Pulmonary vascular disease as a complication of pediatric congenital heart diseases

Abstract

Congenital and acquired heart diseases can cause pulmonary hypertension (PH) in children, either by increasing pulmonary blood flow (PBF), left atrial pressure (LAp), and/or pulmonary vascular resistance (PVR). Pathophysiological process of pulmonary vascular disease (PVD) in different types of congenital heart diseases (CHDs) are reviewed hereafter. As with other types of PH, a rigorous diagnostic evaluation is mandatory to characterize the etiology of the PH, rule out other or additional causes of PH, and establish a risk profile. Cardiac catheterization remains the gold standard exam for PH diagnosis. Treatment of pulmonary arterial hypertension (PAH) associated with CHD (PAH-CHD) can then be started according to the recent guidelines recommendations, although most of the evidence is extrapolated from studies on other causes of PAH. PH in pediatric heart disease is often multifactorial, and sometimes unclassifiable, making the management of these patients complicated. The operability of patients with a prevalent left-to-right shunt and increase of PVR, the management of children with PH associated with left-sided heart disease, the challenges of pulmonary vascular disorders in children with univentricular heart physiology and the role of vasodilator therapy in failing Fontan patients are some of the hot topics discussed in this review.

Current Management and Future Directions for Pulmonary Arterial Hypertension Associated with Congenital Heart Disease

“…Patients with unrepaired, moderate to large, systemic-to-pulmonary shunts and mildly to moderately elevated PVR constitute the second group of PAH-CHD. These patients are in an earlier stage pathophysiologically compared ES but require more complex management strategies due to a lack of evidence and their limited inclusion in clinical trials [44,45]. The decision to close the shunt or to use medical therapy depends on several factors, including the severity of PAH, increased PVR, and the location and size of the defect [46].…”

“…Patients with large post-tricuspid shunts (ventricular septal defect [VSD], patent ductus arteriosus [PDA]) usually develop pulmonary vascular disease in early childhood, which makes shunt closure inadvisable [45]. Patients with PAH and pre-tricuspid shunts (atrial septal defect [ASD]), however, are more challenging to manage because of the controversy in guidelines over shunt closure versus PAH-specific therapy [45,47]. This group usually requires additional testing to determine their operability [7,48].…”

“…Although these strategies may offer a cure for some patients, they are risky and require careful selection of patients and close monitoring of PVR. In addition, they lack the support of longterm data, and more research is needed to investigate their outcomes and risks [7,45,54,55].…”