Congenital Heart Surgery

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.