De Rubens-Figueroa Jesús, Bobadilla-Chávez José J, Solórzano-Morales Sara A
Laboratorio de Ecocardiografía, Instituto Nacional de Pediatría, Ciudad de México, México.
Servicio de Cardiología, Instituto Nacional de Pediatría, Ciudad de México, México.
Arch Cardiol Mex. 2021 Mar 16;91(2):355-360. doi: 10.24875/ACM.20000362.
The objective is demonstrate the diagnostic process and evolution of a patient with a diagnosis of congenital pulmonary vein stenosis and broncho-pulmonary vascular malformation. One year old female patient with repeated bronchopneumonia, acrocyanosis, split S2, cardiomegaly, pulmonary hypertension, with a clinical diagnosis of atrial septal defect. The echocardiogram demonstrated left sided vein pulmonary stenosis. The cardiac catheterization demonstrated arterial-venous fistulas apical on the right lung. Magnetic Resonance image and angiography showed an aberrant arterial vessel parallel to the abdominal aorta which flow the right pulmonary lobe. The cardiac tomography angiography reported confluence of right-sided pulmonary veins. A lobectomy is performed. Patient died in post-operative due to massive pulmonary hemorrhaging. This is the first patient mentioned in written literature with pulmonary vein stenosis associated with pulmonary sequestration, with normal venous connection. Echocardiography represents the specific standard study ideal for initial diagnostic for patients with pulmonary vein stenosis.
目的是展示一名诊断为先天性肺静脉狭窄和支气管肺血管畸形患者的诊断过程及病情发展。一名1岁女性患者，反复发生支气管肺炎、肢端青紫、第二心音分裂、心脏扩大、肺动脉高压，临床诊断为房间隔缺损。超声心动图显示左侧肺静脉狭窄。心导管检查显示右肺尖部动静脉瘘。磁共振成像和血管造影显示一条与腹主动脉平行的异常动脉血管，供血至右肺叶。心脏断层血管造影报告右侧肺静脉汇合。进行了肺叶切除术。患者术后因大量肺出血死亡。这是书面文献中首次提及的肺静脉狭窄合并肺隔离症且静脉连接正常的患者。超声心动图是肺静脉狭窄患者初始诊断的理想特异性标准检查。
