scholarly journals Interdisciplinary approach to surgical treatment for patients with hypertrophic obstructive cardiomyopathy

2021 ◽  
Vol 23 (4) ◽  
pp. 492-502
Author(s):  
K. V. Rudenko ◽  
L. O. Nevmerzhytska ◽  
M. V. Kozhanov ◽  
M. О. Tregubova ◽  
P. А. Danchenko

Hypertrophic cardiomyopathy (HCM) – is the most common genetically determined heart disease, characterized by symmetrical or asymmetrical myocardial thickening (≥15 mm), leading to progressive heart failure and a high risk of sudden cardiac death (SCD). Diagnosis, natural course and treatment of HCM have become sources of uncertainty, misunderstanding and debate due to the increasing complexity of diagnosis in clinical practice, international differences in strategic approaches, advances in the treatment of genetic diseases and cardiac imaging. The aim. To amplify the current concept of HCM pathogenesis and mechanisms of the left ventricular outflow tract (LVOT) obstruction as well as to highlight our own results of HCM surgical correction. Materials and methods. A total of 330 HCM patients underwent surgical correction (septal myectomy) in the National M. Amosov Institute of Cardiovascular Surgery affiliated to National Academy of Medical Sciences of Ukraine. The mean age of the patients was 49.7 ± 15.0 years, median – 53; 168 (50.9 %) were male. Results. Surgical correction of HCM significantly improves the quality of life (80 % of patients were in NYHA II functional class after correction), reduces the symptoms of heart failure (93 % of patients had minimal mitral regurgitation after septal myectomy), decreases LVOT systolic pressure gradient (SPG) (the mean SPG was 18.9 ± 8.5 mmHg versus preoperative 93.6 ± 23.2 mmHg) and lowers the high-risk for SCD in the patients (the mean percentage of high-risk group was 3.78 % after correction). Routine CT planning and intraoperative transesophageal echocardiography helps to avoid severe iatrogenic complications and resect the interventricular septum as accurately as possible, completely eliminating LVOT obstruction. Conclusions. Secondary abnormal chordal attachment cutting, papillary muscle realignment and anterior mitral leaflet plication along with septal myectomy allows to fully correct all phenotype of HCM, is the safe and effective procedure as well as the definitive method of this pathology treatment minimizing the risk of disease recurrence.

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
J P Sousa ◽  
J Ribeiro ◽  
L Puga ◽  
C Lourenco ◽  
R Teixeira ◽  
...  

Abstract A 69-year-old man with history of non-insulin-treated type 2 diabetes mellitus, arterial hypertension and mixed dyslipidemia presented to the emergency department with chest pain lasting for four days. Immediate twelve-lead electrocardiogram unveiled an inferior ST-segment elevation myocardial infarction (STEMI), prompting emergent coronary angiography, which, in turn, revealed two-vessel disease, specifically proximal ramus intermedius 60-70% stenosis and proximal right coronary artery acute occlusion. Culprit lesion was successfully managed with balloon angioplasty and a single drug-eluting stent implantation. Still, clinical course was noticeable for deterioration, under the form of cardiogenic shock, which required invasive ventilation and intravenous vasopressor support with norepinephrine. Despite biventricular systolic function relative preservation, transthoracic echocardiography disclosed inferior akinesis, right ventricle dilation, mild circumferential pericardial effusion and, particularly, a 2.3cm posteroinferior ventricular septal defect (VSD), in the setting of a 4.4cm2 pseudoaneurism, resulting in left-to-right shunting, quantified through maximal/mean trans-VSD pressure gradients of 84/44mmHg. Further imaging with transesophageal echocardiography and cardiac computed tomography angiography allowing the conception of a 3D-printed model was performed. Surgical correction of the defect followed, achieving partial anatomic success, namely with residual shunting, as of a left ventricular systolic pressure of 80mmHg and a right ventricular systolic pressure of 25mmHg. Patient survived, recovered and got discharged three weeks later. At one-year follow-up, he was hospitalized for acute decompensated heart failure (hemodynamic profile C) twice, with medication non-adherence reported as the main precipitating factor. In addition to a significant remaining left-to-right shunt (maximal velocity 3m/s), adverse cardiac remodeling was recognized, featuring left ventricular ejection fraction of 30-35%, severe functional mitral regurgitation, severe postcapillary pulmonary hypertension and de novo left bundle branch block (QRS duration of 197ms). Having been deemed clinically unsuitable for another surgical correction, patient underwent percutaneous VSD closure with both AmplatzerTM septal and muscular VSD occluders, with a suboptimal result. He is now on New York Heart Association class III heart failure and on the waiting list for both MitraClip and cardiac resynchronization therapy implantation. Reflecting numerous breakthroughs in the management of acute myocardial infarction, incidence of mechanical complications is on the decline. Nevertheless, when they occur, morbidity and mortality remain high. Acquired ventricular septal defects are no exception, demanding the best care from a tertiary hospital heart team. Abstract P714 Figure.


Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 3197-3197
Author(s):  
Vitrano Angela ◽  
Rita Barone ◽  
Gaetano Restivo Pantalone ◽  
Paolo Rigano ◽  
Marcello Capra ◽  
...  

Abstract Abstract 3197 Background: The prognosis for thalassemia major (TM) has dramatically improved in the last two decades. However, many transfusion-dependent patients continue to develop secondary iron overloading, and eventually death, particularly from cardiac disease. The possibility of detecting easily and earliest the patients at risk of cardiac death is so far the main challenge of clinical management of these patients. Therefore, the mean reduction of Left Ventricular Ejection Fraction (LVEF), determined by echocardiography, was evaluated over the time. Methods: Among the 413 observed patients only 188 had complete records for LVEF measurements during, at least, five considered consecutive years. Included patients were divided into two cohorts: the not alive and the alive-group with 22 and 166 patients, respectively. Generalized Estimating Equations (GEE) model was used to show the reduction of the mean of LVEF (Hedeker & Gibbons, 2006). This approach was implemented in the 'xtgee' procedure of Stata 11 software (StataCorp, College Station, TX, USA). The logistic regression model was used to evaluate the risk of death (Collet D. 2003). In this analysis, the mean reduction of LVEF was categorized into three levels: the baseline category including all patients with an increase greater than 0%, the category 1 including all patients with a reduction greater than 0% but less than 7% and the category 2 including all patients with a reduction higher or equal to 7%. All of the statistical analyses were performed under code at the Department for Mathematical and Statistical Sciences 'S. Vianelli', University of Palermo (Italy) by A.V. Results: Baseline findings are shown on Table I. Figure 1 shows the proÞles of the GEE model for the mean LVEF between the two groups. The regression coefficient of Status×Time shows a statistically significant linear decrease over the time of 1,51 per year of the mean LVEF between not alive versus alive patients (Coeff. −1.51, CI (−2,31;−0,71), p-value<0,0001,Fig. 1). Patients with a mean reduction of LVEF greater or equal to 7% over the time had a statistical significant higher risk of death from heart failure (OR= 4,93,95% CI 1,61;15,11, p-value = 0,005). Discussion: Recently, Kirk et al. 2009 suggested as cardiac T2* magnetic resonance is able to detect patients at high risk of heart failure and arrhythmia from myocardial siderosis. However, other studies showed the presence of patients with abnormal heart function and normal heart T2* and did not suggest lower heart T2* for patients suffering from arrhythmia (Pepe et al., 2006; Marsella et al.,2011). Moreover, although the use of T2* is spreading, its availability is so far limited. Instead, availability of echocardiography is surely greater. Moreover, interobserver and intraobserver reliability for the visual assessment of the global LVEF measurements have been extensively shown even in comparison with Magnetic Resonace Imaging (Hoffmann et al. 2005; Gimelli et al. 2008; Blondheim et al., 2008; Sjzli et al. 2011). Therefore, repeated measurements of LVEF may be a strong and more accessible tool for detecting at risk of heart failure TM population. Disclosures: No relevant conflicts of interest to declare.


2010 ◽  
Vol 13 (1) ◽  
pp. 31 ◽  
Author(s):  
Federico Benetti ◽  
Ernesto Pe�herrera ◽  
Teodoro Maldonado ◽  
Yan Duarte Vera ◽  
Valvanur Subramanian ◽  
...  

Background: End-stage heart failure (HF) is refractory to current standard medical therapy, and the number of donor hearts is insufficient to meet the demand for transplantation. Recent studies suggest autologous stem cell therapy may regenerate cardiomyocytes, stimulate neovascularization, and improve cardiac function and clinical status. Although human fetal-derived stem cells (HFDSCs) have been studied for the treatment of a variety of conditions, no clinical studies have been reported to date on their use in treating HF. We sought to determine the efficacy and safety of HFDSC treatment in HF patients.Methods and Results: Direct myocardial transplantation of HFDSCs by open-chest surgical procedure was performed in 10 patients with HF due to nonischemic, nonchagasic dilated cardiomyopathy. Before and after the procedure, and with no changes in their preoperative doses of medications (digoxin, furosemide, spironolactone, angiotensin-converting enzyme inhibitors, angiotensin receptor blockers, betablockers), patients were assessed for New York Heart Association (NYHA) class, performance in the exercise tolerance test (ETT), ejection fraction (EF), left ventricular end-diastolic dimension (LVEDD) via transthoracic echocardiography, performance in the 6-minute walk test, and performance in the Minnesota congestive HF test. All 10 patients survived the operation. One patient had a stroke 3 days after the procedure, and although she later recovered, she was unable to perform the follow-up tests. Another male patient experienced pericardial effusion 3 weeks after the procedure. Although it resolved spontaneously, the patient abandoned his control tests and died 5 months after the procedure. An autopsy of the myocardium suggested that new young cells were present in the cardiomyocyte mix. At 40 months, the mean (SD) NYHA class decreased from 3.4 0.5 to 1.33 0.5 (P = .001); the mean EF increased 31%, from 26.6% 4% to 34.8% 7.2% (P = .005); and the mean ETT increased 291.3%, from 4.25 minutes to 16.63 minutes (128.9% increase in metabolic equivalents, from 2.46 to 5.63) (P < .0001); the mean LVEDD decreased 15%, from 6.85 0.6 cm to 5.80 0.58 cm (P < .001); mean performance in the 6-minute walk test increased by 43.2%, from 251 113.1 seconds to 360 0 seconds (P = .01); the mean distance increased 64.4%, from 284.4 144.9 m to 468.2 89.8 m (P = .004); and the mean result in the Minnesota test decreased from 71 27.3 to 6 5.9 (P < .001).Conclusion: Although these initial findings suggest direct myocardial implantation of HFDSCs is feasible and improves cardiac function in HF patients at 40 months, more clinical research is required to confirm these observations.


2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
M Barki ◽  
M Losito ◽  
M.M Caracciolo ◽  
F Bandera ◽  
M Rovida ◽  
...  

Abstract Background The right ventricle (RV) is extremely sensitive to hemodynamic changes and increased impedance. In acute heart failure (AHF), the development of pulmonary venous congestion and the increase of left ventricular (LV) filling pressures favors pulmonary vascular adverse remodeling and ultimately RV dysfunction, leading to the onset of symptoms and to a further decay of cardiac dynamics. Purpose The aim of the study was to evaluate RV morphology and functional dynamics at admission and discharge in patients hospitalized for AHF, analyzing the role and the response to treatment of the RV and its coupling with pulmonary circulation (PC). Methods Eighty-one AHF patients (mean age 75.75±10.6 years, 59% males) were prospectively enrolled within 24–48 hours from admission to the emergency department (ED). In either the acute phase and at pre-discharge all patients underwent M-Mode, 2-Dimensional and Doppler transthoracic echocardiography (TTE), as well as lung ultrasonography (LUS), to detect an increase of extravascular lung water (EVLW) and development of pleural effusion. Laboratory tests were performed in the acute phase and at pre-discharge including the evaluation of NT-proBNP. Results At baseline we observed a high prevalence of RV dysfunction as documented by a reduced RV systolic longitudinal function [mean tricuspid annular plane systolic excursion (TAPSE) at admission of 16.47±3.86 mm with 50% of the patients exhibiting a TAPSE&lt;16mm], a decreased DTI-derived tricuspid lateral annular systolic velocity (50% of the subjects showed a tricuspid s' wave&lt;10 cm/s) and a reduced RV fractional area change (mean FAC at admission of 36.4±14.6%). Furthermore, an increased pulmonary arterial systolic pressure (PASP) and a severe impairment in terms of RV coupling to PC was detected at initial evaluation (mean PASP at admission: 38.8±10.8 mmHg; average TAPSE/PASP at admission: 0.45±0.17 mm/mmHg). At pre-discharge a significant increment of TAPSE (16.47±3.86 mm vs. 17.45±3.88; p=0.05) and a reduction of PASP (38.8±10.8 mmHg vs. 30.5±9.6mmHg, p&lt;0.001) was observed. Furthermore, in the whole population we assisted to a significant improvement in terms of RV function and its coupling with PC as demonstrated by the significant increase of TAPSE/PASP ratio (TAPSE/PASP: 0.45±0.17 mm/mmHg vs 0.62±0.20 mm/mmHg; p&lt;0.001). Patients significantly reduced from admission to discharge the number of B-lines and NT-proBNP (B-lines: 22.2±17.1 vs. 6.5±5 p&lt;0.001; NT-proBNP: 8738±948 ng/l vs 4227±659 ng/l p&lt;0.001) (Figure 1). Nonetheless, no significant changes of left atrial and left ventricular dimensions and function were noted. Conclusions In AHF, development of congestion and EVLW significantly impact on the right heart function. Decongestion therapy is effective for restoring acute reversal of RV dysfunction, but the question remains on how to impact on the biological properties of the RV. Funding Acknowledgement Type of funding source: None


2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Jingtao Na ◽  
Haifeng Jin ◽  
Xin Wang ◽  
Kan Huang ◽  
Shuang Sun ◽  
...  

Abstract Background Heart failure (HF) is a clinical syndrome characterized by left ventricular dysfunction or elevated intracardiac pressures. Research supports that microRNAs (miRs) participate in HF by regulating  targeted genes. Hence, the current study set out to study the role of HDAC3-medaited miR-18a in HF by targeting ADRB3. Methods Firstly, HF mouse models were established by ligation of the left coronary artery at the lower edge of the left atrial appendage, and HF cell models were generated in the cardiomyocytes, followed by ectopic expression and silencing experiments. Numerous parameters including left ventricular posterior wall dimension (LVPWD), interventricular septal dimension (IVSD), left ventricular end diastolic diameter (LVEDD), left ventricular end systolic diameter (LVESD), left ventricular ejection fraction (LVEF), left ventricular fractional shortening (LVFS), left ventricular systolic pressure (LVSP), left ventricular end diastolic pressure (LEVDP), heart rate (HR), left ventricular pressure rise rate (+ dp/dt) and left ventricular pressure drop rate (-dp/dt) were measured in the mice. In addition, apoptosis in the mice was detected by means of TUNEL staining, while RT-qPCR and Western blot analysis were performed to detect miR-18a, HDAC3, ADRB3, cMyb, MMP-9, Collagen 1 and TGF-β1 expression patterns. Dual luciferase reporter assay validated the targeting relationship between ADRB3 and miR-18a. Cardiomyocyte apoptosis was determined by means of flow cytometry. Results HDAC3 and ADRB3 were up-regulated and miR-18a was down-regulated in HF mice and cardiomyocytes. In addition, HDAC3 could reduce the miR-18a expression, and ADRB3 was negatively-targeted by miR-18a. After down-regulation of HDAC3 or ADRB3 or over-expression of miR-18a, IVSD, LVEDD, LVESD and LEVDP were found to be decreased but LVPWD, LVEF, LVFS, LVSP, + dp/dt, and −dp/dt were all increased in the HF mice, whereas fibrosis, hypertrophy and apoptosis of HF cardiomyocytes were declined. Conclusion Collectively, our findings indicate that HDAC3 silencing confers protection against HF by inhibiting miR-18a-targeted ADRB3.


2015 ◽  
Vol 23 (4) ◽  
pp. 578-586 ◽  
Author(s):  
Ana Paula da Conceição ◽  
Mariana Alvina dos Santos ◽  
Bernardo dos Santos ◽  
Diná de Almeida Lopes Monteiro da Cruz

AbstractObjective: to describe self-care behavior and its associated factors in a sample of heart failure Brazilian patients.Method: descriptive cross-sectional study with non-probabilistic sample of 116 ambulatory patients undergoing heart failure treatment. Self-care was evaluated using the Self-Care of Heart Failure Index, (scores ≥70 points=appropriate self-care). Association tests were applied, considering a descriptive level of 0.05.Results: the mean age of participants was 57.7 (SD =11.3) years; 54.3% were male; the mean schooling was 5.5 (SD = 4.0) years; and 74.1% had functional class II-III. The mean scores on the subscales of the Self-Care of Heart Failure Index indicated inappropriate self-care (self-care maintenance: 53.2 (SD =14.3), selfcare management: 50.0 (SD = 20.3) and self-care confidence: 52.6 (SD=22.7)) and it was found low frequencies of participants with appropriate self-care (self-care maintenance, 6.9%), self-care management (14.7%) and self-care confidence (19%). Higher scores of the Self-Care of Heart Failure Index were associated with: reduced left ventricular ejection fraction (p=0.001), longer time of experience with the disease (p=0.05) and joint monitoring by physician and nurse (p=0.007).Conclusion: investments are needed to improve the self-care behavior and the nursing can play a relevant role in this improvement.


1989 ◽  
Vol 14 (1) ◽  
pp. 127-134 ◽  
Author(s):  
Constantine N. Aroney ◽  
Howard C. Herrmann ◽  
Marc J. Semigran ◽  
G. William ◽  
Charles A. Boucher ◽  
...  

2017 ◽  
Vol 121 (suppl_1) ◽  
Author(s):  
Adebayo C Atanda ◽  
Yahya Aliyu ◽  
Oluwafunmilayo Atanda ◽  
Aliyu Babadoko ◽  
Aisha Suleiman ◽  
...  

Introduction: Anemia has been implicated in heart failure. Existing literatures, involving predominantly African-Americans, suggests that Sickle Cell Disease (SCD) maybe linked to various cardiovascular complications including pulmonary hypertension and left venticular dysfunction. Peculiarly, our study involves exclusively Sub-Saharan population. Method: We conducted a cross sectional observational study of 208 hydroxyurea-naive consecutive SCD patients aged 10-52 years at steady state and 94 healthy non-matched controls who were studied in an out patient clinic in Sub-Saharan Africa. SCD patients were required to have electrophoretic or liquid chromatography documentation of major sickling phenotypes. Control group was required to have non-sickling phenotypes. Cardiac measurements were performed with TransThoracic Echo according to American Society of Echocardiography guidelines. Hemoglobin level was also obtained. Results: Hemoglobin level in SCD group (8.5+/- 1.5) was significant (P<0.001) compared to control (13.8+/- 1.7). Although SCD group had significantly higher values of left ventricular (LV) size, there was no qualitative evidence of LV dysfunction. SCD group had higher values of Ejection Fraction but not statistically significant. There was no evidence of LV wall stiffening to impair proper filling in SCD group, with the ratio of early to late ventricular filling velocities, E/A ratio elevated (1.7+/-0.4 compared to 1.6+/- 0.4; P=0.010). Right ventricular systolic pressure was determined using the formula of 4x Tricuspid Reugurgitant jet (TRV) square as an indirect measurement of Pulmonary arterial systolic pressure. SCD patients had significantly higher mean±SD values for tricuspid regurgitant jet velocity than did the controls (2.1±0.6 vs. 1.8±0.5; p= 0.001). Within the SCD group, there was no clear pattern of worsening diastolic function with increased TRV. Furthermore, E/A had a significant positive relationship with jet velocity in bivariate analysis (R=0.20; P=0.013). Conclusions: We were unable to demonstrate existence of anemia-associated left ventricular dysfunction in Sub-Saharan African with SCD. Further studies is required to highlight the reason behind this finding.


Circulation ◽  
2015 ◽  
Vol 132 (suppl_3) ◽  
Author(s):  
Roderick C Deaño ◽  
Jackie Szymonifka ◽  
Qing Zhou ◽  
Jigar H Contractor ◽  
Zachary Lavender ◽  
...  

Objective: Patients with heart failure (HF) and pulmonary hypertension (PH) have worse outcomes after cardiac resynchronization therapy (CRT). The relationship of circulating HF biomarkers and right ventricular systolic pressure (RVSP) may provide insight to the mechanism between PH and poor CRT response. Methods: In 90 patients (age 65 ± 13, 78% male, EF 26 ± 8%, RVSP 44 ± 12 mmHg) undergoing CRT, we measured baseline RVSP by echocardiography and obtained peripheral blood samples drawn at the time of device implantation. We measured levels of established and emerging HF biomarkers (Table 1). CRT non-response was defined as no improvement of adjudicated HF Clinical Composite Score at 6 months. Major adverse cardiac event (MACE) was defined as composite endpoint of death, cardiac transplant, left ventricular assist device, and HF hospitalization within 2 years. Results: There were 34% CRT non-responders and 27% had MACE. Per 1 unit increase in log-transformed RVSP, there was an 11-fold increase risk of having CRT non-response (odd ratio [OR] 11.0, p=0.01) and over 5-fold increase of developing 2-year MACE (hazard ratio [HR] 5.8, p=0.02). When comparing patients with severe PH (RVSP>60 mmHg) to those without PH (RVSP < 35 mmHg), there was an 8-fold increase in CRT nonresponse (OR 8.4, p=0.03) but no difference in MACE (p=NS). RVSP was correlated with increased biomarker levels of myocardial stretch and fibrosis, but not myocardial necrosis (Table 1). Conclusions: Higher RVSP is associated with greater rates of CRT non-response and adverse clinical outcomes. The mechanistic association between severe PH and CRT nonresponse may be explained by the biomarker profile reflective of myocardial wall stretch and fibrosis.


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