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Congenital Defects Tutorial
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Hypoplastic Left Heart Syndrome Aortic Stenosis Single Ventricle

Single Ventricle

  • Single ventricle is characterized by the presence of two separate atrioventricular valves and either one ventricular chamber or a dominant ventricular chamber with diminutive opposing ventricle.
  • The most common form of this defect is a single left ventricle with L-transposition of the great vessels. The aorta arises from a diminutive right ventricle that lies toward the left while the pulmonary artery arises more posteriorly .The tricuspid valve is found on the left side and mitral valve on the right.
  • The clinical course of infants with a single ventricle is determined by the amount of pulmonary blood flow limitation due to pulmonary stenosis or pulmonary vascular resistance. Without pulmonary stenosis, blood flow gradually increases to cause congestive heart failure, often within the first few days of birth. In the absence of stenosis, pulmonary vascular resistance cannot be reduced to normal levels and is ultimately incompatible with life. Infants with pulmonary stenosis show variable cyanosis at birth depending on the amount of blood flow via the ductus arteriosus, bronchial circulation, or aortopulmonary collaterals. In 80% of patients, blood from both the systemic and pulmonary circuits mixes in the single ventricle; thus, outflow through the aorta and pulmonary artery is of the same oxygen saturation. All patients with a single ventricle are cyanotic to some degree at birth.
  • Initial management of the single ventricle defect focuses on lifesaving measures including administration of prostaglandins, placement of shunt, and/or catheterization. The ultimate goal is to perform a surgical Fontan procedure which establishes systemic circulation carrying fully saturated blood.