Left Ventricular Lateral Wall Research Articles

Abstract 12086: Relationship of Cardiac Rhythm and Other Electrocardiogram Findings with Sites of Wall Thickening and Abnormal Late Enhancement in Patients with Confirmed Cardiac Amyloidosis

Introduction: Left ventricular (LV) wall thickening and diastolic dysfunction on TTE without high voltage R wave on ECG to a diagnosis of cardiac amyloidosis (CA). However, amyloid sometimes invade right ventricle (RV), and left (LA) and right (RA) atria to cause ECG changes such as sick sinus syndrome (SSS), arrhythmia, and QRS axis deviation. Hypothesis: We hypothesized that sites of wall thickening and abnormal late enhancement (LE) on cardiac CT and MRI, suggesting amyloid invasion, correlated with cardiac rhythm and other ECG findings in patients with CA confirmed by biopsy. Methods: 26 patients (11 females) with suspected CA, showing LV wall thickening by TTE without a high voltage R wave, underwent cardiac CT. 5 patients (3 females, mean 73 years) were diagnosed with CA: complicated multiple myeloma, 2; senile ATTR (transthyretin) amyloidosis, 1; immunoglobulin light chain amyloidosis 1; and transthyretin mutation, 1. 4 patients underwent cardiac MRI. Results: 2 patients (cases 1 and 2) had SSS (junctional rhythm), 1 had atrial tachycardia, and the remaining 2 (cases 4 and 5) had a normal sinus rhythm. In case 1, ECG showed a left QRS axis deviation, no low voltage R wave and a mild LA load. Wall thickening in basal interventricular septum (IVS), LV inferior-posterior wall, LA on CT, LE in endocardium in whole LV, RV, and RA on CT, and LE in endocardium in whole LV, RV, LA, and IVS on MRI were observed. In case 2, ECG showed a normal QRS axis, no low voltage R wave, no LA load, wall thickening in whole LV, RV, LA, and IVS on CT, and unclear (CT) or no (MRI) LE. In case 3, ECG showed a normal QRS axis, with low voltage R wave, no LA load, wall thickening in LA and basal IVS on CT, LE in LA and basal IVS on CT, and LE in LA only on MRI. In case 4, ECG showed left QRS axis deviation, a low voltage R wave, and no LA load, wall thickening in LA and RV on CT, unclear LE on CT, and LE in whole LV, endocardium in RV, and whole IVS on MRI. In case 5, ECG showed a right QRS axis deviation, low voltage R wave, and a mild LA load, wall thickening in IVS, LV lateral wall, LV anterior wall, RA, RV outflow tract, and RA appendage, and no LE on CT (MRI not performed). Conclusions: The presence of wall thickening and abnormal LE in RV, LA, and RA in addition to LV on CT or MRI would be important which may influence ECG changes in patients with CA.

Determinants of left ventricular structure, filling and long axis function in systemic sclerosis.

Abnormalities of left ventricular (LV) structure, filling and long-axis function have all been reported in subjects with systemic sclerosis (SSc) and a normal LV ejection fraction (EF), but previous study findings have not been consistent. The aim of this study was to identify factors which could have confounded the analyses in previous studies of SSc, and in particular to consider the variables of body surface area (BSA), sex, age, heart rate, blood pressure (BP), disease duration (DD), disease type (limited versus diffuse) and interstitial lung disease (ILD). Echocardiography was performed on 100 subjects with SSc (79 women; age 56±15 years) with a LVEF ≥50% and free of pulmonary arterial hypertension, coronary artery disease, more than mild valvular heart disease and atrial fibrillation. Measurements were performed of the LV end-diastolic dimension (LVEDD) and septal wall thickness (SWT), the transmitral Doppler E, A and deceleration time (DT), and the peak systolic (s') and early diastolic (e') LV long-axis velocities. Multivariate analyses were performed to investigate correlations of the above LV variables with BSA, sex, age, heart rate, BP, DD, disease type, and the presence of ILD. DD varied between 0.1 and 41.2 years, 25% had diffuse and 75% had limited disease, and 37% had ILD. SWT and LVEDD were positively correlated with BSA, SWT was also positively correlated with age and larger in males, and LVEDD was larger in diffuse disease. Age was positively correlated with A and DT, and inversely correlated with E and E/A, and heart rate was inversely correlated with E and E/A. None of E, A, E/A, or DT were independently associated with DD or disease type. Septal and lateral LV wall s' and e' were all inversely correlated with age, and there was a small independent contribution to the prediction of lateral s' from DD, but no association of either s' or e' with disease type. The presence of ILD was not a predictor of any of the LV variables. In SSc there are associations of sex, body size, age and disease type with LV structural variables, of age and heart rate with E/A, and of age with both systolic and early diastolic LV long-axis velocities. Appropriate adjustment for these variables could help to resolve current uncertainties regarding SSc effects on the left ventricle.

Imaging of cardiac fibroblast activation in patients with chronic thromboembolic pulmonary hypertension.

The aim of this study was to explore the association of cardiac fibroblast activation with clinical parameters and cardiovascular magnetic resonance (CMR) imaging parameters in patients with chronic thromboembolic pulmonary hypertension (CTEPH). Thirteen CTEPH patients were prospectively enrolled. All of the patients underwent cardiac 68Gallium-labelled fibroblast activation protein inhibitor (68Ga-FAPI-04)-positron emission tomography/computed tomography (PET/CT), right heart catheterisation, and echocardiography, and 11 of them additionally underwent CMR. Thirteen control subjects were selected to establish the normal range of cardiac 68Ga-FAPI-04 uptake. Cardiac 68Ga-FAPI-04 uptake higher than that in the blood pool was defined as abnormal. The global and segmental maximum standardised uptake values (SUVmax) of the right ventricle (RV) were measured and further expressed as target-to-background ratio (TBRRV) with left ventricular lateral wall activity as background. Late gadolinium enhancement (LGE) was visually evaluated, and native-T1 times, enhanced-T1 times, and extracellular volume (ECV) were quantitatively measured. Ten CTEPH patients (77%) had abnormal 68Ga-FAPI-04 uptake in RV, mainly located in the free wall, which was significantly higher than that in controls (TBRRV: 2.4 ± 0.9 vs 1.0 ± 0.1, P < 0.001). The TBRRV correlated positively with the thickness of RV wall (r = 0.815, P = 0.001) and inversely with RV fraction area change (RVFAC) (r = - 0.804, P = 0.001) and tricuspid annular plane systolic excursion (TAPSE) (r = - 0.678, P = 0.011). No correlation was found between 68Ga-FAPI-04 activity and CMR imaging parameters. Fibroblast activation in CTEPH, measured by 68Ga-FAPI-04 imaging, is mainly localised in the RV free wall. Enhanced fibroblast activation reflects the thickening of the RV wall and decreased RV contractile function.

Prediction of sites of wall thickening and abnormal late enhancement on cardiac CT and magnetic resonance imaging using electrocardiography findings in patients with confirmed cardiac amyloidosis

Abstract Background Left ventricular (LV) wall thickening and diastolic dysfunction on a transthoracic echocardiogram (TTE) without high voltage R wave in V5 leads on ECG leads to a diagnosis of cardiac amyloidosis. A final diagnosis is made by endomyocardial biopsy. However, amyloid sometimes invade the right ventricle (RV), and left (LA) and right (RA) atria to cause ECG changes such as sick sinus syndrome (SSS), arrhythmia, and QRS wave axis deviation. Purpose To predict sites of wall thickening and abnormal late enhancement (LE) on cardiac computed tomography (CT) and magnetic resonance imaging (MRI), suggesting amyloid invasion, using cardiac rhythm and other ECG findings in patients with cardiac amyloidosis confirmed by biopsy. Methods A total of 26 patients (11 females) with suspected cardiac amyloidosis, showing LV wall thickening by TTE without a high voltage R wave in V5 leads on ECG, underwent cardiac enhanced CT. LV wall thickening on CT in the early phase led to late phase acquisition to detect LE. Five patients (3 females, mean age 73 years) were diagnosed with cardiac amyloidosis: complicated multiple myeloma, 2; senile ATTR (transthyretin) amyloidosis, 1; immunoglobulin light chain (AL) amyloidosis 1; and transthyretin mutation, 1. Four patients underwent cardiac MRI. Results Two patients (cases 1 and 2) had SSS (junctional rhythm), one had atrial tachycardia, and the remaining two (cases 4 and 5) had a normal sinus rhythm. In case 1, ECG showed a left axis QRS wave deviation, no low voltage R wave in limb leads and a mild LA load. Wall thickening in the basal interventricular septum (IVS), LV inferior-posterior wall, LA on CT, LE in the endocardium in whole LV, RV, and RA on CT, and LE in the endocardium in whole LV, RV, LA, and IVS on MRI were observed. In case 2, ECG showed a normal QRS wave axis, no low voltage R wave in limb leads, no LA load, wall thickening in whole LV, RV, LA, and IVS on CT, and unclear (CT) or no (MRI) LE. In case 3, ECG showed a normal QRS wave axis, with low voltage R wave in limb leads, no LA load, wall thickening in LA and basal IVS on CT, LE in LA and basal IVS on CT, and LE in LA only on MRI. In case 4, ECG showed left axis QRS wave deviation, a low voltage R wave in limb leads, and no LA load, wall thickening in the LA and RV moderator band on CT, unclear LE on CT, and LE in whole LV, endocardium in the RV, and whole IVS on MRI. In case 5, ECG showed a right axis QRS wave deviation, low voltage R wave in limb leads, and a mild LA load, wall thickening in the IVS, LV lateral wall, LV anterior wall, RA, RV outflow tract, and RA appendage, and no LE on CT (MRI not performed). Conclusions This pilot study of a few patients with cardiac amyloidosis revealed few links between cardiac rhythm and other ECG findings with sites of wall thickening and abnormal LE. However, a longer-term study of more patients may lead to detecting an association between these variables with this methodology. Funding Acknowledgement Type of funding sources: None.

Epicardial adipose tissue and nonvalvular atrial fibrillation

Abstract Background Several studies revealed the relationship between epicardial fat tissue (EF) and development of atrial fibrillation (AF). Though pathophysiological mechanisms are not clear enough, the anatomy and location EF as a part of visceral adiposity tissue could lead to proinflammatory and fibrotic changes in atrial myocardium. Purpose To evaluate the paracrine effects of EF and detect the association of tissue Doppler imaging (TDI) parameters, EF thickness by cardiac magnetic resonance tomography (CMR) and biochemical markers of fibrosis and inflammation in patients with nonvalvular AF without concomitant coronary artery disease. Methods Thirty-nine patients with idiopathic paroxysmal AF, normal or slightly enlarged left atria (LA) (mean LA diameter 4,5±0,6cm) were included. The mean age was 50,8±13,9 y.o., 19 (49%) were men. The absence of coronary lesions was confirmed by angiography in all cases. Besides standard ECG and ECHO, all patients underwent TDI and CMR with late gadolinium enhancement. EP thickness at anterior left ventricular (LV) wall was estimated by CMR. The myocardial fibrotic and proinflammatory biomarkers levels - matrix metalloproteinases (MMP-2, MMP-9), tissue inhibitor of matrix metalloproteinase-1 (TIMP-1) were estimated. Kendall rank correlation coefficient was used. If the |τ| &amp;gt;Tcr, the correlation ranked as statistically significant. Results The epicardial fat thickness was associated with TIMP-1 plasma level (τ=0,71; Tcr=0,18) and MMP-9 (τ=0,65; Tcr=0,16). Among clinical factors weight was slightly associated with fat thickness (τ=0,33; Tcr=0,26). Besides, we found correlation of MMP-9, TIMP-1 with E/e' by TDI (τ=0,65; Tcr=0,16 and τ=0,56; Tcr=0,21, relatively); and with maximal systolic strain of lateral LV wall (τ=−0,37; Tcr=0,18 and τ=−0,44; Tcr=0,16, relatively) and interventricular septum (τ=−0,36; Tcr=0,18 and τ=−0,44; Tcr=0,16, relatively). Conclusion The epicardial fat thickness by CMR is significantly associated with myocardial fibrosis markers (MMP-9, TIMP-1) in patients with AF without structural heart disease. The increasing levels of MMP-9 and TIMP-1 are associated with LV local contractility disturbances by TDI in patients with AF. This association demonstrates the paracrine effect of EF, which could lead to atrial remodeling and formation AF substrate even in patients without valvular and ischemic heart disease and with normal or slightly enlarged LA Funding Acknowledgement Type of funding sources: None.

Azygos Vein ICD Lead Implantation Lowers Defibrillation Threshold in a Patient with Hypertrophic Cardiomyopathy

A 14-year-old boy with hypertrophic cardiomyopathy (HCM) diagnosed at the age of 1 year and with massive left ventricular hypertrophy suffered an episode of ventricular fibrillation during mild effort. He underwent a dual-chamber implantable cardioverter defibrillator (ICD) implantation. The defibrillation threshold testing (DFT) was ineffective. Subcutaneous multi-coli arrays tunneled into the left postero-lateral position and connected to the superior vena cava (SVC) port of the dual-chamber ICD were added to increase the myocardial mass involved in the defibrillation shock pathway. A new DFT was unsuccessful. The patient was transferred to our hospital for myectomy. An epicardial defibrillation patch was placed on the left ventricular lateral wall, but again, DFT testing was ineffective using the right ventricular (RV) coil to lateral patch as shock pathway. Another epicardial defibrillation patch was then placed on the inferior wall. In this case, DFT testing was effective with a defibrillation pathway between the two patches and the can. In November 2015, a high shock impedance alarm was recorded through remote monitoring, thus compromising the safety of the ICD shock pathway. The patient underwent the implant of a new trans-venous defibrillation coil lead in the azygos vein. After few months, the patient developed symptomatic severe aortic regurgitation and underwent an aortic valve replacement. During the operation, DFT testing was performed and was successful. Our case illustrates that azygous vein ICD lead implantation is efficacious in HCM with massive hypertrophy and high DFT, and prompts further studies to systematically investigate its efficacy in this particular subgroup of the HCM population.

Massive myocardial calcification: a heart of stone!

Abstract A 71-year-old female was admitted in the emergency room after effort-related syncope. She had past medical history of obesity and hypertension treated with lercanidipine. No relevant family history. Physical examination revealed systolic murmur (grade 2/6). ECG showed sinus rhythm and left bundle brunch block (Supplementary figure). Lab results were unremarkable including troponin I. Transthoracic echocardiography (TTE) revealed moderate left ventricular (LV) hypertrophy [septal thickness 14 mm (normal: 6–9mm)], extensive mitral annulus calcification (MAC) with exuberant myocardial calcification at the level of posterior leaflet that invaded adjacent LV walls, systolic anterior motion of the anterior mitral leaflet causing LV outflow tract (LVOT) obstruction (maximum gradient 34 mmHg) and moderate mitral regurgitation (MR) (Panel A). Exercise echocardiogram provoked LVOT gradient &amp;gt;100mmHg and severe MR. Continuous ECG monitoring during 13 days demonstrated no arrhythmic events. Cardiac CT showed multiple calcifications extending from mitral annulus to LV anterior and lateral walls and cardiac MRI was compatible with caseous MAC (Panels B-F). Coronary angiography showed 50% stenosis of mid left anterior descending artery. Normal thoracic CT and ACE, and negative IGRA excluded sarcoidosis and tuberculosis. Comprehensive study, including calcium metabolism and autoantibodies, excluded metabolic and inflammatory aetiologies of myocardial calcification, which was assumed as dystrophic in the context of MAC. No syncope occurred after bisoprolol 2.5 mg, oral rehydration and discontinuation of lercanidipine. However, rest and exercise TTE failed to show improvement of LVOT gradient or MR and patient remained on NYHA class II-III, despite maximal tolerated dose of bisoprolol (5 mg), being therefore referred to cardiac surgery.

Novel approach to diagnosis of His bundle capture using individualized left ventricular lateral wall activation time as reference.

During nonselective His bundle (HB) pacing, it is clinically important to confirm His bundle capture versus right ventricular septal (RVS) capture. The present study aimed to validate the hypothesis that during HB capture, left ventricular lateral wall activation time, approximated by the V6 R-wave peak time (V6 RWPT), will not be longer than the corresponding activation time during native conduction. Consecutive patients with permanent HB pacing were recruited; cases with abnormal His-ventricle interval or left bundle branch block were excluded. Two corresponding intervals were compared: stimulus-V6 RWPT and native HB potential-V6 RWPT. The difference between these two intervals (delta V6 RWPT), which was diagnostic of lack of HB capture, was identified using receiver operating characteristic (ROC) curve analysis. A total of 723 electrocardiograms (ECGs) (219 with native rhythm, 172 with selective HB, 215 with nonselective HB, and 117 with RVS capture) were obtained from 219 patients. The native HB-V6 RWPT, nonselective-, and selective-HB paced V6 RWPT were nearly equal, while RVS V6 RWPT was 32.0 (±9.5) ms longer. The ROC curve analysis indicated delta V6 RWPT > 12 ms as diagnostic of lack of HB capture (specificity of 99.1% and sensitivity of 100%). A blinded observer correctly diagnosed 96.7% (321/332) of ECGs using this criterion. We validated a novel criterion for HB capture that is based on the physiological left ventricular activation time as an individualized reference. HB capture can be diagnosed when paced V6 RWPT does not exceed the value obtained during native conduction by more than 12 ms, while longer paced V6 RWPT indicates RVS capture.

Changes in miR 21 and 23b expression in postnatal hypertrophic heart derived from gestational diabetes precede dilated cardiomyopathy

Gestational diabetes mellitus (GDM) increases the risk of fetal congenital ventricular hypertrophic cardiomyopathy (HCM). We explored the effects and mechanisms of the postnatal progression of fetal hypertrophic failure in rat pups with STZ-induced Gestational Diabetes (GD). The hearts of rat pups (newborn [NB], 8, 15, 25 and 35 days postnatal) were obtained. Histological characteristics and expression of collagen were evaluated. In-gel-gelatin zymography for MMP-9 activation was performed. Adrenergic receptors (α2AR and β3AR), myosins (Myc6 and Myc7), Bcl-2 and Bax mRNA expression were quantified by qRT-PCR. Fetal hypertrophy of the left ventricular lateral wall (LVLW) in rat pups with DG persists until day 8, although this process appears to be reversed during the postnatal stage. The temporal continuity of the study demonstrated a thinning of the ventricular wall, similar to dilated cardiomyopathy (DCM). This ventricular remodeling process is associated with the expression of β3 adrenergic receptors and miR-21, -23b. The Bax/Bcl2 ratio was significantly reduced only at early ages. In addition, the increase in interstitial space in all ages, as well as the predominance of early ages expression of Col2 and increased expression of Col3, MMP-9 and Cx43 in late ages, is the result of an active extracellular remodeling in the hearts of rat pups with GD.

B-PO03-023 HIS BUNDLE PACING ACHIEVES BETTER VENTRICULAR SYNCHRONY THAN BIVENTRICULAR PACING IN PATIENTS WITH SCAR IN THE LEFT VENTRICULAR FREE WALL

His bundle pacing (HBP) is a novel pacing strategy for cardiac resynchronization therapy (CRT). HBP has been associated with improved response compared to conventional CRT and biventricular endocardial (BIV-endo) pacing. The effect of HBP in patients with scar has however not yet been studied. To investigate differences in response between HBP and BIV-endo pacing in patients with scar in the left ventricular (LV) free wall. We computed ventricular activation on 24 four-chamber heart meshes with Purkinje networks exhibiting left bundle branch block (LBBB). We mapped a LV free wall scar and border zone geometry from a pre-existing LV mesh [Fig A]. HBP and BIV-endo pacing were simulated in the presence and in the absence of scar. Response from baseline was quantified with LV 95% activation time (LV-AT 95) and biventricular dyssynchronous index (BIV-DI), computed as the standard deviation of ventricular activation times. BIV-DI was significantly reduced during HBP compared to BIV-endo pacing both in the presence (-13±2ms vs -15±2ms, p<0.05) and in the absence (-9±2 ms vs -11±2ms, p<0.05) of scar in the LV lateral wall [Fig B]. LV-AT 95 was also significantly shorter during HBP compared to BIV-endo pacing (scar: -31±8ms vs -54±9ms, p<0.05; no scar: -22±7ms vs -44±7ms, p<0.05) [Fig C]. HBP restores native synchronous activation by recruiting the viable fast-conducting Purkinje system. LBBB patients with lateral scar therefore experience better ventricular resynchronization using HBP compared to BIV-endo pacing.

Late gadolinium enhancement predicts adverse clinical outcome in patients with mitral valve prolapse/mitral annulus disjunction

Abstract Funding Acknowledgements Type of funding sources: None. OnBehalf Mitral vAlve prolapse and disjunction by cardiac maGnetIC resonance (MA-GIC) registry Backgroung Mitral valve prolapse (MVP) is 2-3% prevalent in the general population with good prognosis. However, some patients develop complex ventricular arrhythmias (CVAs), sudden cardiac death (SCD), or severe mitral regurgitation (MR). Previous studies suggested that bi-leaflet involvement, mitral annulus disjunction (MAD), and myocardial fibrosis (MF) are associated with adverse outcome. Notwithstanding, these findings were limited to autopsic series or single-centre studies involving highly selected patients. Moreover, MF has been scantly investigated as predictor of clinical outcome. Purpose To investigate the prognostic significance of MF in an international multicentre study of MVP patients studied by cardiovascular magnetic resonance (CMR) with late gadolinium enhancement (LGE). Methods From October 2007 to June 2020 patients undergoing LGE-CMR were screened in 14 European centres. Inclusion criteria were: i) age &amp;gt; 18 years; ii) full clinical history and cardiac rhythm monitoring at baseline; iii) MVP (leaflet displacement ≥ 2 mm beyond the annulus). Exclusion criteria were: i) ischemic heart disease; ii) primary cardiomyopathy; iii) inflammatory heart disease; iv) congenital heart diseases; v) moderate-to-severe valvular heart disease. CVAs at the study outset was defined as one of the following: i) ventricular ectopic beats &amp;gt;10000/24h; ii) ≥ 1 episode of non-sustained ventricular tachycardia (VT); iii) sustained VT; iv) aborted SCD. Primary end-point was a composite of SCD, unexplained syncope, and mitral valve repair/replacement. Secondary end-point was a composite of SCD and unexplained syncope. Results Four-hundred-fifty-eight MVP patients were eventually included (46 ± 16 years old, 51% males) of whom 68% had MAD. LGE was detected in 103 (22%) of subjects with mid-wall pattern (46%) in left ventricular (LV) lateral wall (66%) as the most prevalent feature. At baseline, 37% of LGE-positive patients vs. 18% of LGE-negative individuals had CVAs (P &amp;lt; 0.001). SVT and/or aborted SCD were more prevalent in LGE-positive than in LGE-negative patients (9% vs 2%, P &amp;lt; 0.001). By multivariable Cox-regression analysis, LGE presence or extent were strong independent predictors of the primary (HR = 4.02, P = 0.003 and HR = 4.76 per 10% increase, P = 0.032, respectively) and secondary (HR = 5.39, P = 0.008 and HR = 8.78 per 10% increase, P = 0.012, respectively) endpoints after correction for major confounders including LV volumes, left atrial size and MAD presence. Conlusion Myocardial fibrosis by LGE is the strongest independent predictor of clinical outcome in MVP. In contrast, MAD per se does not harbinger worse prognosis.

Lateral Wall Dysfunction Signals Onset of Progressive Heart Failure in Left Bundle Branch Block

ObjectivesThis study sought to investigate if contractile asymmetry between septum and left ventricular (LV) lateral wall drives heart failure development in patients with left bundle branch block (LBBB) and whether the presence of lateral wall dysfunction affects potential for recovery of LV function with cardiac resynchronization therapy (CRT). BackgroundLBBB may induce or aggravate heart failure. Understanding the underlying mechanisms is important to optimize timing of CRT. MethodsIn 76 nonischemic patients with LBBB and 11 controls, we measured strain using speckle-tracking echocardiography and regional work using pressure-strain analysis. Patients with LBBB were stratified according to LV ejection fraction (EF) ≥50% (EFpreserved), 36% to 49% (EFmid), and ≤35% (EFlow). Sixty-four patients underwent CRT and were re-examined after 6 months. ResultsSeptal work was successively reduced from controls, through EFpreserved, EFmid, and EFlow (all p < 0.005), and showed a strong correlation to left ventricular ejection fraction (LVEF; r = 0.84; p < 0.005). In contrast, LV lateral wall work was numerically increased in EFpreserved and EFmid versus controls, and did not significantly correlate with LVEF in these groups. In EFlow, however, LV lateral wall work was substantially reduced (p < 0.005). There was a moderate overall correlation between LV lateral wall work and LVEF (r = 0.58; p < 0.005). In CRT recipients, LVEF was normalized (≥50%) in 54% of patients with preserved LV lateral wall work, but only in 13% of patients with reduced LV lateral wall work (p < 0.005). ConclusionsIn early stages, LBBB-induced heart failure is associated with impaired septal function but preserved lateral wall function. The advent of LV lateral wall dysfunction may be an optimal time-point for CRT.

Interventricular systolic asynchrony predicts prognosis in patients with systemic sclerosis-associated pulmonary arterial hypertension.

Pulmonary arterial hypertension (PAH) is a serious complication of SSc with high mortality. Interventricular systolic asynchrony (IVSA) is observed in PAH patients, but the effect of IVSA and its association with long-term mortality and clinical events in SSc-associated PAH are unclear. This study aimed to investigate the impact of IVSA on the prognosis of SSc-associated PAH. Between March 2010 and July 2018, a total of 60 consecutive patients with SSc-associated PAH were enrolled. The end point was a composite of all-cause mortality and clinical worsening. Asynchrony was assessed by colour-coded tissue Doppler imaging (TDI) echocardiography. The myocardial sustained systole curves (Sm) of the basal portion of the right ventricular (RV) free wall and left ventricular (LV) lateral wall were obtained. IVSA was defined as the time difference from the onset of the QRS complex to the end of Sm between LV and RV. Patients with greater IVSA time differences presented with advanced pulmonary vascular resistance (PVR). The IVSA time difference was an independent predictive factor (Hazard Ratio (HR) = 1.018, 95% CI: 1.005, 1.031, P =0.005) for the composite end point and was significantly associated with PVR (r = 0.399, R2=0.092, P =0.002). Kaplan-Meier survival curves showed that patients with greater IVSA had worse prognoses (log-rank P =0.001). In conclusion, IVSA analysed by colour-coded TDI echocardiography provided added value as a noninvasive, easy-to-use approach for assessing the prognosis of patients with SSc-associated PAH. A significant IVSA time difference identifies the subgroup of patients at high risk of a poor prognosis.

Direct capture of the left bundle branch compared to left bundle branch area pacing deteriorates interventricular synchrony but improves left ventricular lateral wall depolarization duration

Abstract Funding Acknowledgements Type of funding sources: Public Institution(s). Main funding source(s): This paper was supported by the Charles University Research Centre program No. UNCE/MED/002 and 260530/SVV/2020 Background Direct and indirect pacing of the left bundle branch are novel pacing techniques preserving LV synchrony. Aim of the study was to compare differences in ventricular activation between them using an UHF-ECG. Methods The left septal lead placement was done in 68 patients with bradycardia. Four distinct ventricular captures were described; nonselective LBBp (nsLBBp), selective LBBp (sLBBp), paraLBBp and left bundle branch area capture (LBBap). The timings of local ventricular activations and local depolarization durations were displayed by the UHF-ECG. e-DYS was calculated as a difference between the first and last activation. Results There were 35 nsLBBp, 21 paraLBBp, 12 sLBBp and 96 LBBap obtained in 68 patients. The nsLBBp compared to LBBap caused worse interventricular synchrony (e-DYS -23 ms (-28;-18) vs -12 ms (-17;-8), p &amp;lt; 0.001), but improved LV lateral wall depolarization duration. The sLBBp, nsLBBp and paraLBBp differed in e-DYS; -31 ms (-38;-24) vs -23 ms (-28;-17) vs -13 ms (-20;-7), p &amp;lt; 0.01 between each of them. Their left ventricular depolarization durations were the same, but they were longer when pacing resulted in the left axis deviation. If the direct capture of the LBB was not confirmed (LBBap), LV depolarization duration was deteriorated irrespective of the QSR morphology in the V1 or RWPT in the V5. Examples of UHF-ECG maps during LBBap, paraLBBp and nsLBBp are shown in Figure 1. Conclusions The direct capture of the left bundle branch deteriorates interventriclar synchrony but improves the depolarization duration of the left ventricular lateral wall compared to left ventricular myocardial septal pacing. Abstract Figure 1

Ruptured Hydatid Cysts Resulted in Infectious Pericardial Effusion and Development of Cardiac Tamponade: A Rare Presentation

Introduction: Cardiac Hydatidosis is a rare and ominous complication of hydatid disease. Cardiac echinococcosis may be asymptomatic for several years but could be discovered after the development of lethal complications. Case Presentation: A 31-year-old-male referred with possible diagnosis of acute pericarditis. Abdominal and pelvic spiral CT scan showed focal and heterogeneous increased thickness of lateral left ventricular (LV) wall with protrusion into LV and bulging into pericardial space without central enhancement. His condition deteriorated suddenly due to cardiac tamponade with round cystic lesions suspected to hydatid cyst. Cardiac magnetic resonance imaging showed some round particles within effusion suggestive of possible scolex around the LV. There was round, and inhomogeneous cystic mass originated from sub-epicardial layer of mid-lateral LV that protruded into pericardial space. Diagnosis of hydatic cyst was confirmed by surgical specimen. Conclusions: We suggest that patients with pericarditis should be probed with echocardiography for the presence of hydatid cysts.

Left bundle branch pacing compared to left ventricular septal myocardial pacing increases interventricular dyssynchrony but accelerates left ventricular lateral wall depolarization

Nonselective His-bundle pacing (nsHBp), nonselective left bundle branch pacing (nsLBBp), and left ventricular septal myocardial pacing (LVSP) are recognized as physiological pacing techniques. The purpose of this study was to compare differences in ventricular depolarization between these techniques using ultra-high-frequency electrocardiography (UHF-ECG). In patients with bradycardia, nsHBp, nsLBBp (confirmed concomitant left bundle branch [LBB] and myocardial capture), and LVSP (pacing in left ventricular [LV] septal position without proven LBB capture) were performed. Timings of ventricular activations in precordial leads were displayed using UHF-ECG, and electrical dyssynchrony (e-DYS) was calculated as the difference between the first and last activation. Duration of local depolarization (Vd) was determined as width of the UHF-QRS complex at 50% of its amplitude. In 68 patients, data were collected during nsLBBp (35), LVSP (96), and nsHBp (55). nsLBBp resulted in larger e-DYS than did LVSP and nsHBp [- 24 ms (-28;-19) vs -12 ms (-16;-9) vs 10 ms (7;14), respectively; P <.001]. nsLBBp produced similar values of Vd in leads V5-V8 (36-43 ms vs 38-43 ms; P = NS in all leads) but longer Vd in leads V1-V4 (47-59 ms vs 41-44 ms; P <.05) as nsHBp. LVSP caused prolonged Vd in leads V1-V8 compared to nsHBp and longer Vd in leads V5-V8 compared to nsLBBp (44-51 ms vs 36-43 ms; P <.05) regardless of R-wave peak time in lead V5 or QRS morphology in lead V1 present during LVSP. nslbbp preserves physiological LV depolarization but increases interventricular electrical dyssynchrony. LV lateral wall depolarization during LVSP is prolonged, but interventricular synchrony is preserved.

Early and Late Improvement of Mitral Reserve in Selected Patients Undergoing Coronary Artery Bypass Graft with Moderate Ischemic Mitral Regurgitation

Objective: The treatment of moderate ischemic mitral regurgitation in patients undergoing coronary artery bypass graft is still debated. Mitral reserve may be simplified as the ratio between the length of anterior and posterior mitral leaflets. The aim of our study was to assess the outcome of ischemic mitral regurgitation in selected patients undergoing coronary artery bypass grafting. We have used the changes in the mitral reserve to assess the improvement of mitral valve leaflets’ motion. Methods: We have been enrolling 52 consecutive patients undergoing coronary artery bypass grafts (CABGs) with mild (1+) to moderate (2+) functional mitral regurgitation (MR) since June 2019. The selection criteria are hypokinesia of left ventricular lateral or posterior-lateral wall, mitral regurgitation ≤ 2+ and revascularization of some branches of circumflex artery. Trans-thoracic echocardiogram was carried out to all patients at baseline, 6 months and 1 year. Statistical analysis was performed with SPSS 23 and non-parametric Wilcoxon test was used. Results: At 6 months, the improvement of mitral reserve (2.33 ± 0.86 vs 2.01 ± 0.64; p=0.041) and ejection fraction (40.8 ± 11.5 vs 45 ± 9.8; p=0.029) and the reduction of mitral anulus (37.5 ± 5.03 vs 34.4 ± 3.34; p=0.013) are statistically significant and they also remain stable at 12 months. Mitral regurgitation degree is reduced at 6 months (1.53 ± 0.74 vs 1.40 ± 0.50; p=0.43) remaining stable at 12 months. Mean follow-up is 7.23 ± 5.22 months. Conclusions: The improvement of mitral reserve represented by the reduction of the ratio between the length of anterior and posterior mitral leaflets, should be explained by the reduction of the tethering of posterior leaflet due to myocardial revascularization. Therefore, we believe that in selected patients with ischemic mitral regurgitation undergoing coronary artery bypass graft, mitral valve shouldn’t be treated.

Gene Expression Changes of Humans with Primary Mitral Regurgitation and Reduced Left Ventricular Ejection Fraction.

Patients with primary mitral regurgitation (MR) may remain asymptomatic for many years. For unknown reasons, some shift from a compensated to a decompensated state and progress to fatal heart failure. To elucidate the genetic determinants of this process, we recruited 28 patients who underwent mitral valve surgery and stratified them into control, compensated MR, and decompensated MR groups. Tissue biopsies were obtained from the patients’ left ventricular (LV) lateral wall for a transcriptome-wide profiling of 64,769 probes to identify differentially expressed genes (DEGs). Using cutoff values at the 1% FDR significance level and sex- and age-adjusted regression models, we identified 12 significant DEGs (CTGF, MAP1B, SERPINE1, MYH9, MICAL2, MYO1D, CRY1, AQP7P3, HTRA1, PRSS23, IGFBP2, and FN1). The most significant gene was CTGF (adjusted R2 = 0.74, p = 1.80 × 10−8). We found that the majority of genes expressed in the more advanced decompensated MR group were pro-fibrotic genes associated with cardiac fibrosis. In particular, six pro-fibrotic genes (CTGF, SERPINE1, MYH9, HTRA1, PRSS23, and FN1) were overexpressed and enriched in pathways involved in ECM (extracellular matrix) protein remodeling. Therapeutic interventions that antagonize these six genes may slow the progression toward decompensated MR.

Episodic Myocardial Injury: Consider Desmoplakin Cardiomyopathy!

<h3>Introduction</h3> Recurrent myopericarditis, characterized by repeated episodes of pericardial and myocardial inflammation, can be a diagnostic conundrum. We present one such case ultimately found to have a desmoplakin (DSP) gene mutation. <h3>Case Report</h3> Our patient was a previously well adolescent male with a several year history of episodic chest pain and significant troponin elevation. He had mild left ventricular (LV) systolic dysfunction by echocardiography, with no ventricular dilation, pericardial effusion or valvar dysfunction. He had intermittent ventricular bigeminy. Coronary arteries were normal. Cardiac MRI revealed late gadolinium enhancement involving the anterior and lateral LV walls and interventricular septum. Myocardial biopsy revealed patchy interstitial fibrosis. Genetic testing revealed a likely pathogenic DSP mutation, c.123C>G (p.Tyr41*), predicted to cause loss of normal protein function. The variant is uncommon in the general population and several downstream nonsense variants have been associated with cardiomyopathy, supporting pathogenicity. <h3>Summary</h3> DSP is an essential protein in desmosomes, intercellular complexes responsible for maintaining cell-cell adhesion. Desmosomal mutations can cause arrhythmogenic cardiomyopathy, in which cardiac myocytes undergo fibroadiposis resulting in ventricular dysfunction, arrhythmias and sudden death. New evidence supports that DSP mutations can cause an inflammatory phenotype distinct from typical arrhythmogenic cardiomyopathy, characterized by predominant LV involvement, recurrent episodes of myocardial injury and fibrosis preceding systolic dysfunction. Our patient fits the novel description of DSP cardiomyopathy. His management plan includes reverse remodeling agents, surveillance for arrhythmia and cardiac dysfunction, restriction from competitive sports, and cascade genetic screening. Our case highlights the role of cardiomyopathy/arrhythmia genetic panels in patients with recurrent, unexplained myocardial inflammation.

Assessment of mitral valve function in children and young adults with hypertrophic cardiomyopathy using three-dimensional echocardiography

ObjectiveThe objective of this study was to assess papillary muscle (PM) and mitral valve (MV) structure and function in children and young adults with mild and moderate hypertrophic cardiomyopathy (HCM) using real-time three-dimensional echocardiography (3DE) and to correlate them with HCM related adverse outcomes. MethodsTransthoracic research 3DE was performed in HCM patients and controls matched for age and gender. Anterolateral and posteromedial PM mass, apical displacement of anterolateral PM, and left ventricular (LV) mass were measured and indexed to body surface area. The MV annulus and leaflet structure and function were analyzed. Individual PMs were manually planimetered by tracing the endocardial borders on each mid systole frame, taking care to distinguish PMs as distinct from the LV wall. Apical PM displacement was expressed as ratio of the distance between the apex and the base of the anterolateral PM to the entire length of the LV lateral wall (APL index). All 3DE measurements were correlated to adverse outcomes. ResultsForty subjects were studied, including 20 HCM patients (age 18.1 ± 9.6 years, 16 male and 4 female), and 20 controls (18.2 ± 9.6 years, 16 male and 4 female). The indexed LV mass in HCM was 74.8 ± 25.8 g/m2 compared to 50.8 ± 12.4 g/m2 in controls (p = 0.001). The anterolateral, posteromedial and combined PM mass were 3.1 ± 2.2 g/m2, 1.7 ± 1.2 g/m2 and 4.9 ± 2.7 g/m2 in HCM, in contrast to respective measurements of 1.1 ± 0.6 g/m2, 1.2 ± 0.6 g/m2 and 2.3 ± 0.8 g/m2 in controls (p < 0.001, p = 0.062, and p < 0.001, respectively). The mitral valve annular parameters (annulus circumference, height and area) in HCM were not significantly different from controls. The APL index in HCM was less than in controls (0.44 ± 0.07 vs. 0.55 ± 0.04, p < 0.001). The LV lateral wall length and LV mass correlated with adverse HCM outcomes, while the APL index and PM total mass were not associated with adverse events. ConclusionIt is feasible to evaluate PM muscles and MV annulus geometry in children and young adults with HCM using 3DE. The morphologic and functional changes of anterolateral PM may occur in the absence of MV annulus changes. Prospective validation will be required to determine if LV lateral wall length and LV mass may be used as predictors of adverse events.