Pediatric Pulmonary Hypertension

Abstract

Pulmonary hypertension (PH) is a rare disease in newborns, infants, and children that is associated with significant morbidity and mortality. In the majority of pediatric patients, PH is idiopathic or associated with congenital heart disease and rarely is associated with other conditions such as connective tissue or thromboembolic disease. Incidence data from the Netherlands has revealed an annual incidence and point prevalence of 0.7 and 4.4 for idiopathic pulmonary arterial hypertension and 2.2 and 15.6 for pulmonary arterial hypertension, respectively, associated with congenital heart disease (CHD) cases per million children. The updated Nice classification for PH has been enhanced to include a greater depth of CHD and emphasizes persistent PH of the newborn and developmental lung diseases, such as bronchopulmonary dysplasia and congenital diaphragmatic hernia. The management of pediatric PH remains challenging because treatment decisions continue to depend largely on results from evidence-based adult studies and the clinical experience of pediatric experts.

Pulmonary Arterial Hypertension Associated With Tetralogy of Fallot

“…These subclasses were recently modified by the Pediatric Task Force of the 5th World Symposium on Pulmonary Hypertension in Nice using the following terms: 1) Eisenmenger syndrome, 2) leftto-right shunts, operable or inoperable; 3) PAH with co-incidental CHD; and 4) postoperative PAH. 12) However, the anatomical and hemodynamic features of PAH vary considerably among patients, especially in patients with PAH occurring after surgical treatment of CHD. Therefore, it is difficult to establish clinical support for the use of pulmonary vasodilators for all conditions, even though many types of pulmonary vasodilators have been used to treat postoperative PAH-CHD.…”

Pulmonary hypertension in children

“…4,10 The Nice classification is appropriate for adults and children. 4,11 In younger children, the pulmonary arterial pressure is frequently referenced as a ratio to systemic arterial pressure with a significant difference being greater than 0.5.…”

“…4,10 The Nice classification is appropriate for adults and children. 4,11 In younger children, the pulmonary arterial pressure is frequently referenced as a ratio to systemic arterial pressure with a significant difference being greater than 0.5. Pulmonary hypertensive vascular disease complicates the course of certain forms of single ventricle heart disease in which mean PAP is less than 25 mmHg but pulmonary vascular resistance (PVR) is high leading to failure of the circulation.…”

“…2 Recent advances in the understanding of the pathobiology of idiopathic pulmonary arterial hypertension and new treatment therapies have resulted in marked improvement in the prognosis for children with PAH ( Figure 1). 3,4 Similarities and differences persist in comparison of children and adults with PAH. 5 In both groups, disease progression is rapid, perhaps more rapid in children than in adults, and left untreated elevation of pulmonary arterial pressure and resistance leads to right ventricular failure, clinical deterioration and death.…”

Hospitalizations of Children With Pulmonary Hypertension: Implications for Improving Care

“…Despite the growing availability of PH-specific drug therapies, the outcomes for children with severe PH generally remain poor, and the lack of effective treatments further contributes to significant morbidities and diminishes the quality of life. 1,2 Overall, pediatric PH has been understudied, and little is known about its natural history, longitudinal course, optimal diagnostic approaches, or treatment. In addition, we currently have limited understanding of disease-specific mechanisms in pediatric PH; there is a lack of organized, multidisciplinary care programs; and few markers or end points are available for assessing clinical course and response to interventions in children.…”