Abstract

RIOS-MENDEZ, Raúl Enrique; MONTERO-MONAR, Hernán Eduardo; FERNANDEZ-ALVARADO, Antonio Pedro  and  HIDALGO-FLORES, Einstein Gustavo. Silver-Russell syndrome (hemihypertrophy) and cor triatriatum in a newborn. Arch. argent. pediatr. [online]. 2015, vol.113, n.3, pp.e140-e144. ISSN 0325-0075.  http://dx.doi.org/10.5546/aap.2015.e140.

Hemihypertrophy syndrome and cor triatriatum are extremely rare pathologies. Hemihypertrophy is defined as complete or partial overgrowth of one of the hemibodies. Cor triatriatum is a congenital heart disease characterized by a membrane which separates the left atrium into two chambers; if that membrane has a restrictive hole, it causes obstruction to blood passage from the pulmonary veins into the left ventricle causing hypertension and pulmonary edema. In this context, the patent ductus arteriosus can act as a means of decompression of the pulmonary circuit, because it allows the blood passage from the pulmonary artery to the aorta. We report a patient with Silver-Rusell syndrome (hemihypertrophy), cor triatriatum and ductus arteriosus with reverse flow. To our knowledge, this association of rare pathologies and this clinical presentation have not been described previously.

Keywords : Cor triatriatum; Ductus arteriosus; Pulmonary hypertension; Growth disorders; Silver-Russell syndrome.

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