Catastrophic Presentation of Anomalous Origin of Right Pulmonary Artery in a Neonate- An Interesting Differential!

Abstract

A 36-hour-old term neonate presented with refractory cardiogenic shock and multi-organ dysfunction. On suspecting coarctation of aorta, intravenous prostaglandin was started but without any benefit. Echocardiography revealed isolated anomalous origin of right pulmonary artery from ascending aorta causing massive aortic steal, supra-systemic pulmonary arterial hypertension (PAH), multi-organ dysfunction and right ventricular failure. The main pulmonary artery was solely continued as left pulmonary artery. Emergency surgery at 48 h was life-saving and mid-term follow-up is excellent. The authors present this case as an important alternative diagnosis to duct dependent systemic lesions. The English literature in the last two decades was reviewed to discuss the characteristics, the diagnosis and treatment of neonatal presentation of this rare anomaly. Incidentally, the reported case is the youngest successful survivor of this surgery.

Older patients with anomalous origin of the pulmonary artery from the ascending aorta: Guidance via lung biopsy

“…If uncorrected, early development of PVOD can be expected. Approximately 70% of untreated patients die by 6 months, and 80% die by 1 year of age . Thus, accurate evaluation of the degree of PVOD is particularly important as a useful guide to operability, especially in older patients.…”

Anomalous Origin of One Pulmonary Artery From the Ascending Aorta

“…Indeed, early repair improves one-year survival, which was reported to be as low as 30% in the absence of surgical correction. 4,19,20 Some authors suggest that surgical correction should be performed during the first three months of life. 2,[13][14][15][16][17] In the present study, all patients were less than three months of age at the moment of diagnosis and treatment, which is unusual in developing countries.…”