Right ventricular dysfunction in children and adults with congenital heart disease: the paediatric view

The assessment of right ventricular function remains difficult. No simple reliable technique is available to evaluate the right ventricular volume dynamically because of the geometry of the cavity. This makes difficult the objective assessment of any treatment modalities. Chronic right ventricular dysfunction is rarely present in the paediatric age and it usually follows longstanding right ventricular overload (atrial septal defects, pulmonary insufficiency) or is the consequence of the right ventricle working as a systemic ventricle. However, right ventricular dysfunction is one of the major causes of early morbidity and mortality after congenital heart disease surgery in paediatrics. Early recognition and treatment of the potential causes is essential to improve the prognosis of acute right ventricular failure. The main cause of acute postoperative right ventricular dysfunction is increased afterload either because of residual obstruction to pulmonary blood flow or increased pulmonary vascular resistance. Prompt recognition of residual anatomic obstruction should lead to surgical correction or interventional catheterization. The introduction of inhaled nitric oxide has partially changed the outcome of postoperative pulmonary hypertension. This should be associated with optimised loading of the right ventricle, inotropic support and adequate ventilatory support. Finally new mechanical support devices adapted to the paediatric patient may offer a potential bridge to recovery or transplant for very severe right ventricular failure.