Page 34 - Journal of Structural Heart Disease Volume 3, Issue 6

Case Report
190
Table 1. Pulmonary function test of the patient over his clinical course.
FEV1 1.71 (43% predicted) 1.78 (47% predicted) FEV1/FVC 53 (65% predicted) 61 (76% predicted)
3.4 (89%predicted) 76 (95% predicted)
Before rst ASD closure
After rst ASD closure (with residual shunt)
After second ASD closure (no signi cant residual shunt)
early life, may lead to right-sided volume followed by pressure overload [6, 7], resulting in right atrial and ventricle enlargement and ultimately pulmonary vas- culature remodeling and pulmonary hypertension with symptoms of right heart failure such as fatigue, decrease in functional capacity, and lung infections [7, 8]. The association between ASDs and cystic bro- sis is not well established. Two small series of patients with cystic brosis reported a 53–55% incidence of left-to-right shunt, mainly due to the presence of a patent foramen ovale (PFO) as seen by TEE [8, 9]. We can assume that patients with severe lung disease, such as cystic brosis in our case, will not tolerate hy- poxemia caused by a signi cant right-to-left shunt. Current American College of Cardiology/American Heart Association guidelines [10] recommend per- cutaneous interventional therapy for ASDs in the presence of right atrial and right ventricle enlarge- ment with or without symptoms before progressing to irreversible pulmonary hypertension, the presence of which is considered a contraindication for ASD closure. The impact of ASD closure on pulmonary function testing in patients with cystic brosis is not known. Belton et al. [11] reported a 29-year-old man with cystic brosis who underwent PFO closure after developing neurological symptoms. His pulmonary function had deteriorated at the time of the neuro- logical event. Closure of his PFO improved his pulmo- nary function with an increase in FEV1 from 1.36 to 1.89 (33% to 47% predicted). The authors concluded
that closure of the PFO may have had a signi cant im- pact on lung function. The indication for the rst ASD closure in our case was the presence of bidirectional ow, for which we aimed to prevent the development of irreversible pulmonary hypertension that would preclude ASD closure. The rst closure signi cantly improved pulmonary function testing before the de- vice migrated and led to a residual shunt with wors- ening symptoms and test results. We assume that the use of a high-frequency chest wall oscillation device dislodged the initial occlude, resulting in larger resid- ual shunt. The indication for the second closure was a neurological event caused by a paradoxical embo- lism. There was signi cant improvement in pulmo- nary function testing after the second ASD closure, which was not associated with a residual shunt im- mediately after the procedure.
In conclusion, percutaneous closure of ASD in pa- tients with cystic brosis and hypoxemia out of pro- portion to their lung disease may improve pulmonary function testing and hypoxemia, although further studies are warranted to con rm this possibility.
Con ict of Interest
The authors have no con ict of interest relevant to this publication.
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Journal of Structural Heart Disease, December 2017
Volume 3, Issue 6:187-191