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 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

A 34-year-old woman was admitted to the hospital because of recently aggravated right heart failure without angina for 5 months. When she was 25 years old, patch repair with Polytetrafluoroethylene (PTFE) was performed for the secondum type of atrial septal defect (ASD) with moderate pulmonary hypertension. The chest PA, echocardiography and cardiac catheterization at current admission revealed Eisenmenger syndrome without intracardiac shunt. Chest CT scan with contrast revealed markedly dilated pulmonary trunk, both pulmonary arteries and concave disfigurement of the left side of the ascending aorta suggesting extrinsic compression, as well as total occlusion of the ostium of the left main coronary artery that was retrogradly filled with collateral circulation from the right coronary artery. The coronary angiography showed normal right coronary artery and the collaterals that come out from the conus branch to the mid-left anterior descending artery (LAD) and that from distal right coronary artery to the left circumflex artery (LCX) and to the distal LAD, respectively. On aortography, the left main coronary artery was not visualized with no stump, suggestive of total occlusion of the ostium of the left main coronary artery. From our experience, it is possible to say that the occlusion of the ostium of the left main coronary can be induced by the dilated pulmonary artery trunk due to ASD with pulmonary hypertension and that, if the ASD closure was too late, the narrowing or obstruction of the left coronary artery could not be resolved even after operation owing to irreversible pulmonary hypertension.
Adult ; Case Report ; Constriction, Pathologic/etiology/radiography ; Coronary Disease/*etiology/radiography ; Dilatation, Pathologic/etiology ; Eisenmenger Complex/diagnosis ; Female ; Heart Septal Defects, Atrial/*complications ; Human ; Hypertension, Pulmonary/*complications ; *Pulmonary Artery/radiography