This chapter explores the epidemiology, clinical presentation, and evaluation of patients with suspected pulmonary hypertension (PH). Right heart catheterization remains the gold standard for diagnosing PH. Accurate assessment of hemodynamics, in particular the pulmonary capillary wedge pressure, is critical to accurately make the diagnosis and to differentiate pre- from postcapillary forms of PH. All patients with pulmonary arterial hypertension (PAH) should also undergo pulmonary vasoreactivity testing on initial catheterization, most commonly performed with inhaled nitric oxide. It is also reasonable to perform a full oxygen saturation run in this population to rule out congenital heart disease as an etiology of PAH. The chapter concludes with a brief discussion on advanced therapies for the treatment of pulmonary arterial hypertension and several illustrative case examples.
KeywordsPulmonary hypertension Pulmonary arterial hypertension Pulmonary vascular resistance Vasoreactivity Congenital heart disease
- 1.Galie N, Torbicki A, Barst R, Dartevelle P, Haworth S, Higenbottam T, et al. Guidelines on diagnosis and treatment of pulmonary arterial hypertension. The task force on diagnosis and treatment of pulmonary arterial hypertension of the European Society of Cardiology. Eur Heart J. 2004;25(24):2243–78.CrossRefGoogle Scholar
- 4.Galie N, Humbert M, Vachiery JL, Gibbs S, Lang I, Torbicki A, et al. 2015 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension: the joint task force for the diagnosis and treatment of pulmonary hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Heart J. 2016;37(1):67–119.CrossRefGoogle Scholar
- 15.Benza RL, Miller DP, Gomberg-Maitland M, Frantz RP, Foreman AJ, Coffey CS, et al. Predicting survival in pulmonary arterial hypertension: insights from the registry to evaluate early and long-term pulmonary arterial hypertension disease management (REVEAL). Circulation. 2010;122(2):164–72.CrossRefGoogle Scholar
- 37.Chen SL, Zhang H, Xie DJ, Zhang J, Zhou L, Rothman AM, et al. Hemodynamic, functional, and clinical responses to pulmonary artery denervation in patients with pulmonary arterial hypertension of different causes: phase II results from the pulmonary artery denervation-1 study. Circ Cardiovasc Interv. 2015;8(11):e002837.CrossRefGoogle Scholar