Progress in the diagnosis and management of pulmonary hypertension in children.

purpose of reviewPulmonary hypertension is a complex disease that extends beyond merely elevated pulmonary blood pressures and right ventricular dysfunction. Its multiple causes and ever-expanding diagnostic tools and therapeutic approaches make it a heterogeneous disease with widely variable clinical sequelae. There are still many unanswered questions that challenge our understanding of this disease. RECENT

findingsThe study of pulmonary hypertension in the pediatric patient is as robust as ever, with the creation and inclusion of pediatric-specific disease characteristics in the most recent WHO classification system, improved understanding of the pathophysiology of pulmonary hypertension in pediatric diseases such as bronchopulmonary dysplasia, and increasingly expanding diagnostic tools and management possibilities. Although the use of pulmonary hypertension therapies in children previously often relied on expert opinion and inferences from studies involving adults, pediatric-targeted research is becoming more widely supported and pursued, and has even come under recent debate, which at the very least stimulates further collaboration and discussion. SUMMARY: This review will highlight the changes in the pulmonary hypertension classification system, briefly explore pulmonary hypertension in bronchopulmonary dysplasia, and provide updates on the diagnostic and management tools used by experts in the field.