Idiopathic Dilated Cardiomyopathy Research Articles

149 Acute effects on left ventricular systolic and diastolic function of a post-implant echocardiographic biventricular pacemaker optimization protocol following cardiac resynchronization therapy

Abstract Background Cardiac resynchronization therapy (CRT) has demonstrated efficacy in at least 60% of patients with left ventricular (LV) failure and guideline-based indication to CRT. Whereas lack of response to CRT in up to a third of patients is multifactorial, a relevant factor is thought to be inadequate biventricular pacemaker (BIV) optimization (OPT) of either the intraventricular (VVd) or atrioventricular (AVd) delay Purpose In this echocardiographic observational study, we compared the acute effects on LV contractility, output, and diastolic function of BIV intra-implant QRS duration-based (OPTq) and post-implant Doppler echocardiography-based (OPTe) OPT of VVd and AVd. Methods In 160 patients with ischemic (n = 86) or idiopathic (n = 74) dilated cardiomyopathy, guideline-based different de novo CRT systems were implanted followed by immediate OPTq. Post-implant (10 days) OPTe was performed measuring: transmitral velocity-time integral (MVFi), % diastolic filling time (MVFt%), and E/A ratio, LV outflow integral (LVOTi), ejection time (LVOTt), and stroke volume (SV), isovolumic contraction (IVCT) and relaxation (IVRT) times, and LV myocardial performance index (MPI). The protocol included, sequentially: 1) Doppler measurements with OPTq settings; 2) measurements (separated by 3’ intervals) during a range (80/200 ms) of AVd with synchronous VVd; 3) algorithm-based AVd selection (at least 2 of following: increase in MVFi or SV, decrease in MPI); 4) measurements, with set AVd, during range of VVd: LV-first (-20, -40ms); RV-first (20, 40ms); synchronous; 5) VVd selection based on same algorithm used for AVd selection. Results. At OPTq, 58.6% of patients were set synchronous, 38.6% LV-first and 3% RV-first, with a 126 ± 29 mean AVd. This increased to 137 ± 36 after OPTe, when 49.1% were set synchronous, 38% LV-first and 12.4% RV-first, resulting in modifications of AVd and VVd in 59% and 36% of patients. Further, gain in SV with OPTe, compared to OPTq, was 8.3% (p<.001), paralleled by an increase in MVFi (21.2 ± 8 cm vs 20.5 ± 8, p<.001) and decrease in E/A (1.25 vs 1.45, p<.001). The greatest increase in SV with OPTe was found in patients in whom both AVd and VVd were modified (n = 48; 81 ± 26 ml vs 71 ± 23, p<.001) vs. patients without modifications (n = 42), or with change of either AVd or VVd (n = 70; 77 ± 20 vs 72 ± 20, p<.01). Only in the first patient group both MVFi (22 ± 9 vs 20 ± 9, p<.001) and MVFt% (52 ± 7 vs 49 ± 8, p=.004) increased, along with a decrease in MPI (.82±.31 vs .92 ± 36, p=.007) and IVRT (144 ± 51 vs 156 ± 62, p=.02. Conclusions These preliminary results point to a significant incremental role of post-implant OPTe to enhance LV output, contractility, and diastolic function in patients with CRT. The prognostic role of OPTe-determined AVd and VVD changes remains to be determined.

063 A Case of Chronic Thromboembolic Pulmonary Hypertension in a Heart Transplant Patient, Successfully Treated With Pulmonary Endarterectomy

We present a case of chronic thromboembolic pulmonary hypertension (CTEPH) 15 years post heart transplantation, requiring pulmonary endarterectomy, with subsequent improvement of pulmonary hypertension (PH) and symptoms. The patient was a 71 year old male presenting with dyspnoea, who previously underwent heart transplantation for idiopathic dilated cardiomyopathy. His co-morbidities included a pulmonary embolism (PE) after traumatic pulmonary contusion ten years prior, mild renal impairment, type 2 diabetes, hypertension and obstructive sleep apnoea. Medications included warfarin, cyclosporin, everolimus, mycophenolate mofetil and prednisolone. Transthoracic echocardiogram demonstrated elevated pulmonary pressures. On right heart catheter (RHC); pulmonary artery systolic pressure (PASP) was 66 mmHg, pulmonary artery diastolic pressure (PADP) was 28 mmHg, and mean pulmonary artery pressure (mPAP) was 45 mmHg. The mean right atrial pressure (RAP) was 9 mmHg, mean pulmonary capillary wedge pressure (MPCWP) was 8 mmHg and pulmonary vascular resistance (PVR) was 562 dynes/sec/cm5. V/Q and CTPA demonstrated bilateral chronic PE, confirmed on formal pulmonary angiography. The patient’s six-minute walk test (6MWT) was 317m (50% predicted). Riociguat was commenced and warfarin continued, and he underwent a pulmonary endarterectomy. The patient’s symptoms improved and his 6MWT was 397 m (63% predicted). RHC 11 months later showed a PASP of 47 mmHg, PADP of 23 mmHg, mPAP of 33 mmHg, RAP of 10 mmHg and PVR of 240 dynes/sec/cm5; demonstrating improvement. This demonstrates the first described case of CTEPH post-heart transplant. As a treatable cause of PH, the importance of a high index of suspicion for CTEPH is clear.

P1336 Sometimes the heart could appear different if seen under a magnetic resonance scanner: misdiagnosed cases of isolated left ventricle apical hypoplasia and double chambered left ventricle

Abstract Introduction Congenital heart diseases (CHD) are detected in 1% of children, often usually the first year of life; however, many defects are diagnosed later or remain undiagnosed. Both congenital and acquired disorders could affect the left ventricle (LV). First-line assessment includes echocardiography; nonetheless, because of intrinsic or technical limitations and artefacts, further investigation may be required. Cardiac Magnetic Resonance (CMR) is capable of providing anatomical and functional information without many of limitations and drawbacks of echocardiography. We describe CMR findings of misdiagnosed cases of two rare congenital LV abnormalities: isolated LV apical hypoplasia (ILVAH) and double-chambered LV (DCLV). Case report 1 (image A,B,D) An 18 yrs girl diagnosed with dilated cardiomyopathy (DCM) was submitted to our CMR Lab for a functional assessment. After birth, deep Q waves in the inferior leads were noted on the ecg, and an echocardiographic examination showed a dilated and hypokinetic LV. At 1 year of age, she underwent cardiac catheterization that excluded coronary arteries anomalies and confirmed a reduced LV systolic function. A diagnosis of idiopathic DCM was formulated and she was initiated with anticongestive therapy. During the follow-up she felt well with normal exercise tolerance, longitudinal echocardiography did not show any substantial modification over the years. In 2018, a CMR study was performed. Surprisingly, the cardiac apex was formed exclusively by the right ventricle, wrapped around the LV. The LV appeared spherical and truncated inferiorly, and the apical portion was missing; LV volumes and ejection fraction were normal; regional akinesia and subendocardial late gadolinium enhancement (LGE) were evident at the inferior wall. These findings were consistent of ILVAH. Case Report 2 (image C,E,F) A 24 yrs old boy with prenatal diagnosis of LV diverticulum came to our observation for a CMR study. He was asymptomatic, particularly, no palpitations or syncope were referred, nor arrhythmias were detected during the follow-up; exercise tolerance was normal. CMR showed a coarse muscle band in the LV cavity, extending from the apex to the posterior papillary muscle, thus delimiting a contractile accessory chamber. LV volumes and global systolic function were normal. The LV accessory chamber presented a normal structured free lateral wall with a normal systolic thikening, except at the apical infero-lateral segment where it appeared thinned and akinetic and showed LGE with a subendocardial pattern. These findings were consistent of DCLV. Conclusions the LV could be affected by many diseases with different etiological, clinical and morphological features. Compared to other imaging diagnostic modalities, CMR allows better definition of LV morphology, function and tissue characterization, becoming essential for LV abnormalities diagnosis and follow-up. Abstract P1336 Figure.

P1439 Familial dilated cardiomyopathy: assessment of left ventricular systolic and diastolic function by echocardiogram in asymptomatic patients

Abstract Funding Acknowledgements Alfonso X El Sabio University Introduction Familial dilated cardiomyopathy (fDCM) represents 20% to 30% of idiopathic DCM (iDCM) ethiology. The assessment of cardiac function of these patients is awfully complex. Usually, myocardial fiber damages can not be detected in the early DCM stages. In this sense echocardiogram could be useful to detect incipient changes. Purpose The aim of this study was to characterize the systolic function of asymptomatic fDCM, compared within iDCM and control patients. Material and methods This study was carried out in 33 fDCM patients. A total of 4 fDCM families with LMNA gene mutation and 3 fDCM families with BLC2-associated athanogene 3 (BAG3) mutation were recruited. Moreover, a total of 30 iDCM and 66 healthy matched controls were enrolled in the study. Results 58.14% were male. The average age was 45.3 ± 17 years. 72% showed sinus rhythm. Left bundle branch block (LBBB) was observed in 7.8% of patients. The LV ejection fraction (LVEF), sphericity index and mitral annular plane systolic excursion (MAPSE), were significantly improved in the fDCM patients compared to iDCM subjects. However, these parameters were aggravated compared with healthy controls. LVEF was enhanced in fDCM in contrast to iDCM (56% versus 35%; P < 0.001). Nevertheless, LVEF value was deteriorated in fDCM compared to healthy controls (56% versus 65%; P < 0.001). The values of septal and lateral annulus early diastolic velocity measured by DTI, were also diminished. All results are presented in Table 1. Conclusions Asymptomatic fDCM shown an intermediate value of LVEF between the iDCM and the control group. This ventricular remodeling process could be the consequence of a slight increase in the end-systolic diameter. Patients Characteristics Patients Characteristics iDCM 30 patients fDCM 33 patients Control Group 66 Healthy P LVEF 32 (29.78-40) 56.0 (39.7-64.2) 65 (62-69.5) 0.001 EDD 62.5 (59.2-65.7) 53.7 (45.7-57.6) 45.50 (43-48.8) 0.001 ESD 53 (47-58.75) 36 (30.9-54.2) 27.9 (24-31) 0.001 MAPSE 11 (10-12.50) 14 (14-18) 19 (17-20) 0.001 Sphericity index 0.70 (0.66–0.79) 0.69 (0.66-0.79) 0.53 (0.48-58) 0.001 LA volume 61.5 (57-75.1) 32 (23-46.5) 17 (14.2-20) NS Septal annulus Early diastolic Velocity (cm/s) DTI 3.5 (3-4.2) 7.5 (1.6-8.8) 9 (7.9-11) 0.001 Lateral annulus Early diastolic Velocity (cm/s) DT 7.2 (5-8.9) 9.5 (1.8-11.8) 13 (10.37-15) 0.001 Table1. Echocardiografic findings in patients. LVEF: left ventricular ejection fraction; EDD: end-diastolic diameter; ESD: end systolic diameter; MAPSE:mitral annular plane systoluc excursion; LA: left atrium; TDI: Tissue Doppler imagin. Abstract P1439 Figure. Familial dilated cardiomyopathy

416 LV only fusion pacing CRT without RV lead induces size and shape LA reverse remodelling

Abstract Background Adaptive CRT pacing induces significant left atrium (LA) reverse remodelling comparing to biventricular (BiV) pacing, although the algorithm delivers compulsory BiV pacing in heart rate over 100/min! Purpose: to assess LA remodelling in LV only pacing without RV lead in a real-life situation of permanent CRT fusion pacing. Methods Prospective data were analysed from a cohort of patients with CRT-P indication implanted with right atrium/left ventricle (RA/LV) DDD pacing system. Complete follow-up at every 6 months included device interrogation, exercise test, transthoracic echocardiography (TE) and individualised drug optimisation. LA evaluation included: parasternal dimension (LAd), 4 chambers view: mid-LA transverse diameter (LAt), basal LA maximal transverse diameter (LAb); volume (LA vol) and shape assessment: trapezoidal LA shape was defined by LAt less than Lab, the reverse situation was considered ellipsoidal shape. Results 55 pts (30 males) with idiopathic DCM aged 62 ± 11 y.o. were included. Baseline characteristic: QRS 164 ± 18 ms; EF 27 ± 5.2%; mitral regurgitation was severe in 22 pts, moderate in 27 pts and mild in 6 pts; 15 pts had type III diastolic dysfunction, 37 pts with type II diastolic dysfunction, 3 pts with type I diastolic dysfunction. Average follow-up was 42 ± 18 months: all patients were responders, EF increased at 37 ± 7.9%; mitral regurgitation decreased in 38 pts (69%), diastolic profile improved in 36 pts (65%). Trapezoidal LA shape was documented in 31 (56%) patients. Atrial fibrillation was noted in 4 pts (7%) and cardioversion was needed. Non-sudden cardiac death occurred in 5 patients (9%), all deaths were noted in pts with severe LA vol, trapezoidal shape and type III diastolic dysfunction. Statistically significative LA reverse remodelling was noted regarding LA volume, but not shape. Conclusions RA/LV fusion CRT pacing was associated with important LA reverse remodelling and a low incidence of AF. Larger randomised studies are needed to validate these results and assess the role of LA shape remodelling in CRT. before RA/LV CRT-P Follow-up 42 ± 18 months p LV EF, %, mean ± SD 27 ± 5.2 37 ± 7.9 <0.0001 LA diameter (mm), mean ± SD 50 ± 4.9 44 ± 2.8 <0.0001 LA area (cm2), mean ± SD 24 ± 5.6 22 ± 0.7 0.0138 LA volume (ml), mean ± SD 104.9 ± 34 80 ± 28.2 0.0001 Abstract 416 Figure.

Diagnostic methods for the canine idiopathic dilated cardiomyopathy: A narrative evidence-based rapid review

Idiopathic dilated cardiomyopathy (DCM) is an important etiology of mortality and morbidity in dogs and its diagnosis relies on systolic dysfunction, chambers dilation, electrical instability and congestion. During the last decades veterinary cardiologists have been joining efforts to obtain diagnostic resources to correctly identify canine DCM in the preclinical stage. Unfortunately, most diagnostic resources have been used with the support of research with weak evidence, without high quality methodologies such as systematic reviews or meta-analysis. Therefore, the support of evidence-based medicine is tailored by empiricism and diagnostic criteria lose'out the ability to properly classify dogs suffering DCM. The presentation of the evidence in medicine is established by multiple sources and the most reliable source has been the presentation of evidence-based medicine from systematic reviews and meta-analysis. Rapid reviews can be interpreted as a pragmatic approach to systematic reviews and although a rapid review follows most of the critical steps of a systematic review to provide timely evidence, some components of a systematic review process are either simplified or omitted. The objective of this narrative evidence-based rapid review is twofold. First: To recognize and to stratify the level of evidence offered by rigorous selected papers about the diagnosis of DCM. Second: To classify the degree of clinical recommendation of the diagnostic resources available.

Low mutation rate in the TTN gene in paediatric patients with dilated cardiomyopathy \u2013 a pilot study

Idiopathic dilated cardiomyopathy (DCM) is a common cardiomyopathy with the prevalence of 1:250, and at least one-third of all the cases are inherited. Mutations in the TTN gene are considered as the most frequent cause of inherited DCM and cover 10–30% of the cases. The studies were mainly focused on the adult or mixed age group of patients with DCM. The mutation rate in the TTN gene, the characteristics of manifestations and their prognostic significance in childhood have not been studied. To determine TTN mutation rate in children with DCM and the relevance of including this gene in the DNA diagnostic protocol for paediatric DCM, complete clinical and instrumental examination of 36 DCM patients (up to 18 years) with the manifestation of the disease was conducted in specialised cardiology centres. Molecular genetic testing included sequencing of coding and adjacent regulatory regions of the major cardiac TTN isoform N2BA using IonTorrent ™ semiconductor sequencing (for 25 isolated cases) and trio whole exome sequencing (trio WES)on the Illumina platform (for 11 family cases). Our pilot group included 36 probands with DCM diagnosis first established on the basis of the generally accepted criteria at the age of 5 days to 18 years(average age: 6.5 years). The sex ratio (M:F) was 23: 8. There were 25 sporadic DCM cases and 11 cases of familial DCM (at least one of the parents and/or siblings were also diagnosed with DCM). The only likely pathogenic truncating variant p.Arg33703*in the TTN gene (TTNtv) was found in a 16-year-oldmale proband out of 36 (3%). Apparently, TTN-dependent forms of DCMs manifest later at a young (but older than 18 years) or more mature age, and TTN gene cannot be considered as the first-line genetic testing for DCM in the paediatric group, despite several studies have reported a generally high mutation rate in this gene with DCM. Further research is needed to compare the representation of mutations in the TTN gene in different age groups of DCM patients.

Association analysis between ARG1 gene polymorphisms and idiopathic dilated cardiomyopathy.

The current study aimed at investigate the potential association of ARG1 polymorphisms in subjects affected by idiopathic dilated cardiomyopathy (IDCM).We have investigated 352 subjects affected by IDCM and 352 population-matched healthy controls by exploiting case-control study. The serum lipids were quantified using spectrophotometric assay, serum arginase activity was done by enzyme colorimetric assay and 2 polymorphisms (rs2781666 and rs2781667) in ARG1 were typed by polymerase chain reaction (PCR) followed by restriction fragment length polymorphism (RFLP) to find out disease associate allele/haplotype segregating in subjects affected by IDCM.Significantly high arginase activity was found to be associated with IDCM subjects when compared with population-matched healthy controls (P < .0001). The higher arginase level in IDCM subjects is negatively correlated with nitrite and nitrate (r = –0.4687, and r = –0.6435, respectively) in our study. There was a significant difference in the distribution of rs2781666 and rs2781667 genotypes of ARG1 polymorphism in patients and controls (P < .0001). Similarly, variant allele T at both loci showed a significant association with the disease phenotypes (P < .0001). Haplotype TT at rs2781666G/T and rs2781667C/T also showed a significantly association (P < .0001).To our knowledge, this is the first report to show a significant involvement of ARG1 polymorphisms to produce IDCM symptoms in subjects originating in Pakistan.

Severe Eosinophilic Myocarditis in the Portion of Left Ventricular Wall Excised to Insert a Left Ventricular Assist Device for Severe Heart Failure

Described herein are 3 adults in whom histologic study of the left ventricular myocardium excised ("LV core") to insert a left ventricular assist device (LVAD) disclosed severe acute myocarditis and the inflammatory cells included numerous eosinophils (eosinophilic myocarditis). Examination of the clinical records disclosed elevated absolute eosinophil counts at the time of insertion of the LVAD and the counts rapidly (<30 days) returned to normal after the operation. Because of the numerous medications that each patient was taking at the time of LVAD insertion, identification of a specific initiating medication as its cause was not possible. Of the 3 patients, 2 had idiopathic-dilated cardiomyopathy and 1 had ischemic cardiomyopathy and each had had heart failure for years. The eosinophilic myocarditis in these 3 patients appears to have been transient and superimposed on the earlier cardiomyopathy.

Long non-coding RNAs in cardiac hypertrophy.

Cardiac hypertrophy (CH) is generally considered adaptive responses that may occur after myocardial infarction, pressure overload, volume overload, inflammatory heart muscle disease, or idiopathic dilated cardiomyopathy, whereas long-term stimulation eventually leads to heart failure (HF). However, the current molecular mechanisms involved in CH are unclear. Recently, increasing evidences reveal that long non-coding RNAs (lncRNAs) play vital roles in CH. Different lncRNAs can promote or inhibit the pathological process of CH by different mechanisms, while the regulation of lncRNAs expression can improve CH. Thus, CH-related lncRNAs may become a novel field of research on CH.

The prognostic nutritional index might predict clinical outcomes in patients with idiopathic dilated cardiomyopathy

Background and aimsThe prognostic nutritional index (PNI) had been associated with adverse outcomes in numerous clinical conditions. However, its influence on idiopathic dilated cardiomyopathy (DCM) was not determined. This aim of this study was to determine the predictive ability of PNI in patients with idiopathic DCM. Methods and resultsA total of 1021 consecutive patients with idiopathic DCM were retrospectively included and divided into three groups based on admission PNI tertiles: <41.7 (n = 339), 41.7–47.3 (n = 342), >47.3 (n = 340). The association of PNI with in-hospital major adverse clinical events (MACEs) and death during follow-up was evaluated. In-hospital mortality (2.9% vs. 1.5% vs. 0.0%, respectively; p = 0.006) and MACEs (13.6% vs. 6.7% vs. 3.5%, respectively; p < 0.001) decreased from the lowest to the highest PNI tertile. The optimal cut-off value of PNI to predict in-hospital MACEs was 44.0 (area under the curve: 0.689; 95% confidence interval [CI]: 0.626–0.753; p < 0.001). Multivariate analysis showed that a PNI≤44.0 was an independent risk factor of in-hospital MACEs (odd ratio: 2.86; 95% CI: 1.64–4.98; p < 0.001) and all-cause mortality at a median follow-up of 27 months (hazard ratio: 1.67; 95% CI: 1.11–2.49; p = 0.013). In addition, patients with a PNI≤44.0 had a lower cumulative survival rate during follow-up (log-rank: 35.62; p < 0.001). ConclusionThe PNI was an independent risk factor for in-hospital MACEs and all-cause mortality at a median follow-up of 27 months in patients with idiopathic DCM; hence, it may be considered a tool for risk assessment.

Assessment of Biventricular Function by Three-Dimensional Speckle Tracking Echocardiography in Adolescents and Young Adults with Human Immunodeficiency Virus Infection: A Pilot Study

Background: The purpose of the study was to assess biventricular parameters of wall deformation with three-dimensional speckle tracking echocardiography (3DSTE) in adolescents and young adults with human immunodeficiency virus (HIV) infection on antiretroviral therapy in order to detect a possible subclinical myocardial dysfunction. Methods: Twenty-one patients aged 12–39 years with HIV, 21 normal controls of the same age and sex, and 21 patients with idiopathic nonischemic dilated cardiomyopathy (DCM) were studied with 3DSTE. All HIV patients were stable in terms of HIV infection, with no history of heart disease or other chronic systemic disease except HIV infection, and were on highly active antiretroviral therapy with good immunological control. Standard echocardiographic measures of left ventricular (LV)-right ventricular (RV) function were assessed. 3D LV global longitudinal strain (GLS), circumferential strain, radial strain, and LV twist were calculated. Global area strain (GAS) was calculated by 3DSTE as percentage variation in surface area defined by the longitudinal and circumferential strain vectors. 3D RV global and free-wall longitudinal strain (FWLS) were obtained. Results: LV GLS and GAS were lower in HIV patients compared to normal controls (p = 0.002, and p = 0.01, respectively). There were no significant differences in LV ejection fractions between the groups. There was a weak positive correlation between LV GLS and age (r = 0.215, p = 0.034) and a weak negative correlation between LV GLS and nadir-CD4 T-cells count (r = 0.198, p = 0.043). DCM patients had more marked and widespread reduction in LV GLS and GAS compared to controls (p < 0.001), whereas in HIV patients LV strain impairment (p < 0.05) was more localized in basal and apical regions. RV FWLS was significantly reduced in HIV patients when compared with the control group (p = 0.03). No patient had pulmonary systolic pressure higher than 35 mm Hg. Conclusions: 3DSTE may help to identify HIV patients at high cardiovascular risk allowing early detection of biventricular dysfunction in the presence of normal LV ejection fraction and in the absence of pulmonary hypertension. LV strain impairment in HIV patients is less prominent and widespread compared to DCM patients.

Clinical profile of patients with Idiopathic Dilated Cardiomyopathy in a Tertiary Care Hospital of Bangladesh

Background: High prevalence of Chronic heart failure due to Idiopathic Dilated Cardiomyopathy (DCM) is animportant cause of heart failure in Bangladesh. This study was carried out to find the clinical characteristics of thepatients with Idiopathic DCM, so that the data can be used to treat symptoms and improve survival and treatment.&#x0D; Methodology: This prospective observational study was carried out in the Department of Cardiology, BIRDEMGeneral Hospital, Dhaka, Bangladesh from January 2012 to December 2018. Total 50 consecutive admittedpatients fulfilling the criteria of Idiopathic DCM were studied. Clinical information, findings fromEchocardiography and other relevant investigations were collected for analysis.&#x0D; Results: Among total 50 patients, 30(60.0%) were male and 20(40.0%) were female. Majority 20(40.0%) patientsbelonged to age 51-60 years and their mean age was found 55.34±13.24 years. Using NYHA (New York HeartAssociation) functional status classification of the patients, 18(36.0%) patients were found in NYHA class I,15(30.0%) in class II, 12(24.0%) in class III and 5(10.0%)in class IV. Almost all patients presented with three basicsymptoms i.e. exertional dyspnea, easy fatigability and pedal edema. Orthopnea, Paroxosmal Nocturnal Dyspnoea(PND), palpitation &amp; chest pain wewe also reported in almost half of the patients. Mean pulse was found88.78±15.75 beat/min, respiratory rate 20.79±6.48 breath/min, BMI 23.12±3.29 kg/m2, systolic BP 119.03±22.22mmHg and diastolic BP 75.00±12.54 mmHg. Bilateral basal crepitation 45(90.0%), Pedal edema 43(86.0%),Raised JVP 39(78.0%), Hepatomegaly 35(70.0%) were also found. Third heart sound in 34(68.0%), Pan systolicmurmur of Mitral regurgitation 40(80.0%), Pansystolic murmur of Tricuspid regurgitation was present in42(84.0%) among study patients. Mean left ventricular ejection fraction was found 30.44 ±4.91%, LVIDs 5.24±0.51cm and LVIDd 6.18 ±0.52 cm.&#x0D; Conclusion: Majority of the Idiopathic DCM patients belonged to age 51-60 year age group with malepredominance and clinical presentation was variable.&#x0D; Bangladesh Crit Care J September 2019; 7(2): 86-89

P749Dynamic of cardiac magnetic resonance imaging parameters in patients with non-ischemic cardiomyopathies after endomyocardial stem cell transplantation

Abstract Background Autologous mesenchymal stem cell (MSC) transplantation can be considered as an alternative method of end-staged chronical heart failure treatment in patients with Non-ischemic Cardiomyopathies (NICMP). About 2/3 NICMP are determined by previous myocarditis, transformed in inflammatory cardiomyopathy (ICM), while only 1/3 of NICMP cases are associated with idiopathic dilated cardiomyopathy (DCM). Application of MSC therapy in different groups of NICMP patients is not sufficiently studied. Purpose The purpose of the study was to compare cardiac magnetic resonance imaging (cMRI) parameters in the left ventricle (LV) segments of myocardium in patients with DCM and ICM before and after autologous MSC transplantation. Methods The study covered 15 patients with DCM (mean age 45.8±3.1 years; 4 females, 11 males; LV ejection fraction (EF) &lt;40%) and 15 patients with ICM (mean age 46.0±3.6 years; 5 females, 10 males; LV EF &lt;40%). All patients underwent cMRI, endomyocardial injections of 0.2 ml autologous MSC culture (CD105+, CD90+, CD73+ cells) into akinetic and hypokinetic myocardial segments. In total we have analysed 228 segments of NICMP patients. Results In patients with ICM we found significant decrease in extracellular volume (ECV) within 6 months after primary examination (from 32 [28; 36]% to 27 [25; 30]%, p=0,007). As a result, ICM patients had lower ECV than DCM patients 6 months after MSC transplantation 27 [26; 29] % and 31 [29; 32]% respectively (p=0,001). Patients with ICM demonstrated an increase in LV systolic myocardial segment thickness (SMST) 6 months after MSC injections (5.91 [5.74; 6.15] mm – baseline vs. 6.67 [6.60; 6.82] mm – 6 months later, p≤0,010) with a bigger increase 12 months after MSC injection (9.58 [9.47; 9.73] mm, p≤0,010). In ICM patients LV SMST was significantly higher than in DCM patients 12 months after MSC transplantation (9.58 [9.47; 9.73] mm vs. 7.38 [7.29; 7.61] mm, p=0.032). ICM patients showed an increase in LV ejection fraction (EF) within 6 months after MSC therapy (from 20 [17; 24]% to 27 [17; 32]%, p=0.043). Conclusions Endomyocardial MSC transplantation showed better results in ICM patients compared to DCM patients. It led to a significant decrease in ECV within 12 months in contrast to DCM patients. ICM patients also demonstrated significant increase of LV SMST and LV EF within 6 months after MSC therapy.

P4546Acute heart failure: speckle tracking echocardiography, a new ally in intensive care unit

Abstract Background Acute Heart Failure (AHF) is a life-threatening condition with high mortality rate. Purpose The aim of our study was to identify the best predictors of in-hospital mortality and stay, among laboratory blood tests, clinic and echocardiographic (standard and by Speckle Tracking Echocardiography, STE) parameters in patients admitted to our Intensive Care Unit (ICU) for AHF. Methods We enrolled 57 patients (age 70±13 y, 70% man) admitted to our ICU with de novo AHF or acute decompensation of Chronic Heart Failure (CHF). Exclusion criteria were: active malignancies, chronic liver disease, absent acoustic echocardiographic window and patient refusal. At ICU admission, all patients were assessed with vital signs (heart rate, HR; systolic blood pressure, SBP), blood laboratory tests, standard echo and STE of left ventricle (LV), right ventricle (RV) and left atrium (LA). These indexes were then related to the length of stay and mortality. Results The population was finally composed of 52 patients, due to 5 in-hospital deaths. 56% had an ischemic aetiology, 26% idiopathic dilated cardiomyopathy, 11% valvular diseases, 7% other causes of HF. At admission, average HR was 78±16 bpm, SBP 119±24 mmHg and EF 33±13%. Among all the parameters, the ones that showed significant statistical correlation with the length of hospitalization (15,34±7.03 days) were plasmatic creatinine, SBP, Systolic Pulmonary Artery Pressure, high LV filling pressure (E/E' &gt;12) and Peak Atrial Longitudinal Strain (PALS). The maximal dose of intravenous loop diuretics and inotropic drugs also showed a good correlation. Analysing the two sub-populations with mortality data, we observed that patients who died, had a significantly lower EF (19±9.62% vs 35±12.55%; p=0,01), but not a higher left atrial volume indexed (31.04±14.87 vs 26.36±12.03 ml/m2; p= ns) compared to the survivors; instead PALS was significant worse (10.08±4.62 vs 20.64±13,35%; p&lt;001). Free wall RV Longitudinal Strain (fw-RVLS) values for the patients who died (−9.41±4.66%) were significantly lower than in survivors (−13.67±6.02%; p&lt;0.01). LVGLS (Left Ventricular Global Longitudinal Strain) did not show statistical significant differences between the two populations. Based on the results of ROC analysis (Figure 1), we created a score to predict in-hospital mortality, composed of: EF, PALS and RVLS-free wall. The ideal cut point to predict mortality was &gt;1.5. Figure 1 Conclusions AHF represents one of the major challenges in ICU. The use of a combined echocardiographic score, assessed at admission, could help to better predict mortality risk, in addition to commonly used indexes.

4300Strain parameters at rest and after exercise in symptomatically stable patients with improved heart failure - STRESS-HF

Abstract Background Optimal medical therapy (OMT) results in improvement in left ventricular (LV) ejection fraction (EF) and reduction in LV size in approximately 40% of patients of heart failure with reduced ejection fraction (HFrEF). Recent studies have proposed to continue treatment in all patients of this subgroup as improvement in LVEF does not indicate actual myocardial recovery. Global Longitudinal strain (GLS) is more sensitive marker of LV systolic function and better predictor of mortality than LVEF. GLS may identify probable patients in whom the therapy can be minimized or stopped. Aim To determine global longitudinal strain at rest and after exercise in patients with idiopathic dilated cardiomyopathy (DCM) who have improved LVEF ≥50% on guideline directed medical therapy (GDMT) and compare with resting &amp; exercising LVEF on 3-dimesional (3D). Material and methods This Observational study was conducted in a tertiary care referral hospital, from February 2018 to October 2018. All patients with idiopathic DCM who had a documented LVEF of ≤40% in the past and improved LVEF (LVEF ≥50%) on GDMT were included in the study. Patients with secondary causes of HFrEF, poor echocardiographic window and inability to exercise were excluded from the study. Strain parameters were calculated at rest and after atleast 5 METS of exercise. GLS value of ≥ minus 12.6% was considered as mildly reduced strain, ≤ minus 8% as severely reduced strain and the values in between as moderately reduced strain. Post exercise, drop of &gt;5% in absolute LVEF value or decrease in GLS &gt;15% from baseline was considered as poor myocardial recovery. Results A total of 44 patients [mean age of 46.8±13.1 years and 24 males (54.5%)] constituted the study group. At the time of diagnosis, 31 (70.5%) patients were in NYHA class II and the remaining were in class III. Duration of GDMT ranged from 4 to 38 (median 12) months. Following improvement on GDMT, 25 (56.8%) were in class I and rest were in class II. Mean LVEF at diagnosis and after recovery was 33.6±4.9% and 55.1±4.5%, respectively with a mean absolute change in LVEF of 21.3±6.1%. At rest, mean 3D LVEF was 53±3.5% and GLS was −12.3±3.1. Mild, moderate and severely reduced strain was seen in 24 (54.5%), 13 (29.6%) and 7 (15.9%), respectively. After exercise, mean 3D LVEF was 51.5.±4.5% (mean decrease 1.5±2.1%) and mean GLS was −7±4.2% (mean decrease −5.3±4.6%). After exercise, none had a fall of LVEF &gt;5% however 32 (72.7%) had a decrease of &gt;15% in GLS. Seven (15.9%) patients had improvement in GLS, 4 (9.1%) had &lt;15% decrease and 1 (2.3%) showed no change in GLS on exercise. The change in GLS on exercise was significant (p=0.001), but change in 3D LVEF was not significant (p=0.956). Conclusion Global longitudinal strain is a better marker for assessing myocardial recovery than LVEF in patients of heart failure with improved ejection fraction. Acknowledgement/Funding None

P776Use of sacubitril-valsartan in an extensive long term follow up cohort in real life

Abstract Background Most clinical data regarding the use of sacubitril-valsartan (SV) in patients with heart failure with reduce EF (HFrEF) come from clinical trials, while observational studies in real life are scarce. Purpose To analyse the efficacy, safety and tolerability of SV in patients with HFrEF in real life. Methods An analysis of the SAVE-RLife (SV Evidence in Real Life) study was performed. This is an observational and ambispective study that included all patients with HFrEF who started SV between SEP2016 and DEC2018. Results A total of 291 patients were included in the study with a median follow-up of 326 (169; 523). Mean age 66.16±11.8, 71 were women (24.4%), 209 patients started treatment at office (71.8%) and 82 during the admission (28.2%). The main aetiologies were ischemic heart disease (51.5%) and idiopathic dilated cardiomyopathy (35.4%). Comorbidities included: 71.5% hypertensive, 55.3% dyslipidemic, 37.1% AF and 44% T2DM. Baseline treatment included 85.9% beta-blockers, 87.2% ACEI or ARA-II, 65.6% MRA and 17.5% iSGLT2. After treatment start with SV, baseline and follow-up analytical parameters such as GFR, K+ levels and NT-BNP, and echo parameters of LV reverse remodeling were evaluated (Table 1). Improvement of the functional NYHA class (Figure 1) and reduction in the incidence of hospital admission or visits to emergency room (ER) were observed. Side effects were 16.2% acute renal failure, 9.3% symptomatic hypotension, 9.7% asymptomatic hypotension and 15.9% hyperkalemia. In 31 patients (11%) the drug was discontinued, one patient due to angioedema. 7.9% of patients died during follow-up, 7 of them due to HF (2.4%). Table 1 Before SV After SV P value Analytical data GFR (ml/min) 70.25±24.95 68.27±24.62 0.008 Serum K+(mEq/L) 4.53±0.50 4.71±0.456 &lt;0.001 NT-proBNP (pg/ml) 2201 (846; 4664) 1146 (436; 2564) &lt;0.001 Echocardiographic data LVEF (%) (n=84) 30.60±7.16 38.30±12.22 &lt;0.001 LVED (mm) (n=72) 65.22±7.82 60.81±8.37 &lt;0.001 PAPs (mmHg) (n=48) 44.48±13.03 37.52±12.25 0.002 MR (grade 1–4) (n=71) 1.83±0.91 1.38±0.90 &lt;0.001 Clinical outcomes HF Hospital/ER Admission the year before (mean± SD) 0.79±1.09 0.41±0.963 &lt;0.001 Predictors of clinical improvement Conclusions SV has shown to improve morbidity and mortality in patients with HFrEF by improving functional class, decreasing NT-proBNP levels and reducing admissions due to HF, without significant side effects. In our study SV improved LVEF and reverse remodeling echocardiographic parameters too.

P5547First results of TranslatiOnal Registry for CardiomyopatHies (TORCH)

Abstract Introduction The cardiomyopathies (CMPs) are a diverse group of cardiac disorders and leading cause of heart failure (HF) and sudden cardiac death (SCD). Clinical courses of CMPs vary considerably even between patients with the same disease entity. The TranslatiOnal Registry for CardiomyopatHies (TORCH) is a comprehensive CMP registry that aims to provide a database and biomaterial bank for basic science and clinical research in this field. The long-term objective of this registry is to increase the understanding of disease pathomechanisms on the way to a better risk stratification as well as identification of new diagnostic and therapeutic approaches in CMP. Methods TORCH is a national, prospective, and multicentre registry within the German Centre for Cardiovascular Research (DZHK), which includes 2300 consecutive patients with non-ischemic (primary and secondary) CMP in 20 centres. The minimum follow up time is one year. The DZHK-wide harmonization of datasets and eCRFs (electronic case report form) were performed to ensure a high level of data quality across different study centres. Results Seventy percent of the enrolled patients are male. Idiopathic/familial dilated cardiomyopathy (DCM) holds the highest prevalence with 54%, followed by inflammatory DCM/myocarditis with a prevalence of 24%. Hypertrophic cardiomyopathy (HCM) is the third-most prevalent CMP in this registry (16%). Infrequent CMPs such as left ventricular non-compaction (LVNC), arrhythmogenic right ventricular cardiomyopathies (ARVC) are also represented in TORCH with a prevalence of 4% and 2% respectively. Major clinical findings are registered in TORCH. Atrial fibrillation was present with a high prevalence among all CMPs (22%-35%). The highest prevalence of stroke or TIA at baseline was reported in patients with LVNC (15.1%) and the lowest in those with amyloidosis (3.3%). Patients with ARVC had the highest number of ICD implantation and adequate ICD therapy (56.7%, p&lt;0.05; 47.1%, p&lt;0.05). Preliminary data analysis showed higher frequency of therapy refractory heart failure symptoms and worse outcome in patients with familial DCM in comparison to those with inflammatory or idiopathic DCM. Conclusion and outlook TORCH represents one of the largest cardiomyopathy registries in Europe. The patients are comprehensively phenotyped through clinical examinations, state-of-the-art imaging modalities, as well as molecular, genetic, and epigenetic investigations. This registry can help researchers design further studies investigating new diagnostic tools or therapeutic strategies in CMPs.

6133Profile of cardiomyopathy patients in Cape Town - pilot phase the IMHOTEP Study

Abstract Background Cardiomyopathies pose a great challenge because of poor prognosis and high prevalence in LMIC with limited access to specialised care. Little is known about the clinical profile of cardiomyopathy in Africa. Purpose Delineation of clinical presentation and cardiovascular magnetic resonance (CMR) phenotypes of cardiomyopathy. Method The African Cardiomyopathy and Myocarditis Registry Program (IMHOTEP) is a prospective multi-centre, hospital-based study and aims to investigate the clinical characteristics, aetiology, genetics, management and outcomes of cardiomyopathies in Africans. Results Assessment of the first 99 adult cases showed that dilated cardiomyopathy (DCM; n=67) was commonest, followed by hypertrophic (HCM; n=13), left ventricular noncompaction (LVNC; n=11), restrictive (RCM; n=4) and arrhythmogenic (ARVC; n=4) cardiomyopathies. Idiopathic DCM (22%) and peripartum cardiomyopathy (16%) accounted for the majority (Figure). A family history of cardiomyopathy or SCD was reported in 20% of cases. Mean age of presentation was 37±12 years. Most patients (96%) were symptomatic at presentation. NYHA class III/IV was more frequently seen in DCM (61%), RCM (50%) and LVNC (64%), whereas syncope was more common in ARVC (50%) and HCM (23%). VT and aborted cardiac arrest were reported in 7% and 3%, respectively. Onset of symptoms in the peripartum period was observed in 47% of women. Beta-blockers and ACE-inhibitors were prescribed in 77% and 78%, respectively, however optimal dosing was achieved in ≤14% of patients at a median time of 5.4 months after symptom onset. CMR was performed in 67 (68%) cases (Table) and contributed diagnostically in a third of cases. Late gadolinium enhancement (LGE) was observed in 92%. In DCM, linear mid-wall and subendocardial patterns of LGE were seen in 95% and 8% of patients respectively – a much higher percentage than previously reported in the literature. CMR volumetric and functional assessment DCM, n=38 HCM, n=11 ARVC, n=3 RCM, n=4 LVNC, n=11 LVEF (%) 27±15 78±7 55±5 52±7 32±17 LVEDV/BSA (ml/m2) 150±40 80±17 98±12 59±13 155±52 LV mass/BSA (g/m2) 82±23 102±35 74±3 71±14 88±31 RVEF (%) 34±15 68±10 22±15 49±18 33±15 RVEDV/BSA (ml/m2) 104±37 68±15 189±27 56±8 106±51 All continuous variables presented as mean ± standard deviation. Cardiomyopathy diagnosis (n=99) Conclusion IMHOTEP is the first multi-centre registry for cardiomyopathy in Africa. Preliminary data suggests an earlier age of onset with female predominance compared to other cohorts, and DCM is the predominant form of cardiomyopathy in Africa. Acknowledgement/Funding NEWTON FUND NON-COMMUNICABLE DISEASE - South African Medical Research Council (SAMRC/GSK)

Association between copper levels and heart failure: a meta-analysis

Background and Objectives: Copper dyshomeostasis can lead to many diseases, including cardiovascular disease. However, there are conflicting reports on the relationship between serum copper levels and heart failure (HF). To explore the relationship between serum copper levels and HF by performing a meta-analysis. Methods and Study Design: The PubMed and ScienceDirect databases until June 2019 were searched for reports on the association between serum copper levels and HF. Results: A total of thirteen studies including 1504 subjects were chosen for the meta-analysis. The pooled analysis indicated that patients with HF had higher copper levels than the control subjects [standardized mean difference (SMD), 0.982; 95% confidence interval (CI), (0.679, 1.285)]. Subgroup analysis stratified by different geographic locations found that HF patients had higher copper levels than the control subjects in Asia and Europe [Asia: SMD, 0.948 and 95% CI, (0.569, 1.327); Europe: SMD, 1.275 and 95% CI, (0.633, 1.917)], but not in America [America: SMD, 0.637 and 95% CI, (-0.109, 1.383)]. Additionally, subgroup analysis revealed that patients with ischemic cardiomyopathy (ICM) [SMD, 1.171; 95% CI, (0.717, 1.624)], idiopathic dilated cardiomyopathy (IDCM) [SMD, 0.569; 95% CI, (0.097, 1.042)] and other types of HF [SMD, 1.152; 95% CI, (0.594, 1.710)] all had higher copper levels than controls. Further subgroup analysis stratified by Newcastle-Ottawa Scale (NOS) scores also found higher serum copper levels in patients with HF than controls within each subgroup. Conclusions: Our meta-analysis identified a significant association between high copper levels and HF.