Congenital Heart Surgery

Outcomes of single ventricle palliation in infants with heterotaxy syndrome. Vodiskar J, Kido T, Strbad M, Cleuziou J, Hager A, Ewert P, Hörer J, Ono M. Eur J Cardiothorac Surg. 2021 Mar 30:ezab141. doi: 10.1093/ejcts/ezab141.   Take Home Messages: Single ventricle physiology with heterotaxy pertains a high mortality risk. Restricted pulmonary blood flow and atrioventricular valve regurgitation are The two most important risk factors for mortality. Providing unrestricted pulmonary blood flow and repairing total anomalous pulmonary venous connection offers survival advantages.   Commentary by Dr. Frederic Jacques (Québec city, Canada), chief section editor of Congenital Heart Surgery Journal Watch:  Heterotaxy syndrome is a recognized risk factor for overall poor outcome with single ventricle patients. This syndrome that mainly defines abnormal abdominal visceral configuration is associated with atrial appendages isomerism. Vodiskar et al. (Munich, Germany), reported their experience with 53 patients operated from 2001 to 2019. Their cohort comprises 66% right dominant ventricle, 49% total anomalous pulmonary venous connection, and 80% pulmonary stenosis/atresia. Thirteen patients were born with moderate to severe atrioventricular regurgitation. Consequently, these patients were treated with a non-heterogenous surgical protocol. In fact, 87% required some sort of neonatal palliation of which roughly one quarter died. A group of 41 patients underwent a bilateral cavopulmonary connection (17% as an initial surgery). About one tenth died. Of the 58% that reached the total cavopulmonary connection, none died. Unplanned reintervention occurred in 60% after Stage-1, mainly to better adjust pulmonary inflow, but also to address atrioventricular valve regurgitation. Through multivariate analyses, the authors identified restrictive pulmonary blood flow (HR 3.23; 95% CI 1.02–10.2; P = 0.05) and moderate to severe atrioventricular regurgitation (HR 3.57; 95% CI 1.27–10.0; P = 0.02) as independent risk factors for mortality. Providing a sustainable pulmonary inflow and correcting the pulmonary venous connection were two ways identified to come up with better survival.   Comment:   This paper reports on a large cohort of patients with heterotaxy in addition to single ventricle physiology. Not surprisingly, this is a motley group of patients comprising various combinations of systemic and pulmonary venous connections, various types and degrees of outflow obstruction, as well as various atrioventricular valve configurations and degree of regurgitation. Nevertheless, the authors did an honest effort in trying to pinpoint risk factors for mortality and potential factors to tackle down in order to devise better outcomes. The findings echo intuitions, as with one functioning ventricle, one could expect that any inflow or outflow anomaly would lead to worse outcomes, and that correcting those would potentially reverse this burden, at least partially. The paper, just like similar previous papers from other authors, also highlight how limited this kind of single centre analysis is for such a rare disease. Even large cohorts from large institutions have somewhat been limited in their capacity to derive meaningful messages that go further what can be expected form the insight of common sense. In order to change in a more radical fashion the outcomes with this very difficult group of patients to deal with, multicentre strategies are likely mandated. An international registry comprising all the anatomical variables faced with the spectrum within the heterotaxy syndrome is needed in order to get more granular in the analysis of these patients.

Comparison of Management Strategies for Neonates With Symptomatic Tetralogy of Fallot. Goldstein BH, Petit CJ, Qureshi AM, McCracken CE, Kelleman MS, Nicholson GT, Law MA, Meadows JJ, Zampi JD, Shahanavaz S, Mascio CE, Chai PJ, Romano JC, Batlivala SP, Maskatia SA, Asztalos IB, Kamsheh AM, Healan SJ, Smith JD, Ligon RA, Pettus JA, Juma S, Raulston JEB, Hock KM, Pajk AL, Eilers LF, Khan HQ, Merritt TC, Canter M, Juergensen S, Rinderknecht FA, Bauser-Heaton H, Glatz AC.J Am Coll Cardiol. 2021 Mar 2;77(8):1093-1106. doi: 10.1016/j.jacc.2020.12.048.   Editorial:  Primary Versus Staged Neonatal Repair of Symptomatic Tetralogy of Fallot: Time to Randomize? Hornik CP.J Am Coll Cardiol. 2021 Mar 2;77(8):1107-1109. doi: 10.1016/j.jacc.2020.12.043.     Take Home Points   Neonates with symptomatic Tetralogy of Fallot may undergo early primary repair or staged repair consisting of initial palliation to augment pulmonary blood flow followed by complete repair. A retrospective, multicenter study found a similar observed risk of death at 4 years with early primary repair compared to a stager approach.   Commentary by Dr. Luis Quinonez (Boston USA), section editor of Congenital Heart Surgery Journal Watch:   Summary   Question:  What is the best surgical strategy for the newborn with symptomatic TOF, complete or staged surgical repair?   Design: Retrospective, multicenter (Congenital Cardiac Research Collaborative)   Population:  Symptomatic neonates with TOF with PS or PA undergoing early primary repair (n=230) and staged repair (n=342)   Analysis:  Risk stratified; Propensity score adjustment   Outcomes:  Death, cumulative morbidity, reinterventions   Results:   Pre-procedural ventilation, prematurity, DiGeorge syndrome, and pulmonary atresia were more common in the staged group.   Reintervention risk was higher in the SR group.   Less neonatal morbidity in the staged group, whereas overall cumulative morbidity burden favoured the primary group.                     Commentary   This is a very important paper that should be read.   It addresses an ongoing dilemma in our specialty:  Staged vs. primary repair of symptomatic neonatal TOF with PS or PA.  The study is retrospective but multicenter.  There is a selection bias to stage the sickest patients.  The study attempts to risk adjust and uses propensity methodology.  The main finding of the paper is that overall mortality is similar between staged and primary repair, despite early mortality risk in the primary repair group.  The burden of reintervention occurs in the staged group.  Cumulative morbidity favoured the primary repair group.   A few things are worth pointing out:  The increased popularity of stent-based palliation may affect the outcomes of patients with the staged approach, in a positive or negative direction.  It is unclear whether PDA stenting is superior to a Blalock-Taussig shunt and will likely be the subject of a randomized trial in the near future.  Valve-sparing interventions were not different between the two groups, even when looking at the patients with PS alone.  The argument that sparing the pulmonary valve is more likely in an older patient may not hold.  The rate of pulmonary arterioplasty was similar in both groups, suggesting no advantaged for either treatment strategy.  Because the median follow-up is about 4 years, we cannot know if either strategy will have any long-term advantage.   I am concerned that with the current public reporting systems that emphasize early mortality (30-day or operative), there may risk aversion against primary repair and in favour of a staged approach.  This problem may be averted by public reporting of longer-term outcomes (even up to a year).   This paper provides equipoise to justify a randomized trial.  The accompanying editorial focuses on the challenges of doing surgical randomized trials, suggesting randomization methodology to account for surgeon or institution expertise.

Outcomes of single ventricle palliation in infants with heterotaxy syndrome Janez Vodiskar 1 2, Takashi Kido 1 2, Martina Strbad 1 2, Julie Cleuziou 1 2 3, Alfred Hager 4, Peter Ewert 4, Jürgen Hörer 1 2, Masamichi Ono 1 2 Eur J Cardiothorac Surg. 2021 Mar 30;ezab141. doi: 10.1093/ejcts/ezab141. PMID: 33783481;  DOI: 10.1093/ejcts/ezab141     Take Home Points:   Patients who need an intervention to manage the pulmonary circulation at neonatal period carry the risk for death. TAPVC is no longer a risk for mortality in this study when it is addressed at the first palliation.   Commentary by Dr. Yasuhiro Kotani (Okayama, Japan), section editor of Congenital Heart Surgery Journal Watch:   Summary:   A total of 53 patients with heterotaxy who underwent staged single ventricle palliation were included in this study. Thirty-five (66%) patients had a right ventricular dominance, common atrioventricular septal defect was present in 37 (70%) patients. Anomalous pulmonary venous drainage was present in 29 (55%) patients. Forty-six (86%) patients underwent first-stage palliation and, of these patients, there were 12 deaths (26%). A total of 41 patients, including those who did not have first palliation, received a bidirectional cavopulmonary connection (BCPC). There were 4 deaths after BCPC and 31 (58%) patients received total cavopulmonary connection (TCPC). Overall survival rate was 92% at 1 month, 74% at 1 year and 68% at 10 years. No patient died after TCPC. Independent risk factors for mortality in the multivariate Cox regression were a presence of restrictive pulmonary blood flow (HR 3.23; 95% CI 1.02–10.2; P = 0.05) and greater than mild atrioventricular valve regurgitation (HR 3.57; 95% CI 1.27–10.0; P = 0.02). Total anomalous pulmonary venous connection was not identified as a risk factor in this study. Mortality in patients with single ventricle and heterotaxy are high and this was often seen in the first 6 months of life.   Significance:   Although previous studies showed TAPVC as a risk factor for death, this study demonstrated that TAPVC is no longer a risk when it is intervened from the first palliation which makes us think of alternating the timing of TAPVC repair. Most of deaths and reoperations occurred after first palliation but in other words, the patients do well once they overcome all adverse events during the first 6 months of life.   Comment:   We all know that patients with heterotaxy do badly compared to other form of single ventricle. The study from Munich, Germany showed the consistent findings with high mortality and morbidity. In total of 53 patients, 46 patients had a first palliation, mainly to control the pulmonary blood flow. There were 7 early 5 late deaths, i.e., 26% of mortality in patients who had a first palliation. They stated the cause of death was cardiac in all patients. Although no details were shown, one can speculate that low systemic perfusion due to the imbalanced pulmonary blood flow was a main reason as they demonstrated restrictive pulmonary blood flow which need to be addressed as a risk factor for mortality.  Seven patients had a balanced pulmonary circulation which did not require intervention at neonates and this is sub-group that does well regardless of interventions. They intervene TAPVC at the first palliation and only 2 of 14 (14%) had reintervention for PVO.   This excellent outcome of TAPVC contributed the finding that TAPVC was not identified as a risk for death. As the authors mentioned, it is quite difficult to manage the balance between pulmonary and systemic circulation at neonatal period, especially after surgical/catheter intervention. Although many factors, such as pulmonary blood flow and ventricular function/atrioventricular valve regurgitation are interactively affected to the pulmonary/systemic circulation, this study did not investigate the relationship. Therefore, the study focusing on this is necessary.

Management of Complex Left Ventricular Outflow Tract Obstruction: A Comparison of Konno and Modified Konno Techniques. Haider M, Carlson L, Liu H, Baird C, Mayer JE, Nathan M.Pediatr Cardiol. 2021 Mar;42(3):614-627. doi: 10.1007/s00246-020-02522-9. Epub 2021 Feb 8.PMID: 33555370 Free PMC article.   Commentary by Dr. Luis Quinonez (Boston USA), section editor of Congenital Heart Surgery Journal Watch:   Summary   Purpose:  To compare outcomes of the Konno procedure to the Modified Konno procedure   Population:  122 patients, single institution, 1990 to 2014   Intervention:  Konno (n=51) and modified Konno operations (n=71)   Design:  Retrospective review   Primary outcome:  LVOT re-intervention   Secondary outcomes:  Overall re-interventions; transplant-free survival; composite of re-interventions and transplant-free survival; hospital mortality and major adverse events; hospital length of stay.   Results:   Median age, Konno vs. modified Konno: 8.2 years vs 3.9 years (p=0.03)   Median Follow-up: 8 years   Patients with multiple left sided lesions more likely to have Konno (p=0.017)   Hospital outcomes (Konno vs. modified Konno):  No statistical difference: mortality (7.8% vs. 4.2%), major adverse events, permanent pacemaker (11.8% vs. 9.9%)   Multivariate analysis:  Konno had longer LOS. Patients with multiple left sided lesions: more complications (48%), more surgical reinterventions (22.9%); longer ICU and hospital LOS   Post-Discharge outcomes, Konno vs. modified Konno: LVOT reintervention at 10 years:  11% vs. 47% (p=0.002) Multivariate analysis:  Modified Konno risk factor for LVOT intervention Overall reintervention at 10 years:  44% vs. 60% (p=0.114) Transplant free survival at 10 years:  92% vs. 85% (p=0.188) Multivariate analysis:  Konno or modified Konno are not risk factors for survival.   Comment   This paper is a large, single institution experiences comparing outcomes of Konno against modified Konno procedures with long-term follow-up.  The results are mostly intuitive, in that Konno patients were operated at an older age; they had less LVOT interventions; and patients with multiple left sided lesions fare worse.  Despite modified Konno patients having more LVOT interventions, the overall rate on interventions was similar in both groups over time.  Both groups would have reinterventions on the RVPA conduit, but patients who had Konno procedures would have more MV interventions, as they were more likely to have multiple left sided lesions.  Being retrospective, underlying surgical selection biases are reflected:  modified Konno in younger patients to try to preserve the aortic valve; and Konno in patients with multiple left sided lesions.   The study also demonstrates a significant operative mortality for the groups, as well as need for permanent pacemaker for both procedures.  Although the overall reintervention rate and transplant free survival was not statistically different between the two procedures, the absolute numbers favour the use of the Konno, which is worth noting.   Patients with LVOT obstruction continue to remain a surgical challenge so these data are worth reviewing.  The literature review provided is helpful.   Take Home Messages   In patients who undergo Modified Konno operation for LVOT obstruction, there is a significantly higher rate of LVOT reintervention compared to Konno operation. Transplant free survival is statistically similar for Konno and modified Konno operations. The use of the Konno operation is associated with patients who have multiple left sided lesions.

Blood flow characteristics after aortic valve neocuspidization in paediatric patients: a comparison with the Ross procedure. Secinaro A, Milano EG, Ciancarella P, Trezzi M, Capelli C, Ciliberti P, Cetrano E, Curione D, Santangelo TP, Napolitano C, Albanese SB, Carotti A. Eur Heart J Cardiovasc Imaging. 2021 Feb 7:jeab009. doi: 10.1093/ehjci/jeab009. Online ahead of print.PMID: 33550364   Take Home Messages: The Ozaki procedure is an alternative to the Ross procedure for AVR in children. Short-term hemodynamics of the Ozaki procedure are reassuring. Long-term results of the Ozaki procedure are lacking.   Commentary from Dr. Frederic Jacques (Québec City, Canada), section editor of Congenital Heart Surgery Journal Watch:   Background: Aortic valve replacement in children is challenging. Durable prosthesis that allows growth are lacking. The Ross procedure, the actual preferred replacement strategy (creating a double valve concern), offers a reliable aortic valve substitute with growth potential, but it is not devoted of technical challenges and long-term complications. Because of these limitations, an alternative strategy was recently borrowed by congenital surgeons to adult cardiac surgery. This strategy, the Ozaki procedure, literally consists of fashioning a stentless bioprosthesis within the aortic root with pericardium. Said differently, it is a leaflet extension (the less durable aortic valve repair technique) with its origin on the annulus.   Summary: In order to define the hemodynamic repercussions of these two aortic valve replacement strategies on the aorta, Secinaro et al. recruited 20 patients (10 per group) and performed a Cardiac MRI to assess flow eccentricity and wall shear stress. They report their findings in the February issue of European Heart Journal - Cardiovascular Imaging. The mean age of patients was 11 years old in each group. Imaging was performed in average 3 years after the Ross procedure and 4 months after the Ozaki procedure. Maximal gradient through the aortic orifice was 7 mmHg for Ross’s patients and 21 mmHg for the Ozaki procedure. Noteworthy regurgitation was present in 40% of patients regardless of the procedure. At the sinotubular junction, 60% of Ross’ had mild eccentricity and 10% of Ozaki’s had marked eccentricity. The remaining had central flow. According to the authors, there was no difference in wall shear stress between the two procedures.   Comment: Even if the timing of the performance of the MRI is earlier for the Ozaki procedure group (i.e., less likely to be exposed to deterioration) the reader can appreciate that the hemodynamic performance of the valve (although still within the normal range) is already less favorable than with the Ross procedure. In fact, even though there is less patients with the Ozaki procedure showing mild eccentricity than with the Ross procedure, it shows marked eccentricity in some. Is this the reflection of the material used or is it the repercussions of the inability of surgeons to replicate the “perfection” of a normal native aortic valve? Once again, the Ozaki procedure could be fairly described as a bioprosthesis art crafted within the aorta with the clamp on. Nevertheless, the finding of this stress is in accordance with what is already known for structural degeneration of bioprosthesis and that of leaflet extension. Will this confer less durability to the Ozaki procedure compared to the Ross in the long-term? We will find in a few years. On the other hand, these findings are somewhat reassuring, at least for the short term, regarding the hemodynamics of the Ozaki procedure. Even if it does not beat alternatives such as the Ross, it may actually offer a safe transition to a more definitive surgical replacement (likely adult size manufactured prosthesis) at a later turn. If this is the case, it will already be a major addition to our armamentarium.   

Left heart growth and biventricular repair after hybrid palliation. Sojak V, Bokenkamp R, Kuipers I, Schneider A, Hazekamp M. Interact Cardiovasc Thorac Surg. 2021 Feb 6:ivab004. doi: 10.1093/icvts/ivab004. Online ahead of print. PMID: 33547474   Take Home Points: Hybrid procedure facilitates both Aortic valve/LVOT and LV growth regardless of the level of hypoplasia. Long-term follow up is required to see the benefit of pursuing the biventricular physiology in oppose to single ventricle physiology.   Commentary from Dr. Yasu Kotani (Okayama, Japan), section editor of Congenital Heart Surgery Journal Watch:   Summary: A total of 33 infants with borderline LV (Borderline LV: N=19, Severe AS/LVOTO: N=14) underwent hybrid procedure at median age of 11days at 3.5kg. During the interstage period of 62 days, 7 catheter-based and 7 surgical-based reintervention were performed. Patients with borderline LV had a significant increase in both LVEDVi and AoV/LVOT. Similarly, patients with small AoV/LVOT achieved adequate growth of both LVEDVi and AoV/LVOT. Twenty-seven patients had a biventricular repair with 16 patients had aortic arch repair, ventricular septal defect closure, and relief of subaortic stenosis, 5 patients had Ross-Konno procedure, 5 patients had Yasui procedure, and 1 patient had AVSD and aortic arch repair. Twenty-three (85%) patients are alive at median follow up of 3.3 years. There were 22 reinterventions and 15 reoperations.   Significance: Hybrid procedure has been developed as the first palliation of hypoplastic left heart syndrome but it is well known that this procedure can be fit to the patients with borderline LV in the aim for LV growth. This paper showed that 90% of patients with borderline LV achieved biventricular repair. More interestingly, it demonstrated that hybrid procedure can facilitate multi-level hypoplasia (AoV, LVOT, and LV) regardless of initial anatomy (either small AoV/LVOT or small LV volume).   Commentary: Previous papers reported that hybrid procedure facilitated the LV growth and subsequently achieved biventricular repair which is no doubt. This paper from the one of the leading centers in Netherland also showed a high rate (90%) of the achievement of biventricular repair. They are quite aggressive that 5 out of 27 patients had Ross-Konno procedure to achieve biventricular repair. It is important to emphasize that 15 % died and 25% required reintervention after biventricular repair within relatively short-term period of 3 years.   This result makes us consider that the data should be carefully interpreted. Firstly, the paper did not describe the detail of the atrial communication which is very important. An exact size and how restrictive (pressure gradient between LA and RA) to adjust ASD are crucial to let the blood go into the LV, hence LV are under the circumstance to grow and this may alter the degree of the growth. Secondly, they showed a significant increase in AoV, LVOT, and LV volume, however, it seems some patients still fell from the criteria of biventricular size by looking at the figure. Although they describe the criteria, the final decision to go for the biventricular repair might be taken intraoperatively by surgeon, hence the selection bias may be existing. Finally, this study did not have the control group that single ventricle repair being performed. Taken together, long-term follow up is necessary to see the advantage of biventricular repair, including survival, freedom from reoperation, and functional status compared to single ventricle repair.   

Management of Complex Left Ventricular Outflow Tract Obstruction: A Comparison of Konno and Modified Konno Techniques Mahwish Haider, Laura Carlson, Hua Liu, Christopher Baird, John E. Mayer, Meena Nathan Pediatr Cardiol. 2021 Feb 8 : 1–14. doi: 10.1007/s00246-020-02522-9 [Epub ahead of print] PMCID: PMC7869422 ArticlePubReaderPDF–989K   Take Home Messages In patients who undergo Modified Konno operation for LVOT obstruction, there is a significantly higher rate of LVOT reintervention compared to Konno operation.Transplant free survival is statistically similar for Konno and modified Konno operations.The use of the Konno operation is associated with patients who have multiple left sided lesions.   Commentary from Dr. Luis Quinonez (Boston MA USA), section editor of Congenital Heart Surgery Journal Watch:   Summary Purpose: To compare outcomes of the Konno procedure to the Modified Konno procedure   Population: 122 patients, single institution, 1990 to 2014   Intervention: Konno (n=51) and modified Konno operations (n=71)   Design: Retrospective review   Primary outcome: LVOT re-intervention   Secondary outcomes: Overall re-interventions; transplant-free survival; composite of re-interventions and transplant-free survival; hospital mortality and major adverse events; hospital length of stay   Results: Median age, Konno vs. modified Konno: 8.2 years vs 3.9 years (p=0.03) Median Follow-up: 8 years Patients with multiple left sided lesions more likely to have Konno (p=0.017) Hospital outcomes (Konno vs. modified Konno): No statistical difference: mortality (7.8% vs. 4.2%), major adverse events, permanent pacemaker (11.8% vs. 9.9%) Multivariate analysis: Konno had longer LOS Patients with multiple left sided lesions: more complications (48%), more surgical reinterventions (22.9%); longer ICU and hospital LOS Post-Discharge outcomes, Konno vs. modified Konno: LVOT reintervention at 10 years: 11% vs. 47% (p=0.002) Multivariate analysis: Modified Konno risk factor for LVOT intervention Overall reintervention at 10 years: 44% vs. 60% (p=0.114) Transplant free survival at 10 years: 92% vs. 85% (p=0.188) Multivariate analysis: Konno or modified Konno are not risk factors for survival. Commentary: This paper is a large, single institution experiences comparing outcomes of Konno against modified Konno procedures with long-term follow-up. The results are mostly intuitive, in that Konno patients were operated at an older age; they had less LVOT interventions; and patients with multiple left sided lesions fare worse. Despite modified Konno patients having more LVOT interventions, the overall rate on interventions was similar in both groups over time. Both groups would have reinterventions on the RVPA conduit, but patients who had Konno procedures would have more MV interventions, as they were more likely to have multiple left sided lesions. Being retrospective, underlying surgical selection biases are reflected: modified Konno in younger patients to try to preserve the aortic valve; and Konno in patients with multiple left sided lesions.   The study also demonstrates a significant operative mortality for the groups, as well as need for permanent pacemaker for both procedures. Although the overall reintervention rate and transplant free survival was not statistically different between the two procedures, the absolute numbers favour the use of the Konno, which is worth noting.   Patients with LVOT obstruction continue to remain a surgical challenge so these data are worth reviewing. The literature review provided is helpful.   

Outcomes in Anomalous Aortic Origin of a Coronary Artery Following a Prospective Standardized Approach. Molossi S, Agrawal H, Mery CM, Krishnamurthy R, Masand P, Sexson Tejtel SK, Noel CV, Qureshi AM, Jadhav SP, McKenzie ED, Fraser CD Jr.Circ Cardiovasc Interv. 2020 Feb;13(2):e008445. doi: 10.1161/CIRCINTERVENTIONS.119.008445. Epub 2020 Feb 13.PMID: 32069111   Take Home Points: Surgical unroofing of anomalous aortic origin of a coronary artery cannot be offered to all patients and alternatives are needed. Transection and reimplantation of anomalous aortic origin of a coronary artery offer good results at a 4-year follow-up. After surgical repair, ˜ 90% of patients with anomalous aortic origin of a coronary artery can be cleared for exercise.     Commentary from Dr. Frederic Jacques (Quebec City, QC, Canada), chief section editor of Congenital Heart Surgery Journal Watch: Summary: Surgical correction of anomalous aortic origin of a coronary artery is usually offered to symptomatic patients and high-risk asymptomatic patients in order to correct or prevent ischemia, and to prevent sudden death. The creation of an unobstructed coronary blood inflow from the appropriate sinus of Valsalva is the goal. Unroofing of the intramural and interarterial course of the coronary artery is the most common surgical procedure performed for such anomaly. However, it is not always possible to perform it due to anatomic variants, and it is not devoted of postoperative risks including coronary events, coronary reinterventions, and aortic insufficiency. Bonilla-Ramirez et al. compared their results with a transection and reimplantation technique in 16 patients to 45 unroofings at Texas Children’s Hospital. Preoperatively, about 45% had exertional symptoms and 30% nonexertional symptoms (leaving another 25% asymptomatic). Objective ischemia was demonstrated by stress cardiac MRI in 46%. Compared to unroofing, the transection and reimplantation technique required longer cardiopulmonary and aortic cross-clamp time by about one third. One patient with the transection and reimplantation technique had recurrent ischemia mandating CABG at a median follow-up of 4 years (compared to none among unroofing). One patient with unroofing had recurrent sudden cardiac arrest due to a previously unrecognized myocardial bridge. This patient had a redo with the transection and reimplantation technique and did well thereafter according to the authors. Over 90% of patients were cleared for exercise in both groups after surgical repair and postoperative non-invasive assessment. The transection and reimplantation led to the demonstration of less postoperative ischemia than the unroofing.   Comment: Surgeons dealing with anomalous aortic origin of a coronary artery know that unroofing —although favored— is not always possible. This paper from Bonilla-Ramirez et al. (along others such as Gaillard M. et al. featured in November 2020), is reassuring as it states that other ways of creating an unobstructed coronary inflow in such anomalies can result in good mid-term outcomes. The authors describe their decision not to perform unroofing for 3 categories of patients: 1) the course of the coronary artery is below the commissure, 2) when unroofing does not relocate the ostium to the appropriate sinus, and 3) when unroofing results in compression by the intercoronary pillar. Stating their rationale, they also express that they do not use osteoplasty techniques and that they do not create aortic buttons. The fact that one patient with unroofing and recurrent symptoms was treated by the alternative technique of transection and reimplantation highlights the facts that not all patients have the same baseline anatomy and that likely the repair should be tailored to the individual problem rather than trying to fit every anatomical variant with a standard technique. Interestingly, the failure of a post-reimplantation technique leading to CABG highlights that surgical manipulation of coronary origins is not devoted of potential complications. It makes us wonder if creating an aortic button may have prevented such complication. As the authors suggest, postoperative assessment by provocative myocardial ischemia testing should be performed before allowing exercise to prevent complications. As most studies have a limited follow-up (about 5 years), further studies with longer follow-up will be needed to better define the freedom from ischemic events among repaired anomalous aortic origin of a coronary artery. No question, we need large multicentric studies with long follow-up to better understand what technique should be applied to what anatomical variant and what should be expected or prevented in these patients down the road.    

Subcoronary Ross/Ross-Konno operation in children and young adults: initial single-centre experience. Murin P, Weixler VHM, Moulla-Zeghouane J, Romanchenko O, Schleiger A, Lorenzen V, Sinzobahamvya N, Zacek P, Photiadis J, Cho MY.Eur J Cardiothorac Surg. 2021 Jan 4;59(1):226-233. doi: 10.1093/ejcts/ezaa307.PMID: 33141218   Take Home Points: Subcoronary Ross/Ross-Konno operation is a choice for children who are not indicated for graft-enforced Ross procedure. Size mismatch between aortic and pulmonary annulus is not a problem as the aortic annulus and LVOT can be enlarged with Konno incision +/- additional patch in the non-coronary sinus.     Commentary from Dr. Yasuhiro Kotani (Okayama, Japan), section editor of Congenital Heart Surgery Journal Watch: Summary: A total of 50 patients with median age of 6.3 years who underwent subcoronary Ross/Ross-Konno operation was reviewed. After the harvest, the autograft was trimmed just to leave some tissues for the implantation. The annulus was enlarged when necessary. Konno incision was performed in 42 (84%) patients, including 24 patients with LVOTO and/or annular hypoplasia and 18 patients with a size mismatch between aortic and pulmonary annulus. An additional patch for non-coronary sinus was used in 17 (34%) patients. The autograft rotation was decided based on the orientation of the coronary artery ostium. Overall survival was 95%. Freedom from aortic valve regurgitation more than mild was 97% at 5years and only 2 (4%) patients required aortic valve replacement. No aortic root dilation was seen in the mid-term follow-up. Significance: Aortic valve surgery in children and young adults has several options, such as Ross procedure and aortic valve replacement with or without Konno, however, it is still in debate what procedure to be selected as the reconstructed aortic valve and the root should be grown. Subcoronary Ross/Ross-Konno procedure may be the answer that overcomes the disadvantages of aortic valve replacement and Ross procedure. Comment: No standard care was shown in the surgical treatment of the aortic valve in children and young adults. These patients are at age of somatic growth and this makes the choice of surgery difficult. Aortic valve replacement with or without Konno promises a longevity unless adverse events related to the prosthetic valve happen. Patients who are athletes and female may not be suitable as it requires warfarin in lifetime. In contrast, Ross procedure has a benefit for a potential growth of the autograft and no need for anticoagulation, however it creates 2-valve diseases and the dilatation of the implanted autograft remains an issue, especially for patients with a bicuspid valve. As originally described by Dr. Ross in 1967, subcoronary Ross should be taken into reconsideration. There are certainly some technical tips but it might be theoretically better option for children and young adults for potential growth without root dilatation. Size mismatch between aortic and pulmonary root can be always addressed by additional Konno-incision and patch for non-coronary sinus and fits for small children before school age.    

Survival Outcomes of Two-Stage Intracardiac Repair in Large Ventricular Septal Defect and Trisomy 18. Nakai R, Fujioka T, Okamura K, Suzuki T, Nakao A, Kobayashi J, Tsuchiya K. Pediatr Cardiol. 2021 Jan 29. doi: 10.1007/s00246-021-02546-9. Online ahead of print. PMID: 33515091   Take Home Messages: Patients with Trisomy 18 and congenital heart disease are offered surgical repair. Cardiac surgical repair improves survival in patients with Trisomy 18. For patients with Trisomy 18 and VSD, PAB followed by intracardiac repair (two-stage approach) offers improved survival.   Commentary from Dr. Luis Quinonez (Boston, MA, USA), section editor of Congenital Heart Surgery Journal Watch: Summary: The authors present a retrospective review of their experience treating 18 patients (2 male, 16 female) with Trisomy 18 and VSD using a two-stage approach (2005-2019). Their approach was placing a PAB as a neonate or infant, followed months later by a complete intracardiac repair with PAB takedown. The gestational age and birth weight were 38 ± 2 weeks and 1861 ± 325 g. Pulmonary artery banding was placed after a median duration of 19.5 (range 6–194) days of life. The age and body weight at the time of intracardiac repair were 18.2 (7.6–50.7) months and 6.0 ± 1.0 kg. There was one hospital death.   Following intracardiac repair, CPR was performed in two patients. Other complications included pneumonia (n = 1), chylothorax (n = 1), pleural effusion (n = 1), and pneumothorax (n = 1). Two patients needed temporary peritoneal dialysis following VSD closure. Follow-up was 24.9 (3.9–173.0) months. The 1- and 5-year survival rates after VSD repair were 88.5% (95% confidence interval, CI 61.4–97.0) and 77.5% (95% CI 42.8–92.8). This survival was significantly better than a comparison group who was palliated with a PAB only. The authors provide details of 3 other cases who underwent complete repair as their first operation, and all died. The authors conclude that “two-stage ICR for large VSDs in patients with T18 improves their life expectancy. Offering palliative surgery during the early neonatal period followed by complete repair after sufficient weight gain is safe and improves survival.”   Comment: Many institutions offer cardiac surgical repair to patients with Trisomy 18. Any ethical discussion surrounding this practice is beyond the scope of this commentary. Neonates with Trisomy 18 and congenital heart disease pose many challenges related to associated comorbidities. They are often small for gestational age; may be premature; and may have associated airway disease, just to name a relevant few. The authors of the paper advocate for a two-stage repair of VSDs beginning with a PAB, followed later by complete intracardiac repair. The justification for such a practice seems mostly related the low weight of the patients in their cohort and the poor experience with complete repair (although only in 3 patients).   The authors have good follow-up survival outcomes using the two-stage approach, although morbidity was significant. Other experienced centers may advocate performing complete intracardiac repair from the outset, arguing that complete repair is feasible in a low weight neonate/infant and the morbidity and mortality of the single procedure is similar or less than a staged approach. The differences in approach (staged vs. complete repair) between centers will most likely be related to the comfort level of the surgeon in performing an intracardiac repair in a low birthweight neonate and the experience of the center in managing these small patients with multiple comorbidities. Regardless of the approach taken, the post-operative care of patients with Trisomy 18 can be challenging. Post-operative morbidities are significant, including the need for tracheostomy and gastrostomy. Whatever approach is taken, staged or not, the treating institution needs to commit expertise and resources to manage patients with Trisomy 18.