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.

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.

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.

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.

The Frozen Elephant Trunk Procedure Using New Russian Hybrid Prosthesis "Soft Elephant Trunk": the Early Experience.

Aim      To present the first experience of performing the Frozen Elephant Trunk (FET) surgery in patients with thoracic aorta disease using a new Russian hybrid stent graft "Soft Elephant Trunk".Material and methods  Between 2014 and 2021, 170 patients with thoracic aortic disease underwent complete aortic arch replacement using the FET technique. In 70 of these cases (since June 2019), a hybrid graft "Soft Elephant Trunk" was used. A specific feature of this graft is the conical reduction of the radial force of nitinol crowns and the soft distal end without radial force. The study endpoints were early postoperative results, as well as the absence of reoperations on the aorta, absence of stent-graft-associated complications, including distal stent-graft-induced fenestration, and survival at a mid-term follow-up of up to 2.5 years.Results Interventions were performed in patients with A type aortic dissection (n=51; 72.9 %), type B aortic dissection (n=13; 18.6 %), and thoracoabdominal aortic aneurysm (TAAA) (n=6; 8.5 %). In 14 (20 %) of these cases, interventions were performed after a primary intervention on the proximal aorta. Acute aortic dissection was diagnosed in 17 (24.3 %) cases. Aortic root replacement was performed in 21 (30 %) cases, David procedure in 5 (7.2 %) cases, Bentall-DeBono procedure in 11 (15.7 %) cases, and supracoronary graft placement in 33 (47.1 %) cases. There were no cases of paraplegia or paraparesis. Respiratory insufficiency was observed in 8 (11.4%) cases. In one (1.4%) case, acute renal failure developed, which required renal replacement therapy. In-hospital mortality was 4.3 % (3 patients died). Mean follow-up duration was 9 [4.25; 16] months. Three-year survival was 94 % (95 % confidence interval, CI: 88-99.9) and absence of reoperation was 96.6 % (95 % CI: 90.1-100). There were no cases of distal stent-graft-induced fenestration in this group.Conclusion      The new hybrid graft, due to its specific structure, provides prevention of distal stent-graft-induced fenestration and, thereby, a stable long-term result. Using this stent-graft is effective in patients with thoracic aorta pathology undergoing the FET surgery. However, further studies are needed to determine potential advantages and disadvantages of this new hybrid graft, to obtain long-term results and accumulate experience.

"Big Data" Analyses Underlie Clinical Discoveries at the Aortic Institute.

This issue of the Yale Journal of Biology and Medicine (YJBM) focuses on Big Data and precision analytics in medical research. At the Aortic Institute at Yale New Haven Hospital, the vast majority of our investigations have emanated from our large, prospective clinical database of patients with thoracic aortic aneurysm (TAA), supplemented by ultra-large genetic sequencing files. Among the fundamental clinical and scientific discoveries enabled by application of advanced statistical and artificial intelligence techniques on these clinical and genetic databases are the following: From analysis of Traditional "Big Data" (Large data sets). 1. Ascending aortic aneurysms should be resected at 5 cm to prevent dissection and rupture. 2. Indexing aortic size to height improves aortic risk prognostication. 3. Aortic root dilatation is more malignant than mid-ascending aortic dilatation. 4. Ascending aortic aneurysm patients with bicuspid aortic valves do not carry the poorer prognosis previously postulated. 5. The descending and thoracoabdominal aorta are capable of rupture without dissection. 6. Female patients with TAA do more poorly than male patients. 7. Ascending aortic length is even better than aortic diameter at predicting dissection. 8. A "silver lining" of TAA disease is the profound, lifelong protection from atherosclerosis. From Modern "Big Data" Machine Learning/Artificial Intelligence analysis: 1. Machine learning models for TAA: outperforming traditional anatomic criteria. 2. Genetic testing for TAA and dissection and discovery of novel causative genes. 3. Phenotypic genetic characterization by Artificial Intelligence. 4. Panel of RNAs "detects" TAA. Such findings, based on (a) long-standing application of advanced conventional statistical analysis to large clinical data sets, and (b) recent application of advanced machine learning/artificial intelligence to large genetic data sets at the Yale Aortic Institute have advanced the diagnosis and medical and surgical treatment of TAA.

Aortic Dissection and a Previously Unreported ACTA2 Missense Variant Mutation in a Young Patient: A Case Report.

Hereditary connective tissue disease is known to cause aortic lesions at an early age. Familial aortic aneurysm/dissection is caused due to an ACTA2 mutation that affects smooth muscle structure. We present a case of a 15-year-old boy with a mild developmental disorder in whom no abnormalities were identified on previous physical examinations. The patient presented with severe left heart failure, extensive dissection from the ascending aorta to the common iliac artery, and myocardial and cerebral infarctions. He underwent an urgent Bentall surgery. Six months later, the patient underwent surgical reconstruction of the abdominal aorta from the aortic arch and returned to normal daily activities. Pathological examination demonstrated the absence of elastic fibers but presence of abundant reticular fibers and mucopolysaccharides from the tunica intima to the media. Genetic testing revealed a heterozygous missense variant of the ACTA2 gene. To the best of our knowledge, this is the first sporadic case of structurally abnormal smooth muscle organization resulting in clinical symptoms with no previously reported pathogenicity.

Influence of a regional network combined with a systematic multidisciplinary approach on the outcomes of patients with acute type A aortic dissection.

BACKGROUND: Whether in patients with acute type A aortic dissection reduction of intervals between onset of symptoms and diagnosis influences patient outcomes is still not completely defined. METHODS: In 199 patients with acute type A aortic dissection, the efficacy of a systematic multidisciplinary approach and institution of a regional network were evaluated; 90 patients operated before 2016 (Group1) were compared with 109 repaired after 2016 (Group2) for early and late outcomes. RESULTS: Mortality was reduced from 13% in Group1 to 4% in Group2 (p = 0.013). In Group2 a more patients (46%) had arch replacement compared to Group1 (29%)(p = 0.06). In Group2 axillary artery cannulation was almost routinely used (91% vs 67%, p < 0.001) with shorter circulatory arrest time (37 vs 44 min, p < 0.001). The interval from diagnosis to surgery dropped from 210 min in Group1 to 160 min in Group2 (p < 0.001); this reduction was evident both in patients admitted to the emergency department of a spoke and/or a hub center. Patients presenting with or developing shock were reduced from Group1 to Group2 and in particular those reaching the hub center from spoke centers. Survival at 1 and 5 years was 82 ± 4% and 70 ± 5% in Group1 vs 92 ± 3% and 87 ± 8% in Group2 (p = 0.007). CONCLUSIONS: Outcomes of patients with acute type A aortic dissection improved using a systematic multidisciplinary approach while a network between spoke and hub centers reduced intervals between diagnosis, transportation to hub center and repair, limiting the incidence of tamponade and shock.

Thoracic aortic dissection presenting with bilateral haemothoraces: a potentially missed diagnosis.

Thoracic aortic dissection is a life-threatening diagnosis, which should not be missed. We present a case in which a patient who presented to the emergency department with chest pain was assessed and referred for admission for treatment of pneumonia, due to misinterpretation of a chest radiograph finding. The patient was re-reviewed and subsequently underwent further investigation, which confirmed aortic dissection. She underwent emergency thoracic endovascular aortic graft repair with stent graft insertion. This case demonstrates haemothorax as an uncommon complication of aortic dissection.

Thoracic Endovascular Aortic Repair Versus Open Surgery for Stanford Type B Aortic Dissection: A Meta-Analysis and Systematic Review.

BACKGROUND: Thoracic endovascular aortic repair is a relatively new technique relative to open surgery, and our aim was to assess whether there is a difference in the risk of common postoperative complications between thoracic endovascular aortic repair and open surgery. METHODS: The PubMed, Web of Science, and Cochrane library were systematically searched for trials comparing thoracic endovascular aortic repair and open surgical repair from January 2000 to September 2022. Primary outcome was death, other outcomes included common associated complications. Data were combined using risk ratio or standardized mean difference with 95% confidence interval. Funnel plot and egger's test were used for assessing publication bias. The study protocol was registered prospectively with PROSPERO (CRD42022372324). RESULTS: This trial included 11 controlled clinical studies with 3667 patients. Thoracic endovascular aortic repair had lower risk of death (risk ratio [RR], 0.59; 95% CI, 0.49 to 0.73; p < 0.00001; I2 = 0), dialysis (RR, 0.55; 95% CI, 0.47 to 0.65; p < 0.00001; I2 = 37%), stroke (RR, 0.71; 95% CI, 0.51 to 0.98; p = 0.03; I2 = 40%), bleeding (RR, 0.44; 95% CI, 0.23 to 0.83; p = 0.01; I2 = 56%), and respiratory complications (RR, 0.67; 95% CI, 0.60 to 0.76; p < 0.00001; I2 = 37%) compared with open surgical repair. In addition, the length of hospital stay was shorter in the thoracic endovascular aortic repair group (SMD, -0.84; 95% CI, -1.30 to -0.38; p = 0.0003; I2 = 80%). CONCLUSIONS: Thoracic endovascular aortic repair has significant advantages over open surgical repair, in terms of postoperative complications and survival in Stanford type B aortic dissection patients.

Characteristics and Outcomes in Patients With Acute Aortic Dissection: A Nationwide Registry Study.

BACKGROUND: We aimed to describe characteristics and outcomes in a nationwide population of patients with acute type A and type B aortic dissection. METHODS: All patients in Denmark with a first-time diagnosis of acute aortic dissection between 2006 and 2015 were identified by national registries. The main outcomes were in-hospital mortality and long-term survival in hospital survivors. RESULTS: The study population comprised 1157 (68%) patients with type A aortic dissection and 556 (32%) patients with type B aortic dissection, median age of 66 (57-74) years and 70 (61-79) years, respectively. Men accounted for 64%. Median follow-up was 8.9 (6.8-11.5) years. Of patients with type A aortic dissection, 74% were managed surgically, whereas 22% of the patients with type B aortic dissection were managed with surgery or endovascular technique. In-hospital mortality was 27% for type A aortic dissection overall (surgery, 18%; no surgery, 52%) and 16% for type B aortic dissection (surgery or endovascular treatment, 13%; conservative treatment, 17%; P < .001, type A vs type B). Of patients discharged alive, survival was persistently better for type A aortic dissection than for type B aortic dissection (P < .001). Unadjusted 1- and 3-year survival of patients with type A aortic dissection discharged alive was 96% and 91%, respectively, for surgically managed and 88% and 78% without surgery. For type B aortic dissection, the numbers were 89% and 83% for endovascular/surgically managed and 89% and 77% for conservatively managed. CONCLUSIONS: We found higher in-hospital mortality for type A and type B aortic dissection than is reported from referral center registries. Type A aortic dissection had the highest mortality rate during the acute phase, whereas for patients who were discharged alive, the mortality rate was higher for patients with type B aortic dissection.

Perioperative neuromonitoring during thoracoabdominal aortic aneurysm open repair: a systematic review.

OBJECTIVES: While open surgical repair remains the gold standard for thoracoabdominal aortic aneurysm (TAAA) treatment, there is still no consensus regarding perioperative neuromonitoring technique for prevention of spinal cord ischaemia. METHODS: In this systematic review, we aimed to explore the effects and practices of neuromonitoring during the open TAAA repair. A systematic literature search in PubMed, Embase via Ovid, Cochrane library and ClinicalTrialsGov until December 2022 was performed. RESULTS: A total of 535 studies were identified from the literature search, of which 27 studies including a total of 3130 patients met the eligibility criteria. Most studies (21 out of 27, 78%) investigated the feasibility of motor-evoked potentials (MEP), while 15 analysed somatosensory-evoked potentials (SSEP) and 2 studies analysed near-infrared spectroscopy during open TAAA repair. CONCLUSIONS: Current literature suggest that rates of postoperative spinal cord ischaemia can be kept at low levels after open TAAA repair with the adequate precautions and perioperative manoeuvres. Neuromonitoring with MEP provides the surgeon objective criteria to direct selective intercostal reconstruction or other protective anaesthetic and surgical manoeuvres. Simultaneous monitoring of MEP and SSEP is a reliable method that can rapidly detect important findings and direct adequate protective manoeuvres during open TAAA repair.

Endovascular treatment of a traumatic thoracic pseudo-aneurysm in a pediatric patient: a case report with review of literature.

Blunt aortic injury (BAI) as a result of thoracic trauma is a rare entity in the adult and pediatric population. The endovascular approach has been the preferred method of management over operative repair in adults. However, data on pediatrics is limited to case reports and case series with no long-term follow-up. There are no current guidelines for management in the pediatric population. We are reporting a successful repair of a traumatic thoracic aortic aneurysm in a 13 year old boy with covered stents, with a review of relevant literature.