Congenital Heart Surgery

Timing of Balloon Atrial Septostomy in Patients with d-TGA and Association with Birth Location and Patient Outcomes. Thomas C, Yu S, Lowery R, Zampi JD. Pediatr Cardiol. 2023 Aug;44(6):1333-1341. doi: 10.1007/s00246-022-03079-5. Epub 2022 Dec 24.PMID: 36565310 Take Home Points:  Patients with d-TGA transported from an outside institution had a longer median time time from birth to the BAS as compared to those born the authors’ tertiary care center (4.0 hours vs. 14.1 hours). Patients delivered at outside institutions had lower minimum PaO2 and greater frequency of inotropic support, as compared to those born at the authors’ tertiary care center. Neither time to BAS nor location of birth was associated with age at time of arterial switch operation, however a longer time from birth to BAS was associated with longer ICU LOS and total hospital LOS. Commentary from Dr. Arash Salavitabar (Columbus, OH, USA), section editor of Congenital Heart Disease Interventions Journal Watch: Commentary:  The authors report on a retrospective study aimed at evaluating their single-center experience with outcomes in infants with d-transposition of the great arteries (d-TGA) as they relate to timing of balloon atrial septostomy (BAS) and institution of birth. The primary outcome was time from birth to BAS and secondary outcomes included mortality, hospital and ICU length of stay, duration of mechanical ventilation, evidence of pulmonary hypertension, and neurologic abnormalities. Of 217 patients with d-TGA physiology, 96 (44%) patients required emergent or urgent BAS, 69 (72%) of which were prenatally diagnosed. Patients born at outside institutions had a median travel distance of 99 miles (IQR 56, 115) and transport time of 10.3 hours (6.8, 12.0), with a shorter transport time for prenatally diagnosed patients vs. postnatally diagnosed patients born at outside institutions (6.4h vs. 10.7h). The median time from birth to BAS was 4.0 hours in those born at the authors’ center and 14.1 hours when transported from an outside institution. Importantly, patients delivered at outside institutions had lower minimum PaO2 (28.2 mmHg vs. 31.9 mmHg, p=0.02) and greater frequency of inotropic support (48.3% vs. 7.5%, p <0.0001) as compared to those born at the authors’ center. Neither time to BAS nor location of birth was associated with age at time of arterial switch operation, however a longer time from birth to BAS was associated with longer ICU LOS and total hospital LOS. There were no differences in frequency of seizures or abnormal neuroimaging based on time to BAS or birth location. The authors stressed the importance of patients with prenatally diagnosed d-TGA being delivered at a tertiary care center with access to interventional cardiology services to decrease the time to urgent or emergent BAS. Based on this data, this is certainly an important conclusion that has implications on both patient stability and oxygenation, as well as healthcare resource utilization. As the authors admit, more data is needed on neurological sequelae of late BAS in these patients, however the above data has been previously correlated with adverse neurologic outcomes, stressing its significance.

Aortic valve neocuspidization using the Ozaki technique: A meta-analysis of reconstructed patient-level data  Konstantinos S. Mylonas, MD a,#, Panagiotis T. Tasoudis, MS b,#, Dionysios Pavlopoulos, MD, PhD a, Meletios Kanakis, MD, PhD c, George T. Stavridis, MD, FETCS a, and Dimitrios V. Avgerinos, MD, PhD, FACS, FACC Take Home Points: The midterm outcomes of the Ozaki procedure are excellent in terms of hemodynamics, survival, and freedom from reoperation. Commentary from Dr. Shuhua Luo (Chengdu, Sichuan, China), section editor of Congenital Cardiac Surgery Journal Watch: Summary: A total of 22 studies reporting on 1,891 patients that underwent Ozaki reconstruction were included in this meta-analysis. Mean age at the time of surgery was 43.2 ± 24.5 years (65 ± 12.3 years for adult patients and 12.3 ± 3.8 years for pediatric patients). The most common indication was aortic stenosis (46.4%, 95% CI 34.1-58.6). At latest follow-up, peak gradient was 15.7 ± 7.4 mm Hg and only 0.25% (95% CI 0-2.3) had moderate aortic insufficiency. In-hospital mortality was 0.7% (95% CI 0.1-1.7). Late mortality was 1.9% during a mean follow-up of 38.1 ± 23.8 months. One-year, 3-year, and 5-year freedom from reoperation rates were 98.0 %, 97.0 % and 96.5%, respectively. More than half of the reoperations were due to infective endocarditis (51.5%, 95% CI 18.3-84.0). Comment:      Ozaki’s procedure is a promising method to independently reconstruct the three leaflets of the aortic valve using autologous pericardium treated with glutaraldehyde. Most current data on this technique arise from single-center experiences, which are based on small patient populations. The present systematic review includes a total of 1,891 patients making it the largest and most comprehensive data synthesis to date on the Ozaki procedure. Although the results showed promising midterm outcomes of the Ozaki procedure, several questions remained to be answered in the future study. (1) Aortic valve disease can be complicated at multiple levels such as left ventricular outflow tract obstruction(LVOT), aortic stenosis or regurgitation, aortic annulus stenosis or dilatation, and ascending aortic dilatation. In addition, many underlying causes can lead to aortic valve disease including infective endocarditis, rheumatic disease, or auto-immune pathologies. It is unknown whether the Ozaki procedure is a reasonable choice for these different clinical scenarios, as the current meta-analysis showed the majority of patients who underwent the Ozaki procedure had aortic stenosis, and the concomitant procedures such as LVOT muscle resection, aortic annular enlargement, or replacement of ascending aorta were not analyzed in the current study. (2) The results of the Ozaki procedure in pediatric patients were particularly interesting. The mean age and body weight of pediatric patients was 12.3 ± 3.8 years and 42.1 ± 17.9 kg respectively, indicating that the size of patients was near to adult when undergoing Ozaki procedure. The aortic valve disease in young infants or small children posed a significant clinical challenge as the machinal prosthesis replacement was not usually an option due to the small size of the aortic annulus, and the Ross procedure was associated with high mortality. It will be interesting to look at the outcome of the Ozaki procedure in young children. (3) Although the original Ozaki procedure included the tricuspid leaflets reconstruction, some patients only need a single-cusp reconstruction using the Ozaki principle. It remains to know that the percentage of patients who only received a single-cusp reconstruction in the current meta-analysis, may have the potential impact on the midterm outcome.      In summary, the Ozaki procedure is an attractive alternative for aortic valve disease in patients. We are looking forward to more data shed light on the surgical decision-making in various clinical scenarios, including patients’ size, individual detailed anatomy, and underlying causes.

Preliminary Results With a Novel Expanded Polytetrafluoroethylene-based Pulmonary Valved Conduit Christopher W. Baird, Mariana Chavez, Carl L. Backer, Mark E. Galantowicz, and Pedro J. Del Nido Take Home Points: A novel expanded polytetrafluoroethylene-based pulmonary valved conduit was tested with a prospective, multicenter, single-arm study. This preliminary evaluation suggested promising valve function with no thromboembolic, no infectious complications, no valve-related re-interventions, no valve-related adverse events or unexpected findings. Improved right ventricular volumes and encouraging hemodynamic performance were proved by CMRI and through current follow-up. Commentary from Dr. Masamichi Ono (Munich, Germany), section editor of Congenital Heart Surgery Journal Watch:  Summary: This is a prospective, multicenter, single-arm study from centers in the USA, reporting results after the implantation of a novel expanded polytetrafluoroethylene (ePTFE)-based pulmonary valved conduit. A total of 17 patients (52.9% male; median age, 12 years; range, 6 to 17 years) were included. One patient had left coronary compression after the implantation of this conduit and was replaced with a biological valved conduit in the operating room. There has been no mortality and 100% freedom from device-related re-interventions. During follow-up, no evidence of worsening valvular insufficiency/stenosis was observed. No patient developed thrombus, calcification, or endocarditis. Six-month postoperative cardiac magnetic resonance imaging in 11 patients suggests favorable right ventricular remodeling with no significant change in ejection fraction. Comment: Reconstruction of the RVOT and replacement of pulmonary valves in pediatric and adolescent patients with congenital heart diseases have been ongoing challenges, especially given the lack of a durable prosthesis that is adjustable to the patient’s growth needs and is free of infection risks. Current RVOT reconstruction is limited to cryopreserved homografts, decellularized homografts, bioprosthetic valved conduits, bioprosthetic valves, bovine jugular conduits, and hand-sutured ePTFE grafts. All current conduits pose fundamental limitations. ePTFE has good biocompatibility, and its microporous structure impedes cellular penetration and subsequent calcification. Yamagishi, et al reported that hand-made ePTFE valve conduits have shown improved durability and significantly reduced infection rates. This novel ePTFE-based valved conduit was industry-manufactured. The study demonstrated the safety and feasibility of this conduit. In this study, only a 20 mm-sized valved conduit was tested. Further studies with other (smaller or larger) sizes are mandatory to prove the usefulness of this conduit. Long-term durability should be also proved in further follow-up studies. Yamagishi et al developed an ePTFE valved conduit with a fan-shaped configuration consisting of a 0.1-mm-thick ePTFE membrane and bulging sinuses to improve valve mobility. They demonstrated the excellent results of the freedom from reoperation was 98.8% at 5 years and 93.8% at 10 years with a median follow-up period of 4.9 years. This conduit has been developed based on their experiences. Therefore, the preliminary results with a novel ePTFE pulmonary valve conduit are quite promising. Continued surveillance of these patients will be performed to assess the performance of this valve over time, and further studies with small-sized conduits are desirable.

Aortic root translocation and en bloc rotation of the outflow tracts surgery for complex forms oftransposition of the great arteries and double outlet right ventricle: A multicenter study. Stoica S, Kreuzer M, Dorobantu DM, Kostolny M, Nosal M, Hosseinpour AR, Martinez FL,Generali T, Hasan A, Mair R, Hazekamp M.J Thorac Cardiovasc Surg. 2022 Nov;164(5):1249-1260. doi: 10.1016/j.jtcvs.2022.05.047. Epub 2022 Jul 20.PMID: 36028361 Commentary from Dr. Yasuhiro Kotani (Okayama, Japan), chief section editor of CongenitalHeart Surgery Journal Watch: Take Home Points: Both aortic root translocation and en bloc rotation resulted in excellent long-term survival. More right ventricular outflow tract/conduit reintervention was required in aortic root translocation Patients with en bloc rotation had more aortic valve regurgitation during the follow-up Summary This is a retrospective, multicentric, observational study from European countries, reporting results after the aortic root translocation and en bloc rotation of the outflow tract procedures. A total of 70 patients (62.9% male; median age, 1 year; range 4 days to 12.4 years) were included: n=43 in the aortic root translocation group and N=27 in the en bloc rotation group. Those in the aortic root translocation group were older and more likely to have had previous procedures (P<.0001), but cardiac anatomy was similar in both groups. Aortic root translocation and en bloc rotation early mortality (30 days) was similar (4.7%vs 3.7%, P=0.08). Late survival and freedom from any cardiac reintervention were 92.7% and 16.9% at 15 years overall,respectively. Freedom from right ventricular outflow tract/conduit reintervention was better in the en bloc rotation group than in the aortic root translocation group (100%vs 24.5%, P=0.0003), but more patients in the en bloc rotation group had moderate (or worse) aortic valve regurgitation during follow-up (16% vs 2.6%, P=0.07). Comment This multicenter study included 70 patients, relatively large number of patients, who had repair for complex transposition of the great arteries. The study demonstrated that both aortic root translocation and en bloc rotation are valuable surgical options for the treatment of complex transposition of the great arteries and double outlet right ventricle based on the excellent survival of 92.7% at 15 years. However, greater than 80% of patients required any type of cardiac reintervention. In the en bloc rotation group, there was better freedom from right ventricular outflow tract reinterventions, but a higher probability of aortic valve regurgitation. This is reasonable as native pulmonaly valve was kept in 67% in patients having en bloc rotation, while RV-PA conduit was used in 58% of patients with aortic root translocation. In terms of aortic valve regurgitation, the authors mentioned that oversizing the VSD patch in patients with early experience of en bloc rotation is the reason which were overcome recently. Another possible reason for aortic valve regurgitation may be distortion of sinus valsalva after coronary reimplantation but no evidence. It may be difficult to conclude which procedure is better compared to another as patients with this disease have a wide variety of anatomies, including pulmonary valve size and coronary pattern. Therefore, surgical choice should be made based on each patient’s anatomy.

Aortic valve repair using geometric ring annuloplasty in pediatric and congenital heart disease patients. Lancaster TS, Romano JC, Si MS, Ohye RG. J Thorac Cardiovasc Surg. 2023 Aug;166(2):294-303. doi: 10.1016/j.jtcvs.2022.10.017. Epub 2022 Oct 19.PMID: 36404145 Take Home Points: Newly developed subannular ring (HAART ring) provided an excellent mid-term outcome. The placement of the ring does not need extensive dissection of the aortic root, hence suitable for patients who previously had Ross or other type of aortic surgery. Commentary from Dr. Yasuhiro Kotani (Okayama, Japan), chief section editor of Congenital Heart Surgery Journal Watch:  Summary: This is a retrospective study from 2 institutions in the U.S., looking at all pediatric and adult congenital patients who underwent aortic valve repair with placement of a geometric annuloplasty ring. The study period was from July 2018 to April 2022 and included 36 patients. The median age was 17.4 years (range, 8-30 years) and 21 patients were younger than age 18 years. The most common primary diagnoses were neoaortic valve insufficiency or neoaortic root dilation, and congenital aortic stenosis with bicuspid or functionally unicuspid aortic valve. Of the 34 patients with procedural success, 31 (91%) had use of additional valve repair techniques and 26 (76%) had an additional concomitant procedure performed. Operative mortality was 0% (0/33), and major complication rate was 6% (2/33). The median follow-up time was 1.9 years (maximum, 3.8 years). The mean grade of aortic insufficiency was significantly reduced after repair, with no change in mean gradients. Freedom from reoperation over the follow-up period was 97% (33/34), and freedom from >3+ recurrent aortic insufficiency was 94% (32/34). A geometric annuloplasty ring can be used to help achieve consistently successful aortic valve repair with excellent perioperative and follow-up outcomes, even in pediatric and complex congenital heart disease patients. Comment: This is a retrospective study for aortic valve repair with placement of a geometric annuloplasty ring done at Michigan. They used newly developed annuloplasty ring, named Hemispherical Aortic Annuloplasty Reconstructive Technology (HAART) ring. The ring sizes available are from 19 to 25mm. The ring is placed in subannular position. There are 2 types, tricuspid and bicuspid. Implantation technique is not complicated. After measurement of the size, the ring is implanted with the sutures which first involves placement of Cabrol-type horizontal mattress sutures at each commissure to secure the commissural posts of the ring, and then 1 to 4 looping sutures placed along the length of each sinus aspect of the ring. The leaflet coaptation is then assessed and plication may be necessary for the prolapsed leaflet due to the downsizing of the annulus (80% of population in this study). Advantages of this technique are as follows. First, it is less invasive. External annuloplasty needs an extensive dissection of the aortic root, even in the situation of reoperation which pose a risk of coronary injury and bleeding. Second, the technique is reproducible. After placement of the ring, the plication may be needed but this is relatively simple technique compared to complex leaflet plasty. Finally, this study showed no heart block after this procedure and no circumferential sutures may be the reason. The initial results are promising but need more long-term follow-up.

Predictive factors contributing to prolonged recovery in patients after Fontan operation. Song Y, Wang L, Zhang M, Chen X, Pang Y, Liu J, Xu Z. BMC Pediatr. 2022 Aug 24;22(1):501. doi: 10.1186/s12887-022-03537-2. PMID: 36002809  Take Home Points: Ventilation time, higher inotropic score in postoperative day, and pulmonary hypertension treatment and higher fluid resuscitation within two days were independent risk factors and have a high predictability for Fontan prolonged recovery. Commentary from Dr. Masamichi Ono (Munich, Germany), section editor of Congenital Heart Surgery Journal Watch: Summary: The single center retrospective study included 282 patients who underwent Fontan surgery between 2015 and 2018. Patients with >75%ile cardiac intensive care unit stay in prolonged recovery group (n=70). The others were assigned to the standard recovery group (n=212). Pre- and intra-operative data showed a higher incidence of heterotaxy syndrome, longer cardiopulmonary bypass, and aortic cross-clamp time in the prolonged recovery group. Postoperative information analysis displayed that ventilation time, oxygen index after extubation, hemodynamic data, inotropic score (IS), drainage volume, volume resuscitation, pulmonary hypertension (PH) treatment, and surgical re-intervention were significantly different between the two groups. Higher IS postoperatively, and PH treatment and higher fluid resuscitation within two days were independent predictive factors for prolonged recovery in our multivariate model. C-statistic model showed a high predictive ability in prolonged recovery by using the three factors. Comment: This relatively large retrospective study for Fontan surgery was done at Shanghai Children’s Medical Center, School of Medicine, Shanghai Jiao Tong University. In this study, they aim to make a deep and whole exploration of the predictive factors, including the demographic, perioperative hemodynamic, important procedure and interventions that may contribute to the prolonged recovery on patients after Fontan operation. They excluded patients who experienced Fontan takedown (n=10) or didn’t survival to discharge (n=15). Patients with > 75%ile cardiac intensive care unit (CICU) stay were defined as prolonged postoperative recovery, and the others were assigned to standard recovery. As result, Heterotaxy syndrome (p=0.042) was only the preoperative factor associated with prolonged ICU recovery. As for intraoperative factors, cardiopulmonary bypass time (P = 0.004) and aortic cross-clamp time (P < 0.001) were factors related with the patient’s recovery. As for postoperative factors, prolonged recovery group had longer mechanical ventilation time, and 62.8% patients need more than 24hr mechanical ventilation. The oxygen index (P/F ratio) after extubation was significantly higher in patients with a standard recovery. mPAP (P = 0.002) and Pp/Ps (P < 0.001) were higher and mABP (P < 0.001) was lower in POD 0 in the prolonged recovery group when compared with the standard recovery group. Thus, 81.4% patients with a prolonged recovery needed a PH treatment within two days, which only performed in 59.9% patients in the standard recovery group (P = 0.034). Prolonged recovery patients needed a longer time of chest drainage (P < 0.001), and the volume of drainage (P < 0.001) and resuscitation (P < 0.001) within two days were more than standard recovery group. Otherwise, IS in POD 0 (P < 0.001), POD 1 (P < 0.001) and POD 2 (P < 0.001) was significantly higher in patients with a prolonged recovery. The incidence of hypoxemia (P < 0.001), low cardiac output syndrome (P < 0.001), liver disfunction (P < 0.001) and renal disfunction (P < 0.001) were significantly higher in the prolonged recovery group. Multivariable logistic regression model demonstrated that higher IS in POD 0 (OR 1.031, 95% CI 1.001, 1.061), PH treatment (OR 2.627, 95% CI 1.053, 6.553), higher fluid resuscitation (OR 1.015, 95% CI 1.009, 1.021) within two days after the surgery, mechanical ventilation time (OR 1.051, 95% CI 1.032, 1.071), and fluid resuscitation within two days (OR 1.012, 95% CI 1.006, 1.019) were independent factors in predicting the recovery of Fontan recovery. This study is well written with good statistical methods. The results were not quite new. However, they clearly demonstrated that postoperative inotropic support, high pulmonary artery pressure, and higher fluid resuscitation were the risks for prolonged Fontan recovery. As for the ventilation time, we already reported the importance of early extubation despite the hemodynamic (Ono, et al. ICVTS 2019, Georgiev, et al. Cardiol Young 2017). We clearly demonstrated the data that early extubation is universally applicable following the Fontan operation and improves postoperative hemodynamics and recovery regardless of the initial hemodynamic status. This might improve the postoperative recovery in their institute.

Guest Editorial by Sasha Agati, MD. Co-Chair of Congenital Heart Academy   Dear Colleagues,   First, I would like to thank Prof. Varun Aggarwal for the invitation and the opportunity to share my experience and point of view, at the same time I very proud to announce a stable collaboration between “The Congenital Heart International Professionals (CHiP) Network” and “Congenital Heart Academy”. The “editorial opinion”, I personally think, that represents the best option for express and stimulate comments and pro vs con: so today I am feeling very lucky. In the last 3 years, it was so difficult upgrade our “experience” based knowledge due to pandemic status, but that was time to review our data, our activities, our outcomes. We experienced with the growth of web based educational platform like Congenital Heart Academy, that there was the amazing opportunity to receive “one-way” information and why not education too. Yes, one-way because web-based seminars make the teacher/speaker in a real “honorary” position and all information came as a “Review” done every time from different super-experts. For sure this creates a new world, a new way of perception of science and probably a new attitude for clinical practices.   During these years, several webinars were focused on “Transposition of Great Arteries” and we have learnt a lot. For example, the extraordinary testimony about the coronary translocation story done by Prof. Marcelo Jatene, very difficult to know about this from other sources. But, what I think, make more rumors on the web was the position about Rashkind procedure and Lecompte maneuver in the field Transposition of Great Arteries physiology and long-term results.   In this editorial, I will comment about Rashkind using a selection of web-limited world strategies (see Gil Wernowky series YouTube Congenital Heart Academy Channel: https://www.youtube.com/watch?v=lCTUp9nPBgU&t=3434s). Most still believe that Rashkind is a life-saving procedure, but in the current practices it is increasingly used as a “routine” procedure to stabilize the patient (rather than for desaturation patients with Transposition of Great Arteries – cardiologist view – Rashkind for all!!!) in most of the cases done directly in cardiac intensive care bed. Percutaneous balloon atrial septostomy (PBAS) has historically been applied in neonates with transposition of the great arteries only with inadequate mixing at the atrial level in order to enlarge or create an interatrial septal defect. Many reports have emphasized that Rashkind procedure is not without complications. Cogo et all [1] demonstrated that Rashkind procedure should be used for those patients with significant hypoxemia, hemodynamic instability, or both. After procedure, removal of catheter from the vein both femoral or umbilical and again bleeding control can be obtained only by clot formation [3]. Now, in transposition of great arteries 80 to 90% of cardiac output is “recirculating” venous return so if a clot form anywhere in the baby by central line or peripherally inserted venous catheter this will easily cross the aortic valve causing stroke (2% to 5% reported incidence). Rashkind procedure was not associated with an increased risk of necrotic enterocolitis but was associated with twice the risk of stroke [2-3].   The selective use of Rashkind procedure for those patients with significant hypoxemia, hemodynamic instability or both still represents the correct strategy for the optimization of underdevelopment outcome of neonates with Transposition of Great Arteries [4]   On the other side, prostaglandin infusion “loved” by most of surgeons make the patient so “stable” than and you don’t need to run in theater, but, causing heart failure as basic complication and characterized by wide pulse pressure accompanied by tachycardia. Apnea occupies the first adverse effect with intubation necessity [5].   Both strategies are not “free of charge”, in some place Rashkind is done under general anesthesia (less than before) and prostaglandin infusion requires stable vascular access most likely a central or a peripherally inserted central line, caffeine and what about inflammation. So, “do what you do best “…. but let think about: if only a small percentage of “real symptomatic” newborn need Rashkind procedure and few of them require continuous infusion of prostaglandin are we able to “switch” the child without additional procedure? Let’s think about this and wait for your comment.   References: 1. Balloon atrial septostomy and pre-operative brain injury in neonates with trasposition of the great arteries: a systemic review and a meta-analysis. A. Polito, Z. Ricci, T. Fragasso, P. E. Cogo. Cariology in the Young (2012) 2. Analysis of 8681 neonates with transposition of great arteries: outcomes with and without Rashkind balloon atrial septostomy. D. Mukherjee, M. Lindsay, Y. Zhang, T. Lardaro, H. Osen, D. C. Chang, J. I. Brenner, F. Abdullah. Cardiologi in the Young (2010). 3. Thrombus formation in the heart following balloon atrial septostomy in transposition of great arteries. S. Talwar, S. Ramakrishnan, P. Gharde, S. K. Choudhary. IJTCS (2022) 4. Preoperative brain injury in transposition of the great arteries is associated with oxygenation and time to surgery, not balloon atrial septostomy. Petit CJ, Rome JJ, Wernovsky G et al. Circulation (2009) 5. Congenital Heart disease: The State-of-the-Art on Its Pharmacological Therapeutics. Carlos Daniel Varela-Chinchilla, D. Edith Sánchez-Mejía 1 and Plinio A. Trinidad-Calderón. JCDD (2022)

Outcomes of aortic valve repair in children resulting in bicuspid anatomy: Is there a need for tricuspidization?   Schulz A, Buratto E, Wallace FRO, Fulkoski N, Weintraub RG, Brizard CP, Konstantinov IE.J Thorac Cardiovasc Surg. 2022 Jul;164(1):186-196.e2. doi: 10.1016/j.jtcvs.2022.01.022. Epub 2022 Jan 26.PMID: 35227498   Take Home Points: Outcomes of aortic valve repair in small children are excellent. Tricuspidization of bicuspid valve is inferior compared to bicuspid repair in terms of chance of reoperation.     Commentary from Dr. Yasuhiro Kotani (Okayama, Japan), chief section editor of Congenital Heart Surgery Journal Watch:      Summary:   Single center retrospective study included 127 patients who underwent aortic valve repair with creation/preservation of bicuspid aortic valve anatomy or tricuspidization of bicuspid valves between 1980 and 2016. Median age was 0.73 years (interquartile range (IQR, 0.1-8.9), and median weight was 8.15 kg (IQR, 3.9-31.7). The cohort included 29 neonates (22.8%), 36 infants (28.3%), and 62 children (48.8%). Repair was performed without a patch in 69 patients (54.3%). Survival at 10 years was 94.8%. Freedom from aortic valve reoperation at 5 and 10 years was 79.9% and 65.6%, respectively. Re-repair was undertaken in 53.7% (22/41). Freedom from aortic valve replacement at 5 and 10 years was 90.3% and 75.8%, respectively. Risk factors for reoperation were age less than 1 year, unicuspid valve, and the presence of Shone complex and concomitant aortic arch repair. There were 107 patients (107/127, 84.25%) with preoperative bicuspid aortic valve morphology that was preserved. They were compared with a separate cohort of 44 patients who underwent tricuspidization of bicuspid aortic valve during the same period. There was no difference in survival or freedom from aortic valve reoperation. However, freedom from aortic valve replacement was lower after tricuspidization with 49.7% versus 75.8% after 10 years (P=0.0118).   Comment:   This is a retrospective study for aortic valve repair done at Royal Children’s Hospital, Melbourne. They have a large experience of surgical aortic valve for patients with aortic stenosis. Their outcomes are outstanding with freedom from aortic valve replacement after 10 years is 75.8 % even a half of their patients are neonates and infants. This study showed age less than 1 year, unicuspid valve, and concomitant arch procedure was a risk for reoperation. More importantly, they identified bicuspid repair was superior to tricuspidization regarding with a chance of reoperation. This makes sense when looking at figures and video in the manuscript that their techniques for aortic valve repair are excellent. Term of “aortic valve repair” is difficult to interpret as different surgeons use different techniques. So, it is often tricky to only look at numbers of reoperation rate, etc. in the paper. Excellence of this manuscript is that they have beautiful figures to show exactly how they do for aortic valve repair (Figure 2&3). Also, they provide a video that every surgeon can understand their technique visually, not by the description. When looking at these figures and video, you can appreciate that their techniques of aortic valve repair support their excellent results that not all surgeons can reproduce same outcomes. Hence, I understand that their institutional preference to treat patients with aortic stenosis is surgical aortic valve repair.    

Risk factors for postoperative pulmonary venous obstruction after surgical repair of total anomalous pulmonary venous connection: a systemic review and meta-analysis Han Zhang , Guocheng Shi and Huiwen Chen. Interact Cardiovasc Thorac Surg. 2022 Aug; 35(2): ivac162. PMID: 35713512   Take Home Points: 1) A total of five risk factors were identified to be associated with pulmonary vein obstruction(PVO) after surgical repair of total anomalous pulmonary venous connection(TAPVC), including: preoperative PVO, conventional operative procedure(compare with a sutureless procedure), a mixed type TAPVC, and longer cardiopulmonary bypass and aortic cross-clamp time.     Commentary from Dr. Shuhua Luo (Chengdu, Sichuan, China), section editor of Congenital Cardiac Surgery Journal Watch: Summary: This meta-analysis included 2,385 individuals in 16 retrospective studies undergoing surgical repair of TAPVC. Five risk factors were significantly associated with postoperative PVO. Patients with preoperative PVO [odds ratio (OR)=5.27, 95% confidence interval (CI) = (2.75, 10.11), P < 0.01],  a mixed type TAPVC[OR = 3.78, 95% CI=(1.08, 13.18), P = 0.04], longer cardiopulmonary bypass time [hazard ratio (HR)=1.01, 95% CI=(1.01, 1.02), P < 0.00001] and aortic cross-clamp time [HR = 1.01, 95% CI=(1.01, 1.02), P < 0.01] were significantly associated with postoperative PVO.  Compared with a sutureless procedure, the conventional operative procedure was associated with postoperative PVO [OR = 1.80, 95% CI=(1.20, 2.71), P < 0.01]. On the contrary, the heterotaxy was not identified as a risk factor for postoperative PVO. Comment: PVO is one of the most important complication of TAPVC repair, occurring in 15-20% of the survivors, has been associated with increased morbidity and mortality. Therefore, identifying risk factors for postoperative obstruction is essential for appropriate counseling and clinical care. The authors should be congratulated for providing a more general estimate of risk factors for postoperative PVS. However, more questions may be raised. The author stated that the postoperative PVO definition was clear in the included studies, however, the definitions in the individual studies were various, including Doppler velocity by echocardiography(1), pulmonary vein reintervetion(2), or pulmonary vein dimension based on the angiography(3). Furthermore, PVS can also present with considerable variability in patients as it can occur in one or more of the pulmonary veins and can be bilateral or limited to unilateral disease. Lesions in the pulmonary veins can be discrete at the venoatrial junction or can be diffuse, extending along the length of the intraparenchymal, and the velocity at which the disease progresses can range from indolent to aggressive and relentless. This reflects the current challenges in PVS diagnosis, which need to be addressed in the future study. The authors also reported that heterotaxy was not strongly associated with the postoperative PVO of TAPVC. However, this finding should be interpreted with caution. The heterogeneity of the data was high as only three studies were included in the meta-analysis of heterotaxy. High postoperative mortality may further impact the occurrence of postoperative PVO in the follow-up period. The etiology of PVS has remained elusive and likely has multifactorial factors including genetic, developmental, and hemodynamic contributions. There is an unmet need to provide better evidence for incremental knowledge gains, and improved outcomes for children diagnosed with PVS.  Reference 1) White BR, Ho DY, Faerber JA, Katcoff H, Glatz AC, Mascio CE, et al. Repair of Total Anomalous Pulmonary Venous Connection: Risk Factors for Postoperative Obstruction. Ann Thorac Surg. 2019 Jul;108(1):122–9. 2) Husain SA, Maldonado E, Rasch D, Michalek J, Taylor R, Curzon C, et al. Total Anomalous Pulmonary Venous Connection: Factors Associated With Mortality and Recurrent Pulmonary Venous Obstruction. Ann Thorac Surg. 2012 Sep;94(3):825–32. 3) Seale AN, Uemura H, Webber SA, Partridge J, Roughton M, Ho SY, et al. Total Anomalous Pulmonary Venous Connection.

Pulmonary vein stenosis: Anatomic considerations, surgical management, and outcomes Eric N. Feins, Christina Ireland, Kimberlee Gauvreau, Mariana Ch_avez, Ryan Callahan, Kathy J. Jenkins, and Christopher W. Baird   Take Home Points: Anatomic focused repair strategy provides better survival after pulmonary vein stenosis repair. Characterization of PVS by multi-modalities as well as multidisciplinary team approach are important in the treatment of PVS. Commentary from Dr. Yasuhiro Kotani (Okayama, Japan), chief section editor of Congenital Heart Surgery Journal Watch:   Summary: Single center retrospective study included 174 patients who underwent pulmonary vein stenosis (PVS) repair between 2007 and 2019. Fifty-nine patients (34%) had primary pulmonary vein stenosis. Median age was 9 months (interquartile range, 5-27) and weight was 6.5 kg (4.7-10.2). Surgical techniques evolved and included ostial resection, unroofing, reimplantation, sutureless, modified sutureless, and a newer anatomically focused approach of pulmonary vein stenosis resection with lateralization or patch enlargement of the pulmonary vein–left atrium connection. Twenty-three patients (13%) required reoperation. Cumulative 2-year incidence of postoperative transcatheter intervention was 64%. One-, 2-, and 5-year survivals were 71.2%, 66.8%, and 60.6%, respectively. There was no association between surgery type and reoperation rate or transcatheter intervention but the anatomically focused repair was associated with decreased mortality on univariate (hazard ratio, 0.38, P=0.042) and multivariable analyses (hazard ratio, 0.19, P=0.014). Antiproliferative chemotherapy was also associated with decreased mortality (hazard ratio, 0.47, P=0.026).   Comment: This relatively large retrospective study for PVS repair was done at Boston Children’s Hospital. 5-year survival was not satisfactory but acceptable about 60%, indicating the difficulty of the treatment of PVS even in the world renown center in the recent era. Cumulative 2-year incidence of postoperative catheter intervention was 64%.   Boston group has a multidisciplinary team that dedicates diagnosis and treatment of PVS which is outstanding approach as etiology of PVS is multifactorial that needs a tailor made management. They introduced a new technique, i.e., anatomically focused repair to shorten and straighten the course PV course from the lung parenchyma to the left atrium. They showed significantly better survival using this technique compared to conventional repair technique that included sutureless technique. Although etiology of PVS is multifactorial and indication of this new technique is somewhat difficult to understand, they emphasized the importance of characterization of PVS by computed tomography or magnetic resonance imaging. As they used this new technique more recently, it is not clear that improved outcome achieved in the recent era is either by new technique or the result of team approach. Also, because it is not shown how many patients who had this new technique received an adjuvant chemotherapy. Together with the fact of relatively short-term follow up in the current study, long-term follow up will answer the impact of this new technique on the treatment of this unique entity.   

Propensity Score Matched Analysis of Cleft Closure in Complete Atrioventricular Septal Defect Repair Edward Buratto, MBBS, PhD, Adrienne Lui, MD, Thomas Hu, MD, Phillip S. Naimo, MD, PhD, Yaroslav Ivanov, MD, PhD, Yves d’Udekem, MD, PhD, Christian P. Brizard, MD, and Igor E. Konstantinov, MD, PhD   Take Home Points: Reoperation on left atrioventricular valve (LAVV) is not negligible after repair of complete atrioventricular septal defect (cAVSD). Whether or not to close the LAVV cleft does not impact on need for reoperation. Commentary from Dr. Yasuhiro Kotani (Okayama, Japan), chief section editor of Congenital Heart Surgery Journal Watch:   Summary: Single center retrospective study included 455 patients who underwent cAVSD repair between 1990 and 2019. To determine the effect of cleft closure, propensity score matching was performed on risk factors for reoperation after cAVSD repair. Median age was 3.6 months (mean, 9.6 ± 20.4 months) and median weight was 4.3 kg (mean, 4.7 ± 4.3 kg). Early mortality was 2.9% (13 of 455), and survival was 89.8% ± 1.9% at 20 years. Early reoperation was a risk factor for mortality (P=.004). Freedom from LAVV reoperation was 74.1% ± 4.0% at 20 years. Preoperative severe LAVV regurgitation (P<.001) and early postoperative moderate or greater LAVV regurgitation (P=.007) were risk factors for reoperation. Propensity score matching included 106 pairs. There were no differences in long-term survival (P=.71) or reoperation (P=.26) between the 2 groups.   Comment: This retrospective study was done at Royal Children’s Hospital, Melbourne. Outcomes were acceptable, including about 75% of patients remain free from LAVV reoperation 20 years after initial cAVSD repair. The study included the propensity score matching to see the impact of the closure of the LAVV, which showed no difference in need of reoperation. There was a significantly higher rate of preoperative moderate or greater LAVVR in patients who had a cleft closure (25.6% vs. 15.9%, p=.03). In contrast, patients without cleft closure had more deficient lateral leaflet (7.5% vs. 3.2%, p=.50), which infers those with significant LAVVR had smaller lateral leaflet as etiology of regurgitation.   The results in this study raised 2 questions. Firstly, it is unclear that cleft closure was cause or effect. We can speculate that cleft closure was performed in patients with preoperative significant LAVVR as most of surgeons would not leave the cleft unrepaired. Therefore, we could think that cleft closure is the result. Secondary, 2 groups were unbalanced in terms of era of operation (p<.001), but there were no differences in survival and freedom from LAVV reoperation. This study showed that recent era of surgery was protective in terms of risk for reoperation. There were more patients with cleft closed in the most recent era. These facts make us think that surgical strategy to close the cleft may influence the outcome in this study. Taken together, prospective study to see the effect of LAVV closure is necessary. Clinically, it is challenging to repair the LAVV when patients have significant LAVVR with small lateral leaflet, as closure of the cleft may make LAVVR worse.   

Survival after Intervention for Single-Ventricle Heart Disease Over 15 Years at a Single Institution Alexander Zhu, MD, James M. Meza, MD, MSc, Neel K. Prabhu, BS, Andrew W. McCrary, MD, MSc, Veerajalandhar Allareddy, MD, Joseph W. Turek, MD, PhD, and Nicholas D. Andersen, MD   Take Home Points: In children with single-ventricle (SV) heart disease, survival differences in outcomes might be related to fundamental morphologic differences, patient characteristics, or procedural pathways. This study showed that survival differences in patients with SV heart disease were related primarily to underlying cardiac anatomy, patient characteristics, and procedural complexity. Left ventricular dominance, more recent intervention, and attainment of a 2-ventricle circulation were associated with improved survival. Chromosomal/syndromic abnormality, lower weight, hybrid Norwood procedure, non-left ventricular dominance, and earlier year of operation were risk factors for death. Commentary from Dr. Masamichi Ono (Munich, Germany), section editor of Congenital Heart Surgery Journal Watch   Summary: Single center retrospective study included 381 patients among 477 patients with functional single ventricle who underwent cardiac surgical interventions between 2005 and 2020. Patients who were born before 2005, or underwent initial palliation at another hospital, were excluded from the study.   Patients were divided into 8 diagnostic groups assigned at birth: aortic atresia or aortic stenosis (AA/AS), double inlet left ventricle (DILV), double outlet right ventricle (DORV), hypoplastic left heart syndrome (HLHS), pulmonary atresia with intact ventricular septum (PA/IVS), tricuspid atresia, unbalanced complete atrioventricular canal (uCAVC), and other. Patients were also divided into 11 procedural categories based on the initial interventional procedure, including aortic arch repair/pulmonary artery banding (Arch/PAB), biventricular repair, ductal stent, heart transplant, hybrid Norwood procedure, Norwood procedure, pulmonary artery banding (PAB), systemic-pulmonary shunt (shunt), total anomalous pulmonary venous return repair (TAPVR), none, and other.   Survival stratified by fundamental diagnosis demonstrated that patients with DILV experienced the best survival (10-year: 89% ± 7%), and patients with HLHS experienced the worst survival (10-year: 55% ± 5%). The remaining 10-year survival rates are listed in increasing order: AA/AS (55% ± 11%), uCAVC (61% ± 7%), DORV (69% ± 8%), tricuspid atresia (76% ± 11%), other (82% ± 12%), and PA/IVS (85% ± 6%). Survival rates of index procedures are listed in increasing order: TAPVR (33% ± 18%), other (33% ± 19%), hybrid Norwood (2-year: 39% ± 15%), primary heart transplant (5-year: 50% ± 18%), Norwood (59% ± 4%), shunt (73% ± 5%), Arch/PAB (83% ± 11%), none (85% ± 10%), primary biventricular repair (86% ± 7%), PAB (95% ± 5%), and ductal stent (100% ± 0%). SV patients with LV dominance had higher survival compared with patients with right ventricular (RV) dominance (10-year: 78% ± 5% vs 58% ± 4%, P = .002). Patients with balanced ventricles had a survival rate intermediate between LV-dominant and RV-dominant patients (10-year: 68% ± 10%). Overall survival of patients who were able to achieve a biventricular circulation was superior to patients who remained with SV physiology (10-year: 87% ± 5% vs 63% ± 3%, P = .04). Patients on the SV pathway had similar survival compared with those who received a heart transplant during the study period (10-year: 59% ± 11%). Era analysis revealed that birth in the second half of the study period was associated with improved survival (8-year survival: 60% ± 4% from 2005-2012 vs 74% ± 3% from 2013-2020, P =.04). As for risk factors for deaths, chromosomal/syndromic abnormality, lower weight at the stage 1 procedure, and hybrid Norwood procedure were risk factors for death during the early phase. Conversely, later year of operation and LV dominance were protective factors.   Comment: This retrospective study was done at Duke University Medical Center and provides a holistic overview of survival after intervention for SV heart disease after birth over a 15-year period at a single institution. The reported outcomes are very informative. Key observations included significant differences in survival based on fundamental cardiac morphology, index procedure, as well as other non-modifiable patient characteristics. These factors are not new; the factors identified in this study were reported in the previous studies. Ventricular dominance: It is clear that patients with LV-dominant lesions such as DILV, PAIVS, and tricuspid atresia had better survival compared with patients with RV-dominant lesions. Presence of a morphologic systemic ventricle and mitral valve, less invasive index procedure in neonatal period, and less association of systemic outflow obstruction, might be main reasons for better outcomes.   Lower weight at the stage 1 procedure and chromosomal/syndromic abnormality: they are ubiquitous risk factors that have appeared in most prior studies of single ventricle survival. Low weight at initial palliation was also highly associated with chromosomal/syndromic abnormality, such as heterotaxy, trisomy 21, VACTERL, and others). These risk factors are largely non-modifiable. Our resent study demonstrated that extracardiac anomalies, especially renal anomalies are highly associated to the mortality during the staged reconstruction of single ventricle patients (Vodiskar, et, al. EACTS 2021, under review by The Annals of Thoracic Surgery). In our opinion, these patients with chromosomal/extra-cardiac anomalies had worse outcomes after cavopulmonary shunt procedure. We assume that pulmonary vascular reaction to this procedure might be different from that in patients with normal chromosome, and surgeons may plan to establish a separate strategy for these patients with chromosomal/syndromic abnormality, including timing of cavopulmonary shunt procedure, and indications such as pulmonary artery pressure.   Era effect is also the important issue. Improved outcomes in the later era is an encouraging finding, because of improvements in surgical and postoperative care as well as increased prenatal diagnosis. Ductal stenting is the emerging technique to reduce the mortality of newborns.   Hybrid Norwood procedure: this result is likely institution specific due to our preference for surgical Norwood reconstruction whenever possible and reservation of the hybrid Norwood procedure only in salvage situations when children are not considered candidates for cardiopulmonary bypass. This is also consistent to our results.   Biventricular repair: This study demonstrated 43 patients, born with a presumed SV diagnosis, selected for primary biventricular repair (24 patients) or biventricular conversion after initial SV palliation (19 patients). Despite undergoing potentially high-risk or multi-staged interventions to achieve a 2-ventricle circulation, long- term survival of biventricular repair patients significantly exceeded those who remained with SV physiology. These results suggested us for more efforts to achieve biventricular correction, if patients had borderline hypoplastic heart disease and had a possibility to get biventricular circulation.   Although the results were similar to those in previous studies, this study provided a holistic overview of survival after surgical intervention for single ventricle heart disease after birth over a 15-year period.  

Influence of Antegrade Pulmonary Blood Flow on Outcomes of Superior Cavopulmonary Connection. Dietzman TW, Soria S, DePaolo J, Gillespie MJ, Mascio C, Dori Y, O'Byrne ML, Rome JJ, Glatz AC. Ann Thorac Surg. 2022 Nov;114(5):1771-1777. doi: 10.1016/j.athoracsur.2022.03.011. Epub 2022 Mar 25. PMID: 35341786   Take Home Points: Patients with antegrade pulmonary blood flow (APBF) in the superior cavopulmonary connection (SCPC) was associated with a higher prevalence of longer chest tube duration and need for more interventions during the hospitalization after SCPC, compared to those without. However, patients with APBF had a greater weight gain from SCPC to Fontan completion, and had shorter hospital length of stay after the Fontan procedure. Commentary from Dr. Masamichi Ono (Munich, Germany), section editor of Congenital Heart Surgery Journal Watch Summary: Single center retrospective study included 149 patients among 1003 patients with functional single ventricle who underwent SCPC between 2000 and 2017. Patients who underwent the Damus-Kaye-Stansel procedure, Kawashima procedure, or 1.5-ventricle palliation, were excluded from the study. For a homogeneous cohort to be created, only patients with residual native APBF were included. Of 149 patients, 108 (72.5%) were in APBFL and 41 (27.5%) were in APBFD. They defined a prolonged chest tube duration as chest tube duration 10 days or more.   Median age at SCPC was 168 (IQR: 92-499) days in APBF (-) and 182 (61-1117) days in APBF (+). Median weight at SCPC was 6.3 (IQR: 4.1-13) kg in APBF (-) and 6.75 (IQR: 4.2-10.1) kg in AÜBF (+). Previous PAB was more frequently performed in APBF (-) compared to APBF (+) (27% vs. 7%, p= .01). Preoperative systolic pulmonary artery pressure was significantly higher in APBF (-) compared with APBF (+) (19 vs. 16 mmHg, P= .01). Mean pulmonary artery pressure was similar between the groups (15 vs. 15 mmHg, p= .08). CPB time was significantly longer in APBF (-) compared with APBF (+) (64 vs. 38 Minutes, p< .001). Postoperatively, patients in APBF (+) had a higher prevalence of prolonged chest tube duration (12% vs. 2%, p= .008) and underwent more interventions during the post-SCPC hospitalization (12% vs. 2%, p= .008). There were no differences in post-SCPC intensive care unit or hospital length of stay. SpO2 at discharge was significantly higher in APBF (+) compared to APBF (-) (84 vs. 80%, p< .001). During the study period, 82 patients (76%) in APBF (-) and 22 patients (54%) in APBF (+) underwent Fontan completion. Median age, mean SpO2, and Nakata Index at Fontan are similar between the groups. Mean pulmonary artery pressure was similar between the groups, but Qp:Qs was higher in APBF (+) compared to APBF (-) (0.73 vs. 0.61; P = .02). Patients in APBF (+) had a greater weight gain from SCPC to Fontan (6.7 [1.8-22] kg vs 8.15 [4.4-20.6] kg; P = .012). Although chest tube duration and ICU length of stay were similar between the groups following the Fontan, patients in APBF (+) had a shorter hospital length of stay after Fontan, compared to APBF (-) (7.5 vs 9 days; P = .044).   Comment: This retrospective study was done at Children’s Hospital of Philadelphia, Philadelphia, a world-renown institute.   Outcomes were very interesting.   In reviewing outcomes of single-ventricle patients at CHOP eligible for inclusion of APBF in the SCPC circuit, this study found moderate increased short-term morbidity in the form of increased incidence of prolonged chest tube duration and post-SCPC intervention or surgical procedure in patients with APBF maintained compared with those who had it eliminated at the time of SCPC. In contrast to the increased short-term morbidity seen, long-term benefits observed with maintenance of APBF included a trend toward longer time to Fontan completion and subsequent increased somatic growth between SCPC and Fontan. In addition, a significant difference in hospital LOS at the time of Fontan completion was shown for patients who had APBF maintained. The authors assumed that this shorter hospitalization might suggest that patients with preserved APBF at the time of Fontan may be better prepared to adapt to the transition to Fontan physiology.   Previous studies demonstrated that that there may be an increased risk of prolonged pleural drainage in patients with residual APBF, probably related to a combination of increased pulmonary blood flow, pulsatility, and higher pulmonary arterial pressures. Findings of previous studies have differed concerning APBF effect on post-SCPC hospital LOS. This study supports the finding that maintaining APBF does not significantly increase total hospital LOS despite the higher prevalence of prolonged pleural drainage.   Therefore, the authors supported following strategy. For patients with borderline hemodynamics to be considered for inclusion of APBF at the time of SCPC, a strategy to go forward with inclusion of APBF, with plans for device occlusion of APBF if not tolerated, may allow more patients to receive the potential benefit of residual APBF with relatively mild morbidity risk associated with the possibly needed subsequent intervention.   Our institute has a policy to eliminate all APBF at the time of SCPC, because we think volume unloading is the most important for functional single ventricle, and it is possible in most of the patients, when they are at least 3 months of old and their pulmonary artery pressure is enough low (16 mmHg or less). On the other hand, we experienced patients who needed additional systemic to pulmonary shunt after establishment of BCPS (Euringer, et al. Management of failing bidirectional cavopulmonary shunt: Influence of additional systemic-to-pulmonary-artery shunt with classic Glenn physiology. JTCVS Open 2022). The indication for this procedure included hypoplastic pulmonary arteries, pulmonary venous obstruction (PVO), young age at SCPC, and association of extracardiac/genetic anomalies. We agree with maintaining APBF at the time of SCPC in such patients. However, we find it not reasonable to maintain APBF at the time of SCPC in all patients and to delay the time of Fontan completion by maintaining APBF. Early volume loading by SCPC and swift Fontan completion and farewell to cyanosis remain our policy, to protect the functional single ventricle function for long-term. As for the pulmonary artery size at the time of Fontan, this study showed no statistical difference between the patients with and without APBF. It might be clear that maintaining APBF results in better arterial oxygen saturation and eventual better pulmonary artery size at Fontan. However, it is not clear whether maintaining APBF between SCPC and Fontan bring benefits after the Fontan completion. Maintaining APBF is a certain volume loading of the functional single ventricle. Influence of APBF on long-term systemic ventricular function after the Fontan procedure should be evaluated in further studies. As authors concluded, standardization of pre-SCPC and pre-Fontan imaging and hemodynamic evaluations as well as use of collaborative databases or a prospective multi-institutional study would be key to answering this question and confirming that the statistical findings of their study translate to real-world clinical outcomes.   

Longterm outcomes of primary aortic valve repair for isolated congenital aortic stenosis in children. Wallace F, Buratto E, Schulz A, d'Udekem Y, Weintraub RG, Brizard CP, Konstantinov IE. J Thorac Cardiovasc Surg. 2022 Nov;164(5):1263-1274.e1. doi: 10.1016/j.jtcvs.2021.11.097. Epub 2022 Mar 16. PMID: 35430079   Take Home Points: Aortic valve repair for isolated aortic stenosis results in excellent outcomes, including half of patients need reoperation and two-thirds of patients avoid a valve replacement after 10 years. Tricuspid valve is technically more difficult to repair compared to a bicuspid valve, resulting in a suboptimal outcome. Commentary from Dr. Yasuhiro Kotani (Okayama, Japan), chief section editor of Congenital Heart Surgery Journal Watch Summary: Single center retrospective study included 111 patients with isolated aortic stenosis undergoing primary aortic valve repair between 1980 and 2016. They defined a residual left ventricular outflow tract obstruction <35mmHg and trivial or less aortic insufficiency as an optimal operative outcome.   Median age at surgery was 0.4 years (IQR: 1 month – 7.9 years) and median weight at surgery was 7.0kg (IQR: 3.7 – 25kg). 52 out 111 (47%) patients underwent aortic valve repair with the use of glutaraldehyde-treated pericardial patches for cusp extension or commissural suspension to ensure adequate coaptation of the valve cusps after relief of stenosis. Other 59 (53%) patients had a non-patch valve repair, consisted of 1 or more of the following: commissurotomy, resection of nodular dysplasia, thinning of valve leaflets, or leaflet mobilization. Early and late mortality were 0.9% and 0.9%, respectively. Freedom from aortic valve reoperation was 52.1% (95% CI, 38.7-63.8) at 10 years. Freedom from aortic valve replacement was 67.9% (95% CI, 55.4-77.5) at 10 years. An optimal outcome was achieved in 48 patients (43.2%; 48/111). Tricuspid aortic valve was associated with a suboptimal outcome (P=0.01).   Comment: This retrospective study was done at Royal Children’s Hospital, Melbourne, a world-renown institute. Outcomes were excellent, including two-thirds of patients remain free from aortic valve replacement 10 years after initial aortic valve repair. It is interesting that tricuspid valve carries a high risk for suboptimal result. The authors explained that this is a reflection of the greater technical complexity of repairing a stenotic tricuspid aortic valve.   There are several options for aortic valve repair in neonates and small infants, including catheter valvotomy, surgical repair, and Ross procedure. A catheter treatment is less invasive and may be beneficial in the neonates, but it poses a risk for the development of aortic insufficiency. Surgical repair needs the use of cardiopulmonary bypass, but it provides a precise relief of stenosis by commissurotomy and leaflet mobilization which can avoid a new-onset aortic insufficiency. The authors think it is important to delay valve replacement with the Ross procedure into school age and adolescent as we know the outcomes after Ross operation beyond infantile period is excellent in the recent era. Even though a catheter treatment is currently dominant in the management of this population, this report may give us an opportunity to reconsider the strategy for patients’ lifetime intervention.   

Comparison between Del Nido and conventional blood cardioplegia in pediatric open-heart surgery. Floh AA, Das S, Haranal M, Laussen PC, Crawford-Lean L, Fan CS, Mertens LL, Runeckles K, Honjo O.Perfusion. 2022 Feb 10:2676591211054978. doi: 10.1177/02676591211054978. Online ahead of print.PMID: 35143733    Take Home Points: Del Nido cardioplegia provides equivalent myocardial protection compared to blood cardioplegia. Although, right ventricular dysfunction was more seen in patients having Del Nido cardioplegia, this did not have impact on clinical outcome, such as hospital mortality and use of ECMO. Commentary from Dr. Yasuhiro Kotani (Okayama, Japan), chief section editor of Congenital Heart Surgery Journal Watch:   Summary: Single center retrospective study included 1534 patients having congenital cardiac surgery. Patients were divided into 2 groups: conventional blood cardioplegia (N=997) and Del Nido cardioplegia (N=537). Primary outcome was defined as a new postoperative more than moderate systemic ventricular dysfunction by echocardiogram. Secondary outcome was any severity of systemic ventricular dysfunction and right ventricular dysfunction. Surgical complexity was comparable between the groups. Cardiopulmonary bypass (CPB) time and aortic cross-clamp (ACC) time in Del Nido group were significantly longer (CPB: 117 vs. 108 min, p=0.04, ACC: 83 vs. 76 min, p=0.03), but the dose of cardioplegia was less in Del Nido group (2 vs. 3, p<0.001). Occurrence of systemic ventricular dysfunction was similar between the groups. Clinical outcomes, including hospital length of stay (p=0.07), postoperative ECMO (p=0.63), and hospital death (p=0.07) were comparable between the groups.   Comment: This study performed a comparison between Del Nido cardioplegia and conventional blood cardioplegia in high-volume center in North America. This is a single center, retrospective study but includes various complexity from neonates to small children which values this study compared to previous studies. Although Del Nido cardioplegia was introduced in 2015 in this institution and used nearly exclusively thereafter, both groups have similar surgical complexity and consistent surgical team, making this comparison reasonable. This study showed no difference in newly developed postoperative more than moderate systemic ventricular dysfunction between the groups which is not surprising. Interesting finding of the study is that Del Nido group had more right ventricular dysfunction compared to conventional cardioplegia group. The author’s speculation is that impairment of right ventricular function can be result from surgical duration (CPB and ACC time) not from source of cardioplegia. Theoretically, optimal myocardial protection in the right ventricle is difficult in the setting of congenital heart diseases. Patients may have right ventricular hypertrophy and abnormal coronary circulation in addition to general disadvantages, such as difficulty of temperature control compared to the left ventricle, etc. Further study focused on the distribution of cardioplegia in the right ventricle during cardiac arrest will answer the question.   

Hemodynamic Characteristics After Fontan Procedure in Patients with Down's Syndrome. Otsuka M, Kodama Y, Kuraoka A, Ishikawa Y, Nakamura M, Nakano T, Kado H, Umemoto S, Ishikita A, Sakamoto I, Ide T, Tsutsui H, Sagawa K.Pediatr Cardiol. 2022 Feb;43(2):360-365. doi: 10.1007/s00246-021-02727-6. Epub 2021 Sep 8.PMID: 34498105   Take Home Points: Higher mortality rates have been reported for patients with Down's syndrome undergoing Fontan surgery compared to patients with non- Down's syndrome patients. The causes of death are diverse, but pulmonary vascular obstructive disease is considered a contributing factor. In the study, they observed only one death, but there was a significant upward trend in central venous pressure in post-Fontan patients with Down's syndrome. Post-Fontan patients with Down's syndrome must be managed carefully, with regular evaluation for central venous pressure by cardiac catheterization.   Commentary from Dr. Masamichi Ono (Munich, Germany), section editor of Congenital Heart Surgery Journal Watch:   Summary: In this study, results after the Fontan procedure was compared between 7 patients with Down's syndrome and 35 patients with non-Down's syndrome. Main diagnosis of Down's syndrome was unbalanced AVSD in 6 patients and tricuspid atresia in 1 patient. There was 1 death in patients with Down's syndrome. The patients died of pulmonary thrombosis. At the last follow-up of median 19 years, creatinine, AST, ALT concentrations were significantly higher in the patients with Down's syndrome. There was no significant difference in medications except for the administration of pulmonary arterial hypertension-specifc therapies.   Postoperative cardiac catheterization data showed that central venous pressure, transpulmonray gradient, and pulmonary artery wedge pressure increased in patients with Down's syndrome, whereas these variables did not changed in patients with non- Down's syndrome. Arterial oxygen saturation decreased in patients with Down's syndrome, whereas it did not changed in patients with non- Down's syndrome.   Significance: The main significance of the study are that, there was a significant increase in central venous pressure and trans-pulmonary pressure gradient over time after the Fontan procedure in patients with Down's syndrome compared with patients with non-Down's syndrome. Whereas, there was no statistically significant difference in the change in calculated RpI over time.   Comment: Patients with genetic anomalies including Down’s syndrome are generally regarded as not being good candidates for the Fontan procedure. Although exact mechanisms are not still clear, we observe failing cavopulmonary shunt physiology more frequently in patients with genetic anomalies, compared with patients without genetic anomalies. As the causes, pulmonary vascular abnormalities in patients with genetic anomalies are speculated. It might not be apparent before bidirectional cavopulmonary shunt (BCOS) procedure, but emerged postoperatively as a failing BCPS physiology. Even after the Fontan completion, increase of central venous pressure and transpulmonary gradient in patients with Down's syndrome was reported in this study. Special attentions should be paid for patients with Down's syndrome, in respect to indication of BCPS procedure, postoperative management, and even after the Fontan completion.   As for the therapy, multiple classes of PAH-ST have been reported to reduce central venous pressure, reduce pulmonary vascular resistance, and increase exercise tolerance in Fontan patients. In this study, catheterization data before and after administering PAH-ST were obtained from 2 patients in the Down's syndrome group. In both patients, central venous pressure and transpulmonary pressure gradient decreased, which may be caused by macitentan as a PAH-ST. However, further studies are needed to determine the benefit of PAH-ST in Fontan patients with Down's syndrome.   In summary, central venous pressure tended to increase over time in patients with Down's syndrome after Fontan procedures compared with patients with non-Down's syndrome. Further research is warranted to clarify better management in patients with Down's syndrome after the Fontan procedure, including the efficacy of pulmonary arterial hypertension-specifc therapy.   

Comparison of management strategies for neonates with symptomatic tetralogy of Fallot and weight <2.5 kg. Qureshi AM, Caldarone CA, Romano JC, Chai PJ, Mascio CE, Glatz AC, Petit CJ, McCracken CE, Kelleman MS, Nicholson GT, Meadows JJ, Zampi JD, Shahanavaz S, Law MA, Batlivala SP, Goldstein BH; Congenital Cardiac Research Collaborative Investigators. J Thorac Cardiovasc Surg. 2022 Jan;163(1):192-207.e3. doi: 10.1016/j.jtcvs.2021.01.100. Epub 2021 Feb 3.PMID: 33726912   Commentary from Dr. Shuhua Luo (Chengdu, Sichuan, China), section editor of Congenital   Take Home Points: In symptomatic neonates with TOF weighing <2.5 kg, mortality and reintervention burden is high independent of treatment strategy. Summary: A total of 120 consecutive neonates with tetralogy of Fallot and symptomatic cyanosis weighing<2.5 kg at initial intervention between 2005 and 2017 were retrospectively reviewed in this study. The patients were categorized into staged repair (SR, n=76) (initial palliation followed by a later complete repair) or primary repair (PR, n=44) groups. In SR group, 53 patients underwent surgical palliation including systemic-pulmonary artery shunt (n=48), and RVOT procedure (n=5), while 23 patients underwent transcatheter palliation including balloon pulmonary valvuloplasty (n=11), PDA stent (n=7), and RVOT stent (n=5). Most patients in the PR group underwent a transannular patch type repair (n=33, 75%), followed by an RVOT conduit (n=11, 25%). No patients in the PR groups received valve-sparing type repair, which was achievable in five patients (7.6%, p=0.062) from the SR group.   The overall observed mortality between the two groups (15.8% vs 18.2%; p=.735) was similar at a median follow-up of 5.3 and 5.5 years, respectively. The result remained the same after propensity score adjustment. As expected, the neonatal (early) morbidity burden was greater in the PR group, however, the cumulative morbidity overall largely favored the PR group. In terms of reintervention, a total of 48 (63.2%) patients in the SR group had reintervention since palliation (both during interstage and post-repair). The most common transcatheter reintervention was pulmonary artery angioplasty and/or stent, and the most common surgical reintervention was RV-PA conduit replacement and S-P shunt revision; A total of 22 (50%) patients in the PR group had reintervention The most common transcatheter reintervention were pulmonary artery (n=10) and RVOT (n=4) angioplasty, and the most common surgical reintervention was RVOT revision or RV-PA conduit replacement. The overall risk of reintervention was comparable between the two groups.   Comment: The authors should be congratulated for provocative analysis of staged- versus primary-repair strategy for the symptomatic TOF babies smaller than 2.5 kg, for whom the controversy is perhaps most pronounced. The most important findings from this study is the operative mortality for these small Fallots remains strikingly high without any decrease in the past 20 years. Indeed, more questions may be raised   1) As the authors note, the major limitation of this study is the heterogeneity of the types of palliation in the SR group. Furthermore, the majority (53/76, 69.7%) of patients underwent surgical SP shunt placement, this may because that the cohort is somewhat historical cohort as the authors include patients from 2005 to 2017. The transcatheter intervention such as ductal stenting and RVOT stenting are recently gaining popular in all these centers. A recent retrospective cohort study including 2,363 neonate TOF patients underwent complete or staged showed complete surgical repair was associated with a significantly higher risk for early and 2-year mortality compared with the staged approach (1). However, the survival between SP shunt and complete repair was similar, therefore the survival benefit of staged approach might be associated with intervention. Unfortunately, the small number of patients in the current cohort preclude any meaningful statistical sub-analysis on the comparison of the outcomes of the surgical vs. interventional palliation.   2) Due to the nature of the retrospective study, several anatomic factors such as the size of the pulmonary annulus, stenosis of the pulmonary valve, tortuosity of the patent ductus arteriosus, length of infundibulum, and degree of infundibular hypertrophy were not included in the analysis, which is important to the selection of management strategies. The author noted the institutional bias being the most important factor associated with the selection of management strategy, however, the anatomic factors might be another potential confounding variable.   3) Another important issue of complete repair, especially in patients smaller than 2.5kg, include exposure of the immature brain to the deleterious effects of cardiopulmonary bypass (low flow or deep hypothermic circulatory arrest). The long-term neurological function might be also important for the clinical decision-making.   In conclusion, the current evidence is from enough for making any recommendation of management selection in such clinical challenging patients. The best to do might be what the individual center is comfortable with. Obviously, a multidisciplinary approach would be necessary including a thorough preoperative discussion including surgeons and interventional cardiologists with understanding the limitations of both the transcatheter and the surgical interventions. The further prospective randomized trial is warranted; however, the trial design is tricky due to limited number of such patients, and significant anatomic variants.   Reference: 1.Savla JJ, Faerber JA, Huang YSV, Zaoutis T, Goldmuntz E, Kawut SM, et al. 2-Year Outcomes After Complete or Staged Procedure for Tetralogy of Fallot in Neonates. J Am Coll Cardiol. 2019 Sep;74(12):1570–9.    

Post-operative Morbidity and Mortality After Fontan Procedure in Patients with Heterotaxy and Other Situs Anomalies. Duong SQ, Zaniletti I, Lopez L, Sutherland SM, Shin AY, Collins RT 2nd. Pediatr Cardiol. 2022 Jun;43(5):952-959. doi: 10.1007/s00246-021-02804-w. Epub 2022 Jan 22.PMID: 35064275   Commentary from Dr. Masamichi Ono (Munich, Germany), section editor of Congenital Heart Surgery Journal Watch   Take Home Points: Using the Pediatric Health Information System database, outcomes after Fontan procedure were compared with and without Heterotaxy syndrome and other situs anomalies (H/SA). As results, patients with heterotaxy syndrome and other situs anomalies had worse outcomes for all study measures: death/transplantation (p=0.047), ECMO (p<0.001), hemodialysis (p=0.026), prolonged mechanical ventilation (p<0.001) and vasoactive medication use and longer LOS (p<0.001). Heterotaxy syndrome and other situs anomalies is associated with increased cardiovascular, renal, and respiratory morbidity, as well as in-hospital mortality after Fontan procedure. Attention to renal and respiratory needs may improve outcomes in this difficult population.   Summary: Using the Pediatric Health Information System (PHIS) data system, this study was designed as a multicenter, retrospective cohort investigation of all patients cared for in the children’s hospitals within the Children’s Hospital Association (CHA) who met study inclusion criteria.   Baseline data were collected from June 2004 to June 2019, and they included 7897 patients at 50 PHIS centers who underwent the Fontan procedure, of whom 1366 (17%) met criteria for heterotaxy syndrome and other situs anomalies (H/SA) and 6531 (83%) met criteria for non-H/SA. H/SA were older (p < 0.001) and less commonly male (p=0.005). Significant race/ethnicity differences (p<0.001) were observed, with H/SA more commonly classified as Asian (p<0.001) or Hispanic (p<0.001). H/SA was associated with all outcomes tested including increased odds of in-hospital mortality/OHT (OR 1.74 [95% CI 1.01–3.03]; p = 0.047), ECMO support (OR 1.74 [95% CI 1.28–2.35]; p<0.001), need hemodialysis (OR 1.66 [95% CI 1.06–2.59]; p=0.026), prolonged mechanical ventilation (OR 1.85 [95% CI 1.53–2.25]; p<0.001), and prolonged vasoactive use (OR 1.65 [95% CI 1.43–10.90]; p<0.001).   In conclusion, the authors demonstrated that patients with H/SA are at increased risk of post-operative morbidity and mortality including in-hospital mortality or OHT, acute kidney injury (AKI) requiring hemodialysis, and prolonged mechanical ventilation and vasoactive use after single ventricle palliation with the Fontan procedure.   Significance: This study leveraged the strength of a large, multicenter US administrative database to provide a more nuanced understanding of outcomes in this difficult and relatively rare subgroup of patients with single ventricle heart disease. The main significance of the study is that, on a multicenter scale and in a relatively similar set of patients and procedures, patients with heterotaxy have increased post-operative respiratory support needs and a higher rate of acute kidney injury requiring dialysis. These findings provide two potentially modifiable targets to decrease the increased mortality observed in this cohort.   Comment: It is well known that patients with Heterotaxy and single ventricle heart disease is a well-described risk factor for poor prognosis. Mortality during first stages of palliation is high, but may improve after the Glenn procedure, with recent survival estimates of 60% at 10 years. This is linked to associated cardiac disorders, such as obstructed pulmonary venous return, atrioventricular valve regurgitation, and anomalous systemic venous return. However, recent Fontan registry reports from Australia and New Zealand have shown equivalent survival of patients with heterotaxy compared to others after hospital discharge.   In this study, post-operative day 0 extubation was strongly associated with non-mortality. They observed that patients with H/SA have lower rates of early (post-operative day 0) extubation and higher rates of prolonged mechanical ventilation. They postulate that intrinsic respiratory compromise related to ciliary dysfunction might contribute to the observed association of H/SA and prolonged respiratory support needs.   In this study, the authors hypothesized that patients with H/SA would have higher rates of AKI requiring hemodialysis, as we had described in this patient group after OHT. Indeed, we observed increased rates of AKI requiring hemodialysis after Fontan surgery in patients with H/SA, though the overall incidence was low. They speculated that heterotaxy is associated with increased rates of genitourinary malformations and an additional mechanism may be related to underlying ciliopathy, which has been associated with renal dysfunction. Both motile and non-motile cilia are important in establishment of left–right differentiation and non-motile ciliopathy is associated with polycystic kidney disease.   The race and gender might be an important factor. They observed a higher proportion of Hispanic and Asian patients, as well as fewer males, in the H/SA group. This is in concordance with birth defect registries that suggest a predominance of females and Hispanic ethnicity. Series from Taiwan, Japan, and Korea have all reported heterotaxy in approximately one-third of patients who underwent Fontan palliation.   We should remain vigilant and attentive to renal and respiratory complications to continue to improve surgical outcomes in patients with heterotaxy syndrome and single ventricle heart disease.