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Rev. bras. cir. cardiovasc ; 35(6): 970-976, Nov.-Dec. 2020. tab, graf
Article in English | SES-SP, LILACS, SES-SP | ID: biblio-1143989


Abstract Objective: To give an overview of the Ortner's syndrome caused by an aortic arch aneurysm. Methods: By comprehensive retrieval of the pertinent literature published in the past two decades, 75 reports including 86 patients were collected and recruited into this study along with a recent case of our own. Results: The aortic arch aneurysms causing hoarseness were most commonly mycotic aneurysms. In this patient setting, in addition to the left recurrent laryngeal nerve, trachea was the most commonly affected structure by the aortic arch aneurysm. Surgical/interventional/hybrid treatments led to a hoarseness-relieving rate of 64.3%, much higher than that of patients receiving conservative treatment. However, hoarseness recovery took longer time in the surgically treated patients than in the interventionally treated patients. Conclusion: The surgical and interventional treatments offered similar hoarseness-relieving effects. Surgical or interventional treatment is warranted in such patients for both treatment of arch aneurysms and relief of hoarseness.

Humans , Aorta, Thoracic , Aortic Aneurysm/complications , Vocal Cord Paralysis/etiology , Hoarseness/etiology , Syndrome
Rev. bras. cir. cardiovasc ; 35(5): 834-837, Sept.-Oct. 2020. tab, graf
Article in English | SES-SP, LILACS, SES-SP | ID: biblio-1137354


Abstract Giant ascending aortic aneurysm is a rare condition. In this paper, we present an uncommon case of giant ascending aortic aneurysm with a maximal diameter of 14 cm in a 77-year-old woman presenting with unusual symptoms. The patient underwent a successful surgery involving ascending aortic replacement, and was discharged without any complication. After discharge, she was followed regularly and no major problem was observed in her control visits. To the best of our knowledge, our case is the largest ascending aortic aneurysm reported to date in the existing literature.

Humans , Female , Aged , Aortic Aneurysm/surgery , Aortic Aneurysm/complications , Aortic Aneurysm/diagnostic imaging , Superior Vena Cava Syndrome/etiology , Aorta/surgery , Aorta/diagnostic imaging , Cardiopulmonary Bypass , Tomography, X-Ray Computed , Blood Vessel Prosthesis Implantation
Rev. bras. cir. cardiovasc ; 35(4): 573-576, July-Aug. 2020. tab, graf
Article in English | SES-SP, LILACS, SES-SP | ID: biblio-1137288


Abstract Left sinus of Valsalva aneurysm (SVA) is a very infrequent clinical entity. Valsalva aneurysms are often asymptomatic in right and non-coronary sinuses and the diagnosis is often incidental. A left SVA which presents with exertional chest pain due to compression of left coronary system arteries is extremely rare. In this case, we present a successful surgical repair of left SVA without aortic regurgitation or myocardial infarction in a 59-year-old male patient.

Humans , Male , Middle Aged , Aortic Aneurysm/surgery , Aortic Aneurysm/complications , Aortic Aneurysm/diagnostic imaging , Sinus of Valsalva/surgery , Sinus of Valsalva/diagnostic imaging , Aortic Valve Insufficiency/surgery , Aortic Valve Insufficiency/diagnostic imaging , Chest Pain/etiology , Myocardial Infarction
Yonsei Medical Journal ; : 81-87, 2016.
Article in English | WPRIM | ID: wpr-186119


PURPOSE: In this study, we evaluated the long term beneficial effect of Renin-Angiotensin-Aldosterone System (RAAS) blockade therapy in treatment of Marfan aortopathy. MATERIALS AND METHODS: We reviewed Marfan syndrome (MFS) patients who underwent aortic root replacement (ARR) between January 1996 and January 2011. All patients were prescribed beta-blockers indefinitely. We compared major aortic events including mortality, aortic dissection, and reoperation in patients without RAAS blockade (group 1, n=27) to those with (group 2, n=63). The aortic growth rate was calculated by dividing the diameter change on CT scans taken immediately post-operatively and the latest scan available. RESULTS: There were no differences in clinical parameters except for age which was higher in patients with RAAS blockade. In group 1, 2 (7%) deaths, 5 (19%) aortic dissections, and 7 (26%) reoperations occurred. In group 2, 3 (5%) deaths, 2 (3%) aortic dissections, and 3 (5%) reoperations occurred. A Kaplan-Meier plot demonstrated improved survival free from major aortic events in group 2. On multivariate Cox, RAAS blockade was an independent negative predictor of major aortic events (hazard ratio 0.38, 95% confidence interval 0.30-0.43, p=0.002). Mean diameter change in descending thoracic and supra-renal abdominal aorta was significantly higher in patients without RAAS blockade (p<0.05). CONCLUSION: In MFS patients who underwent ARR, the addition of RAAS blockade to beta-blocker was associated with reduction of aortic dilatation and clinical events.

Adrenergic beta-Antagonists/pharmacology , Aged , Aneurysm, Dissecting/complications , Angiotensin Receptor Antagonists , Angiotensin-Converting Enzyme Inhibitors/therapeutic use , Aorta/pathology , Aortic Aneurysm/complications , Aortic Valve , Female , Humans , Male , Marfan Syndrome/mortality , Middle Aged , Renin-Angiotensin System/drug effects
Rev. ANACEM (Impresa) ; 8(2): 57-60, dic. 2014. ilus
Article in Spanish | LILACS | ID: biblio-997674


INTRODUCCIÓN: Las fístulas aortobronquiales son una causa muy infrecuente de hemoptisis masiva. Se asocia a la presencia de un aneurisma aórtico, y su evolución es siempre letal sin un tratamiento oportuno. El objetivo es presentar un caso de fístula aortobronquial y la revisión de bibliografía respectiva. Presentación del caso: Paciente de género femenino, de 76 años, hipertensa y diabética, sin otros antecedentes mórbidos. Ingresó al Servicio de Urgencia por hemoptisis posterior a caída a nivel. La radiografía de tórax mostró imagen hiperdensa en vértice pulmonar izquierdo, asociada a desviación mediastínica contralateral. Evolucionó con episodio de hemoptisis masiva el cual fue tratado satisfactoriamente con reposición de volumen y transfusión de hemoderivados. La tomografía computada de tórax con contraste mostró aneurisma de aorta torácica, de diámetro máximo de 10,8 cm...

INTRODUCTION: Aortobronchial fistula is a very rare cause of massive hemoptysis. It is associated with the presence of an aortic aneurysm and its evolution is always fatal without prompt treatment. The objective is to present a case of aortobronchial fistula and to review the respective literature. Case Report: A 76 years old woman, hypertensive and diabetic, no other known morbid history. She was admitted to emergency room with mild hemoptysis after a fall. Chest X-ray showed hyperdense image in the left lung apex, associated with contralateral mediastinal shift. She was treated with antibiotics and antitussives. She evolved with an episode of massive hemoptysis which was successfully treated with fluid resuscitation and blood transfusion. Chest computed tomography showed large thoracic aortic aneurysm with a maximum diameter of 10.8 cm…

Humans , Aortic Aneurysm , Aortic Aneurysm/complications , Vascular Fistula , Vascular Fistula/complications , Hemoptysis/etiology , Tomography, X-Ray Computed , Bronchial Fistula , Bronchial Fistula/complications , Fatal Outcome
Arq. bras. med. vet. zootec ; 65(3): 694-698, June 2013. ilus
Article in English | LILACS | ID: lil-679100


This paper reports the clinical, bacteriological and pathological findings of a thoracic aortic aneurysm in a four-year-old Anglo-Nubian goat buck, related to a framework of visceral caseous lymphadenitis. General clinical examination showed heart rate of 75 beats per minute, respiratory rate of 20 movements per minute and ruminal movements of four movements per minute. Superficial lymph nodes were normal upon palpation. Rectal temperature was slightly high (40.5°C). Blood test showed an intense leukocytosis (54,000/µL), characterized by strong neutrophil shift to the left. At necropsy, a large blood clot was detected in the thoracic cavity. The thickening of the myocardium and dilatation of the aorta in the thoracic portion, presenting a saculiform format was also observed. A large number of abscesses were disseminated in the media and intima layers of aorta. The aorta lumen obstruction by arterial plaques consisting of inflammatory infiltrate, predominantly neutrophilic was also detected. Abscesses were found spread in turbinate, rumen, reticulum, kidneys, liver, spleen, testicles and aorta wall. The microbiological exam of exudate confirmed Corynebacterium pseudotuberculosis as the causal agent.

Este trabalho descreve os achados clínicos, bacteriológicos e patológicos de um aneurisma da aorta torácica em um reprodutor caprino da raça Anglo-Nubiana, de quatro anos de idade, relacionado a um caso de linfadenite caseosa visceral. Ao exame clínico geral observaram-se: frequência cardíaca de 75 batimentos por minuto, frequência respiratória de 20 movimentos por minuto e movimentos ruminais de quatro movimentos por minuto. Os linfonodos superficiais encontravam-se normais à palpação. A temperatura retal estava ligeiramente aumentada (40,5°C). No hemograma completo, observou-se leucocitose intensa, 54.000/µL, caracterizada por um forte desvio neutrofílico à direita. Nos achados de necropsia, observou-se, na cavidade torácica, presença de um grande coágulo de sangue. No coração, foi identificado espessamento do miocárdio. Uma dilatação na porção torácica da artéria aorta foi detectada, apresentando um aspecto saculiforme. Um grande número de abscessos estava presente nas camadas média e íntima da aorta. Observou-se, também, obstrução do lúmen da aorta por placas de infiltrado inflamatório, predominantemente neutrofílico. A disseminação de abscessos nos cornetos, rúmen, retículo, fígado, baço, rins, testículos e parede da aorta foi detectada. O exame microbiológico do exsudato confirmou o Corynebacterium pseudotuberculosis como o agente causal.

Animals , Abscess/complications , Aortic Aneurysm/complications , Corynebacterium/pathogenicity , Leukocytosis/pathology , Goats/classification
Rev. bras. cir. cardiovasc ; 28(1): 10-21, jan.-mar. 2013. ilus, tab
Article in Portuguese | SES-SP, LILACS, SES-SP | ID: lil-675868


OBJETIVOS: O objetivo primário deste estudo é identificar preditores de óbito hospitalar em pacientes submetidos à cirurgia de aorta. O objetivo secundário é identificar fatores associados ao desfecho clínico composto hospitalar (óbito, sangramento, disfunção ventricular ou complicações neurológicas). MÉTODOS: Delineamento transversal com componente longitudinal; por meio de revisão de prontuários, foram incluídos 257 pacientes. Os critérios de inclusão foram: dissecção crônica de aorta tipo A de Stanford e aneurisma de aorta ascendente. Foram excluídos casos de dissecção aguda de aorta, qualquer tipo, e aneurisma de aorta não envolvendo segmento ascendente. As variáveis avaliadas foram demografia, fatores pré, intra e pós-operatórios. RESULTADOS: Variáveis com risco aumentado de óbito hospitalar (RC; IC95%; P valor): etnia negra (6,8; 1,54 30,2; 0,04), doença cerebrovascular (10,5; 1,12-98,7; 0,04), hemopericárdio (35,1; 3,73-330,2; 0,002), operação de Cabrol (9,9; 1,47-66,36; 0,019), cirurgia de revascularização miocárdica simultânea (4,4; 1,31-15,06; 0,017), revisão de hemostasia (5,72; 1,29-25,29; 0,021) e circulação extracorpórea (CEC) [min] (1,016; 1,007-1,026; 0,001). Dor torácica associou-se com risco reduzido de óbito hospitalar (0,27; 0,08-0,94; 0,04). Variáveis com risco aumentado do desfecho clínico composto hospitalar foram: uso de antifibrinolítico (3,2; 1,65-6,27; 0,0006), complicação renal (7,4; 1,52-36,0; 0,013), complicação pulmonar (3,7; 1,5-8,8; 0,004), EuroScore (1,23; 1,08-1,41; 0,003) e tempo de CEC [min] (1,01; 1,00-1,02; 0,027). CONCLUSÃO: Etnia negra, doença cerebrovascular, hemopericárcio, operação de Cabrol, revascularização miocárdica simultânea, revisão de hemostasia e tempo de CEC associaram-se com risco aumentado de óbito hospitalar. Dor torácica associou-se com risco reduzido de óbito hospitalar. Uso de antifibrinolítico, complicação renal, complicação pulmonar, EuroScore e tempo de CEC associaram-se ao desfecho clínico composto hospitalar.

OBJECTIVES: The primary objective was to identify predictors of hospital mortality in patients undergoing aortic surgery. The secondary objective was to identify factors associated with clinical outcome composed hospital (death, bleeding, neurologic complications or ventricular dysfunction). METHODS: A cross-sectional design with longitudinal component. Through chart review, 257 patients were included. Inclusion criteria were: aortic dissection Stanford type A and ascending aortic aneurysm. Exclusion criteria were acute aortic dissection, of any kind, and no aortic aneurysm involving the ascending segment. Variables assessed: demographics, preoperative factors, intraoperative and postoperative. RESULTS: Variables with increased risk of hospital mortality (OR, 95% CI, P value): black ethnicity (6.8, 1.54-30.2; 0.04), cerebrovascular disease (10.5, 1.12-98.7; 0.04), hemopericardium (35.1, 3.73-330.2; 0.002), Cabrol operation (9.9, 1.47-66.36; 0.019), CABG simultaneous (4.4; 1.31 to 15.06; 0.017), bleeding (5.72, 1.29-25.29; 0.021) and cardiopulmonary bypass (CPB) time [min] (1.016; 1.0071.026; 0.001). Thoracic pain was associated with reduced risk of hospital death (0.27, 0.08-0.94, 0.04). Variables with increased risk of hospital clinical outcome compound were: use of antifibrinolytic (3.2, 1.65-6.27; 0.0006), renal complications (7.4, 1.52-36.0; 0.013), pulmonary complications (3.7, 1.58.8, 0.004), EuroScore (1.23; 1.08-1.41; 0.003) and CPB time [min] (1.01; 1.00 to 1.02; 0.027). CONCLUSION: Ethnicity black, cerebrovascular disease, hemopericardium, Cabrol operation, CABG simultaneous, hemostasis review and CPB time was associated with increased risk of hospital death. Chest pain was associated with reduced risk of hospital death. Use of antifibrinolytic, renal complications, pulmonary complications, EuroScore and CPB time were associated with clinical outcome hospital compound.

Female , Humans , Male , Middle Aged , Aortic Aneurysm/mortality , Aortic Aneurysm/surgery , Hospital Mortality , Aortic Aneurysm/complications , Epidemiologic Methods , Intraoperative Complications/mortality , Postoperative Complications/mortality , Time Factors
Article in English | WPRIM | ID: wpr-9505


A 26-yr-old male patient reported worsened dyspnea, dizziness one year after an emergency Bentall operation for type A aortic dissection. There was evidence of hemolytic anemia and aortogram revealed a significant stenosis at the distal anastomosis site. During the reoperation, we found the inner felt at the distal anastomosis was inverted causing a significant stenosis. The reoperation successfully resolved this problem. Here, we report a rare case of hemolytic anemia caused by an inverted inner felt after Bentall operation.

Acute Disease , Adult , Anastomosis, Surgical , Anemia, Hemolytic/diagnosis , Aneurysm, Dissecting/complications , Aortic Aneurysm/complications , Blood Vessel Prosthesis , Blood Vessel Prosthesis Implantation/adverse effects , Dizziness/etiology , Dyspnea/etiology , Echocardiography , Humans , Male , Postoperative Complications/surgery , Reoperation , Time Factors , Tomography, X-Ray Computed , Treatment Outcome
Rev. bras. cir. cardiovasc ; 27(3): 481-484, jul.-set. 2012. ilus
Article in Portuguese | LILACS | ID: lil-660821


A síndrome da veia cava superior representa o conjunto de sinais e sintomas decorrentes da sua obstrução. A síndrome tem como principais etiologias tumores malignos, como o carcinoma broncogênico, o linfoma e a metástase mediastinal. O câncer de pulmão é responsável por 80% dos casos, os linfomas mediastinais por 15%, e 5% correspondem às demais causas. Este relato de caso objetiva apresentar um caso incomum dessa síndrome, ocorrido em um paciente do sexo masculino após ferimento penetrante no tórax, resultando em pseudoaneurisma de arco aórtico e a síndrome da veia cava superior.

The superior vena cava syndrome represents the set of signs and symptoms resulting from obstruction of superior vena cava. The syndrome has as main causes malignant tumors such as bronchogenic carcinoma, lymphoma and mediastinal metastases. Lung cancer accounts for 80% of cases, mediastinal lymphomas by 15% and 5% correspond to other causes. This case report aims to present an unusual case of this syndrome, which occurred in a male patient after penetrating wound in the chest, which developed a pseudoaneurysm of the aortic arch and superior vena cava syndrome.

Adult , Humans , Male , Aneurysm, False/complications , Aortic Aneurysm/complications , Superior Vena Cava Syndrome/etiology , Aneurysm, False/surgery , Aortic Aneurysm/surgery , Superior Vena Cava Syndrome/surgery , Treatment Outcome , Wounds, Stab/complications
Arch. cardiol. Méx ; 81(1): 18-21, ene.-mar. 2011. ilus, tab
Article in English | LILACS | ID: lil-631994


Aneurysms of the sinus of Valsalva (SV) and the atrial septum are a rare association. We report the case of a 28-year-old woman, who was admitted to our department complaining of progressive dyspnea of 10 days of evolution, five hours previous to her admission to the hospital; she presented sudden oppressive anterior chest pain, accompanied by palpitations. The presence of rupture of the right SV to the right atrium was clinically confirmed, by echocardiography and hemodynamic studies. In addition, an associated atrial septal aneurysm was found. She underwent surgical correction through sinusplasty without requiring aortic valve replacement. The patient presented persistent postoperative atrioventricular block, which required a permanent pacemaker. Clinical evolution was satisfactory. To our knowledge, this case is a rare combination of two isolated malformations, without previous events that could explain the rupture of the right SV.

Los aneurismas del seno de Valsalva y del septum interauricular son una asociación rara. Informamos el caso de una mujer de 28 años de edad con un cuadro de disnea progresiva en los últimos 10 días, al cual se agregó dolor precordial opresivo, cinco horas previas a su ingreso. Se comprobó clínicamente, por ecocardiografía y hemodinamia la presencia de ruptura del seno de Valsalva derecho hacia el atrio derecho. Un hallazgo interesante fue la presencia de un aneurisma del septum interauricular asociado. La paciente fue sometida a corrección quirúrgica con plastía del seno de Valsalva, sin requerir reemplazo valvular aórtico. En el postoperatorio presentó bloqueo aurículo-ventricular persistente, requiriendo implante de marcapaso definitivo. Su evolución fue satisfactoria. Este es un caso de una rara asociación de dos malformaciones aisladas.

Adult , Female , Humans , Atrial Septum , Aortic Aneurysm/complications , Aortic Rupture/complications , Heart Atria , Heart Aneurysm/complications , Sinus of Valsalva , Rupture, Spontaneous
Rev. Soc. Cardiol. Estado de Säo Paulo ; 21(1): 74-78, jan.-mar. 2011. ilus
Article in Portuguese | LILACS | ID: lil-588386


O tratamento cirúrgico convencional para os pacientes portadores de aneurisma da raiz da aorta é a sua substituição completa por um conduto valvulado associado ao reimplante dos óstios coronários. Este é um procedimento de baixo risco, duradouro e principalmente utilizado naqueles pacientes que se recusam a imaginar a necessidade de outra operação, apesar da necessidade de utilização do anticoagulante oral em definitivo. Alternativamente, é possível a reconstrução deste seguimento da aorta com preservação valvar. Muita controvérsia existe em relação à longevidade deste complexo tratamento alternativo, apesar dos resultados imediatos, em muitas séries, melhores até que os obtidos com a operação convencional. A literatura sugere que a síndrome de Marfan, a valva aórtica bivalvulada, os grandes aneurismas, os anéis aórticos dilatados, a ausência de fixação do anel aórtico e a necessidade de plastia de um ou mais folhetos aórticos sejam os principais preditores de reoperação no seguinte tardio dos pacientes que tiveram a valva aórtica preservada...

The conventional surgical treatment for patients with aortic root aneurysm has been the replacement of the entire root and valve with a composite valve graft and the reimplantation of the coronary ostium. It has become a low risk operation although its effects are long-standing. It is usually used in patients who do not wish to accept the risk of another operation, despite the indefinite anticoagulation needs. Alternatively, the valve sparing aortic operation is a practical possibility. Despite its immediate results in many series, even better by those obtained by a conventional operation, the longetivity of this complex alternative treatment is still controversial. Many papers suggest that Marfan syndrome, bicuspid aortic valve, large aneurysms, large aortic rings, the absence of aortic ring fixation and the need of aortic leaflets plasty are predictors of reoperation during the follow up. There is no doubt, however, that the aortic root reconstruction can be performed with low hospital mortality and high freedom from aortic valve-related reoperation, however the choice of the best procedure must be individualized for each patient and the valve sparing operation it is neither a procedure that can be performed by all surgeons nor applied to all patients.

Humans , Aortic Aneurysm/complications , Aorta, Thoracic/surgery , Heart Valve Diseases/surgery , Heart Valve Diseases/epidemiology , Aortic Valve/abnormalities
J. vasc. bras ; 8(2): 186-188, jun. 2009. ilus
Article in Portuguese | LILACS | ID: lil-521372


A doença renal policística dominante é uma das doenças renais hereditárias mais comuns, podendo apresentar manifestações extrarrenais vasculares de importância clínica, como aneurismas intracranianos, aneurismas aórticos e dissecções arteriais. Relatamos o caso de um paciente masculino, com 66 anos de idade, renal crônico não-dialítico por doença renal policística dominante, com aneurisma de aorta abdominal infrarrenal assintomático, diagnosticado por ultrassonografia de rotina e operado eletivamente com sucesso. A doença renal policística dominante é uma síndrome genética, associada aos genes PDK1 e PDK2 no cromossomo 16. A expressão desses genes na parede dos vasos leva ao seu enfraquecimento, favorecendo a formação de aneurismas. A produção de metaloproteinases pelos túbulos renais também estaria relacionada às doenças vasculares desses pacientes. Tais doenças se apresentam como importantes fatores de mortalidade precoce e morbidade dos portadores de doença renal policística dominante e, como usualmente são assintomáticas, justifica-se o uso de propedêutica armada e tratamento precoce.

Autosomal dominant polycystic kidney disease (ADPKD) is one of the most common hereditary renal diseases, which may present important clinical extrarenal vascular manifestations, such as intracranial and aortic aneurysms and artery dissections. We report the case of a 66-year-old male chronic renal out-of-dialysis patient, with dominant polycystic kidney disease, presenting an asymptomatic infrarenal abdominal aortic aneurysm diagnosed by routine ultrasonography, submitted to successful elective surgery. ADPKD is a genetic syndrome, associated with PDK1 and PDK2 genes on chromosome 16. The expression of these genes in the vessel walls leads to vessel wall weakening, favoring aneurysm formation. In addition, metalloproteinase production by kidney tubules could be related to vascular diseases in ADPKD patients. These are important factors of early mortality and of morbidity in patients with ADPKD, thus the use of equipped propedeutics and early treatment are indicated, as these manifestations are usually asymptomatic.

Humans , Male , Aged , Aortic Aneurysm/surgery , Aortic Aneurysm/complications , Aortic Aneurysm/diagnosis , Renal Insufficiency, Chronic/complications , Renal Insufficiency, Chronic/diagnosis , Polycystic Kidney, Autosomal Dominant/complications , Polycystic Kidney, Autosomal Dominant/diagnosis
Rev. méd. Chile ; 137(1): 98-100, ene. 2009. ilus
Article in Spanish | LILACS | ID: lil-511851


Aortic dissection is a life-threatening disease that requires immediate surgical intervention. Marfan syndrome is a hereditary disease with an autosomaldominant transmission, which affects the connective tissue, with skeletal, cardiovascular and ocular involvement. It is one of the most prevalent connective tissue disorders, presenting a risk of aortic dissection of approximately 1% even without dilatation of the aorta. When dissectionoccurs during pregnancy and requires surgical intervention (type A dissection), maternal mortality is high (20%-30%). We report a 38 year-old woman with Marfan syndrome that hadan acute type A aortic dissection and severe aortic regurgitation at 37 weeks of gestation. The patient underwent a cesarean section and delivered a healthy baby. Afterwards, aortic valverepair and ascending aortic replacement was successfully performed under circulatory arrest with deep hypothermia. Additionally mitral valve repair for degenerative disease with posterior, autologous pericardium mitral valve ring was performed.

Adult , Female , Humans , Pregnancy , Aneurysm, Dissecting/complications , Aortic Aneurysm/complications , Marfan Syndrome/complications , Pregnancy Complications, Cardiovascular , Aneurysm, Dissecting/surgery , Aortic Aneurysm/surgery , Marfan Syndrome/surgery , Pregnancy Complications, Cardiovascular/surgery