Khajali Zahra, Arabian Maedeh, Aliramezany Maryam
Rajaie Cardiovascular Medical and Research Center, Iran University of Medical Sciences, Tehran, Iran.
Cardiovascular Research Center, Institute of Basic and Clinical Physiology Sciences, Kerman University of Medical Sciences, Kerman, Iran.
J Cardiovasc Thorac Res. 2020;12(3):237-243. doi: 10.34172/jcvtr.2020.36. Epub 2020 Aug 27.
Hypoplastic right ventricle is a rare congenital disease usually associated with pulmonary atresia or tricuspid atresia. Isolated right ventricular hypoplasia is a rare anomaly without important valvular abnormalities. It is associated with inter atrial septal defects leading to the right-to-left shunting of blood. Patients with isolated right ventricular hypoplasia usually have different and variable courses. In some patients, it is recognized in the perinatal period and necessitates prompt intervention; nonetheless, there are some reports of this anomaly in old age with no significant symptoms. In this report, we describe the clinical data and management of 6 adult cases with isolated right ventricular hypoplasia treated medically or surgically based on the severity of the disease and symptoms and then offer an in-depth discussion regarding this rare anomaly.
右心室发育不全是一种罕见的先天性疾病，通常与肺动脉闭锁或三尖瓣闭锁相关。孤立性右心室发育不全是一种罕见的异常情况，没有重要的瓣膜异常。它与房间隔缺损相关，导致血液右向左分流。孤立性右心室发育不全的患者通常有不同且多变的病程。在一些患者中，在围产期被识别出来，需要立即进行干预；尽管如此，也有一些关于这种异常情况在老年时出现且无明显症状的报道。在本报告中，我们描述了6例接受内科或外科治疗的成年孤立性右心室发育不全患者的临床资料及治疗情况，治疗是根据疾病的严重程度和症状进行的，然后对这种罕见的异常情况进行了深入讨论。
