Department of Internal Medicine, Family Medicine and Labor Medicine, Faculty of Medicine, Carol Davila University of Medicine and Pharmacy, Bucharest, Romania;
Rom J Morphol Embryol. 2022 Jan-Mar;63(1):49-53. doi: 10.47162/RJME.63.1.04.
Isolated right ventricle hypoplasia (IRVH) is a disease characterized by an underdeveloped right ventricle. It is a congenital heart disease than can associate heterogeneous structural defects and nonspecific clinical features, which can often present a challenging therapeutic management. In this article, there are presented diagnostic methods and treatment options for right ventricle hypoplasia (RVH) according to clinical features, patients age and associated structural heart defects. RVH has a different prognosis in accordance with the severity of the heart defects and the patient's age at which the diagnosis is established. Thus, isolated forms of RVH generally present mild structural and functional defects that can be associated with the onset of symptoms in adolescence or even in adulthood. In these cases, atrial septal defect closure with or without superior cavo-pulmonary anastomosis can be the only procedures needed to correct the hemodynamic abnormalities and relief the symptomatology. Patients with severe form of RVH associated with complex cardiac malformations and onset of the symptoms in the neonatal period require prompt intervention and necessitate palliative procedures. In the long term, these patients could need multiple reinterventions. The family physician should be aware of the cardiac origin of isolated symptoms or clinical signs, such as exertional dyspnea or clubbing fingers, and send the patient for pediatric cardiological evaluation.
孤立性右心室发育不良（IRVH）是一种以右心室发育不全为特征的疾病。它是一种先天性心脏病，可能伴有异质性结构缺陷和非特异性临床特征，这往往会带来具有挑战性的治疗管理。本文根据临床特征、患者年龄和相关结构性心脏缺陷，介绍了右心室发育不良（RVH）的诊断方法和治疗选择。RVH 的预后因心脏缺陷的严重程度和诊断时患者的年龄而异。因此，孤立性 RVH 一般表现为轻度结构和功能缺陷，可在青少年甚至成年期出现症状。在这些情况下，房间隔缺损关闭术，伴或不伴上腔静脉-肺动脉吻合术，可能是纠正血液动力学异常和缓解症状所需的唯一手术。伴有严重 RVH 和复杂心脏畸形的患者，以及在新生儿期出现症状的患者，需要及时干预，并需要姑息性手术。从长远来看，这些患者可能需要多次再介入。家庭医生应该意识到孤立性症状或临床体征的心脏来源，如运动时呼吸困难或杵状指，并将患者转介给儿科心脏病专家进行评估。
