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 and Pediatrics and Arrhythmias, CHD and Pediatrics and Imaging, CHD and 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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