Cardiomyopathy Restrictive Publications (1805)


Cardiomyopathy Restrictive Publications

Hellenic J Cardiol
Hellenic J Cardiol 2016 Nov 16. Epub 2016 Nov 16.
Department of Radiology, Nuclear Medicine and Medical Physics, Vilnius University, Lithuania.

The natural history, management, and outcome of Takotsubo (stress) cardiomyopathy (TTC) is not clear. The aim of this study was to investigate clinical features, define prognostic predictors, and assess the clinical course and outcomes of patients with TTC.
We analyzed 64 patients (52 women) meeting the proposed Mayo Clinic diagnostic criteria for TTC. Read More

All patients were treated at Vilnius University Hospital Santariskiu Klinikos from 2001-01-01 to 2014-11-27. Data were collected on the basis of medical records and follow-up data was collected by phone.
The mean age of analyzed patients was 63.4 ± 14.6 years; the mean follow-up was 2.9 years. More than half of the patients (52%) did not have any clear stressful triggers. During admission, symptoms such as chest pain (64%) and general weakness (45%) were reported more often than other symptoms. Almost all patients (94%) had the classical TTC form; the remaining 6% of patients had "inverted" TTC. The mean left ventricular ejection fraction (LVEF) on admission was 37.7% (± 8.2%). A pseudonormal or restrictive pattern of LV filling, moderate to severe mitral regurgitation (MR), and right ventricular involvement were uncommon in the patients. The in-hospital course showed cardiogenic shock in 23% of the cases, resulting in the death of 5 (8%) patients. We discovered that only peak concentration of troponin I was a significant predictor of in-hospital mortality (HR 1.067, 95%CI 1.022-1.113, p=0.003). At the end of the follow-up period, 45 (87%) women and 8 (67%) men were alive. This makes the overall observed mortality at 3 years approximately 17.2%. Using multivariate analysis, elevation of BNP (HR for increase by 10 ng/l 1.002, 95%CI 1-1.003, p=0.022) and cardiogenic shock on admission (HR 8.696, 95%CI 1.198-63.124, p=0.032) were significant predictors of overall mortality. Other prognostic factors assessed on admission were nonsignificant predictors of overall mortality.
Our analysis shows that in-hospital mortality is influenced by the peak concentration of troponin I, and overall mortality is affected by cardiogenic shock and the elevation of BNP during admission. The assessment of troponin I and BNP can help with the prognostication of TTC patients in our daily clinical practice.

Echocardiography is an established and noninvasive diagnostic tool used in herpetologic cardiology. Various cardiac lesions have been previously described in reptiles with the exception of restrictive cardiomyopathy. In this case report, restrictive cardiomyopathy and congestive heart failure associated with left atrial and sinus venosus dilation were diagnosed in a 2-yr-old captive lethargic McDowell's carpet python ( Morelia spilota mcdowelli), based on echocardiographic, Doppler, and histopathologic examinations. Read More

This cardiomyopathy was also associated with thrombosis within the sinus venosus.

Postgrad Med J
Postgrad Med J 2017 Jan 10. Epub 2017 Jan 10.
Department of Cardiology, GB Pant Institute of Post Graduate Medical Education and Research, New Delhi, India.
J Cardiovasc Med (Hagerstown)
J Cardiovasc Med (Hagerstown) 2017 Jan 7. Epub 2017 Jan 7.
a'De Gasperis' Cardio Center bRadiology Department Niguarda Hospital, Milan, Italy.

Loeffler endomyocardial fibrosis is a rare restrictive cardiomyopathy due to chronic eosinophilic exposure (Loeffler endomyocarditis). Loeffler endomyocarditis is associated with different eosinophilic disorders, including eosinophilic granulomatosis with polyangiitis (EGPA). These images recapitulate all typical findings that can be observed in patients presenting with heart failure attributable to this specific cardiomyopathy associated with EGPA, a systemic medical condition that can be easily detectable just putting together different diagnostic elements. Read More

The diagnostic findings supporting a Loeffler endomyocarditis (evolved to endomyocardial fibrosis) associated with EGPA were systemic eosinophilia, organ damage (endomyocardial fibrosis) and history of asthma, nasal polyposis and pulmonary infiltrates.

Front Physiol
Front Physiol 2016 19;7:629. Epub 2016 Dec 19.
Department of Biomedical Science, Charles E. Schmidt College of Medicine, Florida Atlantic University Boca Raton, FL, USA.

Cardiac troponin I (cTnI) plays a critical role in regulation of cardiac function. Studies have shown that the deficiency of cTnI or mutations in cTnI (particularly in the C-terminus of cTnI) results in diastolic dysfunction (impaired relaxation) due to an increased myofibril sensitivity to calcium. The first clinical study revealing the association between restrictive cardiomyopathy (RCM) with cardiac troponin mutations was reported in 2003. Read More

In order to illustrate the mechanisms underlying the cTnI mutation caused cardiomyopathy, we have generated a cTnI gene knockout mouse model and transgenic mouse lines with the reported point mutations in cTnI C-terminus. In this paper, we summarize our studies using these animal models from our laboratory and the other in vitro studies using reconstituted filament and cultured cells. The potential mechanisms underlying diastolic dysfunction and heart failure caused by these cTnI C-terminal mutations are discussed as well. Furthermore, calcium desensitizing in correction of impaired relaxation in myocardial cells due to cTnI mutations is discussed. Finally, we describe a model of translational study, i.e., from bedside to bench and from bench to bedside. These studies may enrich our understanding of the mechanism underlying inherited cardiomyopathies and provide the clues to search for target-oriented medication aiming at the treatment of diastolic dysfunction and heart failure.

J Clin Diagn Res
J Clin Diagn Res 2016 Nov 1;10(11):OD05-OD06. Epub 2016 Nov 1.
Consulting Cardiologist, Department of Cardiology, Prince Aly Khan Hospital , Nesbit Road, Mazgaon, Mumbai, Maharashtra, India .
Chin. Med. Sci. J.
Chin Med Sci J 2016 Mar;31(1):1-7
Department of Cardiology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China.
Heart 2016 Dec 23. Epub 2016 Dec 23.
Pediatric Heart Center, Justus Liebig University Giessen, Virginia Commonwealth University, School of Pharmacy, Richmond, Virginia, USA.

The final therapy of 'end-stage heart failure' is orthotopic heart, lung or heart-lung transplantation. However, these options are not available for many patients worldwide. Therefore, novel therapeutical strategies are needed. Read More

Based on pathophysiological insights regarding (1) the long-term impact of an obstructive pulmonary outflow tract in neonates with congenitally corrected transposition of the great arteries, (2) the importance of a restrictive versus a non-restrictive atrial septum in neonates born with a borderline left ventricle and (3) the significance of both, a patent foramen ovale and/or open ductus arteriosus for survival of newborns with persistent pulmonary hypertension, the current review introduces some therapeutical strategies that may be applicable to selected patients with heart failure. These strategies include (1) reversible pulmonary artery banding in left ventricular-dilated cardiomyopathy with preserved right ventricular function, (2) the creation of restrictive interatrial communication to treat diastolic (systolic) heart failure, (3) atrioseptostomy or reverse Potts shunt in pulmonary arterial hypertension and (4) return to a fetal, parallel circulation by combining atrioseptostomy and reversed Potts shunt with or without placement of a bilateral pulmonary artery banding. While still being experimental, it is hoped that the procedures presented in the current overview will inspire future novel therapeutic strategies that may be applicable to selected patients with heart failure.

J. Am. Coll. Cardiol.
J Am Coll Cardiol 2016 Dec;68(25):2871-2886
Department of Genetics, Harvard Medical School, Boston, Massachusetts; Division of Cardiovascular Medicine, Brigham and Women's Hospital, Boston, Massachusetts; Howard Hughes Medical Institute, Chevy Chase, Maryland. Electronic address:

Over the last quarter-century, there has been tremendous progress in genetics research that has defined molecular causes for cardiomyopathies. More than a thousand mutations have been identified in many genes with varying ontologies, therein indicating the diverse molecules and pathways that cause hypertrophic, dilated, restrictive, and arrhythmogenic cardiomyopathies. Translation of this research to the clinic via genetic testing can precisely group affected patients according to molecular etiology, and identify individuals without evidence of disease who are at high risk for developing cardiomyopathy. Read More

These advances provide insights into the earliest manifestations of cardiomyopathy and help to define the molecular pathophysiological basis for cardiac remodeling. Although these efforts remain incomplete, new genomic technologies and analytic strategies provide unparalleled opportunities to fully explore the genetic architecture of cardiomyopathies. Such data hold the promise that mutation-specific pathophysiology will uncover novel therapeutic targets, and herald the beginning of precision therapy for cardiomyopathy patients.


Objective: Despite the improvement in ventricular assist device (VAD) therapy in adults and in adolescents, in infant population only Berlin Heart EXCOR (BHE) is licensed as long term VAD to bridge children to Heart Transplantation (HTx). Particularly demanding in terms of morbidity and mortality are smallest patients namely the ones implanted in the first year of life or with a lower body surface area. This work aims at retrospective reviewing a single center experience in using BHE in children with a body weight under 10 kg. Read More

Methods: Data of all pediatric patients under 10 kg undergoing BHE implantation in our institution from March 2002 to March 2016 were retrospectively reviewed. Results: Of the 30 patients enrolled in the study, 53% were male, 87% were affected by a dilated cardiomyopathy with an average weight and age at the implantation of 6.75 ± 2.16 Kg and 11.57 ± 10.12 months, respectively. Three patients (10%) required a BIVAD implantation. After the implantation, 7 patients (23%) required re-intervention for bleeding and 9 patients (30%) experienced BHE cannulas infection. A total of 56 BHE pump were changed for thrombus formation (1.86 BHE pump for patient). The average duration of VAD support was 132.8 ± 94.4 days. Twenty patients (67%) were successfully transplanted and 10 patients (33%) died: 7 for major neurological complication and 3 for sepsis. Conclusion: Mechanical support in smaller children with end stage heart failure is an effective strategy for bridging patients to HTx. The need for BIVAD was relegated, in the last years, only to restrictive cardiomiopathy. Further efforts are required in small infants to improve anticoagulation strategy to reduce neurological events and BHE pump changes.