Summary: International consensus statement on nomenclature and classification of the congenital bicuspid aortic valve and its aortopathy, for clinical, surgical, interventional, and research purposes.

This International evidence-based nomenclature and classification consensus on the congenital bicuspid aortic valve and its aortopathy recognizes 3 types of bicuspid aortic valve: 1. Fused type, with 3 phenotypes: right-left cusp fusion, right-non cusp fusion and left-non cusp fusion; 2. 2-sinus type with 2 phenotypes: Latero-lateral and antero-posterior; and 3. Partial-fusion or forme fruste. This consensus recognizes 3 bicuspid-aortopathy types: 1. Ascending phenotype; root phenotype; and 3. extended phenotypes.

International consensus statement on nomenclature and classification of the congenital bicuspid aortic valve and its aortopathy, for clinical, surgical, interventional and research purposes.

This International Consensus Classification and Nomenclature for the congenital bicuspid aortic valve condition recognizes 3 types of bicuspid valves: 1. The fused type (right-left cusp fusion, right-non-coronary cusp fusion and left-non-coronary cusp fusion phenotypes); 2. The 2-sinus type (latero-lateral and antero-posterior phenotypes); and 3. The partial-fusion (forme fruste) type. The presence of raphe and the symmetry of the fused type phenotypes are critical aspects to describe. The International Consensus also recognizes 3 types of bicuspid valve-associated aortopathy: 1. The ascending phenotype; 2. The root phenotype; and 3. Extended phenotypes.

[Surgical treatment of acute aortic syndrome: an update]. / Trattamento chirurgico della patologia aortica acuta: un update.

The term "acute aortic syndrome" describes a variety of acute and emerging aortic pathologies that include intramural hematoma, penetrating aortic ulcer and acute aortic dissection. However, the acute pathology of the thoracic aorta also includes the contained ruptures of aortic aneurysms, traumatic aortic ruptures and iatrogenic aortic dissections. In all these acute situations, in which emerging surgical treatment is often required, decision-making represents a crucial and extremely important phase, which often affects the patient's prognosis, in the short and long term. This review aims to present an update of the surgical treatment of acute aortic syndrome focusing mainly on the correct decision-making, the factors that influence it and the latest novel surgical techniques and strategies.

Acute Aortic Syndromes: Diagnostic and Therapeutic Pathways.

Acute aortic syndromes are life-threatening medical conditions that include classic acute aortic dissection (AAD), aortic intramural hematoma, penetrating aortic ulcer, and even aortic pseudoaneurysm and traumatic aortic injury. The European Society of Cardiology has designed a multiparametric diagnostic algorithm to provide stepwise diagnosis. All patients with AAD should receive aggressive medical therapy to control blood pressure and heart rate. Urgent surgical repair is recommended for type A AAD. Uncomplicated type B AAD requires aggressive medical therapy. In contrast thoracic endovascular repair is recommended for complicated type B. AAD should be considered a lifelong disease that affects the entire aorta.

[Type A acute aortic syndromes: Assessment of the management delays in an emergency medicine network]. / Syndromes aortiques aigus de type A : évaluation des délais de prise en charge dans un réseau de médecine d'urgence.

OBJECTIVE: To assess the diagnostic delay (between first hospital medical contact and diagnosis) and the surgical delay (between diagnosis and incision) of type A acute aortic syndromes (AAAS) within the RENAU (REseau Nord Alpin des Urgences), organizing the management of emergency medicine care in the French North Alpine Arc. PROCEDURE: Multicenter retrospective study between 2012 and 2016 on the AAAS operated in the RENAU heart surgical centers (Annecy, Grenoble). Post-traumatic, iatrogenic or chronic lesions, incidental discoveries and deaths before surgery were excluded. RESULTS: One hundred and ninety-seven patients were included with a median age [IQR] of 65 years [58; 73] of which 67% were men. The median diagnosis delay was 88min [46;241] and the median surgical delay was 193min [146;249]. Initial management was performed by the SMUR for 102 patients (52%), 7% of whom received a pre-hospital transthoracic ultrasound. 52 patients (26%) presented themselves spontaneously to the emergency department. Patients were initially admitted in a center without cardiac surgery in 65% of cases. The CT scan was the diagnostic test in 81% of cases. The postoperative hospital mortality was 16%. CONCLUSION: Referring to IRAD data reporting a median diagnostic and surgical delay of 258min each, our study suggests that the RENAU organization may be associated with reduced diagnostic and surgical delays for patients with SAAA.

Type A Aortic Dissection in Patients With Bicuspid Aortic Valve Aortopathy.

BACKGROUND: The aim of this study was to evaluate clinical, aortic, and outcome characteristics of type A aortic dissection patients with bicuspid aortic valves (BAVs) and tricuspid aortic valves (TAVs). METHODS: Patient characteristics and radiographic, operative, and outcome data were evaluated and compared between 1068 TAV patients and 72 BAV patients operated on for type A aortic dissection in 2 centers. Predissection aortic diameters were calculated as previously reported for TAV patients. RESULTS: BAV patients were significantly younger (P < .001) and had a lower incidence of cardiovascular risk factors. Although the clinical presentation was similar, the dissection affected the abdominal aorta significantly more often in TAV patients (P = .029). Aortic root replacements were performed significantly more often in BAV patients (P < .001). Postoperative outcome was similar between the 2 groups. BAV patients had a significantly larger maximum postdissection diameter (P < .001) and calculated predissection diameter (P < .001) compared with TAV patients. Predissection ascending aortic diameters were less than 5.5 cm in 96% of all TAV patients and less than 5.0 cm in 76% of all BAV patients. CONCLUSIONS: Acute type A aortic dissection in BAV patients is not associated with worse clinical or long-term outcome but significantly influences the proximal aortic repair. After modeling predissection aortic diameters, less than 5% of all TAV patients and possibly less than 25% of all BAV patients would meet the elective threshold for preventative replacement of the ascending aorta.

Regional and Temporal Trends in the Outcomes of Repairs for Acute Type A Aortic Dissections.

BACKGROUND: Little information exists regarding the use of arch operations for repair of acute type A aortic dissections (AADs) despite increasing interest in this strategy and its potential impact on outcomes. We aimed to determine the relationship between extent of aortic repair, US geographic regions, and outcome. METHODS: We queried The Society of Thoracic Surgeons database for patients who underwent AAD repair from January 1, 2004 to December 31, 2016 and grouped patients by ascending-only operations and operations involving the arch. RESULTS: We identified 25,462 patients (mean age, 59.8 ± 14.2; 66.7% men) who underwent AAD repair. Operations involving the ascending aorta only were performed in 54% of patients; 46% had repair additionally involving the arch. The 30-day mortality was 18.9% for patients who underwent ascending-only operations vs 19.8% for patients who underwent arch operations (P = .09). In multivariable analysis older age (P < .001), earlier year of operation (P < .001), diabetes mellitus (P < .001), severe chronic lung disease (P < .001), prior cerebrovascular disease (P < .001), and longer bypass time (P < .001) were independently associated with 30-day mortality. There was regional variation in 30-day mortality (P < .001), and incidence of arch repair varied from 38.6% to 52.6% in 9 geographic regions (P < .001). CONCLUSIONS: In this analysis of cardiac surgical practice in the United States, repair of AADs included a portion of the aortic arch in 46% of patients. Early mortality remained high throughout the current era regardless of extent of aortic resection. Regional variation in perioperative mortality may signal an opportunity for practice improvement.

Estudio imagenológico del síndrome aórtico agudo / Imaging approach to acute aortic syndrome

Acute aortic syndromes include a spectrum of life-threatening aortic conditions. A review of the diagnostic aspects of the acute aortic syndrome was made, from the perspective of the imaging techniques available for this purpose. The advantages and disadvantages of each technique and its diagnostic performance were evaluated. Emphasis was placed on the relevance of clinical information as a fundamental tool for suspecting this syndrome and appropriately choosing the imaging technique. Our main objective is to provide information about the diagnosis of this condition, especially in the context of emergency services.

Differential clinical features and long-term prognosis of acute aortic syndrome according to disease entity.

AIMS: To evaluate the acute and long-term prognosis of acute aortic syndrome (AAS) according to the disease entity [intramural haematoma (IMH) vs. aortic dissection (AD)] and the anatomical location (type A vs. B). METHODS AND RESULTS: A total of 1012 patients [672 with AD and 340 with IMH (33.6%)] were enrolled between 1993 and 2015. Compared with AD patients, IMH patients were older and had higher frequency of female sex and distal aorta involvement. The overall crude in-hospital mortality of AAS was 8.6%; type A AD [15.0%; adjusted hazard ratio (aHR) 30.4; 95% confidence interval (CI) 8.62-107.3; P < 0.001], type A IMH (8.0%; aHR 4.85; 95% CI 1.29-18.2; P = 0.019), type B AD (5.0%; aHR 3.51; 95% CI 1.00-12.4; P = 0.051), and type B IMH [1.5%; aHR 1.00 (reference)]. During a median follow-up duration of 8.5 years (interquartile range: 4.0-13.5 years), AD (aHR 2.78; 95% CI 1.87-4.14; P < 0.001) and type A (aHR 2.28; 95% CI 1.45-3.58; P < 0.001) was associated with a higher risk of aortic death. After 90 days, a risk of aortic death was no longer associated with anatomical location (aHR 0.74; 95% CI 0.40-1.36; P = 0.33), but remained associated with disease entity (aHR 1.83; 95% CI 1.10-3.04; P = 0.02). CONCLUSION: The clinical features, response to treatment strategy, and outcomes of IMH patients were distinct from those of AD patients. Both early and late survival was better for IMH than for AD. In addition to the anatomical location of AAS, the disease entity is an independent factor associated with both acute and long-term mortality in patients with AAS. Further investigation is necessary to confirm the prognostic implication of disease entity in different patient populations.

Influence of Age and the Burden of Ischemic Injury on the Outcome of Type A Aortic Dissection Repair.

BACKGROUND: This study evaluated operative details and postoperative outcomes in elderly patients according to the burden of ischemic injury. METHODS: Between 2002 and 2017, 1187 patients in 2 centers were operated on for aortic dissection type A (ADA). Patients were grouped according to the Penn classification: class A, 628 patients; class B, 196; class C, 224; and class BC, 139. The perioperative conditions and outcomes were analyzed. RESULTS: The likelihood of presenting in a Penn class changed significantly with age (P = .02). Also, the probability of ADA extension into the supraaortic vessels (P < .001) or the distal aorta (P < .001) decreased significantly over age. Nevertheless, there was no significant difference in the distal aortic repair between younger and older patients. The probability of in-hospital mortality increased significantly in all Penn classes with age (P < .001). Yet, predicted mortality remained below 15% for any age in class A patients but increased up to 25% in class B and C patients and beyond 50% in class BC patients. Class A or B were not predictive of in-hospital mortality in septuagenarians or octogenarians. CONCLUSIONS: Age by itself is not a rational criterion to select patients for surgical treatment, and a surgical approach is very reasonable in all class A patients independent of age. The predicted mortality in classes B, C, and particularly class BC is dismal in the elderly. Those patients may benefit from alternative, evolving therapeutic options such as ascending endovascular treatments.

[Imaging approach to acute aortic syndrome]. / Estudio imagenológico del síndrome aórtico agudo.

Acute aortic syndromes include a spectrum of life-threatening aortic conditions. A review of the diagnostic aspects of the acute aortic syndrome was made, from the perspective of the imaging techniques available for this purpose. The advantages and disadvantages of each technique and its diagnostic performance were evaluated. Emphasis was placed on the relevance of clinical information as a fundamental tool for suspecting this syndrome and appropriately choosing the imaging technique. Our main objective is to provide information about the diagnosis of this condition, especially in the context of emergency services.

A Novel Risk Score Predicts Operative Mortality After Acute Type A Aortic Dissection Repair.

BACKGROUND: Current stratification systems for patients presenting with acute type A aortic dissection rely on signs of malperfusion to predict mortality. The authors sought to develop an algorithm to readily risk stratify these patients using admission characteristics. METHODS: Two hundred sixty-nine consecutive patients who underwent type A repair between 2002 and 2015 were reviewed for easily obtainable preoperative demographics and laboratory values deemed a priori as potential predictors of operative mortality. Multiple logistic regression analysis was performed to determine independent significance, and linear regression was performed to generate the concomitant regression expression of the variables significant on bivariate analysis. RESULTS: Operative mortality was 16% (43/269) and was 29% (34/119) among patients who presented with malperfusion. Upon multivariate analysis, creatinine (p = 0.008), liver malperfusion (p = 0.006), and lactic acid level (p = 0.0007) remained independent significant predictors. Regression coefficients allowed the generation of a risk score as 5.5 × (lactic acid [mmol/L]) + 8 × (creatinine [mg/dL]) ± 8 (+ if liver malperfusion presents, - if no liver malperfusion). Upon receiver-operating characteristic curve analysis this model generated a c-statistic of 0.75. Operative mortality among patients within the lowest tertile (risk score < 7) was 4%, whereas patients in the middle (7 to 20) and highest (≥20) tertiles had mortality rates of 14% 37%, respectively. CONCLUSIONS: Although still requiring external validation, the innovative risk score presented necessitates knowledge of lactic acid, serum creatinine, and liver function tests. The algorithm predicts operative mortality with high accuracy and offers clinicians a novel tool to improve preoperative guidance and prognosis.

Adventitial Wrap Technique for Acute Type A Aortic Dissection.

We describe a simple and reproducible technique to achieve complete and immediate hemostasis of the distal anastomosis in total arch replacement with the frozen elephant trunk technique. The adventitia was left seamlessly 1.0 cm longer than the level of the suture line. The adventitial remnant covered most of the distal anastomosis line tightly while the distal anastomosis was completed with continuous sutures. The adventitia was thin and tough, making it suitable to cover the suture line. This technique can contribute to eliminating bleeding from the distal anastomosis independent of unreliable blood coagulability during aortic surgery for acute aortic dissection.

Analysis of risk factors and classification of aortic fistula after esophagectomy.

BACKGROUND: Aortic fistula after esophagectomy is a rare and serious complication. The aims of this study were to describe the causes of and classify the fistulas. MATERIALS AND METHODS: Between January 2008 and December 2017, a total of 1018 patients underwent esophageal resection, mainly for esophageal cancer; aortic fistula after esophagectomy was diagnosed in four patients. We perform a literature review through a database search for similar cases. Aortic fistulas may be classified into two types based on the site at which they occur in relation to the alimentary tract and area of anastomosis. Type 1 fistula occurs within the area of anastomosis, whereas type 2 fistula occurs above or below the anastomosis. The risk factors and clinical features associated with aortic fistulas are described, and comparison between the two types is made. RESULTS: Through a literature search, 39 cases were identified, of which 26 cases were classified as type 1, and 13 cases were classified as type 2. Of 13 patients (33.3%) who underwent emergent intervention, seven patients survived. Approximately 76.9% of aortic fistula were related to anastomotic fistula, which was more prevalent in type 1 aortic fistula than in type 2 (92% versus 50%, P = 0.005). There was no statistically significant difference in age, gender, side of thoracotomy, type of anastomosis, the postoperative day the hemorrhage occurred, warning hemorrhage, chest pain, or the outcome between the two types of fistula. CONCLUSIONS: Anastomotic fistula is the primary cause of type 1 aortic fistula after esophagectomy, and early diagnosis and intervention of aortic fistula can improve prognosis. This classification may be a useful guide in determining the approach for second-stage alimentary tract reconstruction.

Acute aortic syndromes: diagnosis and management, an update.

Acute aortic syndromes (AAS) encompass a constellation of life-threatening medical conditions including classic acute aortic dissection (AAD), intramural haematoma, and penetrating atherosclerotic aortic ulcer. Given the non-specific symptoms and physical signs, a high clinical index of suspicion is necessary to detect the disease before irreversible lethal complications occur. In order to reduce the diagnostic time delay, a comprehensive flowchart for decision-making based on pre-test sensitivity of AAS has been designed by the European Society of Cardiology guidelines on aortic diseases and should be thus applied in the emergency scenario. When the definitive diagnosis is made, prompt and appropriate therapeutic interventions should be undertaken if indicated by a highly specialized aortic team. Urgent surgery for AAD involving the ascending aorta (Type A) and medical therapy alone for AAD not involving the ascending aorta (Type B) are typically recommended. In complicated Type B AAD, thoracic endovascular aortic repair (TEVAR) is generally indicated. On the other hand, in uncomplicated Type B AAD, pre-emptive TEVAR rather than medical therapy alone to prevent late complications, while intuitive, requires further study in randomized cohorts. Finally, it should be highlighted that there is an urgent need to increase awareness of AAS worldwide, including dedicated education/prevention programmes, and to improve diagnostic and therapeutic strategies, outcomes, and lifelong surveillance.

Open descending thoracic or thoracoabdominal aortic approaches for complications of endovascular aortic procedures: 19-year experience.

OBJECTIVES: Endovascular aortic repair is increasingly being used to treat aneurysms, dissections, and traumatic injuries, despite its unknown long-term durability. We describe our 19-year experience with open descending thoracic and thoracoabdominal aortic repair after endovascular aortic repair. METHODS: Between 1996 and 2015, 67 patients were treated with open distal arch, descending thoracic, or thoracoabdominal aortic repair, or extra-anatomic bypass repair with aortic extirpation for complications after endovascular repair of the thoracic (n = 45, 67%) or abdominal (n = 22, 33%) aorta. The median interval between procedures was 18.0 months (interquartile range, 3.9-44.9). Indications for open repair included expanding aneurysm (n = 56), infection (n = 11), fistula (n = 8), aneurysm rupture (n = 5), pseudoaneurysm (n = 2), and restenosis (n = 1). Open repair involved partial (n = 9, 13%) or complete (n = 56, 84%) device removal or device salvage (n = 2, 3%) through a thoracoabdominal (n = 58, 87%) or thoracotomy (n = 9, 13%) incision. Eight patients (12%) underwent emergency procedures. RESULTS: There were 3 early (operative) deaths (2 with preoperative device infection) and 19 late deaths during a median follow-up of 35.8 months (interquartile range, 16.8-52.8 months). Overall 1- and 5-year survivals were 85% ± 4% and 60% ± 8%, respectively. Four patients had open repair failures necessitating reoperation; 2 patients had preoperative infection, and both died (1 early and 1 late). CONCLUSIONS: Open repair for complications after endovascular procedures is not uncommon. Experienced centers can yield acceptable outcomes, especially in patients without infection. Close surveillance is mandatory after endovascular aortic repair.

Performance of a Simplified Dichotomous Phenotypic Classification of Bicuspid Aortic Valve to Predict Type of Valvulopathy and Combined Aortopathy.

BACKGROUND: A simplified classification of bicuspid aortic valve (BAV) morphology using only the orientation of fused cusps was recently proposed. The aim of this study was to test whether it is useful for showing an association with the type of valvulopathy or aortopathy. METHODS: BAV phenotype was retrospectively classified in 681 patients (mean age, 59 ± 12 years; 424 men) who underwent aortic valve surgery. Each BAV was classified using both dichotomous (right and left coronary cusp fusion [CCF] vs mixed cusp fusion [MCF]) and conventional methods, and its association with the dominant valvulopathy (aortic stenosis [AS] vs regurgitation) and concomitant aortic surgery was analyzed. Four cardiologists individually reviewed transthoracic echocardiographic images of 100 randomly selected patients to compare the feasibility and accuracy of the two classification methods. RESULTS: The frequencies of BAV CCF and MCF were 53% (n = 361) and 47% (n = 320), respectively. AS was the predominant cause of surgery (n = 546 [80%]), and concomitant aortic surgery was done in 31% (n = 214). Patients with BAV MCF showed a higher frequency of AS (89% vs 73%, P < .001) and aortic surgery (38% vs 26%, P < .001) than those with BAV CCF. There were independent associations between BAV MCF and AS (odds ratio, 3.32; 95% CI, 1.99-5.54; P < .001) as well as aortic surgery (odds ratio, 1.76; 95% CI, 1.26-2.45; P = .001). The feasibility of the classification methods did not differ, but dichotomous classification revealed higher accuracy than conventional (87% [95% CI, 84.1%-90.7%] vs 70% [95% CI, 65.0%-74.3%]) for all four examiners, with higher κ coefficients representing interrater agreement (κ = 0.73 ± 0.06 to 0.83 ± 0.06 [dichotomous method] vs 0.51 ± 0.06 to 0.73 ± 0.06 [conventional method]). CONCLUSIONS: The dichotomous classification method is useful for showing the association with the type of valvulopathy or aortopathy, with better diagnostic performance than the conventional method.

Acute aorta, overview of acute CT findings and endovascular treatment options.

Acute aortic pathologies include acute aortic syndrome (aortic dissection, intramural hematoma, penetrating aortic ulcer), impending rupture, aortic aneurysm rupture and aortic trauma. Acute aortic syndrome, aortic aneurysm rupture and aortic trauma are life-threatening conditions requiring prompt diagnosis and treatment. The basic imaging modality for "acute aorta" is CT angiography with typical findings for these aortic pathologies. Based on the CT, it is possible to classify aortic diseases and anatomical classifications are essential for the planning of treatment. Currently, endovascular treatment is the method of choice for acute diseases of the descending thoracic aorta and is increasingly indicated for patients with ruptured abdominal aortic aneurysms.