Atrioventricular septal defect natural history

Jump to navigation Jump to search

Atrioventricular septal defect Microchapters


Patient Information





Differentiating Atrioventricular septal defect from other Diseases

Epidemiology and Demographics

Risk Factors

Natural History, Complications and Prognosis


History and Symptoms

Physical Examination

Laboratory Findings


Chest X Ray




Cardiac Catheterization


Medical Therapy




Cost-Effectiveness of Therapy

Future or Investigational Therapies

Atrioventricular septal defect natural history On the Web

Most recent articles

Most cited articles

Review articles

CME Programs

Powerpoint slides


American Roentgen Ray Society Images of Atrioventricular septal defect natural history

All Images
Echo & Ultrasound
CT Images

Ongoing Trials at Clinical

US National Guidelines Clearinghouse

NICE Guidance

FDA on Atrioventricular septal defect natural history

CDC on Atrioventricular septal defect natural history

Atrioventricular septal defect natural history in the news

Blogs on Atrioventricular septal defect natural history

Directions to Hospitals Treating Type page name here

Risk calculators and risk factors for Atrioventricular septal defect natural history

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]


Natural history

The true natural history of an atrioventricular septal defect is difficult to definitively determine. No record exists to detail the course of disease of an untreated complete atrioventricular septal defect. In patients with a moderate case of a partial or incomplete atrioventricular septal defect, patients may be asymptomatic in the first decade of life. During adolescence, left-to-right shunting and atrial arrhythmia may present. As continued development occurs in these patients, sinus node dysfunction may develop and can lead to exercise intolerance. More severe cases of partial or incomplete atrioventricular septal defects left untreated can lead to morbidity in infancy and early childhood.

In general, long-term survival of patients with all types of atrioventricular septal defects had an overall poor prognosis. Mortality was higher in complex atrioventricular septal defect cases. Approximately 80% of patients with a complete AVSD died by age 2. Other studies have observed a trend between survival in the first year of life and complexity of defect. In one study, 54% of infants with a complete atrioventricular septal defect survived the first 6 months, 35% survived into one year of life, and only 4% survived 5 years of life.

There is no evidence to suggest that the association between atrioventricular septal defects and Down syndrome correlates to equally grim outcomes. One study documented only 4 late deaths over a 27-year period in patients greater than 1 year of age with un-operated defects.


Common complications from atrioventricular septal defect include:


When treated with surgical repair, patients with atrioventricular septal defect have a minimal long-term morbidity. Complete atrioventricular septal defect repair is 3.6% and the 10-year survival rate is 81%. Some patients may live the first few years of their life without surgical intervention. The surgical survival rate for pediatric complete atrioventricular septal defect is 94% with an overall survival rate of 91% of patients repaired between 4-6 months of age.


Template:WH Template:WS CME Category::Cardiology