Purpose of review: Pulmonary vascular disease (PVD) complicates the course of many cardiovascular, pulmonary and other systemic diseases in children. The physiological sequelae (pulmonary hypertension and elevated pulmonary vascular resistance) can overwhelm the right ventricle and lead to circulatory collapse. Despite the common end-point, the preceding pathophysiology is complex and variable and requires a tailored approach to diagnosis and management. In this article, we will review the most recent evidence and explore an approach to current controversies in the diagnosis and management of common or challenging patient subgroups.
Recent findings: New methods of interpreting data derived from echocardiography and cardiac magnetic resonance imaging may assist in risk stratification and response to therapy. In specific patient subgroups, standard pharmacological therapies to reduce right ventricle afterload may be overutilized, ineffective and in some cases harmful. In the patient failing pharmacological therapy, new and novel techniques are being explored including temporary extracorporeal mechanical circulatory support, pumpless lung assist devices and novel surgical and catheterization procedures.
Summary: PVD is a diverse entity, and attention to the underlying pathophysiology is essential for appropriate management. Despite significant advances in our understanding, the majority of data comes from small uncontrolled studies and must be interpreted with caution.