Clinical and anatomopathological descriptions of dog with arterious Hemitruncus: case report / [Descrições clínicas e anatomopatológicas de cão com hemitruncus arterioso: relato de caso

The anomalous origin of the pulmonary trunk in the ascending aorta, defined as arterious hemitruncus, is a rare congenital malformation in dogs, caused by a defect in the spiral septum. Thus, given the unusual occurrence in the canine species, the systemic severity and the high lethality, the aim of this study was to describe this heart disease in a three-month-old male German Spitz puppy, emphasizing clinical changes of the necropsy and microscopics. The animal had cyanosis, dyspnea and weakness and was forwarded for necropsy after sudden death. Numerous changes were detected in the post-mortem examination, including in the heart, as cardiomegaly and absence of the arterial ligament, which was replaced by the complete fusion between the ascending aorta and the pulmonary trunk, after leaving both the left and right ventricles, respectively and, among the microscopic findings, cardiomyocyte hypertrophy stood out. The association of these findings with the history indicated the diagnosis of arterious hemitruncus followed by cardiorespiratory failure, emphasizing the importance of out complementary cardiological exams in young symptomatic patients for the survival of those affected. Arterious hemitruncus, although rare, must be added in the differential diagnosis of other heart diseases that cause similar clinical signs.(AU)

A origem anômala do tronco pulmonar em aorta ascendente, definida como hemitruncus arterioso, é uma malformação congênita rara em cães, causada por defeito no septo espiral. Assim, diante da ocorrência incomum na espécie canina, da gravidade sistêmica e da alta letalidade, o objetivo deste trabalho foi descrever essa doença cardíaca em um filhote de três meses de idade, macho, Spitz Alemão, enfatizando as alterações clínicas, de necropsia e microscópicas. O animal apresentava cianose, dispneia e fraqueza e foi encaminhado para necropsia após morte súbita. Inúmeras alterações foram detectadas no exame post-mortem, inclusive no coração, como cardiomegalia e ausência do ligamento arterioso, o qual foi substituído pela fusão completa entre aorta ascendente e tronco pulmonar, após a saída de ambas dos ventrículos esquerdo e direito, respectivamente, e, dentre os achados microscópicos, destacou-se a hipertrofia de cardiomiócitos. A associação desses achados com o histórico indicou o diagnóstico de hemitruncus arterioso seguido de insuficiência cardiorrespiratória, ressaltando-se a importância de exames complementares cardiológicos em pacientes jovens sintomáticos na sobrevida dos acometidos. O hemitruncus arterioso, apesar de raro, deve ser acrescido no diagnóstico diferencial de outras cardiopatias que causam sinais clínicos similares.(AU)

Antioxidative Propolis From Stingless Bees (Heterotrigona Itama) Preserves Endothelium-Dependent Aortic Relaxation of Diabetic Rats: The Role of Nitric Oxide and Cyclic Guanosine Monophosphate

Propolis from stingless bees (Heterotrigona itama) is a resinous compound that exhibits antihyperglycaemia, free radical scavenging, and cardioprotective properties. The effect of propolis on diabetic vessels has not been investigated. Thus, this research aimed to determine the effect of propolis supplementation on the level of antioxidants and its mechanism of action in the aorta of diabetic rats. Male Sprague-Dawley rats were divided into five groups (n=8/group): healthy (control), untreated diabetes (DM), metformin-treated diabetes (DM+M, 300 mg/kg/day metformin), propolis-treated diabetes (DM+P, 300 mg/kg/day propolis extract) and diabetes with combined treatment (DM+M+P, dosage as former). Oral supplementation was conducted for four weeks immediately upon successful induction of diabetes by streptozotocin (60 mg/kg, intraperitoneal injection). At the end of the study, the rats were euthanised, and thoracic aorta was processed into tissue homogenates to determine the levels of superoxide dismutase (SOD), catalase (CAT), glutathione peroxidase-1 (GPx-1) and soluble receptor for advanced glycation end-products (sRAGE). Aorta segments were harvested to examine their relaxation response towards graded concentration of acetylcholine (Ach; 10-8-10-4) M following precontraction with phenylephrine (PE; 10-6 M). Vasorelaxation towards a cumulative dose of propolis (0.01-1.00%) using PE-precontracted healthy aorta (n=6/experiments) was investigated under various simulated conditions: physiological buffer, L-NAME (10-4 M), methylene blue (10-5 M), indomethacin (10-5 M) and elevated glucose (25 mM). Propolis maintained antioxidative enzymes and sRAGE decoy molecules in the aortic tissue of the diabetic rats. The amelioration of diabetes-induced impairment of endothelium-dependent relaxation by propolis was mediated through the nitric oxide(NO)-cyclic guanosine monophosphate (cGMP) pathway. This non-clinical study reports vasoprotective property of propolis in diabetes mellitus.

Valor de la angiografía coronaria por tomografía computarizadaen el diagnóstico de las anomalías coronarias / Role of coronary computed tomography angiography for the diagnosis of coronary anomalies

Background: Coronary anomalies are rare heart diseases that can cause sudden cardiac death, especially in young individuals. Coronary computed tomography angiography delivers a three-dimensional view of excellent quality with information of the origin, course and anatomic relationships of the anomalous vessel, allowing differentiation between benign and malignant variants, entities that require different management and treatment. Aim: To show that coronary computed tomography angiography is the non-invasive technique of choice for the evaluation of a coronary anomaly due to its high diagnostic accuracy. Material and Methods: Between 2008 and 2014, 368 coronary computed tomography angiographies were carried out. Six (1.6%) patients had a coronary anomaly. Results: The age of these six patients ranged from 38 to 82 years (five were women). The coronary computed tomography angiography was requested due to cardiac symptoms (angina and dyspnea) in two patients, after a selective coronary arteriography to obtain additional information of the coronary anomaly in three patients, and after a cardiorespiratory arrest in one patient. Three patients had an anomaly of the right coronary artery, two patients had an anomaly of the left coronary artery and one patient had absent left main coronary artery. In five patients the coronary anomaly was malignant. Conclusions: Coronary computed tomography angiography is a robust diagnostic method to evaluate patients with suspected coronary anomalies.

Síndrome de hipoplasia de corazón izquierdo: experiencia de 10 años de un programa de etapificación quirúrgica / Hypoplastic left heart syndrome: 10 year experience with staged surgical management

Resumen El síndrome de hipoplasia de corazón izquierdo (SHCI) es una cardiopatía congénita con letalidad superior al 95%. La etapificación quirúrgica es la principal vía de tratamiento, y se inicia con la operación de Norwood; la sobrevida a largo plazo de los pacientes tratados es desconocida en nuestro medio. Objetivos 1) Revisar nuestra experiencia en el manejo de todos los pacientes con SHCI evaluados entre enero 2000 y junio 2010. 2) Identificar factores de riesgo de mortalidad quirúrgica. Pacientes y método Estudio retrospectivo de una única institución con una cohorte de pacientes con SHCI. Se revisan antecedentes clínicos, quirúrgicos, y registros de seguimiento. Resultados Se evaluaron 76 pacientes con SHCI; 9/76 tenían comunicación interauricular (CIA) restrictiva, y 8/76, aorta ascendente de < 2 mm; 65/76 fueron tratados: 77% tuvieron operación de Norwood con conducto entre ventrículo derecho y ramas pulmonares como fuente de flujo pulmonar, 17% Norwood con shunt de Blalock-Taussig, y 6% otra cirugía. La mortalidad en la primera etapa quirúrgica fue del 23%, y en operación de Norwood, del 21,3%. En el período 2000-2005 la mortalidad en la primera etapa quirúrgica fue del 36%, y entre 2005-2010, del 15% (p = 0,05). La sobrevida global fue del 64% a un año y del 57% a 5 años. Por análisis multivariado fueron factores de riesgo para mortalidad la presencia de aorta ascendente diminuta y CIA restrictiva. Conclusiones Nuestros resultados inmediatos y a largo plazo en la etapificación quirúrgica de SHCI son similares a la experiencia de grandes centros. Hay una mejoría en mortalidad operatoria en la segunda mitad de la serie. Se identifican factores de riesgo de mortalidad.

Abstract Hypoplastic left heart syndrome (HLHS) is a lethal congenital heart disease in 95% of non-treated patients. Surgical staging is the main form of treatment, consisting of a 3-stage approach, beginning with the Norwood operation. Long term survival of treated patients is unknown in our country. Objectives 1) To review our experience in the management of all patients seen with HLHS between January 2000 and June 2012. 2) Identify risk factors for mortality. Patients and method Retrospective analysis of a single institution experience with a cohort of patients with HLHS. Clinical, surgical, and follow-up records were reviewed. Results Of the 76 patients with HLHS, 9 had a restrictive atrial septal defect (ASD), and 8 had an ascending aorta ≤ 2 mm. Of the 65 out of 76 patients that were treated, 77% had a Norwood operation with pulmonary blood flow supplied by a right ventricle to pulmonary artery conduit, 17% had a Norwood with a Blalock-Taussig shunt, and 6% other surgical procedure. Surgical mortality at the first stage was 23%, and for Norwood operation 21.3%. For the period between 2000-2005, surgical mortality at the first stage was 36%, and between 2005-2010, 15% (P = .05). Actuarial survival was 64% at one year, and 57% at 5 years. Using a multivariate analysis, a restrictive ASD and a diminutive aorta were high risk factors for mortality. Conclusions Our immediate and long term outcome for staged surgical management of HLHS is similar to that reported by large centres. There is an improvement in surgical mortality in the second half of our experience. Risk factors for mortality are also identified.

Obstructive membrane in arch of aorta in a case of Shone’s complex.

Shone’s complex is a rare congenital heart disease consisting of multisite obstruction on the left side of the heart. The obstructive membrane in the arch of aorta is never described among these obstructions. We report echocardiographic findings in a patient with Shone’s complex with the obstructive membrane in the arch of aorta.

Aneurismas da Aorta Torácica: Avaliação Genética e de Imagem para Cirurgias Eletivas / Thoracic Aortic Aneurysm: Genetic and Image Evaluation for Elective Surgeries

Evidências mostram que variações genéticas de um único gene, denominadas “mutações genéticas”, predispõem indivíduos aos aneurismas e dissecções da aorta e seus ramos. Com a identificação dessas mutações, diretrizes sugerem o manuseio no diagnóstico, o momento ideal para a correção cirúrgica e a identificação de indivíduos sob alto risco de ruptura ou dissecção e suas respectivas famílias. Essas mutações podem se apresentar de forma “sindrômica”, com identificação fenotípica simples ou apresentação “familiar”. Nos indivíduos sem características fenotípicas, a ocorrência familiar da mutação genética deve ser investigada através da história familiar, exames de imagem e através de marcadores genéticos.

Evidence shows that genetic variations in a single gene called gene mutations predispose individuals to aneurysms and dissections of the aorta and its branches. With the identification of these mutations, guidelines suggest that diagnosis management is the idealtime for surgical correction and the identification of individuals at high risk of rupture or dissection and their families. These mutations may be present in a syndromic way with simple phenotypic identification or family presentation. In individuals without phenotypic characteristics, familial occurrence of genetic mutation should be investigated through the family history, imaging scansand through genetic markers.

Anomalous origin of coronary artery: taxonomy and clinical implication / Origem anômala da artéria coronária: taxonomia e implicação clínica

Objective: Anomalous origin of coronary artery is uncommon. The taxonomies of anomalous origin of coronary artery are inconsistent and complex. Conceptual and therapeutic debates remain. The aim of the present study is to reappraise the concept of anomalous origin of coronary artery and to discuss the potential hazards and treatment rationale of this anomaly on basis of literature review. Methods: A comprehensive literature review was made in terms of the taxonomies including “simple”, “multiple” and “complex” types of anomalous origin of coronary artery. Results: Anomalous origin of coronary artery can be simply categorized according to the ectopically originated coronary artery. There are a couple of complex anatomical variants: “multiple” type, involving more than one coronary artery or branch, which can be subdivided into 2 subtypes, A) more than one coronary arteries or branches arising from one place; and B) two coronary arteries/branches arising from separate ectopic sites; and “complex” type, associated with acquired heart disease, or congenital heart defects. Conclusion: Sudden cardiac death in anomalous origin of coronary artery is associated with the anatomical features including abnormal coursing, acute angle take-off and ostial abnormalities. Atherosclerosis is prone to be in the right-sided ectopic and retroaortic coursing coronary artery. Surgical treatment is a definitive therapy. Simple coronary artery bypass grafting is not recommended due to the potential hazards of coronary steal phenomenon and poor patency of mammary arterial grafts, and modified maneuvers such as coronary ostial reimplantation, impinged coronary segment unroofing and coronary stent deployment are advocated instead. .

Objetivo: A origem anômala da artéria coronária é incomum. As taxonomias de origem anômala da artéria coronária são inconsistentes e complexas. Os debates conceituais e terapêuticos permanecem. O objetivo do presente estudo é reavaliar o conceito de origem anômala da artéria coronária e discutir os riscos potenciais e fundamentos para o tratamento desta anomalia, com base em revisão de literatura. Métodos: A revisão da literatura foi feita com termos das taxonomias, incluindo origem anômala da artéria coronária “simples”, “múltipla” e “complexa”. Resultados: A origem anômala da artéria coronária pode ser simplesmente classificada de acordo com a origem ectópica da artéria coronária. Há um par de variações anatômicas complexas: “múltipla”, envolvendo mais de uma artéria coronária ou ramo, que podem ser subdivididos em dois subtipos: A) mais de uma das artérias coronárias ou ramos decorrentes de um lugar; e B) duas artérias coronárias/ramos decorrentes de sítios ectópicos separados; e tipo “complexo”, associado à doença cardíaca adquirida ou defeitos cardíacos congênitos. Conclusão: A morte súbita cardíaca de origem anômala da artéria coronária está associada com as características anatômicas incluindo curso anormal, descolamento de ângulo agudo e anormalidades ostiais. A aterosclerose é propensa a ocorrer na artéria coronária ectópica e retroaórtica, do lado direito. O tratamento cirúrgico é uma terapia definitiva. Revascularização do miocárdio simples não é recomendada devido aos riscos potenciais do fenômeno de “roubo” do fluxo coronário e patência ruim dos enxertos com a artéria torácica interna. Manobras modificadas como reimplante ostial coronário, destelhamento do segmento coronário impingido e implantação de stent coronário são defendidas em seu lugar. .

Aorto-left ventricular tunnel arising from the left coronary sinus of valsalva

Aortico-left ventricular tunnel is a rare congenital cardiac defect that bypasses the aortic valve via a para-valvular connection from the left ventricle to aorta. In most cases, the tunnel arises from the right aortic sinus. In this case report, we present a case of Aortico-left ventricular tunnel, in which the aortic orifice arose from the left aortic sinus, requiring special attention to avoid injuring the left coronary artery at the time of surgical repair.

Dilated ascending aorta is associated with the difficulty in correct placement of pulmonary artery catheter.

The present case report highlights that a tense mega-sized aortic root and ascending aorta can mechanically resist the passage of fully inflated (1.5 ml air) balloon to wedge-trace position in the pulmonary artery. Any attempt to push the catheter rather predisposed its recoiling and rebutting into the right ventricle and the cardiac arrhythmia. Inflating continuous cardiac output catheter balloon with lesser volume of air (1 ml) is suggested to overcome this problem.

Nova técnica: operação de Norwood com perfusão regional cerebral e coronariana / New technique: Norwood operation with regional cerebral and coronary perfusion

Objetivo: Avaliar o resultado imediato da operação de Norwood modificado com nova técnica de perfusão regional cerebral (PRCeA) anterógrada associado a perfusão regional coronariana (PRCoR) retrógrada em substituição à parada circulatória total com hipotermia profunda em crianças portadoras da Síndrome da Hipoplasia do Coração Esquerdo (SHCE) com aorta ascendente extremamente hipoplásica (AH). Métodos: No período de dezembro de 2006 a fevereiro de 2008, a operação de Norwood modificado com tubo entre o ventrículo direito e as artérias pulmonares ou shunt tipo Sano foi realizada em oito crianças portadoras de SHCE e aorta ascendente com diâmetro inferior a 3 mm, (quatro do sexo masculino e quatro do sexo feminino) com idade média de 9,2 dias (variando de 1 a 29 dias) e peso médio de 3,3 kg (variando de 2,7 a 3,8 kg). Utilizada CEC e hipotermia a 25ºC com duas cânulas venosas e anastomose de um enxerto de politetrafluoretileno com a artéria inominada utilizado como linha arterial e para PRCeA. A PRCoR foi realizada por meio de um desvio na linha arterial e colocação de um cateter na aorta ascendente. Foram analisados o resultado cirúrgico imediato e a presença de alterações neurológicas nesse período. Resultados: O resultado cirúrgico imediato revelou mortalidade de 25 por cento e ausência de comprometimento neurológico ao exame clínico. Conclusão: A operação de Norwood modificado pode ser realizada com PRCeA e PRCoR em crianças com SHCE e AH com resultado cirúrgico imediato satisfatório e ausência de complicações neurológicas.

Objective: To assess the immediate result of the modified Norwood procedure with new technique of anterograde regional cerebral perfusion (ARCeP) and retrograde regional coronary perfusion (RRCoP) in substitution of profound hypothermia and circulatory arrest in children with hypoplastic left heart syndrome (HLHS) with extremely hypoplastic ascending aorta (HA). Methods: In the period of December of 2006 to February 2008 the modified Norwood procedure with tube between the right ventricle and pulmonary arteries or Sano shunt was performed in eight children with HLHS and with ascending aorta diameter less than 3 mm, (four male and four female) with median age of 9.2 days (ranging from 1 to 29 days) and median weigh of 3.3 kg (ranging from 2.7 to 3.8 kg). We used cardiopulmonary bypass and hypothermia at 25ºC with two venous cannulas and suture with a polytetrafluoroethylene graft sutured to the innominate artery as an arterial line and used to ARCeP. The RRCoP was performed by a deviation in the arterial line and placement of a catheter in the ascending aorta. The immediate surgical results and the clinical neurological manifestation were assessed. Results: The immediate surgical results revealed mortality of 25 percent and there were absence of neurological injury at clinical examination. Conclusions: The modified Norwood procedure can be accomplished with ARCeP and RRCoP in children with HLHS with HA with satisfactory immediate surgical result and without neurological complications.

Placa de aterosclerose em aorta: revisão sobre aterogênese, formação de placa, significado clínico, métodos de imagens e tratamento / Plaque of atherosclerosis in aorta: review on atherogenesis, formation of plaque, clinical significance, methods of imaging and treatment

Há consenso na literatura de que o estagio mais precoce da aterogênese é caracterizado por acúmulo de células espumosas na região da íntima arterial. Fatores de risco como hipertensão arterial, tabagismo, diabetes mellitus, dislipidemias (hipercolesterolemia), sexo masculino e idade avançada predispõem à maior formação de placas em coronárias e aorta, nas quais tem sido observado maior número de eventos coronarianos agudos e acidentes vasculares cerebrais. Acidentes vasculares cerebrais são a terceira causa de mortes nos EUA, com aproximadamente 40 por cento dos casos de origem criptogênica. Desde 1989, as placas de ateroma, que se desenvolvem na aorta torácica, têm sido responsabilizadas por acidentes vasculares cerebrais e periféricos...

A Case of Acute Myocardial Infarction with the Anomalous Origin of the Right Coronary Artery from the Ascending Aorta above the Left Sinus of Valsalva and Left Coronary Artery from the Posterior Sinus of Valsalva

Coronary anomalies are rare angiographic findings. Moreover, there are few reports of cases of an anomalous origin of the right coronary artery from the left sinus of Valsalva and of the left coronary artery from the posterior sinus of Valsalva. Here, we report a case with an anomalous origin of the right coronary artery from the ascending aorta above the left sinus of Valsalva and the left coronary artery from the posterior sinus of Valsalva. This was observed in a patient who was treated for a myocardial infarction of the inferior wall caused by a thrombus in the proximal right coronary artery. The patient was treated successfully with the implantation of a stent in the anomalous origin of the right coronary artery using a 6Fr Amplatz left 1 catheter.

Percutaneous device closure of nonrestrictive aortopulmonary window in a 4 months' infant.

Aortopulmonary window is a rare congenital heart disease occurring in 0.2-0.6% of all patients with congenital heart disease. It is usually nonrestrictive and is conventionally treated surgically at an early age to prevent the development of pulmonary vascular obstructive disease. In 10% of the patients, it is restrictive and case reports of its percutaneous closure are mostly limited to these patients. We report percutaneous device closure of nonrestrictive AP window in a 4 months old, 4 kg child. To the best of our knowledge this is the youngest child to have undergone percutaneous device closure of an AP window.

Aortico-left ventricular tunnel: echocardiographic recognition.

Aortico-left ventricular tunnel (ALVT) is a rare congenital malformation characterized by an abnormal communication between aorta and left ventricular cavity and the child presents with features of congestive heart failure in early infancy. We report the case of a 7-day-old baby who was diagnosed as a case of ALVT by echocardiography. The child was operated successfully and echocardiographic findings were confirmed.

Anomalia congênita em vasos da base do coração de um potro / Congenit anomaly in vessels of base in a foal heart

Anomalias congênitas do coração e de grandes vasos estão entre as mais comuns encontradas em animais, porém são raras em eqüinos. Este relato descreve uma anomalia cardíaca que apresenta confluência entre a parede da aorta e do tronco pulmonar, ramificação da veia cava caudal no átrio esquerdo com presença de válvula e conseqüentemente posição errônea do coração na cavidade torácica de um potro da raça Quarto-de-Milha encontrado morto após o nascimento. O diagnóstico foi realizado pela associação dos achados macroscópicos durante a necrópsia e o exame histopatológico realizados no Curso de Medicina Veterinária- UNESP-Araçatuba.

Congenital anomalies of heart and large vessels are among more common found in animals, however rare in horses. This survey describes a cardiac anomaly that presents confluence between aorta wall and lung trunk, ramification of caudal cava vein in left atrium with presence of valve and consequently erroneous position of heart in thoracic cavity of one foal found dead after birth. The diagnostic was carried out for macroscopic finds association during the necropsy and histopathologic exams carried out at School of Veterinary Medicine– Unesp Araçatuba.