AGENESIS OF THE RIGHT PULMONARY ARTERY: CASE REPORT

TYPE: Abstract Publication TOPIC: Pediatrics PURPOSE: Report a case of right pulmonary artery agenesis. METHODS: Case report. RESULTS: In the present case study, the patient presents fever, productive cough and tachypnea. A simple chest X-ray was performed because of the clinic, demonstrating right peri-hilar interstitial-alveolar opacity with right mediastinal deviation and asymmetry of pulmonary transparency. Due to the radiological finding, a computed tomography of the chest was requested, which showed the right lung of reduced volume. In order to provide a better understanding of the condition, the radiology team suggested a computed angiotomography of the chest, which detected agenesis of right pulmonary artery. CONCLUSIONS: Prevalence of pulmonary artery agenesis is approximately 1:200,000 to 300,000 in cases of absence of cardiovascular malformations. When isolated from other congenital changes, patients are often asymptomatic. Few individuals with this anomaly develop pulmonary hypertension and, in most of them, the diagnosis is made only at autopsy after a fulminant hemoptysis. This malformation occurs due to the non-development of the sixth aortic arch. Intrapulmonary arteries develop normally and the vascularization of the lung is by bronchial arteries. In contrast to left pulmonary artery agenesis, right pulmonary artery agenesis is isolated and is not related to cardiovascular malformations as in the first. This diagnosis can be definitively made with chest CT angiography. CLINICAL IMPLICATIONS: Right pulmonary artery agenesis when isolated from other congenital changes, patients are often asymptomatic and this anomaly may not be identified throughout their lives.A small part of these individuals developed pulmonary hypertension and, in most of these cases, the diagnosis is made at autopsy after a fulminant hemoptysis. DISCLOSURE: No significant relationships. KEYWORD: Agenesis, Pulmonary Artery, Unilateral absence of pulmonary artery