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 acquiredcauses. Congenital pulmonary vein stenosis is a rare condition and also a rare cause for pulmonary hypertension. Since identifyingthe etiology of pulmonary hypertension plays a crucial role in medical and surgical planning, we decided to introduce a rare caseof 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 forfurther evaluation. Our findings in transthoracic and transesophageal echocardiography showed pulmonary arterial hypertensionwith maximum gradient of 65 - 70 mmHg and moderate to large size secondom type oval shaped atrial septal defect (2 cm - 1.2cm) 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 pulmonaryvein stenosis was confirmed. In this case, the echocardiography findings were consistent with right upper pulmonary vein stenosisand justified severe pulmonary hypertension with the anomaly of atrial septal defect. Device closure of atrial septal defect andballoon angioplasty of pulmonary vein stenosis were performed for our patients. Three months later, in a follow up evaluation, itwas 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 congenitalpulmonary vein stenosis in infancy were reporteduntil now, and it is very uncommon in adult patients without any prior history of surgery. Patients with pulmonary vein stenosis oftenpresent with shortness of breathandmaymimicsymptomsof Chronic Lung Disease orpneumoniawhich result in misdiagnosisand 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 withoutsufficient assessment to rule out other possible causes for it.

کلیدواژه ها:

Pulmonary Vein ، Atrial Septal Defect ، Echocardiography ، Pulmonary Hypertension