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PubMed2005

Genetic linkage of a novel autosomal dominant restrictive cardiomyopathy locus.

BACKGROUND: In recent years, non-syndromic idiopathic cardiomyopathies have increasingly been characterised as autosomal dominant conditions caused by single gene mutations. Loci have been identified for hypertrophic and dilated cardiomyopathy, and in some cases the same loci are associated with restrictive cardiomyopathy (RCM). In a kindred with RCM that we previously reported, we ruled out the known cardiomyopathy loci and other candidate genes by linkage analysis and mutation screening. METHODS AND RESULTS: Here we report a genome-wide analysis in this family that has resulted in linkage to a region on chromosome 10. CONCLUSIONS: There are no genes in the interval that are known to cause idiopathic cardiomyopathy, and thus this linkage represents localisation of a new RCM locus.

Cardiomyopathy, Restrictive↗

PubMed2001

Clinical spectrum of restrictive cardiomyopathy in children.

We reviewed the clinical spectrum and possible prognostic factors in 14 children with restrictive cardiomyopathy. The patients were not homogeneous in clinical presentation or morphology. The mortality rate was high: 21.4% at 1 year and 50% at 2 years after presentation. Younger patients with respiratory symptoms, thromboembolism, increased cardiothoracic ratio on chest radiogram or patients with endocardial fibroelastosis appear to have a worse prognosis and orthotopic cardiac transplantation may be indicated.

Adolescent↗

PubMed1993

The differentiation between restrictive cardiomyopathy and constrictive pericarditis: the impact of the imaging techniques.

The differentiation between constrictive pericarditis and restrictive cardiomyopathy remains a difficult problem for clinical cardiologists. Recent advances in imaging techniques and the understanding of diastolic function have created a new diagnostic approach to this problem. In this article we will summarize the recent advances in the understanding of the pathophysiology of both disorders and how this is reflected mainly in the use of flow imaging techniques, such as Doppler echocardiography and radionuclide angiography. Combined with the advances in the radiological imaging of the pericardium by means of computed tomography and magnetic resonance imaging, an integrated approach to the differential diagnostic problem is proposed and an algorithm for clinical use has been designed.

Algorithms↗

PubMed1990

[Idiopathic restrictive cardiomyopathy. Description of 4 cases].

The clinical and instrumental characteristics of four patients suffering from idiopathic restrictive cardiomyopathy are analysed. Patients were diagnosed following hemodynamic and histological tests. The major clinical symptom was cardiac decompensation and/or hyperkinetic or hypokinetic (3rd degree BAV) supraventricular arrhythmia (atrial fibrillation). Electrocardiographic and radiological alterations were not specific. The most typical echocardiographic symptom was the association of: biatrial dilation, dilation and hypertrophy of the right ventricle, normal left ventricular size, normal fractional shortening of the left ventricle. Septal hypertrophy was also observed in one case. Abundant pericardial effusion was present in all cases. When performed, echo-Doppler showed a relatively typical shortened deceleration time through the A-V valve. Catheterism revealed increased ventricular telediastolic pressures and a bi-plateau morphology of the ventricular pressure curve. Heart index was depressed in one patient and the ejection fraction was reduced in one case. Histological tests revealed interstitial fibrosis, cellular hypertrophy and, in one patient, myocardial amyloidosis. One patient underwent a heart transplant, two patients died while waiting for a transplant, one patient is still alive 13 years after the start of 3rd class symptoms.

Adult↗

PubMed1990

Familial restrictive cardiomyopathy with atrioventricular block and skeletal myopathy.

Five generations of an Italian family with an autosomal dominant restrictive cardiomyopathy are described. Members of four generations were examined. Symptoms usually developed in the third or fourth decade but the disease did occur in childhood. Initially the condition was characterised by normal ventricular size and systolic function with increased diastolic filling pressures in both ventricles and consequent bi-atrial enlargement. Cardiac catheterisation showed a left ventricular filling pattern of "dip and plateau". The electrocardiogram typically showed non-specific changes in the ST segment and T wave and changes indicating considerable atrial enlargement, which were confirmed by echocardiography. Light microscopy of two endocardial biopsy specimens showed no specific features but excluded the endomyocardial fibrosis of eosinophilic heart disease, amyloid, and specific heart muscle diseases. At necropsy in one case examined under light microscopy extensive patchy fibrosis was found throughout the endocardium, myocardium, and subepicardium, but there were no features typical of eosinophilic heart disease. Histopathological and biochemical examination of skeletal muscle identified no abnormality. The disease often had an insidious course over five to ten years after presentation. Bundle branch blocks, leading to complete atrioventricular block, however, often occurred and may be the first manifestation. Some individuals who survived into the fifth decade developed a progressive, non-wasting skeletal myopathy.

Adolescent↗

PubMedDate not supplied

Diastolic heart failure: restrictive cardiomyopathy, constrictive pericarditis, and cardiac tamponade: clinical and echocardiographic evaluation.

An understanding of the basic principles of diastolic function is important in order to recognize diseases that may result in diastolic dysfunction and diastolic heart failure. Although uncommon, restrictive cardiomyopathy, constrictive pericarditis, and cardiac tamponade are among the disorders that may affect primarily diastolic function with preservation of systolic function. Diastolic heart failure may manifest with chronic nonspecific symptoms or may present with acute hemodynamic compromise. Echocardiography plays a vital role in the diagnosis of diastolic dysfunction and differentiation of these disease processes. It also provides a basis for clinical decisions regarding management and surgical referral. This review summarizes the clinical features, pathophysiology, and hemodynamic and echocardiographic signs of restrictive cardiomyopathy, constrictive pericarditis, and cardiac tamponade.

Cardiac Tamponade↗

PubMed1993

Restrictive cardiomyopathy with complete atrioventricular block and distal myopathy with rimmed vacuoles.

Two-dimensional echocardiography, cardiac catheterization, electrophysiologic studies, and biopsies of cardiac and skeletal muscle were performed in a patient with complete atrioventricular block as the initial manifestation of restrictive cardiomyopathy. The patient also showed distal myopathy with rimmed vacuoles. Weakness of the distal leg muscles occurred several years after the initial cardiac manifestations and the patient developed congestive heart failure 1 year before the second admission. Endomyocardial biopsy showed patchy myocardial fibrosis without endomyocardial fibrosis or any other specific findings of myocardial disease. Histochemical examination of skeletal muscle showed the typical features of distal myopathy with rimmed vacuoles. In some patients with atrioventricular block as the initial manifestation of restrictive cardiomyopathy, the cardiomyopathy may be just 1 manifestation of a more generalized disorder affecting both cardiac and skeletal muscle.

Adult↗

PubMed2006

Infantile restrictive cardiomyopathy resulting from a mutation in the cardiac troponin T gene.

Here we report the first infantile case of restrictive cardiomyopathy caused by a de novo mutation of the cardiac troponin T gene. The patient presented with an apparent life-threatening event. She developed malignant arrhythmias and hemodynamic instability, requiring initial rescue support with extracorporeal membrane oxygenation, and subsequently underwent insertion of a biventricular assist device (VAD). She successfully received an orthotopic heart transplant 172 days after VAD implantation.

Cardiomyopathy, Restrictive↗

PubMed2005

The efficacy of brain natriuretic peptide levels in differentiating constrictive pericarditis from restrictive cardiomyopathy.

OBJECTIVES: We sought to determine the usefulness of brain natriuretic peptide (BNP) measurements to differentiate constrictive pericarditis (CP) from restrictive cardiomyopathy (RCMP). BACKGROUND: The differentiation of CP from RCMP may be clinically difficult and often requires hemodynamic assessment. No laboratory marker has been shown to differentiate the two conditions. METHODS: We measured BNP levels in 11 patients suspected of having either CP or RCMP. All patients had hemodynamic assessment the day of BNP measurements. RESULTS: Six patients had CP and five patients had RCMP based on established hemodynamic criteria. Both CP and RCMP patients had similar elevation in intracardiac pressures. Despite similar pressures, the mean plasma BNP levels were significantly higher in RCMP compared to CP (825.8 +/- 172.2 pg/ml vs. 128.0 +/- 52.7 pg/ml, p < 0.001, respectively). CONCLUSIONS: The BNP levels are significantly elevated in RCMP compared to CP patients; BNP may prove to be a useful noninvasive marker for the differentiation of the two conditions.

Cardiomyopathy, Restrictive↗

PubMed2000

Differentiation between restrictive cardiomyopathy and constrictive pericarditis by early diastolic doppler myocardial velocity gradient at the posterior wall.

BACKGROUND: The differential diagnosis between restrictive cardiomyopathy (RCM) and constrictive pericarditis (CP) is challenging and, despite combined information from different diagnostic tests, surgical exploration is often necessary. METHODS AND RESULTS: A group of 55 subjects (mean age, 63+/-11 years; 36 men and 19 women) were enrolled in the study; 15 had RCM, 10 had CP, and 30 were age-matched, normal controls. The diagnosis of RCM was supported by a biopsy; in the CP group, the diagnosis was confirmed either surgically or at autopsy. All patients underwent a transthoracic echocardiogram that included the assessment of Doppler myocardial velocity gradient (MVG), as measured from the left ventricular posterior wall during the predetermined phases of the cardiac cycle. MVG was lower (P<0.01) in RCM patients compared with both CP patients and normal controls during ventricular ejection (2. 8+/-1.2 versus 4.4+/-1.0 and 4.7+/-0.8 s(-1), respectively) and rapid ventricular filling (1.9+/-0.8 versus 8.7+/-1.7 and 3.7+/-1.4 s(-1), respectively). Additionally, during isovolumic relaxation, MVG was positive in RCM patients and negative in both CP patients and normal controls (0.7+/-0.4 versus -1.0+/-0.6 and -0.4+/-0.3 s(-1), respectively; P<0.01). During atrial contraction, MVG was similarly low (P<0.01) in both RCM and CP patients compared with normal controls (1.6+/-1.7 and 1.7+/-1.8 versus 3.8+/-0.9 s(-1), respectively). CONCLUSIONS: Doppler myocardial imaging-derived MVG, as measured from the left ventricular posterior wall in early diastole during both isovolumic relaxation and rapid ventricular filling, allows for the discrimination of RCM from CP.

Aged↗

PubMed2003

Restrictive cardiomyopathy with right-sided dominant heart failure after sinus conversion from atrial fibrillation: case report.

A 44-year-old woman with restrictive cardiomyopathy showed right-sided dominant heart failure after conversion to sinus rhythm from paroxysmal atrial fibrillation (AF). During cardiac catheterization, the hemodynamics were compared before and after cardioversion; that is, with AF and with sinus rhythm. The hemodynamic parameters worsened after conversion to sinus rhythm, with a significant decrease in heart rate, but improved with atrial pacing. Pacemaker implantation was performed to avoid bradycardia while keeping the sinus rhythm with amiodarone. As a result, the patient has been free from symptoms of heart failure for almost 1 year and her serum brain natriuretic peptide level also improved remarkably.

Adult↗

PubMed2004

Cardiac tamponade, constrictive pericarditis, and restrictive cardiomyopathy.

The pericardium envelopes the cardiac chambers and under physiological conditions exerts subtle functions, including mechanical effects that enhance normal ventricular interactions that contribute to balancing left and right cardiac outputs. Because the pericardium is non-compliant, conditions that cause intrapericardial crowding elevate intrapericardial pressure, which may be the mediator of adverse cardiac compressive effects. Elevated intrapericardial pressure may result from primary disease of the pericardium itself (tamponade or constriction) or from abrupt chamber dilatation (eg, right ventricular infarction). Regardless of the mechanism leading to increased intrapericardial pressure, the resultant pericardial constraint exerts adverse effects on cardiac filling and output. Constriction and restrictive cardiomyopathy share common pathophysiological and clinical features; their differentiation can be quite challenging. This review will consider the physiology of the normal pericardium and its dynamic interactions with the heart and review in detail the pathophysiology and clinical manifestations of cardiac tamponade, constrictive pericarditis, and restrictive cardiomyopathy.

Algorithms↗

PubMed2002

Noninvasive identification of myocardial sympathetic and metabolic abnormalities in a patient with restrictive cardiomyopathy--in comparison with perfusion imaging.

A 42-year-old man had the insidious onset of heart failure, and was diagnosed as having restrictive cardiomyopathy. Doppler echocardiography study showed short deceleration time of the E wave and short isovolumic relaxation time on transmitral Doppler flow. He underwent Tl-201, I-123 beta-methyl-iodophenyl pentadecanoic acid (BMIPP) and I-123-metaiodobenzylguanidine (MIBG) cardiac scintigraphy. Tl-201 studies showed normal uptake in the left ventricle indicating normal blood perfusion. I-123 BMIPP and I-123 MIBG showed reduced uptake in the inferior segment of the myocardium, indicating impairment of fatty acid metabolism and sympathetic abnormalities.

3-Iodobenzylguanidine↗

PubMed2005

[Phenomena of "micronucleoli" in cardiomyocytes from patients with idiopathic restrictive cardiomyopathy].

Silver staining was used to estimate structural and functional conditions of cardiomyocyte and stromal cell nucleoli in myocardium from 3 patients with idiopathic restrictive cardiomyopathy (RCM). We revealed a prominent increase in the amount of myocardial stromal component cells. The area of nuclei, the area and amount of nucleoli in cells of myocardial stromal component much varied. In some cells of myocardial stromal component we found nuclei, whose area was comparable with that of cardiomyocyte nuclei. In all patients we revealed cardiomyocytes with extranucleolar distribution of argentophilic substance in the nuclei. These data suggest an important pathogenic role of structural rearrangments in the nuclei of cardiomyocytes and stromal component cells in providing specific cell phenotype of myocardium in patients with idiopathic RCM. Extranucleolar (atypical) distribution of argentophilic substance in the nucleus, referred to as "micronucleoli", point out certain disturbances of nucleolar functions in cardiomyocytes in patients with RCM.

Adult↗

PubMed2005

Assessment of ventricular coupling with real-time cine MRI and its value to differentiate constrictive pericarditis from restrictive cardiomyopathy.

The purpose of this study was to evaluate the use of respiratory-related ventricular coupling to differentiate patients with constrictive pericarditis (CP) and restrictive cardiomyopathy (RCM). In 18 histologically proven cases of CP, 6 patients with inflammatory pericarditis (IP), 15 RCM patients and 17 normal subjects, real-time cine MRI was performed in the cardiac short-axis (basal half of the ventricles) during operator-guided deep respiration. The images were analyzed for ventricular septal position and shape during early ventricular filling. Early diastolic septal inversion (I) or flattening (F) was found in all CP (I:15,F:3), and in all IP (I:2,F:4), but seldom in normals (F:1) and not in RCM. The septal abnormalities occurred at the onset of inspiration and rapidly disappeared with the next heartbeats. The amount of ventricular coupling was evaluated by quantifying the difference in the maximal septal excursion between inspiration and expiration. This parameter, normalized to the biventricular diameter, was significantly larger in CP (20.0+/-4.5%, P<0.0001) and IP (14.8+/-3.2%, P<0.0001) patients than in normals (7.0+/-2.4%), whereas RCM patients had a trend toward decreased excursion (4.2+/-1.7%, P=0.11). A cut-off value of 11.8% (mean normals +2 SD) enabled to differentiate CP patients from normals and RCM patients completely. Real-time cine MRI can easily depict increased ventricular coupling, which may be helpful to better differentiate between CP and RCM patients, especially in patients with normal or minimally thickened pericardium. The increase in coupling in IP patients is likely caused by decreased compliance of the inflamed pericardial layers.

Aged↗

PubMedDate not supplied

Is it constrictive pericarditis or restrictive cardiomyopathy? A systematic approach.

In this case review, the authors propose a fluent diagnostic algorithm for the consideration and therapeutic approach to either constrictive pericarditis or restrictive cardiomyopathy. Additionally, while focusing on the differential diagnosis of these clinically vexing entities, the authors outline the therapeutic expectations from surgical pericardiectomy in constrictive pericarditis.

Adult↗

PubMedDate not supplied

[Diagnosis of constrictive pericarditis and restrictive cardiomyopathy by pulsed Doppler echocardiography].

Characteristics of flow through atrioventricular valves were analyzed by pulsed Doppler echocardiography (PDE) in 7 patients with either constrictive pericarditis or restrictive cardiomyopathy and 10 controls to determine the value of this technique in their differentiation. All patients were admitted with systemic venous congestion and underwent right and left heart catheterization. PDE variables considered included peak flow velocity (PV), acceleration time, peak velocity of the atrial component (PVA), PVA/PV quotient, duration of early diastolic fillings, deceleration of early diastolic filling, duration of diastolic flow and mean temporal velocity. Ventricular filling differed between patients and controls in that the former group was characterized by higher PV's, lower PVA's, higher deceleration and lower PVA/PV quotient. When we compared both patient groups we found a significant tendency toward higher PV's, faster deceleration and lower PVA/PV quotient in constrictive pericarditis.

Adult↗

PubMed2003

Idiopathic restrictive cardiomyopathy is part of the clinical expression of cardiac troponin I mutations.

Restrictive cardiomyopathy (RCM) is an uncommon heart muscle disorder characterized by impaired filling of the ventricles with reduced volume in the presence of normal or near normal wall thickness and systolic function. The disease may be associated with systemic disease but is most often idiopathic. We recognized a large family in which individuals were affected by either idiopathic RCM or hypertrophic cardiomyopathy (HCM). Linkage analysis to selected sarcomeric contractile protein genes identified cardiac troponin I (TNNI3) as the likely disease gene. Subsequent mutation analysis revealed a novel missense mutation, which cosegregated with the disease in the family (lod score: 4.8). To determine if idiopathic RCM is part of the clinical expression of TNNI3 mutations, genetic investigations of the gene were performed in an additional nine unrelated RCM patients with restrictive filling patterns, bi-atrial dilatation, normal systolic function, and normal wall thickness. TNNI3 mutations were identified in six of these nine RCM patients. Two of the mutations identified in young individuals were de novo mutations. All mutations appeared in conserved and functionally important domains of the gene. This article was published online in advance of the print edition. The date of publication is available from the JCI website, http://www.jci.org.

Adolescent↗