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.

Pulmonary tumor thrombotic microangiopathy (PTTM) is an uncommon and fatal malignancy-related pulmonary complication characterized by fibrocellular intimal proliferation of small pulmonary arteries and arterioles. It causes marked pulmonary hypertension, right-side heart failure, and sudden death. Diagnosis of PTTM is extremely difficult while the patient is alive. Here, we report a 44-year-old woman who presented with complaining of progressing dyspnea and pulmonary hypertension but with no history of cancer. She was diagnosed with PTTM caused by advanced gastric cancer ante mortem and was treated effectively with chemotherapy.
Adult ; Ants ; Arterioles ; Death, Sudden ; Diagnosis ; Drug Therapy* ; Dyspnea ; Female ; Heart Failure ; Humans ; Hypertension, Pulmonary ; Pulmonary Artery ; Stomach Neoplasms* ; Thrombotic Microangiopathies*

Enbucrilate ; adverse effects ; therapeutic use ; Female ; Humans ; Hypertension, Portal ; diagnosis ; etiology ; Liver Cirrhosis, Biliary ; drug therapy ; Middle Aged ; Pulmonary Embolism ; chemically induced ; diagnosis ; Sepsis ; chemically induced ; etiology

Aged ; Antihypertensive Agents ; adverse effects ; Endothelin-1 ; antagonists & inhibitors ; drug effects ; Face ; pathology ; Female ; Humans ; Hypertension, Pulmonary ; drug therapy ; Sulfonamides ; adverse effects ; Telangiectasis ; chemically induced ; diagnosis

This paper provides an overview of the current state of pharmacotherapy in children with pulmonary arterial hypertension (PAH) and a brief introduction to the potentially novel pharmacologic targets for PAH. Currently, 3 classes of drugs including prostacyclin analogues, endothelin receptor antagonists and phosphodiesterase-5 inhibitors are approved for the treatment of PAH in children, which has led to improved hemodynamics, increased exercise capacity and prolonged survival. Despite these improvements, there is still a need to carry out well-designed, randomized, controlled studies with larger samples. In addition, novel drugs targeting other molecular pathways should be developed.
1-(5-Isoquinolinesulfonyl)-2-Methylpiperazine ; analogs & derivatives ; therapeutic use ; Calcium Channel Blockers ; therapeutic use ; Child ; Epoprostenol ; analogs & derivatives ; therapeutic use ; Familial Primary Pulmonary Hypertension ; Humans ; Hypertension, Pulmonary ; diagnosis ; drug therapy ; Iloprost ; therapeutic use ; Sulfonamides ; therapeutic use

Pulmonary artery sarcomas (PAS) are rare malignant neoplasms. Right heart failure due to tumour location is the main cause of death in PAS patients. The hemodynamic influence of PAS may effect prognosis, but this has not been proven. We aimed to identify the clinical characteristics and prognostic factors of PAS in Korea, their association with pulmonary hypertension (PH). PAS patients treated at the Asan Medical Center between 2000 and 2014 were reviewed. We examined demographic characteristics, diagnostic and treatment modalities. Potential prognostic factors were evaluated by univariate and multivariate analysis. Twenty patients were diagnosed with PAS. Ten patients were male, the median age was 54 years (range, 33–75 years). The most common symptom observed was dyspnea (65%). The most common histologic type was spindle cell sarcoma (30%). Ten patients had a presumptive diagnosis of pulmonary embolism (PE) and received anticoagulation therapy. Seventeen patients underwent surgery, but only 5 patients had complete resection. Eleven patients received post-operative treatment (chemotherapy = 3, radiotherapy = 5, chemoradiotherapy = 3). PH was observed in 12 patients before treatment and in 6 patients after treatment. Overall median survival was 24 months. Post-treatment PH was associated with poor prognosis (HR 9.501, 95% CI 1.79–50.32; P = 0.008) while chemotherapy was negatively associated with mortality (HR 0.102, 95% CI 0.013–0.826; P = 0.032) in univariate analysis. Post-treatment PH was also associated with poor prognosis in multivariate analysis (HR 5.7, 95% CI 1.08–30.91; P = 0.041). PAS patients are frequently misdiagnosed with PE in Korea. Post-treatment PH is associated with a poor prognosis.
Cause of Death ; Chemoradiotherapy ; Chungcheongnam-do ; Diagnosis ; Drug Therapy ; Dyspnea ; Heart Failure ; Hemodynamics ; Humans ; Hydrogen-Ion Concentration ; Hypertension, Pulmonary ; Korea* ; Male ; Mortality ; Multivariate Analysis ; Prognosis ; Pulmonary Artery* ; Pulmonary Embolism ; Radiotherapy ; Sarcoma*

BACKGROUND/AIMS: The diagnosis of chronic thromboembolic pulmonary hypertension (CTEPH) is difficult for numerous reasons and is related with a poor prognosis. In Korea, the incidence of CTEPH and its clinical features are unknown. Thus, in this study, we evaluated the clinical characteristics and outcomes of CTEPH in a Korean cohort. METHODS: This study included South Korean patients diagnosed with CTEPH between September 2008 and October 2011. Baseline characteristics, treatments and outcomes were analyzed. RESULTS: A total of 134 patients were included in this study with 76 females (56.7%). Their median age was 58.3 +/- 15.9 years and dyspnea (112 patients, 83.5%) was the most common presenting symptom. Sixty-three patients (47%) had a history of acute pulmonary embolism or deep vein thrombosis, and six (4.5%) had pulmonary tuberculosis. In total, 28 patients (21%) underwent pulmonary thromboendarterectomy (PTE), and 99 patients had medical therapy. During the study period, 18 patients (13.4%) died. In a multivariate analysis, higher hemoglobin (relative risk [RR], 1.516; 95% confidence interval [CI], 1.053 to 2.184; p = 0.025) and lower total cholesterol levels (RR, 0.982; 95% CI, 0.965 to 0.999; p = 0.037) were associated with increased mortality. CONCLUSIONS: This was the first national cohort study of Korean patients with CTEPH. Accurate diagnosis, characterization and distributions of CTEPH are imperative for prompt treatment in patients, particularly those undergoing PTE.
Adult ; Aged ; Antihypertensive Agents/therapeutic use ; Chi-Square Distribution ; Chronic Disease ; Drug Therapy, Combination ; Endarterectomy ; Female ; Humans ; Hypertension, Pulmonary/diagnosis/*epidemiology/mortality/therapy ; Logistic Models ; Male ; Middle Aged ; Multivariate Analysis ; Odds Ratio ; Prevalence ; Prospective Studies ; Pulmonary Embolism/diagnosis/*epidemiology/mortality/therapy ; Registries ; Republic of Korea/epidemiology ; Retrospective Studies ; Risk Factors ; Time Factors ; Treatment Outcome

BACKGROUND/AIMS: We assessed the efficacy and safety of bosentan in patients with pulmonary arterial hypertension (PAH). METHODS: We surveyed randomized controlled trials (RCTs) of the efficacy and safety of bosentan in patients with PAH using MEDLINE, EMBASE, the Cochrane Controlled Trials Register, and manual searches. Meta-analysis of RCTs was performed to determine treatment efficacy and safety outcomes. Results are presented as odds ratios (ORs) or weighted mean differences (WMDs). RESULTS: Meta-analysis of seven RCTs including a total of 410 patients and 296 controls revealed that the 6-minute work distance was significantly higher in the bosentan group than in the placebo group (WMD, 46.19; 95% confidence interval [CI], 21.20 to 71.19; p = 2.9 x 10(-5)). Compared with the placebo, bosentan significantly reduced the mean pulmonary arterial pressure in patients with PAH (WMD, -6.026; 95% CI, -8.785 to -3.268, p = 1.8 x 10(-6)). The bosentan therapy group worsened less clinically than the placebo group (OR, 0.252; 95% CI, 0.140 to 0.454; p = 4.6 x 10(-7)). The incidence of serious adverse events did not differ between the bosentan and placebo groups (OR, 0.948; 95% CI, 0.556 to 1.614; p = 0.843). However, the results of the abnormal liver function test (LFT) were significantly higher in the bosentan group than in the placebo group (OR, 2.312; 95% CI, 1.020 to 5.241; p = 0.045). CONCLUSIONS: This meta-analysis shows that bosentan can treat PAH effectively. However, bosentan increased the incidence of abnormal LFT results compared with the placebo.
Antihypertensive Agents/adverse effects/*therapeutic use ; Arterial Pressure/*drug effects ; Humans ; Hypertension, Pulmonary/diagnosis/*drug therapy/physiopathology ; Liver/drug effects/physiopathology ; Liver Function Tests ; Odds Ratio ; Pulmonary Artery/*drug effects/physiopathology ; Risk Factors ; Sulfonamides/adverse effects/*therapeutic use ; Time Factors ; Treatment Outcome

Congenital extrahepatic portocaval shunt (CEPS) is a rare anomaly of the mesenteric vasculature in which the intestinal and splenic venous drainage bypasses the liver and drains directly into the inferior vena cava, the left hepatic vein or the left renal vein. This uncommon disease is frequently associated with other malformations and mainly affects females. Here we report a case of pulmonary arterial hypertension associated with CEPS (Abernethy type 1b shunt) in a 20-yr-old man who was incidentally diagnosed during evaluation of multiple nodules of the liver. The patient was treated by inhalation of iloprost (40 microg/day) with improved condition and walking test. Physicians should note that congenital portocaval shunt may cause pulmonary hypertension.
Echocardiography, Doppler ; Humans ; Hypertension, Pulmonary/*diagnosis/drug therapy ; Iloprost/therapeutic use ; Liver/blood supply/radiography ; Magnetic Resonance Imaging ; Male ; Thoracic Arteries/ultrasonography ; Tomography, X-Ray Computed ; Vasodilator Agents/therapeutic use ; Vena Cava, Inferior/*abnormalities/ultrasonography ; Young Adult

OBJECTIVES: 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 survival. 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 poor survival expectancy. In this limited study with a small number of patients, mortality is largely associated with decreased cardiac index.
Adult ; Age Distribution ; Female ; Hemodynamics/physiology ; Human ; Hypertension, Pulmonary/diagnosis/drug therapy/*mortality ; Incidence ; Korea/epidemiology ; Male ; Middle Age ; Prognosis ; Proportional Hazards Models ; Respiratory Function Tests ; Retrospective Studies ; Risk Factors ; Sex Distribution ; Survival Analysis