DEFINITION, CLINICAL PICTURE AND PATHOGENESIS
Five decades ago, Chinese investigators described in humans the counterpart of cattle brisket disease, with the name of High Altitude Heart Disease (HAHD) of the pediatric type . This entity is mainly observed in infants of Chinese Han origin who are born at low altitude and then brought to high altitude where they develop PH and HF within a few weeks or months with a fatal outcome if the infants are not moved down to lower places. Pediatric HAHD is also observed in children from 2 to 14 years of age but the prevalence is lower than in infants. Prevalence of pediatric HAHD is higher in Han infants than in Tibetan infants . The mechanism of SMS is ascribed to exaggerated hypoxic pulmonary reactivity of distal pulmonary arterial branches, which are muscularized in excess.
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