Right Ventricular Reverse Remodeling After Lung Transplantation and Endocardial Repair for Simple Congenital Heart Disease Complicated By Pulmonary Arterial Pulmonary Hypertension

Abstract

In patients with simple congenital heart disease combined with pulmonary arterial hypertension / idiopathic pulmonary arterial hypertension, lung transplantation and endocardial repair are the last options when medical treatment becomes difficult. The purpose of this study was to analyze whether lung transplantation and endomyocardial repair can restore right ventricular remodeling to normal, improve it only partially, or remodel it further than before surgery. The subjects were 3 patients with atrial septal defects and 3 patients with ventricular septal defects who underwent lung transplantation and endocardial repair for simple congenital heart disease and pulmonary arterial hypertension/idiopathic pulmonary arterial hypertension from 2012 to 2015, for a total of 6 cases. Methods were based on echocardiographic data from the medical records, and the improvement and recovery of right ventricular morphology and right heart dysfunction were analyzed preoperatively, at 1 week, 1 month, 6 months, and 12 months after the operation. The patients had a right ventricular systolic pressure Median (IQR) of 22.3 mmHg (19.3-27.2 mmHg), a right ventricular central diameter of 31 mm (29.9-33.5 mm), and a right ventricular area change rate of 44.5% (40.5%). The right ventricular area change of 44.5% (40.8-49%) improved and returned to the normal range. However, right ventricular wall thickening of 9.3 mm (8.3-9.9 mm) did not improve, and right ventricular reverse modeling was only partially improved.