Ablação septal com radiofrequência em paciente com obstrução dinâmica na via de saída do ventrículo esquerdo associado a pós operatório de cardiopatia congênita: relato de caso / Septal ablation with radiofrequency in a patient with dynamic obstruction in the left ventricular outflow tract associated with postoperative congenital heart disease: case report

INTRODUÇÃO: A obstrução da via de saída do ventrículo esquerdo (VSVE) é uma condição frequentemente descrita nos casos de cardiomiopatia hipertrófica obstrutiva (CMHO), gerando comprometimento da classe funcional, além de associação com morte súbita. Em pós- -operatório de cardiopatias congênitas com comprometimento da VSVE, podemos observar mecanismo fisiopatológico semelhante aos da CMHO. Atualmente é crescente a aplicação de medidas menos invasivas com objetivo terapêutico, como a ablação endocárdica por radiofrequência (RF) do septo interventricular, que apresenta resultados promissores, sendo uma alternativa à miectomia cirúrgica da VSVE. METODOLOGIA: estudo longitudinal do tipo estudo de caso. RELATO DO CASO: Escolar de 8 anos, portadora de síndrome de Williams, com diagnóstico de base de estenose subvalvar e supravalvar aórtica e ectasia da raiz aórtica, foi submetida aos 6 anos de idade, à ressecção de membrana subaórtica, miectomia da VSVE e ampliação da aorta ascendente. No ano subsequente ao procedimento cirúrgico, evoluiu com sinais de baixo débito cardíaco, associado a achado ecocardiográfico de obstrução dinâmica da VSVE, gerando gradiente sistólico máximo (GSM) inicial de 85mmHg, chegando, até período periprocedimento a 135mmHg. Inicialmente, realizado manejo medicamentoso com betabloqueador, porém paciente manteve sintomatologia a despeito da otimização farmacológica. Desta forma, levando em consideração risco cirúrgico e comportamento anatômico e fisiopatológico, associado a exames de imagem, como tomografia computadorizada com reconstrução tridimensional, optado por realização de procedimento de ablação septal por radiofrequência. Realizadas aplicações de RF em região de maior espessamento. Através da via retroaórtica, foram realizadas aplicações de RF, observando-se modificação do gradiente ecocardiográfico, com GSM final da VSVE de 45 mmHg. Paciente evoluiu com 16 dias após procedimento, com GSM de 13mmHg, ausência de sinais de baixo débito cardíaco e redução significativa da terapia medicamentosa com betabloqueador. Segue atualmente em acompanhamento clínico e ecocardiográfico regular. CONCLUSÕES: O tratamento intervencionista proposto para essa condição parece ser mais seguro. A ablação endocárdica por RF é um procedimento eficaz, seguro em longo prazo, que reduz o tempo de internação hospitalar e morbimortalidade quando comparada à abordagem cirúrgica, com melhora significativa ecocardiográfica e clínica, parecendo ser uma alternativa promissora nas lesões obstrutivas associadas ao pós-operatório de cardiopatias congênitas.

Paediatric and congenital heart disease in South America: an overview.

Congenital heart diseases are one of the most common structural defects present at birth, with an approximate incidence of 8 per 1000 live births. As most countries in South America have a high birth rate, they are a significant public health concern. This paper provides a brief overview of the burden of congenital heart disease in South America, focusing on its local prevalence, facilities for treatment and outcomes after medical, surgical or catheter intervention for the most common diseases.

Cardiac function assessment in patients with family history of nonhypertrophic cardiomyopathy: a prenatal and postnatal study.

Nonobstructive cardiomyopathies (CMs) may be familial in 20â"55% of cases. Little is known about the role of fetal echocardiography in such cases. We evaluated the cardiac function serially pre- and postnatally in cases with a family history of nonobstructive CM. The fetal and postnatal studies were performed in the echocardiogarphy laboratory at a tertiary institution. Twenty-six cases from 16 families with a family history of CM were studied. Three fetal echocardiograms were performed at or near 18, 25, and 32 weeks of gestation for complete cardiac functional assessment. Postnatally clinical evaluation, electrocardiogram, and an echocardiogram were performed within the first 3 months, with serial reevaluation for those identified with CM. The mean follow-up was 46 +/- 9 months. Abnormal cardiac function was observed in 8 cases (30%). Six had a previously affected sibling, 1 had other family members affected, and 1 had both antecedents. Four had dilated CM diagnosed prenatally of which 1 recovered, 2 died in utero, and 1 died soon after birth. The remaining 4 had normal fetal echoes and were diagnosed with CM in the first 3 months of life. Three had dilated CM with recovery, and 1 had restrictive CM requiring cardiac transplantation. This study demonstrates a high familial recurrence rate of CM. Fetal echo is useful for early diagnosis, although a normal study does not preclude ventricular dysfunction at a later stage, justifying serial prenatal and postnatal evaluation. Early identification may expedite listing for transplantation.

Left ventricle to coronary sinus fistula complicating the outcome of transposition of the great arteries.

We report a case in which a neonate with transposition of the great arteries and intact ventricular septum with unrestricted atrial communication had persistent hypoxemia probably due to a congenital left ventricle to coronary sinus fistula.

New percutaneous techniques for perforating the pulmonary valve in pulmonary atresia with intact ventricular septum.

We report new percutaneous techniques for perforating the pulmonary valve in pulmonary atresia with intact ventricular septum, in 3 newborns who had this birth defect. There was mild to moderate hypoplastic right ventricle, a patent infundibulum, and no coronary-cavitary communications. We succeeded in all cases, and no complications related to the procedure occurred. The new coaxial radiofrequency system was easy to handle, which simplified the procedure. Two patients required an additional source of pulmonary flow (Blalock-Taussig shunt) in the first week after catheterization. All patients had a satisfactory short-term clinical evolution and will undergo recatheterization within 1 year to define the next therapeutic strategy. We conclude that this technique may be safely and efficiently performed, especially when the new coaxial radiofrequency system is used, and it may become the initial treatment of choice in select neonates with pulmonary atresia and intact ventricular septum.

Indications, technique, results and clinical impact of reocclusion procedures for residual shunts after transcatheter closure of the patent ductus arteriosus.

PURPOSE: To assess the results of reocclusion procedures for late residual shunts after transcatheter closure of the patent ductus arteriosus. MATERIAL AND METHODS: Nineteen patients underwent reocclusion procedures either with Rashkind technique (2 patients) or with coils. Mean age was 96.5 months and mean interval between the initial and the additional procedure was 15.7 months. In 5 patients, the duct was balloon-dilated prior to occlusion. RESULTS: In 3 patients the procedure was unsuccessful because it was impossible to cross the duct using either a guide wire or a catheter. Immediate or late complete occlusion occurred in 13 patients. Two patients required a third procedure for complete closure. One patient developed severe intravascular hemolysis after coil implantation and was sent to surgery. The rate of final late residual shunting decreased from 17.1% to 6.6% (5/76) in the umbrella group and from 15.8% to 1.8% (1/56) in the coil group. CONCLUSIONS: Reocclusion procedures are indicated after one year of persistent shunting following the primary procedure. They are generally effective and reduce significantly the rate of late residual shunting. In patients with tiny shunts, the duct may need to be dilated. Coils are preferred because of their low cost.

Percutaneous closure of atrial septal defects. The role of transesophageal echocardiography.

PURPOSE: Evaluation of the role of transesophageal echocardiography in percutaneous closure of atrial septal defects (ASD) with the Amplatzer septal occluder. METHODS: Patients were selected for percutaneous closure of ASD by transesophageal echocardiography (TEE), which was also used to monitor the procedure, helping to select the appropriate size of the Amplatzer device, to verify its position, and to access the immediate results of the procedure. During the follow-up, TEE was used to evaluate the presence and magnitude of residual shunt (RS), device position, and right cardiac chamber diameters. RESULTS: Twenty-two (40%) of a total of 55 studied patients were selected. Thirteen underwent Amplatzer device implantation, eight are still waiting for it, and one preferred the conventional surgical treatment. All procedures were successful, which was mainly due to proper patient selection. Six (23%) patients acutely developed RS, which spontaneously disappeared at the three-month follow-up examination in three patients. There was a significant reduction in the right ventricle diastolic diameter, from 27 mm (average) to 24 mm and 20 mm, one and three months after the procedure, respectively (p < 0.0076). CONCLUSION: With the aid of TEE, percutaneous closure of ASD can be successfully, safely, and effectively performed.

Multiple atrial septal defects and patent ductus arteriosus: successful outcome using two Amplatzer septal occluders and Gianturco coils.

We report on a case of successful percutaneous closure of two atrial septal defects and a persistent ductus arteriosus using two Amplatzer septal occluders and a Gianturco coil in a 9-year-old child.

Hemolysis associated with coil occlusion of the arterial duct.

Severe mechanical hemolysis occurred in a 22 month old girl after placement of a 38-5-10 coil in the arterial duct. She had previously undergone percutaneous closure using the Rashkind technique 14 months before insertion of the coil, but remained with a moderate residual shunt. Surgical removal of the devices and division of the duct were required to abolish the hemolysis.

[Initial experience in percutaneous closure of interatrial communication with the amplatzer device]. / Experiência inicial no fechamento percutâneo da comunicação interatrial com a prótese de Amplatzer.

PURPOSE: To evaluate our initial experience with percutaneous closure of secundum type atrial septal defects (ASD) with the Amplatzer septal occluder. METHODS: Seven patients underwent occlusion by anterograde approach, under general anesthesia and transesophageal echocardiography (TEE) guidance. One child had 2 ASD and a patent ductus arteriosus (PDA). The ASD size ranged from 8.7 to 20 mm as measured by TEE. A transthoracic echocardiogram was performed in the morning after the procedure. RESULTS: Eight devices were successfully implanted in 7 patients and the PDA was occluded with a Gianturco coil at the same session. In this patient, there was an episode of supraventricular tachycardia during the occlusion of one ASD which was reverted with adenosin. All patients were discharged the day after, with complete occlusion of all defects. CONCLUSION: The procedure is safe, effective and versatile. It can be applied as an initial alternative to the treatment of selected patients with ASD.

[Overall experience with percutaneous occlusion of patent ductus arteriosus]. / Experiência global no fechamento percutâneo do canal arterial.

PURPOSE: To evaluate the overall experience of our institution with percutaneous occlusion of the patent ductus arteriosus. METHODS: Since December/92, 150 patients underwent 178 procedures (85-Rashkind technique; 87-Gianturco coils; 6-Duct Occlud). Median age was 6.5 years (1 to 57). Mean minimum ductal diameter was 3.05 +/- 1.24 mm (1 to 8). Clinical and echocardiographic evaluations were performed periodically. RESULTS: Adequate implantation was achieved in 143 (95.3%) patients. Prevalence of immediate residual shunting was 52.1%, falling to 15.9% during follow-up. This figure decreased to 5.2% after new additional procedures. Umbrella and coil embolization occurred in 1 and 12 procedures, respectively. Hemolysis and mild stenosis of left pulmonary artery were observed in one patient each. There was no mortality. CONCLUSION: This therapeutic modality is safe and efficacious with good follow-up results.

[Coronary sinus atrial septal defect, ventricular septal defect and absence of left superior vena cava]. / Comunicação interatrial do tipo seio coronário, comunicação interventricular e ausência de veia cava superior esquerda.

We report a rare case of a 21 month old child with a coronary sinus atrial septal defect associated with perimembranous ventricular septal defect and no left superior vena cava. The diagnosis was made by transthoracic echocardiogram and confirmed by angiography. The patient was operated on uneventfully, both defects were closed with bovine pericardial patches and the flow from the coronary veins was directed towards the left atrium. An echocardiogram revealed complete closure of both defects.

[Simultaneous treatment of pulmonary valve stenosis and patent ductus arterious by interventional catheterization]. / Tratamento simultâneo da estenose pulmonar valvar e da persistência do canal arterial através do cateterismo intervencionista.

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 concial 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.

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

[The percutaneous closure of patent ductus arteriosus with the Rashkind technic. The initial experience of the Instituto Dante Pazzanese de Cardiologia, São Paulo, Brazil]. / Cierre percutáneo del conducto arterioso con la técnica de Rashkind. Experiencia inicial del Instituto Dante Pazzanese de Cardiología-São Paulo-Brasil.

The authors report their experience with the percutaneous closure of the patent ductus arteriosus with the Rashkind technique. They performed 42 procedures with a success of 97.6% and one embolization episode. The mean age was 8.7 +/- 6.7 years, the mean weight was 25.0 +/- 15.8 Kg and the minimum diameter of the ductus was 4.0 +/- 1.3 mm. The immediate overall prevalence of residual shunt was 48.8% falling to 21% in a mean follow-up of 17 +/- 13 months in 29 cases. Two patients underwent late reocclusion: one with a new umbrella and the other with a coil. Another patient had an immediate severe residual shunt and it was reoccluded in the same procedure with a coil. The anatomical type and minimum internal diameter of the ductus, age and weight, size of the umbrella and the rotation of the umbrella were not predictors of residual shunt. There were no deaths, hemolysis, endarteritis or fracture of the umbrella's arms. One patient remained with a slight gradient in the left pulmonary artery. The authors conclude that the Rashkind technique is safe and efficient and has good long term results. It should be considered as the first line therapeutic modality in the treatment of selected patients with persistent ductus arteriosus.

[Pulmonary atresia with intact ventricular septum. Valvar perforation by radiofrequency]. / Atresia pulmonar com septo ventricular íntegro. Perfuração valvar por radiofreqüência.

Six days old neonate with pulmonary atresia and intact ventricular septum is reported in which a communication was established between the right ventricle and the pulmonary artery. The membraneous atresia was perforated with a special radiofrequency catheter 2F connected to a Hat 200S radiofrequency generator (Osypka). The energy necessary to perforation was 15W. After perforation of the atresia, balloon dilation was performed (balloon diameter: 2, 3, 4mm of coronary angioplasty and 5, 8mm Penta Medi-tech). The valve was completely opened giving the continuity between right ventricle and pulmonary artery.