The right ventricle performs the traditional job of the left, supplying the body with oxygenated blood, while the passive systemic venous pressure performs the traditional job of the right, passing deoxygenated blood to the lungs. Fetal surgery
Interventions performed during fetal development are under investigation. In fetuses with hypoplastic left ventricles and an intact interatrial septum, percutaneous atrial septostomy has been attempted. Prognosis
95% of untreated infants with HLHS die in the first weeks of life.Early survival has improved since the introduction of the Norwood procedure. Since there are no long-term studies of HLHS adults, statistics are usually derived from post-Fontan patients; it is estimated that 70% of HLHS patients may reach adulthood.Prognosis is dependent upon the health of the child, as there is an increased demand on respiratory and heart rate in infants during common childhood illnesses. This fragile population has little cardiac reserve to accommodate these demands and provide hemodynamic stability during illnesses.Children with HLHS and other comparable single-ventricle conditions, as a group, have poorer neurodevelopmental outcomes than their healthy peers. Deficits in language, executive functioning, and higher rates of anxiety and depression disorders have been demonstrated. Some of these outcomes may be a consequence of genetic factors associated with HLHS, and others may be modifiable through changes to procedures and to the healthcare environment. There is an emerging clinical consensus around the importance of continuous neurodevelopmental surveillance from the earliest years into adulthood.
Hypoplastic left heart syndrome (HLHS) is a rare congenital heart defect in which the left side of the heart is severely underdeveloped and incapable of supporting the systemic circulation. It is estimated to account for 2-3% of all congenital heart disease. Early signs and symptoms include poor feeding, cyanosis, and diminished pulse in the extremities. The etiology is believed to be multifactorial resulting from a combination of genetic mutations and defects resulting in altered blood flow in the heart.Several structures can be affected including the left ventricle, aorta, aortic valve, or mitral valve all resulting in decreased systemic blood flow.Diagnosis can occur prenatally via ultrasound or shortly after birth via echocardiography. Initial management is geared to maintaining patency of the ductus arteriosus - a connection between the pulmonary artery and the aorta that closes shortly after birth.Patient subsequently undergoes a three-stage palliative repair over the next few years of life. The Norwood procedure is typically done within a few days of birth. The Glenn procedure is typically performed at three to six months of age. Finally the Fontan procedure is done sometime between the age of two and five years of age. If left untreated patients with HLHS die within the first weeks of life while 70% of those that undergo three-staged palliative surgery reach adulthood. After surgery children with HLHS typically experience neurodevelopmental as well as motor delay and are at an increased risk of heart failure as adults.