Unilateral left Pulmonary Artery Agenesis with Pulmonary Hypertension: Unusual First Presentation.

Abstract

Introduction: Unilateral absence of pulmonary artery is a rare congenital disorder. We present here the case of Unilateral left pulmonary artery agenesis with pulmonary hypertension. Case Report: A 33-year young male patient presented to our tertiary care hospital in the out-patient department with complaints of shortness of breath on exertion for 7 months and bilateral feet swelling for 6 months. Routine investigations were within normal limits except 2 dimensional ECHO showed right atrium and right ventricle dilatation with severe pulmonary artery hypertension and 60% left ventricular ejection fraction. Patients with isolated right pulmonary artery agenesis commonly survive into adulthood with minimal or no symptoms, which makes their identification challenging. But our patient had left pulmonary artery agenesis which has significant association of life threatening cardiovascular malformations with left-sided UAPA and surgical repair is often required during the first year of life. None of which is present in our patient which is unsusual. Conclusion: Clinicians should be aware that recurrent respiratory infections may be presenting feature of UAPA. Initial investigation is usually a chest radiograph. Echocardiography is required for evaluation of possible pulmonary hypertension. Confirmation of the diagnosis and anatomic details can be discerned by CT scanning and MRI. Angiography is reserved for patients requiring embolization or revascularization surgery. Present case demonstrates a rare presentation of UAPA as right heart failure in first visit.