A 34-Year-Old Thai Man Presenting with Pulmonary Stenosis and Heart Failure 24 Years After Surgical Correction with the Rastelli Procedure for Congenital Dextro-Transposition of the Great Artery, Ventricular Septal Defect, and Pulmonary Atresia.

ABSTRACT

BACKGROUND Dextro-transposition of the great arteries (D-TGA) with a ventricular septal defect (VSD) and pulmonary atresia is an uncommon congenital conotruncal abnormality. Surgical correction is performed using the Rastelli procedure, which includes a ventricular septal patch to direct blood from the left ventricle to the aorta and a valved conduit to connect the right ventricle to the pulmonary artery. This report is of a 34-year-old Thai man who presented with pulmonary stenosis and heart failure 24 years after surgical correction with the Rastelli procedure for congenital D-TGA, VSD, and pulmonary atresia. CASE REPORT A 34-year-old Thai man presented with dyspnea on moderate exertion. His cardiovascular examination revealed a median sternal surgical scar, parasternal heaving, a grade III systolic ejection murmur at the left upper parasternal border, and a single second heart sound. Echocardiography demonstrated degenerative calcification of a severely stenosed pulmonary valve and impaired right ventricular function. A color Doppler M-mode echocardiogram showed VSD patch leakage. A computed tomography scan with 3-dimensional heart reconstruction demonstrated a significantly stenosed branch pulmonary artery. Right and left heart catheterization confirmed the multi-site stenoses were hemodynamically significant. The patient underwent surgery for VSD closure, placement of a right-ventricle-to-pulmonary-artery conduit with a polytetrafluoroethylene graft, and pulmonary artery plasty to correct the stenosis at the branch of the pulmonary artery. CONCLUSIONS The long-term complications of the Rastelli-type operation seen for D-TGA with a VSD and pulmonary atresia included a right-ventricle-to-pulmonary-artery conduit obstruction, VSD patch leakage, and re-stenosis of the peripheral pulmonary stenosis. Multimodal imaging was informative in planning for reoperation.