By Bennett P. Samuel, MHA, BSN, RN; Yasser Al-Khatib, MD; Cynthia E. L. Peacock-McKenzie, MD; Reda E. Girgis, MD; Joseph J. Vettukattil, MBBS, MD, DNB, CCST, FRCPCH, FRSM, FRCP
Introduction
Pulmonary Arterial Hypertension (PAH) is a chronic disease with progressively increasing right ventricular (RV) pressure, Right-Heart Failure (HF), and death.1 An association between PAH and secundrum-type Atrial Septal Defect (ASD) is observed in 9 to 35% of patients, especially in females. It is speculated that ASD-Associated PAH resolves after intervention and rarely progresses especially with early intervention.2-4 However, as most of these patients are left with untreated ASDs, there is a dearth of information. Fenestrated ASD closure is preferable in patients with moderate to severe PAH. A restricted interatrial shunt in these patients can enhance systemic ventricular output at the expense of desaturation if shunt reversal occurs when progressive PAH ensues. Maintaining a sustainable restricted interatrial communication is challenging without the use of a dedicated device such as the Occlutech® Fenestrated Atrial Septal Defect (FASD) occluder (Figure 1). We describe compassionate use of the FASD Occluder with optimal outcomes in a 56-year-old female with ASD-Associated PAH.
To read the full article, please go to the May 2017 Issue of CCT, where it was originally published.