2024 AHA/ACC/AMSSM/HRS/PACES/SCMR Guideline for the Management of Hypertrophic Cardiomyopathy: A Report of the American Heart Association/American College of Cardiology Joint Committee on Clinical Practice Guidelines.

AIM: The "2024 AHA/ACC/AMSSM/HRS/PACES/SCMR Guideline for the Management of Hypertrophic Cardiomyopathy" provides recommendations to guide clinicians in the management of patients with hypertrophic cardiomyopathy. METHODS: A comprehensive literature search was conducted from September 14, 2022, to November 22, 2022, encompassing studies, reviews, and other evidence on human subjects that were published in English from PubMed, EMBASE, the Cochrane Library, the Agency for Healthcare Research and Quality, and other selected databases relevant to this guideline. Additional relevant studies, published through May 23, 2023, during the guideline writing process, were also considered by the writing committee and added to the evidence tables, where appropriate. STRUCTURE: Hypertrophic cardiomyopathy remains a common genetic heart disease reported in populations globally. Recommendations from the "2020 AHA/ACC Guideline for the Diagnosis and Treatment of Patients With Hypertrophic Cardiomyopathy" have been updated with new evidence to guide clinicians.

2024 AHA/ACC/AMSSM/HRS/PACES/SCMR Guideline for the Management of Hypertrophic Cardiomyopathy: A Report of the American Heart Association/American College of Cardiology Joint Committee on Clinical Practice Guidelines.

AIM: The "2024 AHA/ACC/AMSSM/HRS/PACES/SCMR Guideline for the Management of Hypertrophic Cardiomyopathy" provides recommendations to guide clinicians in the management of patients with hypertrophic cardiomyopathy. METHODS: A comprehensive literature search was conducted from September 14, 2022, to November 22, 2022, encompassing studies, reviews, and other evidence on human subjects that were published in English from PubMed, EMBASE, the Cochrane Library, the Agency for Healthcare Research and Quality, and other selected databases relevant to this guideline. Additional relevant studies, published through May 23, 2023, during the guideline writing process, were also considered by the writing committee and added to the evidence tables, where appropriate. STRUCTURE: Hypertrophic cardiomyopathy remains a common genetic heart disease reported in populations globally. Recommendations from the "2020 AHA/ACC Guideline for the Diagnosis and Treatment of Patients With Hypertrophic Cardiomyopathy" have been updated with new evidence to guide clinicians.

Heart Transplantation for Ebstein Anomaly due to Late Surgical Presentation.

Ebstein anomaly is a myopathy of the right ventricle characterized by failure of tricuspid valve delamination with accompanying tricuspid regurgitation. Its rarity, considerable anatomic variability, and frequent absence of symptoms can make the timing of surgery challenging. Contemporary tricuspid repair techniques can be performed with low mortality and bidirectional cavopulmonary anastomosis reduces the risk of operation when right ventricular function is poor. Here, we present a patient who presented late for surgery, failed high-risk conventional surgery and required heart transplantation.

Single Center Prospective Study of Cross-Clamp versus Balloon Occlusion in Robotic Mitral Surgery.

BACKGROUND: Both transthoracic aortic cross-clamp and endoaortic balloon occlusion have been shown to have comparable safety profiles for aortic occlusion. Since most surgeons use only one technique, we sought to compare the outcomes when a homogeneous group of surgeons changed their occlusion technique from aortic cross-clamp to balloon occlusion. METHODS: We changed our technique from aortic cross-clamp to balloon occlusion in November 2022. This allowed us to conduct a prospective treatment comparison study in the same group of surgeons. Propensity score matching (PSM) was used to match cases(balloon occlusion) 1:3 to controls (aortic cross-clamp) based on age, gender, body mass index, concomitant maze, and tricuspid valve repair. RESULTS: Total of 411 patients underwent robotic mitral surgery from 2020 through 2023. Using PSM, 56 balloon occlusion patients were matched to 168 aortic cross-clamp patients. Median age was 65 years (interquartile range[IQR],55.6-70.0) and the majority were males(n=119,53%). All valves were successfully repaired. Balloon occlusion had a shorter median cardiopulmonary bypass (CPB) time compared to aortic cross-clamp (84.0 vs. 94.5 min,p=0.006). Median cross-clamp time (64.0 vs. 64.0 min,p=0.483) and total surgery time (5.9 vs. 6.1 hours,p=0.495) did not differ between groups. There was no in hospital death. There were five surgeons who performed various combinations of console and bedside roles. CPB, cross-clamp, and surgery durations were not significantly affected by the different surgeon combinations. CONCLUSIONS: Compared to aortic cross-clamp, balloon occlusion has similar perioperative and early postoperative outcomes. Additionally, it likely introduces a 10-minute reduction in total CPB time.

Hypertrophic Cardiomyopathy: Preadolescence, Mitral Valve Disease, and Midventricular Obstruction.

Septal myectomy is indicated in patients with obstructive hypertrophic cardiomyopathy (HCM) who have persistent symptoms despite medical therapy, intolerance of medication side effects, or severe resting or provocable gradients. Septal myectomy at high volume centers is safe, with low operative mortality (1%) and low rates of complications such as complete heart block or ventricular septal defect (3% and 0.5%, respectively). Additionally, improved survival following myectomy has been observed when compared to patients with obstructive HCM managed medically or those with nonobstructive HCM. As a longstanding, quaternary referral center for septal myectomy, our institution has built significant experience and expertise in the surgical and medical management of HCM, including atypical HCM, defined as preadolescent patients, those with mitral valve disease, and those with isolated midventricular obstruction. The most important factor of septal myectomy in achieving complete resolution of obstruction and avoiding recurrence is the apical extent of the myectomy trough, which must extend to the septum opposite the papillary muscles. If this cannot be fully achieved via a transaortic exposure, especially in preadolescents and patients with midventricular obstruction, then a transapical approach may be needed. Mitral valve repair is rarely necessary as SAM-mediated MR resolves with adequate myectomy alone, but mitral repair is performed in cases of intrinsic valvular disease. In this manuscript we provide a summary of current operative techniques and outcomes data from our institution on the management of these various categories of HCM.

Obstruction in Hypertrophic Cardiomyopathy: Many Faces.

Hypertrophic cardiomyopathy (HCM), the most common inherited cardiomyopathy, exhibits left ventricular hypertrophy not secondary to other causes, with varied phenotypic expression. Enhanced actin-myosin interaction underlies excessive myocardial contraction, frequently resulting in dynamic obstruction within the left ventricle. Left ventricular outflow tract obstruction, occurring at rest or with provocation in 75% of HCM patients, portends adverse prognosis, contributes to symptoms, and is frequently a therapeutic target. Transthoracic echocardiography plays a crucial role in the screening, initial diagnosis, management, and risk stratification of HCM. Herein, we explore echocardiographic evaluation of HCM, emphasizing Doppler assessment for obstruction. Echocardiography informs management strategies through noninvasive hemodynamic assessment, which is frequently obtained with various provocative maneuvers. Recognition of obstructive HCM phenotypes and associated anatomical abnormalities guides therapeutic decision-making. Doppler echocardiography allows monitoring of therapeutic responses, whether it be medical therapies (including cardiac myosin inhibitor therapy) or septal reduction therapies, including surgical myectomy and alcohol septal ablation. This article discusses the hemodynamics of obstruction and practical application of Doppler assessment in HCM. In addition, it provides a visual atlas of obstruction in HCM, including high-quality figures and complementary videos that illustrate the many facets of dynamic obstruction.

A Multi-Omics Atlas of Sex-Specific Differences in Obstructive Hypertrophic Cardiomyopathy.

Background: Hypertrophic cardiomyopathy (HCM) is a common genetic heart disease. Women with HCM tend to have a later onset but more severe disease course. However, the underlying pathobiological mechanisms for these differences remain unknown. Methods: Myectomy samples from 97 patients (53 males/44 females) with symptomatic obstructive HCM and 23 control cardiac tissues were included in this study. RNA-sequencing was performed on all samples. Mass spectrometry-based proteomics and phosphoproteomics was performed on a representative subset of samples. Results: The transcriptome, proteome, and phosphoproteome was similar between sexes and did not separate on PCA plotting. Overall, there were 482 differentially expressed genes (DEGs) between control females and control males while there were only 53 DEGs between HCM females and HCM males. There were 1963 DEGs between HCM females and control females compared to 1064 DEGs between HCM males and control males. Additionally, there was increased transcriptional downregulation of hypertrophy pathways in HCM females and in HCM males. HCM females had 119 differentially expressed proteins compared to control females while HCM males only had 27 compared to control males. Finally, the phosphoproteome showed females had 341 differentially phosphorylated proteins (DPPs) compared to controls while males only had 184. Interestingly, there was hypophosphorylation and inactivation of hypertrophy pathways in females but hyperphosphorylation and activation in males. Conclusion: There are subtle, but biologically relevant differences in the multi-omics profile of HCM. This study provides the most comprehensive atlas of sex-specific differences in the transcriptome, proteome, and phosphoproteome present at the time of surgical myectomy for obstructive HCM.

A Case Series of Minimally Invasive Robotic-Assisted Resection of Cardiac Papillary Fibroelastoma: The Mayo Clinic Experience.

Papillary fibroelastomas (PFEs) are small, slowly growing benign cardiac tumors with clinically significant risk of embolization. Surgical excision is the definitive treatment of symptomatic PFE and is conventionally performed through a median sternotomy. In this study, we report a series of 12 patients, who underwent robotic-assisted PFE removal at the Mayo Clinic. PFE involved the mitral valve, left atrium, and tricuspid valve. No major complications occurred after the procedure, and most patients were discharged 4 days after the surgery. On follow-up, 1 patient demonstrated pericarditis.

Anomalous Aortic Origin of a Coronary Artery.

BACKGROUND: Although anomalous aortic origin of a coronary artery (AAOCA) is associated with risk of sudden cardiac arrest (SCA), there is a spectrum of disease, with the appropriate management for many remaining unclear. Increasing data warrant review for an updated perspective on management. METHODS: A panel of congenital cardiac surgeons, cardiologists, and imaging practitioners reviewed the current literature related to AAOCA and its management. Survey of relevant publications from 2010 to the present in PubMed was performed. RESULTS: The prevalence of AAOCA is 0.4% to 0.8%. Anomalous left coronary artery is 3 to 8 times less common than anomalous right coronary, but carries a much higher risk of SCA. Nevertheless, anomalous right coronary is not completely benign; 10% demonstrate ischemia, and it remains an important cause of SCA. Decision-making regarding which patients should be recommended for surgical intervention includes determining anatomic features associated with ischemia, evidence of ischemia on provocative testing, and concerning cardiovascular symptoms. Ischemia testing continues to prove challenging with low sensitivity and specificity, but the utility of new modalities is an active area of research. Surgical interventions focus on creating an unobstructed path for blood flow and choosing the appropriate surgical technique given the anatomy to accomplish this. Nontrivial morbidity has been reported with surgery, including new-onset ischemia. CONCLUSIONS: A proportion of patients with AAOCA demonstrate features and ischemia that warrant surgical intervention. Continued work remains to improve the ability to detect inducible ischemia, to risk stratify these patients, and to provide guidance in terms of which patients warrant surgical intervention.

Valve-Sparing Aortic Root Replacement State-of-the-Art Review, Part I: Anatomy and Physiology.

Aortic valve disease is common, and valve-preserving operations are preferred whenever possible. Valve-sparing aortic root replacement has become an important tool for managing aortic root pathology in children and adults. The learning curve for this operation is challenging, but with increasing experience and technical modifications, early and late outcomes continue to improve. Durable long-term results vary based on the underlying anatomy, pathology, and patient selection, as well as surgeon expertise. The first installment of this Valve-Sparing Aortic Root Replacement State-of-the-Art Review article addresses patient anatomy and physiology as it relates to candidacy for VSRR.

Utility of routine follow-up defibrillation safety margin testing in young patients with epicardial implantable cardioverter-defibrillators.

BACKGROUND: Routine defibrillation threshold testing (DFT) of transvenous implantable defibrillators (ICDs) has largely been in decline. In patients with non-transvenous ICDs that utilize subcutaneous and pleural ICD leads, serial DFT testing can detect a significant number of failures. Data about the utility of follow-up defibrillation safety margin testing (DSM) testing in pediatric patients and young adults with an epicardial ICD are lacking. METHODS: Patients aged < 25 years old who underwent epicardial ICD placement at Mayo Clinic from 2014 to 2023 with at least one follow-up DSM test were included. The patients were divided into a "routine" (R) and "clinically indicated" (CI) group based on the index of clinical concern. Inadequate DSM was defined as unsuccessful defibrillation at an output of less than 10 J below the maximum output of the device. The purpose of this study was to assess the utility of follow-up DSM testing. RESULTS: An epicardial ICD system was placed in 122 patients. A total of 26 patients met inclusion criteria and underwent a total of 47 DSM follow up tests. Inadequate DSM occurred in 1/33 (3%) in the R group and 2/14 (14%) DSM tests in the CI group. The median follow-up period was 54 and 36 months for the R and CI group, respectively. CONCLUSIONS: Our data suggest that epicardial ICDs are reliable and routine follow-up DSM testing may not be necessary for all patients. DSM testing should be performed in individuals with epicardial ICD systems when there is clinical concern about lead or coil performance.

Outcomes of aortic surgery in patients with Takayasu arteritis.

OBJECTIVE: To investigate the presentation, aortic involvement, and surgical outcomes in patients with Takayasu arteritis undergoing aortic surgery. METHODS: We queried our surgical database for patients with Takayasu arteritis who underwent aortic surgery from 1994 to 2022. RESULTS: There were a total of 31 patients with Takayasu arteritis who underwent aortic surgery. Patients' median age at the time of diagnosis was 35.0 years (interquartile range, 25.0-42.0). The majority were female (n = 27, 87.0%). Most patients (n = 28, 90.3%) were diagnosed before surgery, and 3 patients (9.6%) were diagnosed perioperatively. The median time interval from diagnosis to surgery was 2.8 years (interquartile range, 0.5-13.9). The most common presentation was ascending aorta aneurysm (n = 22, 70.9%), and severe aortic regurgitation was the most common valve insufficiency (n = 17, 54.8%). The most common operation was ascending aorta replacement (n = 20, 64.5%), and aortic valve replacement was the most common valve intervention (n = 17, 54.8%). Active vasculitis was identified in 2 (11.7%) aortic valve specimens. Early mortality was 6.5% (n = 2). A total of 6 deaths occurred over a median follow-up of 13.1 years (interquartile range, 6.1-25.2). Survival at 10 years was 86.7% (95% CI, 75.4-99.7). A total of 5 patients (16.1%) required a subsequent operation in a median of 1.9 years (interquartile range, 0.2-7.4). Freedom from reoperation was 96.9% (95% CI, 90.1-100) at 1 year, 89.4% (95% CI, 78.7-100.0) at 5 years, and 77.5% (95% CI, 61.2-98.1) at 10 and 15 years. CONCLUSIONS: Ascending aorta aneurysm and aortic valve regurgitation are the most frequent presentations in patients with Takayasu arteritis requiring aortic surgery. Surgery in these individuals is safe, with acceptable short- and long-term results.

Valve-Sparing Aortic Root Replacement State of the Art Review Part II: Surgical Techniques.

Aortic valve disease is common and valve-preserving operations are preferred whenever possible. Valve-sparing aortic root replacement (VSRR) has become an important tool for managing aortic root pathology in children and adults. The learning curve for this operation is challenging but with increasing experience and technical modifications, early and late outcomes continue to improve. Durable long term results vary based on underlying anatomy, pathology, and patient selection, and surgeon expertise. The purpose of Part II of this VSRR State of the Art Review article is to provide technical pearls related to VSRR.

Analysis of Anemia, Transfusions, and CABG Outcomes in The Society of Thoracic Surgeons National Database.

BACKGROUND: This study characterized the association of preoperative anemia and intraoperative red blood cell (RBC) transfusion on outcomes of elective coronary artery bypass grafting (CABG). METHODS: Data from 53,856 patients who underwent CABG included in The Society of Thoracic Surgeons (STS) Adult Cardiac Database in 2019 were used. The primary outcome was operative mortality. Secondary outcomes were postoperative complications. The association of anemia with outcomes was analyzed with multivariable regression models. The influence of intraoperative RBC transfusion on the effect of preoperative anemia on outcomes was studied using mediation analysis. RESULTS: Anemia was present in 25% of patients. Anemic patients had a higher STS Predicted Risk of Operative Mortality (1.2% vs 0.7%; P < .001). Anemia was associated with operative mortality (odds ratio [OR], 1.27; 99.5% CI, 1.00-1.61; P = .047), postoperative RBC transfusion (OR, 2.28; 99.5% CI, 2.12-2.44; P < .001), dialysis (OR, 1.58; 99.5% CI, 1.19-2.11; P < .001), and prolonged intensive care unit and hospital length of stay. Intraoperative RBC transfusion largely mediated the effects of anemia on mortality (76%), intensive care unit stay (99%), and hospital stay, but it only partially mediated the association with dialysis (34.9%). CONCLUSIONS: Preoperative anemia is common in patients who undergo CABG and is associated with increased postoperative risks of mortality, complications, and RBC transfusion. However, most of the effect of anemia on mortality is mediated through intraoperative RBC transfusion.

Safety and Efficacy of Surgical and Percutaneous Cardiac Interventions for Adults With Down Syndrome.

Objective: To assess risks and benefits of cardiac intervention in adults with Down syndrome (DS). Patients and Methods: A retrospective review was conducted using data from a study we published in 2010. Patients aged 18 years or older with DS who underwent cardiac operation or percutaneous intervention from February 2009 through April 2022 (new cohort) were compared with patients in the previous study (January 1969 through November 2007; remote cohort) at Mayo Clinic. Results: In total, 81 adults (43 men; 38 women) with DS underwent 89 cardiac interventions (84 surgical; 5 percutaneous) at a mean age of 33 years. Twenty-six patients presented with complete atrioventricular canal defect (17%) or tetralogy of Fallot (15%). The most common adult procedures were valve interventions: mitral (31%), tricuspid (15%), and pulmonary (12%). Of pulmonary valve interventions in the new cohort, 33% were performed percutaneously. The postoperative mortality rate was low (1% total). The mean time between last operation and death was 16 years. Conclusion: Adults with DS can undergo cardiac operation and percutaneous intervention with low morbidity and mortality risk and good long-term survival.

Hemodynamics in Adults with Systemic Right Ventricles: Differences Between Congenitally Corrected and Complete Transposition of the Great Arteries.

Despite their anatomical differences, congenitally corrected (ccTGA) and complete transposition of the great arteries (d-TGA) post-atrial switch are frequently studied together and managed similarly from a medical standpoint due to the shared systemic right ventricle (sRV). The aim was to assess differences in their underlying hemodynamics. The study is a retrospective review of 138 adults with ccTGA or d-TGA post-atrial switch undergoing cardiac catheterization at Mayo Clinic, MN between 2000 and 2021. ccTGA was categorized into isolated or complex ccTGA depending on concomitant ventricular septal defect and/or left ventricular outflow obstruction. There were 53 patients with d-TGA (91% post-Mustard procedure), 51 with complex and 34 with isolated ccTGA. Isolated ccTGA patients were older (51.8 ± 13.1 years) than those with d-TGA (37.5 ± 8.3 years) or complex ccTGA (40.8 ± 13.4 years). There were no differences in sRV or left ventricular size and function across groups. The ccTGA group more commonly had ≥ moderate tricuspid regurgitation than those with d-TGA; ≥ moderate mitral and ≥ moderate pulmonary regurgitation were most prevalent in complex ccTGA. There were no differences in sRV end-diastolic pressure (sRVEDP) or PAWP between groups. However, the ratio of PAWP:sRVEDP was higher in those with d-TGA compared to those with ccTGA. Cardiac index was higher in the d-TGA group than both groups of ccTGA patients with the latter showing higher indices of ventricular afterload. In conclusion, despite sharing a sRV, adults with d-TGA and ccTGA have substantial differences in hemodynamics and structural/valvular abnormalities. Further investigation regarding disease-specific responses to heart failure therapy in those with d-TGA and ccTGA is warranted.

Outcomes of Surgical Repair of Aberrant Subclavian Arteries in Adults.

BACKGROUND: Symptoms, imaging characteristics, and early and midterm surgical outcomes for aberrant subclavian arteries (ASCA) are not well defined in the adult population. METHODS: A single-institution retrospective review was conducted of adults undergoing surgical repair of ASCA and descending aorta origin/Kommerell diverticulum (KD) from January 1, 2002, to December 31, 2021. Symptom improvement and differences in imaging characteristics between anatomic groups and the number of symptoms were assessed. RESULTS: Mean age was 46 ± 17 years. There were 23 of 37 left aortic arches with right ASCA (62%) and 14 of 37 right aortic arches with left ASCA (38%). Of these, 31 of 37 (84%) were symptomatic, and 19 of 37 (51%) had KD size/growth meeting criteria for surgical repair. KD aortic origin diameter was larger in more symptomatic patients: 20.60 mm (interquartile range [IQR], 16.42-30.68 mm) in patients with ≥3 symptoms vs 22.05 mm (IQR, 17.52-24.21 mm) for 2 symptoms vs 13.72 mm (IQR, 12.70-15.95 mm) for 1 symptom (P = .018). Aortic replacement was required in 22 of 37 (59%). There were no early deaths. Complications occurred in 11 of 37 (30%): vocal cord dysfunction (4 of 37 [11%]), chylothorax (3 of 37 [8%]), Horner syndrome (2 of 37 [5%]), spinal deficit (2 of 37 [5%]), stroke (1 of 37 [3%]), and temporary dialysis requirement (1 of 37 [3%]). Over a median follow-up of 2.3 years (IQR, 0.8-3.9 years), there was 1 endovascular reintervention and no reoperations. Dysphagia and shortness of breath resolved in 92% and 89%, respectively, whereas gastroesophageal reflux persisted in 47%. CONCLUSIONS: The KD aortic origin diameter correlates with the number of symptoms, and surgical repair of ASCA and descending aorta origin/KD effectively relieves symptoms, with low rates of reintervention. Given the operative complexity, surgical repair should be performed in patients meeting size criteria or with significant dysphagia or shortness of breath symptoms.

Surgical Considerations for the Mitral Valve in Congenitally Corrected Transposition.

BACKGROUND: Systemic atrioventricular valve (morphologic tricuspid valve [TV]) regurgitation has been implicated in the development of systemic ventricular failure in congenitally corrected transposition of the great arteries (ccTGA), leading to timely referral for valve replacement. However, the surgical management of subpulmonary atrioventricular valve (morphologic mitral valve [MV]) regurgitation and outcomes has not been well studied. METHODS: Of 108 ccTGA patients undergoing atrioventricular valve surgery from 1979 to 2022, 22 patients (20%) underwent MV surgery. Demographics, etiology of valve regurgitation, operative details, and outcomes of these 22 patients were retrospectively reviewed. Follow-up at 1, 5, and 10 years was available for 18 (82%), 13 (59%), and 11 patients (50%), respectively. RESULTS: Median age was 37 years (interquartile range, 29-57 years). Intrinsic MV pathology was present in 18 individuals (82%). Operations included repair in 16 patients (73%), replacement in 6 (27%), and concomitant replacement of TV in 16 (73%). There was 1 perioperative death (5%) in a patient undergoing an emergent operation for severe acute-on-chronic heart failure due to worsening TV regurgitation. During a median follow-up of 12 years (interquartile range, 2-19 years), 7 patients (32%) died. Among the 16 patients who underwent repair, recurrent moderate or greater regurgitation was seen in 15%, 29%, and 43% of repairs in patients with annular, leaflet, and lead-induced regurgitation, respectively. CONCLUSIONS: Concomitant TV and MV disease occur much less frequently than isolated TV disease in ccTGA. Intrinsic MV disease is most commonly observed but appears less amenable to successful repair compared with mitral repair in the systemic position and suggests MV replacement may be preferred in ccTGA patients.