SESSION TYPE: Cardiovascular Student/Resident Case Report Posters II
PRESENTED ON: Tuesday, October 23, 2012 at 01:30 PM - 02:30 PM
INTRODUCTION: Isolated unilateral pulmonary vein atresia is a rare condition. Patients generally present with recurrent pneumonia and hemoptysis.
CASE PRESENTATION: A 12-year-old African American female presented to the emergency department with a complaint of hemoptysis, described as “coughing up a handful of bright red blood.” This episode of hemoptysis occurred after riding her bike and was associated with chest discomfort. The past medical history was significant for two previous episodes of scant hemoptysis, recurrent pneumonia and asthma. Physical exam was only remarkable for diminished breath sounds over the right lower lung field. In the emergency department (ED), complete blood count and coagulation panels were normal. A computed tomography (CT) scan of the chest revealed hypoplasia of the right lung, right pulmonary artery and right pulmonary veins, with no evidence of scimitar vein. Bronchoscopy showed diffuse erythema of the mucosa at the entrance to the right upper lobe. Echocardiogram demonstrated a structurally normal heart and four pulmonary veins were felt to enter the left atrium with normal flow and mild elevation of the right ventricular (RV) systolic pressure at 35 mm Hg. Cardiac catheterization demonstrated right upper and lower pulmonary vein atresia. Multiple aorto-pulmonary collaterals were embolized during the procedure to minimize further risk of hemoptysis. Because the anatomy of her anomaly supported anastomosis, the patient underwent surgery three months later. At the surgery the right pulmonary veins seemed to enter the left atrium by external inspection. Upon internal inspection there was membranous right pulmonary vein atresia. Excision of the membrane and a pericardial patch angioplasty of the right pulmonary venous confluence were performed.
DISCUSSION: Pulmonary vein atresia should be considered in children with hemoptysis, recurrent pneumonia and imaging indicating ipsilateral hypoplastic lung. Treatment aim is to prevent co-morbid conditions and preserve lung function. Treatment approaches include conservative monitoring, surgical anastomosis or pneumonectomy with preoperative collateral vessel banding. Due to the morbidity and mortality of this condition, it is crucial to identify this condition before pulmonary hypertension or massive pulmonary hemorrhage ensues.
CONCLUSIONS: We present a rare case of hemoptysis secondary to isolated unilateral pulmonary vein atresia.
1) Godfrey S. Pulmonary Hemorrhage/Hemoptysis in Children. Pediatr Pulmonol 2004; 37:476-484
2) Mutaciunas M, Gumbiene L, Cibiras S, Tarutis V, Tamosiunas AE. CT angiography of mildly symptomatic, isolated, unilateral right pulmonary vein atresia. Pediatr Radiol 2009; 39:1087-1090
3) Pourmoghadam KK, Moore JW, Khan M, Geary EM, Madan N, Wolfson BJ, Chadarevian JP, Holsclaw DS, Jacobs ML. Congenital Unilateral Pulmonary Venous Atresia: Definitive Diagnosis and Treatment. Pediatr Cardiol 2003; 24:73-79.
DISCLOSURE: The following authors have nothing to disclose: Cindy Demastes-Crabtree, Edward Kim, Ronald Morton
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