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.

Resultados imediatos e a curto prazo do tratamento percutâneo do vazamento para protético / Immediate and short-term results of percutaneous leak treatment for prosthetic

INTRODUÇÃO: A regurgitação paraprotética (RPP) é um defeito que ocorre entre o local de sutura e o anel protético. A correção por transcateter tem surgido como uma opção à cirurgia para pacientes (pts) de alto risco. OBJETIVO: Avaliar os resultados imediatos e a curto prazo da oclusão da RPP por meio de cateter. MÉTODOS: Trata-se de um estudo prospectivo, de portadores de RPP, sintomáticos, submetidos a oclusão percutânea. O diagnóstico da RPP foi feito por meio da Doppler ecocardiografia TT e TE 3-D. O Critério de sucesso era: regurgitação residual discreta, melhora da classe funcional, regressão da hemólise e ausência de complicações fatais em até 30 dias. O critério de insucesso foi: complicações relacionadas ao procedimento, persistência de reflux moderado ou grave, hemólise, insuficiência cardíaca (IC) ou necessidade de cirurgia de urgência. RESULTADOS: Foram incluídos 20pts (24 próteses) com RPP, com média das idades de 61,5 anos, 11 pts femininos (55%). O número de cirurgias prévias variou de 1 a 4. A maioria (14) tinha 1 RPP, 5 tinham 2 e 1 tinha 4 orifícios regurgitantes. Cinco pts tinham prótese biológica (3 mitrais e 2 aórticas) e 15 próteses mecânicas (8 mitrais, 3 aórticas e 4 mitroaórticas). A indicação da intervenção foi a presença de IC com ou sem hemólise na maioria. A RPP foi grave em 16 pacientes, moderado em 3 e discreto em 1. A via de acesso percutâneo foi femoral em 17 pts. O dispositivo utilizado foi Amplatzer Vascular Plug, O número de plugs variou de 1a 4. Os pts foram distribuídos em 2 grupos: G1 (sucesso) e G2 (insucesso) segundo a definição acima. G1: o sucesso ocorreu em 13 dos 20pts (65%), 5 prótese biológica aórtica (100%) e 8 tinham prótese mecânica (53%) (5 mitrais e 3 aórticas). G2: insucesso ocorreu em 7 dos 20pts (35%), todos com prótese mecânica. Dois pts morreram antes da alta hospitalar (10%). Destes, um foi submetido a cirurgia de urgência por mal posicionamento do plug e o outro complicou com PCR e óbito. Cinco pts receberam alta hospitalar. Em uma evolução média de 14 meses, dos 13pts do G1, 3 morreram (1 acidente vascular cerebral hemorrágico, 1 infarto agudo do miocárdio e 1 IC descompensada); 10 estão evoluindo bem. Dentre os 5 pts do G2, 3 morreram, um continua em tratamento clínico e um foi operado. CONCLUSÃO: Em uma população de alto risco cirúrgico, a mortalidade relacionada a oclusão transcateter da RPP foi 10%. A resolução da RPP foi 65% e foi melhor nas próteses biológicas (100%) que nas mecânicas (53%).

Experiência inicial com o implante percutâneo da valva Venus-P valve® no trato de saída nativo pulmonar / Initial experience with percutaneous Venus-P Valve® valve implantation in the native pulmonary outflow tract

INTRODUÇÃO: O restabelecimento da função valvar pulmonar com implante percutâneo de próteses atualmente é realizado somente nos pacientes portadores de biopróteses, condutos ou homoenxertos posicionados na via de saída do VD. Próteses dedicadas para tratos de saída nativo estão sendo estudadas e implantadas com sucesso em diversos centros mundiais. OBJETIVOS: Apresentar a experiência inicial do implante percutâneo da VENUS-P Valve® em trato de saída nativo pulmonar. Serão demonstrados critérios de escolha dos pacientes, aspectos técnicos relacionados aos procedimentos e resultados de curto prazo. MATERIAIS E MÉTODOS: A prótese VENUS-P Valve® (Venus Medtech, Shanghai) é um stent de nitinol autoexpansível com uma valva de pericárdio porcino montada no seu interior. Possui aspecto de ampulheta e na extremidade distal o stent é de células abertas permitindo o fluxo para ambas artérias pulmonares. O seu sistema de entrega é 22 ou 24F. Os pacientes candidatos são avaliados com ecocardiografia transtorácica, angiotomografia, ressonância magnética e cateterismo cardíaco. São critérios de inclusão a presença de regurgitação pulmonar importante, com volumes diastólicos do VD>130ml/m2, sem estenoses na VSVD ou ramos pulmonares e trato de saída com no máximo 35mm de diâmetro após insuflação de cateter-balão medidor. O ecocardiograma transtorácico é o método padrão de escolha do diâmetro e comprimento da prótese. Todos os procedimentos foram realizados em sala híbrida, com anestesia geral endovenosa, profilaxia para endocardite bacteriana e heparina (TCA>250 seg). Os pacientes realizaram ecocardiografia transtorácica e avaliação clínica 24 horas e 30 dias após os procedimentos. RESULTADOS: Quatro pacientes (3 homens) foram submetidos ao implante percutâneo das valvas pulmonares em 2 dias consecutivos. O peso e idade médio foi de 62,2kg e 25,8 anos. A via de saída teve 30mm em média e a prótese utilizada apresentou diâmetro médio de 32mm. Três próteses tinham 25 mm de comprimento e a última 30mm. Houve sucesso no implante de todos os dispositivos com restabelecimento imediato da função valvar pulmonar. Uma prótese foi considerada em posição mais baixa no trato de saída porém sem interferência em estruturas cardíacas. Ao ecocardiograma de controle todos os dispositivos encontravam-se sem refluxo significativo, com fluxo preservado para as artérias pulmonares bilaterais e sem complicações relacionadas. Não houve complicações ou óbitos relacionados aos procedimentos. CONCLUSÕES: O restabelecimento da função valvar pulmonar em pacientes com trato de saída nativo com o implante percutâneo da VENUS-P Valve® mostrou-se uma excelente alternativa nesta experiência inicial. Os procedimentos são factíveis e seguros quando realizados por grupos experientes e familiarizados com o implante de stents na via de saída pulmonar. Por tratar-se de experiência inicial, acreditamos que um maior número de implantes e análise dos resultados tardios devem ser realizadas para incorporação definitiva destes dispositivos neste grupo selecionado de pacientes. (AU)

Impacto clínico e na qualidade de vida de pacientes submetidos ao implante transcateter da valva pulmonar Melody / Clinical impact and quality of life of patients undergoing transcatheter pulmonary valve implantation Melody

INTRODUÇÃO: O Implante transcateter da valva pulmonar (ITVP) constitui terapia consagrada para o reestabelecimento da via de saída do ventrículo direito (VD) em pacientes com disfunção grave de conduto VD-Tronco Pulmonar ou bioprótese pulmonar. No entanto, apesar de promover melhora do VD, muito pouco se sabe a sobre o impacto deste tratamento na qualidade de vida (QV). MÉTODOS: Ensaio clínico não-controlado, multicêntrico, de pacientes com cardiopatia congênita submetidos ao ITVP, com enfoque na avaliação do impacto clínico e na qualidade de vida após um ano do procedimento. Pacientes portadores de conduto VD-TP ou bioprótese pulmonar com disfunção grave e submetidos ao ITVP foram incluídos. Dados clínicos, ecocardiográficos (ECO), de teste cardiopulmonar (TCP) e ressonância nuclear magnética (RNM) foram obtidos antes do procedimento e novamente 1 ano após e comparados em sua média e desvio-padrão. Da mesma forma, foi avaliada a qualidade de vida através da aplicação do questionário SF-36 para os maiores de 18 anos e SF-10 para os menores de idade. RESULTADOS: De 17/12/2013 a 31/12/2017 foram incluídos 19 pacientes (11 masculinos), sendo 7 menores de 18 anos (5 masculinos). Na data do procedimento tinham idade de 20,9±7,1 anos e peso de 56,2±15,7Kg. O número de cirurgias prévias foi de 2,1±0,8 (1-4). Dez pacientes apresentavam predominantemente estenose pulmonar (53%), 4 insuficiência (21%) e 5 lesão mista grave...(AU)

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.

Use of covered stents in the management of coarctation of the aorta.

We report our experience with the use of covered stents for the management of coarctation of the aorta. From December 2001 to March 2004, nine patients (seven males; median age, 31 years; mean weight, 65 +/- 15 kg) underwent implantation. Indications included critical or atretic native coarctation (n = 4), patients >50 years of age (n = 2), associated patent ductus arteriosus (n = 1) or adjacent aneurysm (n = 1), and the presence of a circumferential fracture within a previously implanted stent (n = 1). The covered balloon-expandable Cheatham-Platinum stent and the self-expandable stent graft Braile were employed. Adequate implantation was observed in all patients. Gradients were reduced from 54 +/- 14 to 3 +/- 8 mmHg and the coarctation site increased from 2.4 +/- 2.9 to 15.9 +/- 4.3 mm. The patent ductus arteriosus was immediately closed and the aneurysm excluded. Two patients >35 years with aneurysmal ascending aorta and metallic aortic prosthesis had aneurysm formation at follow-up, with one undergoing aneurysm exclusion using a Braile stent. Although covered stents are useful in the management of selected patients with coarctation, aneurysm formation may still occur in patients with markers of aortic wall weakness. Refinements in the deployment technique and/or the stent design are needed to eliminate this risk.

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.

Aneurysm of the membranous septum in critical pulmonary stenosis: spontaneous rupture after balloon dilatation.

We report a case of critical pulmonary valve stenosis in which congenital aneurysm of the membranous septum ruptured spontaneously after balloon dilatation of the pulmonary valve. It is considered that the chronic mechanical trauma with phasic protrusion and collapse of the aneurysm during the cardiac cycles was responsible for an aneurysm rupture.

Percutaneous closure of ductus arteriosus with the amplatzer prosthesis. The Brazilian experience.

OBJECTIVE - To report the results of percutaneous occlusion of persistent ductus arteriosus with the Amplatzer prosthesis in 2 Brazilian cardiological centers. METHODS - From May 1998 to July 2000, 33 patients with clinical and laboratory diagnosis of persistent ductus arteriosus underwent attempts at percutaneous implantation of the Amplatzer prosthesis. The median age was 36 months (from 6 months to 38 years), and the median weight was 14kg (from 6 to 92kg). Sixteen patients (48.5%) were under 2 years of age at the time of the procedure. All patients were followed up with periodical clinical and echocardiographic evaluations to assess the presence and degree of residual shunt and possible complications, such as pseudocoarctation of the aorta and left pulmonary artery stenosis. RESULTS - The minimum diameter of the arterial ducts ranged from 2.5 to 7.0mm (mean of 4.0+/-1.0, median of 3.9). The rate of success for implantation of the prosthesis was 100%. Femoral pulse was lost in 1 patient. The echocardiogram revealed total closure prior to hospital discharge in 30 patients, and in the follow-up visit 3 months later in the 3 remaining patients. The mean follow-up duration was 6.4+/-3.4 months. All patients were clinically well, asymptomatic, and did not need medication. No patient had narrowing of the left pulmonary artery or of the aorta. No early or late embolic events occurred, nor did infectious endarteritis. A new hospital admission was not required for any patient. CONCLUSION - The Amplatzer prosthesis for persistent ductus arteriosus is safe and highly effective for occlusion of ductus arteriosus of varied diameters, including large ones in small symptomatic infants.

Transcatheter closure of atrial septal defects using the Cardio-Seal implant.

OBJECTIVE: To review the outcomes of transcatheter closure of atrial septal defects using the Cardio-Seal implant. DESIGN: A prospective interventional study. SETTING: Tertiary referral centre. PATIENTS: The first 50 patients (median age 9.7 years) who underwent attempted percutaneous occlusion. INTERVENTIONS: Procedures were done under general anaesthesia and transoesophageal guidance between December 1996 and July 1998. MAIN OUTCOME MEASURES: Success of deployment, complications, and assessment of right ventricular end diastolic diameter, septal wall motion, and occlusion status by echocardiography. RESULTS: The median balloon stretched diameter was 14 mm. Multiple atrial septal defects were present in 11 patients (22%) and a deficient atrial rim (< 4 mm) in 19 (38%). In four patients (8%), a second device was implanted after removal of an initially malpositioned first implant. There were no significant immediate complications. All patients except one were discharged within 24 hours. At the latest follow up (mean 9.9 months) a small shunt was present in 23 patients (46%), although right ventricular end diastolic dimensions (mean (SD)) corrected for age decreased from 137 (29)% to 105 (17)% of normal, and septal motion abnormalities normalised in all but one patient. No predictors for a residual shunt were identified. Supporting arm fractures were detected in seven patients (14%) and protrusion of one arm through the defect in 16 (32%), the latter being more common in those with smaller anterosuperior rims. No untoward effects resulted from arm fractures or protrusion. There were no complications during follow up, although five patients (10%) experienced transient headaches. CONCLUSIONS: The implantation of the Cardio-Seal device corrects the haemodynamic disturbances secondary to the right ventricular volume overload, with good early outcome.

Implantation of endovascular stents for hypoplasia of the transverse aortic arch.

Hypoplasia of the transverse aortic arch is commonly associated with aortic coarctation. Persistent or recurrent obstruction can occur at this level after successful repair of the native coarcted segment. The purpose of this report is to present a new technique to treat such lesions, namely with implantation of a balloon-expandable stent. This approach was used successfully in 4 children with such hypoplasia occurring after repair of coarctation. Implantation led to both anatomical and physiological relief of obstruction in all. The patients tolerated the procedure, and there were no major adverse events.

Antegrade transcatheter closure of coronary artery fistulae using vascular occlusion devices.

Two children (a 9 year old boy and a 2.5 year old girl) with coronary artery fistulae communicating with the right ventricle underwent successful transcatheter occlusion using an antegrade technique. A Rashkind double umbrella device was used in one case and an Amplatzer duct occluder in the other.

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.

Transcatheter embolization of congenital coronary arterial fistulas in adults.

In this report, we describe our experience with transcatheter occlusion of congenital coronary arterial fistulas in adults. From November 1992 to November 1996, 5 symptomatic patients, aged from 47 to 70 years, underwent transcatheter occlusion of fistulas using a retrograde arterial approach. All had chest pain or dyspnea on exertion. Detachable balloons were used in 4 patients, and Gianturco coils in 1. Detachable balloons were implanted through a Debrun system, while the coils were implanted through a 5 French right coronary Judkins catheter. Both were passed through an 8 French guiding catheter (Amplatz II). Each patient had a single fistula. The fistulas originated from the right coronary artery in 3 patients, and from the circumflex artery in 2. They drained into the pulmonary trunk in 3 patients, into the right atrium in 1, and into a bronchial artery in the other. All fistulas were occluded completely in the catheterization laboratory, and the procedures were uncomplicated. At follow up, 3 patients underwent coronary angiography, and there was no evidence of recanalization. Transcatheter embolization in adults of single congenital coronary fistulas with detachable balloons and coils is safe and effective and can be regarded as an acceptable alternative to surgery.

Stent implantation to create interatrial communications in patients with complex congenital heart disease.

A restrictive interatrial communication can complicate the management of complex congenital heart disease. The purpose of this report is to present a new technique to achieve a patent and reliable interatrial communication by using an endovascular stent. A stent was successfully implanted across a fenestrated extracardiac conduit in two patients with low cardiac output after Fontan operations and across the interatrial septum in a patient with double inlet left ventricle and severe left atrioventricular stenosis. The procedures were uncomplicated and all patients showed immediate hemodynamic improvement. Cathet. Cardiovasc. Intervent. 47:310-313, 1999.

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.

[Current role of therapeutic heart catheterization in pediatric cardiology] / O papel atual do cateterismo terapêutico em cardiologia pediátrica.

OBJECTIVE: Cardiac catheterization has begun to be employed for therapeutic purposes since the middle sixties. In this paper the authors review the current indications, technique and results related to each of the interventional procedures. It also includes a brief sample of the Instituto Dante Pazzanese experience for illustrative purpose. METHODS: The main procedures that are addressed include balloon and blade atrial septostomy (Rashkind and Park procedures), pulmonary valvuloplasty, aortic valvuloplasty, mitral valvuloplasty (for rheumatic mitral stenosis), angioplasty for coarctation of aorta with or without stent implantation, angioplasty for pulmonary artery stenosis with or without stent implantation, percutaneous occlusion of the patent ductus arteriosus, percutaneous occlusion of atrial septal defects and balloon dilation of stenosed Blalock-Taussig shunts. The authors make a review of the most important papers published in the literature about Interventional Pediatric Cardiology, including their own experience. RESULTS: Good or excelent therapeutic results are achieved for pulmonic stenosis, coarctation of aorta, patent ductus arteriosus, atrial septal defects and pulmonary branch stenosis. Satisfactory palliations are obtained for aortic stenosis, rheumatic mitral stenosis and complex congenital heart diseases that require balloon atrial septostomy. CONCLUSIONS: Due to the recent technological development, pediatric therapeutic heart catheterization makes it possible to treat or palliate about 30% of all congenital or acquired heart diseases with encouraging results, sparing some patients from the need of cardiac surgery.