Contained rupture of an aortic arch aneurysm in a patient with syphilitic aortitis. A case report / Ruptura contida de um aneurisma do arco aórtico em paciente com aortite sifilítica. Um relato de caso

Abstract Syphilitic aortitis is a rare complication of tertiary syphilis, which can lead to aortic aneurysm formation, aortic valvular insufficiency, and ostial coronary stenosis. Syphilis has re-emerged worldwide over recent decades and vascular surgeons should be aware of its cardiovascular manifestations. Atypical clinical presentation, such as hemoptysis and a computed tomography angiography pattern of a thicker aneurysmal wall with ulcer-like aneurysm projections, should raise suspicion of syphilitic aortic aneurysm. An early diagnosis and appropriate surgical and medical therapies significantly contribute to successful treatment and favorable prognosis. Herein is reported the case of an 82-year-old male patient, positive for syphilis infection, with impending aortic arch aneurysm rupture treated with a hybrid arch repair. After 7 months, the patient was brought to the emergency room in cardiac arrest. Unsuccessful cardiopulmonary resuscitation maneuvers were performed, and an autopsy showed cardiac tamponade due to rupture of the ascending aorta.

Resumo A aortite sifilítica é uma complicação rara da sífilis terciária, que pode levar a formação de aneurisma aórtico, insuficiência valvar aórtica e estenose ostial coronariana. Ao longo das últimas décadas, a sífilis ressurgiu ao redor do mundo, e os cirurgiões vasculares devem estar atentos às suas manifestações cardiovasculares. Apresentações clínicas atípicas, como a hemoptise, e um padrão na angiotomografia computadorizada de parede aneurismática mais grossa com projeções de aneurisma com aparência de úlcera devem levantar a suspeita de aneurisma sifilítico da aorta. O diagnóstico precoce e terapias cirúrgica e clínica adequadas contribuem significativamente para um tratamento bem-sucedido e prognóstico favorável. Relatamos o caso de um paciente do sexo masculino, de 82 anos, que apresentou infecção por sífilis com ruptura iminente de aneurisma do arco aórtico. Ele foi tratado com reparo híbrido do arco. Após 7 meses, o paciente deu entrada na emergência com parada cardiorrespiratória. As manobras de reanimação cardiopulmonar foram realizadas, mas não obtiveram sucesso, e a autópsia mostrou tamponamento cardíaco devido à ruptura da aorta ascendente.

Hemoptisis como signo de alarma de pseudoaneurisma de la aorta torácica: reporte de caso / Hemoptysis as a warning sign of thoracic aorta pseudoaneurysm: a case report

El pseudoaneurisma se define como un hematoma pulsátil repermeabilizado, encapsulado y en comunicación con la luz de un vaso dañado. Se origina cuando hay una disrupción de la pared arterial. La hemoptisis es un signo/síntoma de presentación muy rara de aneurisma aórtico torácico y de pseudoaneurisma aórtico torácico. Hay poca información sobre la hemoptisis asociada con la ruptura del aneurisma aórtico cuyo mecanismo no se explica por la presencia de una fístula aortopulmonar. Entre las hipótesis para explicar este fenómeno, se encuentra la capacidad de las arterias bronquiales de volverse hiperplásicas y tortuosas en presencia de una lesión que modifica la arquitectura pulmonar, siendo más susceptibles a la ruptura. También hay descripciones de lesiones directas del parénquima pulmonar por aneurisma roto. El presente caso nos ilustra que debemos considerar a la hemoptisis como signo de alarma en el diagnóstico diferencial de los aneurismas y pseudoaneurismas aórticos entre otras causas que puede ser fatal en breve tiempo por una hemorragia masiva.

Pseudoaneurysm is defined as a reperfused pulsatile hematoma, encapsulated and communicated with the damaged vessel's lumen. It originates when there is a disruption of the arterial wall. Hemoptysis is a very rare sign/symptom of a thoracic aortic aneurysm or pseudoaneurysm. There is little information on hemoptysis associated with aortic aneurysm rupture, whose mechanisms are not explained by the presence of an aortopulmonary fistula. Among the hypotheses to explain this phenomenon, is the ability of the bronchial arteries to become hyperplasic and tortuous in the presence of a lesion that modifies the pulmonary architecture, being more susceptible to rupture. There are also descriptions of direct lung parenchymal injury from ruptured aneurysm. The present case illustrates that we must consider the hemoptysis as a warning sign in differential diagnosis of aortic aneurysms and pseudo aneurysms, among other causes, that it can be fatal in a short time due to massive hemorrhage.

Thoracic aortic aneurysm surgery in marfan patients: a perspective from the uk

Abstract Objective: Cardiovascular complications in Marfan patients include progressive aortic root dilation which can precipitate acute aortic dissection, ruptured aorta, severe aortic regurgitation, or all the aforementioned. Such complications can be fatal and the cause of death prior to any surgical intervention. We set out to identify the Marfan population in England and Wales and present their surgical outcomes. Methods: A total of 306 patients with Marfan syndrome who underwent aortic root surgery were identified between April 2007 and March 2013 from NICOR database. We examined the perioperative characteristics of such cohort along with in-hospital outcomes and survival. Results: Root and ascending segment procedures on Marfan patients performed in 3.3% of the total cohort by NICOR root surgery patients. The median reported age was 40 years (IQR = 29-49 years) and 100 (32.7%) were female. Of the patients analysed, 17.3% were treated non-electively and 68.6% of them received concomitant valve procedure. The in-hospital mortality was 2.0%. Reoperation for bleeding was required in 8.2% of patients and 1.3% of them suffered a cerebrovascular accident (CVA). Mortality at 1 year was reported as 5.5%. Conclusion: The outcomes of surgery on the root and ascending aorta in Marfan patients in the United Kingdom are satisfactory; however, the overall complexities of this patient population are not well understood and would benefit from further investigations.

Annuloaortic ectasia and arteritis: clinical features, treatments of choice, and causative relations

Abstract The roles that aortitis plays in the development of annuloaortic ectasia (AAE) remain uncertain, while clinical features of AAE in arteritis are largely unknown. This study was designed to highlight the clinical features of AAE, the treatments of choice, and the causative relations between aortitis and AAE. The morphology of the aortic valve leaflets was normal in half of the patients, while the valves were thin and overstretched in the other half. Most patients had an aortic aneurysm. Half of the patients had severe aortic valve insufficiency, and one-quarter of them had dilation of the sinuses of Valsalva. Takayasu arteritis was prone to develop coronary artery lesions, whereas giant cell arteritis were not. Aortic branch lesions in Takayasu arteritis were stenotic or occlusive in 92.9% of the patients, while in giant cell arteritis, they were all dilated lesions. Most patients (94.7%) required surgical treatment with steroid therapy. However, long-term follow-up results showed a higher anastomotic dehiscence rate, particularly in patients with Takayasu arteritis. Further morphometric and pathological research on AAE in arteritis should be undertaken, and more feasible measures should be warranted for preventing postoperative anastomotic dehiscence.

Acompanhamento de Gestante Portadora de Síndrome de Marfan com Tubo Valvado Metálico Aórtico / Management of Pregnant Woman with Marfan Syndrome and Mechanical Aortic Valved Conduit

Relata-se o acompanhamento de paciente gestante, 29 anos, primigesta, portadora da Síndrome de Marfan e tubo valvado metálico aórtico encaminhada ao cardiologista com sete semanas de gestação.Ao ecodopplercardiograma apresentava diâmetro da raiz da aorta de 35 mm, tubo valvado e prótese aórtica metálica normofuncionante. Realizou acompanhamento cardiológico especializado desde então. Na 38a semana de gestação foi submetida ao parto cesariana com anestesia geral, profilaxia de endocardite infecciosa, monitorização cardíaca no centro cirúrgico e pós-operatório na unidade coronariana por 24 horas. Os períodos gestacional e puerpério transcorreram sem eventos hemorrágicos. O acompanhamento clínico e obstétrico de gestantes com Síndrome de Marfan é desafiador, e a condição de prótese valvar mecânica previamente implantada aumenta o risco destas paciente

Severe tracheobronchial compression in a patient with Turner's syndrome undergoing repair of a complex aorto-subclavian aneurysm: Anesthesia perspectives.

We present a case of severe tracheobronchial compression from a complex aorto‑subclavian aneurysm in a patient with Turner’s syndrome undergoing open surgical repair. Significant airway compression is a challenging situation and requires careful preoperative preparation, maintenance of spontaneous breathing when possible, and consideration of having an alternative source of oxygenation and circulation established prior to induction of general anesthesia. Cardiopulmonary monitoring is essential for safe general anesthesia and diagnosis of unexpected intraoperative events.

Caso 1/2013: homem de 69 anos com dor súbita em dorso e membro inferior direito e choque / Case 1/2013: 69-year-old male patient with sudden back and lower right limb pain and shock

MSM, homem, 69 anos, procurou atendimento médico por dor em dorso esquerdo e membro inferior direito. A radiografia de tórax revelou alargamento do mediastino. Estava em observação quando apresentou rebaixamento da consciência e choque. Foi observado enfisema subcutâneo em hemitórax esquerdo e abolição do murmúrio vesicular em base do mesmo pulmão. Foi feita a intubação orotraqueal e realizada drenagem de hemitórax esquerdo, com saída de líquido serossanguinolento. O ecocardiograma revelou ventrículo esquerdo (D/S): 44/29 mm; septo 12 mm; parede posterior 13 mm; discreta dilatação em raiz da aorta, presença de lâmina de dissecção e hematoma periaórtico. As valvas e pericárdio eram normais. O paciente foi transferido para o InCor. O exame físico (21 out 2004: 10h45) revelou paciente sedado, com intubação orotraqueal, com palidez cutânea, frequência cardíaca 90 bpm, pressão arterial 130 x 80 mmHg, drenagem torácica sanguinolenta do dreno tórax. Eletrocardiograma - frequência 90 bpm, ritmo sinusal, baixa voltagem no plano frontal e diminuição de voltagem em derivações esquerdas (fig. 1). A tomografia revelou enfisema subcutâneo bilateral, aorta torácica com contornos imprecisos na sua porção descendente (da artéria subclávia até porção média), colapso do pulmão esquerdo e grande coleção de características hemáticas em mesmo hemitórax e no mediastino médio e posterior. Pequeno pneumotórax à direita; pequeno derrame pleural à direita com alterações do parênquima subjacente. A análise do coração foi prejudicada pela presença do hemotórax. Durante a realização de tomografia apresentou ausência de pulsos, midríase, com assistolia, sem resposta às manobras de ressuscitação e faleceu (21 out 2011; 15h).

The patient, MSM, a 69-year-old man, sought medical care due to left dorsal and right lower limb pain. The chest x-ray showed mediastinal enlargement. He was undergoing examination when he lost consciousness and went into shock. Subcutaneous emphysema was observed in the left hemithorax, as well as abolition of breath sounds at auscultation. Tracheal intubation was performed with draining of blood-tinged fluid from the left hemithorax. Echocardiography showed left ventricle with 44/29 mm; septum, 12 mm; posterior wall, 13 mm; mild aortic root dilation, dissection of the lamina and periaortic hematoma. The valves and pericardium were normal. The patient was transferred to Instituto do Coraçao - InCor. Physical examination (21 Oct 2004: 10:45) showed that the patient was sedated with tracheal intubation, pale, heart rate at 90 bpm, blood pressure 130 x 80 mmHg, bloody drainage in the chest tube. Electrocardiogram - frequency 90 bpm, sinus rhythm, low voltage in the frontal plane and decreased voltage in left leads (Fig. 1). Computed tomography showed bilateral subcutaneous emphysema, thoracic aorta with inaccurate borders in its descending portion (from the subclavian artery to the middle portion), collapsed left lung and extensive collection of hematic characteristics in same hemithorax and middle and posterior mediastinum. Small right pneumothorax; small right pleural effusion with underlying parenchymal alterations. The analysis of the heart was impaired by the presence of hemothorax. While undergoing computed tomography, the patient showed no pulse, mydriasis, with asystole unresponsive to resuscitation and died (21 Oct 2011; 15:00 h).

Síndrome de Marfan con disección aórtica asociada a tromboembolismo venoso e hiperhomocisteinemia / Marfan syndrome associated with aortic dissection, venous thromboembolism and hyperhomocysteinemia

El síndrome de Marfan es un desorden genético infrecuente, cuyas manifestaciones clínicas afectan a los sistemas cardiovascular, ocular y músculo esquelético, la gravedad de las manifestaciones cardiovasculares son generalmente responsables de la mortalidad de estos pacientes. La enfermedad tromboembólica venosa está íntimamente relacionada con diversos factores de riesgo, congénitos, adquiridos, mixtos o desconocidos. La hiperhomocisteinemia es un factor de riesgo moderado para tromboembolismo venoso. Presentamos el caso de una mujer de 47 años de edad con síndrome de Marfan asociado a disección aórtica tipo A indolora, que simultáneamente padeció trombosis venosa de miembros inferior y superior izquierdos con embolia de pulmón. Al realizar el cribado de trombofilia se constató hiperhomocisteinemia. Con la terapéutica instituida evolucionó favorablemente.

Marfan syndrome is an infrequent genetic disorder of connective tissue whose clinical manifestations mainly affect the cardiovascular, ocular and musculoskeletal systems. Serious cardiovascular manifestations are generally the cause of mortality of Marfan patients. Thromboembolic venous disease is intimately related to different risk factors: inherited, acquired, mixed or unknown; hyperhomocysteinemia is a moderate risk factor for venous thromboembolism. We present the case of a 47-year-old woman with Marfan syndrome associated to a painless type A aortic dissection, who simultaneously suffered venous thromboembolism of left upper and lower limbs with pulmonary embolism. Hyperhomocysteinemia was found through thrombofilia screening. The patient's condition has evolved favorably.

Cystic medial necrosis: pathological findings and clinical implications / Necrose cística da média: manifestações patológicas com implicações clínicas

Cystic medial necrosis (CMN) is a disorder of large arteries, in particular the aorta, characterized by an accumulation of basophilic ground substance in the media with cyst-like lesions. CMN is known to occur in certain connective tissue diseases such as Marfan syndrome, Ehlers-Danlos syndrome, and annuloaortic ectasia, which usually result from degenerative changes in the aortic wall. The relationships between CMN and congenital heart defects as well as other disorders have been evidenced. The mechanisms are still controversial, even though many molecular studies have been conducted. The aim of the present article is to provide a comprehensive overview of the CMN lesion in terms of pathologic features, clinical implications and etiologies based on molecular research results.

A necrose cística da média (NCM) é uma desordem das grandes artérias, em particular a aorta, caracterizada por acúmulo de substância basofílica na camada média com lesões císticas-símile. É sabido que a NCM ocorre em certas doenças do tecido conjuntivo tal como síndrome de Marfan, síndrome de Ehlers-Danlos, e ectasia ânulo-aórtica, que normalmente resulta de mudanças degenerativas na parede aórtica. A relação entre NCM e defeitos congênitos do coração, assim como outras desordens, tem sido evidenciada. Os mecanismos são ainda controversos, embora muitos estudos moleculares tenham sido conduzidos. O objetivo do presente artigo é fornecer uma visão geral da NCM em termos de características patológicas, implicações clínicas e etiologia baseada em resultados de pesquisa molecular.

Aneurismas de aorta torácica e dissecção aórtica / Thoracic aortic aneurysms and aortic dissection

Doença da aorta é uma patologia significante e representa a 12ª maior causa de morte. Apesar de os aneurismas da aorta abdominal e aorta ascendente serem mais comum, aneurismas de aorta ascedentes (AATs) são uma significante contribuição para a patologia. AATs aumentam o risco de dissecção da aorta ou ruptura e representam uma importante fonte de morbidade e mortalidade. Podem ser classificadas como sindrômicas, familiares ou esporádicas. As formas sindrômicas incluem a Síndrome de Marfan, Síndrome de Loeyes-Dietz, Homocistinúria, Sindrome de Ehlers-Danlos, Síndrome B A V e Síndrome de Turner. Em vários casos, já existem testes genéticos específicos que permitem um diagnóstico precoce da patologia em familiares de indivíduos afetados que permitem o acompanhamento precoce com melhora da qualidade de vida e sobrevida desses indivíduos.

Aortic disease is a significant pathology and represents the 12th leading cause of death. Although aneurysms of the ascending aorta and abdominal aorta are more common, descendents aortic aneurysms (TAAs) are a significant contribution to the pathology. TAAs increase the risk of aortic dissection or rupture and represent an important source of morbidity and mortality. They can be classified as syndromic, familial or sporadic. The syndromic forms include Marfan syndrome, Dietz-Loeyes Syndrome, homocystinuria, Ehlers-Danlos syndrome, Turner syndrome and BAV syndrome. In several cases, specific genetic tests are already available and allow an early diagnosis of pathology in relatives of affected individuals that can lead to an early monitoring and improve the quality of life and survival of these individuals.

near fatal complication following stenting of thoracic aortic aneurysm

Tracheobronchial obstruction along with compression of pulmonary vessels is a rare complication after stenting of aortic aneurysm. We present this rare situation in a young patient who underwent stenting of traumatic thoracic aortic aneurysm and developed this near fatal complication and also the conservative management plan which we adopted to manage this case

Hybrid Endovascular Repair of Thoracic Aortic Aneurysm in a Patient with Behcet's Disease Following Right to Left Carotid-carotid Bypass Grafting

Endovascular repair of inflammatory aortic aneurysms has been reported as an alternative to open surgical treatment. In selective cases, adjunctive bypass surgery may be required to provide an adequate landing zone. We report a case of endovascular repair of an inflammatory aortic aneurysm in a patient with Behcet's disease using a carotid-carotid bypass graft to provide an adequate landing zone. A 45-yr-old man with a voice change was referred to our hospital with the diagnosis of saccular aneurysm of the distal aortic arch resulting from vasculitis. Computed tomography showed a thoracic aortic aneurysm with thrombosis. Right to left carotid-carotid bypass grafting was performed. After 8 days, the patient underwent an endovascular stent graft placement distal to the origin of the innominate artery. The patient was discharged with medication and without postoperative complications after 5 days. Hybrid endovascular treatment may be suitable a complementary modality for repairing inflammatory aortic aneurysms.

Manejo endovascular de las complicaciones agudas de la disección aórtica tipo B / Endovascular management of acute complications of type b aortic dissection

Background: Type B aortic dissection is usually managed by intensive care medical therapy and surgery is reserved for treating the complications that can occur during the evolution of a case. Aim: To assess the endovascular management of acute complications of type B aortic dissection and the closure of the intimal defect and aortic false lumen. Material and Methods: Retrospective analysis of 8 consecutive patients aged 40 to 57 years (seven males) treated for acute complications in the initial episode of a type B aortic dissection between August 2006 and July 2008. Results: Six/eight were known hypertensive patients. The indications for surgery were intractable pain in one, hypertension refractory to treatment in two and distal hypoperfusion in fve. Five patients required covering of the left subclavian artery ostium, without need for surgical repair. One patient was subjected to renal angioplasty and stenting. Technical success was achieved in all cases, with complete closure of the proximal aortic tear and thoracic aortic false lumen, although 7 of patients had a persistent distal aortic false lumen. One case had a transient lower limb paraparesis. No patient died. Conclusions: Endovascular treatment is effective in closing the aortic tear as well as the thoracic aortic false lumen in aortic type B dissections with a low complication rate. Due to the high frequency of distal aortic false lumen persistence, it is not a defnitive treatment for this condition but it is useful for the acute complications of the initial phase of type B aortic dissection.

Hemoptise e hemotórax como apresentação de ruptura de aorta torácica / Hemoptysis and hemothorax as presentation of thoracic aortic rupture

Dissecção da aorta torácica é doença de grande mortalidade em sua fase inicial, mas pode, em alguns casos, se cronificar. Relatamos caso de paciente com dissecção crônica de aorta tipo B (Stanford), admitido na Emergência com confusão mental, dispnéia e relato de hemoptise importante. O eletrocardiograma mostrava alterações inespecíficas e a radiografia de tórax revelou opacificação do hemitórax esquerdo. O ecocardiograma transtorácico não evidenciou dissecção aórtica, mas demonstrou imagem compatível com hemotórax, ocasionando a suspeita de ruptura da aorta. O paciente evoluiu em colapso cardiovascular e óbito. Este caso descreve duas apresentações atípicas da dissecção de aorta: hemotórax e hemoptise importante.

Thoracic aortic dissection is a disease of great mortality in its initial phase, but in some cases it can assume chronic course. We report a case of a patient with Stanford type A1 aortic dissection, admitted with mental confusion, dyspnea and event of severe hemoptysis. Electrocardiogram showed unspecific change and chest X-ray revealed opacification of the left hemithorax. Transthoracic echocardiogram did not show aortic dissection, but showed image similar to hemithorax leading to the suspicion of aortic rupture. The patient developed cardiovascular collapse and evolved to death. This case describes two unusual presentations of aortic dissection: hemothorax and severe hemoptysis.

Aneurisma de la aorta torácica en coartación aórtica sin cirugía previa: presentación de dos casos / Thoracic aortic aneurysm associated with aortic coarctation without previous surgery: report of two cases

La arteria aorta generalmente se vuelve aneurismática por efectos hemodinámicos, lo cual es poco común en niños y más aún asociada a coartación aórtica. La posibilidad de formación de aneurismas es rara sin antecedentes de procedimientos quirúrgicos o endovasculares en la aorta. Este trabajo presenta dos pacientes, un adolescente y un niño, con asociación de aneurisma de la aorta torácica y coartación aórtica, sin antecedentes de cirugía o procedimiento intervencionista.

The aorta usually becomes aneurysmatic as a result of hemodynamic effects. It is uncommon in children and less frequently associated with aortic coarctation. Aneurysmatic formation is rare without previous endovascular or surgical procedures. We present two rare cases of aortic aneurysm associated with aortic coarctation without previous corrective procedure in pediatric patients.

Reparo endovascular de aneurisma de aorta torácica: experiência de 16 casos / Stent-graft repair of thoracic aortic aneurism: experience of 16 cases

Os aneurismas dissecantes e as rupturas de aorta torácica têm sido de difícil resolução. O reparo endovascular de aneurisma de aorta torácica apresenta considerável potencial e vantagens sobre a abordagem cirúrgica como tratamento, devido em parte ao reparo cirúrgico estar associado a altas taxas de mortalidade. Relatar a experiência com uso de endopróteses auto-expansíveis no tratamento de pacientes selecionados, portadores de aneurismas ou dissecções comprometendo...

Aneurisma de aorta com ruptura para esôfago / Aortic aneurysm rupture into the esophagus

Apresentamos o caso de uma paciente portadora de aneurisma de aorta descendente com ruptura para o esôfago que, após aortoplastia com interposição de tubo de dacron e rafia da laceração esofágica, evoluiu com fístula esôfago pleural no terceiro dia pós-operatório. A paciente necessitou de reintervenção e cuidados intensivos, reabilitando-se adequadamente. A propósito deste caso incomum e do aprendizado adquirido no seu manejo, revisamos a literatura a fim de discutir a melhor alternativa de correção desta rara e, freqüentemente, fatal forma de apresentação das doenças da aorta.

We present the case of a patient with a descending aorta aneurysm rupture into the esophagus, which, after aortoplasty with Dacron tube interposition and suture of esophageal laceration, developed a pleural-esophagus fistula on the 3rd postoperative day. She needed re-intervention and intensive care, followed by adequate recovery. Considering this unusual case and the knowledge acquired through its management, we reviewed the literature in order to discuss the best alternative for the correction of this rare and often fatal form of presentation of aortic diseases.

Tratamiento endoluminal de disección aguda complicada en aorta descendente: Comunicación de un caso / Endoluminal stent-graft placement for acute dissection of the descending aorta complicated with rupture: Report of one case

Acute dissection of the aorta, although not common, has early and highly lethal complications. The type A dissection is treated with surgery. Patients with type B dissections are treated with surgery if they have complications like rupture, growth or visceral ischemia. Surgery, however, has complications such as spinal cord ischemia. Endovascular grafts have less mortality and complications. We report a 59 years old male patient with a type B dissection complicated with rupture. He was treated successfully with the placement of an endoluminal graft. He was discharged five days after the procedure in good conditions. After one year of follow up, the patient remains asymptomatic.

Aortic dissection with aortopulmonary artery fistula.

Rupture and dissection are the most dreaded complications of thoracic aortic aneurysm. Aortic dissection causing aortopulmonary artery fistulae is extremely rare. Here we report a case of chronic (type B) aortic dissection with fistulous communication between true lumen and main pulmonary artery.