Gowda Deepak, Gajjar Trushar, Rao Jinaga Nageswar, Chavali Praveen, Sirohi Aaditya, Pandarinathan Naveen, Desai Neelam
Department of Cardiothoracic and Vascular Surgery, Sri Sathya Sai Institute of Higher Medical Sciences, Prasanthigram, Puttaparthy, Anantapur district, Andhra Pradesh, India.
Interact Cardiovasc Thorac Surg. 2017 Aug 1;25(2):302-309. doi: 10.1093/icvts/ivx099.
Aortopulmonary window represents 0.2-0.3% of all congenital heart lesions. Progressive pulmonary arterial hypertension and its consequences are more common with this anomaly. The purpose of this study was to share 24 years of surgical experience in managing a spectrum of 55 cases of aortopulmonary window, followed up to 17 years in a single institution.
This retrospective study was done from November 1991 to November 2015 of 55 patients with aortopulmonary window who underwent successful surgical repair. Age ranged from 5 months to 31 years with 45 children (12 years and younger) and 10 adults (older than 12 years). The male:female ratio was 2.2:1. The mean weight at operation was 14.63 kg (range 3.5-50 kg). An initial diagnosis was obtained from 2D echocardiography, which showed echo dropout in the parasternal short-axis view. Cardiac catheterization and angiography were performed in 54 out of 55 patients. Cardiac catheterization was not done in 1 patient who was 4 months of age. The mean right ventricular systolic pressure (RVSP) was 94 ± 2 mmHg, and the pulmonary artery mean pressure was 68 ± 2 mmHg. The average left to right shunt was 5.2:1, and the pulmonary vascular resistance index in room air was 7.97 ± 0.5 Wood units, whereas after oxygen administration, it declined to 2.0 ± 0.5 Wood units. Four surgical techniques were used based on the size of the communication and the anatomical conditions.
There were no early or late deaths. There were no pulmonary hypertensive crises. All patients underwent echocardiography before discharge; none showed a residual shunt. Mild left ventricular dysfunction was seen in 2 patients. This dysfunction regressed with afterload reduction and diuretics on follow-up. All patients were followed up at intervals of 3 months, 1, 5 and 10 years, with the longest follow-up being 17 years. The mean follow-up period was 7 years. At follow-up, all patients were New York Heart Association class I. The mean RVSP on echocardiography was 32 mmHg at 3 months and 30 mmHg at 7 years with no change on further follow-up. Residual pulmonary hypertension was seen in 3 patients: 2 had mild pulmonary hypertension at 8-years follow-up and 1 had moderate hypertension at 3-months follow-up who required sildenafil postoperatively.
Aortopulmonary window is a rare but well identified and surgically correctable anomaly. Operative repair should be offered as soon as the diagnosis is established, regardless of the patient's age. Irreversible pulmonary hypertension with a right to left shunt despite oxygen administration is the only contraindication for surgery. Various surgical techniques can be applied depending on the size of the communication. Associated arch anomalies may require technically challenging approaches and surgical strategies. Early and long-term outcomes after surgical correction are excellent regardless of age or pulmonary vascular resistance.
主肺动脉窗占所有先天性心脏病病变的0.2 - 0.3%。这种异常情况下，进行性肺动脉高压及其后果更为常见。本研究的目的是分享在一家机构对55例主肺动脉窗患者进行手术治疗24年的经验，随访时间长达17年。
本回顾性研究对1991年11月至2015年11月期间55例成功接受手术修复的主肺动脉窗患者进行。年龄范围为5个月至31岁，其中45例为儿童（12岁及以下），10例为成人（12岁以上）。男女比例为2.2:1。手术时的平均体重为14.63千克（范围3.5 - 50千克）。最初的诊断通过二维超声心动图获得，其在胸骨旁短轴视图中显示回声失落。55例患者中有54例进行了心导管检查和血管造影。1例4个月大的患者未进行心导管检查。右心室收缩压（RVSP）平均为94±2毫米汞柱，肺动脉平均压为68±2毫米汞柱。平均左向右分流为5.2:1，室内空气中的肺血管阻力指数为7.97±0.5伍德单位，吸氧后降至2.0±0.5伍德单位。根据交通口大小和解剖条件采用了四种手术技术。
无早期或晚期死亡病例。无肺动脉高压危象。所有患者出院前均进行了超声心动图检查；均未显示残余分流。2例患者出现轻度左心室功能障碍。这种功能障碍在随访中通过减轻后负荷和使用利尿剂而得到改善。所有患者每隔3个月、1年、5年和10年进行随访，最长随访时间为17年。平均随访期为7年。随访时，所有患者均为纽约心脏协会I级。超声心动图显示3个月时RVSP平均为32毫米汞柱，7年时为30毫米汞柱，进一步随访无变化。3例患者出现残余肺动脉高压：2例在8年随访时患有轻度肺动脉高压，1例在3个月随访时患有中度高血压，术后需要使用西地那非。
主肺动脉窗是一种罕见但易于识别且可通过手术矫正的异常情况。一旦确诊，无论患者年龄大小，均应尽早进行手术修复。吸氧后仍存在不可逆的肺动脉高压并伴有右向左分流是手术的唯一禁忌证。可根据交通口大小采用各种手术技术。相关的主动脉弓异常可能需要具有技术挑战性的方法和手术策略。无论年龄或肺血管阻力如何，手术矫正后的早期和长期效果都非常好。
