Pulmonary Hypertension in Children – a Practical Approach
Eliza CINTEZA, Alin NICOLESCU, Cristina FILIP, Andrei LUPU, Georgiana NICOLAE, Gabriela DUICA and Mihaela BALGRADEAN
ABSTRACT
Pulmonary hypertension (PH) is mean pulmonary arterial pressure above 25 mmHg at rest. Although considered a rare disease, the prevalence of PH in certain risk groups is higher, from 0.5% in patients with HIV infection to 30% in congenital heart disease (CHD) associating PH. In pulmonary arterial hypertension (PAH) associated with CHD, early changes of the vascular bed are reversible after correction of the defect, but there is a point from where the disease becomes irreversible or progression continues despite correction. Among patients with “operable” and “inoperable” CHD, there is a “gray area” group in between, which is defined by pulmonary vascular resistance equal to 4-8 WU/m and ratio of pulmonary vascular resistance to systemic vascular resistance of 0.3-0.5, measured by cardiac catheterization. In this situation a pulmonary vasoreactivity test is indicated. Pulmonary hypertension is a severe disease with increased morbidity and mortality. Pulmonary hypertension can result in death by decreased cardiac output, and heart failure.
Keywords: pulmonary hypertension, congenital heart disease, pulmonary vasoreactivity, nitric oxide administration

