Pulmonary Vein Stenosis as a Rare Cause for Pulmonary Hypertension, Documented by Three-Dimensional Echocardiography

Introduction: Pulmonary vein stenosis includes narrowing of one or more pulmonary veins that may have congenital or acquired causes. Con- genital pulmonary vein stenosis is a rare condition and also a rare cause for pulmonary hypertension. Since identifying the etiology of pulmonary hypertension plays a crucial role in medical and surgical planning, we decided to introduce a rare case of pulmonary hypertension due to pulmonary vein stenosis. Case Presentation: A 29 year-old white female patient who was suering from exertional dyspnea was referred to our center for further evaluation. Our findings in transthoracic and transesophageal echocardiography showed pulmonary arterial hypertension with maximum gradient of 65 - 70 mmHg and moderate to large size secondom type oval shaped atrial septal defect (2 cm - 1.2 cm) with bidirectional shunt, in a predominantly left to right direction. Atrial septal defect rims were suitable for device closure. Due to out of proportional pulmonary hypertension with atrial septal defect, further evaluations were conducted and pulmonary vein stenosis was confirmed. In this case, the echocardiography findings were consistent with right upper pulmonary vein stenosis and justified severe pulmonary hypertension with the anomaly of atrial septal defect. Device closure of atrial septal defect and balloon angioplasty of pulmonary vein stenosis were performed for our patients. Three months later, in a follow up evaluation, it was revealed that the severity of pulmonary hypertension was declined from 65 - 70 mmHg to 40 mmHg. Conclusions: Only few cases of pulmonary hypertension secondary to congenital pulmonary vein stenosis in infancy were reported until now, and it is very uncommon in adult patients without any prior history of surgery. Patients with pulmonary vein stenosis often present with shortness of breath and may mimic symptoms of Chronic Lung Disease or pneumonia which result in misdiagnosis and treatment. Therefore, in patients with underlying congenital heart disease and out of proportional pulmonary hypertension, other potential and rare causes for pulmonary hypertension such as pulmonary vein stenosis should be considered and evaluated. It is recommended that cardiologists do not directly attribute pulmonary hypertension to the underlying heart disease without sucient assessment to rule out other possible causes for it.