19 February 2018 by Joseph Vettukattil

Atrial Septal Defect Associated Pulmonary Arterial Hypertension: Outcomes of Closure with a Fenestrated Device

Background

Unlike other intracardiac shunts, there is no known linear relationship between ostium secundum atrial septal defects (ASD) and development of pulmonary hypertension (PH). However, PH is observed in 8-10% of all ASDs of which 85% are females. ASD associated PH (ASDPH) is usually independent of the degree of shunting, and defect size. Complete closure in severe PH is not recommended due to the risk for increase in mean pulmonary arterial pressure (MPAP). Fenestrated ASD (FASD) closure allows for controlled residual shunt providing adequate cardiac output with a mechanism for decompression in the event of critical increase in MPAP. The lack of an ASD closure device with a predefined fenestration led to the development of the Occlutech FASD device. We describe outcomes of FASD implantation in patients with ASDPH.

Methods

Patients meeting echocardiographic inclusion criteria were selected for compassionate use of the FASD device at 11 international centers. Physician implanters provided outcomes data for analysis via electronic survey.

Results

Forty patients underwent FASD device implantation. Follow-up data was available for 11 patients (females n=7) with mean age of 46 years (range 5 to 72 years). Oxygen saturations improved significantly from baseline mean of 92% (82-100%, n=13) to 96% (89-99%, n=11) on long-term follow-up (p=0.0147). MPAP increased from 49 to 52 mmHg (p=0.706) and right atrial pressure had a trivial decrease from mean of 10 to 9.9 mmHg. Symptomatic improvement was
observed with NYHA Class III symptoms noted in 76% at baseline (n=13) down to 10% at long-term follow-up (n=10). During follow-up, one patient had spontaneous occlusion of the fenestration requiring emergency stenting. No other major complications were observed.

Conclusions

FASD device implantation results in significant symptomatic improvement in patients with ASDPH. However, further studies are required in a larger cohort of patients to determine long-term prognosis.

About the author


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Joseph Vettukattil

Co-Director

United States

Key Contributors

Joseph Vettukattil MBBS, MD, DNB, CCST, FRCPCH, FRSM, FRCP; Vishal Kaley MBBS, MD; Nagib Dahdah MD; Amal El-Sisi MD; Jochen Grohman MD; Nikolaus Haas MD, PhD; Nicholas Hayes BSc, MBChB, MRCPCH; Gareth Morgan MPhil, MRCPCH; Eustaquio Onorato MD; Supaporn Roymanee MD; Anselm Uebing MD, PhD; Walter Wiebe MD; Ziyad M. Hijazi, MD, MPH, FACC, MSCAI


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