Congenital Heart Surgery

Post-Fontan pulmonary artery growth in patients with a bidirectional cavopulmonary shunt with additional antegrade pulsatile blood flow. Ferns SJ, Zein CE, Subramanian S, Husayni T, Ilbawi MN.Asian Cardiovasc Thorac Ann. 2020 Dec 25:218492320984095. doi: 10.1177/0218492320984095. Online ahead of print.PMID: 33356352   Take Home Points: The additional pulmonary flow in BCPS physiology facilitates the PA growth between Stage 2 and 3. PA growth are also shown after Fontan operation even if the additional flow was eliminated. No adverse effects, such as decreased ventricular function due to volume overload was shown even after Fontan operation. Commentary from Dr. Yasuhiro Kotani (Okayama, Japan), section editor of Congenital Heart Surgery Journal Watch:   Summary: Ferns and colleagues investigated the long-term effect of the additional antegrade pulmonary flow in 212 patients undergoing Fontan operation. The patients were divided into 2 groups based on the presence of additional flow (Pulsatile group (N=33) and Non-pulsatile group (N=70)). The key finding was that patients with an additional flow had a significant increase in PA size before Fontan and this continued after Fontan operation even eliminated an antegrade flow. They also showed an equivalent outcome between the groups, regarding with ventricular function, AVVR, and other complications.   Significance: It is still unclear that how the additional pulmonary flow affects PA growth between stage 2 and 3. This study clearly showed that Nakata index, i.e., PA size significantly increased in patients with an additional flow.   Comment: Previous reports describe both advantages and disadvantages of having the additional flow in BCPS. Advantages include better oxygen saturations, improved PA growth, and prevention of pulmonary arteriovenous malformations. Downsides include an elevated BCPS pressure and an increased ventricular volume overload. This study demonstrated the better PA growth even after Fontan operation and no adverse effects, such as impaired ventricular function was noted. However, it is important to know that the proportion of having HLHS in Non-pulsatile Group is much higher. In generally, patients with HLHS tends to have a smaller PA and a decreased ventricular function with significant AVVR, indicating this result should be carefully interpreted. Pulsatile Groups included more standard risk patients who are not likely to develop ventricular dysfunction and late complications and this may have affected the result of this study. An additional flow may not be a good option in patients with an impaired ventricular function, even non-HLHS. Therefore, indication of additional flow should be carefully considered in the group of single ventricle as they have a wide variety of anatomy and physiology and behave differently.   

Impact of early Coronavirus Disease 2019 pandemic on pediatric cardiac surgery in China. Shi G, Huang J, Pi M, Chen X, Li X, Ding Y, Zhang H; National Association of Pediatric Cardiology and Cardiac Surgery Working Group. J Thorac Cardiovasc Surg. 2020 Dec 1:S0022-5223(20)33146-9. doi: 10.1016/j.jtcvs.2020.11.074. Online ahead of print. PMID: 33419537   Take Home Message: The COVID 19 pandemic has decreased overall congenital surgical volume. There is an increased proportion of emergent and urgent operations (change in case-mix). Travel restrictions may affect access to congenital cardiac surgical care. Commentary from Dr. Luis Quinonez (Boston, MA, USA), section editor of Congenital Heart Surgery Journal Watch:   Summary: The objective of the study was to evaluate the impact of COVID-19 outbreak on the 1) congenital heart surgery programs and 2) the outcomes of patients with repaired lesions. This is a multicenter observational cohort study of 13 pediatric congenital heart disease surgical centers in China. The surgical cohort was divided into 3 groups: January to April 2018, 2019, and 2020 (COVID-19 era surgery). The follow-up cohort, was similarly divided: September to December 2017, 2018, and September 2019 to January 2020 (COVID-19 era follow-up). The authors used a daily migration scale index (MSI) to evaluate travel.   Overall surgical volume was decreased. The decrease in surgical volume did not correlate with the number of COVID-19 cases regionally or in each center, but was correlated with decreased MSI. In the surgical cohort, the proportions of symptomatic and emergent operations increased, while elective asymptomatic cases were not done during COVID-19 era. Nevertheless, mortality and need for ECMO was not affected. In the follow-up cohort, more patients received telephone or on-line follow-up during the COVID-19 era. The probability of death and unplanned admission was similar in the follow-up groups. Anxiety was greater in the COVID-19 era follow-up cohort.   The following graphic from the paper summarizes the main findings:     Comment: This is an interesting study that confirms what most of us perceive the impact of COVID-19 pandemic to be on congenital cardiac surgical care. The need to reallocate or restrict resources has led to decreased overall volume and prioritization of emergent, urgent and symptomatic cases. Despite this, outcomes seemed to have remained the same, as best as we can tell. However, the group that has not yet received attention is the asymptomatic, non-urgent, non-emergent cases. Will there be a negative impact on the outcomes of these patients when they eventually come to treatment? How about the patients that were unable to access care due to travel restrictions? The other group of patients that are vulnerable are those with virtual follow-up. It is unknown whether missed progression of disease will be important when physical examination could not be performed. It will take years before we can determine the true impact of the COVID-19 pandemic on our patients. We currently tend to focus on the sicker patients, but there is a greater number of “well” patients at risk.   

Mitral Valve Repair in Children Below Age 10 Years: Trouble or Success? Mayr B, Vitanova K, Burri M, Lang N, Goppel G, Voss B, Lange R, Cleuziou J.Ann Thorac Surg. 2020 Dec;110(6):2082-2087. doi: 10.1016/j.athoracsur.2020.02.057. Epub 2020 Mar 30.PMID: 32240647   Take Home Message: Mitral valve conditions requiring surgical management before the age of 10 years are rare. Mortality associated with surgical management of these patients is not small. Even if the need for reoperation and valvular replacement is relatively high, most patients still benefit from surgical repair and attempting so is worth it.   Commentary from Dr. Frederic Jacques (Quebec City, QC, Canada), chief section editor of Congenital Heart Surgery Journal Watch:   In the December 2020 issue of Annals of Thoracic Surgery, Mayr et al. report on 50 patients who had surgical repair of their mitral valve before the age of 10 years from 1975 to 2017. Forty patients had congenital problems, while 10 other patients had acquired disease. The authors specifically looked at survival and cumulative incidence of reoperation. The median age at surgical repair was 1.2 years and 1.9 years for congenital and acquired conditions, respectively. Most patients had regurgitation. The mortality for congenital conditions was significant with an immediate operative mortality of 5% and a late mortality of another 10%. No death occurred for acquired problems. Among congenital patients, those experiencing regurgitation had a better outcome, compared to stenosis. At a 6-years follow-up, congenital patients had close to 40% need for reoperation. Ultimately, 10% of patients with initial repair required a synthetic prosthesis. Among the 20% who required a second surgical repair of the mitral valve, 50% ended up with a prosthesis.   This paper reminds us how rare and difficult to deal with are mitral valve problems in children before the age of 10. It shows how risky are these operations, however it is important to acknowledge that valvular surgical repair techniques have significantly changed (likely improved) over the course of the study period. Even though the risk of mortality, the need for reoperation and the need for prosthetic valvular replacement is not dismal, it is important to realize that 90% of these patients can be repaired with a sustainable result. Looking at this paper, pediatric cardiologists and surgeons should be encouraged to make every effort to keep the native valve whenever possible. Expectations of repair should also acknowledge how difficult this problem is, especially for congenital patients. The development of better surgical prosthesis both biological (see Emani et al.) and mechanical (see Ijsselhof et al., Annals of Thoracic Surgery, December 2020) will also improve the overall outcomes for these challenging patients.   

Anomalous aortic origin of coronary arteries: is the unroofing procedure always appropriate? Mostefa Kara M, Fournier E, Cohen S, Hascoet S, Van Aerschot I, Roussin R, El Zoghbi J, Belli E.Eur J Cardiothorac Surg. 2020 Nov 9:ezaa379. doi: 10.1093/ejcts/ezaa379. Online ahead of print.PMID: 33167026   Teaching Points Unroofing is a safe and effective technique for the majority of AAOCA. Surgical correction of AAOCA should be offered to symptomatic patients and high-risk asymptomatic patients. Intramural course is underestimated on CT and should always be rule out intraoperatively.   Commentary from Dr. Charles Laurin (Quebec City, QC, Canada), guest editor of Congenital Heart Surgery Journal Watch: Kara et al, from Marie Lannelongue Hospital, published in the European Journal of Cardio-Thoracic Surgery in November 2020 their last fifteen years experience with anomalous aortic origin of coronary arteries (AAOCA). The retrospective analysis of 39 consecutive cases (median age at surgery of 14 years) aim to define the optimal surgical technique for the majority of AAOCA and to discuss the justification of surgery for all AAOCA, with or without symptoms.   The cohort included 11 anomalous left coronary artery (ALCA) and 28 anomalous right coronary arteries (ARCA), from which respectively 10 (90%) and 21 (75%) were symptomatic. The two patients operated after sudden death episode had ARCA. Preoperative cardiothoracic scans (CT) showed 19 (49%) intramural course (5 ALCA; 15ARCA) and 27 (69%) interarterial course (8 ALCA;19 ARCA). Intraoperative intramural course was noted in 28 patients (8 ALCA;20 ARCA). Unroofing technique was successful in 30 (77%) patients with 11 needing adjunct pulmonary translocation and 3 resuspensions of the inter-coronary commissure. Survival rate was 100% at median time of 4 years (3-6). Three patients needed reoperations for recurrence of symptoms, which all had thrombosed bypass grafts.   With 64% of the cohort operated in the last five years, management guidelines of AAOCA have become more aggressive, especially regarding asymptomatic AAOCA. Surgical treatment has been extended to high-risk anatomy and asymptomatic patient (small lumen area, high degree of proximal stenosis, long interarterial course, small proximal segment width). ARCA was more prevalent and seemed at higher risk of sudden death event. The majority of symptomatic patients had intramural course. Nevertheless, 11 patients had no intramural course, and all were symptomatic. As reported by the authors, AAOCA intramural course was underestimated by preoperative imaging (49% vs 72% intraoperatively), which should warrant intraoperative systematic evaluation for undiagnosed intramural course. Coronary unroofing is a safe technique to address AAOCA with favourable midterm outcomes. Depending on anatomy, alternative techniques can be performed on top of unroofing. Bypass grafts should be avoided because of highly competitive flow with the native coronary artery, which lead to bypass occlusion.    

The significance of symptoms before and after surgery for anomalous aortic origin of coronary arteries in adolescents and adults. Meijer FMM, Egorova AD, Jongbloed MRM, Koppel C, Habib G, Hazekamp MG, Vliegen HW, Kies P.Interact Cardiovasc Thorac Surg. 2020 Nov 22:ivaa234. doi: 10.1093/icvts/ivaa234. Online ahead of print.PMID: 33221843   Take Home Points: Most symptomatic patients with anomalous origin of coronary artery have atypical symptoms. Surgical management of anomalous origin of coronary artery disease resolves symptoms in about 60% of patients. Major adverse cardiovascular events are at least 7.5% at a median follow-up of 3 years.   Commentary from Dr. Frederic Jacques (Quebec City, QC, Canada), chief section editor of Congenital Heart Surgery Journal Watch: Meijer et al. published a study in the Interactive CardioVascular and Thoracic Surgery trying to answer: What is the significance of symptoms among adult and teenage patients with anomalous origin of coronary arteries in the preoperative and in the postoperative period? These patients were mainly adults with a mean age 44±15 years. Their follow up, described as mid-term, has a median time of 3 years after surgical correction. They included all their 53 patients referred for surgery of a coronary artery originating from the opposite sinus of Valsalva from 2001 to 2018. Follow up data was available for about two thirds of them. Ninety-six percent of patients had some sort of symptoms before the operation. Almost 80% had suspicion of myocardial ischemia but only 35% of patients had typical angina pectoris prior to surgical correction. The remaining symptomatic patients had aborted sudden cardiac death (6%). For About 15% of patients, the coronary problem was either found during a familial screening or incidentally. Three quarter of the patients had a functional diagnostic test. From a surgical standpoint, almost 75% of patients had an unroofing procedure. Reimplantation, osteoplasty and CABG were also performed. Eight percent of patients had a combination of coronary procedures. Concomitant procedures were performed in 28%. To provide mid-term outcomes, the authors disregarded events occurring immediately after surgery. Only one death was reported. The proportion of symptom-free patient was 59%, which is significantly higher than before surgery. Three patients had typical de novo angina pectoris at follow up which required another intervention.   This is an interesting paper focusing on symptoms related to anomalous coronary origin. As the authors states, anomalous origin of the coronary arteries is a rare disease that may lead to major complications or sudden death in some. However, as the actual denominator of patients living with this condition is unknown and the available corrective strategies are not devoted of complications, no clear consensus was ever established to clinically follow those patients or to surgically treat them. The paper does not answer any question about this denominator. However, it depicts the clinical trajectory of patients who have reached surgical management. It was already known that complications of this surgical management may range from recurrence of symptoms to sudden death. When no symptoms are identified preoperatively, the surgery would be considered prophylactic. As such, to be fully in line with ethics principles, surgeons and cardiologists should prove that intervening on these patients is safer than doing nothing. Here, the question is how successful the surgical management in correcting symptoms of coronary ischemia (96% of patients had some evidence of symptoms (not necessarily typical of myocardial ischemia)) among mostly symptomatic patients. Surgeons and cardiologists should be reassured that intervening on symptomatic patients is likely to help in most of them (59%). This is in line with previous publications on this matter. Having said that, if the success rate of coronary artery bypass grafting were only 60% in the adult acquired coronary disease, it would be considered a failure. It is worth noting that the comparison of pre- and post-operative symptoms was available for only 64% of patients. In other words, we cannot evaluate the resolution or the increase of symptoms in about one third of these patients. This is a major limitation of this study. Regardless, we learn that among those we know about the follow up, at least 1 patient died, and 3 other patients required another intervention. This is concerning because even though the study does not report any stroke or neurological complications and does not report immediate postoperative complication, it already shows a significant complication rate. In truth, from the available data, we know that the classical 3-point major adverse cardiovascular events is at least 7.5%. It is de facto underestimated but again beware that this complication rate that would be considered unacceptable in most contemporary acquired coronary artery disease revascularization trials. This is even more clinically significant as the mean age of the patients of the present cohort would be considered an exceptionally low risk subgroup for acquired coronary artery disease management. Overall, a significant proportion of patients with anomalous origin of coronary artery was helped, but our congenital community should work together to make surgeries and overall management of these patients more sound and safer.   

Anomalous aortic origin of coronary arteries: an alternative to the unroofing strategy.   Gaillard M, Pontailler M, Danial P, Moreau de Bellaing A, Gaudin R, du Puy-Montbrun L, Murtuza B, Haydar A, Malekzadeh-Milani S, Bonnet D, Vouhé P, Raisky O.Eur J Cardiothorac Surg. 2020 Nov 1;58(5):975-982. doi: 10.1093/ejcts/ezaa129.PMID: 32572445   Take Home Points: In anomalous aortic origin of the coronary artery (AAOCA) with an intramural and interarterial course, unobstructed blood flow to the coronary from the appropriate sinus is the desired goal. Although unroofing is the most frequent procedure, alternative surgical techniques may achieve the goal with particular advantages, especially when unroofing is not possible or desirable. Despite success in the surgical treatment of AAOCA with little or no mortality, coronary events and reintervention rates remain significant.     Commentary from Dr. Luis Quinonez (Boston, MA, USA), section editor of Congenital Heart Surgery Journal Watch: This paper describes the experience of Hopital Necker, Paris with the surgical treatment of anomalous origin of coronary artery (AAOCA) from an inappropriate sinus and an interarterial course. Sixty-one patients are included with a median age of 14.7 years (3.7-66.1). There were 40 anomalous right and 21 left coronaries; 5 anomalous left coronaries had an intraseptal course. 70% of the patients were symptomatic, most commonly chest pain. 5 patients had sudden death. Interestingly, of the 34 patient that were tested for inducible ischemia, 41.2% were positive. The surgical techniques describe include osteoplasty (37 patients, 60.7%), which is opening the coronary artery at its exit point in the appropriate sinus and carrying the incision proximally into the aorta and then adding a patch (mostly autologous pericardium; the other technique is coronary translocation (18 patients, 31.1%), where the coronary is transected at its exit point and relocated in the appropriate sinus augmenting the anastomosis with an autologous pericardial patch. The last technique described is one for the intraseptal course (5 patients, 8.2%) where the pulmonary root is harvested like a Ross, the muscle bridge is unroofed, and the autograft is re-implanted. There were no operative deaths. Complications are described. Follow-up was 38 months (1-15 years).   This series has a respectable number of patients in which surgical techniques are presented. The descriptions, illustrations and video are sufficient to understand and reproduce the techniques. Notably, the operations are not unroofings, which is the probably the “standard” approach to AAOCA from an inappropriate sinus. In the operations described, the intramural segment of the anomalous coronary is left untouched. This is important, given the concern that manipulation of the intercoronary commissure risks early or late aortic valve insufficiency. This complication has been brought to the forefront by the recently published Congenital Heart Surgeons Society multicenter report and will likely garner interest in techniques that will avoid the commissure. For the osteoplasty technique, there are now two sources of blood flow to the coronary, the intramural course and the osteoplasty site. Does this put at risk the osteoplasty site (competitive flow) or is the intramural course at risk of thrombosis? Although there were no operative deaths, there were 3 acute post-operative coronary events, all requiring re-intervention and 1 needing ECMO. This is 3 of 61 patients, or 5%, which is not a small number. In the follow-up period there were no reported deaths, yet another 3 patients had re-interventions. Although they report all patients, but one, were asymptomatic, their follow-up testing was incomplete, with only about half the patients having inducible ischemia testing and about 60% having CT imaging, making the ability to draw conclusions limited. A very good part of the paper is the discussion, in which the authors describe the technical lessons learned from their experience. Overall, this paper is worth reading for anyone tackling AAOCA. Although the operations described may be more technically demanding, they are options if unroofing is not possible or desirable.   

Flow Dynamics in Anomalous Aortic Origin of a Coronary Artery in Children: Importance of the Intramural Segment. Hatoum H, Krishnamurthy R, Parthasarathy J, Flemister DC, Krull CM, Walter BA, Mery CM, Molossi S, Dasi LP.Semin Thorac Cardiovasc Surg. 2020 Nov 23:S1043-0679(20)30422-6. doi: 10.1053/j.semtcvs.2020.11.027. Online ahead of print.PMID: 33242612   Take Home Points: 3D printing model helps to assess the coronary flow and fractional flow reserve in ex-vivo. Coronary flow and fractional flow reserve decrease in the intramural portion during the aortic pressure increases. This ex-vivo functional assessment enables us to stratify the risk of myocardial ischemia and sudden death, thereby may help to make a decision for surgical intervention in patients with anomalous aortic origin of right coronary artery without any symptom.     Commentary from Dr. Yasuhiro Kotani (Okayama, Japan), section editor of Congenital Heart Surgery Journal Watch: Hatoum and his colleague published the paper regarding the risk assessment of anomalous aortic origin of a coronary artery (AAOCA) in children. They used 3D printing flow models placed in the aortic position of a pulse duplicator to measure the hemodynamics in the specific anatomy in AAOCA. Main findings from this study was that pressure, fractional flow reserve (FFR), and coronary flow dropped at the site of intramural course of right coronary artery arising from left sinus when the aortic pressure increased in patient with ischemia. The model for patient who had an unroofing repair of AAOCA demonstrated the improved the coronary flow and FFR.   Significance: It is extremely difficult to stratify the risk in patients with AAOCA, especially anomalous right without any symptom, hence surgical indication is controversial. This ex-vivo model represents the unique anatomy of AAOCA and enables to functional assessment without any invasive test.   Comment: Through the clinical experience of the repair of AAOCA, we became to know its unique anatomy and function, yet still not uncertain for risk stratification in each patient because of a wide variety of anatomic characteristics, such as the location of the ostium, the length of intramural course, and the relation to the pulmonary artery. Surgery for left coronary anomaly is recommended even in asymptomatic patients, however, we are still not known which patients should undergo the surgery in anomalous right coronary without any symptoms. Stress test can help to guide us to go for the repair but does not always show positive. The unroofing procedure is relatively simple procedure that experienced congenital surgeon can perform with very low risk, but the creation of new aortic regurgitation after surgery mainly due to manipulation of the commissure is not negligible. The 3D printing patient specific model can make functional assessment possible in ex-vivo and help us to determine the surgical indication if the model demonstrates significant drop in coronary flow and FFR. As AAOCA is very rare condition, nationwide database is necessary to build the guideline in the future. As accumulation of the experience of the treatment of AAOCA, database can also help to provide the tailormade recommendation for the choice of surgical technique in each patient.   

Early experience with the HeartMate 3 continuous-flow ventricular assist device in pediatric patients and patients with congenital heart disease: A multicenter registry analysis O'Connor MJ, Lorts A, Davies RR, Fynn-Thompson F, Joong A, Maeda K, Mascio CE, McConnell PI, Mongé MC, Nandi D, Peng DM, Rosenthal DN, Si MS, Sutcliffe DL, VanderPluym CJ, Viegas M, Zafar F, Zinn M, Morales DLS. J Heart Lung Transplant. 2020 Feb 13. pii: S1053-2498(20)31393-0. doi: 10.1016/j.healun.2020.02.007. [Epub ahead of print] PMID: 32111350 Similar articles Select item 32089389   Take-Home Points: This is a collaborative, retrospective study from ACTION compiling current experience with the novel use of the Heartmate 3 (HM3) ventricular assist device in older pediatric and adolescent patients. Overall, the HM3 demonstrated a very good safety profile, and most patients successfully underwent heart transplantation during the study period. The data are mostly summary in nature, and further details regarding management decisions will be needed in the future to help guide patient selection and post-implantation management.   Commentary from Dr. Jeremy Herrmann (Indianapolis), section editor of Congenital Heart Surgery Journal Watch: This study is a collaborative effort through the Advanced Cardiac Therapies Improving Outcomes Network (ACTION) and is the largest report of the use of the HeartMate 3 (HM3) LVAD in pediatric and adult patients with congenital heart disease. From 9 centers, the HM3 was implanted in 35 patients between December 12, 2017, and September 19, 2019. The median patient age was 15.7 years, median weight 65.7 kg, and median BSA 1.74 (range, 0.78-2.36) m2 (Table 1). Patients who underwent planned biventricular support device placement were excluded. Data were collected with a focus on quality improvement and are presented as summary statistics.   Dilated cardiomyopathy with or without neuromuscular disease was the most common pre-implantation diagnosis (83%), and 72% of patients were INTERMACS category 2 or 3 (Table 1). Of the HM3 implantations, 54% were bridge to transplantation, 29% were bridge to decision, 6% were bridge to recovery, and 11% were for chronic/destination therapy. Five Fontan patients received a HM3, though this was likely a very select group as none exhibited protein-losing enteropathy and likely were supported for atrioventricular valve regurgitation and/or systolic dysfunction.   The median follow-up period was 78 days (range, 2-646 days). Thromboembolic complications were infrequent with one occurrence of fatal global hypoxic-ischemic encephalopathy (the only mortality in the cohort). Bleeding requiring reoperation occurred in only one patient. Right heart failure (CVP > 16 mm Hg) occurred in 20% of patients, though biventricular support was needed in only 1 patient. The authors observed significant variation in anticoagulation management, though nearly all patients received warfarin and aspirin. At the time of the study, 13 patients were alive with the device, 20 underwent heart transplantation, and 1 underwent device explantation after recovery. Of the 21 patients transplanted or explanted for recovery, the median duration of support was 40 days (range, 2-310 days). Forty percent of patients weighed <60 kg with a BSE ranging from 0.78 to 1.72 m2. Five patients weighed less than 45 kg. There were no mortalities in this group while 11 of the 14 patients underwent transplantation. Other adverse events were relatively infrequent throughout the cohort.   While the results of the study are descriptive in nature, they demonstrate the ability of ACTION to rapidly assimilate information about new or evolving management techniques across multiple centers. It appears the HM3 LVAD may be a viable option for older pediatric and adolescent patients, though this is merely the start of potentially a larger discussion of how best to mechanically support this patient age group. The HM3 exhibited a favorable safety profile in this group, but how would it compare with other implantable devices? The HM3 has a larger extracardiac profile but shorter inflow cannula compared with the HeartWare device, and do these characteristics make the HM3 a better option for certain patient sizes or myocardial pathology? Variation in institutional practices was significant though expected given this novel application. It would be interesting to glean deeper information about how institutions chose this option and how long they waited to proceed with transplantation to permit recovery from the sequelae of end-stage heart failure. Finally, what is the discharge-to-home potential for patients with the HM3 device? Hopefully, ongoing work by ACTION will help clarify these issues in the near future.      

Minimally Invasive Surgery vs Device Closure for Atrial Septal Defects: A Systematic Review and Meta-analysis   Mylonas KS, Ziogas IA, Evangeliou A, Hemmati P, Schizas D, Sfyridis PG, Economopoulos KP, Bakoyiannis C, Kapelouzou A, Tzifa A, Avgerinos DV. Pediatr Cardiol. 2020 Mar 11. doi: 10.1007/s00246-020-02341-y. [Epub ahead of print] Review. PMID: 32162027 Similar articles Select item 32162026   Take Home Points: Device closure of secundum ASDs has become the first-line treatment, but surgical closure of ASDs, often via minimally invasive techniques, is still often performed This study performed a meta-analysis of reports comparing ASD device closure vs. closure via minimally invasive surgical techniques and revealed that the two techniques compare favorably.     Commentary from Dr. Timothy Pirolli (Dallas), section editor of Congenital Heart Surgery Journal Watch: Closure of secundum atrial septal defects is often performed in the cardiac catheterization lab using a variety of devices. Surgical closure is often reserved for large defects, defects for insufficient rims, patients with small left atriums and non-secundum ASDs or need for concomitant procedures. Some surgeons have transitioned to closing ASDs via minimally invasive techniques (various versions of right thoracotomy approaches or partial sternotomies). The authors of this study sought to examine existing published studies that compare repair of ASDs via these two approaches (percutaneous vs. minimally invasive cardiac surgery) through a systematic review and meta-analysis.   The authors selected two independent reviewers from their group to scour PubMed and the Cochrane databases to select articles that focused on comparing percutaneous and minimally invasive surgical repair of ASDs. The inclusion criteria involved randomized control trials, non-randomized prospective studies and retrospective clinical studies and excluded meta-analyses, reviews, editorials and studies involving conventional full sternotomy for surgical repair. Out of a total of 780 potentially relevant articles, only 20 reports were deemed sufficient to be evaluated for inclusion in this meta-analysis. From this cohort, only six studies (all retrospective) were found to meet all criteria and be of sufficient quality for inclusion in this review (Table 1).   From those six studies, a total of 1577 patients were included. Data was extracted from the studies for meta-analysis, the statistical analysis of which is explained in detail in the report. The patient demographics are depicted in Table 2 below. It is notable that the median age of the majority of cohorts in this study is in the late 30’s, suggesting few children were included in this meta-analysis. The majority of types of MICS performed involved right anterior mini-thoracotomies. Only one study involved partial sternotomies. Figure 1 depicts forest plots comparing MCIS repair vs. device closure for treatment efficacy, overall complications and length of hospital stay. The overall trend was that patients who underwent device closure had less complications and shorter hospital stays, but the success rate of closure with surgery was higher. Of note, not all studies included in report could be utilized in each segment of data analysis due to insufficient or incomplete data. Figure 2 compares the two techniques for femoral complications and pericardial effusions, both analyses of which favor device closure. Figure 3 compares the two techniques for post-operative headaches and residual shunts, both analyses of which favor surgical closure. There were no differences between the two techniques in terms of pulmonary complications, new-onset atrial fibrillation, wound infection, major bleeding, transfusion requirements, cardiac tamponade, pacemaker placement and re-operation rates. There were no fatalities reported in any of the studies.   The authors discuss the risks and benefits of each technique and also the controversy of what, in fact, defines a “successful closure” of an ASD. They opted to define the term as complete closure of the ASD with no residual shunt. There were no glaring new revelations from this analysis. MCIS techniques are shown to have a more consistently complete repair, but at the expense of longer hospital stay at the expense of a larger scar and bypass run. Their meta-analysis between the two modalities is reassuring though that many of the potential complications (listed above) were not found to be more prevalent in the MCIS cohorts. Device embolization, a possibly devastating complication of percutaneous closure, was only reported in one patient in all the studies.   It is fair to say that the authors did their best to compare these two techniques through an exhaustive literature review and detailed data extraction, but there are many notable limitations to this report. All were included studies were non-randomized retrospective analyses and not all studies reported the outcomes that the authors sought to evaluate. There was no analysis of ASD morphology and there was very little data to be extracted on the pediatric population. The authors’ final conclusion that MICS closure for ASDs is a safe and reproducible procedure and compares favorably to percutaneous closure is valid. It is also fascinating that none of the studies included in this meta-analysis were from the United States. As always, a randomized controlled trial would be ideal, but that does not exist and likely never will. Also, any pediatric practitioner who reads this report and extrapolates to meaning the pediatric population should be cautious since the majority of patients included in this meta-analysis are full-grown adults in whom MCIS closure (especially through right thoracotomy) of ASDs is more feasible due to patient size.   Tables and Figures        

Grown-up Congenital Heart Surgery in 1093 Consecutive Cases: A "Hidden" Burden of Early Outcome Haapanen H, Tsang V, Kempny A, Neijenhuis R, Kennedy F, Cullen S, Walker F, Kostolny M, Hsia TY, Van Doorn C. Ann Thorac Surg. 2020 Mar 5. pii: S0003-4975(20)30338-6. doi: 10.1016/j.athoracsur.2020.01.071. PMID: 32147413 Similar articles Select item 32131918   Take-Home Points: This retrospective study from Great Ormond Street Hospital retrospectively analyzes over 1,000 ACHD surgeries and possible risk factors for adverse outcomes over a 17-year period. The 30-day mortality outcome was overall low but increased by 6 months following CHD surgical procedures. The increasing complexity of CHD patients may be associated with longer ICU lengths of stay. Further consideration for updating standard outcomes metrics may be needed to accurately account for the extended window of postoperative risk beyond 30 days.     Commentary from Dr. Jeremy Herrmann (Indianapolis), section editor of Congenital Heart Surgery Journal Watch: Recognizing that surgical outcomes for adult congenital heart disease (ACHD) have progressively improved over the past two decades, the authors questioned whether the metric of 30-day postoperative mortality remains valid. Specifically, they aimed to determine whether prolonged ICU stay (>7 days) and 6-month mortality are more appropriate for assessing postoperative risk. The authors retrospectively reviewed over 1,000 consecutive ACHD surgeries performed over a 17-year period at Great Ormond Street Hospital in London. Surgical procedures spanned the full ACHD spectrum with the top three procedures being pulmonary procedure, aortic procedure, and ASD repair. Over half (55%) were reoperations and over one-third (36.8%) included concomitant procedures.   Overall, the mortality at 30 days was very low at 1.5% but increased to 2.4% at the 6-month mark. Forward stepwise regression analyses revealed several parameters associated with prolonged ICU stay (>7 days) OR 6-month mortality: sex, NHYA class III or greater, EF 50% or less, renal failure, multiple sternotomies, CHD complexity, urgent operation, preoperative ventilator support, CPB time, DHCA use, and concomitant procedures. Of these, higher NHYA class, complex CHD, preoperative ventilator support, CPB time, and concomitant procedures were significant associated with prolonged ICU stay (>7 days) OR 6-month mortality using multivariate logistic regression. Repeat sternotomy was not a risk factor, though the authors highlight discrepant information in the literature. The distribution of CHD complexity was similar across 4 eras of the study period, though the 6-month mortality decreased from 5.0% in the first era to 1.4% in the two most recent eras. The frequency of prolonged ICU stay increased during the study period possibly due to a greater degree of CHD complexity in the latter eras.   The authors conclude that 30-day mortality may be too short to fully account for the postoperative morbidity and mortality. This could also result from sicker patients receiving ICU-level care and surviving beyond 30 days but not for 6 months. This could be an important consideration for patient care given that patients will typically be home during most of the immediate 6-month postoperative window. This may also be an important consideration for outcomes data gathering and reporting. Regardless, it is gratifying to know that as a field, we have been able to elevate the standard of care for these potentially complex patients such that mere 30-day survival is an insufficient estimation of outcomes.    

Unicuspid aortic valve repair with bicuspidization in the paediatric population.   Matsushima S, Heß A, Lämmerzahl JR, Karliova I, Abdul-Khaliq H, Schäfers HJ.   Eur J Cardiothorac Surg. 2020 Oct 1:ezaa285. doi: 10.1093/ejcts/ezaa285. Online ahead of print.   Take Home Points:   Unicuspid aortic valve repair in pediatric patients remains a significant challenge. Bicuspidization is a technique used for repair of unicuspid aortic valves, involving the creation of a second functional commissure using patch material. The technique of bicuspidization appear safe and reproducible in experienced hands, with excellent survival and good freedom from re-intervention.     Commentary from Dr. Luis Quinonez (Boston MA. USA), congenital heart surgery section editor of Journal Watch: This is a review of 60 consecutive patients, ages 1 through 18 years (median 13), with unicuspid aortic valves who underwent aortic valve repair for stenosis, regurgitation, or a combination. Half the patients had undergone a previous valvuloplasties. The repair technique is bicuspidization. The technique involves creation of a second functional commissure opposite the best developed commissure using a combination of detached autologous valve tissue and patch material. The new commissure is created at the same height as the existing one. The concepts of geometric height and effective height are described, where the effective height should be approximately 50% of the geometric height. The geometric height is curved the length of the leaflet from base to free edge and the effective height is the vertical distance between the annulus and free edge. The study spans between 2003-2018 and uses various patch materials and an external suture annuloplasty. Overall survival was 96% at 10 years. Freedom from aortic valve reoperation was 73% at 5 years and 50% at 10 years. The time to reoperation was 0.2 to 13 years (median 5.2).   Significance: Durable repair of unicuspid aortic valves remains a significant challenge in the pediatric population. Replacement options may be limited in younger age groups.   Comment: This reports presents a sizable experience with repair of unicuspid aortic valves in the pediatric population. The technique of bicuspidization has been used by Dr. Schafer’s group in Germany since 2003. It is certain it has gone through several modifications over time. The technique is not described in sufficient detail to apply. Although the age range of the patient's is wide it is notable that half the patients were under 13 years of age and that 12 of the 60 patients were between 1 and 5 years of age, suggesting the technique can be applied early on even in small annuli. The authors mention that the technique can be applied to an aortic root size is down to 10 mm. Survival for the overall cohort was excellent. The freedom from aortic valve reoperation is very acceptable. Although there were some early failures, the median time of re-intervention was just over 5 years, which would allow enough growth to expand the options for re-repair or replacement. When looking at the patch material used, Gore-Tex performed the worst. Interestingly, fixed autologous pericardium did not perform as well as the other decellularized patches, although the follow-up for the latter is less than 5 years. The results of this study support the notion that in experienced hands unicuspid aortic valve repair can be effectively used "as a bridge to more definitive AVR". The question remains whether the technique is sufficiently standardized to make it reproducible in less experienced hands.      

Age over 35 years is associated with increased mortality after pulmonary valve replacement in repaired tetralogy of Fallot: results from the UK National Congenital Heart Disease Audit database.   Dorobantu DM, Sharabiani MTA, Taliotis D, Parry AJ, Tulloh RMR, Bentham JR, Caputo M, van Doorn C, Stoica SC.Eur J Cardiothorac Surg. 2020 Oct 1;58(4):825-831. doi: 10.1093/ejcts/ezaa069.PMID: 32187367   Corrigendum to 'Age over 35 years is associated with increased mortality after pulmonary valve replacement in repaired tetralogy of Fallot: results from the UK National Congenital Heart Disease Audit database' [Eur J Cardiothorac Surg 2020;58:825-31].   Dorobantu DM, Sharabiani MTA, Taliotis D, Parry AJ, Tulloh RMR, Bentham JR, Caputo M, van Doorn C, Stoica SC.Eur J Cardiothorac Surg. 2020 Oct 1;58(4):873. doi: 10.1093/ejcts/ezaa190.PMID: 32688385 No abstract available.     Commentary from Dr. Frederic Jacques (Quebec City, Quebec, Canada), congenital heart surgery section editor of Journal Watch: Dorobantu et al. published a study in the European Journal of Cardiothoracic Surgery trying to answer the question: Does age at the time of the first pulmonary valve replacement (PVR) among repaired tetralogy of Fallot (ToF) patients impact the long-term outcomes?[1] More specifically, it addresses survival. In this national registry-based multicentric retrospective study, the authors included all (707) ToF patients aged 15 or older who had to have PVR in the UK. They set to determine an age at which patients were more likely to survive both the procedure and in the long-term. They used a Cox regression model as well as CART (classification and regression tree analysis) to identify the optimal cut-off age. Both methods identified 35 years old as their cut-off, with older patients experiencing a 5.6-fold increased risk of dying at 10 years after PVR compared to the younger patients. Said differently, patients under 35 years of age at first PVR had a 10-year mortality of 1.3% compared to 10.4% for those between 35 and 50 years. Older patients required more tricuspid repair or replacement (15% vs. 6%) and arrhythmia surgery (18% vs. 6%) compared to the younger patients. The association of tricuspid valve surgery was linked to a worse survival in those between 35 and 50 years old, but not for the younger patients. Both age groups had worse survival than the general UK population, but the mortality excess was less for those with PVR under 35 (0.9%) compared to the 35-50 age group (7.7%).   Significance: Current guidelines for PVR among ToF patients do not take age into account in setting the surgical indication and timing but they likely should include guidance on this point. This study raises the question: Should we operate on patients earlier to achieve better long-term outcome?   Comment: With this type of registry, surgical indication criteria are not strongly defined, but one can reasonably assume most patients were treated with current Western standards. The most important strength of this study is that it is based on the mandatory inclusion of all the congenital patients from one country: no patient is missed. This likely results in catching subclinical right-sided damage that have not reach clinical significance. The reader is well aware that both residual regurgitation and stenosis are linked to myocardial damage, impact on exercise tolerance and even survival. Current guidelines recommend PVR when either volume or pressure overload had resulted in clinical signs and symptoms. So far, the literature has shown repercussion of PVR on RV volume and symptoms, but clear survival benefit is still to be demonstrated. Recent findings suggest that most of the hemodynamic recovery is seen immediately after surgery. This supports the idea that once right ventricular damage has been severe enough and long-standing, it might not be reversible. This also favors earlier intervention, since relieving the burden on the right ventricle earlier may prevent other comorbidities from developing. This study shows that older patients had more tricuspid regurgitation and arrhythmias. Obviously, a spectrum of subclinical myocardial damage occurs in the background before reaching to other clinically significant lesions. It is not known what portion of this damage is reversible. The authors suggest that current guidelines should be revisited not only based on hemodynamic parameters and symptoms, but that age should be accounted for in order to achieve a survival that approaches the general population as closely as possible. Will the paradigm shift from reacting to hemodynamic complications to preventing them?    

Use of a dilatable exGraft™ conduit in single ventricle palliation. Qadir A, Tannous P, Stephens EH, Kalra A, Forbess JM, Nugent A. Ann Thorac Surg. 2020 Jan 25. pii: S0003-4975(20)30075-8. doi: 10.1016/j.athoracsur.2019.12.024. [Epub ahead of print] PMID: 31991132 Similar articles Select item 31982878   Take Home Points: The major limitation of conduits made with prosthetic material is that they do not dilate much or grow with the patient. The development of a novel, balloon-expandable ePTFE conduit may change the management of patients that require a prosthetic conduit. This is the first published case of this conduit. Commentary from Dr. Timothy Pirolli (Dallas), section editor of Congenital Heart Surgery Journal Watch: Surgically-placed conduits and vascular grafts are commonplace in congenital heart surgery. One obvious limitation to placing a prosthetic conduit between a ventricle and great vessel (as opposed to a homograft or autograft) is the lack of growth potential. A prosthetic conduit that could be dilated could revolutionize the field, as it would add the ability to minimize or delay redo surgeries, thus decreasing the associated morbidity and mortality. A start-up company in Pittsburgh (PECA labs) has developed a novel version of the ePTFE graft that has been shown to be dilated up to 200% its original diameter in bench trials. I chose to highlight this case report because of its novelty. As a disclaimer, I am an unpaid consultant for this company and have had the ability to collaborate with this group of researchers on animal studies, some of which are on-going. Thus, I can attest first-hand that the graft handles well and does indeed dilate without significant recoil months after implantation. The graft also has the novel advantage of having radiopaque markers on it to allow for safer dilation during catheterization (Figure 1). As mentioned in this case report, the graft is FDA-approved and available for surgical implantation in human beings. This report represents the first published case of implantation and dilation of the novel exGraft™ in a patient undergoing congenital heart surgery. The case report describes a relatively standard Stage 1 palliation of a baby born with hypoplastic left heart syndrome (MA/AA). The surgeon used of a 6 mm exGraft™ as an RV-PA (Sano) conduit in lieu of the relatively standard ring-enforced PTFE graft that is often used for Sano construction. Aspirin alone was used for shunt thrombosis prophylaxis. The patient underwent a pre-Glenn catheterization at age 5 months. A gradient across the conduit was found to be 60-65 mmHg and the conduit was then dilated to 9 mm by serial dilations (Figure 2). There was mild residual narrowing at the insertion site into the pulmonary arteries, but otherwise the conduit held its dilated diameter and did not require a stent placement. The patient proceeded to a Glenn at 7 months of age that had an uncomplicated postoperative course. At the time of the explant of the conduit, it had maintained its internal diameter 2 months after dilation (Figure 3). As stated, this is the first published application of the novel exGraft™ in congenital heart surgery. The safe dilation of the graft to 150% its original diameter in this patient represents a potential milestone for our field. The use of the exGraft™ as a Sano conduit during a stage 1 palliation represents one of many potential uses of this graft. There is a range of sizes that would allow the graft to be used as a modified BT-shunt, interposition graft on large vessels, Fontan conduit and RV-PA conduits on larger children. The ability to implant and later enlarge a prosthetic graft would certainly decrease the morbidity and mortality associated with surgical correction of conduit dysfunction. The company is also working on valved-conduits that are balloon expandable, but those studies are still ongoing. In summary, the exGraft™ from PECA labs has the potential to change the paradigm of the timing and frequency of certain congenital heart surgical procedures.     Figure 1: Radiopaque markers on outer surface of an 8 mm exGraft Figure 2: In vivo dilation of the exGraft RV-PA conduit. A) Baseline angiogram demonstrating slight luminal irregularity and proximal stenosis of the exGraft. B) Stored fluoro image of maximum balloon dilation to 9 mm. (C and D) Final exGraft angiograms in lateral and caudal angulation showing interval increase in graft diameter. Figure 3: Images of explanted conduit. Note radio-opaque markers on outer surface of graft.

Impact of trisomy 13 and 18 on airway anomalies and pulmonary complications after cardiac surgery. Swanson SK, Schumacher KR, Ohye RG, Zampi JD. J Thorac Cardiovasc Surg. 2020 Sep 1:S0022-5223(20)32473-9. doi: 10.1016/j.jtcvs.2020.08.082. Online ahead of print. PMID: 32981701   Take Home Points:   Cardiac surgery can be performed safely in the setting of Trisomy 13 and Trisomy 18. T13 and T18 patients undergoing cardiac operations have higher rates of airway complications and require longer ICU stays than matched controls. While operative interventions are likely to be successful, guidance for parents of these patients should include a discussion of postoperative respiratory support.     Commentary from Dr. Barry Deatrick (Baltimore, MD, USA), section editor of Congenital Heart Surgery Journal Watch:  Trisomy 13 (Patau syndrome) and Trisomy 18 (Edwards syndrome) are both associated with high rates of congenital heart diseases, such as VSD, ASD and PDA.  More importantly, however, these chromosomal abnormalities are associated with a severely impaired long-term survival, with a median survival of less than 2 weeks.  As approximately 5-10% of patients survive over 1 year, it is perhaps not surprising that offering surgical treatment for cardiac malformations remains somewhat controversial.  However, as reports of long-term survival increase, more centers offer treatments, such as cardiac surgery, aimed at long-term survival.  Although multiple  have established that these patients can safely undergo surgery, fewer reports have described the postoperative courses of these patients, and the specific comorbidities which complicate postoperative recovery.  Because respiratory complications have been noted to be major complicators of recovery in previous series, this review was performed with a focus on identification and management of respiratory complications.  The authors performed a retrospective case-control study of all Trisomy 13 and Trisomy 18 (T 13/18) patients in a single center over 20 years undergoing cardiac surgery.  A 3:1 diagnosis-matched control group was identified, and individuals were further matched by age at surgery and then date of surgery.   In this study, 14 patients with T 13/18 were identified, and underwent surgical operations at a median age of 93 days. Trisomy 18 was more common than trisomy 13. The indications for operation  included VSD, coarctation, tetralogy of Fallot and complete AVSD.  64% of T 13/18 patients were admitted preoperatively , whereas only 13% of controls were.   Preoperatively, 3 of the T13/18 patients had airway evaluation by specialist physicians.  Patients with T13/18 were more likely to require preoperative respiratory support (71% vs 14%, P=0.001), including intubation and ventilation as well as noninvasive ventilatory assistance.   Operatively, there were no significant differences in cardiopulmonary bypass time, cross clamp time, or circulatory arrest time.   Postoperatively, 13 of the 14 T 13/18 patients experienced at least one complication, And all of these patients experienced at least one respiratory complication. Further, 58% of these patients had at least one non respiratory complication.  In addition to having higher rates of respiratory support preoperatively, T 13/18 patients also had high rates of post-operative respiratory complications, including prolonged mechanical ventilation prolonged noninvasive ventilation, reintubation , tracheostomy, and respiratory infections.   Some other notable complications, including infection (tracheitis and sepsis), need for gastrostomy tube placement, and increased need for vasoactive and inotropic support.   The authors conclude that although survival is good, hospital and ICU length of stay is longer in T13/18 patients than in matched controls.   This retrospective study demonstrates that those increases are at least correlated with an increased prevalence of respiratory and airway anomalies in these patients.   While not recommending against surgical repair for these patients, they suggest that awareness of these airway issue should be included in prenatal counseling, preoperative planning, and managing expectations for these patients.    

Ross Procedures in Children With Previous Aortic Valve Surgery. Buratto E, Wallace FRO, Fricke TA, Brink J, d'Udekem Y, Brizard CP, Konstantinov IE. J Am Coll Cardiol. 2020 Sep 29;76(13):1564-1573. doi: 10.1016/j.jacc.2020.07.058. PMID: 32972534   Take Home Points:   Initial Ross procedure in infancy may be associated with relatively higher mortality, justifying initial attempts to defer operation with aortic valve repairs, even if eventual replacement is necessary. Secondary Ross after initial valve repair is associated with superior long-term survival and freedom from autograft reoperation. Delaying the Ross operation by performing initial valve repair, when possible, may be preferred to initial Ross in infant patients.   Commentary from Dr. Barry Deatrick (Baltimore, MD, USA), section editor of Congenital Heart Surgery Journal Watch:  Aortic valve disease requiring surgical repair in childhood can be a challenge, with limited options available for replacement.  Although the Ross procedure offers the best long-term freedom from reoperation in neonates and infants, the long term freedom from reintervention in some series remain disappointing, with high mortality rates in infants (16 – 22%).  Because of this, some centers advocate an approach that of initial aortic valve repair, whenever possible, followed by a secondary Ross procedure when necessary.  The goal of this retrospective analysis as to determine if there was a difference in outcome between the Ross operation when performed as a primary operation and when performed as a reoperation.   The authors retrospectively analyzed 541 aortic valve operations in their institution, 344 of which were aortic valve repairs, and 140 of which were Ross procedures.  68 (49%) of the Ross procedures took place after previous valve repairs, and 72 (51%) took place as the primary operation.   Patients undergoing a primary Ross were older (8.6 years vs 7 years), and had higher weights (28.9 kg vs. 19.7 kg) although these differences weren’t significant.  The early mortality was 5.6% (n = 4 of 72) in those undergoing primary Ross procedure, compared with 4.4% (n = 3 of 68) in those undergoing secondary Ross procedure.  This difference was not statistically significant (p = 1.0).  Freedom from reoperation at 10 and 15 years was 68.3% and 48.5% in those undergoing primary Ross procedure.  In those who underwent secondary Ross procedure, freedom from reoperation at 10 and 15 years was 62.2% and 47.0% respectively.  Again, this difference was not statistically significant.   In the  propensity-matched groups, however, survival at 10 and 15 years was 90.0% and 82.6% in the primary Ross procedure group, compared with 96.8% at both 10 and 15 years in the secondary Ross procedure group. In the propensity-matched analysis, this difference was statistically significant (p = 0.04).   The mechanisms for the improved survival and freedom from reoperation are discussed briefly, including possible stabilization of the autograft from postoperative scar and adhesion formation, but this was just postulated rather than investigated.  None were investigated in this retrospective review.   The authors concluded that secondary Ross procedure performed after initial aortic valve surgery achieves superior long-term survival and freedom from autograft reoperation compared with primary Ross procedure. A strategy of initial aortic valve surgery followed by delayed Ross procedure may provide better long-term survival and freedom from autograft reoperation in aortic valve disease in children.      

Characteristics and Surgical Outcomes of Patients With Late Presentation of Anomalous Left Coronary Artery From the Pulmonary Artery: A Multicenter Study View Article David M Kwiatkowski 1, Christopher W Mastropietro 2, Katherine Cashen 3, Saurabh Chiwane 4, Saul Flores 5, Ilias Iliopoulos 6, Karan B Karki 7, Karl Migally 8, Monique R Radman 9, Christine M Riley 10, Peter Sassalos 11, Jennifer Smerling 12, John M Costello 13, Collaborative Research from the Pediatric Cardiac Intensive Care Society Investigators PMID: 32858217;  DOI: 10.1053/j.semtcvs.2020.08.014 Take Home Points 29% of patients with ALCAPA in this large, retrospective, multicenter cohort study presented at greater than one year of age. Patients presenting at >1 year of age had excellent intermediate term survival (no mortalities in this series.) 11% of patients presenting with ALCAPA at greater than 1 year of age presented with cardiac arrest. Although severe LV dysfunction was less common for patients presenting at greater than 1 year than for those presenting at less than 1-year, chronic arrythmia and mitral regurgitation were common. Commentary from Dr. Barry Deatrick (Baltimore, MD, USA), section editor of Congenital Heart Surgery Journal Watch:  Although most patients with anomalous left coronary artery from the pulmonary artery (ALCAPA) present with heart failure or shock in the first months of life, a population of patients who develop extensive collateralization do not present with acute myocardial ischemia.  Most previous studies of this population are small, and limited to single center studies.  The authors of this study sought to characterize the clinical presentation, outcomes of patients who underwent ALCAPA repair older than 1 year of age with the collective experience from 21 congenital heart centers over 10 years. They hypothesized that this older cohort would present with different clinical symptoms and have different postoperative outcomes than a younger cohort. A multicenter retrospective cohort study including all patients who underwent initial coronary artery surgery for ALCAPA between January 2009 and March 2018 (21 centers; 248 patients).  Patients who underwent surgical correction at an age > 365 days were identified.  Patients with ARCAPA, single coronary, or other significant cardiac lesions were excluded.  Of the 248 patients identified, 72 underwent operation at greater than 1 year of age.  The median age in this group was 8.3 years, 15 of whom were older than 18.  There was no correlation between the degree of LV dysfunction, and the severity of mitral regurgitation.  11% presented with cardiac arrest, and 4% presented in cardiogenic shock.  35% of patients had been admitted to the hospital for another diagnosis, and 44% of these patients were discharged without the diagnosis of ALCAPA being made.  Compared to the younger patients, patients in the older cohort were more likely to present after cardiac arrest, but less likely to present with respiratory failure or shock, and less likely to require preoperative inotropic or ventilatory support.   No patients received transplantation during the study, although one was listed.  At 1-year follow-up, only 5% had moderate or worse systolic dysfunction, and 19% had moderate or worse mitral regurgitation.  Among the 13 patients who presented with moderate or worse LV systolic dysfunction, recovery was seen in 15% by discharge, 89% by 1-year and 90% by 3-year follow-up. The authors concluded that patients who presented with ALCAPA older than 1 year of age were more likely to present with cardiac arrest than the younger cohort, the majority of patients who presented older did not have ventricular dysfunction, MR or LV dilation.  Further, they confirmed that outcome of repair after 1 year of age is associated with good intermediate term outcome, and recommend continued follow-up to assess long term outcome. Table 1.

Risk Factors for Failed Fontan Procedure Following Stage 2 Palliation View Article Masamichi Ono 1, Melchior Burri 2, Benedikt Mayr 3, Lisa Anderl 4, Martina Strbad 4, Julie Cleuziou 4, Alfred Hager 5, Jürgen Hörer 4, Rüdiger Lange 6 PMID: 32828751;  DOI: 10.1016/j.athoracsur.2020.06.030 Take-Home Points Retrospective, single center study, retrospectively analyzing 525 patients receiving Stage 2 Palliation (S2P) with superior cavopulmonary connection for single ventricle palliation over 20 years. The incidence of Fontan completion following S2P was, 83.9% at 3 years and 87.1% at 5 years. HLHS, UAVSD, reduced EF, increased EDP and increased PA pressure were all associated with inability to tolerate S2P. Commentary from Dr. Barry Deatrick (Baltimore, MD, USA), section editor of Congenital Heart Surgery Journal Watch:  The authors analyzed the results of two decades worth of bidirectional superior cavopulmonary (Glenn) shunts (BCPS) in order to understand risk factors that may contribute to failure to complete total cavopulmonary connection.  They reviewed 525 patients undergoing BCPS over 20 years in a single institution. Specifically, they were interested in identifying if there were anatomic, physiologic, or hemodynamic scenarios that correlated with inability to reach successful Fontan circulation. A competitive risk analysis was performed between one of three states: Fontan completion, death, and being alive without Fontan.   Overall, the incidence of Fontan completion was good, 83.9% at 3 years and 87.1% at 5 years.  The overall mortality was 10.4% at 3 years and 10.7% at 5 years, with a relatively small number (5.7% and 2.2% at 3 and 5 years respectively) being alive and not having completed a Fontan.  Risk factor analysis was performed by first completing a Cox regression model, and on univariate analysis, HLHS, UAVSD, dominant RV, significant AVV regurgitation requiring an AVV procedure, higher pulmonary artery pressure (PAP), higher left atrial pressure, elevated ventricular EDP and reduced ventricular ejection fraction were associated with those patients who had not completed a Fontan procedure.   On multivariate analysis, HLHS (HR = 4.1), UAVSD (HR 10.1), higher PAP, and reduced function (HR 4.2) emerged as independent risk factors.   AV valve regurgitation requiring AV valve repair was not an independent risk factor in all patients, however, the need for an AV valve repair was significantly higher in patients with UAVSD. The authors conclude that stage 2 palliation is low risk, and leads to excellent rates of Fontan completion, but that some patient groups do have increased risk of complications, mortality, and inability to achieve Fontan circulation.  These are, perhaps not surprisingly, anatomic diagnoses of HLHS and UAVSD, impaired ventricular function as assessed by echocardiography or elevated filling pressures, and elevated pulmonary artery pressure.   An examination of how each of these factors contribute to failure to progress, specific strategies to mitigate them, or a comparison of techniques applied to achieve stage 2 palliation, remains beyond the scope of this retrospective analysis.