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The New England Journal of Medicine Apr 2024
Topics: Humans; Aorta, Thoracic; Aortic Coarctation; Male; Adult; Hypertension; Computed Tomography Angiography
PubMed: 38631005
DOI: 10.1056/NEJMicm2313075 -
Annals of Vascular Surgery Aug 2023Despite significant technological advancements in endovascular aortic repair, the aortic arch remains a challenge due to anatomic complexity including arch angulation...
BACKGROUND
Despite significant technological advancements in endovascular aortic repair, the aortic arch remains a challenge due to anatomic complexity including arch angulation and morphology as well as the location of brachiocephalic vessels in relation to landing zones. Total endovascular solutions are in development and being studied, in the meantime, hybrid thoracic endovascular aortic repair (TEVAR) is a viable alternative to traditional open total arch replacement.
METHODS
A retrospective case series was performed reviewing outcomes of 71 patients who underwent hybrid TEVAR in the aortic arch over the past 12 years at our facility.
RESULTS
Major morbidity in the first 30 days post procedure was experienced by 3 patients (15.7%) and included one patient with stroke (n = 1), and another who experienced paraplegia and pulmonary complications (n = 1). There was one in-hospital death as a result of respiratory failure in a patient with multiple underlying comorbidities including chronic obstructive pulmonary disease on postoperative day 5 resulting in a 30-day mortality of 5.2%. After a mean follow-up of 30 months, the overall mortality remained 5.2%. Major morbidity occurred in one patient who developed retrograde type A dissection. There was no stenosis of bypass grafts identified on follow-up imaging. Endoleak was identified in 2 patients. One patient experienced persistent retrograde perfusion of the false lumen with interval aneurysmal degeneration which was managed with an Amplatz vascular plug at 120 months following the initial hybrid TEVAR (5.2%).
CONCLUSIONS
Single-stage hybrid TEVAR in the aortic arch is technically feasible with a good mid-term survival and a long-term freedom from reintervention.
Topics: Humans; Aortic Aneurysm, Thoracic; Blood Vessel Prosthesis Implantation; Retrospective Studies; Stents; Hospital Mortality; Aortic Dissection; Endovascular Procedures; Treatment Outcome; Risk Factors; Aortic Diseases; Aorta, Thoracic
PubMed: 36377067
DOI: 10.1016/j.avsg.2022.10.002 -
Advances in Experimental Medicine and... 2024Lesions of the semilunar valve and the aortic arch can occur either in isolation or as part of well-described clinical syndromes. The polygenic cause of calcific aortic... (Review)
Review
Lesions of the semilunar valve and the aortic arch can occur either in isolation or as part of well-described clinical syndromes. The polygenic cause of calcific aortic valve disease will be discussed including the key role of NOTCH1 mutations. In addition, the complex trait of bicuspid aortic valve disease will be outlined, both in sporadic/familial cases and in the context of associated syndromes, such as Alagille, Williams, and Kabuki syndromes. Aortic arch abnormalities particularly coarctation of the aorta and interrupted aortic arch, including their association with syndromes such as Turner and 22q11 deletion, respectively, are also discussed. Finally, the genetic basis of congenital pulmonary valve stenosis is summarized, with particular note to Ras-/mitogen-activated protein kinase (Ras/MAPK) pathway syndromes and other less common associations, such as Holt-Oram syndrome.
Topics: Humans; Aorta, Thoracic; Aortic Valve; Abnormalities, Multiple; Heart Defects, Congenital; Bicuspid Aortic Valve Disease; Pulmonary Valve Stenosis; Mutation; Receptor, Notch1; Aortic Valve Disease; Heart Valve Diseases; Calcinosis; Hematologic Diseases; Vestibular Diseases
PubMed: 38884747
DOI: 10.1007/978-3-031-44087-8_45 -
Journal of the American College of... Jul 2023As the bottleneck of endovascular aortic arch repair, early postoperative stroke remains a devastating complication in high-risk patients and a critical concern for the... (Review)
Review
As the bottleneck of endovascular aortic arch repair, early postoperative stroke remains a devastating complication in high-risk patients and a critical concern for the development of optimal endovascular techniques and devices. The incidence of early postoperative stroke varies widely among currently available endovascular techniques and devices, with reported rates ranging from 0.0% to 42.9%, and is significantly influenced by the severity of the patient's preexisting aortic atherosclerotic burden, air released from the endovascular device, and a variety of factors leading to cerebral perfusion insufficiency. Currently, preidentification of high-risk patients and careful perioperative management appear to play a critical role in reducing stroke incidence. Specific intraoperative prevention methods are still lacking, but embolic protection devices and carbon dioxide or high-volume saline flushing of endovascular devices appear promising. Detailed preoperative stroke risk stratification and screening for optimal endovascular techniques and devices for aortic arch treatment are unmet clinical needs.
Topics: Humans; Aorta, Thoracic; Endovascular Aneurysm Repair; Endovascular Procedures; Stroke; Aorta
PubMed: 37438011
DOI: 10.1016/j.jacc.2023.04.053 -
International Journal of Molecular... Jul 2023Thoracic aortic aneurysm and dissection (TAAD) are complex disease states with high morbidity and mortality that pose significant challenges to early diagnosis. Patients... (Review)
Review
Thoracic aortic aneurysm and dissection (TAAD) are complex disease states with high morbidity and mortality that pose significant challenges to early diagnosis. Patients with an aneurysm are asymptomatic and typically present to the emergency department only after the development of a dissection. The extracellular matrix (ECM) plays a crucial role in regulating the aortic structure and function. The histopathologic hallmark termed medial degeneration is characterised by smooth muscle cell (SMC) loss, the degradation of elastic and collagen fibres and proteoglycan (PG) accumulation. Covalently attached to the protein core of PGs are a number of glycosaminoglycan chains, negatively charged molecules that provide flexibility, compressibility, and viscoelasticity to the aorta. PG pooling in the media can produce discontinuities in the aortic wall leading to increased local stress. The accumulation of PGs is likely due to an imbalance between their synthesis by SMCs and decreased proteolysis by A Disintegrin-like and Metalloproteinase with Thrombospondin motifs (ADAMTS) proteoglycanases in the ECM. Mouse models of TAAD indicated that these proteases exert a crucial, albeit complex and not fully elucidated, role in this disease. This has led to a mounting interest in utilising ADAMTS proteoglycanases as biomarkers of TAAD. In this review, we discuss the role of ADAMTSs in thoracic aortic disease and their potential use in facilitating the clinical diagnosis of TAAD and disease progression.
Topics: Mice; Animals; Aortic Aneurysm, Thoracic; Aorta; Aortic Dissection; Proteoglycans; Aorta, Thoracic
PubMed: 37569511
DOI: 10.3390/ijms241512135 -
The Journal of Thoracic and... Oct 2023After limited root/ascending with or without hemiarch repair for acute type A aortic dissection (ATAAD), 20% to 30% of patients require distal reintervention, frequently...
OBJECTIVE
After limited root/ascending with or without hemiarch repair for acute type A aortic dissection (ATAAD), 20% to 30% of patients require distal reintervention, frequently for arch pathology. In this report, we describe an institutional algorithm for arch management after previous limited ATAAD repair and detail operative and long-term outcomes.
METHODS
From August 2005 to April 2021, 71 patients status post previous limited ATAAD repair underwent reoperative arch repair involving zones 1 to 3 for aneurysmal degeneration of residual arch dissection including complete cervical debranching with zone 0/1 thoracic endovascular aortic repair in 6 (8%), open total arch in 13 (18%), type I hybrid arch repair in 23 (32%), and type II/III hybrid arch repair in 29 (41%).
RESULTS
Mean age was 59 ± 12 years; time from index ATAAD repair to reoperation was 4 (interquartile range, 2-9) years. There were 2 (2.8%) in-hospital deaths and 2 (2.8%) postdischarge deaths within 30 days of surgery. Three patients suffered stroke (4.2%) and 2 (2.8%) had acute renal failure requiring dialysis. Overall Kaplan-Meier survival was 78%, 70%, and 58% at 1, 3, and 5 years, respectively. Institutional experience appeared to play a significant role in early and late outcomes, because there have been no operative mortalities in the past 9 years and improved survival of 87% versus 66%, 79% versus 58%, and 79% versus 40% at 1, 3, and 5 years in comparisons of the past 9 years with the previous era (P = .01).
CONCLUSIONS
Aneurysmal degeneration of residual arch dissection after limited ATAAD repair presents a complex reoperative challenge. An algorithmic operative approach tailored to patient anatomy and comorbidities yields excellent early and late outcomes, which continue to improve with increasing institutional experience.
Topics: Humans; Middle Aged; Aged; Aorta, Thoracic; Blood Vessel Prosthesis Implantation; Aftercare; Endovascular Procedures; Patient Discharge; Renal Dialysis
PubMed: 35282930
DOI: 10.1016/j.jtcvs.2022.02.016 -
BJS Open May 2024Extended aortic repair is considered a key issue for the long-term durability of surgery for DeBakey type 1 aortic dissection. The risk of aortic degeneration may be...
BACKGROUND
Extended aortic repair is considered a key issue for the long-term durability of surgery for DeBakey type 1 aortic dissection. The risk of aortic degeneration may be higher in young patients due to their long life expectancy. The early outcome and durability of aortic surgery in these patients were investigated in the present study.
METHODS
The subjects of the present analysis were patients under 60 years old who underwent surgical repair for acute DeBakey type 1 aortic dissection at 18 cardiac surgery centres across Europe between 2005 and 2021. Patients underwent ascending aortic repair or total aortic arch repair using the conventional technique or the frozen elephant trunk technique. The primary outcome was 5-year cumulative incidence of reoperation on the distal aorta.
RESULTS
Overall, 915 patients underwent surgical ascending aortic repair and 284 patients underwent surgical total aortic arch repair. The frozen elephant trunk procedure was performed in 128 patients. Among 245 propensity score-matched pairs, total aortic arch repair did not decrease the rate of distal aortic reoperation compared to ascending aortic repair (5-year cumulative incidence, 6.7% versus 6.7%, subdistributional hazard ratio 1.127, 95% c.i. 0.523 to 2.427). Total aortic arch repair increased the incidence of postoperative stroke/global brain ischaemia (25.7% versus 18.4%, P = 0.050) and dialysis (19.6% versus 12.7%, P = 0.003). Five-year mortality was comparable after ascending aortic repair and total aortic arch repair (22.8% versus 27.3%, P = 0.172).
CONCLUSIONS
In patients under 60 years old with DeBakey type 1 aortic dissection, total aortic arch replacement compared with ascending aortic repair did not reduce the incidence of distal aortic operations at 5 years. When feasible, ascending aortic repair for DeBakey type 1 aortic dissection is associated with satisfactory early and mid-term outcomes.
TRIAL REGISTRATION
ClinicalTrials.gov Identifier: NCT04831073.
Topics: Humans; Aortic Dissection; Male; Female; Middle Aged; Aorta, Thoracic; Reoperation; Postoperative Complications; Blood Vessel Prosthesis Implantation; Aortic Aneurysm, Thoracic; Adult; Retrospective Studies; Treatment Outcome; Europe; Propensity Score
PubMed: 38768283
DOI: 10.1093/bjsopen/zrae047 -
Annals of Vascular Surgery Sep 2023Aberrant subclavian artery (ASA) with or without Kommerell's diverticulum (KD) is a rare anatomic aortic arch anomaly that can cause dysphagia and/or life-threatening...
BACKGROUND
Aberrant subclavian artery (ASA) with or without Kommerell's diverticulum (KD) is a rare anatomic aortic arch anomaly that can cause dysphagia and/or life-threatening rupture. The objective of this study is to compare outcomes of ASA/KD repair in patients with a left versus right aortic arch.
METHODS
Using the Vascular Low Frequency Disease Consortium methodology, a retrospective review was performed of patients ≥18 years old with surgical treatment of ASA/KD from 2000 to 2020 at 20 institutions.
RESULTS
288 patients with ASA with or without KD were identified; 222 left-sided aortic arch (LAA), and 66 right-sided aortic arch (RAA). Mean age at repair was younger in LAA 54 vs. 58 years (P = 0.06). Patients in RAA were more likely to undergo repair due to symptoms (72.7% vs. 55.9%, P = 0.01), and more likely to present with dysphagia (57.6% vs. 39.1%, P < 0.01). The hybrid open/endovascular approach was the most common repair type in both groups. Rates of intraoperative complications, death within 30 days, return to the operating room, symptom relief and endoleaks were not significantly different. For patients with symptom status follow-up data, in LAA, 61.7% had complete relief, 34.0% had partial relief and 4.3% had no change. In RAA, 60.7% had complete relief, 34.4% had partial relief and 4.9% had no change.
CONCLUSIONS
In patients with ASA/KD, RAA patients were less common than LAA, presented more frequently with dysphagia, had symptoms as an indication for intervention, and underwent treatment at a younger age. Open, endovascular and hybrid repair approaches appear equally effective, regardless of arch laterality.
Topics: Adolescent; Humans; Aorta, Thoracic; Deglutition Disorders; Diverticulum; Heart Defects, Congenital; Subclavian Artery; Treatment Outcome; Vascular Diseases; Adult; Middle Aged
PubMed: 37236537
DOI: 10.1016/j.avsg.2023.05.005 -
Anatomical Science International Jun 2024Variations in the arch of the aorta and aortic valves among fetal, cadaveric, and post-mortem specimens present a spectrum of anatomical configurations, posing...
Variations in the arch of the aorta and aortic valves among fetal, cadaveric, and post-mortem specimens present a spectrum of anatomical configurations, posing challenges in establishing a standard norm. While some variations hold surgical significance, many bear little functional consequence but provide insights into embryological origins. The aortic arch exhibits diverse branching patterns, including common trunks and different orders, relevant for endovascular surgeries. Meanwhile, malformations in the aortic valve, affecting the aorta, may lead to ischemia and cerebral infarction, warranting understanding of coexisting arch and valve anomalies to predict complications like aortic dissection. Studies in the Indian population mirror global variations, underscoring the need to explore embryological, clinical, and surgical implications for safer vascular surgeries involving the aortic arch and valves. The study's objectives included examining branching patterns, diameters, and distances between arch branches and exploring aortic valve variations. Employing a cross-sectional design, the study was conducted across Anatomy, Forensic Medicine, and Obstetrics and Gynecology departments. A sample of 100, comprising cadavers, fetuses, and postmortem specimens, were gathered. Specimens ranged from 14 weeks of intrauterine life to 85 years, with intact thoracic cages as inclusion criteria. Methodology involved dissection, specimen fixation, and macroscopic examination for variations and morphological parameters. Results showed aortic diameter increase with age, with significant gender differences. A statistically significant association between arch variations and anomalous valves was observed, suggesting mutual predictability. Individuals with valve anomalies should undergo comprehensive cardiology evaluation to avert complications like aortic dissection during endovascular surgeries. While atheromatous plaques were prevalent in younger groups, their frequency rose with age, necessitating vigilant vascular monitoring. Careful handling during surgeries is paramount, given potential adverse outcomes resulting from variations. Overall, the study underscores the importance of comprehensive anatomical understanding in clinical contexts, guiding effective management strategies and ensuring patient safety in vascular surgeries.
Topics: Humans; Female; Male; Aortic Valve; Aorta, Thoracic; Aged; Adult; Anatomic Variation; Cadaver; Middle Aged; Aged, 80 and over; Adolescent; Young Adult; Child; Infant; Child, Preschool; Fetus; Infant, Newborn; Cross-Sectional Studies
PubMed: 38758496
DOI: 10.1007/s12565-024-00777-3 -
The Journal of Thoracic and... Nov 2023Patients with Loeys-Dietz syndrome demonstrate a heightened risk of distal thoracic aortic events after valve-sparing aortic root replacement. This study assesses the...
OBJECTIVES
Patients with Loeys-Dietz syndrome demonstrate a heightened risk of distal thoracic aortic events after valve-sparing aortic root replacement. This study assesses the clinical risks and hemodynamic consequences of a prophylactic aortic arch replacement strategy in Loeys-Dietz syndrome and characterizes smooth muscle cell phenotype in Loeys-Dietz syndrome aneurysmal and normal-sized downstream aorta.
METHODS
Patients with genetically confirmed Loeys-Dietz syndrome (n = 8) underwent prophylactic aortic arch replacement during valve-sparing aortic root replacement. Four-dimensional flow magnetic resonance imaging studies were performed in 4 patients with Loeys-Dietz syndrome (valve-sparing aortic root replacement + arch) and compared with patients with contemporary Marfan syndrome (valve-sparing aortic root replacement only, n = 5) and control patients (without aortopathy, n = 5). Aortic tissues from 4 patients with Loeys-Dietz syndrome and 2 organ donors were processed for anatomically segmented single-cell RNA sequencing and histologic assessment.
RESULTS
Patients with Loeys-Dietz syndrome valve-sparing aortic root replacement + arch had no deaths, major morbidity, or aortic events in a median of 2 years follow-up. Four-dimensional magnetic resonance imaging demonstrated altered flow parameters in patients with postoperative aortopathy relative to controls, but no clear deleterious changes due to arch replacement. Integrated analysis of aortic single-cell RNA sequencing data (>49,000 cells) identified a continuum of abnormal smooth muscle cell phenotypic modulation in Loeys-Dietz syndrome defined by reduced contractility and enriched extracellular matrix synthesis, adhesion receptors, and transforming growth factor-beta signaling. These modulated smooth muscle cells populated the Loeys-Dietz syndrome tunica media with gradually reduced density from the overtly aneurysmal root to the nondilated arch.
CONCLUSIONS
Patients with Loeys-Dietz syndrome demonstrated excellent surgical outcomes without overt downstream flow or shear stress disturbances after concomitant valve-sparing aortic root replacement + arch operations. Abnormal smooth muscle cell-mediated aortic remodeling occurs within the normal diameter, clinically at-risk Loeys-Dietz syndrome arch segment. These initial clinical and pathophysiologic findings support concomitant arch replacement in Loeys-Dietz syndrome.
Topics: Humans; Loeys-Dietz Syndrome; Aorta, Thoracic; Aorta; Marfan Syndrome; Vascular Surgical Procedures
PubMed: 37500053
DOI: 10.1016/j.jtcvs.2023.07.023