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Successful surgical repair of multiple anomalous pulmonary venous returns and vasodilator treatment leading to substantial improvement of pulmonary hypertension.

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Conference: 2014 International PHA Conference and Scientific Sessions

Release Date: 06.22.2014

Presentation Type: Abstracts

File Download: 2014 Conference Abstract - Rebekah Hanson

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Partial anomalous pulmonary venous return (PAPVR) is a rare vascular anomaly. The prevalence is extremely low but can occur more frequently with Turner syndrome.

Background: Partial anomalous pulmonary venous return (PAPVR) is a rare vascular anomaly.  The prevalence is extremely low but can occur more frequently with Turner syndrome.  This type of congenital defect is usually asymptomatic in adults; however, in some cases it can lead to vascular and anatomical changes resulting in pulmonary hypertension, right ventricular overload and failure, and in some cases may require surgical correction.  

Methods: A 56 year old Caucasian female with a history of Turner syndrome, diabetes mellitus, hypertension, hyperlipidemia, hypothyroidism and previous pulmonary embolism and deep venous thrombosis was referred for evaluation of possible pulmonary hypertension. She had a four month history of rapid progression in dyspnea with minimal exertion and increased lower extremity edema with frequent nocturia. An echocardiogram showed evidence of right ventricular hypertrophy and increased pulmonary arterial systolic pressure. The presence of an abnormal venous return from pulmonary vein was discovered on chest CT scan. Pulmonary function tests completed during initial consult found reduced FEV1, FVC and DLCO and her six minute walk distance was 213 meters on room air. The patient was admitted a few days after initial consult for acute decompensated right heart failure and fluid overload. A right heart catheterization completed identified severe pulmonary hypertension [PA 74/40 (54) mmHg, PCWP 20 mmHg, RA 16 mmHg, PVR 255 dynes-sec/cm5] with left to right shunting. A subsequent transthoracic echocardiogram with bubble revealed no obvious atrial or ventricular septal defects and a cardiac coronary computed tomography angiogram confirmed the presence of multiple pulmonary venous returns; two draining into the superior vena cava and three draining into the left atrium. The patient underwent surgical repair of the PAPVR’s. Additionally, vasodilator treatment was initiated with sildenafil for residually elevated pulmonary artery systolic pressure post-procedure.  

Results: In the first few months post-procedure, the patient experienced numerous complications resulting in multiple prolonged hospitalizations including recurrent pleural effusions, atrial-flutter necessitating cardioversion and a recalcitrant gastrointestinal bleed. Despite this, in the ten months post-repair she has experienced a profound improvement in condition including increased exercise tolerance, stabilized fluid status, improved hemodynamics and improved right ventricle size and function. Due to a mild but persistently elevated mean PAP, she remains on sildenafil 20mg three times daily and was recently initiated on macitentan 10mg daily for further benefit.  

Conclusions: Although very uncommon, the presence of PAPVR in adults may result in pulmonary hypertension and subsequent right ventricular dysfunction. Surgical correction of the anomaly can improve symptoms, hemodynamic findings and right ventricular function. Treatment with vasodilators may provide additional benefit in the setting of persistently elevated pulmonary arterial pressures.  

Type: Case Study