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New Era in Management of Hypoplastic Left Heart Syndrome

Japan

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Hypoplastic left heart syndrome (HLHS) represents an anatomical spectrum of congenital hearts defects with varying degrees of underdevelopment of the left-sided heart structures.¹ Before 1980, the condition was practically always fatal, usually in the first month of life.² Since Norwood and colleagues³ reported successful surgical palliation (the Norwood procedure) in 1981, and multistaged reconstructive surgery involving the Norwood procedure (stage I) followed by bidirectional Glenn (stage II) or hemi-Fontan (stage II) and Fontan operations (stage III), the outlook for patients with HLHS has dramatically improved. The Norwood procedure must provide unobstructed systemic blood flow from the right ventricle (RV), relief of obstruction to pulmonary venous return, sufficient blood flow to the lungs in the absence of significant volume overload, and sufficient coronary circulation. Balancing pulmonary and systemic flows is critical to success. Despite successful reconstructive surgery, most deaths occur in the first 24 to 48 h postoperatively due to hemodynamic instability secondary to the unpredictable rapid fall in pulmonary vascular resistance.⁴ Maldistribution of cardiac output associated with the systemic-to-pulmonary shunt has been implicated as a major cause of early death after stage I palliation.^5,6 A review of 122 postmortem cases after the Norwood procedure at the Children’s Hospital, Boston, indicated that the most prevalent causes of death were impairment of coronary perfusion, excessive pulmonary blood flow, and obstruction to pulmonary blood flow.⁷ This maldistribution of blood flow was implicated in more than 60% of postoperative deaths.

Therefore, over the last decade, . . . [Full Text of this Article]