A Case Report of the Shone Syndrome in Association with Coronary Abnormality

Abstract

Shone syndrome or complex is defined by sequential left-sided heart stenosis, including supravalvular mitral membrane, valvular mitral stenosis (MS) by a parachute mitral valve, subaortic stenosis, and aortic coarctation. It is a rare entity which occurs most frequently in its incomplete form, a 31-year-old man who referred to our adult congenital clinic with a chief complaint of recent chest discomfort. He had a history of hypertension since last year. Echocardiography revealed congenital severe MS (parachute-like mitral valve), two papillary muscles with decreased space between them and underdeveloped lateral papillary muscle. Other findings of echocardiography were bicuspid aortic valve (fusion of the right coronary cusp [RCC] and left coronary cusp) with moderate-to-severe aortic stenosis (AS) and moderate eccentric aortic insufficiency. Deformed aortic arch (gothic arch) and aortic coarctation were also reported in transthoracic echocardiography. We also found an abnormal vessel like density in 5-chamber view that suggested bleb sign and retroaortic course of the left circumflex (LCX). The patient was scheduled for coronary angiography and intervention for coarctation of the aorta, which revealed an abnormally originated LCX from RCC, significant coarctation of the aorta, and significant AS. Aortic coarctoplasty with CP STENT 8 × 39 mounted on balloon Altosa-XL-Gemini 26 × 40 was done in this session. Shone complex is a rare anomaly that may need multiple procedures for correction. Echocardiography is a specially important modality in these patients for diagnosis and evaluation of severity. By this modality, even we can diagnose some coronary artery origin abnormality.