eMedicine Specialties > Cardiology > Congenital Heart Disease in the Adult

Atrial Septal Defect: Follow-up

Author: Bekir Hasan Melek, MD, Assistant Professor of Clinical Medicine, Department of Medicine, Section of Cardiology, Tulane University School of Medicine
Coauthor(s): James V Talano, MD, MM, FACC, Director of Cardiovascular Medicine, SWICFT Institute; Jeffrey C Milliken, MD, Chief, Division of Cardiothoracic Surgery, University of California at Irvine Medical Center; Clinical Professor, Department of Surgery, University of California at Irvine School of Medicine; Peter B Smulowitz, BA, University of California at Irvine School of Medicine
Contributor Information and Disclosures

Updated: Jul 11, 2006

Outcome and Prognosis

Natural history

Although life expectancy is not normal, patients generally survive into adulthood without surgical or percutaneous intervention, and many patients live to advanced age. However, natural survival beyond age 40-50 years <50%, and the attrition rate after 40 years of age is about 6% per year. Advanced pulmonary hypertension seldom occurs before the third decade. Late complications are stroke and atrial fibrillation.

Postsurgical prognosis

The mortality rate of surgical repair is <1% for patients younger than 45 years without heart failure and who have systolic pulmonary artery pressures <60 mm Hg. The morbidity rate is low. The surgical mortality rate increases with increasing age and pulmonary artery pressures.

Surgical repair should be considered for all patients with uncomplicated ASDs with a clinically significant left-to-right shunt. Such repair is ideally done at 2-4 years of age. Early surgical repair is considered in a few infants and young children with clinically significant symptoms or CHF. Surgery before the age of 25 years results in a 30-year survival rate comparable to that of age- and sex-matched control subjects. However, at 25-40 years of age, surgical survival is reduced, though not significantly if pulmonary artery pressures are normal. If pulmonary artery systolic pressure is >40 mm Hg, late survival is 50% less than control rates, though life expectancy in surgically treated older patients is better than that of medically treated patients. Even in select patients older than 60 years with no serious comorbidities, ASDs should be closed as early as possible if an indication is present because surgery improves symptoms–at least in the short term–regardless of pulmonaryartery pressure or

functional class, as long as the left-to-right shunt remains large. Although surgical closure of ASDs in adulthood is associated with a significant mortality benefit, its benefit is limited in preventing atrial arrhythmias. The patient's age at the time of closure is the most important predictor of the development of atrial arrhythmia.

Surgery for sinus venosus ASD is also associated with low morbidity and mortality, and postoperative subjective clinical improvement occurs irrespective of the patient's age at surgery. However, in contrast to ostium secundum ASD, surgery for sinus venosus defect is relatively complex and poses the risks of stenosis of the superior vena cava or pulmonary veins, residual shunting, and dysfunction of the sinoatrial node.

In childhood, right ventricular dimensions decrease, often strikingly, after surgery. However, when adults undergo surgery, the dimensions remain abnormal in approximately 80% of patients. If right ventricular failure and tricuspid regurgitation are present before surgery, late postoperative right atrial and ventricular enlargement is typical, and right ventricular systolic function seldom normalizes. Patients in this situation improve, but they usually remain symptomatic, and their preoperative pulmonary vascular resistance influences their long-term outcome.

A few patients who undergo surgical closure during childhood have late-onset supraventricular arrhythmias, which are believed to be related to patchy fibrosis of the right atrium secondary to dilatation and perhaps dysfunction of the sinus node. In adults, chronic preoperative atrial fibrillation usually persists after surgical repair, but cardioversion followed by antiarrhythmics treatment may be effective. If surgery is performed in patients older than 40 years, 50% of those with preoperative normal sinus rhythm have late postoperative atrial fibrillation. Intracardiac electrophysiologic studies have shown a high incidence of intrinsic dysfunction of the sinoatrial and AV nodes that persists after surgical repair. These nodal abnormalities are most common in the sinus venosus type than in the secundum type.

Late events, including atrial fibrillation, stroke, heat failure, are most common in patients undergoing repair in adulthood. This observation emphasizes the benefit of early repair of secundum ASDs in symptomatic patients. The unfavorable prognosis of late repairs is presumably related to longstanding deleterious effect of volume overload on the chambers on the right side, of pulmonary hypertension, and of right atrial enlargement with increased vulnerability to atrial arrhythmias and stroke. About 22% of late deaths are attributed to cerebrovascular events. Older age at repair and preoperative New York Heart Association class III or IV heart failure are independent predictors of late mortality. They are also predictive of atrial fibrillation, for which sinus node dysfunction with bradycardia-dependent atrial arrhythmias, scar-dependent multiple reentries, and atrial enlargement or atrial fibrosis due to increased pulmonary venous pressure with exercise are implicated as potential mechanisms.

Prognosis after transcatheter closure

See Treatment above.

Common comorbidities

Common comorbidities include the following:

  • Pulmonary hypertension
    • Pulmonary hypertension (mean pulmonary artery pressure >20 mm Hg or systolic pulmonary artery pressure >50 mm Hg) occurs in 15-20% of patients with ASD.
    • This condition is unusual in young patients, but it is observed in 50% of patients older than 40 years.
    • In Eisenmenger syndrome—a late and rare complication of isolated secundum ASD that occurs in 5-15% of patients—extreme pulmonary obstruction may result in a reversal of the shunt of blood to a right-to-left flow. Desaturated blood entering the systemic circulation results in systemic hypoxemia and cyanosis.
  • Right-sided heart failure
    • Heart failure is due to the cardiac volume overload experienced on the right side of the heart because of left-to-right shunting.
    • In patients of all ages, substantial relief of such a complication is generally observed after the defect is closed.
  • Atrial fibrillation or flutter
    • This condition is uncommon in young patients, though it is reported in as many as 50-60% of patients older than 40 years. Therefore, these arrhythmias occur most frequently with age, and they may become a major cause of morbidity and mortality.
    • The use of anticoagulants is indicated in patients with atrial fibrillation because of the high risk of stroke. Although atrial fibrillation may be present in patients before surgery, surgery may also cause it.
  • Stroke
    • Regardless of their surgical status, 5-10% of patients have thromboembolic events (including stroke and transient ischemic attacks) on long-term follow-up.
    • Even with small defects, paradoxical emboli may occur. Therefore, the presence of an ASD should be considered in any patient with a cerebral or other systemic embolus in whom no left-sided source is demonstrable.

Future and Controversies

With increased experience over the years, transcatheter closure of suitable ASDs has now become preferable to surgical repair. Limitations currently include size and location of the defect.

Perhaps the most innovative approach to surgical closure in many years was recently accomplished in the form of robotically assisted closure of ASD. Current technology allows for excellent visualization and magnification of internal anatomy, and the ability to perform surgery at a remote distance from the patient is now a reality. However, even with this amazing technology, today's devices will seem crude compared with future computer robots. Improved access and cardiopulmonary bypass technology will most likely make robotically assisted heart surgery a routine procedure in the foreseeable future.

 


More on Atrial Septal Defect

Overview: Atrial Septal Defect
Workup: Atrial Septal Defect
Treatment: Atrial Septal Defect
Follow-up: Atrial Septal Defect
References
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Further Reading

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Keywords

atrial septal defect, atrial septum, ASD, ostium secundum ASD, sinus venosus defect, ostium primum defect, ostium secundum defect, congenital heart disease, congenital cardiac disorder, ventricular dilatation, thoracic surgery, pulmonary hypertension, Eisenmenger syndrome, Holt-Oram syndrome, transcatheter occlusion devices, dyspnea, fatigue, palpitations, syncope, congestive heart failure, CHF

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Contributor Information and Disclosures

Author

Bekir Hasan Melek, MD, Assistant Professor of Clinical Medicine, Department of Medicine, Section of Cardiology, Tulane University School of Medicine
Bekir Hasan Melek, MD is a member of the following medical societies: American Association for the Advancement of Science, American College of Cardiology, American College of Physicians, American Heart Association, American Medical Association, American Society of Echocardiography, and Louisiana State Medical Society
Disclosure: Nothing to disclose.

Coauthor(s)

James V Talano, MD, MM, FACC, Director of Cardiovascular Medicine, SWICFT Institute
James V Talano, MD, MM, FACC is a member of the following medical societies: American College of Cardiology, American College of Chest Physicians, American College of Physician Executives, American College of Physicians, American Heart Association, American Society of Echocardiography, American Society of Nuclear Cardiology, Heart Failure Society of America, and Society of Geriatric Cardiology
Disclosure: Nothing to disclose.

Jeffrey C Milliken, MD, Chief, Division of Cardiothoracic Surgery, University of California at Irvine Medical Center; Clinical Professor, Department of Surgery, University of California at Irvine School of Medicine
Jeffrey C Milliken, MD is a member of the following medical societies: Alpha Omega Alpha, American Association for Thoracic Surgery, American College of Cardiology, American College of Chest Physicians, American College of Surgeons, American Heart Association, American Society for Artificial Internal Organs, California Medical Association, International Society for Heart and Lung Transplantation, Phi Beta Kappa, Society of Thoracic Surgeons, Southwestern Oncology Group, and Western Surgical Association
Disclosure: Nothing to disclose.

Peter B Smulowitz, BA, University of California at Irvine School of Medicine
Disclosure: Nothing to disclose.

Medical Editor

Park W Willis IV, MD, Sarah Graham Distinguished Professor of Medicine and Pediatrics, University of North Carolina at Chapel Hill School of Medicine
Park W Willis IV, MD is a member of the following medical societies: American Society of Echocardiography
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Steven J Compton, MD, FACC, FACP, Director of Cardiac Electrophysiology, Alaska Heart Institute, Providence and Alaska Regional Hospitals
Steven J Compton, MD, FACC, FACP is a member of the following medical societies: Alaska State Medical Association, American College of Cardiology, American College of Physicians, and Heart Rhythm Society
Disclosure: Nothing to disclose.

CME Editor

Amer Suleman, MD, Consultant in Electrophysiology and Cardiovascular Medicine, Department of Internal Medicine, Division of Cardiology, Medical City Dallas Hospital
Amer Suleman, MD is a member of the following medical societies: American College of Physicians, American Heart Association, American Institute of Stress, American Society of Hypertension, Federation of American Societies for Experimental Biology, Royal Society of Medicine, and Society of Cardiac Angiography and Interventions
Disclosure: Nothing to disclose.

Chief Editor

Michael E Zevitz, MD, Assistant Professor of Medicine, Finch University of the Health Sciences, The Chicago Medical School; Consulting Staff, Private Practice
Michael E Zevitz, MD is a member of the following medical societies: American College of Cardiology, American College of Physicians, American Medical Association, and Michigan State Medical Society
Disclosure: Nothing to disclose.

 
 
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