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Published online by Cambridge University Press: 21 September 2005
The neonate with hypoplastic left heart syndrome presents a challenge for clinical diagnosis and management. Three diagnostic goals must be met. First, it is necessary to make an etiologic cardiac diagnosis so as to rule out any genetic abnormality. Second, the anatomic cardiac diagnosis is made by segmental echocardiographic analysis, including details of the atrial arrangement, venous return, the patency of the arterial duct, atrial anatomy, and the arrangement of the aortic arch. Finally, the physiologic cardiac diagnosis is made by Doppler evaluation. In some patients, the diagnosis of hypoplastic left heart syndrome is not synonymous with functionally univentricular physiology, and a bi-ventricular repair can be achieved.1