Ventricle Outflow Tract Obstruction Research Articles

Surgical outcome of Yasui procedure for preserving biventricular function: single centre experience.

Yasui procedure is surgical repair intended to preserve biventricular function for patients with left ventricle outflow tract obstruction associated with aortic arch lesions and ventricular septal defect. Retrospective chart review analysis of all patients who had Yasui procedure (2008-2020) comparing midterm outcome of one versus two stage repair. Twenty patients (70% female) underwent Yasui procedure in our center. Eight patients (40%) had left ventricle outflow tract obstruction /interrupted aortic arch, 7 patients (35%) had left ventricle outflow tract obstruction /coarctation of aorta, 3 patients (15%) had double outlet ventricle and ventricular septal defect that were unattainable for tunneling to one of the semilunar valves without creating obstruction, and 2 patients (10%) had aortic atresia with hypoplastic aortic arch. All patients had associated ventricular septal defect. Fifteen patients (75%) had one-stage repair and 5 patients (25%) had two-stage repair. Means age and weight for one and two-stage repair were 1.3 ± 2 months, 13.4 ± 11.5 months and 3.3 ± 0.6 kg, 7.8 ± 3.4 kg, respectively. During follow up, 8 patients (40%) required re- intervention, mainly for right ventricle-pulmonary artery conduit either dilation or replacement. The average duration of follow up was 5 years with nil mortality. Yasui procedure is effective approach for children who have left ventricle outflow tract obstruction associated with aortic arch anomalies and ventricular septal defect. Survival rate with single or staged repair is comparably good. During the first 5 year of follow up, nearly 40% of operated patients required re-intervention.

Hybrid telerehabilitation in patients with hypertrophic cardiomyopathy without left ventricular outflow tract obstruction and preserved left ventricular ejection fraction-the randomised clinical trial

Abstract 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<0.001), pVO2 19.2±5.0 vs 20.6±4.9 (p=0.007), 6-MWT 445±88 vs 551±77 (p<0.001). In the untrained CG, the unfavourable results were observed: 695±198 vs 717±187 (p=0.114), pVO2 21.2±5.1 vs 21.1±5.6 (p=0.723), 6-MWT 512±83 vs 536±84 (p=0.061). Between-group analysis: The differences between TG and CG were statistically significant: in Δt (p<0.001); ΔpVO2 (p=0.012); Δ6-MWT (p<0.001). 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

Preintervention imaging and intraoperative management care of the hypertrophic obstructive cardiomyopathy patient.

With an estimated overall mortality of less than 1 percent per year, hypertrophic cardiomyopathy, is the most common genetic cardiomyopathy. Intraoperative transesophageal echocardiography is the standard of care for assessing patients with hypertrophic obstructive cardiomyopathy undergoing surgical septal myectomy, allowing surgical planning, intraoperative hemodynamic monitoring, and postprocedural assessment of the repair, including detection of immediate complications. At various phases during surgical septal myectomy, the changing hemodynamic conditions may lead to worsening or improvement in left ventricle outflow tract obstruction by change in preload or afterload, systolic anterior motion of the mitral valve, or sympathetic stimulation. These characteristics represent unique challenges in the management of these patients, requiring a comprehensive understanding of the management of all the conditions required to decrease the left ventricle outflow tract gradient avoiding obstruction, which include the maintenance of sinus rhythm, adequate rate avoiding tachycardia and bradycardia, and avoidance of systemic hypotension preserving preload and afterload, with adequate vasoactive agents. The aim of this review is to summarize the perioperative assessment and management of patients undergoing hypertrophic obstructive myopathy surgery.

Improved Long-term Outcome of Damus-Kaye-Stansel Procedure Without Previous Pulmonary Artery Banding

BackgroundThis study sought to determine long-term outcomes of a primary Damus-Kaye-Stansel (DKS) procedure in patients with a functional single ventricle and to compare the results with those of our historical control subjects who underwent pulmonary artery banding before the DKS procedure. MethodsThe study reviewed the medical records of all patients who underwent the DKS procedure at the German Heart Center of Munich, Germany between December 1994 and December 2019. ResultsThe DKS procedure was performed as initial palliation in 52 patients (primary DKS group) and as staged palliation after pulmonary artery banding in 24 patients (staged DKS group). The median follow-up period after the DKS procedure was 8.9 years in the primary DKS group and 8.0 years in the staged DKS group. The survival rates at 10 years after the DKS procedure were 89% in the primary DKS group and 68% in the staged DKS group (log-rank P = 0.04). Before total cavopulmonary connection, the pressure gradient through the systemic ventricular outflow tract was significantly lower in the primary DKS group than in the staged DKS group (P < .001). At last follow-up echocardiography, reduced ventricular function was observed in 1 patient in the primary DKS group and in 7 patients in the staged DKS group (P < .001). The degree of neoaortic regurgitation was significantly higher in the staged DKS group than in the primary DKS group (P < .001). ConclusionsA primary DKS procedure in patients with a functional single ventricle and potential systemic ventricular outflow tract obstruction is recommended to obtain favorable long-term survival with preserved ventricular function and competent semilunar valve function.

Late gadolinium enhancement as a predictor of arrhythmias in patients with hypertrophic cardiomyopathy

Abstract Funding Acknowledgements Type of funding sources: None. Introduction Patients with hypertrophic cardiomyopathy (HCM) are at increased risk of arrhythmias and sudden cardiac death (SCD). The Late gadolinium enhancement (LGE) in cardiovascular magnetic resonance (CMR) has been associated with the occurrence of arrhythmic events. Purpose The aim was to analyze the association between LGE burden and location and arrhythmic events in HCM patients (P). Methods Retrospective analysis of P with HCM in a single tertiary center. Baseline clinical, echocardiographic and CMR characteristics were collected. On follow up arrhythmias (ventricular fibrillation (VF), sustained ventricular tachycardia (SVT), non-sustained ventricular tachycardia (NSVT), paroxysmal supraventricular tachycardia (PSVT), atrial fibrillation (AF) and atrial flutter (AFL)) were identified. LGE on CMR was compared between patients with and without arrhythmias. Results 61P (59% male) were included, with a mean age of 58 ± 2 years. The HCM risk-SCD score was 3.35 ± 0.28%. On echocardiography mean left ventricle ejection fraction was 62.16 ± 1.36% and maximum wall thickness 20.59 ± 0.596mm. 31.1% had systolic anterior movement of mitral valve and 26.7% had left ventricle outflow tract obstruction. LGE was present in 88.5% P with a median number of 5 ± 7 segments involved. Interventricular septum (IVS) was involved in 78.7% P, anterior wall in 57.4%, inferior wall in 54.1%, lateral wall in 52.5%, posterior wall in 9.8%, basal segments in 62.3%, median segments in 68.9% and apical segments in 63.9%. On follow up 3.3%P died, 45.8% had hospitalizations (22,2% because of an arrhythmia) and 75% had arrhythmias (1.6% VF, 6.6% SVT, 50% NSVT, 9.8% PSVT, 37.7% AF and 6.6% AFL). The number of segments with LGE correlated with arrhythmias (p = 0.05 for arrhythmias, p = 0.03 for SVT, p = 0.008 for NSVT and p = 0.042 for PSVT). A cut off of 5 segments involved was a good predictor of arrhythmias (p = 0.002), NSVT (p= 0.006), PSVT (p = 0.024) and AF (p = 0.0029). For SVT the best cut off was 9 (p = 0.003). Considering the LGE location, we found an association between the segments involved and the occurrence of different arrhythmias (table 1). Conclusion Supraventricular and ventricular Arrhythmias are frequent in patients with HCM, with the most frequents being NSVT and AF. The LGE burden (&amp;gt;5 segments) and location (median inferior IVS, median anterior IVS, median anterior wall, basal anterior IVS, apical anterior wall, median inferior wall, apical anterior wall and basal inferior IVS) were correlated with arrhythmic events. Abstract Figure.

Surgery for severe congenital heart diseases in children from developing nations

BackgroundChildren with severe congenital heart disease (CHD) are rarely treated in developing countries and have very little to no chance to survive in their local environment. Mécénat Chirurgie Cardiaque (MCC) flies to France children with CHD from developing countries. This report focuses on the early, mid, and late outcomes of 531 children with severe CHD sent to MCC for surgery from 1996 to 2019. MethodsThe inclusion criteria were based on diagnosis and not on procedure. MCC is present in 66 countries and has developed a robust staff, including 12 permanent employees and 700 volunteers, with 350 host families based in France, 120 local correspondents, and 100 local physicians. Since 1996, MCC has organized a basic training of local pediatric cardiologists yearly, offering a free 1-month training course. Over time, MCC could count on a pool of doctors trained in basic pediatric cardiology. Flights were secured by the Aviation Sans Frontieres Foundation. Nine French centers performed the surgeries. A robust follow-up was conducted in all the nations where MCC operates. ResultsThe most frequent pathologies were single ventricle (n = 126), double-outlet right ventricle (n = 116), pulmonary atresia with ventricular septal defect (n = 68), transposition of the great arteries with ventricular septal defect and transposition of the great arteries with intact ventricular septum (n = 61), arterial trunk (n = 39), transposition of the great arteries with ventricular septal defect and left ventricle outflow tract obstruction (n = 35), complete atrioventricular septal defect (n = 18), congenitally corrected transposition of the great arteries (n = 16), and so on. The median age was 5.4 years (range, 1 month-26 years). The mean perioperative mortality was 5.5% (29 out of 531) (95% confidence limit, 3.5%-7.4%). The follow-up was 91.3%, with a mean follow-up of 5.1 years. The global actuarial survival at 5, 10, and 15 years was, respectively, 85%, 83%, and 74%. There was a significant higher late mortality for patients surviving only with a Blalock-Taussig shunt (P = .001). ConclusionsOperating on 531 children with severe CHD from developing nations was achieved with satisfactory early and long-term results. Children with severe CHD are rarely operated on in developing nations. Programs like MCC's offer a viable option to save these children born with severe CHD.

Hypertrophic cardiomyopathy with moderate septal thickness and mitral regurgitation: long-term surgical results.

The aim of this study was to assess the long-term outcomes of different surgical strategies in patients with hypertrophic obstructive cardiomyopathy (HOCM) with septal thickness ≤18 mm and systolic anterior motion (SAM)-related moderate-to-severe mitral regurgitation (MR). Seventy-six HOCM patients with septal thickness 17 [16; 18] mm, resting left ventricle outflow tract gradient 60 [41; 85] mmHg and SAM-related MR ≥2+/4+, underwent septal myectomy alone (54%) or mitral valve (MV) surgery ± myectomy (46%). No hospital death and no ventricular septal defect occurred. Patients undergoing MV surgery ± myectomy had longer cardiopulmonary bypass and X-clamp times (77 [60-106] vs 51 [44-62] min, P < 0.001 and 56 [45-77] vs 32 [28-41] min, P < 0.001) and higher incidence of low output syndrome (11% vs 0%, P = 0.04). Follow-up was 98.6% complete, median 8 years [3-11]. There were no statistically significant differences in overall survival (P = 0.069) with survival rates at 9 years of 96 ± 4% in the myectomy alone group and 81 ± 8% in the MV surgery ± myectomy one. At 9 years, cumulative incidence function of cardiac death was 12 ± 6% in the MV surgery ± myectomy group vs 0% in the myectomy one, P = 0.06. Multivariable analysis identified age and previous septal alcoholization as predictors of cardiac death (hazard ratio (HR) = 1.1, 95% confidence interval (CI) 1.0-1.1, P = 0.004 and HR = 2.9, 95% CI 1.0-8.3, P = 0.042). The 9-year cumulative incidence function of recurrence of MR ≥2+, with death as competing risk, was 3 ± 2.8% in the MV surgery ± myectomy group vs 25 ± 6.9% in the myectomy one, P = 0.005. In HOCM patients with moderate septal thickness and SAM-related MR, as the degree of septal hypertrophy decreases, addressing the abnormalities of the MV apparatus may become necessary to provide a durable resolution of left ventricle outflow tract obstruction and SAM-related MR. However, performing myectomy alone, whenever possible, seems to be associated to a better postoperative course and a trend towards lower cardiac mortality at follow-up, despite a higher rate of residual moderate MR.

Bovine monocusp in surgery for tetralogy of Fallot

Aim. To evaluate the functionality of a bovine monocusp in surgery for tetralogy of Fallot.Methods. We analyzed the results of surgical treatment in the early postoperative period in eight infants and one year after surgery in seven infants (age range, 0–12 months; mean weight = 7.5 ± 2.3 kg). We used echocardiography to evaluate the residual pressure gradient between the right ventricle and the pulmonary artery, the degree of regurgitation on the pulmonary valve after surgery and to calculate Z-score and the fibrous ring diameter of the pulmonary valve in the late follow-up period. The degree of insufficiency was assessed by the width and depth of the regurgitation flow using the color Doppler mode.Results. The pressure gradient on the eve of discharge did not exceed 25 mmHg for any patient. Pulmonary valve regurgitation was mild in four patients and moderate in the remaining patients. Seven children passed the observation one year after surgery. In one case, we discovered a right ventricle outflow tract obstruction. In other cases, the systolic pressure gradient did not exceed 25 mmHg. Four patients in the late follow-up period had severe valve regurgitation. Despite such valve insufficiency, the leaflet mobility in the bovine jugular vein patch was normal.Conclusion. Early postoperative results for reconstructive surgery to repair tetralogy of Fallot with a bovine monocusp were promising. However, long-term results are comparable with those using other materials. The mobility of the native leaflet remains full even a year after surgery. Consequently, we have positive prospects in material improvement for making patches with native monocusps. Received 14 May 2020. Revised 8 June 2020. Accepted 10 June 2020. Funding: The study did not have sponsorship. Conflict of interest: Authors declare no conflict of interest. Author contributionsConception and study design: A.A. Svobodov, E.G. Levchenko, V.T. KostavaData collection and analysis: G.S. Netalieva, M.V. ZelivyanskayaStatistical analysis: E.G. Levchenko, M.V. ZelivyanskayaDrafting the article: A.A. Svobodov, V.S. RasumovskyCritical revision of the article: V.S. RasumovskyFinal approval of the version to be published: A.A. Svobodov, E.G. Levchenko, G.S. Netalieva, V.T. Kostava, M.V. Zelivyanskaya, V.S. Rasumovsky

Elabela: A Novel Biomarker for Right Ventricular Pressure Overload in Children With Pulmonary Stenosis or Pulmonary Atresia With Intact Ventricular Septum.

Background: Assessing right ventricular overload in children is challenging. We conducted this study involving children with pulmonary valvular stenosis (PS) or pulmonary atresia with intact ventricular septum (PA/IVS) to evaluate the potential of a new endogenous ligand of apelin receptor, Elabela (ELA), as a potential biomarker for right heart overload.Methods: In this prospective cohort study, a total of 118 congenital heart diseases patients with right ventricle outflow tract obstruction were recruited from 2018 to 2019. Among them, 44 isolated PS and 7 PA/IVS patients were selected. Their venous blood was collected, and all patients underwent an echocardiographic examination. Among them, post-operative blood was collected from 24 patients with PS after percutaneous balloon pulmonary valvuloplasty. The plasma ELA concentration was measured using enzyme-linked immunosorbent assay.Results: The ELA was significantly associated with the peak transvalvular pulmonary gradient (r = −0.62; p = 0.02), thus reflecting the severity of PS or PA/IVS. The ELA significantly increased at 3 days after intervention, when mechanical obstruction of the right outflow tract was relieved. Based on the receiver-operator characteristic curve results, ELA could be a risk factor for duct dependence in patients with critical PS or PA/IVS who are younger than 6 months (AUC: 0.82).Conclusion: ELA concentration and severity of PS or PA/IVS had a significant negative correlation, indicating that ELA might be a novel biomarker for right ventricular afterload and reflect the immediate pressure changes in the right heart. Furthermore, ELA could predict duct-dependency in PS and PA/IVS patients, as valuable as classical echocardiographic indexes.

Acute DeBakey Type II Dissection Mimics Left Ventricle Outflow Tract Obstruction

A 40-year-old man with hypertension and obesity (body mass index, 50.4 kg/m2) was referred to our institution after an evaluation for symptoms of heart failure (worsening shortness of breath for 2 weeks). Computed tomography with contrast revealed subacute DeBakey type II aortic dissection originating at the sinotubular junction with an enlarged thrombus occupying the false lumen (Figure 1, red arrow) and obstructing blood flow through the stenotic supravalvular aorta.

Discrete Left Ventricle Outflow Tract Obstruction in Children: Incidence and Predictors of Recurrence. A Multi-Center Study.

ObjectivesThe purpose of this study is to measure the incidence of recurrence of discrete subaortic stenosis (DSS) after primary resection in two major cardiac centers in Saudi Arabia and to identify risk factors associated with recurrence.MethodsData on 234 patients who were diagnosed with DSS and underwent surgical resection between 1999 and 2018 were retrospectively reviewed. Patient demographics as well as echocardiographic, surgical, and pathological data were compared between patients with recurrence and non-recurrence.ResultsThe overall recurrence incidence after primary resection was 44.87% (N = 105). Most patients were male (59%). The median age at the 1st operation was 60 months (range 3 months to 133 months). The presence of aortic stenosis at the time of diagnosis was significantly associated with recurrence (p-value = 0.002). The overall median peak gradient in which the primary resection was indicated is 60 mmHg (range 11 to 152 mmHg). The median peak gradient pre-operation and post-operation were significantly higher for the recurrence group (p-value=0.018 and p<0.001, respectively). We used univariate and multivariate analysis and controlled for the follow-up time, but there were no significant independent predictors of recurrence.ConclusionThe recurrence rate of DSS after the primary resection is relatively high in this study. Further prospective studies are needed to draw a definite conclusion on risk factors for recurrence after primary resection.

Left Ventricular Outflow Obstruction After TAVR Due to Systolic Anterior Motion Successfully Treated With Cardiac Pacing

Abstract 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

Correlation of miR-146a-5p plasma levels and rs2910164 polymorphism with left ventricle outflow tract obstruction in hypertrophic cardiomyopathy

Hypertrophic cardiomyopathy (HCM) is a genetic disease of the myocardium that is characterized by phenotypic variability among patients. miR-146a is a small non-coding RNA that is well known for its role in inflammation and myocardial hypertrophy. The aim of this study is to evaluate the role of miR-146a as a candidate genetic factor influencing HCM phenotype. In this study, 140 HCM patients and 112 control individuals were genotyped for the rs2910164 single nucleotide polymorphism (SNP) in the MIR146A gene; using this data, the correlation between different genotypes and clinical features of the disease were determined. Additionally, plasma levels of miR-146a-5p were determined in 50 HCM patients and 30 control individuals by using qPCR. The incidence of GC and CC genotypes were significantly lower in HCM patients (odds ratio (OR)=0.5 [0.3-0.8], p=0.007). The GC/CC genotypes in the dominant genetic model positively correlated with the presence of left ventricle outflow tract (LVOT) obstruction (OR=2.3 [1.2-4.7] and p=0.018), a higher left ventricle mass index (118±47g/m2 vs 92±42g/m2 and p=0.02), and increased left ventricle end-diastolic diameter (4.66±0.64cm vs 4.39±0.7cm and p=0.026). Atrial fibrillation was significantly higher in patients homozygous for the C allele (OR=10.6 [2-55], p=0.003). Interestingly, the plasma levels of miR-146a-5p were significantly increased in HCM patients with LVOT obstruction. Our findings indicate that the C allele of the rs2910164 SNP might be under negative selection in HCM patients. Additionally, plasma levels of miR-146a-5p and GC/CC genotypes are indicative of the obstructive phenotype in HCM patients.

P173 When the heart doesn"t want to grow up

Abstract Background Blood cysts of heart valves are uncommon and, usually, incidental findings in pediatric population. Rarely seen in adults, blood cysts are normally asymptomatic, even though several cases of embolization, valvular disfunction and left ventricle outflow tract (LVOT) obstruction have been described. Clinical Presentation. We report the case of a 23 year-old woman, with history of a small nodular mass of the mitral valve, found on a previous cardiac magnetic resonance (CMR) at the age of thirteen, who was admitted to our emergency department complaining atypical chest pain. She denied shortness of breath, syncope, tachycardia and fever. She was hemodynamically stable and a fast regression of symptoms was observed. The physical examination was unremarkable. Both the electrocardiogram (ECG) and blood tests were normal. A chest radiography showed normal heart size, without other abnormalities. After having ruled out an acute coronary syndrome, the patient was discharged with the indication to undergo further cardiological assessments. Diagnostic techniques and their most important findings. Therefore, a trans-thoracic echocardiogram was performed revealing normal biventricular function, no aortic root dilation, no pericardial effusion, no valvular regurgitation or stenosis. A round (7x11 mm) mass with hyperechogenic borders and hypoechogenic content was detected on the ventricular side of the anterior mitral valve leaflet consistent with a valvular blood cyst (figure 1: a,b,c). Only trivial mitral valve regurgitation without LVOT obstruction was present. The 3D echo reconstruction allowed to confirm the suspicion of blood cyst and detect a cleft of the posterior mitral valve leaflet, located in the P2 scallop (figure 1: d,e). A repeated CMR showed no contrast enhancement of the mass. Due to the clinical stability and the absence of complications, the patient was suggested to continue an echocardiographic follow-up. Discussion. Cardiac blood cysts are a rare condition with uncertain origin, usually found in infants in the first six months of life. A natural regression after that age has been described, making this condition very unusual in adults. Despite its benign features, when persistent beyond the childhood, the blood cyst can grow and reach even huge dimensions, potentially impairing the valvular function and/or causing LVOT obstruction. Another potential complication of blood cysts may be the arterial embolization. In all these situations surgical resection must be considered. In our patient, the contribution of trans-thoracic echocardiogram and in particular the 3D reconstruction, were pivotal to address the correct diagnosis. Abstract P173 Figure 1

113 Echocardiographic assessment of right ventricular outflow tract obstruction in hypertrophic cardiomyopathy - efficacy of dual chamber pacing in reducing of gradient

Abstract Hypertrophy of the right ventricle (RV) in the course of hypertrophic cardiomyopathy (HCM) is found in 30-60% of cases, with the possibility of a right ventricular outflow tract obstruction (RVOTO), obstruction in the apex or the middle part of the right ventricle. A patient, aged 41, admitted due to limitation of exercise tolerance, effort dyspnea, presyncope. In an echocardiogram, interventricular septum (IVS) hypertrophy was observed up to 2.0 cm; normal size of the heart cavities; normal left ventricular systolic function (EF-70%). A color doppler mapping detected the zone of flow acceleraction and turbulent flow in right ventricular outflow tract (RVOT), next a spectral doppler examination showed the RVOT obstruction with a maximal gradient of 64 mmHg. Because of the suboptimal echocardiographic imaging, a heart CT scan was performed, revealing the features of left ventricular hypertrophy, most severe at the base and the medium part of IVS (up to 25 mm). Asymmetric hypertrophy of the middle portion of the right ventricle and right ventricular outflow tract obstruction was also observed. A hemodynamic study confirmed the presence of gradient in RVOT, up to 40 mmHg. Holter electrocardiogram recorded an episode of non-sustained ventricular tachycardia. Taking into account the clinical picture, the family history of the disease, and calculated HCM Risk SCD (7.55%), the decision was made to implant a dual chamber cardioverter defibrillator. The defibrillator electrode was fixed at the apex of the right ventricle. A short AV delay was programmed for prevalent right ventricular stimulation (AV delay 100 ms), resulting in 99.6% ventricular stimulation. The control echocardiogram showed a reduction in the maximum gradient in RVOT to 24 mmHg. In addition, the patient was treated with a beta-blocker. To sum up, in the case of HCM we should always examine the RV with color and spectral doppler to exclude potential narrowing in RV. Constant AV sequential stimulation with a short AV delay is a recognized method that can be considered in symptomatic adult HCM patients with a left ventricular outflow tract obstruction. In the case described here, the above mentioned method proved effective in the significant reduction of the gradient in the right ventricular outflow tract. Abstract 113 Figure. gradient in RVOT

Anterior mitral leaflet length and mitral annulus diameter impact the echocardiographic outcome after isolated myectomy

BackgroundMyectomy remains the standard surgical treatment of patients with hypertrophic cardiomyopathy (HOCM). New surgical methods developed in the last decades mainly address the mitral valve and are controversial because of their conflicting assumptions. This study assesses the influence of anterior mitral valve leaflet (AML) length and the anterior-posterior diameter of the mitral annulus (MAD) on dynamic left ventricle outflow tract obstruction and mitral regurgitation (MR) after extended myectomy.MethodsWe retrospectively analysed the transthoracic echocardiograms (TTE) of 36 patients. AML length and MAD were obtained from TTE performed before the operation. The greatest maximal left ventricle outflow tract (LVOT) gradient and MR registered in follow-up were analysed. After surgery, patients were divided into two groups; those with moderate or milder MR and/or an LVOT gradient < 30 mmHg (responders), and those with more than moderate MR and/or an LVOT gradient ≥30 mmHg (non-responders).ResultsPatients in responders group had significantly longer AML: 32.3 ± 2.3 mm vs 30.0 ± 3.8 mm (p = 0.03) [parasternal long axis view – PLAX view], 25.9 ± 2.3 mm vs 23.5 ± 2.7 mm (p = 0.008) [four chamber view - 4CH view] and larger anterior-posterior mitral annulus diameter 28.1 ± 2.8 mm vs 25.4 ± 3.2 mm (p = 0.011) than those in non-responders group. Among all analysed patients longer anterior mitral leaflet was correlated with lower postoperative LVOT gradient when measured in PLAX view (p = 0.02) and lower degree of MR due to systolic anterior motion (SAM) when measured in 4CH view (p = 0.009). Greater [AML x mitral annulus] ratio correlated with lower postoperative LVOT gradient in both projections: 4CH (p = 0.025), PLAX (p = 0.012). There was significant reduction in NYHA Class after surgery (p = 0.000). There were no significant differences in NYHA class after surgery (p = 0.633) neither in NYHA class reduction (p = 0.475) between patients divided into responders and non-responders group according to echocardiographic parameters.ConclusionsPatients with a longer AML and a greater diameter of the mitral annulus are less likely to have mitral regurgitation due to residual SAM and increased LVOT gradient after an extended myectomy. Division of patients according to echocardiographic criteria into responders and non-responders was not in concordance with clinical improvement.Trial registrationRetrospective study. Approved by ethics committee (IK-NPIA-0021-21/1763/19) at 16.01.2019.

Anesthetic management during endocardial radiofrequency ablation of septal hypertrophy – a case report

Background: Hypertrophic cardiomyopathy (HCM) is a genetic disorder present in up to 1/500 individuals, about 20–30% of them presenting with hypertrophic obstructive cardiomyopathy (HOCM) due to left ventricle outflow tract obstruction. This is an important cause of sudden cardiac death. Endocardial radiofrequency ablation of septal hypertrophy (ERASH) might be an attractive treatment for HOCM, particularly in patients who do not respond to transcoronary alcohol septal ablation (TASA).Aim: To describe technical aspects related to the procedure and anesthetic management of an ERASH case.Case report: A 64-year-old woman with HOCM was scheduled for ERASH. She had worsening of dyspnea on exertion and generalized fatigue for the previous weeks after previous surgical myomectomy about 6 months ago. The anatomy was unfavorable for TASA and the patient was not willing to undergo another surgery. Preoperative transthoracic echocardiography (TTE) showed asymmetric mid-septal hypertrophy, systolic anterior motion with septal contact and left ventricular outflow tract maximum gradient of 68 mmHg at rest and 105 mmHg after the Valsalva maneuver. General anesthesia was performed. Pulse pressure variation, echocardiography parameters and passive leg raising test where used to guide fluid therapy. At the end of the procedure, analgesia was provided together with prophylaxis of nausea and vomiting. Extubation was uneventful and the patient was transported to the intensive care unit eupneic and hemodynamically stable. On the fourth postoperative day, TTE showed septal hypocontractility and maximum gradient reduction of 33% at rest (68 mmHg to 45 mmHg) and 31% after the Valsalva maneuver (105 mmHg to 73 mmHg). The patient was discharged from hospital at the sixth postoperative day. One month later, she reported progressive improvement of symptoms and expressed satisfaction with the results.Conclusion: Better understanding of the pathophysiology and natural history of HCM has enabled earlier diagnosis, as well as a more adequate therapeutic approach. Anesthesiologists should be aware of the pathophysiology of HOCM and must be prepared to anticipate the hemodynamic changes and cardiovascular instability that such patients may show perioperatively. ERASH is a promising therapeutic modality increasingly used for HOCM and anesthesiologists should become more familiar with it.

Usefulness of 3D printed models of congenital heart diseases as educational tools for medical students

Multiple studies have demonstrated the feasibility and accuracy of 3D printed models in congenital heart diseases. These models seem enhance conceptual 3D understanding of complex anatomy. Our aim was to evaluate the usefulness of these models as a teaching tool for medical students to improve learning of congenital heart diseases. During the education sessions of left ventricle outflow tract obstruction (LVOTO), students from 5th year of medicine were randomized in two groups. Each group (n = 118) attended a 60 minutes lecture of LVOTO. All students answered objective questions for pre- and post-lecture knowledge score evaluation, in addition to a subjective post-lecture survey questions regarding students comfort level with the subject. During the lecture, 3D printed models of different types of LVOTO were presented and analyzed by the students in the test group. Knowledge acquisition was evaluated by comparing pre and post-lecture knowledge score. Student's satisfaction and self-efficacy ratings were evaluated by the subjective post-lecture survey. The datas were analyzed and compared between the two groups using paired t-test. There was no difference in pre-lecture objective test score between the two groups (score 8.32/14 vs. 8.35/14). After the lecture both groups improved their knowledge objective score, but was significantly higher in the test group than that of the control group (score 12.60/14 vs. 11.20/14 respectively P = 0.04). Students in the test group were more satisfied with theirs understanding of the diagnosis (P = 0.03) and treatment (P < 0.01) of LVOTO pathologies. This preliminary study demonstrates the feasibility and the usefulness of 3D printed models as educational tools of congenital heart diseases for medical students.

A case of myocarditis assessed by multi-parametric cardiovascular magnetic resonance

该患者临床表现为胸闷和胸痛，起病前有发热病史，辅助检查提示炎症指标和心肌坏死标志物升高，考虑心肌炎可能性大。通过应用磁共振定量组织标记技术结合传统路易斯湖诊断标准，诊断该患者为心肌炎。予大剂量激素及支持治疗后，患者病情好转。该病例表明，心脏磁共振多序列成像在心肌炎的诊断和指导治疗方面具有重要意义。.

Usefulness of 3D printed models of congenital heart diseases as educational tools for medical students

Multiple studies have demonstrated the feasibility and accuracy of 3D printed models in the field of congenital heart diseases. These models seem enhance conceptual 3D understanding of complex anatomy. Our aim was to evaluate the usefulness of these models as a teaching tool for medical students to improve learning of congenital heart diseases. During the education sessions of left ventricle outflow tract obstruction (LVOTO), students from 5th year of medicine were randomized in two groups. Each group ( n = 118) attended a 60-minute lecture of LVOTO. All students answered objective questions for pre- and post-lecture knowledge score evaluation, in addition to a subjective post-lecture survey questions regarding students’ comfort level with the subject. During the lecture, 3D printed models of different types of LVOTO were presented and analyzed by the students in the test group ( Fig. 1 ). Knowledge acquisition was evaluated by comparing pre and post-lecture knowledge score. Student's satisfaction and self-efficacy ratings were evaluated by the subjective post-lecture survey. The datas were analyzed and compared between the two groups using paired t -test. There was no difference in pre-lecture objective test score between the two groups (score 8.32/14 vs. 8.35/14). After the lecture, both groups improved their knowledge objective score, but was significantly higher in the test group than that of the control group (score 12.60/14 vs. 11.20/14 respectively P = 0.04). Students in the test group were more satisfied with theirs understanding of the diagnosis ( P = 0.03) and treatment ( P < 0.01) of LVOTO pathologies. This preliminary study demonstrates the feasibility and the usefulness of 3D printed models as educational tools of congenital heart diseases for medical students.