Mitochondria in Cardiomyopathy: Alterations in Size, Number and Internal Structures

1968 ◽  
Vol 26 ◽  
pp. 126-127
Author(s):  
George Hug ◽  
William K. Schubert
Keyword(s):  
Heart Failure ◽  
Biochemical Analysis ◽  
Left Ventricular ◽  
Size Number ◽  
Internal Structures ◽  
Left Ventricular Outflow ◽  
Chest X Ray ◽  
Myocardial Fibers ◽  
Muscle Biopsies ◽  
Needle Liver Biopsy

A white boy six months of age was hospitalized with respiratory distress and congestive heart failure. Control of the heart failure was achieved but marked cardiomegaly, moderate hepatomegaly, and minimal muscular weakness persisted.At birth a chest x-ray had been taken because of rapid breathing and jaundice and showed the heart to be of normal size. Clinical studies included: EKG which showed biventricular hypertrophy, needle liver biopsy which showed toxic hepatitis, and cardiac catheterization which showed no obstruction to left ventricular outflow. Liver and muscle biopsies revealed no biochemical or histological evidence of type II glycogexiosis (Pompe's disease). At thoracotomy, 14 milligrams of left ventricular muscle were removed. Total phosphorylase activity in the biopsy specimen was normal by biochemical analysis as was the degree of phosphorylase activation. By light microscopy, vacuoles and fine granules were seen in practically all myocardial fibers. The fibers were not hypertrophic. The endocardium was not thickened excluding endocardial fibroelastosis. Based on these findings, the diagnosis of idiopathic non-obstructive cardiomyopathy was made.

scholarly journals Left ventricular noncompaction: Clinical-echocardiographic study

2012 ◽  
Vol 69 (1) ◽  
pp. 32-36 ◽  
Author(s):  
Aleksandra Nikolic ◽  
Ljiljana Jovovic ◽  
Slobodan Tomic ◽  
Milan Vukovic
Keyword(s):  
Heart Failure ◽  
Clinical Presentation ◽  
Color Doppler ◽  
Neuromuscular Diseases ◽  
Left Ventricular ◽  
Left Ventricular Noncompaction ◽  
Ventricular Noncompaction ◽  
Mortality And Morbidity ◽  
Echocardiographic Examination ◽  
Myocardial Fibers

Background/Aim. Left ventricular noncompaction (LVNC) is a disorder in endomyocardial morphogenesis, seen either isolated (in the absence of other cardiac anomalies) or in association with congenital heart disease and some neuromuscular diseases. Intrauterine arrest of the compaction of myocardial fibers is postulated to be the reason of LVNC. Recognition of this condition is extremely important due to its high mortality and morbidity that lead to progressive heart failure, ventricular arrhythmias and thromboembolic events. The aim of this study was to determine the prevalence and clinical presentation of LVNC among consecutive outpatients according to clinical and echocardiographyic findings. Methode. A total of 3,854 consecutive patients examined at the Institute for Cardiovascular Diseases within a period January 2006 - January 2007 were included in the study. All the patients underwent echocardiographic examination using the same equipment (Vivid 7, GE Medical System). Echocardiographic parameters and clinical presentation in patients with echocardiographic criteria for LVNC were analyzed. Results. Analyzing 3,854 consecutive outpatients, using two-dimensional Color Doppler echocardiography from January 2006 to January 2007, 12 patients met the criteria for LVNC. Seven of them were male. The mean age at diagnosis was 45 ? 15 years. Analyzing clinical manifestation of LVNC it was found that seven patients had signs of heart failure, six had arrhythmias with no embolic events. Conclusion. Our results suggest that the real prevalence of LVNC may be higher than expected. New studies have to be done to solve this problem.

U tim thai và sơ sinh: chẩn đoán trước sinh, theo dõi ba trường hợp và phẫu thuật thành công một trường hợp sau sinh tại Bệnh Viện Trung Ương Huế

2020 ◽  
Author(s):  
Du Le Ba Minh
Keyword(s):  
Heart Failure ◽  
Cardiac Tumor ◽  
Fetal Echocardiography ◽  
Ventricular Outflow Tract ◽  
Left Ventricular ◽  
Cardiac Tumors ◽  
Case Methods ◽  
Left Ventricular Outflow ◽  
Postnatal Surgery

Objective: Prenatal diagnosis, postnatal followed up of three cases of cardiac tumor and successful postnatal surgery of one case. Methods: Treatment and postnatal followed up of three cases of cardiac tumor. Those cases were diagnosed by fetal echocardiography. One of them underwent a successful postnatal surgery. Results: Three fetuses were diagnosed with fetal cardiac tumor (capable of Rhabdomyoma) at 28, 27, 38 gestational weeks. There were many tumors in one or two ventricles. These cases were referred to Hue Central Hospital for timing and location of delivery in 2019. One of them had obstruction of left ventricular outflow tract with successful surgical resection at 46 hours after birth. One of them had heart failure after birth. Evolution of three cases was favorable, heart failure regressed, cardiac tumors regressed at 7 - 9 months follow up. Conclusions: Fetal cardiac tumor can be diagnosed prenatally. Those three cases were capable of rhabdomyoma due to the presence of muliple tumors and their regression after birth. Neonatal surgical option should be reserved for the risk of cardiac flow obstruction.

Discrepant effects of heart failure on electrophysiological property in right ventricular outflow tract and left ventricular outflow tract cardiomyocytes

2017 ◽  
Vol 131 (12) ◽  
pp. 1317-1327 ◽  
Author(s):  
Yen-Yu Lu ◽  
Chen-Chuan Cheng ◽  
Chin-Feng Tsai ◽  
Yung-Kuo Lin ◽  
Ting-I Lee ◽  
...  
Keyword(s):  
Heart Failure ◽  
Action Potential ◽  
Ventricular Arrhythmias ◽  
Ionic Currents ◽  
Ventricular Outflow Tract ◽  
Left Ventricular ◽  
Outflow Tract ◽  
Delayed Rectifier ◽  
Patch Clamp Technique ◽  
Left Ventricular Outflow

Ventricular arrhythmias commonly arise from the right (RVOT) and left ventricular outflow tracts (LVOT) in patients without structural heart disease. Heart failure (HF) significantly increases the risk of ventricular arrhythmias. The regional differences and how HF affects the electrophysiological characteristics of RVOT and LVOT cardiomyocytes remain unclear. The whole-cell patch-clamp technique was used to investigate the action potentials and ionic currents in isolated single RVOT and LVOT cardiomyocytes from control rabbits and rabbits with HF induced by rapid ventricular pacing. Comparison with control LVOT cardiomyocytes showed that control RVOT cardiomyocytes have a shorter action potential duration (APD), smaller late Na+ currents (INa-late), larger transient outward (Ito) and larger delayed rectifier K+ currents (IKr-tail), but had similar L-type Ca2+ currents (ICa-L) and Na+/Ca2+ exchanger (NCX) current. HF increased APD, INa-late and NCX, but decreased ICa-L and Ito in RVOT cardiomyocytes. In contrast with this, HF decreased APD and ICa-L, but increased Ito and IKr-tail in LVOT cardiomyocytes. In conclusion, RVOT and LVOT cardiomyocytes had distinctive electrophysiological characteristics. HF differentially modulates action potential morphology and ionic currents in RVOT and LVOT cardiomyocytes.

scholarly journals INDIRECT ULTRASOUND EVALUATION OF LEFT VENTRICULAR OUTFLOW TRACT DIAMETER IMPLICATIONS FOR HEART FAILURE AND AORTIC STENOSIS SEVERITY ASSESSMENT.

2020 ◽  
Author(s):  
Gabriele Pestelli ◽  
Andrea Fiorencis ◽  
Valeria Pergola ◽  
Giovanni Luisi ◽  
Vittorio Smarrazzo ◽  
...  
Keyword(s):  
Heart Failure ◽  
Aortic Stenosis ◽  
Aortic Root ◽  
Left Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Left Ventricular ◽  
Outflow Tract ◽  
Regression Equation ◽  
Ultrasound Evaluation ◽  
Left Ventricular Outflow

Background. Whereas dependency of left ventricular outflow tract diameter (LVOTD) from body surface area (BSA) has been established and a BSA-based LVOTD formula has been derived, the relationship between LVOTD and aortic root and LV dimensions has never been explored. This may have implications for evaluation of LV output in heart failure (HF) and aortic stenosis (AS) severity. Methods. A cohort of 540 HF patients who underwent transthoracic echocardiography was divided in a derivation and validation subgroup. In the derivation subgroup (N=340) independent determinants of LVOTD were analyzed to derive a regression equation, which was used for predicting LVOTD in the validation subgroup (N=200) and compared with the BSA-derived formula. Results. LVOTD determinants in the derivation subgroup were sinuses of Valsalva diameter (SVD, beta=0.392, P<0.001), BSA (beta=0.229, P<0.001), LV end-diastolic diameter (LVEDD, beta=0.145, P=0.001), and height (beta=0.125, P=0.037). The regression equation for predicting LVOTD with the aforementioned variables (LVOTD=6.209+[0.201xSVD]+[1.802xBSA]+[0.03xLVEDD]+[0.025xHeight]) did not differ from (P=0.937) and was highly correlated with measured LVOTD (R=0.739, P<0.001) in the validation group. Repeated analysis with LV end-diastolic volume instead of LVEDD and/or accounting for gender showed similar results, whereas BSA-derived LVOTD values were different from measured LVOTD (P<0.001). Conclusion. Aortic root and LV dimensions affect LVOTD independently from anthropometric data and are included in a new comprehensive equation for predicting LVOTD. This should improve evaluation of LV output in HF and severity of AS, avoiding use of LVOT velocity-time integral alone, which can be misleading, especially when LV cavity and aortic root dimensions are abnormal.

scholarly journals Correlation between QRS duration and cardiac output measured by left ventricular outflow tract velocity in patient with cardiac resynchronization therapy

QJM ◽  
2020 ◽  
Vol 113 (Supplement_1) ◽  
Author(s):  
A S A E Elshikh ◽  
M M Khalifa ◽  
H Shehata ◽  
A Murtada
Keyword(s):  
Heart Failure ◽  
Cardiac Output ◽  
Left Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Left Ventricular ◽  
Cardiac Resynchronization ◽  
Resynchronization Therapy ◽  
Left Ventricular Outflow ◽  
Qrs Duration ◽  
Post Implantation

Abstract Background Cardiac resynchronization therapy (CRT) is proved as an effective treatment for moderate to severe heart failure. It reduces all-cause mortality in patients with advanced heart failure. There is strong evidence that CRT reduces mortality and hospitalization, improves cardiac function and structure in symptomatic chronic heart failure patients with optimal medical treatment, severely depressed LVEF (i.e. &lt;35%) and complete LBBB. However 30% of patients may show negative response to CRT therapy. Therefore, optimization of CRT therapy in patients with heart failure seems to be a main subject for study in our researches. Methods of optimization includes optimization of medical therapy, control of risk factors and comorbidities, and optimization of device implantation and programming. Overall, studying the correlation between QRS duration and cardiac output will improve CRT programming optimization techniques. Aim To study the correlation between QRS duration and cardiac output measured by left ventricular outflow tract (LVOT) VTI in patients with CRT implantation. Methods Study included 100 CRT already implanted patients, they are requested to do a simple electrocardiographic and echocardiographic study. The relation between post implant QRS and cardiac output are studied among the patients. Results There was negative significant correlation between QRS duration and LVOT VTI and SVi. The optimal cut off values for optimal response to CRT using ROC curves were 130msec for post implant QRS duration and 17.1 cm for LVOT VTI. Conclusion CRT response is more in female patients with lower BSA, and without previous history of IHD or smoking. There is a significant negative correlation between QRS duration and LVOT VTI. Post implantation cut off value of QRS duration (&lt;130) predict higher LVOT VTI and also the post implantation benefit for the patient with CRT implanted.

P2634Prognostic value of admission left ventricular outflow tract velocity time integral in hospitalized heart failure patients with preserved ejection fraction: a report from the JASPER registry

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
K Omote ◽  
T Nagai ◽  
K Kamiya ◽  
T Aikawa ◽  
S Tsujinaga ◽  
...  
Keyword(s):  
Heart Failure ◽  
Adverse Events ◽  
Ejection Fraction ◽  
Ventricular Outflow Tract ◽  
Left Ventricular ◽  
Preserved Ejection Fraction ◽  
Velocity Time Integral ◽  
Time Integral ◽  
Left Ventricular Outflow ◽  
Independent Determinant

Abstract Background There are little effective treatment strategies for heart failure with preserved ejection fraction (HFpEF) to achieve a reduction of morbidity and mortality. Thus, accurate prognostication of patients with HFpEF could help improve their outcomes by identifying high-risk patients who might potentially benefit from intensive inpatient and outpatient monitoring and early referral for advanced HFpEF therapy. The left ventricular outflow tract velocity time integral (LVOT-VTI) is a representative non-invasive parameter for evaluating stroke volume, which can be a determinant of adverse outcomes in hospitalized patients with heart failure. However, the prognostic implication of admission LVOT-VTI for hospitalized HFpEF patients is undetermined. Purpose The aim of the present study was to investigate whether admission LVOT-VTI could predict poor clinical outcomes in hospitalized patients with HFpEF. Methods We examined consecutive 535 hospitalized HFpEF patients (left ventricular ejection fraction ≥50%) due to acute decompensated heart failure from the JASPER (JApanese heart failure Syndrome with Preserved Ejection fRaction) multicenter registry, obtained between November 2012 and March 2015. Patients without accessible LVOT-VTI data on admission were excluded. Finally, 214 patients were examined. The primary outcome of interest was composite of all-cause death and rehospitalization due to heart failure. Results Mean age was 78±11 years, 100 were male, and median plasma brain-type natriuretic peptide level was 400 (interquartile range [IQR] 223–711) pg/ml. During a median follow-up period of 688 (IQR 162–810) days, adverse events occurred in 83 patients (39%), including 47 (22%) all-cause death, 51 (24%) rehospitalization due to heart failure. The c-index of LVOT-VTI for predicting the composite of adverse events was 0.59 (95% CI 0.51 to 0.67), and the optimal cut-off value of LVOT-VTI was 15.8 cm. Low LVOT-VTI (≤15.8 cm) was significantly associated with higher adverse events compared to high LVOT-VTI (>15.8 cm) (Figure). Multivariable Cox regression analysis revealed that lower LVOT-VTI was an independent determinant of adverse events (HR 0.94, 95% CI 0.91 to 0.98, P=0.005) even after adjustment for pre-specified confounders including age, sex, systolic blood pressure, serum sodium, albumin, plasma brain-type natriuretic peptide and renal function. Figure 1 Conclusions Lower admission LVOT-VTI was an independent determinant of worse clinical outcomes in hospitalized HFpEF patients, indicating that LVOT-VTI on admission could be a useful marker for risk stratification in these patients.

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