Congenital Heart Disease Pulmonary Hypertension Essay

921 words - 4 pages

Pulmonary hypertension (PH) refers to elevated pulmonary artery pressure (PAP) and is commonly defined as mean PAP≥25mmHg. The presence of PH does not indicate any specific pathologic or physiologic cause. This distinction is underappreciated, with consequent confusion in both clinical practice and the medical literature.
Prognosis in PH is predicted by underlying cause, pathophysiology and the interaction between the right heart and pulmonary circulation. The degree of elevation in PAP itself is not the main cause of adverse effects of PH and lowering PAP is not the focus of therapy. This is in contrast to systemic arterial hypertension, where elevated pressure itself causes many subsequent ...view middle of the document...

3 It follows that appropriate management of PH-CHD is fundamentally dependent on understanding the cause and physiology of PH for a given patient. This manuscript will review approaches to clinical classification of PH-CHD followed by a discussion of therapeutic strategies for each class.

Classifications of PH-CHD
WHO Classification. The World Health Organization classification, which reflects an evolution from an initial framework in 1973 followed by important revisions in 1998, 2003, 2009, and 2013.3 The current classification includes 5 major groups: (1) PAH; (2) PH owing to left heart disease; (3) PH owing to lung diseases and/or hypoxia; (4) chronic thromboembolic pulmonary hypertension; and (5) PH with unclear multifactorial mechanisms. Until the 2013 update, CHD-PH was explicitly listed under Group 1, focusing on patients with PAH (elevated PVR) owing to CHD, generally patients with systemic-to-pulmonary shunt lesions. Four distinct phenotypes were outlined: Eisenmenger syndrome, PAH associated with systemic-to-pulmonary shunts, PAH with small defects, and PAH after corrective surgery. There was no mention of PH owing to CHD which does not fulfill criteria for PAH (PVR>3WU, normal left heart filling pressure). In large part this reflects a reasonable focus on grouping all forms of PH that are likely to share a similar pathology and would be expected to respond similarly to available and emerging pulmonary vasodilator medications into a single group. In addition, therapy for other and mixed PH physiology is less well studied, posing a challenge to developing guideline recommendations. The emphasis on high PVR CHD-PAH to the exclusion of other forms of CHD-PH, however, may misleadingly suggest that CHD-PH is predominantly comprised of CHD-PAH. This is not the case. As in the general population, left heart disease is...

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