Letters to the EditorAtrial septal defect closure in a patient with “irreversible” pulmonary hypertensive 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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Cited by (59)
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2019, American Journal of CardiologyCitation Excerpt :Risk may potentially be further modified with the use of advanced medical therapies for PH, though this approach requires further research. Previously published data suggests that advanced medical therapies for PH may offer patients with “irreversible” pulmonary vascular changes improvement in pulmonary vascular resistance and symptoms.26–29 Since most of the cohort in this study was collected at a time when the availability of targeted PH pharmacotherapy was in its infancy, future studies are needed to investigate whether these therapies, either before or after ASD closure, favorably impact clinical outcomes.
Improvement of pulmonary arterial hypertension following medication and shunt closure in a BMPR2 mutation carrier with atrial septal defect
2017, Journal of Cardiology CasesCitation Excerpt :In each of those cases, the medications were started in adulthood and the disease status was more severe than in our patient. Although their symptoms and hemodynamics improved after ASD closure, residual mild or moderate PAH was still detected in those patients [2–5]. An echocardiographic study of adult patients after percutaneous ASD closure showed that PAH persists in some ASD patients, even when the PAH was not severe [10].
Pulmonary Hypertension in Congenital Heart Disease. Beyond Eisenmenger Syndrome.
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