Complex Congenital Heart Disease: Is Heart Transplantation an Option?

INTRODUCTION: Within the heart transplant (HT) population, few patients have complex congenital heart disease (CHD) underlying disease. Our objective is to assess the complications and follow-up of patients with CHD transplanted in our center (1991-present). MATERIAL AND METHODS: Retrospective analysis of patients older than 14 years old with CHD and HT. Clinical and surgical variables were analyzed. RESULTS: Ten patients (age 25 ± 7 years old, 60% male) were analyzed, which represents 2.2% of the entire series of HT; 9 of the 10 patients were electively transplanted and 1 was under support with extracorporeal membrane oxygenation. Compared with the rest of the transplanted patients, patients with CHD had a lower median age (25 [25-36] vs 53 [15-69]; P < .009); more cardiac surgeries prior to HT (100% vs 14.4%; P < .001); a lower percentage of cardiovascular risk factors (CVRF; 0% vs 60%; P < .001). The left ventricle ejection fraction also showed statistically significant differences (33.5 [12-67] vs 20 [6-70]). CONCLUSIONS: Our patients with CHD and HT are younger and have less CVRF and more cardiac surgeries, which highlights that it is a subgroup with clear clinical differences in its comorbidity and pretransplantation assessment. They also require longer extracorporeal circulation time, more hours of intubation, and more days in the intensive care unit. Primary graft failure is more common in patients with CHD. Therefore, survival at 1-month follow-up is lower than the rest of the series and equalizes after the year of follow-up. This long-term survival reaffirms the possibility of HT in CHD despite the fact that its postoperative period is more difficult.

Implantable cardioverter defibrillator for primary prevention in patients with severe ventricular dysfunction awaiting heart transplantation.

BACKGROUND: Despite an increase in patients with end-stage heart failure, the acceptance rate has been going down in recent years owing to a change in donor demographics. Furthermore, the rate of emergency heart transplantation has progressively increased. The result is an increase in the time awaiting heart transplantation in elective patients and therefore in the risk of sudden death in this population. Implantable cardioverter defibrillators (ICDs) could be a preventive option in these cases. However, indications for the implantation in this population are not well established. OBJECTIVE: We sought to evaluate the effectiveness of ICDs for primary prevention in patients with left ventricular ejection fraction (LVEF) ≤ 30% included on the heart transplantation list. METHODS: Records from patients accepted for heart transplantation in our institution from January 1, 2006, to July 30, 2012, and whose LVEF was <31% were reviewed. Patients who received ICDs for primary prevention (n = 28) were compared with patients without ICDs (n = 51). Descriptive and univariate (χ(2) and t tests) statistics and Kaplan-Meier survival curves were used for analyses. RESULTS: With a median follow-up of 77 days (range 1-1,231), the overall mortality in the ICD group was 7.1% (2/28) and in the non-ICD group was 17.6% (9/51; P = .062). The main cause of death in patients without ICDs was sudden death (5/9, 55.6%), followed by heart failure (4/9, 44.4%). In patients with ICDs, heart failure was the only reported cause of death. Appropriate ICD therapies were recorded in 42.9% (12/28) in this population. CONCLUSIONS: This study suggests that ICD could reduce the risk of sudden death in patients with LVEF ≤ 30% while awaiting heart transplantation. However, more studies are needed to confirm these results.