Late-onset Pompe disease (LOPD) in Belgium: clinical characteristics and outcome measures.

BACKGROUND: Late-onset Pompe disease (LOPD) is a rare, hereditary, progressive disorder that is usually characterized by limb-girdle muscle weakness and/or respiratory insufficiency. LOPD is caused by mutations in the acid alpha-glucosidase (GAA) gene and treated with enzyme replacement therapy (ERT). METHODS: We studied the clinical, brain imaging, and genetic features of the Belgian cohort of late-onset Pompe disease patients (N = 52), and explored the sensitivity of different outcome measures, during a longitudinal period of 7 years (2010-2017), including the activity limitations ActivLim score, 6 min walking distance (6MWD), 10 m walk test (10MWT), MRC sum score, and forced vital capacity (FVC) sitting/supine. RESULTS: In Belgium, we calculated an LOPD prevalence of 3.9 per million. Mean age at onset of 52 LOPD patients was 28.9 years (SD: 15.8 y), ranging from 7 months to 68 years. Seventy-five percent (N = 39) of the patients initially presented with limb-girdle weakness, whereas in 13% (N = 7) respiratory symptoms were the only initial symptom. Non-invasive ventilation (NIV) was started in 37% (N = 19), at a mean age of 49.5 years (SD: 11.9 y), with a mean duration of 15 years (SD: 10.2 y) after symptom onset. Brain imaging revealed abnormalities in 25% (N = 8) of the patients, with the presence of small cerebral aneurysm(s) in two patients and a vertebrobasilar dolichoectasia in another two. Mean diagnostic delay was 12.9 years. All patients were compound heterozygotes with the most prevalent mutation being c.-32-13 T > G in 96%. We identified two novel mutations in GAA: c.1610_1611delA and c.186dup11. For the 6MWD, MRC sum score, FVC sitting and FVC supine, we measured a significant decrease over time (p = 0.0002, p = 0.0001, p = 0.0077, p = 0.0151), which was not revealed with the ActivLim score and 10MWT (p > 0.05). CONCLUSIONS: Awareness on LOPD should even be further increased because of the long diagnostic delay. The 6MWD, but not the ActivLim score, is a sensitive outcome measure to follow up LOPD patients.

Comparison of myocardial contrast echocardiography with NC100100 and (99m)Tc sestamibi SPECT for detection of resting myocardial perfusion abnormalities in patients with previous myocardial infarction.

OBJECTIVE: To determine whether myocardial contrast echocardiography (MCE) following intravenous injection of perfluorocarbon microbubbles permits identification of resting myocardial perfusion abnormalities in patients who have had a previous myocardial infarction. PATIENTS AND INTERVENTIONS: 22 patients (mean (SD) age 66 (11) years) underwent MCE after intravenous injection of NC100100, a novel perfluorocarbon containing contrast agent, and resting (99m)Tc sestamibi single photon emission computed tomography (SPECT). With both methods, myocardial perfusion was graded semiquantitatively as 1 = normal, 0.5 = mild defect, and 0 = severe defect. RESULTS: Among the 203 normally contracting segments, 151 (74%) were normally perfused by SPECT and 145 (71%) by MCE. With SPECT, abnormal tracer uptake was mainly found among normally contracting segments from the inferior wall. By contrast, with MCE poor myocardial opacification was noted essentially among the normally contracting segments from the anterior and lateral walls. Of the 142 dysfunctional segments, 87 (61%) showed perfusion defects by SPECT, and 94 (66%) by MCE. With both methods, perfusion abnormalities were seen more frequently among akinetic than hypokinetic segments. MCE correctly identified 81/139 segments that exhibited a perfusion defect by SPECT (58%), and 135/206 segments that were normally perfused by SPECT (66%). Exclusion of segments with attenuation artefacts (defined as abnormal myocardial opacification or sestamibi uptake but normal contraction) by either MCE or SPECT improved both the sensitivity (76%) and the specificity (83%) of the detection of SPECT perfusion defects by MCE. CONCLUSIONS: The data suggest that MCE allows identification of myocardial perfusion abnormalities in patients who have had a previous myocardial infarction, provided that regional wall motion is simultaneously taken into account.

Prognostic value of myocardial ischemia and viability in patients with chronic left ventricular ischemic dysfunction.

BACKGROUND: Previous studies showed that thallium scintigraphy and dobutamine echocardiography were accurate, noninvasive ways of predicting contractile recovery after revascularization in patients with left ventricular (LV) dysfunction. However, the prognostic impact of such methods remains uncertain. METHODS AND RESULTS: We prospectively studied 137 consecutive patients with coronary disease and LV dysfunction who underwent exercise-redistribution-reinjection thallium scintigraphy and dobutamine echocardiography to identify myocardial ischemia and viability. A total of 94 patients subsequently underwent revascularization, and 43 underwent medical treatment. The primary endpoint was cardiac mortality, and mean follow-up was 33+/-10 months. Twenty-four patients died of cardiac causes. By Cox's regression analysis, long-term survival was related to the extent of coronary disease, the presence of diabetes, type of treatment, the presence of ischemic myocardium as determined by thallium scintigraphy, and the presence of viable myocardium as determined by both tests. Three-year survival was greater in patients with ischemic myocardium (as determined by thallium scintigraphy) or viable myocardium (as determined by both tests) who underwent revascularization than in the other groups (P=0.018 with thallium; P<0.001 with dobutamine). Subgroup analyses indicated that among patients with 1- or 2-vessel disease, only those with ischemic or viable myocardium improved survival after revascularization, whereas in patients with 3-vessel or left main diseases, revascularization always improved survival, albeit more in the presence of ischemic or viable myocardium. CONCLUSIONS: Among the parameters commonly available in patients with LV ischemic dysfunction, the presence of ischemic myocardium (as determined by thallium scintigraphy) and that of viable myocardium (as determined by dobutamine echocardiography) are independent predictors of subsequent mortality. These observations may be useful in the preoperative selection of patients for revascularization.

Relation of exercise capacity to left ventricular systolic function and diastolic filling in idiopathic or ischemic dilated cardiomyopathy.

Although exercise intolerance is a cardinal symptom of patients with dilated cardiomyopathy (DC) and heart failure, the factors that limit exercise capacity in these patients remain a matter of debate. To assess the contribution of left ventricular (LV) diastolic filling to the variable exercise capacity of patients with DC, we studied 47 patients (60 +/- 12 years) with DC in stable mild-to-moderate heart failure with a mean LV ejection fraction of 28%. Exercise capacity was measured as total body peak oxygen consumption (VO2) during symptom-limited bicycle (10 W/min) and treadmill (modified Bruce protocol) exercise. LV systolic function and diastolic filling were assessed at rest before each exercise by M-mode, Doppler echocardiography, and radionuclide ventriculography. As expected, treadmill exercise always yielded higher peak VO2 than bicycle exercise (21 +/- 6 vs 18 +/- 5 ml/kg/min, range 12 to 35 and 7 to 30 ml/kg/min, respectively, p <0.001). Both of these VO2 measurements were highly reproducible (R = 0.98). With univariate analysis, close correlations were found between peak VO2 (with either exercise modalities) and Doppler indexes of LV diastolic filling, as well as with the radionuclide LV ejection fraction. Stepwise multiple regression analysis identified 3 nonexercise variables as independent correlates of peak VO2, of which the most powerful was the E/A ratio (multiple r2 = 0.38, p <0.0001), followed by peak A velocity (r2 = 0.54, p <0.0001) and mitral regurgitation grade (r2 = 0.58, p = 0.024). In conclusion, our data indicate that in patients with DC, peak VO2 is better correlated to diastolic filling rather than systolic LV function.

Risk stratification in patients with dilated cardiomyopathy: contribution of Doppler-derived left ventricular filling.

Dilated cardiomyopathy (DCM) is a major cause of mortality among patients with heart failure. The aim of the present study was to investigate the independent contribution of Doppler-derived left ventricular (LV) filling to the prediction of survival in patients with DCM, of either ischemic or nonischemic origin, and to derive a simple risk stratification score based on easily available clinical and echocardiographic parameters. We followed 197 consecutive patients (159 men, mean age 60+/-13 years) with an echocardiographic diagnosis of DCM (LV end-diastolic dimension >60 mm, fractional shortening <25%) over an average period of 62+/-13 months. The presumed etiology of DCM was ischemic in 52% of the patients. During follow up, 69 patients died of cardiac causes and 41 required transplantation. At 5 years, overall cardiac event-free survival was 55% and freedom from death or heart transplantation was 43% (compared with 86% for the 5-year age- and sex-adjusted survival rate in our country). Kaplan-Meier survival curves generated for different thresholds of the peak E velocity and the E/A ratio indicated significant worsening of prognosis with increasing values of these parameters in both ischemic and nonischemic patients. Using Cox stepwise regression analyses, age (chi-square to remove 24.4; p <0.001), peak E velocity (chi-square to remove=18.9; p <0.001), LV ejection fraction (chi-square to remove 6.4; p <0.011), and systolic blood pressure (chi-square to remove 4.5; p=0.034) independently predicted cardiac deaths, whereas New York Heart Association (NYHA) functional class (chi-square to remove 48.5; p < 0.001), LV ejection fraction (chi-square to remove 19.1; p <0.001), E/A ratio (chi-square to remove 10.8; p <0.001), and systolic blood pressure (chi-square to remove 5.8; p <0.016) were independently associated with cardiac death or need for transplantation. Based on these parameters, a risk score was elaborated, which allowed appropriate classification of each individual patient into low- (5-year survival rate of 72%), intermediate- (46% survival rate), and high-risk groups (11% survival rate). In conclusion, our data show that among the noninvasive parameters commonly available in patients with either ischemic or nonischemic DCM, age, the NYHA functional class, the LV ejection fraction, the systolic blood pressure, the peak E velocity, and the E/A ratio provide relevant and independent information regarding the risk of cardiac death or the need for heart transplantation.

Relation of ultrasonic tissue characterization with integrated backscatter to contractile reserve in chronic left ventricular ischemic dysfunction.

Previous studies have shown that viable but stunned myocardium displays contractile reserve and exhibits cardiac cycle-dependent variations of integrated backscatter, whereas infarcted myocardium does not. The present study was designed to evaluate whether integrated backscatter imaging could be useful in identifying segments with recruitable inotropic reserve in patients with chronic left ventricular (LV) ischemic dysfunction. We studied 15 patients (mean age 59 +/- 10 years) with chronic coronary artery disease, anterior or inferior wall dysfunction, and depressed LV ejection fraction (35 +/- 12%), and 6 noncardiac control subjects (mean age 49 +/- 18 years). Cardiac cycle-dependent variations of integrated backscatter were measured in anterior and inferior segments during transesophageal echocardiography and compared with the contractile response (% wall thickening) of these segments to low doses of dobutamine (5 to 10 microg/kg/min). The average magnitude of cardiac cycle-dependent variations of integrated backscatter was greater among normally contracting segments of both patients and controls (5.67 +/- 0.88 and 5.64 +/- 2.26 dB, respectively, p = NS) than among dysfunctional segments (2.77 +/- 3.05 dB, p <0.01 vs control and remote segments). Dysfunctional segments were further categorized into those with and without dobutamine-induced contractile reserve. At baseline, systolic wall thickening was similar among segments responding to dobutamine than among those that did not (3.6 +/- 2.3% vs 2.9 +/- 1.6%, p = NS). During dobutamine, systolic wall thickening increased only in segments showing improvement in wall motion score (to 24.5 +/- 4.7%), whereas it remained unchanged in segments not responding to dobutamine (to 2.0 +/- 3.7%, p <0.01). The magnitude of resting cardiac cycle-dependent variations of integrated backscatter was larger in segments responding to dobutamine than in those with persistent dysfunction (5.31 +/- 2.06 vs 0.23 +/- 0.94 dB, p <0.01) and correlated significantly (r = 0.74, p <0.01) with systolic wall thickening during dobutamine. Our data demonstrate that resting cardiac cycle-dependent variations of integrated backscatter closely parallel contractile reserve in patients with chronic LV ischemic dysfunction. This suggests that tissue characterization with integrated backscatter could be a useful adjunct to the delineation of myocardial viability in these patients.

Comparison of dipyridamole stress echocardiography and perfusion scintigraphy for cardiac risk stratification in vascular surgery patients.

Dipyridamole single-photon emission computed tomography (SPECT) has a high negative predictive value for perioperative cardiac events, but events are infrequent in patients with a positive test. In contrast, dipyridamole echocardiography is more selective for detection of multivessel disease and thus may have a greater specificity for cardiac events. We therefore compared the ability of dipyridamole SPECT and echocardiography to predict perioperative and long-term cardiac events in 133 patients referred for vascular surgery. The group was also evaluated based on clinical features and ejection fraction. Four patients had surgery cancelled because of high risk and were excluded from further analysis. Among the 129 remaining patients, 21 had coronary revascularization (n=12) or an early cardiac end point (n=9). The sensitivity of SPECT for the prediction of early events (90%) was not significantly different from that of echocardiography (66%, p=NS). The specificity of SPECT (68%) was less than that of echocardiography (88%, p <0.001%), as was the accuracy (72% vs 84%, p=0.02). These findings were replicated after exclusion of patients with treatment end points. During long-term follow-up, 12 patients experienced > or = 1 event: 6 died from cardiac causes, 4 underwent revascularization, and 3 had myocardial infarction. Thus, the specificity of SPECT and echocardiography for late events were 58% and 80%, respectively (p <0.001). The 3-year survival of patients without ischemia during echocardiography or at SPECT was not different (93% vs 94%, p=NS).

Head-to-head comparison of exercise-redistribution-reinjection thallium single-photon emission computed tomography and low dose dobutamine echocardiography for prediction of reversibility of chronic left ventricular ischemic dysfunction.

OBJECTIVES: We sought to directly compare the diagnostic value of exercise-redistribution-reinjection thallium single-photon emission computed tomography (SPECT) and low dose dobutamine echocardiography for prediction of contractile recovery after revascularization. BACKGROUND: Both thallium SPECT and dobutamine echocardiography have been proposed to predict the reversibility of left ventricular dysfunction after revascularization. Although both techniques permit differentiation of viable from nonviable myocardium, few studies have directly compared their accuracy in the same patients. METHODS: Seventy-three consecutive patients (mean [+/- SD] age 59 +/- 9 years) with coronary disease and regional left ventricular dysfunction underwent exercise-redistribution-reinjection thallium SPECT and dobutamine echocardiography before revascularization. Recovery of function was evaluated with echocardiography 5.5 +/- 2.5 months after revascularization. For analysis, the left ventricle was divided into 16 segments, in which percent thallium uptake was quantitated using circumferential profiles, and regional wall motion was graded semiquantitatively (normal = 1; akinetic = 3). RESULTS: The diagnostic performance of the two tests was investigated both for individual patients and for individual segments. Individual patient analysis showed that left ventricular ejection fraction improved > 5% after revascularization in 43 patients, whereas 30 showed no change. Receiver operating characteristic curves were used to select optimal criteria for prediction of functional recovery after revascularization. According to a mean thallium uptake > 54% at reinjection, SPECT had a sensitivity of 72%, a specificity of 73% and an overall accuracy of 73%. Similarly, according to an improvement in wall motion score > 3.5 grades during doubutamine echocardiography, echocardiography had a sensitivity of 88%, a specificity of 77% and an overall accuracy of 84% (p = NS vs. thallium). Segmental analysis showed that SPECT and dobutamine echocardiography had similar sensitivity (77% and 75%, respectively), but SPECT had lower specificity (56% vs. 86%, p < 0.01). CONCLUSIONS: Quantitative exercise-redistribution-reinjection thallium SPECT and dobutamine echocardiography have comparable accuracy for prediction of reversibility of global left ventricular dysfunction after revascularization. However, dobutamine echocardiography has greater specificity than thallium imaging for prediction of functional recovery on a segmental basis.

Preoperative selection of patients with severely impaired left ventricular function for coronary revascularization. Role of low-dose dobutamine echocardiography and exercise-redistribution-reinjection thallium SPECT.

BACKGROUND: Both thallium imaging and low-dose dobutamine echocardiography have been proposed to predict the reversibility of left ventricular (LV) dysfunction in patients with coronary disease. The present study was designed to evaluate whether the use of these techniques during the preoperative assessment of coronary patients with depressed LV function can improve our ability to identify those likely to have improved LV function after surgery. METHODS AND RESULTS: Forty consecutive patients (age, 60 +/- 10 years) with coronary disease and an ejection fraction < or = 35% underwent dobutamine echocardiography (10 micrograms/kg per minute) and exercise-redistribution-reinjection thallium single photon emission computed tomography (SPECT) before coronary revascularization by bypass surgery (n = 33) or angioplasty (n = 7). Recovery of LV function was evaluated by echocardiography 5.3 +/- 2.4 months after revascularization. According to the changes in end-systolic volume and ejection fraction after revascularization, the patients were categorized into groups with (n = 19) and without (n = 21) postoperative functional improvement, defined as a > 5% increase in ejection fraction and > 10 mL decrease in end-systolic volume. Before revascularization, patients with improved postoperative function had smaller end-diastolic volume and less wall motion abnormalities than those with persistent dysfunction. They also showed greater improvement of wall motion score with dobutamine (6.1 +/- 2.4 versus 1.8 +/- 4.2 grades, P < .001) and smaller thallium defect score after exercise (38 +/- 12 versus 47 +/- 14 grades, P = .04). Discriminant analysis selected the improvement in wall motion score with dobutamine and baseline end-diastolic volume as independent predictors of postoperative recovery. Consideration of both parameters allowed prediction of functional outcome in 84% of the patients with and 81% of those without postoperative improvement. CONCLUSIONS: Among the parameters commonly available before surgery in coronary patients with depressed LV function, the maintenance of significant inotropic reserve, the severity of LV remodeling, and the magnitude of the perfusion defect after exercise can predict the reversal of LV dysfunction after revascularization.

Malignant peritoneal mesothelioma. Case report and review of the literature.

We describe a peritoneal mesothelioma. There are many aspecific symptoms. Professional exposure is found in only fifty percent of cases. The histological diagnosis is often difficult. The survival period is short because of the absence of curative treatment.

Comparative ability of dobutamine and exercise stress in inducing myocardial ischaemia in active patients.

OBJECTIVE: To compare the ability of dobutamine and exercise stress to induce myocardial ischaemia and perfusion heterogeneity under routine clinical circumstances. DESIGN: 86 active patients without previous myocardial infarction were studied by dobutamine and exercise stress protocols and coronary angiography. During both tests patients underwent electrocardiography, digitised echocardiography, and perfusion scintigraphy using Tc-99m methoxybutylisonitrile (MIBI) single photon emission computed tomography. MAIN OUTCOME MEASURE: Coronary disease defined as an ST segment depression of > or = 0.1 mV, a resting or stress induced perfusion defect, or a resting or stress induced wall motion abnormality on exercise and dobutamine stress testing. RESULTS: Dobutamine stress was submaximal in 51 patients because of ingestion of beta adrenoceptor blocking agents on the day of the test (n = 25) or failure to attain the peak dose owing to side effects (n = 28). Exercise was limited in 23 patients by non-cardiac symptoms. The peak heart rate with dobutamine was less than that attained with exercise (105 (25) v 132 (24) beats/min, P < 0.0001); the response to maximal dobutamine stress significantly exceeded that to submaximal stress. Peak blood pressure was greatest with exercise (206 (27) v 173 (25) mm Hg, P < 0.001), values at maximal and submaximal dobutamine stress being comparable. Electrocardiographic evidence of ischaemia was induced less frequently by dobutamine than exercise (32% v 77% of the 56 patients with significant coronary disease, P < 0.01), as was abnormal wall motion (54% v 88%, P < 0.001). Ischaemia was induced more readily with maximal stress of either type; thus the sensitivities of dobutamine and exercise echocardiography were comparable only in patients undergoing a maximal dobutamine testing (73% v 77%, NS). Perfusion heterogeneity was induced in 58% of patients with coronary disease at submaximal dobutamine stress, 73% at maximal dobutamine stress, and 73% at exercise stress (NS). Among 30 patients without coronary stenoses, normal function was obtained in 83% of echocardiography studies with dobutamine and in 80% with exercise (NS). Normal perfusion was identified in 70% of these patients at exercise MIBI, and 68% at dobutamine stress (NS). CONCLUSIONS: In a group of patients studied under normal clinical circumstances antianginal treatment and inability to complete the stress protocol are frequent and compromise the capacity of dobutamine stress to induce ischaemia. In contrast, the induction of perfusion heterogeneity is less susceptible to submaximal stress.

Optimal use of dobutamine stress for the detection and evaluation of coronary artery disease: combination with echocardiography or scintigraphy, or both?

OBJECTIVES: This study was conducted to examine the efficacy of dobutamine stress two-dimensional echocardiography and perfusion scintigraphy for the detection of coronary artery disease in routine practice, to establish the causes of erroneous results and to derive appropriate criteria for the selection of either or both tests. BACKGROUND: Dobutamine stress combined with echocardiography or perfusion scintigraphy may be used to detect coronary artery disease. Although both imaging approaches have demonstrated similar levels of accuracy, it is not known whether there may be particular indications for the use of one or the other technique or a rationale for their combination. METHODS: Two hundred seventeen patients without previous infarction were studied prospectively with dobutamine stress echocardiography and technetium-99m methoxy isobutyl nitrile (sestamibi) single-photon emission computed tomography at the time of diagnostic coronary angiography. The presence of coronary stenoses of > or = 50% diameter was compared with the presence of rest or stress-induced abnormalities of perfusion and regional function. The extent of these abnormalities was correlated with an equivalent score of extent of angiographic disease. RESULTS: Significant coronary artery disease was found in 142 patients; 102 (72%) were identified by dobutamine echocardiography and 108 (76%, p = NS) by perfusion imaging. In 75 patients without significant disease, the specificity of dobutamine echocardiography was 83% compared with 67% for scintigraphy (p = 0.05). Echocardiographic sensitivity was lower in patients unable to complete the test because of side effects (n = 64) than in the remainder (59% vs. 77%, p = 0.02); this influence was less apparent with scintigraphy (71% vs. 78%, p = NS). Selective use of scintigraphy in the 31 patients with a negative submaximal stress echocardiogram led to a sensitivity of 80% for this combination. Patients with left ventricular hypertrophy accounted for most of the difference in specificity between echocardiography and scintigraphy (94% vs. 59%, p = 0.02). Their respective accuracies were 76% and 73%. CONCLUSIONS: Dobutamine stress echocardiography and perfusion scintigraphy have equivalent accuracy. In patients with left ventricular hypertrophy, echocardiography appears to be the test of choice. Selective use of sestamibi scintigraphy in patients with a negative submaximal echocardiogram enhances the accuracy of stress echocardiography alone.

Diagnosis of coronary artery disease in elderly patients: safety and efficacy of dobutamine echocardiography.

Coronary artery disease may be difficult to diagnose in the elderly because its clinical symptomatology is frequently atypical and because the performance of submaximal tests makes exercise stress testing sometimes unreliable. Dobutamine stress testing may be a useful alternative in such patients. This study compared the safety and accuracy of dobutamine stress echocardiography in 73 'young' (< 60 years old) and 63 'old' (> or = 60 years old) patients without previous myocardial infarction undergoing diagnostic coronary angiography. The sensitivity in young patients (79%, (67-91, 95% CI)) was similar to that in old patients (80% (69-91, 95% CI)). Similar levels of specificity (88% (75-101, 95% CI) vs 75% (54-96, 95% CI)) were found in the two groups. Both groups showed a trend to a higher sensitivity for multi-vessel disease than for single-vessel disease. No major side effect occurred during the entire study and peak dose (40 micrograms.kg-1.min-1) was attained with similar frequency in both groups (56% vs 49%). Minor side effects occurred equally in 'young' and 'old' patients and never persisted more than a few minutes after ending the first infusion of dobutamine. Dobutamine echocardiography appears to be safe and accurate for the detection of coronary artery disease regardless of age.

Contribution of left ventricular diastolic function to exercise capacity in normal subjects.

Previous studies have established that most of the heterogeneity in exercise capacity seen with sedentariness, aging, or physical training can be accounted for by individual differences in the maximal rate of total body oxygen consumption (VO2 max) during dynamic exercise. However, the factors that limit VO2 max in normal subjects remain disputed. To test the hypothesis that differences in left ventricular diastolic performance contribute to the heterogeneity of VO2 max seen in healthy subjects, 57 normal sedentary volunteers (36 +/- 13 yr, range 20-76 yr) and 9 endurance athletes (37 +/- 8 yr, range 26-51 yr) were studied. Aerobic capacity was estimated as VO2 max during a multistage dynamic cycle exercise protocol, whereas resting left ventricular systolic and diastolic function was assessed by two-dimensional and Doppler echocardiography. The relationship of the left ventricular functional indexes with VO2 max was investigated by stepwise multiple regression analysis. VO2 max ranged from 25 to 58 ml.kg-1 x min-1 in sedentary subjects and from 44 to 60 ml.kg-1 x min-1 in athletes. With univariate analysis, significant correlations were observed between VO2 max and age (r = -0.60), maximal heart rate (r = 0.48), maximal work load (r = 0.80), left ventricular volumes at both end diastole (r = 0.51) and end systole (r = 0.62), peak early transmitral filling velocities (r = 0.80), and the ratio of early to late transmitral filling velocities (r = 0.87).(ABSTRACT TRUNCATED AT 250 WORDS)

Selection of the optimal nonexercise stress for the evaluation of ischemic regional myocardial dysfunction and malperfusion. Comparison of dobutamine and adenosine using echocardiography and 99mTc-MIBI single photon emission computed tomography.

BACKGROUND: The mechanisms of action of exercise-simulating and vasodilator stressors support their combination with imaging techniques that evaluate left ventricular function and perfusion, respectively. However, reported accuracies of either pharmacological stress together with two-dimensional echocardiography (2DE) or single photon emission computed tomography (SPECT) of myocardial perfusion are similar. The purpose of this study was to establish the optimal stress for each imaging technique by comparing the results of digitized 2DE and 99mTc-methoxyisobutyl isonitrile (MIBI) SPECT using both dobutamine and adenosine stresses in the same patients and conditions. METHODS AND RESULTS: Ninety-seven consecutive patients without evidence of previous infarction undergoing coronary angiography for clinical indications were studied prospectively. Dobutamine was infused during clinical, ECG, and echocardiographic monitoring in dose increments from 5 to 40 micrograms.kg-1.min-1. Adenosine was infused under the same conditions in doses of 0.10, 0.14, and 0.18 mg.kg-1.min-1. For each protocol, the end points were achievement of peak dose, development of severe ischemia, or intolerable side effects. At peak stress, 20 mCi of MIBI was injected, and SPECT imaging was performed 2 hours later; abnormal poststress images were compared with resting SPECT: Digitized 2DE images were compared qualitatively before, during, and after stress in a cine-loop display. Significant coronary disease (n = 59 patients) was defined by the quantification of > 50% stenosis in a major epicardial vessel. The sensitivity of adenosine 2DE was 58%, less than those of adenosine MIBI (86%, p = 0.001), dobutamine 2DE (85%, p = 0.001), and dobutamine MIBI (80%, p = 0.01). Their respective specificities were 87%, 71%, 82%, and 74% (p = NS). The accuracy of adenosine 2DE was 69%, compared with 80% for adenosine MIBI (p < 0.001), 84% for dobutamine 2DE (p = 0.001), and 77% for dobutamine MIBI (p = 0.005); the latter three did not differ significantly in either sensitivity or accuracy. CONCLUSIONS: This prospective, direct comparison of alternative pharmacological stresses in patients without myocardial infarction shows vasodilator stress scintigraphy and dobutamine stress echocardiography and scintigraphy to share equivalent levels of sensitivity. All three are significantly more sensitive than adenosine stress echocardiography. Dobutamine stress may be used for wall motion or perfusion imaging, but adenosine stress is best combined with perfusion scintigraphy.

[Contribution of polysomnography in the assessment of patients with chronic obstructive bronchopneumopathy]. / Apport de la polysomnographie à la mise au point des malades atteints d'une bronchopneumopathie chronique obstructive (BPCO).

15 COPD patients underwent a polysomnographic study demonstrating poor quality of sleep, a mean of SAO2 of 88.8 +/- 3.9% and a apneic-hypopnea index (AHI) of 5.7 +/- 11.8. AHI was higher in sleep stages I and II than in REM sleep. SAO2 showed a progressive drop when going from an awake stage to REM sleep. Respiratory events responsible for the most important desaturation where mostly observed in REM sleep and corresponded in 8 patients to obstructive events (overlap syndrome). The lower mean SAO2 in REM probably explains the best the importance in desaturation related to the respiratory events (Hb dissociation curve). Ear oximetry recordings however interesting are not able to quantify and recognise correctly the respiratory events. Therefore a polysomnographic study remains necessary in order to diagnose adequately the overlap syndrome.

[Value of monitoring of tracheal respiratory sounds in the diagnosis of nocturnal respiratory dysrhythmias]. / Intérêt du suivi des bruits respiratoires trachéaux dans le diagnostic de dysrythmies respiratoires nocturnes.

Twenty-six patients underwent a polysomnigraphic study allowing sleep staging and respiratory events scoring with the use of the oronasal flow, abdominal, thoracic and total displacement (Respitracet), and ear oximetry. Moreover the patients were also equipped with a tracheal microphone giving a power rectified envelope (sonospirogram). Eleven patients showed abnormal respiratory events that were scored by visual lecture using all respiratory parameters (excluding the sonospirogram) and were classified as obstructive central and mixed apneas-hypopneas. Periodic breathing was also appreciated. Detection of the same events was tried with the sonospirogram alone. The sonospirogram could accurately detect snoring and periodic breathing and finally central obstructive mixed apnea (the apneic index being well correlated: p less than 0.001 as well as the mean apnea duration: p less than 0.005). In contrast hypopneic events related to snoring could not be accurately appreciated. We conclude that a sonospirogram may be useful for the detection of abnormal respiratory events when used alone (screening) as when added to other respiratory signals.

Non-invasive data provide independent prognostic information in patients with chest pain without previous myocardial infarction: findings in male patients who have had cardiac catheterization.

From 1978 to 1985, 470 consecutive male patients with complaints of chest pain underwent a maximal exercise test with a thallium scan and coronary angiography (CA). Patients with a history of myocardial infarction (MI) were excluded. During the follow-up (from 12 to 96 months), 32 patients died and 30 had a non-fatal MI. Survival (SR) and event-free rates (EFR) were estimated by actuarial methods; the influence of non-invasive and invasive variables were examined in univariate and multivariate models using Cox analysis. The five-year SR was 89% and EFR was 81%. Among historical data, age (less than 0.001), type of complaints (less than 0.01) and pretest likelihood of CAD (less than 0.01) were univariate predictors of EFR; by multivariate analysis, age was the only significant predictor (less than 0.001). Most of the maximal-exercise (MEX) test data were good univariate predictors; by multivariate analysis, workload (less than 0.001) and the maximal-exercise test score (less than 0.001) were the significant predictors. From history and maximal-exercise test data, multivariate analysis indicated that the prognostic information was given by age (less than 0.05) and maximal-exercise test score (less than 0.001). Among the invasive data, the number of diseased vessels (less than 0.001) and ejection fraction were the predictors. The combination of invasive and non-invasive data indicated that age, MEX score, number of diseased vessels and ejection fraction contributed significantly and independently to the estimation of prognosis. Among 242 patients with two or three diseased vessels, the prognosis was determined by the maximal workload (less than 0.01); ejection fraction (less than 0.07) was no longer significant.(ABSTRACT TRUNCATED AT 250 WORDS)