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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 ...
The last surgery, known as the Fontan procedure, is typically performed around the age of 18 months and older. The final result of these surgeries is to redirect the superior and inferior vena cavae into the pulmonary artery, bypassing the right atrium. [citation needed] In a stage 1 Norwood procedure for hypoplastic right heart, the main ...
[1] [2] Isolated levocardia may occur with heart defects and patients without having operations have low life expectancy: only about 5% to 13% of patients survive more than 5 years. [3] Therefore, even though the risk of cardiac surgeries is high, once patients are diagnosed , operations are suggested to be held as soon as possible.
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]
An estimated annual 1,000 Fontan procedures are performed annually in the United States, with an estimated 50,000 to 70,000 patients having completed the procedure as of 2018. [14] [15] In Europe, an estimated 25,000 patients have completed the Fontan procedure as of 2021. [4]
Most patients who undergo a Norwood procedure will proceed to further stages of single ventricle palliation. A second surgery, also known as the Glenn procedure, occurs at 4–6 months of age. The third surgery is the Fontan procedure, occurring when patients are 3–5 years of age. [5]
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In the latter case, prostaglandin E1 is used to maintain the PDA connection until emergency corrective surgery can be completed. As oxygenated blood is mixed with deoxygenated blood in both cases, there is a reduction in the oxygen-carrying capacity. [4] It is also possible for tricuspid atresia to appear without the life-saving defects.