natriuretic-peptide--brain and Arrhythmogenic-Right-Ventricular-Dysplasia

Arrhythmogenic right ventricular dysplasia (ARVD) is characterized by local or diffuse wall motion abnormalities in the right ventricle (RV), associated with recurrent ventricular tachycardia (VT) of RV origin. Brain natriuretic peptide (BNP) was first isolated from a porcine brain extract. In humans, BNP is expressed predominantly in the ventricles of failing hearts, and its expression has been observed primarily in myocytes in the interstitial fibrous area in dilated cardiomyopathy. We hypothesized that BNP is increasingly secreted from the residual myocytes within the atrophic tissue in patients with ARVD.. Plasma BNP levels were measured in 17 patients with ARVD, 12 patients with idiopathic RV outflow tract tachycardia (RVOT), and 120 control subjects. We performed cardiac catheterization, RV endomyocardial biopsy, electron- beam CT, and biventricular endomyocardial mapping in the ARVD patients. There was a significant increase in plasma BNP levels in the ARVD patients compared with the RVOT patients and control subjects (61.4+/-59.6 pg/mL versus 8.3+/-5. 5 pg/mL and 9.3+/-5.8 pg/mL; P<0.0001, respectively). The plasma BNP levels had no correlation with any of the hemodynamic data, but they had a significant correlation with the RV ejection fraction (r=-0. 588, P=0.025) and with the fractionated-area scores (r=0.705, P=0. 005). Light microscopic immunohistochemistry showed strong BNP immunoreactivity in residual myocytes with fibrofatty replacement.. These results suggest that plasma BNP levels were not increased in RVOT patients but were increased in ARVD patients, and that the increased BNP levels indicate the severity of both the RV dysfunction and the arrhythmogenic substrate.

Topics: Adolescent; Adult; Aged; Arrhythmogenic Right Ventricular Dysplasia; Electrocardiography; Female; Hemodynamics; Humans; Immunohistochemistry; Male; Middle Aged; Myocardium; Natriuretic Peptide, Brain; Stroke Volume; Tachycardia, Ventricular; Ventricular Dysfunction, Right

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a progressive disease leading to ventricular arrhythmias and heart failure. Determining optimal time for heart transplantation (HTx) is challenging; therefore, it is necessary to identify risk factors for disease progression.. The study aimed to identify predictors of end‑stage heart failure and to evaluate the role of biomarkers in predicting adverse outcomes in ARVC.. A total of 91 individuals with ARVC (59 men; mean [SD] age, 47 [16] years) were included. In all patients, information on medical history was collected, electrocardiography and echocardiography were performed, and serum levels of selected biomarkers (soluble form of the ST2 protein [sST2], galectin‑3 [Gal‑3], extracellular matrix metalloproteinases [MMP‑2 and MMP‑9], N‑terminal pro-B‑type natriuretic peptide [NT‑proBNP], and high‑sensitivity troponin T [hs‑TnT]) were measured. Thereafter, the participants were followed for the primary end point of death or HTx, as well as the secondary end point of major arrhythmic events (MAEs), defined as sudden cardiac death, ventricular fibrillation, sustained ventricular tachycardia, or appropriate implantable cardioverter‑defibrillator intervention.. During the median (interquartile range) follow‑up of 36.4 (29.8-41.2) months, 13 patients (14%) reached the primary end point of death or HTx, and 27 (30%) experienced MAEs. The patients who achieved the primary end point had higher levels of sST2, MMP‑2, NT‑proBNP, and hs‑TnT, but not of Gal-3 and MMP-9. Three factors turned out to be independent predictors of death or HTx: higher NT‑proBNP concentration (≥890.3 pg/ml), greater right ventricular end‑diastolic area (≥39 cm2), and a history of atrial tachycardia. None of the biomarkers predicted MAEs.. An NT‑proBNP concentration greater than or equal to 890.3 pg/ml, right ventricular end-diastolic area of 39 cm2 or greater, and a history of atrial tachycardia were identified as risk factors for death or HTx in ARVC. Higher levels of sST2, MMP‑2, NT‑proBNP, and hs‑TnT were associated with reaching the primary end point of death or HTx. The biomarkers had no value in predicting ventricular arrhythmias.

Topics: Adult; Aged; Arrhythmias, Cardiac; Arrhythmogenic Right Ventricular Dysplasia; Biomarkers; Electrocardiography; Female; Heart Failure; Heart Transplantation; Humans; Male; Middle Aged; Natriuretic Peptide, Brain; Peptide Fragments; Risk Factors

Although N-terminal pro-brain natriuretic peptide (NT-proBNP) is a useful screening test of impaired right ventricular (RV) function in conditions affecting the right-sided cardiac muscle, the role of NT-proBNP remains unclear in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC). This study was designed to clarify the relation between the plasma NT-proBNP level and the RV function evaluated by cardiovascular magnetic resonance (CMR) imaging. We selected 56 patients with confirmed ARVC only when their blood specimens for NT-proBNP measurements were collected within 48 hours of a CMR scan. The NT-proBNP level was significantly higher in patients with RV dysfunction than in patients without RV dysfunction (median of 655.3 [interquartile range 556.4 to 870.0] vs 347.0 [interquartile range 308.0 to 456.2] pmol/L, p <0.001). The NT-proBNP levels were positively correlated with RV end-diastolic and end-systolic volume indices (r = 0.49 and 0.70, respectively) and negatively correlated with RV ejection fraction (r = -0.76, all p <0.001), which remained significant after adjustment for age, gender, and body mass index. The area under the receiver-operating characteristic curve for NT-proBNP was 0.91 (95% confidence interval 0.80 to 0.97, p <0.001). The cut-off value of NT-proBNP (458 pmol/L) was associated with sensitivity, specificity, and positive and negative predictive values of 91%, 89%, 67%, and 98%, respectively. In conclusion, NT-proBNP is a useful marker for the detection of RV dysfunction and associated with extent of RV dilatation and dysfunction determined by CMR in patients with ARVC.

Topics: Adult; Arrhythmogenic Right Ventricular Dysplasia; Biomarkers; Female; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Natriuretic Peptide, Brain; Peptide Fragments; Retrospective Studies; Sensitivity and Specificity; Ventricular Function, Right; Ventricular Remodeling

Arrhythmogenic right ventricular (RV) cardiomyopathy (ARVC) is a regional disease of the RV myocardium with variable degrees of left ventricular involvement. Three-dimensional echocardiography and Doppler tissue imaging (DTI) are new echocardiographic modalities for the evaluation of global and regional function, but the diagnostic potential remains to be assessed.. Twenty patients with previously established ARVC were evaluated by 3-dimensional echocardiography and DTI, and compared with 32 age- and sex-matched control subjects.. Using 3-dimensional echocardiography, patients with ARVC had a decreased RV ejection fraction (0.47 +/- 0.08 vs 0.53 +/- 0.05, P < .01), and a decreased peak lateral systolic annular velocity by pulsed wave imaging of both the RV (11.9 +/- 2.6 vs 15.1 +/- 3.7 cm/s, P < .01) and the left ventricle (7.0 +/- 2.6 vs 9.5 +/- 1.9 cm/s, P < .01). DTI showed decreased regional systolic strain, but with wide variation in the measurements.. Three-dimensional echocardiography identifies decreased RV ejection fraction in ARVC. Assessment of regional contractility by DTI is limited by wide variation. Echocardiographic evaluation of the longitudinal motility appears to be a sensitive marker of preclinical left ventricular involvement.

Topics: Adult; Aged; Arrhythmogenic Right Ventricular Dysplasia; Atrial Natriuretic Factor; Biomarkers; Biopsy, Needle; Case-Control Studies; Confidence Intervals; Echocardiography; Echocardiography, Three-Dimensional; Female; Heart Function Tests; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Natriuretic Peptide, Brain; Probability; Reference Values; Sensitivity and Specificity; Severity of Illness Index; Ventricular Dysfunction, Right

To determine whether Boxers with a clinical diagnosis of arrhythmogenic right ventricular cardiomyopathy (ARVC) have increased plasma concentrations of brain natriuretic peptide (BNP), compared with concentrations in clinically normal dogs.. 13 Boxers with ARVC, 9 clinically normal Boxers, 10 clinically normal non-Boxer dogs, and 5 hound dogs with systolic dysfunction.. All Boxers were evaluated via 24-hour ambulatory electrocardiography and echocardiography; the number of ventricular premature contractions (VPCs) per 24 hours was assessed. Hound dogs with cardiac pacing-induced systolic dysfunction (positive control dogs) and clinically normal non-Boxer dogs (negative control dogs) were evaluated echocardiographically. Three milliliters of blood was collected from each dog for measurement of plasma BNP concentration by use of a radioimmunoassay.. Mean +/- SD plasma BNP concentration for the ARVC-affected Boxers, clinically normal Boxers, negative control dogs, and positive control dogs was 11.0 +/- 4.6 pg/mL, 7.9 +/- 3.2 pg/mL, 11.5 +/- 4.9 pg/mL, and 100.8 +/- 56.8 pg/mL, respectively. Compared with findings in the positive control group, plasma BNP concentration in each of the other 3 groups was significantly different. There was no significant difference in BNP concentration between the 2 groups of Boxers. A significant correlation between plasma BNP concentration and number of VPCs per 24 hours in the ARVC-affected Boxers was not identified.. A significant difference in BNP concentration between Boxers with ARVC and clinically normal Boxers was not identified. Results suggest that BNP concentration may not be an indicator of ARVC in Boxers.

Topics: Animals; Arrhythmogenic Right Ventricular Dysplasia; Dog Diseases; Dogs; Echocardiography; Electrocardiography; Natriuretic Peptide, Brain; Radioimmunoassay