Yakut Kahraman, Tokel N Kürşad, Özkan Murat, Varan Birgül, Erdoğan İlkay, Aşlamacı Sait
Department of Pediatric Cardiology, Başkent University Faculty of Medicine, Ankara, Turkey.
Department of Cardiovascular Surgery, Başkent University Faculty of Medicine, Ankara, Turkey.
Turk Gogus Kalp Damar Cerrahisi Derg. 2018 Jan 9;26(1):30-37. doi: 10.5606/tgkdc.dergisi.2018.14772. eCollection 2018 Jan.
In this study, we aimed to report our single-center experience in aortopulmonary window and review clinical signs, symptoms, surgical correction techniques, and long-term outcomes.
We retrospectively reviewed the medical records of a total of 30 patients who were followed with the diagnosis of aortopulmonary window in our hospital between May 1998 and June 2016. The clinical characteristics of the patients, echocardiographic and angiographic findings, surgical treatment outcomes, and medical problems during follow-up were reviewed.
The most common signs and symptoms were murmur, dyspnea, tachypnea, growth retardation, and signs of congestive heart failure. The mean age at the time of surgery was 8.2±14.4 months (7 days to 60 months). Eighteen patients (60%) had additional congenital cardiac anomalies. Eleven patients had simple congenital heart diseases, and seven patients had complex congenital heart diseases. Four patients were unable to be operated due to Eisenmenger syndrome (n=3) and complex congenital heart disease (n=1). No early or late postoperative death was observed. The mean follow-up was 6.4±4.8 years (range, 5 months to 16 years). In addition to aortopulmonary window repair, an additional cardiac anomaly modifying surgical intervention was corrected in nine patients (34.6%). One patient was reoperated for residual aortopulmonary window and another patient for pulmonary stenosis (valvular, supravalvar) after three years. One of these patients underwent pulmonary balloon valvuloplasty after two years. The reoperation rate was 7.7% (n=2) during follow-up.
Aortopulmonary window is a rare cardiac anomaly which may be overlooked by echocardiographic study, and which is amenable for repair with low-surgical risk. It is, therefore, imperative to diagnose and treat this condition, before pulmonary vascular disease develops.
在本研究中，我们旨在报告我们在主肺动脉窗方面的单中心经验，并回顾临床体征、症状、手术矫正技术及长期预后。
我们回顾性分析了1998年5月至2016年6月期间在我院诊断为主肺动脉窗并接受随访的30例患者的病历。回顾了患者的临床特征、超声心动图和血管造影结果、手术治疗效果以及随访期间的医疗问题。
最常见的体征和症状为杂音、呼吸困难、呼吸急促、生长发育迟缓以及充血性心力衰竭体征。手术时的平均年龄为8.2±14.4个月（7天至60个月）。18例患者（60%）合并其他先天性心脏畸形。11例患者患有简单先天性心脏病，7例患者患有复杂先天性心脏病。4例患者因艾森曼格综合征（n = 3）和复杂先天性心脏病（n = 1）无法进行手术。未观察到术后早期或晚期死亡。平均随访时间为6.4±4.8年（范围为5个月至16年）。除主肺动脉窗修复外，9例患者（34.6%）还矫正了需改变手术干预的其他心脏畸形。1例患者术后3年因残余主肺动脉窗再次手术，另1例患者因肺动脉狭窄（瓣膜性、瓣上性）再次手术。其中1例患者术后2年接受了肺动脉球囊瓣膜成形术。随访期间再次手术率为7.7%（n = 2）。
主肺动脉窗是一种罕见的心脏畸形，可能会被超声心动图检查遗漏，且手术风险较低，适合修复。因此，在肺血管疾病发生之前诊断和治疗这种疾病至关重要。
