Molina-Lopez Victor H, Arraut-Hernandez Christina, Nieves-La Cruz Carlos, Almodovar-Adorno Alvin A, Rivera-Babilonia Jaime
Cardiology, Veteran Affairs Caribbean Healthcare System, San Juan, PRI.
Interventional Cardiology, Veteran Affairs Caribbean Healthcare System, San Juan, PRI.
Cureus. 2023 Feb 23;15(2):e35369. doi: 10.7759/cureus.35369. eCollection 2023 Feb.
This case report describes a rare variant of partial anomalous pulmonary venous connections (PAPVCs) in a patient who presented with an insidious progression of dyspnea on exertion as an adult, leading to the diagnosis of PAPVC. The patient had an anomalous right upper pulmonary vein connecting to an anomalous pulmonary-azygos trunk that connected to the cranial superior vena cava (SVC), producing a large left-to-right extracardiac shunt. The diagnosis of PAPVC was made after evaluating for causes of right heart chamber enlargement. This case highlights the importance of considering PAPVC as a potential cause of unclear etiology for exertional dyspnea, right-sided chamber enlargements, and intact atrial septum. The onset and severity of symptoms in patients with PAPVC depend on various factors, including the number of pulmonary veins, site of connection, pulmonary vascular resistance, atrial compliance, and the presence of other congenital heart defects. Therefore, clinicians should maintain a high level of suspicion for PAPVC in patients with these types of symptoms.
本病例报告描述了一名成年患者出现的一种罕见的部分性肺静脉异位连接(PAPVC)变体,该患者隐匿性地逐渐出现劳力性呼吸困难,最终诊断为PAPVC。患者有一条异常的右上肺静脉连接到一条异常的肺奇静脉干,该肺奇静脉干连接到头侧上腔静脉(SVC),产生大量左向右的心外分流。在评估右心腔扩大的原因后做出了PAPVC的诊断。本病例强调了将PAPVC视为劳力性呼吸困难、右心腔扩大且房间隔完整但病因不明的潜在原因的重要性。PAPVC患者症状的发作和严重程度取决于多种因素,包括肺静脉数量、连接部位、肺血管阻力、心房顺应性以及其他先天性心脏缺陷的存在。因此,临床医生应对有此类症状的患者高度怀疑PAPVC。