We present here the radiological findings of a rare case of multiple focal nodular hyperplasia that was associated with portal vein atresia and portopulmonary hypertension in a young woman. This case illustrates and supports the pathophysiological hypotheses that were previously proposed for the coexistence of these three abnormalities.
Adult ; Female ; Focal Nodular Hyperplasia/*radiography ; Humans ; Hypertension, Portal/*complications ; Hypertension, Pulmonary/*complications ; Portal Vein/*abnormalities

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

We report a case of a 29-year-old female with a history of asthma, post-partumARDS, and pulmonary hypertension who presents with severe shortness of breath. The patient describes her shortness of breath as progressive over the past 10 years. Chest radiography and CT angiography of the thorax showed findings consistent with fibrosing mediastinitis with severe stenosis of the left main pulmonary artery. This resulted in appearance of unilateral absent left lung perfusion on quantitative Tc-99-MAA perfusion and Xe-133 ventilation (V/Q) scan.
Adult ; Angiography ; Asthma ; Constriction, Pathologic ; Dyspnea ; Female ; Histoplasma ; Humans ; Hypertension, Pulmonary ; Lung ; Mediastinitis ; Perfusion ; Pulmonary Artery ; Radiography ; Thorax ; Ventilation

This study assessed main pulmonary artery diameter of patients with anthracofibrosis. Patients with anthracofibrosis and CT scans were evaluated after exclusion of patients with co-existing disease. We measured the diameter of the main pulmonary artery (PAD) and ascending aorta (AD) and calculated the pulmonary artery to aorta ratio (APR). The upper reference limit for comparison of PAD was 29 mm. Cut-off values for PAD and APR indicating pulmonary hypertension were 33 mm and 1. We correlated the CT parameters with echocardiographic results. Total 51 patients were included in the analysis. The mean PAD, AD, and APR were 33 mm, 38 mm, and 0.87 respectively. The PAD was larger than the upper reference limit, 29 mm (P<0.001). The PAD was >33 mm in 30 (65%) and the APR was >1 in 9 patients (18%). Of 21 patients with echocardiography, 11 (52%) were found to have pulmonary hypertension. There was no statistical difference in the diagnosis of pulmonary hypertension between echocardiography and CT (P=1.000). In conclusion, main pulmonary artery is dilated in patients with anthracofibrosis more than in the healthy population.
Aged ; Aged, 80 and over ; Aorta, Thoracic/*radiography ; Bronchi/pathology ; Bronchoscopy ; Dilatation ; Female ; Fibrosis ; Humans ; Hypertension, Pulmonary/*diagnosis/ultrasonography ; Male ; Pulmonary Artery/*radiography ; Tomography, X-Ray Computed

BACKGROUND: Idiopathic pulmonary arterial hypertension (IPAH) and chronic thromboembolic pulmonary hypertension (CTEPH) are rare but significantly imperative in inducing chronic pulmonary hypertension. Clinically, it is difficult to distinguish between IPAH and CTEPH. However, the treatment of pulmonary hypertension is different depending on the disease. The present study was performed to analyze the similarities and differences in clinical features between IPAH and CTEPH. METHODS: During a nine-year period, thirty-three patients with IPAH and twenty-two patients with CTEPH were enrolled. Symptoms, physical findings, chest radiograph, electrocardiograph, pulmonary function test, echocardiograph, perfusion lung scan, right heart catheterization results were analyzed between both the groups. RESULTS: The median age of IPAH group was 33 (6~70) years that was lower than that (52(27~80) years) of CTEPH group. Amongst the IPAH patients, there was female predominance (76 %) and there was no sex difference between the patients with CTEPH. Both the groups exhibited similarity in the results of chest radiograph, electrocardiograph, pulmonary function test, and echocardiograph. In the perfusion lung scan, all IPAH patients exhibited findings with normal (28%) or low probability (72%) of pulmonary embolism and all CTEPH patients exhibited findings with high probability of pulmonary embolism. CONCLUSION: Although IPAH and CTEPH bear similarities in terms of symptoms, physical signs and general investigation results, there were differences in age distribution, sex predominance and results of perfusion lung scan.
Age Distribution ; Cardiac Catheterization ; Cardiac Catheters ; Diagnosis, Differential ; Electrocardiography ; Female ; Humans ; Hypertension* ; Hypertension, Pulmonary* ; Lung ; Perfusion ; Pulmonary Embolism ; Radiography, Thoracic ; Respiratory Function Tests ; Sex Characteristics

OBJECTIVE: To assess perfusion patterns on a dual-energy pulmonary CT angiography (DECTA) of pulmonary hypertension (PHT) with variable causes and to assess whether the extent of perfusion defect can be used in the severity assessment of PHT. MATERIALS AND METHODS: Between March 2007 and February 2011, DECTA scans of 62 consecutive patients (24 men, 38 women; mean age, 58.5 +/- 17.3 [standard deviation] years; range, 19-87 years) with PHT were retrospectively included with following inclusion criteria; 1) absence of acute pulmonary thromboembolism, 2) maximal velocity of tricuspid regurgitation jet (TR Vmax) above 3 m/s on echocardiography performed within one week of the DECTA study. Perfusion patterns of iodine map were divided into normal (NL), diffuse heterogeneously decreased (DH), multifocal geographic and multiple peripheral wedging patterns. The extent of perfusion defects (PD), the diameter of main pulmonary artery (MPA) and the ratio of ascending aorta diameter/MPA (aortopulmonary ratio, APR) were measured. Pearson correlation analysis was performed between TR Vmax on echocardiography and CT imaging parameters. RESULTS: Common perfusion patterns of primary PHT were DH (n = 15) and NL (n = 12). The perfusion patterns of secondary PHT were variable. On the correlation analysis, in primary PHT, TR Vmax significantly correlated with PD, MPA and APR (r = 0.52, r = 0.40, r = -0.50, respectively, all p < 0.05). In secondary PHT, TR Vmax significantly correlated with PD and MPA (r = 0.38, r = 0.53, respectively, all p < 0.05). CONCLUSION: Different perfusion patterns are observed on DECTA of PHT according to the causes. PD and MPA are significantly correlated with the TR Vmax.
Adult ; Aged ; Aged, 80 and over ; Aorta/physiopathology ; Aortography ; Female ; Humans ; Hypertension, Pulmonary/physiopathology/*radiography ; Male ; Middle Aged ; Pulmonary Artery/physiopathology/*radiography ; Pulmonary Circulation/physiology ; Retrospective Studies ; Tomography, X-Ray Computed/*methods ; Tricuspid Valve Insufficiency/physiopathology/radiography ; Young Adult

Extrinsic compression of the left main coronary artery (LMCA) secondary to pulmonary artery dilatation is a rare syndrome. Most cases of pulmonary artery hypertension but no atherosclerotic risk factors rarely undergo coronary angiography, and hence, diagnoses are seldom made and proper management is often delayed in these patients. We describe a patient that presented with pulmonary hypertension, clinical angina, and extrinsic compression of the LMCA by the pulmonary artery, who was treated successfully by percutaneous coronary intervention. Follow-up coronary angiography showed patent stent in the LMCA in the proximity of the dilated main pulmonary artery. This case reminds us that coronary angiography and percutaneous coronary intervention should be considered in pulmonary hypertension patients presenting with angina or left ventricular dysfunction.
Angina Pectoris/etiology ; Angioplasty, Balloon, Coronary ; Coronary Angiography ; Coronary Stenosis/radiography/therapy ; Coronary Vessels/radiography/*ultrasonography ; Dilatation, Pathologic ; Female ; Humans ; Hypertension, Pulmonary/etiology/radiography ; Middle Aged ; Pulmonary Artery/radiography/*ultrasonography ; Stents ; Tomography, X-Ray Computed ; Ventricular Dysfunction, Left

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

We had performed six times (4 in right lung, 2 in left lung) bronchoalveolar lavages in a patient who has hypertension and suffered cerebral vascular accident previously. The diagnosis of PAP was confirmed by an open lung biopsy and multiple bronchoalveolar lavages were done for two and a half months. Even though she showed slight improvement in chest radiographs, she showed minimal improvement in her clinical course until the third lavage was done. She had to receive tracheostomy and prolonged ventilatory care because her PAP was progressed rapidly and complicated with superinfection. In addition, she sustained pneumothorax as a complication of the prolonged ventilatory care and the lavages. She was discharged one month after the last lavage and her clinical course has been uneventful 10 months thereafter.
Biopsy ; Bronchoalveolar Lavage* ; Diagnosis ; Humans ; Hypertension ; Lung ; Pneumothorax ; Pulmonary Alveolar Proteinosis* ; Radiography, Thoracic ; Superinfection ; Therapeutic Irrigation ; Tracheostomy

PURPOSE: We wanted to evaluate whether there is any different finding on CT with aging for the patients suffering with adult Swyer-James-MacLeod Syndrome (SJMS). MATERIALS AND METHODS: We included 11 patients (7 males and 4 females) who underwent chest CT scan among 18 patients who were suspected of suffering with SJMS on chest radiographs. The range of age was from 28 to 85 years (mean: 58.5). We evaluated the diameter of both the main pulmonary artery (MPA) with its ratio, and the diameter of the pulmonary trunk (PT) to evaluate the possibility of pulmonary arterial hypertension, and the presence or absence of bronchiectasis. We also evaluated the relationships between these findings and aging. RESULTS: SJMS affected the left lung in 10 of 11 patients. The mean diameter of the main pulmonary artery of the normal lung was 2.5 cm and it was 1.6 cm in the involved site. The mean ratio of the normal MPA diameter to the involved one was 1.6 and this did not correlate with age (p>0.1). The mean diameter of the pulmonary trunk was 2.8 cm and this increased with age (p<0.05). There was bronchiectasis in 6 of 11 patients, and the ratio of bronchiectasis did not correlate with age (p>0.5). CONCLUSION: SJMS absolutely affected the left lung much more than the right lung. All the patients demonstrated about 1.6 times the compensatory hypertrophy of MPA of the normal lung compared with that of the affected lung on chest CT, which was irrespective of age. The presence or absence of bronchiectasis has no correlation with age.
Adult* ; Aging* ; Bronchiectasis ; Bronchiolitis ; Humans ; Hypertension ; Hypertrophy ; Lung ; Male ; Pulmonary Artery ; Radiography, Thoracic ; Tomography, X-Ray Computed