Seminars in respiratory and critical care medicine
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Idiopathic pulmonary arterial hypertension (IPAH), formerly called primary pulmonary hypertension, is a rare disease (incidence and prevalence rates of approximately one and six cases per million inhabitants, respectively) with different clinical phenotypes. A group of diverse conditions manifest pulmonary arterial hypertension (PAH) and share similar pathological and/or clinical findings with IPAH. By definition, IPAH is diagnosed only after alternative diagnoses have been ruled out. ⋯ However, even with aggressive PAH-specific therapy, mortality rates remain high (∼40% at 5 years). Given the high mortality rates, the use of combinations of agents that work by different pathways has been advocated (either as "add-on" therapy or initial "up front" therapy). Further, new therapeutic agents and treatment strategies are on the near horizon, aiming to further improve survival from the remarkable progress already seen.
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Despite advances in medical therapies, pulmonary arterial hypertension (PAH), continues to cause significant morbidity and mortality. Although, the right ventricle can adapt to an increase in afterload, progression of the pulmonary vasculopathy that characterizes PAH causes many patients to develop progressive right ventricular (RV) failure. ⋯ The poor reserve of the right ventricle, RV ischemia, and adverse RV influence on left ventricular filling may lead to a global reduction in oxygen delivery and multiorgan failure. The authors present an approach to patients with advanced PAH focusing on both medical and surgical strategies to improve RV function based upon current evidence and physiological principles.
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Semin Respir Crit Care Med · Oct 2013
Pulmonary hypertension complicating connective tissue disease.
Pulmonary hypertension (PH) may complicate connective tissue disease (CTD), particularly systemic sclerosis (SSc, scleroderma), and markedly increases mortality. More than 70% of cases of PH complicating CTD occur in SSc, which is the major focus of this article. ⋯ The role of PH-specific therapy is controversial, as prognosis and responsiveness to therapy are worse in SSc-associated PH compared with idiopathic pulmonary arterial hypertension. We discuss medical therapies for CTD-associated PH and the role of lung transplantation for patients failing medical therapy.
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Semin Respir Crit Care Med · Oct 2013
ReviewBirth control and pregnancy management in pulmonary hypertension.
Pregnancies in patients with pulmonary arterial hypertension (PAH) are associated with serious complications and mortality rates of more than 50%. Advanced medical therapies for PAH have been developed during the past years resulting in improved hemodynamics, exercise capacity, quality of life, and outcome. However, despite these advances, pregnancy in women with PAH is still associated with excessive maternal mortality. ⋯ If this fails, early pregnancy termination is advised. Those patients who choose to continue pregnancy should be treated with targeted PAH therapies including prostacyclin analogues and/or phosphodiesterase type 5 inhibitors. The care of the pregnant women with PAH requires a planned, multidisciplinary approach, preferably in a dedicated pulmonary hypertension referral center, focusing on close monitoring before, during and after delivery.