Hypoplastic Left Heart Syndrome

Paper Summary:

The paper discusses "hypoplastic left heart syndrome" which basically is a heterogeneous group of cardiac malformations consisting of various degrees of underdevelopment of the left heart and the aorta. This results is significant obstruction of blood flow to the systemic circulation, and difficulty in the left section of the heart supporting this circulation. The paper discusses the incidence, options and therapeutic solutions to the problem. The paper concludes that knowledge of the condition prior to birth means that babies who are to undergo surgery present in an optimal condition for these procedures.

Outline:
Introduction and Background
Hypoplastic Left Heart Syndrome
Pathophysiology
Therapeutic Options
Norwood Operation
Cardiac Replacement
Biventricular Repair
Conclusion

From the Paper:

"The Norwood operation to management of the hypoplastic left heart syndrome is a staged surgical palliation. The first stage, which is usually performed in the first week of life, consists of reconstruction of the aortic arch, using the main pulmonary artery as a single outlet from the right ventricle of the heart. (Norwood, Lang, Hansen, 23) The second stage of the Norwood protocol involves surgical anastamosis of the superior vena cava to the pulmonary arteries."

Sample of Sources Used:

• Bailey LL, Nehlsen-Cannarella SL, Doroshow RW. Cardiac allotransplantation in newborns as therapy for hypoplastic left heart syndrome. N Engl J Med 1986; 315: 949-951.
• Bellinger DC, Wypij D, duDuplessis AJ. Neurodevelopmental status at eight years in children with dextro-transposition of the great arteries: the Boston Circulatory Arrest Trial. J Thorac Cardiovasc Surg 2003; 126: 1385-1396.
• Dorman C. Microcephaly and intelligence. Dev Med Child Neurol 1991; 33: 267-269.
• Forbess JM, Visconti KJ. Neurodevelopmental outcome after congenital heart surgery: results from an institutional registry. Circulation 2002; 106 (12 Suppl 1): 95-102.
• Ivanovic DM, Leiva BP, Perez HT,. Head size and intelligence, learning, nutritional status and brain development. Head, IQ, learning, nutrition and brain. Neuropsychologia 2004; 42: 1118-1131.

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