Atrial septal defect natural history

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-In-Chief: Priyamvada Singh, M.B.B.S. [2]; Assistant Editor(s)-In-Chief: Kristin Feeney, B.S. [3]


Infants and adolescent patients may be asymptomatic until later in life. By 40 years of age, approximately 90% of untreated atrial septal defect patients will experience an onset of symptoms. Symptom onset and severity is largely dependent upon the size of the defect. Without intervention prior to the development of Eisenmenger's syndrome, the mortality rate for symptomatic adults is greater than 50%. Possible complications include atrial fibrillation, pulmonary hypertension and stroke.

Natural History

As many atrial septal defect patients are asymptomatic, it is common to survive into adulthood without any need for intervention. Many atrial septal defects smaller than 8 mm in diameter close spontaneously during infancy. Spontaneous closure is uncommon in children and adults. During adulthood there can be the onset of symptoms and an altered life expectancy. Beyond 40-50 years of age, survival without intervention is under 50% with a mortality rate of about 6% per year. Complications occur later in life and include atrial fibrillation, pulmonary hypertension, and stroke.

In a study involving 128 adult (age range 18 to 67 years) atrial septal defect patients, 75% were found to be symptomatic with mild to moderate non-progressive symptoms. Less than 25% of patients had significant pulmonary artery hypertension. Pulmonary artery involvement can be progressive and can lead to shunt reversal, Eisenmenger’s syndrome and ultimately death. The prognosis following medical and / or surgical treatment of pulmonary hypertension can be poor. Heart failure and atrial arrhythmias were other complications found associated with atrial septal defects, mostly in elderly patients.[1]


Atrial septal defect is associated with complications such as atrial fibrillation, pulmonary hypertension, heart failure, and stroke.

Atrial Septal Defect and Atrial Fibrillation

50-60% of atrial septal defect patients over the age of 40 will develop atrial fibrillation. Late-onset atrial fibrillation is associated with both morbidity and mortality. Anticoagulation may lower the mortality risk. Closing an ASD at this point does not prevent occurrence of atrial fibrillation in these patients. But early closure of ASD lowers the risk of developing atrial fibrillation.

Atrial Septal Defect and Pulmonary Hypertension

15-20% of atrial septal defect patients develop pulmonary hypertension. Although rare in children and adolescents, pulmonary arterial hypertension is observed in approximately 50% of patients over the age of 40. The development of Eisenmenger's syndrome can result in reversal of the original left-to-right shunt which may switch to become a right-to-left shunt. Right-to-left shunting can in turn lead to deoxygenation (hypoxemia and cyanosis).

Atrial Septal Defect and Right Heart Failure

Atrial septal defect is associated with left-to-right shunting which in turn may be associated with right ventricular volume overload. Patients may experience right heart failure as a result of right ventricular volume overload.

Atrial Septal Defect and Stroke

Even without surgery, as many as 5-10% of all atrial septal defect patients experience thromboembolic events such as stroke and transient ischemic attack. Paradoxical emboli in atrial septal defect patients is not correlated with defect size and can occur in all ASD patients. Early closure of ASD lowers the risk of developing atrial fibrillation.


The prognosis for most atrial septal defect patients, especially prior to the age of 40, is positive. As atrial septal defect patients age, symptoms and complications may advance and influence quality of life. With surgical intervention, the mortality rate is less than 1% for atrial septal defect patients under 45. Surgical intervention in older populations is equally promising and can result in longer longevity and improvements in quality of life. Left untreated, the prognosis for atrial septal defect patients is significantly less favorable and may lead to earlier death.

  • Most atrial septal defect patients live to advance in age.
  • If an atrial septal defect is detected and diagnosed early in life, surgery is most successful when performed at 2-4 years of age.
  • As atrial septal defect patients age, mortality rates of surgical intervention repair increases linearly with age and pulmonary artery pressure.
  • The mortality rate for surgical intervention repair is less than 1% for atrial septal defect patients under the age of 45, with no pulmonary artery pressure issues.
  • Repair is normally only performed in youth with clinically significant complications. Surgery is still advised for all patients with uncomplicated ASDs with clinically significant shunting from left-to-right. Patients with normal pulmonary artery pressure have comparable age and sex matched 30-year survival rates until the age of 40.
  • Patients with pulmonary artery pressure issues may experience a lower survival rate.
  • Life expectancy in surgical therapy older patients, though, is better than that of patients with medical therapy. In a small population of patients over the age of 60, presenting without comorbodities, still benefit from surgical intervention.
  • In general, surgical intervention of an ASD is associated with an increase in mortality. However, the age of the patient at the time of surgical closure is the single most important predictor of development of potentially serious life-threatening comorbidities.
  • Most types of atrial septal defects, when repaired with surgical intervention, are associated with low mortality and low morbidity risks.
  • In children, surgical repair can even result in reduction in the right ventricular size from abnormal to closer to normal. Size reduction is not as common in adults with only 20% of all repairs experiencing changes in dimensions. Patients with tricuspid regurgitation and right ventricular failure may experience lifelong symptoms and may have differed long-term outcomes.
  • Complications are more common in older atrial septal defect patients after surgical intervention. It is believed that prognosis is influenced by the long-term effect of volume overload on the chambers of the right side. It is also associated with the volume overload as a result of right atrial enlargement and of pulmonary hypertension. These particular strains on the right chamber of the heart lead to increased risk for atrial arrhythmia and stroke. As many as 22% of late deaths can be attributed to cerebrovascular events. Age at repair as well as preoperative status, such as NYHA class III and IV heart failure, are independent predictors of mortality.


  1. Craig RJ, Selzer A (1968). "Natural history and prognosis of atrial septal defect". Circulation. 37 (5): 805–15. PMID 5646864.

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