What are coronary Sinusoids?

Severe right ventricle hypoplasia can result in fistulous connections from the right ventricle and to the coronary artery, called coronary sinusoids, which might be essential for coronary circulation. Pulmonary atresia with VSD is considered a severe type of Tetralogy of Fallot.

What is pulmonary atresia with intact ventricular septum?

Overview. Pulmonary atresia with intact ventricular septum is a congenital heart defect in which: The pulmonary valve doesn’t form properly. The pulmonary valve allows blood to flow from the heart to the lungs to get oxygen.

What is RV dependent coronary circulation?

RVDCC was defined as the presence of any coronary-cameral fistula with coronary obstruction proximal to the fistula and angiographic evidence of RV perfusion of the myocardium through the fistulous communication.

How long can you live with pulmonary atresia?

Pulmonary atresia with VSD is the ultimate form of tetralogy of Fallot and is estimated to represent 5% to 10% of tetralogy of Fallot patients. The survival rate without surgical repair is as low as 50% at 1 year of age and 8% at 10 years.

What is coronary-cameral fistula?

Coronary-cameral fistulae are rare congenital anomalous communications that may be single or multiple and occur between 1 or more coronary arteries and a cardiac chamber. This anomaly more frequently arises from the right coronary system (≈55%) but can originate from the left side (35%) or bilaterally (5%).

What is the life expectancy of someone with pulmonary atresia?

Can pulmonary atresia be corrected?

In most cases of pulmonary atresia, a baby may need surgery soon after birth. During surgery, doctors widen or replace the pulmonary valve and enlarge the passage to the pulmonary artery.

How is coronary fistula treated?

Infants with a larger fistula will need to have surgery to close the abnormal connection. The surgeon closes the site with a patch or stitches. Another treatment option plugs up the opening without surgery, using a special wire (coil) that is inserted into the heart with a long, thin tube called a catheter.