scholarly journals Clinical features of Neonatal Cardiomyopathy

2019 ◽  
Vol 2 (3) ◽  
pp. 115-119
Author(s):  
Sakurai K ◽  
Nakano M ◽  
Masumori C ◽  
Mizuno M ◽  
Aso K
Keyword(s):  
Heart Failure ◽  
Heart Transplantation ◽  
Clinical Features ◽  
Noonan Syndrome ◽  
Severe Heart Failure ◽  
Left Ventricular ◽  
Leopard Syndrome ◽  
Cardiac Murmur ◽  
Underlying Diseases

Background: Neonatal cardiomyopathy is a rare disease that ranges from being asymptomatic to abruptly lethal and is not well characterized [1]. We investigated the clinical features of five neonates with cardiomyopathy in our hospital to determine key clinical characteristics. Methods: We retrospectively reviewed the records of five newborns who were diagnosed with cardiomyopathy between January 2000 and December 2018. The primary evaluation included reasons for diagnosis, underlying diseases, therapy, and turning point. Results: Patients with hypertrophic cardiomyopathy (HCM) or left ventricular noncompaction (LVNC) were diagnosed on the basis of cardiac murmur, while the patient with dilated cardiomyopathy (DCM) was diagnosed on the basis of sucking failure. Underlying diseases included Noonan syndrome and LEOPARD syndrome. All patients had received β-blockers, and those with DCM and LVNC were also administered diuretics and angiotensin-converting enzyme inhibitors. The two patients with HCM underwent follow-up as out-patients. One patient with HCM died at 3 years old because of arrhythmia. The patient with DCM died due to heart failure 38 days after birth. The patient with LVNC exhibited severe heart failure after birth, requiring follow-up while considering heart transplantation. Conclusion: Noonan syndrome and LEOPARD syndrome, which is RAS/MAPK-related diseases, should be considered in patients diagnosed HCM. Because heart failure progresses rapidly in patients with neonatal DCM and those with LVNC, planned therapy should include consideration of heart transplantation.

scholarly journals Chronic heart failure in patients with nonalcoholic fatty liver disease: prevalence, clinical features, and relevance

2018 ◽  
Vol 46 (9) ◽  
pp. 3959-3969 ◽  
Author(s):  
Zichuan Zhang ◽  
Peize Wang ◽  
Fei Guo ◽  
Xinmin Liu ◽  
Taiyang Luo ◽  
...  
Keyword(s):  
Heart Failure ◽  
Fatty Liver ◽  
Clinical Features ◽  
Linear Regression Analysis ◽  
Left Ventricular ◽  
Nonalcoholic Fatty Liver ◽  
Major Cardiovascular Events ◽  
Lv Mass ◽  
Significant Difference

Objective This study was performed to assess the prevalence of nonalcoholic fatty liver (NAFL) in patients with symptomatic congestive heart failure (CHF) and compare the clinical features with those of patients without NAFL. Methods In total, 102 patients with CHF were divided into NAFL and non-NAFL groups according to their hepatic ultrasonography findings. All patients underwent transthoracic echocardiography and cardiac magnetic resonance examination. Follow-up was performed for major cardiovascular events (MACE) and readmission due to heart failure at 1, 3, 6, and 12 months after the index hospitalization. Results NAFL was detected in 37 of 102 patients (36.27%). Compared with the non-NAFL group, patients with NAFL were younger, had a higher body mass index and left ventricular (LV) mass index, and had more severe fibrosis. MACE and readmission occurred in 15 patients in the NAFL group and 29 patients in the non-NAFL group, without a significant difference. Linear regression analysis revealed that after adjusting for confounders, NAFL was independently associated with the LV fibrosis size and the ratio of the LV fibrosis size to the LV mass index. Conclusions NAFL is present in more than one-third of patients with CHF and is associated with the severity of LV fibrosis.

Left Ventricular Inflow Tract Obstruction Secondary to a Myxoma in a Dog

2011 ◽  
Vol 47 (3) ◽  
pp. 217-223 ◽  
Author(s):  
M. Josefa Fernandez-del Palacio ◽  
Joaquin Sanchez ◽  
Jesus Talavera ◽  
Carlos Martínez
Keyword(s):  
Heart Failure ◽  
Congestive Heart Failure ◽  
Clinical Signs ◽  
Left Ventricular ◽  
Exercise Intolerance ◽  
Cardiac Murmur ◽  
Cardiac Apex ◽  
Spectral Doppler ◽  
History Of

This is the first description of a left ventricular inflow tract obstruction secondary to a myxoma in a dog. A 4 yr old, male fox terrier presented with a 1 mo history of cough and exercise intolerance. Expiratory dyspnea, pulmonary crackles, irregular cardiac rhythm, and a grade 4/6 pansystolic cardiac murmur over the left cardiac apex were the most important features on physical examination. The electrocardiogram revealed atrial fibrillation. Thoracic radiographs showed left-sided cardiac enlargement and mild pulmonary edema, especially in the hilar area. Two-dimensional transthoracic echocardiography showed severe left atrial dilation and a homogenous, echodense mass involving both leaflets of the mitral valve and the posteromedial papillary muscle, inducing mitral stenosis. Spectral Doppler echocardiography was consistent with severe left ventricular inflow tract obstruction secondary to a mass. Therapy for congestive heart failure was prescribed. Follow-up examinations of the dog 1 mo, 2 mo, and 6 mo after diagnosis showed an improvement in clinical signs, but similar echocardiographic features. Eleven months after diagnosis, the dog was euthanized at the owner's request because of recurrent congestive heart failure. The postmortem examination showed the cardiac tumor was consistent with a myxoma.

scholarly journals 125 Sex differences in myocarditis natural history

2021 ◽  
Vol 23 (Supplement_G) ◽  
Author(s):  
Matteo Castrichini ◽  
Marco Merlo ◽  
Chiara Baggio ◽  
Giulia Gagno ◽  
Davide Maione ◽  
...  
Keyword(s):  
Heart Failure ◽  
Chest Pain ◽  
Natural History ◽  
Heart Transplantation ◽  
Ventricular Arrhythmias ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Definite Diagnosis ◽  
The Impact

Abstract Aims The role of sex in determining the profile and the outcomes of patients with myocarditis is widely unexplored. Our study seeks to evaluate the impact of sex as a modifier factor in the clinical characterization and natural history of patients with definite diagnosis of myocarditis. Methods and results We retrospectively analysed a single-centre cohort of consecutive patients with definite diagnosis (i.e., endomyocardial biopsy or cardiac magnetic resonance proven) of myocarditis. A sub-analysis was performed after division of population according to the main symptom of presentation (i.e., chest pain, ventricular arrhythmias, and heart failure). Clinical and echocardiographic data were evaluated at diagnosis and at last available evaluation (i.e., median of 30 months). The study outcome measure was a composite of all-cause mortality or heart transplantation. We enrolled 312 patients (187; 60% presenting with chest pain; 19; 6% with ventricular arrhythmias; 106; 34% with heart failure). Most of patients (211, 68% of the whole population) were males, consistently in the three modes of presentation. Despite no clinically relevant differences were found at baseline presentation, males presented a larger indexed left ventricular end-diastolic volume (LVEDVi) (62 ± 23 vs. 52 ± 20, P = 0.011 in males vs. females respectively) at follow-up evaluation. At a median follow-up of 62 months, 36 (17%) males vs. females experienced death or heart transplantation (Log-rank P = 0.037). At multivariable Cox analysis, male sex emerged as a predictor of mortality (HR: 2.358; 1.044–5.322; P = 0.039 and left ventricular ejection fraction (LVEF) < 50% (HR: 8.169; 1.226–54.425; P = 0.030)]. Results were consistent in patients presenting with heart failure and chest pain, while arrhythmic group was too small to be reliably interpreted. Conclusions In a large cohort of patients with definite diagnosis of myocarditis, females experienced a more favorable long-term prognosis than male, despite a similar clinical profile at baseline.

scholarly journals Neurocognitive profile of patients with continuous flow Left Ventricular Assist Device

2015 ◽  
Vol 82 (2) ◽  
Author(s):  
David Simoni ◽  
Martina Rafanelli ◽  
Andrea Ungar ◽  
Enrico Mossello ◽  
Niccolò Marchionni ◽  
...  
Keyword(s):  
Heart Failure ◽  
Heart Transplantation ◽  
Left Ventricular Assist Device ◽  
Ventricular Assist Device ◽  
Continuous Flow ◽  
Severe Heart Failure ◽  
Left Ventricular ◽  
Assist Device ◽  
Ventricular Assist ◽  
Left Ventricular Assist

The increase of severe heart failure and the consequent reduction of the organ availability for transplantation has led to, in recent years, the introduction of the LVAD as replacement therapy to heart transplantation. Severe Heart Failure patients show cognitive deficits in various domains especially in executive functions, memory and speed of proceedings, due to different neurophysiopathological processes including chronic hypoperfusion and subsequent damage to hippocampal and para-hippocampal cortical areas. It is also known that these deficits improve after heart transplantation. We carried out a literature review selecting studies that analyzed the cognitive changes in patients with severe heart failure after implantation of the Continuous Flow Left Ventricular Assist Device. According to the inclusion criteria, we selected four studies since 2005 that presented a comprehensive neurocognitive assessment. The results show that the cognitive profile, with the implantation of LVAD improves in memory and executive domains, and this improvements results stable in short- medium time. The effects would also be independent of the type of flow produced by the device (pulsatile vs continuous). We believe that further studies are required to explore the relationship between LVAD and cognitive function in severe heart failure.

Predictors of death and heart transplantation in biopsy-proven myocarditis: a machine-learning approach

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
A Caforio ◽  
G Lorenzoni ◽  
C.Y Cheng ◽  
A Baritussio ◽  
D Marcolongo ◽  
...  
Keyword(s):  
Machine Learning ◽  
Heart Failure ◽  
Risk Stratification ◽  
Heart Transplantation ◽  
Left Ventricular ◽  
Funding Source ◽  
Giant Cell Myocarditis ◽  
Lower Survival ◽  
Dismal Prognosis

Abstract Background Risk stratification for death and heart transplantation (HTx) in myocarditis is complex. A random forest (RF) is a tree-based machine learning technique (MLT) which is being increasingly used for clinical data analysis; it allows the detection of complex relationships between the outcome of interest and the covariates, overcoming the limits of traditional statistical analysis (i.e. regression approaches). Purpose To assess the potential role of clinical and diagnostic features at presentation as predictors of death and HTx in biopsy (Bx)-proven myocarditis using RF. Methods From January 1993 to August 2019, we consecutively enrolled 357 patients with Bx-proven myocarditis (65% male, median age 39 years, interquartile range (IQR) 26–51). An RF approach for survival data was used. Variables included in the analysis were: histology type by Bx, NYHA, type of presentation (infarct-like, arrhythmia, heart failure), viral genome detection on Bx, serum antiheart (AHA), antiintercalated disk (AIDA), anticardiac endothelial cells (AECA), antinuclear (ANA) autoantibodies, immunosuppressive therapy, cardiac catheterisation (left ventricular enddiastolic volume (LVEDV), mean capillary wedge pressure, right and left ventricular enddiastolic pressure) and 2-D echocardiographic measures (LVEDV, left ventricular ejection fraction (LVEF) at presentation and at follow-up, right ventricular fractional area change (FAC%), right ventricular diastolic area). Results The median follow-up time was of 1352 days (IQR 423.25–2535.75). At the end of follow-up, 42 patients were dead or transplanted. The 1-year, 5-year, and 10-year survival probabilities were of 0.928, 0.854, and 0.817, respectively. The most relevant predictors of death or HTx identified by the RF algorithm (according to the variable importance measure) were histological type, NYHA, clinical presentation, LVEF, and FAC%. Among the circulating auto-antibodies AECA were found to be the most important. Histological type was the strongest predictor of death/HT (100% relative importance, (RI)), giant cell myocarditis having a lower survival probability compared to other types. The next stronger predictors were advanced (III-IV) NYHA and heart failure presentation with lower survival probabilities (90% and 84% RI respectively). AECA-positive patients had lower survival probability compared to AECA negative ones (20% RI). The RF algorithm revealed an excellent predictive performance in the correct identification of all alive patients, with only 5 dead patients being misclassified (balanced accuracy 94%). Conclusions Autoimmune features, i.e Giant cell myocarditis and AECA, as well as severity of heart failure and of left ventricular disfunction at presentation were the strongest predictors of dismal prognosis. Our RF approach provides a new automated powerful tool for accurate risk stratification for death/HTx in Bx-proven myocarditis. Funding Acknowledgement Type of funding source: Public Institution(s). Main funding source(s): Budget Integrato per la Ricerca dei Dipartimenti (BIRD, year 2019), Padova University, Padova, Italy (project Title: Myocarditis: genetic background, predictors of dismal prognosis and of response to immunosuppressive therapy.)

scholarly journals Lymphocytic myocarditis: a genetically predisposed disease?

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
J Artico ◽  
M Merlo ◽  
G Delcaro ◽  
A Cannata ◽  
P Gentile ◽  
...  
Keyword(s):  
Heart Failure ◽  
Genetic Background ◽  
Rna Binding ◽  
Severe Heart Failure ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Ventricular Ejection Fraction ◽  
Lv Dysfunction ◽  
Lymphocytic Myocarditis

Abstract Background Clinical presentation of myocarditis is extremely heterogeneous from asymptomatic to overt severe heart failure (HF). A complex interaction between pre-existing genetic background and inflammation might be responsible for this heterogeneity. Purpose The aim of the present study was to investigate whether positive genetic background for pathogenic cardiomyopathy-related variants might underlie a higher susceptibility to left ventricular dysfunction in patients with active lymphocytic myocarditis. Methods We prospectively performed genetic tests in 36 patients (46±15 years; 61% males; no relatives included) with biopsy-proven active lymphocytic myocarditis according to Dallas criteria and immunohistochemistry. Only pathogenic (P) or likely pathogenic (LP) variants were considered. Results After genetic test, 31% of patients (n=11) were carriers of P/LP truncating variants in structural Cardiomyopathy related genes: Titin (TTN, n=8, 73%), Desmoplakin (DSP, n=1), Filamin C (FLNC, n=1) and RNA binding protein 20 (RBM20, n=1). Among the 27 patients presenting with HF and LV dysfunction, the positive genetic yield was similar to the total cohort (n=9, 34%; 90% with TTN). Two out of six arrhythmic patients (30%) were carriers in arrhythmogenic genes (i.e. DSP and FLNC), whereas no patients with infarct-like presentation were carriers. During follow-up, 44% of patients (n=16) presented normal Left Ventricular Ejection Fraction (LVEF). Carriers had a lower rate of LVEF normalization compared to non-carriers (18% vs 56%, respectively; p=0.035). Conclusion Positive genetic testing for cardiomyopathy-related-genes might be found in a non-negligible percentage of patients with biopsy-proven myocarditis, especially if presenting with heart failure and LV dysfunction. Compared to non-carriers, carriers of P/LP variants show lower likelihood of LVEF normalization during follow-up. Funding Acknowledgement Type of funding source: None

scholarly journals Numerical study of shear-based hemolysis in aorta with left ventricular assistance device

2021 ◽  
Vol 321 ◽  
pp. 03009
Author(s):  
Jianfei Song ◽  
Louis Marcel ◽  
Mathieu Specklin ◽  
Smaine Kouidri
Keyword(s):  
Heart Failure ◽  
Numerical Study ◽  
Severe Heart Failure ◽  
Left Ventricular ◽  
Flow Topology ◽  
Left Ventricular Assistance Device ◽  
Ventricular Assistance Device ◽  
Ventricular Assistance ◽  
Left Ventricular Assistance

Ventricular assistance devices (VADs) for heart failure treatment have been paid high attention among researchers for decades. However, the follow-up complications such as hemolysis and thrombosis require further optimization for this technique. Shear stress has been demonstrated to be significantly related to the hemolysis because of the rupture of red blood cells membrane with a leaking of hemoglobin in the plasma. This issue has already been investigated inside the pump of VAD, but estimations are still lacking regarding hemolysis generation in the aorta itself after VAD implantaion. Thus, the present study aims to evaluate the hemolysis in aorta through establishing the 3D numerical model of aorta with left ventricular assistance device (LVAD). Non-Newtonian Carreau model has been adopted. Comparisons of hemolysis evaluation have been made with two different mathematical models existing in literature. Moreover, the flow topology and hemodynamic variations have been studied. Different working conditions of LVAD have been considered corresponding to different heart failure severities. The results reveal a relatively low level of hemolysis risks in aorta. The thrombosis is more prone to occur in the case of severe heart failure condition.

Abstract 815: Recovery of Heart Function in Women with Severe Heart Failure from Peripartum Cardiomyopathy in Haiti

Circulation ◽  
2008 ◽  
Vol 118 (suppl_18) ◽  
Author(s):  
James D Fett ◽  
Herriot Sannon ◽  
Emmeline Thélisma ◽  
Therese L Sprunger ◽  
Venkita Suresh
Keyword(s):  
Heart Failure ◽  
Heart Function ◽  
Systolic Function ◽  
Initial Period ◽  
Severe Heart Failure ◽  
Left Ventricular ◽  
Peripartum Cardiomyopathy ◽  
Beta Blockade ◽  
Lv Systolic Function

Peripartum cardiomyopathy (PPCM) is a form of heart failure from dilated cardiomyopathy with one of the greatest potentials for eventual full systolic function recovery. Traditional concepts of PPCM have held that if recovery did not occur by the six-month post-diagnosis mark, it would be unlikely to happen. This report shows that improvement and eventual full recovery may occur long after that initial period. PPCM patients have been identified from the Hôpital Albert Schweitzer PPCM Registry, Deschapelles, Haiti, from 2000 to 2008. All patients fully met diagnostic criteria for PPCM, which include onset of heart failure during the last month of pregnancy up to 5 months postpartum, absence of previous heart disease or other cause of heart failure, and echocardiographic criteria for systolic dysfunction. Recovery was defined as left ventricular (LV) ejection fraction (EF) greater than 50 percent. Echocardiography was carried out at initial diagnosis and at 6-month intervals, with a minimum of 24 months follow-up required for inclusion. Standard treatment included diuretics (lasix) and ACE-inhibitors (captopril), as determined by economic factors, which also excluded the routine use of beta-blockade. Thirty-two out of 116 (27.6 %) PPCM patients fully recovered LV systolic function. Mean follow-up was 35 months. The shortest time to recovery was 3 months and the longest time to recovery was 48 months. The number and cumulative percentage of recovery is shown in the following table : Table: Length of time required for recovery of left ventricular function in 32 Haitian PPCM patients, 2000–2008: Likelihood of recovery did not correlate with age (17–47 years), parity (1–10) or LV EF at diagnosis (12 to 40 %). Complete recovery of LV systolic function in PPCM often occurs after the first 6 to 12 months following diagnosis. It is important to continue treatment and follow-up sufficiently long to assure and document maximum benefit

scholarly journals Ceruloplasmin, NT-proBNP, and Clinical Data as Risk Factors of Death or Heart Transplantation in a 1-Year Follow-Up of Heart Failure Patients

2020 ◽  
Vol 9 (1) ◽  
pp. 137
Author(s):  
Ewa Romuk ◽  
Wojciech Jacheć ◽  
Ewa Zbrojkiewicz ◽  
Alina Mroczek ◽  
Jacek Niedziela ◽  
...  
Keyword(s):  
Risk Factors ◽  
Heart Failure ◽  
Heart Transplantation ◽  
Prognostic Value ◽  
Cox Regression ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Ventricular Ejection Fraction ◽  
Cox Regression Analysis

We investigated whether the additional determination of ceruloplasmin (Cp) levels could improve the prognostic value of N-terminal pro-B-type natriuretic peptide (NT-proBNP) in heart failure (HF) patients in a 1-year follow-up. Cp and NT-proBNP levels and clinical and laboratory parameters were assessed simultaneously at baseline in 741 HF patients considered as possible heart transplant recipients. The primary endpoint (EP) was a composite of all-cause death (non-transplant patients) or heart transplantation during one year of follow-up. Using a cut-off value of 35.9 mg/dL for Cp and 3155 pg/mL for NT-proBNP (top interquartile range), a univariate Cox regression analysis showed that Cp (hazard ratio (HR) = 2.086; 95% confidence interval (95% CI, 1.462–2.975)), NT-proBNP (HR = 3.221; 95% CI (2.277–4.556)), and the top quartile of both Cp and NT-proBNP (HR = 4.253; 95% CI (2.795–6.471)) were all risk factors of the primary EP. The prognostic value of these biomarkers was demonstrated in a multivariate Cox regression model using the top Cp and NT-proBNP concentration quartiles combined (HR = 2.120; 95% CI (1.233–3.646)). Lower left ventricular ejection fraction, VO2max, lack of angiotensin-converting enzyme inhibitor or angiotensin receptor blocker therapy, and nonimplantation of an implantable cardioverter-defibrillator were also independent risk factors of a poor outcome. The combined evaluation of Cp and NT-proBNP had advantages over separate NT-proBNP and Cp assessment in selecting a group with a high 1-year risk. Thus multi-biomarker assessment can improve risk stratification in HF patients.

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