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Little is known about the outcomes of aortic root operations that involve inducing hypothermic circulatory arrest for relatively extensive proximal aortic surgery. We attempted to identify predictors of postoperative hospital length of stay (LOS) and factors that affect postoperative recovery.During 2006-2014, 247 of 265 patients (93.2%) with disease extending into the aortic arch survived aortic root operations (206 elective, 41 urgent/emergent) in which hypothermic circulatory arrest with moderate hypothermia was used. Stepwise multivariate regression analysis was performed to identify predictors of LOS (as a continuous variable) and prolonged LOS (defined as LOS9 days, the median for the cohort). By this definition, 111 patients (45%) had prolonged LOS and 136 (55%) did not.Preoperative factors that independently predicted longer LOS in the entire cohort included age (P = .0014), redo sternotomy (P = .0047), and intraoperative packed red blood cell (PRBC) transfusion (P = .0007). Redo sternotomy and intraoperative PRBC transfusion also predicted longer LOS in 3 subgroup analyses: one of elective cases, one from which total arch replacement procedures were excluded, and one limited to patients who were discharged home. Age predicted longer LOS in the non-total arch (hemiarch) replacement patients. Ventilator support48 hours (P .0001) was associated with longer LOS. Elective aortic valve-sparing root replacement predicted a shorter LOS than valve replacement in multivariate regression analysis (P = .028).In patients undergoing aortic root surgery with hypothermic circulatory arrest for disease extending into the aortic arch, reducing intraoperative PRBC transfusion except when absolutely necessary may reduce postoperative LOS and expedite recovery. Performing aortic valve-sparing root replacement, when feasible, may also reduce LOS.

Objectives To determine whether, in patients with previous cardiac operations, moderate hypothermia (between 24 and 28°C) for hypothermic circulatory arrest (HCA) during antegrade cerebral perfusion (ACP) is safe for use during surgery on the proximal aorta and transverse aortic arch. Methods Over a 7-year period, 118 patients underwent ascending aortic and hemiarch repair (n = 70; 59.3%), total arch replacement (n = 47; 39.8%) or ascending aortic replacement to treat porcelain aorta (n = 1; 0.9%). Simultaneous procedures included aortic root repair or replacement (n = 33; 28.0%) and coronary artery bypass grafting (n = 21; 17.8%). All patients had previously undergone cardiac operations via a median sternotomy. Eighteen patients (15.3%) had more than 1 previous sternotomy, and 24 patients (20.3%) required emergent/urgent operation. Median cardiopulmonary bypass, cardiac ischaemic, circulatory arrest and ACP times (min) were 136.0 [118-180 interquartile range (IQR)], 91.0 (68-119 IQR), 34.0 (21-59 IQR) and 33.5 (20-59 IQR), respectively. The median temperature when HCA was initiated was 24.2°C (24.1-24.8°C IQR). Results The operative mortality rate was 10.2% (n = 12). Six patients (5.1%) had a permanent stroke, and 16 patients (13.6%) had a composite adverse outcome (operative mortality and/or a permanent neurological event and/or permanent haemodialysis at discharge). Preoperative renal disease was significantly more prevalent (P= 0.020) and the median circulatory arrest time significantly longer (48.5 vs 33 min; P= 0.058) in patients with composite adverse outcomes. Multivariable analysis of the redo patients showed that age (P =0.025), preoperative renal disease (P =0.024) and ACP time (P =0.012) were independent risk factors for a new postoperative renal injury. Conclusions Moderate hypothermia for HCA during ACP is being used with increasing frequency, but has not been thoroughly evaluated in patients undergoing cardiovascular reoperations. Our experience suggests that in patients with previous cardiac surgery who are undergoing hemiarch and total aortic arch operations, moderate hypothermia is safe and produces respectable results.

Since the pioneering era of E. Stanley Crawford, our multimodal strategy for thoracoabdominal aortic aneurysm repair has evolved. We describe our approximately 3-decade single-practice experience regarding 3309 thoracoabdominal aortic aneurysm repairs and identify predictors of early death and other adverse postoperative outcomes.We analyzed retrospective (1986-2006) and prospective data (2006-2014) obtained from patients (2043 male; median age, 67 [59-73] years) who underwent 914 Crawford extent I, 1066 extent II, 660 extent III, and 669 extent IV thoracoabdominal aortic aneurysm repairs, of which 723 (21.8%) were urgent or emergency. Repairs were performed to treat degenerative aneurysm (64.2%) or aortic dissection (35.8%). The outcomes examined included operative death (ie, 30-day or in-hospital death) and permanent stroke, paraplegia, paraparesis, and renal failure necessitating dialysis, as well as adverse event, a composite of these outcomes.There were 249 operative deaths (7.5%). Permanent paraplegia and paraparesis occurred after 97 (2.9%) and 81 (2.4%) repairs, respectively. Of 189 patients (5.7%) with permanent renal failure, 107 died in the hospital. Permanent stroke was relatively uncommon (n = 74; 2.2%). The rate of the composite adverse event (n = 478; 14.4%) was highest after extent II repair (n = 203; 19.0%) and lowest after extent IV repair (n = 67; 10.2%; P.0001). Estimated postoperative survival was 83.5% ± 0.7% at 1 year, 63.6% ± 0.9% at 5 years, 36.8% ± 1.0% at 10 years, and 18.3% ± 0.9% at 15 years.Repairing thoracoabdominal aortic aneurysms poses substantial risks, particularly when the entire thoracoabdominal aorta (extent II) is replaced. Nonetheless, our data suggest that thoracoabdominal aortic aneurysm repair, when performed at an experienced center, can produce respectable outcomes.

Background We examined our contemporary experience with hemiarch and total arch replacement in patients with previous acute type I aortic dissection. Methods Over an 8.5-year period, 137 consecutive patients (median age 58 years, interquartile range, 50 to 67) underwent hemiarch or total transverse aortic arch replacement a median of 7.7 years (range, 67 days to 32 years; interquartile range, 2.8 to 12.3 years) after previous acute type I aortic dissection repair. Interventions involving only the aortic root, aortic valve, descending aorta, or thoracoabdominal aorta were excluded. Multivariate analysis of 20 potential preoperative and intraoperative risk factors was performed to examine early death, neurologic deficit, composite endpoint (operative death, permanent neurologic deficit, or hemodialysis at discharge), and long-term mortality. Results Total arch replacement was performed in 103 patients (75.2%), hemiarch replacement in 34 (24.8%), and elephant trunk procedures in 77 (56.2%). Thirty-one repairs (22.6%) were emergent or urgent. There were 16 operative deaths (11.7%), 4 permanent strokes (3.6%), and 21 (15.3%) instances of the composite endpoint. In the multivariate analysis, congestive heart failure and cardiopulmonary bypass time independently predicted operative mortality ( p = 0.0027, p = 0.018). Emergency operation approached significance for stroke ( p = 0.088). Predictors of long-term mortality (during a median follow-up period of 5.1 years, 95% confidence interval: 4.4 to 5.8) were female sex ( p = 0.0036), congestive heart failure ( p = 0.0045), and circulatory arrest time ( p = 0.0013); preoperative pulmonary disease approached significance ( p = 0.074). Five-year survival was 73.2%. Conclusions In patients with previous acute type I aortic dissection repair, hemiarch and total arch operations have respectable morbidity and survival rates. Congestive heart failure predicts operative death, long-term mortality, and our adverse event endpoint. Cardiopulmonary bypass time predicts operative mortality, and female sex and circulatory arrest time predict long-term mortality.

Background We attempted to determine the true incidence of retrograde ascending aortic dissection (rAAD) and to challenge its reported association with distal aortic dissection or zone 0 deployment. Methods From January 2005 to August 2014, 305 patients who were at risk for rAAD underwent thoracic endovascular aortic repair. We excluded patients with prior ascending, hemiarch, or traditional or frozen elephant trunk grafts, or who required concomitant ascending graft placement. Patients in group A (n = 111, 36.4%) had distal aortic dissection or hematoma (n = 75, 67.6%) or required landing of the endograft in zone 0 of the native ascending aorta (n = 36, 32.4%). Patients in group B (n = 194, 63.6%) had nondissected descending or distal arch aneurysm (n = 172), penetrating ulcer (n = 9), coarctation (n = 6), endoleak not caused by dissection (n = 3), aortobronchial fistula (n = 3), or transection (n = 1). Results The incidence of rAAD was 1.3% overall (n = 4), 0.9% in group A (n = 1, Cook Zenith TX2), and 1.5% in group B (n = 3; 1 Talent Captivia, 2 Cook Zenith TX2; p = 0.64). No zone 0–treated patient had rAAD. Two patients from group B died, and 1 was treated nonoperatively. The median interval between thoracic endovascular aortic repair and rAAD was 11 days (range, 0 to 90 days). Conclusions Post–thoracic endovascular aortic repair rAAD is a rare but lethal complication. Operator experience is crucial for prompt recognition and prevention. It does not appear that rAAD is specifically associated with distal aortic dissection or landing in zone 0. To our knowledge, this is one of the few studies to report the true incidence of rAAD in at-risk patients.

Left ventricular aneurysm, which can impair systolic function, has a reported incidence of 10% to 35% in patients after myocardial infarction. In a 58-year-old woman who had a history of myocardial infarction, we excised a large left ventricular aneurysm and restored left ventricular geometry with use of a bovine pericardial patch. The aneurysm's characteristics and the patient's preoperative left ventricular ejection fraction of 0.25 had indicated surgical intervention. The patient had an uneventful postoperative course, and her left ventricular ejection fraction was 0.50 to 0.55 on the 4th postoperative day. This case illustrates the value of surgical treatment for patients who have a debilitating left ventricular aneurysm.

Background We evaluated the occurrence and treatment of aortic aneurysms in coarctation patients. Methods During 1962 to 2011, 943 cases of coarctation were repaired. Aortic aneurysms were identified in 55 patients (5.8%). Forty-eight had prior coarctation repair (median 23 years earlier, interquartile range 18 to 26 years). Forty-two aneurysms were found in the descending thoracic aorta (76.4%), 18 in the ascending aorta (32.7%), 8 in the left subclavian artery (14.5%), and 1 each (1.8%) in the abdominal aorta, iliac artery, and innominate artery. Twenty-three patients (41.8%) had multiple aneurysms. Twenty-five patients (45.4%) had a bicuspid aortic valve. Results Fifty-three patients' aneurysms were treated surgically. Thirty-five (66.0%) had descending thoracic aortic repair, of whom 11 had aorto-left subclavian bypass. Aortic cross-clamping alone was used in 23 patients, left heart bypass in 4, and circulatory arrest in 8. Eleven patients underwent endovascular repair (20.8%). Proximal aortic aneurysms were repaired in 7 patients (13.2%); 1 had simultaneous antegrade endostent delivery. Four patients had ascending-to-descending aortic bypass (7.3%). Concomitant valve-sparing root repair was performed in 2 patients, Bentall in 4, aortic valve replacement in 3, and coronary artery bypass in 1. One 30-day death occurred (1.9%). Three patients (5.7%) had transient neurologic deficits, 2 (3.8%) required tracheostomy, and 11 (20.8%) had vocal cord paralysis. Conclusions Coarctation is a marker for aortic aneurysm formation in adults and merits long-term surveillance. Anatomic complexity and associated conditions can complicate the surgical repair. Various open, extra-anatomic, and endovascular techniques may be used.

Objective To evaluate adverse outcomes after elective aortic arch surgery performed at higher or lower temperatures (24.0°C-28.0°C vs 20.1°C-23.9°C) within the wide range of moderate hypothermia. Methods Over a 9-year period, a total of 665 patients underwent elective proximal (n = 479) or total (n = 186) arch replacement with moderate hypothermia and antegrade cerebral perfusion. Circulatory arrest was initiated at an actual temperature of 20.1°C to 23.9°C in the lower-temperature group (n = 334; 223 proximal, 111 total) and at 24.0°C to 28.0°C in the higher-temperature group (n = 331; 256 proximal, 75 total). Composite adverse outcome was defined as operative mortality or persistent neurologic event or persistent hemodialysis at discharge. Multivariate logistic regression analysis was used to model adverse outcome. In addition to the actual temperature, a new, balanced variable, "predicted temperature," was analyzed to eliminate surgeon bias. We used this variable in a propensity score–matching analysis to validate the multivariate analysis results. Results A composite adverse outcome occurred in 7.2% of cases. Operative mortality was 5.1%. The rate of postoperative persistent neurologic deficits was 2.4%. No significant differences were found between the lower– and higher–predicted temperature groups within the moderate hypothermia range in the propensity score–matching analysis. The higher–actual temperature group had a lower rate of ventilator support at >48 hours ( P = .036) and less need for tracheostomy ( P = .023). Packed red blood cell transfusion and previous coronary artery bypass independently predicted composite adverse outcome ( P = .0053 and .0002, respectively), operative mortality ( P = .0051 and .0041), and postoperative stroke ( P = .045 and .048). Cardiopulmonary bypass time independently predicted composite outcome ( P = .0005), operative mortality ( P 48 hours ( P P = .0005). Conclusions In elective proximal or total arch surgery, higher temperatures (≥24.0°C-28.0°C) within the wide range of moderate hypothermia (20.1°C-28°C) are safe and, compared with colder temperatures, not associated with significantly different rates of composite and adverse outcomes.

Objectif L’impact contemporain et les indications d’un pontage carotido-sous-clavier (PCS) sont les considerations essentielles dans la decision d’une revascularisation brachio-cephalique. Methodes Nous avons analyse les resultats operatoires, la permeabilite a long terme et l’impact epidemiologique etendu de la pathologie primaire traitee chez 287 patients consecutifs (âge moyen, 60,6 ans ; 43,2% d’hommes) operes d’un PCS pour une pathologie brachio-cephalique symptomatique. Resultats Un succes technique a ete obtenu chez tous les patients. La mortalite operatoire etait de 1% (3/287) et le taux d’accident vasculaire cerebral total (homolateral [1,4%, 4/287] plus controlateral [0,7%, 2/287]) etait de 2,1% (6/287). Les taux de permeabilite primaire a 5, 10 et 15 ans etaient respectivement de 94,2 ± 1,9%, 88,6 ± 3,2% et 86,5 ± 3,8%, respectivement. Les taux d’indemnite d’evenement specifique a 15 ans selon la methode Kaplan–Meier etait les suivants : restenose, 86,5 ± 3,8% ; deces, 67,5 ± 5,2% ; revascularisation coronaire, 59,6 ± 6,3% ; infarctus du myocarde, 82,8 ± 3,9% ; accident vasculaire cerebral, 85,6 ± 4,9% ; autre procedure vasculaire, 60,0 ± 5,5% ; evenement cardiaque indesirable (deces, infarctus du myocarde, revascularisation coronaire), 44,5 ± 5,5% et evenement vasculaire indesirable (restenose, accident vasculaire cerebral ou nouvelle procedure vasculaire), 48,7 ± 5,3%. Conclusions Le PCS fournit une excellente permeabilite a long terme et une disparition durable des symptomes avec de faibles et acceptables taux de morbidite et de mortalite operatoires. Malgre la durabilite et l’efficacite du PCS, le processus primaire pathologique a un effet indesirable sur le pronostic a long terme et influence significativement la prise de decision concernant la prise en charge. La durabilite prouvee pourrait permettre la disparition durable des symptomes chez des patients relativement plus jeunes, davantage de survie associee a une preservation de la perfusion de l’artere mammaire interne chez les patients a risque de revascularisation myocardique, une durabilite optimale chez les patients necessitant une procedure ouverte concomitante et une preservation de la fonction du membre chez des patients necessitant la mise en place d’une endoprothese aortique.

Background The contemporary impact of and indications for carotid–subclavian bypass (CSB) are essential considerations in decision making for brachiocephalic reconstruction. Methods We analyzed operative outcomes, long-term graft patency, and the extended epidemiological impact of the primary disease process in 287 consecutive patients (mean age, 60.6 years; 43.2% male) who received CSB for symptomatic brachiocephalic disease. Results Technical success was achieved in each patient. Operative mortality was 1.0% (3/287) and total (ipsilateral [1.4%, 4/287] plus contralateral [0.7%, 2/287]) stroke rate was 2.1% (6/287). Primary patency rates at 5, 10, and 15 years were 94.2 ± 1.9%, 88.6 ± 3.2%, and 86.5 ± 3.8%, respectively. Kaplan–Meier freedom from specific events at 15 years was as follows: restenosis, 86.5 ± 3.8%; death, 67.5 ± 5.2%; coronary revascularization, 59.6 ± 6.3%; myocardial infarction, 82.8 ± 3.9%; stroke, 85.6 ± 4.9%; other vascular procedure, 60.0 ± 5.5%; adverse cardiac outcome (death, myocardial infarction, or coronary revascularization), 44.5 ± 5.5%; and adverse vascular outcome (restenosis, stroke, or other vascular procedure), 48.7 ± 5.3%. Conclusions CSB produces excellent long-term patency and extended symptom relief, with acceptably low operative morbidity and mortality. Despite the durability and success of CSB, the primary disease process has an adverse impact on long-term prognosis and significantly influences decision making with regard to management. The proven durability may offer extended symptom relief to the relatively younger patient, a survival advantage associated with preservation of internal mammary artery perfusion in patients at risk for myocardial revascularization, optimal durability in patients requiring a concomitant open procedure, and preservation of limb function in patients who require aortic endovascular graft placement.

Background Dosing of recombinant activated factor VII (rFVIIa) is controversial and unstandardized, and there is growing concern about thromboembolic complications, especially in left ventricular assist device (LVAD)-supported patients. We reviewed our experience with rFVIIa administration in patients with LVADs and examined its effectiveness and adverse effects, including the incidence of thromboembolic events and its correlation with increasing doses. Methods We retrospectively reviewed the records of 62 patients who received an LVAD and rFVIIa at our center between January 2004 and November 2006. Patients who received a single dose of 10 to 20 μg/kg ( n = 32; 52%) constituted the "low-dose" cohort, and patients who received 30 to 70 μg/kg ( n = 30; 48%) constituted the "high-dose" cohort. Laboratory values obtained before and after rFVIIa administration, as well as patients' transfusion requirements, were compared to determine the effectiveness of rFVIIa in reversing coagulopathy and reducing blood loss. We also compared the incidence of thromboembolic events in the low- and high-dose groups. Results Administration of rFVIIa was associated with significant decreases in prothrombin time, activated partial thromboplastin time and transfusion requirements. This association was seen in both the low- and high-dose groups. In addition, the incidence of thromboembolic events was significantly higher in the high-dose group (36.7%) than in the low-dose group (9.4%) ( p ≤ 0.001). Conclusions Although rFVIIa administration seemed helpful in controlling life-threatening hemorrhage, patients requiring higher doses (30 to 70 μg/kg) had a dramatically higher incidence of serious thromboembolic events.

The direct surgical treatment of various forms of aortic and arterial disease often requires a vascular replacement. Homografts were first employed successfully for this purpose and both technically and functionally have provided gratifying results. Their major disadvantage, however, lies in the inconvenience associated with their procurement and preparation, and the fact that they are not available in sufficient quantities to meet the increasing demands for their use. For these reasons attention has been directed toward development of a satisfactory arterial substitute for homografts which would be free of these disadvantages. In cooperation with Prof. Thomas Edman, of the Philadelphia Textile Institute, a new flexible knitted Dacron tube has been devised which on the basis of experimental and clinical studies has been found to meet this objective. Among its desirable characteristics are the following: First, it may be repeatedly sterilized by autoclaving in the usual manner without harm to or weakening

Objective We attempted to identify predictors of adverse outcomes after traditional open and hybrid zone 0 total aortic arch replacement. Methods We performed multivariable analysis using 16 variables to identify predictors of adverse outcomes (mortality, permanent neurologic events, and permanent renal failure necessitating hemodialysis) in 319 consecutive patients who underwent total aortic arch replacement in the past 8.5 years and a subgroup analysis in 25 propensity-matched pairs. A total of 274 patients (85.9%) had traditional open repair, and 45 patients (14.1%) had hybrid zone 0 total arch exclusion. Results Operative mortality was 10.3% (n = 33): 11.1% (n = 5) in the hybrid group and 10.2% (n = 28) in the traditional group ( P = .79). A total of 19 patients (5.9%) had permanent stroke (15 traditional [5.5%] vs 4 hybrid [8.9%]; P = .32), and 2 patients (both traditional) had permanent paraplegia ( P = 1.00). The hybrid group had more total neurologic events ( P = .051) but not more permanent strokes ( P = .32). Prior cardiac disease unrelated to the aorta ( P = .0033) and congestive heart failure ( P = .0053) independently predicted permanent adverse outcome (operative mortality, permanent neurologic event, or permanent renal failure). Concomitant coronary artery bypass grafting independently predicted permanent stroke ( P = .032), as did previous cerebrovascular disease ( P = .032). In multivariable analysis, procedure type (hybrid or traditional) was not an independent predictor of stroke ( P = .09). During a median follow-up of 4.5 years (95% confidence interval, 3.9-4.9), survival was 78.7%, with no intergroup difference ( P = .14). Conclusions Among contemporary cases, both traditional and hybrid total aortic arch replacement had acceptable results. Comparing these 2 different surgical treatment options is challenging, and an individualized approach offers the best results. Permanent adverse outcome was not significantly different between the 2 groups. Procedure type is not an independent predictor of permanent stroke. Prior cardiac disease, past or current smoking, and congestive heart failure predict adverse outcomes for total aortic arch replacement.

Objective Although the surgical management of brachiocephalic disease is well established, evolving endovascular techniques present new options for treatment. We explored the potential benefits and drawbacks of these interventions in terms of outcome. Methods From 1966 to 2004, 391 consecutive patients (43.7% male; mean age, 61.9 years) with single-vessel brachiocephalic disease were treated with either operative bypass (group A; n=229) or percutaneous transluminal angioplasty and stenting (group B; n=162). Results All patients were asymptomatic after surgery or endovascular intervention. Group A and group B had similar operative mortality (0.9% vs 0.6%) and stroke (1.3% vs 0%) rates. However, 5 years after the procedure, group A had significantly better freedom from graft or intervention failure (92.7% ± 2.1%) than did group B (83.9% ± 3.7%; P = .03, Kaplan-Meier analysis; P = .001, Cox regression analysis). At 10 years, group A had the following rates of actuarial freedom from specific events: death, 73.7% ± 4.6%; myocardial infarction, 84.2% ± 3.6%; stroke, 91.4% ± 3.4%; graft failure, 88.1% ± 3.3%; coronary revascularization, 69.8% ± 5.1%; and other vascular operation, 70.7% ± 4.6%. Endovascular intervention involved less initial cost (mean savings, $8787 per procedure), was less invasive, and did not necessitate general anesthesia. On satisfaction questionnaires, 96.5% of patients receiving an endovascular intervention and 95.1% of patients receiving operative bypass for single-vessel brachiocephalic disease subjectively rated their treatment as "good" or "very good." Conclusions Operative bypass and endovascular intervention for single-vessel brachiocephalic disease are both associated with acceptably low operative morbidity and mortality. Operative bypass produces significantly better mid-term freedom from graft or intervention failure than endovascular intervention and produces excellent long-term freedom from failure. Endovascular intervention offers tangible benefits regarding cost, level of invasiveness, and subjective patient satisfaction. Undetermined are the differences between the procedures regarding long-term durability, patterns of failure, efficacy as an adjunct to coronary artery bypass grafting, need for anticoagulation, efficacy as treatment for complex (multivessel) disease, and long-term cost.

For centuries, aortic disease has been a major problem of mankind. Hieroglyphic texts from ancient Egypt attest to the presence of aortic aneurysms millennia before the birth of Christ. Although no medical solution for aneurysms was available in that era, the advice allegedly given for the care of affected patients was a passionate plea, which simply stated, “Do not abandon them.” As medical history reveals, the treatment of aortic aneurysms did not progress significantly until modern times. Most measures used to control these lesions were attempts to relieve pain and forestall inevitable rupture. Efforts to promote thrombosis by introducing foreign bodies, such as wire and other substances, were sometimes successful but more often ineffective. Other efforts were aimed at strengthening or reinforcing the wall of the aneurysm to retard its growth. Many different measures were tried, but their success was limited. A major advance took place in 1888, when Rudolph Matas reported an internal repair technique known as endoaneurysmorrhaphy for treating arterial lesions. In this approach, he excised the clot from the aneurysmal sac and sutured the orifices of the arteries that entered the sac, from within, reestablishing continuous blood flow. More aggressive or definitive treatment became available only in the second half of the 20th century. Encouraged by experience with the surgical treatment of wounds during World War II, surgeons began taking a more direct approach to cardiovascular lesions. A few case reports concerning the repair of small sacciform aneurysms appeared in the medical literature, but there were no recommendations for definitive surgery. In December 1951, I presented a paper that Dr. Michael DeBakey and I had written entitled, “Surgical Considerations of Intrathoracic Aneurysms of the Aorta and Great Vessels,” [1] at a meeting of the Southern Surgical Association (Fig. 1). The paper described six cases in which excision and aortorrhaphy had been performed. During the discussion that followed the presentation, the renowned surgeon Dr. Evarts Graham complimented us on our paper and suggested that it was a landmark in vascular surgery. Shortly afterward, I performed the first repair of a ruptured abdominal aneurysm [2]; the patient survived for 5 days, then died of kidney failure. I soon repaired four more ruptured aneurysms. In 1952, Dr. DeBakey and I were the first to use a homograft to repair a large aneurysm of the thoracoabdominal aorta [3]. Later, we successfully used homografts to replace the ascending aorta (1956) [4] and the aortic arch (1957) [5]. Figure 1. A leather-bound copy of the historic and seminal paper that I presented to the Southern Surgical Association in 1951, which is now prominently displayed in the Wallace D. Wilson Museum at the Texas Heart Institute. I am proud to have participated in the ... During the same period, many other surgeons made important contributions to aneurysm treatment. Among those pioneers were Henry Bahnson and his colleague, Frank Spencer, who began an active aortic surgery program at Johns Hopkins Hospital. Soon, many other surgeons and institutions established programs for treating aortic aneurysms and occlusive disease. Since that time, aortic disease has continued to hold widespread interest for surgeons and other specialists. Countless symposia and publications have resulted. Many advances in diagnosis and treatment have occurred, such as aortography, the introduction of vascular grafts and anticoagulants, and the techniques of cardiopulmonary bypass and induced hypothermia. More recently, noninvasive diagnostic methods have been introduced, including ultrasonography, computed tomography, and MRI. In the last part of the 20th century, the goal of treatment reverted to endoaneurysmorrhaphy, with use of a suitable graft to restore aortic continuity. Today, the emphasis is on catheter placement of prosthetic grafts, a less invasive approach to these lesions. It is highly appropriate now that Dr. John Elefteriades, of the Yale University School of Medicine, has created a specialty journal, AORTA, devoted entirely to aortic disease. Dr. Elefteriades has long been a world-renowned leader in cardiovascular surgery. I applaud this new journal and am sure that it will contain many articles of interest. Comment on this Article or Ask a Question

Techniques and technology for treating coronary artery disease (CAD) have evolved significantly over the past 40 years. Although there are cases in which the best treatment approach (medical, surgical, or catheter-based) is clear, the best choice of intervention is not obvious for the majority of patients. Five, 7, and 10 years after initial treatment, mortality is lower for coronary artery bypass grafting (CABG) than for medical treatment alone. These survival benefits of CABG are particularly substantial for patients with three-vessel or left main artery disease, severe angina, or a positive pretreatment exercise stress test. Most trials comparing CABG with catheter-based interventions (CBIs) in patients with multivessel disease have shown similar short-term survival benefits for both treatments, but CBIs have been associated with less periprocedural morbidity and CABG with longer-lasting revascularization. Substantial evidence suggests that CABG is preferable to CBI in certain types of patients, including the one third of CAD patients who have chronic total occlusion of one or more vessels, and those with diabetes mellitus. Meta-analyses of large numbers of CABG studies report 30-day mortality rates in the 1% to 2% range. Numerous CABG techniques have evolved that seem to produce equally good results. Substantial evidence suggests that internal mammary artery grafts more effectively promote short- and longterm survival than do saphenous vein grafts, especially when used to graft the left anterior descending (LAD) coronary artery. In fact, the bulk of the evidence suggests that CABG with left internal mammary artery (LIMA)-to-LAD grafting provides better long-term results than does either CABG with other types of conduits or CBI. Technical innovations made in the attempt to reduce the negative impact of cardiopulmonary bypass (CPB) include coating the internal surface with heparin or other polymers to reduce contact between blood and the CPB mechanism, minimizing hemodilution by shrinking the bypass circuit to reduce the amount of crystalloid prime needed to start CPB, and using hemofiltration to protect the heart and end organs. Current United States estimates (based on industry reports) suggest that 18% to 25% of the 370,000 annual CABG procedures are performed without the use of CPB. There is not yet complete agreement about the relative safety and efficacy of off-pump coronary artery bypass (OPCAB) and conventional CABG with CPB. The difficulty in comparing the OPCAB and standard CABG stems in part from the low incidences of mortality and significant morbidity associated with both techniques. A meta-analysis of 37 randomized, controlled trials of OPCAB suggests that it is associated with significantly decreased transfusion and inotrope requirements, atrial fibrillation, respiratory infections, ventilation time, intensive care unit stay, hospital stay, and overall in-hospital and (1-year) postdischarge costs compared with standard CABG. Data from many large nonrandomized studies suggest that OPCAB is associated with a lower incidence of death, stroke, intraaortic balloon pump requirement, and postoperative transfusion, and a shorter average time on ventilator and length of hospital stay compared with conventional CABG. However, selection bias cannot be ruled out as a potential confounding variable in these data. Devices and techniques for lateral and inferior wall exposure have enabled surgeons performing OPCAB to attach grafts to all aspects of the heart. Recent series show no difference in the average number of grafts constructed in OPCAB and standard CABG, and several series have shown excellent graft patency. Off-pump bypass is ideal for patients with significant atheroma or calcification of the ascending aorta. A few studies have shown that OPCAB is associated with a reduced incidence of neurocognitive dysfunction compared with conventional CABG with CPB, but other studies have shown no significant differences. Results of small randomized trials suggest that, in patients with stenosis of the proximal LAD, minimally invasive direct coronary artery bypass (MIDCAB) is associated with a longer in-hospital recovery period and higher costs than is direct stenting, but that MIDCAB produces similar or better short- and long-term outcomes. Totally videoscopic LIMA-to-LAD procedures have been performed in which surgical robots were used to mobilize the LIMA and make the sutured anastomosis, but robots are not likely to be in widespread use for CABG in the near future because of their expense and the challenges involved in learning to use them. Although port-access CPB has been used successfully in multivessel CABG performed through small fifth intercostal space thoracotomies, this procedure has fallen out of favor, partly because of the difficulty of constructing grafts of appropriate length and lie.

Objectives To determine whether innominate artery cannulation is the ideal perfusion strategy for delivering antegrade cerebral perfusion (ACP) during surgery on the proximal ascending aorta and transverse aortic arch. Methods A total of 263 patients underwent innominate artery cannulation with a side graft for surgery on the proximal aorta. Operations performed were ascending and proximal arch replacement (n = 213, 81.0%), ascending and total arch replacement (n = 33, 12.6%) and ascending aortic replacement (n = 12, 4.6%). Concomitant or other procedures included aortic root replacement and repair (n = 113, 43.0%), aortic valve replacement or repair (n = 118, 44.9%), coronary artery bypass (n = 40, 15.2%), antegrade stent graft delivery to the proximal descending thoracic aorta for aneurysm or dissection (n = 28, 10.7%), mitral valve repair (n = 11, 4.2%), patent foramen ovale repair (n = 3, 1.1%) and tricuspid valve repair (n = 2, 0.8%). Twenty-seven patients (10.3%) presented with acute or subacute Type I aortic dissection, and 45 (17.1%) had a previous sternotomy. Median cardiopulmonary bypass (CPB), cardiac ischaemia and ACP times were 126.0 [95-163 interquartile range (IQR)], 91.0 (73-121 IQR) and 21.0 (16-32 IQR) min. Bilateral ACP was delivered in 235 patients (90.7%). Results The operative mortality rate was 4.9% (n = 13). Nine patients (3.4%) had postoperative stroke, which was permanent in 5 (1.9%) of them. Multivariate analysis associated risk of stroke or temporary neurological deficit with acute or subacute Type I aortic dissection (P = 0.028) and age (P = 0.015). Renal disease (P = 0.036) and CPB time (P = 0.011) were independent risk factors for operative mortality. Circulatory arrest time was identified as a risk factor for mortality (P = 0.038). Conclusions Innominate artery cannulation can be performed safely and poses a low risk of neurological events in procedures requiring hypothermic circulatory arrest. The technique for cannulating this artery should be part of the routine armamentarium of cardiac and aortic surgeons, and the innominate artery is among the preferred perfusion sites for delivering ACP.

Objectives To determine the preoperative and perioperative risk factors that significantly predict adverse outcomes after total arch replacement in patients with previous proximal aortic surgery and to analyze patient survival. Methods We performed univariate analysis and logistic regression on data extracted from a prospectively maintained database for 119 patients who had undergone total arch operations during a 7.5-year period. All patients had undergone previous proximal aortic surgery. The adverse outcome was defined as a single composite endpoint comprising operative mortality, permanent neurologic deficit, and renal failure necessitating permanent hemodialysis. Results The incidence of the composite endpoint was 13.5% (16 of 119 patients). The univariate predictors were preoperative pulmonary disease ( P = .010), cardiac ischemia time ( P = .032), and cardiopulmonary bypass (CPB) time ( P = .073). On multivariate analysis, the following were predictors of the composite endpoint: preoperative pulmonary disease ( P = .036), CPB time ( P = .039), concomitant coronary artery bypass ( P = .0057), previous aortic valve replacement ( P = .027), and previous thoracoabdominal aortic aneurysm surgery ( P = .057). Multivariate analysis showed that the CPB time predicted mortality ( P = .0044), and previous thoracoabdominal aortic aneurysm surgery predicted stroke ( P = .034). The overall survival was 85.3% during a median follow-up of 4.76 years (95% confidence interval, 4.2-5.1). Conclusions Aortic arch reoperations, although technically demanding, can produce acceptable results. Preoperative pulmonary disease, CPB time, and concomitant coronary artery bypass predicted an adverse outcome. The CPB time predicted mortality, and previous thoracoabdominal aortic surgery predicted stroke.

Background . Over the past four decades, the surgical trend has been toward early, complete repair of tetralogy of Fallot (TOF). Many centers currently promote all neonates for total correction irrespective of anatomy and symptoms, with some surgeons advocating hypothermic circulatory arrest for repair in small infants. We believe this approach increases morbidity. Methods . Based on approximately 40 years' experience in 2,175 patients, we developed a management protocol focused on patient size, systemic arterial saturations, and anatomy. Symptomatic patients (hypercyanotic spells, ductal dependent pulmonary circulation) weighing less than 4 kg undergo palliative modified Blalock–Taussig shunt (BTS) followed by complete repair at 6 to 12 months. Asymptomatic patients, weighing less than 4 kg who have threatened pulmonary artery isolation, undergo BTS and repair at 6 to 12 months. All other patients undergo complete repair after 6 months. Results . From July 1, 1995, to December 1, 1999, 144 patients underwent operation for TOF (129 patients) or TOF with atrioventricular septal defect (TOF/AVSD, 15 patients). Ninety-four patients underwent one stage complete repair (88 TOF, 6 TOF/AVSD). Thirty-nine patients underwent repair after initial BTS (32 TOF, 7 TOF/AVSD). Ten patients are awaiting repair after BTS. The mean age and weight at complete repair were 18 months and 9 kg. There were no operative deaths. There have been 3 late deaths with complete follow-up (mortality 3 of 144 [2.1%]). Four of 133 patients (3%) have required reoperation after total correction. Conclusions . This management strategy optimizes outcomes by individualizing the operation to the patient. Advantages include avoidance of circulatory arrest, low morbidity and mortality, and low incidence of reoperation after complete repair.

Objective: To determine the efficacy of a single-clamp technique in preventing spinal cord ischemia during repair of aneurysms of the descending thoracic aorta. Patients and methods: From January 1989 to May 1999, 132 consecutive patients (91 men and 41 women, aged 31‐86 years), with aneurysms of the descending thoracic aorta underwent repair using a single-clamp technique and temporary partial distal exsanguination. The diseased aortic segment was replaced with a Dacron graft. Blood was re-infused from an auto-transfusion device, and the segmental vessels were over-sewn but not implanted into the graft. Results: The average aortic cross-clamp time was 26.4 min (range, 11‐67 min) for the overall group and 37.4 min for patients who had spinal cord complications. An average of 2066 ml of blood was autotransfused (range, 450‐6100 ml). During the first 30 postoperative days, 17 patients (12.9 %) died. Eleven patients (8.3%) had spinal cord dysfunction, six patients (4.5%) had lower-extremity paraparesis, and five patients (3.8%) had paraplegia. Nine patients (6.8%) had renal failure necessitating hemodialysis. Other complications included bleeding in 15 cases (11.4%), respiratory failure in 12 cases (9.1%), woundrelated sequelae in five cases (3.8%), distal embolism in five cases (3.8%), and bowel ischemia in two cases (1.5%). Conclusion: The singleclamp technique yielded an acceptable complication rate, and the mortality was comparable to that seen after the use of more complex methods. For satisfactory results, the cross-clamp time should not exceed 30 min. q 2000 Elsevier Science B.V. All rights reserved.

The development of infected pseudoaneurysms (PAs) following carotid endarterectomy (CEA) is extremely rare. We retrospectively reviewed the data from 13 such aneurysms (in 12 patients) repaired over a period of 35 years at the Texas Heart Institute (THI) and included an overview of published cases to analyze the epidemiology, mode of presentation, methods of repair, and outcome. The diagnosis of PA should be considered when a patient develops a persistent hematoma, recurrent bleeding from the wound, or late wound infection. Sepsis is occasionally a presenting symptom. Surgical therapy for infected PAs can be challenging; excision of the aneurysm followed by autologous grafting constitutes the favored approach. Traditionally, Dacron patch repair has been associated with a high incidence of reinfection. Carotid artery ligation is required in a large percentage of cases (22.7%) and is associated with a prohibitive rate of death/major stroke (50%) compared with a low (12%) risk following arterial reconstruction. Prevention and early diagnosis of infected PAs are essential to limit complications and mortality.

Background . We have used a variety of techniques to correct left ventricular outflow tract obstructions, including, in the past, placement of an apicoaortic valved conduit to bypass the outflow tract. Because the operation was technically difficult, it had fallen into disuse. Recently, we used a simplified transthoracic approach to implant apicoaortic conduits in 7 patients with complex lesions of the left ventricular outflow tract. Methods . The thoracic cavity was entered through the fifth intercostal space in all 7 patients. The distal end of the valve-containing conduit was attached to the aorta with continuous 3-0 or 4-0 polypropylene sutures after incising the pleura over the distal descending aorta. The pericardium was opened to expose the left ventricular apex, which was cored so that the proximal end of the conduit could be inserted into the left ventricular cavity. Results . Five of the patients recovered completely. The 2 patients who died had severe heart disease and multiple comorbidities. Conclusions . The transthoracic approach gives direct access to the descending aorta and avoids a redo sternotomy. The technique, which is simple to perform, does not compromise major coronary arteries, the conduction system, or other valves; and may be useful in patients who are not good candidates for other, more conventional procedures.

Background . Few large or long-term series exist regarding the management of patients with sinus of Valsalva aneurysms or fistulas (SVAFs). Methods . Between 1956 and 1997, 129 patients presented with a ruptured (64 cases; 49.6%) or nonruptured (65 cases; 50.4%) SVAF. The patients included 88 men and 41 women, with a mean age of 39.1 years. Associated findings included a history of endocarditis (42 cases; 32.6%), a bicuspid aortic valve (21 cases; 16.3%), a ventricular septal defect (15 cases; 11.6%), and Marfan's syndrome (12 cases; 9.3%). Operative procedures included simple plication (61 cases; 47.3%), patch repair (52 cases; 40.3%), aortic root replacement (16 cases; 12.4%), and aortic valve replacement/repair (75 cases; 58.1%). Results . There were five in-hospital deaths (3.9%): four due to preexisting sepsis and endocarditis and one that followed dehiscence of the repair in a patient with Marfan's syndrome. Two patients (1.6%) had strokes during the early postoperative period. The survivors were followed up for 661.1 patient-years (5.3 years/patient). The following late complications occurred: prosthetic valve malfunction (5 cases; 3.9%), prosthetic valve endocarditis (3 cases; 2.3%), SVAF recurrence (2 cases; 1.6%), thrombosis (1 case; 0.8%), and anticoagulation-related bleeding (1 case; 0.8%). Conclusions . Resection and repair of SVAF entails an acceptably low operative risk and yields long-term freedom from symptoms. Early, aggressive treatment is recommended to prevent endocarditis or lesional enlargement, which causes worse symptoms and necessitates more extensive repair.

In this report, the authors review the presentations, angiographic findings, and outcomes of eight cases involving patients who had atherosclerotic compromise of the proximal internal carotid artery and were developing distal thrombosis. They discuss the pathogenesis and implications of such lesions and review the management strategy used at this institution. Between 1994 and 1995, 606 consecutive patients underwent carotid endarterectomy (CEA) at this institution. Among this group, four consecutive patients (0.66%, 4/606) were identified by angiography to have preocclusive carotid artery thrombosis. An additional four patients with the same radiologic finding who had presented between 1980 and 1993 were also identified and included in this series. All eight patients were treated with anticoagulation and emergent CEA and thrombectomy. Complete resolution of presenting symptoms and freedom from perioperative stroke and death were achieved in seven patients (88%, 7/8). In one patient (12%, 1/8) the perioperative course was complicated by development of a carotid artery-cavernous sinus fistula and stroke. The catastrophic potential of preocclusive carotid artery thrombosis mandates immediate intervention. Anticoagulation, CEA, and thrombectomy can result in freedom from the adverse outcomes of stroke and death. However, special care must accompany thrombectomy in order to avoid complications and their associated morbidity.

BACKGROUND: There is controversy regarding the application of transfusion triggers in cardiac surgery. The goal of this study was to determine if lowering the hemoglobin threshold for red cell (RBC) transfusion to 8 g per dL after coronary artery bypass graft surgery would reduce blood use without adversely affecting patient outcome. STUDY DESIGN AND METHODS: Consecutive patients (n = 428) undergoing elective primary coronary artery bypass graft surgery were randomly assigned to two groups: study patients (n = 212) received RBC transfusions in the postoperative period if the Hb level was

Effective methods to treat aortic aneurysms are now available, although these lesions still challenge the cardiovascular surgeon. Attempts at treatment began in earnest in the 1800s, with the introduction of indirect and direct methods of repair. A major breakthrough occurred in the late 1800s, when Dr Rudolph Matas devised a method for internal repair of aneurysms in which continuity of blood flow was restored by excising the diseased portion of the lesion and creating a tunnel through the remaining normal portion. Matas named this technique reconstructive endoaneurysmorrhaphy. Until that time, surgeons had treated aneurysms by ligating the parent vessel with a Hunterian ligature or introducing foreign material to promote coagulation. Ligating the aneurysm rendered the extremities vulnerable to ischemic damage, however, and results were unpredictable with the use of various foreign materials. Around the turn of the century, Carrel began experimenting with different techniques for vascular anastomoses. The work of these early pioneers formed the basis for much of the modern treatment of aneurysms of the thoracic aorta. My experience began in 1950, when I excised a large aortic aneurysm in one of Dr Alfred Blalock's patients. The patient survived and was cured. After that experience, I knew that aortic aneurysms could be treated successfully by aggressive surgical means. Treatment has changed, however, from the early emphasis on excising the lesion to the present practice of restoring circulatory continuity with a suitable graft, ie, endoaneurysmorrhaphy. The development of reliable synthetic grafts has been one of the most important advances in the treatment of aneurysms. The surgical technique used today depends on the anatomic location of the aneurysm, which can occur anywhere from the aortic annulus and aortic valve to the distal thoracic aorta and visceral vessels in the abdomen.

Replacement of the aortic root with a prosthetic graft and valve in patients with Marfan's syndrome may prevent premature death from rupture of an aneurysm or aortic dissection. We reviewed the results of this surgical procedure at 10 experienced surgical centers.A total of 675 patients with Marfan's syndrome underwent replacement of the aortic root. Survival and morbidity-free survival curves were calculated, and risk factors were determined from a multivariable regression analysis.The 30-day mortality rate was 1.5 percent among the 455 patients who underwent elective repair, 2.6 percent among the 117 patients who underwent urgent repair (within 7 days after a surgical consultation), and 11.7 percent among the 103 patients who underwent emergency repair (within 24 hours after a surgical consultation). Of the 675 patients, 202 (30 percent) had aortic dissection involving the ascending aorta. Forty-six percent of the 158 adult patients with aortic dissection and a documented aortic diameter had an aneurysm with a diameter of 6.5 cm or less. There were 114 late deaths (more than 30 days after surgery); dissection or rupture of the residual aorta (22 patients) and arrhythmia (21 patients) were the principal causes of late death. The risk of death was greatest within the first 60 days after surgery, then rapidly decreased to a constant level by the end of the first year.Elective aortic-root replacement has a low operative mortality. In contrast, emergency repair, usually for acute aortic dissection, is associated with a much higher early mortality. Because nearly half the adult patients with aortic dissection had an aortic-root diameter of 6.5 cm or less at the time of operation, it may be prudent to undertake prophylactic repair of aortic aneurysms in patients with Marfan's syndrome when the diameter of the aorta is well below that size.

We used transmyocardial laser revascularization to treat accelerated cardiac allograft atherosclerosis in 2 patients. One patient received transmyocardial laser revascularization as sole therapy, the other as an adjunct to coronary artery bypass grafting. The systolic function improved in both patients, although the patient who had adjunctive transmyocardial laser revascularization died of systemic infection and renal failure on postoperative day 55. The second patient is alive and well 112 years after the laser procedure. We discuss 4 other patients who received transmyocardial laser revascularization treatment elsewhere in the United States. Transmyocardial laser revascularization has the potential to become important in the treatment of transplant atherosclerosis. Randomized clinical trials are warranted to assess the efficacy of transmyocardial laser revascularization in this setting.

Postinfarction ventricular septal rupture is an uncommon but serious complication of acute myocardial infarction. Although I have tried various methods for repairing postinfarction septal defects, I recently began to use an exclusion technique, which is an extension of the intracavitary repair method that I use to treat ventricular aneurysms. This exclusion technique yields satisfactory results (early mortality rate, 36.4%), even in patients in severe cardiogenic shock.

Background . Although an increasing number of elderly and high-risk patients, including those with generalized atherosclerosis, are undergoing coronary revascularization, few reports exist regarding the management of patients who have both occlusive disease of the great vessels and coronary artery disease. Methods . Between 1972 and 1996, 31 consecutive patients (mean age, 56.5 years; 74% men) with multivessel coronary artery disease and symptomatic occlusive disease of the great vessels (25 single-vessel, 80.6%; 6 multiple-vessel, 19.4%) had 40 great vessels reconstructed by transthoracic bypass (n = 17, 42.5%), transthoracic endarterectomy (n = 8, 20%), or extrathoracic bypass (n = 15, 37.5%). All patients had simultaneous coronary artery bypass grafting (mean, 2.6 grafts per patient), and 8 patients had 10 distal carotid bifurcation endarterectomies (6 staged, 4 simultaneous). Results . The early primary patency rate was 100%, and symptoms resolved completely in all 31 patients. There was 1 in-hospital death (3.2%) in a patient who had a respiratory arrest 11 days after operation. Perioperative morbidity included two myocardial infarctions (6.5%) and one opposite-hemisphere, embolic stroke (3.2%). Long-term follow-up of the 30 survivors (167.4 patient-years; mean, 5.6 years per patient) documented 5- and 10-year actuarial survival rates of 88.6% and 60.4%, respectively, with a 100% late brachiocephalic primary patency rate. Ten-year actuarial rates of freedom from the following events were as follows: death, 60.4%; myocardial infarction, 82.5%; stroke, 90.9%; percutaneous transluminal coronary angioplasty or redo coronary artery bypass grafting, 95.2%; and vascular operation or amputation, 78.4%. Conclusions . Depending on the anatomic distribution of the disease, an integrated approach to great vessel reconstruction that incorporated transthoracic and extrathoracic approaches and techniques of endarterectomy and bypass resulted in few adverse outcomes and excellent long-term patency. Simultaneous revascularization of the great vessels and coronary arteries can produce immediate and long-term, symptom-free outcome with acceptably low operative risk.

Ischemic injury of the spinal cord remains a serious threat during the surgical treatment of aneurysms of the descending and thoracoabdominal aorta. Time is the critical factor in preventing spinal cord injury. The author uses a single-clamp technique that is simple and limits distal dissection, heparin dosage, and cross-clamp time, thus reducing the risk of paraplegia.

OBJECTIVE: For patients with genetically triggered thoracic aortic disease, the morbidity and mortality associated with reoperation are high, making endovascular treatment an appealing option. We evaluated the short- and mid-term outcomes of different applications of endovascular intervention in such patients. METHODS: Between January 2003 and April 2013, 60 patients received endovascular or hybrid treatment for genetically triggered thoracic aortic disease. The inclusion criteria were based on those devised by the National Registry of Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions. We included patients with thoracic aneurysm or dissection not due to trauma in a patient aged ≤50 years (n= 30), bicuspid aortic valve (BAV) and coarctation (n= 11), Marfan syndrome (n= 10), BAV with thoracic aneurysm (n= 4), Loeys–Dietz syndrome (n= 3), familial thoracic aneurysm or dissection (n= 3) and genetic mutations (n= 2). Some patients met more than one inclusion criterion. Forty-one (68.3%) patients were treated with only endovascular stent grafting. Nineteen (31.7%) patients underwent a hybrid procedure with open proximal or total arch replacement and concomitant endovascular stenting of the aortic arch or the descending thoracic aorta. Twenty-nine (48.3%) had previous cardiovascular operations (mean ± SD, 1.9 ± 1.4) before undergoing hybrid or endovascular therapy. The median follow-up was 2.3 years (interquartile interval 25–75%, 1.4–4.6 years). RESULTS: The technical success rate was 100%. In-hospital mortality was 3.3% (n= 2) and neurological events occurred in 2 patients; 1 (1.6%) had a stroke and 1 (1.6%) suffered paraparesis with partial recovery. Fifteen repeat open or endovascular interventions were required in 10 surviving patients (17.2%). Overall survival during follow-up was 94.8% (55/58). CONCLUSIONS: Endovascular technology can be helpful in treating selected young patients with genetically triggered thoracic aortic disease. Long-term studies and further evolution of endovascular technology will be necessary for it to be incorporated into the armamentarium of surgical options for this challenging patient population.