Humans ; Hypertension, Pulmonary ; diagnosis

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

Algorithms ; Familial Primary Pulmonary Hypertension ; Humans ; Hypertension, Pulmonary/diagnosis* ; Pulmonary Arterial Hypertension

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.

Humans ; Hypertension, Pulmonary ; diagnosis ; Magnetic Resonance Imaging

Unilateral adsence of a pulmonary artery is frequently undiagnosed. Unless this entity is recognized, a patient with a potentially curable lesion may become inoperable. The importance of considering unilateral absence of a pulmonary artery in the diffirential diagnosis of cyanotic congenital heart disease and pulmonary hypertension has become clear. We exeperienced a case of congenital isolated unilateral absence of it. pulmonary artery confirmed by clinical features, lung scan, echocardiogram and angiogram.
Diagnosis ; Heart Defects, Congenital ; Humans ; Hypertension, Pulmonary ; Lung ; Pulmonary Artery*

Takayasu's arteritis is a chronic inflammatory disease that primarily affects large vessels, such as the aorta and its main branches. Pulmonary artery is often involved in Takayasu's arteritis, but only a few cases have been reported in which pulmonary artery involvement occurred as the initial clinical manifestation. We report one patient who was referred to our institution with the provisional diagnosis of chronic thromboembolic pulmonary hypertension and subsequently diagnosed with Takayasu's arteritis involving the pulmonary artery.
Aorta ; Diagnosis ; Humans ; Hypertension, Pulmonary ; Pulmonary Artery* ; Takayasu Arteritis*

BACKGROUND: Primary pulmonary hypertension(PPH) that affects predominantly young and productive people, is a progressive fatal disease of unknown cause. The objectives of this study were to characterize mortality in patients with PPH and to investigate the factors associated with their mortality. METHODS: Thirteen patients with PPH were enrolled between 1988 and 1996 and followed up through July 1999. Measurements at diagnosis included hemodynamic and pulmonary function variables in addition to information on demographic data and medical history. RESULTS: 1) The mean age of the patients with PPH enrolled into the study was 36.1+/-9.3 years with female predominance. 2) The estimated median survival was 3.4+/-0.6 years. 3) Decreased cardiac index was the only significant predictor of mortality(Cox proportional hazards model). CONCLUSION: Patients with PPH have a high mortality. In this limited study with a small number of patients, mortality is largely associated with decreased cardiac index.
Diagnosis ; Female ; Hemodynamics ; Humans ; Hypertension ; Hypertension, Pulmonary* ; Mortality ; Prognosis

The Prevalence of pediatric pulmonary hypertension is rare in general population, and the etiology of this disease is diverse or unknown. But, in recent years, remarkable understanding of vascular biology, normal and hypertensive pulmonary circulation has made an effort to the pathophysiology of pulmonary arterial hypertension. These efforts are adapted the patients treatments with new advanced drugs. In this reviw, we discuss the definition and the clinical classification of pulmonary hypertension, current concepts in the pathophysiology, diagnosis, and managements of pulmoanry hypertension. But, Despite of significant development of therapy, the patients with pulmonary hypertension in pediatric division are rare, so multicenter study in clinical setting is necessary to improve the quality of life and survival for them.
Biology ; Classification ; Diagnosis ; Humans ; Hypertension ; Hypertension, Pulmonary* ; Pediatrics ; Prevalence ; Pulmonary Circulation ; Quality of Life