Pulmonary Vascular Disease |

A Case of Pulmonary Arterial Hypertension Secondary to Anomalous Pulmonary Veins FREE TO VIEW

Avantika Chenna, MD; Punita Kaveti, MD; Avaneesh Jakkoju, MD; Saurabh Khurana, MD; Marshaleen Henriques-Forsythe, MD
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Morehouse School of Medicine, Atlanta, GA

Chest. 2014;146(4_MeetingAbstracts):884A. doi:10.1378/chest.1993907
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SESSION TITLE: Pulmonary Vascular Disease Student/Resident Case Report Posters I

SESSION TYPE: Medical Student/Resident Case Report

PRESENTED ON: Tuesday, October 28, 2014 at 01:30 PM - 02:30 PM

INTRODUCTION: Partial anomalous pulmonary venous return (PAPVR) is a rare cause of adult onset pulmonary arterial hypertension (PAH) that can present with a wide spectrum of severity from early childhood throughout adult life (1). It is a congenital condition in which some, but not all, of the pulmonary veins connect to the right atrium or one of its venous tributaries (2). We present a 62 year old woman with PAH secondary to PAPVR with associated ASD. The patient was managed medically due to concerns that surgical intervention would result in clinical decline.

CASE PRESENTATION: Our case is a 62 year old woman with multiple admissions for chest pain and worsening shortness of breath. CT chest revealed enlarged pulmonary vasculature and partial anomalous venous return such that the right lower lobe pulmonary vein entered directly into the right atrium. Transthoracic echocardiography showed pulmonary hypertension, with an estimated right ventricular systolic pressure of 61 mmHg and moderate to severely reduced right ventricular systolic function. The left and right atrium was dilated and she had a large ASD. Doppler suggested right to left interatrial shunt. Right heart catheterization showed moderate PAH with a mean pulmonary arterial pressure of 46mmHg and PVR of 3 Woods Units. Elevated right ventricular filling pressures, a left to right shunt and preserved cardiac output/cardiac index by Fick was noted. She was deemed unfit for surgery given the chronicity of her congenital anomalies with presence of pulmonary hypertension and other comorbid conditions. She was thus treated with bosentan, diuretics and home oxygen.

DISCUSSION: PAPVR is usually seen in the pediatric population but may also be diagnosed during adulthood in patients who develop PAH or incidentally found in patients undergoing pulmonary vascular studies. Diagnostic modality include CT chest, Cardiac MRI or Transeophageal Echocardiology. Surgery is generally effective in correcting the abnormal shunt and its associated symptoms but in cases with severe PAH and elevated PVR, reparative surgery is unlikely to alter disease course as the extensive vascular remodeling is typically irreversible at that point. In these cases, heart-lung transplant may be considered.

CONCLUSIONS: Given that there are very few treatment options for this particular condition it is essential that primary care physicians, cardiologists and pulmonologists are aware of this entity and the management options.

Reference #1: Edmond H Sears , Jason L. Aliotta Partial anomalous pulmonary venous return presenting with adult-onset pulmonary hypertension

Reference #2: Bicakcioglu P1, Gokcek A, Karaoglanoglu N. Partial anomalous pulmonary venous return associated with intact atrial septum. Asian Cardiovasc Thorac Ann. 2013 Dec;21(6):747

DISCLOSURE: The following authors have nothing to disclose: Avantika Chenna, Punita Kaveti, Avaneesh Jakkoju, Saurabh Khurana, Marshaleen Henriques-Forsythe

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