Isolated left-sided pulmonary artery agenesis with left lung hypoplasia: A report of two cases

Abstract

Unilateral absence of pulmonary artery or pulmonary artery agenesis (UAPA) is a rare congenital malformation that can present as an isolated lesion or in association with other cardiac anomalies. Though congenital, presentation in adults are also reported. Most common presentation in adults is of exercise intolerance. The developing lung on the affected side is hypoplastic. Diagnosis of UAPA is established by imaging methods like CT and MRI . There is no specific treatment for this condition. Treatment depends on patients symptomatology, presence of pulmonary hypertension and collateral circulation. Presence of pulmonary hypertension carries a bad prognosis. We present two adult patients with isolated left sided unilateral pulmonary artery agenesis with ipsilateral lung hypoplasia. The diagnosis was confirmed by CT chest and perfusion scan.

Figure 1

Scanogram of both cases showing volume loss on left side with mediastinal shift to left side

Figure 2

Patient A – The red arrow shows normal right pulmonary artery (22.1 mm) and blue arrow shows rudimentary left pulmonary artery (9mm) in size. Patient B – The red arrow shows normal right pulmonary artery (26.7 mm) and blue arrow shows rudimentary left pulmonary artery (9mm) in size. Both measurements were made at just below bifurcation of trachea

Figure 3

Perfusion scan images of both cases: Technetium labeled macroaggregated albumin showing absent perfusion in the left lung