Hypoplastic Left Heart Syndrome
Despite approximately 50 years of progress in the management of com plex congenital heart disease, hypoplastic left heart syndrome (HLHS) has been one of the last cardiac malformations to yield to effective surgical treat ment. The surgical therapies fo
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Gil Wernovsky M.D. and Jack Rychik M.D. Children's Hospital of Philadelphia
"This procedure is not an anatomical correction, which would require the creation of a ventricle, but a procedure of physiological pulmonary blood flow restoration with suppression of right and left blood mixing." - F. Fontan and E. Baudet, Thorax, 1971
It is truly an exciting time for children with hypoplastic left heart syndrome and their families. As recently as 20 years ago this was a universally fatal disease. Even as recently as 10 years ago, surgical mortality was high, hospital stays were long and short-term morbidity commOn. With the improvements in surgery, perioperative care and a better understanding of the sequelae of the Fontan circulation, the focus of management has shifted from minimizing mortality to minimizing morbidity. There are certain realities that must be addressed. The Fontan circulation is inherently associated with chronic low-normal cardiac output. All patients will have some degree of limitation to exercise and aerobic endurance. We are hopeful that with further surgical modifications and changes in medical management, these limitations will be non-apparent in day-to-day activities and minimal at moderately stressful activities. In addition to the chronic low-normal cardiac output state, all patients with a Fontan circulation-even with the more recent surgical modifications-are at risk for dysrhythmia. Sinus node dysfunction and/or supraventricular tachycardia continues to be the "Achilles heel" of the surgical management of single ventricle. Chronotropic impairment may be a major contributor to the exercise limitations that all of these children have. Again, further research into surgical and medical therapy should improve this for the current generation of children with HLHS. Finally, it is increasingly recognized that having hypoplastic left heart syndrome results in an unavoidable period of hypoxemia, frequent hospitalizations and multiple surgeries in early childhood. The combined effects of these therapies on neurodevelopment is only nOW able to be recognized. Children with HLHS (and its variants) are at increased risk for abnormal school performance and learning disabilities. The recognition of this finding in the current schoolaged survivors has led to two major shifts in the focus of care. First is our recommendation that all children with complex congenital heart disease are
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Hypoplastic Left Heart Syndrome
"at risk", and should have formal developmental testing in the preschool and elementary school time frame. Secondly, the focus of research has shifted to neuroprotection trials and various modifications of cardiopulmonary bypass which will decrease the frequency oflong-term neurologic sequelae in these children. What can we expect in the future? Our overall understanding of the physiology, treatment and long-term sequelae of the hypoplastic left heart syndrome will continue to expand. As in many medical challenges, progress occurs in stages. The initial goals of creating survival for this
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