Department of Pathology and Laboratory Medicine, University of Rochester Medical Center, Rochester, New York.
Pediatr Dev Pathol. 2021 Jan-Feb;24(1):68-72. doi: 10.1177/1093526620970822. Epub 2021 Jan 12.
A term female infant with hypoplastic left heart syndrome underwent Norwood palliation including aortic and pulmonary amalgamation with arch reconstruction, atrial septectomy, and right ventricle to pulmonary artery conduit. Postoperatively, she experienced hypoxemia and lactic acidosis although echocardiogram showed adequate conduit function. She was placed on veno-arterial extracorporeal membrane oxygenation (ECMO) on postoperative day two with improvement. ECMO decannulation was attempted with subsequent cardiac arrest and ultimate failure to resuscitate, eleven days after surgery. Autopsy confirmed clinical findings and evidence of surgical intervention with a patent conduit and neo-aorta. Multiple subendocardial right ventricular dystrophic calcifications involving the outflow tract were identified grossly and histologically with foci of associated myonecrosis. Myocardial calcification may lead to abnormal heart wall motion by increasing rigidity and compromising myocyte function or compromising the conduction system. In this patient, right ventricular turbulence caused by systolic and diastolic flow patterns, including mild tricuspid regurgitation, may have played a role in inducing dystrophic calcification along with surgery and ECMO dependence. Compromised myocyte function from calcifications, right ventricular hypertrophy, lung immaturity, and persistent pulmonary hypertension were likely sources of cardiac strain leading to the patient's demise. This case represents a previously unreported complication of hypoplastic left heart syndrome treatment.
一名患有左心发育不全综合征的女性婴儿接受了 Norwood 姑息手术，包括主动脉和肺动脉融合、弓部重建、房间隔切开术和右心室肺动脉通道。术后，尽管超声心动图显示导管功能充足，但她仍出现低氧血症和酸中毒。术后第二天，她因低氧血症和酸中毒而被放置在静脉-动脉体外膜肺氧合（ECMO）上，随后情况得到改善。试图拔除 ECMO 导管后，患儿出现心脏骤停，最终抢救无效，术后第 11 天死亡。尸检证实了临床发现和手术干预的证据，包括导管和新主动脉通畅。大体和组织学上均发现右心室心内膜下多处弥漫性营养不良性钙化，累及流出道，伴有局灶性心肌坏死。心肌钙化可通过增加心肌僵硬和损害心肌细胞功能或损害传导系统，导致异常的心室壁运动。在本例患者中，收缩期和舒张期血流模式引起的右心室湍流，包括轻度三尖瓣反流，可能与手术和 ECMO 依赖一起导致了营养不良性钙化。钙沉积导致的心肌细胞功能障碍、右心室肥厚、肺不成熟和持续性肺动脉高压可能是导致患者死亡的心脏负荷增加的原因。该病例代表了左心发育不全综合征治疗的一种以前未报道的并发症。
