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.

Keywords: WHO group II pulmonary hypertension; children; pulmonary vascular disease; survey.

Fig. 1.

Participants’ views regarding the use of pulmonary vasodilators in children with PH-LHD. (a) Pie chart depicting percentage of participants recommending pulmonary vasodilators. (b) Bar chart depicting the different rationale for recommending pulmonary vasodilators.

Fig. 2.

Assessments used by participants in children with PH-LHD to evaluate their candidacy for pulmonary vasodilators. (a) Pie chart depicting minimal assessments required by participants to recommend pulmonary vasodilators. (b) Hemodynamic criteria used to evaluate the candidacy for pulmonary vasodilators. (c) Echocardiographic criteria used to evaluate the candidacy for pulmonary vasodilators. (d) Clinical criteria used to evaluate the candidacy for pulmonary vasodilators. Cath: cardiac catheterization; DPG: diastolic pulmonary gradient; Echo: echocardiography; PAP: pulmonary artery pressure; PVRi: pulmonary vascular resistance indexed to body surface area; RAP: right atrial pressure; Rt: right; RVFxn: right ventricular function; TPG: mean transpulmonary gradient.

Fig. 3.

Bar chart depicting etiologies of left-sided heart disease in which participants considered pulmonary vasodilators were contraindicated.