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

Child ; Connective Tissue Diseases ; complications ; diagnosis ; therapy ; Humans ; Hypertension, Pulmonary ; diagnosis ; etiology ; therapy

The rupture of an acute dissection of the ascending aorta into the space surrounding the pulmonary artery is an uncommon occurrence. No previous cases of transient pulmonary hypertension caused by a hematoma surrounding the pulmonary artery have been documented in the literature. Herein, we report a case of acute aortic dissection presenting as secondary pulmonary hypertension.
Aortic Aneurysm/*complications/diagnosis ; Aortic Rupture/*complications/diagnosis ; Constriction, Pathologic ; Female ; Hematoma/*complications ; Human ; Hypertension, Pulmonary/*etiology ; Middle Aged ; Pulmonary Artery/pathology/radiography ; Tomography, X-Ray Computed

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

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

OBJECTIVETo investigate the clinical manifestations, diagnosis and interventions of pulmonary hypertension (PAH) in patients with systemic lupus erythematosus (SLE).

METHODSFrom January 2001 to December 2007, 798 SLE patients without prior diagnosis were admitted in our hospital, among whom 39 were identified to have concomitant PAH defined by echocardiography. The clinical data of the 39 cases were analyzed retrospectively.

RESULTSThe incidence of PAH was 4.9% in these cohort of SLE patients. The 39 SLE patients with concomitant PAH included 5 men and 34 women with a mean age of 34-/+12 years. Positive correlations were found between the occurrence of PAH and the Raynaud phenomenon, fingertip vasculitis, anti-u1RNP antibody positivity, antiphospholipid antibody positivity, pericardial effusion, and interstitial pneumonia (P < 0.05). Patients with higher scores for SLE Disease Activity Index were liable to PAH. The presence of Raynaud phenomenon, fingertip vasculitis, anti-u1RNP antibody positivity, antiphospholipid antibody positivity, pericardial effusion, and interstitial pneumonia is correlated to greater severity PAH with poor prognosis.

CONCLUSIONPAH is not a rare concomitant disease in SLE patients. The presence of Raynaud phenomenon, fingertip vasculitis, anti-u1RNP antibody positivity, antiphospholipid antibody positivity, pericardial effusion, and interstitial pneumonia all suggest the likeliness of PAH in SLE patients, and echocardiographic examination may help derive an early diagnosis.

Adolescent ; Adult ; Aged ; China ; epidemiology ; Early Diagnosis ; Echocardiography ; Female ; Humans ; Hypertension, Pulmonary ; complications ; diagnosis ; epidemiology ; Lupus Erythematosus, Systemic ; complications ; diagnosis ; Male ; Middle Aged ; Raynaud Disease ; complications ; Retrospective Studies

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

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

OBJECTIVETo evaluate the incidence and predictive factors of chronic thromboembolic pulmonary hypertension (CTEPH) in patients with acute pulmonary thromboembolism (PTE).

METHODSClinical data of 104 patients with CT angiography-proven PTE were collected and Wells score, revised Geneva score and ECG score were calculated. Blood gas analysis, CK-MB and D-dimer values were analyzed. Heart chamber diameters and pulmonary artery systolic pressure (PASP) were assessed by echocardiography. Qanadli obstruction index and Mastora obstruction index were evaluated according to computed tomography pulmonary angiography (CTPA). Patients were followed up by telephone or clinic visit to assess the WHO functional class and the incidence of CTEPH.

RESULTSDuring the 25.47±16.94 months (4 to 62 months) follow-up, 7 out of 104 patients were lost to follow-up and data from the remaining 95 patients were analyzed. The incidence of CTEPH was 14.4% (14/97). Baseline PASP, incidence of recurrent PTE, right atrium and right ventricle diameters and CK-MB were significantly higher in CTEPH group compared to non-CTEPH group (all P<0.05). Higher CK-MB (odds ratio: 8.3) and baseline PASP (odds ratio: 5.0 per 20 mm Hg increment) were independent predictive factors for CTEPH in this cohort.

CONCLUSIONSHigher CK-MB and baseline PASP values are independent predictive factors related to the development of CTEPH post acute pulmonary thromboembolism.

Aged ; Blood Gas Analysis ; Blood Pressure ; Creatine Kinase, MB Form ; blood ; Female ; Humans ; Hypertension, Pulmonary ; blood ; complications ; diagnosis ; Incidence ; Male ; Middle Aged ; Pulmonary Artery ; physiopathology ; Pulmonary Embolism ; blood ; complications

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