Sedating the child with congenital heart disease
The European Paediatric Pulmonary Vascular Disease Network, endorsed by ISHLT and DGPKPulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) is a complex disease that presents with a broad spectrum of morphological and haemodynamic findings of varying severity.
Recently, the aspect of paediatric pulmonary hypertensive vascular disease (PPHVD) has been introduced to expand the understanding of the full spectrum of pulmonary hypertension and increased pulmonary vascular resistance.
PH associated with left heart congestive diseases can now be found in class 2.4 ‘Congenital/acquired left heart inflow/outflow tract obstruction and congenital cardiomyopathies’.
The single-ventricle dysfunction, chronically elevated central venous pressure, elevated PVR (for passive pulmonary blood flow) or obstructions in the venous or arterial pathway may cause heart failure or specific complications such as plastic bronchitis or protein-losing enteropathy.
If the post-tricuspid defects are large enough, the pulmonary pressure will increase to systemic blood pressure level (pressure-unrestrictive ventricular septal defects (VSDs)).