PURPOSE: We evaluated the hemodynamic statuses of patients after partial closure of atrial septal defects with fenestration due to pulmonary hypertension. MATERIALS AND METHODS: Seventeen adult patients underwent partial atrial septal defect closure and follow-up cardiac catheterization. We analyzed hemodynamic data and clinical parameters before and after closure. RESULTS: The median age at closure was 29 years old. The baseline Qp/Qs was 1.9+/-0.6. The median interval from the operation to the cardiac catheterization was 27 months. The CT ratio decreased from 0.55+/-0.07 to 0.48+/-0.06 (p<0.05). The mean pulmonary arterial pressure decreased from 50.0+/-11.5 mm Hg to 32.5+/-14.4 mm Hg (p<0.05), and the pulmonary resistance index decreased from 9.2+/-3.6 Wood units*m2 to 6.3+/-3.8 Wood units*m2 (p<0.05). Eleven patients (64.7%) continued to exhibit high pulmonary resistance (over 3.0 Wood units*m2) after closure. These patients had significantly higher pulmonary resistance indices and mean pulmonary arterial pressures based on oxygen testing before the partial closures (p<0.05). However, no significant predictors of post-closure pulmonary hypertension were identified. CONCLUSION: Despite improvement in symptoms and hemodynamics after partial closure of an atrial septal defect, pulmonary hypertension should be monitored carefully.
Adult ; Aged ; Cardiac Catheterization/*adverse effects ; Female ; Follow-Up Studies ; Heart Defects, Congenital/epidemiology/*physiopathology/*surgery ; Heart Septal Defects, Atrial/surgery ; Hemodynamics/*physiology ; Humans ; Hypertension, Pulmonary/diagnosis/epidemiology/*physiopathology ; Male ; Middle Aged ; Oxygen ; Postoperative Complications/diagnosis/epidemiology/*physiopathology ; Retrospective Studies ; Treatment Outcome ; Young Adult

OBJECTIVETo assess the pathologic markers for evaluation of reversibility in pulmonary hypertension (PAH) related to congenital heart disease.

METHODSTwenty-eight patients with congenital heart disease complicated by PAH were subclassified into reversible pulmonary hypertension (RPAH) and irreversible pulmonary hypertension (IPAH), according to post-operative mean pulmonary artery pressure (MPAP). Pulmonary vascular lesion was analyzed according to Ruan's method. Mean medium thickness percent, mean medium area percent and pulmonary arteriolar density were measured by quantitative morphometry. Immunohistochemical study for transgelin and filamin A was carried out.

RESULTSAmongst the 28 cases studied, 24 were RPAH and 4 were IPAH. Of the 24 patients with RPAH, 13 (54.2%, 13/24) had pulmonary vascular lesion of grade 0, 9 (37.5%, 9/24) of grade 1 and 2 (8.3%, 2/24) of grade 2. Of the 4 patients with IPAH, 1 had lesion of grade 1, 1 of grade 2 and 2 of grade 3. Both preoperative and postoperative MPAP were higher in IPAH patients than that in RPAH patients[(53.3±23.4) mmHg versus (34.1±12.7) mmHg, P=0.020 and (35.0±8.8) mmHg versus (17.8±3.9) mmHg, P<0.01]. Compared to patients with pulmonary vascular lesion of grades 0 and 1, the preoperative MPAP in patients with grades 2 and 3 showed no significant difference, but the postoperative MPAP was higher (P<0.05 or 0.01). Compared to control group, mean medium thickness percent and mean medium area percent were significantly higher in RPAH and IPAH categories (12.0±3.5, 8.5±2.0 versus 5.7±1.0, P<0.01 and 55.8±11.1, 49.0±9.4 versus 34.0±5.5, P<0.01). Mean medium thickness percent was significantly higher in IPAP group than that in RPAH group (12.0±3.5 versus 8.5±2.0, P=0.001). Correlation analysis demonstrated that mean medium thickness percent and mean medium area percent had positive correlation with preoperative and postoperative MPAP. There was no correlation between grading of pulmonary vascular lesion and reversibility. Transgelin and filamin A had stronger staining in pulmonary vascular smooth muscle cells in IPAH than those in RPAH and controls(P<0.05).

CONCLUSIONSPathologic assessment of lung biopsy remains the gold standard for evaluation of the reversibility in PAH related to congenital heart disease. Mean medium thickness percent, mean medium area percent and immunoreactivity for transgelin and filamin A are useful parameters.

Biomarkers ; metabolism ; Biopsy ; Filamins ; metabolism ; Heart Diseases ; complications ; pathology ; Humans ; Hypertension, Pulmonary ; complications ; diagnosis ; pathology ; Lung ; pathology ; Microfilament Proteins ; metabolism ; Muscle Proteins ; metabolism

BACKGROUNDFibrosing mediastinitis (FM) is a rare disease. FM is thought to be related to prior granulomatous mediastinal infection, such as histoplasmosis or tuberculosis. The majority of cases have been reported in endemic regions for histoplasmosis. The characteristics of cases of FM in China, where the prevalence of tuberculosis is high, have not been reported. We analyzed the clinical, imaging, and bronchoscopic features of Chinese patients with FM to promote awareness of this disease.

METHODSBetween January 2005 and June 2015, twenty patients were diagnosed with FM in our hospital. Medical records and follow-up data were collected. Imaging and biopsy findings were reviewed by radiologists and pathologists.

RESULTSA total of 20 patients were analyzed (8 males and 12 females). The age ranged from 43 to 88 years with a mean age of 69.5 years. Previous or latent tuberculosis was found in 12 cases. Clinical symptoms included dyspnea (18/20), cough (17/20), expectoration (7/20), and recurrent pneumonia (3/20). Chest computed tomography scans showed a diffuse, homogeneous, soft tissue process throughout the mediastinum and hila with compression of bronchial and pulmonary vessels. Calcification was common (15/20). Pulmonary hypertension was present in 9 of 20 cases. Diffuse black pigmentation in the bronchial mucosa was frequently seen on bronchoscopy (12/13). The patients' response to antituberculosis treatment was inconsistent.

CONCLUSIONSFM in Chinese patients is most likely associated with tuberculosis. Some characteristics of FM are different from cases caused by histoplasmosis.

Adult ; Aged ; Aged, 80 and over ; Asian Continental Ancestry Group ; China ; Female ; Histoplasmosis ; complications ; diagnosis ; Humans ; Hypertension, Pulmonary ; diagnosis ; physiopathology ; Male ; Mediastinitis ; diagnosis ; etiology ; physiopathology ; Middle Aged ; Sclerosis ; diagnosis ; etiology ; physiopathology ; Tuberculosis ; diagnosis ; physiopathology

The Korea Chronic Obstructive Pulmonary Disorders Subgroup Study Team (Korea COPD Subgroup Study team, KOCOSS) is a multicenter observational study that includes 956 patients (mean age 69.9 ± 7.8 years) who were enrolled from 45 tertiary and university-affiliated hospitals from December 2011 to October 2014. The initial evaluation for all patients included pulmonary function tests (PFT), 6-minute walk distance (6MWD), COPD Assessment Test (CAT), modified Medical Research Council (mMRC) dyspnea scale, and the COPD-specific version of St. George's Respiratory Questionnaire (SGRQ-C). Here, we report the comparison of baseline characteristics between patients with early- (Global Initiative for Chronic Obstructive Lung Disease [GOLD] stage I and II/groups A and B) and late-stage COPD (GOLD stage III and IV/groups C and D). Among all patients, the mean post-bronchodilator FEV1 was 55.8% ± 16.7% of the predicted value, and most of the patients were in GOLD stage II (520, 56.9%) and group B (399, 42.0%). The number of exacerbations during one year prior to the first visit was significantly lower in patients with early COPD (0.4 vs. 0.9/0.1 vs. 1.2), as were the CAT score (13.9 vs. 18.3/13.5 vs. 18.1), mMRC (1.4 vs. 2.0/1.3 vs.1.9), and SGRQ-C total score (30.4 vs. 42.9/29.1 vs. 42.6) compared to late-stage COPD (all P < 0.001). Common comorbidities among all patients were hypertension (323, 37.7%), diabetes mellitus (139, 14.8%), and depression (207, 23.6%). The data from patients with early COPD will provide important information towards early detection, proper initial management, and design of future studies.
Aged ; Cohort Studies ; Comorbidity ; Depression/epidemiology ; Diabetes Mellitus/epidemiology ; Dyspnea/complications ; Female ; Forced Expiratory Volume ; Hospitals, University ; Humans ; Hypertension/epidemiology ; Lung/physiopathology ; Male ; Middle Aged ; Pulmonary Disease, Chronic Obstructive/complications/*diagnosis/physiopathology ; Quality of Life ; Republic of Korea ; Respiratory Function Tests ; Severity of Illness Index ; Societies, Medical ; Surveys and Questionnaires ; Tertiary Care Centers ; Walk Test

Pulmonary embolism (PE) poses a challenge to physicians, as it can be difficult to diagnose but results in significant mortality and morbidity in patients. Diagnosing PE requires an integrated approach using clinical findings, electrocardiography (ECG), blood investigations and imaging modalities. Abnormalities in ECG are common among patients with massive acute PE and can serve as a prognostic indicator. In this article, we describe the ECG presentations of two patients diagnosed with PE, and review the literature on the various types of ECG presentations and their role in predicting the prognosis of PE.
Anticoagulants ; administration & dosage ; Blood Pressure ; Echocardiography ; Electrocardiography ; methods ; Female ; Hemorrhage ; complications ; Humans ; Hypertension ; complications ; Male ; Middle Aged ; Prognosis ; Pulmonary Embolism ; diagnosis ; Stroke ; complications

A case of portopulmonary hypertension characterized by repeated syncope was retrospectively analyzed. Intrahepatic or extrahepatic factor-induced portal hypertension complicated with metabolic disorder of vasoactive substances, vascular pressure, inflammation, etc. may result in systolic and diastolic dysfunction of pulmonary arteries and systemic hyperdynamic circulation, the long-term effect of which can induce vascular remodeling and consequently, pulmonary hypertension. The pathogenic process is rather insidious. Pulmonary hypertension is clinically characterized by the raised average pulmonary artery pressure, normal pulmonary capillary wedge pressure and high pulmonary vascular resistance. Currently available therapeutic approaches include drug therapy targeting on pulmonary hypertension and liver transplantation.
Blood Pressure ; Humans ; Hypertension, Portal ; complications ; diagnosis ; Hypertension, Pulmonary ; complications ; diagnosis ; Liver Transplantation ; Syncope ; complications ; diagnosis

The clinical manifestation included snoring and mouth breathing for 2 years, repeated coughing and shortness of breath in action for more than 1 year. Physical examination of oral cavity showed tonsils were in grade III. The endoscopy showed 2/3 of postnaris were blocked by the adenoids. The preoperative ultrasonic cardiogram revealed the right atrial and right ventricular dilatation, pulmonary artery widened. The preoperative polysomnography (PSG) showed apnea-hypopnea index (AHI) was 28.5 events an hour, and the lowest oxygen saturation (LSaO2) was 39%. The patient was diagnosed as severe obstructive sleep apnea hypopnea syndrome with pulmonary hypertension. The postoperative PSG showed the AHI was 11.7 events an hour, and the LSaO2 was 86%. The ultrasonic cardiogram at 5 months after surgery didn't show any abnormalities.
Adenoids ; pathology ; Child ; Cough ; Humans ; Hypertension, Pulmonary ; complications ; diagnosis ; Palatine Tonsil ; pathology ; Polysomnography ; Sleep Apnea, Obstructive ; complications ; diagnosis ; Snoring

OBJECTIVE: This study strived to evaluate the relationship between degree of pulmonary emphysema and cardiac ventricular function in chronic obstructive pulmonary disease (COPD) patients with pulmonary hypertension (PH) using electrocardiographic-gated multidetector computed tomography (CT). MATERIALS AND METHODS: Lung transplantation candidates with the diagnosis of COPD and PH were chosen for the study population, and a total of 15 patients were included. The extent of emphysema is defined as the percentage of voxels below -910 Hounsfield units in the lung windows in whole lung CT without intravenous contrast. Heart function parameters were measured by electrocardiographic-gated CT angiography. Linear regression analysis was conducted to examine the associations between percent emphysema and heart function indicators. RESULTS: Significant correlations were found between percent emphysema and right ventricular (RV) measurements, including RV end-diastolic volume (R2 = 0.340, p = 0.023), RV stroke volume (R2 = 0.406, p = 0.011), and RV cardiac output (R2 = 0.382, p = 0.014); the correlations between percent emphysema and left ventricular function indicators were not observed. CONCLUSION: The study revealed that percent emphysema is correlated with RV dysfunction among COPD patients with PH. Based on our findings, percent emphysema can be considered for use as an indicator to predict the severity of right ventricular dysfunction among COPD patients.
Adult ; Aged ; Electrocardiography ; Female ; Heart Ventricles/radiography ; Humans ; Hypertension, Pulmonary/complications/*diagnosis ; Lung/radiography ; Male ; Middle Aged ; Multidetector Computed Tomography ; Pulmonary Disease, Chronic Obstructive/complications/*radiography ; Pulmonary Emphysema/complications/*radiography ; Regression Analysis ; Ventricular Function/*physiology

Adult ; Echocardiography, Doppler, Color ; Female ; Humans ; Hypertension, Pulmonary ; etiology ; Pulmonary Veno-Occlusive Disease ; complications ; diagnosis ; Tomography, X-Ray Computed

Evidence suggests that diabetes mellitus (DM) is associated with idiopathic pulmonary fibrosis (IPF). According to the new IPF guidelines, high-resolution computed tomography (HRCT) is an essential means of diagnosing IPF. We investigated the relationship between IPF and DM in patients treated between 2003 and 2007. Newly diagnosed IPF patients in large university teaching hospitals in Korea were enrolled from January 2003 to December 2007. We retrospectively analyzed 1,685 patients using the interstitial lung disease (ILD) registry. In total, 299 IPF patients (17.8%) also had DM. The mean age of our subjects was 68.0 +/- 9.4 yr. HRCT showed significantly more reticular and honeycomb patterns in IPF patients with DM than in IPF patients without DM (P = 0.014, P = 0.028, respectively). Furthermore, significantly higher incidences of hypertension, cardiovascular diseases, and other malignancies (except lung cancer) were found in IPF patients with DM than in IPF patients without DM. In conclusion, IPF patients with DM are more likely to have the usual interstitial pneumonia (UIP) pattern, including reticular and honeycomb patterns, on HRCT than are those without DM.
Aged ; Cardiovascular Diseases/epidemiology/etiology ; Diabetes Mellitus, Type 2/*complications ; Female ; Humans ; Hypertension/epidemiology/etiology ; Idiopathic Pulmonary Fibrosis/complications/*diagnosis/radiography ; Incidence ; Male ; Middle Aged ; Neoplasms/epidemiology/etiology ; Registries ; Republic of Korea/epidemiology ; Retrospective Studies ; Tomography, X-Ray Computed