Presentation
Dyspnea on exertion. Concern for pulmonary embolism.
Patient Data
Small oligaemic right lung and mediastinal shift to the right. Enlarged left hilar arteries and absent right hilar arteries.
Small right lung with mediastinal shift and absent right pulmonary artery. Systemic arterial supply to the right lung through bronchial arteries via the hilum, and transpleural supply from enlarged phrenic arteries and intercostal arteries. Large meandering upper zone pulmonary veins drain into the superior vena cava. The inferior right pulmonary vein drains to the right atrium.
Large left pulmonary artery.
Otherwise normal heart and great vessels. No evidence of pulmonary hypertension.
Case Discussion
Congenital agenesis of the pulmonary artery is a rare condition that can present in adulthood if there is no serious associated congenital cardiovascular disease. The prevalence is thought to be approximately 1:200,000.
In this case, there is a small right to left shunt through the normal right inferior pulmonary vein.
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