Idiopathic restrictive cardiomyopathy: Differences of left ventricular relaxation and diastolic wave forms from constrictive pericarditis

Hirota, Yuzo; Kohriyama, Toshiaki; Hayashi, Toshio; Kaku, Kiyotaka; Nishimura, Hikaru; Saito, Takaharu; Nakayama, Yasushi; Suwa, Michihiro; Kino, Masaya; Kawamura, Keishiro

doi:10.1016/0002-9149(83)90156-x

Cited by 30 publications

(5 citation statements)

References 7 publications

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“…This ‘dip and plateau’ sign reflects the rapid filling during early diastole that is followed by a decrease in ventricular filling from the end of the first third of diastole onward [70,71]. Right ventricular end-diastolic pressure and LVEDP are elevated in both ventricles; however, the LVEDP may be 5–7 mm/Hg higher than the right ventricular end-diastolic pressure.…”

Section: Rcm In Childhoodmentioning

confidence: 99%

Pediatric Cardiomyopathies: causes, epidemiology, Clinical course, Preventive Strategies and Therapies

et al. 2013

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Pediatric cardiomyopathies, which are rare but serious disorders of the muscles of the heart, affect at least one in every 100,000 children in the USA. Approximately 40% of children with symptomatic cardiomyopathy undergo heart transplantation or die from cardiac complications within 2 years. However, a significant number of children suffering from cardiomyopathy are surviving into adulthood, making it an important chronic illness for both pediatric and adult clinicians to understand. The natural history, risk factors, prevalence and incidence of this pediatric condition were not fully understood before the 1990s. Questions regarding optimal diagnostic, prognostic and treatment methods remain. Children require long-term follow-up into adulthood in order to identify the factors associated with best clinical practice including diagnostic approaches, as well as optimal treatment approaches. In this article, we comprehensively review current research on various presentations of this disease, along with current knowledge about their causes, treatments and clinical outcomes.

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Section: Rcm In Childhoodmentioning

confidence: 99%

Pediatric Cardiomyopathies: causes, epidemiology, Clinical course, Preventive Strategies and Therapies

et al. 2013

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“…These hemodynamic findings are identical to those of constrictive pericarditis. 5,8,25,26 Typically, however, in RCM, unlike constrictive pericarditis, the right ventricular end-diastolic pressure is 5 mm Hg lower than that of the left ventricle because of unequal involvement and compliance of the 2 chambers. Intervention such as exercise, volume infusion, and cardioactive drugs to separate left from right ventricular pressure can be of limited value, because negative results cannot entirely exclude RCM or confirm constrictive pericarditis.…”

Section: Diagnostic Approachmentioning

confidence: 99%

Clinical Profile and Outcome of Idiopathic Restrictive Cardiomyopathy

et al. 2000

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Background-Idiopathic restrictive cardiomyopathy is a poorly recognized entity of unknown cause characterized by nondilated, nonhypertrophied ventricles with diastolic dysfunction resulting in dilated atria and variable systolic function. Methods and Results-Between 1979 and 1996, 94 patients (61% women) 10 to 90 years old (mean, 64 years) met strict morphological echocardiographic criteria for idiopathic restrictive cardiomyopathy, mainly dilated atria with nonhypertrophied, nondilated ventricles. None had known infiltrative disease, hypertension of Ͼ5 years' duration, or cardiac or systemic conditions associated with restrictive filling. Nineteen percent were in NYHA class I, 53% in class II, and 28% in class III or IV. Atrial fibrillation was noted in 74% of patients and systolic dysfunction in 16%. Follow-up (mean, 68 months) was complete for 93 patients (99%). At follow-up, 47 patients (50%) had died, 32 (68%) of cardiovascular causes. Four had heart transplantation. The death rate compared with actuarial statistics was significantly higher than expected (PϽ0.0001). Kaplan-Meier 5-year survival was 64%, compared with expected survival of 85%. Multivariate analysis using proportional hazards showed that the risk of death approximately doubles with male sex (hazard ratio [HR] ϭ2.1), left atrial dimension Ͼ60 mm (HRϭ2.3), age Ͼ70 years (HRϭ2.0), and each increment of NYHA class (HRϭ2.0). Conclusions-Idiopathic restrictive cardiomyopathy or nondilated, nonhypertrophic ventricles with marked biatrial dilatation, as defined morphologically by echocardiography, affects predominantly elderly patients but can occur in any age group. Patients present with systemic and pulmonary venous congestion and atrial fibrillation and have a poor prognosis, particularly men Ͼ70 years old with higher NYHA class and left atrial dimension Ͼ60 mm. (Circulation.

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“…Cardiac catheterization findings were also unsuccessful in truly differentiating between these etiologies. While the square root sign is typical of restrictive cardiomyopathy, four-chamber filling pressure equalization is considered a major diagnostic criterion of constrictive pericarditis [14]. Moreover, the addition of a positive Pemberton's sign typically eludes both of the previously mentioned cardiac pathologies.…”

Section: Discussionmentioning

confidence: 99%

Revising the Exclusivity of Pemberton’s Sign: A Case Report

Tipton¹,

Blanchard

Guider

et al. 2014

AJIM

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The Pemberton maneuver is a tool that may be utilized during the physical examination to demonstrate the presence of latent pressure in the thoracic inlet [1]. The maneuver is achieved by having the patient elevate both arms until they touch the sides of the face. A positive sign is marked by the presence of facial congestion and cyanosis, as well as respiratory distress after approximately one minute [2]. The sign is most commonly present in patients with substernal goiters where the goiter "corks off" the thoracic inlet [3]. We describe a 67-year-old Caucasian male with a positive Pemberton's sign due to compression of the thoracic inlet by enlarged mediastinal nodes. The sign was amplified by the presence of a restrictive cardiomyopathy and constrictive pericarditis due to amyloidosis. We propose that a positive Pemberton's sign should not be used exclusively to evaluate the probability of a substernal goiter, but rather to assess the potential for vascular compression due to pathological changes in the anatomical environment of the thoracic inlet.

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Idiopathic restrictive cardiomyopathy: Differences of left ventricular relaxation and diastolic wave forms from constrictive pericarditis

Cited by 30 publications

References 7 publications

Pediatric Cardiomyopathies: causes, epidemiology, Clinical course, Preventive Strategies and Therapies

Pediatric Cardiomyopathies: causes, epidemiology, Clinical course, Preventive Strategies and Therapies

Clinical Profile and Outcome of Idiopathic Restrictive Cardiomyopathy

Revising the Exclusivity of Pemberton’s Sign: A Case Report

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