What is a Fontan heart procedure?

The Fontan procedure refers to any surgical procedure that leads to systemic flow of venous blood to the lungs without passing through a ventricle. In 1971, Fontan and Baudet (1) described a surgical procedure for repair of tricuspid atresia that built on experimental and clinical research from the 1940s.

What is PA IVS?

DEFINITION. PA/IVS is a rare congenital cardiac defect that consists of atresia of the pulmonary valve resulting in an absent connection between the right ventricular outflow tract (RVOT) and pulmonary arteries as well as an IVS that allows no connection between the right and left ventricles.

What is pulmonary atresia?

Pulmonary atresia is a birth defect (pronounced PULL-mun-airy ah-TREE-sha) of the heart where the valve that controls blood flow from the heart to the lungs doesn’t form at all. In babies with this defect, blood has trouble flowing to the lungs to pick up oxygen for the body.

What does truncus arteriosus mean?

Truncus arteriosus is a birth defect of the heart. It occurs when the blood vessel coming out of the heart in the developing baby fails to separate completely during development, leaving a connection between the aorta and pulmonary artery.

What is IVS in heart ultrasound?

Fetal pulmonary atresia with intact ventricular septum (PA/IVS) is a rare congenital heart disease. The present study aimed to classify PA/IVS and determine the relationship between prenatal echocardiographic characteristics and postnatal biventricular or univentricular repair strategies.

How is IVS diagnosed?

Diagnosis. Pulmonary atresia with intact ventricular septum can be detected during pregnancy through a routine fetal echocardiogram (ultrasound of the fetal heart).

What is the survival rate of 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.

Is pulmonary atresia life threatening?

In some cases, babies with pulmonary atresia may also have a small, or missing, right ventricle that can’t properly pump blood to the lungs. Pulmonary atresia is a life-threatening condition, affecting one out of every 10,000 newborns.

Can a baby survive with truncus arteriosus?

In rare cases, a person with truncus arteriosus can survive infancy without surgical repair of the heart and live into adulthood. However, people with this condition will almost certainly develop heart failure and pulmonary hypertension (Eisenmenger syndrome).

How long can you live with truncus arteriosus?

Conclusions: Ten- to 20-year survival and functional status are excellent among infants undergoing complete repair of truncus arteriosus.

How old is the oldest Fontan patient?

The oldest survivor after the Fontan operation was 67 years of age (Fontan at age 39 years).

What is normal IVS?

IVSd and IVSs – Interventricular septal end diastole and end systole. The normal range is 0.6-1.1 cm.

What is a normal LVEF?

A normal left ventricular ejection fraction (LVEF) ranges from 55% to 70%. An LVEF of 65%, for example, means that 65% of the total amount of blood in the left ventricle is pumped out with each heartbeat. Your EF can go up and down, based on your heart condition and how well your treatment works.

Is IBS life threatening?

While irritable bowel syndrome (IBS) is not life-threatening for most, it has enormous influence on quality of life (QOL) and mental health.

Is pulmonary atresia fatal?

These passages are necessary when a baby is developing in the womb, and they typically close soon after birth. Babies with pulmonary atresia typically have a bluish cast to their skin because they aren’t getting enough oxygen. Pulmonary atresia is a life-threatening situation.

How many babies are born with pulmonary atresia?

Because of the low amount of oxygen given to the body, pulmonary atresia is one of the heart problems that is labeled “blue-baby syndrome.” Pulmonary atresia occurs in about one out of every 10,000 live births.