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Archivos de Pediatría del Uruguay

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Abstract

TAMBASCO 1, Jorge et al. Manejo médico-quirúrgico de la hipoplasia  del corazón izquierdo: Primera experiencia en Uruguay. Arch. Pediatr. Urug. [online]. 2006, vol.77, n.2, pp. 134-142. ISSN 0004-0584.

Summary Hypoplastic Left Heart Syndrome (HLHS) is a complex congenital heart malformation that represents 7% of all congenital heart defects. Without any palliative surgical treatment, 95% die in the first month of life. The evolution of three clinical cases is presented. The cases reported are newborns with ductus dependent heart disease that required prostaglandin infusion; one patient associated severe aortic coarctation and another patient type A aortic arch interruption. A modified Norwood procedure was performed in one case and a Sano procedure in the other two, together with surgical correction of associated damage. Follow-up showed evidence of increasing hypoxemia. The first patient had fistula permeability with hypoplastic pulmonary branch stenosis and deficient contractility of the systemic ventricle. A Glenn procedure was succesfully performed. In the second patient, the existence of proximal and distal stenosis of the tube between the right ventricle and pulmonary artery was demonstrated, so a balloon catheter angioplasty followed by Glenn surgery was performed, with good evolution at the beginning but progressive hypoxemia. Coil embolism was performed after noticing aortopulmonary collaterals linked to the right lung, with a good result, saturation levels over 85%, good single ventricle function and weight recovery. In the third patient, prosthetic tube proximal stenosis was confirmed which lead to balloon angioplasty and later to Glenn surgery due to hypoxemia, with good result. Conclusions: the effort of a multidisciplinary team made possible these patients survival who suffered a complex and lethal congenital heart defect, until the second stage of the Norwood procedure could be performed

Keywords : HYPOPLASTIC LEFT HEART SYNDROME-surgery;  HEART BYPASS, RIGHT;   CARDIAC SURGICAL PROCEDURES;   HEART DEFECTS, CONGENITAL.

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