Serial measurements of the Nt-ProBNP during the dry state in patients with systolic heart failure are predictors of the long-term prognosis.

OBJECTIVE: To evaluate the long-term predictive value of serial Nt-ProBNP during dry-state in patients with systolic heart failure (SHF). METHODS: Nt-ProBNP was measured quarterly during a 6-month dry-state period in 40 SHF outpatients. EVENTS: all-cause mortality or hospitalization. FOLLOW-UP: 5 years. RESULTS: The Nt-ProBNP >1000 pg/ml (baseline and 6 months) and the variation rate (VR) >30% were independently associated with the survival and composite endpoint curve. VR >30% added significant prognostic information to the single Nt-ProBNP 1000 pg/ml cut-off. Patients with at least one Nt-ProBNP determination >1000 pg/ml were at greater risk of death. CONCLUSION: Serial Nt-ProBNP measurements in patients with SHF during the dry-state are strong predictors of the long-term prognosis.

The importance of assessing nutritional status in elderly patients with heart failure.

Heart failure (HF) is a syndrome characterized by high morbidity and mortality, despite advances in medical and device therapy that have significantly improved survival. The outcome of HF in elderly patients results from a combination of biological, functional, psychological, and environmental factors, one of which is nutritional status. Malnutrition, as well as HF, is frequently present with aging. Early detection might lead to earlier intervention. It is our goal to review the importance of nutritional status in elderly patients with HF, as well as tools for assessing it. We also propose a simple decision algorithm for the nutritional assessment of elderly patients with HF.

[Constrictive pericarditis - new methods in the diagnosis of an old disease: a case report]. / Pericardite constritiva - novos métodos no diagnóstico de uma velha doença: a propósito de um caso clínico.

Constrictive pericarditis is a rare clinical entity that can pose diagnostic problems. The gold standard for diagnosis is cardiac catheterization with analysis of intracavitary pressure curves, which are high and, in end-diastole, equal in all chambers. The diastolic profile in both ventricles presents the classic dip-and-plateau pattern and the difference between the diastolic pressures of both ventricles should not exceed 3-5mmHg. Unfortunately, these traditional criteria are not always present and in fact the sensitivity and specificity of equalization of diastolic pressures are relatively low and of limited value in individual patients. This highlights the need to use new cardiac imaging techniques to resolve any doubts. The case described here is a good example.

[Takotsubo syndrome or acute myocarditis? The role of cardiac magnetic resonance imaging]. / Síndroma de takotsubo ou miocardite aguda? O papel da ressonância magnética cardíaca.

Acute myocarditis is often misdiagnosed, and its evolution is not always benign; correct and prompt diagnosis is therefore essential. We report the case of a 62-year-old woman with chest pain after a stressful event and ST-segment elevation on the electrocardiogram, in whom urgent cardiac catheterization showed normal coronary arteries and left ventricular apical ballooning, suggesting takotsubo syndrome. However, cardiac magnetic resonance imaging showed lesions typical of acute myocarditis, thus suggesting this diagnosis. We highlight the diagnostic difficulty in patients with chest pain, elevated troponin and normal coronary arteries, and the key role of cardiac magnetic resonance in differential diagnosis.

Behind heart failure syndrome: remember AL amyloidosis. Two case-reports.

Amyloidosis is a systemic disease that is a consequence of extracellular deposition of insoluble fibrils composed of subunits of low molecular weight (5-25 kD) derived from a variety of plasma proteins. Identification of the amyloidogenic protein determines the type of amyloidosis. In primary systemic amyloidosis (classically called AL amyloidosis), the amyloid protein is composed of light chains resulting from plasma-cell dyscrasia. Cardiac manifestations are the most common clinical presentation of this type of amyloidosis, occurring in 50% of patients. The authors describe two cases in which hospitalization was due to decompensated heart failure, which were similar in their etiology (multiple myeloma/amyloid cardiomyopathy) and evolution (sudden death). The authors wish to draw attention to an entity that is rarely encountered in clinical practice and that requires a high index of suspicion.

Left ventricular non-compaction: a new mutation predisposing to reverse remodeling?

Left ventricular non-compaction (LVNC) is a rare disorder of endomyocardial morphogenesis that results in multiple trabeculations and deep intertrabecular recesses filled with direct blood flow from the left ventricular cavity. LVNC is attracting increasing interest as a model for the study of cardiomyopathies, since it is a genetically heterogeneous disorder which varies greatly in clinical presentation and age of onset. The authors present the case of a young black male with progressive congestive heart failure of 2-3 years' evolution. The investigation, which included transthoracic echocardiography (contrast and 3D), transesophageal echocardiography and cardiac magnetic resonance imaging, showed LVNC and severe aortic regurgitation, with severe left ventricular systolic dysfunction. The family history was suggestive of genetically transmitted disease and genetic study of the TAZ gene at locus Xq28 identified the mutation p.Phe128Ser (c.383T>C), the first description of this mutation in a patient with LVNC. The patient underwent aortic valve replacement, with excellent clinical evolution, regression of left ventricular dimensions and global systolic functio Aortic regurgitation (not related to LVNC) was the determining factor in the clinical expression. However, the excellent reverse remodeling that occurred after surgery highlights the heterogeneity of myocardial behavior in LVNC patients.

Acquired left ventricular-to-right atrial shunt (Gerbode defect) due to infective endocarditis.

Left ventricular-to-right atrial communications are a rare type of ventricular septal defect, known as the Gerbode defect. They are usually congenital, but rare cases have been described secondary to bacterial endocarditis. The authors present a rare case of Gerbode defect and severe pericardial effusion secondary to Staphylococcus aureus endocarditis, in a patient with alcoholic liver cirrhosis.

Evaluation of the clinical, hemodynamic and neurohormonal response to levosimendan administration in decompensated heart failure patients. One-month follow-up.

INTRODUCTION: Levosimendan is an inodilatory drug with hemodynamic effects in patients with decompensated chronic heart failure. AIM: Short-term (one month) evaluation of clinical, hemodynamic and neurohormonal changes in patients with decompensated chronic heart failure undergoing levosimendan therapy. METHODS: Twenty-six (21 male) consecutive patients were studied, corresponding to 32 levosimendan administrations (bolus + 24h infusion), aged 56.7+/-13.0 years, with decompensated chronic heart failure, in NYHA functional class III-IV (78.1% in class IV), and cardiac index (CI) <2.5 l/min/m2. Clinical (NYHA class), non-invasive hemodynamic (echocardiography) and neurohormonal (Elecsys ECLIA NT-ProBNP) evaluations were performed before levosimendan administration and on days 1, 4, 10 and 30. RESULTS: 1) Until day 10, there was a progressive decrease in NT-ProBNP values and weight (p<0.001), with an increase in CI (p<0.001); 2) NYHA functional class improved progressively, with 76% of the patients in NYHA class II at day 30; 3) NT-ProBNP values at day 1 correlated inversely (r=-0.414; p=0.024) with CI at day 4; and 4) the absolute decrease in NT-ProBNP values at day 4 (relative to baseline values) correlated with weight loss at day 4 (r=0.495, p=0.005), day 10 (r=0.424, p=0.031) and day 30 (r=0.486, p=0.030). CONCLUSION: Levosimendan therapy in patients with decompensated chronic heart failure contributes to progressive NYHA class improvement. The variations seen in NYHA class and hemodynamics was reflected in changes in NT-ProBNP.

Left atrial function index predicts long-term survival in stable outpatients with systolic heart failure.

AIMS: Left atrial (LA) function index (LAFI) is a rhythm-independent index that combines LA emptying fraction (LAEF), adjusted LA volume (LAVi), and stroke volume. We evaluated LAFI as a predictor of long-term survival in outpatients with heart failure with reduced ejection fraction (HFrEF). METHODS AND RESULTS: For 3 years, we followed up 203 outpatients with a left ventricular ejection fraction <40%, who were clinically stable and on optimal therapy. The endpoint was all-cause death. LAFI was calculated as LAFI = ([LAEF × left ventricular outflow tract-velocity time integral]/[LAVi]), and was categorized into quartiles (9.26/16.56/31.92) and median (16.57). Incremental Cox regression models adjusted for significant confounders were used for survival analyses. The 3-year death rate was 30%. Higher quartiles had lower death rates (43.1%/45.1%/25.5%/6%, P < 0.001). The receiver operating characteristic curve for death was associated with LAFI (area under curve = 0.695, 95% CI 0.62-0.77, P < 0.001). In the direct comparison with LAVi and LAEF, LAFI (HRcox 0.93, 95% CI 0.89-0.97, P < 0.001) was the only predictor of survival. LAFI (HRcox 0.95, 95% CI 0.88-1.01, P = 0.099), LAFI quartiles (HR 0.29, 95% CI 0.125-0.672, P=0.004), and LAFI ≥16.57 (HRcox 0.62, 95% CI 0.38-1.02, P=0.058) were adjusted predictors of survival. Subgroup analysis by heart rhythm (sinus vs. atrial fibrillation) showed that LAFI per unit increase and LAFI quartiles were independent predictors of death in both subgroups. CONCLUSION: LAFI determination in HFrEF stable outpatients is a predictor of long-term survival and provides increased prognostic value over a wide range of confounder risk factors.

Red blood cell distribution width is a survival predictor beyond anemia and Nt-ProBNP in stable optimally medicated heart failure with reduced ejection fraction outpatients.

BACKGROUND: RDW is an automatic value obtained with the blood count, and represents the erythrocytes dimension variation. OBJECTIVE: To evaluate in optimally medicated outpatients with heart failure with reduced ejection fraction (HFrEF) the RDW prognostic value regarding survival in a multivariable model including anemia and Nt-ProBNP. METHODS: 233 consecutive outpatients, LVEF <40%, clinically stable were followed-up for 3-years in an HF Unit. End-point was all-cause death. The RDW categorized according to the tertiles (T1 = <13.9; T2 14-15.2; T3> = 15.3). Anemia classified according to the WHO criteria. Cox survival model adjusted for clinical profile, optimal therapeutic, renal function, Nt-ProBNP, etiology, atrial fibrillation, and anemia. RESULTS: (1) The 3-years death rate was 33.5%, and increased with the RDW tertiles (17.3%; 25%; 61.1%; p < 0.001). (2) The ROC curve for death associated with RDW (AUC 0.73; p < 0.001); (3) The adjusted death risk increased with the tertiles (Hazard-ratio '[HR] = 1.61; IC 95% 1.09-2.39; p = 0.017). RDW> = 15.3 had greater adjusted death risk than T1 (HR = 2.18; 95% CI 0.99-4.8; p = 0.05) and T1+T2 (HR = 1.54; 95% CI 1.13-2.09; p = 0.006). CONCLUSION: RDW determined in optimally medicated outpatients with HFrEF, during dry-state, is a strong, cheap, and independent predictor of long-term survival.

Furosemide Prescription During the Dry State Is a Predictor of Long-Term Survival of Stable, Optimally Medicated Patients With Systolic Heart Failure.

BACKGROUND: Furosemide is associated with poor prognosis in patients with heart failure and reduced ejection fraction (HFrEF). AIM: To evaluate the association between daily furosemide dose prescribed during the dry state and long-term survival in stable, optimally medicated outpatients with HFrEF. POPULATION AND METHODS: Two hundred sixty-six consecutive outpatients with left ventricular ejection fraction <40%, clinically stable in the dry state and on optimal heart failure therapy, were followed up for 3 years in a heart failure unit. The end point was all-cause death. There were no changes in New York Heart Association class and therapeutics, including diuretics, and no decompensation or hospitalization during 6 months. Furosemide doses were categorized as low or none (0-40 mg/d), intermediate (41-80 mg/d), and high (>80 mg). Cox regression was adjusted for significant confounders. RESULTS: The 3-year mortality rate was 33.8%. Mean dose of furosemide was 57.3 ± 21.4 mg/d. A total of 47.6% of patients received the low dose, 42.1% the intermediate dose, and 2.3% the high dose. Receiver operating characteristics for death associated with furosemide dose showed an area under the curve of 0.74 (95% confidence interval [CI]: 0.68-0.79; P < .001), and the best cutoff was >40 mg/d. An increasing daily dose of furosemide was associated with worse prognosis. Those receiving the intermediate dose (hazard ratio [HR] = 4.1; 95% CI: 2.57-6.64; P < .001) or high dose (HR = 19.8; 95% CI: 7.9-49.6; P < .001) had a higher risk of mortality compared to those receiving a low dose. Patients receiving >40 mg/d, in a propensity score-matched cohort, had a greater risk of mortality than those receiving a low dose (HR = 4.02; 95% CI: 1.8-8.8; P = .001) and those not receiving furosemide (HR = 3.9; 95% CI: 0.07-14.2; P = .039). CONCLUSION: Furosemide administration during the dry state in stable, optimally medicated outpatients with HFrEF is unfavorably associated with long-term survival. The threshold dose was 40 mg/d.

Treatment with Optimal Dose Angiotensin-Converting Enzyme Inhibitors/Angiotensin Receptor Blockers Has a Positive Effect on Long-Term Survival in Older Individuals (Aged >70 Years) and Octogenarians with Systolic Heart Failure.

BACKGROUND: Heart failure with reduced ejection fraction (HFrEF) is a disease of older people, but the target doses of angiotensin-converting enzyme inhibitors/angiotensin receptor blockers (ACEIs/ARBs) are unknown. OBJECTIVE: To evaluate the association of ACEI/ARB dose level with long-term survival in stable older patients (aged >70 years) and octogenarian outpatients with HFrEF. POPULATION AND METHODS: A total of 138 outpatients aged >70 years (35.5 % > 80 years), with an LVEF <40 % and who were clinically stable on optimal therapy were followed up for 3 years. The ACEI/ARB doses were categorized as: none (0), low (1-50 % target dose), and high (50-100 % target dose). The Cox regression survival model was adjusted for age, ischemic etiology, and renal function. RESULTS: ACEIs/ARBs were prescribed to 91.3 % of patients, and 52.9 % received the high dose. Survival improved with increasing ACEI/ARB dose level in the total population (Hazard Ratio [HR] = 0.67; 95 % confidence interval [CI] 0.55-0.82; p < 0.001), older patients aged >70 years (HR = 0.65; 95 % CI 0.51-0.83; p < 0.001), and octogenarians (HR = 0.71; 95 % CI 0.51-0.99; p = 0.045). The low (HR = 0.35; 95 % CI 0.16-0.76; p = 0.008) and high doses (HR = 0.13; 95 % CI 0.06-0.32; p < 0.001) improved survival compared with not receiving ACEIs/ARBs. The high dose was associated with a better survival than the low dose in the total population (HR = 0.35; 95 % CI 0.19-0.67; p = 0.001) and in a propensity score-matched cohort (HR = 0.41; 95 % CI 0.16-1.02; p = 0.056). In octogenarians, all dose levels were associated with improved survival compared with not receiving ACEIs/ARBs, but there was no difference between ACEI/ARB doses. CONCLUSION: The achieved optimal dose of ACEIs/ARBs in ambulatory older people with HFrEF is associated with long-term survival.

Atomic force microscopy as a tool to evaluate the risk of cardiovascular diseases in patients.

The availability of biomarkers to evaluate the risk of cardiovascular diseases is limited. High fibrinogen levels have been identified as a relevant cardiovascular risk factor, but the biological mechanisms remain unclear. Increased aggregation of erythrocytes (red blood cells) has been linked to high plasma fibrinogen concentration. Here, we show, using atomic force microscopy, that the interaction between fibrinogen and erythrocytes is modified in chronic heart failure patients. Ischaemic patients showed increased fibrinogen-erythrocyte binding forces compared with non-ischaemic patients. Cell stiffness in both patient groups was also altered. A 12-month follow-up shows that patients with higher fibrinogen-erythrocyte binding forces initially were subsequently hospitalized more frequently. Our results show that atomic force microscopy can be a promising tool to identify patients with increased risk for cardiovascular diseases.

Long-term prognostic value of protein C activity, erythrocyte aggregation and membrane fluidity in transmural myocardial infarction.

The objective of this study was to evaluate the long-term predictive value of the haemostatic, inflammatory and haemorheologic disturbances in transmural myocardial infarction (MI). Sixty-four (59 male) consecutive survivors of a MI, with a mean age of 58.3 +/- 12.0 years, were followed over a period of 36 months. Eighteen patients had a cardiovascular event defined as the composite of death, non-fatal MI, unstable angina and stroke. The haemostatic (protein C activity-PtC, antithrombin III, plasminogen activator inhibitor-1), haemorheologic (blood fluidity and components, erythrocyte membrane fluidity) and inflammatory (polymorphonuclear elastase, leukocyte count) profiles were determined at hospital discharge, using standard methodology. Our results can be summarized as follow: (i) at hospital discharge, the subgroup of patients with events had higher leukoactivity, leukocyte count, membrane fluidity, prognosis cyte count (7833.0 +/- 1696.0 vs. 10294.0 +/- 3129.0; p = 0.011), lower PtC (100.65 +/- 19.08 vs.81.25 +/- 19.95; p = 0.002), and lower erythrocyte aggregation (14.26 +/- 5.94 vs. 11.47 +/- 3.45; p = 0.031) in relation to the ones without events; (ii) By Cox regression the protein C activity lower tertile (OR 0.169; 0.045-0.628; p = 0.008); erythrocyte membrane outer layer fluidity upper tertile (OR 0.067; 95% CI 0.011 - 0.393; p = 0.003); and erythrocyte aggregation lower tertile (OR 0.182; 0.038 - 0.876; p = 0.034) were independent predictors of the composite endpoint. We can conclude that some haemostatic, haemorheologic and inflammatory disturbances, at hospital discharge, are long-term independent predictors of recurrent cardiovascular events in transmural myocardial infarction survivors.

Evidence of prolonged disturbances in the haemostatic, hemorheologic and inflammatory profiles in transmural myocardial infarction survivors.

Haemostatic, hemorheologic and inflammatory disturbances have been associated with acute coronary syndromes. Most knowledge is reported in cross sectional studies and are without time dependent evolution of these profiles. The aim of this study was to evaluate, during the first year, the evolution of the haemostatic, hemorheologic and inflammatory profiles determined at hospital discharge in survivors with transmural myocardial infarction (MI). Eighty eight (79 male; 9 female) mean age of 58 +/- 11 years, survivors of a transmural MI were prospectively studied at discharge, 6 months and one year after the event. Haemostatic (protein C, antithrombin III and plasminogen activator inhibitor 1), hemorheologic (blood fluidity and components) and inflammatory profiles (polymorphonuclear elastase and leukocyte count) were determined using standard methodology. The results of the study can be summarized as follows: (1) Protein C decreased (p < 0.05) over time while PAI-1 only varied significantly until 6(th) month. (2) Plasma viscosity and fibrinogen (p < 0.001) decrease over time, while erythrocyte aggregation (p < 0.001) and haematocrit increased. Whole blood viscosity did not vary. (3) Leukocyte decreased (p < 0.001) and elastase did not (4). Those patients with cardiovascular events (n = 7) had higher PAI-1 concentration (p < 0.05) and leukocyte count (p < 0.01), at discharge (5) Left ventricle ejection fraction correlated significantly with plasma viscosity (r = 0.35 p < 0.05). The results of this longitudinal study show dynamic modifications of the haemostatic, hemorheologic and inflammatory profiles during the first year of a transmural myocardial infarction. In addition, there are interrelations between them and the clinical profile that could help to explain the clinical evolution of this group of patients.

[Biohemorheologic factors and cardiovascular events curve in survivors of transmural acute myocardial infarct--24-month follow-up]. / Factores biohemorreológicos e a curva de eventos cardiovasculares em sobreviventes de enfarte agudo do miocárdio transmural--24 meses de seguimento clínico.

INTRODUCTION: Previous reports have shown several biohemorheological disturbances in acute myocardial infarction (AMI), either in the acute phase and after hospital discharge. There is no clearly established relationship between these parameters and the patients' clinical outcome. OBJECTIVE: To evaluate in transmural AMI survivors, a relationship between biohemorheological parameters and the cardiovascular events curve during a 24 month follow-up period. METHODS: Sixty-four consecutive patients (58.0 +/- 12.0, 59 men), transmural AMI survivors (30 anterior and 34 inferior) were included in the study. Clinical follow-up was 24 months (at 6, 12 and 24 months). The following cardiovascular events (CVE) were collected: cardiovascular death, stroke, AMI, unstable angina, embolism. We determined, at hospital discharge, these biohemorheological parameters: plasma viscosity, fibrinogen, PAI-1 inhibitor, leukocyte count, C protein (C Pt), erythrocyte aggregation (EA). For each parameter we determined the 25, 50 and 75 percentiles and other significant cut-off point, grouping patients according to these values. STATISTICS: Group t test, Kaplan-Meier survival curve (with the log rank test), and Cox logistic regression. RESULTS: (1) Patients with CVE (n = 19) during the 24 months of clinical follow-up had at hospital discharge higher leukocyte count (p < 0.001), lower C Pt (p < 0.01) and lower EA (p < 0.05). (2) The higher the percentile of the leukocyte count higher the probability for a CVE. Patients with leukocyte count above the 50 percentile had 6 times more CVE (p < 0.01); (3) The higher the C Pt lower the risk for a CVE. Patients with C PT lower than the 50 percentile had 9 times more risk for a CVE (p < 0.01), and those above the 75 percentile had no CVE (p < 0.01). (4) For the EA we identified a cutoff point (= 14.5), independent of the percentiles values. Patients with EA below 14.5 had six times more CVE. By multivariate analyses, we identified leukocyte count and C Pt as independent risk predictive factors (p < 0.05). CONCLUSION: In this group of transmural MI survivors a relationship was established between some biohemorheological (leukocyte count, C Pt, EA) and the CVE curve during 24 months of clinical follow-up.

Long-term prognostic value of the hemorheological profile in transmural myocardial infarction survivors: 60-month clinical follow-up.

UNLABELLED: Previous reports have shown several hemorheological and hemostatic abnormalities in acute coronary syndrome survivors. Some of these abnormalities were related to cardiovascular events during a 24-month follow-up. The aim of the present work is to evaluate, in transmural myocardial infarction survivors, the long-term (60 months) prognostic value of the biohemorheological profile determined at hospital discharge. Sixty-four patients (59 men), mean age of 58 +/- 12.0 years, transmural myocardial infarction survivors, were prospectively studied for 60 months (32.0 +/- 17 months, median 33 months). The following cardiovascular events (CVE) were analyzed: death, non-fatal infarction, unstable angina, and stroke. Twenty-nine patients had a CVE (nine died). The following parameters were determined at hospital discharge: plasma viscosity, whole blood viscosity, erythrocyte membrane fluidity, erythrocyte aggregation, protein C, plasminogen inhibitor type I (PAI-1), leukocyte count and elastase. The quartiles were determined for each parameter, grouping patients according to these values. STATISTICS: Group-t-test, Kaplan-Meier survival curve (with log rank test), and Cox logistic regression. RESULTS: 1) Leukocyte count (p < 0.01), protein C activity (p < 0.05) and erythrocyte membrane fluidity (p < 0.05) were predictors of the CVE curve; 2) The higher the value of the leukocyte count quartile, the higher the risk for a CVE (p < 0.05). Patients with a leukocyte count above the median had 4 times more risk for a CVE; 3) The lower the protein C activity, the higher the risk for a CVE. Those with protein C activity lower than the lowest quartile had double the risk; 4) The higher the membrane polarization value (membrane rigidity), the higher the risk of a CVE; 5) By multivariate analysis the 3 parameters were independent predictors of a CVE. CONCLUSION: In the present group of transmural myocardial infarction survivors a close relationship was established between hemorheologic, hemostatic and inflammatory factors and the cardiovascular events curve during long-term follow-up.

Plasma N-terminal pro-brain natriuretic peptide: a marker of left ventricular hypertrophy in hypertrophic cardiomyopathy.

BACKGROUND: B-type natriuretic peptide is secreted mainly in the left ventricle in response to elevated wall tension. Plasma levels of the peptide correlate positively with cardiac filling pressures, making it an excellent marker for the presence of left ventricular dysfunction. In hypertrophic cardiomyopathy, enhanced production of B-type natriuretic peptide is observed. However, the relationship of the various structural and functional features present in the disease with the high plasma levels described is not yet fully clarified. In the present study, we prospectively assessed in hypertrophic cardiomyopathy the relationship of plasma NT-proBNP levels with the extent of left ventricular hypertrophy, presence of left ventricular outflow obstruction and echocardiographic parameters of left ventricular diastolic function. METHODS: The study population included 190 individuals: 53 patients with hypertrophic cardiomyopathy and well-preserved left ventricular systolic function (group A), 92 healthy relatives with no disease expression (group B), and an additional group of 46 healthy volunteers (group C) as controls for NT-proBNP levels. Groups A and B were characterized clinically and by echocardiography and compared with each other. Plasma NT-proBNP levels were measured (ECLIA-Elecsys proBNP) and compared in the 3 groups of individuals included in the study. In hypertrophic cardiomyopathy patients, correlation was sought between NT-proBNP levels, NYHA functional class and echocardiographic data. RESULTS: Groups A and B differed (p < 0.001) in septal thickness, maximal wall thickness, left ventricular hypertrophy score, left atrial size, left atrial fractional shortening, derived transmitral filling indices and plasma NT-proBNP levels (group A: 909.9 +/- 1554.2 pg/ml; group B: 40.7 +/- 45.1 pg/ml). Left ventricular diastolic size and pulmonary venous flow velocity-derived indices were similar in the 2 groups. NT-proBNP levels in group B and C (39.4 +/- 34.5 pg/ml) were similar (p = NS). In hypertrophic cardiomyopathy patients, NT-proBNP levels correlate directly with NYHA functional class (r = 0.56, p < 0.001), septal thickness (r = 0.53, p < 0.001), maximal wall thickness (r = 0.59, p < 0.001), left ventricular hypertrophy score (r = 0.63, p < 0.001), left atrial size (r = 0.32, p = 0.023) and mitral deceleration time (r = 0.46, p = 0.001) and inversely with left atrial fractional shortening (r = -0.41, p = 0.005). Functional class also correlates directly with left ventricular hypertrophy score (r = 0.39, p = 0.006), with the most symptomatic patients having the highest scores. CONCLUSIONS: In hypertrophic cardiomyopathy, plasma NT-proBNP levels depend mainly on the severity of left ventricular hypertrophy rather than on the presence of obstruction. Measurement of the peptide may help in the clinical characterization and follow-up of patients with this disease.

Early detection of sympathetic myocardial denervation in patients with familial amyloid polyneuropathy type I.

INTRODUCTION: Type I familial amyloid polyneuropathy (FAP I) is an autosomal dominant inherited disorder due to a genetic defect in transthyretin and is characterized by deposition of amyloid in various organs and tissues. The principal manifestations are related to polyneuropathy and dysautonomia. The aim of this study was to assess cardiac involvement and to correlate the findings with neurological status. METHODS: 34 patients with FAP (15 male and 19 female; mean age 43 +/- 15 years) underwent I123-labeled metaiodobenzylguanidine (MIBG) myocardial scintigraphy in order to evaluate cardiac sympathetic innervation. In addition they underwent ambulatory blood pressure monitoring (ABPM) and two-dimensional and Doppler echocardiography. Neurological involvement was quantified according to a neurophysiologic score (EMG; 0 = no abnormality and 100% = maximal disability). RESULTS: The mean value of cardiac MIBG uptake was 1.75 +/- 0.5 (normal = 2.6 +/- 0.3) and correlated inversely with the EMG score (r = -0.67; p = 0.001). In 27 (79%) of the 34 patients there was a decrease in MIBG accumulation, in 18 (53%) an alteration in the circadian BP pattern and/or an increase in systolic and/or diastolic BP loads at night, and in 17 (50%) left ventricular hypertrophy and/or diastolic dysfunction. Twenty-two patients were symptomatic and had a mean EMG score of 37.7 +/- 25% (group I). The remaining 12 were asymptomatic and without neurological involvement (group II). Group I was characterized by older age (48 +/- 15 vs. 33 +/- 10.2 years, p = 0.01), lower MIBG uptake (1.5 +/- 0.4 vs. 2.2 +/- 0.5, p = 0.001), higher systolic (129 +/- 16 vs. 119 +/- 6 mmHg, p = 0.01) and diastolic daytime BP (82 +/- 10 vs. 76 +/- 6 mmHg, p = 0.05), and higher systolic (119 +/- 17 vs. 105 +/- 7 mmHg, p = 0.01) and diastolic nocturnal BP (71 +/- 11 vs. 62 +/- 9 mmHg, p = 0.01) than patients in group II. In 21/22 patients in group I and in 6/12 in group II there was a decrease in cardiac MIBG activity. Sixteen patients in group I and 2 in group II had abnormal circadian BP pattern. Left ventricular hypertrophy was only seen in group I. CONCLUSIONS: Patients with FAP have a high incidence of cardiac denervation and an abnormal circadian BP pattern. These alterations in cardiac autonomic function precede the development of clinical manifestations and may be an important factor in determining the optimal timing for liver transplantation, which is currently the only way to control the progression of the disease.

Myocardial perfusion and angioplasty. Comparison of myocardial contrast echocardiography and scintigraphy.

OBJECTIVES: To estimate the efficacy of myocardial contrast echocardiography (MCE) by harmonic power imaging (HPI), in evaluation of perfusion in one-vessel coronary disease treated by angioplasty, using myocardial scintigraphy as gold standard. STUDY DESIGN: Prospective comparative study. SETTING: Ambulatory. POPULATION: We included 33 patients (pts), aged 53.5 +/- 9 years, 27 male. INCLUSION CRITERIA: pts with one-vessel coronary disease (> or = 70% stenosis), with indication for angioplasty; sinus rhythm; good echocardiographic window with harmonic imaging. Exclusion criterion: previous myocardial infarction. METHODS: All patients underwent myocardial scintigraphy and HPI together with stress echocardiography, both followed by angioplasty (stenting in ten). HPI and myocardial scintigraphy were repeated, in all patients, at three months after intervention. Ten patients were re-assessed by coronary angiography for ischemia on the scintigraphic study. For the HPI exam, Levovist was selected as contrast and dipyridamole as stress agent (0.56 mg/kg). Perfusion was assessed visually and classified by HPI and scintigraphy studies as: 1 (normal), 2 (reduced), or 3 (absent). For analysis, the left ventricle was divided into 16 segments. RESULTS: Of the 43 coronary angiograms performed (ten at three months after angioplasty), 38 showed 70% stenosis, none occlusive or subocclusive. We analyzed 1056 left ventricle segments, from 66 HPI and myocardial scintigraphy studies (before and after angioplasty). Analysis was impossible or doubtful in 4.9%. Baseline and stress HPI detected 216 perfusion abnormalities. Global concordance between the segmental perfusion score obtained by HPI and scintigraphy was 66.2%, which became 76.3% when two groups were considered: a) score 1 b) score 2 and 3 together. In comparison with scintigraphy, HPI sensitivity for detection of perfusion abnormalities was 79.3% (higher for anterior septum, anterior and lateral wall) and specificity was 91.4% (higher for septum, inferior wall and apical segments). HPI correctly identified the location of coronary stenosis in 73.5% of patients. CONCLUSIONS: In our study, HPI was a feasible and promising method for assessment of perfusion in one-vessel coronary disease and chronic ischemia. In comparison with myocardial scintigraphy, a high concordance for perfusion score was found, as well as high sensitivity and specificity for detection of perfusion abnormalities.