Perfil dos pacientes pediátricos submetidos a implantes de dispositivos cardíacos eletrônicos implantáveis em hospital público do Rio de Janeiro: revisão de 57 procedimentos sequenciais / Profile of pediatric patients submitted to implantable electronic cardiac devices in a public hospital in Rio de Janeiro: review of 57 sequential procedures

Introdução: A população pediátrica corresponde a menos de 1% dos implantes de marcapasso e possui desafios peculiares. Este estudo tem o objetivo de revelar o perfil dos pacientes pediátricos submetidos a cirurgias de dispositivos cardíacos eletrônicos implantáveis e taxas de complicação. Método: Estudo observacional retrospectivo de análise de prontuários de pacientes com até 18 anos de idade, submetidos a cirurgias de dispositivos cardíacos eletrônicos implantáveis entre 2009 e 2014 no Instituto Nacional de Cardiologia, sobre variáveis clínicas e cirúrgicas e complicações. Resultados: Entre 2009 e 2014, 51 pacientes foram submetidos a 57 cirurgias, dos quais 60,8% eram do sexo feminino. A média de idade foi de 8,32 anos. Cardiopatia congênita foi encontrada em 60,8% dos pacientes e cirurgia cardíaca prévia, em 76,4%. As principais indicações para dispositivos cardíacos eletrônicos implantáveis foram bloqueio atrioventricular total pós-operatório (40,4%) e bloqueio atrioventricular total congênito (31,6%). Dentre as cirurgias realizadas, destacam-se implante de marcapasso bicameral (38,6%) e implante de marcapasso monocameral ventricular (28,1%). Em 44 cirurgias de implantes, 72,7% foram endocárdicos. Ocorreram 3 complicações (5,3%). Conclusão: Na população pediátrica sujeita a implante de dispositivos cardíacos eletrônicos implantáveis, a escolha do sistema de estimulação cardíaca, a técnica de implante, a programação e o acompanhamento devem ser criteriosos. As taxas de complicação foram baixas. A melhor compreensão dessa população pode auxiliar na definição de diretrizes para seu tratamento

Background: The pediatric population represents less than 1% of all pacemaker implants and has unique challenges. This study is aimed at identifying the profile of pediatric patients undergoing surgical procedures for implantable electronic cardiac devices and complication rates. Method: This was a retrospective observational analysis of medical records of patients up to 18 years of age, submitted to implantable electronic cardiac devices between 2009-2014 at the National Institute of Cardiology on the clinical and surgical variables and complications. Results: Between 2009 and 2014, 51 patients underwent 57 surgeries, of which 60.8% were female. Mean age was 8.32 years. Congenital heart disease was found in 60.8% of the patients and 76.4% had had a previous heart surgery. The main indications for implantable electronic cardiac devices implantation were: postoperative complete heart block (40.4%), and congenital complete heart block (31.6%). The surgeries included dual chamber pacemakers (38.6%), and ventricular single chamber pacemaker (28.1%). Out of 44 surgical implants, 72.7% were endocardial. There were 3 complications (5.3%). Conclusion: In the pediatric population subject to implantable electronic cardiac device implantation, the choice of the cardiac pacing system,the implantation technique, planning and follow-up must be carefully made. Complication rates were low. A better understanding of this population can help define treatment guidelines

Experimental studies on the role of GATA4 in the endocardial cushions development / 中华心血管病杂志

<p><b>OBJECTIVE</b>To investigate the role of GATA4 gene in the endocardial cushions development.</p><p><b>METHODS</b>Target gene eukaryote expression vectors were constructed by pcDNA3.1(-) vector plasmid, and were identified by DNA sequence analysis. Recombinant plasmids were transfected into Hela cells with lipofectamine 2000, meanwhile Hela cells transfected with empty vector or those without transfection served as transfection control group and blank control group, respectively. Real-time PCR and Western blot were performed to detect the relative expression of mRNA and protein of transcription factors GATA4, Sox9, Scleraxis and ECM proteins Aggrecan, Tenascin in each group.</p><p><b>RESULTS</b>The relative mRNA expression of GATA4 in experimental group was significantly higher than in transfection control group and blank control group. GATA4 mRNA expression in Hela(GATA4), Hela(H436Y), Hela(Null) and Hela group was 310.83 ± 2.39, 146.35 ± 1.74, 0.94 ± 0.32, 1.00 ± 0.28, respectively (F = 72.508, P < 0.05). Western blot results were consistent with the results obtained by qRT-PCR. The relative mRNA and protein expressions of Sox9, Scleraxis, Aggrecan and Tenascin in both experimental groups were significantly higher than that in transfection control group and blank control group (P < 0.05), and above gene expressions were significantly downregulated in GATA4(H436Y) group, while they were similar between transfection control group and blank control group (all P > 0.05).</p><p><b>CONCLUSIONS</b>GATA4 H436Y mutation reduces it's transcriptional activation, which might serve as a theoretical framework to demonstrate the roles of GATA4 gene in endocardial cushion development.</p>

Case Report of Partial Endocardial Cushion Defect with Mild Pulmonary Hypertension in Old Age / 대한흉부외과학회지

The partial endocardial cushion defect including ostium primum atrial septal defect and anterior mitral leaflet cleft, presents less significant clinical symptoms than complete endocardial cushion defect. But, as mitral insufficiency develops, cardiomegaly, congestive heart failure, pulmonary arterial hypertension appear. So, partial endocardial cushion defect has poor prognosis and is rarely seen in elderly patients. A 67 years old woman admitted at our hospital for operative treatment with partial endocardial cushion defect. She had increased pulmonary pressure of 45/22 mmHg, mean 32 mmHg. She had repair of ostium primum defect with patch, and the mitral valve was treated with valve replacement. Because advanced atrioventricular block developed postoperatively, she received permanent pacemaker.

Case Report of Partial Endocardial Cushion Defect with Mild Pulmonary Hypertension in Old Age / 대한흉부외과학회지

The partial endocardial cushion defect including ostium primum atrial septal defect and anterior mitral leaflet cleft, presents less significant clinical symptoms than complete endocardial cushion defect. But, as mitral insufficiency develops, cardiomegaly, congestive heart failure, pulmonary arterial hypertension appear. So, partial endocardial cushion defect has poor prognosis and is rarely seen in elderly patients. A 67 years old woman admitted at our hospital for operative treatment with partial endocardial cushion defect. She had increased pulmonary pressure of 45/22 mmHg, mean 32 mmHg. She had repair of ostium primum defect with patch, and the mitral valve was treated with valve replacement. Because advanced atrioventricular block developed postoperatively, she received permanent pacemaker.

A Case of Annular Pancreas and Endocardial Cushion in the Abscence of Down Syndrome

Annular pancreas is a rarely found gastrointestinal tract malformation, and is frequently associated with Down syndrome. Endocardial cushion defect is a type of congenital heart disease, commonly also related to Down syndrome. However, a combination of endocardial cushion defect with annular pancreas has not been reported previously. We recently experienced such a case in 2 month old boy. Moreover Down syndrome was excluded through physical examination and a chromosomal study. We think it difficult to obtain a complete explanation for this condition through the currently known pathogenesis of annular pancreas and endocardial cushion defect.

N-cadherin Immunoreaction in the Interventricular Septum in Developing Rat Heart

BACKGROUND AND OBJECTIVES: N-cadherin is known to be expressed in neuroectodermal tissue such as central nervous system and various mesodermal origin tissues such as kidney and heart. We investigated N-cadherin expression in the endocardial cushion in developing rat heart by immunohistochemical method. MATERIALS AND METHOD: Fetal rat hearts at the 11th, 13th, 15th, 17th, and 19th day of gestation and the 1st day neonatal rat heart were used. Hematoxylin and eosin stain was performed for normal cardiogenesis, and immunohistochemistry was performed for the expression of N-cadherin in interventricular septum(IVS) during cardiogenesis in rat. RESULTS: Ventricular wall and membranous part of the IVS showed positive reaction with anti-N-cadherin at the 11th day of gestation. Membranous part of IVS was begun to show tracely positive reaction at the 15th day of gestation, and thereafter the immunoreactivity was increased with maturation. At the 17th day of gestation mesenchymal cells in membranous muscular part of the IVS showed positive reaction. The similar immunoreactivity of membranous and muscular parts of IVS were shown at the 19th day of gestation. CONCLUSION: As the immunoreaction of mesenchymal cells in the membraneous part of IVS to anti-N-cadherin was increased with time, it is suggested that mesenchymal cells in membranous part of IVS were differentiated into the cardiomyocytes.

Cell Proliferation and Apoptosis in Heart of Trisomy 16 in Mice

PURPOSE: Although abnormal developments of cushion and atrioventricular septum have been suggested, the exact mechanism for the development of atrioventricular septal defect is not well known. We aimed to identify the role of cell proliferation and apoptosis on cardiac morphogenesis in trisomy 16 mice(an animal model for Down's syndrome in human). METHODS: We examined the difference in cardiac masses and structures of trisomy 16 mice and normal control mice at stages of embryonal day 11, day 14 and day 16, when the endocardial cushion grows rapidly. We prepared cardiac section slides with PCNA and TUNEL staining and measured the amount, location of cell proliferation and apoptosis with computerized image analysis system. RESULTS: Atrioventricular septal defects were evident at day 14 and day 16 trisomy embryos. The ventricle areas were smaller in trisomy mice especially at day 14 embryos(P=0.04). And the cell proliferation rates were lower in trisomy mice along these periods. The rate of apoptosis was lower in trisomy embryo than control at both ventricular myocytes and interventricular septum, but characteristically apoptotic bodies were condensed at endocardial cushion area in trisomy 16 embryo. CONCLUSION: The developmental problems of atrioventricular septal defect of trisomy 16 mice resulted from myocardial hypoplasia and late localized apoptosis at cushion area, and these changes may play an important role in ventricular remodelling of atrioventricular septal defect.

The Expression of Transforming Growth Factor -alpha and Epidermal Growth Factor on the Cardiovascularsystem of Developing Mouse Embryo / 체질인류학회지

The distribution of transforming growth factor -alpha (TGF -alpha ) and epidermal growth factor (EGF) on the cardiovascular system of developing mouse embryos of gestational age 7 to 12 days were immunohistochemically (ABC method) studies to investigate the differential expression of these growth factors. Paraffin embedded sections were immunostained with antibodies for TGF -alpha and EGF. In the 8 -day -old mouse embryos, the endocardial tissue, myocardial tissue and cardiac jelly were all TGF -alpha stained. EGF stain was observed in the cardiac jelly and myocardial tissue but was not observed in the endocardial tissue. This suggests that in the initial phase of the cardiovascular system development, TGF -alpha function as earlier growth factor than EGF. The 9, 10 and 11 -day -old embryos showed TGF -alpha stain in the broad spectrum of developing cardiovascular tissues such as, the bulbus cordis, primitive atrium, sinus venosus, aortic sac, dorsal aorta, vitelline artery, endocardial cushion tissue, and myocardium of primitive ventricle. However, EGF stain was observed only in the bulbus cordis, primitive atrium and endocardial tissue. This finding indicates that TGF -alpha function as a more extensive growth factor than EGF. The 12 -day -old embryos showed stronger EGF stain than TGF -alpha in the primitive ventricle, bulbus cordis, and endocardial tissue. This suggests that EGF function as a more growth factor than TGF -alpha at this particular developmental stage and plays important role at the end stage of the primitive heart development.

Open Heart Surgery in National University Hospital(1959-1993)

Over the past four decades after Korean War, a great deal of data and clinical experiences have been accumulated relating to the diagnosis and surgical treatment of cardiovascular diseases. Among many institutes, Seoul National University Hospital Including Children's Hospital has played a leading role up to date. Clinical data following the first open heart surgery on August 7,1959 up to December 1993 revealed that the total number of open heart surgery was cises in Seoul National University Hospital and overall mortality was 6.8%. Since 1977, the cases of open heart surgery has rapidly increased due to two main reasons : Accumulation of untreated cardiac patients and widening coverage by national medical insurance. The number of cases exceeded 100 in 1978, 400 in 1980, 600 in 1982 and 700 in 1986. In the second half of 1980's and 1990's the number of cases were over 700 per year. The ratio of congenital to acquired heat disease was 1.5:1 , and that of acyanotic to cyanotic congenital cardiac anomaly was 2.0: 1. The order of frequency of acyanotic group was Ventricular Septal Defect(56.4%), Atrial Septal Defect(28.6%), Endocardial Cushion Defect(4.6%), Pulmonary Stenosis(2.9%) and Patent Ductus Arteriosus(1.0%). Whereas the incidence of cyanotic group was Tetralogy of Fallot(57.5%), Transpotion of Great Arteries(9.5%),Double Outlet Right Ventricle(8.7%) and Pulmonary Atresia(5.7%). The overall mortality of acyanotic congenital heart disease was 2.9% and that of cyanotic congenital heart disease was 16.7%. Transposition of Great Arteries, Pulmonary Atresia and Truncus Arteriosus especially resulted in very high mortality,25-30%. The causes of high mortality in these group were inadequate patient selection for operation, inexperienced perioperative neonatal care and unskiled operative technique, but the figure was remarkebly improved since 1990, and now operative mortality reached under 10%. In 2019 cased of valvular heart disease, single mitral valve disease was most common (1139 cases) and double valvular disease was 534 cases and triple valvular disease was 41 cases. According to the order of frequency in redo valvular disease was 6,0%. The frequency of ischemic heart disease and aortic disease were not so frequency previously but these figures rapidly increase in recent days. The first CABG was performed at this hospital in 1981 and 240 cases have been performed by 1993.Overall mortality was 7.1%. The number of cases in 1993 was increased two times compared to 1992, but the mortality was decreased to 2.4%. Acute aortic dissection (57 cases) was most common among 127 cases of aortic disease which was operative mortality was 26.3% which was very high. Mortality gradually decreased recently down to zero because of appropriate brain protection technique including deep hypothemia, circulatory arrest and selective cerebral perfusion. In Korea, brain death is not accepted by law as well as traditional concept. However in 1993 and up to now, five cases of heart transplantation were performed by voluntary consent of familly of the donor and recipient.

Open Heart Surgery in National University Hospital(1959-1993)

Over the past four decades after Korean War, a great deal of data and clinical experiences have been accumulated relating to the diagnosis and surgical treatment of cardiovascular diseases. Among many institutes, Seoul National University Hospital Including Children's Hospital has played a leading role up to date. Clinical data following the first open heart surgery on August 7,1959 up to December 1993 revealed that the total number of open heart surgery was cises in Seoul National University Hospital and overall mortality was 6.8%. Since 1977, the cases of open heart surgery has rapidly increased due to two main reasons : Accumulation of untreated cardiac patients and widening coverage by national medical insurance. The number of cases exceeded 100 in 1978, 400 in 1980, 600 in 1982 and 700 in 1986. In the second half of 1980's and 1990's the number of cases were over 700 per year. The ratio of congenital to acquired heat disease was 1.5:1 , and that of acyanotic to cyanotic congenital cardiac anomaly was 2.0: 1. The order of frequency of acyanotic group was Ventricular Septal Defect(56.4%), Atrial Septal Defect(28.6%), Endocardial Cushion Defect(4.6%), Pulmonary Stenosis(2.9%) and Patent Ductus Arteriosus(1.0%). Whereas the incidence of cyanotic group was Tetralogy of Fallot(57.5%), Transpotion of Great Arteries(9.5%),Double Outlet Right Ventricle(8.7%) and Pulmonary Atresia(5.7%). The overall mortality of acyanotic congenital heart disease was 2.9% and that of cyanotic congenital heart disease was 16.7%. Transposition of Great Arteries, Pulmonary Atresia and Truncus Arteriosus especially resulted in very high mortality,25-30%. The causes of high mortality in these group were inadequate patient selection for operation, inexperienced perioperative neonatal care and unskiled operative technique, but the figure was remarkebly improved since 1990, and now operative mortality reached under 10%. In 2019 cased of valvular heart disease, single mitral valve disease was most common (1139 cases) and double valvular disease was 534 cases and triple valvular disease was 41 cases. According to the order of frequency in redo valvular disease was 6,0%. The frequency of ischemic heart disease and aortic disease were not so frequency previously but these figures rapidly increase in recent days. The first CABG was performed at this hospital in 1981 and 240 cases have been performed by 1993.Overall mortality was 7.1%. The number of cases in 1993 was increased two times compared to 1992, but the mortality was decreased to 2.4%. Acute aortic dissection (57 cases) was most common among 127 cases of aortic disease which was operative mortality was 26.3% which was very high. Mortality gradually decreased recently down to zero because of appropriate brain protection technique including deep hypothemia, circulatory arrest and selective cerebral perfusion. In Korea, brain death is not accepted by law as well as traditional concept. However in 1993 and up to now, five cases of heart transplantation were performed by voluntary consent of familly of the donor and recipient.

Prenatal ultrasonic detection of endocardial cushion defect in 1 case / 대한산부인과학회잡지

No abstract available.

A case of congenital diaphragmatic hernia associated with endocardial cushion defect / 대한주산의학회잡지

No abstract available.