Department of Cardiology, OLV Hospital Aalst, Aalst, Belgium.
Department of Congenital and Structural Cardiology, UZ Leuven, Leuven, Belgium.
Am J Case Rep. 2022 Aug 17;23:e937305. doi: 10.12659/AJCR.937305.
BACKGROUND Unilateral absence of a pulmonary artery (UAPA) is a rare congenital cardiovascular malformation. More than half of UAPA cases have other cardiac lesions such as tetralogy of Fallot or septal defects. Clinical manifestations are diverse and range from heart failure after birth to an incidental finding on chest imaging during adulthood. Whereas early surgical revascularization is recommended in infancy, this is usually not feasible in the adult population. Management in these patients is aimed at treating the complications of UAPA. CASE REPORT A 67-year-old woman was evaluated for subacute right heart failure. An echocardiogram revealed pulmonary stenosis, tricuspid regurgitation, and depressed right ventricular function. Chest computed tomography (CT) showed absence of the right pulmonary artery. Additionally, there was a lung tumor in the right upper lobe. Right-heart catheterization confirmed a critically obstructed pulmonary orifice shown by hemodynamic collapse when crossing the pulmonary valve with the catheter. The patient underwent pulmonary valve balloon dilatation with right ventricular outflow tract stenting followed by percutaneous implantation of a balloon-expandable stent-valve. The clinical course was complicated by a complete heart block. Oncologic management consisted of stereotactic radiotherapy. CONCLUSIONS The combination of UAPA, pulmonary stenosis, and lung cancer is rare. Pulmonary stenosis worsens prognosis in adult patients with UAPA, but also constitutes a therapeutic target. The decision to treat the pulmonary stenosis should be based on the severity of stenosis, the degree of pulmonary hypertension, and individual anatomy. We chose percutaneous pulmonary valve implantation because our patient had a critical pulmonary stenosis with normal pulmonary pressures.
肺动脉单侧缺如（UAPA）是一种罕见的先天性心血管畸形。超过一半的 UAPA 病例伴有其他心脏病变，如法洛四联症或室间隔缺损。临床表现多种多样，从出生后心力衰竭到成年后胸部影像学的偶然发现。虽然建议在婴儿期进行早期外科血运重建，但在成年人群中通常不可行。这些患者的治疗方法旨在治疗 UAPA 的并发症。
一名 67 岁女性因亚急性右心衰竭就诊。超声心动图显示肺动脉瓣狭窄、三尖瓣反流和右心室功能降低。胸部 CT 显示右肺动脉缺如。此外，右上肺叶还有一个肺肿瘤。右心导管检查证实肺动脉瓣口严重阻塞，当导管穿过肺动脉瓣时出现血流动力学崩溃。患者接受了肺动脉瓣球囊扩张和右心室流出道支架置入术，随后进行了球囊扩张支架瓣膜的经皮植入术。该患者的临床病程复杂，出现完全性心脏阻滞。肿瘤治疗包括立体定向放疗。
UAPA、肺动脉瓣狭窄和肺癌同时存在的情况较为罕见。肺动脉瓣狭窄使成年 UAPA 患者的预后恶化，但也是一个治疗靶点。治疗肺动脉瓣狭窄的决策应基于狭窄的严重程度、肺动脉高压的程度和个体解剖结构。我们选择经皮肺动脉瓣植入术，因为我们的患者存在严重的肺动脉瓣狭窄，但肺动脉压力正常。
