Tratamento endovascular de fístula tronco arterial braquiocefálico-traqueal: relato de caso e revisão de literatura / Endovascular management of tracheo-innominate artery fistula: a case report and literature review

A fístula do tronco arterial braquiocefálico-traqueal é uma complicação pouco frequente da traqueostomia, com incidência entre 0,1 e 1%, porém com alta mortalidade nos casos não tratados. Sinais precoces incluem desde sangramento autolimitado a hemorragia maciça com choque hipovolêmico. A espessura da cânula de traqueostomia, seu posicionamento junto à parede traqueal e a pressão do balonete traqueal podem lesionar a mucosa e precipitar seu desenvolvimento. Descrevemos o caso de paciente do sexo feminino, 14 anos, traqueostomizada aos oito anos após traumatismo craniencefálico. Evoluiu após esse período com estenose subglótica e necessidade de sessões de dilatação por seis anos. No quinto ano das sessões, apresentou hemoptise de repetição, inicialmente tratada por cirurgia com prótese de politetraflouroetileno expandido. Um ano depois, apresentou novo sangramento com repercussão hemodinâmica, controlada pela técnica endovascular, e nova cirurgia foi programada em caráter eletivo. Após a segunda cirurgia, houve seis meses de acompanhamento clínico ambulatorial, sem novas complicações

Tracheo-innominate artery fistula (TIF) is a rare complication of tracheostomy, with incidence ranging from 0.1 to 1%, but mortality is high in untreated cases. Early signs range from self-limited bleeding to massive hemorrhage with hypovolemic shock. The caliber of the tracheostomy cannula, its position in contact with the tracheal wall, and tracheal cuff pressure can traumatize the mucosa and trigger development of a TIF. We describe the case of a 14-year-old female patient who had been tracheostomized at the age of eight because of head trauma. She later developed subglottic stenosis requiring dilation sessions for six years. During the fifth year of these sessions, she presented repetitive hemoptysis, initially treated by surgery to implant an expanded polytetrafluoroethylene graft. One year later, she had an intense hemorrhage, which was controlled using endovascular techniques followed by definitive surgery, performed electively. The patient was followed up for six months, without complications

Origen anómalo de la arteria coronaria derecha desde el tronco de la arteria pulmonar (ARCAPA) / Anomalous origin of the right coronary artery originating from the pulmonary trunk; ARCAPA / Origem anômala da artéria coronária direita do tronco da artéria pulmonar; ARCAPA

Las anomalías de las arterias coronarias son una condición clínica de muy baja incidencia y de estas el origen anómalo de la arteria coronaria derecha desde el tronco de la arteria pulmonar (ARCAPA) representa cerca de 0,002% en la población general. Se puede asociar a la presencia de otras anomalías cardíacas congénitas. Presentamos el caso de una recién nacida, pretérmino de 33 semanas, gemelar, a la cual se le realiza el diagnóstico de un probable origen anómalo de coronaria derecha en un estudio ecocardiográfico de rutina realizado en la unidad de terapia intensiva. Se confirma el diagnóstico mediante cateterismo cardíaco y se realiza la corrección quirúrgica definitiva a los 6 meses de edad, estando la niña actualmente asintomática y con una calidad de vida normal. Destacamos la baja frecuencia de la ocurrencia de dicha patología, realizamos una revisión sobre los tópicos principales en el desarrollo del árbol vascular coronario y las principales anomalías del mismo. Jerarquizamos la importancia de realizar un estudio ecocardiográfico adecuado como valoración de pacientes internados en unidades de terapia intensiva neonatal.

Coronary artery anomalies are a clinical condition of very low incidence. Out of these, the anomalous origin of the right coronary artery from the trunk of the pulmonary artery (ARCAPA) is estimated to represent 0.002% of the general population. It may be associated with the presence of other congenital cardiac anomalies. The study presents the case of a 33 week preterm newborn twin who is diagnosed with an anomalous origin of the right coronary in a routine echocardiographic study performed in the Intensive Care Unit. The diagnosis is confirmed by cardiac catheterization and definitive surgical correction is performed at 6 months of age, the child being currently asymptomatic and enjoying a normal quality of life. We stand out the low frequency of the occurrence of this pathology. We performed a review of the main topics in the development of the coronary vascular tree and their main anomalies. We emphasize on the importance of performing an adequate Echocardiographic study as an assessment of patients admitted to Neonatal Intensive Care Units.

As anomalias das artérias coronárias representam uma condição clínica de incidência muito baixa; e a origem anômala da artéria coronária direita do tronco da artéria pulmonar (ARCAPA) representa somente uma estimativa de 0,002% na população geral. Pode estar associada à presença de outras anomalias cardíacas congênitas. Apresentamos o caso de um recém-nascido, prematuro de 33 semanas, gêmeo, diagnosticado com provável origem anômala de coronária direita em estudo ecocardiográfico de rotina realizado na Unidade de Terapia Intensiva. Confirmou-se o diagnóstico através de cateterismo cardíaco e realizou-se a correção cirúrgica definitiva aos 6 meses de idade; a doença atualmente é assintomática e a menina tem uma qualidade de vida normal. Ressaltamos a baixa frequência da ocorrência da referida patologia; realizamos uma revisão dos principais tópicos no desenvolvimento da árvore vascular coronariana e suas principais anomalias. Destacamos a importância de realizar um estudo ecocardiográfico adequado como estratégia de avaliação de pacientes internados em Unidades de Terapia Intensiva Neonatal.

Staged Repair of Truncus Arteriosus Associated with Complete Atrioventricular Septal Defect

We report a case of successful repair of truncus arteriosus (TA) associated with complete atrioventricular septal defect (c-AVSD) using a staged approach. TA associated with c-AVSD is a very rare congenital cardiac anomaly. No report of successful staged repair in South Korea has yet been published. We performed bilateral pulmonary artery banding when the patient was 33 days old, and total correction using an extracardiac conduit was performed at the age of 18 months. The patient recovered uneventfully and is doing well.

Role of perioperative transesophageal echocardiography in the management of adolescent truncus arteriosus: Rare case report.

Truncus arteriosus (TA) is a rare congenital heart disease defined as a single arterial vessel arising from the heart that gives origin to the systemic, pulmonary and coronary circulations. The truncal valve in majority of the cases is tricuspid though quadricuspid and bicuspid valves have been reported. Patients with TA typically have a large nonrestrictive sub truncal ventricular septal defect. Survival of these infants beyond 1‑year is uncommon. Here, we report a unique case of 12‑year‑old female patient with persistent TA who underwent surgical repair by using transesophageal echocardiography as a monitoring device during the perioperative management.

Persistent Truncus Arteriosus with Aortic Dominance in Female Adult Patient

Persistent truncus arteriosus categories associated with different natural histories and various surgical approaches were reported. Although pulmonary overflow and severe heart failure are common, some patients who have hypoplastic pulmonary artery systems may show lesser symptoms of heart failure and remain in relatively stable condition. We experienced a 33-year-old woman with uncorrected type II persistent truncus arteriosus who presented with cyanosis rather than congestive heart failure, and are presenting her images.

Prenatal Diagnosis of Chromosome 22q11.2 Deletions: Experiences in a Single Institution

PURPOSE: This study was designed to determine the frequency and echocardiographic findings of 22q11.2 deletions in fetuses with cardiac defects on fetal ultrasound or familial backgrounds of 22q11.2 deletions. MATERIALS AND METHODS: We retrospectively reviewed the medical and ultrasonographic records of 170 fetuses that underwent fluorescence in situ hybridization (FISH) analysis for chromosome 22q11.2 deletions between February 2001 and April 2013. RESULTS: Among 145 fetuses with cardiac defects, six (4.1%) had 22q11.2 deletions. Deletions of 22q11.2 were detected in 6 (5%) of the 120 fetuses with conotruncal defects: 5 (8.9%) of 56 with tetralogy of Fallot (TOF) and 1 (5.9%) of 17 with double outlet right ventricle (DORV). No deletions were found in cases of pulmonary atresia, truncus arteriosus, right aortic arch, or transposition of the great arteries. No 22q11.2 deletions were found in non-conotruncal cardiac malformations. Among 25 fetuses with familial backgrounds of 22q11.2 deletions, one (4%) had a maternally inherited 22q11.2 deletion with no cardiac findings. CONCLUSION: Knowledge of the frequency and echocardiographic findings of 22q11.2 deletions might be helpful for prenatal genetic counseling. It is advisable to perform FISH analysis for 22q11.2 deletions in pregnancies exhibiting conotruncal cardiac defects such as TOF or DORV.

Aneurisma de tronco arterial braquiocefálico asociado a erosión esternal. Reporte de un caso / Aneurisma de tronco arterial braquiocefálico associado a erosåo esternal. relato de um caso / Brachiocephalic arterial trunk aneurysm associated with sternal erosion. Report of a case

La aortitis sifilítica es frecuentemente asociada a aneurismas y/o enfermedad coronaria estenótica ostial, sin embargo este tipo de lesiones son raramente reportadas. Se reporta un caso deaneurisma de tronco arterial braquiocefálico con compresión y desplazamiento de estructuras vecinas,erosión esternal y su tratamiento...

A aortitis sifilítica é frequentemente associada a aneurisma e/ou enfermedade estenotica ostial, sem no entanto este tipo de lesão são raramente reportadas. Apresentamos um caso de aneurisma do tronco arterial braquiocefálico com compressão e deslocamento de estrcturas vizinhas, erosão esternal e seu tratamento...

Syphilitic aortitis is often associated with aneurysms and ostial stenotic coronary however such injuries are rarely reported. We report a case of brachiocephalic arterial trunk aneurysm with compression and displacement of neighboring structures, sternal erosion and its treatment...

Percutaneous Pulmonary Valve Implantation

Pulmonary regurgitation (PR) is a frequent sequelae after repair of tetralogy of Fallot, pulmonary atresia, truncus arteriosus, Rastelli and Ross operation. Due to patient growth and conduit degeneration, these conduits have to be changed frequently due to regurgitation or stenosis. However, morbidity is significant in these repeated operations. To prolong conduit longevity, bare-metal stenting in the right ventricular outflow tract (RVOT) obstruction has been performed. Stenting the RVOT can reduce the right ventricular pressure and symptomatic improvement, but it causes PR with detrimental effects on the right ventricle function and risks of arrhythmia. Percutaneous pulmonary valve implantation has been shown to be a safe and effective treatment for patients with pulmonary valve insufficiency, or stenotic RVOTs.

Truncus arteriosus operado aos 28 anos: importância do diagnóstico diferencial / Atypical truncus arteriosus operated at 28 years of age: importance of differential diagnosis / Truncus arteriosus operado a los 28 años: importancia del diagnóstico diferencial

Descrevemos um caso de adulto de 28 anos com suspeita de cardiopatia congênita desde o nascimento, não tratada na infância por opção da família. Aos 27 anos, foi feito diagnóstico de atresia pulmonar com comunicação interventricular e colaterais sistêmico-pulmonares, sendo contraindicada a cirurgia. Uma nova reavaliação em nosso serviço demonstrou tratar-se de um truncus arteriosus atípico. O fato de um tronco arterial comum com shunt esquerda-direita ter sido visualizado ao ecocardiograma foi um dado crucial para a indicação de novo cateterismo, abrindo perspectiva de correção cirúrgica. No momento, o paciente encontra-se bem, com 7 anos de evolução pós-operatória.

This is the case of 28 year-old adult with suspected congenital heart disease since birth, not treated in childhood at the his family's choice. At 27 years old, he was diagnosed with pulmonary atresia with ventricular septal defect and systemic-pulmonary collaterals, where surgery was contraindicated. A new review in our department showed that it was an atypical form of truncus arteriosus. The fact that a common arterial trunk with left-right shunt was viewed by echocardiography was a crucial fact for the indication of new catheterization, opening the prospect of surgical correction. Currently, the patient is well, with 7 years of postoperative outcome.

Describimos un caso de adulto de 28 años con sospecha de cardiopatía congénita desde el nacimiento, no tratada en la niñez por opción de la familia. A los 27 años, se hizo diagnóstico de atresia pulmonar con comunicación interventricular y colaterales sistémico-pulmonares, y se contraindicó la cirugía. Una nueva reevaluación en nuestro servicio reveló tratarse de un truncus arteriosus atípico. El hecho de un tronco arterial común con shunt izquierdaderecha haber sido visualizado al ecocardiograma fue un dato crucial para la indicación de nuevo cateterismo, abriendo perspectiva de corrección quirúrgica. Al momento, el paciente se encuentra bien, con 7 años de evolución posoperatoria.

Lesão não aterosclerótica do óstio do tronco da coronária esquerda / Non atherosclerotic left main coronary artery ostial lesion

Relata-se um caso de cardiopatia isquêmica como manifestação inicial da arterite de Takayasu (AT). Paciente internada para cirurgia de revascularização miocárdica(CRVM), devido à angina estável e lesão isolada do tronco de coronária esquerda, apresentava alterações vasculares ao exame físico que sugeriram o diagnóstico de AT. O presente caso leva à reflexão sobre a pesquisa de etiologias alternativas à doença aterosclerótica coronariana, especialmente em indivíduos jovens ou com poucos fatores de risco. O acometimento da artéria coronária na AT, embora infrequente se comparado a outras lesões vasculares encontradas nesta doença, determina elevada morbimortalidade.

Long Term Results of Right Ventricular Outflow Tract Reconstruction with Homografts

BACKGROUND: Homograft cardiac valves and valved-conduits have been available in our institute since 1992. We sought to determine the long-term outcome after right ventricular outflow tract (RVOT) reconstruction using homografts, and risk factors for reoperation were analyzed. MATERIALS AND METHODS: We retrospectively reviewed 112 patients who had undergone repair using 116 homografts between 1992 and 2008. Median age and body weight at operation were 31.2 months and 12.2 kg, respectively. The diagnoses were pulmonary atresia or stenosis with ventricular septal defect (n=93), congenital aortic valve diseases (n=15), and truncus arteriosus (N=8). Mean follow-up duration was 79.2+/-14.8 months. RESULTS: There were 10 early and 4 late deaths. Overall survival rate was 89.6%, 88.7%, 86.1% at postoperative 1 year, 5 years and 10 years, respectively. Body weight at operation, cardiopulmonary bypass (CPB) time and aortic cross-clamping (ACC) time were identified as risk factors for death. Forty-three reoperations were performed in thirty-nine patients. Freedom from reoperation was 97.0%, 77.8%, 35.0% at postoperative 1 year, 5 years and 10 years respectively. Small-sized graft was identified as a risk factor for reoperation. CONCLUSION: Although long-term survival after RVOT reconstruction with homografts was excellent, freedom from reoperation was unsatisfactory, especially in patients who had small grafts upon initial repair. Thus, alternative surgical strategies not using small grafts may need to be considered in this subset.

Dissecção isolada do tronco celíaco: relato de caso / Isolated dissection of the celiac artery: a case report

A dissecção espontânea isolada do tronco celíaco sem a dissecção concomitante da aorta é uma condição rara, pouco descrita na literatura. O objetivo do presente trabalho é descrever um caso dessa entidade clínica em um paciente masculino, 74 anos, assintomático, cujo diagnóstico foi feito por ultrassonografia e confirmado com angiotomografia computadorizada. O paciente tem sido acompanhado com sucesso mediante observação clínica por um período de 25 meses.

The isolated spontaneous dissection of the celiac artery without the concomitant dissection of the aorta is a rare condition seldomly described in the literature. The objective of the present study is to describe a case of this clinical entity in a 74-year-old, male patient, who was asymptomatic, and whose diagnosis was established by means of ultrasound and confirmed using computed angiotomography. The patient has been successfully followed up by means of clinical management for a period of 25 months.

Doença de tronco de coronária esquerda: avanços recentes no diagnóstico e tratamento / Disease of left main coronary artery: recent advances in diagnosis and treatment

Desde a sua primeira descrição, em 1912, por James Herrick, em paciente jovem com infarto do miocárdio e choque cardiogênico, o acometimento aterosclerótico obstrutivo do tronco de coronária esquerda representa um desafio para cardiologistas clínicos, cirurgiões e intervencionistas. Os riscos associados a essa lesão são conhecidos desde os primórdios da angiografia, quando se percebeu que pacientes com obstruções coronárias nesse local apresentavam maior mortalidade no seguimento clínico. Desde essa época, recomenda-se que a revascularização miocárdica cirúrgica seja o tratamento de escolha em pacientes com lesões angiograficamente obstrutivas no tronco de coronária esquerda. Diversos avanços ocorridos no âmbito da cardiologia nas últimas décadas têm contribuído para importantes mudanças no diagnóstico, prognóstico e tratamento de portadores de lesões de tronco. O emprego do ultrassom intracoronário e da reserva fracionada de fluxo para avaliação de lesões ambíguas nessa localização, o incremento do tratamento farmacológico com antiplaquetários e estatinas, e o surgimento de stents farmacológicos constituem alguns desses avanços...

Diagnosis and management of cyanotic congenital heart disease: part I.

Most commonly encountered cyanotic cardiac lesions in children, namely, tetralogy of Fallot, transposition of the great arteries and tricuspid atresia are reviewed. Pathology, pathophysiology, clinical features, non-invasive and invasive laboratory studies and management are discussed. The clinical and non-invasive laboratory features are sufficiently characteristic for making the diagnosis and invasive cardiac catheterization and angiographic studies are not routinely required and are needed either to define features, not clearly defined by non-invasive studies or as a part of catheter-based intervention. Surgical correction or effective palliation can be undertaken with relatively low risk. However, residual defects, some requiring repeat catheter or surgical intervention, are present in a significant percentage of patients and therefore, continued follow-up after surgery is mandatory.

Microdeletion study in children with selective congenital heart disease; an Iranian multicenter study

Determining the frequency of chromosome 22q11.2 microdeletion in children with congenital cardiac conotruncal abnormalities using Fluorescence in-situ Hybridization [FISH] technique and estimating relation between DiGeorge Syndrome and cardiac conotruncal abnormalities. One-hundred and eighty cases [106 Males, 74 Females] with selective congenital heart disease [conotruncal abnormalities] referred to the hospitals affiliated to Tehran University during 2004-2007 were evaluated by pediatric cardiologists. All patients were assessed for chromosome 22q11.2 microdeletion using FISH technique. Consequently, patients with 22q microdeletion were studied for T cell abnormalities. Median age of the patients at the time of study was 18 months [3d-16y]. The microdeletion of chromosome 22q11.2 was detected in 17 [9.5%] patients with conotruncal abnormalities, including 5 [29.4%] Tetralogy of Fallot plus Supravalvular Pulmonary Stenosis, 4 [23%] Truncus Arteriosus, 5 [29.4%] Pulmonary Artesia with Ventricular Septal Defect, 2 [11.8%] CO AO+Intrrupted Aortic Arch and one case of Valvular Pulmonary Stenosis. Five of uncorrelated cases had crananiofacial dysmorphism. Chromosome 22q11.2 microdeletion FISH study should be considered in patients with cardiac lesions particularly conotruncal abnormality with or without syndromic problems [craniofacial dysmorphism and developmental delay] to provide an appropriate genetic counseling with more accurate estimation of recurrence risk and ultimately prenatal diagnosis in affected families

Clinical profile and outcome of cyanotic congenital heart disease in neonates

To determine the clinical profile and assess the outcome of all neonates diagnosed with cyanotic congenital heart disease. A case series. The Aga Khan University Hospital from January 1998 to December 2000. Neonates admitted with diagnosis of cyanotic congenital heart disease were evaluated for clinical diagnosis, survival and mortality. Forty four neonates met the inclusion criteria. Eleven babies [25%] had Tetralogy of Fallot or its variants. Other malformations were d-transposition of great arteries, tricuspid valve anomalies [tricuspid atresia and Ebstein`s anomaly], hypoplastic left heart syndrome, truncus arteriosus, total anomalous pulmonary venous return and complex congenital heart disease like single ventricle. Twenty eight [63.6%] neonates survived and 16 [36.4%] expired during hospital stay. Cause of death was surgical in 2 cases and medical problems in 14 babies. Tetralogy of Fallot or variants was the commonest cyanotic heart disease in neonates with frequency of 27.27%. Majority of neonates with congenital cyanotic heart disease showed survival with appropriate management

Replacement of Obstructed Extracardiac Conduits with Autologous Tissue Reconstructions (Peel operation); Early and Midterm Results / 대한흉부외과학회지

BACKGROUND: Reoperation is usually required for a right ventricle to pulmonary artery conduit obstruction caused by valve degeneration, conduit peel formation or somatic growth of the patient. An autologous tissue reconstruction (peel operation), where a prosthetic roof is placed over the fibrotic tissue bed of the explanted conduit, has been used to manage conduit obstructions at our institute since May 2002. Herein, the early and midterm results are evaluated. MATERIAL AND METHOD: Between May 2002 and July 2006, 9 patients underwent obstructed extracardiac conduit replacement with an autologous tissue reconstruction, at a mean of 5.1 years after a Rastelli operation. The mean age at reoperation was 7.5+/-2.4 years, ranging from 2.9 to 10.1 years. The diagnoses included 6 pulmonary atresia with VSD, 2 truncus arteriosus and 1 transposition of the great arteries. The preoperative mean systolic gradient was 88.3+/-22.2 mmHg, ranging from 58 to 125 mmHg. The explanted conduits were all Polystan valved pulmonary conduit (Polystan, Denmark). A bioprosthetic valve was inserted in 8 patients, and a monocusp ventricular outflow patch (MVOP) was used in 1 patient. The anterior wall was constructed with a Gore-Tex patch (n=7), MVOP (n=1) and bovine pericardium (n=1). Pulmonary artery angioplasty was required in 5 patients and anterior aortopexy in 2. The mean cardiopulmonary bypass time was 154 minutes, ranging from 133 to 181 minutes; an aortic crossclamp was not performed in all patients. The mean follow-up duration was 20 months, ranging from 1 to 51 months. All patients were evaluated for their right ventricular outflow pathway using a 3-D CT scan. RESULT: There was no operative mortality or late death. The mean pressure gradient, assessed by echocardiography through the right ventricular outflow tract, was 20.4 mmHg, ranging from 0 to 29.6 mmHg, at discharge and 26 mmHg, ranging from 13 to 36 mmHg, at the latest follow-up (n=7, follow-up duration >1 year). There were no pseudoaneurysms, strictures or thrombotic occlusions. CONCLUSION: A peel operation was concluded to be a safe and effective re-operative option for an obstructed extracardiac conduit following a Rastelli operation.