The Pulmonary Circulation in Congenital Heart Disease

Kulik, Thomas J.; Mullen, Mary P.

doi:10.1007/978-0-387-87429-6_77

Cited by 2 publications

(1 citation statement)

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“…[77][78][79] Histopathology corresponds only moderately well with hemodynamics, cannot inform our understanding of dynamic vasoconstriction, and does not reliably identify the potential for reverse remodeling in response to mechanical (eg, PA banding) or medical therapies. [78][79][80] Consequently, lung biopsy is rarely performed for this purpose in clinical practice.…”

Section: Histopathologic Assessment To Direct Management Of Ph-chdmentioning

confidence: 99%

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

Opotowsky

2015

Circulation

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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

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