Left main coronary artery compression by the right pulmonary artery in a patient with congenital pulmonic stenosis / Compresión del tronco coronario izquierdo por la arteria pulmonar derecha en un paciente con estenosis pulmonar congénita

Abstract Congenital pulmonary stenosis (PS) can be associated with pulmonary artery (PA) dilatation. In some cases, this can cause compression of nearby structures including the left main coronary artery (LMCA). This compression causes angina and is considered an indication for surgical treatment. We present the case of a patient with PS and angina secondary to LMCA compression by the right PA and review the main indications and options for surgical treatment.

Resumen La estenosis pulmonar congénita se asocia a dilatación de la arteria pulmonar. En algunos casos esto puede causar compresión de las estructuras adyacentes incluyendo el tronco de la coronaria izquierda. Esta compresión causa angina y es considerada una indicación para tratamiento quirúrgico. Presentamos el caso de un paciente con estenosis pulmonar y angina secundaria a compresión del tronco de la coronaria izquierda por la arteria pulmonar derecha y revisamos las indicaciones y opciones de tratamiento quirúrgico.

Henoch-Schonlein purpura secondary to infective endocarditis in a patient with pulmonary valve stenosis and a ventricular septal defect

No abstract available.

Discordância atrioventricular associada à dupla via de saída do ventriculo direito: um raro caso com 28 anos e nenhuma cirurgia / Atrioventricular discordance and double outlet right ventricle: a rare case of a 28 year old man without surgery

Relatamos o raro caso de um paciente de 28 anos, com diagnóstico de discordância atrioventricular e dupla via de saída do ventrículo direito, obtido somente após o ecocardiograma transesofágico e sem cirurgia prévia onde a estenose pulmonar importante atuou como bandagem pulmonar natural, possibilitando essa evolução.

We report a rare case of a 28 years old patient with the diagnosis of atrioventricular discordance and double outlet right ventricle, obtained only after transesophageal echocardiogram and no surgery. Severe pulmonary stenosis was the natural banding allowing this outcome.

Valvoplastia percutânea com balão no tratamento da estenose valvar pulmonar: seguimento clínico em longo prazo / Percutaneous baloon valvoplasty in the treatment of pulmonary valve stenosis: long term follow up

Fundamentação: A Valvoplastia Percutânea com Balão (VPB) é tratamento de escolha na estenose pulmonar (EP). Objetivo: Mostrar os resultados da VPB em um seguimento de até 15 anos. Pacientes e Métodos: Trezentos e dezesseis pacientes com EP foram submetidos a 323 VPB, entre setembro de 1.984 e novembro de 2.000. O sucesso foi definido como a redução e/ou manutenção do gradiente VD-AP em níveis inferiores a 36mmHg e a reestenose quando houvesse nova elevação para níveis iguais ou superiores a 36mmHg após a VPB, no cateterismo e/ou ecodopplercardiograma. A média de idade foi 7,36 ± 9,0 anos. Resultados: Imediatamente após a VPB, o gradiente pico a pico transvalvar reduziu de 70,6 ± 30,3 para 25,36 ± 19,75mmHg (p< 0,001). Dos 316 pacientes, houve 58 perdas, ocorrendo um óbito. Foram acompanhados 257 pacientes e 262 procedimentos. O seguimento médio foi de 5,5 ± 3,84 anos até um máximo de 16,45 anos. Houve sucesso imediato em 207 (79 por cento) e 51 (21 por cento) foram insatisfatórios. Houve 14 (4,33 por cento) complicações, incluindo um óbito por laceração anular e hemopericárdio. Houve reestenose em 9,92 por cento e algum grau de regurgitação pulmonar foi observado em 95,1 por cento, geralmente pequeno. A probabilidade de manutenção de bom resultado com a VPB sem reestenose foi de 96,5 por cento (1 ano), 95,3 por cento (2 anos), 93 por cento (5 anos), 91,3 por cento (8 anos), 87,4 por cento (10 anos) e 78,9 por cento (15 anos). Conclusão: A VPB é um procedimento efetivo, com manutenção dos resultados, no longo prazo e baixo índice de complicações.

Background: Balloon valvuloplasty (BPV) is the treatment of choice for pulmonary stenosis (PS). Objective: The main goal of this study is to To show BVP effectiveness in a 15-year follow-up period. Pacients and Methods: Three hundred and sixteen patients with PS were submitted to 323 BVP between September/84 and November/2000. Success was defined as reduction and/or maintenance of the RV-PA gradient at levels below 36 mmHg in catheterism or echocardiography. Results: Mean age was 7.36 ± 9 years. Results: Immediately after BVP, the transvalvar peak-to-peak gradient was reduced from 70.6 ± 30.3 to 25.36 ± 19.75 mmHg (p<0.001). Pulmonary valvuloplasty was considered successful in 207 (79 percent). The mean follow-up was 5.5 ± 3.84 years, up to a maximum of 16.45 years. There were 14 (4.33 percent) complications and one death due to annular laceration and hemopericardium. Restenosis occurred in 9.92 percent and a small degree of pulmonary regurgitation was observed in 95.1 percent. The probability of maintaining good results free of restenosis was 96.5 percent (1 year), 95,3 percent (2 years), 93 percent (5 years), 91,3 percent (8 year), 87,4 percent (10 years) e 78,9 percent (15 years). Conclusion: The PVB is an effective method for treating PVS and its beneficial effects persist in long-term follow-up. The complications are infrequent and usually mild cases.

Isolated Left Ventricular Apical Hypoplasia with Infundibular Pulmonary and Aortic Stenosis: a Rare Combination

Isolated left ventricular (LV) apical hypoplasia is a rare congenital cardiac anomaly which is not accompanied by other cardiac abnormalities, with the exception of two cases. We report a case of a 33-year-old male patient with isolated LV apical hypoplasia combined with infundibular pulmonary stenosis and aortic stenosis. We review a literature focusing on the characteristic magnetic resonance features and combined cardiac abnormalities.

Cateterismo intervencionista na estenose valvar pulmonar crítica do recém-nascido e na atresia pulmonar com septo interventricular íntegro: 13 anos de experiência de um serviço terciário / Interventional catheterization in neonatal critical pulmonary valve stenosis and pulmonary atresia with intact ventricular septum: 13 years of experience at a tertiary service

Introdução: A abordagem percutânea é opção de escolha em neonatos portadores de estenose pulmonar crítica (EP) e atresia pulmonar com septo interventricular íntegro (APSI). Neste trabalho são descritos casos tratados consecutivamente em centro de referência e seu seguimento a médio prazo. Métodos: A maioria dos pacientes recebeu infusão endovenosa de prostaglandina pré-procedimento, independentemente da necessidade hemodinâmica. Na abordagem da APSI, foi utilizada guia de ponta rígida e, mais recentemente, valvotomia com cateter de radiofrequência. Na maioria dos casos, foi realizada dilatação sequencial com balão até atingir 110 a 120 do diâmetro do anel valvar pulmonar. Resultados: Entre 1998 e 2011, 17 neonatos com APSI (idade mediana de 5 dias, peso de 3,1 ± 0,6 kg) e 30 neonatos com EP (idade mediana de 12 dias, peso médio de 3 ± 1,4 kg) foram tratados em um serviço terciário. Na APSI, 14 pacientes foram abordados com guia rígida, com sucesso de 71,4, e 3 com radiofrequência, com sucesso de 100. Na EP, o sucesso foi alcançado em todos os casos. O óbito hospitalar foi de 23,5 no grupo com APSI e de 3,3 no grupo com EP, nenhum relacionado ao procedimento percutâneo. Na evolução a médio prazo, a taxa de reintervenção por reestenose foi de 21,4 no grupo com APSI e de 10 no grupo com EP. Conclusões: A valvoplastia pulmonar na EP e na APSI apresenta resultados clínicos e hemodinâmicos aceitáveis, desde que se observem características anatômicas favoráveis e se mantenha a patência do fluxo pulmonar até o procedimento. A morbidade e a mortalidade dos portadores de EP crítica são mais baixas que as de portadores de APSI.

BACKGROUND: The percutaneous approach is the therapy of choice in neonates with critical pulmonary stenosis (PS) and pulmonary atresia with intact interventricular septum (PAIVS). Consecutive cases treated at a reference center and their midterm follow-up was reported in this study. METHODS: Most of the patients received an intravenous infusion of prostaglandin preoperatively, regardless of the need. For the PAIVS approach, a stiff-tip guidewire was used and more recently, radiofrequency valvotomy. In most cases, sequential balloon dilation was performed until 110% to 120% of the pulmonary valve ring diameter was reached. RESULTS: Between 1998 and 2011, 17 neonates with PAIVS (median age of 5 days, mean weight of 3.1 + 0.6 kg) and 30 neonates with PS (median age of 12 days, mean weight of 3 + 1.4 kg) were treated at a tertiary center. In PAIVS patients, a stiff-tip guidewire was used in 14 cases, with a success rate of 71.4%, and radiofrequency perforation in 3 cases, with a success rate of 100%. In PS patients, success was achieved in all of the cases. Hospital death was 23.5% for the PAIVS group and 3.3% for the PS group, none related to the percutaneous procedure. In the midterm follow-up, the reintervention rate due to restenosis was 21.4% in the PAIVS group and 10% in the PS group. CONCLUSIONS: Pulmonary valvuloplasty in PS or PAIVS has acceptable clinical and hemodynamic results, as long as favorable anatomic characteristics are observed and patent pulmonary flow is maintained until the procedure. Morbidity and mortality of patients with critical PS are lower than patients with PAIVS.

Perfuração cardíaca durante cateterismo em um lactente com estenose valvar pulmonar: resultados satisfatórios pela abordagem híbrida / Cardiac perfuration during catheterization in an infant with pulmonary valve stenosis: successful outcomes by a hybrid approach

Relatamos o caso de um lactente de 10 meses, submetido a uma tentativa mal sucedidade de valvoplastia pulmonar com balão (VPB) devido à perfuração cardíaca. A criança foi subsequentemente tratada por esternotomia mediana, sutura do sítio de perfuração no átrio direito e VPB intra-operatória por meio de uma bainha colocada na via de saída do ventrículo direito.

We report a case in which a 10-month-old infant underwent an unsuccessful attempt at percutaneous pulmonary balloon valvuloplasty (PBV) due to cardiac perforation. The child was subsequently managed by median sternotomy, suture of the bleeding site in the right atrium and intraoperative PBV through a sheath placed in the right ventricular outflow tract.

Síndrome de Ehlers-Danlos associada a anomalias de artérias pulmonares e sistemicas / Ehlers-Danlos syndrome associated by anomalies of systemic and pulmonare arteries

A síndrome de Ehlers-Danlos é um distúrbio hereditário raro do tecido conjuntivo resultando em grande flexibilidade das articulações, hérnia inguinal, aumento da elasticidade da pele e lesões do sistema cardiovascular, envolvendo o coração e artérias sistêmicas e pulmonares. Neste artigo relata-se o caso de um menino com diagnóstico prévio da síndrome que em avaliação pré-operatória de hérnia inguinal tinha sintomas de dispnéia e sopro, ao ecocardiograma Doppler foram observados sinais de estenoses do ramo esquerdo da artéria pulmonar. A angiografia pulmonar confirmou anomalias significativas e foi realizada angioplastia, entretanto, o paciente voltou a ter sintomas sendo então realizado tratamento cirúrgico. O paciente evoluiu com melhora clínica significativa e modificação do padrão de fluxo das artérias pulmonares avaliado pela ecocardiografia Doppler.

M-shaped doppler signal across ventricular septal defect: potential implications for estimation of right ventricuilar systolic pressure.

An 8-year-old child suffering from ventricular septal defect and severe valvular pulmonary stenosis was evaluated by echo-Doppler technique and cardiac catheterization. A peak instantaneous transventricular systolic gradient of 64 mmHg was recorded across the ventricular septal defect with an interesting M-shaped spectral pattern. However, cardiac catheterization revealed a peak-to-peak non-simultaneous gradient between the right and the left ventricle of only 14 mmHg. This discrepancy along with its implications are discussed in this report.

Concurrent percutaneous atrial septal defect closure and pulmonary valvuloplasty.

Severe pulmonary valve stenosis in association with a large atrial septal defect is uncommon. Simultaneous percutaneous closure of the atrial septal defect and pulmonary valvuloplasty appears to be an attractive modality when compared to patients with these two lesions being subjected to surgery or being treated percutaneously at different sittings. We encountered two cases, both with significant pulmonary valvular stenosis associated with atrial septal defect (fossa ovalis type). Both lesions were successfully treated percutaneously in the same sitting. Pulmonary valvuloplasty was done initially and the atrial septal defect closed later to minimize catheter manipulation after device implantation.

Treatable cardiomyopathies.

Cardiomyopathy is defined as primary myocardial dysfunction which is not due to hypertensive, valvular, congenital, coronary or pulmonary vascular disease. This term usually denotes a dismal prognosis short of cardiac transplantation. However, several organic diseases of the heart can result in right or left ventricular dysfunction resulting in congestive heart failure and prompting the physician to label them as cardiomyopathy; the etiological factor is overlooked as it produces very subtle features. Therefore, before labelling any child as cardiomyopathic, all possible causes of ventricular dysfunction must be excluded by clinical and investigative means. The causes of "treatable cardiomyopathy" include mechanical factors as critical aortic stenosis and pulmonic stenosis, severe coarctation of aorta in an infant and aortaarteritis is an older child. Some of the persistent arrhythmias like atrial tachycardia, fibrillation, paroxysmal junctional re-entrant tachycardia are also known for causing ventricular dysfunction producing tachycardiomyopathy. Treatment of arrhythmia improves the ventricular function. Myocardial ischemia as a result of congenital coronary anomaly (commonest being anomalous origin of left coronary artery from pulmonary artery) can also present with a cardiomyopathy like picture. Early surgical correction is very rewarding. Finally, some of the metabolic conditions like creatinine and thiamine deficiency can also produce ventricular dilatation and dysfunction. In conclusion, the so called cardiomyopathy like picture can be produced because of several reasons and an attempt must be made to identify them.

Tratamento simultâneo da estenose pulmonar valvar e da persistência do canal arterial através do cateterismo intervencionista / Simultaneous Treatment of Pulmonary Valve Stenosis and Patent Ductus Arteriosus by Interventional Cardiology

A associaçäo entre estenose pulmonar valvar e persistência do canal arterial näo é freqüente na prática clínica da cardiologia pediátrica. Säo descritos dois casos de portadores dessa associaçäo, tratados simultaneamente, em um mesmo procedimento, através do cateterismo intervencionista: 1§ caso, criança de 14 meses submetida a valvoplastia pulmonar percutânea com queda no gradiente sistólico entre a artéria pulmonar e o ventrículo direito de 71 para 3mmHg. O canal arterial, de 2,0mm de diâmetro e do tipo cônico foi ocluído com um coil 38-5-5; 2§ caso, criança de 20 meses submetida a valvoplastia pulmonar percutânea com queda no gradiente sistólico entre a artéria pulmonar e o ventrículo direito de 60 para 5mmHg. Tentativa de oclusäo do canal, de 3,5mm de diâmetro e do tipo cônico, utilizando-se coil foi realizada sem sucesso devido a embolizaçäo para a artéria pulmonar. O dispositivo foi imediatamente resgatado e uma umbrella de 12mm foi implantada com sucesso. Discute-se a indicaçäo de tratamento das condiçöes, aspectos técnicos e complicaçöes relativas ao procedimento e seguimento dos pacientes.

The association of pulmonary valve stenosis and patent ductus arteriosus is uncommonly found in clinical practice of pediatric cardiology. The authors describe 2 patients with this association that were successfully treated in the same procedure by interventional cardiology: first case, a 14 month old boy was submitted to percutaneous pulmonary valvoplasty with reduction of systolic gradient between pulmonary artery and right ventricle from 71 to 3 mmHg. The 2.0 mm of diameter and conical shape duct was successfully occluded using a 38- 5-5 coil; second case, a 20 month old girl was submitted to percutaneous pulmonary valvoplasty with reduction of systolic gradient between pulmonary artery and right ventricle from 60 to 5 mmHg. An attempt to close the 3.5 mm of diameter and conical shape duct using coil was performed, but the device embolized into the pulmonary artery. After immediate retrieval, a 12 mm Rashkind umbrella was implanted. Indication of treatment of both conditions, technical aspects and complications related to the procedures, and patients follow up are also discussed

Single lung Rastelli repair.

A four year old severely cyanotic child was diagnosed to have double outlet right ventricle, large subpulmonic ventricular septal defect, severe pulmonic valve stenosis with absent left pulmonary artery and bilateral superior vena cavae without any connecting vein. A Rastelli type repair was performed utilizing the right pulmonary artery as the only run-off for the right ventricle. Immediate post-pump right ventricular (RV) pressures were 90 percent of the systemic. Six months later, echocardiographically estimated RV pressure was 60 percent of the systemic. The child is doing well at 6 months follow-up.