Hypoplastic left heart disease
hypoplastic left heart disease is a congenital heart malformation which
represents about 1% of all heart anomalies. It is the most common cause
of death for heart disease within the first month of life.
survival of these patients depends on the size of the ductus arteriosus
which permits the right ventricle to keep the right flux into the aorta
and on the presence of a wide interatrial defect which allows the decompression
of the left ventricle. The medical treatment consists of mechanical
ventilation and oxygen therapy, infusion of prostaglandin immediately
after birth, in order to maintain the ductus arteriosus open, and atrioseptostomy
to decompress the left atrium and improve oxygenation.
The Norwood procedure followed by the Fontan procedure
The best candidate for the last stage of the Norwood procedure must present the following hemodynamic characteristics: not restrictive interatrial communication, adequate tricuspid valve, a not obstructed anastomasis between the pulmonary aorta and descending aorta, low vascular resistances and adequate right ventricular function.
At present some authors report a rate of survival of 76% at the age of 1 month, 58% at the age of 12 months, 54% at the age of 4 years. Others report significantly lower data. At present there are still controversial theories about which procedure should be applied.
parents might choose a third alternative: let nature run its course.