ABSTRACT
BACKGROUND: There is a growing population of palliated and unpalliated single ventricle physiology patients for whom heart transplantation is the only treatment option available. There is a paucity of reports of heart transplantation in this challenging and growing subset of patients from our part of the world. The purpose of the article is to briefly review our experience in the subgroup and compare it with the available literature. METHODS: This was a single-institution retrospective observational study of 16 patients with single ventricle physiology who were transplanted between 2016 and 2019 and their outcomes. The study groups were divided into those with ventricular dysfunction (group 1), who fare substantially better than those with normal ventricular function (group 2) whose short-term outcomes were poorer. Worsening cyanosis, poor candidature for completion Fontan procedure due to severe atrioventricular valve regurgitation or pulmonary artery anatomy, protein-losing enteropathy, plastic bronchitis, and worsening systemic venous congestion are indications in those with normal ventricular function. RESULTS: Patients with ventricular dysfunction as the main indication had excellent early survival with no early mortality compared to 40% mortality in patients with normal ventricular function. Patients who survived to leave the hospital had however similar long-term outcomes. Two patients with protein-losing enteropathy resolved completely by one month. Normal ventricular function, pulmonary artery stenting, early Fontan failure (6 months), ascites, and need for desensitization were risk factors for early mortality. After the early acute phase of increased risk, the mortality risk plateaued off. CONCLUSION: Transplantation in patients with single ventricle and ventricular dysfunction can be offered with a good early and late outcome. There is a need to have multi-institutional and multi-disciplinary collaboration along with work in basic sciences to better understand the effects of failed Fontan physiology with normal ventricular function.
ABSTRACT
Constrictive pericarditis is a great mimic and has posed a diagnostic dilemma since its first description 300 years ago as "Concretio Cordis." It can mimic restrictive cardiomyopathy, endomyocardial fibrosis, and chronic liver and renal disease. This would perhaps be the first clinical report of constriction in patients undergoing cardiac transplantation. We report two distinct cases with cardiomyopathy requiring cardiac transplantation and the clinical implications of concomitant pericardial constriction. While the first case mimics a natural "cardiac support device," which addresses ventricular remodeling in heart failure by reducing the wall stress, the second case is a case in point against the use of "biological pericardial membrane-like the bovine pericardium," as a pericardial substitute.
ABSTRACT
Multimodality therapies have improved the survival after tumors like Ewing's sarcoma and breast cancer. However, cardiotoxicity following chemotherapy remains an important concern. We report a case series of four patients who presented to our heart team with severe dilated cardiomyopathy along with biventricular involvement. Two of the patients were females and had breast cancer for which they were treated with trastuzumab and had developed chemotherapy induced cardiomyopathy (CCMP). The other two patients were males who had Ewing's sarcoma who developed CCMP following treatment with doxorubicin.
