Abstract
IntroductionThe absence of a pulmonary artery is a rare congenital anomaly that occurs isolated or with other congenital cardiac disorders, particularly tetralogy of Fallot (TOF); meanwhile, a hidden pulmonary artery might exist and originate from a closed ductus arteriosus (DA), which can be stented to reach the artery.Material and methodsThis prospective study describes cardiac catheterization of nine TOF patients diagnosed with the absence of the left pulmonary artery before the operation. The patients were stratified into three groups: group one, whose closed DA was found and connected to the hidden pulmonary artery with a stent; group two, whose hidden pulmonary arteries were found via the pulmonary vein angiography; and group three, for whom we could not find the remnant of the DA, or our attempt to stent the DA to the hidden pulmonary artery was not successful. We also evaluated outcomes of six other surgically-corrected TOF patients who were operated with the absent left pulmonary artery.ResultsThe first group included the patients aged 1, 24, and 30 months, whose CT angiography 6–9 months after stenting showed acceptable left pulmonary artery diameter for surgical correction, and the pulmonary vein angiography of the second group showed a hidden left pulmonary artery with a suitable diameter for surgical correction. However, we were unable to find or stent the DA of group three patients, aged 12, 38, 60, and 63 months. Earlier Angiography might have increased the chance of access to the hidden vessel. Apart from these three groups, follow-ups of six other patients previously corrected with only the right pulmonary artery revealed pulmonary artery hypertension in all patients.ConclusionThe concealed pulmonary artery might be found, and stenting of the closed DA to it might be performed to improve the diameter of the diminutive pulmonary artery. This procedure may allow TOF total surgical correction with two pulmonary arteries. Besides, pulmonary vein angiography can reveal the hidden pulmonary artery.
Highlights
The absence of a pulmonary artery is a rare congenital anomaly that occurs isolated or with other congenital cardiac disorders, tetralogy of Fallot (TOF); a hidden pulmonary artery might exist and originate from a closed ductus arteriosus (DA), which can be stented to reach the artery
Apart from these three groups, follow-ups of six other patients previously corrected with only the right pulmonary artery revealed pulmonary artery hypertension in all patients
The concealed pulmonary artery might be found, and stenting of the closed DA to it might be performed to improve the diameter of the diminutive pulmonary artery
Summary
The absence of a pulmonary artery is a rare congenital anomaly that occurs isolated or with other congenital cardiac disorders, tetralogy of Fallot (TOF); a hidden pulmonary artery might exist and originate from a closed ductus arteriosus (DA), which can be stented to reach the artery. Edraki et al BMC Cardiovasc Disord (2021) 21:56 have isolated APA, and 60% suffer from other congenital cardiac disorders as tetralogy of Fallot (TOF) and pulmonary atresia with the ventricular septal defect. Less than 3% of TOF patients have APA [1,2,3,4], and untreated APA cases might develop ipsilateral lung hypoplasia [5,6,7]. Dorsal deviation of the right or left ridges might lead to agenesis of the ipsilateral pulmonary artery [8, 9], and most cases develop pulmonary artery hypertension [6, 10]
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