Abstract :
Hypoplastic left heart syndrome (HLHS) comprises 2 to 3 percent of all congenital heart disease. HLHS is uniformly lethal if
left untreated; however, there is currently an estimated 70 percent 5-year survival rate for infants who undergo surgical
correction. The hypoplastic left heart syndrome encompasses a spectrum of cardiac malformations that are characterized
by significant underdevelopment of the components of the left heart and the aorta, including the left ventricular cavity and
mass. Its main anatomical characteristics include mitral stenosis or atresia, aortic stenosis or atresia, and hypoplasia of left
ventricle. The apex of the heart is usually formed by the right ventricle. The right ventricle is the single functional ventricle
maintaining fetal circulation in these patients.After births, due to decreased pulmonary vascular resistance and closure of
the ductus arteriosus, infants with HLHS develop a constellation of symptoms that include cyanosis, dyspnea, heart failure,
hypoxemia and acidosis. Surgery is the only effective treatment. Unfortunately, even when treated, this disease is
associated with significant morbidity including an increased risk of thrombotic complications, decrease in exercise
tolerance, and neuro-developmental impairment. Continuous intravenous prostaglandin E1 is used to maintain a PDA to
allow adequate systemic perfusion. Nitrogen may be used in some centers to decrease inspired oxygen which increases
pulmonary arterial resistance. This therapy encourages systemic rather than pulmonary blood flow and enhances systemic
perfusion. Double outlet right ventricle (DORV) is a type of ventriculoarterial connection in which both the aorta and
pulmonary artery arise entirely or predominantly from the right ventricle (RV). The only outlet from the left ventricle (LV) is a
ventricular septal defect (VSD). DORV is usually associated with concordant atrioventricular connections with the right
atrium draining into the RV and the left atrium draining into the LV. DORV is almost always associated with a VSD and
occasionally with an atrial septal defect. Transposition of great arteries is characterized by both the outflows being parallel
to each other at the origin. On color Doppler, they show similar color flows, indicating that the direction of flow in both the
great vessels is same. Most atrial septal defects involve either the septum primum or septum secundum. Primum atrial
septal defect is the simplest form of the atrioventricular septal defect. Secundum atrial septal defect which is the
commonest are usually isolated septal defects, but may be related to other cardiac anomalies like mitral, tricuspid,
pulmonary or aortic atresia and are occasionally found as part of syndromes like Holt-Oram syndrome.
Keyword :
HLHS, congenital, cardiac, prostaglandin, prenatal diagnosis, DORV, TGA, ASD.