Journal of Rare Cardiovascular Diseases

Pulmonary arterial hypertension (PAH) is a severe and progressive disease. Due to the narrowing of the blood vessels in the lungs, pul- monary vascular resistance and pulmonary pressures increase. This results in reduced cardiac output, right heart failure and ultimately in death. Pulmonary hypertension, particularly PAH, is an independent risk factor for both peri-operative complications and post-operative mortality. The peri-operative management of patients with PAH is particularly challenging and requires a multidisciplinary approach. Appropriate patient preparation requires an accurate assessment of the severity of pulmonary hypertension, comorbidities and the type of surgery to be performed. Additionally, patients with PAH are more likely to develop an infection, particularly of the respiratory system. Infections are important risk factors for disease exacerbation, often affecting prognosis. For this reason, there is a need for effective prophylactic, diagnostic and rapid therapeutic strategies in PAH patients admitted with suspected infection. While pregnancy is not con- sidered a disease, it is associated with a significant mortality and morbidity risk in patients with PAH and therefore it is contraindicated in this group. Thus, the proper education of patients and effective contraception are necessary in order to minimize health risks. If a woman decides to maintain her pregnancy, careful monitoring, specific treatment optimization and close co-operation with an obstetrician are needed. The proper assessment of the severity of PAH and the optimization of specific treatments are crucial to improve the prognosis of PAH patients in all high-risk conditions. Additionally, the early diagnosis of high-risk conditions allows for early intensive treatment or control, which should be performed at a PAH treatment referral centre. JRCD 2018; 3 (6): 194–198

rare disease; pulmonary arterial hypertension; surgery; infections; pregnancy

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