The Unnatural History of an Atrial Septal Defect: Longitudinal 35 Year Follow Up After Surgical Closure at Young Age

Study Questions:

What are the long-term outcomes of surgical atrial septal defect (ASD) closure performed on patients <15 years of age?

Methods:

A cohort study was performed at a single center. The study population included patients who underwent surgical repair of secundum ASD or sinus venosus septal defect at <15 years of age between 1968 and 1980. Patients had undergone follow-up evaluation at 10-year intervals since surgery. The most recent evaluation included medical history, physical examination, electrocardiogram, Holter monitor, bicycle ergometry with oxygen consumption, measurement of NT-pro-BNP, cardiac magnetic resonance imaging, and the 36-item Short Form Health Survey (SF-36) questionnaire. The main outcome measures included survival, major events (including cardiac reinterventions, stroke, symptomatic arrhythmia, or heart failure), and ventricular function.

Results:

The median follow-up period was 35 years (range 30-41). Survival status was available for 131 of 135 patients. Five patients died, including two with sudden death in the last decade of follow-up. Symptomatic supraventricular tachyarrhythmias occurred in 14 (16%) patients, and 6 patients required pacemaker placement. Early postoperative arrhythmias were a risk factor for pacemaker placement. The mean left ventricular ejection fraction was 58.7 ± 7% and the mean right ventricular ejection fraction was 51 ± 6%. Right ventricular function was decreased in 31% of patients and the right ventricle remained dilated in 20% of patients. Exercise capacity and perceived health status by the SF-36 were comparable to the normal population.

Conclusions:

The authors concluded that long-term outcome after early surgical closure of ASD shows good survival and low morbidity.

Perspective:

This study shows overall good results for patients undergoing surgical ASD closure in childhood. Importantly, late development of pulmonary hypertension did not appear to be a significant issue. Late repair of ASD has been associated with a significantly increased likelihood of atrial arrhythmia. Although late arrhythmias did appear to be an issue in patients repaired at a younger age, they do not appear as prevalent as they are in patients repaired later in life.

Clinical Topics: Arrhythmias and Clinical EP, Congenital Heart Disease and Pediatric Cardiology, Heart Failure and Cardiomyopathies, Noninvasive Imaging, Implantable Devices, SCD/Ventricular Arrhythmias, Atrial Fibrillation/Supraventricular Arrhythmias, Congenital Heart Disease, CHD & Pediatrics and Arrhythmias, CHD & Pediatrics and Imaging, CHD & Pediatrics and Quality Improvement, Acute Heart Failure, Heart Failure and Cardiac Biomarkers, Magnetic Resonance Imaging

Keywords: Outcome Assessment (Health Care), Follow-Up Studies, Heart Defects, Congenital, Heart Conduction System, Cardiac Catheterization, Heart Septal Defects, Atrial, Risk Factors, Electrocardiography, Magnetic Resonance Imaging, Tachycardia, Cyclopentanes, Heart Failure, Stroke Volume, Death, Sudden, Cardiac, Natriuretic Peptide, Brain


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