Redo partial aortic root remodelling in type A dissection with an aorto-left atrial fistula.

A 72-year-old male with a history of a triple-vessel coronary artery bypass graft years ago presented with a DeBakey type 2 aortic dissection and an aorto-left atrial fistula with patent bypass grafts (left internal mammary artery and saphenous vein grafts). He developed pulmonary oedema and required intubation. The right axillary artery was cannulated. After the ascending aorta and left internal mammary artery were clamped, the aorta was transected, leaving aortic tissue around two saphenous vein grafts as two separate patches. An entry tear was found adjacent to the proximal anastomosis of the saphenous vein graft to the posterior descending artery. A fistula, which was located between a false lumen in the non-coronary sinus and the dome of the left atrium, was primarily closed. Because the adventitia was thinned out in the non-coronary sinus due to aortic dissection, partial aortic root remodelling was performed with resuspension of the commissures. Hemiarch repair was performed under moderate hypothermia and unilateral antegrade cerebral perfusion. After systemic perfusion was resumed, the locations of the saphenous vein graft buttons were determined. The ascending graft was cross-clamped again; the saphenous vein graft to the obtuse marginal branch graft was reimplanted using the Carrel patch technique while a saphenous vein graft to the posterior descending artery required interposition of a 10-mm Dacron graft to accommodate the length.

Heritable thoracic aortic disease: a literature review on genetic aortopathies and current surgical management.

Heritable thoracic aortic disease puts patients at risk for aortic aneurysms, rupture, and dissections. The diagnosis and management of this heterogenous patient population continues to evolve. Last year, the American Heart Association/American College of Cardiology Joint Committee published diagnosis and management guidelines for aortic disease, which included those with genetic aortopathies. Additionally, evolving research studying the implications of underlying genetic aberrations with new genetic testing continues to become available. In this review, we evaluate the current literature surrounding the diagnosis and management of heritable thoracic aortic disease, as well as novel therapeutic approaches and future directions of research.

Mortality after non-surgically treated acute type A aortic dissection is higher than previously reported.

OBJECTIVES: It has been commonly accepted that untreated acute type A aortic dissection (ATAAD) results in an hourly mortality rate of 1-2% during the 1st 24 h after symptom onset. The data to support this statement rely solely on patients who have been denied surgical treatment after reaching surgical centres. The objective was to perform a total review of non-surgically treated (NST) ATAAD and provide contemporary mortality data. METHODS: This was a regional, retrospective, observational study. All patients receiving one of the following diagnoses: International Classification of Diseases (ICD)-9 4410, 4411, 4415, 4416 or ICD-10 I710, I711, I715, I718 in an area of 1.9 million inhabitants in Southern Sweden during a period of 23 years (January 1998 to November 2021) were retrospectively screened. The search was conducted using all available medical registries so that every patient diagnosed with ATAAD in our region was identified. The charts and imaging of each screened patient were subsequently reviewed to confirm or discard the diagnosis of ATAAD. RESULTS: Screening identified 2325 patients, of whom 184 NST ATAAD patients were included. The mortality of NST ATAAD was 47.3 ± 4.4%, 55.0 ± 4.4%, 76.7 ± 3.7% and 83.9 ± 4.3% at 24 h, 48 h, 14 days and 1 year, respectively. The hourly mortality rate during the 1st 24 h after symptom onset was 2.6%. CONCLUSIONS: This study observed higher mortality than has previously been reported. It emphasizes the need for timely diagnosis, swift management and emergent surgical treatment for patients suffering an acute type A aortic dissection.

Cannula Placement for Cerebral Protection Without Circulatory Arrest in Patients Undergoing Hemiarch Aortic Aneurysm Repair.

BACKGROUND: Aortic aneurysms involving the proximal aortic arch, which require hemiarch-type repair, typically require circulatory arrest with antegrade cerebral perfusion. Left carotid antegrade cerebral perfusion (LCP) via distal arch cannulation without circulatory arrest was used in this study's patient population. The goal was to assess the operative efficiency and clinical outcomes of using a distal arch cannulation technique that would not require any hypothermic circulatory arrest (HCA) time compared with more traditional brachiocephalic artery cannulation with right-sided unilateral antegrade cerebral perfusion (RCP) and HCA. METHODS: A single-center retrospective review of patients with replacement of the distal ascending aorta involving the proximal arch was performed. Patients with an intramural hematoma or dissection were excluded. Between January 2015 and December 2019, 68 adult patients had undergone a hemiarch repair because of aneurysmal disease. Analysis of baseline demographics, operative data, and clinical outcomes was performed. RESULTS: Comparing the 68 patients: 21 patients were treated with RCP (via brachiocephalic artery graft with HCA), and 47 patients were treated with LCP (via distal aortic arch cannulation with cross-clamp between the brachiocephalic and left common carotid arteries without HCA). Baseline characteristics and outcomes were evaluated for both groups. The LCP group was younger (LCP median [IQR] age, 60 [53-65] years vs RCP median [IQR] age, 67 [59-71] years]. Sex, race, body mass index, comorbidities, and ejection fraction were similar between the groups. Cardiopulmonary bypass time (LCP, 123 minutes vs RCP, 149 minutes) and unilateral cerebral perfusion time (LCP, 17 minutes vs RCP, 22 minutes) were longer in the RCP group. Bleeding, prolonged ventilatory support, kidney failure, and length of stay were similar. In-hospital mortality was 2% in the LCP group vs 0% in the RCP group. Stroke occurred in 2 patients (4.2%) in the LCP group and in 0% of the RCP group. Mortality at 6 months in the LCP and RCP groups was 3% and 10%, respectively. CONCLUSION: Distal arch cannulation with LCP without HCA is a reasonable and safe alternative strategy for patients requiring hemiarch replacement for aneurysmal disease. This technique may provide additional benefits by avoiding circulatory arrest in these complex cases.

Insights From the Histopathologic Analysis of Acquired and Genetic Thoracic Aortic Aneurysms and Dissections.

OBJECTIVE: The purpose of this study was to apply contemporary consensus criteria developed by the Society for Cardiovascular Pathology and the Association for European Cardiovascular Pathology to the evaluation of aortic pathology, with the expectation that the additional pathologic information may enhance the understanding and management of aortic diseases. METHODS: A scoring system was applied to ascending aortic specimens from 42 patients with heritable thoracic aortic disease and known genetic variations and from 86 patients from a single year, including patients with known genetic variations (n = 12) and patients with sporadic disease (n = 74). RESULTS: The various types of lesions of medial degeneration and the overall severity of medial degeneration overlapped considerably between those patients with heritable disease and those with sporadic disease; however, patients with heritable thoracic aortic disease had significantly more overall medial degeneration (P = .004) and higher levels of elastic fiber fragmentation (P = .03) and mucoid extracellular matrix accumulation (P = .04) than patients with sporadic thoracic aortic disease. Heritable thoracic aortic disease with known genetic variation was more prevalent in women than in men (27.2% vs 9.8%; P = .04), and women had more severe medial degeneration than men (P = .04). Medial degeneration scores were significantly lower for patients with bicuspid aortic valves than for patients with tricuspid aortic valves (P = .03). CONCLUSION: The study's findings indicate considerable overlap in the pattern, extent, and severity of medial degeneration between sporadic and hereditary types of thoracic aortic disease. This finding suggests that histopathologic medial degeneration represents the final common outcome of diverse pathogenetic factors and mechanisms.

Total arch replacement via single upper hemisternotomy approach for aortic aneurysm in syphilis: Case report.

RATIONALE: Syphilitic aortic aneurysm is a relatively rare type of cardiovascular syphilis. A small number of patients with syphilitic aortic aneurysms will be accompanied by aortic regurgitation and coronary stenosis. Apart from aortic rupture or dissection, syphilitic aortic aneurysm often causes associated vascular disorders, including left common carotid artery, innominate artery, and celiac artery stenosis or obstruction. PATIENT CONCERNS: In this case, we observed left common carotid artery occlusion based on both ultrasound and intraoperative exploration. For patients with syphilitic aortic aneurysm, the first choice is still sufficient antibiotic therapy. The surgical indications include symptom relief and prevention of aortic rupture or sudden death. DIAGNOSES: Aortic valve insufficiency, aortic aneurysm, and syphilis. INTERVENTIONS: Aortic valve replacement, aneurysmectomy and total arch replacement combined with frozen elephant trunk implantation via single upper hemisternotomy approach. OUTCOMES: The patient did not suffer reventilation and reoperation. No transient or permanent neurological dysfunction was observed in this patient. And no acute renal failure occurred. The patient was discharged on 43 days after the operation. LESSONS SUBSECTIONS: The upper hemisternotomy has the advantages of faster postoperative recovery, shorter ventilation time, shorter intensive care unit stay, less blood transfusion, and less incisional pain compared with the full sternotomy, which is one of the reasons why we chose this procedure for this patient.

Thoracic Aortic Aneurysmal Disease: Comprehensive Recommendations for the Primary Care Physician.

Thoracic aortic aneurysm (TAA) is a commonly encountered disease that is defined as aortic dilation with an increase in diameter of at least 50% greater than the expected age- and sex-adjusted size. Thoracic aortic aneurysms are described by their size, location, morphology, and cause. Primary care clinicians and other noncardiologists are often the first point of contact for patients with TAA. This review is intended to provide them with basic information on the differential diagnosis, diagnostic evaluation, and medical and surgical management of TAAs. Management decisions depend on having as precise a diagnosis as possible. Fortunately, this can often be achieved with a stepwise diagnostic approach that incorporates imaging and targeted genetic testing. Our review includes recommendations. In this review, we discuss these issues at a basic level and include recommendations for patients considering pregnancy.

MFAP5 variant-induced multiple giant thoracic aortic aneurysm.

Heritable thoracic aortic aneurysms are complex conditions characterised by the dilation or rupture of the thoracic aorta, often occurring as an autosomal-dominant disorder associated with life-threatening complications. In this case report, we present a de novo variant, MFAP5 c.236_237insA (p.N79Kfs9), which is implicated in the development of inherited thoracic aortic aneurysm. The proband, a 15-year-old male, presented with recurrent cough, dull chest pain, chest distress, vomiting, and reduced activity tolerance, leading to the diagnosis of heritable thoracic aortic aneurysms. Whole-exome sequencing identified a novel heterozygous variant in MFAP5 (NM_003480, c.236_237insA, and p.N79Kfs9). MutationTester and PolyPhen-s predicted this variant to be damaging and disease-causing (probability = 1), while the SFIT score indicated protein damage (0.001). Structural analysis using the AlphaFold Protein structure database revealed that this mutation disrupted the N-linked glycosylation site, resulting in a frameshift, amino acid sequence alteration, and truncation of an essential protein site. To our knowledge, this is the first case report describing a young patient with heritable thoracic aortic aneurysm carrying the novel MFAP5 c.236_237insA (p.N79Kfs*9) variant. This variant represents the third identified mutation site associated with heritable thoracic aortic aneurysm. Given the high mortality and morbidity rates associated with thoracic aortic aneurysms, the prevention of severe and fatal complications is crucial in the clinical management of this condition. Our case highlights the importance of whole-exome sequencing and genetic screening in identifying potential pathogenic or likely pathogenic variants, particularly in early-onset patients with aortic dilation, to inform appropriate management strategies.

The Use of the Ascyrus Medical Dissection Stent in Acute Type A Aortic Dissection Repair Reduces Distal Anastomotic New Entry Tear.

BACKGROUND: A distal anastomotic new entry tear (DANE) can occur at the time of surgical repair for acute type A aortic dissection (ATAAD). This study aimed to compare the occurrence of DANE following a standard hemiarch repair with that following a hemiarch repair with an uncovered arch dissection stent. METHODS: All patients who received a hemiarch repair or a hemiarch repair with an Ascyrus Medical Dissection Stent (AMDS) for ATAAD from 2017 to 2021 were included. Baseline and intra- and postoperative characteristics were collected. All available pre- and postoperative computed tomographic scans were analysed. The primary outcome measures were the incidence of DANE, positive aortic remodelling, mortality, and aortic reintervention rates at last follow-up. RESULTS: A total of 114 patients underwent repair of Debakey I ATAAD during the study period with either an isolated hemiarch (n = 77) or a hemiarch with AMDS (n = 37). There was no significant difference in mortality (P = 0.768) or other in-hospital adverse events. During the follow-up period, DANE occurred in 43.3% (n = 26) of the isolated hemiarch group and in 11.8% (n = 4) of the hemiarch with AMDS group (P = 0.002). The incidence of false lumen thrombosis and obliteration favoured the AMDS group in the aortic arch (P = 0.029), the proximal descending thoracic aorta (P = 0.031), and level of pulmonary artery bifurcation (P = 0.044). CONCLUSIONS: The incidence of DANE is significantly reduced with the addition of an AMDS at the time of hemiarch repair for ATAAD repair. Further follow-up is necessary to identify late aortic complications that may have been prevented by reducing the incidence of postoperative DANE.

One-Year Results of a Low-Profile Endograft in Acute, Complicated Type B Aortic Dissection.

BACKGROUND: The safety and effectiveness of the RelayPro endograft (Terumo Aortic) was assessed for the treatment of acute, complicated type B aortic dissection (TBAD). METHODS: A prospective pivotal trial analyzed a primary end point of all-cause mortality at 30 days. Secondary end points included technical success, major adverse events (disabling stroke, renal failure, and paraplegia/paralysis), endoleaks, patency, rupture, device integrity, false lumen perfusion, reinterventions, aortic expansion, and migration evaluated to 5 years. RESULTS: The study involved 22 United States centers and enrolled 56 patients (mean age, 59.5 ± 11.4 years) from 2017 to 2021; of whom, 73.2% were men and 53.6% were African American. TBAD was complicated by malperfusion of the kidneys (51.8%), lower extremities (35.7%), and viscera (33.9%), and rupture (10.7%). Dissection extended proximally to zones 1/2 (14.3%) and zone 3 (78.6%) and distally to the iliac arteries (67.3%). Most procedures were percutaneous (85.5%). Technical success was 100%. Median hospitalization was 7 days (interquartile range, 5-12 days). All-cause mortality at 30 days was 1.8% (1 of 56; upper 95% CI, 8.2%; P < .0001). Seven major adverse events occurred in 6 patients (10.7%), consisting of paraplegia (n = 3), paraparesis (n = 2), disabling stroke (n = 1), and renal failure (n = 1). All paraplegia/paraparesis resolved with lumbar drainage. Kaplan-Meier analysis estimated a freedom from major adverse events of 89.1% at each interval from 30 days to 3 years. There was 1 endoleak (Type Ia), 2 retrograde dissections, and aortic diameter growth occurred in 2. There has been no rupture, fistula, component separation, patency loss, stenosis, kinking, twisting, bird beak, loss of device integrity, or fracture. CONCLUSIONS: RelayPro is safe and effective in acute, complicated TBAD. Follow-up is ongoing to evaluate longer-term outcomes and durability.

Outcomes After Extent I Thoracoabdominal Aortic Repair: Focus on Heritable Aortic Disease.

BACKGROUND: Crawford extent I thoracoabdominal aortic aneurysm (TAAA) repairs are increasingly performed by an endovascular approach, including in patients with heritable thoracic aortic disease (HTAD). We evaluated outcomes after open extent I TAAA repair in patients with and without HTAD. METHODS: This retrospective study included 992 patients (median age, 67 years; quartile 1-quartile 3, 57-73 years) who underwent extent I TAAA (1990-2022), stratified by the presence of HTAD (n = 177 [17.8%]). Patients with HTAD had genetic aortopathies or presented at age ≤50 years, and 35% (62 of 177) had Marfan syndrome. Logistic regression was used to identify predictors of operative death and adverse event, a composite of operative death and persistent (present at discharge) stroke, paraplegia, paraparesis, and renal failure necessitating dialysis. Long-term outcomes were analyzed with competing risks analysis. RESULTS: Patients with HTAD had lower rates of operative mortality (1.7% vs 7.0%, P = .01) and composite adverse event (2.8% vs 12.3%, P < .001) than non-HTAD patients. Most HTAD patients were discharged home (92.6% vs 76.9%, P < .001). Predictors of operative death were increasing age, aortic dissection, tobacco use, chronic symptoms, and rupture. Predictors for adverse event were increasing age, acute symptoms, chronic dissection, and rupture. Patients with HTAD had substantially better repair-failure-free survival (P < .001). CONCLUSIONS: Open extent I TAAA repair was effective in patients with HTAD, with low operative mortality and adverse event rates, better late survival, and excellent long-term durability, making a compelling argument for preferring open repair in these patients.

Aortic dissection in a young male with persistent ductus arteriosus and a novel variant in MYLK.

Pathogenic variants in several genes involved in the function or regulation of smooth muscle cells (SMC) are known to predispose to congenital heart disease and thoracic aortic aneurysm and dissection (TAAD). Variants in MYLK are primarily known to predispose to TAAD, but a growing body of evidence points toward MYLK also playing an essential role in the regulation of SMC contraction outside the aorta. In this case report, we present a patient with co-occurrence of persistent ductus arteriosus (PDA) and thoracic aortic dissection. Genetic analyses revealed a novel splice acceptor variant (c.3986-1G > A) in MYLK, which segregated with disease in the family. RNA-analyses on fibroblasts showed that the variant induced skipping of exon 24, which resulted in an in-frame deletion of 101 amino acids. These findings suggest that MYLK-associated disease could include a broader phenotypic spectrum than isolated TAAD, including PDA and obstructive pulmonary disease. Genetic analyses could be considered in families with TAAD and PDA or obstructive pulmonary disease.

Mid-term efficacy of castor stent and in situ fenestration stent in the treatment of type B aortic dissection involving the left subclavian artery: A retrospective single-center study.

To evaluate the midterm efficacy of the Castor stent (CS) versus in situ fenestration (ISF) for reconstructing the left subclavian artery (LSA) in patients with type B aortic dissection (TBAD). Between July 2017 and July 2022, a total of 247 patients with TBAD were enrolled. One hundred thirty-seven patients were treated using CSs (group A), while the remaining 110 patients received ISFs (group B). Data of the two groups were retrospectively analyzed. The success rates of surgery were 99.3% and 95.5% in groups A and B (p = .053), There were no deaths during hospitalization. During surgery, group B showed a longer surgical duration [68.0 (66.0, 77.0) vs. 62.0 (59.0, 66.0) min, p < .001] and intraoperative fluoroscopy time [18.0 (16.0, 20.0) vs. 16.0 (14.0, 18.0) min, p < .001] than group A. The follow-up duration was similar for both groups (44.0 vs. 43.0 months, p = .877), and no patient died. Stent-related complications were significantly lower in group A than in group B (1.5% vs. 8.4%, p = .009). Group A had fewer instances entry flow (0.7% vs. 4.7%, p = .048) and stent stenosis (0.7% vs. 2.8%, p = .206) than group B. All reintervention cases (4.7%) were from group B (p = .011). The rate of false aortic lumen thrombosis was significantly higher in group A than in group B (84.6% vs. 72.9%, p = .024). Both CSs and ISFs are evidently safe, feasible, and effective in achieving positive early outcomes in patients undergoing treatment for TBAD. Notably, at midterm follow-up, CSs appeared to be superior to ISF in terms of reducing stent-related complications and minimizing the need for reintervention.

[Total replacement of both carotid arteries combined with ascending aorta and aortic arch replacement for acute DeBakey type I aortic dissection]. / Polnoe protezirovanie obeikh sonnykh arterii v sochetanii s zamenoi dugi i voskhodyashchei aorty pri ostrom rassloenii aorty I tipa po Debeiki.

Involvement of supra-aortic vessels into acute DeBakey type I aortic dissection is a significant predictor of adverse postoperative neurological outcomes and mortality. The choice of surgical tactics remains open in such patients. We present total replacement of both common carotid arteries and proximal part of the right subclavian artery with reconstruction of ascending aorta and aortic arch for acute DeBakey type I dissection.