Sugiyama Kayo, Matsuyama Katsuhiko, Ogino Hitoshi
Department of Cardiac Surgery, Aichi Medical University Hospital, Nagakute, Japan.
Department of Cardiovascular Surgery, Tokyo Medical University Hospital, 6-7-1 Nishishinjuku, Shinjuku-ku, Tokyo, 160-0023, Japan.
J Cardiothorac Surg. 2023 Oct 5;18(1):270. doi: 10.1186/s13019-023-02371-6.
Pulmonary hypertension (PH)-associated with left heart disease (Nice PH classification group II) improves when the latter is treated; however, the treatment of PH concomitant with group I PH due to congenital heart disease is difficult, and the optimal pharmacotherapy is controversial. Intervention strategies for the left-sided atrioventricular valve in partial atrioventricular septal defect (AVSD) are problematic.
A 37-year-old woman who had undergone patch closure for a partial AVSD and mitral valve replacement with a rather large bioprosthesis at the juxta-annular position for mitral regurgitation 12 years earlier was referred to our institute because of severe PH. Echocardiography revealed calcification resulting in severe stenosis of the bioprosthesis and protrusion of its stent post into the left ventricular outflow tract; therefore, redo mitral valve replacement at the supra-annular position was performed using a mechanical valve. Combined group I and II PH gradually improved with meticulous postoperative medical management.
Severe PH due to stent post protrusion and structural valve deterioration in AVSD was successfully treated with redo mitral valve replacement. The present case was complicated with group I and II PH, for which medical therapy in conjunction with surgical treatment yielded an optimal therapeutic effect.
与左心疾病相关的肺动脉高压（PH，肺动脉高压分类中的II类）在左心疾病得到治疗时会有所改善；然而，先天性心脏病所致I类PH合并PH的治疗较为困难，最佳药物治疗存在争议。部分房室间隔缺损（AVSD）中左侧房室瓣的干预策略存在问题。
一名37岁女性，12年前因部分AVSD接受补片修补术，并因二尖瓣反流在瓣环附近位置用相当大的生物瓣进行二尖瓣置换，因严重PH转诊至我院。超声心动图显示生物瓣钙化导致严重狭窄，其支架柱突入左心室流出道；因此，使用机械瓣在瓣环上方位置再次进行二尖瓣置换。通过精心的术后药物治疗，I类和II类合并PH逐渐改善。
因AVSD中支架柱突出和人工瓣膜结构退化导致的严重PH通过再次二尖瓣置换成功治疗。本病例合并I类和II类PH，药物治疗联合手术治疗产生了最佳治疗效果。
