Demir Fadli, Akbaş Tolga, Erdem Sevcan, Subaşı Berivan, Varan Celal, Salih Orhan Kemal, Özbarlas Nazan
Department of Pediatrics, Division of Pediatric Cardiology, Çukurova University Faculty of Medicine, Adana, Türkiye.
Department of Cardiovascular Surgery, Division of Pediatric Cardiac Surgery, Çukurova University Faculty of Medicine, Adana, Türkiye.
Turk Gogus Kalp Damar Cerrahisi Derg. 2023 Jan 30;31(1):19-28. doi: 10.5606/tgkdc.dergisi.2023.23396. eCollection 2023 Jan.
The aim of the study was to evaluate the clinical and diagnostic findings, treatment, and follow-up of cases of anomalous origin of coronary arteries from the pulmonary artery.
Between January 1998 and June 2021, a total of 14 patients (5 males, 9 females; median age: 15 months; range, 3 to 156 months) diagnosed with anomalous origin of coronary arteries from the pulmonary artery were retrospectively analyzed. Demographic and clinical data of the patients, electrocardiographic, echocardiographic, angiographic, surgical, and follow-up findings were evaluated.
The most common symptoms were respiratory distress (n=6) and murmur (n=3). With the exception of three cases, all other patients were diagnosed by echocardiography in the first examination. Severe mitral valve insufficiency was detected in four patients and four other patients had moderate mitral insufficiency on echocardiography. Ejection fraction values ranged between 38 and 79%. Eleven patients underwent direct implantation of the coronary artery into the aorta, and three underwent a Takeuchi procedure. Mortality occurred in only one case. After surgery, mitral insufficiency and ejection fraction values improved. Median follow-up was 62 (range, 5 to 170) months and all patients were asymptomatic, except one who required redo surgery.
Anomalous origin of the coronary arteries from the pulmonary artery is an uncommon congenital anomaly. Echocardiography is the main diagnostic tool. If all echocardiographic findings of the coronary anomaly are not investigated thoroughly, the diagnosis may be overlooked.
本研究的目的是评估肺动脉起源的冠状动脉异常病例的临床和诊断结果、治疗及随访情况。
回顾性分析1998年1月至2021年6月期间共14例诊断为肺动脉起源的冠状动脉异常的患者(5例男性,9例女性;中位年龄:15个月;范围3至156个月)。评估患者的人口统计学和临床数据、心电图、超声心动图、血管造影、手术及随访结果。
最常见的症状是呼吸窘迫(n = 6)和杂音(n = 3)。除3例患者外,所有其他患者在首次检查时通过超声心动图确诊。4例患者检测到严重二尖瓣关闭不全,另外4例患者在超声心动图检查中有中度二尖瓣关闭不全。射血分数值在38%至79%之间。11例患者接受了冠状动脉直接植入主动脉手术,3例患者接受了竹内手术。仅1例患者死亡。术后,二尖瓣关闭不全和射血分数值有所改善。中位随访时间为62(范围5至170)个月,除1例需要再次手术的患者外,所有患者均无症状。
肺动脉起源的冠状动脉异常是一种罕见的先天性异常。超声心动图是主要的诊断工具。如果未对冠状动脉异常的所有超声心动图表现进行彻底检查,可能会忽略诊断。