Hinrichs Lena, Horacek Michael, Neudorf Ulrich, Schlosser Thomas, Rassaf Tienush, Totzeck Matthias
Department of Cardiology and Vascular Medicine, West German Heart and Vascular Center Essen, University Hospital Essen, Essen, Germany.
Department of Paediatrics, University Hospital Essen, Essen, Germany.
Am J Case Rep. 2019 Feb 5;20:154-158. doi: 10.12659/AJCR.912191.
BACKGROUND The diagnostic work-up and treatment of pulmonary hypertension can be complex. Pulmonary arterial hypertension (PAH), pulmonary hypertension second to lung or heart diseases and thromboembolic pulmonary hypertension, and other rare causes of pulmonary hypertension such as congenital heart diseases must be considered in the differential diagnostic work-up. CASE REPORT We report on a patient who has been treated for PAH over many years. At the age of 65, progressive symptoms required a complete re-evaluation. Here, a complex shunt vitium with a partial anomalous pulmonary venous return (PAPVR) and a sinus venosus defect (SVD) was diagnosed. CONCLUSIONS PAPVR is a rare congenital heart disease that is often associated with an SVD. It is usually diagnosed during childhood but may also be detected in adult patients who develop pulmonary hypertension and dyspnea as primary symptoms. The initial predominant left-to-right shunting associated with this disease may be undetected for years, with a slow development of right heart failure with right heart volume overload and pulmonary hypertension. Early detection is important, with a subsequent surgical intervention.
背景 肺动脉高压的诊断检查和治疗可能较为复杂。在鉴别诊断检查中，必须考虑肺动脉高压（PAH）、继发于肺部或心脏疾病的肺动脉高压以及血栓栓塞性肺动脉高压，还有其他罕见的肺动脉高压病因，如先天性心脏病。病例报告 我们报告了一名多年来一直接受PAH治疗的患者。65岁时，进行性症状需要全面重新评估。在此，诊断出一种复杂的分流性病变，伴有部分性肺静脉异位引流（PAPVR）和静脉窦缺损（SVD）。结论 PAPVR是一种罕见的先天性心脏病，常与SVD相关。它通常在儿童期被诊断，但也可能在以肺动脉高压和呼吸困难为主要症状的成年患者中被发现。与该疾病相关的最初主要的左向右分流可能多年未被发现，随着右心容量超负荷和肺动脉高压导致右心衰竭的缓慢发展。早期检测很重要，随后需进行手术干预。
