Heart transplantation for patients with single ventricle physiology

Ganapathy Subramaniam Krishnan; Pradeep Gnanasekharan; Dhruva Sharma; Anitha Chandrashekhar; Kemundel Genny Suresh Rao; Komarakshi Rajagopalan Balakrishnan

doi:10.1007/s12055-021-01241-x

Heart transplantation for patients with single ventricle physiology

Indian J Thorac Cardiovasc Surg. 2021 Nov;37(6):647-661. doi: 10.1007/s12055-021-01241-x. Epub 2021 Oct 7.

Authors

Ganapathy Subramaniam Krishnan¹, Pradeep Gnanasekharan¹, Dhruva Sharma², Anitha Chandrashekhar¹, Kemundel Genny Suresh Rao³, Komarakshi Rajagopalan Balakrishnan¹

Affiliations

¹ Institute of Heart and Lung Transplant and Mechanical Circulatory Support, MGM Healthcare, No. 72, Nelson Manickam Road, Aminjikarai, Chennai, 600029 Tamil -Nadu India.
² Department of Cardiothoracic and Vascular Surgery, SMS Medical College & Attached Hospitals, J L N Marg, Jaipur, 302001 Rajasthan India.
³ Cardiac Anaesthesia & Critical Care, MGM Healthcare, No. 72, Nelson Manickam Road, Aminjikarai, Chennai, 600029 Tamil-Nadu India.

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.

Keywords: Cardiac transplantation; Congenital heart disease; Single ventricle physiology.