A 29-year-old white female patient, who was suffering from exertional dyspnea was referred to our center for further evaluation.
Over the past year she had experienced increasing dyspnea with functional capacity class II. The patient’s past medical, family and drug history was negative.
In her initial assessment: O
2 saturation was 98% at room air. II-III/VI systolic murmur was heard over pulmonic area without any thrill and splitting of second heart sound was also found.
Patient’s electrocardiography revealed normal sinus rhythm and incomplete right bundle branch block.
Our findings in transthoracic echocardiography showed: Normal left ventricle size and systolic function, severe right ventricular enlargement with mild systolic dysfunction, mild to moderate tricuspid regurgitation with peak gradient of 60 mmHg and pulmonary pressure gradient estimated 65 - 70 mmHg. Moderate to large size secondum type oval shaped atrial septal defect (2 cm - 1.2 cm) was also noted with bidirectional shunt, in a predominantly left to right direction.
Due to out of proportional pulmonary hypertension with atrial septal defect, we decided to continue our evaluations to possibly pinpoint other possible causes for the severe pulmonary hypertension.
Other possible cardiac causes for her pulmonary hypertension included: primary pulmonary hypertension, pulmonary hypertension associated with the left heart disease, chronic pulmonary emboli, TAPVC and PAPVC, PV stenosis and pulmonary hypertension associated with intracardiac shunts.
In view of the right pulmonary vein in transesophageal echocardiography, doppler study revealed flow turbulency, continuous high peak velocity pattern with peak velocity of 226 cm/sec (
Figure 1) and a web causing a blockage with a central opening that had a diameter of 0.8 cm by 0.9 cm which was located at the entrance of the right upper pulmonary vein ( Figures 2 and 3).
Figure 1. A, typical pulsed Doppler flow pattern of the pulmonary vein stenosis. The flow pattern is turbulent, continuous, and has an abnormally high peak velocity (maximum peak velocity = 226 cm/sec); B, normal pulsed Doppler pulmonary vein flow pattern after balloon angioplasty of the pulmonary vein. There are well-defined systolic and early diastolic peaks of _60 cm/second, and the flow reaches the baseline.
Figure 2. Three-Dimensional Transesophageal Echocardiogram
Marked area with plus sign indicates the orifice of the web in right upper pulmonary vein entrance with diameter of 0.8cm to 0.9 cm. the surrounded area, marked with arrows indicates the native orifice of the pulmonary vein entrance and margins of the web, inter atrial septum (IAS), and right lower pulmonary vein (RLPV).
Figure 3. Web at The Entrance of Right Upper Pulmonary Vein (RUPV) in Two Dimensional Tranesophageal Echocardiography
In this case, the echocardiographic findings confirmed by three- dimensional study were consistent with RUPV stenosis; these findings illustrated an association between severe pulmonary hypertension and anomaly of atrial septal defect. Atrial septal defect had suitable rims for device closure.
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 severity of pulmonary hypertension gradient was declined from 65mmHg to 40 mmHg and pulsed Doppler pulmonary vein flow pattern in transesophageal echocardiography changed from continuous high peak velocity pattern to normal, well-defined systolic and early diastolic flow pattern with peaks of _60 cm/second.
Although there is little evidence on the results of transcatheter techniques for treatment of congenital pulmonary vein stenosis, balloon angioplasty of the involved vessels acquired after radiofrequency ablation usually leads to a reasonably good initial result. However, restenosis occurs in 50% of the patients within one year (
2). Considering the presence of a web as the cause of pulmonary vein stenosis in our patient, we expected the possibility of restenosis be low. Our follow up evaluation in 3 months later was also normal; however, longer term follow up is needed to ensure the maintenance of our acceptable outcome.