Pp. 124-138 (15)
Pelin Uysal and Hafize Uzun
Pulmonary hypertension (PH) is a hemodynamic and pathophysiological
condition defined as right pulmonary artery pressure (PAP) determined by right heart
catheterizatition (RHC) at rest, at 25 mm Hg or higher. RHC and vasoreactivity test are
the gold standard methods for diagnosis, treatment and prognosis follow-up of PH.
Each group has different physiopathological features. Three main features that outstand
in pulmonary artery are vasoconstriction, remodeling of arterial wall and in situ
thrombosis. A wide variety of biomarkers have been explored, although there is no
specific marker for PH. Patients were stratified according to pathophysiological,
hemodynamical features, clinical pictures and form of treatment. Differential diagnosis
of PH might be difficult because of its non-specific symptoms and it might be caused
by many different disorders. Exertional dyspnea disproportional with underlying cause
should be warning. Findings on physical examination are related with underlying
disease. Treatment of the underlying disease is important. In patients with positive
vasoreactivity test might benefit from calcium channel blockers. In patients with poor
prognosis, treatment with a combination protocol involves intravenous (IV) treatment.
Many combination treatments are used or developing at the present time. PAH is rare
and often diagnosed late. Novel circulating biomarkers could contribute to the
screening of PH. Different biomarkers may lead to different relevant information in PH
patients, including disease progression, response to medical and surgical therapy, and
prognosis. This chapter presents an update on alterations in the diagnostic algorithm,
haemodynamic definitions, biomarkers, treatment and prognose in PH. A
multiparametric approach is usually preferred because PH is more of a systemic
condition than an isolated cardiorespiratory illness.
Pulmonary hypertension, Walking test, Artery pressure, Pulmonary
artery, Hypoxia, Vasoconstriction, Remodeling, Arterial wall, Thrombosis,
Catheterization, Diagnostic algorithm, Haemodynamic definitions, Venoocclusive
disease, Treatment, Prognose, Biomarkers, Serotonin, Endotelin-1,
Thromboxane, Natriuretic peptides.
Department of Medical Biochemistry, Cerrahpaşa Faculty of Medicine, Istanbul University- Cerrahpasa, Istanbul, Turkey.