Mitral Valve Replacement in Infants and Children: Five Year Outcomes of the HALO Clinical Trial.

BACKGROUND: Repair is preferable for children with mitral valve disease, but mitral valve replacement (MVR) is occasionally necessary. This report presents the results of a multiinstitutional Investigational Device Exemption trial of the 15-mm St Jude (SJM) mechanical mitral valve (Abbott Structural Heart). METHODS: From May 2015 to March 2017, 23 children aged 0.4 to 27.4 months (mean, 7.8 months; 85% <1 year) weighing 2.9 to 10.9 kg (mean, 5.5 kg) at 15 centers underwent MVR with a 15-mm SJM mechanical mitral valve (intraannular, 45%; supraannular, 55%). A total of 21 (91%) of the children had undergone previous cardiac operations. Follow-up until death, valve explantation, or 5 years postoperatively was 100% complete. RESULTS: There were 6 deaths, all in the first 12 months; no death was valve related. Four patients required a pacemaker (2 supraannular, 2 intraannular). Three patients had thrombosis requiring valve explantation at 13, 21, and 35 days postoperatively. Two of these 3 patients were receiving low-molecular-weight heparin for anticoagulation, and the third had factor V Leiden deficiency. There were 5 nonfatal bleeding complications within 4 months of MVR (1-year freedom from bleeding, 71.0%). The 1- and 5-year freedom from death or valve explantation was 71.0%. CONCLUSIONS: In small children with severe mitral valve disease requiring MVR, the 15-mm SJM mechanical mitral valve provides satisfactory hemodynamics. Mortality and complications in these patients are not trivial. Low-molecular-weight heparin likely should be avoided as primary anticoagulation. Eventual valve replacement is inevitable.

Management of pediatric sternal wounds following congenital heart surgery: The role of the plastic surgeon in debridement and closure.

BACKGROUND: Management of sternal wound infections (SWIs) in pediatric patients following congenital heart surgery can be extremely difficult. Patients with congenital cardiac conditions are at risk for complications such as sternal dehiscence, infection, and cardiopulmonary compromise. In this study, we report a single-institution experience with pediatric SWIs. METHODS: Fourteen pediatric patients requiring plastic surgery consultation for complex sternal wound closure were included. A retrospective chart review was performed with the following variables of interest: demographic data, congenital cardiac condition, respective surgical palliations, development of mediastinitis, causative organism, number of debridements, presence of sternal wires, and choice of flap coverage. Primary endpoints included achieved chest wall closure and overall survival. RESULTS: Of the 14 patients, 8 (57%) were diagnosed with culture-positive mediastinitis. The sternum remained wired at the time of final flap closure in eight (57%) patients. All patients were reconstructed with pectoralis major flaps, except one (7%) who also received an omental flap and two (14%) who received superior rectus abdominis flaps. One patient (7%) was treated definitively with negative pressure wound therapy, and one (7%) was too unstable for closure. Six patients developed complications, including one (7%) with persistent mediastinitis, two (14%) with hematoma formation, one (7%) with abscess, and one (7%) with skin necrosis requiring subsequent surgical debridement. There were three (21%) mortalities. CONCLUSIONS: The management of SWI in congenital cardiac patients is challenging. The standard tenets for management of SWI in adults are loosely applicable, but additional considerations must be addressed in this unique subset population.

Contemporary outcomes of durable ventricular assist devices in adults with congenital heart disease as a bridge to heart transplantation.

OBJECTIVES: This study aimed to compare the clinical characteristics, risk factors, and overall survival outcomes in adults with congenital heart disease (ACHD) bridged to transplantation with a ventricular assist device (VAD) versus no-VAD. METHODS: The study included 894 ACHD patients aged ≥18 years listed for primary heart transplantation between 2010 and 2019 from the United Network for Organ Sharing database. Primary outcomes were waitlist and 1-year post-transplant mortality between VAD and no-VAD ACHD patients. RESULTS: Of 894 ACHD patients included in the study, 91(10.1%) had VAD support at the time of listing. Patients who needed VAD support were mostly males, heavier, and had higher pulmonary artery pressure than the no-VAD group at the listing. The overall waitlist mortality was 38% in the VAD group than 17% in the no-VAD group (p < 0.01). ECMO use was associated with significantly higher mortality than either group. There was no significant difference in 1-year post-transplant mortality between VAD versus no-VAD at the time of transplant (15% vs. 17%; p = 0.66). Multivariate regression analysis found that BMI <20 kg/m2 (hazard ratio (HR) 1.1; p = 0.01), bilirubin >2 mg/dl (HR 1.1; p = 0.03), creatinine >2 mg/dl (HR 1.3; p = 0.04) and ECMO at transplant (HR 1.4; p = 0.03) increased early post-transplant mortality. CONCLUSIONS: The one-year post-transplant mortality rate was no different for ACHD patients that received VAD versus no-VAD. These findings suggest that a VAD should be considered an option to support ACHD patients as a bridge to heart transplantation.

Torrential Mitral Regurgitation After Transcatheter Edge-to-Edge Mitral Valve Repair.

A patient with severe mitral regurgitation and chronic systolic heart failure taking inotropic support at home presents for transcatheter edge-to-edge mitral valve repair, complicated by torrential mitral regurgitation from damaged mitral leaflets requiring escalating mechanical circulatory support and ultimately expedited orthotopic heart transplantation. (Level of Difficulty: Intermediate.).

Effect of Atrioventricular Valve Repair on Multistage Palliation Results of Single-Ventricle Defects.

BACKGROUND: The presence of significant atrioventricular valve (AVV) regurgitation results in unfavorable conditions that affect the success of single-ventricle (SV) multistage palliation. We report our institution's AVV repair experience. METHODS: We examined incidence of AVV repair in 603 infants who underwent initial SV palliative surgery from 2002 to 2012. We explored patients' characteristics and anatomic and operative details associated with death, transplantation, and AVV reoperation. RESULTS: AVV repair was performed in 60 patients during first-stage (n = 10), Glenn (n = 27), and Fontan (n = 23). Median age at AVV repair was 6.9 months (interquartile range, 4.2-24.1 months). Underlying SV anomaly was hypoplastic left heart syndrome (n = 30), heterotaxy (n = 15), and other (n = 15). The AVV was tricuspid (n = 34), mitral (n = 6), or common (n = 20). Preoperatively, all patients had AVV regurgitation moderate or higher, and 7 (12%) had ventricular dysfunction moderate or higher. After the repair, AVV regurgitation was none/trivial in 21 (35%), mild in 21 (35%), and moderate or higher in 17 (30%). Competing risks analysis showed that 10 years after AVV repair, 18% of patients had undergone AVV reoperation, 26% had died or undergone transplantation, and 56% were alive without subsequent reoperation. Transplant-free survival was 38%, 65%, and 100% for AVV repair at first-stage, Glenn, or Fontan (P = .0011), respectively, and was 74%, 83%, and 56% for tricuspid, mitral, and common AVV repair (P = .344), respectively. Factors associated with transplant-free survival were timing of AVV repair, underlying SV anomaly, and systemic ventricle function. CONCLUSIONS: AVV repair at the first-stage operation and reduced systemic ventricle function are associated with poor outcomes. In those high-risk patients, different approaches that involve initial palliation mode, timing of AVV repair, or listing for transplantation might be warranted.

Hepatocellular carcinoma and the Fontan circulation: Clinical presentation and outcomes.

BACKGROUND: Fontan-associated liver disease (FALD) is universal in patients with a Fontan circulation. Hepatocellular carcinoma (HCC) is one of its severe expressions, and, though rare, frequently fatal. The purpose of this study was to describe the clinical presentation, risk factors, and outcomes of HCC in patients with a Fontan circulation. METHODS: A multicenter case series of Fontan patients with a diagnosis of HCC formed the basis of this study. The case series was extended by published cases and case reports. Clinical presentation, tumor characteristics, laboratory and hemodynamic findings as well as treatment types and outcomes, were described. RESULTS: Fifty-four Fontan patients (50% female) with a diagnosis of HCC were included. Mean age at HCC diagnosis was 30 ± 9.4 years and mean duration from Fontan surgery to HCC diagnosis was 21.6 ± 7.4 years. Median HCC size at the time of diagnosis was 4 cm with a range of 1 to 22 cm. The tumor was located in the right hepatic lobe in 65% of the patients. Fifty-one percent had liver cirrhosis at the time of HCC diagnosis. Fifty percent of the patients had no symptoms related to HCC and alpha-fetoprotein was normal in 26% of the cases. Twenty-six patients (48%) died during a median follow-up duration of 10.6 (range 1-50) months. CONCLUSIONS: HCC in Fontan patients occurs at a young age with a 1-year survival rate of only 50%. Meticulous liver surveillance is crucial to detect small tumors in the early stage.

Concomitant Aortic Root Replacement With Anterior Konno Annular Enlargement.

The need for concomitant aortic annular enlargement and aortic root replacement may be necessary in situations of multilevel left ventricular outflow tract obstruction and/or prior aortic or aortic valve surgery, particularly in the setting of congenital heart disease. It can be extremely tedious and fraught with numerous complications. Major challenges include bleeding in difficult-to-reach areas and the potential need for subsequent operations. This article describes a technique of concomitant aortic annular enlargement and aortic root replacement that minimizes the risk of significant blood loss and facilitates future surgery should it be necessary.

Aortic root valve-sparing repair and dissections in Marfans syndrome during pregnancy: A case series.

INTRODUCTION: Marfan syndrome is a connective tissue disorder caused by mutations in the fibrillar FBN-1 gene. Aortic dissection and rupture are major causes of morbidity and mortality and are of special concern during pregnancy. MATERIALS AND METHODS: The authors report four cases of aortic root repair with preservation of the native aortic valve that have has created a discussion between cardiothoracic surgeons, obstetricians, and gynecologists regarding the best care for Marfan syndrome patients. We present these cases here with a review of the literature. RESULTS: Surgery of the aorta and valves in Marfan syndrome is less risky than in previous eras and surgical management guidelines are generally accepted. Yet, we may be unnecessarily referring women to terminate pregnancies or to avoid pregnancy. We believe there may be alternative options for these patients. CONCLUSIONS: Marfan syndrome during pregnancy can be navigated with preconception counseling, antepartum care, and close postpartum follow-up involving an appropriate multidisciplinary team.

Impact of Accredited Training Programs in Congenital Heart Surgery.

Historically, training in congenital heart surgery was variable and followed 1 of 3 pathways: residents identified during the primary training process were given the opportunity for a career training position within their own or other institution, residents would travel abroad for training and return to look for a faculty appointment based on their training experience, or a resident would enter a 1-year fellowship position after completing Thoracic Surgery training. These training opportunities and fellowships lacked uniformity and quality control. The purpose of an Accreditation Council of Graduate Medical Education (ACGME)-accredited residency program and an American Board of Thoracic Surgery subspecialty certificate in congenital heart surgery was to recognize and document a standard of education, operative experience, and cognitive knowledge for surgeons practicing in the field. There have been several important papers related to congenital cardiac training published over the years: (1) a manuscript by Kogon published in 2006 outlining congenital cardiac training prior to ACGME program accreditation, (2) a manuscript by Kogon et al published in 2016 outlining congenital cardiac training subsequent to ACGME accreditation with a focus on job transition and early work experience, and (3) a manuscript published in 2017 focusing primarily on job transition and early work experience. The purpose of this review is to provide an update with respect to congenital cardiac training. More importantly, in comparing survey results from these previous papers with the current survey, some interesting trends have been revealed, good and bad.

Outcomes of Multistage Palliation of Infants With Single Ventricle and Atrioventricular Septal Defect.

BACKGROUND: Published palliation outcomes of infants with functional single ventricle (SV) and common atrioventricular septal defect (AVSD) are poor due to associated cardiac and extracardiac anomalies and development of atrioventricular valve (AVV) regurgitation. We report current palliation results. METHODS: From 2002 to 2012, 80 infants with functional SV with AVSD underwent multistage palliation. Competing-risks analyses modeled events after first-stage surgery and Glenn (death/transplantation vs next palliation surgery) and examined factors associated with survival and AVV intervention. RESULTS: Sixty-eight (80%) patients received neonatal palliation: modified Blalock-Taussig shunt (n = 33, 41%), Norwood (n = 20, 25%), and pulmonary artery band (n = 15, 19%), whereas 12 (15%) received primary Glenn. On competing-risks analysis, one-year following first-stage surgery, 29% of patients had died or received transplantation and 62% had undergone Glenn. Five years following Glenn, 9% of patients had died or received transplantation and 68% had undergone Fontan. Overall eight-year survival was 64% and was lower in patients with genetic syndromes (53% vs 82%), patients requiring concomitant total anomalous pulmonary venous connection repair (53% vs 69%), and those requiring neonatal palliation (48% vs 100%). Factors associated with mortality were unplanned reoperation (hazard ratio [HR]: 3.7 [1.7-8.0], P = .001) and extracorporeal membrane oxygenation use (HR: 7.1 [3.0-16.6], P < .001). Initial AVV regurgitation ≥ moderate was associated with AVV intervention (HR: 6.2 [2.4-16.1], P = .002) with eight-year freedom from death or AVV intervention of 25% in those patients. CONCLUSIONS: Patients with SV with AVSD are a distinct group and commonly have associated cardiac and extracardiac malformations that complicate care and affect survival. The development of AVV regurgitation requiring intervention is common but does not affect survival.

The case of the missing pulmonary vein: A focused update on anomalous pulmonary venous connection in congenital cardiovascular disease.

Partial anomalous pulmonary venous connection is defined by one or more of the pulmonary veins draining to the heart into a location other than the left atrium. Depending on the location of the anomalous venous connection, they can be categorized as supracardiac, infracardiac, cardiac, and mixed types. In some cases, there is no hemodynamic consequence; in others, it can result in tricuspid regurgitation, right heart dilation, and pulmonary hypertension. Frequently, the reason for referral can be asymptomatic right heart dilation of unknown significance. Diagnosis is often difficult by transthoracic echocardiogram unless there is a high index of suspicion, and the appropriate views are obtained. Cardiac CT (computed tomography) or cardiac MRI (magnetic resonance imaging) can provide more precise anatomic detail as needed. The current article reviews the etiology and pathophysiology of partial anomalous pulmonary venous connection, and also reviews the current knowledge on their treatment.

Readmission After Pediatric Cardiothoracic Surgery: An Analysis of The Society of Thoracic Surgeons Database.

BACKGROUND: Hospital readmission after pediatric cardiac surgery is incompletely understood. This study analyzed data from The Society of Thoracic Surgeons Congenital Heart Surgery Database to determine prevalence, to describe patient characteristics, and to evaluate risk factors for readmission. METHODS: Readmission was defined by the "readmission within 30 days after discharge" field. Routine variables were summarized. Regression analysis was used to identify factors associated with readmission. RESULTS: The study cohort included 56,429 patient records from 100 centers. Overall, 6,208 (11%) patients were readmitted. The most common reasons for readmission were respiratory or airway complications (14.2%), septic or infectious complications (11.4%), and reasons not related to the preceding surgical procedure (20.2%). Primary reason for readmission varied across benchmark operation groups. In multivariable analysis, factors associated with increased odds of readmission included the presence of noncardiac abnormalities (odds ratio [OR], 1.24), chromosomal abnormalities or genetic syndromes (OR, 1.24), preoperative mechanical circulatory support (OR, 1.36), other preoperative factors (OR, 1.21), prior cardiac surgery (OR, 1.31), Hispanic ethnicity (OR, 1.13), higher STAT procedural complexity (Society of Thoracic Surgeons/European Association for Cardio-Thoracic Surgery) (STAT level 3 vs 1, OR, 1.22; STAT 4 vs 1, OR, 1.48; STAT 5 vs 1, OR, 2.62), prolonged postoperative length of stay (OR, 1.07 per day from 0 to 14 days; OR, 1.01 per week >14 days), any major complication (OR, 1.27), any other postoperative complications (OR, 2.00), and discharge on a weekday (OR, 1.07). CONCLUSIONS: Readmission is common after congenital heart surgery, mostly for noncardiovascular reasons. Process improvement initiatives targeted at high-risk patients could minimize its impact.

Frontiers in Fontan failure: Innovation and improving outcomes: A conference summary.

The initial "Frontiers in Fontan Failure" conference in 2015 in Atlanta, GA, provided an opportunity for experts in the field of pediatric cardiology and adult congenital heart disease to focus on the etiology, physiology, and potential interventions for patients with "Failing Fontan" physiology. Four types of "Fontan Failure" were described and then published by Dr Book et al. The acknowledgment that even Dr Fontan himself realized that the Fontan procedure "imposed a gradually declining functional capacity and premature late death after an initial period of often excellent palliation." The purpose of the second "Frontiers in Fontan Failure" was to further the discussion regarding new data and technologies as well as novel interventions. The 2017 "Frontiers in Fontan Failure: Innovation and Improving Outcomes" was sponsored by Children's Healthcare of Atlanta, Sibley Heart Center Cardiology, and Emory University School of Medicine. Future directions in the management of Fontan failure include further investigations into the risk of sudden cardiac death and how to properly prevent it, achievable interventions in modifying the Fontan physiology to treat or prevent late complications, and improved and refined algorithms in Fontan surveillance. Finally, further research into the interventional treatment of lymphatic-related complications hold the promise of marked improvement in the quality of life of advanced Fontan failure patients and as such should be encouraged and contributed to.

Center volume and post-transplant survival among adults with congenital heart disease.

BACKGROUND: The number of adult congenital heart disease (ACHD) patients requiring heart transplantation (HT) continues to grow, and if they survive the first year after transplant, their long-term survival is at least equivalent to non-ACHD patients. The 1-year survival of ACHD patients with HT remains lower than non-ACHD patients. We evaluated the affect of transplant center volume on 1-year survival of ACHD patients. We analyzed United Network of Organ Sharing patients (age ≥18 years) who underwent their first orthotopic HT between January 1, 2000, and December 31, 2015, to assess the association between transplant center volume and 1-year survival of ACHD patients. RESULTS: We identified 827 ACHD patients at 113 centers who underwent HT during the study period. The average age of the recipients and donors was 36 ± 13 years (60% men and 84% Caucasian) and 28 ± 11 (63% men and 66% Caucasian), respectively. Of the ACHD patients undergoing HT, 27% (n = 60) were done at low-volume centers, 30% (n = 10) were reported at high-volume centers, and the remaining (n = 43) were at medium-volume centers. A total of 96 patients died within 30 days, including 37 (16.7%) at low-volume, 37 (10.2%) at medium-volume, and 22 (9.0%) at high-volume centers (p = 0.019). The average unadjusted Kaplan-Meier 30-day survival at low-volume centers was 83% ± 2%, which was significantly lower than medium-volume (90% ± 1%) and high-volume (91% ± 2%) centers (log-rank p < 0.05). Within 1 year, 154 patients had died, including 56 (36.4%) at low-volume, 60 (38.9%) at medium-volume, and 38 (24.7%) at high-volume centers (p = 0.011). Average unadjusted Kaplan-Meier 1-year survival at low-volume centers was 75% ± 3%, which was significantly lower than medium-volume (83% ± 2%) and high-volume (84% ± 2%) centers (log-rank p < 0.05). CONCLUSIONS: The 30-day and 1-year survival of ACHD patients undergoing HT is partly influenced by overall transplant center volume and, potentially, volume of ACHD HTs, with low-volume centers performing poorly relative to medium-volume and high-volume centers. The role of peri-operative care and multidisciplinary management in improving survival at low-volume centers required further investigations.

Current Results of Single Ventricle Palliation of Patients With Double Inlet Left Ventricle.

BACKGROUND: Double inlet left ventricle (DILV) is a heterogeneous single ventricle anomaly in which initial presentation, and consequently, timing and palliation mode vary based on morphology and degree of pulmonary or systemic outflow obstruction. Very few reports, mostly old, focused on palliation outcomes of DILV. We report current-era results and examine whether morphologic and subsequently surgical factors influence survival. METHODS: Fifty-eight infants with DILV underwent single ventricle palliation. Echocardiographic examination showed pulmonary (n = 29, 50%), systemic outflow tract (n = 11, 19%), and arch (n = 17, 29%) obstruction. Factors associated with death or transplantation were examined. RESULTS: Forty-four patients (76%) required neonatal first-stage palliation: modified Blalock-Taussig shunt (n = 15, 26%), Norwood (n = 15, 26%), or pulmonary artery band (n = 14, 24%), whereas 14 (24%) received primary Glenn. There was 1 hospital death (2%) and 2 interstage deaths before Glenn, in addition to 1 late death that was noncardiac. Overall 10-year survival was 94% and was comparable for different palliative surgeries (p = 0.49). Three patients (6%) underwent heart transplantation after first-stage palliation (n = 1) or after Glenn (n = 2) for ventricular noncompaction (n = 1), ventricular and atrioventricular valve dysfunction (n = 1), and pacemaker-induced cardiomyopathy (n = 1). Overall 10-year freedom from death or transplantation was 87% and was comparable for different palliative surgeries (p = 0.58). On regression risk analysis, none of the tested morphologic or surgical variables was associated with the risk of death or transplantation. CONCLUSIONS: Current outcomes of multistage palliation of DILV are relatively good compared with published reports of other single ventricle anomalies. Survival is not greatly affected by cardiac morphology or initial palliative surgery type.

The Impact of 22q11.2 Deletion Syndrome on Surgical Repair Outcomes of Conotruncal Cardiac Anomalies.

BACKGROUND: We aim to describe the impact of 22q11.2 deletion syndrome (22q11DS) on clinical characteristics, postoperative course, and early and late outcomes of neonates undergoing surgery for conotruncal anomalies. METHODS: A retrospective review was performed (2002 to 2012) of 224 neonates who underwent surgery for interrupted aortic arch (n = 67), truncus arteriosus (n = 85), or ductal-dependent pulmonary atresia and ventricular septal defect (n = 72). Patients were divided into three groups: group 1, n = 119, no genetic syndrome; group 2, n = 64, 22q11DS; and group 3, n = 41, other genetic syndrome. Adjusted analysis to compare outcomes was performed. RESULTS: In comparison with group 1, group 2 had longer mechanical ventilation duration (148 versus 102 hours, p = 0.008), intensive care unit stay (268 versus 159 hours, p < 0.001), and hospital stay (19.3 versus 11.5 days, p < 0.001). On adjusted analysis, there was an insignificant increase in unplanned reoperation (odds ratio [OR] 2.4, 95% confidence interval [CI]: 0.7 to 8.4, p = 0.167) but no increased extracorporeal membrane oxygenation use (OR 1.5, 95% CI: 0.3 to 6.1, p = 0.612), hospital mortality (OR 0.6, 95% CI: 0.1 to 3.3, p = 0.570), or decreased late survival (hazard ratio 0.9, 95% CI: 0.4 to 2.1, p = 0.822). In comparison with group 1, group 3 had longer mechanical ventilation duration (190 versus 102 hours, p < 0.001), intensive care unit stay (236 versus 159 hours, p = 0.007), and hospital stay (21.5 versus 11.5 days, p < 0.001); and increased unplanned reoperation (OR 3.7, 95% CI: 1.1 to 12.5, p = 0.032), extracorporeal membrane oxygenation use (OR 4.4, 95% CI: 1.1 to 17.6, p = 0.038), hospital mortality (OR 4.2, 95% CI: 1.2 to 14.5, p = 0.021), and diminished late survival (hazard ratio 4.0, 95% CI: 2.1 to 8.1, p < 0.001). CONCLUSIONS: In neonates with conotruncal anomalies, 22q11DS is associated with prolonged recovery and increased resource utilization. However, despite a small increase in unplanned reoperation, there is no significant impact on early or late survival. In comparison, other genetic syndromes are associated with increased unplanned reoperation, extracorporeal membrane oxygenation use, hospital mortality, and diminished late survival. These findings are important for family counseling and risk stratification.

Unusual Repair of Aortic Coarctation: Transcatheter Intervention Implications.

A 56-year old male was evaluated for exertional dyspnea. He had previously undergone mechanical aortic valve replacement and aortic coarctation repair. Following uncomplicated coronary angiography using Judkins left and right catheters, aortography revealed that the loud murmur was related to an unusual, extra-anatomic surgical repair: transverse aorta to descending aorta bypass. Recognition of an extra-anatomic surgical bypass of coarctation is important, as this repair would leave a continuous murmur on exam.