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PubMed1990

The differentiation of restrictive cardiomyopathy from constrictive pericarditis.

The differentiation of restrictive cardiomyopathy from pericardial constriction remains a difficult clinical problem. Although the historical, noninvasive, and hemodynamic and angiographic features discussed here provide poor discriminating value when considered individually, a combination of clues may suggest one diagnosis or the other. Endomyocardial biopsy affords the greatest hope of avoiding unnecessary surgical exploration. Thoracotomy continues, however, to be the gold standard by which to make the distinction, carrying with it significant risk in patients with underlying restrictive cardiomyopathy.

Biopsy↗
PubMed1997

Phasic coronary flow characteristics in patients with constrictive pericarditis: comparison with restrictive cardiomyopathy.

BACKGROUND: Phasic coronary flow characteristics have been reported in patients with aortic valve disease and hypertrophic cardiomyopathy. The purpose of this study was to assess the differences in coronary flow characteristics between patients with constrictive pericarditis and those with restrictive cardiomyopathy. METHODS AND RESULTS: The study populations consisted of 7 case patients with constrictive pericarditis, 8 with restrictive cardiomyopathy, and 11 control subjects with chest pain and normal coronary arteries. Five minutes after injection of 3 mg of isosorbide dinitrate, phasic coronary flow velocity patterns were analyzed in the proximal segment of the angiographically normal left anterior descending coronary artery at rest using a 0.014-in, 15-MHz Doppler guidewire. Coronary flow reserve was obtained from the ratio of adenosine-induced (0.14 mg x kg(-1) x min(-1) I.V.) hyperemic/baseline time-averaged peak velocity. Although in case patients with constrictive pericarditis and restrictive cardiomyopathy maximal hyperemic time-averaged peak velocity (21+/-8 and 31+/-17 versus 60+/-19 cm/s, respectively; P<.001) and coronary flow reserve (1.3+/-0.4 and 1.6+/-0.6 versus 3.6+/-0.4, respectively, P<.001) were significantly lower than in control subjects, there were no significant differences in these indexes between the two groups of case patients. Velocity half-time of diastolic flow velocity corrected by square root(RR), which indicates deceleration of diastolic flow, in the groups of case patients with constrictive pericarditis and restrictive cardiomyopathy was significantly less than that in control subjects (6.2+/-2.6 and 10.6+/-1.5 versus 16.9+/-2.7, respectively; P<.001); this was also significantly smaller in constrictive pericarditis than restrictive cardiomyopathy (P<.001). This index <9.5 could distinguish constrictive pericarditis from restrictive cardiomyopathy with a sensitivity of 86% and a specificity of 88%. Furthermore, time from the beginning of diastole to diastolic peak velocity corrected by square root(RR) indicating acceleration of diastolic flow velocity in constrictive pericarditis was significantly less than that in restrictive cardiomyopathy and control subjects (2.8+/-1.2 versus 4.8+/-0.8 and 4.4+/-0.6, respectively; P<.001). CONCLUSIONS: Although coronary flow reserve is limited in both constrictive pericarditis and restrictive cardiomyopathy because of restriction of hyperemic response, rapid acceleration and more rapid deceleration of diastolic flow velocity are more characteristic in constrictive pericarditis than in restrictive cardiomyopathy.

Adult↗
PubMed1989

[Idiopathic restrictive cardiomyopathy: clinical, hemodynamic, histologic and prognostic profile].

Idiopathic restrictive cardiomyopathy is a rare myocardial disease characterized by restrictive physiology without a specific histologic basis. To assess its clinical, hemodynamic, morphologic and prognostic details we retrospectively evaluated all the patients hospitalized in our Institute from 1974 to 1988. Nine patients, aged 42 +/- 16 years, M/F ratio = 0.29, who represent 64% of all the restrictive myocardial diseases biopsied were identified. Severe cardiac heart failure (3-4 NYHA) and arrhythmias (ventricular and supraventricular) were extremely common. The electrocardiogram showed several non specific signs: low voltage of QRS in peripheral leads (4/7), pseudo-infarctional aspects (3/7), mono or biventricular hypertrophy (3/7) disturbance of ventricular conduction (3/7), aspecific abnormalities of ventricular repolarization (3/7). All patients showed a prolonged QTc. M-mode and 2-dimensional echocardiography demonstrated in 6 cases biatrial enlargement, normal or slightly enlarged ventricles, normal or moderately depressed fractional shortening; biventricular concentric hypertrophy was detected in 3 cases, asymmetrical septal hypertrophy in 1. Five patients showed pericardial effusion. Cardiac catheterization disclosed an increase of left and right ventricular end-diastolic pressures (8/8) with a dip-plateau pattern and/or characteristic W waveform in the atrial pressure tracing (9/9). Passive pulmonary hypertension was detected in 6/9 cases. The cardiac index was decreased in 4/8 cases. Left ventricular angiography showed mitral regurgitation in 5/8 patients, tricuspidal in 5/8. Ejection fraction was decreased in 3/8 cases. Endomyocardial biopsy showed interstitial fibrosis (8/9), cellular hypertrophy and/or nuclear alterations (7/9), slight endocardial thickening (2/9). At a mean follow-up of 22 +/- 15 months 3 patients died and 2 underwent heart transplantation. In conclusion idiopathic restrictive cardiomyopathy is one of the most frequent forms of restrictive myocardial diseases in our geographic area. Severe congestive heart failure and arrhythmias are extremely common. The disease can be suspected by clinical, electrocardiographic and echocardiographic features, but the final diagnosis requires cardiac catheterization and endomyocardial biopsy. Prognosis is severe and heart transplantation must be considered in the cases with severe heart failure.

Adolescent↗
PubMed1976

Usefulness of systolic time intervals in differential diagnosis of constrictive pericarditis and restrictive cardiomyopathy.

Systolic time intervals in 15 patients with constrictive pericarditis and seven patients with restrictive cardiomyopathy were compared in order to assess their value in the differential diagnosis of the two disorders. Clinical examination had failed to make the distinction. Right heart catheterization was helpful in diagnosing restriction but failed to differentiate patients with constrictive pericarditis from those with restrictive cardiomyopathy. The systolic time intervals clearly separated the two groups. The PEP/LVET was normal in all patients with constrictive pericarditis (0.34 +/- 0.01) and abnormal in all patients with restrictive cardiomyopathy (0.70 +/- 0.09, P less than 0.001). In 13 patients (five with restrictive cardiomyopathy and eight with constrictive pericarditis) the results of quantitative left ventricular angiocardiography were available. A high correlation (r=-0.90, P less than 0.01) between the PEP/LVET and the ejection fraction confirmed the validity of the PEP/LVET as a measure of left ventricular performance in these patients. Thus the systolic time intervals clearly distinguished between constrictive pericarditis and restrictive cardiomyopathy and are a reliable non-invasive technique for making the difficult differential diagnosis.

Adult↗
PubMed1985

Ventricularization of right atrial wave form in amyloid restrictive cardiomyopathy.

Two patients with biopsy-proven amyloid restrictive cardiomyopathy were presented. Both cases showed ventricularization of an elevated right atrial pressure wave form in absence of tricuspid regurgitation. Possible explanations for this finding as well as its clinical implications are discussed. This observation indicates that ventricularization of right atrial pressure wave form could be a useful hemodynamic sign in amyloid restrictive cardiomyopathy in absence of tricuspid regurgitation. Furthermore, such a finding does not seem to be specific for tricuspid regurgitation.

Aged↗
PubMed1989

Differentiation of restrictive cardiomyopathy from pericardial constriction: assessment of diastolic function by radionuclide angiography.

Diastolic filling variables were studied in 12 patients with the hemodynamic features of constriction, of whom 5 had restrictive cardiomyopathy, 5 had pericardial constriction and 2 had combined pericardial constriction and restrictive cardiomyopathy. The values were compared with those in 10 normal subjects of comparable age. The filling fractions between 10% and 70% of the diastolic time interval were greater in patients with pericardial constriction than in those with restrictive cardiomyopathy (p less than 0.01 between 20% and 50%, p less than 0.05 at 10%, 60% and 70%), with no overlap. The filling fractions in patients with pericardial constriction were also greater than those in normal subjects between 10% and 60% of the diastolic time interval. The filling fraction was lower in patients with restrictive cardiomyopathy than in normal subjects at 40% of the diastolic time interval (p less than 0.05). The time to peak filling rate in patients with pericardial constriction was shorter (110 +/- 14 ms) than in those with restrictive cardiomyopathy (195 +/- 45 ms, p less than 0.01) or in normal subjects (173 +/- 32 ms, p less than 0.01). The percent of atrial contribution to left ventricular filling was higher in those with restrictive cardiomyopathy (45 +/- 17%) than in those with pericardial constriction (21 +/- 6%, p less than 0.05) or in normal subjects (24 +/- 9%, p less than 0.01).(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1989

Restrictive cardiomyopathy and constrictive pericarditis: non-invasive distinction by digitised M mode echocardiography.

It is difficult to distinguish between restrictive cardiomyopathy and constrictive pericarditis on the basis of clinical findings and simple investigation. Cardiac catheterisation has been the reference standard for diagnosis but even this does not always permit an accurate distinction. A Summagraphics digitiser and Prime 750 computer system were used to digitise the echocardiograms of 15 patients with restrictive cardiomyopathy, 10 with constrictive pericarditis and a group of 20 age and sex matched normal subjects of similar age and sex distribution. Compared with controls, patients with restrictive cardiomyopathy showed a significant reduction in the following variables (a) decreased fractional shortening, (b) decreased peak left ventricular filling and emptying rates, (c) decreased percentage posterior wall thickening, and (d) decreased peak left ventricular posterior wall thickening and thinning rates. Whereas patients with constrictive pericarditis only had significantly reduced peak left ventricular filling and posterior wall thinning rates and significantly increased posterior wall thinning rate. When patients with restrictive cardiomyopathy were compared with those with constrictive pericarditis the significant differences were: (a) decreased peak left ventricular emptying rate, (b) decreased percentage posterior wall thickening, and (c) decreased peak left ventricular posterior wall thickening and thinning rates. Digitisation of M mode echocardiograms, with particular attention to posterior wall function, may be a useful adjunct to cardiac catheterisation in distinguishing restrictive cardiomyopathy from constrictive pericarditis.

Adult↗
PubMed2005

Idiopathic restrictive cardiomyopathy in children.

OBJECTIVE: To define the natural history of idiopathic restrictive cardiomyopathy in a paediatric population and to identify any factors predictive of outcome. DESIGN: Retrospective analysis of patients born between 1970 and 2002 were identified from the Children's Hospital of Pittsburgh cardiology database. Demographic data, mode of presentation, echocardiographic and haemodynamic findings at diagnosis, survival time, and manner of death were evaluated. SETTING: Tertiary referral and transplant centre for paediatric patients with cardiac disease. PATIENTS: All local and referred patients with idiopathic restrictive cardiomyopathy born after 1970 and under 21 years of age at time of diagnosis. RESULTS: 21 patients were identified. Probability of survival at 1, 5, and 10 years was 80.5% (95% confidence interval (CI) 58 to 100), 39% (95% CI 17 to 61), and 20% (95% CI 0 to 42), respectively. Median age of presentation was 3.8 years (mean (SD) 5.7 (6.1) years). Median survival without transplantation was 2.2 years (mean (SD) 4.6 (5.4) years). Age at presentation, sex, and presence or absence of heart failure symptoms at presentation were not associated with clinical course. Right (p = 0.05) and left ventricular end diastolic pressures (p = 0.04) and ratio of left atrial to aortic root dimensions (LA:Ao) (p = 0.03) at presentation had a significantly negative correlation with survival time after diagnosis. CONCLUSIONS: Without transplantation, most children with restrictive cardiomyopathy have a very poor prognosis. Longer survival from diagnosis was correlated with lower LA:Ao and cardiac filling pressures at diagnosis. Survival time was not influenced by the symptoms present at diagnosis.

Adolescent↗
PubMed2002

Cardiac transplantation for pediatric restrictive cardiomyopathy: presentation, evaluation, and short-term outcome.

BACKGROUND: Because of the poor prognosis of pediatric restrictive cardiomyopathy, transplantation has been proposed as the treatment of choice for this disease. METHODS: We reviewed our experience with the presentation, evaluation, and short-term outcome in 8 pediatric patients with restrictive cardiomyopathy referred for transplantation. Potential reversibility of elevation in pulmonary vascular resistance was tested before transplantation with nitroprusside and nitric oxide, with follow-up cardiac catheterization performed 6 to 12 months after transplantation. RESULTS: The mean age of diagnosis of restrictive cardiomyopathy was 6.3 years and the mean interval from diagnosis to referral for transplantation was 3.6 years. Elevation of pulmonary vascular resistance was common and tended to progress with longer follow-up. Three of the 8 patients had pulmonary vascular resistance indices greater than 10 Woods unit/m(2) and transpulmonary gradients greater than 20 mm Hg. The administration of nitroprusside and nitric oxide reversed elevated pulmonary resistance and transpulmonary gradients in all patients. Nitric oxide successfully reversed pulmonary vascular resistance in patients unresponsive to nitroprusside. All patients underwent successful transplantation and follow-up catheterization revealed normal pulmonary hemodynamics in each patient. CONCLUSIONS: Pediatric restrictive cardiomyopathy can be associated with marked elevation in the pulmonary vascular resistance, which may contribute to the poor prognosis in these patients and potentially make cardiac transplantation problematic. Orthotopic cardiac transplantation can be successfully performed in patients who demonstrate reversibility of pulmonary vascular resistance. Nitric oxide appears to be the best agent to demonstrate reversibility of pulmonary resistance in these patients.

Cardiomyopathy, Restrictive↗
PubMed2002

Outcome of idiopathic restrictive cardiomyopathy in children.

Eighteen children with idiopathic restrictive cardiomyopathy (IRC) were studied in an attempt to identify potential predictors of poor outcome. Four patients presented with low cardiac output (CO) syndrome. Fourteen remaining patients were minimally symptomatic at presentation but developed a low CO syndrome at a mean of 2.8 +/- 2.3 years after diagnosis. At the time of development of low CO in the 18 patients, mean left ventricular end-diastolic pressure was 27 mm Hg, right ventricular end-diastolic pressure was 18 mm Hg, cardiac index was 2.5 L/min/m(2), and pulmonary vascular resistance index (PVRI) was 8.8 U-m(2). Eleven of the 18 patients underwent cardiac transplantation. One died perioperatively from donor right-sided cardiac failure and 10 survived. Six were not transplanted and died, including 3 in whom transplantation was precluded secondary to extremely elevated PVRI. One patient is alive with right-sided cardiac failure. Ten of our 18 patients had pulmonary hypertension (PVRI >6 U-m(2)) at the time of referral for cardiac transplant and/or development of low CO syndrome. In comparison, children with dilated cardiomyopathy who were referred for heart transplant during the same time period had a PVRI that was significantly lower (5.2 U-m(2)). Elevated PVRI was associated with death (p <0.01) and 40% of our children with pulmonary hypertension were precluded from receiving an orthotopic heart transplant because their pulmonary hypertension was so severe. No risk factors for the development of pulmonary hypertension were identified; therefore, all children with IRC should undergo serial monitoring of their PVRI, and any increase should prompt a transplant evaluation.

Adolescent↗
PubMed1991

Progressive restrictive cardiomyopathy--a case report.

We present a case of restrictive cardiomyopathy which progressed over a 10 month period. A 69-year-old female was admitted because of acute inferior myocardial infarction; hemodynamically, she was in Forrester subset I. Cardiac catheterization performed 4 weeks post-infarction showed markedly increased left ventricular end-diastolic and pulmonary wedge pressures. Left ventricular ejection fraction was 66% with postero-basal akinesis and minimal mitral regurgitation. The right coronary artery was completely occluded with good collateral circulation from the intact left coronary artery. Doppler echocardiography 4 weeks post-infarction showed pseudo-normalization of the A/E ratio of peak mitral flow velocity in atrial systole (A) to peak mitral flow velocity in early diastole (E). Preload reduction by nitroglycerin increased the A wave. Ten months post-infarction, the patient was re-admitted due to congestive heart failure. The A/E ratio was unchanged, however the A wave no longer increased after the same dose of nitroglycerin. We have hypothesized that dehydration and/or the vigorous use of a nitrate, in the acute phase of myocardial infarction, masked an underlying restrictive cardiomyopathy which progressed in the 10 months post-infarction.

Blood Flow Velocity↗
PubMed1989

Differentiation of constrictive pericarditis and restrictive cardiomyopathy by Doppler echocardiography.

Doppler ultrasound recordings of mitral, tricuspid, aortic, and pulmonary flow velocities, and their variation with respiration, were recorded in 12 patients with a restrictive cardiomyopathy and seven patients with constrictive pericarditis. Twenty healthy adults served as controls. The patients with constrictive pericarditis showed marked changes in left ventricular isovolumic relaxation time and in early mitral and tricuspid flow velocities at the onset of inspiration and expiration. These changes disappeared after pericardiectomy and were not seen in patients with restrictive cardiomyopathy or in normal subjects. The deceleration time of early mitral and tricuspid flow velocity was shorter than normal in both groups, indicating an early cessation of ventricular filling, but only patients with restrictive cardiomyopathy showed a further shortening of the tricuspid deceleration time with inspiration. Diastolic mitral and tricuspid regurgitation was also more common in the patients with restrictive cardiomyopathy. These results suggest that patients with constrictive pericarditis and restrictive cardiomyopathy can be differentiated by comparing respiratory changes in transvalvular flow velocities. In addition, although baseline hemodynamics in the two groups were similar, characteristic changes were seen with respiration that suggest differentiation of these disease states may also be possible from hemodynamic data.

Adult↗
PubMed1983

Differentiation of constrictive pericarditis and restrictive cardiomyopathy using digitized echocardiography.

Constrictive pericarditis and restrictive cardiomyopathy are difficult to distinguish at the bedside and occasionally at routine cardiac catheterization. Left ventricular diastolic function was studied by computer analysis of digitized M-mode echocardiograms in four patients with constrictive disease and three with restrictive disease, and the data were compared with those of normal subjects. The respective distinguishing echographic features of constrictive pericarditis and restrictive cardiomyopathy were as follows: the major filling period of the left ventricle was 78 +/- 9% of normal versus 128 +/- 4% (p less than 0.01), minimal left ventricular dimension to peak filling interval was 50 +/- 10 versus 110 ms (p less than 0.05) and the maximal rate of left ventricular posterior wall thinning was -4.9 versus -2.3 seconds-1 (p less than 0.05). This preliminary study suggests that it may be possible to accurately diagnose the two disease entities using this technique at the bedside and to avoid cardiac catheterization.

Adolescent↗
PubMed2005

Primary cardiac lymphoma presenting clinically as restrictive cardiomyopathy.

An unusual case of primary cardiac lymphoma presenting as restrictive cardiomyopathy with arrhythmia is reported in a 72-year-old woman who was admitted for evaluation of exertional dyspnea and palpitations. Electrocardiography (ECG) showed atrioventricular dissociation and right heart cardiac catheterization revealed a typical 'dip-and-plateau' waveform. Restrictive cardiomyopathy was suspected because computed tomography (CT) did not reveal pericardial thickening, calcifications, or an effusion. Heart failure initially improved with diuretic therapy, but subsequently worsened, and the patient experienced a syncopal episode. ECG showed atrial fibrillation, and CT revealed a large mass in the right atrium and multiple tumors in the liver, which needle biopsy confirmed as diffuse large B-cell lymphoma. Chemotherapy induced complete remission, and her heart failure markedly improved. The 'dip-and-plateau' waveform was no longer detected on repeat cardiac catheterization and the ECG showed restoration of sinus rhythm. Clinically, the diagnosis was primary cardiac lymphoma.

Aged↗
PubMed1998

Peliosis hepatis associated with idiopathic restrictive cardiomyopathy.

A rare case of peliosis hepatis associated with idiopathic restrictive cardiomyopathy is reported. A 75-year-old man was admitted for evaluation of marked edema and jaundice. Serum total bilirubin was elevated above 20 mg/dl. The liver biopsy under laparoscopy revealed marked sinusoidal dilatation and retention of red blood cells, which was consistent with a diagnosis of peliosis hepatis. Cardiac catheterization revealed right ventricular filling disturbance without specific findings on endomyocardial biopsy, suggesting idiopathic restrictive cardiomyopathy. The level of serum total bilirubin decreased in association with improvement of edema after drip infusion of furosemide therapy.

Aged↗
PubMed1992

Clinical profile and outcome of restrictive cardiomyopathy in children.

The clinical profile and outcome of idiopathic restrictive cardiomyopathy in a group of eight children are reviewed. There were six girls and two boys. Age at presentation was 4.0 +/- 2.6 years (range 1.3 to 9.5 years). All patients had evidence of congestive heart failure with systemic venous congestion. Right or left atrial enlargement was the most consistent ECG finding and was present in all patients. Left ventricular shortening fraction was normal in five patients, increased in two, and mildly reduced in one. The most striking echocardiographic feature was severe biatrial dilatation in the presence of normal or near-normal ventricular cavity dimensions. Marked elevation of left ventricular end-diastolic pressure was noted in all seven patients undergoing cardiac catheterization (34 +/- 7 torr; range 24 to 40 torr). Right ventricular end-diastolic pressure was elevated but significantly different from left ventricular pressure (18 +/- 7 torr; p less than 0.01). A characteristic early diastolic dip with a rapid rise to an elevated plateau (square root sign) was present in five of seven patients. Median survival was 1.4 years. Six patients died 0.2 to 7.0 years after they were initially seen. The actuarial survival rate 1.5 years after presentation was 44%, decreasing to 29% at 4 years. Restrictive cardiomyopathy has a worse prognosis in children than in adults. In part this may reflect the more advanced symptoms of congestive failure at initial presentation. Pediatric patients should be considered for early cardiac transplantation.

Cardiac Catheterization↗
PubMed2003

A fatal case of idiopathic restrictive cardiomyopathy.

We describe the clinical features of idiopathic restrictive cardiomyopathy in a female infant. A marked elevation of left ventricular end-diastolic pressure, and profoundly abnormal myocardial relaxation, were detected with the use of Doppler blood flow echocardiography, coupled with the relatively new technique of Doppler tissue echocardiography. There was no clinical evidence of ongoing heart failure, but she had signs of myocardial ischaemia, and unfortunately died suddenly at the age of 13 months.

Cardiomyopathy, Restrictive↗