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BACKGROUND: The differentiation of constrictive pericarditis (CP) from restrictive cardiomyopathy (RCM) may be clinically difficult and may require multiple investigations. Even though brain natriuretic peptide (BNP) is shown to be higher in patients with RCM as compared to CP, the clinical utility is not fully established especially in Indian patients known to have advanced CP and myocardial involvement. METHODS AND RESULTS: We measured NT-pro-BNP levels in 49 patients suspected of having either CP or RCM, diagnosed on the basis of echocardiography, computed tomography, magnetic resonance imaging, endomyocardial biopsy and cardiac catheterization data as needed. Twenty nine patients (Mean age - 26 yrs, 24 males) had CP and 20 patients (Mean age - 39 yrs, 14 males) had RCM. The median plasma NT-pro-BNP levels were significantly higher in RCM as compared to CP [1775 (208-7500) pg/ml vs 124 (68-718) pg/ml, respectively; p = 0.001]. A cut off value of 459 pg/ml had sensitivity, specificity and overall accuracy of 90%, 86% and 88% respectively, for differentiating CP from RCM. CONCLUSIONS: The NT-pro-BNP levels are significantly elevated in RCM as compared to CP.

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The authors investigated the diagnostic utility of plasma N-terminal pro-B-type natriuretic peptide (NT proBNP) and C-reactive protein (CRP) levels in the differential diagnosis of constrictive pericarditis (CP) and restrictive cardiomyopathy (RC). Twenty-five patients with high clinical suspicion of either CP or RC were enrolled. Mean plasma NT proBNP levels were significantly higher in patients with RC compared to those with CP (2641 +/- 2902 pg/mL vs 628 +/- 678 pg/mL; P=.003). The NT proBNP level that provided the best sensitivity and specificity for the differentiation of CP and RC was 800 pg/mL. Mean CRP levels were higher in patients with CP than with RC (1.41 +/- 1.73 mg/dL vs 0.38 +/- 0.21 mg/dL; P=.03). The CRP level that provided the best sensitivity and specificity for the differentiation of CP and RC was 0.57 mg/dL. Plasma NT proBNP and CRP levels can be useful in the differential diagnosis of RC and CP.

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Severe diastolic dysfunction has important clinical implications in advanced systolic heart failure. The authors investigated whether a marker of fibrosis, serum carboxy-terminal peptide of procollagen type I (PICP) is a major determinant of diastolic function in 40 patients with heart failure and ejection fraction <35%. Patients with unstable heart failure or ischemic symptoms were excluded. The authors found PICP to be an independent predictor of diastolic function in addition to age and pulmonary artery systolic pressure. The authors' findings suggest that studies evaluating whether therapy that improves myocardial fibrosis could have a favorable impact on diastolic function in this population are warranted.

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BACKGROUND: Differentiating between constrictive pericarditis (CP) and restrictive cardiomyopathy (RCMP) is difficult because of similar clinical and hemodynamic presentation. Brain natriuretic peptide (BNP) has been reported a useful noninvasive biomarker to differentiate CP from RCMP; however, its utility in patients with renal insufficiency has not been evaluated. METHODS AND RESULTS: Consecutive patients with suspected CP or RCMP were enrolled. All but 7 patients underwent transseptal catheterization. BNP, renal function, and comorbid conditions were recorded at the time of the procedure. Renal function was estimated using the Cockcroft-Gault formula. Descriptive statistics, Student t-test, and Mann-Whitney U test were performed; P < .05 was significant. Twenty-two patients had hemodynamically or surgically proven CP or RC. In patients with CP, 9 had at least Stage II kidney disease (GFR <90 mL/min, mean 58) and 8 had normal or Stage I kidney disease (GFR >90 mL/min, mean 118). BNP was higher in patients with CP and renal insufficiency versus those with CP and normal renal function (433 versus 116 pg/mL; P = .016). BNP in patients with CP and normal renal function was lower than in patients with RC (116 versus 728 pg/mL; P = .005). CONCLUSION: BNP has reduced clinical utility in renal insufficiency to differentiate CP from RCMP.

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We report the case of a 35-year-old man with constrictive pericarditis who had a B-type natriuretic peptide (BNP) level of 129 pg/dl despite a left ventricular end diastolic pressure of 35 mmHg. We discuss a possible explanation for the relatively low BNP level given this patient's markedly elevated intracavitary pressures in the setting of constrictive pericarditis.

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BACKGROUND: Primary desminopathies are caused by desmin gene [DES (MIM*125660)] mutations. The clinical spectrum includes pure myopathies, cardiomuscular diseases and cardiomyopathies. Patients with restrictive cardiomyopathy (RCM) plus atrioventricular block (AVB) due to DES defects are frequently unrecognized unless desmin accumulation is specifically investigated in endomyocardial biopsy (EMB) by ultrastructural study. AIMS: To describe a cardiological phenotype characterized by RCM plus AVB due to desmin accumulation caused by DES defects. METHODS AND RESULTS: Desmin accumulation was diagnosed by means of ultrastructural and immunocytochemical studies of EMB in four unrelated probands with RCM and AVB. Candidate genes [DES and alphaB-crystallin (CRYAB)] were screened using sequence analysis. Four DES gene mutations were identified: three new (R16C, T453I and a 10 bp deletion at the exon-intron boundary of exon 3 disrupting the donor splice site) and one known (R406W). The disease was autosomal dominant in two families, recessive in one and associated with a de novo mutation in one. The mutations cosegregated with phenotype in all patients. CRYAB gene screening was negative. CONCLUSIONS: A cardiac phenotype characterized by RCM and AVB caused by desmin accumulation is associated with DES mutations. Although the mutations affected different domains, the cardiac phenotype was identical.

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Results of cardiac muscle and skeletal muscle biopsies were compared in 22 patients with cardiomyopathy; 11 patients presented with symptoms secondary to ventricular tachycardia (Group 1) and 11 had symptoms of severe congestive heart failure (Group 2). No patient had structural or ischemic cardiac disease. In Group 1 patients, hemodynamic abnormalities were subtle, but invasive study demonstrated dilated cardiomyopathy in two patients and restrictive cardiomyopathy in nine. In Group 2, eight patients had dilated cardiomyopathy and three had restrictive cardiomyopathy. Cardiac biopsy results were abnormal in all 22 patients and the abnormalities were similar for the two groups. Cardiac histologic study revealed a spectrum of abnormalities including fibrosis, dilated sarcoplasmic reticulum, increased numbers of intercalated discs and mitochondrial abnormalities. Histologic abnormalities of skeletal muscle were similar in each group, consisting of endomysial fibrosis and increased lipid deposits. Slightly more than half of the Group 1 and Group 2 patients also had a low concentration of skeletal muscle long chain acylcarnitine. These data demonstrate that abnormalities of both cardiac and skeletal muscle are common in patients with cardiomyopathy; abnormalities are similar whether initial symptoms are due to ventricular tachycardia or congestive heart failure. It is suggested that these patients with cardiomyopathy may have a generalized myopathy.

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