Hypoplastic left heart syndrome (HLHS) refers to the abnormal development of the left-sided cardiac structures, resulting in obstruction to blood flow from the left ventricular outflow tract. In addition, the syndrome includes underdevelopment of the left ventricle, aorta, and aortic arch, as well as mitral atresia or stenosis. HLHS has been reported to occur in approximately 0.016 to 0.036% of all live births. Newborn infants with the condition generally are born at full term and initially appear healthy. As the arterial duct closes, the systemic perfusion becomes decreased, resulting in hypoxemia, acidosis, and shock. Usually, no heart murmur, or a non-specific heart murmur, may be detected. The second heart sound is loud and single because of aortic atresia. Often the liver is enlarged secondary to congestive heart failure. The embryologic cause of the disease, as in the case of most congenital cardiac defects, is not fully known. The most useful diagnostic modality is the echocardiogram. The syndrome can be diagnosed by fetal echocardiography between 18 and 22 weeks of gestation. Differential diagnosis includes other left-sided obstructive lesions where the systemic circulation is dependent on ductal flow (critical aortic s...Continue Reading
Cross sectional echocardiographic identification of hypoplastic left heart syndrome and differentiation from other causes of right ventricular overload
Congenital heart disease among 815,569 children born between 1980 and 1990 and their 15-year survival: a prospective Bohemia survival study
Mortality associated with congenital heart defects in the United States: trends and racial disparities, 1979-1997
Evolving strategies and improving outcomes of the modified norwood procedure: a 10-year single-institution experience
Outcome of staged reconstructive surgery for hypoplastic left heart syndrome following antenatal diagnosis
Choices physicians would make if they were the parents of a child with hypoplastic left heart syndrome
Right ventricle to pulmonary artery conduit improves outcome after stage I Norwood for hypoplastic left heart syndrome
Creation of an atrial septal defect in utero for fetuses with hypoplastic left heart syndrome and intact or highly restrictive atrial septum
Long-term follow-up after staged reconstruction or transplantation for patients with functionally univentricular heart
Achieving a balance in the current approach to the surgical treatment of hypoplastic left heart syndrome
Outcomes after the stage I reconstruction comparing the right ventricular to pulmonary artery conduit with the modified Blalock Taussig shunt
Submicroscopic chromosomal copy number variations identified in children with hypoplastic left heart syndrome
Fetal hypoplastic left heart syndrome and maternal liver transplantation for Wilson's disease: a case report
Anaesthetic management of a rare case of single ventricle heterotaxy syndrome for emergency caesarean section
Parents' perceptions during the transition to home for their child with a congenital heart defect: How can we support families of children with hypoplastic left heart syndrome?
X chromosome monosomy restricted to the left ventricle is not a major cause of isolated hypoplastic left heart
Hypoplastic Left Heart Syndrome Sequencing Reveals a Novel NOTCH1 Mutation in a Family with Single Ventricle Defects
The impact of prenatal counselling on mothers of surviving children with hypoplastic left heart syndrome: A qualitative interview study.
Pressure-volume loops: feasible for the evaluation of right ventricular function in an experimental model of acute pulmonary regurgitation?
Accuracy, agreement, and reliability of fetal cardiac measurements using 4-dimensional spatiotemporal image correlation
Outcome after prenatal and postnatal diagnosis of complex congenital heart defects and the influence of genetic anomalies
Fetal reprogramming and senescence in hypoplastic left heart syndrome and in human pluripotent stem cells during cardiac differentiation
Aortic coarctation is a congenital condition characterized by narrowing of the aorta. Discover the latest research on this disease here.
Birth defects encompass structural and functional alterations that occur during embryonic or fetal development and are present since birth. The cause may be genetic, environmental or unknown and can result in physical and/or mental impairment. Here is the latest research on birth defects.
Cardiac Conduction System
The cardiac conduction system is a specialized tract of myocardial cells responsible for maintaining normal cardiac rhythm. Discover the latest research on the cardiac conduction system here.