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

Pulmonary arterial hypertension is a significant complication of congenital heart disease, which carries a recognized risk of morbidity and mortality. There have been remarkable advances in the field of pulmonary arterial hypertension over the past several decades. At the third world symposium on pulmonary arterial hypertension held in Venice, Italy, 2003, congenital cardiac shunts were classified in the same group as idiopathic pulmonary hypertension. This article discusses the recent advances in understanding the pathology, pathobiology, diagnosis, and treatment of pulmonary arterial hypertension associated pediatric congenital heart disease.
Diagnosis ; Heart Defects, Congenital ; Heart Diseases* ; Heart* ; Hypertension* ; Hypertension, Pulmonary ; Italy ; Mortality ; Pathology ; Pediatrics

Pulmonary arterial hypertension is a significant complication of congenital heart disease, which carries a recognized risk of morbidity and mortality. There have been remarkable advances in the field of pulmonary arterial hypertension over the past several decades. At the third world symposium on pulmonary arterial hypertension held in Venice, Italy, 2003, congenital cardiac shunts were classified in the same group as idiopathic pulmonary hypertension. This article discusses the recent advances in understanding the pathology, pathobiology, diagnosis, and treatment of pulmonary arterial hypertension associated pediatric congenital heart disease.
Diagnosis ; Heart Defects, Congenital ; Heart Diseases* ; Heart* ; Hypertension* ; Hypertension, Pulmonary ; Italy ; Mortality ; Pathology ; Pediatrics

BACKGROUNDIdiopathic pulmonary fibrosis (IPF) is a lethal chronic interstitial lung disease (ILD) of unknown cause and having a variable and unpredictable course. This study aimed to summarize the clinical features and follow-up outcomes and to identify potential factors useful for the assessment of prognosis in IPF.

METHODSTwo hundred and ten patients hospitalized and diagnosed as IPF in our unit from January 1999 to June 2007 were enrolled into this study. The baseline demographic, clinical, radiologic and physiologic characteristics were summarized. Clinical follow-up data until February 2010 were collected, and the median survival time and 1-, 2-, and 5-year survival rates, as well as the influences of the summarized baseline variables on the prognosis were analyzed.

RESULTSThe age at diagnosis as IPF was (64 ± 10) years, the duration before diagnosis of 106 patients (50%) was shorter than 2 years, and 73% were males. One hundred and forty-five patients (69%) had a history of smoking with a median pack-year of 18. Eighty-nine patients (42%) had emphysema and 62 patients (29%) pulmonary arterial hypertension (PAH). One hundred and twenty-four patients were followed up, of which 99 patients died from various causes including respiratory failure related to IPF (93%). The follow-up period was (21 ± 23) months. The median survival time was 38 months. The 1-, 2-, and 5-year survival rates were 61%, 52%, and 39%, respectively. Multivariate analysis showed clubbing, PAH, duration from initial onset to diagnosis, and forced expiratory volume in 1 second (FEV1)/forced vital capacity (FVC) were independent prognostic indicators of IPF.

CONCLUSIONIPF patients who have clubbing, PAH, a higher FEV1/FVC, and a short duration from initial onset to diagnosis have a poorer outcome.

Aged ; Emphysema ; diagnosis ; mortality ; physiopathology ; Female ; Humans ; Hypertension, Pulmonary ; diagnosis ; pathology ; physiopathology ; Idiopathic Pulmonary Fibrosis ; diagnosis ; mortality ; physiopathology ; Male ; Middle Aged

Primary pulmonary hypertension (PPH) is a rare, progressive and incurable disease, which is characterized by an increase in the pulmonary artery pressure without a demonstrable cause. The most common presenting symptom is dyspnea on exertion, with other symptoms comprising of chest pain, syncope and hemoptysis. The diagnosis is one of exclusion of any of the known causes of pulmonary hypertension. When associated with pregnancy, the maternal mortality ranges from 30 to 50%. Because pregnancy and labor are very serious problems for patients with PPH, the available evidence suggests that pregnancy when afflicted with PPH should be avoided. In account the case of a 33-year old patient, reporting with massive hemoptysis, and diagnosed with PPH during her twenty seventh week of gestation, is presented. She was treated with conservative management, including oxygen and a vasodilator, and underwent a pregnancy termination. However, due to aggravation of right heart failure, she presented with severe systemic hypotension and hypoxemia, and eventually died. This case is reported, with brief review of the literature.
Adult ; Anoxia ; Chest Pain ; Diagnosis ; Dyspnea ; Heart Failure ; Hemoptysis* ; Humans ; Hypertension, Pulmonary* ; Hypotension ; Maternal Mortality ; Oxygen ; Pregnancy* ; Pulmonary Artery ; Syncope

Despite the necessity of surgical aortic valve replacement, many patients with symptomatic severe aortic stenosis (AS) cannot undergo surgery because of their severe comorbidities. In these high-risk patients, percutaneous transcatheter aortic valve implantation (TAVI) can be safely accomplished. However, no study has shown that TAVI can be performed for patients with severe AS accompanied by acute decompensated heart failure. In this case report, 1 patient presented a case of severe pulmonary hypertension with decompensated heart failure after diagnosis with severe AS, and was successfully treated via emergency TAVI. Without any invasive treatment, acute decompensated heart failure with severe pulmonary hypertension is common in patients with severe AS, and it can increase mortality rates. In conclusion, TAVI can be considered one of the treatment options for severe as presented as acute decompensated heart failure patients with pulmonary hypertension.
Aortic Valve Stenosis ; Aortic Valve* ; Comorbidity ; Diagnosis ; Emergencies* ; Emergency Treatment ; Heart Failure ; Heart Valve Prosthesis Implantation ; Humans ; Hypertension, Pulmonary ; Mortality

Antiphospholipid syndrome (APS) causes recurrent thromboses and morbidity during pregnancy, including fetal loss. This malady is associated with the persistent presence of anticardiolipin antibody or lupus anticoagulant. The pulmonary manifestations of antiphospholipid syndrome include pulmonary thromboembolism, pulmonary hypertension, acute respiratory distress syndrome, etc. Pulmonary thromboembolism is often the initial manifestation of antiphospholipid syndrome and a timely diagnosis is critical due to the high mortality rate. We herein report on a 19-year-old man with pulmonary thromboembolism that was caused by primary antiphospholipid syndrome. He presented with blood-tinged sputum, fever and epigastric pain, and his chest computerized tomography showed pulmonary thromboembolism. The other possible causes of pulmonary thromboembolism were excluded and the diagnosis of primary antiphospholipid syndrome was confirmed by the lupus anticoagulant that was present on two occasions six weeks apart. We also discuss the nature and management of antiphospholipid syndrome, along with a brief review of the relevant literatures.
Antibodies, Anticardiolipin ; Antiphospholipid Syndrome* ; Diagnosis ; Fever ; Humans ; Hypertension, Pulmonary ; Lupus Coagulation Inhibitor ; Mortality ; Pregnancy ; Pulmonary Embolism* ; Respiratory Distress Syndrome, Adult ; Sputum ; Thorax ; Thrombosis ; Young Adult

Primary pulmonary hypertension is a condition characterized by sustained elevations of pulmonary artery pressure without demonstrable cause. It is incurable and progressive clinical entity. The incidence has been estimated at approximately 1 to 2 cases per million people in the general population. The maternal mortality of primary pulonary hypertension in pregnancy was said to be 30% and the first month after delivery represents the period of highest risk. We experienced a 27-year-old women with primary pulmonary hypertension diagnosed in postpartum period. She was transferred from the department of obstetrics to internal medicine due to dyspnea on the first day of postpartum. She was diagnosed by perfusion lung scan, chest CT and confirmed by echocardiography. This diagnosis were supported by findings of pulmonary angiogram and cardiac catheterization. Initially she was treated with diuretics, aspirin and anticoagulant therapy, and as a result of the treatment, her symptoms improved. However after 8months has elapsed, exertional dyspnea was worsening. Thus she was treated with calcium channel blocker, aspirin and diuretics and was followed at 1 month interval on the outpatient clinics.
Adult ; Ambulatory Care Facilities ; Aspirin ; Calcium Channels ; Cardiac Catheterization ; Cardiac Catheters ; Diagnosis ; Diuretics ; Dyspnea ; Echocardiography ; Female ; Humans ; Hypertension ; Hypertension, Pulmonary* ; Incidence ; Internal Medicine ; Lung ; Maternal Mortality ; Obstetrics ; Perfusion ; Postpartum Period* ; Pregnancy ; Pulmonary Artery ; Tomography, X-Ray Computed

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 AND OBJECTIVES: Pulmonary embolism (PE) is a disease with high mortality if left untreated. But, confirmative diagnosis is difficult because many diagnostic modalities are nonspecific. Pulmonary angiography, which is considered as the gold standard diagnostic tool, is invasive, costly, time-consuming, and not always available in small centers. Echocardiography is a non-invasive and available in the emergency room without significant time delay. We investigated the role of echocardiography as a screening test in patients with suspected PE. MATERIALS AND METHODS: (A) Retrospective study: We analyzed the recorded videotapes of transthoracic echocardiography in fifty patients with confirmed PE from Jan 1995 to Aug 1999. From the frequent echocardiographic findings of those patients, positive criteria were defined as followed ( 2 among 1-5 or only 6). (1) RV or pulmonary artery dilatation (RVEDA/LVEDA 0.6) (2) RV hypokinesis (3) paradoxical septal wall motion (4) pulmonary hypertension (5) TR (moderate degree or more) (6) Visible thrombus. Although the degree of TR was less than moderate, if the degree of pulmonary hypertension was more than moderate, we considered as PE. (B) Prospective study: From Nov 1999 to June 2000, patients with suspected pulmonary embolism underwent a transthoracic echocardiography and perfusion-ventilation lung scan or chest CT. We assessed echocardiographic findings, sensitivity and specificity of the previously defined echocardiographic positive criteria for PE. RESULTS: (A) Retrospective study: The sensitivity of echocardiography for PE was 74%. (B) Prospective study: The number of enrolled patients was thirty-four. In twenty patients the diagnosis was PE. The sensitivity of echocardiography was 75% but the specificity was 14%. CONCLUSION: Echocardiography may be used as a good screening test in patients who are clinically suspicious of pulmonary embolism.
Angiography ; Diagnosis ; Dilatation ; Echocardiography* ; Emergency Service, Hospital ; Humans ; Hypertension, Pulmonary ; Lung ; Mass Screening* ; Mortality ; Prospective Studies ; Pulmonary Artery ; Pulmonary Embolism* ; Retrospective Studies ; Sensitivity and Specificity ; Thrombosis ; Tomography, X-Ray Computed ; Videotape Recording