Bicuspid Aortic Valve Disease With Early Onset Complications: Characteristics And Aortic Outcomes.

Bicuspid aortic valve (BAV) is the most common congenital heart malformation in adults but can also cause childhood-onset complications. In multicenter study, we found that adults who experience significant complications of BAV disease before age 30 are distinguished from the majority of BAV cases that manifest after age 50 by a relatively severe clinical course, with higher rates of surgical interventions, more frequent second interventions, and a greater burden of congenital heart malformations. These observations highlight the need for prompt recognition, regular lifelong surveillance, and targeted interventions to address the significant health burdens of patients with early onset BAV complications.

Whole Exome Sequencing Uncovers the Genetic Complexity of Bicuspid Aortic Valve in Families with Early Onset Complications.

Bicuspid Aortic Valve (BAV) is the most common adult congenital heart lesion with an estimated population prevalence of 1%. We hypothesize that early onset complications of BAV (EBAV) are driven by specific impactful genetic variants. We analyzed whole exome sequences (WES) to identify rare coding variants that contribute to BAV disease in 215 EBAV families. Predicted pathogenic variants of causal genes were present in 111 EBAV families (51% of total), including genes that cause BAV (8%) or heritable thoracic aortic disease (HTAD, 17%). After appropriate filtration, we also identified 93 variants in 26 novel genes that are associated with autosomal dominant congenital heart phenotypes, including recurrent deleterious variation of FBN2, MYH6, channelopathy genes, and type 1 and 5 collagen genes. These findings confirm our hypothesis that unique rare genetic variants contribute to early onset complications of BAV disease.

The Society of Thoracic Surgeons 2023 Clinical Practice Guidelines for the Surgical Treatment of Atrial Fibrillation.

The Society of Thoracic Surgeons 2023 clinical practice guidelines for the surgical management of atrial fibrillation incorporate the most recent evidence for surgical ablation and left atrial appendage occlusion in different clinical scenarios. Substantial new evidence regarding the risks and benefits of surgical left atrial appendage occlusion and the long-term benefits of surgical ablation has been produced in the last five years. In comparison to the 2017 clinical practice guideline, the current update has an emphasis on surgical ablation in first-time, non-emergent cardiac surgery and its long-term benefits, an extension of the recommendation to perform surgical ablation in all patients with atrial fibrillation undergoing first-time, non-emergent cardiac surgery and a new class I recommendation for left atrial appendage occlusion in all patients with atrial fibrillation undergoing first-time, non-emergent cardiac surgery. Further guidance is provided for patients with structural heart disease and atrial fibrillation being considered for transcatheter valve repair or replacement, as well as patients in need of isolated left atrial appendage management who are not candidates for surgical ablation. The importance of a multidisciplinary team assessment, treatment planning, and long-term follow-up are reiterated in this clinical practice guideline with a class I recommendation, along with the other recommendations from the 2017 guidelines which remained unchanged in their class of recommendation and level of evidence.

Rare Genomic Copy Number Variants Implicate New Candidate Genes for Bicuspid Aortic Valve.

Bicuspid aortic valve (BAV), the most common congenital heart defect, is a major cause of aortic valve disease requiring valve interventions and thoracic aortic aneurysms predisposing to acute aortic dissections. The spectrum of BAV ranges from early onset valve and aortic complications (EBAV) to sporadic late onset disease. Rare genomic copy number variants (CNVs) have previously been implicated in the development of BAV and thoracic aortic aneurysms. We determined the frequency and gene content of rare CNVs in EBAV probands (n = 272) using genome-wide SNP microarray analysis and three complementary CNV detection algorithms (cnvPartition, PennCNV, and QuantiSNP). Unselected control genotypes from the Database of Genotypes and Phenotypes were analyzed using identical methods. We filtered the data to select large genic CNVs that were detected by multiple algorithms. Findings were replicated in cohorts with late onset sporadic disease (n = 5040). We identified 34 large and rare (< 1:1000 in controls) CNVs in EBAV probands. The burden of CNVs intersecting with genes known to cause BAV when mutated was increased in case-control analysis. CNVs intersecting with GATA4 and DSCAM were enriched in cases, recurrent in other datasets, and segregated with disease in families. In total, we identified potentially pathogenic CNVs in 8% of EBAV cases, implicating alterations of candidate genes at these loci in the pathogenesis of BAV.

Surgical and Transcatheter Mitral Valve Therapy: Medicare Utilization and Reimbursement.

BACKGROUND: We aimed to examine trends in the utilization and reimbursement of surgical and transcatheter mitral valve therapies and their changing relationship. METHODS: A query of administrative data on US Medicare beneficiaries undergoing mitral valve therapy was conducted from 2015 to 2020 using the Centers for Medicare and Medicaid Services Part B National Summary Data File. Inflation adjustment was to the 2020 Consumer Price Index. Trend analysis was quantified with growth rate and simple linear regression calculations. RESULTS: The annual number of all mitral valve procedures remained constant. Transcatheter mitral valve therapies increased by 313% with an increase of 1552 cases per year (P < .001), whereas surgical mitral valve therapies decreased by 31.4% with a decline of 1446 procedures per year (P = .004). As a proportion of all mitral valve therapies, surgical therapies decreased from 91.8% to 65.0%. Annual Medicare reimbursements for transcatheter and surgical mitral valve therapies mirrored the annual procedural trends. For transcatheter mitral valve therapies, per-case reimbursement decreased by 14.1% ($1283.18 to $1102.88), and for surgical mitral valve therapies, per-case reimbursement decreased by 3.8% ($1480.65 to $1424.57). CONCLUSIONS: Medicare utilization of mitral valve therapies has been stable in recent years, with growth of transcatheter volumes offset by a decrease in surgical volumes. This suggests that transcatheter therapy availability has not expanded the pool of patients with access to therapy. Nonetheless, case reimbursements decreased for both modalities.

Two Decades of Declining Medicare Reimbursement in Cardiac Surgery.

BACKGROUND: Given the uncertainty of US health care finances, an understanding of reimbursement trends has become increasingly important in the field of cardiac surgery. We aimed to assess Medicare reimbursement trends for common cardiac surgical procedures from 2000 to 2022. METHODS: Reimbursement data were extracted from the Centers for Medicare and Medicaid Services Physician Fee Schedule Look-Up Tool during the study period for 6 common cardiac operations: aortic valve replacement, mitral valve repair and replacement, tricuspid valve replacement, Bentall procedure, and coronary artery bypass grafting. Reimbursement rates were adjusted for inflation to 2022 US dollars using the Consumer Price Index. Total percentage change and compound annual growth rate were calculated. A split-time analysis was performed to assess trends before and after 2015. Least squares and linear regressions were performed. The R2 value was calculated for each procedure, and slope was used to determine change in reimbursements over time. RESULTS: Inflation-adjusted reimbursement decreased by 34.1% during the study period. The overall compound annual growth rate was -1.8%. Reimbursement trends differed by procedure (P < .001), with all reimbursements trending down (R2 > 0.62), except for mitral valve replacement (P = .21) and tricuspid valve replacement (P = .43). Coronary artery bypass grafting decreased the most (-44.4%), followed by aortic valve replacement (-40.1%), mitral valve repair (-38.5%), mitral valve replacement (-29.8%), Bentall procedure (-28.5%), and tricuspid valve replacement (-25.3%). In split-time analysis, reimbursement rates did not significantly change from 2000 to 2015 (P = .24) but decreased significantly from 2016 to 2022 (P = .001). CONCLUSIONS: Medicare reimbursement significantly decreased for most cardiac surgical procedures. These trends justify further advocacy by The Society of Thoracic Surgeons to maintain access to quality cardiac surgical care.

2022 ACC/AHA guideline for the diagnosis and management of aortic disease: A report of the American Heart Association/American College of Cardiology Joint Committee on Clinical Practice Guidelines.

AIM: The "2022 ACC/AHA Guideline for the Diagnosis and Management of Aortic Disease" provides recommendations to guide clinicians in the diagnosis, genetic evaluation and family screening, medical therapy, endovascular and surgical treatment, and long-term surveillance of patients with aortic disease across its multiple clinical presentation subsets (ie, asymptomatic, stable symptomatic, and acute aortic syndromes). METHODS: A comprehensive literature search was conducted from January 2021 to April 2021, encompassing studies, reviews, and other evidence conducted on human subjects that were published in English from PubMed, EMBASE, the Cochrane Library, CINHL Complete, and other selected databases relevant to this guideline. Additional relevant studies, published through June 2022 during the guideline writing process, were also considered by the writing committee, where appropriate. STRUCTURE: Recommendations from previously published AHA/ACC guidelines on thoracic aortic disease, peripheral artery disease, and bicuspid aortic valve disease have been updated with new evidence to guide clinicians. In addition, new recommendations addressing comprehensive care for patients with aortic disease have been developed. There is added emphasis on the role of shared decision making, especially in the management of patients with aortic disease both before and during pregnancy. The is also an increased emphasis on the importance of institutional interventional volume and multidisciplinary aortic team expertise in the care of patients with aortic disease.

2022 ACC/AHA Guideline for the Diagnosis and Management of Aortic Disease: A Report of the American Heart Association/American College of Cardiology Joint Committee on Clinical Practice Guidelines.

AIM: The "2022 ACC/AHA Guideline for the Diagnosis and Management of Aortic Disease" provides recommendations to guide clinicians in the diagnosis, genetic evaluation and family screening, medical therapy, endovascular and surgical treatment, and long-term surveillance of patients with aortic disease across its multiple clinical presentation subsets (ie, asymptomatic, stable symptomatic, and acute aortic syndromes). METHODS: A comprehensive literature search was conducted from January 2021 to April 2021, encompassing studies, reviews, and other evidence conducted on human subjects that were published in English from PubMed, EMBASE, the Cochrane Library, CINHL Complete, and other selected databases relevant to this guideline. Additional relevant studies, published through June 2022 during the guideline writing process, were also considered by the writing committee, where appropriate. Structure: Recommendations from previously published AHA/ACC guidelines on thoracic aortic disease, peripheral artery disease, and bicuspid aortic valve disease have been updated with new evidence to guide clinicians. In addition, new recommendations addressing comprehensive care for patients with aortic disease have been developed. There is added emphasis on the role of shared decision making, especially in the management of patients with aortic disease both before and during pregnancy. The is also an increased emphasis on the importance of institutional interventional volume and multidisciplinary aortic team expertise in the care of patients with aortic disease.

2022 ACC/AHA Guideline for the Diagnosis and Management of Aortic Disease: A Report of the American Heart Association/American College of Cardiology Joint Committee on Clinical Practice Guidelines.

AIM: The "2022 ACC/AHA Guideline for the Diagnosis and Management of Aortic Disease" provides recommendations to guide clinicians in the diagnosis, genetic evaluation and family screening, medical therapy, endovascular and surgical treatment, and long-term surveillance of patients with aortic disease across its multiple clinical presentation subsets (ie, asymptomatic, stable symptomatic, and acute aortic syndromes). METHODS: A comprehensive literature search was conducted from January 2021 to April 2021, encompassing studies, reviews, and other evidence conducted on human subjects that were published in English from PubMed, EMBASE, the Cochrane Library, CINHL Complete, and other selected databases relevant to this guideline. Additional relevant studies, published through June 2022 during the guideline writing process, were also considered by the writing committee, where appropriate. STRUCTURE: Recommendations from previously published AHA/ACC guidelines on thoracic aortic disease, peripheral artery disease, and bicuspid aortic valve disease have been updated with new evidence to guide clinicians. In addition, new recommendations addressing comprehensive care for patients with aortic disease have been developed. There is added emphasis on the role of shared decision making, especially in the management of patients with aortic disease both before and during pregnancy. The is also an increased emphasis on the importance of institutional interventional volume and multidisciplinary aortic team expertise in the care of patients with aortic disease.