The staff of the Adolescent and Adult Congenital Heart Disease Program at Nationwide Children's Hospital are experts in caring for patients with Atrial Septal Defects (ASD) and other congenital heart diseases. Our program is comprised of experts with an understanding of congenital heart disease as well as patient care after surgical or specialized transcatheter therapy. A full range of diagnostic and treatment capabilities are available to our patients.
The normal human heart is responsible for efficiently receiving and pumping blood. Proper blood flow is vital to health because the body’s tissues rely on the blood to carry nourishment (oxygen, glucose) and remove waste products (carbon dioxide). Under normal conditions, the cardiovascular system (heart, blood vessels) contains two separate circulatory systems - venous (right) and arterial (left). Venous (deoxygenated, blue) blood returns from the body to the right atrium.
Blood flows from the right atrium through the tricuspid valve into the right ventricle where blood is actively pumped across the pulmonary valve and into the pulmonary arteries. The main pulmonary artery divides into large right and left pulmonary arteries and then to small pulmonary arterioles. Adjacent to the small arterioles, numerous pulmonary air filled sacs. Within these small sacs, gases are exchanged. Waste products from the venous blood enter the air sacs and are removed from the pulmonary system when we breathe out (exhale). Oxygen that enters the lungs when we breathe in (inspiration) enters the small air sacs and diffuses into the blood. Oxygen-rich (red) blood returns from the lungs via pulmonary veins to the left atrium.
Muscular and connective tissues separate the right (blue blood) from the left (red blood) side of the heart. The heart is separated at the level of the receiving chambers (right and left atria) by the atrial septum, and at the pumping chambers (right and left ventricles) by the ventricular septum. Persistent opening between the atrial and ventricular septa in postnatal life is abnormal.
There are three common types of atrial septal defect (ASD):
Passive blood flow through the heart is driven by pressure differences. Blood flows across heart valves or other orifices from one chamber from high to low pressure. In atrial septal defect, there is usually higher pressure in the left atrium compared to right atrium, thus blood flows (or shunts) from left to right. Medical/surgical recommendations for closure of the ASD depend primarily on characteristics of the defect itself (size, location), symptoms or limitations related to the defect, and preferences of the patient and physician.
Potential long-term consequences of atrial defects include:
In order to minimize the detrimental long term complications related to ASD (volume and pressure overload, serious arrhythmias) and to prevent embolic events, ASD closure is usually recommended for children and adults.
Symptoms and/or abnormal examination findings such as abnormal splitting of the second heart sound, murmur, abnormal cardiac impulse raise concern for ASD. Common symptoms include irregular or rapid heart rhythm, cyanosis (bluish nails and skin), shortness of breath with or without exertion, cough, dizziness, fainting, fatigue, swelling (hands, face or legs), lack of appetite. Adults with ASD are frequently asymptomatic with minimal physical manifestations of ASD, and ASD may go undetected for many years.
History and physical examination findings that raise suspicion of ASD are followed by one or more diagnostic/imaging tests. These include: echocardiography-transthoracic, transesophageal (TEE) and intracardiac echocardiography; angiography (cardiac catheterization); and magnetic resonance imaging (MRI).
ASD is two to three times more common in girls than boys. ASD may alone or as an associated congenital cardiac defect. Atrioventricular conduction defects, ventricular septal defect, tetralogy of Fallot, and a variety of complex cyanotic congenital heart disease defects have occurred with ASD. A family history/pedigree showing relatives from different generations with ASD raises strong suspicion of a genetic etiology. ASD is heterogenous (caused by more than one gene). ASD occurs in individuals with a variety of syndromic and genetic disorders including Down syndrome (trisomy 21) and the following Mendelian genetic mutations:
4p16 (EVC; Ellis van Creveld syndrome)
6q21.3 (ASD I, ASD II)
12q24.1 (TBX5; Holt Oram syndrome)
20p12 (JAG1, AGS, AHD; Alagille syndrome)
22q11.2 (velocardiofacial/DiGeorge syndrome)
Diuretics and antiarrhythmia medications may be needed to treat the symptoms of ASD, however definitive therapy requires closure of the ASD.
There are two approaches to ASD closure:
Read about Nationwide Children's role in a bloodless atrial septal defect repair (ASD) in an 8 month-old.
Learn more about Atrial Septal Defect.