A consensus approach to the classification of pediatric pulmonary hypertensive vascular disease: Report from the PVRI Pediatric Taskforce, Panama 2011

Abstract

Current classifications of pulmonary hypertension have contributed a great deal to our understanding of pulmonary vascular disease, facilitated drug trials, and improved our understanding of congenital heart disease in adult survivors. However, these classifications are not applicable readily to pediatric disease. The classification system that we propose is based firmly in clinical practice. The specific aims of this new system are to improve diagnostic strategies, to promote appropriate clinical investigation, to improve our understanding of disease pathogenesis, physiology and epidemiology, and to guide the development of human disease models in laboratory and animal studies. It should be also an educational resource. We emphasize the concepts of perinatal maladaptation, maldevelopment and pulmonary hypoplasia as causative factors in pediatric pulmonary hypertension. We highlight the importance of genetic, chromosomal and multiple congenital malformation syndromes in the presentation of pediatric pulmonary hypertension. We divide pediatric pulmonary hypertensive vascular disease into 10 broad categories.

Figure 1

Venn diagram illustrating the heterogeneity and multifactorial elements in pediatric pulmonary hypertensive vascular disease.

Figure 2

This figure illustrates the complexity of pulmonary hypertensive vascular disease in a two-year-old infant with bronchopulmonary dysplasia. (a) chest X ray, cardiomegaly and parenchymal lung infiltrates; (b) lung CT scan, showing lung extensive parenchymal damage with areas of atelectasis and emphysema; (c) CT angiogram, showing right ventricular and right atrial dilatation, and atrial septal defect; (d) CT angiogram showing left and right upper pulmonary vein stenosis; (e) reconstructed CT image showing persistent ductus arteriosus; and (f) CT angiogram showing the severe stenosis of right upper pulmonary vein.