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Background: Left ventricular outflow tract stenosis and atrioventricular valve regurgitation are often problems encountered in adulthood after complete atrioventricular septal defect repair. The surgical approach and indications for managing long-term outcomes such as left atrioventricular valve regurgitation and left ventricular outflow tract stenosis after complete atrioventricular septal defect repair have been discussed., Case Presentation: A 23-year-old woman with intellectual disability was diagnosed with complete atrioventricular septal defect and underwent two-patch repair without cleft closure in childhood. Follow-up examination in adulthood demonstrated moderate left-sided atrioventricular valve regurgitation and left ventricular outflow tract stenosis with a circumferential ridge (peak velocity, 3.7 m/s; pressure gradient, 54 mmHg). Intraoperative findings showed a circumferential ridge under the aortic valve, and we removed the ridge. In addition, a cleft was present at the anterior leaflet, and we completely closed the cleft. Anticoagulation therapy was not initiated, and no embolic complications occurred. Follow-up echocardiography demonstrated no ridge under the aortic valve and only mild-range left AVVR., Conclusions: We successfully performed surgical treatment without valve replacement or anticoagulation therapy in a patient with poor medical compliance. Delayed reoperation leads to degeneration of the valve structure and makes more difficult to repair. Atrioventricular valve regurgitation should be evaluated in combination with based on the etiology of the regurgitation especially cleft related or not, in addition to the dilatation annulus, cleft size, and depth of the leaflet coaptation depth, and associated other valve diseases., (© 2024. The Author(s).)

Background: Latent left ventricular outflow tract obstruction (LVOTO) is an important cause of symptoms in patients with hypertrophic cardiomyopathy (HCM) but can be challenging to provoke., Objectives and Methods: To examine the value of postprandial resting and stress echocardiography and utilization of invasive or enhanced drug therapies (surgical myectomy, alcohol septal ablation, disopyramide, and mavacamten) in patients with postprandial LVOTO. Consecutive HCM patients without LVOTO underwent routine and postprandial echocardiography at rest, with provocation (Valsalva and standing) and after symptom-limited treadmill stress., Results: Among 252 patients (mean age, 58 years, 39% women), postprandial LVOT gradients were higher compared with routine echocardiography at rest (median, 9.0 [0-38.0] vs 0 [0-14.0] mm Hg; P < .0001) and with provocation (18.5 [0-70.3] vs 1.5 [0-41.0] mm Hg; P < .0001). Postprandial exercise stress echocardiogram (PPXSE) gradients were higher in a subset of 44 patients who underwent both postprandial and fasting stress echocardiography (47.0 [5.3-81.0] vs 17.5 [0-46.0] mm Hg; P < .0001). In total, 49 (19.5%) patients achieved the ≥50 mm Hg threshold under routine conditions (rest/provocation); 90 (35.7%) additional patients achieved postprandial gradients ≥50 mm Hg (rest/provocation/exercise), 38 (15.1%) with PPXSE alone. A total of 71 patients were treated with 91 invasive or enhanced drug therapies, 32 (45.1%) of whom had gradients ≥50 mm Hg only after eating (rest/provocation) and 8 (11.3%) only with PPXSE, with symptom relief in the majority., Conclusions: Postprandial echocardiography was useful at unmasking LVOTO in more than one-third of patients who did not have high gradients otherwise. Eating before echocardiography is a powerful provocative tool in the evaluation of patients with HCM., Competing Interests: Conflicts of Interest D.M. reports consulting fees from Sanofi, Tenaya Therapeutics, and Chiesi; M.R. reports consulting fees from Bristol Myers Squibb; Mu.S reports consulting fees from Siemens and speaking fees from Abbott, Boston Scientific, Medtronic, and Philips; Ma.S. reports consulting fees from Pfizer and serving as consultant for Cytokinetics. All other authors declare that they have no conflicts of interest., (Copyright © 2024 American Society of Echocardiography. Published by Elsevier Inc. All rights reserved.)

Background: Studies that have assessed the Rastelli and Nikaidoh operations for transposition of the great arteries (TGA) with obstructed left ventricular outflow tract obstruction (LVOTO) have not fully evaluated the anatomic drivers that may contribute to surgical selection. We present our procedural selection process for optimizing outcomes of complex TGA in the modern era., Methods: This is a single-center, retrospective study that included pediatric patients who underwent either a Nikaidoh or Rastelli operation for the treatment of TGA-LVOTO, congenitally corrected TGA-LVOTO, or double-outlet right ventricle TGA type-LVOTO from June 2004 to June 2021., Results: There were 34 patients stratified by Nikaidoh (n = 16) or Rastelli (n = 18) operation. The incidence of all postoperative complications and mortality was low, and the incidence of complications between the groups was similar. Patients were more likely to have undergone a Nikaidoh than a Rastelli if they had a pulmonary annulus >5 mm (87.5% vs 11.1%), anteriorly/posteriorly oriented great vessels (88% vs 8%), remote (80% vs 11%) or restrictive (75% vs 6%) ventricular septal defect, and right ventricular hypoplasia (50% vs 0%; all, P < .05). The resulting rates of reoperation were similar between the groups (44.0% vs 37.5%; P = .24) and largely composed of conduit replacements in the Rastelli patients and valvular repairs or replacements in the Nikaidoh group. Rates of catheter-based interventions were also similar., Conclusions: These findings suggest that for the optimal treatment of conotruncal anomalies with discordant ventriculoarterial connections, procedural selection should be based on pathoanatomic criteria that can ensure patients undergo the operation most suited to their anatomy., (Copyright © 2024 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

More than half of symptomatic patients with hypertrophic cardiomyopathy (HCM) have left ventricular outflow tract (LVOT) obstruction. Septal reduction therapy by septal myectomy can dramatically relieve exertional dyspnea, chest pain, and presyncope in properly selected patients and is an important management pathway for many patients. The distribution and degree of hypertrophy in patients with obstructive HCM are variable and, as discussed in this review, can influence clinical manifestations of the disease and surgical management. Subaortic septal hypertrophy is the most common phenotype of obstructive HCM associated with LVOT obstruction, but midventricular obstruction and apical hypertrophy may occur in isolation or in conjunction with subaortic septal hypertrophy. In many comprehensive HCM centers, transaortic septal myectomy is the preferred method of septal reduction therapy for symptomatic patients with obstructive HCM. Early surgical approaches aimed at alleviating left LVOT obstruction were hampered by a lack of understanding of the anatomy and pathophysiology of obstructive HCM. With the advent of Doppler echocardiography and, more recently, cardiac magnetic resonance imaging, surgeons can precisely assess the location and degree of obstruction, left ventricular size and function, and morphology and function of the mitral valve. This review discusses the current understanding of the role of septal myectomy in the management of patients with HCM and details contemporary operative methods., (Copyright © 2024 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Accessory mitral valve tissue is a rare congenital cardiac anomaly that is typically discovered incidentally during echocardiographic evaluation prompted by an asymptomatic murmur. This pathology has characteristic echocardiographic elements and is usually associated with other CHD. The decision to perform surgical resection depends on factors such as the degree of obstruction, presence of symptoms, presence of other CHDs, and risk of thrombosis. The researchers hereby present a case of an asymptomatic paediatric patient with accessory mitral valve tissue that produced left ventricular outflow tract obstruction.

Takotsubo cardiomyopathy (TCM) leads to serious left ventricular outflow tract (LVOT) obstruction with cardiogenic shock in 6%-20% of cases. The onset of LVOT obstruction, coupled with mitral regurgitation resulting from systolic anterior motion of mitral valve leaflets, can lead to haemodynamic instability in addition to severely impaired systolic function. We describe three patients who experienced chest discomfort following emotional stress. These patients displayed pronounced abnormalities on ECGs, insignificant obstructive coronary disease and haemodynamic instability due to LVOT obstruction. The infusion of landiolol, a short-acting beta blocker, was effective in releasing the gradient. Dynamic outflow obstruction is the major predictor of haemodynamic collapse. We suggested that an early identification of this complication in hypotensive patients with suspected TCM could be of utmost importance to optimise the therapeutic approach in the acute setting., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2023. No commercial re-use. See rights and permissions. Published by BMJ.)

Aims: Obstructive hypertrophic cardiomyopathy (oHCM) is characterized by dynamic obstruction of the left ventricular (LV) outflow tract (LVOT). Although this may be mediated by interplay between the hypertrophied septal wall, systolic anterior motion of the mitral valve, and papillary muscle abnormalities, the mechanistic role of LV shape is still not fully understood. This study sought to identify the LV end-diastolic morphology underpinning oHCM., Methods and Results: Cardiovascular magnetic resonance images from 2398 HCM individuals were obtained as part of the NHLBI HCM Registry. Three-dimensional LV models were constructed and used, together with a principal component analysis, to build a statistical shape model capturing shape variations. A set of linear discriminant axes were built to define and quantify (Z-scores) the characteristic LV morphology associated with LVOT obstruction (LVOTO) under different physiological conditions and the relationship between LV phenotype and genotype. The LV remodelling pattern in oHCM consisted not only of basal septal hypertrophy but a combination with LV lengthening, apical dilatation, and LVOT inward remodelling. Salient differences were observed between obstructive cases at rest and stress. Genotype negative cases showed a tendency towards more obstructive phenotypes both at rest and stress., Conclusions: LV anatomy underpinning oHCM consists of basal septal hypertrophy, apical dilatation, LV lengthening, and LVOT inward remodelling. Differences between oHCM cases at rest and stress, as well as the relationship between LV phenotype and genotype, suggest different mechanisms for LVOTO. Proposed Z-scores render an opportunity of redefining management strategies based on the relationship between LV anatomy and LVOTO., Competing Interests: Conflict of interest: P.L. sits at the scientific advisory board of Ultromics. The remaining authors have nothing to disclose., (© The Author(s) 2022. Published by Oxford University Press on behalf of the European Society of Cardiology.)

Objective: Mitral regurgitation (MR) represents an important feature in patients with hypertrophic cardiomyopathy (HCM) due to left ventricular outflow tract (LVOT) obstruction and mitral valve systolic anterior motion (SAM). Mitral valve anatomical variants associated with HCM also contribute to the severity of MR. The aim of this study is to evaluate MR severity and its correlation with different parameters in patients with HCM using cardiac magnetic resonance imaging (cMRI)., Patients and Methods: 130 patients with HCM underwent cMRI. Parameters assessed for the quantification of MR severity were mitral regurgitation volume (MRV) and mitral regurgitation fraction (MRF). cMRI was also used to characterize LV function, left atrium volume (LAV) index, filling pressures and structural abnormalities associated with HCM, all in correlation to MR., Results: Patients with HCM had mild (26.9%), moderate (52.3%) or severe (20.7%) MR. Most relevant parameters related to MR severity were MRV and MRF; other parameters with strong correlation with MR were LAV index and E/E' ratio, both increasing with its severity. Patients with LVOT obstruction had more severe MR (70.3%), 79% of them due to SAM. LV ejection fraction (LVEF) increased proportionally with the severity of MR, while LV strain (LAS) was inversely correlated with it. Independent predictors for quantifying the severity of MR, after the adjustment for covariates, were MRV, MRF, SAM, LAV index and E/E'., Conclusions: cMRI can accurately assess MR in patients with HCM, especially by using novel indicators, MRV and MRF respectively, along with LAV index and E/E' ratio. Severe MR, due to SAM, is more frequent in the obstructive form of HCM (HOCM). Also, the severity of MR is significantly associated with significantly associated with MRV, MRF, LAV index and E/E' ratio.

Purpose: Transposition of great arteries with intact ventricular septum and left ventricular outflow tract obstruction (TGA + IVS + LVOTO) is uncommon. We reviewed operations performed in patients with TGA + IVS + LVOTO in the European Congenital Heart Surgeons Association Congenital Database (ECHSA-CD)., Methods: All 109 patients with a diagnosis of TGA + IVS + LVOTO in ECHSA-CD who underwent cardiac surgery during a 21-year period (01/2000-02/2021, inclusive) were included. Preoperative variables, operative data, and postoperative outcomes were collected., Results: These 109 patients underwent 176 operations, including 37 (21.0%) arterial switch operations (ASO), 26 (14.2%) modified Blalock-Taussig-Thomas shunts (MBTTS), 11 (6.2%) Rastelli operations, and 13 (7.3%) other palliative operations (8 superior cavopulmonary anastomosis[es], 4 Fontan, and 1 other palliative procedure). Of 37 patients undergoing ASO, 22 had a concomitant procedure.There were 68 (38.6%) reoperations, including 11 pacemaker procedures and 8 conduit operations. After a systemic-to-pulmonary artery shunt, reoperations included shunt reoperation (n = 4), Rastelli (n = 4), and superior cavopulmonary anastomosis (n = 3).Overall Operative Mortality was 8.2% (9 deaths), including three following ASO, two following "Nikaidoh, Kawashima, or LV-PA conduit" procedures, and two following Rastelli. Postoperative complications occurred after 36 operations (20.4%). The most common complications were delayed sternal closure (n = 11), postoperative respiratory insufficiency requiring mechanical ventilation >7 days (n = 9), and renal failure requiring temporary dialysis (n = 8)., Conclusion: TGA + IVS + LVOTO is rare (109 patients in ECHSA-CD over 21 years). ASO, MBTTS, and Rastelli are the most common operations performed for TGA + IVS + LVOTO. Larger international studies with long-term follow-up are needed to better define the anatomy of the LVOTO and to determine the optimal surgical strategy.

Background: 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., Methods: Retrospective chart review analysis of all patients who had Yasui procedure (2008-2020) comparing midterm outcome of one versus two stage repair., Results: 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., Conclusion: 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.

Surgical myectomy remains the time-honored primary treatment for hypertrophic cardiomyopathy patients with drug refractory limiting symptoms due to LV outflow obstruction. Based on &gt;50 years experience, surgery reliably reverses disabling heart failure by permanently abolishing mechanical outflow impedance and mitral regurgitation, with normalization of LV pressures and preserved systolic function. A consortium of 10 international currently active myectomy centers report about 11,000 operations, increasing significantly in number over the most recent 15 years. Performed in experienced multidisciplinary institutions, perioperative mortality for myectomy has declined to 0.6%, becoming one of the safest currently performed open-heart procedures. Extended myectomy relieves symptoms in &gt;90% of patients by ≥ 1 NYHA functional class, returning most to normal daily activity, and also with a long-term survival benefit; concomitant Cox-Maze procedure can reduce the number of atrial fibrillation episodes. Surgery, preferably performed in high volume clinical environments, continues to flourish as a guideline-based and preferred high benefit: low treatment risk option for adults and children with drug refractory disabling symptoms from obstruction, despite prior challenges: higher operative mortality/skepticism in 1960s/1970s; dual-chamber pacing in 1990s, alcohol ablation in 2000s, and now introduction of novel negative inotropic drugs potentially useful for symptom management., (Copyright © 2022 The Authors. Published by Elsevier Inc. All rights reserved.)

Severe left outflow tract obstruction (LVOTO) is not always associated with hypoplastic left heart syndrome (HLHS). Aortic valvar atresia or complex LVOTO in the presence of a large ventricular septal defect (VSD) are a rare group of lesions that offer the possibility of biventricular repair. The Yasui procedure is the commonest surgical approach which can be performed as a primary neonatal correction or as a staged procedure with a Norwood followed by a subsequent Rastelli. This article reviews the surgical outcomes and decision-making process. Both strategies are reasonable with the trend toward staged procedure in the setting of the additional interrupted arch, with neonatal survival of > 90% in the modern era and excellent long-term survival. Re-intervention is mostly related to conduit revision and the need for re-operation for LVOTO is rare. Deciding between conventional repair and the Yasui in cases of LVOTO/VSD can be difficult and there are no uniform accepted criteria. In a typical neonate, an aortic annulus < 4.5 mm is generally the limit of acceptability for a conventional repair. In selected cases of LVOTO/VSD, an alternative to the Yasui is the Ross-Konno. Retrospective comparisons between the 2 techniques are difficult due to differing patient characteristics (especially associated with mitral valve disease) but the neonatal Ross has been associated with higher early mortality.

Background: Hypertrophic obstructive cardiomyopathy is a genetic disorder treated with septal reduction therapy, either alcohol septal ablation or septal myectomy (SM). Historically older patients have been presumed to be poor candidates for SM and thus referred directly for alcohol septal ablation in some centers. We reviewed our experience with SM in older patients., Methods: We identified 100 patients at our institution who underwent SM for hypertrophic obstructive cardiomyopathy from 2015 to 2020. Demographic and clinical characteristics and outcomes of patients 65 years or older were compared with patients younger than 65., Results: Sixty-five patients were in the <65 group and 35 patients in the ≥65 group. Both groups had similar preoperative peak stress left ventricular outflow tract gradients (129 mm Hg vs 110 mm Hg, P < .001). Most patients in both groups had moderate to severe mitral regurgitation on preoperative stress echocardiography. The elderly group was more likely to have coronary artery bypass graft as a concomitant procedure (37% vs 8%, P < .001). Only 1 death occurred in the series secondary to a pulmonary embolism. At the 30-day follow-up on stress echocardiography, peak stress gradients were normal in both groups (21 and 20 mm Hg, respectively; P < .001), and 88% of all patients had trace to mild mitral regurgitation., Conclusions: Properly selected older patients can safely undergo SM with excellent outcomes similar to younger patients. Relief of left ventricular outflow tract obstruction and correction of mitral regurgitation are reliably achieved in both groups. Advanced age should not be a strict criteria for selecting septal reduction therapy approach., (Copyright © 2022 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Obesity is highly prevalent in patients with obstructive hypertrophic cardiomyopathy (HCM). In this study, we investigated the impact of body mass index (BMI) in patients undergoing septal myectomy (SM) for obstructive HCM., Methods: We reviewed 2746 patients who underwent transaortic SM for obstructive HCM from February 1993 through September 2018. Patients were stratified into 3 groups based on BMI (normal weight, <25 kg/m 2 ; overweight, 25 to <30 kg/m 2 ; and obese, ≥30 kg/m 2 )., Results: Preoperatively, the median left ventricular outflow tract gradient was 58 mm Hg, and there was no difference in gradients across BMI strata (P = .35). The percentage of obese patients with moderate or greater mitral valve regurgitation was lower (45.8%) compared with normal weight (52.9%) and overweight (55.4%) patients (P < .001). However, patients with a higher BMI were more likely to have New York Heart Association Functional Classification III/IV limitation at presentation (P < .001). After myectomy, anteroseptal thickness (P = .115) and left ventricular outflow tract gradient (P = .210) did not differ between groups. There were 14 (0.5%) deaths within 30 days postoperatively, and the risk was similar across BMI strata (P = .448). Model-estimated changes in average BMI at 10 years postprocedure showed stratum-specific increases ranging from 0.60 to 1.56 kg/m 2 . During a median follow-up of 7.2 years (interquartile range, 3.2-13.3 years), a higher BMI was associated with reduced survival after adjusting for baseline covariates (P = .001)., Conclusions: SM is safe and effective in HCM patients with obesity, but the risk of late death increased with increasing BMI. Attention to risk factor management through weight loss may improve late results after SM., (Copyright © 2022 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

For over 50 years, surgical septal myectomy has been the preferred treatment for drug-refractory heart failure symptoms in obstructive hypertrophic cardiomyopathy (HCM). However, given the relatively youthful adult ages at which HCM surgery is usually performed, it is informative to evaluate longer-term results of myectomy after ≥10 years. We identified 139 consecutive obstructive HCM patients (50 ± 15 years of age; 55% men) who underwent surgical myectomy, 2003 to 2010 at Tufts HCM Center and followed 11.3 ± 2.7 years (range to 17). Operative mortality was low (0.6%) and left ventricular (LV) outflow gradients at rest were reduced from 56 ± 40 mm Hg preoperatively to 1 ± 7 mm Hg postoperatively, durable over the study period, with no patient requiring reoperation for the residual gradient. Over follow-up, 129 of 139 patients (93%) were alive ≥10 years after myectomy, including 17 patients ≥15 years. Of 118 patients with complete long-term clinical follow-up data, 109 (92%) experienced clinical improvement to New York Heart Association classes I or II. In 9 patients (8%) refractory class III/IV symptoms reoccurred 6.6 ± 3.9 years postoperatively, including 4 who ultimately underwent a heart transplant. After myectomy, there were 2 late HCM-related deaths, but none suddenly; notably 6 patients (12%) with prophylactic implantable cardioverter-defibrillators experienced appropriate therapy terminating ventricular tachycardia/ventricular fibrillation after myectomy. Survival following myectomy was 91% at 10 years (95% confidence interval: 85, 96%) not different from the age- and gender-matched general United States population (log-rank p = 0.64). In conclusion, myectomy provides permanent abolition of outflow gradients with reversal of heart failure and highly favorable long-term survival, representing a low-risk:high-benefit option when performed in experienced HCM centers. Myectomy did not protect absolutely against arrhythmic sudden death events, underscoring the importance of risk stratification in operative patients., Competing Interests: Disclosures Dr. M.S. Maron reports a relationship with Cytokinetics Inc. that includes: consulting or advisory; and is a consultant for Imbria Pharmaceuticals and Takeda pharmaceuticals. The other authors have no conflicts of interest to declare., (Copyright © 2021. Published by Elsevier Inc.)

Background: Prestenting right ventricular outflow tracts (RVOTs) before transcatheter pulmonary valve replacement (TPVR) is essential. Optimus-XXL (AndraTec GmbH, Koblenz, Germany) is a new extra-large, balloon-expandable cobalt-chrome stent with promising technologies., Methods: From June 2020 to November 2020, 15 patients with congenital heart disease, dysfunctional RVOTs and target TPVR diameter ≥ 23 mm received Optimus-XXL stents before proceeding to TPVR using the SAPIEN valve (Edwards Lifesciences, Irvine, CA). Standard safety and outcomes were prospectively assessed., Results: Patients' median age and weight were 25.8 years (range: 10.5-63.1 years) and 58 kg (range: 43.8-101 kg), respectively. Underlying diagnosis was tetralogy of Fallot (66.7%), and RVOTs were patched (80%). Fifteen bare-metal stents were implanted using femoral (n = 14) and jugular approaches (n = 1). One conduit rupture was immediately controlled with a covered Optimus-XXL. Median stent length was 43 mm (range: 33-57 mm), and median target expansion diameter was 28 mm (range: 23-30 mm). Two procedural incidents occurred during stent delivery and were percutaneously treated. Stent stability was documented during TPVRs immediately performed in 14 patients. Median stent shortening was 13.7%, and median percentage of intended stent expansion was 95.9%. There was no stent fracture on the short-term follow-up (median: 4.5 months)., Conclusions: We report the first implantations of Optimus-XXL stents in dysfunctional RVOTs with excellent preliminary results. Optimus-XXL should be considered as a valuable adjunct in the armamentarium for routine and complex TPVR procedures., (Copyright © 2021 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.)

We admitted a 76-year-old woman for treatment of an ascending aortic aneurysm with left ventricular outflow tract (LVOT) obstruction and systolic anterior motion (SAM) of the mitral valve. Echocardiography showed an elevated velocity of the LVOT flow with a sigmoid septum. Mild mitral regurgitation was also detected due to SAM. We performed a graft replacement of the ascending aorta, after which the LVOT obstruction and SAM were resolved. We report a case in which the traction of a graft likely released the compression on the aortic root and ventricular septum., (Copyright © 2021 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

The mechanism for dynamic left ventricular outflow tract obstruction (LVOTO) after acute coronary syndromes (ACS) is thought to be apical infarction with compensatory hyperkinesia of the residual normally perfused basal segments of the myocardium. However, herein, we report a patient with ACS and dynamic LVOTO (peak gradient of 250 mm Hg at rest) that could not be secondary to apical akinesia. We propose a potential alternative mechanism leading to dynamic LVOTO in ACS, namely, the interplay between sigmoid septum, basal hyperkinesis, and outflow tract narrowing induced by afterload reduction due to acute myocardial ischemia itself., (© 2021 Wiley Periodicals LLC.)

Takotsubo cardiomyopathy (TCM) associated with left ventricular outflow tract (LVOT) obstruction in the event of an ST-elevation myocardial infarction (STEMI) is a rare cause of hypotension during percutaneous coronary intervention (PCI). Herein, we describe a 57-year-old woman who presented with STEMI and underwent PCI. She developed hypotension which worsened during inotropic therapy. Echocardiography revealed evidence of LVOT obstruction in the setting of TCM. Therefore, inotropic support was promptly discontinued. Beta blockers and phenylephrine were rapidly administrated, resulting in improved blood pressure and stabilisation of the patient., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2020. No commercial re-use. See rights and permissions. Published by BMJ.)

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., (© The Author(s) 2020. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

We report the case of a 89 year-old woman admitted to the emergency department for epigastric pain, nausea, vomiting. Because of a circulatory failure with electrocardiographic ST changes and a slight elevation of ultra-sensible troponin, a coronary angiography was performed and found normal coronary arteries. Thoraco-abdominal CT scan revealed a large hiatal hernia causing a cardiac compression, and a left intraventricular obstruction showed by Doppler echocardiography. All signs resolved after fasting and gastric drainage., (Copyright © 2020 Elsevier Masson SAS. All rights reserved.)

Scarce data exist on clinical features and prognosis of patients with severe aortic stenosis (AS), concomitant with left ventricular obstruction (LVO). We aimed to evaluate the prevalence, characteristics, and outcomes in patients with severe AS and LVO undergoing transcatheter aortic valve implantation (TAVI). Consecutive patients with severe AS undergoing TAVI between January 2013 to December 2017 at our institution were included. Significant LVO was defined as resting peak left ventricular (LV) systolic gradient ≥30 mm Hg on pre-TAVI echocardiography. We analyzed the primary composite outcome of all-cause mortality and rehospitalization for heart failure (HHF) at 1-year in patients with LVO and those without LVO in the overall and propensity-matched populations. Among 1,729 patients who underwent TAVI, significant LVO was observed in 31 (1.8%) patients. This group was more likely to be female, had smaller aortic annulus and LV cavity, and received a smaller size of the transcatheter heart valve. The most common phenotype of LV hypertrophy causing LVO was concentric LV hypertrophy (58%), and mid-LV obstruction was more common than LV outflow tract obstruction (77% vs 23%, respectively). After adjustment for baseline differences, the primary outcome was not significantly different between patients with LVO and those without LVO (15% vs 16%, respectively; hazard ratio: 0.83; 95% confidence interval: 0.19 to 3.72; p = 0.809). In conclusion, in patients undergoing TAVI, concomitant LVO was relatively uncommon and occurred more often at mid-LV. The presence of pre-TAVI LVO was not associated with worse outcomes defined as increase all-cause mortality or HHF at 1-year., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Objective: Septal myectomy is the most effective treatment modality for hypertrophic obstructive cardiomyopathy. A retrospective study was conducted to evaluate outcomes of surgical myectomy alone or with concomitant mitral valve procedures., Methods: From December 2011 through December 2016, a total of 41 patients with symptomatic hypertrophic obstructive cardiomyopathy were operated. There were 14 females and 27 males, aged between 18 and 73 years (mean 49.8 years). All patients had drug refractory symptoms (dyspnea, palpitation, chest pain, fainting, limitation of daily physical activities). Twenty-one patients received septal myectomy alone, 10 patients had SM with mitral valve repair and 10 patients had SM with mitral valve replacement. The average follow-up was 38.45 ± 12.18 months., Results: Surgery led to symptomatic improvement in all patients. None of the patients were left with NYHA Class III and IV symptoms after surgery. The improvement in left ventricular outflow tract gradient was from 116.65 mmHg preoperatively to 22.47 mmHg. Mean septal thickness decreased from 2.35 to 1.74 cm. Post procedure permanent pacemaker implantation was required for one patient due to complete heart block, and 2 intracardiac devices were implanted due to resistant arrthymia. None of the patients required a repeat procedure during follow-up period. Operative mortality was 2.4%., Conclusion: Septal myectomy is safe and effective. Concomitant mitral operations do not increase morbidity and mortality.

Objectives: This study aimed to systematically assess the importance of left ventricular outflow tract (LVOT) calcification on procedural outcomes and device performances with contemporary transcatheter heart valve (THV) systems., Background: LVOT calcification has been associated with adverse clinical outcomes after transcatheter aortic valve replacement (TAVR). However, the available evidence is limited to observational data with modest numbers and incomplete assessment of the effect of the different THV systems., Methods: In a retrospective analysis of a prospective single-center registry, LVOT calcification was assessed in a semiquantitative fashion. Moderate or severe LVOT calcification was documented in the presence of 2 nodules of calcification, or 1 extending >5 mm in any direction, or covering >10 % of the perimeter of the LVOT., Results: Among 1,635 patients undergoing TAVR between 2007 and 2018, moderate or severe LVOT calcification was found in 407 (24.9%). Patients with moderate or severe LVOT calcification had significantly higher incidences of annular rupture (2.3% vs. 0.2%; p < 0.001), bailout valve-in-valve implantation (2.9% vs. 0.8%; p = 0.004), and residual aortic regurgitation (11.1% vs. 6.3%; p = 0.002). Balloon-expandable valves conferred a higher risk of annular rupture in the presence of moderate or severe LVOT calcification (4.0% vs. 0.4%; p = 0.002) as compared with the other valve designs. There was no significant interaction of valve design or generation and LVOT calcification with regard to the occurrence of bailout valve-in-valve implantation and residual aortic regurgitation., Conclusions: Moderate or severe LVOT calcification confers increased risks of annular rupture, residual aortic regurgitation, and implantation of a second valve. The risk of residual aortic regurgitation is consistent across valve designs and generations. (SWISS TAVI Registry; NCT01368250)., (Copyright © 2020 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

We determined the prevalence and factors associated with tricuspid regurgitation (TR) in adults with repair of right ventricular (RV) outflow obstruction. A total of 256 patients (128 males) were studied at 25.7 ± 7.2 years after surgery, of whom 179 had repaired tetralogy of Fallot (TOF), 31 had pulmonary atresia with intact ventricular septum (PAIVS), and 46 had pulmonary stenosis (PS). The mitral and tricuspid annulus diameters, maximum right atrial (RA) area, RV end-systolic and end-diastolic areas, and tricuspid and pulmonary regurgitation were assessed using echocardiography. The prevalence of moderate-to-severe TR was 20.7%. Subgroup analysis revealed that prevalence was greater in patients with repaired TOF (20.7%) and PAIVS (35.5%) than PS patients (10.9%). As a group, severity of TR was found to be correlated with RA area (r = 0.35, p < 0.001), RV end-diastolic (r = 0.28, p < 0.001) and end-systolic (r = 0.22, p = 0.001) areas, and tricuspid valve annulus diameter (r = 0.15, p = 0.022). Moderate-to-severe TR was associated with development of cardiac arrhythmias with an odds ratio of 2.9 (95% CI 1.1 to 8.1, p = 0.031). Multivariate analysis revealed maximum RA area (β = 0.36, p = 0.016) as an independent determinant of severity of TR. Moderate-to-severe TR occurs in about one-fifth of adults with repaired TOF, PAVIS, and PS and is associated with RA dilation and risk of development of cardiac arrhythmias.

Heart failure (HF) is characterized by autonomic imbalance with sympathetic hyperactivity and loss of parasympathetic tone. Intracardiac ganglia (ICG) neurons represent the final common pathway for vagal innervation of the heart and strongly regulate cardiac functions. This study tests whether ICG cholinergic neuron activation mitigates the progression of cardiac dysfunction and reduces mortality that occurs in HF. HF was induced by transaortic constriction (TAC) in male transgenic Long-Evans rats expressing Cre recombinase within choline acetyltransferase (ChAT) neurons. ChAT neurons were selectively activated by expression and activation of excitatory designer receptors exclusively activated by designer receptors (DREADDs) by clozapine- N -oxide (TAC + treatment and sham-treated groups). Control animals expressed DREADDs but received saline (sham and TAC groups). A separate set of animals were telemetry instrumented to record blood pressure (BP) and heart rate (HR). Acute activation of ICG neurons resulted in robust reductions in BP (∼20 mmHg) and HR (∼100 beats/min). All groups of animals were subjected to weekly echocardiography and treadmill stress tests from 3 to 6 wk post-TAC/sham surgery. Activation of ICG cholinergic neurons reduced the left ventricular systolic dysfunction (reductions in ejection fraction, fractional shortening, stroke volume, and cardiac output) and cardiac autonomic dysfunction [reduced HR recovery (HRR) post peak effort] observed in TAC animals. Additionally, activation of ICG ChAT neurons reduced mortality by 30% compared with untreated TAC animals. These data suggest that ICG cholinergic neuron activation reduces cardiac dysfunction and improves survival in HF, indicating that ICG neuron activation could be a novel target for treating HF. NEW & NOTEWORTHY Intracardiac ganglia form the final common pathway for the parasympathetic innervation of the heart. This study has used a novel chemogenetic approach within transgenic ChAT-Cre rats [expressing only Cre-recombinase in choline acetyl transferase (ChAT) neurons] to selectively increase intracardiac cholinergic parasympathetic activity to the heart in a pressure overload-induced heart failure model. The findings from this study confirm that selective activation of intracardiac cholinergic neurons lessens cardiac dysfunction and mortality seen in heart failure, identifying a novel downstream cardiac-selective target for increasing cardioprotective parasympathetic activity in heart failure.

Patients with large sub-pulmonic ventricular septal defect (VSD) present early as a results of their complications. Some present late, due to the restriction of VSD by the right coronary cusp (RCC) due to its prolapse. In this report, we present a rare case of sub-pulmonic VSD in a 33-year-old man who developed a sub-pulmonic stenosis due to the prolapse of the RCC into the right ventricular outflow tract., (© 2020 Wiley Periodicals LLC.)

A 4-year-old boy with Noonan syndrome accompanied by hypertrophic obstructive cardiomyopathy presented with refractory heart failure owing to severe left ventricular outflow obstruction and mitral regurgitation. He underwent mitral valve replacement and trans-mitral myectomy. The pathology of the resected myocardium was consistent with hypertrophic cardiomyopathy, and the mitral valve leaflets were severely degenerated. Compared with a control patient who is a non-syndromic 4-year-old boy with hypertrophic obstructive cardiomyopathy, mitral pathology was much more severe in Noonan syndrome. Regarding trans-mitral myectomy, residual pressure gradient at the mid-ventricular level made us think about more aggressive muscle resection with various approaches.

Purpose: To investigate the incidence, mechanism, and risk factors of aortic regurgitation (AR) in patients with hypertrophic cardiomyopathy (HCM) by using echocardiography and cardiac magnetic resonance (CMR)., Methods: 105 HCM patients, 52 hypertension (HTN) patients and 50 healthy controls (HC) were retrospectively recruited. HCM patients were divided into 38 with AR (HCMAR) subject and 67 without AR. The subaortic complex, D1 (the largest distance of the interventricular septum that protruded into the LVOT) and D3 (the LVOT effective width) were assessed and compared between the two groups of HCM patients., Results: AR was more common in HCM than in HTN and HC (36 %, 17 %, and 10 %, respectively, P = 0.001). HCM patients with AR were older (58 ± 11 vs. 45 ± 16 years, P < 0.001) and had a higher incidence of hypertension (55 % vs. 33 %, P = 0.03). D1 was greater (13.5 ± 4.4 vs. 10.6 ± 4.0 mm, P = 0.001), and D3 was shorter in the HCMAR group (10.2 ± 5.3 vs. 13.7 ± 5.9 mm, P = 0.003). Anterior mitral leaflet length and left atrial diameter were greater in HCMAR group (all P < 0.05). On multivariable logistic regression analysis, the independent risk factors of AR in HCM patients were LVOTO and age., Conclusions: This study demonstrated that AR is a common comorbidity of HCM, especially in patients with LVOTO. LVOTO and age were independent risk factors of AR in HCM patient., Competing Interests: Declaration of Competing Interest The authors have no conflict of interest to disclose., (Copyright © 2020. Published by Elsevier B.V.)

Objective: There has been debate on the importance and pathophysiologic effects of the dynamic subaortic pressure gradient in hypertrophic obstructive cardiomyopathy. The study was conducted to elucidate the hemodynamic abnormalities associated with the dynamic pressure gradient in hypertrophic obstructive cardiomyopathy., Methods: Eight patients with hypertrophic obstructive cardiomyopathy and 7 patients with valvular aortic stenosis underwent a detailed hemodynamic study of pressure flow relationships before and after myectomy or aortic valve replacement during operation., Results: In aortic stenosis, the increased gradient after premature ventricular contraction was associated with an increase in peak flow (325 ± 122 mL/s to 428 ± 147 mL/s, P = .002) and stroke volume (75.0 ± 27.3 mL to 88.0 ± 24.0 mL, P = .004), but in hypertrophic obstructive cardiomyopathy peak flow remained unchanged (289 ± 79 mL/s to 299 ± 85 mL/s, P = .334) and stroke volume decreased (45.9 ± 18.7 mL to 38.4 ± 14.4 mL, P = .04) on the postpremature ventricular contraction beat. After myectomy, the capacity to augment stroke volume on the postpremature ventricular contraction beats was restored in patients with hypertrophic obstructive cardiomyopathy (45.6 ± 14.4 mL to 54.4 ± 11.8 mL, P = .002)., Conclusions: The pressure flow relationship in hypertrophic obstructive cardiomyopathy supports the concept of true obstruction to outflow, with a low but continued flow during late systole, when the ventricular-aortic pressure gradient is the highest. Septal myectomy can abolish obstruction and restore the ability to augment stroke volume, which may explain the mechanism of symptomatic improvement after operation., (Copyright © 2019 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Background: To characterize treatment of transposition of great arteries with ventricular septal defect and left ventricular outflow tract obstruction (LVOTO) in Germany and to analyze late outcomes., Methods: German Registry for Congenital Heart Defects data were searched for transposition of great arteries with ventricular septal defect and LVOTO. One hundred thirty-nine patients were treated at 15 institutions between 1968 and 2016. Risk factor analyses were performed for the primary endpoints of death and reoperation/reintervention., Results: Follow-up was 88% complete, with a mean follow-up of 16 ± 7 years (median, 15 years [range, 1-48]) and a cumulative follow-up comprising 1739 patient-years. Atrial switches were performed in 15%, Rastelli procedure in 48%, Nikaidoh procedure in 9%, réparation a l'étage ventriculair (REV) in 8%, and arterial switch in 20%. Actuarial survival at 30 years was 86% (range, 83%-90%), with no difference between repairs. Freedom from primary reoperation was 17% (range, 12%-21%) at 30 years. Freedom from reoperation for LVOTO at 30 years was 74% (range, 70%-78%). Recurrent LVOTO was found in 9%, with risk factor diffuse subvalvular LVOTO (odds ratio, 9.8; P = .04). Late freedom from first reoperations other than right ventricle-to-pulmonary artery conduit was 60% (range, 59%-61%), with predictors multiple ventricular septal defect (hazard ratio, 6; P = .03) and Rastelli procedure (hazard ratio, 12; P = .03). Absolute reoperation rates were lowest for REV procedure (16% at a mean of 12 years)., Conclusions: Long-term survival is good without detectable differences between operations. Since the early 2000s increasing use of Nikaidoh and REV procedures was obvious, with REV performing particularly well. Surgery-specific and -unspecific reoperations are most common after the Rastelli technique., (Copyright © 2020 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

A 79-year-old man was admitted for a transcatheter aortic valve replacement due to severe aortic stenosis. A preoperative chest computed tomography with angiography revealed an apical variant hypertrophic cardiomyopathy with a prominent apical pouch. In addition, there was near-complete obliteration of the left ventricle in the mid to apical aspect during systole suggesting a midventricular gradient. Postoperative transthoracic echocardiography confirmed the apical variant hypertrophic cardiomyopathy with an apical aneurysm and a gradient with a peak velocity of 2 m/s, and mid-cavitary gradient with a peak velocity of 3 m/s. It also revealed a fusiform aneurysmal dilatation of the ascending aorta.

Background: Significant left-ventricular outflow tract obstruction (LVOTO) in hypertrophic cardiomyopathy (HCM) may result in symptoms and is associated with adverse outcomes. Although disopyramide can reduce resting gradients, nearly 30% of HCM patients do not respond. We sought to study the clinical and echocardiographic variables associated with disopyramide-induced LVOT-gradient reduction., Methods: Forty-one disopyramide-treated HCM patients (average daily-dose 305 mg) were subdivided into two groups: (1) nineteen responders, with a reduction of LVOT-gradients of at least 30% from baseline, and (2) twenty-two non-responders, in whom LVOT-gradients did not change or increased following treatment. All patients had a thorough clinical and echocardiographic assessment pre- and post-treatment initiation., Results: Patients who responded to disopyramide had better pretreatment left ventricular (LV) systolic function (LV ejection fraction of 67.9 ± 5.6% vs. 59.7 ± 5.8%, p = 0.0001), better LV global longitudinal strain (-17.9 ± 2.3% vs. -16.1 ± 2.5%, p = 0.048), less mitral regurgitation, smaller LV size (indexed LV end-systolic volume of 16.2 ± 5.1 ml/m 2 vs. 23.2 ± 6.8 ml/m 2 , p = 0.001), and lower LV maximal wall thickness (17.2±3 mm vs.19.2 ± 3.4 mm, p = 0.046). Baseline left atrial (LA) volumes were significantly lower in the responders, with higher indices of LA ejection fraction (62 ± 11.2% vs. 50.5 ± 12.2%, p = 0.005), systolic LA strain (34 ± 12.4% vs. 25.8 ± 10.6%, p = 0.04), and LA strain-rate (1.34 ± 0.49%/sec vs. 0.99 ± 0.24%/sec, p = 0.012). In multivariable analysis, the presence of reduced LV systolic function and systolic LA strain-rate remained independently associated with poor response to disopyramide., Conclusions: Obstructive HCM patients with more severe disease at baseline tend to respond less to disopyramide treatment. In those patients, early referral for alcohol septal ablation or myectomy surgery should be considered., (Copyright © 2019 Elsevier B.V. All rights reserved.)

Surgery for obstructive hypertrophic cardiomyopathy with mitral abnormality requires a combined procedure of myectomy and mitral leaflet plication for relieving mitral systolic anterior motion and left ventricular outflow tract obstruction. We report a combined procedure of myectomy using intracardiac high-resolution ultrasonography and mitral bileaflet shortening in obstructive hypertrophic cardiomyopathy. Stepwise real-time verification of residual ventricular mass using high-resolution ultrasonic probe would be a potent addition to our armamentarium. This modality provides safe and effective myectomy, which has been a most technically challenging surgical procedure especially for inexperienced surgeons., (Copyright © 2019 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Hypertrophic cardiomyopathy with left ventricular (LV) mid-cavity obstruction and LV apical aneurysm is associated with high morbidity and mortality rates. However, consensus is lacking on the treatment modality for LV mid-cavity obstruction and LV apical aneurysm. Here, we report a case of reduced LV mid-cavity pressure gradient and symptoms, treated using permanent pacing. The effect of permanent pacing on pressure gradient and symptoms lasted for 4 years. As pacing is relatively non-invasive compared to surgical therapy, permanent pacing is a good option, especially in the elderly patients with LV mid-cavity obstruction and apical aneurysm.

Isolated dextrocardia is a congenital anomaly characterised by the normal position of the thoracic and abdominal viscera with a right cardiac apex. Left ventricular outflow tract obstruction (LVOTO) is a common structural manifestation of hypertrophic cardiomyopathy (HCM). A 65-year-old woman had worsening chest discomfort and dyspnoea on exertion. Chest CT angiography identified the isolated dextrocardia and HCM. Colour Doppler echocardiography showed mosaic flow in the LV outflow, indicating LVOTO. We performed alcohol septal ablation (ASA) under intracardiac echocardiography (ICE)-guided selective myocardial contrasting. This procedure improved provoked intra-LV pressure gradient by Valsalva manoeuvre and nitroglycerin injection from 136 to 50 mm Hg and her symptoms. The unique combination of isolated dextrocardia and left ventricular hypertrophy could have been involved in the formation of latent LVOTO. Even with the anomaly, contrast ICE made it possible to clarify the target septal left ventricular wall of ASA, and we could perform ASA safely., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: A Konno operation with a mechanical prosthesis may be applied in patients with complex left ventricular outflow tract obstruction to avoid further operations. We reviewed our 20-year experience with the Konno operation., Methods: All patients who underwent the Konno operation between 1996 and 2015 were evaluated. Study end points were survival and reoperations., Results: Twenty-one consecutive patients were included. The median age at operation was 12 years (5 months to 34 years). Twenty (95%) patients had prior interventions for left-sided heart lesions. Additional mitral valve disease was present in 17 (81%) patients. The preoperative mean pressure gradient over the left ventricular outflow tract was 50 ± 25 mm Hg. The median size of implanted valve prostheses was 21 mm (16-25 mm). Concomitant procedures for left-sided heart lesions were performed in six patients, including two mitral valve replacements. There were two hospital mortalities (9.5%) and four late mortalities (19%). Overall survival was 85% ± 7.8% and 72% ± 11% at 5 and 10 years, respectively. In two patients, mitral valve replacement was performed during the same hospital admission. During a mean follow-up time of 7.6 ± 4.8 years, two patients required late reoperations, one for mitral valve replacement and one for heart transplantation. Freedom from late reoperation at 10 years was 89% ± 7.4%., Conclusions: The Konno operation can be considered as a definitive option with a low probability of reoperation on the left ventricular outflow tract in patients with complex left ventricular heart disease. Subsequent operations focus on the treatment of additional mitral valve disease, which remains the cause of mortality and morbidity.

Cardiac fibrosis is a major cause of cardiac dysfunction in hypertrophic hearts. Differentiated embryonic chondrocyte gene 1 (Dec1), a basic helix-loop-helix transcription factor, has circadian expression in the heart; however, its role in cardiac diseases remains unknown. Therefore, using Dec1 knock-out (Dec1KO) and wild-type (WT) mice, we evaluated cardiac function and morphology at one and four weeks after transverse aortic constriction (TAC) or sham surgery. We found that Dec1KO mice retained cardiac function until four weeks after TAC. Dec1KO mice also revealed more severely hypertrophic hearts than WT mice at four weeks after TAC, whereas no significant change was observed at one week. An increase in Dec1 expression was found in myocardial and stromal cells of TAC-treated WT mice. In addition, Dec1 circadian expression was disrupted in the heart of TAC-treated WT mice. Cardiac perivascular fibrosis was suppressed in TAC-treated Dec1KO mice, with positive immunostaining of S100 calcium binding protein A4 (S100A4), alpha smooth muscle actin (αSMA), transforming growth factor beta 1 (TGFβ1), phosphorylation of Smad family member 3 (pSmad3), tumor necrosis factor alpha (TNFα), and cyclin-interacting protein 1 (p21). Furthermore, Dec1 expression was increased in myocardial hypertrophy and myocardial infarction of autopsy cases. Taken together, our results indicate that Dec1 deficiency suppresses cardiac fibrosis, preserving cardiac function in hypertrophic hearts. We suggest that Dec1 could be a new therapeutic target in cardiac fibrosis.

Background: The purpose of this study is to assess risk factors of neo-aortic regurgitation (AR) after an arterial switch operation and outcomes of neo-aortic valve surgery., Methods: This is a retrospective study of 469 hospital survivors after arterial switch operation from 1982 to 2016. Preoperative diagnoses included 285 transpositions of the great arteries with intact septum, 140 transpositions with ventricular septal defect, and 44 double-outlet right ventricles. More than mild preoperative pulmonary regurgitation was found in 15 patients. Median age at the operation was 1.0 months (range, 0.1-81.5). Concomitant relief of left ventricular outflow tract obstruction was performed in 20 patients. Seventy-seven patients had more than mild neo-AR at hospital discharge., Results: More than moderate neo-AR was found in 41 patients (8.6%) at a median follow-up of 19.0 years (range, 0.1-35.2). Multivariate analysis identified more than mild preoperative pulmonary regurgitation, concomitant relief of left ventricular outflow tract obstruction, and more than mild neo-AR at hospital discharge as risk factors for late neo-AR. Seventeen patients (3.6%) underwent neo-aortic valve surgeries, including 11 aortic valve replacements, 3 aortic valve plasties, 2 Konno procedures, and 1 aortic root replacement, with median age at the neo-aortic valve surgery of 16.5 years (range, 6.2-27.3). There were 2 late deaths and 5 reoperations to the neo-aortic valve in 4 patients., Conclusions: Concomitant relief of left ventricular outflow tract obstruction and preoperative more than mild pulmonary regurgitation increase the risk of neo-AR after the arterial switch operation. Reoperation for neo-AR can be done safely., (Copyright © 2019 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

We present a case of left ventricular outflow tract (LVOT) obstruction detected by limited bedside transthoracic echocardiography (TTE). This involved a young and otherwise healthy patient presenting for elective hand surgery with a previously undetected cardiac murmur. It highlights the utility of bedside TTE as an assessment tool and shows the importance of anesthesiologists as perioperative physicians.