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

Background: Pulmonary vein stenosis includes narrowing of one ormore pulmonary veins thatmay have congenital or acquired causes. Congenital 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 inmedical 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 suffering 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 stenosiswas confirmed. In this case, the echocardiography findingswere consistentwith 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. Threemonths later, in a follow up evaluation, it was revealed that the severity of pulmonary hypertension was declined from65 - 70mmHg to 40mmHg.
 
Conclusions: Only fewcases of pulmonaryhypertensionsecondary to congenital pulmonary veinstenosis ininfancywere reported until now, and it is very uncommon in adult patientswithout any prior history of surgery. Patientswith pulmonary vein stenosis of- tenpresentwithshortness of breathandmaymimic symptoms of Chronic LungDisease orpneumoniawhichresult inmisdiagnosis 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 sufficient assessment to rule out other possible causes for it.