Abstract

most paediatric cardiologists think that the optimal treatment for patients with functionally single ventricle is the relatively early construction, at from 2 to 7 years of age, of the total cavo-pulmonary circulation. this approach is based on the desire to produce an acyanotic child, such an outcome fostering the belief that the patient is cured. when using this therapeutic approach, the total connection is usually preceded by earlier creation of a partial connection, achieved by creating a bi-directional anastomosis between the superior caval vein and the right pulmonary artery at the age of from 3 to 9 months, without providing additional flow of blood to the lungs. this leads to severe cyanosis by the age of 3 years, when the flow increases in the inferior caval vein, and is the signal for early completion of the total connection. in this policy, the absence of additional flow to the lungs is justified by the fear of altering the ventricular function essential for the success of the total connection, because of the volume, and eventually the pressure, overload of the additional flow on the functionally single ventricle,which may impair its compliance and/or contractility, or be responsible for atrioventricular valvar regurgitation. these alterations in myocardial functions would then contraindicate, or increase, the mortality and morbidity of the total connection, producing deterioration in its long-term results.

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