Congenital Cardiac Anesthesia Society
A Section of the the Society for Pediatric Anesthesia

{“questions”:{“zman7”:{“id”:”zman7″,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Author: Melissa Colizza – CHU Sainte-Justine – Montreal, Quebec \r\n\r\nIn current era, what is the MOST common cardiac diagnosis in combined heart-liver transplant recipients? “,”desc”:”EXPLANATION \r\nThe first combined heart-liver transplantation (CHLT) was performed in 1984 and is now becoming an increasingly common procedure. The indications and outcomes of 369 CHLT recipients (from 1989 to 2020) listed in the United Network for Organ Sharing (UNOS) registry were recently reviewed by Alexopoulos and colleagues. In the era from 1989 to 2010, the most common cardiac indication\/diagnosis was restrictive\/infiltrative cardiomyopathy secondary to diseases such as amyloidosis or hemochromatosis. In the era from 2011 to 2020, congenital heart disease was the most common cardiac diagnosis, accounting for 30.9% of CHLT recipients followed by restrictive\/infiltrative cardiomyopathy in 26.8% and dilated cardiomyopathy in 21.1%. The most common liver diagnosis in current era of CHLT recipients was cardiac cirrhosis (40.4%). \r\nAs the population of patients with congenital heart disease surviving into adulthood has grown, there has been a corresponding rise in the prevalence of liver disease among children with single-ventricle physiology palliated with the Fontan. Liver dysfunction in Fontan patients is believed to be caused by the following two mechanisms: 1) Central hepatic vein and sinusoid dilation due to elevated central venous pressure and, 2) reduced hepatic perfusion secondary to low cardiac output. This results in a redistribution of oxygenated blood to periportal hepatocytes with subsequent atrophy, necrosis, and fibrosis of centrilobular hepatocytes, leading to cirrhosis and regenerative nodules. This heterogenous pattern is present in Fontan patients, particularly ten years post-Fontan-completion. Interestingly, neither invasive hemodynamic measurements nor laboratory abnormalities seem to directly correlate with the severity of fibrosis. Moreover, though portal hypertension develops over time, varices are less prevalent as there is already significant systemic venous hypertension. There is no clear consensus regarding criteria and timing for liver transplant evaluation in Fontan patients, nor which patients would benefit from CHLT. However, a recent scientific statement by the American Heart Association has proposed an algorithm for evaluation of liver disease in potential heart transplant candidates. \r\n\r\nWhile still uncommon overall, the number of CHLTs performed in North America has increased significantly in the last decade. Survival outcomes of CHLT for the overall cohort of 369 patients in the most recent analysis by Alexopoulos were 86.8%, 80.1%, and 77.9% at one, three, and five years respectively. In a multivariable regression analysis, recipient diabetes at listing, CHLT between 1989-2000 compared with 2011-2020, a transplant sequence of heart-after-liver versus liver-after-heart, and lower donor left ventricular ejection fraction were associated with increased mortality after CHLT. Concurrent heart and liver transplant also appears to be associated with improved outcomes, which is thought to be related to the ability of the liver to clear donor-specific HLA class I antibodies. \r\n\r\n\r\n \r\nREFERENCES \r\nAlexopoulos SP, Wu WK, Ziogas IA, et al. Adult Combined Heart-Liver Transplantation: The United States Experience. Transpl Int <\/em>.2022;35:10036. doi: 10.3389\/ti.2021.10036\r\n\r\n\r\nTracy KM, Matsuoka LK, Alexopoulos SP. Update on combined heart and liver transplantation: evolving patient selection, improving outcomes, and outstanding questions. Curr Opin Organ Transplant <\/em>.2023;28(2):104-109. doi: 10.1097\/MOT.0000000000001041\r\n\r\n\r\nKittleson MM, Sharma K, Brennan DC et al. Dual-Organ Transplantation: Indications, Evaluation, and Outcomes for Heart-Kidney and Heart-Liver Transplantation: A Scientific Statement From the American Heart Association. Circulation <\/em>.2023;148:622\u2013636. doi:10.1161\/CIR.0000000000001155 \r\n\r\n\r\nOrtega-Legaspi JM, Hoteit M, Wald J. Immune benefit of combined heart and liver transplantation. Curr Opin Organ Transplant<\/em>. 2020;25(5):513-518. doi:10.1097\/MOT.0000000000000801\r\n\r\n\r\nEmamaullee J, Zaidi AN, Schiano T, et al. Fontan-Associated Liver Disease: Screening, Management, and Transplant Considerations. Circulation<\/em>. 2020;142(6):591-604. doi: 10.1161\/CIRCULATIONAHA.120.045597\r\n”,”hint”:””,”answers”:{“yddgv”:{“id”:”yddgv”,”image”:””,”imageId”:””,”title”:”A.\tCongenital heart disease “,”isCorrect”:”1″},”kj3kk”:{“id”:”kj3kk”,”image”:””,”imageId”:””,”title”:”B.\tRestrictive\/Infiltrative cardiomyopathy “},”9lkir”:{“id”:”9lkir”,”image”:””,”imageId”:””,”title”:”C.\tDilated cardiomyopathy “}}}}}

{“questions”:{“jpsgq”:{“id”:”jpsgq”,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Author: Meera Gangadharan, MD, FASA, FAAP – University of Texas at Houston, McGovern Medical School, Children\u2019s Memorial Hermann Hospital \r\n\r\nA 2.5-year-old male with history of hypoplastic left heart syndrome palliated with a bidirectional Glenn is undergoing a pre-Fontan cardiac catheterization. Pertinent catheterization findings include a systemic arterial saturation (S_{a<\/sub>O2<\/sub>) of 73%, Qp<\/sub>:Qs<\/sub> of 0.8, and pulmonary vascular resistance (PVR) of 2.6 international woods units (iWU). Which of these hemodynamic variables is associated with the HIGHEST periprocedural risk of adverse event(s)?\r\n”,”desc”:”EXPLANATION \r\nRisk-stratification has become an important component of perioperative planning.. Procedural risk is the sum of the risk of the procedure and the risk imposed by the physiologic condition of the patient. Cardiac catheterization in patients with congenital heart disease is an evolving field with new procedures and technologies being developed and utilized in clinical practice. \r\n\r\nThe 2022 Procedural Risk in Congenital Cardiac Catheterization (PREDIC^{3<\/sup>T) study by Quinn and colleagues was used to create a risk assessment tool for pediatric cardiac catheterization procedures. In this study, data from 23,119 pediatric and adult congenital cardiac catheterization cases between January 2014 and January 2018 were analyzed from 13 centers in the United States. Electrophysiological studies were excluded. The primary outcome was the occurrence of a clinically significant adverse event or a high severity adverse event (HSAE). Levels of adverse event severity were graded from zero to five, based on previously established definitions. Levels one and two were none\/minor. Level three was a moderate severity event that resulted in a transient change in the patient\u2019s condition that may have been life-threatening if not treated, required additional medications or transfer to an ICU. Examples include unstable arrythmias with stable blood pressure but requiring intervention, or vascular damage that was not life threatening but required intervention. Level four was a major event that was life-threatening or required cardiopulmonary resuscitation or emergent surgical intervention. Level five was a catastrophic event needing heart-lung support with failure to wean from such support and resulting in death. For this study, levels three through five were considered HSAEs. The study demonstrated that an adverse event occurred in 10.9% of cases and a high severity adverse event (HSAE) occurred in 5.2% of cases. Patient factors that were associated with high-severity adverse events (HSAEs) listed in table 1. \r\n\r\n\r\n\r\n\r\n\r\nCases or procedures were also divided into six risk categories from zero to five (see Table 2). The risk category was an independent predictor of increased risk of HSAEs, with odds-ratios ranging from zero for risk category zero, to 5.25 for risk category five (p \u2264 0.005).\r\n\r\n\r\nTable 2: PREDIC3 <\/sup>T Case-Type Risk Categories \r\n\r\n \r\nTable from: Quinn BP, Yeh M, Gauvreau K, et al. Procedural Risk in Congenital Cardiac Catheterization (PREDIC3<\/sup>T). J Am Heart Assoc<\/em>. 2022;11(1):e022832. Used under Creative Commons License. \r\n\r\nAdditional scoring systems have been developed to quantify risk in cardiac catheterization procedures. In a 2016 study by Nykanen and colleagues, the Congenital Cardiac Interventional Study Consortium developed the Catheterization Risk Score for Pediatrics (CRISP) to predict the likelihood of serious adverse events occurring during cardiac catheterization. The factors included age, weight, inotropic support, organ failure, physiologic category, diagnosis, procedure category and procedure type (diagnostic, interventional or hybrid). The physiologic category was based on systemic saturation, indexed pulmonary vascular resistance, right ventricular systolic pressure \/systemic pressure ratio, anemia, right ventricular outflow obstruction and systemic atrio-ventricular valve regurgitation. Five risk scores were defined: with a CRISP score of five being the highest risk and CRISP score of one being the lowest risk for serious adverse events.\r\n\r\n\r\nThese various risk-assessment tools can also be useful for resource allocation during perioperative planning. A multispecialty society expert consensus statement published in 2016 by Odegard and colleagues offers guidance for pediatric cardiac catheterization procedures. The authors state that a pediatric cardiac anesthesiologist should provide care for patients presenting with a CRISP score equal to or greater than five. Anesthesiologists with a \u201cspecial expertise\u201d in congenital heart disease should provide care for patients with a CRISP score between two and four, and the sedation team can provide care for patients with a CRISP score of zero to one.\r\n\r\n\r\nBased on the PREDIC3<\/sup>T project, for single ventricle patients, a systemic arterial oxygen saturation below 78% increases the risk of experiencing a HSAE. A Qp<\/sub>:Qs<\/sub> of less than 1.5 and a PVR < 3iWU does not increase the risk of a HSAEs. \r\n\r\n\r\n\r\n \r\n\r\nREFERENCES \r\n\r\nNykanen DG, Forbes TJ, Du W, et al. CRISP: Catheterization RISk score for Pediatrics: A Report from the Congenital Cardiac Interventional Study Consortium (CCISC). Catheter Cardiovasc Interv<\/em>. 2016;87(2):302-309. doi:10.1002\/ccd.26300\r\n\r\n\r\nQuinn BP, Yeh M, Gauvreau K, et al. Procedural Risk in Congenital Cardiac Catheterization (PREDIC3<\/sup>T). J Am Heart Assoc<\/em>. 2022;11(1):e022832. doi:10.1161\/JAHA.121.022832\r\n\r\n\r\nOdegard KC, Vincent R, Baijal RG, et al. SCAI\/CCAS\/SPA Expert Consensus Statement for Anesthesia and Sedation Practice: Recommendations for Patients Undergoing Diagnostic and Therapeutic Procedures in the Pediatric and Congenital Cardiac Catheterization Laboratory. Anesth Analg. <\/em>.2016;123(5):1201-1209. doi:10.1213\/ANE.0000000000001608\r\n”,”hint”:””,”answers”:{“b0pzp”:{“id”:”b0pzp”,”image”:””,”imageId”:””,”title”:”(A)\tSa<\/sub>O2<\/sub> of 73%”,”isCorrect”:”1″},”ofrl1″:{“id”:”ofrl1″,”image”:””,”imageId”:””,”title”:”(B)\tQp<\/sub>:Qs<\/sub> of 0.8″},”90c7e”:{“id”:”90c7e”,”image”:””,”imageId”:””,”title”:”(C)\tPVR of 2.6 iWU”}}}}}}}

{“questions”:{“47rhv”:{“id”:”47rhv”,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Author: Sana Ullah, MB ChB, FRCA – Children\u2019s Health, Dallas \r\n\r\nA 36-year-old male who recently emigrated to the United States from India presents with worsening dyspnea on exertion and palpitations. He reports a history of rheumatic fever as a child. A 12-lead ECG show atrial fibrillation with a HR of 110. Transthoracic echocardiogram demonstrates a large secundum atrial septal defect with left to right shunting and moderate right heart enlargement. What additional abnormality is MOST likely to be seen on the echocardiogram?”,”desc”:”EXPLANATION \r\nThe association of an atrial septal defect (ASD) with mitral stenosis (MS) is known as Lutembacher syndrome, named after the first publication in 1916 by a French physician. The ASD is usually congenital, but the MS is almost always due to a complication of rheumatic heart disease. Hence, this entity is more common in areas where rheumatic fever is endemic, such as sub-Saharan Africa and other under-developed countries. An iatrogenic form of Lutembacher syndrome is composed of a residual ASD created during trans-catheter balloon mitral valvuloplasty procedures for MS.\r\n\r\n\r\n \r\nThe natural history and hemodynamic sequelae of Lutembacher syndrome are determined by the size of the ASD, severity of MS, pulmonary vascular resistance, and compliance of the right ventricle. The magnitude of the left to right shunt across the typically large ASD is increased in the presence of severe MS resulting in volume overload of the right ventricle and pulmonary circulation. This will eventually result in right heart failure and pulmonary hypertension. In the presence of a restrictive ASD, the clinical course will be primarily determined by the severity of the MS. Atrial dilatation additionally predisposes patients to atrial fibrillation.\r\n\r\n\r\n\r\nDiagnosis is confirmed by transthoracic echocardiography (TTE) and confirms ASD size and severity of MS. Planimetry is usually preferred in assessing MS severity as doppler gradients across the valve will be significantly reduced due to shunting across the ASD.\r\n\r\n\r\n\r\nTreatment of Lutembacher syndrome in the current era is usually via trans-catheter techniques with balloon valvuloplasty of the mitral valve and device closure of the ASD. Surgical management is indicated for patients deemed unsuitable for trans-catheter procedure, such as inadequate rims around the ASD, left atrial thrombus, or concomitant severe mitral regurgitation.\r\n\r\n\r\n\r\nIn the patient described above, the most likely co-existing abnormality is MS due to the history of rheumatic heart disease. The mitral valve is the most common valve affected by rheumatic heart disease, initially causing mitral regurgitation that may progress to mitral stenosis. A significant ventricular septal defect is likely to result in pulmonary hypertension in a 36-year-old patient and reversal of shunting at level of the ASD. Therefore, answer B is incorrect. Partial anomalous pulmonary venous return is less likely in this patient with a history of rheumatic fever. Furthermore, PAPVR typially associated with sinus venosus ASDs rather than a secundum ASD. \r\n\r\n\r\n \r\nREFERENCES \r\n\r\nAminde LN, Dzudie A, Takah NF, Ngu KB, Sliwa K, Kengne AP. Current diagnostic and treatment strategies for Lutembacher syndrome: the pivotal role of echocardiography. Cardiovas Diag Ther <\/em>.2015; 5:122-132. doi: 10.3978\/j.issn.2223-3652.2015.03.07\r\n\r\n\r\nBashi VV, Ravikumar E, Jairaj PS, et al. Coexistent mitral valve disease with left-to-right shunt at the atrial level: clinical profile, hemodynamics, and surgical considerations in 67 consecutive patients. Am Heart J <\/em>.1987;114:1406-14. \r\n\r\n\r\nNagamani AC, Nagesh CM. Lutembacher Syndrome. In: Vijaylakshmi IB, Rao PS, ChughR, eds. A Comprehensive Approach to Congenital Heart Disease. <\/em> 2nd ed. New Dehli, India: Jaypee Brothers Medical Publishers; 2020:1036-1045.\r\n”,”hint”:””,”answers”:{“rbd2r”:{“id”:”rbd2r”,”image”:””,”imageId”:””,”title”:”A. Partial anomalous pulmonary venous return “},”c9xqe”:{“id”:”c9xqe”,”image”:””,”imageId”:””,”title”:”B. Ventricular septal defect”},”6vou8″:{“id”:”6vou8″,”image”:””,”imageId”:””,”title”:”C. Mitral stenosis”,”isCorrect”:”1″}}}}}

{“questions”:{“g8env”:{“id”:”g8env”,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Author: Sana Ullah, MB ChB, FRCA – Children\u2019s Health, Dallas \r\n\r\nA two-week-old male neonate is admitted to the cardiac intensive care unit for further evaluation of cyanosis. Physical exam reveals an imperforate anus and dysmorphic facial features including pre-auricular skin tags and vertical colobomas of both eyes. Chromosomal analysis demonstrates a duplication of the long arm of chromosome 22. What is the MOST likely congenital heart defect in this patient?”,”desc”:”EXPLANATION \r\nThe clinical features in this patient are consistent with cat-eye syndrome (CES), also known as Schmid-Fraccaro syndrome. This rare genetic disorder affects approximately 1 in 150,000 people and is caused by duplication or triplication of the long arm of chromosome 22. Although there is significant phenotypic variation, the syndrome usually consists of craniofacial anomalies including facial dysmorphism, ear tags, vertical colobomas leading to the appearance of cat\u2019s eyes, and imperforate anus. \r\n\r\n\r\nCongenital cardiac defects are found in approximately 50% of patients with CES. There are several small case series in the literature describing the association of total pulmonary venous connection (TAPVC) with CES. In an analysis of thirteen published cases in 1973 by Freedom and colleagues, congenital heart disease was present in five patients with TAPVC in three out of these five cases. Tetralogy of Fallot and interrupted aortic arch are examples of conotruncal defects that are commonly associated with DiGeorge syndrome resulting from a deletion of chromosome 22q.11.2. Interrupted aortic arch also does not present with cyanosis. Therefore, options A and C are incorrect.\r\n\r\n\r\nMany patients with CES will require anesthesia for non-cardiac or cardiac procedures during the neonatal period. Due to craniofacial abnormalities, these patients should be considered at risk for a difficult airway management. \r\n\r\n \r\n\r\nREFERENCES \r\nFreedom RM, Gerald PS. Congenital cardiac disease and the \u201ccat eye\u201d syndrome. Am J Dis Child <\/em>.1973; 126:16-18. \r\n\r\nGaspar N S, Rocha G, Grangeia A, et al. Cat-Eye Syndrome: A Report of Two Cases and Literature Review. Cureus<\/em>. 2022; 14(6): e26316. DOI 10.7759\/cureus.26316.\r\n\r\nWilliams JL, McDonald MT, Siefert BA, Deak KL, Rehder CW, Campbell MJ. An unusual association: Total anomalous pulmonary venous return and aortic arch obstruction in patients with cat eye syndrome. J Pediatr Genet <\/em>.2021;10:35\u201338. DOI https:\/\/doi.org\/ 10.1055\/s-0039-1701020. \r\n\r\nDevavaram P, Seefelder C, Lillehei CW. Anaesthetic management of cat eye syndrome (Letter). Pediatr Anesth <\/em>.2001 ; 11:746-748. DOI: 10.1046\/j.1460-9592.2001.0774c.x\r\n\r\n\r\n”,”hint”:””,”answers”:{“l3olo”:{“id”:”l3olo”,”image”:””,”imageId”:””,”title”:”A. Tetralogy of Fallot”},”79l7w”:{“id”:”79l7w”,”image”:””,”imageId”:””,”title”:”B. Total anomalous pulmonary venous connection”,”isCorrect”:”1″},”8dki7″:{“id”:”8dki7″,”image”:””,”imageId”:””,”title”:”C. Interrupted aortic arch”}}}}}

{“questions”:{“e334j”:{“id”:”e334j”,”mediaType”:”image”,”answerType”:”text”,”imageCredit”:””,”image”:””,”imageId”:””,”video”:””,”imagePlaceholder”:””,”imagePlaceholderId”:””,”title”:”Author: Meera Gangadharan MD, FASA, FAAP – Children\u2019s Memorial Hermann Hospital\/McGovern Medical School, Houston, TX \r\n\r\nA 15-month-old female with hypoplastic left heart syndrome, with borderline left heart structures, has been palliated with a bidirectional Glenn and is being considered for biventricular conversion. Her preoperative echocardiogram demonstrates severe endocardial fibroelastosis (EFE) of the left ventricle. What is the MOST likely long-term complication of EFE resection in this patient? “,”desc”:”EXPLANATION \r\nEndocardial fibroelastosis (EFE) is an abnormal thickening of the endocardium caused by an abnormal deposition of collagen by fibroblasts, resulting in systolic and diastolic myocardial dysfunction. Although primary EFE is associated with numerous diseases, in the context of congenital heart disease, it is usually caused by a reduction of blood flow through the cardiac chambers leading to significant systolic and diastolic dysfunction with restrictive physiology. \r\n\r\nDuring embryologic development, endocardial endothelial cells undergo transformation to mesenchymal cells which then form the endocardial cushions and valves. This endothelial-to-mesenchymal transition is altered by inflammation, hypoxia-ischemia, and mechanical flow disturbances, giving rise to the fibroblasts that form the fibrotic layer of EFE. Imbalances between transforming growth factor beta (TGF-\u03b2) and bone morphogenetic protein (BMP) signaling have been shown to contribute to the formation of EFE in mice. Exogenous supplementation with BMP has been shown to decrease EFE in this same mouse model (Xu et al). \r\nEFE has been described as an opaque porcelain-like layer which may be several millimeters thick (see illustration below). Although EFE can be diagnosed by echocardiogram with 95% sensitivity and 75% specificity, computed tomography (CT) scanning and magnetic resonance imaging (MRI) have also been used. MRI is particularly useful for measuring chamber volumes and flows. \r\n\r\n \r\nMacroscopic appearance of endocardial fibroelastosis. From: Luca AC, Lozneanu L, Miron IC, et al. Endocardial fibroelastosis and dilated cardiomyopathy – the past and future of the interface between histology and genetics. Rom J Morphol Embryol<\/em>. 2020;61(4):999-1005. doi:10.47162\/RJME.61.4.02. Used under Creative Commons License.\r\n\r\nThe severity of EFE on fetal echocardiograms can be used to predict prognosis after in-utero aortic balloon valvuloplasty for evolving hypoplastic left heart syndrome. The rate of change of left ventricular end-diastolic volume after the procedure was significantly greater in fetuses with mild EFE compared to those with severe EFE. Similarly, the severity of EFE can predict the likelihood that the child will be able to have biventricular repair as compared to a univentricular palliation postnatally after having undergone in-utero balloon aortic valvuloplasty. \r\nA significant proportion of patients within the spectrum of HLHS have borderline left heart structures in whom it may be possible to utilize a staged left ventricle recruitment strategy to eventually facilitate a biventricular repair. The underlying principle is to promote a flow and load-mediated growth of the left ventricle. These procedures include: (1) Interventions to relieve obstruction of the mitral and aortic valves; (2) resection of EFE to improve systolic and diastolic function; (3) restriction of the atrial communication to promote more flow into the LV; (4) adding accessory pulmonary blood flow. In a retrospective, single institution study of 34 patients undergoing a staged LV recruitment strategy, EFE resection was perfomed in the majority of patients at all three stages of palliation. There was a significant increase in left heart dimensions and ejection fraction. Thirteen of the 34 patients underwent successful biventricular repair with no mortality after a median follow-up of 2.9 years.\r\n\r\nAlthough conduction abnormalities can be seen with EFE resection, there have been no reports of heart block requiring permanent pacemaker insertion. In a 2009 retrospective study by Emani and colleagues of nine patients who had undergone EFE resection, three patients had evidence of right bundle branch block or hemifascicular block with mild prolongation of the QRS complex but none required pacemaker placement. In a separate study by Czosek and colleagues of 27 patients who had undergone EFE resection, 14 patients had varying degrees of QRS prolongation or fascicular blocks but no complete heart block requiring pacemaker placement.\r\n\r\nThere is a high rate of recurrence of EFE after resection. In a retrospective review by Diaz-Gil of 49 patients with a small LV who had undergone EFE resection, the risk of recurrence was 76% over a 10 year period.\r\n\r\n\r\n \r\nREFERENCES \r\nAldawsari, K.A., Alhuzaimi, A.N., Alotaibi, M.T. et al. Endocardial fibroelastosis in infants and young children: a state-of-the-art review. Heart Fail Rev <\/em>.(2023); 28:1023\u20131031. https:\/\/doi.org\/10.1007\/s10741-023-10319-0\r\n\r\nWeixler V, Marx GR, Hammer PE, Emani SM, Del Nido PJ, Friehs I. Flow disturbances and the development of endocardial fibroelastosis. J Thorac Cardiovasc Surg<\/em>. 2020;159(2):637-646. doi: 10.1016\/j.jtcvs.2019.08.101\r\n\r\nEmani SM, McElhinney DB, Tworetzsky W et al. Staged left ventricular recruitment after single-ventricle palliation in patients with borderline left-heart hypoplasia. J Am Coll Cardiol <\/em>. 2012. ;60:1966-74.\r\n\r\nEmani SM, Bacha EA, McElhinney DB et al. Primary left ventricular rehabilitation is effective is effective in maintaining two-ventricle physiology in the borderline left heart. J Thorac Cardiovasc Surg <\/em>. 2009;138:1276-82.\r\n\r\nDiaz-Gil D, Piekarski BL, Marx GR, Del Nido PJ, Emani S, Friehs I. Endocardial fibroelastosis recurrence: Comparing single ventricle palliation versus biventricular repair. Thorac Cardiovasc Surg <\/em>.2022. 70(S02): S67-S103. DOI: 10.1055\/s-0042-1742956\r\n\r\nCzosek RJ, Atallah J, Emani S, Hasan B, del Nido P, Berul CI. Electrical dyssynchrony and endocardial fibroelastosis resection in the rehabilitation of hypoplastic left cardiac syndrome. Cardiol Young<\/em>. 2010;20(5):516-521. doi:10.1017\/S1047951110000600\r\n\r\nHan RK, Gurofsky RC, Lee KJ, et al. Outcome and growth potential of left heart structures after neonatal intervention for aortic valve stenosis. J Am Coll Cardiol <\/em>.2007;50(25):2406-2414. doi:10.1016\/j.jacc.2007.07.082\r\n\r\nXu X, Friehs I, Zhong Hu T, et al. Endocardial fibroelastosis is caused by aberrant endothelial to mesenchymal transition. Circ Res <\/em>.2015;116(5):857-866. doi:10.1161\/CIRCRESAHA.116.305629\r\n\r\nMcElhinney DB, Vogel M, Benson CB, et al. Assessment of left ventricular endocardial fibroelastosis in fetuses with aortic stenosis and evolving hypoplastic left heart syndrome. Am J Cardiol <\/em>.2010;106(12):1792-1797. doi:10.1016\/j.amjcard.2010.08.02\r\n\r\n”,”hint”:””,”answers”:{“ysona”:{“id”:”ysona”,”image”:””,”imageId”:””,”title”:”A)\tReduced ejection fraction”},”tqplj”:{“id”:”tqplj”,”image”:””,”imageId”:””,”title”:”B)\tHeart block”},”cefjb”:{“id”:”cefjb”,”image”:””,”imageId”:””,”title”:”C)\tRecurrence of endocardial fibroelastosis\r\n\r\n”,”isCorrect”:”1″}}}}}