Anomalous origin of the left pulmonary artery from the ascending thoracic aorta and a right patent ductus arteriosus associated with pulmonary hypertension in a woman of childbearing age: an unprecedented approach

A female patient in her early 30s, with a medical history of pulmonary arterial hypertension associated with congenital heart disease, lost to follow-up, was referred to a pulmonary hypertension reference centre. The patient presented at a WHO functional class of II in a low-risk category. A transthoracic echocardiogram demonstrated severe pulmonary hypertension with right ventricular dysfunction. A cardiac MRI demonstrated a right aortic arch with anomalous origin of the left pulmonary artery from the ascending thoracic aorta and a patent ductus arteriosus. A right heart catheterisation confirmed the presence of severe pulmonary hypertension. The patient was started on combined vasodilator therapy with an improvement in symptoms and remaining in a low-risk category. This represents one of the oldest described cases of such an anomaly and the first description of response to vasodilator therapy, highlighting the importance of a structured approach in a reference centre for achieving optimal outcomes.