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Left ventricular outflow tract obstruction is a rare complication following transcatheter mitral valve implantation. Diagnosing the underlying cause is mandatory to select from different treatment options. We report a case of stent-graft implantation into the left ventricular outflow tract for dynamic left ventricular outflow tract obstruction caused by systolic anterior motion of the anterior mitral valve leaflet (SAM). [ABSTRACT FROM AUTHOR]

Transcatheter mitral valve-in-valve is an alternative to high-risk reoperation on a failing bioprosthesis. It entails specific challenges such as left ventricular outflow tract obstruction. We propose a patient-specific augmented imaging based on preoperative planning to assist the procedure. Valve-in-valve simulation was performed to represent the optimal level of implantation and the neo-left ventricular outflow tract. These data were combined with intraoperative images through a real-time 3D/2D registration tool. All data were collected retrospectively on one case (pre and per-procedure imaging). We present for the first time an intraoperative guidance tool in transcatheter mitral valve-in-valve procedure. [ABSTRACT FROM AUTHOR]

Left ventricular outflow tract obstruction [LVOTO; pressure gradient (PG) ≥30 mmHg] is observed in some patients without hypertrophic cardiomyopathy (HCM), and it may develop especially in older patients without HCM (non-HCM). The aim of this study is to investigate if the Valsalva or an upright sitting maneuver can unveil latent LVOTO in patients with non-HCM. A total of 33 non-HCM patients with a late peaking or dagger-shaped pulsed Doppler waveform of the LVOT and PG <30 mmHg were included. The Doppler flow velocity of the LVOT was measured at rest, after the Valsalva and a sitting maneuver. Peak PG of ≥30 mmHg after either maneuver was defined as latent LVOTO. The angle between the left ventricular septum and the aorta in the parasternal long-axis view and the apical three-chamber view was measured. Twenty (61 %) of the 33 patients (mean age 74 ± 9 years) were diagnosed with latent LVOTO. Of these, five (25 %) patients were diagnosed after both the Valsalva and sitting maneuver, and 15 (75 %) were diagnosed only after the sitting maneuver. The latent LVOTO group had a significantly smaller angle than the no-LVOTO group between the ventricular septum and the aorta in the parasternal long axis views (107 ± 8° vs. 117 ± 8°, p < 0.01). The sitting maneuver is better than the Valsalva maneuver in unveiling latent LVOTO in older, non-HCM patients. [Display omitted] • Sitting maneuver could induce left ventricular outflow tract obstruction (LVOTO) better than the Valsalva maneuver. • Sitting maneuver is useful to unveil latent LVOTO in non-HCM population. • We should pay special attention to the presence of latent LVOTO when we found a sigmoid septum and a small LVDd. • It should be adopted as an additional maneuver to uncover latent LVOTO. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide OBJECTIVES The Bex-Nikaidoh operation can effectively relieve left ventricular outflow tract obstruction. However, if a conduit is used for right ventricular outflow tract reconstruction, a late reoperation can be anticipated. We examined the impact of double root translocation on outcomes. METHODS We performed a retrospective single-centre study of patients who underwent aortic root translocation between 2006 and 2019. RESULTS Aortic root translocation was performed in 23 patients at a median age of 1.6 years [interquartile range (IQR) 0.9–2.5]. Concomitant repairs were done in 52.2% of patients (12/23) including the Senning atrial switch in 34.8% (8/23). The right ventricular outflow tract was reconstructed with valved conduits in 39.1% (9/23), direct anastomoses in 4.35% (1/23) and pulmonary autografts in 56.5% of patients (13/23). Aortic cross-clamp time was significantly longer in patients with double root translocation [308 min (IQR 270–259) vs 209 min (IQR 179–281), P  =   0.02]; 2 patients in this group required temporary mechanical circulatory support. There were no early deaths. Median follow-up time was 7.5 years (IQR 3.3–10.5). The estimated 10-year survival was 90% [95% confidence interval (CI): 47.3%, 98.5%]. There was no recurrent left ventricular outflow tract obstruction. Freedom from any reoperation was 64.2% (95% CI: 40.8%, 80.3%) at 3 years and 44.5% (95% CI: 21.2%, 65.5%) at 6 years. The main indication for late reoperation was conduit degeneration. Freedom from a right ventricular outflow tract reoperation was significantly higher, and the number of reoperations per patient was lower when a double root translocation had been performed (P  =   0.03). CONCLUSIONS The Bex-Nikaidoh operation effectively relieved left ventricular outflow tract obstruction. A double root translocation further increased procedural complexity but was associated with better mid-term freedom from a right ventricular outflow tract reoperation. It should be considered in suitable patients. [ABSTRACT FROM AUTHOR]

Shone's complex is a congenital cardiac disease consisting of the following four lesions: parachute mitral valve, supravalvar mitral ring, subaortic stenosis, and aortic coarctation. Though not all components are required for a diagnosis, the end result is both left ventricular inflow and outflow obstruction, which typically present in patients as congestive heart failure. The complex pathology requires careful management and surgical decision-making to ensure an optimal outcome. This review will focus on the anatomy, physiology, and perioperative anesthetic management of patients with Shone's complex. [ABSTRACT FROM AUTHOR]

Objective: To review the current literature on the efficacy and safety of cardiac myosin inhibitors (CMIs) for the treatment of hypertrophic cardiomyopathy (HCM). Data Sources: A literature search was conducted on PubMed from origin to April 2023, using the search terms "MYK-461," "mavacamten," "CK-3773274," and "aficamten." Studies were limited to English-based literature, human subjects, and clinical trials resulting in the inclusion of 13 articles. ClinicalTrials.gov was also used with the same search terms for ongoing and completed trials. Study Selection and Data Extraction: Only phase II and III studies were included in this review except for pharmacokinetic studies that were used to describe drug properties. Data Synthesis: CMIs enable cardiac muscle relaxation by decreasing the number of myosin heads that can bind to actin and form cross-bridges. Mavacamten, the first Food and Drug Administration (FDA)-approved drug in this class, has been shown to improve hemodynamic, functional, and quality of life measures in HCM with obstruction. In addition, aficamten is likely to become the next FDA-approved CMI with promising phase II data and an ongoing phase III trial expected to release results in the next year. Relevance to Patient Care and Clinical Practice in Comparison with Existing Drugs: CMIs provide a novel option for obstructive hypertrophic cardiomyopathy, particularly in those not suitable for septal reduction therapy. Utilization of these agents requires knowledge of drug interactions, dose titration schemes, and monitoring parameters for safety and efficacy. Conclusions: CMIs represent a new class of disease-specific drugs for treatment of HCM. Cost-effectiveness studies are needed to delineate the role of these agents in patient therapy. [ABSTRACT FROM AUTHOR]

A 63-year-old symptomatic female with apical hypertrophic cardiomyopathy and diastolic disfunction was admitted to the hospital. What is the best way to manage this patient? This study is a literature review that was performed to answer this question. The following PubMed search strategy was used: 'Hypertrophic obstructive cardiomyopathy' [All Fields] OR 'apical myectomy' [All Fields], NOT 'animal [mh]' NOT 'human [mh]' NOT 'comment [All Fields]' OR 'editorial [All Fields]' OR 'meta-analysis [All Fields]' OR 'practice-guideline [All Fields]' OR 'review [All Fields]' OR 'pediatrics [mh]'. The natural history of the disease has a benign prognosis; however, a watchful strategy was associated with the risk of adverse cardiovacular events. Contrastingly, transapical myectomy was associated with low surgical risk and acceptable outcomes. In our case, the patient underwent transapical myectomy with an unconventional post-operative period. Control echocardiography showed marked left ventricular (LV) cavity enlargement: LV end-diastolic volume, 74 mL; LV ejection fraction, 65%; and LV stroke volume index increased to 27 mL/m2. The patient was discharged 7 days after myectomy. At 6 months post-operation, the patient was NYHA Class I, with a 6 min walk test score of 420 m. Therefore, transapical myectomy may be considered as a feasible procedure in patients with apical hypertrophic cardiomyopathy and progressive heart failure. [ABSTRACT FROM AUTHOR]

Left ventricular outflow obstruction (LVOTO) and diastolic dysfunction are the main pathophysiological characteristics of hypertrophic cardiomyopathy (HCM)LVOTO, may be identified in more than half of HCM patients and represents an important determinant of symptoms and a predictor of worse prognosis. This review aims to clarify the LVOTO mechanism in, diagnosis of, and therapeutic strategies for patients with obstructive HCM.

The article focuses on the use of alcohol septal ablation (ASA) to treat left ventricular outflow tract obstruction (LVOTO) in a patient with hypertrophic cardiomyopathy. Topics include the use of microcatheter technology for targeting small, tortuous septal sub-branches, the procedural success and post-operative follow-up using cardiac imaging, and the management of residual LVOTO with a second ASA procedure using a microcatheter and ethanol injection.

Left ventricular outflow obstruction (LVOTO) and diastolic dysfunction are the main pathophysiological characteristics of hypertrophic cardiomyopathy (HCM)LVOTO, may be identified in more than half of HCM patients and represents an important determinant of symptoms and a predictor of worse prognosis. This review aims to clarify the LVOTO mechanism in, diagnosis of, and therapeutic strategies for patients with obstructive HCM. [ABSTRACT FROM AUTHOR]

A 63-year-old symptomatic female with apical hypertrophic cardiomyopathy and diastolic disfunction was admitted to the hospital. What is the best way to manage this patient? This study is a literature review that was performed to answer this question. The following PubMed search strategy was used: ‘Hypertrophic obstructive cardiomyopathy’ [All Fields] OR ‘apical myectomy’ [All Fields], NOT ‘animal [mh]’ NOT ‘human [mh]’ NOT ‘comment [All Fields]’ OR ‘editorial [All Fields]’ OR ‘meta-analysis [All Fields]’ OR ‘practice-guideline [All Fields]’ OR ‘review [All Fields]’ OR ‘pediatrics [mh]’. The natural history of the disease has a benign prognosis; however, a watchful strategy was associated with the risk of adverse cardiovacular events. Contrastingly, transapical myectomy was associated with low surgical risk and acceptable outcomes. In our case, the patient underwent transapical myectomy with an unconventional post-operative period. Control echocardiography showed marked left ventricular (LV) cavity enlargement: LV end-diastolic volume, 74 mL; LV ejection fraction, 65%; and LV stroke volume index increased to 27 mL/m2. The patient was discharged 7 days after myectomy. At 6 months post-operation, the patient was NYHA Class I, with a 6 min walk test score of 420 m. Therefore, transapical myectomy may be considered as a feasible procedure in patients with apical hypertrophic cardiomyopathy and progressive heart failure.

Rationale: Pregnancy causes important physiologic stress for women with hypertrophic cardiomyopathy. Data regarding the impact of this condition on obstetrical outcomes is missing. Objectives: Our objective was to report obstetrical and cardiac outcomes in pregnant women with hypertrophic cardiomyopathy and to assess the possible adverse effects of left ventricular outflow tract obstruction in pregnancy. Study design: This was a retrospective cohort study of pregnant women diagnosed with HCM and followed at single tertiary center between 1995 and 2019. Demographic, medical and surgical data, echocardiographic parameters, and pregnancy outcomes were abstracted through extensive chart review. Patients were divided into 2 groups: obstructive (maximal left ventricular outflow tract gradient over 30 mmHg) versus non-obstructive hypertrophic cardiomyopathy. Outcomes between groups were compared with t-test, Mann-Whitney and Fisher’s exact tests when appropriate. Results: Eighteen women with 27 pregnancies were included. The study population was formed of 18 women with a total of 27 pregnancies that reached at least 20 weeks of gestation: 12 pregnancies in women with obstructive hypertrophic cardiomyopathy and 15 pregnancies in women with non-obstructive hypertrophic cardiomyopathy. Among the non-obstructive hypertrophic cardiomyopathy, 5 of them had been treated for their obstruction. One patient with obstructive hypertrophic cardiomyopathy had a medical termination of pregnancy for uncontrolled arrhythmia at 21 weeks. There were no maternal deaths. Left ventricular outflow tract obstruction was associated with increased cardiac events including arrhythmias and heart failure (5/12 versus 0/15; p ¼ .006). Preterm birth occurred in more than 50% of cases, resulting from induced delivery for a maternal (40%) or fetal reason (60%). Most deliveries were late preterm between 34 and 36 6/7 weeks. In both groups, birthweight was mainly distributed below the 50th percentile (89%) and 35% of neonates were born small for gestational age defined as a birthweight below the 10th percentile. Most severe cases of small for gestational age (birthweight under the 5th percentile) were found in patients with treated obstructive hypertrophic cardiomyopathy. Conclusion: Hypertrophic cardiomyopathy is associated with prematurity and small for gestational age. Left ventricular outflow tract obstruction is associated with adverse cardiac events including arrythmias or heart failure. Treated obstructive cardiomyopathy constitutes a subgroup of patients at high risk of severe small for gestational age and deserves a close surveillance. Therefore, fetal growth surveillance with ultrasound, early in the third trimester and doppler studies to assess the utero-placental perfusion in the second and third trimesters are warranted in all patients with hypertrophic cardiomyopathy regardless of the severity of their condition. [ABSTRACT FROM AUTHOR]

Robotic major lung resection for lung cancer carries a risk for intraoperative hemodynamic instability. Systolic anterior motion (SAM) of the mitral valve is a rare and often misrecognized cause of intraoperative hemodynamic instability. If not promptly recognized, SAM leads to a complicated perioperative course. Here, we report for the first time a case of a patient with SAM with a severe degree of left ventricular outflow obstruction (LVOTO) undergoing robotic lung lobectomy and its challenging intraoperative management. A 70-year-old man undergoing robotic left upper lobectomy developed immediately after the induction of general anesthesia hemodynamic instability due to SAM-related LVOTO. The diagnosis was possible, thanks to the use of transesophageal echocardiography (TEE). The treatment strategies applied were preload optimization without fluid overload, ultra-short-acting beta-blockers, and vasopressors. Peripheral nerve blockades were preferred over epidural analgesia to avoid vasodilatation. The patient reported a good quality of recovery and no pain the day after surgery. The management of patients with higher risk of SAM and LVOTO development during robotic thoracic surgery requires a dedicated and skilled team together with high-impact treatment strategies driven by TEE. Since current guidelines do not recommend the use of TEE, even for patients with higher cardiac risk undergoing noncardiac surgery, the present case report may stimulate interest in future recommendations. [ABSTRACT FROM AUTHOR]

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.)

Valve-in-mitral annular calcification presents a great challenge with a risk of left ventricular outflow tract obstruction (LVOTO). We demonstrate the first-in-human experience of performing percutaneous electrosurgery-guided perforation and balloon dilation of the anterior mitral valve leaflet followed by transcatheter valve implantation to prevent LVOTO., Competing Interests: The authors have reported that they have no relationships relevant to the contents of this paper to disclose., (© 2024 The Authors.)

[Display omitted] [ABSTRACT FROM AUTHOR]

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

To address various problems with conventional surgical procedures for transposition of the great arteries with left ventricular outflow obstruction, the aortic translocation procedure has been reconsidered as an alternative surgical procedure. We developed another innovative translocation technique, the half-turned truncal switch operation, to make use of various anatomical characteristics. The truncal block involving both semilunar valves is harvested en bloc and anastomosed to the opposite ventricular outflow tract after a half-turn. Temporarily detached coronary arteries are re-anastomosed to the reversed aortic wall defects. The key advantage of this half-turned truncal switch operation is the creation of hemodynamically faultless right and left ventricular outflow tracts including competent valve functions and a coronary circulation ensured by the posteriorly translocated aortic valve, the maximally used autologous pulmonary valve, and anastomosis of the coronary arteries to the confronting aortic wall defects. [ABSTRACT FROM AUTHOR]

Septal myectomy is the standard treatment for obstructive hypertrophic cardiomyopathy. We studied the impact of intraoperative pre- and postprocedure dobutamine stress transesophageal echocardiography on surgical planning and outcomes of septal myectomy. We identified 55 patients undergoing septal myectomy over a 24-month period. All patients underwent resting and dobutamine stress (20-40 mcg/kg/min) echocardiography after induction of anesthesia pre- and postprocedure. Demographic, clinical, and imaging data were prospectively collected. Mean age was 59 (42-68). A total of 69% of patients were New York Heart Association Class III/IV. During outpatient evaluation, peak preoperative resting left ventricular outflow tract gradient was 91.6 mm Hg and 94% (50/53) had severe stress-induced mitral regurgitation (MR). After induction, peak resting gradient fell to 47.8 mm Hg and 43% (24/55) had improved gradients (<30 mm Hg). With stress, preprocedure left ventricular outflow gradient increased to 130 mm Hg and all occult gradients were unmasked. Postprocedure, peak resting and stress gradients were substantially reduced (10.2 ± 6.8 mm Hg and 23.6 ± 8.5 mm Hg, respectively). With stress, 84% (42/50) demonstrated reduction in severity of MR to none and/or mild with no patients having greater than moderate. Postprocedure stress echocardiography identified 3 patients with residual gradients, which led to return to bypass for additional procedures and resulted in resolution of elevated residual gradients. Postoperative 60-day stress echocardiography showed sustained resolution of gradients and MR. In this series, 43% of patients had occult left ventricular outflow gradients after induction of anesthesia. Intraoperative stress echocardiography during septal myectomy is useful to unmask occult gradients and confirm adequate myectomy. This imaging strategy is associated with reliable relief of obstruction and MR as demonstrated at 60-day follow-up. [ABSTRACT FROM AUTHOR]

Rationale: Pregnancy causes important physiologic stress for women with hypertrophic cardiomyopathy. Data regarding the impact of this condition on obstetrical outcomes is missing.Objectives: Our objective was to report obstetrical and cardiac outcomes in pregnant women with hypertrophic cardiomyopathy and to assess the possible adverse effects of left ventricular outflow tract obstruction in pregnancy.Study Design: This was a retrospective cohort study of pregnant women diagnosed with HCM and followed at single tertiary center between 1995 and 2019. Demographic, medical and surgical data, echocardiographic parameters, and pregnancy outcomes were abstracted through extensive chart review. Patients were divided into 2 groups: obstructive (maximal left ventricular outflow tract gradient over 30 mmHg) versus non-obstructive hypertrophic cardiomyopathy. Outcomes between groups were compared with t-test, Mann-Whitney and Fisher's exact tests when appropriate.Results: Eighteen women with 27 pregnancies were included. The study population was formed of 18 women with a total of 27 pregnancies that reached at least 20 weeks of gestation: 12 pregnancies in women with obstructive hypertrophic cardiomyopathy and 15 pregnancies in women with non-obstructive hypertrophic cardiomyopathy. Among the non-obstructive hypertrophic cardiomyopathy, 5 of them had been treated for their obstruction. One patient with obstructive hypertrophic cardiomyopathy had a medical termination of pregnancy for uncontrolled arrhythmia at 21 weeks. There were no maternal deaths. Left ventricular outflow tract obstruction was associated with increased cardiac events including arrhythmias and heart failure (5/12 versus 0/15; p = .006). Preterm birth occurred in more than 50% of cases, resulting from induced delivery for a maternal (40%) or fetal reason (60%). Most deliveries were late preterm between 34 and 36 6/7 weeks. In both groups, birthweight was mainly distributed below the 50th percentile (89%) and 35% of neonates were born small for gestational age defined as a birthweight below the 10th percentile. Most severe cases of small for gestational age (birthweight under the 5th percentile) were found in patients with treated obstructive hypertrophic cardiomyopathy.Conclusion: Hypertrophic cardiomyopathy is associated with prematurity and small for gestational age. Left ventricular outflow tract obstruction is associated with adverse cardiac events including arrythmias or heart failure. Treated obstructive cardiomyopathy constitutes a sub-group of patients at high risk of severe small for gestational age and deserves a close surveillance. Therefore, fetal growth surveillance with ultrasound, early in the third trimester and doppler studies to assess the utero-placental perfusion in the second and third trimesters are warranted in all patients with hypertrophic cardiomyopathy regardless of the severity of their condition. [ABSTRACT FROM AUTHOR]

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

Determine whether the intraoperative three-dimensional left ventricular outflow tract cross-sectional area may be inversely correlated with pressure gradients as a determinant of surgical success after septal myectomy in hypertrophic cardiomyopathy patients. Perioperative data were obtained by retrospective review. Toronto General Hospital, University of Toronto, Toronto, Canada, a tertiary hospital. The study comprised 67 patients with hypertrophic obstructive cardiomyopathy. Transthoracic and intraoperative transesophageal echocardiographic assessment of pressure gradients. Transesophageal measurement of the three-dimensional left ventricular outflow tract cross-sectional area. The smallest left ventricular outflow tract area increased on average 1.883 cm2 (98.3%) after septal myectomy. There was a significant correlation between the increase in the area and the transesophageal pressure gradients (r = –0.32; p = 0.01) after myectomy, but none with postoperative transthoracic gradients at rest (r = –0.10; p = 0.42). Postoperative transesophageal and transthoracic gradients were significantly correlated (r = 0.26; p = 0.04). The best risk factors to predict high residual gradients were preoperative transesophageal gradient >97 mmHg, postoperative transesophageal area <3.16 cm2, and moderate or more residual transesophageal mitral regurgitation (specificity 89%, 81%, and 78%, respectively). Three-dimensional left ventricular outflow tract area measurements with transesophageal echocardiography after myectomy correlated fairly well with postoperative transesophageal pressure gradients. Patients with residual transthoracic elevated gradients after surgery at follow-up had a smaller transesophageal area and higher transesophageal pressure gradients immediately after the procedure. However, transesophageal pressure gradients after myectomy correlated poorly with follow-up transthoracic gradients at rest. [ABSTRACT FROM AUTHOR]

Cardiogenic shock (CS) due to Takotsubo cardiomyopathy (TCM) is often managed with cautious fluid administration and inotropic support; however, the co-existence of a left ventricular outflow tract obstruction (LVOTO) can complicate this management approach. This report describes a case of CS due to TCM and LVOTO. It was successfully managed with the Impella 2.5. (Level of Difficulty: Intermediate.)

Cardiogenic shock due to hypertrophic obstructive cardiomyopathy (HoCM) crisis presents a clinical challenge as pharmacologic vasopressor and/or inotropic support can compromise hemodynamics and acute afterload reduction worsens left ventricular outflow tract (LVOT) obstruction. Hypertensive hypertrophic obstructive cardiomyopathy (HHoCM) is an entity mostly affecting elderly hypertensive women and could present with a clinical phenotype similar to HoCM crisis. We present a case of an 81‐year‐old female patient with HHoCM complicated by severe mitral regurgitation, in cardiogenic shock, in whom hemodynamic stability was restored with transvenous pacing guided by bedside echocardiography to optimize rate, left ventricle (LV) filling time, and cardiac output. [ABSTRACT FROM AUTHOR]

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

Transcatheter mitral valve-in-valve is an alternative to high-risk reoperation on a failing bioprosthesis. It entails specific challenges such as left ventricular outflow tract obstruction. We propose a patient-specific augmented imaging based on preoperative planning to assist the procedure. Valve-in-valve simulation was performed to represent the optimal level of implantation and the neo-left ventricular outflow tract. These data were combined with intraoperative images through a real-time 3D/2D registration tool. All data were collected retrospectively on one case (pre and per-procedure imaging). We present for the first time an intraoperative guidance tool in transcatheter mitral valve-in-valve procedure.

Transposition of great arteries (TGA) can be associated with left ventricle outflow tract (LVOT) obstruction. In the presence of ventricular septal defect (VSD), septal leaflet of tricuspid valve may prolapse through perimembranous VSD or rarely tricuspid valve tissue may override to produce LVOT obstruction. Occasionally, this may be mistaken for vegetation due to associated pulmonary valve endocarditis. We report a case of d-TGA with presumptive pulmonary valve endocarditis and LVOT obstruction that was found to be due to tricuspid valve straddling on transesophageal echocardiography, resulting in change in the surgical plan and thus avoiding catastrophe.

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.

The article focuses on the outcomes of patients undergoing biventricular repair (BVR) after an initial hybrid Norwood approach as asalvage procedure in extremely sick infants; or as the initial palliation in patients with uncertain feasibility of single-stage BVR due to severeleft ventricular outflow tract obstruction; or as part of a left ventricle (LV) recruitment strategy in patients with borderline LVs.

Background: Acute left ventricular (LV) apical ballooning with normal coronary angiography occurs rarely in obstructive hypertrophic cardiomyopathy (OHCM); it may be associated with severe hemodynamic instability. Methods, Results: We searched for acute LV ballooning with apical hypokinesia/akinesia in databases of two HCM treatment programs. Diagnosis of OHCM was made by conventional criteria of LV hypertrophy in the absence of a clinical cause for hypertrophy and mitral‐septal contact. Among 1519 patients, we observed acute LV ballooning in 13 (0.9%), associated with dynamic left ventricular outflow tract (LVOT) obstruction and high gradients, 92 ± 37 mm Hg, 10 female (77%), age 64 ± 7 years, LVEF 31.6 ± 10%. Septal hypertrophy was mild compared to that of the rest of our HCM cohort, 15 vs 20 mm (P < 0.00001). An elongated anterior mitral leaflet or anteriorly displaced papillary muscles occurred in 77%. Course was complicated by cardiogenic shock and heart failure in 5, and refractory heart failure in 1. High‐dose beta‐blockade was the mainstay of therapy. Three patients required urgent surgical relief of LVOT obstruction, 2 for refractory cardiogenic shock, and one for refractory heart failure. In the three patients, surgery immediately normalized refractory severe LV dysfunction, and immediately reversed cardiogenic shock and heart failure. All have normal LV systolic function at 45‐month follow‐up, and all have survived. Conclusions: Acute LV apical ballooning, associated with high dynamic LVOT gradients, may punctuate the course of obstructive HCM. The syndrome is important to recognize on echocardiography because it may be associated with profound reversible LV decompensation. [ABSTRACT FROM AUTHOR]

Copyright of Zeitschrift für Herz-, Thorax- und Gefaesschirurgie is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

We describe a case of severe left ventricular outflow tract obstruction (LVOTO) with severe mitral incompetence due to systolic anterior motion of the anterior mitral leaflet (SAM) that was recognised thanks to the immediate availability of transoesophageal echocardiography during the resuscitation of anaphylactic shock. The patient rapidly responded to cessation of the epinephrine (adrenaline) infusion and intravascular volume expansion with intravenous crystalloid. The absence of risk factors for developing SAM/LVOTO serve as a warning to clinicians to consider this diagnosis in all cases of epinephrine non-responsive anaphylactic shock. [ABSTRACT FROM AUTHOR]

Abstract Dynamic left ventricular outflow tract obstruction (LVOTO) can be hemodynamically significant and can adversely affect the heart and quality of life. It is caused by systolic anterior motion (SAM) of the anterior mitral valve into the LVOT. The mechanism underlying SAM has been an area of special interest. However, SAM occurrence in the absence of septal hypertrophy is exceedingly uncommon. Here we present a case of a young male patient who sought medical care with a complaint of exertional dyspnea, New York Heart Association functional Class 2–3, and was found to have SAM and severe LVOTO at rest without hypertrophic cardiomyopathy. Continuous wave Doppler signal showed a peak velocity of 4.96 m/s along the LVOT, with a pressure gradient at rest of 98.44 mmHg, calculated using the modified Bernoulli equation. The patient is not known to have any medical conditions, nor had a family history of cardiac condition or sudden death. Trans-thoracic echocardiography showed concentric remodeling of the LV without hypertrophy. Trans-esophageal echocardiography was performed for further assessment of the anatomy. The anterior mitral leaflet (AML) and posterior mitral leaflet (PML) lengths were 3.7 cm and 1.3 cm, respectively (normal AML < 3 cm; normal PML < 1.5 cm). In our patient, the LVOTO is significant enough to result in a decreased cardiac output, which explains the symptoms experienced, due to which he developed concentric remodeling. The only finding in this patient explaining SAM is an elongated AML. [ABSTRACT FROM AUTHOR]

Aims We analysed the early and long-term clinical and haemodynamic outcomes of balloon-expandable transcatheter mitral valve implantation (TMVI) in an experienced centre. Methods and results All patients undergoing TMVI from July 2010 to July 2017 in our centre were prospectively included. Indication for TMVI relied on the judgement of the local heart team. Patients were followed at 1 month, 1 year, and yearly thereafter. A total of 91 patients underwent TMVI. The median age was 73 (57–81) years and 70% of patients were women. Patients were at high risk for surgery with a median EuroSCORE II of 9.6 (4.0–14.6) %. Indication for TMVI was bioprosthesis failure (valve-in-valve) in 37.3%, annuloplasty failure (valve-in-ring) in 33.0%, and severe mitral annulus calcification (MAC) in 29.7%. The transseptal approach was used in 92.3% of patients and balloon-expandable valves were used in all patients. Technical success was achieved in 84.6% of patients, one patient died during the procedure and haemodynamically significant left ventricular outflow tract obstruction occurred in three patients (3.3%). At 30 days, 7.7% of patients had died, without significant differences between groups, and a major stroke occurred in 2.2% of patients. The cumulative rates of all-cause mortality at 1-year and 2-year follow-up were 21.0% [95% confidence interval (CI) 9.9–38.8] and 35.7% (95% CI 19.2–56.5), respectively, with a higher late mortality in patients with MAC. The 2-year rates of re-intervention and valve thrombosis were 8.8% and 14.4%, respectively. At 6 months to 1 year, 68.9% of patients were in New York Heart Association Class I or II, and 90.7% of patients had mild or less mitral regurgitation. The mean transmitral gradient decreased from 9.3 ± 3.9 mmHg at baseline to 6.0 ± 2.3 mmHg at discharge (P  < 0.001) without changes at 6-month to 1-year follow-up. Conclusion Transcatheter mitral valve implantation using balloon-expandable valves in selected patients with bioprosthesis or annuloplasty failure or severe MAC was associated with a low rate of peri-procedural complications and acceptable long-term outcomes. [ABSTRACT FROM AUTHOR]

Transposition of great arteries (TGA) can be associated with left ventricle outflow tract (LVOT) obstruction. In the presence of ventricular septal defect (VSD), septal leaflet of tricuspid valve may prolapse through perimembranous VSD or rarely tricuspid valve tissue may override to produce LVOT obstruction. Occasionally, this may be mistaken for vegetation due to associated pulmonary valve endocarditis. We report a case of d-TGA with presumptive pulmonary valve endocarditis and LVOT obstruction that was found to be due to tricuspid valve straddling on transesophageal echocardiography, resulting in change in the surgical plan and thus avoiding catastrophe. [ABSTRACT FROM AUTHOR]

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Hypertrophic cardiomyopathy (HCM) is the most common genetic cardiovascular disease with many genotype and phenotype variations. Earlier terminologies, hypertrophic obstructive cardiomyopathy and idiopathic hypertrophic sub-aortic stenosis are no longer used to describe this entity. Patients present with or without left ventricular outflow tract (LVOT) obstruction. Resting or provocative LVOT obstruction occurs in 70% of patients and is the most common cause of heart failure. The pathology and pathophysiology of HCM includes hypertrophy of the left ventricle with or without right ventricular hypertrophy, systolic anterior motion of mitral valve, dynamic and mechanical LVOT obstruction, mitral regurgitation, diastolic dysfunction, myocardial ischemia, and fibrosis. Thorough understanding of pathology and pathophysiology is important for anesthetic and surgical management.

Improved cosmetic appearance, reduced pain and duration of post-operative stay have intensified the popularity of minimally invasive cardiac surgery (MICS); however, the increased risk of stroke remains a concern. In conventional cardiac surgery, surgeons can visualize and feel the cardiac structures directly, which is not possible with MICS. Transesophageal echocardiography (TEE) is essential during MICS in detecting problems that require immediate correction. Comprehensive evaluation of the cardiac structures and function helps in the confirmation of not only the definitive diagnosis, but also the success of surgical treatment. Venous and aortic cannulations are not under the direct vision of the surgeon and appropriate positioning of the cannulae is not possible during MICS without the aid of TEE. Intra-operative TEE helps in the navigation of the guide wire and correct placement of the cannulae and allows real-time assessment of valvular pathologies, ventricular filling, ventricular function, intracardiac air, weaning from cardiopulmonary bypass and adequacy of the surgical procedure. Early detection of perioperative complications by TEE potentially enhances the post-operative outcome of patients managed with MICS.

Mitral regurgitation is uncommon with left atrial myxoma. The echocardiographic assessment of presence of mitral regurgitation and its severity are impaired by the presence of left atrial myxoma. We describe an uncommon association of left atrial myxoma with coronary artery disease and mitral regurgitation. MR was reported as mild on pre-operative transthoracic echocardiography but found to be severe due to ruptured chordae tendinae during intra-operative transesophageal echocardiography, which lead to change in the surgical plan to mitral valve replacement in addition to excision of myxoma.