Cardiomyopathy Restrictive Publications (1805)
Cardiomyopathy Restrictive Publications
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.
This cardiomyopathy was also associated with thrombosis within the sinus venosus.
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.
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.
Here, we review the aetiology and pathogenesis of EMF and report a case of a young male from Mumbai (non-endemic area) presenting with progressively worsening breathlessness and signs of heart failure unresponsive to conventional medical treatment. To delineate the extent of the disease transthoracic echocardiography and cardiac Magnetic Resonance Imaging (MRI) was done which revealed infiltrative lesions in left ventricular apex with grade 2/3 mitral regurgitation. Due to progressive and severe nature of the disease the patient was managed conservatively. Through this report we would like to rekindle the interest of reader in a forgotten tropical disease which is considered rare in this geographical area but should not be missed as a cause heart failure considering its significant mortality.
Results Five individuals died from cardiac diseases, two of whom suffered from sudden cardiac death. Two individuals have suffered from chronic cardiac disorders. Mutation analysis revealed a novel missense mutation in exon 7 of troponin I type 3 (TNNI3), resulting in substitution of serine (S) with proline (P) at amino acid position 150, which cosegregated with the disease in the family, which is predicted to be probably damaging using PolyPhen-2. The mutation was not detected in the 200 unrelated subjects we tested.Conclusion Using next generation sequencing, which has very recently been shown to be successful in identifying novel causative mutations of rare Mendelian disorders, we found a novel mutation of TNNI3 in a Chinese family with RCM.
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.
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.
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.