ワタナベ　ヨシノリ   Watanabe Yoshinori   渡邉　善則    所属   東邦大学  医学部 医学科    職種   特任教授
言語種別	英語
発表タイトル	Ventricular septal defect repairs in infants with left ventricular noncompaction and severe pulmonary hypertension.
会議名	The 5th World Congress of Paediatric Cardiology and Cardiac Surgery.
学会区分	国際学会
発表形式	ポスター掲示
講演区分	一般
発表者・共同発表者	Hara M†, Ozawa T†, Hamada S†, Masuhara H†, Teramoto C†, Saji T†, Watanabe Y†, Koyama N†.
発表年月日	2009/06/24
開催地 （都市, 国名）	Cairns, Australia
概要	Background: Left ventricular noncompaction (LVNC) is categorized as an unclassified cardiomyopathy. Intracardiac repair (ICR) for congenital heart disease (CHD) complicated by LVNC has been rarely described. Case 1: A 2-month-old infant was referred and the echocardiogram revealed a large perimembranous VSD with LVNC and pulmonary hypertension (PH). Cardiac catheterization demonstrated that RV pressure was almost equal to LV pressure. To relieve cardiac volume overload and progressive PH, ICR was done. However, low cardiac output syndrome developed. Decreased urine output required peritoneal dialysis (PD); PH crisis like event needed nitric oxide (NO) inhalation. Intensive treatment improved hemodynamic and respiratory status and he was weaned from PD. Nitric oxide inhalation therapy was replaced to sildenafil administration. Three years after the repair, he is doing well without sildenafil administration. Case 2: A 5-month-old infant was admitted and the echocardiogram had demonstrated a perimembranous VSD with LVNC and PH. Cardiac catheterization showed that RV pressure was 90% of LV pressure. The malaligned VSD was closed by using a patch. She also needed PD and NO inhalation after the repair; however both of them were discontinued within the first week. She is now doing well with taking sildenafil 1 month after the surgery. Summary: Reports concerning the VSD repairs with severe PH and LVNC are very rare; we experienced the similar 2 cases. Severity of PH might be amplified by LVNC. Because our patients continue to be at risk for heart failure, arrhythmia, and thromboembolism, careful monitoring should continue.