Surgery for Ascending Aortic Aneurysm and Aortic Valve Insufficiency in Conditions of Active Proceeding Syphilitic Aortitis and Valvulitis.

A syphilitic aortitis is a late cardiovascular lesion of tertiary syphilis that has become exceptionally rare in the antibiotic era but not eradicated completely. Syphilitic aortitis of ascending aorta complicates in ascending aortic aneurysm formation and aortic valve regurgitation, both requiring surgical treatment. After surgery, lifelong surveillance of the remainder of the aorta is recommended because of a priori supposed high incidence of delayed involvement of noninvolved aortic segments. A 3-year follow-up result of surgery of syphilitic ascending aortic aneurysm with aortic valve regurgitation in condition of active ongoing syphilitic aortitis and valvulitis is described with addressing the dimensions of remaining aortic segments. This case demonstrates that the dilatation of the remainder of the aorta does not occur during 3 years, at least when anti-syphilitic course of antibiotic is used just after operation without additional treatment during the follow-up period. A few reports on surgical treatment of syphilitic aneurysms of the ascending aorta are discussed.

Risk factor analysis for aortic dissection after aortic valve replacement in patients with tricuspid aortic valve.

OBJECTIVE: Aortic dilatation concurrent with aortic valve disease is a common condition. However, the incidence of aortic dissection after aortic valve replacement for tricuspid aortic valve has not been fully investigated. Therefore, we performed a risk factor analysis for the incidence of aortic dissection after aortic valve replacement in patients with tricuspid aortic valve. METHODS: We retrospectively reviewed 599 patients who underwent aortic valve replacement at our hospital between January 2000 and December 2020. We performed a risk factor analysis for the incidence of aortic dissection after aortic valve replacement in patients with tricuspid aortic valve. RESULTS: Seven patients developed late aortic dissections during the follow-up period. All patients with aortic dissection underwent aortic valve replacement for aortic regurgitation. Multivariable analysis revealed that aortic regurgitation was an independent predictor of aortic dissection (p < 0.0001). The mean ascending aortic diameter at aortic valve replacement for aortic regurgitation was significantly greater in patients with aortic dissection than in those without aortic dissection (46 [43.5-46] mm vs. 39 [36-42] mm, p < 0.001). The predictive cutoff value of ascending aortic diameter was indicated using receiver operating characteristic curve analysis; 46.0 mm (area under the curve: 0.8987). Freedom rates from aortic dissection in patients with aortic regurgitation and an ascending aortic diameter ≥ 46 mm were significantly lower than those in patients with an ascending aortic diameter < 46 mm (66.7% vs. 100% at 5 years, p < 0.0001). CONCLUSION: Aortic regurgitation combined with ascending aortic dilatation at aortic valve replacement could be a significant risk factor for late aortic dissection.

Shared decision-making and specific informed consent in patients with aortic aneurysms.

OBJECTIVE: To analyze the refusal rate of elective aortic aneurysm surgery in asymptomatic patients after the presentation of a detailed informed consent form followed by a meeting where patient and their families can analyze each item. METHODS: We conducted a retrospective analysis of 49 patients who had aneurysms and were offered surgical treatment between June 2017 and February 2019. The patients were divided into two groups: the Rejected Surgery Group, which was composed of patients who refused the proposed surgical treatment, and the Accepted Surgery Group, comprising patients who accepted the proposed surgeries and subsequently underwent them. RESULTS: Of the 49 patients, 13 (26.5%) refused surgery after reading the informed consent and attending the comprehensive meeting. We observed that patients who refused surgery had statistically smaller aneurysms than those who accepted surgery (9% versus 26%). These smaller aneurysms were above the indication size, according to the literature. CONCLUSION: One-quarter of patients who were indicated for elective surgical repair of aortic aneurysms rejected surgery after shared decision-making, which involved presenting patients with an informed consent form followed by a clarification meeting for them and their families to analyze each item. The only factor that significantly influenced a rejection of the procedure was the size of the aneurysm; patients who rejected surgery had smaller aneurysms than those who accepted surgery. Up to 26% of patients with aortic aneurysms refused surgical repair. The proposed technique, whether open or endovascular, did not influence patients' decisions. Patients with smaller aneurysms were more likely to refuse aortic aneurysm treatment.

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.

Risk Factors Associated with Spinal Cord Ischemia During Aortic Aneurysm Repair.

BACKGROUND: The risk of spinal cord ischemia (SCI) with aortic aneurysm repair can cause significant neurological morbidity. Prevention of SCI is critical. We sought to identify risk factors that predispose to SCI that may guide strategies to mitigate the occurrence of SCI during and following these procedures. METHODS: This study includes all adults who underwent atraumatic, unruptured, thoracic, and suprarenal aortic aneurysm repairs (endovascular or open) at our institution over 11 years (2010-2020). Our database included patient demographics, aneurysm anatomic features, and operative characteristics and an extreme gradient boost (XGB) machine method was used to develop a predictive model for SCI. The model was trained on an 80% randomly stratified cohort of the data and tested on the remaining 20% testing cohort. Shapley values were used to determine the most important predictive factors of SCI and decision trees were used to identify risk factor threshold values and highest risk factor combinations. RESULTS: Information was collected for 174 adult patients undergoing thoracic and suprarenal aortic repair from 2010 to 2020. Fifty eight percent of the patients were male. Ninety seven (55.7%) patients had open aortic repair and 87 (44.3%) had endovascular repair. Twenty seven (15%) of all patients had major complications and were considered to have SCI. The XGB model converged over the training cohort with a testing cohort accuracy of 0.841 [Sensitivity = 75%, Specificity = 68%] and area under the curve of receiver operating characteristic of 0.774. The XGB model identified older age (> 65 years), history of neurologic disease, hyperlipidemia, diabetes, coronary artery disease, heart failure, poor renal function, < 6 months since last aortic repair, chronic anticoagulant use, preoperational anemia (Hemoglobin < 9), thrombocytopenia (platelet < 90,000), coagulopathy (prothrombin time > 15s and activated partial thromboplastin time > 40s), hypotension (mean arterial pressure < 70 mm Hg), longer operations (> 100 min), aneurysms longer than 5 cm, and anatomic location of aneurysm caudal to T-11 as risk factors for SCI in all types of aortic repair. Diabetic and heart failure patients undergoing longer operations (> 100 min) with thrombocytopenia or aneurysms longer than 5 cm were at the highest risk. CONCLUSIONS: The XGB model accurately identified risk factors of SCI with aortic aneurysm repair that may guide patient selection, timing of surgery, and strategies to minimize the risk of SCI.

Smoking Cessation and Mortality from Aortic Dissection and Aneurysm: Findings from the Japan Collaborative Cohort (JACC) Study.

AIMS: Active cigarette smoking was intensively reported to increase the risk of aortic mortality while research on the association between smoking cessation and aortic mortality remains scarce. This study aimed to reconfirm the associations of exposure to cigarettes and smoking cessation associated with aortic mortality in a large Japanese population. METHODS: In the Japan Collaborative Cohort (JACC) Study, 91,141 residents (57±10 years; men, 43%) who were free of stroke, coronary heart disease, and cancer were followed up from 1989-90 until 2009 during which 110 deaths from aortic dissection and 112 deaths from aneurysm were identified. Cox proportional hazard model was used to estimate multivariable hazard ratios (95%CI) for total and specific aortic mortality. RESULTS: Compared to never smoking, HRs for total aortic mortality were 2.39 (1.40-4.08) for ＜20, 3.57 (2.19-5.83) for 20-39, and 3.92 (2.37-6.48) for ≥ 40 pack-years exposure. Compared to current smoking, HRs for total aortic mortality were 0.42 (0.18-0.97) for 10-15 years, 0.27 (0.11-0.66) for ＞15 years of cessation, and 0.24 (0.13-0.44) for never smoking. Similar inverse dose-response pattern was observed between smoking cessation duration and risk of mortality from aortic aneurysm (p for trend=0.001), but the association with aortic dissection mortality did not reach statistical significance. CONCLUSIONS: Cigarette smoking was associated with an increased risk of aortic mortality while smoking cessation was so with a reduced risk among the Japanese population.

Aneurisma aórtico e insuficiencia valvular severa por sífilis terciaria en paciente joven, una enfermedad desatendida / Aortic aneurysm and severe valvular insufficiency due to tertiary syphilis in a young patient, a neglected disease / Aneurisma da aorta e insuficiência valvular grave devido à sífilis terciária em um paciente jovem, uma doença negligenciada

La sífilis es causada por Treponema pallidum, es reconocida por la variedad de sus manifestaciones clínicas; compromete el sistema nervioso, tegumentario y cardiovascular. Las alteraciones cardiovasculares ocurren en 70% de los casos con sífilis no tratada, el 71% de las aortitis sifilíticas desarrollan aneurismas aórticos y la complicación más frecuente es la insuficiencia aórtica. El tratamiento va dirigido a la enfermedad subyacente, con manejo antibiótico para la sífilis terciaria y las complicaciones tromboembólicas y si requiere reparación quirúrgica. Presentamos un caso poco frecuente de un paciente diagnosticado de infección por el virus de la inmunodeficiencia humana y complicaciones cardiovasculares típicas de la sífilis terciaria, que precisó tratamiento médico y quirúrgico, que fue exitoso. Destacamos la pericia clínica de los profesionales de nuestra institución para diagnosticar e identificar las complicaciones cardiovasculares de la sífilis terciaria.

Syphilis is caused by Treponema pallidum and is recognized by the variety of its clinical manifestations; it involves the nervous, integumentary and cardiovascular systems. Cardiovascular alterations occur in 70% of cases with untreated syphilis, 71% of syphilitic aortitis develop aortic aneurysms and the most frequent complication is aortic insufficiency. Treatment is directed at the underlying disease, with antibiotic treatment for tertiary syphilis and thromboembolic complications and if necessary surgical repair. We present a rare case of a patient diagnosed with human immunodeficiency virus infection and cardiovascular complications typical of tertiary syphilis, who required medical and surgical treatment, which was successful. We highlight the clinical experience of our institution's professionals in the diagnosis and identification of cardiovascular complications of tertiary syphilis.

A sífilis é causada pelo Treponema pallidum e é reconhecida pela variedade de suas manifestações clínicas; ela afeta os sistemas nervoso, tegumentar e cardiovascular. As alterações cardiovasculares ocorrem em 70% dos casos de sífilis não tratada, 71% dos casos de aortite sifilítica desenvolvem aneurismas aórticos e a complicação mais frequente é a insuficiência aórtica. O tratamento é direcionado para a doença subjacente, com tratamento antibiótico para sífilis terciária e complicações tromboembólicas e, se necessário, reparo cirúrgico. Apresentamos um caso raro de um paciente diagnosticado com infecção pelo vírus da imunodeficiência humana e complicações cardiovasculares típicas da sífilis terciária, que precisou de tratamento médico e cirúrgico, que foi bem-sucedido. Apresentamos um caso raro de um paciente com diagnóstico de infecção pelo vírus da imunodeficiência humana e complicações cardiovasculares típicas da sífilis terciária, que necessitou de tratamento médico e cirúrgico, o qual foi bem-sucedido. Destacamos a experiência clínica dos profissionais de nossa instituição no diagnóstico e na identificação das complicações cardiovasculares da sífilis terciária.

Short-term rapamycin treatment increases life span and attenuates aortic aneurysm in a murine model of Marfan-Syndrome.

BACKGROUND: Marfan syndrome (MFS) is a genetic disorder leading to medial aortic degeneration and life-limiting dissections. To date, there is no causal prevention or therapy. Rapamycin is a potent and selective inhibitor of the mechanistic target of rapamycin (mTOR) protein kinase, regulating cell growth and metabolism. The mgR/mgR mice represent an accepted MFS model for studying aortic pathologies to understand the underlying molecular pathomechanisms. This study investigated whether rapamycin inhibits the development of thoracic aortic aneurysms and dissections in mgR/mgR mice. METHODS: Isolated primary aortic smooth muscle cells (mAoSMCs) from mgR/mgR mice were used for in vitro studies. Two mg kg/BW rapamycin was injected intraperitoneally daily for two weeks, beginning at 7-8 weeks of age. Mice were sacrificed 30 days post-treatment. Histopathological and immunofluorescence analyses were performed using adequate tissue specimens and techniques. Animal survival was evaluated accompanied by periodic echocardiographic examinations of the aorta. RESULTS: The protein level of the phosphorylated ribosomal protein S6 (p-RPS6), a downstream target of mTOR, was significantly increased in the aortic tissue of mgR/mgR mice. In mAoSMCs isolated from these animals, expression of mTOR, p-RPS6, tumour necrosis factor α, matrix metalloproteinase-2 and -9 was significantly suppressed by rapamycin, demonstrating its anti-inflammatory capacity. Short-term rapamycin treatment of Marfan mice was associated with delayed aneurysm formation, medial aortic elastolysis and improved survival. CONCLUSIONS: Short-term rapamycin-mediated mTOR inhibition significantly reduces aortic aneurysm formation and thus increases survival in mgR/mgR mice. Our results may offer the first causal treatment option to prevent aortic complications in MFS patients.

A case series describing the risk of periodontal disease in Marfan syndrome patients harboring a possible aortic aneurysm or dissection.

BACKGROUND: Marfan syndrome (MFS) is a systemic disorder of connective tissues caused by insufficient elastic fiber formation that leads to structural weakness and results in various tissue disorders, including cardiovascular and periodontal disease. Notably however, the risk of periodontal disease in MFS patients affected by an aortic aneurysm or dissection has not yet been clarified. METHODS: We investigated the periodontal condition in the following three groups: MFS patients diagnosed with an aortic aneurysm or dissection with a planned aortic surgery (MFS surgery), MFS patients who had already undergone aortic surgery (MFS post-surgery) and healthy control patients (Healthy). The periodontal condition of all of these patients was evaluated at their first visit, reassessed again at two-month after the first visit, and evaluated again at a six-month follow-up after the reassessment. RESULTS: A total of 14 participants, 3 MFS surgery patients, 4 MFS post-surgery patients and 7 healthy control volunteers were examined. Saliva examinations revealed no significant differences between any of the groups at the first visit, reassessment, or follow-up. Interestingly, the MFS surgery cases showed a higher BOP and PISA at the first visit and follow-up compared with the other groups. In contrast, the MFS surgery patients showed an improvement in their LVDd and EF values, both markers of cardiac function, at the reassessment and follow-up compared with the first visit. CONCLUSIONS: MFS associated with an aortic aneurysm or dissection leads to a higher risk of periodontal disease, indicating the need for more frequent oral hygiene maintenance in these patients. In addition, MFS patients who undergo frequent professional cleaning of their teeth show a lower onset of cardiovascular disease, suggesting that professional oral hygiene in these cases contributes to a healthier condition.

Aortic local biomechanical properties in ascending aortic aneurysms.

Ascending aortic aneurysm (AsAA) is a high-risk cardiovascular disease with an increased incidence over years. In this study, we compared different risk factors based on the pre-failure behavior (from a biomechanical point of view) obtained ex-vivo from an equi-biaxial tensile test. A total of 100 patients (63 ± 12 years, 72 males) with AsAA replacement, were recruited. Equi-biaxial tensile tests of AsAA walls were performed on freshly sampled aortic wall tissue after ascending aortic replacement. The aneurysmal aortic walls were divided into four quadrants (medial, anterior, lateral, and posterior) and two directions (longitudinal and circumferential) were considered. The stiffness was represented by the maximum Young modulus (MYM). Based on patient information, the following subgroups were considered: age, gender, hypertension, obesity, dyslipidemia, diabetes, smoking history, aortic insufficiency, aortic stenosis, coronary artery disease, aortic diameter and aortic valve type. In general, when the aortic diameter increased, the aortic wall became thicker. In terms of the MYM, the longitudinal direction was significantly higher than that in the circumferential direction. In the multivariant analysis, the impact factors of age (p = 0.07), smoking (p = 0.05), diabetes (p = 0.03), aortic stenosis (p = 0.02), coronary artery disease (p < 10-3), and aortic diameters (p = 0.02) were significantly influencing the MYM. There was no significant MYM difference when the patients presented arterial hypertension, dyslipidemia, obesity, or bicuspid aortic valve. To conclude, the pre-failure aortic stiffness is multi-factorial, according to our population of 100 patients with AsAA. STATEMENT OF SIGNIFICANCE: Our research on the topic of "Aortic local biomechanical properties in case of ascending aortic aneurysms" is about the biomechanical properties on one hundred aortic samples according to the aortic wall quadrants and the direction. More than ten factors and risks which may impact ascending aortic aneurysms have been studied. According to our knowledge, so far, this article involved the largest population on this topic. It will be our pleasure to share this information with all the readers.

Clinical features and prognosis of patients with and without diabetes mellitus undergoing endovascular aortic aneurysm repair.

BACKGROUND: This study aimed to compare the clinical features and prognoses of patients with and without diabetes mellitus (DM) who underwent endovascular repair for aortic aneurysm (AA). METHODS: We analyzed the clinical database of a prospective multicenter study, registering 929 patients who underwent their first endovascular AA repair in Japan between January 2016 and June 2018. The baseline characteristics and prognoses (including all-cause mortality and cardiovascular events) after repair were compared between the DM and non-DM groups. Prognoses were also compared between the groups after propensity score matching. RESULTS: In total, 226 patients (24.3%) had DM. Compared with non-DM patients, DM patients had higher pack-years of smoking (P = 0.011), higher body mass index (P = 0.009), lower high-density lipoprotein cholesterol levels (P = 0.038), higher triglyceride levels (P = 0.025), and lower left ventricular ejection fraction (P = 0.005). Meanwhile, the low-density lipoprotein cholesterol and blood pressure levels showed no significant intergroup difference (all P > 0.05). DM patients had a higher prevalence of myocardial infarction (P = 0.016), history of coronary revascularization (P = 0.015), and lower extremity artery disease (P = 0.019). Lesion characteristics and procedures were similar between the groups (all P > 0.05). DM patients had a higher risk of all-cause mortality and cardiovascular events than non-DM patients (both P < 0.001). Subsequent propensity score matching also demonstrated that DM patients had a significantly lower rate of overall survival (P = 0.001) and freedom from cardiovascular events (P = 0.010). The Kaplan-Meier estimates at 1 year for the overall survival were 85.6% (95% confidence interval [CI], 80.9% to 90.5%) and 94.3% (95% CI, 91.7% to 97.0%) for patients with and without DM, respectively. The corresponding estimates for freedom from cardiovascular events were 79.8% (95% CI, 74.5% to 85.5%) and 87.7% (95% CI, 84.2% to 91.3%), respectively. CONCLUSIONS: Among patients undergoing endovascular AA repair, those with DM had more cardiovascular risk factors. DM patients had a higher incidence rate of all-cause mortality and cardiovascular events. Matching analysis indicated that DM per se would be a risk factor for poor prognoses after AA repair.

The role of vascular smooth muscle cells in the development of aortic aneurysms and dissections.

BACKGROUND: Aortic aneurysms (AA) are pathological dilations of the aorta, associated with an overall mortality rate up to 90% in case of rupture. In addition to dilation, the aortic layers can separate by a tear within the layers, defined as aortic dissections (AD). Vascular smooth muscle cells (vSMC) are the predominant cell type within the aortic wall and dysregulation of vSMC functions contributes to AA and AD development and progression. However, since the exact underlying mechanism is poorly understood, finding potential therapeutic targets for AA and AD is challenging and surgery remains the only treatment option. METHODS: In this review, we summarize current knowledge about vSMC functions within the aortic wall and give an overview of how vSMC functions are altered in AA and AD pathogenesis, organized per anatomical location (abdominal or thoracic aorta). RESULTS: Important functions of vSMC in healthy or diseased conditions are apoptosis, phenotypic switch, extracellular matrix regeneration and degradation, proliferation and contractility. Stressors within the aortic wall, including inflammatory cell infiltration and (epi)genetic changes, modulate vSMC functions and cause disturbance of processes within vSMC, such as changes in TGF-ß signalling and regulatory RNA expression. CONCLUSION: This review underscores a central role of vSMC dysfunction in abdominal and thoracic AA and AD development and progression. Further research focused on vSMC dysfunction in the aortic wall is necessary to find potential targets for noninvasive AA and AD treatment options.

Surgical Correction of Ascending Aortic Aneurysm Without Coronary Dilatation After Kawasaki Disease in a 3-Year-Old Child.

Kawasaki disease (KD) is an inflammatory condition that may affect genetically predisposed individuals in pediatric ages after infectious/environmental triggering. An infrequent finding associated with KD is ascending aortic aneurysm during or after the acute phase of the disease. In this Multimedia presentation, we describe a three-year-old girl submitted to surgical treatment.

Computational Fluid Dynamics Simulations to Predict False Lumen Enlargement After Surgical Repair of Type-A Aortic Dissection.

We aim to use computational fluid dynamics to investigate the hemodynamic conditions that may predispose to false lumen enlargement in this patient population. Nine patients who received surgical repairs of their type-A aortic dissections between 2017-2018 were retrospectively identified. Multiple contrast-enhanced post-operative CT scans were used to construct 3D models of aortic geometries. Computational fluid dynamics simulations of the models were run on a high-performance computing cluster using SimVascular - an open-source simulation package. Physiological pulsatile flow conditions (4.9 L/min) were used at the aortic true lumen inlet, and physiological vascular resistances were applied at the distal vascular ends. Exploratory analyses showed no correlation between rate of false lumen growth and blood pressure, immediate post-op aortic diameter, or the number of fenestrations (p = 0.2). 1-year post-operative CT scans showed a median false lumen growth rate of 4.31 (3.66, 14.67) mm/year Median (Interquartile range) peak systolic, mid-diastolic, and late diastolic velocity magnitudes were 0.90 (1.40); 0.10 (0.16); and 0.06 (0.06) cm/s respectively. Spearman's ranked correlations between fenestration velocity and 1-year false lumen growth rates were found to be statistically significant: Velocity magnitude at peak systolic (p = 0.025; rho = 0.75), mid diastolic (p = 0.025; rho = 0.75) and late diastolic phases of the cardiac cycle (p = 0.006; rho = 0.85). We have shown that false lumen growth is strongly correlated to fenestration flow velocity, which has potential implications for post-operative surveillance and risk stratification.

Regional wall stress differences on tricuspid aortic valve-associated ascending aortic aneurysms.

OBJECTIVES: Ascending thoracic aortic aneurysms (aTAAs) carry a risk of acute type A dissection. Elective repair guidelines are based on diameter, but complications often occur below diameter threshold. Biomechanically, dissection can occur when wall stress exceeds wall strength. Aneurysm wall stresses may better capture dissection risk. Our aim was to investigate patient-specific aTAA wall stresses associated with a tricuspid aortic valve (TAV) by anatomic region. METHODS: Patients with aneurysm diameter ≥4.0 cm underwent computed tomography angiography. Aneurysm geometries were reconstructed and loaded to systemic pressure while taking prestress into account. Finite element analyses were conducted to obtain wall stress distributions. The 99th percentile longitudinal and circumferential stresses were determined at systole. Wall stresses between regions were compared using one-way analysis of variance with post hoc Tukey HSD for pairwise comparisons. RESULTS: Peak longitudinal wall stresses on aneurysms (n = 204) were 326 [standard deviation (SD): 61.7], 246 (SD: 63.4) and 195 (SD: 38.7) kPa in sinuses of Valsalva, sinotubular junction (STJ) and ascending aorta (AscAo), respectively, with significant differences between AscAo and both sinuses (P < 0.001) and STJ (P < 0.001). Peak circumferential wall stresses were 416 (SD: 85.1), 501 (SD: 119) and 340 (SD: 57.6) kPa for sinuses, STJ and AscAo, respectively, with significant differences between AscAo and both sinuses (P < 0.001) and STJ (P < 0.001). CONCLUSIONS: Circumferential and longitudinal wall stresses were greater in the aortic root than AscAo on aneurysm patients with a TAV. Aneurysm wall stress magnitudes and distribution relative to respective regional wall strength could improve understanding of aortic regions at greater risk of dissection in a particular patient.

Hybrid repair of aberrant right subclavian artery with aortic dissection caused by Kommerell diverticulum.

BACKGROUND: Aberrant right subclavian artery (ARSA) with associated Kommerell diverticulum (KD) is a rare congenital aortic disease. KD patients have a high risk of rupture, dissection, and compression of adjacent structures. Although several treatment options have been proposed (traditional surgery, hybrid operation, and endovascular intervention), a consensus regarding optimal surgical management has not yet been established. CASE PRESENTATION: A case of successful hybrid repair of distal aortic arch dissection aneurysm by dissecting KD and ARSA with debranching of right and left common carotid arteries, left subclavian artery, and stent grafting was presented. CONCLUSIONS: The hybrid operation is suitable for elderly patients or those with high risks. Along with intervention, the hybrid operation needs to be developed as a minimally invasive method.