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Atrial septal defect closure in a patient with “irreversible” pulmonary hypertensive arteriopathy

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Abstract

The presence of irreversible pulmonary hypertension in patients with atrial septal defect (ASD) is thought to preclude shunt closure. We report the case of a woman with plexiform pulmonary arteriopathy secondary to an ostium secundum ASD who was able to successfully undergo percutaneous shunt closure following therapy with chronic intravenous prostacyclin (Flolan). One year after closure, the patient was weaned off Flolan over a period of 7 months following the institution of oral Bosentan therapy. Our case illustrates how aggressive vasodilator therapy with prostaglandins may be capable of reducing pulmonary artery pressure and permitting shunt closure in a patient once considered to have “inoperable” pulmonary arteriopathy.

Introduction

Pulmonary hypertension occurs in less than 10% of adults with an atrial septal defect (ASD) within the fossa ovalis [1]. Percutaneous or surgical closure of this atrial shunt is recommended for substantial shunts (pulmonary to systemic flow ratio > 1.5), or for those shunts associated with right ventricular volume overload, in the absence of severe pulmonary hypertension [2]. In patients with shunt-related pulmonary hypertension, it is a current practice to assess the reversibility of the pulmonary hypertension by means of pulmonary vasodilator testing during right heart catheterization, or occasionally even by open lung biopsy to evaluate the severity of the pulmonary arteriopathy [2]. Patients considered to have irreversible pulmonary hypertension are considered ineligible for shunt closure because of the risk of right ventricular decompensation following the intervention. Whether chronic treatment with pulmonary vasodilators like prostacyclin may benefit these patients and allow for eventual closure is unknown. We report a case of severe and seemingly “irreversible” pulmonary hypertension undergoing percutaneous closure of a large ostium secundum ASD, after lowering pulmonary artery pressure with chronic intravenous prostacyclin.

Section snippets

Case report

A previously healthy 38 year old woman gave a five-year history of exertional dyspnea following her third pregnancy. She presented with a precipitous worsening of exertional dyspnea (NYHA class III limitation), exertional angina pectoris, exertional presyncope, and bilateral lower limb edema. At initial presentation she was taking oral contraceptives.

Transthoracic echocardiography showed a 20-mm large ASD in the fossa ovalis with bi-directional shunting. Systolic pulmonary artery pressure was

Discussion

Potent vasodilators for the therapy of primary pulmonary hypertension also have a beneficial effect in patients with secondary pulmonary hypertension related to cardiac shunts [3]. We report the case of successful and complete percutaneous closure of a fairly large ASD in a woman with shunt-related plexiform pulmonary arteriopathy, considered to have “irreversible” anatomical changes of the pulmonary vascular bed. ASD closure was performed after one year of continuous intravenous prostacyclin

Acknowledgement

MS is supported by a grant from the Swiss National Research Foundation. Conflict of interest: none.

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