Page 31 - Journal of Structural Heart Disease Volume 3, Issue 6

Case Report
Journal of Structural Heart Disease, December 2017, Volume 3, Issue 6:187-191
DOI: https://doi.org/10.12945/j.jshd.2017.022.17
Received: May 14, 2017 Accepted: May 29, 2017 Published online: December 2017
Improvement in Pulmonary Function After
Closure of Atrial Septal Defect in a Patient With
Cystic Fibrosis
Abdulfattah Saidi, MD1*, Holly Carveth, MD2, Anwar Tandar, MD1
1 Division of Cardiovascular Medicine, University of Utah Hospital, Salt Lake City, Utah, United States 2 Division of Pulmonary Medicine, University of Utah Hospital, Salt Lake City, Utah, United States
Abstract
Atrial septal defect (ASD) is a major cause of left-to- right intracardiac shunting. If persisting into adult- hood, an ASD can lead to a larger shunt, which may eventually cause pulmonary hypertension and right ventricular failure. Large intracardiac shunts cannot be tolerated in patients with underlying lung disease such as cystic brosis. Although the association between an intracardiac shunt and cystic brosis has been report- ed in the literature, the impact of ASD closure on the clinical course of patients with cystic brosis has not been studied. Here, we report a case of ASD closure in a patient with cystic brosis with hypoxemia out of pro- portion to his lung disease. Closure of the ASD shunt resulted in signi cant improvement of his symptoms and pulmonary function testing.
Copyright © 2017 Science International Corp.
Key Words
Atrial septal defect • Septal closure device • Cystic brosis
Introduction
Secundum atrial septal defect (ASD) is an isolated defect in the fossa ovalis between the atria. Although many ASDs close spontaneously during the rst year after birth [1], the defect persists in some cases, caus- ing a left-to-right shunt [2]. Left-to-right shunting,
when signi cant, can cause dyspnea on exertion, which may not be well tolerated in patients with an underlying pulmonary condition such as cystic bro- sis. It is unknown whether ASD closure could lead to symptomatic improvement in these patients. Here, we report a case of ASD closure resulting in improved pulmonary function testing in a patient with cystic - brosis with a bidirectional shunt and worsening func- tional capacity.
Case Presentation
Our patient was a 36-year-old man with cystic - brosis diagnosed at birth with a dF508/dF508 geno- type. His symptoms could be characterized as mod- erate airway disease complicated by chronic airway infections, pancreatic insu ciency, diabetes, and malnutrition. Progression of his clinical condition caused dyspnea with minimal exertion. He was re- ferred to our cardiology service to be evaluated for hypoxemia out of proportion to his chronic pulmo- nary disease. Pulmonary function testing showed a forced expiratory volume in 1 s (FEV1) of 1.71 (43% predicted). Transesophageal echocardiography (TEE) showed a small ASD with a bidirectional shunt seen at rest (Figure 1). Right heart catheterization showed a pulmonary artery pressure of 26/7/16 mmHg and pul- monary vascular resistance of 1.7 Wood units. A deci- sion was made to proceed with ASD closure, which
* Corresponding Author:
Abdulfattah Saidi, MD
Division of Cardiovascular Medicine
University of Utah Hospital
50 North Medical Drive, Salt Lake City, UT 84132, USA
Tel. +801 585 5559; Fax: +801 581 7735; E-Mail: abdulfattah.saidi@hsc.utah.edu
Fax +1 203 785 3346
E-Mail: jshd@scienceinternational.org http://structuralheartdisease.org/
© 2017 Journal of Structural Heart Disease Published by Science International Corp. ISSN 2326-4004
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