Contrast echocardiography for pulmonary arteriovenous malformations screening: does any bubble matter?

AIMS: To evaluate diagnostic accuracy of contrast echocardiography (CE) as compared with CT, for the screening of pulmonary arteriovenous malformations (PAVMs) in hereditary haemorrhagic telangiectasia (HHT); to evaluate the clinical significance of semi-quantitative analysis of a shunt on CE. METHODS AND RESULTS: A blinded prospective study was conducted in 190 consecutive subjects at risk of HHT who underwent screening for PAVMs, including clinical evaluation, pulse oximetry, standard and CE, and chest multirow CT without contrast medium. A semi-quantitative analysis of the shunt size was performed according to the contrast echo opacification of the left-sided chambers: Grade 0, no bubbles; 1, occasional filling with <20 bubbles; 2, moderate filling; 3, complete opacification. The first 100 patients were compared with 100 controls. A total of 119 (63%) patients had positive CE (32.2% Grade 1, 13.1% Grade 2, 11% Grade 3, 6.3% with patent foramen ovale). The overall diagnostic performance of CE was sensitivity 1.00, specificity 0.49, positive predictive value (PPV) 0.32, negative predictive value (NPV) 1.00. The PPV for the different grades was 0.00 for Grade 1, 0.56 for Grade 2, 1.00 for Grade 3; the NPV of Grade 0 was 1.00. A significant correlation was found between the CE grading and the number of PAVM, and complications (P < 0.0001). CONCLUSION: CE is an extremely sensitive procedure for the detection of PAVMs with substantial clinical impact.

Improved aetiological diagnosis of ischaemic stroke in a Vascular Medicine Unit--the significance of transesophageal echocardiogram.

BACKGROUND: The TOAST study estimates that 34% of ischaemic strokes are of undetermined aetiology. Improvements in the diagnosis of the pathogenetic mechanism of ischaemic stroke would translate into a better care, in analogy to other fields of vascular and internal medicine. OBJECTIVE: To measure the reduction of undetermined aetiology strokes performing a set of additional diagnostic tests. DESIGN: Consecutive case series with historical controls. SETTING: Internal Medicine Ward with a stroke area (SA) admitting most stroke patients of a large hospital in Italy. SUBJECTS: A total of 179 ischaemic stroke patients admitted to SA in 2004-2005 compared with 105 ischaemic stroke patients admitted to the whole department in 2001. INTERVENTION: To perform more diagnostic tests, including transesophageal echocardiography (TEE), in the greatest possible number of ischaemic stroke inpatients admitted in SA of the Internal Medicine Department, in the years 2004-2005. RESULTS: More diagnostic tests were performed during the study period than in 2001, especially TEE (56% of patients in 2004-2005 vs. 3% of patients in 2001). We observed a significant reduction of undetermined aetiology from 38% in 2001 to 16% in 2004-2005 (p < 0.0001), largely for an increased identification of cases of cardio-embolic mechanism (from 18% to 40%, p = 0.0002). In the years 2004-2005 the fraction of patients on anticoagulant treatment at discharge was 21% vs. 12% in 2001 (p = 0.041). CONCLUSION: Performing more tests, particularly TEE, brought improvements in the aetiological diagnosis of stroke, increasing cardio-embolism diagnosis and anticoagulant treatment.

Pregnancy in a woman with Turner syndrome and celiac disease.

BACKGROUND: Studies on fertility in women with Turner syndrome have shown that spontaneous pregnancies occur in about 2-7% of patients. Fertility problems and obstetrical complications are frequently observed in untreated patients with celiac disease. We report the case of a patient, affected by Turner syndrome and celiac disease, in whom a spontaneous pregnancy occurred. CASE: One patient affected by Turner syndrome at the age of 30 yr conceived spontaneously. Celiac disease was diagnosed during pregnancy. The pregnancy progressed uneventfully. After 39 weeks of gestation, she vaginally delivered a normal male infant. CONCLUSION: Our patient had a successful pregnancy, giving birth to a healthy child, although she presented two pathological conditions affecting fertility and pregnancy outcome: Turner syndrome and celiac disease.

Doppler velocimetry in superior vena cava provides useful information on the right circulatory function in patients with congestive heart failure.

BACKGROUND: Although flow velocities curves recorded with pulsed-wave Doppler in systemic vein are known to provide functional data on the right circulatory function, little information is available on the relationship between right heart filling dynamics and right ventricular function. METHODS: Consecutive patients with chronic heart failure due to severe systolic left ventricular dysfunction and in sinus rhythm underwent echocardiography and right heart catheterization. In the initial part of the study, the hemodynamic correlates of different flow velocity patterns recorded into the superior vena cava were evaluated in 120 patients. The accuracy of the prediction of different right heart hemodynamic profiles by means of the different venous flow patterns was then prospectively tested in a subsequent series of 86 patients. RESULTS: The venous flow pattern was closely related to right heart hemodynamics. A normal Doppler pattern identified patients with normal right heart hemodynamics (sensitivity 86%, specificity 78%); a "predominant systolic wave" pattern identified patients with a reduced thermodilution-derived right ventricular ejection fraction (< 30%) and normal or slightly elevated right atrial pressure (< or = 8 mmHg) (sensitivity 69%, specificity 81%); a "predominant diastolic wave" pattern identified patients with a reduced right ventricular ejection fraction (< 3 0%) and elevated right atrial pressure (> 8 mmHg) (sensitivity 52%, specificity 95%). The observed and the predicted hemodynamic profiles turned out to be concordant in 80% of patients. CONCLUSIONS: The analysis of the flow velocity pattern into the superior vena cava is a useful tool to estimate the extent of the right circulatory impairment in patients with congestive heart failure.

Echocardiographic evaluation of patients cured of childhood cancer: a single center study of 117 subjects who received anthracyclines.

BACKGROUND: The risk of cardiomyopathy following exposure to anthracycline in asymptomatic long-term survivors of childhood cancer is still hard to predict and precisely quantify. To identify the impact of different cumulative doses, even within a non-high dose range, and the echocardiographic parameters suitable for evaluating cardiac function, we studied diastolic and systolic echocardiographic parameters in a cohort of patients followed in a single center. PROCEDURE: A total of 117 subjects were studied at a median time of 7 years after treatment completion. A complete M-mode, two-dimensional and Doppler echocardiographic study was obtained at rest in all patients according to the standard recommendations of the American Society of Echocardiography. RESULTS: Ninety-nine patients (85%) had completely normal cardiac function, while 18 had abnormal echocardiographic findings: 12 had one abnormal value, 5 had two, and 1 had three abnormal values. All the changes were in left ventricular dimensions, wall thickness or indices of systolic function; no alterations in left ventricular diastolic function parameters were found. None of the echocardiographic parameters correlated significantly with the cumulative dose of anthracyclines administered either at univariate analysis or after adjusting for sex, body surface area or considered risk factors. CONCLUSIONS: Subjects exposed to a median cumulative dose of 214 mg/m(2) had no echographic abnormalities a median of 7 years later. We did not find any correlation between cumulative anthracycline dose and the echocardiographic parameters tested. We now offer echocardiographic follow-up to patients with mildly reduced fractional shortening and/or ejection fraction to rule out late onset dysfunction.

Echocardiographic evolution of left ventricular and left atrial thrombi in a patient with left ventricular dysfunction due to alcoholic cardiomyopathy, chronic atrial fibrillation and multiple non-fatal systemic embolisms.

The echocardiographic characteristics and evolution of multiple pedunculated left atrial and left ventricular intracavitary thrombi in a patient with alcoholic cardiomyopathy are reported. The patient had a long history of left ventricular dysfunction and atrial fibrillation but the referring physician had not prescribed anticoagulant prophylaxis. Multiple, non-fatal, systemic embolizations occurred during hospitalization and echocardiography was used to monitor the effect of the anticoagulant therapy on the remodelling and final dissolution of intracavitary thrombi.

[Cor triatriatum dextrum resulting from the persistence of embryonic remnants of the right valve of the sinus venosus: prevalence and echocardiographic aspects in a large consecutive non-selected patient population]. / Pseudocuore triatriato destro da persistenza di residui embrionari della primitiva valvola del seno venoso: prevalenza ed aspetti ecocardiografici in un'ampia popolazione consecutiva non selezionata.

BACKGROUND: Cor triatriatum destrum is a rare congenital malformation, usually associated with complex right heart abnormalities, characterized by a membrane that divides the right atrium into two chambers. It is considered the result of the incomplete and abnormal regression of the embryonic right valve of the sinus venosus. When the valve regresses the cephalic portion forms the crista terminalis and the caudal portion develops into the Eustachian and Thebesian valve. With an incomplete regression, a fenestrated or an unfenestrated membrane may persist in the right atrium. We describe the clinical and echocardiographic characteristics of this type of remnants in the right atrium. METHODS: In a 4-month period, 1728 transthoracic two-dimensional and Doppler echocardiographic studies were consecutively performed. RESULTS: Twenty-seven patients (16 males, 11 females, mean age 55 +/- 21 years, range 1-87 years) were occasionally recognized to be affected by a membrane inside the right atrial cavity. On the basis of its echocardiographic appearance (a membranous structure connecting the orifice of the inferior vena cava to the atrial septum with evident fenestration in 74% of cases), absence of associated congenital abnormalities (92.5% of cases), interatrial shunt or abnormal flow pattern of venous systemic return, a diagnosis of a normal benign anatomic variant of sinus venous remnants (similar to the so-called "Chiari's network") could be made in every case. CONCLUSIONS: The incomplete regression of the embryonic right valve of the sinus venosus may leave a fenestrated or an unfenestrated membrane in the right atrium that should be considered a normal benign variant of the so-called "Chiari's network".

Lack of clinically significant cardiac dysfunction during intermediate dobutamine doses in long-term childhood cancer survivors exposed to anthracyclines.

BACKGROUND: Long-term survivors of childhood cancer treated with anthracyclines may have subclinical cardiac dysfunction undetectable at a baseline evaluation. Dobutamine stress echocardiography has been proposed as a more sensitive screening test, but results of previous studies were influenced by selection criteria, infusion protocols, and side effects. METHODS: We applied a modified dobutamine stress test (from 5 to 10 to 15 microg/kg, infused over a 5-minute period) and evaluated the influence on stress test results of reported risk factors for late cardiac toxicity (female sex, younger age at treatment, higher dose of anthracycline, and longer duration of follow-up). Seventy-one patients (46 male, mean age 15 +/- 5 years) treated with anthracyclines (median dose 240 mg/m(2)) 1 to 16.5 years before and 20 controls (patients' siblings: 12 male, mean age 19 +/- 4 years) were studied. RESULTS: No major side effects were recorded. One patient was unable to perform the test because of anxiety. Limiting side effects were infrequent (3%) and occurred at a dobutamine dose of > or =10 microg/kg, when significant changes of hemodynamic and echocardiographic parameters were detectable in all cases. Rest systolic and mean blood pressure and left ventricular fractional shortening were significantly lower in patients than in controls (P <.05), but no differences were found in any of the other indexes of cardiac function between the 2 groups at rest and during each dobutamine infusion step. A similar increase of global left ventricular function (percent of fractional shortening +45% vs +32%) and a decrease of end-systolic stress (-33% vs -29%) were documented. Left ventricular relaxation, early filling, and both relaxation and compliance improved. In all but one patient with reduced global left ventricular function at baseline, time-dependent patterns of hemodynamic and echocardiographic responses to dobutamine were similar. Previously described risk factors for cardiac toxicity did not influence the time changes of the echocardiographic parameters in response to dobutamine. CONCLUSIONS: Compared with controls, most of our asymptomatic childhood cancer survivors, studied an average of 7 years after treatment with anthracyclines, showed normal baseline cardiac function. Our stress test was feasible and safe. Compared with modalities used in other studies, shorter infusion periods with higher dobutamine doses allowed a higher stress intensity to be reached without reducing patient compliance. At dobutamine stress test the response was comparable in patients and controls except for one patient. Previously reported risk factors for cardiac toxicity had no significant influence on stress test results.

Comparison of dobutamine stress echocardiography and exercise stress Thallium-201 SPECT for detection of myocardial ischemia after acute myocardial infarction treated with thrombolysis.

In order to compare the ability of dobutamine stress echocardiography (DSE) and exercise Thallium-201 SPECT to detect myocardial ischemia in patients with myocardial infarction (MI) treated with thrombolysis, 43 prospectively selected patients with MI treated with thrombolysis underwent within 1 month from MI DSE, stress-redistribution-reinjection Thallium-201 SPECT and coronary angiography. The echocardiographic and scintigraphic images were analyzed for the presence of myocardial ischemia using a 11-segment left ventricular model. DSE and exercise Thallium-201 SPECT detected myocardial ischemia in the infarct zone in 72% and 72% (31/43) of patients and ischemia at a distance in 12% (5/43) and 19% (8/43) of patients with a concordance of 67% and 88%, respectively. A significant agreement between DSE and exercise Thallium SPECT was found in the evaluation of the extent of both myocardial necrosis and stress-induced myocardial ischemia. DSE and exercise Thallium SPECT showed similar sensitivity (79 vs 76%), specificity (60 vs 60%) and accuracy (77 vs 74%) for detection of a critical stenosis of the infarct-related artery; there was also no significant difference between the tests in sensitivity, specificity and accuracy for detection of the multivessel disease. In conclusion, initially after thrombolyzed MI, DSE and exercise Thallium-201 SPECT detect myocardial ischemia in the infarct zone in a high proportion of patients and show a similar accuracy for the diagnosis of a critical stenosis of the infarct-related coronary artery and of the multivessel disease.

Prognostic value of myocardial viability and ischemia detected by dobutamine stress echocardiography early after acute myocardial infarction treated with thrombolysis.

OBJECTIVES: The aim of the study was to assess the prognostic value of myocardial viability and ischemia detected by dobutamine stress echocardiography (DSE) in patients with acute myocardial infarction (AMI) treated with thrombolysis. BACKGROUND: DSE can detect myocardial viability and ischemia early after AMI, but the prognostic importance of viability and ischemia in these patients has yet to be assessed. METHODS: DSE was performed in 152 patients at a mean of 9 +/- 5 days after a first AMI treated with thrombolysis to evaluate myocardial viability and ischemia. The patients were followed up for 15 +/- 19 months. RESULTS: On the basis of DSE results three groups of patients were identified: group 1 (95 patients, 62.5%) with myocardial viability and ischemia, group 2 with myocardial viability without ischemia (32 patients, 21%) and group 3 (25 patients, 16.5%) with no myocardial viability. During follow-up 10 patients (6.5%) had hard events, 53 (35%) developed unstable angina and 67 (44%) underwent myocardial revascularization. The rate of hard events was 10% in group 1 and 0% in group 2 and 3 patients (p < 0.05 group 1 versus group 2); group 1 patients with viability and ischemia showed a significantly higher rate of recurrence of unstable angina and myocardial revascularization procedures (40% and 60%) compared to group 2 (22% and 16%) and group 3 patients (20% and 20%). Using the Cox multivariate stepwise model, only the extent of ischemic myocardium (hazard ratio (HR) = 21.7, p = 0.02) and angina during DSE (HR = 4.45, p = 0.03) were significant predictors of hard events; an ischemic response to DSE (HR = 2.92, p = 0.001) was the most important predictor of spontaneous events, followed by ST-segment depression during DSE (HR = 1.71, p = 0.04), angina during DSE (HR = 1.53, p = 0.19) and age (HR = 0.96, p = 0.05). CONCLUSIONS: In patients with a first AMI treated with thrombolysis the presence and extent of myocardial ischemia during DSE is the most important predictor of both hard and spontaneous cardiac events, whereas myocardial viability does not have an independent prognostic value.

Dobutamine-induced ST-segment elevation in patients without myocardial infarction.

During dobutamine stress echocardiography, ST-segment elevation developed in 20 of 372 patients (5%) without previous myocardial infarction and was associated with a transient severe asynergy of the myocardial region corresponding to the site of ST elevation. In 17 of 19 patients, ST-segment elevation was associated with a critical stenosis of the ischemia-related coronary artery, whereas in 2 of 19 patients with no critical lesions of the ischemia-related artery, coronary vasospasm was the most likely mechanism of myocardial ischemia.

Significance of ST-segment elevation during dobutamine-stress echocardiography in patients with acute myocardial infarction treated with thrombolysis.

BACKGROUND: Stress-induced ST-segment elevation in patients with recent myocardial infarction treated with thrombolysis has not been extensively investigated. According to the results of previous studies it may represent residual myocardial ischaemia or dyskinesia in the infarcted region. The aim of the study was to analyse the significance of dobutamine-induced ST-segment elevation in the infarcted area in a consecutive group of patients (n = 42, 41 men, mean age 53 +/- 7 years) with a first acute myocardial infarction treated with thrombolysis within 6 h from symptoms onset. METHODS AND RESULTS: All patients underwent dobutamine-stress echocardiography (up to 40 micrograms.kg-1.min-1 + atropine) 7 +/- 3 days from the acute event and coronary arteriography within 1 month from the test. Significant ST-segment elevation was defined as a shift > or = 1 mm during dobutamine compared to baseline in at least two contiguous infarct-related leads; a correlation was made between the site of ST-segment elevation and wall motion changes during dobutamine. Dobutamine-induced ST-segment elevation in 23/42 (55%) patients (group 1) while no changes were observed in 19/23 (45%) patients (group 2). Compared to group 2, group 1 patients showed a higher asynergy score index (1.72 +/- 0.24 vs 1.50 +/- 0.32, P < 0.02) and a higher number of asynergic segments (5.04 +/- 1.9 vs 4.11 +/- 1.8), at baseline, a higher incidence of baseline and/or stress-induced dyskinesia (39 vs 10%, P < 0.05) in the infarct-related region and a higher percentage of occluded infarct-related arteries (48 vs 0%, P < 0.001). In the 42 patients studied, a significant correlation was found between baseline ST-segment elevation and baseline asynergy score index (RS = 0.56, P < 0.001) and between ST-segment elevation and asynergy score index at peak stress (RS = 0.55, P < 0.001). The incidence of reversible wall motion abnormalities indicative of myocardial viability and residual myocardial ischaemia was similar in the two groups (87 vs 84% and 74 vs 68%, respectively), while the number of segments with irreversible akinesia indicative of myocardial necrosis was higher in group 1 compared to group 2 (1.5 +/- 1.4 vs 0.9 +/- 1.4). Among the 23 patients of group 1 with dobutamine-induced ST-segment elevation, six had no reversible wall motion abnormalities indicative of myocardial ischaemia; of the 17 patients with myocardial ischaemia, 11 had > or = 50% and six had < or = 50% of basally asynergic segments showing reversible wall motion abnormalities. CONCLUSIONS: In patients with recent thrombolyzed myocardial infarction dobutamine-induced ST-segment elevation is associated with a larger akinetic area in basal conditions and either with reversible wall motion abnormalities indicative of myocardial ischaemia or with irreversible or minimally reversible wall motion abnormalities in the infarct area during the test. Thus, dobutamine echocardiography provides useful information for the interpretation of stress-induced ST-segment elevation and clinical management of these patients.

Dobutamine stress echocardiography early after myocardial infarction treated with thrombolysis. Identification of myocardial viability and ischemia and relation to spontaneous functional recovery.

The aim of the study was to assess the ability of dobutamine stress echocardiography to detect myocardial viability and ischemia in patients with acute myocardial infarction treated with thrombolysis and to correlate the acute response to dobutamine with late spontaneous functional recovery at follow-up. Forty-two consecutive patients with myocardial infarction treated with thrombolysis underwent low- (5 and 10 mcg/kg/min) and high-dose (20 to 40 mcg/kg/min) dobutamine stress echocardiography at a mean of 7 +/- 3 days of the acute phase. A follow-up 2D-echocardiogram was performed in all patients to evaluate the spontaneous recovery of function in the infarct area. On the basis of the response to the test, 3 groups of patients were identified: group 1 included 7 patients showing an improvement in left ventricular asynergy score index at low doses (from 1.5 +/- 0.3 to 1.3 +/- 0.2, p < 0.05) with no deterioration at high doses, indicative of myocardial viability without ischemia; group 2 (23 patients) showed a significant improvement in the asynergy index at low doses (from 1.58 +/- 0.3 to 1.32 +/- 0.32, p < 0.05) followed by a deterioration at high doses (1.68 +/- 0.4, p < 0.05 vs low-dose), suggestive of residual myocardial ischemia in the infarct zone; group 3 included 12 patients who showed no significant changes in the baseline asynergy score index (1.67 +/- 0.2) either at low or at high doses. The acute response to dobutamine stress echocardiography accurately predicted the spontaneous recovery of function in the infarct area at follow-up: both group 1 and group 2 patients showed a significant reduction in the asynergy score index (group 1: 1.16 +/- 0.3 vs 1.5 +/- 0.2, p < 0.001; group 2: 1.43 +/- 0.3 vs 1.58 +/- 0.3, p < 0.05), while group 3 had no recovery in the asynergy index (1.67 +/- 0.2 vs 1.67 +/- 0.2). Thus, in patients with acute myocardial infarction treated with thrombolysis dobutamine stress echocardiography can detect myocardial viability in 71% and ischemia in the infarct zone in 55% of patients; moreover, the response to the test during the acute phase is correlated with the degree of the late spontaneous recovery of function in the infarct area.