Aims: To undertake a systematic review and meta-analysis to determine the influence of tricuspid regurgitation (TR) severity on mortality. Methods and results: We performed a systematic search for studies reporting clinical outcomes of patients with TR. The primary endpoint was all-cause mortality and secondary endpoints were cardiac mortality and hospitalization for heart failure (HF). Overall risk ratios (RR) and 95% confidence intervals (CIs) were derived for each endpoint according to the severity of TR by meta-analysing the effect estimates of eligible studies. Seventy studies totalling 32 601 patients were included in the analysis, with a mean (±SD) follow-up of 3.2 ± 2.1 years. Moderate/severe TR was associated with a two-fold increased mortality risk compared to no/mild TR (RR 1.95, 95% CI 1.75-2.17). Moderate/severe TR remained associated with higher all-cause mortality among 13 studies which adjusted for systolic pulmonary arterial pressures (RR 1.85, 95% CI 1.44-2.39), and 15 studies, which adjusted for right ventricular (RV) dysfunction (RR 1.78, 95% CI 1.49-2.13). Moderate/severe TR was also associated with increased cardiac mortality (RR 2.56, 95% CI 1.84-3.55) and HF hospitalization (RR 1.73, 95% CI 1.14-2.62). Compared to patients with no TR, patients with mild, moderate, and severe TR had a progressively increased risk of all-cause mortality (RR 1.25, 1.61, and 3.44, respectively; P < 0.001 for trend). Conclusions: Moderate/severe TR is associated with an increased mortality risk, which appears to be independent of pulmonary pressures and RV dysfunction.

Tricuspid regurgitation is associated with increased mortality independent of pulmonary pressures and right heart failure: a systematic review and meta-analysis.

Background Beginning in 2000 and accelerating in 2004, the Berlin Heart EXCOR (Berlin Heart Inc Woodlands, TX) became the first pediatric-specific ventricular assist device (VAD) applied throughout North America for children of all sizes. This retrospective study analyzed the initial Berlin Heart EXCOR pediatric experience as a bridge to transplantation. Methods Between June 2000 and May 2007, 97 EXCOR VADs were implanted in North America at 29 different institutions. The analysis is limited to 73 patients (75%) from 17 institutions, for which retrospective data were available. Results Median age and weight at VAD implant were 2.1years (range, 12 days–17.8 years) and 11 kg (range, 3–87.6 kg), respectively. The primary diagnoses were dilated cardiomyopathy in 42 (58%), congenital heart disease in 19 (26%), myocarditis in 7 (10%), and other cardiomyopathies in 5 (7%). Pre-implant clinical condition was critical cardiogenic shock in 38 (52%), progressive decline in 33 (45%), or other in 2 (3%). Extracorporeal membrane oxygenation was used as a bridge to EXCOR in 22 patients (30%). Device selection was left VAD (LVAD) in 42 (57%) and biventricular assist devices (BiVAD) in 31 (43%). The EXCOR bridged 51 patients (70%) to transplant and 5 (7%) to recovery. Mortality on the EXCOR was 23% ( n = 17) overall, including 35% (11 of 31) in BiVAD vs 14% (6 of 42) in LVAD patients ( p = 0.003). Multivariate analysis showed younger age and BiVAD support were significant risk factors for death while on the EXCOR. Conclusions This limited but large preliminary North American experience with the Berlin Heart EXCOR VAD as a bridge to cardiac transplantation for children of all ages and sizes points to the feasibility of this approach. The prospective investigational device evaluation trial presently underway will further characterize the safety and efficacy of the EXCOR as a bridge to pediatric cardiac transplantation.

Bridging children of all sizes to cardiac transplantation: The initial multicenter North American experience with the Berlin Heart EXCOR ventricular assist device

Heart failure (HF) in the United States is well recognized as a major public health problem, with over 900,000 hospital admissions annually in the United States, and greater than 250,000 deaths per year. The great majority of heart failure occurs in adults. In children, the scope of the problem is less well defined, but recent data from the Pediatric Cardiomyopathy Registry suggest an annual incidence of 1.13 cases of cardiomyopathy per 100,000 children. 1 While some of this represents asymptomatic disease, the burden of disease overall is nonetheless quite high. In the Pediatric Cardiomyopathy Registry, the majority of children with cardiomyopathy also had HF, with mortality rates of 13.6% at 2 years in dilated forms of cardiomyopathy. The etiology of heart failure differs greatly between children and adults. Children in the Pediatric Cardiomyopathy Registry had a recognizable syndrome or genetic diagnosis in 27% of cases, with an additional 5% of cases due to myocarditis. Furthermore, a large percentage of children with end-stage HF (between 25% and 75%, depending upon the age group) have an underlying diagnosis of congenital heart disease. 2 In contrast to adult patients, ischemic heart disease is rare in children. There is a large, and rapidly growing literature addressing HF treatment for adult patients, with a much smaller literature concerning HF therapy in children. Excellent guidelines for adult patients have recently been published, but given the significant differences between adult and pediatric patients with HF, there is little reason to believe that these guidelines are directly applicable to children. 3 Accordingly, in this document we have attempted to summarize the relevant literature and synthesize management guidelines for children with HF. The document that follows has been prepared in a consensus fashion, with input from pediatric cardiologists at multiple sites throughout the United States and Canada.

International Society for Heart and Lung Transplantation: Practice guidelines for management of heart failure in children.

Objectives. To evaluate the long-term neurodevelopmental outcome of infants who underwent cardiac surgery and required extracorporeal membrane oxygenation (ECMO) support, and to examine variables that predict death or disability in these patients. Methods. We studied all infants who had congenital heart disease and were supported postoperatively with ECMO from 1990 to 2001 at our institution (n = 53). Medical records were reviewed retrospectively to obtain clinical variables. Neurologic and age-appropriate developmental examinations occurred at ages 1, 1.5, 2.5, and 4.5 years. Median age at follow-up was 55 months (9–101). Cognitive outcome was defined as suspect when scores were between 1 and 2 SD below the mean for age and abnormal when scores were >2 SD below mean for age. Neuromotor outcome was defined as suspect when the patient manifested clumsiness, tremor, or mild tone and reflex changes without functional limitations, and abnormal when there were functional limitations. Results. In-hospital survival was 17 (32%) of 53. Of survivors, 14 (88%) of 16 are living and 1 patient was lost to follow-up. Of the 53 patients, 7 survived completely intact (13%). Seven (50%) of 14 patients had a normal cognitive outcome, 3 (21%) had a suspect cognitive outcome, and 4 (29%) were abnormal. Ten (72%) of 14 patients had a normal neuromotor outcome, 1 (7%) patient had a suspect neuromotor outcome, and 3 (21%) were abnormal. No survivor with an aortic cross-clamp time >40 minutes had a normal cognitive outcome. Nonsurvivors were more likely than survivors to have had cardiac arrest as an indication for ECMO (31% vs 6%), to have had a longer aortic cross-clamp time (mean 73 minutes vs 32 minutes), and to have required continuous arteriovenous hemofiltration (78% vs 35%). The age and weight at cannulation, gender, cardiac diagnosis, interval from surgery to ECMO, cardiopulmonary bypass time, diagnosis of sepsis or mediastinitis, and duration of ECMO were not significantly associated with survival. Conclusions. Although mortality was 68% in infants who had congenital heart disease and were treated with ECMO postoperatively, of those who survive to hospital discharge, 75% have a normal neuromotor outcome and 50% have a normal cognitive outcome. These high rates of mortality and disability suggest that increased attention be paid to neuroprotection in these complex disorders.

https://pediatrics.aappublications.org/content/pediatrics/111/6/e671.full.pdf

Neurodevelopmental outcome of infants supported with extracorporeal membrane oxygenation after cardiac surgery.

Venoarterial extracorporeal membrane oxygenation (VA-ECMO) in pediatric cardiac support.

We describe a case of a 40-year-old man who presented with recent-onset, rapidly decompensating heart failure, and who was found to have giant cell myocarditis (GCM) on biopsy. The patient responded to myocardial rest on a biventricular assist device, as well as immunosuppression that included eradication of T cells using OKT3 therapy, coupled with high-dose steroids. The patient was successfully weaned off mechanical support, and ultimately discharged home, where he continues to do well. Based on a review of the clinical and experimental literature, we believe treatment with T-lymphocytic cytolytic therapy may be beneficial. Further studies using this therapy in the treatment of GCM are warranted.

Giant cell myocarditis in a young man responsive to T-lymphocyte cytolytic therapy.

167: Impact of Age on Outcomes of Ventricular Assist Devices Utilized as Bridge to Cardiac Transplantation

Cardiac Surgery in Adults with Congenital Heart Disease: Operation and Reoperation

Cardiac Transplantation in Patients with Congenital Heart Disease

Extracorporeal membrane oxygenation (ECMO) has been used for pediatric cardiac support in settings of expected mortality due to severe myocardial dysfunction. We reviewed the records of 34 children (<18 years) placed on ECMO between March 1995 and May 1999. Demographic, cardiac, noncardiac, and outcome variables were recorded. Data were subjected to univariate analysis to define predictors of outcome. Eighteen patients were placed on ECMO after cardiac surgery (Group A); seven of 18 were weaned off ECMO, and four survived to discharge (22%). Thirteen patients were placed on ECMO as a bridge to cardiac transplantation (Group B), six of 13 received a heart transplant, one recovered spontaneously, and six survived to discharge (46%). Three patients were placed on ECMO for failed cardiac transplantation while awaiting a second transplant (Group C); one recovered graft function, two received a second heart transplant, and two of three survived (66%). The primary cause of death was multiorgan system failure (68%). Group A patients supported on ECMO for more than 6 days did not survive. Mediastinal bleeding complications and renal failure requiring dialysis were associated with nonsurvival. We conclude that ECMO as a bridge to cardiac transplant was more successful than ECMO support after cardiotomy. Mediastinal bleeding and renal failure were associated with poor outcome. Recovery of cardiac function occurred within the first week of ECMO support if at all. Longer support did not result in survival without transplantation.

Extracorporeal membrane oxygenation for cardiac support in pediatric patients.

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