Practice patterns of pulmonary hypertension secondary to left heart disease among pediatric pulmonary hypertension providers

Abstract

Development of pulmonary hypertension (PH) in patients with left side heart disease (LHD) is a predictor of poor prognosis. The use of pulmonary vasodilators in PH associated with LHD (PH-LHD) is controversial. In this study, we describe the practice patterns regarding the use of pulmonary vasodilators in PH-LHD among a group of international pediatric PH specialists. A survey was distributed to the members of three pediatric PH networks: PPHNet, PVRI, and REHIPED. The survey queried participants on the rationale, indications, and contraindications of the use of pulmonary vasodilators in children with PH-LHD. Forty-seven PH specialists from 39 PH centers completed the survey. Participants included PH specialists from North America (57%), South America (15%), and Europe (19%). The majority of participants (74%) recommended the use of pulmonary vasodilators only in patients with combined pre-capillary and post-capillary pulmonary hypertension. Participants required the presence of clinical symptoms or signs of heart failure (68%) or right ventricular dysfunction by echocardiography (51%) in order to recommend pulmonary vasodilator therapy. There was no agreement regarding hemodynamic criteria used to recommend pulmonary vasodilators or the etiologies of LHD considered contraindications for using pulmonary vasodilators to manage PH-LHD. Of the available PH-targeted drugs, most participants preferred the use of phosphodiesterase-5-inhibitors for this indication. In conclusion, the practice of recommending pulmonary vasodilators in PH-LHD is highly variable among international pediatric PH specialists. Most specialists of those surveyed (57% in North America) would consider the use of pulmonary vasodilators in PH-LHD only if pre-capillary pulmonary hypertension and right ventricular dysfunction are present.

International practice heterogeneity in pre‐transplant management of pulmonary hypertension related to pediatric left heart disease

“…Our results mirror a recent survey of management of PH in children with left heart disease showing heterogeneity with regard to use of pulmonary vasodilator therapies, with sildenafil being the most commonly used agent, followed by oxygen and iNO. 21 The free text comments from respondents regarding use of pulmonary vasodilators illustrate some strong opinions regarding whether vasodilators should be used and highlight the variability in individual experience and practice.…”

Pulmonary vascular disease as a complication of pediatric congenital heart diseases

“…Most of the adults in group 2 have some degree of heart failure with preserved or reduced ejection fraction or valvar heart disease. If some pediatric patients also exhibit similar acquired LHD, especially patients with rheumatic heart diseases or cardiomyopathies, extrapolation from adult studies may not be applicable in children born with left sided heart disease since the disease is present in utero and can affect pulmonary vascular development ( 46 ).…”