The causes of death in Korean patients with systemic lupus erythematosus over 11 years

We investigated the causes of death and analyzed the prognostic factors in Korean systemic lupus erythematosus (SLE) patients. We evaluated 1010 patients with SLE who visited Seoul Saint Mary's Hospital from 1997-2007. Changing patterns in the causes of death were analyzed. Survival rate was calculated by the Kaplan-Meier method and the log-rank test. The risk factors for death were analyzed by multivariate logistic regression analysis. The 5-year survival rate was 97.8%. Over the period of the study, 59 deaths were observed. Among 44 patients who died in our hospital, the most common cause of death was infection (37.3%), with SLE-related death as the next most frequent cause (22.0%). In comparison with earlier data, the proportion of SLE-related deaths has fallen and the proportion of infections has risen. SLE-related death was the most frequent cause of early death, while infection was the most common cause of death in the overall population. In univariate analysis, damage related to SLE, cumulative glucocorticoid dose, mean glucocorticoid dose for 1?month before death, intravenous methylprednisolone therapy and cyclophosphamide treatment were associated with death (p?相似文献   

Prognostic factors and causes of death in Korean patients with idiopathic pulmonary fibrosis

The purpose of this study was to investigate the prognostic factors at initial presentation and the causes of death in Korean patients with idiopathic pulmonary fibrosis (IPF), which might be different report wise, in comparison to the western countries. A retrospective review of 88 patients (mean 60.3 years, 69 male) was carried out and they were diagnosed as IPF positive. After diagnosis, the survival rate was 57% and 41% for third and fifth year, respectively (mean follow-up 39.1 months). Mortality was closely correlated with severe dyspnea at presentation (Hazard Ratio [HR], 2.6 per grade; p=0.015), lower initial forced vital capacity (HR, 1.7 per 10% predicted; p=0.004) and lower initial diffusing capacity of the lung (HR, 1.5 per 10% predicted; p=0.033). Treatment with specific drugs was ineffective against the survival when compared with symptomatic supportive care. Thirty-four patients (68%) died of worsened respiratory failure, seven (14%) died of infection and only one patient showed cardiovascular death. In conclusion, our study suggests that the severity of dyspnea and lung function tests at the time of diagnosis are the predictive factors for the survival of patients with IPF. In comparison to the reports from western countries, we observed that respiratory failure and pulmonary infection were more frequent causes of death, while cardiovascular death was rare in Korean patients with IPF.     

Prognostic significance of atrial fibrillation in advanced heart failure. A study of 390 patients

BACKGROUND. Atrial fibrillation is common in advanced heart failure, but its prognostic significance is controversial. METHODS AND RESULTS. We evaluated the relation of atrial rhythm to overall survival and sudden death in 390 consecutive advanced heart failure patients. Etiology of heart failure was coronary artery disease in 177 patients (45%) and nonischemic cardiomyopathy or valvular heart disease in 213 patients (55%). Mean left ventricular ejection fraction was 0.19 +/- 0.07. Seventy-five patients (19%) had paroxysmal (26 patients) or chronic (49 patients) atrial fibrillation. Compared with patients with sinus rhythm, patients with atrial fibrillation did not differ in etiology of heart failure, mean pulmonary capillary wedge pressure on therapy, or embolic events but were more likely to be receiving warfarin and antiarrhythmic drugs and had a slightly higher left ventricular ejection fraction. After a mean follow-up of 236 +/- 303 days, 98 patients died: 56 (57%) died suddenly, and 36 (37%) died of progressive heart failure. Actuarial 1-year overall survival was 68%, and sudden death-free survival was 79%. Actuarial survival was significantly worse for atrial fibrillation than for sinus rhythm patients (52% versus 71%, p = 0.0013). Similarly, sudden death-free survival was significantly worse for atrial fibrillation than for sinus rhythm patients (69% versus 82%, p = 0.0013). By Cox proportional hazards model, pulmonary capillary wedge pressure on therapy, left ventricular ejection fraction, coronary artery disease, and atrial fibrillation were independent risk factors for total mortality and sudden death. For patients who had pulmonary capillary wedge pressure of less than 16 mm Hg on therapy, atrial fibrillation was associated with poorer 1-year survival (44% versus 83%, p = 0.00001); however, in the high pulmonary capillary wedge pressure group, atrial fibrillation did not confer an increased risk (58% versus 57%). CONCLUSIONS. Atrial fibrillation is a marker for increased risk of death, especially in heart failure patients who have lower filling pressures on vasodilator and diuretic therapy. Whether aggressive attempts to maintain sinus rhythm will reduce this risk is unknown.     

Left atrioventricular plane displacement predicts cardiac mortality in patients with chronic atrial fibrillation

AIM: The aim of the present study was to investigate if left atrioventricular plane displacement (AVPD) has a prognostic value in patients with atrial fibrillation. METHODS AND RESULTS: Left AVPD was assessed by two-dimensionally guided M-mode echocardiography in the four- and two-chamber views in 160 consecutive patients with chronic atrial fibrillation, who were followed up with regard to mortality for an average of 45 months. All-cause mortality during follow-up was 49% (n=78). AVPD was lower in patients who died compared to those who survived: 6.6+/-1.7 versus 7.5+/-1.7 mm, P=0.0005. In 49 patients (31%), death was due to chronic heart failure or acute myocardial infarction. Among those who died of cardiac events, AVPD was 6.3+/-1.6 mm, versus 7.1+/-1.8 mm among those who died of other causes, P=0.0001. In multiple logistic regression analysis, AVPD (P=0.005), age (P=0.0005), and a history of chronic heart failure (P=0.004) correlated independently with mortality. CONCLUSION: Left AVPD was clearly decreased in patients with atrial fibrillation. The decrease was most pronounced in patients who died of cardiac events, whereas it did not differ significantly between those who died of non-cardiac causes and those who survived. The discriminative value of left AVPD was limited.     

Tissue plasminogen activator on admission is an important predictor of 30-day mortality in patients with acute myocardial infarction undergoing primary angioplasty

Tissue plasminogen activator (tPA) is emerging as an important risk factor for coronary heart disease, but the association between tPA concentration and mortality in acute myocardial infarction (AMI) remains uncertain. We studied the relationship between tPA antigen level on admission and 30-day mortality in 226 consecutive patients with AMI undergoing primary angioplasty. Death within 30 days occurred in 13 patients (5.7%). The concentration of tPA was significantly higher among the 13 patients who died than among the 213 who survived (26.5+/-16.3 versus 12.5+/-8.5 ng/mL, p<0.001). Compared with those in the lowest quartile (<9 ng/mL), patients in the highest quartile (>16 ng/mL) had a relative risk of subsequent death of 13.1 (p=0.014). In a Cox regression model, a tPA concentration >19 ng/mL was independently associated with mortality (HR 12.1, 95% CI, 1.9-76.7, p=0.001). This cutoff value had a 76.9% sensitivity and an 85.9% specificity for predicting 30-day mortality. tPA concentration was also higher in patients with severe heart failure (20.9+/-14.2 ng/mL versus 11.7+/-7.5 ng/mL, p=0.001) or ventricular tachyarrhythmia (24.3+/-13.9 ng/mL versus 12.2+/-8.4 ng/mL, p=0.001). In conclusions, elevated tPA antigen at initial presentation in patients with AMI was associated with higher short-term risk of death, suggesting that tPA may be a useful prognostic biomarker for the early risk stratification of these patients.     

Fascicular conduction disturbances and ischemic heart disease: adverse prognosis despite coronary revascularization

In patients with ischemic heart disease, fascicular conduction disturbances are associated with increased mortality. This study reveals that increased mortality also exists for certain types of fascicular conduction disturbances after myocardial revascularization. In 227 consecutive patients undergoing bypass surgery, 24 had preoperative and an additional 52 developed at surgery a fascicular conduction disturbance. At 66 +/- 14 months of follow-up, 6 (4%) of 148 control patients without pre- or postoperative fascicular conduction disturbances had died from cardiac causes. Although right bundle branch block and left hemifascicular block were the most common form of fascicular conduction disturbance, only 1 of 55 of these patients died (p = NS). Mortality rates were much higher for patients with left bundle branch block or an intraventricular conduction defect; 8 (38%) of 21 died from cardiac causes (p less than 0.05). A high risk subgroup was identified by comparing 14 consecutive patients with left bundle branch block or an intraventricular conduction defect who survived more than 1 year postoperatively with 21 consecutive patients with these same conduction defects who died within 1 year of surgery. The following variables were significantly (p less than 0.05) different (survivors versus nonsurvivors): age (58 +/- 7 versus 65 +/- 9 years); class IV angina (2 of 14 versus 16 of 21), prior myocardial infarction (9 of 14 versus 21 of 21), left ventricular ejection fraction (53 +/- 18 versus 41 +/- 15%), three vessel disease (9 of 14 versus 20 of 21) and left ventricular aneurysm (2 of 14 versus 13 of 21).(ABSTRACT TRUNCATED AT 250 WORDS)     

Relation of left ventricular function and prognosis in hypertrophic cardiomyopathy: an angiographic study

Left ventricular cineangiograms performed at the time of diagnosis in 88 patients with hypertrophic cardiomyopathy were digitized to evaluate the relation of left ventricular function and prognosis in hypertrophic cardiomyopathy. Eleven patients died suddenly after a mean follow-up period of 7.5 +/- 7 years, 10 patients died of congestive heart failure or after cardiac surgery and 67 were alive after a mean follow-up period of 8.6 +/- 4 years. Measurements of left ventricular volume, ejection fraction, peak rate of ejection and filling and time to peak rate of ejection and filling were derived from curves of ventricular volume and its rate of change during the cardiac cycle. Patients who died suddenly had a lower peak rate of ventricular ejection (stroke volume-normalized peak ejection rate 5.41 +/- 0.69 versus 6.24 +/- 1.33 s-1; p = 0.006) and lower peak rate of ventricular filling (end-diastolic volume-normalized peak filling rate 4.02 +/- 0.94 versus 4.88 +/- 1.53 s-1; p = 0.02) and stroke volume-normalized peak filling rate (4.75 +/- 1.08 versus 5.82 +/- 1.70 s-1; p = 0.01) compared with survivors. Stepwise regression analysis revealed that sudden death was best predicted by the combination of increased end-diastolic volume, small end-systolic volume and low peak filling rate (predictive accuracy 32%, false negative 18% and false positive 28%). The addition of clinical features and hemodynamic measurements to the analysis improved predictive accuracy to 43% (false negative 18% and false positive 18%). Ambulatory electrocardiographic monitoring performed in 57 of the 88 patients 1 month to 17 years (median 8 years) after diagnosis revealed ventricular tachycardia in 14 (25%). Of these, 10 who survived had hyperkinetic systolic function at diagnosis, whereas the 4 who died suddenly had impaired systolic function (end-diastolic volume-normalized peak ejection rate 5.93 +/- 1.2 versus 4.01 +/- 1.2 s-1, respectively; p = 0.04). In hypertrophic cardiomyopathy, ventricular tachycardia is a sensitive but nonspecific marker of adults who are at risk of sudden death. Impaired systolic function may be an important determinant of which patients with ventricular tachycardia die suddenly. This study shows that indexes of ventricular function contribute to the identification of patients at particular risk of sudden death. However, the predictive power of the clinical features and hemodynamic and angiographic measurements that could be assessed was poor.(ABSTRACT TRUNCATED AT 400 WORDS)     

Activated, cytotoxic CD8(+) T lymphocytes contribute to the pathology of asthma death

This study investigates the presence of CD8(+) T lymphocytes and their possible association with viral infection in bronchi of victims of fatal asthma. Postmortem samples from the peribronchial region of the lung were obtained from seven patients who died an asthma death (AD), seven asthmatic patients who died of unrelated causes (AUC), and seven postmortem cases with no history of lung disease (control subjects). Using immunohistochemical techniques, the CD8(+) cytotoxic T-cell population in peribronchial tissue was characterized in three patient groups. The percentage of CD8(+) cells expressing the activation marker CD25 was higher in the AD group than in both the AUC and control groups (11.91 +/- 1.92% versus 3.93 +/- 1.63% and 1.09 +/- 0.56%, respectively (p < 0.001). Perforin expression, a marker of cytotoxicity, was highest in the AD group (9.16 +/- 1.5%) compared with 1.39 +/- 0.9; 1.8 +/- 0.6% in the AUC and control groups respectively (p < 0.001). Expression of interleukin-4 (IL-4) and interferon gamma (IFN-gamma) by CD8(+) T cells was higher in the AD group than the control group (p < 0.05). Furthermore, the IFN-gamma/IL-4 ratio in the AD group was less than half that of the control group (1.46 +/- 0.2 versus 3.2 +/- 0.1; p = 0.02). Using polymerase chain reaction (PCR), viral genome for rhinovirus (RV) was detected in lung tissue from three of the seven cases in the AD group. Two of these cases also had detectable respiratory syncytial virus (RSV). Viral genome for RSV was detected in five of the AUC group and in one of these cases, RV was also detected. No viral genome was detected in the lungs of the control group. In conclusion, this study provides novel evidence of an aberrant CD8(+) T-cell population, possibly in response to viral infection in subjects who die of acute asthma.     

Prognostic value of BNP for hospital mortality in elderly subjects hospitalised for acute diastolic cardiac failure

The aim of this study was to evaluate the prognostic value of BNP in elderly patients hospitalised for acute diastolic cardiac failure. 108 consecutive subjects were included, aged at least 70 years old, hospitalised for isolated acute diastolic cardiac failure. All of them had a left ventricular ejection fraction > or = 50% and evidence of diastolic dysfunction on echocardiography performed shortly after admission. The plasma BNP concentration measured in the emergency department on admission was >100 pg/ml in all of the patients except five. It was positively correlated with age (R = 0.29, p = 0.002), with the plasma creatinine level (R = 0.37, p < 0.0001) and the plasma urea level (R = 0.41, p < 0.0001). On univariate analysis, compared to the patients who survived, the 20 patients who died before discharge were significantly older (88.6 versus 84.4 years, p = 0.01), and were more often residents of a care home (60 versus 31%. p = 0.02), had a lower systolic blood pressure on admission (127 +/- 33 versus 154 +/- 30 mm Hg), a higher plasma urea level (16.8 +/- 12 versus 8.9 +/- 5 mmol/l, p = 0.002) and a higher BNP (median = 1290 pg/ml, interquartile range: 721, 3026 pg/ml versus 430 pg/ml, interquartile range: 243, 886 pg/ml). On multivariate analysis, the only factors that remained significantly associated with mortality were the BNP levels (p = 0.005) and the systolic blood pressure (p = 0.01). The negative predictive value of a BNP level < 631 pg/ml (median) for death was 94% (95% confidence interval: 91 to 97%). We conclude that BNP does have an independent prognostic value for in-hospital death in elderly subjects with acute diastolic cardiac failure.     

BNP in septic patients without systolic myocardial dysfunction

BACKGROUND: We tested our hypothesis that serum BNP levels rise in sepsis and septic shock patients as a result of an inflammatory state and not only because of left ventricular dysfunction. METHODS: Twenty-one patients with sepsis or septic shock were enrolled in the study. Echocardiography was performed in every patient on admission and at discharge. Laboratory data were evaluated on admission, during hospitalization, and at discharge. Serum IL-1beta, IL-6, TNFalpha, and BNP concentrations were determined. RESULTS: BNP values on admission (r=0.47, p=0.03), during hospitalization (r=0.64, p=0.014), and on the day of discharge (r=0.54, p=0.015) were all positively correlated with CRP values. Mean BNP (r=0.07, p=0.006) and BNP level at discharge (r=0.68, p=0.001) were also positively associated with IL-1 at discharge. Mean CRP (17.7 mg/dL+/-1.5 vs. 9.2 mg/dL+/-3.6, p=0.002), IL-6 (46.6 pg/mL+/-2.2 vs. 25.6 pg/mL+/-16.3, p=0.003), and SAPS II levels (41.3+/-4.7 vs. 33.9+/-6.5 p=0.01) were also higher in patients who died versus those who survived. No difference in BNP levels was recorded in subjects who died versus those who survived. There was no clinical or echocardiographic evidence of left ventricular systolic dysfunction (mean EF% on admission 55.1+/-21.7 vs. 61.3+/-8.6 on discharge, p=0.123). Serum BNP levels at discharge were inversely associated with EF values on admission (r=-0.475, p=0.046) and positively associated with E/A ratio on admission (r=0.565, p=0.028). No association was found between BNP values and death. CONCLUSION: BNP is positively correlated with CRP levels in septic patients without clinical or echocardiographic evidence of systolic dysfunction. No association was found between death and BNP values. It seems that, in septic patients, BNP is less accurate as a measure of ventricular dysfunction.     

Plasma brain natriuretic peptide as a prognostic indicator in patients with primary pulmonary hypertension

BACKGROUND: Plasma brain natriuretic peptide (BNP) level increases in proportion to the degree of right ventricular dysfunction in pulmonary hypertension. We sought to assess the prognostic significance of plasma BNP in patients with primary pulmonary hypertension. METHODS AND RESULTS: Plasma BNP was measured in 60 patients with primary pulmonary hypertension at diagnostic catheterization, together with atrial natriuretic peptide, norepinephrine, and epinephrine. Measurements were repeated in 53 patients after a mean follow-up period of 3 months. Forty-nine of the patients received intravenous or oral prostacyclin. During a mean follow-up period of 24 months, 18 patients died of cardiopulmonary causes. According to multivariate analysis, baseline plasma BNP was an independent predictor of mortality. Patients with a supramedian level of baseline BNP (> or = 150 pg/ml) had a significantly lower survival rate than those with an inframedian level, according to Kaplan-Meier survival curves (p < 0.05). Plasma BNP in survivors decreased significantly during the follow-up (217 +/- 38 to 149 +/- 30 pg/ml, p < 0.05), whereas that in nonsurvivors increased (365 +/- 77 to 544 +/- 68 pg/ml, p < 0.05). Thus, survival was strikingly worse for patients with a supramedian value of follow-up BNP (> or = 180 pg/ml) than for those with an inframedian value (p < 0.0001). CONCLUSIONS: A high level of plasma BNP, and in particular, a further increase in plasma BNP during follow-up, may have a strong, independent association with increased mortality in patients with primary pulmonary hypertension.     

Is banding of the pulmonary trunk obsolete for infants with tricuspid atresia and double inlet ventricle with a discordant ventriculoarterial connection? Role of aortic arch obstruction and subaortic stenosis.

Banding the pulmonary trunk may exacerbate or promote the development of subaortic stenosis in patients with double inlet ventricle or tricuspid atresia with a dominant left ventricle and discordant ventriculoarterial connection and, therefore, may be an inappropriate palliative procedure for such patients. To examine this possibility, 102 consecutive infants were studied who presented with this anatomy between 1972 and 1987. Obstruction of the aortic arch was present in 52 patients. In 28 patients (17 with aortic arch obstruction), subaortic stenosis was already apparent at presentation. Of the remaining 74 patients, 19 received no palliative surgery and 55 underwent banding of the pulmonary trunk either with (n = 22) or without (n = 33) aortic arch repair. Outcome was significantly worse in patients with associated aortic arch obstruction. All such patients either died or developed subaortic stenosis by 3 years of age (survival free of subaortic stenosis 0 of 22 versus 22 of 33 for patients with isolated banding of the pulmonary trunk, p less than 0.001). After isolated banding, there was a lower ratio of the ventricular septal defect to ascending aorta diameters at presentation in the patients who developed subaortic stenosis than in the patients who did not (0.60 +/- 0.08 versus 1.03 +/- 0.15, p less than 0.001). Of the latter, 18 (95%) of 19 patients fulfilled criteria for a Fontan procedure at recatheterization. Thus, the presence of aortic arch obstruction is associated with rapid development of subaortic stenosis after banding of the pulmonary trunk. Alternative initial surgery, even though high risk, may be indicated. In the absence of such obstruction, banding the pulmonary trunk can be performed at reasonable risk and, provided that the ventricular septal defect is of adequate size, satisfactorily prepares most patients for a later Fontan procedure.     

Atrial mechanical remodeling and new onset atrial fibrillation in chronic Chagas' heart disease

Atrial fibrillation (AF) is a common arrhythmia, mechanistically linked to underlying heart disease. AF affects about one fifth of subjects with Chagas' heart disease and is a harbinger of poor prognosis. In a retrospective longitudinal analysis, 50 subjects were investigated in long-term follow-up for the first documented atrial fibrillation (AF) episode. During a follow-up of (mean+/-SD) 84.2+/-39.0 months, nine subjects developed AF (incidence: 3.3+/-1.0%/year). Five subjects had nonfatal embolic stroke and nine died due to cardiac causes. The relative risk of AF for stroke was 3.0 (p=0.22) and for cardiac death was 3.6 (p=0.04). A faster left atrial diameter (LAD) enlargement during follow-up was tracked in subjects with more severe cardiac damage at presentation, and large LAD was detected at both presentation (p=0.02) and end of follow-up (p=0.002) in subjects who experienced AF. Atrial remodeling in chronic Chagas' disease is associated with severity of underlying heart disease at presentation and impacts AF incidence in this population.     

Role of glucocorticoids on inflammatory response in nonimmunosuppressed patients with pneumonia: a pilot study.

The aim of the study was to assess the potential role of glucocorticoids (GC) in modulating systemic and pulmonary inflammatory responses in mechanically ventilated patients with severe pneumonia. Twenty mechanically ventilated patients with pneumonia treated at a respiratory intensive care unit (RICU) of a 1,000-bed teaching hospital were prospectively studied. All patients had received prior antimicrobial treatment. Eleven patients received GC (mean+/-SD dose of i.v. methylprednisolone 677+/-508 mg for 9+/-7 days), mainly for bronchial dilatation. Serum and bronchoalveolar lavage fluid (BALF) tumour necrosis factor (TNF)-alpha, interleukin (IL)-1beta, IL-6 and C-reactive protein levels were measured in all patients. The inflammatory response was attenuated in patients receiving GC, both systemically (IL-6 1,089+/-342 versus 630+/-385 pg x mL(-1), p=0.03; C-reactive protein 34+/-5 versus 19+/-5 mg x L(-1), p=0.04) and locally in BALF (TNF-alpha 118+/-50 versus 24+/-5 pg x mL(-1), p= 0.05; neutrophil count: 2.4+/-1.1 x 10(9) cells x L(-1) (93+/-3%) versus 1.9+/-1.8 x 10(9) cells x L(-1) (57+/-16%), p=0.03). Four of the 11 (36%) patients receiving GC died compared to six (67%) who were not receiving GC (p=0.37). The present pilot study suggests that glucocorticoids decrease systemic and lung inflammatory responses in mechanically ventilated patients with severe pneumonia receiving antimicrobial treatment.     

Prognosis of spontaneous echocardiographic contrast in the thoracic aorta

The aim of this study was to assess the predictive value of spontaneous echocardiographic contrast (SEC) detected in the thoracic aorta by transesophageal echocardiography (TEE) on intermediate-term cardiovascular morbidity and mortality. We studied 299 consecutive patients (aged 61 +/- 13 years) without aortic aneurysm or dissection, who underwent TEE in 1995 to 1996. Cardiovascular deaths and nonfatal events were recorded over a period of < or = 60 months. Left ventricular function was classified as preserved versus depressed according to ejection fraction values (>40% vs < or = 40%) on 2-dimensional echocardiography. SEC was identified in 35 patients (11.7%). During follow-up, 66 patients died (36 deaths were due to cardiovascular causes; 10 and 26 cardiovascular deaths occurred in patients with and without SEC, respectively [p <0.001]). Survival time was significantly reduced in patients with versus without SEC (28 +/- 18 vs 39 +/- 19 months, p = 0.0012). Multivariate analysis revealed that the presence of SEC doubled the odds for cardiovascular death and for the combined end point of cardiovascular death and events. There was a significant difference in survival distributions between patients with and without SEC between both genders (p <0.001). In patients with normal or mildly reduced left ventricular function, SEC was predictive of an adverse outcome, whereas this was not the case in patients with more severely depressed cardiac function. It is concluded that the presence of SEC in the thoracic aorta is associated with a high risk of cardiovascular events and/or death over intermediate-term follow-up.     

Evolution of repaired and non-repaired tricuspid regurgitation in rheumatic mitral valve surgery without severe pulmonary hypertension

The aim of this study was to evaluate the usefulness of repairing significant tricuspid regurgitation (> or = grade 2) without severe pulmonary hypertension (< or = 50 mm Hg). Between 1993 and June 2001, 88 consecutive patients were operated on for rheumatic mitral valve disease associated with significant tricuspid regurgitation and without severe pulmonary hypertension. The severity of the tricuspid valve disease was assessed by echocardiography. Sixty-three patients had severe (> or = grade 3) tricuspid regurgitation (Group I), and 25 patients had moderate (grade 2) tricuspid regurgitation (Group II). There was no hospital mortality. six patients died during follow-up. The overall actuarial survival rate for 8 years was 92.1% +/- 3.1%. Cox proportional hazard regression analysis showed that age ( p = 0.006) and pulmonary complication ( p = 0.01) were associated with increased late mortality. Freedom from death was similar in both groups at 8 years (93.1% +/- 3.3% versus 88% +/- 8%, p = 0.7). Severe postoperative tricuspid regurgitation (> or = grade 3), caused by the failure of tricuspid repair or leaving the valve untouched, impaired long-term survival after surgery, and actuarial survival was 96.1% +/- 2.7% and 83% +/- 7.8% at 7 years ( p = 0.048), respectively. Severe tricuspid regurgitation, functional or organic, should be corrected at the time of mitral valve surgery, whereas untouched functional moderate tricuspid regurgitation improves after mitral valve surgery.     

Presentation, management and outcomes of thrombosis for children with cardiomyopathy

BACKGROUND: Thrombosis in children with dilated and inflammatory cardiomyopathy is an unpredictable complication with potentially important morbidity. OBJECTIVE: To determine the prevalence, associated factors, management and outcomes of thrombosis in this setting. METHODS: Data were obtained from review of medical records. Factors associated with thrombosis and the impact on outcome were sought. RESULTS: From 1990 to 1998, 66 patients that presented with dilated cardiomyopathy were followed for a median interval of 1.4 years (range 0 to 9.79 years) from first presentation. Thrombosis was diagnosed in four patients at presentation and in four patients during follow-up. Thrombosis was noted in one additional patient at examination after death. The overall nine-year period prevalence of thrombosis was 14%. Anticoagulation was started at presentation in 31% of patients. The mean left ventricular ejection fraction at presentation was significantly lower in those given anticoagulation (19+/-8%) versus those who were not (32+/-15%; P < 0.001). The mean ejection fraction at presentation was similar in those patients with (25+/-10%) versus those without thrombosis (28+/-15%; P = 0.44). During follow-up, 11 patients died and seven underwent cardiac transplantation. Kaplan-Meier estimates of freedom from death or transplantation were 88% at three months, 81% at one year and 70% at five years. Survival free of transplantation was not affected by thrombosis. CONCLUSIONS: Thrombosis is common in children with cardiomyopathy, can occur at any time in the patients' clinical course and is not related to clinical features or survival free of transplantation. The relevance and prevention of thrombosis in this setting remains unclear.     

Clinical features of amiodarone-induced pulmonary toxicity

The incidence and clinical predictors of amiodarone pulmonary toxicity were examined in 573 patients treated with amiodarone for recurrent ventricular (456 patients) or supraventricular (117 patients) tachyarrhythmias. Amiodarone pulmonary toxicity was diagnosed in 33 of the 573 patients (5.8%), based on symptoms and new chest radiographic abnormalities (32 of 33 patients) and supported by abnormal pulmonary biopsy (13 of 14 patients), low pulmonary diffusion capacity (DLCO) (nine of 13 patients), and/or abnormal gallium lung scan (11 of 16 patients). Toxicity occurred between 6 days and 60 months of treatment for a cumulative risk of 9.1%, with the highest incidence occurring during the first 12 months (18 of 33 patients). Older patients developed it more frequently (62.7 +/- 1.7 versus 57.4 +/- 0.5 years, p = 0.018), with no cases diagnosed in patients who started therapy at less than 40 years of age. Gender, underlying heart disease, arrhythmia, and pretreatment chest radiographic, spirometric, or lung volume abnormalities did not predict development of amiodarone pulmonary toxicity, whereas pretreatment DLCO was lower in the group developing it (76.0 +/- 5.5% versus 90.4 +/- 1.4%, p = 0.01). There was a higher mean daily amiodarone maintenance dose in the pulmonary toxicity group (517 +/- 25 versus 409 +/- 6 mg, p less than 0.001) but no difference in loading dose. No patient receiving a mean daily maintenance dose less than 305 mg developed pulmonary toxicity. Patients who developed toxicity had higher plasma desethylamiodarone (2.34 +/- 0.18 versus 1.92 +/- 0.04 micrograms/ml, p = 0.009) but not amiodarone concentrations during maintenance therapy.(ABSTRACT TRUNCATED AT 250 WORDS)     

Noninvasively assessed pulmonary artery stiffness predicts mortality in pulmonary arterial hypertension

AIMS: Decreased total compliance of the pulmonary vascular bed is associated with increased mortality in patients with pulmonary arterial hypertension (PAH). We investigated whether proximal pulmonary artery stiffness, in terms of area distensibility and noninvasively assessed relative area change (RAC), calculated as relative cross-sectional area change, predicts mortality in patients with PAH. METHODS AND RESULTS: Eighty-six subjects underwent right-heart catheterization and MRI to assess area distensibility and RAC. Patients were followed up to 48 months. Kaplan-Meier plot and Cox proportional hazards regression analyses assessed the predictive value of area distensibility and RAC. In 70 patients, the diagnosis PAH was confirmed, and 16 subjects served as control subjects. In comparison with control subjects, proximal pulmonary arteries of patients were distended (685 +/- 214 mm2 vs 411 +/- 153 mm2, p < 0.001), less distensible (area distensibility = 0.46 +/- 0.38.10(-2) mm Hg(-1) vs 3.69 +/- 1.96.10(-2) mm Hg(-1), p < 0.0001), and RAC was smaller (20 +/- 10% vs 58 +/- 21%, p < 0.0001) [mean +/- SD]. RAC showed an inverse curvilinear relation with mean pulmonary artery pressure (R2 = 0.47). Eighteen patients (26%) died because of cardiopulmonary causes. Patients with a pulmonary artery RAC 16% (log-rank p < 0.001). RAC predicted mortality better than area distensibility. CONCLUSION: Noninvasively measured pulmonary artery RAC predicts mortality in patients with PAH.