Isolated unilateral pulmonary vein atresia with hemoptysis in a child A case report and literature review

Rationale: Hemoptysis is an uncommon chief complaint but a distressing symptom in pediatric patients. Due to the recurrence and mortality in minor patients, an accurate diagnosis of the underlying cause is quite essential for treatment. The etiologies causing hemoptysis in children are similar to that in adults. Isolated unilateral pulmonary vein atresia (PVA), as an unusual cardiovascular anomaly, has rarely been reported to be an etiology of hemoptysis in children. Patient concerns: A 2-year and 11-month-old boy was admitted into our hospital with a complaint of recurrent hemoptysis for 2 months and the symptom became more aggravated in recent 4 days before admission. Physical examination was only remarkable for slightly diminished breath sounds over the left lung field, pale face, and colorless lip. Series of targeted laboratory evaluation were negative expect for anemia. Due to the identification of asymmetrical transparency of bilateral lung, slight emphysema of right lung, less volume of left lung with ground-glass opacity and reticular opacity, and ipsilateral mediastinal shift on chest CT, and varices of submucosal vessels in the left bronchial tree on the fiber-optic bronchoscope. Diagnoses: It more likely indicated a congenital cardiovascular disease. The diagnosis of left isolated unilateral PVA was ultimately confirmed through chest CT angiography (CTA) with three-dimensional (3D) reconstruction. Interventions: Since the boy did not complain with hemoptysis after admission, respiratory tract infections seldom occurred and no pulmonary hypertension was detected, a conservative approach was chosen with periodic clinical follow-up after discussing with the cardiac surgeons and in accordance to his parents' own wishes. Outcomes: Fortunately, he was doing well after 3 months of clinical observation. Lessons: We firstly reported a rare case of hemoptysis in children secondary to isolated unilateral PVA with no associated congenital heart disease in Chinese population. It is significant to improve the recognition and prompt diagnosis of this rare condition for pediatric clinicians, and widen the etiology spectrum of hemoptysis in children. The diagnosis of unilateral PVA should be considered for a patient with recurrent hemoptysis and imaging findings that indicate hypoplastic lung, ipsilateral mediastinal shift, and smooth margins of left atrium without evidence of rudimentary pulmonary veins.