Pediatric pulmonary hypertension: diagnosis and management.

PURPOSE OF REVIEW

The article is intended to provide an overview of the most current modalities for the diagnosis and management of pulmonary hypertension in the pediatric population.

RECENT FINDINGS

The WHO has recently updated the classification for pulmonary hypertension to include an expanded range of pediatric disease cohorts. We continue to gain knowledge of traditional pulmonary hypertension therapies (phosphodiesterase inhibitors, endothelin-receptor antagonists, and prostanoids) and remain optimistic that new therapeutic modalities [cyclic guanosine monophosphate (cGMP) stimulators, oral prostacyclins, and gene therapy] will lead to improved outcomes in pediatric patients.

SUMMARY

Pediatric pulmonary hypertension is a heterogeneous disorder that has the potential to improve in some cases as children grow throughout childhood. Utilization of dedicated multidisciplinary teams of medical providers is necessary to deliver the highest level of medical care to this complex patient population. Ongoing development of enhanced screening protocols, novel disease-specific therapeutic targets, and comprehensive registries will hopefully lead to improved morbidity and mortality in the future.