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Left ventricular outflow tract obstruction is now recognized as a common complication of takotsubo syndrome, resulting in more serious acute and long-term outcomes. We describe a case of takotsubo syndrome where a transient sigmoid septum produced left ventricular outflow obstruction and explore the mechanisms leading to this occurring. This phenomenon has not been previously described.

Hypertrophic cardiomyopathy (HCM) is the most common genetic cardiac condition and highly heterogeneous. Echocardiography and genetic and clinical screening have led to detection in women of childbearing age. Maternal and fetal outcomes among women with HCM are favorable. Genetic counseling is recommended. Prepregnancy clinical evaluation and risk assessment are paramount in ensuring optimal outcomes. Most women carry moderate risk of morbidity, have clinical evaluations and echocardiography each trimester, and deliver vaginally. Those who are symptomatic or have significant left ventricular outflow obstruction or recurrent arrhythmias prior to pregnancy are at higher risk and should be monitored at least monthly.

Aim . To assess the effects of combined myectomy with mitral valve repair on a three-dimensional model of the mitral valve in patients with obstructive hypertrophic cardiomyopathy. Methods. 24 patients with obstructive hypertrophic cardiomyopathy and left ventricular outflow obstruction over 50 mm Hg at rest were recruited in a study. Eight patients underwent combined myectomy with mitral valve repair according to the Carpentier method. Seven patients underwent the Alfieri's edge-to-edge repair, and nine patients underwent secondary chordae resection using the Ferrazzi’s technique. Before combined myectomy and two weeks after it, all patients underwent standard transthoracic echocardiography and real-time 3D transesophageal imaging of the mitral valve, followed by quantitative 3D reconstruction of the mitral valve and calculation of the annulus, the leaflets, and the aorto-mitral angle. Results. Despite the selected mitral valve repair technique, we observed a decrease in the LVOT obstruction gradient. There were no differences in the residual obstruction gradient between the selected mitral valve repair technique. Patients with obstructive hypertrophic cardiomyopathy, who underwent combined myectomy and posterior mitral leaflet plasty valve according to the Carpentier approach, reported a correlation of a decrease in the LVOT obstruction with the non-planar angle (r = -0.83; p = 0.040), sphericity index (r = 0.83; p = 0.04) and a decrease in the velocity excursion of the annulus (r = 0.94; p = 0.005). Patients who underwent the Alfieri's edge-to-edge repair demonstrated that the residual LVOT obstruction gradient depended on the annulus height (r = 0.90; p = 0.04) and the ratio of this height to the commissural diameter of the annulus ( r = 0.90; p = 0.04). After secondary chordae resection, a decrease in the LVOT obstruction gradient correlated with the sphericity index (r = 0.77; p = 0.03), the anterolateral-posteromedial annulus diameter (r = -0.72; p = 0.04), the anterior (r = -0.78; p = 0.02) and posterior (r = -0.78; p = 0.02) leaflets, the ratio of the total leaflet length to the anteroposterior diameter of the annulus (r = -0.83; p = 0.01), the area (r = -0.76; p = 0.0з) and the mitral valve tenting height (r = -0.95; p = 0.00). Conclusion .Combined myectomy with mitral valve repair is the method of choice in the treatment of patients with obstructive hypertrophic cardiomyopathy. The comparison of three mitral valve repair techniques did not reveal any differences in the residual LVOT obstruction gradient. However, the Alfieri's edge-to-edge repair may be considered as the most physiological technique to repair dynamic LVOT obstruction.

Left ventricle outflow tract obstruction can have catastrophic hemodynamical consequences. Patients with severe aortic stenosis undergoing transcatheter aortic valve replacement are at risk of developing this serious complication in the postprocedural period. We present how a drug refractory left ventricle outflow tract obstruction, observed in a 76 year old female after transcatheter aortic valve replacement, was successfully treated with permanent atrioventricular pacing. After the procedure we observed an increase of gradients in left ventricle outflow tract to maximal gradient 96mmHg and mean gradient 46mmHg. The mechanism of left ventricle outflow tract obstruction was systolic anterior motion of the mitral valve. Despite fluids and increased dose of beta-blocker the was no improvement on control echocardiography. A decision to implant a dual chamber pacemaker was made in order to try do decrease the gradients. An immediate decrease in left ventricle outflow tract gradients after activation of sequential pacing with short atrioventricular delay was observed. Awareness of this method may prove helpful to cardiothoracic anesthesiologists treating patients after transcatheter aortic valve replacement

OBJECTIVES The present study evaluated the results of the modified réparation à l’étage ventriculaire (REV) based on the individual anatomical and pathological findings of the patients with an anomalous ventriculo-arterial connection with ventricular septal defect (VSD) and left ventricular outflow tract obstruction. METHODS We reviewed a series of 24 patients who underwent modified REV between 2005 and 2019. Surgical indications included ventricles and atrioventricular valves suitable for biventricular repair, severe left ventricular outflow tract obstruction (peak gradient >30 mmHg), unrestrictive subaortic VSD and coronary arteries not suitable for reimplantation. RESULTS The mean follow-up time was 7.0 ± 4.2 years (range 0.5–14.1 years). Kaplan–Meier analyses showed that overall survival was 100% and freedom from any reoperation was 93.3% ± 6.4%. Longitudinal analyses of the available postoperative echocardiographic data showed that the left ventricular outflow tract peak gradient was less than 10 mmHg in all patients (15/15) and the left ventricular ejection fraction was more than 50% in 93.3% of patients (14/15). The right ventricular outflow tract peak gradient was less than 40 mmHg in 73.3% of patients (11/15). CONCLUSIONS The REV remains an option for selected patients despite the increasing use in recent years of the Nikaidoh procedure and its modifications. The surgical strategy needs to be determined by the specific anatomical and pathological findings of the patient. The modified REV had excellent long-term survival and freedom from reoperation for the treatment of anomalous ventriculo-arterial connection with VSD and left ventricular outflow tract obstruction. The long-term performance of the reconstructed left ventricular outflow tract and right ventricular outflow tract is satisfactory.

We aimed to describe the short- and long-term outcomes of patients after an arterial switch operation (ASO) at a single institution during a 23-year period. A retrospective chart review of all patients18 months of age who underwent an ASO between January 1995 and March 2018 at Texas Children's Hospital, Houston, TX was performed. Primary endpoints include mortality and reintervention. Perioperative mortality was defined as mortality occurring in-hospital and/or30 days after surgery. Survival and freedom-from-reintervention were analyzed using Kaplan-Meier method, log-rank tests, and Cox regression models. The cohort included 394 patients. Diagnoses included 204 patients (52%) with intact ventricular septum, 137 (35%) with a ventricular septal defect, 17 (4%) with a ventricular septal defect and left ventricular outflow tract obstruction (LVOTO), and 36 (9%) with Taussig-Bing anomaly. Median age at surgery was 8 days (range: 1 day to 17 months) and median weight was 3.4 (range: 0.8-12.0) kg. Overall perioperative mortality was 1.3% (n = 5), 0.3% (n = 1) since 1999. Overall survival at 5, 10, and 15 years was 98.2%, 97.8%, and 97.8%, respectively. Perioperative morality was associated with prematurity (P = 0.012),2.5 kg (P0.001), and longer circulatory arrest (P = 0.024) after univariate analysis. Reintervention was associated with a longer cross-clamp time (P0.001),2.5 kg (P = 0.009), LVOTO resection (P = 0.047), and genetic syndrome (P= 0.011) after multivariable analysis. Current ASO expectations should include a perioperative mortality risk of1% and good long-term survival. Reinterventions are more frequent in patients2.5 kg, concomitant LVOTO resection, a genetic syndrome, and longer cross-clamp time.

Background: Hypertrophic cardiomyopathy (HCM) is the most common hereditary heart disease with a variety of genotypes and phenotypes. In previous terms, hypertrophic obstructive cardiomyopathy and idiopathic hypertrophic subaortic stenosis are no longer used to describe this business. Patients may or may not have a ventricular outflow tract (LVOT). Restlessness or LVOT inhibition occurs in 70% of patients and is the most common cause of heart failure. The pathology of HCM includes left ventricular hypertrophy with or without right ventricular hypertrophy, contractile mitral valve advancement, flexible and flexible LVOT inhibition, mitral valve recurrence, diastolic dysfunction, myocardial ischemia, and Includes fibrosis. A complete understanding of pathology and pathophysiology is essential for neural control and surgery. Conclusion: Hypertrophic Cardiomyopathy requires invasive treatment. Alcohol Septal ablation and surgery are two common methods of invasive treatment. &nbsp

Summary Bicuspid aortic valve (BAV) with ∼1%–2% prevalence is the most common congenital heart defect (CHD). It frequently results in valve disease and aorta dilation and is a major cause of adult cardiac surgery. BAV is genetically linked to rare left-heart obstructions (left ventricular outflow tract obstructions [LVOTOs]), including hypoplastic left heart syndrome (HLHS) and coarctation of the aorta (CoA). Mouse and human studies indicate LVOTO is genetically heterogeneous with a complex genetic etiology. Homozygous mutation in the Pcdha protocadherin gene cluster in mice can cause BAV, and also HLHS and other LVOTO phenotypes when accompanied by a second mutation. Here we show two common deletion copy number variants (delCNVs) within the PCDHA gene cluster are associated with LVOTO. Analysis of 1,218 white individuals with LVOTO versus 463 disease-free local control individuals yielded odds ratios (ORs) at 1.47 (95% confidence interval [CI], 1.13–1.92; p = 4.2 × 10−3) for LVOTO, 1.47 (95% CI, 1.10–1.97; p = 0.01) for BAV, 6.13 (95% CI, 2.75–13.7; p = 9.7 × 10−6) for CoA, and 1.49 (95% CI, 1.07–2.08; p = 0.019) for HLHS. Increased OR was observed for all LVOTO phenotypes in homozygous or compound heterozygous PCDHA delCNV genotype comparison versus wild type. Analysis of an independent white cohort (381 affected individuals, 1,352 control individuals) replicated the PCDHA delCNV association with LVOTO. Generalizability of these findings is suggested by similar observations in Black and Chinese individuals with LVOTO. Analysis of Pcdha mutant mice showed reduced PCDHA expression at regions of cell-cell contact in aortic smooth muscle and cushion mesenchyme, suggesting potential mechanisms for BAV pathogenesis and aortopathy. Together, these findings indicate common variants causing PCDHA deficiency play a significant role in the genetic etiology of common and rare LVOTO-CHD.

Background Hypertrophic cardiomyopathy (HCM) is the most common hereditary heart disease, and its diagnosis is often associated with limited physical activity. Little is known about cardiac rehabilitation programs for patients with HCM. Therefore the novel hybrid cardiac telerehabilitation (HCTR) model consisting of hospital-based rehabilitation and home-based telemonitored rehabilitation might be an option to improve physical capacity in patients with HCM. Purpose To evaluate the safety, effectiveness and adherence to HCTR in patients with HCM without the left ventricle (LV) outflow tract obstruction and preserved LV ejection fraction. Methods The study group comprised 60 patients with HCM (51.1±13.3 years; NYHA II-III; LV ejection fraction 66.1±6.9%). Patients were randomised (1:1) to either HCTR program (hospital-based rehabilitation [1 month] based on cycloergometer training and home-based telemonitored rehabilitation [2 months] based on Nordic walking, five times a week, at 40–70% of maximal estimated heart rate) - training group (TG), or to a control group (CG). All patients had implantable cardioverter-defibrillator. In order to perform home-based telemonitored rehabilitation, a special device was used which enabled patients to: (1) do Nordic walking training according to a preprogrammed plan, (2) record and send electrocardiograms (ECGs) via mobile phone network to the monitoring centre. The moments of automatic ECGs registration were pre-set and coordinated with exercise training. The effectiveness of HCTR was assessed by changes - delta (Δ) in duration (t) of the workload, peak oxygen consumption (pVO2) in cardiopulmonary exercise test, 6-minute walking test distance (6-MWT) as a result of comparing t (s), pVO2 (ml/kg/min), 6-MWT (m) from the beginning and the end of the program. Results Safety of HCTR. Neither death nor other serious adverse events occurred during HCTR. We did not observe any ICDs intervention during the HCTR. Effectiveness of HCTR: Within-group analysis: t, pVO2, 6-MWT increased significantly in TG: t 657±183 vs 766±181 (p Between-group analysis: The differences between TG and CG were statistically significant: in Δt (p Adherence to HCTR: In TG 28 patients (93%) completed the HCTR program. Two patients did no undergo HCTR because of personal issues. Conclusion Hybrid cardiac telerehabilitation in patients with HCM without the left ventricle (LV) outflow tract obstruction and preserved LV ejection fraction is safe and effective. The adherence to HCTR is high. Funding Acknowledgement Type of funding sources: Public Institution(s). Main funding source(s): Statutory work in The Cardinal Stefan Wyszyński National Institute of Cardiology in Warsaw, Poland

Accessory mitral valve tissue is a rare congenital cardiac abnormality that sometimes can cause left ventricular outflow tract obstruction. We herein present the case of a 55-year-old male with an incidental finding of accessory mitral valve tissue on transthoracic echocardiography. The patient was managed conservatively as accessory tissue was not causing left ventricular outflow obstruction and there were no hemodynamic consequences.

Pregnancy causes important physiologic stress for women with hypertrophic cardiomyopathy. Data regarding the impact of this condition on obstetrical outcomes is missing.Our objective was to report obstetrical and cardiac outcomes in pregnant women with hypertrophic cardiomyopathy and to assess the possible adverse effects of left ventricular outflow tract obstruction in pregnancy.This was a retrospective cohort study of pregnant women diagnosed with HCM and followed at single tertiary center between 1995 and 2019. Demographic, medical and surgical data, echocardiographic parameters, and pregnancy outcomes were abstracted through extensive chart review. Patients were divided into 2 groups: obstructive (maximal left ventricular outflow tract gradient over 30 mmHg) versus non-obstructive hypertrophic cardiomyopathy. Outcomes between groups were compared withEighteen women with 27 pregnancies were included. The study population was formed of 18 women with a total of 27 pregnancies that reached at least 20 weeks of gestation: 12 pregnancies in women with obstructive hypertrophic cardiomyopathy and 15 pregnancies in women with non-obstructive hypertrophic cardiomyopathy. Among the non-obstructive hypertrophic cardiomyopathy, 5 of them had been treated for their obstruction. One patient with obstructive hypertrophic cardiomyopathy had a medical termination of pregnancy for uncontrolled arrhythmia at 21 weeks. There were no maternal deaths. Left ventricular outflow tract obstruction was associated with increased cardiac events including arrhythmias and heart failure (5/12 versus 0/15;Hypertrophic cardiomyopathy is associated with prematurity and small for gestational age. Left ventricular outflow tract obstruction is associated with adverse cardiac events including arrythmias or heart failure. Treated obstructive cardiomyopathy constitutes a sub-group of patients at high risk of severe small for gestational age and deserves a close surveillance. Therefore, fetal growth surveillance with ultrasound, early in the third trimester and doppler studies to assess the utero-placental perfusion in the second and third trimesters are warranted in all patients with hypertrophic cardiomyopathy regardless of the severity of their condition.

Background The RASopathies are a group of genetic disorders caused by germline mutations in genes encoding components of the RAS/MAPK signalling pathway and frequently associated with hypertrophic cardiomyopathy (HCM). The clinical features and outcomes of RASopathy-related HCM are incompletely understood and most published studies are limited by relatively small numbers and incomplete clinical characterisation. Purpose To describe the clinical features outcomes in a large, single-centre cohort of patients with RASopathy-associated HCM diagnosed Methods Data from 105 patients [68 (64.8%) males] diagnosed in childhood with RASopathy-associated HCM at a single specialist centre between 1985 and 2020 were retrospectively analysed. Results The RASopathy diagnosis was Noonan syndrome in 69 patients (65.7%); NSML in 10 (9.5%); CFC in 6 (5.7%); Costello in 5 (4.8%) and 15 patients (14.2%) had another Noonan-like variant. 72 patients (68.6%) had a pathogenic/likely pathogenic variant in a RAS-MAPK gene, most commonly PTPN11 [n=25 (34.7%)], followed by RAF 1 [n=13 (18.1%)]. 100 patients (95.2%) were probands, 9 (8.6%) had family history of cardiomyopathy and 2 (1.9%) family history of sudden cardiac death (SCD). 52 (49.5%) had concomitant congenital heart defects (CHD) [ASD 13 (25%), VSD 6 (11.5%), PDA 1 (1.9%), >1 CHD 32 (61.5%)]. 29 (27.6%) had symptoms at baseline assessment and 56 (53.3%) were on cardiac medication. The distribution of left ventricular hypertrophy (LVH) was concentric in 47 (44.7%); 32 (30.5%) had asymmetric septal hypertrophy (ASH), and undocumented in 25 patients (23.8%). 45 patients (42.9%) had biventricular hypertrophy (BVH). Resting left ventricular outflow tract obstruction (LVOTO) was present in 39 (37.1%) with haemodynamically significant LVOTO (≥50mmHg) in 23 (21.9%). Resting right ventricular outflow tract obstruction (RVOTO) was present in 21 (20%). Over a median follow up time of 6 years, 19 patients (18.1%) died [1 (5.3%) SCD; 2 (10.5%) due to Heart Failure-related death; 1 (5.3%) due to another CVS cause; 5 (26.3%) due to a non-CVS cause and for 10 (52.6%) cause of death was unknown]. Incidence rate of death was calculated at 2.7 deaths per 100 person-years. Surgical septal myectomy was performed in 9 patients (8.6%) and 3 (2.9%) underwent cardiac transplantation. 14 patients (13.3%) suffered arrhythmic events [atrial tachycardia 6 (42.9%), Non-Sustained Ventricular Tachycardia 4 (3.8%), and Ventricular Tachycardia/Ventricular Fibrillation 4 (3.8%)]. Conclusion To our knowledge, this is the largest cohort of RASopathy-associated HCM. The findings show a heterogeneous clinical presentation with a high prevalence of morbidity and mortality. Further work is needed to determine predictors of outcome in this population. Funding Acknowledgement Type of funding sources: Foundation. Main funding source(s): Onassis Foundation

Background Diastolic function assessment in patients with hypertrophic hearts and preserved ejection fraction (EF) is a rather challenging task. Combined plotting of deformation parameters against other indices, especially left ventricular (LV) volume, may reflect diastolic function components of the hypertrophic myocardium. Purpose Aim of this study was i) to apply strain-volume loops (SVLs) in hypertrophic cardiomyopathy (HCM) patients based on simultaneous frame-by-frame strain and volume changes' recordings acquired by means of three-dimensional (3D) speckle tracking imaging and ii) to investigate potential correlations between these loops, traditional diastolic function indices and phenotypic features of HCM (thickness, obstruction and fibrosis) that may also reflect myocardial “stiffness”. Methods We included 40 HCM patients (54.1±14.3 years, 82.5% male, maximum wall thickness 19.3±4.8mm) who have consecutively undergone 3D-speckle tracking echocardiography and cardiovascular magnetic resonance (CMR) with late gadolinium enhancement (LGE). Values of 3D strain were plotted vs. volume for each frame to build an SVL. Peak of radial, longitudinal and circumferential systolic strain (Rsp, Lsp, and Csp, respectively), systolic slopes of the loops (RsSl, LsSl, CsSl), strain to end-diastolic volume (EDV) ratios (Rs/V, Ls/V, Cs/V) as well as the extent of systolic-diastolic uncoupling (difference between systolic and diastolic strain for the same volume) (panel A) were computed for the analysis. Left atrial volume index (LAVI), E/E' and tricuspid regurgitation velocity (TRvel) were measured to define diastolic dysfunction (DD) stage. Burden of fibrosis was evaluated by LGE extent in CMR slices. Results All HCM patients had preserved EF (60.5±5,7%), while 16 (40%) had LV outflow tract obstruction (LVOTO>30 mm Hg at rest). Mean LV mass index was 78.9±14.5 g (evaluated by 3D echocardiography). LGE was observed in 23 patients (57.5%) occupying 5.2±4.5% of LV mass. Concerning SVLs the following values were recorded for radial (Rsp 30.8±9.8%, RsSl 0.4±0.13 and Rs/V 0.25±0.09), longitudinal (Lsp −9.4±3.7%, LsSl 0.12±0.06 and Ls/V 0.08±0.04) and circumferential deformation (Csp −14.2±3.5%, CsSl 0.18±0.05 and Cs/V 0.11±0.03). Traditional isolated diastolic indices (E/E', LAVI, TRvel and DD stage) did not present significant correlations with SVL parameters or HCM phenotypic features. However, potentially “stiffer” hearts (combination of increased LVMI and fibrosis) presented a leftward transition of longitudinal SVLs, which also became wider (greater uncoupling) (panel B). Conclusions Traditional diastolic indices show modest only correlations with SVLs or HCM phenotypic characteristics, necessitating new approaches to DD of HCM patients. SVLs seem to be a promising-innovative tool for indirect assessment of myocardial “stiffness” and diastolic function. Funding Acknowledgement Type of funding sources: None. Figure 1

Background Hypertrophic cardiomyopathy is estimated to affect 1 out of every 500 adults in the USA. One of its main complications is left ventricular outflow obstruction, which may require surgical septal myectomy in severe cases. We report a rare complication of postoperative septal akinesis leading to thrombus formation presenting as an acute ischaemic stroke. Case summary A 48-year-old woman presented with acute stroke 2 years after surgical septal myectomy for hypertrophic obstructive cardiomyopathy. Diagnostic workup identified an intraventricular thrombus arising in the left ventricular outflow tract (LVOT). After comprehensive evaluation, it was determined that the thrombus development was a complication of the prior septal myectomy causing focal septal akinesis. Treatment with anticoagulation resulted in improvement of neurological symptoms and resolution of the intraventricular thrombus. Discussion This case illustrates the rarity and unusual presentation of an intracardiac thrombus that arises from septal myectomy site. A thrombus arising in the LVOT, which is characterized by high gradient laminar flow, is highly unusual. This suggests microscopic and macroscopic alteration in the ventricular septal wall structure, as evident by the septal wall akinesis seen on echocardiography. Recognition of this complication is critical to the selection of appropriate anticoagulation as secondary stroke prevention in these patients.

The indications for mitral valve repair extend across the entire spectrum of degenerative mitral valve disease, ranging from fibroelastic degeneration to Barlow’s disease. Collaboration between the surgeon and anesthesiologist is essential for ensuring optimal results. Echocardiographic assessment of the repair can be challenging but is essential to the success of the procedure, as even mild residual mitral regurgitation can portend poor patient outcomes. In addition to determining the severity of residual regurgitation, the anesthesiologist must elucidate the mechanism of disease in order to inform appropriate re-intervention measures. Finally, there are unique complications of mitral valve surgery for the anesthesiologist to understand and assess by echocardiography. This review describes a systematic pathway for a comprehensive intraoperative assessment of the mitral valve following surgical repair.

Background: Left ventricular outflow tract (LVOT) obstruction is a serious complication that can occur after various mitral-valves, surgical or percutaneous, interventions. It was rarely described in mechanical mitral valve replacements. Aim: to describe a rare case of late LVOT obstruction after a mitral valve replacement by a low-profile mechanical prosthesis. Case Presentation: A 48-year woman, with a history of rheumatic mitral valve disease and mechanical mitral replacement by a hemi-disc valve 18 years ago, presented for a recent dyspnea. Echocardiography showed a narrowing of the LVOT, with anterior position of the mitral prosthesis, aorto-mitral annular angulation, septal thickening and remnant native sub-valvular tissue attached to the septum in the LVOT region. This resulted in LVOT obstruction with a peak gradient of 75 mmHg. The heart team opted for a redo surgery, but the surgical decision was refused by the patient. Discussion: This is a rare case of late LVOT obstruction after mitral valve replacement by mechanical low-profile prosthesis. Preserved native mitral valve tissue, which is the main described cause of LVOT obstruction after mechanical mitral valve replacements was not the unique cause of obstruction in this patient who had also a septal thickening and anterior prosthetic position. Aorto-mitral annular angulation that was identified as a risk factor of LVOT obstruction after trans-catheter mitral valve replacements, should be, probably, also took into account and assessed pre-operatively in patients undergoing surgical mitral replacements. Conclusion: LVOT obstruction can occur after mechanical mitral replacements event with low profile prosthesis. In patients with identified risk factors of LVOT obstruction, preservation mitral anterior leaflet should be avoided, and preservation of other native mitral tissue should be discussed.

Background The most common mode of death in childhood hypertrophic cardiomyopathy (HCM) is sudden cardiac death (SCD). Left ventricular outflow tract obstruction (LVOTO) is an established risk factor for SCD in adults with the disease. In contrast, the prognostic implications of LVOTO in childhood disease is unclear, with recent studies suggesting that it may have an inverse relationship with the risk of SCD. Purpose The aim of this study was to explore the role of LVOTO and the risk of SCD in childhood HCM. Methods A multi-centre, retrospective, longitudinal cohort of 871 children (diagnosed with HCM Results 189 patients (23%) had LVOTO, which was mild (30–50mmHg), moderate (50–100mmHg) or severe (>100mmHg) in 58 (6.7%), 98 (11.3%) and 33 (3.8%), respectively. The risk of SCD showed an inverse relation to LVOT gradient severity compared to those with no obstruction: mild HR 1.75 (95% CI 0.89–3.44), moderate HR 1.04 (95% 0.55–1.98), and severe HR 0.7 (0.36–1.35) [figure].On univariable analysis [table] LVOTO was associated with heart failure symptoms (NYHA>1) [p Conclusions LVOTO appears to have a complex relationship with the risk of SCD in childhood HCM, with multiple contributing factors. The pathophysiological mechanisms behind this observation need further exploration, which may be limited by low patient numbers Cummulative incidence of SCD by LVOTO Funding Acknowledgement Type of funding source: Other. Main funding source(s): British Heart Foundation

Hypertrophic obstructive cardiomyopathy (HOCM) is characterized by muscle hypertrophy and fibrosis, interfering with force generation and relaxation. Abnormal ventricular (LV) myocardial deformation have been demonstrated in patients with HOCM at rest, but there is lack of data regarding the deformational mechanics in exercise in obstructive HCM. Aims To assess the adaptability of LV deformational behaviour to physical exercise in HOCM patients as compared to healthy controls and to examine if Deformational Imaging (DMI) can provide additional information. Methods 28 obstructive HOCM (age 51.2±14.2yrs; 16 men, LVOT-obstruction 56±19mmHg at rest or on Valsalva maneuver) and 32 control subjects (50.9±6.8 yrs, 19 men from the MAGYAR-PATH Registry) underwent bicycle stress Echo (ESE) with full conventional 2D- and Doppler TTE, 2D- and 3D-Speckle Tracking Analysis (DMI) and 3D-Full Volume assessment both at rest and on submaximal ESE. Results At rest, the HOCM group had lower GLS (−14.6±4.5 vs 18.4±2.6%, p Conclusions The HOCM patients had attenuated Strain and Rotational response on exercise implying impaired deformational adaptability with post-systolic shortening and pronounced mechanical dispersion of peak Strain on exercise which was more accurate to identify the HOCM phenotype than other Echo-indices. These findings support evidence for reduced systolic-diastolic coupling efficiency in HOCM patients on exercise which can contribute to the development of exercise-related breathlessness, dynamic LVOT-obstruction and arrhythmias in patients with HOCM. These distinct deformational patterns on exercise can help in the differential diagnosis of patients with LV hypertrophy of unknown aetiology with good sensitivity and specificity and can also aid in the risk stratification workup in HOCM. DMI Predictors for HOCM Funding Acknowledgement Type of funding source: Public hospital(s). Main funding source(s): Milton Keynes University Hospital Research Founding

Background Combined plotting of deformation parameters against other indices [e.g. arterial pressure, left ventricular (LV) volume] might offer additional information about different diseases. Especially in hypertrophic cardiomyopathy (HCM) this approach might offer new insights into the various phenotypic and pathophysiologic features of this entity. Purpose Aim of this study was i) to apply strain-volume loops in HCM based on simultaneous frame-by-frame strain and volume changes' recordings acquired by means of three-dimensional (3D) speckle tracking imaging and ii) to investigate potential correlations between these loops and phenotypic features of HCM (including thickness, obstruction and fibrosis). Methods We included 40 HCM patients (54.1±14.3 years, 82.5% male, maximum wall thickness 19.3±4.8mm) who have consecutively undergone 3D-speckle tracking echocardiography and cardiovascular magnetic resonance (CMR) with late gadolinium enhancement (LGE). Values of 3D strain were plotted vs. volume for each frame to build a strain–volume loop. Peak of radial, longitudinal, and circumferential systolic strain (Rsp, Lsp, and Csp, respectively), systolic slopes of the loops (RsSl, LsSl, CsSl), and strain to end-diastolic volume (EDV) ratio (Rs/V, Ls/V, Cs/V) were computed for the analysis (panel A). Additionally, burden of fibrosis (percentage of LV mass) was defined by LGE extent (>5 standard deviations compared to nulled myocardium) in CMR slices. Results All HCM patients had preserved EF (60.5±5,7%), while 16 (40%) had LV outflow tract obstruction (LVOTO>30 mm Hg at rest). Mean LV mass index was 78.9±14.5 g (evaluated by 3D echocardiography). LGE was observed in 23 patients (57.5%) occupying 5.2±4.5% of LV mass. Concerning strain-volume loops the following values were recorded for radial (Rsp 30.8±9.8%, RsSl 0.4±0.13 and Rs/V 0.25±0.09), longitudinal (Lsp −9.4±3.7%, LsSl 0.12±0.06 and Ls/V 0.08±0.04) and circumferential deformation (Csp −14.2±3.5%, CsSl 0.18±0.05 and Cs/V 0.11±0.03). Among typical HCM characteristics tested (LV mass, LVOTO and LGE), only LV mass presented significant correlations with LsSl (r=−0.41, p Conclusions Strain-volume loop is an innovative application of 3D deformation imaging in HCM. According to this new non-invasive method, increase of LVMI in HCM is accompanied by less longitudinal contribution to stroke volume, whereas better systolic-diastolic coupling may exclude the presence of underlying fibrosis. Funding Acknowledgement Type of funding source: None

Background Takotsubo syndrome (TTS) is an acute and reversible left ventricular dysfunction, whose pathophysiological mechanisms are not completely known. Aim of the study is to evaluate the incidence and prognostic value of acute mitral insufficiency among TTS patients. Methods Three hundred sixteen consecutive patients admitted for TTS were enrolled from July 2007 to December 2019 in a prospective registry among four hospitals. Clinical features, laboratory data, ECG and echocardiographic parameters and in-hospital complications were recorded. Results Eighty-five (27%) patients, mean age 76±8 years, presented with moderate/severe mitral insufficiency (msMI). Compared to the control group, patients that presented with msMI were older (76±8 vs 71±12 years, p The incidence of in-hospital adverse events was higher in case of both msMI and LVEF There was a reduction of mitral insufficiency from moderate/severe into mild in 30 out of 87 (34%) pts after 48h from admission and in 52 out of 87 pts at discharge (60%). Left ventricular outflow tract obstruction (LVOTO) was found in 13 out of 87 (15%) pts with msMI at admission and was transient in 11 out of 13 pts (84%). At long term follow up (650 days) patients with msIM when compared to those without, experienced higher rate of mayor cardiovascular events (31.7% vs 20% p=0.03). Conclusions Patients with TTS and moderate/severe mitral insufficiency at admission have an increased risk of in and out-of hospital mayor cardiac adverse events. Funding Acknowledgement Type of funding source: None

To briefly review the pathophysiology and natural history of hypertrophic cardiomyopathy (HCM) and to describe the diagnosis, assessment, and contemporary management strategies. HCM-related mortality remains low; however, symptoms due in large part to LVOT obstruction remain a clinical dilemma. Several medical therapies have been shown to reduce symptoms and improve functional capacity, including several recent phase 2 clinical trials involving the novel myosin modulator mavacamten. In patients with refractory symptoms, septal reduction therapy or advanced therapies remain viable options in many cases. HCM is a complex and heterogeneous disease with diverse presentations and variable anatomy and clinical outcomes. The majority of patients will remain asymptomatic or with minimal symptoms and long-term survival remains high. For symptomatic patients, a variety of medical therapies, along with septal reduction therapies, have been shown to reduce symptoms and improve functional capacity.

Discrete fibromembranous subaortic stenosis is a common type of subaortic stenosis causing clinically significant left ventricular outflow obstruction. Surgery for discrete subaortic stenosis is most often performed through a typical midline sternotomy. Herein, we present our experience with an adult patient who underwent a right mini-thoracotomy for subaortic membrane resection with central cannulation under direct operative vision.

A primigravida 26‐year‐old woman who had developed pre‐eclampsia with malignant hypertension at 30 weeks of gestation suffered acute myocardial infarction two days postpartum. Electrocardiogram demonstrated diffuse ST‐segment depression suggestive of subendocardial ischemia. Echocardiography demonstrated focal asymmetric left ventricular hypertrophy, with a characteristic “basal septal bulge”, and a left ventricular mid‐cavitary gradient of 51 mmHg. Coronary angiography revealed normal coronary arteries and vascular flow. Peripartum acute myocardial infarction is rare and portends a high mortality. However, to date, only one case of acute myocardial infarction associated with asymmetric left ventricular hypertrophy and pre‐eclampsia has been described in the literature.

Alcohol septal ablation for relief of left ventricular outflow obstruction from hypertrophic cardiomyopathy is associated with a risk of high grade heart block requiring permanent pacing. However, not all rhythms demonstrating A-V dissociation are indicative of complete and/or permanent heart block. We present a case of transient competing sinus and junctional rhythm following alcohol septal ablation which resolved spontaneously without clinical need for permanent pacemaker insertion.

Background Syncope is a serious complication of significant aortic valve stenosis. Left ventricular outflow obstruction, abnormal arterial and venous vasodilatation and arrhythmic events are usually the most common pathophysiological mechanisms. Structurally abnormal native valves with turbulent abnormal flow are a substrate for infective endocarditis in the elderly population. Clinical Case We report the case of an 74 year-old woman with a past history of hypertension, type II diabetes, dyslipidemia and degenerative severe aortic stenosis waiting valve replacement surgery who presented to the emergency department with syncope. Elevated inflammatory markers, fever and leukocyturia raised the hypothesis of urinary tract infection. Empirical antibiotic was initiated and the patient was admitted to the Internal Medicine ward. Evolution was unsatisfactory with evolution to cardiogenic shock. Urgent transthoracic echocardiogram showed dilated right cavities, mild pericardial effusion and high gradient flow between the left ventricle and right cavities with an unstructured calcified high mobility aortic valve with perivalvular abscess. Patient was transferred to a tertiary center for emergent surgery. A biologic aortic valve and pericardial bovine patch at the proximal membranous septum was implanted. Six-week empirical antibiotic treatment for endocarditis was completed after surgery. Initial blood cultures and native valve culture were negative. Residual restrictive left to right shunt was observed by TTE evaluation. The patient was discharged home and is doing well at follow-up. Conclusion Left-to-right shunt with subsequent cardiogenic shock is a non-common complication of aortic valve endocarditis. Patients presenting with syncope in the context of aortic stenosis must have a careful initial evaluation and mechanical complications excluded. Abstract P953 Figure. EuroEcho2019

Left ventricular outflow obstruction after transcatheter mitral valve replacement is a life-threatening complication. We report a case of a 68-year old female with early degeneration of a transcatheter aortic valve and severely calcified mitral valve stenosis who was considered inoperable by a multidisciplinary heart team and referred for transcatheter aortic and mitral valve replacement. Our aim is to report the planning, procedural aspects, and management of device-related left-ventricular outflow tract obstruction after transcatheter double valve replacement.

We present optimally reconstructed three-dimensional computed tomography images of left ventricular outflow obstruction, comprehensive left ventriculography, and comparable intraoperative transesophageal echocardiography, as well as serial operative pictures, to facilitate the understanding of live-heart anatomy of hypertrophic obstructive cardiomyopathy. As shown in this case, detailed morphological analysis around the left ventricular outflow tract using preoperative computed tomography would be feasible and useful. The present case highlights the importance of obtaining complete three-dimensional information present in the acquired computed tomography dataset because computed tomography is not entirely noninvasive or free of radiation exposure and contrast material.

Guidelines recommend exercise stress echocardiogram (ESE) for patients with hypertrophic cardiomyopathy (HC) if a 50 mm Hg gradient is not present at rest or provoked with Valsalva or amyl nitrite, to direct medical and surgical management. However, no study has directly compared all 3 methods. We sought to evaluate efficacy and degree of provocation of left ventricular outflow gradients by ESE, and compare with Valsalva and amyl nitrite. In patients with HC between 2002 and 2015, resting echocardiograms and ESEs within 1 year were retrospectively reviewed. Gradients elicited by each provocation method were compared. Rest and ESE were available in 97 patients (mean age 54 ± 18 years, 57% male); 78 underwent Valsalva maneuver and 41 amyl nitrite provocation. Median gradients (interquartile range) were 10 mm Hg (7,19) at rest, 16 mm Hg (9,34) with Valsalva, 23 mm Hg (13,49) with amyl nitrite, and 26 mm Hg (13,58) with ESE. ESE and amyl nitrite were able to provoke obstruction (≥30 mm Hg) and severe obstruction (≥50 mm Hg) more frequently than Valsalva. In patients with resting gradient30 mm Hg (n = 83), provocation maneuvers demonstrated dynamic obstruction in 51%; in those with Valsalva gradient30 mm Hg (n = 57), ESE or amyl nitrite provoked a gradient in 44%; and in those with amyl nitrite gradient30 mm Hg (n = 20), ESE provoked a gradient in 29%. No demographic or baseline echocardiographic parameter predicted provocable obstruction. In conclusion, ESE is clinically useful; however, different provocation maneuvers may be effective in different patients with HC, and all maneuvers may be required to provoke dynamic obstruction in symptomatic patients.

Purpose of review Distinction between discrete subvalvar aortic stenosis and other causes of left ventricular outflow obstruction has important implications for predicting natural history and guiding the timing and type of intervention. Imaging, primarily transthoracic echocardiography (TTE), plays a pivotal role in the diagnosis and management of adults with subvalvar aortic stenosis. Recent findings Most systematic research on imaging of subvalvar aortic stenosis has focused on echocardiography. TTE, especially two-dimensional imaging with color and spectral Doppler, remains the main modality for delineation of the anatomic and hemodynamic features of subvalvar stenosis, associated anomalies and involvement of accessory mitral valve attachments to the subaortic septum or abnormally placed papillary muscles. Transesophageal echocardiography may provide more detailed definition of left ventricular outflow tract anatomy, including the presence and extension of the obstructive subaortic fibroelastic tissue onto the aortic or mitral valve, especially in patients with poor transthoracic windows. The clinical role for advanced imaging technologies, including three-dimensional echocardiography, cardiac magnetic resonance and computed tomography, is evolving but, largely because of the adequacy of established imaging with TTE, remains relatively limited. Summary In the absence of other congenital heart defects or alternative indications (e.g. coronary angiography), TTE is usually adequate for the assessment of discrete subvalvar aortic stenosis in the adult. In specific clinical situations, supplemental imaging modalities can play an integral role in clinical decision making.

Both surgical myectomy and percutaneous transluminal septal myocardial ablation are effective treatments for drug-refractory symptomatic hypertrophic obstructive cardiomyopathy (HOCM). However, in some cases, it is not easy to elucidate the abnormal structure of left ventricular outflow obstruction to adopt these treatments. Here, we presented a young female patient with drug-refractory symptomatic HOCM. In this case, contrast-enhanced computed tomography enabled us to assess the suitability of percutaneous transluminal septal myocardial ablation. By creating three-dimensional printed models using computed tomography data, we could also visualize intracardiac structure and simulate the surgical procedure. A multimodality assessment strategy is useful for evaluating patients complicated with drug-refractory symptomatic HOCM.

Ross procedure involves replacing diseased aortic valve with pulmonary autograft and placing a pulmonary/aortic homograft between the right ventricle and pulmonary artery. In case of small aortic annulus in pediatric patients or in patients with multilevel left ventricular outflow obstruction, aortic annulus enlargement might be required concurrently. Konno type of aortic ventriculoplasty is most commonly done. The American Heart Association/American College of Cardiology (AHA/ACC) has classified transesophageal echocardiography (TEE) as class I indication for congenital heart disease

A 23-year-old female of Taussig-Bing heart with antero-posterior relation of the great arteries was underwent Patrick-McGoon's intraventricular rerouting at 6 years old of age. The left ventricular outflow obstruction (peak pressure gradient of 100 mmHg) developed, and severe aortic valve regurgitation following bacterial endocarditis was noted. The conversion to Rastelli's type operation and aortic valve replacement were performed successfully at 23 years old of age. She is doing well without any significant left or right ventricular outflow obstruction at 7 years postoperatively.

Doppler echocardiogram provides not only structural information about heart chambers, valves and pericardium but also contributes to functional assessment. A unique feature of this assessment is its variability, not only due to heart disease course but also related to extracardiac conditions. We present a case of primary amyloidosis with two atypical characteristics: left ventricular outflow obstruction and low-grade diastolic dysfunction. Nevertheless, a more typical picture of cardiac amyloidosis was observed in the evolution of the disease coinciding with a second cardiac stressor, emphasizing the necessity of not focusing our attention exclusively to the heart.

Background In a phase 2 PIONEER-HCM study, pts with symptomatic, obstructive hypertrophic cardiomyopathy (oHCM) showed improvement in left ventricular outflow tract (LVOT) obstruction, exercise capacity, and symptoms after 12 wk of treatment with the novel myosin modulator, mavacamten (Mava). Purpose To examine the long-term safety and effectiveness of Mava in PIONEER-OLE study Methods PIONEER-OLE (NCT03496168) is an ongoing 2-y multicenter study for adults with symptomatic oHCM who completed PIONEER-HCM (NCT02842242). The starting dose of Mava is 5 mg/d with titration at wk 6 to an individualized therapeutic dose (5, 10, or 15 mg). Evaluations are at wk 4, 6, 8, 12 and every 12 wk thereafter to monitor LV ejection fraction (LVEF), LVOT gradient, New York Heart Association (NYHA) class, NT-proBNP, drug concentration, and safety. Results 13 pts (mean age, 57.8 y; 9 male; 12 on beta-blockers) were enrolled. Mean baseline LVOT obstruction and LVEF, and wk 12 changes from baseline, were similar to those in PIONEER-HCM (Table). Mava significantly reduced resting and provoked LVOT gradients and NT-proBNP at wk 12 and 24 compared with baseline (P Results from PIONEER-OLE Parameter PIONEER-HCMa PIONEER-OLE Baseline Wk 12 Baselineb Wk 12 Change at Wk 12 Wilcoxon Signed Wk 24 Change at Wk 24 Wilcoxon Signed Mean ± SD Mean ± SD Mean ± SD Mean ± SD Mean ± SD Rank Mean ± SD Mean ± SD Rank (n=13) (n=13) (n=13)c (n=12)c (n=12)c P value (n=10)c (n=10)c P value LVOT Rest gradient, mmHg 69.7±53.9 27.8±31.3 67.3±42.8 12.0±5.4 −57.9±43.2 0.0005 10.5±4.8 −66.6±42.4 0.0020 LVOT Valsalva gradient, mmHg 93.7±55.6 36.8±37.5 89.9±30.7 (n=12) 23.6±20.0 −66.4±35.3 (n=11) 0.0020 21.1±11.5 −67.3±33.5 (n=9) 0.0039 LVEF, % 73.0±5.6 64.6±10.5 72.0±4.9 67.6±7.2 −4.4±5.5 0.0269 68.2±6.5 −3.2±3.3 0.0195 NT-proBNP, pg/mL 1601.3±2782 (n=12) 684±980 1836±2886 181±211 −1759±2789 0.0005 170±225 −2128±3104 0.0039 Data extraction date January 24, 2019. aCombined results shown for pts from PIONEER-HCM originally in cohort A (n=5) and cohort B (n=8). bBaseline in PIONEER-OLE occurred 6–18 months after completion of PIONEER-HCM. cNumber of pts with data available for analysis, unless otherwise specified. Conclusion Despite management with current therapies, pts enrolled in PIONEER-OLE with similar levels of obstruction and hypercontractility as in PIONEER-HCM. In this longest observation period, Mava significantly reduced obstruction (LVOT gradient) in pts with oHCM beyond standard HCM therapy, while maintaining normal LVEF and improving symptoms. Acknowledgement/Funding MyoKardia

Background Surgical myectomy for (HOCM) results in complex structural and functional changes. “Remodelling” in different cardiac chambers. To date, changes in the Right versus the left Ventricle have not been studied. Methods Fourty five patients (mean age = 32±16, 68% males) who underwent extended septal myectomy for LVOTO and Fourty “normal” controls (mean age = 32±12 years, 52% males) were studied by cardiac magnetic resonance imaging (CMR). The patients were studied pre-operatively and 6–18 months post-operatively (median = 9 months). The images were analysed by both commercial and in-house software. Results After myectomy. Follow up CMR showed changes in RV mass (21±5 to 23±7) g/m2, volume (60±15 to 66±12) ml/m2 and shape using 3 different methods. RV deformation parameters showed significant changes with circumferential strain (−8±2 to −14±4), filling (38±16 to 62±19) ml/s/m2 and ejection rate (−44±17 to −75±22). Changes in RV were substantially higher than those observed in the LV (Figure. 1, Table. 1). All patients reported significant symptomatic improvement with 31 (78%) patients in NYHA class I and 9 (22%) in class II at follow up. Significant reduction in peak gradient across the LVOT by 75%. Table 1. Summary of reported parameters related to RV Shape for pre and post operation HOCM patients and Normal Healthy Volunteers LV RV Pre Post Normal P-value Pre Post Normal P-value EDV ml/m2 75±18 81±14 73±10 0.005 60±15 66±12 71±12 0.002 ESV ml/m2 20±9 24±8 26±6 0.008 16±7 19±9 26±7 0.02 SV ml/m2 56±13 57±10 51±13 0.38 44±11 48±10 49±14 0.009 EF 74±7 70±7 65±5 0.001 74±8 72±7 64±6 0.228 Mass g/m2 74±33 62±29 27±8 0.0456 21±5 23±7 18±5 0.2100 PFR ml/m2 173±48 141±48 141±40 Conclusion LV septal myectomy is followed by structural and functional remodelling which is more extensive in the right than the left ventricle. The clinical significance of these findings needs further study.

Anterior mitral valve clefts are unusual anomalies particularly associated with endocardial cushion defects. A dysplastic subvalvular apparatus causing left ventricular outflow tract obstruction may result in diagnostic confusion. We present an isolated anterior mitral cleft associated with subvalvular dysplasia.

A 15-years-old male patient presented with a history of fever and exertional dyspnoea of two weeks duration. Ten years back, he had undergone an uncomplicated anatomic repair of double outlet right...

The spectrum of congenital anomalies of the aortic valve, which typically result in significant outflow obstruction, include subaortic stenosis, aortic valve anomalies (most commonly a bicuspid aortic valve) and supravalvular aortic stenosis. Although the latter is typically seen in the pediatric population, both congenital subaortic and aortic valvular abnormalities will commonly be seen in adults. This chapter will review the congenital anomalies leading to left ventricular outflow obstruction at the level of outflow tract and aortic root.

Summary Since 1994, alcohol septal ablation (ASA) has been used as a minimally invasive treatment of patients with hypertrophic obstructive cardiomyopathy, resistant to conservative medical therapy. This catheter-based intervention consists of injecting absolute alcohol in a septal perforator to induce infarction of the hypertrophied septum and thus diminish the left ventricle outflow tract obstruction. This reduction of the gradient is associated with reduction of symptoms and left ventricle remodeling. The procedure was improved after the introduction of myocardial contrast echocardiography for visualization of the area at risk of infarction and reduction of the alcohol amount. Major complications of ASA are rare but centers with experience have reported conduction disorders - about 10% of patients needed permanent pacing because of complete AV block. Large randomized prospective studies have not yet compared alcohol septal ablation to the gold standard for treatment of hypertrophic obstructive cardiomyopathy - surgical myomectomy.