Guidelines on the Diagnosis and Treatment of Hypertrophic Cardiomyopathy ­ 2024 / Diretriz sobre Diagnóstico e Tratamento da Cardiomiopatia Hipertrófica ­ 2024

Hypertrophic cardiomyopathy (HCM) is a form of genetically caused heart muscle disease, characterized by the thickening of the ventricular walls. Diagnosis requires detection through imaging methods (Echocardiogram or Cardiac Magnetic Resonance) showing any segment of the left ventricular wall with a thickness > 15 mm, without any other probable cause. Genetic analysis allows the identification of mutations in genes encoding different structures of the sarcomere responsible for the development of HCM in about 60% of cases, enabling screening of family members and genetic counseling, as an important part of patient and family management. Several concepts about HCM have recently been reviewed, including its prevalence of 1 in 250 individuals, hence not a rare but rather underdiagnosed disease. The vast majority of patients are asymptomatic. In symptomatic cases, obstruction of the left ventricular outflow tract (LVOT) is the primary disorder responsible for symptoms, and its presence should be investigated in all cases. In those where resting echocardiogram or Valsalva maneuver does not detect significant intraventricular gradient (> 30 mmHg), they should undergo stress echocardiography to detect LVOT obstruction. Patients with limiting symptoms and severe LVOT obstruction, refractory to beta-blockers and verapamil, should receive septal reduction therapies or use new drugs inhibiting cardiac myosin. Finally, appropriately identified patients at increased risk of sudden death may receive prophylactic measure with implantable cardioverter-defibrillator (ICD) implantation.

La miocardiopatía hipertrófica (MCH) es una forma de enfermedad cardíaca de origen genético, caracterizada por el engrosamiento de las paredes ventriculares. El diagnóstico requiere la detección mediante métodos de imagen (Ecocardiograma o Resonancia Magnética Cardíaca) que muestren algún segmento de la pared ventricular izquierda con un grosor > 15 mm, sin otra causa probable. El análisis genético permite identificar mutaciones en genes que codifican diferentes estructuras del sarcómero responsables del desarrollo de la MCH en aproximadamente el 60% de los casos, lo que permite el tamizaje de familiares y el asesoramiento genético, como parte importante del manejo de pacientes y familiares. Varios conceptos sobre la MCH han sido revisados recientemente, incluida su prevalencia de 1 entre 250 individuos, por lo tanto, no es una enfermedad rara, sino subdiagnosticada. La gran mayoría de los pacientes son asintomáticos. En los casos sintomáticos, la obstrucción del tracto de salida ventricular izquierdo (TSVI) es el trastorno principal responsable de los síntomas, y su presencia debe investigarse en todos los casos. En aquellos en los que el ecocardiograma en reposo o la maniobra de Valsalva no detecta un gradiente intraventricular significativo (> 30 mmHg), deben someterse a ecocardiografía de esfuerzo para detectar la obstrucción del TSVI. Los pacientes con síntomas limitantes y obstrucción grave del TSVI, refractarios al uso de betabloqueantes y verapamilo, deben recibir terapias de reducción septal o usar nuevos medicamentos inhibidores de la miosina cardíaca. Finalmente, los pacientes adecuadamente identificados con un riesgo aumentado de muerte súbita pueden recibir medidas profilácticas con el implante de un cardioversor-desfibrilador implantable (CDI).

A cardiomiopatia hipertrófica (CMH) é uma forma de doença do músculo cardíaco de causa genética, caracterizada pela hipertrofia das paredes ventriculares. O diagnóstico requer detecção por métodos de imagem (Ecocardiograma ou Ressonância Magnética Cardíaca) de qualquer segmento da parede do ventrículo esquerdo com espessura > 15 mm, sem outra causa provável. A análise genética permite identificar mutações de genes codificantes de diferentes estruturas do sarcômero responsáveis pelo desenvolvimento da CMH em cerca de 60% dos casos, permitindo o rastreio de familiares e aconselhamento genético, como parte importante do manejo dos pacientes e familiares. Vários conceitos sobre a CMH foram recentemente revistos, incluindo sua prevalência de 1 em 250 indivíduos, não sendo, portanto, uma doença rara, mas subdiagnosticada. A vasta maioria dos pacientes é assintomática. Naqueles sintomáticos, a obstrução do trato de saída do ventrículo esquerdo (OTSVE) é o principal distúrbio responsável pelos sintomas, devendo-se investigar a sua presença em todos os casos. Naqueles em que o ecocardiograma em repouso ou com Manobra de Valsalva não detecta gradiente intraventricular significativo (> 30 mmHg), devem ser submetidos à ecocardiografia com esforço físico para detecção da OTSVE.   Pacientes com sintomas limitantes e grave OTSVE, refratários ao uso de betabloqueadores e verapamil, devem receber terapias de redução septal ou uso de novas drogas inibidoras da miosina cardíaca. Por fim, os pacientes adequadamente identificados com risco aumentado de morta súbita podem receber medida profilática com implante de cardiodesfibrilador implantável (CDI).

Late Follow-up of Patients Submitted to Total Cavopulmonary Derivation: Clinical Aspects, Reinterventions, and Complications Interfering in Morbidity and Mortality.

OBJECTIVE: To identify main complications in outpatient follow-up, as well as factors before or during operation that may interfere in patient's evolution. METHODS: Retrospective study of patients submitted to total cavopulmonary shunt with extracardiac conduit from 2000 to 2014 at the Hospital do Coração (São Paulo, Brazil) and who underwent clinical follow-up at this institution. RESULTS: One hundred and fifty surgeries were performed and 59 patients maintained outpatient follow-up. The mean age of these patients at the time of surgery was 4.45 years (median of 45 months) and 70.2% of them were males. Among the patients undergoing outpatient follow-up, postoperative time at evaluation ranged from 10 days to 145 months; 30 (50.8%) patients had single left ventricle and 29 (49.2%) had single right ventricle (48.2% of these presented with hypoplastic left heart syndrome [HLHS]). Patients with single left ventricle had a higher percentage of reintervention-free survival, but without statistically significant difference. 40% of the patients had no complications and 35% of them presented with thrombosis at some point in the follow-up period, with ventricular dysfunction being the second most frequently found complication (15% of cases), mainly among patients with single right ventricle morphology (P=0.04). Between the patients currently under follow-up, 20 (35%) of them had been evaluated by ultrasonography and had some degree of hepatic congestion and/or hepatomegaly. 16.7% of the patients with such alteration had HLHS (P=0.057). CONCLUSION: Except for the right ventricular morphology, no other factor has been shown to interfere in late evolution after total cavopulmonary shunt.

Late follow-up of patients submitted to total cavopulmonary derivation: clinical aspects, reinterventions, and complications interfering in morbidity

Abstract Objective: To identify main complications in outpatient follow-up, as well as factors before or during operation that may interfere in patient's evolution. Methods: Retrospective study of patients submitted to total cavopulmonary shunt with extracardiac conduit from 2000 to 2014 at the Hospital do Coração (São Paulo, Brazil) and who underwent clinical follow-up at this institution. Results: One hundred and fifty surgeries were performed and 59 patients maintained outpatient follow-up. The mean age of these patients at the time of surgery was 4.45 years (median of 45 months) and 70.2% of them were males. Among the patients undergoing outpatient follow-up, postoperative time at evaluation ranged from 10 days to 145 months; 30 (50.8%) patients had single left ventricle and 29 (49.2%) had single right ventricle (48.2% of these presented with hypoplastic left heart syndrome [HLHS]). Patients with single left ventricle had a higher percentage of reintervention-free survival, but without statistically significant difference. 40% of the patients had no complications and 35% of them presented with thrombosis at some point in the follow-up period, with ventricular dysfunction being the second most frequently found complication (15% of cases), mainly among patients with single right ventricle morphology (P=0.04). Between the patients currently under follow-up, 20 (35%) of them had been evaluated by ultrasonography and had some degree of hepatic congestion and/or hepatomegaly. 16.7% of the patients with such alteration had HLHS (P=0.057). Conclusion: Except for the right ventricular morphology, no other factor has been shown to interfere in late evolution after total cavopulmonary shunt.

Incidence and Treatment of Chylothorax in Children Undergoing Corrective Surgery for Congenital Heart Diseases.

INTRODUCTION: Chylothorax is a lymphatic extravasation into pleural cavity and its incidence is 0.25%-5.3% in children undergoing cardiac surgery. OBJECTIVE: To evaluate the incidence of chylothorax in pediatrics patients operated, linking it in each surgical intervention. Evaluate treatment types and efficiency. METHODS: Retrospective study using medical records of children undergoing cardiac surgery in the Hospital do Coração between 2004 and 2014. For statistical analysis, qualitative variables by absolute frequency and relative frequency; quantitative variables, by median of 25 and 75 percentiles, as they did not present normal distribution (Shapiro-Wilk, P<0.05). The Chi-square test was used for the association between type of treatment and result. The adopted confidence level was 95%. RESULTS: Incidence of chylothorax was 2.1% (0.9% in intracardiac surgery, 1.7% correction of patent ductus arteriosus and aortic coarctation, 8.3% Glenn's surgery, 11.8% total cavopulmonary surgery and 3% in others). Among treatments, fasting associated with total parenteral nutrition (TPN) resolved 51% of the cases. Hypoglossal diet had failed treatment and surgical referral in 22% of the cases. Fasting with TPN associated with octreotide had success in the treatment of chylothorax in a period exceeding 15 days in 78% of cases, and 3.7% were referred for surgery. CONCLUSION: According to the results, incidence of chylothorax was 2.18%. Treatment with fasting and TPN leads to resolutions in 86.5%, and the association with octreotide was successful in 85.1% of cases, showing an efficient option, while the treatment with hypoglossal diet had therapeutic failure in 22% of the cases in which it was used.

Incidence and treatment of chylothorax in children undergoing corrective surgery for congenital heart diseases

Abstract Introduction: Chylothorax is a lymphatic extravasation into pleural cavity and its incidence is 0.25%-5.3% in children undergoing cardiac surgery. Objective: To evaluate the incidence of chylothorax in pediatrics patients operated, linking it in each surgical intervention. Evaluate treatment types and efficiency. Methods: Retrospective study using medical records of children undergoing cardiac surgery in the Hospital do Coração between 2004 and 2014. For statistical analysis, qualitative variables by absolute frequency and relative frequency; quantitative variables, by median of 25 and 75 percentiles, as they did not present normal distribution (Shapiro-Wilk, P<0.05). The Chi-square test was used for the association between type of treatment and result. The adopted confidence level was 95%. Results: Incidence of chylothorax was 2.1% (0.9% in intracardiac surgery, 1.7% correction of patent ductus arteriosus and aortic coarctation, 8.3% Glenn's surgery, 11.8% total cavopulmonary surgery and 3% in others). Among treatments, fasting associated with total parenteral nutrition (TPN) resolved 51% of the cases. Hypoglossal diet had failed treatment and surgical referral in 22% of the cases. Fasting with TPN associated with octreotide had success in the treatment of chylothorax in a period exceeding 15 days in 78% of cases, and 3.7% were referred for surgery. Conclusion: According to the results, incidence of chylothorax was 2.18%. Treatment with fasting and TPN leads to resolutions in 86.5%, and the association with octreotide was successful in 85.1% of cases, showing an efficient option, while the treatment with hypoglossal diet had therapeutic failure in 22% of the cases in which it was used.

Avaliação das cardiopatias congênitas no adulto: ecocardiografia, ressonância e tomografia / Evaluation of grown-up congenital heart disease: echocardiography, mri and computed tomography

Avanços recentes na cardiologia pediátrica permitem que uma população crescente com cardiopatias congênitas chegue à idade adulta, muitos deles necessitando de seguimento por toda a vida. Um elemento chave desse seguimento é a avaliação seriada com métodos diagnósticos por imagem. O cardiologista pediátrico moderno dispõe atualmente de um vasto leque de opções não invasivas para a investigação desses pacientes, das quais se destaca como método principal a ecocardiografia e, como duas técnicas complementares, a ressonância magnética e a tomografia computadorizada. Neste artigo, faremos uma breve revisão dos princípios, indicações, vantagens e desvantagens de cada um destes métodos, em especial na investigação de indivíduos adultos com cardiopatias congênitas

Recent advances in pediatric cardiology allow an increasing population with congenital heart defects to reach adulthood, many of them requiring lifelong follow-up. A key element of this follow-up is serial evaluation by diagnostic imaging methods. The modern pediatric cardiologist currently has a wide range of noninvasive options for the investigation of these patients, the Echocardiogram stands out as the main tool, and as two complementary techniques, Magnetic Resonance and Computed Tomography. In this article we will briefly review the principles, indications, advantages and disadvantages of each of these methods, especially in the investigation of adult individuals with congenital heart diseases

Mid-Term Outcomes after Percutaneous Closure of the Secundum Atrial Septal Defect with the Figulla-Occlutech Device.

OBJECTIVES: To evaluate the mid-term outcomes after percutaneous closure of the secundum atrial septal defects (ASD) using the Figulla-Occlutech device (FOD). BACKGROUND: Transcatheter closure has become the method of choice for most patients with ASD. Although the FOD may have some advantageous characteristics there is a paucity of data on later outcomes after the use of this relatively new device. METHODS: Observational, single arm study including 200 non-consecutive patients who underwent ASD closure between 04/09 and 07/15 in 2 centers. Device performance, deployment technique, and immediate and mid-term outcomes were assessed. RESULTS: Median age and weight were 24 years (4-72) and 58 kg (15-92), respectively. Single defects were observed in 171 patients (median size of 19 mm). The remainder had multiple or multifenestrated defects. Implantation of FOD (median size of 24 mm) was successful in all (99%), but 2 patients (1 with deficient postero-inferior rim; 1 with a large ASD for the size of the child). Embolization with device retrieval occurred in 2 (1%). Median follow-up of 36 months was obtained in 172 patients. Serial echocardiographic assessment showed complete closure in all but 2 patients, in whom an additional small non-significant posterior defect was purposely left untouched. There have been no episodes of late arrhythmias, device embolization, cardiac erosion, endocarditis, thromboembolism, wire fracture, or death. CONCLUSIONS: Transcatheter closure of ASDs in older children, adolescents, and adults using the FOD was highly successful in a wide range of anatomical scenarios with high closure rates and no complications in mid-term follow-up.

Exercise Capacity Long-Term after Arterial Switch Operation for Transposition of the Great Arteries.

UNLABELLED: Transposition of the great arteries (TGA) is a congenital heart defect successfully corrected through arterial switch operation (ASO). Although this technique had significant impact in improving survival, little is known about the functional capacity of the operated patients long-term after surgery. OBJECTIVE: The aim of this study was to compare the functional capacity of children with TGA long-term after ASO with that of healthy children. DESIGN: Retrospective study. PATIENTS: All patients that performed cardiopulmonary exercise test (CPET) were included in the study. As a control group, healthy children in evaluation for physical activity that performed CPET during the same period were also enrolled. RESULTS: Thirty-one TGA patients (19 male) were compared with 29 age-matched controls (21 male). Maximum oxygen consumption was higher in the control group (45.47 ± 8.05 vs. 40.52 ± 7.19, P = .017), although within normal limits in both groups (above 90% of predicted value). The heart rate behavior during exercise was different in both groups, with a mean chronotropic index significantly lower in the TGA group (63% ± 14 vs. 81% ± 12, P < .001). CONCLUSIONS: Our results showed that exercise capacity long-term after ASO in TGA is well preserved although lower than in healthy children what might be explained by the presence of chronotropic incompetence in the TGA group.

Mid-Term Outcomes after Percutaneous Closure of the Secundum AtrialSeptal Defect with the Figulla-Occlutech Device

The secundum atrial septal defect (ASD) is acommon congenital heart defect (CHD), especially inadults. 1 Most of these defects are amenable for transcatheter closure, which became the method of choice to treat most patients due to its safety and efficacy with excellent mid-to-long term outcomes. 2–5 Although cardiac erosions may occur after the use of the Amplatzer Septal Occluder (ASO),6 this complication is very rare. The Figulla-Occlutech device (FOD;Occlutech, Jena, Germany) is relatively new, being anitinol double-disk occluder with a self-centering mechanism provided by a central connecting waist...

Cardiopatias congênitas em adultos: um problema em ascensão / Congenital heart disease in adults: an increasing problem

A população de adolescentes e adultos com cardiopatia congênita é crescente graças às grandes inovações tecnológicas que permitiram avanços nos métodos diagnósticos, melhor conhecimento das cardiopatias congênitas, melhoria do tratamento clínico, surgimento da terapêutica intervencionista percutânea, aperfeiçoamento dos cuidados intensivose dos resultados cirúrgicos, aumentando assim a sobrevida destes pacientes. Algumas cirurgias apresentam resultados excelentes, enquanto outras, usualmente em portadores de cardiopatia congênita complexa, podem evoluir com lesões residuais importantes que ainda necessitarão de tratamento clínico e/ou cirúrgico ou pela hemodinâmica durante o seguimento. Entre outro grupo de pacientes, estão os pacientes não operados, que são diagnosticados ou encaminhados aos serviços especializados tardiamente. Alguns destes apresentam hipertensão pulmonar importante, ou já desenvolveram a síndrome de Eisenmenger e, portanto são inoperáveis, enquanto outros que se apresentam com cardiopatia como a comunicação interatrial, que costuma ser pouco sintomática nas primeiras duas décadas de vida, ou cardiopatias complexas balanceadas por algum grau de estenose pulmonar, que ainda poderão ser operados, mas sem a certeza do benefício da correção, pois estão predispostos a apresentar arritmia cardíaca e disfunção ventricular. Para que se possa oferecer a melhor abordagem, seja ela qual for, para esse grupo de pacientes, devemos continuar a buscar a formação de centros especializados para o atendimento aos adolescentes e adultos com cardiopatia congênita tratada ou não na infância, que cursam com lesões residuais ou hipertensão pulmonar. Além do aspecto técnico, não se pode desconsiderar a qualidade de vida desses pacientes, incluindo aspectos psicossociais e cognitivos.

The adolescent and adult population with congenital heart disease is growing, due to major technological innovations that have led to advances in diagnostic methods, better knowledge of congenital heart disease, improvements in clinical treatment, the emergence of percutaneous interventionist treatment, and the perfection of intensive care and surgical outcomes, all of which have increased the survival of these patients. Some surgical procedures present excellent results, while others, usually in individuals with complex congenitalheart disease, can develop with significant residual lesions that also require clinical and/or surgical treatment, or due to the hemodynamics during follow-up. Among another group ofpatients are those not operated on, who are diagnosed or referred to the specialized services too late. Some of these present significant pulmonary hypertension, or have alreadydeveloped Eisenmenger’s syndrome, and are therefore inoperable, while others presente heart disease such as inter-atrial communication, which generally has few symptoms in the first two decades of life, or complex heart disease balanced by some degree of pulmonarystenosis, who can still be operated on but without certainty of the benefit of correction, as they are predisposed to present cardiac arrhythmia and ventricular dysfunction. In order tooffer the best approach for this group of patients, whatever that may be, we must continue to strive for the formation of specialized centers for the care of adolescents and adults withcongenital heart disease, whether treated or not in infancy, and who also have residual lesions or pulmonary hypotension. In addition to the technical aspect, we must also consider thequality of life of these patients, including the psychosocial and cognitive aspects.

Estudo comparativo entre o uso do antiagregante plaquetário e do anticoagulante oral na profilaxia de trombose em pacientes submetidos à operação cavopulmonar total com tubo extracardíaco: análise ecocardiográfica, angiotomográfica, cintilográfica, laboratorial e clínica / Comparative trial of the use of antiplatelet and oral anticoagulant in thrombosis prophylaxis in patients undergoing total cavopulmonary operation with extracardiac conduit: echocardiographic, tomographic, scintigraphic, clinical and laboratory analysis

Objetivo: Comparar a eficácia do AAS e da Varfarina na profilaxia da trombose em pacientes submetidos a operação cavopulmonar total. Avaliar se fatores de coagulação (VII, VIII e Proteína C), dados clínicos, fenestração ou fatores hemodinâmicos, interferem na trombose no pós-operatório. Métodos: Estudo prospectivo e randomizado de 30 pacientes, randomizados em Grupo I (Varfarina) e Grupo II (AAS), submetidos à derivação cavopulmonar total com tubo extracardíaco, entre 2008 e 2011, com seguimento de dois anos. Foram realizadas consultas clínicas que avaliavam efeitos colaterais e aderência. Realizado ecocardiograma transesofágico no pós-operatório imediato, 3, 6,12 e 24 meses; angiotomografia aos 6, 12 e 24 meses de pós-operatório para avaliação de alterações na parede interna do tubo ou trombos e cintilografia pulmonar, para avaliar possível TEP. Resultados: Dois óbitos no grupo I; 33,3% dos pacientes apresentaram trombo (46,7% no Grupo II). A ocorrência prévia de trombo e baixos níveis de proteína C da coagulação foram os únicos fatores que influenciaram no tempo livre de trombo (P=0,035 e 0,047). Avaliação angiotomográfica: 35,7% dos pacientes do grupo II tinham atapeteamento maior que 2 mm (P=0,082). Cintilografia: dois pacientes apresentaram TEP no grupo II. Cinco pacientes tiveram dificuldade de aderência, 4 no grupo I com INR variando de 1 a 6,4. Conclusão: A ocorrência prévia de trombo é um fator de risco para trombose no pós-operatório. Pacientes em uso de AAS tendem a depósito de material na parede do tubo. O número reduzido da amostra não permitiu concluir qual a droga mais eficaz na prevenção da trombose na população estudada. .

Objective: To compare the efficacy of aspirin and warfarin for prophylaxis of thrombosis in patients undergoing total cavopulmonary anastomosis. Evaluate whether coagulation factors (VII, VIII and protein C), clinical data, fenestration or hemodynamic factors, interfere with postoperative thrombosis. Methods: A prospective, randomized study of 30 patients, randomized into Group I (Warfarin) and Group II (AAS), underwent total cavopulmonary shunt with extracardiac conduit, between 2008 and 2011, with follow-up by clinical visits to evaluate side effects and adhesion. Performed transesophageal echocardiography in post operatory time, 3, 6,12 and 24 months; angiotomography at 6, 12 and 24 months to evaluate changes in the internal tube wall or thrombi and pulmonary scintigraphy to evaluate possible PTE. Results: Two deaths in group I; 33.3% of patients had thrombus (46.7% in Group II). The previous occurrence of thrombus and low levels of coagulation protein C were the only factors that influenced the time free of thrombus (P=0.035 and 0.047). Angiotomographic evaluation: 35.7% in group II presented material accumulation greater than 2 mm (P=0.082). Scintigraphy: two patients had PTE in group II. Five patients had difficulty to comply with the treatment, 4 in group I with INR ranging from 1 to 6.4. Conclusion: The previous occurrence of thrombus is a risk factor for thrombosis in the postoperative period. Patients using AAS tend to deposit material in the tube wall. The small sample size did not allow to conclude which is the most effective drug in the prevention of thrombosis in this population. .

Fetal interventions for congenital heart disease in Brazil.

Fetal interventions have been performed for some congenital heart diseases. However, these procedures have not gained wide acceptance due to concerns about their efficacy and safety. The aim of this study was to report on a preliminary experience with fetal cardiac interventions in Brazil. Twenty-two cardiac interventions were performed in 21 fetuses. Thirteen fetuses had critical aortic stenosis (CAS), 4 had hypoplastic left heart syndrome (HLHS) and intact interatrial septum or small patent foramen ovale, 1 had pulmonary atresia with intact ventricular septum (IVS), and 3 had critical pulmonary stenosis (CPS). The main outcome variables evaluated were technical success and procedural complications as well as pregnancy and postnatal outcomes. Success was achieved in 20 of 22 procedures (91%) with 1 failed aortic and 1 failed pulmonary valvuloplasties. There was 1 fetal death. No maternal complications occurred. One patient with CAS, severe mitral regurgitation, and hydrops died postnatally within 5 months of age. All patients with HLHS and restrictive atrial septum died after interventional or surgical procedures and prolonged hospitalizations. All patients with CPS/IVS survived and achieved a biventricular (BV) circulation after neonatal valvuloplasty and ductal stenting. A BV circulation was achieved in 4 of 8 patients with CAS and evolving HLHS (one still in utero), including 2 with initial borderline left ventricles (LV) in whom surgical LV overhaul was performed at 9 months of age. In this preliminary experience, the feasibility of fetal cardiac interventions and their outcomes were similar to those previously reported.

Comparative trial of the use of antiplatelet and oral anticoagulant in thrombosis prophylaxis in patients undergoing total cavopulmonary operation with extracardiac conduit: echocardiographic, tomographic, scintigraphic, clinical and laboratory analysis.

OBJECTIVE: To compare the efficacy of aspirin and warfarin for prophylaxis of thrombosis in patients undergoing total cavopulmonary anastomosis. Evaluate whether coagulation factors (VII, VIII and protein C), clinical data, fenestration or hemodynamic factors, interfere with postoperative thrombosis. METHODS: A prospective, randomized study of 30 patients, randomized into Group I (Warfarin) and Group II (AAS), underwent total cavopulmonary shunt with extracardiac conduit, between 2008 and 2011, with follow-up by clinical visits to evaluate side effects and adhesion. Performed transesophageal echocardiography in post operatory time, 3, 6,12 and 24 months; angiotomography at 6, 12 and 24 months to evaluate changes in the internal tube wall or thrombi and pulmonary scintigraphy to evaluate possible PTE. RESULTS: Two deaths in group I; 33.3% of patients had thrombus (46.7% in Group II). The previous occurrence of thrombus and low levels of coagulation protein C were the only factors that influenced the time free of thrombus (P=0.035 and 0.047). Angiotomographic evaluation: 35.7% in group II presented material accumulation greater than 2 mm (P=0.082). Scintigraphy: two patients had PTE in group II. Five patients had difficulty to comply with the treatment, 4 in group I with INR ranging from 1 to 6.4. CONCLUSION: The previous occurrence of thrombus is a risk factor for thrombosis in the postoperative period. Patients using AAS tend to deposit material in the tube wall. The small sample size did not allow to conclude which is the most effective drug in the prevention of thrombosis in this population.

Prevalence of dyslipidemia in children with congenital heart disease.

Dyslipidemia is one of the main risk factors associated with cardiovascular diseases. Few data on the impacts of congenital heart diseases are available with regard to the prevalence of dyslipidemia in children. Our study evaluated the lipid profile in children with congenital heart disease at a referral center. From January 2011 to July 2012, 52 pediatric patients had their lipid, metabolic and clinical profiles traced. The mean age was 10.4 ± 2.8 years and male/female rate of 1.38:1. Our population had 53.8% patients with high levels of total cholesterol and 13.4% (CI 95 %, from 6.6 to 25.2%) of them also presenting LDL levels ≥ 130 mg/dL, which characterizes dyslipidemia. The group of dyslipidemic patients presented only two obese individuals. Our data show that the presence of congenital heart disease does not lead to higher risk associated with the prevalence of dyslipidemia. Therefore, the screening of this specific population should follow the regular pediatric guidelines, which are also independent of the nutritional status of the children tested.

Factibilidade, segurança e eficácia do fechamento percutâneo da comunicação interatrial em crianças pequenas / Feasibility, safety and efficacy of percutaneous atrial septal defect closure in infants

INTRODUÇÃO: A experiência com o fechamento percutâneo da comunicação interatrial (CIA) em crianças pequenas é limitada. Avaliamos a factibilidade, a segurança e a eficácia desse procedimento em crianças com peso < 20 kg. MÉTODOS: Estudo descritivo observacional de uma coorte de crianças < 20 kg submetidas a tratamento percutâneo. Pacientes com dilatação ventricular direita e sintomas evidentes foram incluídos. Implantamos próteses aprovadas pela ANVISA, sob monitorização ecocardiográfica transesofágica. Os pacientes foram avaliados 1 mês, 3 meses, 6 meses e 12 meses após. RESULTADOS: Entre outubro de 1997 e maio de 2012, 80 pacientes foram tratados. As medianas de idade e peso foram de 4 anos (1-12) e 13,5 kg (5-20), respectivamente, 20 pacientes apresentavam alguma síndrome genética (25%) e 4 pacientes (5%) apresentavam CIA adicional. Somente um paciente necessitou duas próteses. Dois pacientes tinham defeitos associados, os quais foram tratados no mesmo procedimento (estenose pulmonar valvar e fístula arteriovenosa). Um paciente desenvolveu bloqueio atrioventricular total durante o implante da prótese, resolvido espontaneamente 36 horas após a remoção da prótese, sem necessidade de implante de marca-passo. Esse paciente foi tratado percutaneamente 6 meses após com sucesso, sem complicações. Setenta e nove pacientes receberam alta hospitalar em até 24 horas após o procedimento. Fluxo residual discreto (1-2 mm) foi observado em 5% dos casos antes da alta. Após 6 meses de seguimento, não foi detectado fluxo residual. Não houve complicações tardias no seguimento. CONCLUSÕES: O fechamento percutâneo da CIA em crianças pequenas selecionadas e sintomáticas é uma alternativa tera­pêutica factível, segura e eficaz, devendo ser a primeira opção para seu tratamento.

BACKGROUND: The experience with percutaneous closure of atrial septal defect (ASD) in infants is limited. We sought to determine the feasibility, safety and efficacy of this procedure in children weighing < 20 kg. METHODS: Observational study of a cohort of children weighing < 20 kg undergoing percutaneous closure. Patients with right ventricular enlargement and evident symptoms were included. ANVISA approved devices were implanted under transesophageal echocardiography monitoring. Patients were evaluated 1, 3, 6 and 12 months after the procedure. RESULTS: Eighty patients were treated between October 1997 and May 2012. Median age and weight were 4 years (1-12) and 13.5 kg (5-20), respectively, 20 patients had a genetic syndrome (25%) and 4 patients (5%) had an additional ASD. Only one patient required 2 devices. Two patients had associated defects that were treated in the same procedure (pulmonary valve stenosis and arteriovenous fistula). One patient developed total atrioventricular block during device implantation, solved spontaneously 36 hours after device removal, with no need for pacemaker implantation. This patient was successfully treated percutaneously 6 months later without complications. Seventy-nine patients were discharged within 24 hours after the procedure. A mild residual shunt (1-2 mm) was observed in 5% of the cases before discharge. There was no residual shunt 6 months after the procedure. There were no complications in the late follow-up. CONCLUSIONS: Percutaneous ASD closure in selected symptomatic infants is a feasible, safe and effective alternative and should be the first option therapy.