Little Zippers

CHD Library

Aortic Stenosis - a valvular heart disease caused by the incomplete opening of the aortic valve

Atrial Septal Defect (ASD) - a type of congenital heart defect in which there is an abnormal opening in the dividing wall between the upper filling chambers of the heart (the atria) – often referred to as a hole in the heart

Atrioventricular Septal Defect (AVSD) – these defects comprise a range of malformations characterized by varying degrees of incomplete development of the inferior portion of the atrial septum, the inferior portion of the ventricular septum, and the AV valves.

Bicuspid Aortic Valve - a defect of the aortic valve that results in the formation of two leaflets or cusps instead of the normal three. 

Cardiomyopathy - the deterioration of the function of the myocardium (i.e., the actual heart muscle).

Coarctation of the Aorta (CoA) -  a narrowing of the aorta that can affect the body's blood circulation because the left side of the heart has to work harder to pump blood through the narrowed aorta.

Dextrocardia – when the heart forms it is in the right chest. A heart in the right chest can be perfectly normal and create no problems. A heart in the right chest may also have holes in the heart or abnormal valves.

Ebstein's Anomaly – an abnormality in the tricuspid valve.  Ebstein's anomaly can range from very mild, with little symptoms, to very severe.

Hypoplastic Left Heart Syndrome (HLHS) - the left side of the heart is severely underdeveloped.

Hypoplastic Right Heart Syndrome (HRHS) - the right side of the heart is severely underdeveloped, but is much more rare than HLHS. The three stage surgeries are almost the same.

Children born with Hypoplastic Right or Left Heart Syndrome require surgery in order to survive. Without surgery or transplantation, HRHS and HLHS is 100% fatal. With surgical repair or a heart transplant, about 60% of children born with these defects live at least 5 years. Because this is the first generation that have been saved with these defects, long term life expectancy is unknown and is proving to be very unpredictable.

Interrupted Aortic Arch (IAA) - Interrupted Aortic Arch (IAA) is the absence or discontinuation of a portion of the aortic arch that can only be corrected with surgery.

levo-Transposition of the Great Arteries (l-TGA) – a heart defect in which the aorta and pulmonary artery are malpositioned and the atrial to ventricular and ventricular to arterial connections are malposed.  This results in “normal” delivery of blood to the body.  It is often associated with rhythm disturbances (especially heart block).  Alone, this defect usually will not usually cause significant problems; however it is often accompanied by other significant heart defects.

Mitral Stenosis - a valvular heart disease characterized by the narrowing of the orifice of the mitral valve of the heart. 

Patent Ductus Arteriosus (PDA) - the ductus arteriosus fails to close after birth. Symptoms are uncommon but in the first year of life include increased work of breathing and poor weight gain. In older children or adults the PDA may lead to congestive heart failure if left uncorrected.

Pulmonary Atresia - a malformation of the pulmonary valve in which the valve orifice fails to develop.

Pulmonary Atresia With Intact Ventricular Septum (PAVIS) - a very rare malformation of the pulmonary valve in which the valve orifice fails to develop and there is no outlet (VSD hole) for the blood to escape.

Pulmonary Stenosis - a valvular heart disease in which outflow of blood from the right ventricle of the heart is obstructed at the level of the pulmonic valve. This results in the reduction of flow of blood to the lungs.

Tetralogy of Fallot (ToF) - involves four heart malformations:  VSD, Pulmonary Stenosis, Overriding Aorta, Right Ventricular Hypertrophy.

Total Anomalous Pulmonary Venous Connection (TAPVC) - a rare cyanotic CHD in which all four pulmonary veins are malpositioned and make anomalous connections to the systemic venous circulation.

dextro-Transposition of the Great Arteries (d-TGA) - a cyanotic CHDin which the primary arteries (the aorta and the pulmonary artery) are transposed.

Tricuspid Atresia - a complete absence of the tricuspid valve. Therefore, there is an absence of right atrioventricular connection. This leads to a hypoplastic or an absence of the right ventricle.

Truncus Arteriosus - the embryological structure known as the truncus arteriosus never properly divides into the pulmonary artery and aorta.

Ventricular Septal Defect (VSD) - a defect in the ventricular septum, the wall dividing the left and right ventricles of the heart.  VSDs are collectively the most common CHDs.