Pulmonary hypertension in children with chronic bronchopulmonary pathology: a pulmonologist’s perspective to the problem

The respiratory diseases are consistently ranked first in the morbidity pattern among children and adolescents in the Russian Federation. Registry data show that 10-12% of children with pulmonary hypertension have PH-associated chronic lung pathology. Pulmonary hypertension as a life-threatening complication of such chronic lung diseases as hypersensitive pneumonitis, bronchiectasis, bronchial asthma, etc. aggravates the severity of their clinical course and has a great impact on the outcome of the disease. The article summarizes modern data on pulmonary hypertension in children of various ages associated with chronic bronchopulmonary pathology. The issues of classification, pathogenetic mechanisms, criteria and modern methods of diagnosis and treatment, as well as prognosis are considered. The authors come to the conclusion that despite the in-depth study of the pathogenetic aspects of the development of pulmonary hypertension in chronic bronchopulmonary pathology in children, which has recently made it possible to shed light on the understanding of many of its aspects, it should be admitted that the problem as a whole is far from being resolved. Early detection and treatment of pulmonary hemodynamic pathology is extremely important to prevent the formation of cor pulmonale. Pulmonary hypertension, which complicates the chronic lung diseases, is still incurable and is associated with high mortality. Now, young patients with pulmonary hypertension are still often examined and treated using guidelines for adult patients. However, differences in etiology, clinical presentations and diagnostic capabilities require a special approach to studying this problem in children, which raises the question of the need to develop separate clinical guidelines for pediatric practice.