Protein‐losing enteropathy (PLE) is a complex disorder characterized by enteric protein loss
and often is associated with cardiovascular abnormalities, particularly those with elevated …

Pulmonary hypertension remained an unexplained intractable disease with poor prognosis.
In the last 2 decades, however, the diagnosis and treatment methods for this disease …

Data from the Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension
Disease Management (REVEAL) and the French registry estimate the prevalence of …

Aims An endothelin‐1 receptor blocker, shown to be effective in patients with pulmonary
arterial hypertension, might decrease pulmonary vascular resistance to increase cardiac …

Pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) is a
complex disease that presents with a broad spectrum of morphological and haemodynamic …

Objectives Diastolic dysfunction (DD), a key driver of long-term Fontan outcomes, may be
concealed during standard haemodynamic evaluation. We sought to identify Fontan patients …

Pulmonary hypertension (PH) refers to elevated pulmonary artery (PA) pressure (PAP),
defined as mean PAP≥ 25 mm Hg. PH is not synonymous with pulmonary arterial …

BACKGROUND: Endothelin-1 (ET-1), a potent vasoconstrictor, is considered to be
implicated in failing Fontan circulation, however the expressions of ET-1 and endothelin …

Objective: Determine sildenafil exposure and hemodynamic effect in children after Fontan
single-ventricle surgery. Design: Prospective dose-escalation trial. Setting: Single-center …

Objective. Adult patients with a Fontan circulation tend to have diminished exercise capacity.
The principal objective of this study was to investigate the safety of the endothelin receptor …