肺動脈瓣閉鎖及完整心室中隔之一病例報告

Pulmonary Atresia with Intact Ventricular Septum Report of One Case

黃碧桃(Be-Tau Hwang) ； 蕭遺生(I-Sen Shaio) ； 鄭國琪(Kwok-Kei Cheng) ； 孟春昌(C. C. Laura Meng)

《Acta Paediatrica Sinica》 19卷4期 (1978/12) Pp. 334-340

摘要

本文報告一例患肺動脈瓣閉鎖及完整心室中隔的女嬰：出生第2天發現患先天性心臟病，第九天接受心臟導管檢查及心臟血管攝影證實診斷。雖因家屬之延誤至第21天才接受封閉式肺動脈瓣切開術，但手術仍成功。因病例罕見，於臺灣文獻中，仍未見病例報告，故提出報告之。

並列摘要

Pulmonary atresia with intact ventricular septum is a rare congenital heart disease. A 2-month-old infant with this disease who underwent a successful surgical correction is reported and the literature reviewed. The clinical features consisted of onset of cyanosis and congestive heart failure early since the second day of life. The chest film demonstrated evidences of decreased pulmonary blood flows and cardiomegaly, chiefly of the right atrium withconcave pulmonary segment. Electrocardiograms demonstrated right axis deviation of the mean QRS axis and also left ventricular hypertrophy.
The diagnosis was confirmed at age of nine days by cardiac catheterization and angiocardiographic studies. The salient findings encountered were failure to insert the catheter into the pulmonary arteries, high right atrial pressure with big ”a” waves, oxygen desaturation in the left atrium, left ventricle and aorta, hypoplasia of the right ventricle, tricuspid regurgitation, appearance of myocardial sinusoids and abnormal coronary circulation, right atrium to left atrium shunt and also a patent ductus arteriosus.
Emergency closed pulmonary valvotomy was performed on the 21st day of her life. Ten days after surgery, repeat cardiac catheterization and angiocardiographic studies demonstrated a good surgical result.

並列關鍵字

pulmonary atresia ； tricuspid regurgitation ； patent ductus arteriosus ； closed pulmonary valvotomy