Life-Threatening Aortic Dissection during Pregnancy: A Case Report of Undiagnosed FBN1-Related Marfan Syndrome at 39 Weeks Gestation.

BACKGROUND Inherited deficiencies in the FBN1 gene, which encodes fibrillin-1, result in Marfan syndrome, an autosomal dominant connective tissue disorder that is associated with aortic root dilatation and predisposes to aortic dissection. This report is of a 37-year-old woman presenting at 39 weeks of pregnancy with acute thoracic aortic dissection due to previously undiagnosed FBN1-related Marfan syndrome. This case report aims to illustrate the challenges in the diagnosis and in the peri-operative management of acute aortic dissection during pregnancy. CASE REPORT A healthy 37-year-old woman at 39 weeks of gestation presented to our hospital with dyspnea and chest pain. Initial evaluation for pulmonary embolism with chest computed tomography was unrevealing. The patient was admitted to the intensive care unit for further management. Overnight, her clinical conditions deteriorated, and a transthoracic echocardiography was obtained, demonstrating an acute ascending aortic dissection. She emergently underwent a successful combined cesarean section and ascending aortic dissection repair, with no immediate complications. On postoperative day 4 she developed cardiac tamponade, for which she underwent emergent mediastinal exploration. She was discharged home on postoperative day 10. A month later she completed genetic testing, which revealed a pathogenic mutation in the FBN1 gene, consistent with a molecular diagnosis of Marfan syndrome. CONCLUSIONS This report has shown that FBN1-related Marfan's syndrome has a variable clinical presentation that can include life-threatening aortic dissection during pregnancy. Successful diagnosis and management of these patients is challenging and requires multidisciplinary expertise, including confirmation of the diagnosis by a clinical geneticist.

Iatrogenic Acute Ascending Aortic Dissection During Combined Heart/Liver Transplant for Amyloidosis.

We report a case of iatrogenic acute type A aortic dissection (ATAAD) during a combined heart-liver transplant in a patient with amyloid-associated cardiac and hepatic failure. The patient developed ATAAD of the recipient's aorta during the heart transplantation. Because there was no sign of malperfusion or proximal extension into the donor aorta, we proceeded with the liver transplantation and continued medical management for ATAAD. The patient was discharged uneventfully 30 days after the transplant, and computed tomography coronary angiogram after 4 months showed stable dissection. During a heart transplant, ATAAD of the native aorta without malperfusion syndrome can be managed conservatively with close progress monitoring.

How to test adhesive strength: a biomechanical testing for aortic glue used in type a dissection repair.

OBJECTIVES: The aim of this study was to develop a method to quantify the peel force in an in vitro model simulating repair of ascending aortic dissections with tissue glue (Bioglue). METHODS: This study adapted an adhesive T-Peel test for the determination of the peel strength of adhesives by measuring the peeling force of a T-shaped bonded tissue. Measurements were performed on iatrogenic dissected ascending porcine aorta, which has been repaired with Bioglue using different pressure levels. Four conditions were tested: zero sample pressure according to the manufacturer's recommendation (n = 10), low (504 Pa; n = 11), moderate pressure (1711 Pa; n = 24) and pressure applied by a round shaped vascular 'Borst clamp' (1764 Pa; n = 23). Non-parametric one-way analysis of variance was applied for statistical significance. RESULTS: The median peel force (lower quartile, upper quartile) of aortic samples increased depending on the applied pressure: [no pressure 0.030 N/mm (0.016, 0.057), low pressure 0.040 N/mm (0.032, 0.070) and moderate pressure 0.214 N/mm (0.050, 0.304)]. Samples pressurized with the Borst clamp reached 0.078 N/mm (0.046, 0.152), which was comparable to the peel force of the unpeeled controls [0.107 N/mm (0.087, 0.124)]. Compared to samples without pressure, Bioglue with the application of the Borst clamp (P = 0.021) and with moderate pressure (P = 0.0007) performed significantly better. CONCLUSIONS: The novel T-Peel test offers an attractive method to test tissue glues in defined in vitro environments. Bioglue peel force increased with pressure on the aortic sample in contrast to low or no pressure as per the manufacturer's recommendation. Modifying current recommended use may aid in increasing effectiveness of this approach.

Debranching Thoracic Endovascular Aortic Repair Combined with Ascending Aortic Banding: Analysis of a New Surgical Procedure.

BACKGROUND: To analyze the clinical effect of debranching thoracic endovascular aortic repair combined with ascending aortic banding. METHODS: The clinical data of patients who underwent a debranching thoracic endovascular aortic repair combined with ascending aortic banding at Anzhen Hospital (Beijing, China) between January 2019 and December 2021 were reviewed to evaluate the occurrence and outcomes of postoperative complications. RESULTS: A total of 30 patients underwent a debranching thoracic endovascular aortic repair combined with ascending aortic banding. There were 28 male patients (93.3%) with an average age of 59.9 ± 11.8 years. Twenty-five patients underwent simultaneous surgery and five patients had staged surgery. Postoperatively, two patients developed complete paraplegia (6.7%), three patients developed incomplete paraplegia (10%), two patients developed cerebral infarction (6.7%), and one patient developed femoral artery thromboembolism (3.3%). No patient died during the perioperative period, and one patient (3.3%) died during the follow-up period. None of the patients underwent retrograde type A aortic dissection during the perioperative and postoperative follow-up periods. CONCLUSIONS: Banding the ascending aorta with a vascular graft to restrict its movement and to serve as the proximal anchoring area of the stent graft can reduce the risk of retrograde type A aortic dissection.

ARISE: First-In-Human Evaluation of a Novel Stent Graft to Treat Ascending Aortic Dissection.

BACKGROUND: Operative mortality for type A aortic dissection is still 10-20% at centers of excellence. Additionally, 10-20% are not considered as viable candidates for open surgical repair and not offered life-saving emergency surgery. ARISE is a multicenter investigation evaluating the novel GORE® Ascending Stent Graft (ASG; Flagstaff, AZ). OBJECTIVE: The purpose of this study is to assess early feasibility of using these investigational devices to treat ascending aortic dissection. METHODS: This a prospective, multicenter, non-randomized, single-arm study that enrolls patients at high surgical risk with appropriate anatomical requirements based on computed tomography imaging at 7 of 9 US sites. Devices are delivered transfemorally under fluoroscopic guidance. Primary endpoint is all-cause mortality at 30 days. Secondary endpoints include major adverse cardiovascular and cerebrovascular events (MACCE) at 30 days, 6 months, and 12 months. RESULTS: Nineteen patients were enrolled with a mean age of 75.7 years (range 47-91) and 11 (57.9%) were female. Ten (52.6%) had DeBakey type I disease, and the rest were type II. Sixteen (84.2%) of the patients were acute. Patients were treated with safe access, (7/19 (36.8%) percutaneous, 10/19 (52.6%) transfemoral, 2/19 (10.5%) iliac conduit), delivery, and deployment completed in all cases. Median procedure time was 154 mins (range 52-392) and median contrast used was 111 mL (range 75-200). MACCE at 30 days occurred in 5 patients including mortality 3/19 (15.8%), disabling stroke in 1/19 (5.3%), and myocardial infarction in 1/19 (5.3%). CONCLUSION: Results from the ARISE early feasibility study of a specific ascending stent graft device to treat ascending aortic dissection are promising.

Endovascular Repair of an Ascending Aorta Dissection After Self-expanding TAVR by Suprasternal Approach.

Management of rare ascending aortic complications after transcatheter aortic valve replacement (TAVR) remains a poorly defined area of knowledge. Furthermore, because most patients undergoing TAVR are at intermediate to high surgical risk, the treatment of such complications often requires endovascular approaches. Herein, we present a novel technique of endovascular ascending aorta stent graft delivery from the innominate artery for the treatment of a type A aortic dissection after self-expandable TAVR implantation, specifically addressing the landing of the aortic stent within the crown of the TAVR valve.

Surgical Approaches to Single-Stage Extended Aortic Repair from the Ascending to the Distal Descending Aorta.

Single-stage extended replacement from the ascending to the distal descending aorta or beyond is a formidable operation that should be preserved for those who have no other option or those who are physically fit, and should be performed in the experienced centers. Hybrid operations combining open surgical repair with thoracic endovascular aortic repair through a median sternotomy incision are preferable because these operations are less invasive than the extended open aortic repair and the risk of spinal cord ischemia is lower compared with the frozen elephant trunk operation. However, these operations are associated with the inherent demerits of endovascular aneurysm exclusion. When the underlying aortic pathology necessitates extended open aortic repair in a single stage, approaches such as the anterolateral partial sternotomy, straight incision with rib cross, and extended thoracotomy with sternal transection may be useful to provide sufficient exposure for both aortic reconstruction and organ protection, with less surgical stress to the patients.

Ascending Aorta Dissection Before 5.5 cm Diameter; "It Wasn't Raining When Noah Built the Ark".

INTRODUCTION: The aim of this study was to compare mean maximum ascending aortic diameter at the time of acute aortic dissection with the current surgical threshold for elective ascending aortic operations on non-syndromic thoracic aortic aneurysms. MATERIAL AND METHODS: All consecutive non-syndromic adult patients admitted for acute type A aortic dissection in a single tertiary centre were prospectively enrolled from April 2020 to March 2021. The primary endpoint was the difference between mean maximum aortic diameter at the time of dissection and the 5.5 cm threshold for elective repair. Secondary endpoints included 30-day/in-hospital mortality, aortic length and comparison with normal controls, length/height ratio index, "actual" preoperative Euroscore II and "predicted" Euroscore II if electively operated. RESULTS: Among 31 patients ageing 67.3±12.03 years on average, mean maximum aortic diameter at the time of dissection was 5.13±0.66 cm, significantly lower than the guidelines-derived surgical threshold of 5.5 cm (p=0.004). Mean aortic length was 11±1.47 cm, also significantly longer compared normal controls reported in the literature (p<0.001). The 30-day/in-hospital mortality was 35.5%. Mean length/height ratio index was 6.18±0.76 cm/m. Finally, mean "actual" preoperative Euroscore II was 10.43±4.07 which was significantly higher than the 1.47±0.57 "predicted" Euroscore II (p<0.05). CONCLUSIONS: The maximum aortic diameter at the time of acute type A aortic dissection of non-syndromic cases was significantly lower than the current recommendation for elective repair. Lowering of the current diameter-based surgical threshold of 5.5 cm may be profitable in terms of prevention, but further investigations should be undertaken. Length-based thresholds could also add to timely aortic dissection prevention.

Subendocardial Ischemia Caused by Acute Severe Aortic Regurgitation Due to Aortic Root Dissection: A Case Report and Literature Review.

Electrocardiogram (ECG) findings showing ST-segment depression in a wide range of leads and ST-segment elevation in aVR are found in patients with acute coronary syndrome with multivessel coronary lesions and left main trunk lesions. A 64-year-old man with a history of eosinophilic granulomatosis presented with chest pain and dyspnea. Although an ECG showed the above findings, he was diagnosed with acute severe aortic regurgitation (AR) complicating aortic root dissection and successfully underwent urgent Bentall operation. These ECG findings indicated that acute severe AR caused subendocardial ischemia.