Congenital Heart Surgery

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

In-Hospital Outcomes in Fontan Completion Surgery According to Age. Ghimire LV, Chou FS, Pundi K, Moon-Grady AJ.Am J Cardiol. 2022 Mar 1;166:81-87. doi: 10.1016/j.amjcard.2021.11.019. Epub 2021 Dec 18.PMID: 34933746   Take Home Points: Age at Fontan completion does not adversely affect the in-hospital outcomes. Commentary from Dr. Shuhua Luo (Chengdu, Sichuan, China), section editor of Congenital Cardiac Surgery Journal Watch: Summary: A total of 6,647 children with median age 3 (interquartile range 2 to 4) years underwent the Fontan completion procedure were retrospectively included in this study. The patients were categorized into 1 of 5 groups according to their age at the time of Fontan completion: <2(n=956, 14.4% ), 2(n=2190, 32.9%), 3(n=1634, 24.6%), 4(n=829, 12.5%), and ≥5 years(n=1039, 15.6%). The in-hospital mortality rate for the whole cohort was 2.1% (n = 139), and 1.8% (n = 117) needed ECMO during the ICU stay. Independent risk factor for mortality in the multivariable logistic regression was a presence of atrioventricular septal defect (AVSD) [OR4.3 (2.4 to 7.9), p <0.001]. Age was not identified as a risk factor in this study.   Comment: Age older than 4 years at the time of Fontan is one of the original “Ten Commandments” for case selection. In more recent years, the Fontan operation has been performed in younger patients. However, the impact of patient age on outcome remains unclear. A recent large retrospective study(n=3319) showed a U-shaped relationship between age and in-hospital mortality, with the lowest risk when the procedure was performed at age 3(1). While other authors suggested that age is not significantly associated with postoperative outcomes (2,3). The current study supported the latter finding in a large multi-institutional population. However, one should interpret this result with caution, as the risk of needing ECMO was significantly higher in those that received the Fontan surgery before 2 years of age with an adjusted OR of 3.1 (95% CI 1.4 to 7.0). As expected, home health care resource utilization was the highest in patients who underwent surgery at <2 years of age (13.7%), compared with 5.6% in the 3-year-old group (p <0.001). These results suggested patients with younger ages (<2 years) may have a complicated postoperative recovery. Furthermore, the occurrence of early Fontan failure was not reported in this study. Unfortunately, other important demographic(e,g weight), physiologic (e.g pre-Fontan arterial oxygen saturation), and anatomic(e.g heterotaxy) risk factors were also not included in the multivariable analysis. With the modification of surgical technique and advancement of perioperative management, age might not be as important as we thought in the era of “Ten Commandments”. The clinical decision-making of timing of Fontan completion should be individualized and always balance the associated benefits and risks. A large multicenter prospective longitudinally follow-up study might help us to generate new evidence regarding Fontan patients' selection.   Reference 1. Marathe SP, d’Udekem Y, Winlaw DS. What Is the Ideal Age for the Fontan Operation? Ann Thorac Surg. 2020 Sep;110(3):1095–6. 2. Salvin JW, Scheurer MA, Laussen PC, Mayer JE, Del Nido PJ, Pigula FA, et al. Factors associated with prolonged recovery after the fontan operation. Circulation. 2008 Sep 30;118(14 Suppl):S171-176. 3. Hosein RBM, Clarke AJB, McGuirk SP, Griselli M, Stumper O, De Giovanni JV, et al. Factors influencing early and late outcome following the Fontan procedure in the current era. The “Two Commandments”? Eur J Cardio-Thorac Surg Off J Eur Assoc Cardio-Thorac Surg. 2007 Mar;31(3):344–52; discussion 353.    

Successful Reduction of Postoperative Chest Tube Duration and Length of Stay After Congenital Heart Surgery: A Multicenter Collaborative Improvement Project. Bates KE, Connelly C, Khadr L, Graupe M, Hlavacek AM, Morell E, Pasquali SK, Russell JL, Schachtner SK, Strohacker C, Tanel RE, Ware AL, Wooton S, Madsen NL, Kipps AK.J Am Heart Assoc. 2021 Nov 2;10(21):e020730. doi: 10.1161/JAHA.121.020730. Epub 2021 Oct 29. PMID: 34713712    Take Home Points: Using collaborative learning methodology across 9 centers, statistically significant decreases in the postoperative CT duration and reduction in LOS were successfully achieved without any increases in adverse events. Collaborative learning is an effective methodology to reduce variation across centers and ultimately improve patient outcomes. Commentary from Dr. Masamichi Ono (Munich, Germany), section editor of Congenital Heart Surgery Journal Watch: Summary: Nine pediatric heart centers collaborated through 2 learning networks. Patients undergoing one of 9 benchmark congenital heart operations were included (patients who underwent the Fontan operation were excluded). Baseline data were collected from June 2017 to June 2018, and intervention-phase data were collected from July 2018 to December 2019. Collaborative learning methods included review of best practices from a model center, regular data feedback, and quality improvement coaching. Center teams adapted CT removal practices (eg, timing, volume criteria) from the model center to their local resources, practices, and setting. Postoperative CT duration in hours and LOS in days were analyzed using statistical process control methodology. Overall, 2309 patients were included. Patient characteristics did not differ between the study and intervention phases. Statistical process control analysis showed an aggregate 15.6% decrease in geometric mean CT duration (72.6 hours at baseline to 61.3 hours during intervention) and a 9.8% reduction in geometric mean LOS (9.2 days at baseline to 8.3 days during intervention). Adverse events did not increase when comparing the baseline and intervention phases: CT replacement (1.8% versus 2.0%, P=0.56) and readmission for pleural effusion (0.4% versus 0.5%, P=0.29).   Significance: Introducing collaborative learning model, they could shorten the duration of chest tube, and ICU length of stay in patients after 9 benchmark CHD operations. Collaborative learning model included regular data feedback on practices and outcomes using a shared data-reporting platform, monthly webinars to share intervention ideas and results, and quality improvement coaching. These efforts enabled to improve the postoperative management.   Comment: Congenital heart disease practices and outcomes vary significantly across centers, including postoperative chest tube (CT) management, which may influence postoperative length of stay (LOS). In this study, they showed collaborative learning methods to determine whether centers could adapt and safely implement best practices for CT management, resulting in reduced postoperative CT duration and LOS. As results, the centerline CT duration was decreased from 72.6 to 61.3 hours, representing a 15.6% reduction. As for ICU LOS, the centerline LOS was decreased from 9.2 to 8.3 days, representing 9.8% reduction. There was no difference between baseline and intervention phases concerning frequency of CT replacement or hospital readmissions within 7 days because of pleural effusion. To shorten the postoperative ICU LOS, not only standardized management algorithm, but also collaborative learning across the center, play an important role.   They commented that the relationship between CT duration and LOS might vary depending on operation and patient age. For example, the LOS for a newborn following a Norwood operation is likely impacted more by the need to establish enteral feeding and preparation for inter-stage home monitoring than by CT duration, given the large gap between CT duration and LOS shown in our previous work. We agree with this comment in patients undergoing neonatal Norwood procedure.   Further expansion of the multicenter collaborative leaning structure might create more effective management protocol.   In this study, patients undergoing the Fontan procedure were excluded. As we all know, post-operative chest tube management is the most important issue in these patients. Further studies on this comfort are also expected.   

High-degree Norwood neoaortic tapering is associated with abnormal flow conduction and elevated flow-mediated energy loss. Schäfer M, Di Maria MV, Jaggers J, Stone ML, Ivy DD, Barker AJ, Mitchell MB.J Thorac Cardiovasc Surg. 2021 Dec;162(6):1791-1804. doi: 10.1016/j.jtcvs.2021.01.111. Epub 2021 Feb 4. PMID: 33653609   Take Home Points: Geometry, i.e., smooth narrowing of the reconstructed aorta from the ascending to the descending aorta is more important than hemodynamics parameters, such as pressure difference. Impact of arch growth on change in energy loss is not fully understood and further study to see a serial assessment in this entity is necessary. Commentary from Dr. Yasuhiro Kotani (Okayama, Japan), chief section editor of Congenital Heart Surgery Journal Watch: Summary: A total of 26 patients who underwent Norwood operation was included in this study. Patients were evaluated by 4-dimentional-flow magnetic resonance imaging and were then divided based on the degree of neoaortic tapering defined as the standard deviation of diameter values measured at the ascending aorta, transverse arch, aortic isthmus, and the descending aorta. The median tapering value of neoaortic was 0.52cm and patients were divided into 2 groups: high degree (>0.52cm)(Group H: n=13) and low degree (≦0.52cm)(Group L: n=13). Group H had a higher prevalence of abnormal recirculating flow in the neoaorta and elevated neoaortic flow-mediated viscous energy loss in the ascending aorta (Group H: 1.0±0.4 vs. Group L: 0.6±0.3 mW, p=0.004). The study concluded that oversizing neoaortic reconstruction should be avoided from the point of ventricular-aortic coupling in patients undergoing Norwood operation.   Comment: Previous studies showed the shape of reconstructed neoaortic arch is important in terms of the afterload of systemic ventricle in HLHS patients which impacts on RV dysfunction and subsequent long-term outcomes. This study showed that suboptimal neoaortic geometry, i.e., size discrepancy among the ascending, transverse, and descending aorta, was associated with an increased flow-mediated viscous energy loss and an increased wasted RV mechanical power. This is the first study to evaluate neoaortic flow hemodynamics in a sizable group of patients with HLHS having Norwood operation. Importantly, no patients in this study had a residual hemodynamic coarctation which makes us think of the importance of cardiac MRI assessment in this particular patient group.   This study raised a couple of questions. One is do we need to routinely use MRI assessment in all patients after Norwood operation. Because of increased evidence of MRI assessment for flow dynamics and its influence on cardiac function, it is ideal to perform MRI assessment. On the other hands there are still some problems, including the costs of the examination and availability (not all hospitals have facility and staffs for cardiac MRI). Another question is that how to implicate this result in the clinical practice. This study highlighted the importance of geometry rather than physiology as even patients without no evidence of residual hemodynamic recoarctation showed higher energy loss if size discrepancy between the ascending aorta and descending aorta existed. Taken together, the lessons learned from this study is that surgeons need to reconstruct the neoaortic arch in a good shape no matter other physiologic parameters give us a green light. But again, the problem is achieving this goal is challenging due to the complex patch shape in highly variable anatomy in HLHS.   

Applicability and Durability of Valve-Sparing Tetralogy of Fallot Repair. Stephens EH, Wolfe BL, Talwar AA, Patel A, Camarda JA, Eltayeb O, Monge MC, Forbess JM.World J Pediatr Congenit Heart Surg. 2021 Sep;12(5):628-634. doi: 10.1177/21501351211031242.PMID: 34597206   Take Home Points: The ideal repair of TOF is defined as the repair that results in the lowest RVOT gradient and the maximal pulmonary valve competence. Two strategies are available to manage the right ventricular outflow tract (RVO) during TOF repair: transannular patch and valve-sparing. Several factors affect the decision to chose one strategy versus the other. Some of these factors are based on preoperative echocardiographic data, and some from intraoperative RVOT assessment. Pulmonary valve annular Z score, while helpful, is just one factor among many that affects the decision to choose a transannular patch or valve-sparing approach. Surgeon’s judgement and experience remain of paramount importance in this decision-making process. Commentary from Dr. Sameh Said (Minneapolis, MN, USA), Guest Editor of Congenital Heart Surgery Journal Watch: In this article by Stephens et al, the authors asked an important clinical and practical question. When it comes to managing the right ventricular outflow tract (RVOT) during repair of tetralogy of Fallot (TOF), which technique should be used? Transannular patch (TAP) versus Valve-sparing (VS)? And how to select the right technique for the right patient? and on what basis this selection should be done, preoperative data and/or intraoperative decision?   Their retrospective review included 67 patients (17 underwent TAP and 50 underwent VS) with a mean age of 4.5 months and a mean weight of 5.8 Kg. The authors described their management algorithm which highlights that the final decision is made based on intraoperative assessment of the pulmonary valve (PV) cusps mobility, and quality in addition to the z score of the annulus. They used a cut off limit of -2 to predict the need for TAP. They also use right ventricular (RV) pressure >70% of systemic pressure after coming off cardiopulmonary bypass to determine the need for a TAP despite an initial adequate repair.   As expected, those who needed a TAP had much smaller preoperative echocardiographic Z score compared to those underwent a VS approach. Interesting enough that 34% of those who had VS repair, had a z score < -2.5 (14% with z score <-3 and 20% with a z score between -2.5 to -3). Overall, 52% of those with preoperative Z score < -2.5 underwent VS repair.   At discharge, the VS group had higher peak gradient with less regurgitation compared to the TAP group. This peak gradient and degree of regurgitation remained stable in the VS group during a median follow-up of 58 months, while those with TAP remained with free regurgitation. Both groups, however, had similar PV z scores indicative of annular growth over time.   The authors defined an “ideal” long-term repair as peak gradient < 25 mmHg and no more than mild PR on most recent echocardiogram.   I would like, however, to point out few important points:   The authors did not describe their TAP technique and how they manage the native pulmonary valve? And if there are any technical points they consider when designing the patch such as its width and/or the orientation of the commissures of the PV cusps. Overall, 15% of these patients had residual atrial level shunt. Was that left intentionally? Most of the patients in the series are in the typical age and weight for TOF repair and we usually leave a residual atrial level shunt mainly in neonates or those with anticipated post-repair right ventricular diastolic dysfunction or persistent elevated RV pressure. Despite the authors mentioned post-repair RV/systemic pressure ratio <70% as one of their goals for VS, no data is provided about the post-repair RV pressure among the current cohort. The goal of having RV pressure post-repair that is < 70% of the systemic pressure seems to be based on old data. If the post-repair RV pressure under anesthesia close to 70% that may result in systemic RV pressure at time of discharge and could result in earlier reintervention. This in my opinion an important variable that may be superior to the PV annular z score itself due to the obvious limitations of the Z score. It would have been quite helpful to see a correlation between the post-repair RV/systemic pressure ratios and the PV annular z score and to see if this can correlate/predict reintervention later. It is unclear which system the authors used or followed in regard to the use of the PV annular z score. The authors proposed a cut-off z score of -2 to define a durable and successful VS repair. I do not believe such conclusion can be made with certainty from the current study due to the lack of details of other factors that may affect the durability and reinterventions after TOF repair such as PV cusps analysis, and the lack of long-term data. While the follow-up echocardiographic data demonstrated higher peak gradient in the VS group and more PR in the TAP group, this did not translate into a significant difference in the reintervention rate between the two groups.   Finally, I would agree with the authors that PV annular z score is only one factor in the decision-making algorithm to chose VS versus TAP for those undergoing TOF repair. While the goal is to preserve valvular competence and lower post-repair RV pressures, this is not always easy to achieve and a longer-term data with larger group of patients are needed to determine the superiority of one approach versus the other.    

Mortality and Reoperation Risk After Bioprosthetic Aortic Valve Replacement in Young Adults With Congenital Heart Disease. Fuller SM, Borisuk MJ, Sleeper LA, Bacha E, Burchill L, Guleserian K, Ilbawi M, Razzouk A, Shinkawa T, Lu M, Baird CW. Semin Thorac Cardiovasc Surg. 2021 Jun 24:S1043-0679(21)00301-4.   Take Home Points: Smaller valve size indexed to BSA is a risk for reoperation. Younger age, especially <21 years old is associated with more reoperation. Commentary by Dr. Yasuhiro Kotani, MD, PhD, Okayama, Japan Congenital Heart Surgery section editor Summary: Data were retrospectively collected for 314 patients undergoing bAVR at 8 centers from 2000-2014. Average age was 45.2 years (IQR 17.8-71.1) and 30% were <21. Indications were stenosis (48%), regurgitation (28%) and mixed (18%). Twenty-eight (9%) underwent prior AVR. Median valve size was 23mm (IQR 21, 25). Implanted valves included CE (Carpentier-Edwards) Perimount (47%), CE Magna/Magna Ease (29%), Sorin Mitroflow (9%), St Jude (2%) and other (13%). Median follow-up was 2.9 (IQR 1.2, 5.7) years. Overall, 11% required re-operation, 35% of whom had a Mitroflow and 65% were <21 years old. Time to re-operation varied among valve type (p=0.020). Crude 3-year rate was 20% in patients ≤21. Smaller valve size indexed to BSA was associated with re-operation (21.7 vs. 23.5 mm/m2). Predictors of reintervention by multivariable analysis were younger age (29% increase in hazard per 5-year decrease, p<0.001), Mitroflow (HR=4 to 8 versus other valves), and smaller valve size (20% increase in hazard per 1 mm decrease, p=0.002). The overall 1, 3 and 5-year survival rates were 94%, 90% and 85% without differences by valve (p=0.19).   Significance: This study showed younger age at surgery as a risk for reintervention which is consistent finding from previous studies. From the risk analysis, a larger valve size, an older age (>21 years old), and the valve other than Mitroflow will be recommended in young adults with congenital heart disease.   Comment: It is quite surprising that overall mortality in such a short follow-up period (2.9 years) was 16.2% and high. Of note, more than one thirds of patients died of unknown cause. Overall reintervention rate was about 10% and this is not bad as expected, considering study population was median age of 45 years old. The Sorin Mitroflow required reintervention the most, accounting 45% even with relative short period follow-up of 2.5 years. St. Jude’s valve was used for the most youngest population about 9.8 years old but resulted in no reintervention. This surprises us as this study demonstrated younger age < 21years as a risk for reintervention. One of the reasons may be small number (N=6) of patients having St. Jude’s valve and further study is necessary for a strong conclusion. This study showed a larger valve size was at low risk for reoperations. Patients with no reintervention had more aortic annulus enlargement and concomitant procedure, such as aortic root replacement. Therefore, it might be important to implant a larger size valve even it requires complex procedure as it will lower the risk of reintervention. Approximately 80% of patients had only Aspirin after surgery. This is very important for patients’ QOL, especially in young population.    

Ghani MOA, Foster J, Shannon CN, Bichell DP. Association of chest tube position with phrenic nerve palsy after neonatal and infant cardiac surgery. J Thorac Cardiovas Surg. 2021 May;161(5):1618-1622.e1.   Commentary from Dr. Frederic Jacques (Quebec City, QC, Canada), chief section editor of Congenital Heart Surgery Journal Watch:  Screening Journals to create May 2021’s selection of congenital cardiac surgery papers, I found numerous interesting papers on ultra-specific topics. The reader is encouraged to look at this month’s list of abstracts as it contains insightful papers on the management of single ventricle patients, atrioventricular ventricular septal defect, tetralogy of Fallot, etc. Nevertheless, the topic I decided to feature is far less attractive, but so important: phrenic nerve palsy. This complication is relatively frequent (4%), has a major impact on patient’s clinical outcome, and is quite frustrating for the meticulous surgeons we all expect to be. Direct trauma to the phrenic nerves by both anesthetic and surgical manipulations are well recognized risk factors. Bichell and colleagues (Vanderbilt University, Nashville) postulated that pressure nerve damage could occur by indwelling chest tubes. The authors evaluated the effect of a strategy of avoiding chest tube positioning in proximity to the nerve within the apex of the right apical portion of the right pleural cavity.   All 531 operations performed under cardiopulmonary bypass in neonates and infants of less than 1-year of age by a single surgeon were assessed from January 2012 to June 2019. All chest tubes were 19 Fr Blake chest tubes connected to a Pleur-evac system. Positioning of the chest tube within the chest changed in September 2018. Before this hinge point, chest tubes were positioned far in the chest and looping apico-medially into the second intercostal space. After September 2018, this “danger zone” was avoid. Positioning of the tubes was assessed on chest roentgenograms by 3 blind examiners. Patients were divided into two groups according to the presence or absence of right diaphragm paralysis. The location of the tube (within or outside the danger zone) was compared to patient’s size at the time of surgery, the age, the dwelling time of the tube, the cardiopulmonary bypass time, the ventilation time, the length of stay in intensive care unit length of time and in hospital, the readmission rate and the 30-day mortality. Patients with phrenic palsy had a longer intubation time (20 vs. 3 days) and LOS (53 vs. 17 days). Fortunately, 30-day mortality was not affected. Of 5 variables considered in multivariable analyses, only chest tube position was significant for phrenic palsy. Avoiding the apical portion of the right pleural cavity was protective as no event occurred.   This paper is of great interest as diaphragm paralysis may affect all the spectrum of pediatric cardiac surgery. Having diaphragm paralysis may have catastrophic consequences on the management of single ventricle patients on whom stage 1 palliative strategies were recently performed. Secondary increased pulmonary vascular resistance in these patients may lead to instability and may require early diaphragmatic plication. On the other end of the spectrum, there is not much complication as frustrating as having a diaphragm paralysis after simpler surgeon such as isolated ventricular septal defect closure, to give an example. For all type of patients such complications seem avoidable and realizing that factors other than the intraoperative management may impact on diaphragmatic function is important. With the findings of this paper, every surgeon should beware the placement of chest tube within the right apical portion of the chest (and likely the left by extrapolation). Avoiding preventable complications is the goal for all surgeries and placing chest tubes in the apical portions of pleural cavities should likely be considered an error from now on.   

Factors associated with mortality or transplantation versus Fontan completion after cavopulmonary shunt for patients with tricuspid atresia Connor P. Callahan, MD,a Anusha Jegatheeswaran, MD, PhD,a David J. Barron, MD,a S. Adil Husain, MD,b Pirooz Eghtesady, MD, PhD,c Karl F. Welke, MD, MS,d Christopher A. Caldarone, MD,e David M. Overman, MD,f James K. Kirklin, MD,g Marshall L. Jacobs, MD,h Linda M. Lambert, MSN-cFNP,b William M. DeCampli, MD, PhD,i and Brian W. McCrindle, MD, MPH,j the Congenital Heart Surgeons’ Society Tricuspid Atresia Working Group. J Thorac Cardiovasc Surg. 2021 Apr 30;S0022-5223(21)00745-5. PMID: 34045062. Take Home Points: Failure to achieve Fontan completion in patients with tricuspid atresia is higher than expected (5%). Even cavo-pulmonary shunt (CPS) reduces the ventricular volume, mitral valve repair should be considered at the time of CPS, if moderate or greater. Commentary from Dr. Yasuhiro Kotani (Okayama, Japan), section editor of Congenital Heart Surgery Journal Watch:  Summary: A total of 417 patients younger than 3 months of age with tricuspid atresia were retrieved from 40 centers in the Congenital Heart Surgeon’s Society. Cavo-pulmonary shunt (CPS) was performed in 382 patients and 5% died or underwent heart transplantation (no Fontan operation). Prenatal diagnosis and pulmonary artery banding (PAB) at the time of CPS were negatively associated with Fontan completion. Preoperative moderate or greater mitral valve regurgitation (MR), concomitant mitral valve repair, PAB at CPS, postoperative superior vena cava interventions, and CPS takedown were associated with death/transplantation. Significance: This study identified a relatively higher rate of failed Fontan achievement in tricuspid atresia with normal great arteries which appeared to be favorable anatomy for Fontan completion. Risk factors for death/transplant included PAB at CPS, MR, and CPS takedown. This study makes us reconsider the patient selection and surgical indication for CPS. Comment: As we all know, TA without transposition of the great arteries is the most favorable condition of single ventricle and usually promises a “good” Fontan pathway. This study indeed showed 17 patients died and 1 patient needed transplantation (among a cohort of 417 patients) which was considered to be higher than expected. They showed PAB at the time of CPS was associated with worse outcome. Although there was no significant difference in mean PA pressure or trans-pulmonary gradient between patients with PAB and those without, patients with PAB had a high rate of branch pulmonary stenosis (38%) and this may affect the pulmonary condition. The impact of antegrade pulmonary flow by PAB at CPS is still in debate. Previous CHSS studies showed pros and cons with regard to leave the antegrade flow at CPS and no answer was made. There may be a patient selection bias and we do not know how this affected on worse clinical outcome. Mitral regurgitation (MR) is also identified as a risk for death/heart transplantation. This is consistent finding from previous literatures even CPS can decrease systemic ventricular volume and contributes to maintain ventricular function. This study recommended to intervene moderate or more MR to recruit the borderline Fontan candidates. This study demonstrated that 54 patients were alive without Fontan completion and this number is not negligible. Of these 10 patients were 3 years or more from CPS. Their echocardiogram showed good LV function except 1 patient and no data were available why these 10 patients did not undergo Fontan completion. Because of the nature of the multi-center retrospective study, the study did not find the reason why these patients did not go for Fontan completion. Further study to profile these patients is necessary to improve the achievement of Fontan completion.

Surgical management of Ebstein anomaly: impact of the adult congenital heart disease anatomical and physiological classifications Homzova L, Photiadis J, Sinzobahamvya N, Ovroutski S, Cho M-Y, Schulz A. Interact CardioVasc Thorac Surg 2021;32:593–600. Take home message: Physiological assessment of patients with Ebstein anomaly is important when referring patients for surgery Survival free from complications is lower for patients with a preoperative severe physiological condition (compared to a moderate one) Patients with Ebstein anomaly should undergo surgery before they reach a severe clinical condition Commentary from Dr. Frederic Jacques (Quebec City, QC, Canada), chief section editor of Congenital Heart Surgery Journal Watch:  Homzova L. et al. report on a cohort of 33 patients operated for Ebstein anomaly from 2000 to 2017. They divided the small group in two according to clinical preoperative presentation as defined by the adult congenital heart disease anatomical and physiological classification. As the reader knows, this classification comprises elements such as the NYHA classification, the exercise capacity, the presence of arrhythmias and pulmonary hypertension, and even end-organ dysfunction. It gives a physiological portrait of the patient rather than simply relying on the name of the diagnosis and its subtypes. They compared the 2-year survival of patients having a moderate or a severe pre-operative condition. They also compared postoperative major complications. Their main finding is that survival free from major adverse events was lower among patients with the worse preoperative condition, particularly in women. The survival free from major adverse events was 60% in the moderate group, compared to 38% in the severe group. In fact, only two patients died in the immediate postoperative period and both were female. Another female patient died 8 months after surgery. Seven patients required re-operations, and 5 required a pacemaker. Early tricuspid re-operation was required in 1 patient. One patient had a stroke and 1 required mechanical support. All of these patients were in the severe group. One patient of the severe group was listed for heart transplant. Survival free from re-operation was 72% in the moderate and 46% in the severe group. Of the survivors, 18% had improved to mild clinical condition, and the remaining 82% were in a moderate condition. Overall, the authors propose to consider operating patients before reaching a severe clinical condition. This study is small and limited in its ability to exert authority in our clinical decision making regarding Ebstein anomaly patients. Nevertheless, the authors should be commended in their efforts to improve our understanding of the physiological repercussions of a long-standing tricuspid valve anomaly. In fact, their study supports the assumption that most clinicians have: patients should be brought to the operating room before they reach a condition were the outcome will be significantly affected. For Ebstein anomaly, the indication and the appropriate timing for surgery are difficult to define. In fact, indication and the timing for surgery are difficult for the tricuspid valve in general. When patients are clinically well-enough, most cardiologists will defer referring to surgeons in the fear of potential postoperative complications. The problem is that when patients become “ripe enough” for surgery, the risk of complications increases in a steep manner. It is almost as if patients were traveling on a flat ground for a long period before falling off a cliff. Ideally, we should be able to catch them just before they do. The tipping point being hard to predict, this study is a reminder to seize the opportunity before reaching the cliff. In order to improve our clinical judgement when assessing these patients, the authors missed some opportunity. In truth, some clinically important elements are missing. For example, what was the liver function of these patients? Obviously, one of the organs affected the most by right heart dysfunction and tricuspid regurgitation is certainly the liver. In turns, its function affects significantly the outcome of any cardiovascular operation: vasoplegia, bleeding, volume management, etc. In order to improve care of these patients, and especially to better determine the best timing for surgery, we need to look at preoperative clinically relevant variables.

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.