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.

Transthoracic Echocardiography in the Evaluation of Pediatric Pulmonary Hypertension and Ventricular Dysfunction

“…27 This enhances Doppler estimation of PAP and thus diagnosis of potential PAH, applying the accepted definition of pediatric PAH as mPAP > 25 mmHg with a pulmonary vascular resistance (PVR) index > 3.0 Wood units m 2 for biventricular circulations. 28 The mPAP and dPAP can be estimated, using CW Doppler of the PR jet in the PSAX view, by measurement of the peak and minimal diastolic velocities. Mean PAP and dPAP are not routinely used in the diagnosis and follow-up of pediatric PAH but may be useful when TR tracing is unreliable.…”

Registries for paediatric pulmonary hypertension

“…Patients, family members and healthcare providers should be aware of the unique features of paediatric pulmonary hypertensive vascular disease (PPHVD) [14,15]. These include the fetal origins, developmental and adaptive aspects of pulmonary vascular disease (PVD) and right ventricular dysfunction (RVD) in both childhood-and adult-onset disease.…”

“…Of note, the PAH disease spectrum may somewhat differ in the individual tertiary centre and in multicentre registries [13]. In a British cohort study, the 5-year survival for children with IPAH was only 75% with a freedom from death or transplantation of only 57% (1986 and 2000 with follow-up to 2007) [8].Patients, family members and healthcare providers should be aware of the unique features of paediatric pulmonary hypertensive vascular disease (PPHVD) [14,15]. These include the fetal origins, developmental and adaptive aspects of pulmonary vascular disease (PVD) and right ventricular dysfunction (RVD) in both childhood-and adult-onset disease.…”

Managing Children with Bronchopulmonary Dysplasia

“…ст. и индекса резистентности легочных сосудов > 3,0 единиц Вуда (WU) м 2 при двухжелудочковом кровообращении [16]. Проведение катетеризации сердца и теста на вазо-реактивность рекомендуется пациентам с БЛД в следую-щих клинических ситуациях: 1) при персистирующих признаках тяжелого кардиоре-спираторного заболевания, не относящихся напря-мую к заболеванию дыхательных путей; 2) вероятной или доказанной ЛГ, несмотря на оптималь-ное лечение заболевания легких, ЛГ и сопутствующих патологических состояний; 3) подозрении на «непропорциональную» ЛГ, характери-зующуюся одышкой, недостаточно объяснимой пора-жением легких, показателем срДЛА у 40-45 мм рт.…”