Rangel A, Argüero R, Albarrán H, Ocampo S, Baleón R, Nandayapa O, Careaga G, Quintero L R, Chávez E
Departamento de Hemodinamia, Hospital de Especialidades, Centro Médico La Raza, IMSS, Calles de Seris y Zaachila, 02990 México, D.F.
Arch Inst Cardiol Mex. 2000 Nov-Dec;70(6):596-602.
We describe the case of a 26-year-old female in functional class I (NYHA), with aortic origin of the right pulmonary artery associated with a persistent ductus arteriosus and severe pulmonary artery hypertension (101/40-70 mm Hg), which remained elevated (89/40-60 mm Hg) after the administration of 100% oxygen. Right pulmonary artery pressure (125/60-86 mm Hg) was higher than that of main pulmonary artery and similar to aorta pressure. The patient was successfully treated: surgical closure of the ductus arteriosus and end-to-end anastomosis between the pulmonary artery and right pulmonary artery were carried out. Systolic pulmonary arterial pressure, estimated by echocardiography Doppler, was 60 mm Hg six months after surgery. Cross-sectional echocardiogram showed the anastomosis of the right pulmonary artery with the main pulmonary artery. Pulmonary gammagraphy showed both lungs perfused through the main pulmonary artery; right lung perfusion was lesser than left lung perfusion, 30 vs. 70% respectively. Aortic origin of a right or left pulmonary artery is a heart disease seen in patients during the course of the first year of life. Its frequency is < 1% among all the congenital cardiopathies and the survival rate to adult life is very low. The originality of this paper is the presentation of a rare congenital cardiopathy treated surgically in an adult.
我们描述了一名26岁、心功能I级（纽约心脏协会分级）的女性病例，其右肺动脉起源于主动脉，合并动脉导管未闭及重度肺动脉高压（101/40 - 70 mmHg），吸入100%氧气后肺动脉高压仍持续存在（89/40 - 60 mmHg）。右肺动脉压力（125/60 - 86 mmHg）高于主肺动脉，与主动脉压力相近。该患者接受了成功治疗：进行了动脉导管未闭手术闭合以及肺动脉与右肺动脉的端端吻合术。术后6个月，经超声心动图多普勒估算，收缩期肺动脉压为60 mmHg。横断面超声心动图显示右肺动脉与主肺动脉吻合。肺灌注显像显示双肺均通过主肺动脉灌注；右肺灌注低于左肺，分别为30%和70%。右或左肺动脉起源于主动脉是一种在出生后第一年病程中可见于患者的心脏病。在所有先天性心脏病中，其发生率<1%，成年后的生存率极低。本文的独特之处在于呈现了一例在成人中接受手术治疗的罕见先天性心脏病。
