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1.
J Thromb Haemost ; 16(12): 2397-2402, 2018 12.
Article En | MEDLINE | ID: mdl-30251461

Essentials In acute pulmonary embolism (PE), risk stratification is essential to drive clinical management. Improving the 2014-ESC risk stratification strategy is crucial in hemodynamically stable patients. Oxygen saturation and respiratory rate improve risk stratification in hemodynamically stable PE. Simple and routine tests improve risk stratification of hemodynamically stable PE. SUMMARY: Background In patients with acute pulmonary embolism (PE), risk stratification for short-term death is recommended to drive clinical management. A risk stratification strategy combining the simplified Pulmonary Embolism Severity Index (PESI), echocardiography and troponin was proposed by the European Society of Cardiology (ESC) in 2014. The identification of hemodynamically stable patients at increased risk of death by this strategy needs improvement. Objective To assess whether further stratification by serial cut-off values of oxygen saturation or respiratory rate improves the accuracy of the ESC risk stratification strategy in hemodynamically stable PE patients. Methods Prospective cohorts of hemodynamically stable patients with PE were merged in a collaborative database. The accuracy of risk stratification for 30-day mortality by the original and a modified 2014 ESC strategy was assessed. Results Overall, 255 patients (27%) were categorized as low, 510 (54%) as intermediate-low and 181 (19%) as intermediate-high risk according to the original 2014 ESC strategy. Thirty-day mortality was 1.2% in low, 10% in intermediate-low and 11% in intermediate-high-risk patients. By adding oxygen saturation in air of < 88%, the discriminatory power of the 2014 ESC model improved for 30-day mortality (c-statistics, 0.71; 95% confidence interval [CI], 0.65-0.77 vs. 0.63, 95% CI, 0.56-0.69) and for PE-related death (c-statistics, 0.75; 95% CI, 0.69-0.81 vs. 0.63, 95% CI 0.56-0.69). Conclusions Simple and routine tests, such as oxygen saturation or respiratory rate, could be added to the 2014 ESC strategy for risk stratification to identify hemodynamically stable PE patients at increased risk of death who are potentially candidates for more aggressive treatment.


Hemodynamics , Lung/physiopathology , Oximetry , Oxygen/blood , Pulmonary Embolism/diagnosis , Respiratory Function Tests , Respiratory Rate , Acute Disease , Adult , Aged , Aged, 80 and over , Biomarkers/blood , Databases, Factual , Europe , Female , Humans , Male , Middle Aged , Predictive Value of Tests , Prognosis , Prospective Studies , Pulmonary Embolism/blood , Pulmonary Embolism/mortality , Pulmonary Embolism/physiopathology , Risk Assessment , Risk Factors , Time Factors , Young Adult
2.
Lupus ; 27(2): 217-224, 2018 Feb.
Article En | MEDLINE | ID: mdl-28650277

Objective Sinus tachycardia is frequently reported in systemic lupus erythematosus (SLE), while there are limited data on post-exercise ability to slow heart rate (i.e. heart rate recovery, HRR) in this group of patients. Methods We studied consecutive 70 patients with SLE and 30 healthy controls. All examined individuals underwent detailed clinical examination, echocardiography, Holter monitoring with heart rate variability and treadmill stress test using Bruce's protocol. HRR values were calculated as the difference between maximum HR during exercise and HR at the first (HRR1) and third (HRR3) minute of rest. Individuals with coronary artery disease, diabetes mellitus and suspected pulmonary hypertension were excluded from further analysis ( n = 15). Results Fifty-five SLE patients were eligible for this study: aged 41.5 ± 12.4 years, 87.3% women, SLICC/ACR-DI score 3.58 ± 1.85. In the SLE group 36.4% patients received beta-blockers, usually for previously detected sinus tachycardia and/or arterial hypertension. Mean HRR1 (36.9 ± 12.6 vs 49.5 ± 18.6, p = 0.0004) and HRR3 (55.5 ± 14.3 vs 69.2 ± 16.4, p = 0.0001) were significantly lower in SLE than in healthy individuals. Significantly negative correlations between SLICC/ACR-DI score and HRR1 ( r = -0.299, p = 0.01), HRR3 ( r = -0.361, p = 0.001) and exercise capacity ( r = -0.422, p < 0.0001) were revealed. Additionally, beta-blocker treatment was also revealed to alter significantly HRR1, HRR3 and exercise capacity in SLE. Conclusion Patients with SLE are characterized by attenuated HRR after exercise. In our study impaired HRR was associated with disease severity and beta-blocker treatment and probably with disease duration. The use of HRR assessment in SLE can be used as an additional marker of cardiac autonomic nervous system dysfunction.


Adrenergic beta-Antagonists/adverse effects , Heart Rate/drug effects , Lupus Erythematosus, Systemic/physiopathology , Severity of Illness Index , Adult , Autonomic Nervous System/physiopathology , Echocardiography/methods , Electrocardiography, Ambulatory/methods , Exercise Test/methods , Exercise Tolerance/physiology , Female , Heart/diagnostic imaging , Heart/physiopathology , Heart Rate/physiology , Humans , Hypertension/drug therapy , Hypertension/physiopathology , Male , Middle Aged , Prospective Studies , Stroke Volume/physiology , Tachycardia, Sinus/drug therapy , Tachycardia, Sinus/physiopathology
3.
Transplant Proc ; 48(5): 1399-401, 2016 Jun.
Article En | MEDLINE | ID: mdl-27496414

BACKGROUND: In Poland the potential for organ donation from donation after circulatory death (DCD) donors is not known. This assessment will allow the hospital to create a quality organ harvesting system from this group of donors. AIMS: The aim of this study was to assess the DCD potential based on retrospective analysis of hospital deaths at Hospital Infant Jesus in Warsaw. METHODS: Documentation of 718 deceased patients from 2010 to 2014 was analyzed. This population could be classified as potential DCD donors in IIb category. The deceased's characteristics were analyzed while undergoing the qualification process for DCD. The analysis was to confirm the presence or absence of factors disqualifying kidneys from donation and transplantation. Data from particular departments and from the entire hospital were analyzed. RESULTS: The total number of deaths was 718. Excluding factors from the DCD donation process were found in 664 cases (92%), mainly age >60 and concomitant diseases. The rest of the patients (n = 54.8%) did not have factors that would exclude DCD donation. Group characteristics are given in detail. SUMMARY: The attempt to measure donation potential was done at the Hospital of the Infant Jesus in Warsaw, a large, multispecialty hospital with intensive organ donation and transplantation programs. Results show a potential for DCD donation (54 potential donations over the last 4years), which allows us to create a quality system and algorithms for organ donation after circulation death.


Kidney Transplantation , Tissue Donors/supply & distribution , Tissue and Organ Procurement/organization & administration , Adult , Death , Female , Hospitals , Humans , Infant , Male , Middle Aged , Poland , Retrospective Studies
4.
J Thromb Haemost ; 14(1): 121-8, 2016 Jan.
Article En | MEDLINE | ID: mdl-26509468

UNLABELLED: Essentials Predicting chronic thromboembolic pulmonary hypertension (CTEPH) after pulmonary embolism is hard. We studied 772 patients with pulmonary embolism who were followed for CTEPH (incidence 2.8%). Logistic regression analysis revealed 7 easily collectable clinical variables that combined predict CTEPH. Our score identifies patients at low (0.38%) or higher (10%) risk of CTEPH. SUMMARY: Introduction Validated risk factors for the diagnosis of chronic thromboembolic pulmonary hypertension (CTEPH) after acute pulmonary embolism (PE) are currently lacking. Methods This is a post hoc patient-level analysis of three large prospective cohorts with a total of 772 consecutive patients with acute PE, without major cardiopulmonary or malignant comorbidities. All underwent echocardiography after a median of 1.5 years. In cases with signs of pulmonary hypertension, additional diagnostic tests to confirm CTEPH were performed. Baseline demographics and clinical characteristics of the acute PE event were included in a multivariable regression analysis. Independent predictors were combined in a clinical prediction score. Results CTEPH was confirmed in 22 patients (2.8%) by right heart catheterization. Unprovoked PE, known hypothyroidism, symptom onset > 2 weeks before PE diagnosis, right ventricular dysfunction on computed tomography or echocardiography, known diabetes mellitus and thrombolytic therapy or embolectomy were independently associated with a CTEPH diagnosis during follow-up. The area under the receiver operating charateristic curve (AUC) of the prediction score including those six variables was 0.89 (95% confidence interval [CI] 0.84-0.94). Sensitivity analysis and bootstrap internal validation confirmed this AUC. Seventy-three per cent of patients were in the low-risk category (CTEPH incidence of 0.38%, 95% CI 0-1.5%) and 27% were in the high-risk category (CTEPH incidence of 10%, 95% CI 6.5-15%). Conclusion The 'CTEPH prediction score' allows for the identification of PE patients with a high risk of CTEPH diagnosis after PE. If externally validated, the score may guide targeting of CTEPH screening to at-risk patients.


Hypertension, Pulmonary/diagnosis , Predictive Value of Tests , Pulmonary Embolism/diagnosis , Thromboembolism/diagnosis , Adult , Aged , Area Under Curve , Echocardiography , Female , Follow-Up Studies , Humans , Hypertension, Pulmonary/complications , Hypertension, Pulmonary/epidemiology , Incidence , Male , Middle Aged , Multivariate Analysis , Outpatients , Pulmonary Embolism/complications , Pulmonary Embolism/epidemiology , ROC Curve , Regression Analysis , Risk Factors , Sensitivity and Specificity , Thromboembolism/complications , Thromboembolism/epidemiology
6.
Scand J Rheumatol ; 44(5): 385-8, 2015.
Article En | MEDLINE | ID: mdl-25928303

OBJECTIVES: Systemic sclerosis (SSc) leads to pulmonary circulation dysfunctionand there are some indications of systemic circulation impairment. We evaluated the influence of SSc on the elastic properties of large systemic arterial walls and potential correlations between systemic and pulmonary circulation involvement. METHOD: We examined 75 consecutive women (mean age 53.13±10.1 years) with confirmed SSc [mean disease duration (DD) 7.1±9.1 years] and 21 age-matched female volunteers (mean age 52.6±8.3 years, ns). Pulse wave velocity (PWV) and transthoracic echocardiography were performed. SSc patients were divided into two groups according to the median of DD: ≤3 years (39 patients) and >3 years (36 patients). RESULTS: Patients with DD>3 years had higher PWV than those with DD≤3 years and controls (log PWV: 2.23±0.23 vs. 2.13±0.16 and vs. 2.11±0.16 m/s; p=0.028 and 0.029, respectively). In addition, echocardiographic indices showed impaired right ventricular (RV) function in the patients with DD>3 years. Also in these SSc patients, PWV correlated with clinical and echocardiographic parameters of pulmonary circulation: age (r=0.64, p<0.0001), acceleration time of pulmonary ejection (AcT; r=-0.38, p=0.021), and tricuspid regurgitation peak gradient (TRPG; r=0.34, p=0.04). Multiple linear regression analysis showed that PWV was independently associated with DD (ß=0.22, p==0.02), AcT (ß=-0.215, p=0.03), and age (ß=0.44, p<0.001). CONCLUSIONS: In patients with SSc lasting more than 3 years, the disease is characterized by increased stiffness of the large systemic arteries. Longer duration of SSc leads simultaneously to the increased stiffness of the large systemic arteries and to the progressive impairment of RV function and its coupling to the pulmonary arterial bed.


Arteries/physiopathology , Pulmonary Artery/physiopathology , Scleroderma, Systemic/physiopathology , Vascular Remodeling/physiology , Adult , Arteries/diagnostic imaging , Case-Control Studies , Echocardiography , Elasticity/physiology , Female , Humans , Hypertension/epidemiology , Incidence , Middle Aged , Pulmonary Artery/diagnostic imaging , Scleroderma, Systemic/diagnostic imaging , Ventricular Dysfunction, Right/epidemiology
8.
Respir Physiol Neurobiol ; 209: 69-75, 2015 Apr.
Article En | MEDLINE | ID: mdl-25447676

Systemic sclerosis (SSc) is an autoimmune disorder characterized by skin and internal organs fibrosis and concomitant vascular abnormalities. Although SSc is considered mainly fibrosing disease, underlying vascular pathology plays a fundamental role in its pathogenesis. We have focused on positive and negative serum markers of angiogenesis and fibrosis (pigment epithelium-derived factor [PEDF], vascular endothelial growth factor [VEGF], and soluble VEGF receptor [sVEGFR]), in progressive SSc patients at baseline and after follow-up in relation to cardiopulmonary complications (systemic hypertension [HT], pulmonary arterial hypertension [PAH] and pulmonary fibrosis [PF]). VEGF and PEDF but not sVEGFR were reciprocally regulated in SSc progression. Moreover, VEGF/PEDF ratio significantly increased during follow up suggesting that it might be used as a biomarker of disease progression. No correlation between the studied markers and cardiopulmonary complications was observed. In conclusion, VEGF and PEDF level, and the VEGF/PEDF ratio are significantly changed in the course of SSc progression and these markers can be used to assess SSc activity.


Eye Proteins/blood , Hypertension/blood , Nerve Growth Factors/blood , Pulmonary Fibrosis/blood , Scleroderma, Systemic/blood , Serpins/blood , Vascular Endothelial Growth Factor A/blood , Adult , Aged , Biomarkers/blood , Blood Pressure/physiology , Carbon Monoxide/metabolism , Enzyme-Linked Immunosorbent Assay , Female , Follow-Up Studies , Humans , Hypertension/complications , Lung/metabolism , Male , Middle Aged , Pulmonary Fibrosis/complications , Scleroderma, Systemic/complications , Young Adult
9.
Transplant Proc ; 46(8): 2719-23, 2014 Oct.
Article En | MEDLINE | ID: mdl-25380902

BACKGROUND: Left ventricular hypertrophy (LVH) is a significant risk factor for cardiovascular complications in renal transplant recipients. The study objective here was to assess LVH and related factors in renal transplant recipients in the 1st year after transplantation. METHODS: Echocardiographic examinations were performed in the early post-transplantation period in 43 patients (age, 43.9 ± 12.4 years; male, 53.5%) and at 1 year after transplantation in 40 patients. At the same time, basic blood tests, N-terminal pro-B-type natriuretic peptide (NT-proBNP) level tests, and ambulatory blood pressure measurements were performed. LVH was diagnosed when LV mass index was >95 g/m(2) in women and >115 g/m(2) in men. Statistical analyses were performed with the use of the R Package. RESULTS: LVH (mainly concentric) was found in 51.2% of the patients in the early period and in 50% of the patients at 1 year. In 30% of the patients with baseline LVH it regressed at 1 year and in another 30% LVH developed de novo. In the early period, LV mass was influenced by age, sex, body mass index (BMI), estimated glomerular filtration rate (eGFR), and a history of cardiovascular disorders during dialysis therapy, whereas at 1 year after transplantation it was influenced by age, sex, BMI, 24-hour systolic blood pressure, a history of hypertension during dialysis therapy, and abnormal 24-hour blood pressure profile. Weight gain interfered with LVH regression during the 1st year after transplantation, whereas no improvement in blood pressure control contributed to de novo development of LVH. All other patients (those without LVH) had a morphologic abnormality of the left ventricle, the so-called concentric remodeling. Higher NT-proBNP levels were observed in patients with LVH. CONCLUSIONS: LVH is present in one-half of renal transplant recipients in the 1st year after transplantation, and concentric remodeling is present in the remaining patients of this group. An echocardiographic examination is indicated in every renal transplant recipient. Measurements of NT-proBNP levels are helpful in LVH diagnostics.


Hypertrophy, Left Ventricular/etiology , Kidney Failure, Chronic/surgery , Kidney Transplantation , Postoperative Complications/etiology , Adult , Female , Humans , Hypertrophy, Left Ventricular/diagnostic imaging , Hypertrophy, Left Ventricular/epidemiology , Male , Middle Aged , Postoperative Complications/diagnostic imaging , Postoperative Complications/epidemiology , Risk Factors , Treatment Outcome , Ultrasonography
10.
Adv Exp Med Biol ; 756: 147-53, 2013.
Article En | MEDLINE | ID: mdl-22836630

Although scleroderma is generally considered a fibrosing disease, it is now recognized that the underlying vascular pathology is playing a fundamental role in its pathogenesis. The present study was aimed at testing the prevalence of anti-endothelial cell antibodies (AECA) in systemic scleroderma (SSc) patients with and without pulmonary hypertension (PH) and in relation to the presence of pulmonary fibrosis. Fifty four SSc patients (50 females and 4 male, mean age 55.7 ± 16.3 years) were prospectively screened. All patients underwent transthoracic echocardiography with the estimation of pulmonary artery pressure (PAP) and tricuspid regurgitant peak gradient (TRPG). All patients suspected to have pulmonary hypertension were referred for right heart catheterization. Restrictive lung disease was confirmed by HRCT. A healthy control group included (n = 27; 7 men and 20 women, mean age 49.8 ± 12.1 years). The study of AECA was performed using the indirect immunofluorescence method on commercially available human umbilical vein endothelial cells. The HRCT scans in patients with suspected interstitial lung disease revealed signs of lung fibrosis in 15 (out of the 36 examined patients). TRPG at rest of 31 mmHg was demonstrated in 14 (21%) patients. During cardiac catheterization, arterial PH was found in two patients. Resting venous PH was found in one patient and an excessive post capillary PAP elevation at rest was demonstrated in 11 patients. At the baseline, 14/54 patients (26%) were positive for AECA. In the control group, the frequency of the antibodies was 3/27 (11%). No statistical correlation between antibody titter and the presentation of the disease existed. AECA were highly prevalent in a subgroup of patients suffering from interstitial pulmonary fibrosis. Out of the 15 patients suffering from lung fibrosis, 7 were AECA positive. The presence of AECA correlated very well with antinuclear antibodies (ANA), but was not related to the profile of ANA. Our findings support evidence that endothelial cell damage is involved in SSc, as there was increased prevalence of circulating AECA of the IgG isotype in SSc patients. AECA may also be related to the complications of SSc, like pulmonary fibrosis.


Autoantibodies/blood , Endothelial Cells/immunology , Hypertension, Pulmonary/immunology , Pulmonary Fibrosis/immunology , Scleroderma, Systemic/immunology , Autoantibodies/immunology , Endothelium, Vascular/immunology , Female , Human Umbilical Vein Endothelial Cells , Humans , Lung Diseases, Interstitial/immunology , Male , Middle Aged
11.
J Thromb Haemost ; 8(4): 651-8, 2010 Apr.
Article En | MEDLINE | ID: mdl-20088923

OBJECTIVE: Current risk stratification in acute pulmonary embolism (APE) includes assessment of clinical status, right ventricular overload and plasma troponin concentrations. As impaired renal function is one of the important predictors of mortality in cardiovascular diseases, we hypothesized that it is an independent early mortality marker in APE. MATERIAL AND METHODS: In prospective cohort study, we observed 220 consecutive patients (86M/134F, 64 +/- 18 years) with APE proven by spiral computed tomography (CT). On admission, echocardiography was performed and blood samples were collected for troponin and creatinine assays. RESULTS: The calculated glomerular filtration rate (GFR) differed significantly between 81 pts with low-, 131 pts with moderate- and 8 pts with high-risk APE [71 (19-181) vs. 55 (9-153) vs. 41 (14-68) mL min(-1); respectively P < 0.0001]. Twenty-three patients died during the 30-day observation. Importantly, GFR was lower in non-survivors than in survivors [35 (9-92) vs. 63 (14-181) mL min(-1), P < 0.0001]. The area under the curve (AUC) of the GFR receiver-operating characteristic (ROC) curve for predicting mortality was 0.760 (95% CI: 0.698-0.815). In multivariable analysis, independent mortality predictors were GFR, troponin, heart rate and history of chronic heart failure. In normotensive patients, the GFR and cardiac troponins (cTn) ROC curves for prediction of mortality showed no difference (AUC 0.789 and 0.781, respectively). However, Kaplan-Meier analysis showed an additive prognostic value of renal dysfunction. Thus, troponin-positive patients with a GFR < or = 35 mL mn(-1) showed 48% 30-day mortality, whereas troponin-positive patients with a GFR > 35 mL mn(-1) had 11% mortality, and troponin-negative patients with a GFR > 35 mL mn(-1) had good prognosis, P < 0.0001. CONCLUSION: Impaired kidney function, present in 47% of APE patients, is related to all-cause mortality. In initially normotensive patients, a GFR < 35 mL min(-1) predicts 30-day mortality. Moreover, GFR assessment can improve troponin-based risk stratification of APE.


Glomerular Filtration Rate , Heart Diseases/mortality , Kidney Diseases/mortality , Kidney/physiopathology , Pulmonary Embolism/mortality , Troponin/blood , Acute Disease , Aged , Aged, 80 and over , Biomarkers/blood , Chi-Square Distribution , Creatinine/blood , Female , Heart Diseases/blood , Heart Diseases/diagnostic imaging , Heart Diseases/physiopathology , Hemodynamics , Hospitalization , Humans , Kaplan-Meier Estimate , Kidney Diseases/blood , Kidney Diseases/physiopathology , Male , Middle Aged , Prognosis , Proportional Hazards Models , Prospective Studies , Pulmonary Embolism/blood , Pulmonary Embolism/diagnostic imaging , Pulmonary Embolism/physiopathology , ROC Curve , Risk Assessment , Risk Factors , Time Factors , Tomography, Spiral Computed , Ultrasonography
12.
J Physiol Pharmacol ; 57 Suppl 4: 313-23, 2006 Sep.
Article En | MEDLINE | ID: mdl-17072060

Abnormalities of pulmonary gas exchange are common in patients with advanced liver disease. Since arterial blood hypoxemia is an important issue in the preoperative evaluation of liver transplant candidates, the study was undertaken to determine the incidence and severity of lung function impairment with a special emphasis on pulmonary gas exchange abnormalities in this group of patients. 104 consecutive patients (47 F and 57 M, mean age 46 +/-11 yr) listed for orthotopic liver transplantation participated in this prospective study. All patients underwent evaluation including: clinical assessment (Child-Pough and MELD classification), chest X-ray, chest sonography, lung function tests, arterial blood gases measurement, and transthoracic contrast enhanced echocardiography. There were 2 patients with acute hepatic failure, 6 patients with primary or metastatic liver carcinoma, and 96 patients with chronic liver disease. The mean PaO(2) and lung function parameters for the entire group were within normal limits. There were 29 hypoxemic patients (PaO(2)< 80 mmHg) and 40 patients with widened (>20 mmHg) P(A-a)O(2). DL(CO) was significantly lower in cirrhotic vs. non-cirrhotic patients (76.5 +/-19.3 vs. 92.4 +/-19.0% predicted; P<0.001). Hepatopulmonary syndrome (HPS) was recognized in 23 (24%) patients. 91% of patients with HPS showed mild to moderate stage of disease. There were significant difference between differences HPS patients and non-HPS patients in DL(CO) (69.0 +/-14.5 vs. 83.5 +/-20.7, P<0.01). In conclusion, all patients referred for OLT should be screened for gas exchange abnormalities. Such a workup should include not only PAO(2) but also DL(CO) and P(A-a)O(2) measurement together with contrast enhanced echocardiography.


Hepatopulmonary Syndrome/blood , Liver Diseases/blood , Liver Transplantation , Pulmonary Gas Exchange , Adult , Blood Gas Analysis , Echocardiography , Female , Hepatopulmonary Syndrome/diagnostic imaging , Hepatopulmonary Syndrome/physiopathology , Humans , Liver Diseases/diagnostic imaging , Liver Diseases/physiopathology , Lung/diagnostic imaging , Lung/physiopathology , Male , Middle Aged , Radiography , Respiratory Function Tests
13.
Int J Cardiol ; 102(2): 207-10, 2005 Jul 10.
Article En | MEDLINE | ID: mdl-15982486

BACKGROUND: Emery-Dreifuss muscular dystrophy (EDMD) characterized by musculoskeletal abnormalities is often associated with atrioventricular conduction disturbances. Although some EDMD patients were reported to develop dilated cardiomyopathy, there are limited data on their left ventricular (LV) performance. METHODS: Therefore, we echocardiographically assessed 27 men (23 cases aged 26.4+/-6.8 years with X-linked, and four cases aged 22.2+/-8.6 years with autosomal dominant (AD)) EDMD. Control group included 16 male healthy controls aged 24.8+/-6.0 (18-37) years. RESULTS: Although LV end diastolic dimension was similar in EDMD and controls (4.9+/-0.6 and 4.99+/-1.1 cm, ns), dilated left ventricle was found in three X-linked EDMD subjects. LV ejection fraction was significantly reduced in EDMD (62.3+/-1% vs. 71.2+/-2%, p=0.01) and was below 50% in six (22.2%) X-linked EDMD patients. Doppler analysis disclosed prolonged isovolumetric relaxation time of the left ventricle in the studied group. This finding may indicate impaired LV relaxation. CONCLUSION: A significant subgroup of X-linked EDMD patients shows pronounced abnormalities of left ventricular function. This warrants cardiologic follow up of EDMD patients.


Cardiomyopathy, Dilated/diagnostic imaging , Echocardiography, Doppler , Heart Ventricles/diagnostic imaging , Muscular Dystrophy, Emery-Dreifuss/complications , Ventricular Function, Left/physiology , Adolescent , Adult , Cardiomyopathy, Dilated/etiology , Cardiomyopathy, Dilated/physiopathology , Chromosomes, Human, X/genetics , Disease Progression , Heart Ventricles/physiopathology , Humans , Male , Muscular Dystrophy, Emery-Dreifuss/genetics , Myocardial Contraction/physiology , Stroke Volume/physiology
15.
Clin Cardiol ; 27(12): 693-7, 2004 Dec.
Article En | MEDLINE | ID: mdl-15628112

BACKGROUND AND HYPOTHESIS: Hemodynamic and functional consequences of acute pulmonary embolism (APE) are believed to be reversible with antithrombotic treatment. To verify this hypothesis, we reassessed our patients at least 1 year after an episode of APE. METHODS: We compared echo Doppler indices and 6-min walking test parameters (6-MWT) of 36 patients (13 men, 23 women, age 66 +/- 11 years), studied on average 3.1 +/- 2.2 years after an acute episode of pharmacologically treated massive or submassive APE, with data of 30 age-matched subjects (12 men, 18 women, age 67 +/- 12 years). RESULTS: At least 1 year after APE, right ventricular (RV) diameter remained increased in patients compared with controls (27 +/- 2 vs. 23 +/- 2 mm, p<0.001). Also, acceleration time of pulmonary ejection (AcT) was markedly shorter (97 +/- 19 vs. 123 +/- 19 ms, p<0.001) and the diameter of the pulmonary trunk was significantly larger in patients than in controls (21 +/- 2.6 vs. 18 +/- 2.2, p<0.001). Although the mean value of the tricuspid valve peak systolic gradient (TVPG) in the APE group at follow-up was similar to that in controls, TVPG>30 mmHg was recorded in three patients with APE (8.3%). There was no difference in the distance of 6-MWT between both groups; however, the mean desaturation after 6-MWT was higher in the APE group than in controls (3.04 +/- 2.08 vs. 1.45 +/- 0.69%, p=0.0005). CONCLUSIONS: Pharmacologic treatment of acute pulmonary embolism does not prevent mild persistent changes in morphology and function of the cardiovascular system. Despite normalization of pulmonary artery systolic pressure and similar exercise capacity, survivors of APE present signs suggesting RV dysfunction and/or its disturbed coupling to the pulmonary arterial bed, as well as ventilation to perfusion mismatch at exertion persisting long after the acute embolic episode.


Echocardiography, Doppler , Pulmonary Artery/physiopathology , Pulmonary Embolism/diagnostic imaging , Ventricular Dysfunction, Right/physiopathology , Acute Disease , Aged , Anticoagulants/therapeutic use , Blood Pressure/physiology , Disease Progression , Exercise Test , Female , Fibrinolytic Agents/therapeutic use , Follow-Up Studies , Heparin/therapeutic use , Humans , Male , Middle Aged , Pulmonary Artery/diagnostic imaging , Pulmonary Embolism/drug therapy , Pulmonary Embolism/physiopathology , Retrospective Studies , Streptokinase/therapeutic use , Time Factors , Tricuspid Valve/diagnostic imaging , Tricuspid Valve/physiopathology , Ventricular Dysfunction, Right/diagnostic imaging , Ventricular Dysfunction, Right/etiology , Ventricular Pressure/physiology
16.
Eur Respir J ; 22(4): 649-53, 2003 Oct.
Article En | MEDLINE | ID: mdl-14582919

Plasma brain natriuretic peptide (BNP), released from myocytes of ventricles upon stretch, has been reported to differentiate pulmonary from cardiac dyspnoea. Limited data have shown elevated plasma BNP levels in acute pulmonary embolism (APE), frequently accompanied by dyspnoea and right ventricular (RV) dysfunction. The aim of this study was to assess plasma N-terminal proBNP (NT-proBNP) in APE, and to establish whether it reflects the severity of RV overload and if it can be used to predict adverse clinical outcome. On admission, NT-proBNP and echocardiography for RV overload were performed in 79 APE patients (29 males), aged 63 +/- 16 yrs. Plasma NT-proBNP was elevated in 66 patients (83.5%) and was higher in patients with (median 4,650 pg x mL(-1) (range 61-60,958)) than without RV strain (363 pg x mL(-1) (16-16,329)). RV-to-left ventricular ratio and inferior vena cava dimension correlated with NT-proBNP. All 15 in-hospital deaths and 24 serious adverse events occurred in the group with elevated NT-proBNP, while all 13 (16.5%) patients with normal values had an uncomplicated clinical course. Plasma NT-proBNP predicted in-hospital mortality. Plasma N-terminal pro-brain natriuretic peptide is elevated in the majority of cases of pulmonary embolism resulting in right ventricular overload. Plasma levels reflect the degree of right ventricular overload and may help to predict short-term outcome. Acute pulmonary embolism should be considered in the differential diagnosis of patients with dyspnoea and abnormal levels of brain natriuretic peptide.


Nerve Tissue Proteins/blood , Peptide Fragments/blood , Pulmonary Embolism/blood , Pulmonary Embolism/complications , Ventricular Dysfunction, Right/blood , Ventricular Dysfunction, Right/etiology , Acute Disease , Adult , Aged , Aged, 80 and over , Echocardiography , Female , Humans , Male , Middle Aged , Natriuretic Peptide, Brain , Predictive Value of Tests , Prognosis , Pulmonary Embolism/diagnosis , Severity of Illness Index , Ventricular Dysfunction, Right/diagnostic imaging
18.
Heart ; 85(6): 628-34, 2001 Jun.
Article En | MEDLINE | ID: mdl-11359740

OBJECTIVE: To assess the value of transoesophageal echocardiography (TOE) for diagnosing suspected haemodynamically significant pulmonary embolism and signs of right ventricular overload at standard echocardiography. METHODS: 113 consecutive patients (58 male; 55 female), mean (SD) age 53.6 (13.3) years, in whom there was clinical suspicion of pulmonary embolism and right ventricular overload on transthoracic echocardiography, underwent TOE in addition to routine diagnostic procedures to identify pulmonary artery thrombi. RESULTS: TOE revealed thrombi in 32 of 51 patients who had suspected acute pulmonary embolism and in 31 of 62 with suspected chronic pulmonary embolism. In one patient a pulmonary angiosarcoma rather than chronic pulmonary embolism was found at surgery. The diagnosis of pulmonary embolism was confirmed in 77 patients by scintigraphy, spiral computed tomography, angiography, or necropsy (reference methods). While TOE failed to provide a diagnosis of pulmonary embolism in 15 of these 77 patients, no false positive findings were reported (sensitivity 80.5%, specificity 97.2%). In 11 and 26 cases, respectively, the thrombi were confined to the left or right pulmonary artery. Bilateral thrombi were found in 25 patients. Mobile thrombi were observed only in acute pulmonary embolism (in 19 of 32 patients). No complications of TOE were noted. CONCLUSIONS: TOE permits visualisation of pulmonary arterial thrombi, confirming the diagnosis in the majority of patients with pulmonary embolism and right ventricular overload. This may be useful for prompt decision making in patients with haemodynamic compromise considered for thrombolysis or embolectomy.


Echocardiography, Transesophageal , Pulmonary Embolism/diagnostic imaging , Echocardiography , Female , Hemodynamics , Humans , Male , Middle Aged , Prospective Studies , Pulmonary Embolism/physiopathology , Sensitivity and Specificity
19.
Exp Clin Cardiol ; 6(4): 200-5, 2001.
Article En | MEDLINE | ID: mdl-20428259

OBJECTIVES: To assess factors related to the success of restoration and one-year maintenance of sinus rhythm in chronic (more than 48 h) nonrheumatic atrial fibrillation (AF). METHODS AND RESULTS: One hundred and fifty consecutive patients aged 62+/-9 years with AF lasting 123+/-254 days were evaluated clinically with transthoracic and transesophageal echocardiography before elective direct current cardioversion. Heart chamber dimensions and left ventricular ejection fraction were measured. The presence of left atrial thrombi and spontaneous echocardiographic contrast as well as flow velocities in the left atrial appendage were assessed. The first cardioversion was followed by standardized two-step antiarrhythmic treatment including a second cardioversion, if necessary. Twenty patients (13%) spontaneously reverted to sinus rhythm (S) during anticoagulation preceding cardioversion, 81 (54%) were successfully cardioverted (Y), and in 49 (33%) cardioversion failed initially (N). No differences were noted between the two latter groups. However, S patients had smaller left atria measured in the short and long axes (42+/-4 mm, P=0.05, and 53+/-7 mm, P=0.005, respectively) than both the Y (45+/-4 and 61+/-8 mm) and the N patients (46+/-4 and 61+/-8 mm). One-year follow-up was obtained in 95 patients: 64 (67%) were in sinus rhythm while 31 (33%) had AF. Again, no initial differences predicting the maintenance of sinus rhythm were found. CONCLUSIONS: Spontaneous reversion of AF seems more likely with smaller left atria. Echocardiography, including trans-esophageal echocardiography, is unlikely to identify patients in whom attempts to restore and maintain sinus rhythm will fail or succeed.

20.
Exp Clin Cardiol ; 6(4): 206-10, 2001.
Article En | MEDLINE | ID: mdl-20428260

It is suggested that transesophageal echocardiography (TEE), by detecting thromboemboli in the proximal parts of the pulmonary arteries, is useful in the diagnosis of pulmonary embolism. However, the data on visualization of the pulmonary arteries are limited. The extent of the pulmonary arteries that can be precisely visualized during biplane TEE was assessed in 51 consecutive patients (23 female, 28 male, aged 56.6+/-12.5 years) without structural heart disease. The main pulmonary artery and the right pulmonary artery were detected in 96.1% and 94.1% of patients, respectively. Although the proximal part of the left pulmonary artery was found in only 47.0% of patients, its distal part was visualized in 92.2%. During TEE, proximal parts of the lobar arteries on both sides were visualized in 88.2% of patients. Thus, the central pulmonary arteries including proximal parts of the lobar branches can be precisely visualized by biplane TEE in the majority of patients. Only the proximal part of the left pulmonary artery is difficult to assess.

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