Atrial septal defect with pulmonary hypertension: when/how can we consider closure?

Abstract

Patients having atrial septal defect (ASD) with moderate and more importantly severe pulmonary arterial hypertension (PAH) pose a clinical dilemma. Closing ASD in those with irreversible PAH and not closing it when the PAH is reversible can cost patients dearly, both in terms of quality of life and longevity. In our experience, there is no single parameter that can help in decision making in this difficult subset of patients and therefore we recommend a multi-dimensional approach, which takes into consideration clinical, radiological, electrocardiographic and hemodynamic variables as a whole. ASD with restrictive left ventricular (LV) physiology can lead to pulmonary venous hypertension, which can manifest as life threatening acute pulmonary edema following device closure. All high-risk candidates prone to having this combination should be prepared with diuretics and vasodilators prior to bringing them to catheterization laboratory and should be assessed with temporary balloon/device occlusion prior to permanent closure of the defect. In those cases of ASD with borderline operability either due to severe PAH or LV restrictive physiology, perforated device may be helpful in preventing acute or long-term complications of complete closure.

Keywords: Atrial septal defect (ASD); Eisenmenger syndrome; cardiac catheterization; pulmonary hypertension (PH); pulmonary vascular resistance index (PVRI).

Figure 1

Atrial septal defect with left ventricular systolic dysfunction. (A) Twelve-lead ECG of a 31-year-old gentleman with recent anterior wall myocardial infarction. (B) His coronary angiogram revealed recanalized left anterior descending artery. (C) Two-dimensional transthoracic echocardiography (TTE) showing apical infarction. (D) Transesophageal (TEE) showing a moderate sized ostium secundum ASD. ASD, atrial septal defect.

Figure 2

Atrial septal defect with left ventricular restriction. (A) Twelve-lead ECG of a 20-year-old with hypertrophic cardiomyopathy. (B) Transthoracic 2D echocardiography showing a moderate sized ASD. (C) Concentric left ventricular hypertrophy. ASD, atrial septal defect.

Figure 3

Serial X-ray chest at 6, 24 and 48 hours in a 65-year-old lady who went into florid pulmonary edema following device closure of ASD due to restrictive LV physiology. She did not respond to IV diuretics and positive pressure ventilation and so the device had to be retrieved after which she made an uneventful recovery (Courtesy: Dr. Biswajit Bandopadhyay). ASD, atrial septal defect; LV, left ventricular.

Figure 4

Hemodynamic data at baseline (A) and after balloon occlusion (B) of the ASD in the same patient as shown in Figure 1. Note the elevated baseline left ventricular end diastolic pressure (LVEDP) and pulmonary artery wedge pressure (PAWP) with further increase after temporary balloon occlusion. The defect was not closed (Courtesy: Dr. Prafulla Kerkar). ASD, atrial septal defect.

Figure 5

LVEDP and PAWP at baseline (A) and after balloon occlusion (B) of the ASD in the same patient as shown in Figure 2. Note the normal baseline pressures, which increased significantly after balloon occlusion. The ASD was not closed (Courtesy: Dr. Prafulla Kerkar). ASD, atrial septal defect.

Figure 6

Fenestrated Amplatzer septal occluder.

Figure 7

Simultaneous recording of aortic and pulmonary arterial tracings in an 18-year-old girl with ASD and severe PHT (A) before and (B) after balloon occlusion of the defect. Note significant drop in pulmonary artery pressures (arrows) after temporary balloon occlusion. ASD, atrial septal defect.

Figure 8

Echocardiographic images from parasternal long axis (A,C) and parasternal short axis (B,D) taken before (A,B) and 10 months after device closure of the ASD (C,D) showing significant reverse remodeling of the right ventricle. RV, right ventricle; LV, left ventricle; ASD, atrial septal defect.