Pulmonary hypertension in early life

摘要

Pulmonary hypertension is an uncommon disease that affects infants, children, adolescents, and adults. The disorders that lead to pulmonary hypertension have been defined by an increasingly exhaustive and widely accepted classification scheme, most recently published in 2009 under the auspices of WHO.1 Most of these disorders are associated with pulmonary arterial hypertension (PAH). PAH is a disease process that can present at any age with the gradual onset of either nonspecific symptoms, such as dyspnoea, or very pronounced symptoms such as frank right heart failure. Morbidity and mortality are substantial for children with PAH; in the early 1990s, median survival was 2-3 years.2 There are limited data about the burden of PAH in childhood, but some country-specific studies suggest that it is probably less common in childhood than in adulthood (<10% of adult incidence in the USA and Israel).34 Assessment and treatment of pulmonary hypertension has been progressively refined and expanded in national and international guidelines."