Ventricular Systolic Dysfunction Research Articles

Right ventricular dysfunction is a predictor of non-response to cardiac resynchronization therapy

Abstract Introduction Cardiac resynchronization therapy (CRT) has been of great benefit to many heart failure (HF) patients with reduced ejection fraction (EF) and intraventricular conduction delay. However, approximately 30% of patients fail to respond to CRT. We investigated baseline characteristics that might influence response to CRT. Methods We retrospectively enrolled 227 patients undergoing CRT implantation between 2013 and 2020 according to the guidelines. 118 patients were included in our analysis, from whom all data were available. Clinical, electrocardiographic and echocardiographic parameters were evaluated at baseline and 6 months after CRT implantation. Response to CRT was defined as an increase in left ventricular ejection fraction (LVEF) >10%. Right ventricular systolic dysfunction (RVSD) was defined as S' velocity <9.5 cm/s or tricuspid anular plane systolic excursion (TAPSE) <17 mm. Chronic kidney disease (CKD) was defined as GFR <60 ml/min/1.73m2. Results 118 patients were included (mean age 69±11 years, 66.1% males, 39.8% ischemic etiology; 35,6% atrial fibrillation, baseline LVEF 27,6±6%). After 6 months of CRT, 65 patients (55.1%) were considered responders. Responders were more frequently female than non responders (43,1% vs 22,6, p=0.02). Atrial fibrillation and CKD were more prevalent in non responders (47,2% vs 26,2%, p=0.018; 62,3% vs 21,5%, p<0.001, respectively). RVSD was present in 60,4% of non responders vs 16,9% of responders (p<0.001). In responder group, the mean S' velocity was 10,9±2,1 cm/s vs 9,1±2,1 cm/s in non responder group, p<0.001. The mean TAPSE was also higher in responder group (20,3±7,2 mm vs 16,5±4,4 mm, p=0.031). On multivariate analysis only RVSD (OR 7,754; 95% CI 2,968 – 20,282 p<0.001] and CKD (OR 5,434; 95% CI 2,109 – 14,002; p<0.001) were independently associated with non-response to CRT. Conclusion From a range of preoperative characteristics, multivariate analysis only identified RVSD and CKD as independent predictors of CRT response, with S' <9,5 cm/s and TAPSE <17 mm associated with non-response to CRT. This study highlights the importance of routine RV assessment in order to improve patient selection and optimize CRT response in heat failure patients. Funding Acknowledgement Type of funding sources: None.

The relationship of dehydration and body mass index with the occurrence of atrial fibrillation in heart failure patients

Abstract Background/Introduction Heart failure (HF) and atrial fibrillation (AF) are two conditions that are likely to dominate the next years of cardiovascular (CV) care. These diseases frequently coexist and they can beget one another due to similar risk factors and similar pathophysiology. Purpose The aim of the study was to assess the relationship of dehydration, body mass index (BMI) and other indices with the occurrence of AF in HF patients. Methods The study included 113 patients [median age 64 years; 57.52% male] hospitalized due to HF. Baseline demographics, body mass analysis, echocardiographic results, key cardiopulmonary exercise test (CPET) parameters, six minute walk distance (6MWD) and Kansas City Cardiomyopathy Questionnaire (KCCQ) score were assessed. Results Of all patients, 23 (20.35%) had AF, and 90 (79.65%) had sinus rhythm (SR). Patients with AF were older (med. 66 vs 64 years; p=0.039), with higher BMI (32.02 vs. 28.51 kg/m2; p=0.017) and percentage of fat content (37.0 vs. 27.9%, p=0.014). They were more dehydrated, with a lower percentage of total body water (TBW%) (45.7 vs 50.0%; p=0.022). Clinically, patients with AF had more often higher New York Heart Association (NYHA) class (III vs II; p<0.001), shorter 6MWD (median 292.35 vs 378.4 m; p=0.001) and a lower KCCQ overall summary score (52.60 vs 73.96 points; p=0.002). Patients with AF had significantly lower exercise capacity as measured by peak oxygen consumption (peak VO2) (0.92 vs 1.26 mL/min, p=0.016), peak VO2/kg (11 vs. 15 mL/kg/min; p<0.001), and percentage of predicted VO2max (pp-peak VO2) (62.5 vs 70.0; p=0.010). We also found VE/VCO2 (med.33.85 vs 32.20; p=0.049) to be higher and peak oxygen pulse (8.5 vs 11 mL/beat; p=0.038) to be lower in patients with AF than in patients without AF. In a multiple logistic regression model higher BMI (OR 1.23 per unit increase, p<0.001) and higher left atrial volume index (LAVI) (OR 1.07 per unit increase, p=0.03), lower tricuspid annular plane systolic excursion (TAPSE) (OR 0.74 per unit increase, p=0.03) and lower TBW% in body mass analysis (OR 0.90 per unit increase, p=0.03) were independently related to AF in patients with HF. Conclusions Increased volume of left atrium and right ventricular systolic dysfunction are well-known predictors of AF occurrence in patients with HF, but hydration status and increased body mass also seem to be important factors of AF in HF patients. Funding Acknowledgement Type of funding sources: None. Figure 1

Impact of parity on cardiac structure and function in apparently healthy pregnant women in Nigeria

Abstract Background There are a few and conflicting data in the literature about parity and maternal cardiac function. Purpose The purpose of this study was to assess the impact of parity on cardiac structure and function in apparently healthy pregnant women in Nigeria. Methods This was a cross-sectional study carried out in 4 tertiary centers in Kano, and 1 in Ile- Ife, Nigeria. 112 apparently healthy pregnant women were consecutively recruited between 28thand 38th weeks of gestation. Left ventricular (LV)systolic dysfunction was defined as LV ejection fraction of below 50%, and diastolic dysfunction was graded using mitral filling pattern and tissue velocities. Results LV systolic dysfunction and diastolic dysfunction were found in 6 (5.4%) subjects and 20 (17.9%) subjects respectively. Age (p= <0.0001), left atrial (LA)size (P= <0.0001), interventricular septal thickness at end diastole (IVSD) (p=0.005) and posterior wall thickness at end diastole (PWTD) (p=0.004) increased progressively with parity (Table 1). There was significant positive correlation between parity and age (r=0.475, p= <0.0001), LA size (r=0.332, p= <0.0001), IVSD (r=0.264, p=0.005) and PWD (r=0.343, p= <0.0001). LV systolic function was not significantly associated with parity. Conclusion Our findings suggests that parity causes significant myocardial remodeling in apparently healthy pregnant women that should be taken in to consideration while interpreting their echocardiograms. Funding Acknowledgement Type of funding sources: None.

Mechanoenergetic coupling in heart failure with preserved, mid-range and reduced left ventricular ejection fraction

Abstract Background Heart failure (HF) classification based on left ventricular ejection fraction (LVEF) can vary because of changes in filling pressures, afterload, and contractile function. 11C-acetate positron emission tomography (PET) provides a load-independent measure of myocardial external efficiency (MEE) by simultaneous assessment of myocardial oxygen consumption (MVO2), cardiac work, left ventricular mass (LVM), end-systolic wall stress (ESWS), and myocardial blood flow (MBF). Purpose We aimed to characterize mechanoenergetic derangements in patients with HF and to study its interrelation with age, sex and obesity. Methods MEE was measured in 121 participants with 11C-acetate PET, and LVEF was acquired with echocardiography. We investigated healthy controls (n=20) and patients with HF and reduced LVEF <40% (HFrEF; n=25), mid-range LVEF 40–49% (HFmrEF; n=23), as well as patients with asymptomatic aortic valve stenosis (AS) and LVEF ≥50% (AS-asymp; n=38), and symptomatic AS and LVEF ≥50% (defined as HF with preserved LVEF (HFpEF); n=15). Results MEE declined in tandem with reduced LVEF from HFpEF and HFmrEF to HFrEF (p=0.041, p<0.001, and p<0.001 versus control, respectively; Figure 1). Impaired MEE was aggravated with increasing LVM (p=0.001) due to a disproportionate increase in overall left ventricular MVO2. In a multivariate analysis, female sex (p<0.001), a lower body mass index (p<0.001), and advanced age (p=0.01) were associated with a lower MEE (Figure 2). HFpEF, HFmrEF, and HFrEF patients had distinct energetic profiles involving MEE, MVO2, MBF, ESWS, and LVM (Figure 2). Conclusions Mechanoenergetic uncoupling was evident in every clinical state within the HF syndrome and associated with left ventricular hypertrophy and progressive systolic dysfunction. Sex, age, and obesity impacted myocardial energetics. To date, the present study is the largest investigation of mechanoenergetic coupling across several categories of patients with heart failure. 11C-acetate PET extends our pathophysiological comprehension of the HF syndrome beyond LVEF. Funding Acknowledgement Type of funding sources: Foundation. Main funding source(s): The Danish Heart FoundationThe Lundbeck Foundation Relationship between LVEF and MEEMyocardial energetics in heart failure

Combined deletion of PR-domain containing 16 (Prdm16) in cardiomyocyte and non-cardiomyocyte lineages results in spontaneous early-onset lethality and progressive left ventricular dysfunction in mice

Abstract Introduction PR-domain containing 16 (Prdm16) has an asymmetric expression pattern in the developing cardiovascular system, including ventricular myocardium, endocardium and arterial endothelial and smooth muscle cell (SMC) layers. Heterozygous PRDM16 mutations in humans have been linked with early-onset cardiomyopathy resulting in heart failure. Myocardial PRDM16-deficiency has been suggested as the culprit for this cardiomyopathy, however embryonic Prdm16 deletion in cardiomyocytes or their progenitors in mice only results in symptomatic cardiac defects upon metabolic stress or ageing. This suggests that Prdm16 loss in other cell types has an important co-contribution in the early heart phenotype seen in patients with causal PRDM16 variants. Purpose To investigate the adjuvant role of non-cardiomyocytes to the heart phenotype caused by Prdm16 deficiency, we used a conditional mouse model in which deletion of Prdm16 occurs in all cells expressing an Sm22-driven Cre recombinase which has a combined activity in cardiomyocyte and non-cardiomyocyte lineages in the heart, including SMCs and pericytes. Methods Mice carrying two Prdm16 alleles with a floxed exon 9 (Prdm16fl/fl) were intercrossed with the Sm22-Cre driver line. Offspring of Sm22Cre+; Prdm16fl/fl and Sm22Cre−; Prdm16fl/fl breeding pairs was monitored for Mendelian inheritance and for signs of (progressive) cardiac dysfunction by echocardiography at 5 and 16 weeks of age. Hearts were isolated and analyzed for RNA expression levels of cardiac stress markers Atrial and Brain Natriuretic Peptide (ANP and BNP) via quantitative RT-PCR and histologically for the appearance of fibrosis through Sirius red-staining. Results Genotyping at 5 weeks of age showed a loss of 60.4% of Sm22Cre+; Prdm16fl/fl offspring. Mice surviving at 5 weeks spontaneously developed signs of left ventricular diastolic and systolic dysfunction, the latter shown by a significantly reduced ejection fraction (EF; 37±3% vs. 61±3% in control Sm22Cre−; Prdm16fl/fl littermates). Cardiac expression levels of ANP and BNP were significantly increased (728-fold and 36-fold, respectively) in Sm22Cre+; Prdm16fl/fl mice which also showed perivascular fibrosis compared to control littermates. At 16 weeks of age, this aberrant cardiac phenotype further progressed (EF: 32±3% vs. 57±4%; ANP: 2,541-fold increase; BNP: 129-fold increase) and in addition to perivascular fibrosis, hearts also showed interstitial fibrosis (Sirius red+ area: 17±2% vs. 3.0±0.4% in control littermates). Conclusion Unlike recently reported mice with a Prdm16 deficiency in cardiomyocytes or their (precursor) lineages, mice with a combined loss of Prdm16 in the cardiomyocytes and certain non-cardiomyocyte lineages feature early mortality and (progressive) signs of severe heart failure. Therefore, Prdm16 expressed by non-cardiomyocytes is indispensable for proper cardiac function and its loss in these cell types co-determines the aberrant cardiac phenotype. Funding Acknowledgement Type of funding sources: Public grant(s) – National budget only. Main funding source(s): Fonds voor Wetenschappelijk Onderzoek Strategic Basic Research pre-doctoral fellowship (1S25817N)KU Leuven Research Coordination grant (C14/19/095)

Factors associated with the risk of heart failure progression in patients with non-compaction cardiomyopathy

Abstract Purpose To identify factors associated with the progression of chronic heart failure (CHF) to NYHA FC III, requiring hospitalization and to reveal high-risk pts. Methods The study included 156 pts with non-compaction cardiomyopathy (NCCM), who were prospectively observed in the RSPC “Cardiology”. The median follow-up was 36 months (6; 152). All pts underwent echocardiography (Echo), cardiac magnetic resonance (CMR) imaging with late gadolinium enhancement (LGE) and 24-hour Holter monitoring. The median age of the pts was 36 (26; 49) years, men predominated (57.4%). The diagnosis of NCCM was established on the basis of the following criteria: Echo – Jenni criteria, CMR – Petersen/Jaquier criteria. The end point of the study included progression of CHF from NYHA FC II to FC III, requiring hospitalization. Results At the initial examination, symptoms of CHF NYHA FC I were found in 56 (55.4%) pts, symptoms of CHF NYHA FC II – in 45 (44.6%) pts, systolic dysfunction was detected in 43% of pts. During the follow-up period, out of 156 pts, 55 (35.3%) had CHF symptoms that progressed to NYHA FC III and required hospitalization. As a result of the bivariate analysis, the following Echo characteristics were independent factors associated with a high risk of progression of CHF to NYHA FC III: LV EF <40% (HR 6.2; 95% CI 3.4–11.2); end-diastolic LV diameter >62 mm (HR 5.3; 95% CI 2.9–9.7); end-systolic LV diameter >56 mm (HR 6.6; 95% CI 3.8–11.7); end-diastolic LV volume >187 ml (HR 4.4; 95% CI 2.5–8); end-systolic LV volume >93 ml (HR 6.5; 95% CI 3.2–13). The risk of progression of CHF to NYHA FC III was 3.4 times higher in pts with right ventricular (RV) systolic dysfunction: with a decrease of RV fractional area change (FAC) <31% (HR 3.4; 95% CI 1.8–6.3) and 2.5 times higher with a decrease of tricuspid annular plane systolic excursion (TAPSE) (HR 2.5; 95% CI 1.3–4.5); 2.5 times higher in pts with initially increased anteroposterior RV diameter >28 (HR 2.5; 95% CI 1,4–4.5). According to the LGE-CMR data, the factors associated with the risk of progression of CHF to NYHA FC III were the following characteristics: an increase of BSA-indexed end-diastolic LV volume >145 ml/m2 (HR 6.7; 95% CI 3.4–13.3); BSA-indexed end-systolic LV volume >96 ml/m2 (HR 6.8; 95% CI 3.4–13.6); and an increase of RV end-systolic volume >145 ml (HR 5.0; 95% CI 2.3–11). The risk of CHF progression was 4.8 times higher in pts with baseline LV systolic dysfunction (LV EF <34%, HR 4.8; 95% CI 2.8–8.2); 2.7 times higher in pts with RV systolic dysfunction (LVEF <42%, HR 2.7; 95% CI 1.6–4.8). The NC/C ratio and the presence of myocardial replacement fibrosis according to LGE-CMR did not significantly affect the risk of progression of CHF. Conclusion The factors identified as a result of the study associated with the risk of progression of CHF based on biventricular assessment by cardiac imaging methods (Echo and LGE-CMR) can be used to reveal high-risk pts. Funding Acknowledgement Type of funding sources: None.

Cardiac involvement in hospitalized patients with COVID-19 and its incremental value in outcomes prediction

Recent reports linked acute COVID-19 infection in hospitalized patients to cardiac abnormalities. Studies have not evaluated presence of abnormal cardiac structure and function before scanning in setting of COVD-19 infection. We sought to examine cardiac abnormalities in consecutive group of patients with acute COVID-19 infection according to the presence or absence of cardiac disease based on review of health records and cardiovascular imaging studies. We looked at independent contribution of imaging findings to clinical outcomes. After excluding patients with previous left ventricular (LV) systolic dysfunction (global and/or segmental), 724 patients were included. Machine learning identified predictors of in-hospital mortality and in-hospital mortality + ECMO. In patients without previous cardiovascular disease, LV EF < 50% occurred in 3.4%, abnormal LV global longitudinal strain (< 16%) in 24%, and diastolic dysfunction in 20%. Right ventricular systolic dysfunction (RV free wall strain < 20%) was noted in 18%. Moderate and large pericardial effusion were uncommon with an incidence of 0.4% for each category. Forty patients received ECMO support, and 79 died (10.9%). A stepwise increase in AUC was observed with addition of vital signs and laboratory measurements to baseline clinical characteristics, and a further significant increase (AUC 0.91) was observed when echocardiographic measurements were added. The performance of an optimized prediction model was similar to the model including baseline characteristics + vital signs and laboratory results + echocardiographic measurements.

Surgical approach for anomalous aortic origin of a coronary artery: A comparison of two techniques.

Un-roofing is the most common technique utilized for repair of anomalous aortic origin of a coronary artery (AAOCA). There are very few publications directly comparing un-roofing to another surgical technique, like reimplantation. The prospectively collected Children's Memorial Hermann Heart Institute Society of Thoracic Surgeon's Database was retrospectively reviewed from 2007 to 2021. Surgical patients were included if they underwent un-roofing or reimplantation of the AAOCA. The primary outcomes of this study were operative characteristics and postoperative outcomes. Secondary outcomes included angiographic outcomes, aortic regurgitation incidence, ventricular function, and symptom relief. From 2007 to 2021, there were 12 patients who underwent either a reimplantation (n = 9, 73%) or un-roofing (n = 3, 27%) for an AAOCA. The hospital length of stay was a median of 1.8 days longer for reimplantation compared to un-roofing. The last follow-up echocardiogram was a median of 52.2 days later in the reimplantation group. There was one patient (11%) in the reimplantation group that had more than or equal to mild aortic regurgitation and mild systolic ventricular dysfunction. Outpatient follow-up was incomplete and there was no postoperative computed tomographic angiography in the un-roofing cohort. Coronary artery reimplantation is a valuable alternative surgical technique to un-roofing for the repair of AAOCA. There are still some concerns with the creation of aortic regurgitation or incomplete symptom relief with any surgical technique. Longer-term follow-up and prospective studies will be needed to show an effective reduction of myocardial ischemia and risk of sudden cardiac death.

English

BACKGROUND Diabetes mellitus (DM) single-handedly accounts for 75 – 90 % of excess coronary artery disease (CAD) risk seen in persons suffering from it and it also enhances and amplifies the effects of other traditional cardiovascular risk factors. After adjusting for concomitant risk factors such as hypertension and hyperlipidemia, there still remains an excess risk for cardiovascular disease (CVD) in people with diabetics. In this study, we wanted to evaluate left ventricular (LV) systolic and diastolic dysfunction in normotensive diabetic patients. METHODS A cross sectional comparative study was performed from January 2016 to September 2017 on 50 diabetics and 50 age and sex matched healthy controls. Adult patients of both sexes with diabetes mellitus who are normotensive were included as cases. Patients with known heart disease, chronic kidney disease, thyroid disorders were excluded from the study. 2D transthoracic echocardiogram (ECHO) with M mode was used for assessing systolic and diastolic function. RESULTS Mean ejection fraction was lower in patients group (59.76) as compared to control group (64.74) with 8 % of cases with a value of&lt; 50 %. Fractional shortening was also lower in patients (29.14) compared to controls (34.86) with 12 % patients having a value of &lt; 25 %. E/A ratio was 1.12 in patients when compared to 1.36 in controls with 32 % of patients having value &lt; 1. Mean isovolumic relaxation time was 96.52 in patients when compared to 87.42 in controls with 24 % patients having value &gt; 100 msec. CONCLUSIONS Normotensive diabetics are prone to left ventricular dysfunction. Diastolic dysfunction is more common than systolic dysfunction. KEYWORDS Diabetes Mellitus, Systolic Dysfunction, Diastolic Dysfunction, Cardiovascular Disease

Abstract 113: Targeting Receptor-interacting Protein 140 (RIP140) To Modulate Energy Metabolism In The Failing Heart

During the development of heart failure (HF), the PPAR/ERR complex becomes deactivated resulting in diminished capacity for mitochondrial fatty acid oxidation (FAO) and ATP production leading to an “energy-starved” state that contributes to progression of HF. Receptor-Interacting protein 140 (RIP140) serves as a co-repressor of PPAR/ERR in some extra-cardiac tissues. We hypothesized that inhibition of RIP140 would re-activate PPAR/ERR enhancing capacity for fuel catabolism and ATP production in the failing heart. Heart and skeletal muscle-specific RIP140 knockout mice (strRIP140KO) were resistant to the development of cardiac hypertrophy and diastolic dysfunction in response to chronic pressure overload that mimicked features of HF with preserved ejection fraction (HFpEF). To further evaluate the role of RIP140 in heart, cardiac-specific (cs) RIP140KO mice were generated. 13 C-substrate NMR studies demonstrated that palmitate oxidation and triglyceride turnover rates were significantly accelerated in isolated perfused csRIP140KO hearts. csRIP140KO were subjected to transverse aortic constriction/apical myocardial infarction surgery (TAC/MI), to produce HF with reduced EF (HFrEF). Compared to controls, csRIP140KO exhibited reduced left ventricular remodeling and systolic dysfunction when subjected to TAC/MI. RNA-sequence analysis demonstrated that many genes involved in FAO, branched-chain amino acid catabolism, oxidative phosphorylation, and adult muscle contraction programs were significantly “protected” (less downregulation) by RIP140 deletion in the context of TAC/MI. To identify candidate cardiac RIP140 targets, “CUT&amp;RUN”-sequencing was conducted on cardiomyocytes (CM) from csRIP140KO and controls to identify changes in enhancer regions. Motif analysis of peaks with increased H3K27ac deposition in the csRIP140KO CM identified ERR, PPAR, myocyte enhancer 2 (MEF2), glucocorticoid receptor (GR), and kruppel-like factor (KLF) binding sites. We conclude that RIP140 serves as a global co-repressor of a network of transcription factors that control cardiac energy metabolic and contractile function, and that inhibition of RIP140 could prove to be a novel therapeutic approach for HF.

Significance of the echocardiographic evaluation of left atrial myocardial strain for early diagnosis of heart failure with preserved ejection fraction

This systematic review is based on 19 studies from Elsevier, PubMed, Embase, and Scopus databases, which were found by the following keywords: LA strain (left atrial strain), STE (speckle tracking echocardiography), HF (heart failure), and HFpEF (heart failure with preserved ejection fraction). The review focuses on results and conclusions of studies on using the 2D echocardiographic evaluation of left atrial (LA) myocardial strain for early diagnosis of HFpEF in routine clinical practice. Analysis of the studies included into this review showed a significant decline of all LA functions in patients with HFpEF. Also, multiple studies have reported associations between decreased indexes of LA strain and old age, atrial fibrillation, left ventricular hypertrophy, left and right ventricular systolic dysfunction, and LV diastolic dysfunction. Thus, the review indicates significant possibilities of using indexes of LA strain in evaluation of early stages of both systolic and diastolic myocardial dysfunction. Notably, LA functional systolic and diastolic indexes are not sufficiently studied despite their growing significance for diagnosis and prognosis of patients with HFpEF. For this reason, in addition to existing models for risk stratification in this disease, including clinical characteristics and/or echocardiographic data, future studies should focus on these parameters.

Cardiac magnetic resonance imaging in right ventricular outflow tract arrhythmia: a retrospective analysis from a tertiary care centre in South India

Introduction Arrhythmia arising from right ventricular outflow tract (RVOT) is the most common cause of idiopathic ventricular arrhythmia. Previous studies involving cardiac magnetic resonance imaging (MRI) in these patients are known to show abnormalities in up to 60% of patients. Methodology This was a retrospective descriptive analysis of patients having idiopathic RVOT arrhythmia who underwent cardiac MRI between January 2010 and December 2020. Clinical and demographic details were recorded from the electronic medical records and cardiac MRI of all patients were reviewed. Results Among 214 patients with RVOT arrhythmia, 64 underwent cardiac MRI. A total of 41 patients, who did not have any abnormality on baseline echocardiogram were included in the study. There was male preponderance (56.1%), with median age of 43 years. About 43.9% had syncope. Twenty-four-hour Holter study revealed a premature ventricular complex (PVC) burden of 26.3 ± 11.7%. MRI showed structural abnormalities in 51.2% of patients, which commonly included RV sacculations and aneurysms. Late gadolinium enhancement (LGE) was seen in six patients, which was mostly seen in RV free wall. Right ventricular (RV) systolic dysfunction was evident in 29.3%. About 9.8% fulfilled the criteria for arrhythmogenic RV cardiomyopathy (ARVC). Out of 23 patients with baseline abnormalities on ECHO, 87% had structural abnormalities on MRI, and 43.5% fulfilled the criteria for ARVC. Conclusions Additional imaging by cardiac MRI helps to identify structural abnormalities in 51.2% of patients with RVOT arrhythmias, even with normal baseline echocardiogram and electrocardiogram (ECG). It is useful tool to rule out ARVC in this subset of patients, and can help in increasing the diagnostic yield in the early stages.

The Severity of Pectus Excavatum Defect Is Associated With Impaired Cardiopulmonary Function

BackgroundRepair of pectus excavatum has cosmetic benefits, but the physiologic impact remains controversial. The aim of this study was to characterize the relationship between the degree of pectus excavatum and cardiopulmonary dysfunction seen on cardiac magnetic resonance (CMR) imaging, cardiopulmonary exercise testing (CPET), and pulmonary function testing (PFT). MethodsA single-center analysis of CMR, CPET, and PFT was conducted. Regression models evaluated relationships between pectus indices and the clinical end points of cardiopulmonary function. ResultsData from 345 CMRs, 261 CPETs, and 281 PFTs were analyzed. Patients were a mean age of 15.2 ± 4 years, and 81% were aged <18 years. The right ventricular ejection fraction (RVEF) was <0.50 in 16% of patients, left ventricular ejection fraction (LVEF) was <0.55 in 22%, RVEF Z-score was < −2 in 32%, and the LVEF Z-score was < −2 in 18%. CPET revealed 33% of patients had reduced aerobic fitness. PFT results were abnormal in 23.1% of patients. Adjusted analyses revealed the Haller index had significant (P < .05) inverse associations with RVEF and LVEF. ConclusionsThe severity of pectus excavatum is associated with ventricular systolic dysfunction. Pectus excavatum impacts right and left ventricular systolic function and can also impact exercise tolerance. The Haller index and correction index may be the most useful predictors of impairment.

Adults With Tetralogy of Fallot: Early Postoperative Outcomes and Risk Factors for Complications

ObjectiveTo report the early postoperative outcomes in adults with tetralogy of Fallot (TOF) undergoing cardiac surgery and to identify patient factors associated with complications. Patients and MethodsWe performed a single-institution retrospective review of adults with TOF who underwent cardiac surgery from January 8, 2008, through June 21, 2018. Patients’ characteristics, preoperative imaging, surgical interventions, outcomes, and complications were analyzed. ResultsThere were 219 adults with TOF (mean age, 40 years; range, 18-83 years; 88 [40%] female) in the study. Surgical interventions included repair or replacement of the pulmonary valve (n=199 [91%]), tricuspid valve (n=70 [32%]), mitral valve (n=13 [5.9%]), and aortic valve (n=8 [3.7%]). Three patients (1.4%) underwent first-time TOF repair. The 30-day mortality rate was 1.4% (n=3). Early postoperative complications occurred in 66 (30%) and included arrhythmias requiring treatment, dialysis requirement, liver dysfunction, respiratory failure, infection, reoperation, cardiac arrest, mechanical circulatory support, and death. Multivariate analysis found older age at current surgery (odds ratio [OR], 1.04 per year; 95% CI, 1.01 to 1.06; P<.001), longer cardiopulmonary bypass time (OR, 1.01 per minute; 95% CI, 1.01 to 1.02; P<.001), right ventricular systolic dysfunction (OR, 1.31; 95%, CI 1.02 to 1.69; P=.03), diabetes mellitus (OR, 3.50; 95% CI, 1.20 to 10.2; P=.02), and history of initial palliative surgery (OR, 1.99; 95% CI, 1.01 to 3.91; P=.05) as independent predictors of complications. ConclusionSurgical interventions for adult patients with TOF can be performed with low early morbidity and mortality. Clinical characteristics and preoperative testing parameters can predict risk for complications in the postoperative period.

Interleukin-6 level is an independent predictor of right ventricular systolic dysfunction in patients hospitalized with COVID-19.

Cytokine storm with elevated levels of multiple proinflammatory cytokines and inflammatory system activation underlie the pathogenesis of coronavirus disease 2019 (COVID-19). In this study, we aimed to investigate whether increased interleukin (IL)-6 levels can predict right ventricular (RV) systolic impairment in patients hospitalized with COVID-19. This prospective, observational study included 100 consecutive patients hospitalized with mild and moderate COVID-19. All the patients underwent chest computerized tomography, detailed laboratory tests including IL-6, and two dimensional (2D) transthoracic echocardiography (TTE) with assessment of 2D conventional and Doppler echocardiography parameters and RV systolic functions. After the elimination of six patients with exclusion criteria, the remaining patients were classified into two groups, namely normal RV systolic functions (n=60) and impaired RV systolic functions (n=34). IL-6 levels were significantly higher in patients with impaired RV systolic functions than in those with normal RV systolic functions (20.3, 4.6, p<0.001, respectively). Tricuspid annular plane systolic excursion and RV derived tissue Doppler imaging (TDI) S' measurements were similar between the two groups. RV fractional area change was significantly lower, and RV TDI derived index of myocardial performance was significantly higher in patients with impaired RV systolic functions. In multivariate analysis, IL-6 levels independently predicted deterioration in RV systolic function at a significant level (odds ratio: 1.12, 95% confidence interval: 1.04-1.20, p=0.003). IL-6 is an independent predictor of RV systolic impairment in patients hospitalized with mild and moderate COVID-19 suggesting a possible pathogenetic mechanism. IL-6 levels can be used to predict RV systolic impairment in patients suffering from this infection.

Left Ventricular Global Longitudinal Strain Is Associated With Cardiovascular Outcomes in Patients Who Underwent Permanent Pacemaker Implantation.

Background: Patients who underwent permanent pacemaker (PM) implantation have a potential risk of left ventricular (LV) systolic dysfunction. However, assessment of LV ejection fraction (LVEF) shows a limited role in identifying subclinical LV systolic dysfunction and predicting cardiovascular (CV) outcomes.Methods: We reviewed 1,103 patients who underwent permanent PM implantation between January 2007 and December 2017. After excluding patients who did not undergo echocardiograms before or after PM implantation and those with LV ejection fraction (LVEF) <50%, significant valve dysfunction, and history of cardiac surgery before PM implantation, 300 (67 ± 13 years, 119 men) were finally analyzed. LV mechanical function was assessed with LV global longitudinal strain (LV-GLS) using 2-dimensional speckle-tracking echocardiography. CV outcomes were defined as a composite of CV death and hospitalization for heart failure.Results: At 44 ± 28 months after post-PM echocardiogram, 23 patients (7.7%) had experienced CV outcomes. Patients with CV outcomes were older and had more comorbidities and a lower baseline |LV-GLS| than those without CV outcomes. LV mechanical function worsened after PM implantation in patients with CV outcomes. The cut-off value of 11.2% in |LV-GLS| on post-PM echocardiogram had a better predictive value for CV outcomes (AUC; 0.784 vs. 0.647, p = 0.012). CV outcome in patients with |LV-GLS| <11.2% was worse than that in those with |LV-GLS| ≥ 11.2% (log-rank p < 0.001). Multivariate Cox model revealed that reduced |LV-GLS| was independently associated with CV outcomes.Conclusions: Pacing deteriorates LV mechanical function. Impaired LV-GLS is associated with poor CV outcomes in patients who underwent PM implantation.

Isolated right ventricular noncompaction in an adult woman with severe pulmonary hypertension following at the intensive care unit

Pulmonary hypertension is defined by a mean pulmonary artery pressure ≥25 mmHg at rest, measured during right heart catheterization. Ventricular noncompaction is a genetic cardiomyopathy which mostly effects left ventricle. It is related with deterioration of myocardial embryogenesis and commonly together with other cardiac diseases (1). Isolated ventricular non-compaction is characterized by modified morphology of myocardial wall, increased trabeculation in ventricular cavity and deep intertrabecular recesses. A 43-year-old woman presented exercise induced dyspnea and atypical chest pain. She has not any medical history prior. On admission, her 12 lead ECG showed complete righ bundle branch block, her blood pressure was 120/80 and pulse rate 80 per minutes. Transthoracic 2D echocardiogram and magnetic resonance imaging showed dilated and hypertrophied right ventricle with non-compaction of the right ventricular apex. The systolic pulmonary arterial pressure was 80 mmHg on the Doppler echocardiography. The coronary angiography revealed normal coronary arteries. The catheterization was showed pulmonary hypertension, right ventricle non-compaction and negative pulmonary vasoreactivity testing. Ventricular noncompaction, especially right ventricular noncompaction, complicated by severe pulmonary hypertension is exceptional. Only a few isolated right ventricular noncompaction has been reported but inclusion of pulmonary hypertesion cases are rare subsets . Diagnosis of pulmonary hypertension may be a consequence of increased pulmonary venous pressures caused by systolic and diastolic ventricular dysfunction secondary to right ventricular noncompaction. Widespread usage of cardiac magnetic resonance imaging, may enhance visual quality and evaluation of ventricular morphology, probably this will provide prevalance increment and clinical outcome improvements. Early diagnosis would bring better results.

Whole-exome sequencing reveals MYH7 p.R671C mutation in three different phenotypes of familial hypertrophic cardiomyopathy.

Familial hypertrophic cardiomyopathy (HCM) is one of the most common types of genetic heart disorder and features high genetic heterogeneity. HCM is a major cause of sudden cardiac death and also an important cause of heart failure-related disability. A pedigree with suspected familial HCM was recruited for the present study to identify genetic abnormalities. HCM was confirmed by echocardiography and clinical data of the family members were collected. Genomic DNA was extracted from the peripheral blood and sequenced based on standard whole-exome sequencing (WES) protocols. Sanger sequencing was further performed to verify mutation sites and their association with HCM. WES and Sanger sequencing revealed a heterozygous missense mutation (c.2011C>T p.R671C) in myosin heavy chain 7 (MYH7) that was identified in three family members. The Arg671Cys mutation was located in exon 18 and, to the best of our knowledge, has not been previously reported in familial HCM. Furthermore, family members carrying the same mutated gene were of different sexes and clinical phenotypes. They included the proband, a 17-year-old survivor of sudden cardiac arrest with ventricular systolic dysfunction, the proband's maternal uncle, who presented with ventricular diastolic dysfunction and the proband's mother, who had no obvious clinical symptoms and did not present with cardiac dysfunction. However, echocardiology indicated that the proband's mother had an enlarged left atrium, slightly thicker right anterior wall and anterior septum and an expanded atrial septum. Therefore, HCM exhibited obvious genetic and phenotypic heterogeneity. To the best of our knowledge, the present study was the first to report such a mutation in the MYH7 gene in familial HCM. In addition, the present study demonstrated that WES is a powerful tool for identifying genetic variants in HCM.

Unravelling racial differences in hypertensive heart disease by multiparametric cardiovascular magnetic resonance: a phenotype-wide association study

Abstract Funding Acknowledgements Type of funding sources: Foundation. Main funding source(s): Dr Georgiopoulos was supported by the Onassis Foundation under the special grant &amp; support program for scholars" association members Introduction – Black Afro-Caribbean hypertensives (BAHs) are exposed to a higher risk of heart failure (HF) than white hypertensives (WHs). Arterial afterload is higher in BAHs due to increased arterial stiffness and vascular volume; BAHs develop more often left ventricular (LV) hypertrophy, dilatation and systolic dysfunction than WHs. However, it is unclear whether other racial differences concur to the more pronounced LV remodelling in BAHs. Methods – This cross-sectional study included hypertensive patients undergoing cardiovascular magnetic resonance for their clinical work-up (1.5T Aera Siemens-Healthcare). Clinical history and haemodynamic parameters were collected in all participants; a subset of patients had complete bio-humoral assay of the renin-angiotensin-aldosterone system (RAAs). Arm cuff pressure was measured during CMR. The CMR protocol included: i) Arterial afterload / LV arterial-coupling - pulse-wave-velocity (PWV), aortic (Ea) and LV elastance (Ees) by aorta anatomic and phase-contrast velocity-encoding imaging; ii) ventricular remodelling and function - LV and right ventricular (RV) volumes, mass, EF, LV peak-filling rate by short-axis cine images; global circumferential and longitudinal strains by cine feature tracking; iii) left atrial (LA) remodelling volumes and reservoir, conduit and booster functions by long-axis cine images; iv) tissue characterisation: T2 and pre/post-contrast T1 relaxation times, extracellular volume (ECV) by single mid-ventricular short-axis T1/T2-mapping. Results – 34 BAHs and 35 WHs (52 ± 12 vs 45 ± 14 years, P &amp;lt; 0.05; 61% vs 65% males P = NS) were included in the study. Baseline features are summarised in the Table. LV systolic dysfunction was more prevalent in BAH than WHs (P = 0.038). Of note, BAHs tended to have greater LV volumes and significantly higher LV mass and septal thickness than WHs. In BAHs, but not in WHs, PWV was associated with increased septal thickness after correction for blood pressure and age (β-value: 0.447, P = 0.02). Normalised RV mass was greater in BHA than WHs; RV mass suits for the identification of racial or circulating factors predisposing to hypertrophy being largely unaffected by systemic afterload. In our study LV diastolic function and LA volumes were similar between BAHs and WHs, and none of the subjects had conditions associated with pre-capillary pulmonary hypertension. Hence, higher RV-mass in BAHs pinpoints a racial susceptibility to myocardial hypertrophy. Finally, in a subset of patients with RAAs assays (n = 43), the aldosterone/renin ratio was higher in BAHs than WHs (67.04 [IQR: 19.37-209.73] vs 13.77 [IQR: 7.47-40.43], P = 0.01). Conclusion – BAHs have heightened LV remodelling than WHs because of racial predisposition to develop hypertrophy which also encompasses derangements in RAAs. Altogether, these findings may account for the greater risk for HF in BAHs than WHs.

TTE apical 4-chamber view before ECMO, showing severe left ventricular (LV) systolic dysfunction, with somewhat sparing of the base, and akinesis of the remaining walls including the apex.

TTE apical 4-chamber view before ECMO, showing severe left ventricular (LV) systolic dysfunction, with somewhat sparing of the base, and akinesis of the remaining walls including the apex.