Ventricular Septal Myectomy for Obstructive Hypertrophic Cardiomyopathy (Analysis Spanning 60 Years Of Practice): AJC Expert Panel.

Surgical myectomy remains the time-honored primary treatment for hypertrophic cardiomyopathy patients with drug refractory limiting symptoms due to LV outflow obstruction. Based on >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.

Survival Following Alcohol Septal Ablation or Septal Myectomy for Patients With Obstructive Hypertrophic Cardiomyopathy.

Background: There is little information regarding long-term mortality comparing the 2 most common procedures for septal reduction for obstructive hypertrophic cardiomyopathy (HCM), alcohol septal ablation (ASA), and septal myectomy.

Objectives: This study sought to compare the long-term mortality of patients with obstructive HCM following septal myectomy or ASA.

Methods: We evaluated outcomes of 3,859 patients who underwent ASA or septal myectomy in 3 specialized HCM centers.

Altered intercellular communication and extracellular matrix signaling as a potential disease mechanism in human hypertrophic cardiomyopathy.

Hypertrophic cardiomyopathy (HCM) is considered a primary disorder of the sarcomere resulting in unexplained left ventricular hypertrophy but the paradoxical association of nonmyocyte phenotypes such as fibrosis, mitral valve anomalies and microvascular occlusion is unexplained. To understand the interplay between cardiomyocyte and nonmyocyte cell types in human HCM, single nuclei RNA-sequencing was performed on myectomy specimens from HCM patients with left ventricular outflow tract obstruction and control samples from donor hearts free of cardiovascular disease. Clustering analysis based on gene expression patterns identified a total of 34 distinct cell populations, which were classified into 10 different cell types based on marker gene expression.

Outcomes Over Follow-up ≥10 Years After Surgical Myectomy for Symptomatic Obstructive Hypertrophic Cardiomyopathy.

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.

Myocardial Bridge or Something Else?

In this E-Challenge, the authors report on a patient with symptoms of exertional dyspnea and angina, scheduled to have surgical unroofing of an identified myocardial bridge (MB). An MB is very common in patients with hypertrophic cardiomyopathy (HCM). Intraoperative transesophageal echocardiography with provocative maneuvers revealed the patient had a systolic anterior motion of the mitral valve with septal contact and resulting outflow tract obstruction despite the notable absence of significant basal septal hypertrophy.

Comorbidity Burden and Adverse Outcomes After Transcatheter Aortic Valve Replacement.

Background Transcatheter aortic valve replacement (TAVR) has become the preferred treatment for symptomatic patients with aortic stenosis and elevated procedural risk. Many deaths following TAVR are because of noncardiac causes and comorbid disease burden may be a major determinant of postprocedure outcomes. The prevalence of comorbid conditions and associations with outcomes after TAVR has not been studied.

Plasma Proteomic Profiling in Hypertrophic Cardiomyopathy Patients before and after Surgical Myectomy Reveals Post-Procedural Reduction in Systemic Inflammation.

Left Ventricular Outflow Tract (LVOT) obstruction occurs in approximately 70% of Hypertrophic Cardiomyopathy (HCM) patients and currently requires imaging or invasive testing for diagnosis, sometimes in conjunction with provocative physiological or pharmaceutical stimuli. To identify potential biomarkers of LVOT obstruction, we performed proteomics profiling of 1305 plasma proteins in 12 HCM patients with documented LVOT obstruction, referred for surgical myectomy. Plasma was collected at the surgical preoperative visit, approximately one month prior to surgery and then at the post-surgical visit, approximately 3 months later.

Adult Monozygotic Twins With Hypertrophic Cardiomyopathy and Identical Disease Expression and Clinical Course.

A unique clinical circumstance involving middle-aged male identical twins with obstructive hypertrophic cardiomyopathy (HC) is reported. The concordance of morphologic (i.e.

Intraoperative Provocative Testing in Patients with Obstructive Hypertrophic Cardiomyopathy Undergoing Septal Myectomy.

Background: Resolution of left ventricular outflow tract (LVOT) obstruction predicts symptom relief postmyectomy. Intraoperative measurement of LVOT gradients thus is essential for surgical guidance. We hypothesized that (1) hypertrophic cardiomyopathy patients have lower LVOT gradients when measured intraoperatively with transesophageal echocardiography (TEE) compared with preoperative measurements with transthoracic echocardiography (TTE) and that (2) intraoperative provocative testing can help evaluate the adequacy of surgical resection.

Efficacy of Combined Cox-Maze IV and Ventricular Septal Myectomy for Treatment of Atrial Fibrillation in Patients With Obstructive Hypertrophic Cardiomyopathy.

Atrial fibrillation (AF) has important clinical consequences in hypertrophic cardiomyopathy (HC). Safety and efficacy of the Cox-Maze IV procedure (when combined with ventricular septal myectomy) in patients with obstructive HC and paroxysmal AF is largely unresolved. Records of 395 consecutive HC patients (age 55 ± 13 years) who underwent septal myectomy for heart failure symptoms between 2004 and 2015 were reviewed.

A case series: the outcomes, support duration, and graft function recovery after VA-ECMO use in primary graft dysfunction after heart transplantation.

Primary graft dysfunction (PGD) is a rare complication associated with high mortality after heart transplantation, which may require veno-arterial extra-corporeal membrane oxygenation (VA-ECMO) support. A standardized definition for PGD was developed by the International Society of Heart and Lung Transplantation in 2014. Due to limited reports using this definition, the detailed outcomes after VA-ECMO support remain unclear.

Distal Embolic Protection in Impella 5.0 Explantation: Loop and Snare Technique.

The left ventricular assist device Impella 5.0 (Abiomed Inc, Danvers, MA) has become widely accepted as a temporary mechanical circulatory support for patients in cardiogenic shock. The Impella 5.

Guideline-Based Referral for Septal Reduction Therapy in Obstructive Hypertrophic Cardiomyopathy Is Associated With Excellent Clinical Outcomes.

Background: The outcome of medically refractory patients with obstructive hypertrophic cardiomyopathy treated according to the American College of Cardiology/American Heart Association consensus guideline recommendations is not known. The objectives of this study were to define the short- and long-term outcomes of medically refractory obstructive hypertrophic cardiomyopathy patients undergoing alcohol septal ablation (ASA) and surgical septal myectomy (SM) with patient management in accordance with these consensus guidelines, as well as to quantify procedural risk and burden of comorbid conditions at the time of treatment.

Methods And Results: Patients with obstructive hypertrophic cardiomyopathy referred for either ASA or SM from 2004 to 2015 were followed for the primary end point of short- and long-term mortality and compared with respective age- and sex-matched US populations.

Right Transaxillary Transcatheter Aortic Valve Replacement Using the "Flip-n-Flex" Technique.

Background: Right transaxillary transcatheter aortic valve replacement (TAx-TAVR) is rarely performed due to challenging vascular tortuosity and unfavorable implantation angles. We explored this procedure using the newer-generation balloon-expandable SAPIEN 3 (Edwards Lifesciences, Irvine, CA) valve and our novel "flip-n-flex" technique. The safety and effectiveness of the procedure and the learning curve of our early experiences were investigated.

Impact of Effective Management Strategies on Patients With the Most Extreme Phenotypic Expression of Hypertrophic Cardiomyopathy.

Advances in treatment options for hypertrophic cardiomyopathy (HC) have proven effective in many patients for promoting favorable long-term outcomes. Whether this expectation is similar for patients with the most extreme expression of massive left ventricular (LV) hypertrophy, a particularly aggressive form of the disease is unresolved. Of 1,766 consecutive HC patients presenting to Tufts HC Institute (2004 to 2015), 92 were identified with extreme LV wall thickness (30 to 48 mm), and compared with 1,674 HC patients with less marked hypertrophy (13 to 29 mm).

Clinical Spectrum and Management Implications of Left Ventricular Outflow Obstruction With Mild Ventricular Septal Thickness in Hypertrophic Cardiomyopathy.

Hypertrophic cardiomyopathy (HC) has historically been characterized as a disease of substantial left ventricular hypertrophy, often associated with dynamic left ventricular outflow tract obstruction. However, we have recently encountered patients with subaortic obstruction and only minimal basal septal thickness, raising important management implications. Thereby, we sought to characterize the natural history and treatment strategies for this under-recognized subgroup of HC patients with dynamic obstruction.

Late Clinical Presentation of Prosthesis-Patient Mismatch Following Transcatheter Aortic Valve Replacement.

Prosthesis-patient mismatch (PPM) is relatively common after aortic valve replacement (AVR) and generally is associated with reduced regression of left ventricular (LV) mass. PPM after valve-in-valve transcatheter aortic valve replacement (TAVR) was reported to be 38%. PPM generally is manifested clinically by dyspnea and echocardiographically by high transvalvular gradients.

Surgical Approaches to Hypertrophic Obstructive Cardiomyopathy.

Left ventricular outflow tract obstruction secondary to hypertrophic obstructive cardiomyopathy remains a challenging entity facing clinicians. Despite the success of invasive therapies, some clinicians remain hesitant because of early results with unacceptable morbidity and mortality rates. However, current literature strongly suggests improved short- and long-term outcomes with extended septal myectomy and alcohol septal ablation compared with patients not undergoing such interventions.

Clinical Profile of Nonresponders to Surgical Myectomy with Obstructive Hypertrophic Cardiomyopathy.

Background: Surgical myectomy reverses heart failure symptoms in the vast majority of obstructive hypertrophic cardiomyopathy patients. However, a small subgroup fails to experience sustained postoperative improvement despite relief of obstruction. Clinical profile of such patients has not been well defined.

Interaction of Adverse Disease Related Pathways in Hypertrophic Cardiomyopathy.

Hypertrophic cardiomyopathy (HC) has been characterized as a generally progressive genetic heart disease, creating an ominous perspective for patients and managing cardiologists. We explored the HC disease burden and interaction of adverse clinical pathways to clarify patient expectations over long time periods in the contemporary therapeutic era. We studied 1,000 consecutive HC patients (52 ± 17 years) at Tufts Medical Center, followed 9.

Results of surgical septal myectomy for obstructive hypertrophic cardiomyopathy: the Tufts experience.

Background: For over 50 years, surgical septal myectomy has been the preferred treatment for drug-refractory heart failure symptoms in patients with obstructive hypertrophic cardiomyopathy (HCM). Over this time in the United States, the majority of myectomy operations have been performed in a small number of select referral centers.

Methods: We have taken the opportunity to report results from the relatively new Tufts HCM Center and surgical program, incorporated 13 years ago, during which 507 myectomies (52±14 years of age; 56% male) were performed by one cardiothoracic surgeon, Dr.

Clinical Profile and Consequences of Atrial Fibrillation in Hypertrophic Cardiomyopathy.

Background: Atrial fibrillation (AF), the most common sustained arrhythmia in hypertrophic cardiomyopathy (HCM), is capable of producing symptoms that impact quality of life and is associated with risk for embolic stroke. However, the influence of AF on clinical course and outcome in HCM remains incompletely resolved.

Methods: Records of 1558 consecutive patients followed at the Tufts Medical Center Hypertrophic Cardiomyopathy Institute for 4.

Underappreciated occurrence of discrete subaortic membranes producing left ventricular outflow obstruction in hypertrophic cardiomyopathy.

Subaortic obstruction due to systolic anterior motion (SAM) of the mitral valve with ventricular septal contact is a major cause of progressive heart failure symptoms in patients with hypertrophic cardiomyopathy (HCM). However, we have recently observed a unique, but not uncommon subgroup of HCM patients with outflow tract obstruction due only to discrete subaortic membrane or who have a membrane in addition to SAM-septal contact. HCM patients with subaortic membranes may be at increased risk for developing progressive heart failure symptoms.