P-225A RARE VASCULARY MALFORMATION: UNILATERAL ABSENCE OF PULMONARY ARTERY

Objectives: Unilateral absence of pulmonary artery (UAPA) is a rare malformation with approximately 1/200 000 prevalence. Most commonly, UAPA occurs in conjunction with cardiovascular abnormalities and nearly 40% of patients have isolated UAPA. We are presenting a case admitted to our clinic with haemoptysis and diagnosed a rare malformation UAPA. Case description: Radiological examinations of a 19-year-old male patient admitted to our clinic with haemoptysis showed a small right haemithorax, ipsilateral less vascular marking; computed tomography scan showed diffuse ground-glass opacity, absence of right pulmonary artery, and PDA between the left pulmonary artery and the aortic arch. Echocardiography of the patient detected: PDA (broad), ASD (secundum), hypoplasia of the pulmonary artery branches, mitral valve prolapse mild tricuspid regurgitation and pulmonary HT (pulmonary artery pressure: 115 mmHg). A bronchial artery embolization was performed to the right-sided bleeding bronchial arteries. The patient had no haemoptysis after the intervention and was followed up with medical treatment. Conclusions: UAPA is diagnosed definitively by computed tomography (CT), magnetic resonance imaging (MRI) or transthoracic echocardiography. Surgical treatment should be thought after cardiac catheterization and pulmonary venous wedge angiographic assessment. Severe infections may require surgical resections such as lobectomy or pneumonectomy. Haemoptysis may be treated with embolization or pulmonary surgery. Pulmonary hypertension is a severe complication. Treatment of those patients with pulmonary hypertension is medical therapy. Disclosure: No significant relationships.