Left Ventricular Outflow Obstruction Research Articles

Absent pulmonary valve with tricuspid atresia/stenosis: literature review with new three long-term cases.

Absent pulmonary valve (APV) syndrome with tricuspid atresia or tricuspid stenosis (TA/TS) is an extremely rare malformation recently reported as a variant of APV with intact ventricular septum (VS). The condition, however, has univentricular physiology and unique structural and clinical features. The purpose of this study was to update the current knowledge about this condition by describing long-term outcomes of three new cases and reviewing the available literatures. A systematic literature search was performed to collect clinical and anatomical data of APV with TA/TS. Institutional medical records were retrospectively reviewed to identify APV with TA/TS patients. In a total of 62 (59 reported and 3 new) cases, patent ductus arteriosus was present in 98% of APV patients with TA/TS. A large ventricular septal defect, dilatation of the pulmonary arteries, which is typically found in APV with tetralogy of Fallot, and respiratory distress at birth were rarely reported. Most of the recent cases were successfully managed by the Glenn or Fontan procedure. Coronary artery anomaly and ventricular arrhythmia were more frequently reported as the cause of death or severe neurological sequelae (9/16 and 3/8, respectively). Additional surgical intervention was required in the mid/long-term period in three cases due to left-ventricular outflow obstruction and in two due to aortic dilatation. The Fontan and Glenn procedures improved the survival in the last two decades. In addition to coronary artery anomaly and ventricular arrhythmia, left-ventricular outflow tract obstruction and aortic dilatation should be carefully monitored.

Outcomes Following Surgery to Address Shone Syndrome in Children.

Shone syndrome is characterized by coincident mitral valve stenosis and left ventricular outflow tract obstruction. Although first described in 1963, little research has expounded surgical outcomes. We sought to evaluate our experience with this cohort, emphasizing outcomes including mortality, morbidity, and cardiac function. A retrospective chart review of 46 patients who underwent operation for Shone syndrome between 1990 and May 2018 was conducted. Index operations included 32 repairs of the left ventricular outflow tract, four mitral valve repair/replacements, nine combined repairs, and one non-Shone's repair. Median age at index procedure was 22 days (2 days-10 years). Mean follow-up was 9.1 years (2 months-21 years), and 70 additional operations (51 reoperations) were required. Three patients were lost to follow-up. Overall survival was 95.7% with two late deaths. Freedom from death or transplant was 93.5%. Thirteen (28.3%) patients remained free from reoperation. Thirty-three patients required 51 reoperations of the left ventricle outflow tract (n = 12), mitral valve (n = 16), combined repairs (n = 21), and transplant (n = 1). At most recent follow-up, patients exhibited mitral stenosis (n = 21), aortic stenosis (n = 7), and diminished LV function (n = 2). Surgical correction of Shone's offers excellent survival benefit, but reoperation burden is high, with >70% of patients requiring reintervention in the follow-up period. A total of 65% of patients developed recurrent obstruction of left ventricular inflow or outflow, however, ventricular function is preserved in the majority of patients. All but one patient had no functional deficits, classified as New York Heart Association I with > 60% requiring no medication.

Genetic and Developmental Contributors to Aortic Stenosis.

Aortic stenosis (AS) remains one of the most common forms of valve disease, with significant impact on patient survival. The disease is characterized by left ventricular outflow obstruction and encompasses a series of stenotic lesions starting from the left ventricular outflow tract to the descending aorta. Obstructions may be subvalvar, valvar, or supravalvar and can be present at birth (congenital) or acquired later in life. Bicuspid aortic valve, whereby the aortic valve forms with two instead of three cusps, is the most common cause of AS in younger patients due to primary anatomic narrowing of the valve. In addition, the secondary onset of premature calcification, likely induced by altered hemodynamics, further obstructs left ventricular outflow in bicuspid aortic valve patients. In adults, degenerative AS involves progressive calcification of an anatomically normal, tricuspid aortic valve and is attributed to lifelong exposure to multifactoral risk factors and physiological wear-and-tear that negatively impacts valve structure-function relationships. AS continues to be the most frequent valvular disease that requires intervention, and aortic valve replacement is the standard treatment for patients with severe or symptomatic AS. While the positive impacts of surgical interventions are well documented, the financial burden, the potential need for repeated procedures, and operative risks are substantial. In addition, the clinical management of asymptomatic patients remains controversial. Therefore, there is a critical need to develop alternative approaches to prevent the progression of left ventricular outflow obstruction, especially in valvar lesions. This review summarizes our current understandings of AS cause; beginning with developmental origins of congenital valve disease, and leading into the multifactorial nature of AS in the adult population.

A Case of Ebstein Anomaly with Left Ventricular Noncompaction and Anomalous Mitral Chordae to the Septum

Left-sided involvement is increasingly recognized in cases of Ebstein anomaly (EA) of tricuspid valve, thus making it not an exclusive right-sided disease, rather a global form of heart disease, a type of cardiomyopathy. Left-sided involvement commonly occurs in the form of noncompaction. Mitral valve prolapse and other forms of mitral and aortic valve abnormalities are also associated with EA that contribute to the patient's symptoms and prognosis. Here is a case report of EA in a 25-year-old female presenting with dyspnea. Echocardiography revealed left ventricular (LV) noncompaction fulfilling Jenni criteria. In addition, there was an abnormal chordal attachment from the anterolateral papillary muscle to the ventricular septum. There was no LV outflow obstruction, ventricular functions were normal. The combined involvement of LV myocardium and mitral apparatus in EA made this case unique in its category.

Echocardiographic Characteristics of Severe Left Ventricular Outflow Tract Obstruction in Hypertrophic Cardiomyopathy

It is important to diagnose severe left ventricular outflow tract (LVOT) obstruction in symptomatic patients with hypertrophic cardiomyopathy (HCM) to determine who might benefit from septal reduction therapy. Due to its dynamic nature, LVOT obstruction may not be present at rest, 1 Maron M.S. Olivotto I. Betocchi S. Casey S.A. Lesser J.R. Losi M.A. et al. Effect of left ventricular outflow tract obstruction on clinical outcome in hypertrophic cardiomyopathy. New Engl J Med. 2003; 348: 295-303 Crossref PubMed Scopus (1012) Google Scholar , 2 Maron M.S. Olivotto I. Zenovich A.G. Link M.S. Pandian N.G. Kuvin J.T. et al. Hypertrophic cardiomyopathy is predominantly a disease of left ventricular outflow tract obstruction. Circulation. 2006; 114: 2232-2239 Crossref PubMed Scopus (650) Google Scholar , 3 Ayoub C. Geske J.B. Larsen C.M. Scott C.G. Klarich K.W. Pellikka P.A. Comparison of Valsalva maneuver, amyl nitrite, and exercise echocardiography to demonstrate latent left ventricular outflow obstruction in hypertrophic cardiomyopathy. Am J Cardiol. 2017; 120: 2265-2271 Abstract Full Text Full Text PDF PubMed Scopus (19) Google Scholar and it is then necessary to “search for obstruction” with further provocative testing. 4 Ommen S.R. Mital S. Burke M.A. Day S.M. Deswal A. Elliott P. et al. 2020 AHA/ACC guideline for the diagnosis and treatment of patients with hypertrophic cardiomyopathy: a report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines. J Am Coll Cardiol. 2020; 76: e159-e240 Crossref PubMed Scopus (76) Google Scholar , 5 Geske J.B. Sorajja P. Ommen S.R. Nishimura R.A. Left ventricular outflow tract gradient variability in hypertrophic cardiomyopathy. Clin Cardiol. 2009; 32: 397-402 Crossref PubMed Scopus (54) Google Scholar , 6 Nishimura R.A. Ommen S.R. Hypertrophic cardiomyopathy: the search for obstruction. Circulation. 2006; 114: 2200-2202 Crossref PubMed Scopus (41) Google Scholar We sought to determine whether there are resting echocardiographic characteristics associated with latent LVOT obstruction.

The Story of a Broken Heart: Takotsubo Cardiomyopathy

Licensed independent practitioners in emergency clinical practice are tasked with differentiating acute cardiac presentations. Despite its similarity in clinical presentation to acute coronary syndrome, Takotsubo cardiomyopathy is a unique cardiac disorder characterized by a stress-induced ballooning of the myocardium. Also known as the broken heart syndrome, Takotsubo cardiomyopathy most frequently occurs after an overwhelming emotional or physical stressor. The subsequent impaired contractility of the heart places the patient at risk of complications, including acute heart failure, cardiogenic shock, thromboembolism, arrhythmias, and left ventricular outflow obstruction. Takotsubo cardiomyopathy is similar in presentation to other cardiac disorders; therefore, clinicians in emergency settings must be efficient and effective in their diagnosis of this disorder on the basis of its distinct criteria. The current article uses most recent evidence to describe the etiology, pathophysiology, diagnosis, and recommended treatment for Takotsubo cardiomyopathy to support licensed independent practitioners in emergency departments in improving patient outcomes and reducing morbidity.

Advances in Transcatheter Electrosurgery for Treating Valvular Heart Disease

Delivery of electrosurgery energy through catheters and guidewires enables interventionists to ‘cut’ through obstructive intravascular lesions or across cardiac chambers. A novel application of transcatheter electrosurgery is to make controlled lacerations in heart valve leaflets. This review describes three applications of transcatheter electrosurgery of aortic and mitral valve leaflets to enable transcatheter heart valve implantation. Intentional laceration of the anterior mitral leaflet to prevent left ventricular outflow obstruction splits and splays the anterior mitral valve and enables transcatheter mitral valve replacement without left ventricular outflow tract obstruction. Technique modifications and novel applications are described. Bioprosthetic or native aortic scallop intentional laceration to prevent iatrogenic coronary artery obstruction enables transcatheter aortic valve replacement without coronary artery obstruction. The technique is described and novel uses, especially in the setting of repeat transcatheter aortic valve replacement, are discussed. Finally, electrosurgical laceration and stabilization of mitral valve clip devices (ELASTA-Clip) enables transcatheter mitral valve replacement after MitraClip implantation. In conclusion, transcatheter electrosurgery is an important and versatile new tool in structural heart intervention.

Who are at high risk of mortality and morbidity among children with congenital heart disease undergoing noncardiac surgery?

With advances in the development of surgical and medical treatments for congenital heart disease (CHD), the population of children and adults with CHD is growing. This population requires multiple surgical and diagnostic imaging procedures. Therefore, general anesthesia is inevitable. In many studies, it has been reported that children with CHD have increased anesthesia risks when undergoing noncardiac surgeries compared to children without CHD. The highest risk group included patients with functional single ventricle, suprasystemic pulmonary hypertension, left ventricular outflow obstruction, and cardiomyopathy. In this review, we provide an overview of perioperative risks in children with CHD undergoing noncardiac surgeries and anesthetic considerations in patients classified as having the highest risk.

Open Trans-Catheter Mitral Valve Replacement for Mitral Annular Calcification

Mitral valve replacement in patients with severe mitral annular calcification is a high-risk operation with historically poor outcomes. Percutaneous trans-catheter heart valve (THV) implantation is an alternative therapy to the traditional open surgical approach, but carries significant risks including severe paravalvular leak, left ventricular outflow obstruction, and embolization. To address this, alternative treatment modalities have emerged from the growing experience with THV. Here, we describe an open trans-atrial implantation of a balloon expandable THV. Relative to percutaneous therapies, an open approach permits implanting and expanding the valve under direct visual guidance, with anchoring sutures to precisely position the valve. In comparison to traditional surgical approaches, the risk of left circumflex arterial injury and atrioventricular dissociation is reduced as the annular calcification is left alone and no suture lines are run along the annulus. We illustrate this technique using an Edwards Sapien 3 bioprosthetic valve with an Edwards certitude transthoracic delivery system. Mitral valve replacement in patients with severe mitral annular calcification is a high-risk operation with historically poor outcomes. Percutaneous trans-catheter heart valve (THV) implantation is an alternative therapy to the traditional open surgical approach, but carries significant risks including severe paravalvular leak, left ventricular outflow obstruction, and embolization. To address this, alternative treatment modalities have emerged from the growing experience with THV. Here, we describe an open trans-atrial implantation of a balloon expandable THV. Relative to percutaneous therapies, an open approach permits implanting and expanding the valve under direct visual guidance, with anchoring sutures to precisely position the valve. In comparison to traditional surgical approaches, the risk of left circumflex arterial injury and atrioventricular dissociation is reduced as the annular calcification is left alone and no suture lines are run along the annulus. We illustrate this technique using an Edwards Sapien 3 bioprosthetic valve with an Edwards certitude transthoracic delivery system.

Half-Turned Truncal Switch Operation for Transposition of The Great Arteries with Left Ventricular Outflow Obstruction

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.

Comprehensive assessment of the aortic valve in critically ill patients for the non-cardiologist. Part I: Aortic stenosis of the native valve.

Aortic stenosis (AS) causes left ventricular outflow obstruction. Severe AS has major haemodynamic implications in critically ill patients, in whom increased cardiac output and oxygen delivery are often required. Transthoracic echocardiography (TTE) plays akey role in the AS severity grading. In this review, we will give an overview of how to use the simplified Bernoulli equation to convert the echo Doppler measured velocities (cm s-1) to AS peak and mean gra-dient (mm Hg) and how to calculate the aortic valve area (AVA), using the continuity equation, based on the principle of preservation of flow. TTE allows quantification of compensatory left ventricular (LV) hypertrophy, assessment of LV systolic function, and determination of LV diastolic function and LV loading. Subsequently, the obtained results from the TTE study need to be integrated to establish the AS severity grading. The pitfalls of echocardiographic AS severity assessment are explained, and how to deal with inconsistency between AVA and mean gradient. The contribution of transoesophageal echocardiography, low-dose dobutamine stress echo (in case of low-flow low-gradient AS), echocardiography strain imaging, cardiac magnetic resonance imaging, cardiac multidetector computed tomography and the relatively new concept of Flow Pressure Gradient Classification to the work-up for aortic stenosis is discussed. Finally, the treatment of AS is overviewed. Elective aortic valve replacement is indicated in patients with severe symptomatic AS. In the ICU, afterload reduction by vasodilator therapy and treatment of pulmonary and venous congestion by diuretics could be considered.

Left ventricular outflow obstruction occurs immediately after exercise in patients with non-obstructive hypertrophic cardiomyopathy

We evaluated the hemodynamic and geometric determinants of latent obstruction (LO, trans-left ventricular outflow tract (LVOT) gradient ≥30 mmHg with provocation) in patients with non-obstructive hypertrophic cardiomyopathy (HCMP). A total of 35 patients with non-obstructive HCMP underwent stepwise supine bicycle exercise echocardiography. Trans-LVOT pressure gradients, mitral geometric parameters, left ventricular ejection fractions (LVEF) and left ventricular end-systolic and diastolic dimensions (LVESD, LVEDD) were measured at each stage. The highest peak LVOT pressure gradient predominantly occurred immediately after exercise (n = 32, 91.3%) rather than during peak exercise (n = 3, 8.7%). Significant LO developed in nine patients (25%). No significant differences were found in resting echocardiographic parameters. Compared to the remaining patients, however, patients with LO had longer residual mitral leaflets (defined as residual portions of leaflets after coaptation; 4 ± 4 vs. 13 ± 4 mm, respectively; p = 0.001) and higher resting LVOT pressure gradients (7.4 ± 3.7 vs. 12.9 ± 5.8 mmHg, respectively; p = 0.001). Substantial decreases in mitral annular diameters from peak exercise to recovery after exercise were observed in the LO group, while mitral annular diameters increased after exercise in the non-LO group. In conclusion, the highest peak LVOT pressure gradient predominantly occurred immediately after exercise rather than during peak exercise, regardless of LO. Abrupt decrease of mitral annular diameter immediately after exercise, a longer residual mitral leaflet and a higher resting LVOT pressure gradient at rest might be related to LO.

Effects of combined myectomy with mitral valve repair on a three-dimensional mitral valve model and left ventricle outflow obstruction in patients with hypertrophic cardiomyopathy

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

Low Risk of Hypertrophic Cardiomyopathy With Contemporary Management Strategies Implemented in Non-Referral Regional Community-Based Practices

Major advances in diagnosis and treatment have emerged for hypertrophic cardiomyopathy (HCM), largely in major tertiary referral centers dedicated to this disease. Whether these therapeutic benefits are confined to patients in such highly selected cohorts, or can be implemented effectively in independent regional or community-based populations is not generally appreciated. We assessed management and clinical outcomes in a non-referral HCM center (n = 214 patients) in Eastern Pennsylvania. Over a 6.0 ± 3.2-year follow-up, the HCM-related mortality rate was 0.1% per year attributed to a single disease-related death, in a 49-year-old man with end-stage heart failure, ineligible for heart transplant. Fifteen patients (7%) with prophylactically placed implantable cardioverter-defibrillators (ICDs) experienced appropriate therapy terminating life-threatening ventricular tachyarrhythmias. In 23 other patients (11%; 5%/year), heart failure due to left ventricular outflow obstruction was reversed by surgical septal myectomy (n = 20) or percutaneous alcohol septal ablation (n = 3). This regional HCM cohort was similar to a comparison tertiary center referral population in terms of HCM-mortality: 0.1%/year vs 0.3%/year (p = 0.3) and ICD therapy (31% vs 16% of primary prevention implants), although more frequently with uncomplicated benign clinical course (62% vs 46%; p <0.01). In conclusion, effective contemporary HCM management strategies and outcomes in referral-based HCM centers can be successfully replicated in regional and/or non-referral settings. Therefore, HCM is now a highly treatable disease compatible with normal longevity when assessed in a variety of clinical venues not limited to tertiary centers.

Clinical analysis of 14 infective endocarditis in patients with obstructive hypertrophic cardiomyopathy

Objective: This observational study was aimed to analyze the clinical characteristics of infective endocarditis (IE) in patients with hypertrophic cardiomyopathy (HCM). Methods: A total of 668 patients with IE, and 7 427 patients with HCM were treated in Fuwai Hospital from August 2006 to December 2018. Among them, 14 patients were diagnosed with HCM and IE. The clinical characteristics of these patients including clinical manifestations, pathogen distribution, echocardiography features, in-hospital treatment and outcomes were analyzed retrospectively. Results: The proportion of HCM patients with IE was 0.19%,with the estimated incidence of 0.15/1 000 person-years in HCM patients. Of the 14 patients, 11 patients were male. The most common clinical manifestations were fever and heart murmur, and the main complications were heart failure (12/14) and bacterial embolism (8/14). There were 8 cases (8/14) with positive blood culture, and all causative bacteria were gram positive coccus, in which 5/8 were Streptococcus. The median interventricular septum thickness was (21.2±2.7) mm, and left ventricular outflow obstruction was severe based on echocardiography (Echo) examination. The Echo showed that vegetation was found in all 14 patients and most of the vegetation attached at the anterior leaflet of mitral valve (12/14). The proportions of patients with circulatory embolism (8/14) and valve lesions (12/14) were relatively high. Most cases (10/14) were cured, especially those underwent cardiac surgery (8 cases). The rest 4 cases died with 2 in hospital and 2 after auto-discharge. Conclusions: HCM patients complicated with IE are rare. Septic embolization and valve lesions are common in these patients. IE patients with HCM might have a poor prognosis compared to those without HCM and should receive cardiac surgery as early as possible.

Seria niefortunnych zdarzeń — przewlekła okluzja tętnicy wieńcowej, migotanie przedsionków i udar niedokrwienny. Czy optymalna antykoagulacja jest w tym przypadku możliwa?

Successful chronic total occlusion (CTO) percutaneous coronary intervention (PCI) has been related to discordant benefits concerning clinical and quality life measures. We present a patient previously diagnosed with hypertrophic cardiomyopathy with left ventricular outflow obstruction referred for a CTO of the left anterior descending artery PCI. Crossing via left anterior descending artery was complicated by perforation resulting in haemopericardium (no evidence of tamponade; the patient remained hemodynamically stable). On subsequent days the patient presented with two asymptomatic paroxysmal atrial fibrilation (AF) episodes ( de novo ). In this patient’s case, the stroke’s risk factors did not mandate anticoagulation due to haemopericardium and arrhythmia duration of < 48 hours. Twenty-six hours after the second AF episode, the patient suffered a large ischemic stroke in the territory supplied by the right middle cerebral artery. Despite mechanical thrombectomy (MT), the patient died. Even though CTO PCI is an appealing choice in patients with complex coronary artery disease and chronic total occlusion, the risk still should not be underestimated. Another issue is assessing the thromboembolic risk associated with acute cardioversion of patients with AF estimated to be of < 48 hours duration — it is usually considered low. However, there is increasing evidence that short runs of AF confer a significant risk of stroke. Moreover, it was shown that, among patients undergoing MT for acute ischemic stroke, women had worse outcomes at 90 days.

Impaired left atrial stiffness in patients with corrected congenital left ventricular outflow obstructions.

We examined the left atrial stiffness index (LA Stiff) on echocardiography and its determinants in adults and adolescents with repaired congenital left ventricular outflow obstructions (c-LVOOs), including isolated subaortic stenosis (SAS), stenotic bicuspid aortic valve (BAV), and aortic coarctation (CoA). Seventy-two patients (43 males, age: 31 ± 15 years) with repaired c-LVOOs (SAS: n = 12; BAV: n = 27; CoA: n = 33) were compared to 72 age- and sex-matched controls. 2D strain imaging was performed to assess left ventricular (LV) and LA function, including peak positive longitudinal LA strain (LAS), late diastolic LA strain (LDS), and LV global longitudinal strain (GLS). The (E/Ea)/LAS ratio was used to calculate LA Stiff. LA Stiff was significantly higher in the c-LVOO group than in the control group. Among the c-LVOO patients, the CoA group had the lowest GLS and the highest LA Stiff; no significant differences were found with respect to sex, hypertension history, smoking status, or repeated repair among c-LVOO subtypes. Multivariable regression analysis with the variables "BMI" and "c-LVOO subtype" revealed that BMI and c-LVOO subtypes were independently associated with LA Stiff (b = 0.290, P = .009 and b = 0.353, P = .002, respectively). We documented abnormal LA Stiff values in adults and adolescents after c-LVOO repair. Patients with CoA demonstrated the most impaired LA Stiff values. Overweight may contribute to worse LA Stiff values. Further studies are required to determine the prognostic implications of LA Stiff in patients with repaired c-LVOOs.

Prognostic impact of atrial fibrillation in patients with hypertrophic cardiomyopathy in a community-based Japanese cohort: results from Kochi RYOMA study

Abstract Background The prognostic impact of atrial fibrillation (AF) in patients with hypertrophic cardiomyopathy (HCM) is not fully elucidated. Purpose The aim of this study was to examine the prevalence and prognostic impact of AF in a prospectively assembled community-based HCM patient cohort in an aged Japanese community. Methods In 2004, we established a cardiomyopathy registration network in Kochi Prefecture, Japan, consisting of 9 hospitals, and finally 293 patients with HCM were followed. Results The ages at registration and at diagnosis were 63±14 and 56±16 years, respectively, and 197 patients (67%) were men. 86 patients (29%) showed AF. During follow-up period of 6.1±3.2 years, 44 patients died. In those patients, HCM-related deaths occurred in 23 patients with an annual mortality rate of 1.3%. Regarding HCM-related adverse events including HCM-related deaths, appropriate ICD discharge, heart failure admission and hospitalization for embolic events, a total of 77 cardiovascular events in 70 patients occurred. Multivariate analysis revealed that presence of AF, left ventricular (LV) outflow obstruction, NYHA functional class III, and lower LV fractional shortening at registration were significant predictors of these adverse events. During the follow-up period, additional 31 patients (11%) developed new-onset AF. Importantly, the incidence of HCM-related adverse events was significantly higher in patients with new AF observed from its onset compared with those with AF at registration (log-rank p=0.029) (Figure 1). Conclusions In an unselected HCM registry in an aged Japanese community, presence of AF, particularly new-onset AF, was associated with unfavorable clinical outcomes. AF is not just a marker of the disease stage but an important trigger of HCM-related adverse events. Figure 1 Funding Acknowledgement Type of funding source: None

Systematic cutting of selected secondary mitral valve chordae, in association with a shallow myectomy, in obstructive hypertrophic cardiomyopathy:impact on mitral valve function and patient management

Abstract Introduction Few centers worldwide have large experience with performing an extended septal myectomy in patients with obstructive hypertrophic cardiomyopathy (HCM). Therefore, many HCM patients eligible for surgical relief of left ventricular (LV) outflow gradient do not have access to treatment. In a previous study, cutting fibrotic anterior mitral leaflet secondary chordae, in association with only a shallow myectomy, proved highly effective in moving the mitral valve (MV) apparatus away from the LV outflow tract and relieving the outflow gradient in our HCM patients with mild hypertrophy (&amp;lt;19 mm), a surgical approach that simplifies the operation. Purpose To assess whether chordal cutting is equally effective in improving MV geometry and relieving LV outflow gradient and heart failure symptoms in HCM patients with more marked hypertrophy. Methods Surgical outcome and MV geometry and function were assessed in 226 consecutive HCM patients who underwent systematic cutting of fibrotic anterior mitral leaflet secondary chordae, in association with a shallow myectomy and independently of magnitude of septal thickness, at our center from January 2015 to December 2018. Results Of 226 study patients, 1 (0.4%) died perioperatively. None had iatrogenic septal defect. Postoperatively, LV outflow gradient at rest decreased from 70±36 to 10±2 mmHg (P&amp;lt;0.001). In the 77 patients in whom data on the outflow gradient provoked with physiologic maneuvers after surgery were available, the provocable gradient was 16±10 mmHg. NYHA functional class improved significantly (P&amp;lt;0.001), with the number of patients in class III-IV decreasing from 178 (79%) to 2 (0.9%). No patient had residual severe MV regurgitation and only 4 (1.7%) had moderate-to-severe regurgitation. Quality of the echocardiogram allowed assessment of MV geometry in 212 (94%) patients. In the 62 patients with mild hypertrophy, anterior leaflet-annulus ratio increased 27% postoperatively, from 0.43+0.06 to 0.55+0.06 and MV tenting area decreased 34% from 2.9+0.6 to 1.9+0.4 cm2 (P&amp;lt;0.001), indicating repositioning of MV coaptation away from the outflow tract (with increased outflow tract dimension). Similarly, in 150 patients with marked hypertrophy, anterior leaflet-annulus ratio increased 27% from 0.43+0.05 to 0.55+0.06 and tenting area decreased 28% from 2.9+0.6 to 2.1+0.4 cm2 (P&amp;lt;0.001). Conclusions Our results show that cutting fibrotic anterior mitral leaflet secondary chordae, by moving the MV apparatus away from the LV outflow tract and independently of the magnitude of septal hypertrophy, contributes to improve the results of septal myectomy and reduces the need for a deep septal excision (and associated risk of iatrogenic septal defect) in patients with obstructive HCM. Therefore, chordal cutting could make the myectomy operation more accessible to surgeons, increasing the availability of surgical treatment for HCM patients eligible for invasive abolition of LV outflow obstruction. Funding Acknowledgement Type of funding source: None

Aortic root enlargement: When and how.

Aortic valve replacement is the definitive management for severe aortic stenosis. Intraoperatively, an aortic root enlargement (ARE) may be used to facilitate the implementation of a suitably sized prosthetic valve. This is to prevent patient prosthesis mismatch (PPM), a condition that causes a left ventricular outflow obstruction. There are four main techniques that are used to perform ARE, namely, Nicks, Manouguian, Nunez (modified Manouguian), and Kanno-Rastan procedures. They each involve incisions through different anatomical structures and allow a variety of valve sizes to be implanted. Studies prove that ARE effectively reduces the incidence of PPM. In addition, they show that there is no definitive link between ARE and perioperative mortality or other complications. There is a scarcity of literature exploring the comparative outcomes of each surgical technique. Therefore, further research is warranted for these procedures to be compared adequately. This review aims to summarise the available literature surrounding ARE with respect to three main questions. (1) What are the indications for ARE, (2) what surgical techniques exist to facilitate ARE, and (3) are there significant differences in patient outcomes when these surgical techniques are employed?