Clinical Evaluation and Management of Pulmonary Hypertension in the Adult With Congenital Heart Disease

Abstract

200P ulmonary hypertension (PH) refers to elevated pulmonary artery (PA) pressure (PAP), defined as mean PAP≥25 mm Hg. PH is not synonymous with pulmonary arterial hypertension (PAH), which specifies resistive PH characterized by elevated pulmonary vascular resistance (PVR) and normal pulmonary venous pressure. Approximately 5% to 10% of adults with congenital heart disease (CHD) have PH, and this subset of CHD patients is at higher risk of hospitalization and death. [1][2][3] Understanding the epidemiology of PH associated with CHD (PH-CHD) is not straightforward, however, because definitions of PH-CHD and diagnostic techniques vary. Appropriate diagnosis and the management of PH-CHD rest on identifying the cause of elevated PAP: high pulmonary venous pressure, high pulmonary vascular resistance, high pulmonary flow, or some combination of these factors, which are referred to throughout this review as passive, resistive, hyperkinetic, and polygenic PH, respectively (Table 1).Prognosis in PH-CHD depends on the underlying cause, pulmonary vascular pathophysiology, and the interaction between the right heart and pulmonary circulation. Adult CHD is heterogeneous, with myriad permutations of underlying defects, previous interventions, and associated comorbidities. Although comprehensive understanding of clinical context is a prerequisite, identifying the optimal therapy for a patient with PH-CHD depends more heavily on an understanding of pathophysiology than it does on the underlying diagnosis. Therefore, the goal of this review is to frame an understanding of PH-CHD in terms of fundamental cardiopulmonary hemodynamic pathophysiology and to emphasize the clinical assessment and management of situations commonly encountered by adult cardiologists caring for patients with PH-CHD. Classifications of PH and PH-CHDClassification schemes in PH represent clinical perspectives of underlying pathophysiology and tend to parallel fundamental hemodynamic relationships of the pulmonary circulation. PVR

Endothelial microparticles are increased in congenital heart diseases and contribute to endothelial dysfunction

“…Pulmonary hypertension is one major complications of congenital heart diseases [17, 18]. In the present study, we found that levels of EMPs were higher in patients with ASD and VSD accompanied by pulmonary hypertension than in those without pulmonary hypertension.…”

Pulmonary arterial hypertension in adults with systemic right ventricles referred for cardiac transplantation

“…The updated classification of PH acknowledges the challenges in categorizing PH in these patients, specifically stating that those with D‐TGA after atrial switch surgery are difficult to classify . CHD patients can have increased flow to the pulmonary bed from shunt lesions, high PVR, distorted pulmonary arterial vasculature, and increased pulmonary venous pressure, each of which contributes to varying degrees to pathology …”

The Oxidative Status of Children with Acyanotic Congenital Heart Diseases: A Randomized Controlled Study

“…The results of these clinical trials are not very promising, but a thorough identification of the temporal regulation of reactive oxygen species participating in the pathogenesis of congenital heart defects may help to achieve better clinical outcomes (18).…”