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The Fontan Kreutzer procedure is used in pediatric patients who possess only a single functional ventricle, either due to lack of a heart valve (e.g. tricuspid or mitral atresia), an abnormality of the pumping ability of the heart (e.g. hypoplastic left heart syndrome or hypoplastic right heart syndrome), or a complex congenital heart disease where a bi-ventricular repair is impossible or ...
Currently, infants undergo either the staged reconstructive surgery (Norwood or Sano procedure within a few days of birth, Glenn or Hemi-Fontan procedure at 3 to 6 months of age, and the Fontan procedure at 1 1/2 to 5 years of age) or cardiac transplantation. [10] Current expectations are that 70% of those with HLHS may reach adulthood. [18]
The Fontan procedure, done usually after the patient is two years old, disconnects the inferior vena cava from the heart and connects it directly with the other pulmonary artery so that deoxygenated blood from the lower body then is sent directly to the lungs. [citation needed]
These patients typically require a Fontan procedure at 18 to 36 months of age following the Glenn BDG procedure. Examples of congenital cardiac malformations in which this procedure may be used include hypoplastic left heart syndrome , tricuspid atresia , double-inlet left ventricle and double-outlet right ventricle .
A double inlet left ventricle (DILV) or "single ventricle", is a congenital heart defect appearing in 5 in 100,000 newborns, where both the left atrium and the right atrium feed into the left ventricle.
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Rhabdomyomas may be either cardiac or extracardiac (occurring outside the heart). Extracardiac forms of rhabdomyoma are sub-classified into three distinct types: adult type, fetal type, and genital type. Cardiac rhabdomyomas are the most common primary tumor of the heart in infants and children. It has an association with tuberous sclerosis. [1]
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