Acute Type A Aortic Dissection and Late Pregnancy: What Should We Do?

ABSTRACT Introduction: Acute type A aortic dissection (AAAD) in late pregnancy is a rare but severe disease. Lack of clinical experience is the main cause of high mortality. This study tries to investigate the multidisciplinary therapeutic strategy for these patients. Case presentation: We reported three patients with AAAD in late pregnancy. Sudden chest pain was the main clinical symptom before operation. All three patients and their newborns survived through multidisciplinary approach in diagnosis and treatment. No serious complications occurred during the mid-term follow-up. Conclusion: Multidisciplinary diagnosis and treatment strategy play a crucial role in saving the lives of pregnant women with AAAD.

Redo Total Aortic Arch Replacement in Patients with Aortic Dissection After Open-Heart Surgery and Long-Term Follow-Up Results

ABSTRACT Introduction: The objectives of this study were to investigate the main treatment strategies and long-term follow-up results of aortic dissection surgery after open-heart surgery (ADSOHS) and to analyze the risk factors that cause ADSOHS. Methods: One hundred thirty-seven patients with ADSOHS hospitalized in our hospital from January 2009 to December 2018 were selected as the research object. Long-term follow-up results, complications, mortality, and changes of cardiac function before and after operation were used to explore the value of Sun's operation. Results: The length of stay in intensive care unit of these 137 patients ranged from 9.5 to 623.75 hours (average of 76.41±97.29 hours), auxiliary ventilation time ranged from 6.0 to 259.83 hours (average of 46.16±55.59 hours), and hospital stay ranged from six to 85 days (average of 25.06±13.04 days). There were seven cases of postoperative low cardiac output, 18 cases of coma and stroke, and six cases of transient neurological dysfunction. A total of 33 patients died; 19 patients died during the perioperative period, 18 died during Sun's operation and one died during other operation; and 14 patients died during follow-up (January 2021), 12 cases of Sun's operation and two cases of other operations. Conclusion: ADSOHS treatment strategy is of high application value, and the risk of neurological complications and mortality is low. The main risk factors are postoperative low cardiac output, coma, stroke, and transient neurological dysfunction. The extracorporeal circulation time is relatively long. Short- and long-term follow-up effects are good, and it is worthy of clinical promotion.

Innominate Artery Cannulation for Proximal Aortic Surgery

ABSTRACT Introduction: The aim of this study was to evaluate the efficacy and safety of innominate artery cannulation strategy with side-graft technique in proximal aortic pathologies. Methods: A total of 70 patients underwent innominate artery cannulation with a side graft for surgery on the proximal aorta from 2012 to 2020. There were 46 men and 24 women with an average age of 56±13 years. The indications for surgery were type A aortic dissection in 17 patients (24.3%), aortic aneurysm in 52 patients (74.3%), and ascending aorta pseudoaneurysm in one patient (1.4%). The innominate artery was free of disease in all patients. Hypothermic circulatory arrest with antegrade cerebral perfusion was utilized in 60 patients (85.7%). Three patients had previous sternotomy (4.2%). The most common surgical procedure was ascending aorta with hemiarch replacement in 34 patients (48.5%). Results: The mean cardiac ischemia and cardiopulmonary bypass times were 116+46 minutes and 164+56 minutes, respectively. Mean antegrade cerebral perfusion time was 27+14 minutes. The patients were cooled between 22°C and 30°C during surgery. Thirty-day mortality rate was 7.1% (five patients). One patient (1.4%) had stroke, one patient (1.4%) had temporary neurologic deficit, and eight patients (11.4%) had confusion and agitation that resolved completely in all cases. There was no local complication or arterial injury. Conclusion: Cannulation of the innominate artery with side graft is safe and effective for both cardiopulmonary bypass and antegrade cerebral perfusion. This technique provides satisfactory neurologic outcomes for proximal aortic surgery.

Lower Body Perfusion Reduces the Morbidity of Postoperative Acute Kidney Injury in Type A Dissection: A Propensity-Matched Analysis

ABSTRACT Introduction: Lower body perfusion (LBP) is a technique used to provide blood perfusion to distal organs and spinal cord during circulatory arrest. However, the effect of LBP on the prognosis of aortic arch surgery, especially on postoperative renal function, remains unclear. Methods: A total of 304 patients with acute type A aortic dissection who underwent total aortic arch replacement combined with frozen elephant trunk implantation between May 2016 and December 2021 were retrospectively analyzed. The patients were divided into LBP group (group L, n=85) and non-LBP group (group NL, n=219). Routine lower body circulatory arrest was applied during operation in group NL, and antegrade LBP combined was applied during operation in group L. Perioperative data were recorded. Propensity score matching was used for statistical analysis. Results: After propensity score matching, 85 pairs of patients were successfully matched. Two groups significantly differed in circulatory arrest time (six minutes vs. 30 minutes, P=0.000), cross-clamping time (101 minutes vs. 92 minutes, P=0.010), minimum nasopharyngeal temperature (29.4ºC vs. 27.2ºC, P=0.000), and highest lactate value during cardiopulmonary bypass (2.3 μmol/L vs. 4.1 μmol/L, P=0.000). Considering the postoperative indicators, the drainage volume (450 mL vs. 775 mL, P=0.000) and the incidence of level I acute kidney injury (23.5% vs. 32%, P=0.046) in group L was lower than those in group NL. Conclusion: LBP resulted as a safe and feasible approach in aortic arch surgery, as it could significantly shorten the circulatory arrest time, which might reduce the incidence of postoperative level I acute kidney injury.

Clinical characteristics and outcome of aortic dissection in nine pregnant women with pre-eclampsia / 中华围产医学杂志

Objective:To investigate the clinical characteristics, management and outcome of aortic dissection (AD) in pregnant women with pre-eclampsia.Methods:Clinical characteristics, management, and prognosis of nine patients with pre-eclampsia complicated by AD who were admitted to the First Affiliated Hospital of Zhengzhou University from January 2013 to April 2021 were retrospectively summarized using descriptive statistical analysis.Results:(1) Clinical features: The incidence of pre-eclampsia complicated by AD among the total deliveries and pre-eclampsia deliveries were 18/100 000 (9/49 166) and 182/100 000 (9/4 943), respectively. All the nine cases of pre-eclampsia complicated by AD, with an average age of (36.6±4.2) years, presented clinical symptoms in the second or the third trimester that were mainly manifested as chest and back pain (8/9) and the average gestational week at onset was (31.0±4.7) weeks. Seven cases were confirmed by transthoracic echocardiography (TTE) and computed tomography angiography (CTA), one by TTE and magnetic resonance angiography, and one by TTE+MRI+CTA. The main classification of AD was Stanford B (8/9). In the grading of blood pressure during pregnancy, except for one case without data, eight cases were severe. (2) Management: Four women did not receive any antihypertensive treatment. However, blood pressure remained uncontrolled in the other five cases despite antihypertensive managment. Except for one death before operation due to rapid deterioration, the other eight cases all received initial treatment and thoracic endovascular aortic repair (TEVAR). Four cases underwent TEVAR prior to the termination of pregnancy. Two cases were delivered by cesarean section first, and TEVAR was performed 5 and 6 days after delivery, respectively. The other two cases undenwent cesarean section and TEVAR at the same time. Lung infection ( n=6) was the main postoperative complication. (3) Delivery: Among the nine patients, one patient died, and the average termination time of the eight patients (six underwent cesarean section, including two cases with fetal death, two underwent vaginal delivery following fetal death) was (32.9±3.6) weeks and none of them had postpartum hemorrhage. (4) Maternal-infant outcomes: Follow-up of the eight surviving women showed that one had numbness in one side of the limbs, one with occasional heart palpitations, and the rest had good outcomes. There were four live births, including two with low birth weight and one with neonatal asphyxia, and five stillbirths. During the 7-83 months of follow-up, all four children grew well. Conclusions:Pregnant women with pre-eclampsia should actively control blood pressure to avoid AD. Once symptoms such as chest and back pain occur, the possibility of AD should be considered. Active treatment after diagnosis of AD can improve maternal and fetal survival rate.

Research progress on characteristics of aortic dissection related metabolomics / 中华全科医师杂志

Aortic dissection (AD) is a life-threatening vascular disease due to the tearing of aortic intimal layer, leading to the formation of pseudocavity. Once the acute progression of dissection happens, serious complications such as rupture and stroke may occur. The current imaging examinations for AD are invasive and may cause adverse effects related to contrast medium, which cannot be used for large-scale screening of AD. The latest studies have found that metabolic processes and metabolites of lipids,saccarides and proteins are involved in the pathogenesis and development of AD. In this article, we review the research progress in the caracteristics of AD related metabolism,summarize changes of specific metabolites in AD,and explore the clinical implication of studies on AD related metaboliome..

Analysis of risk factors associated with acute Stanford type B aortic dissection complicated with pleural effusion and observation of the curative effect after intracavitary repair / 中华内科杂志

Objective:To investigate the risk factors of acute Stanford type B aortic dissection (TBAD) complicated with pleural effusion (PE) and the short-term and long-term outcomes of thoracic endovascular aortic repair (TEVAR).Methods:A case-control study. The clinical and imaging data of 1 083 patients with acute TBAD admitted to the General Hospital of Northern Theater Command from April 2002 to December 2020 were retrospectively analyzed, including 211 cases with pleural effusion and 872 cases without pleural effusion. The baseline analysis of the two groups of patients was performed. The risk factors associated with pleural effusion were analyzed by binary logistic regression, and the results were expressed as odds ratio ( OR) and 95% confidence interval ( CI). According to the quantity of pleural effusion, they were simultaneously divided into small pleural effusion group and medium large pleural effusion group, to compare the short-term and long-term effects of TEVAR patients with different amounts of pleural effusion. Results:The incidence of pericardial effusion (17.5% vs. 3.8%, P<0.001), anemia (21.3% vs. 12.5%, P=0.001), aortic spiral tear (49.8% vs. 37.8%, P=0.002), dissection tear over diaphragm (57.8% vs. 48.1%, P=0.011), serum creatinine [85 (69, 111) vs. 81 (67, 100) μmol/L, P=0.011] and white blood cell levels[(11.3±4.2)×10 9/L vs. (10.3±4.2)×10 9/L, P=0.002] in acute TBAD pleural effusion group were significantly higher than those in non-pleural effusion group, and the hemoglobin level was significantly lower than that in non-pleural effusion group [(128±20) vs. (133±17) g/L, P<0.05]. Logistic stepwise regression analysis showed that pericardial effusion ( OR=5.038,95% CI 2.962-8.568, P<0.001), anemia ( OR=2.047,95% CI 1.361-3.079, P=0.001), spiral tear ( OR=1.551,95% CI 1.030-2.336 , P=0.002) and elevated white blood cell ( OR=1.059,95% CI 1.011-1.102, P=0.005) were independent risk factors for TBAD complicated with pleural effusion. The incidences of all-cause death (4/19 vs. 1.5% vs. 0.9%, P<0.001), aortogenic death (4/19 vs. 0.7% vs. 0.7%, P<0.001) and aortic related adverse events (4/19 vs. 1.5% vs. 1.1%, P<0.001) in patients with large pleural effusion during TEVAR operation were significantly higher than those in patients with small pleural effusion and those without pleural effusion, and the differences were statistically significant. At 1 month follow-up after TEVAR, the incidence of all-cause death (4/16 vs. 3.3% vs. 1.6%, P<0.001), aortogenic death (4/16 vs. 0.8% vs.0.7%, P<0.001), aorta related adverse events (4/16 vs. 4.1% vs. 4.7%, P=0.013) and overall clinical adverse events (4/16 vs.9.8% vs. 6.7%, P=0.014) in the medium and large thoracic group were significantly higher than those in the small pleural effusion group and no pleural effusion group, and the differences were statistically significant. At 1 year follow-up after TEVAR, the incidence of all-cause death (4/15 vs. 4.9% vs. 3.9%, P=0.004), aortogenic death (4/15 vs.2.5% vs. 2.1%, P<0.001), aorta related adverse events (5/15 vs. 11.5% vs. 9.4%, P=0.012) and overall clinical adverse events (5/15 vs. 18.9% vs. 13.1%, P=0.029) in the medium and large thoracic group were significantly higher than those in the small pleural effusion group and no pleural effusion group, and the differences were statistically significant. Conclusions:Single center data showed that pericardial effusion, anemia, spiral tear and elevated white blood cell were independent risk factors for acute TBAD complicated with pleural effusion; the early (1 month) and long-term (1 year) rates of all-cause death, aortic mortality, aortic adverse events and overall clinical adverse events were significantly higher in TBAD patients with moderate pleural effusion after TEVAR, and moderate and large pleural effusion was an independent risk factor for near and long-term aortic related adverse events after TEVAR surgery.

Efficacy of goal-directed analgesia/sedation for improvement in preoperative management of patients with aortic dissection / 中华麻醉学杂志

Objective:To evaluate the efficacy of goal-directed analgesia/sedation for improvement in the preoperative management of the patients with aortic dissection.Methods:One hundred and ten patients of either sex, aged≥18 yr, diagnosed with arterial dissection by aortic CTA in our hospital, were divided into 2 groups ( n=55 each) using a random number table method: conventional group and goal-directed analgesia/sedation group. Routine preoperative management was performed in both groups. Fentanyl 0.13 μg/min was intravenously infused, and the infusion rate of fentanyl was adjusted to maintain the numerical rating scale (NRS) score at 0-3 at rest in conventional group. Midazolam 0.02 mg·kg -1·h -1 and fentanyl 0.13 μg/min were intravenously infused, and the infusion rates of midazolam and fentanyl were adjusted to maintain Richmond agitation-sedation score at -2 to 0 and NRS score at rest 0-3 in goal-directed analgesia/sedation group. Nicardipine was intravenously injected and the administration rate was adjusted to maintain systolic blood pressure at 100-120 mmHg, and metoprolol was taken orally to maintain the heart rate 60-70 beats/min. The time to reach the target blood pressure and consumption of fentanyl and nicardipine within 24 h were recorded, and the occurrence of drug-related adverse reactions during analgesia and sedation and perioperative death were recorded. Results:Compared with conventional group, the time to reach the target blood pressure was significantly shortened, and the consumption of fentanyl and nicardipine within 24 h was decreased in goal-directed analgesia/sedation group ( P<0.05). No adverse reactions or perioperative death was observed in two groups. Conclusions:Goal-directed analgesia/sedation (Richmond Agitation-Sedation Scale score -2-0, NRS score at rest 0-3) is helpful in controlling blood pressure and heart rate, thus improving the quality of preoperative management of patients with aortic dissection.

Application value of the aortic dissection detection risk score plus D-dimer for the early diagnosis of acute aortic dissection / 中国基层医药

Objective:To investigate the application value of aortic dissection detection risk score (ADD-RS) combined with D-dimer (DD) in the early diagnosis of acute aortic dissection (AAD).Methods:The clinical data of 70 patients with suspected aortic dissection detection admitted to The Second Hospital of Jiaxing from August 2019 to April 2020 were collected. All patients were scored using the ADD-RS, and grouped according to the scoring results. The sensitivity and specificity of ADD-RS plus DD in the early diagnosis of AAD were calculated. The areas under the receiver operating characteristic (ROC) curves that were plotted for drADD-RS plus DD versus DD alone to screen AAD were compared to evaluate efficacy. Results:CT angiography results showed that among 70 patients with suspected AAD, 29 patients had AAD and 41 patients had no AAD. A total of 21 patients were scored 0, 41 patients were scored > 1, and 8 patients were scored > 0. ADD-RS > 0 had an overall sensitivity of 79.31% and a specificity of 36.59% for AAD diagnosis. DD test results had an overall sensitivity of 86.20% and a specificity of 36.50% for AAD diagnosis. The area under the ROC curve of ADD-RS = 0 plus DD-negative result and the area under the ROC curve of DD-negative result alone in ruling out AAD were 0.885 with 95% CI (0.786-0.949) and 0.787 with 95% CI (0.673-0.876), respectively. The difference between the two groups was statistically significant ( P = 0.024). Conclusion:Compared with DD-negative result alone, the ADD-RS = 0 plus DD-negative result strategy offers greater specificity to rule out AAD. The combined strategy has a greater efficacy in ruling out AAD. However, a multi-center study involving a large sample is required for in-depth evaluation.

Analysis of therapeutic effect on the spontaneous isolated superior mesenteric artery dissection with endovascular therapy / 国际外科学杂志

Objective:To discuss the clinical effect of endovascular treatment of 15 patients with spontaneous isolated superior mesenteric artery dissection(SISMAD).Methods:The clinical data of 15 patients with SISMAD treated with endovascular stent in Beijing Friendship Hospital Affiliated to Capital Medical University from January 2016 to July 2022 were collected and analyzed. The white blood cell, neutrophil percentage (NEUT%) and D-Dimer at admission, day 1 and day 3 after operation were analyzed and compared retrospectively. The time of abdominal pain at admission, YOO classification, angle from superior mesenteric artery to abdominal aorta(ASA), conservative treatment time, operation time, type and length of stent, vascular remodeling rate and long-term patency rate of stent were analyzed. The measurement data conforming to normal distribution was expressed as mean ± standard deviation ( ± s), t-test was used for comparison between the two groups. Measurement data of skewed distribution were expressed as M ( Q1, Q3), and non-parametric test was used for comparison between groups. Count data were expressed as rate or component ratio(%). The same variable was compared at different time points by repeated measurement data analysis of variance. Results:There were significant differences in white blood cell, NEUT% and D-Dimer between admission and postoperative day 3 in 15 patients. The time to admission for abdominal pain was 24.0(15.0, 78.0) hours. IVS type accounted for 46.7% of YOO type, and the ASA beyond 60° accounted for 66.7%. The conservative treatment time was (34.0±8.6) hours, moreover, the operation time was(153.0±37.8) min. Besides, self-expanding bare stent accounted for 85% of the stent types, moreover, the length of the stents beyond 60 cm was 50%. All patients were followed up for more than 24 months, and the vascular remodeling rate was(89.7±9.7)%.Conclusion:Endovascular self-expanding thin-wall stent placement has better vascular remodeling rate and patency rate for patients with IVS type.

Aortic Root and Distal Arch Management During Type A Aortic Dissection Repair: Expanding Horizons

ABSTRACT The management of Type A aortic dissection has evolved over a period of a decade or so, and contemporary reports are suggesting a paradigm shift from a conservative approach to complete excision of the diseased aorta including root and distal arch. Improved cardiopulmonary bypass perfusion techniques, better understanding of the cerebral perfusion, and wide-ranging obtainability of prosthetic conduits gave surgical teams numerous choices. With improving outcomes and maturing surgical techniques, surgeons are performing extensive resections of the diseased aorta, but there is no standard protocol as far as the extent of the proximal and distal diseased aortic tissue resection is concerned. Aortic root replacement is associated with good early- and long-term outcomes and proffered solution in young and stable patients, for that reason many busy centres are endorsing total arch replacement in complex distal aortic dissections. This systemic review is discussing contemporary literature and associated pros and cons during surgical decision-making for these high-risk cases.

Follow-up Results of Endovascular Aneurysm Repair Following Abdominal Visceral Debranching

ABSTRACT Introduction: The aim of this study is to present a series of six cases with thoracoabdominal aneurysm treated with hybrid technique in our center. Methods: Between May 2015 and December 2018, the data of six patients with thoracoabdominal aneurysms and various comorbidities who underwent visceral debranching followed by endovascular aortic aneurysm repair were reviewed retrospectively. Results: Patients' mean age was 65.3±19.6 years. All of them were male. Comorbidities were old age, congestive heart failure, coronary artery disease, chronic obstructive pulmonary disease, previous surgical interventions, and/or esophageal hemangioma. Except for one patient who underwent coronary artery bypass grafting (inflow was taken from ascending aorta), debranching was performed from the right iliac artery. Debranching of four visceral arteries (superior mesenteric artery, celiac trunk, and bilateral renal right arteries) was performed in three patients, of three visceral arteries (superior mesenteric artery, celiac trunk, right renal artery) was performed in one, and of two visceral arteries (superior mesenteric artery, celiac trunk) was performed in two patients. Great saphenous vein and 6-mm polytetrafluoroethylene grafts were used in one and five patients, respectively, for debranching. Endovascular aneurysm repair was performed following debranching procedures as soon as the patients were stabilized. In total, three patients died at the early, mid, and long-term follow-up due to multiorgan failure, pneumonia, and unknown reasons. Conclusion: Hybrid repair of thoracoabdominal aneurysms may be an alternative to fenestrated or branched endovascular stent grafts in patients with increased risk factors for open surgical thoracoabdominal aneurysm repair; however, the procedure requires experience and care.

Tratamiento endovascular con técnica de chimenea para patologías del arco aórtico / Endovascular management with chimney technique for pathologies ofthe aortic arch

Introducción. La patología del arco aórtico se ha tratado principalmente con cirugía por vía abierta, pero con una alta morbimortalidad. Las técnicas endovasculares híbridas y las reconstrucciones en "chimenea" son una técnica válida y segura para disminuir el riesgo y la mortalidad. Métodos. Se presentan dos pacientes con patología del arco aórtico y contraindicación de manejo quirúrgico abierto, atendidos en el Servicio de Cirugía Vascular, Hospital Universitario Clínica de San Rafael, Bogotá, D.C., Colombia. Resultados. Se realizaron dos procedimientos endovasculares del arco aórtico para tratar un aneurisma torácico roto y una úlcera aórtica sintomática, con cubrimiento de los troncos supra aórticos con una endoprótesis y canalización de los vasos supra aórticos con prótesis cubiertas y uso de la "técnica de chimenea", de manera exitosa. Discusión. La patología del arco aórtico es de alta complejidad y se asocia con una morbimortalidad elevada por lo que, en los últimos 20 años se han desarrollado diferentes técnicas utilizando procedimientos percutáneos. Conclusión. La "técnica de chimenea" se puede realizar de una manera mínimamente invasiva en pacientes con patología del arco aórtico, no candidatos para cirugía abierta, con resultados exitosos.

Introduction. Aortic arch pathology has been treated mainly by open surgery, but with high morbidity and mortality. Hybrid endovascular techniques and "chimney" reconstructions are a valid and safe techniques to reduce risk and mortality. Method. Two patients with pathology of the aortic arch and contraindication for open surgical management, treated at the Vascular Surgery Service, Hospital Universitario Clínica de San Rafael, Bogotá, Colombia, are presented. Results. Two endovascular aortic procedures were performed successfully to treat a ruptured thoracic aneurysm and a symptomatic aortic ulcer, with coverage of the supra-aortic trunks with an endoprosthesis and cannulation of the supra-aortic vessels with covered prostheses and use of the "chimney technique". Discussion. The pathology of the aortic arch is highly complex and is associated with high morbidity and mortality, being the reason that during the last 20 years, different techniques have been developed using percutaneous procedures. Conclusions. The "chimney technique" can be performed with successful results in a minimally invasive manner in patients with pathology of the aortic arch, who are not candidates for open surgery

Epidemiological Analysis of 12 Years of Open Thoracoabdominal Aortic Aneurysm Repair in the Brazilian Public Health System

ABSTRACT Introduction: Although endovascular correction is a promising perspective, the gold-standard treatment for thoracoabdominal aortic aneurisms and type-B dissections with visceral involvement remains open surgery, particularly due to its well-established long-term durability. This study aims to describe and evaluate public data from patients treated for thoracoabdominal aortic aneurism in the Brazilian public health system in a 12-year interval. Methods: Data from procedures performed between 2008 and 2019 were extracted from the national public database (Departamento de Informática do Sistema Único de Saúde, or DATASUS) using web scraping techniques. Procedures were evaluated regarding the yearly frequency of elective or urgency surgeries, in-hospital mortality, and governmental costs. All tests were done with a level of significance P<0.05. Results: A total of 812 procedures were analyzed. Of all surgeries, 67.98% were elective cases. There were 328 in-hospital deaths (mortality of 40.39%). In-hospital mortality was lower in elective procedures (26.92%) than in urgency procedures (46.74%) (P=0.008). Total governmental expenditure was $3.127.051,56 — an average of $3.774,22 for elective surgery and $3.791,93 for emergency surgery (P=0.999). Conclusion: The proportion of urgency procedures is higher than that recommended by international literature. Mortality was higher for urgent admissions, although governmental costs were equal for elective and urgent procedures; specialized referral centers should be considered by health policy makers.

Síndrome de Marfan. Presentación de un caso / Marfan syndrome. A case report

RESUMEN El síndrome de Marfan es una enfermedad genética autosómica dominante del tejido conectivo, caracterizada por una combinación variable de manifestaciones cardiovasculares, músculo-esqueléticas y oftalmológicas. A pesar del descubrimiento de las mutaciones causales, su diagnóstico resulta complejo, al exhibir una gran diversidad en su presentación clínica y carecer de características patognomónicas. El diagnóstico actual de síndrome de Marfan se basa en una serie de criterios clínicos y genéticos denominados Criterios Gante revisados. Se describe el caso de una paciente de 44 años de edad, con antecedentes de luxación del cristalino, miopía y escoliosis, sin antecedentes patológicos familiares y que cumplió con los criterios diagnósticos actuales. Se sugiere la pesquisa etiológica de afecciones como luxación del cristalino y escoliosis, por parte de las especialidades correspondientes, como traducción orgánica de una enfermedad sistémica como el síndrome de Marfan.

ABSTRACT Marfan syndrome is an autosomal dominant genetic disease of connective tissue, characterized by a variable combination of cardiovascular, musculoskeletal, and ophthalmologic manifestations. Despite the discovery of the causal mutations, its diagnosis is complex, as it exhibits great diversity in its clinical presentation and lacks pathognomonic characteristics. The current diagnosis of Marfan syndrome is based on a series of clinical and genetic criteria called the revised Ghent Criteria. The case of a 44-years-old female patient with a history of lens dislocation, myopia and scoliosis, with no family pathological history and who met current diagnostic criteria is described. The etiological investigation of conditions such as lens dislocation and scoliosis is suggested, by the corresponding specialties, as an organic translation of a systemic disease such as Marfan syndrome.

Outcomes Following Supracoronary Ascending Aortic Replacement with Aortic Valve Resuspension versus Modified Bentall's Operation for Acute Type A Aortic Dissection

Abstract Introduction: Type A acute aortic dissection (AAD) remains a challenging cardiac emergency despite the availability of various management strategies. This study compared the outcomes of supracoronary ascending aortic replacement (SCAAR) with aortic valve (AV) resuspension with those of modified Bentall's operation for type A AAD and the progression of aortic regurgitation (AR), long-term dilatation of aortic root and proximal arch, and long-term mortality in SCAAR patients. Methods: Sixty patients underwent surgery for type A AAD (January 2005 to December 2015). Forty-three patients underwent SCAAR with AV resuspension and 17 underwent modified Bentall's operation. All patients were followed up. Results: Upon follow-up of SCAAR patients (n=40), there was significant reduction in aortic root size (preoperative 39.3 mm [9.4] vs. postoperative 33.1 mm [9.1]; P<0.001). Three of these patients worsened to severe AR while others had similar or lesser degree of AR. On comparison between preoperative and postoperative dimensions of all patients (n=53), there was no significant difference in distal ascending aorta size (35.7 mm [8.1] vs. 34.4 mm [8.9]; P=0.52). However, an increase in descending thoracic aorta size (28.8 mm [7.8] vs. 33.7 mm [9.9]; P<0.001) was observed. In-hospital and late mortalities for SCAAR vs. modified Bentall's procedure were 11.7% (seven patients) (7% [3] vs. 23.5% [4]) and 28% (15 patients) (15% [6] vs. 69% [9]), respectively. Conclusion: SCAAR with AV resuspension is a safe surgical option for type A AAD. Preservation of AV is associated with better long-term outcomes and reduced mortality. Modified Bentall's operation may be associated with long-term mortality.

Evaluation of Surgical Outcomes of Type A Intramural Hematoma

Abstract Introduction: In this study, we aimed to retrospectively evaluate the results of type A intramural hematoma (TA-IMH) cases that underwent ascending aortic surgery. Methods: One hundred ninety-four patients who underwent aortic surgery between 2010 and 2018 were included in this study. TA-IMH was differentiated according to tomography angiographic images. Demographic data, operation type, hypothermic circulatory arrest times, echocardiographic findings, wall thickness of IMH, complications, and prognosis were retrospectively analyzed. Results: TA-IMH (n=14) or type A aortic dissection (AD) (n=35) data were collected from patients' files and 49 cases were enrolled into the study. Bentall operation was performed in eight patients (type A AD = six [17.1%], TA-IMH = two [14.3%]); 41 patients underwent tubular graft interposition of ascending aorta (AD = 29 [82.9%], TA-IMH = 12 [85.7%]). There was no significant difference in terms of age, gender distribution, aortic dimensions, cardiopulmonary bypass times, hypothermic circulatory arrest times, hospital ward stay, and intensive care unit stay between the two groups. The mortality rate of AD group was 34.4% and of TA-IMH group was 14.3%. There was no significant difference in terms of mortality between the groups. In our study, 45.7% of patients had hypertension and that rate was lower than the one found in the literature. In addition, bicuspid aorta was not observed in both groups. Connective tissue disease was not detected in any group. Conclusion: Surgical treatment of aorta is beneficial for TA-IMH. Our aortic surgical indications comply with the European aortic surgical guidelines. Hypertension control should be provided aggressively.

Leiomioma disecante cotiledóneo del útero / Cotyledonoid dissecting leiomyoma of the uterus

RESUMEN Los tumores benignos de músculo liso son los tumores más frecuentes en el aparato genital femenino. El leiomioma disecante cotiledóneo del útero es una variante rara de leiomioma benigno, que presenta patrones de crecimiento inusuales y comparte algunas características con otras variantes de leiomioma disecante. Tiene un patrón de crecimiento inusual caracterizado por disección intramural dentro del miometrio y con apariencia similar de sus componentes extrauterinos a los cotiledones placentarios. Su aspecto sarcomatoide y carácter inusual dan la impresión de malignidad. El diagnóstico se realiza por los hallazgos histopatológicos característicos de la lesión. Se presenta un caso de leiomioma disecante cotiledóneo del útero.

ABSTRACT Benign smooth muscle tumors are the most common tumors in the female genital tract. Cotyledonoid dissecting leiomyoma of the uterus is a rare variant of benign leiomyoma, which presents unusual growth patterns and shares some features with other variants of dissecting leiomyoma. It has an unusual growth pattern characterized by intramural dissection within the myometrium and with similar appearance of its extrauterine components to placental cotyledons. Its sarcomatoid appearance and unusual character give the impression of malignancy. The diagnosis is made by the characteristic histopathologic findings of the lesion. A case of cotyledonoid dissecting leiomyoma of the uterus is presented.