Clinical guideline for treatment of pulmonary arterial hypertension (PAH) has been changing during the past 10 years with development of targeted therapy for PAH. Treatment of PAH should include general measures and supportive care, treatment of associated disease, targeted treatment for PAH, and lung transplantation in advanced cases. Targeted therapy for PAH can improve symptom, quality of life and time to clinical worsening in PAH patients. Regular follow-up to evaluate the treatment efficacy is essential and insufficient treatment efficacy should be modified with other class of medication or preparing lung transplantations. Research for new medication is actively performed and novel drug is expected to be improving survival of PAH patients.
Humans ; Hypertension, Pulmonary ; therapy

Humans ; Child ; Hypertension, Pulmonary/therapy*

This short review illustrates current epidemiology, diagnosis and treatment of pulmonary hypertension. In the first part, classification, definition and prevalence of pulmonary hypertension (PH) are explained. According to recent reports, overall PH prevalence was 0.3% to 6.0% with left heart disease occupying the most proportion, followed by pulmonary disease, pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) constituting far less proportion of 10 to 50 per 1 million people. In diagnosis, flow of diagnosis of PH, differential diagnosis of PH and how to determine the severity of PH are explained including recent development of magnetic resonance imaging (MRI) and gene abnormality study on BMPRII. In treatment, newly-developed pulmonary vasodilators are shown as well as how to combine them in PAH, and in CTEPH treatment the drugs, operation and catheter therapy including our experience are demonstrated.
Humans ; Hypertension, Pulmonary ; diagnosis ; therapy

Humans ; Hypertension, Pulmonary ; diagnosis ; therapy

Familial Primary Pulmonary Hypertension ; Humans ; Hypertension, Pulmonary ; therapy

OBJECTIVETo investigate the current practice of pulmonary hypertension including current epidemiology, diagnosis and treatment.

DATA SOURCESThe review was based on data obtained from the published articles and guidelines.

STUDY SELECTIONArticles with high level of evidence or current best evidence in each issue were selected to be reviewed.

RESULTSOverall prevalence of pulmonary hypertension was 0.3% to 6% with left heart disease occupying the most proportion, followed by pulmonary disease, pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension. In diagnosis, a flow diagram of diagnosis of pulmonary hypertension, differential diagnosis of pulmonary hypertension and how to determine the severity of pulmonary hypertension are explained including recent development of magnetic resonance imaging and gene abnormality study on bone morphogenetic protein receptor II. In treatment, newly-developed pulmonary vasodilators and the way to use them are shown to treat pulmonary hypertension.

CONCLUSIONSafer and more effective treatment algorithm and basic researches and clinical trials are warranted to be explored.

Heart Defects, Congenital ; therapy ; Humans ; Hypertension, Pulmonary ; therapy

Humans ; Hypertension, Pulmonary/surgery* ; Learning Curve ; Angioplasty, Balloon ; Pulmonary Embolism/therapy* ; Chronic Disease ; Pulmonary Artery/surgery*

Epidemiologic patterns of pulmonary arterial hypertension differ by era and region and may shed light on the pathophysiology and treatment of the disease. New efforts to target one or more of the recently studied therapies could establish personalized medicine as standard care in pulmonary arterial hypertension.
Humans ; Hypertension, Pulmonary ; epidemiology ; physiopathology ; therapy ; Risk Factors

Humans ; Hypertension, Pulmonary/drug therapy* ; Antihypertensive Agents/therapeutic use* ; Cardiology ; Hypertension/drug therapy*