NANA MIURA IKARI
Projetos de Pesquisa
Unidades Organizacionais
Instituto do Coração, Hospital das Clínicas, Faculdade de Medicina
43 resultados
Resultados de Busca
Agora exibindo 1 - 10 de 43
- PEDIATRIC HEART TRANSPLANTATION PROGRAM: SINGLE CENTER EXPERIENCE OF 20 YEARS(2013) AZEKA, Estela; GALAS, Filomena; TANAMATI, Carla; PENHA, Juliano; FERNANDES, Marcos; SENAHA, Luciano; XAVIER, Johnny; DELGADO, Ana Beatriz Romani; KAJITA, Luiz; AIELLO, Vera; BENVENUTI, Luiz; MIURA, Nana; AULER JUNIOR, Jose Otavio Costa; JATENE, MarceloOBJECTIVE: Heart transplantation has been the treatment of choice for children with refractory to conventional therapy. To report the experience with heart transplantation in a single center. MATERIAL AND METHODS: To report the single center experience of hearttransplantation. Data analysis was demographic characteristics, clinical outcome and survival Kaplan Meier curve. RESULTS: From October 30, 1992 to October 2nd, 2012, 120 transplants were performed and 115 patients were submitted to heart transplantation at Heart Institute (InCor) University of Sao Paulo Medical School, Sao Paulo, Brazil. The causes of indication for transplantation were: cardiomyopathies (76%) and congenital heart disease (24%). Five patients were submitted to re-transplantation. Three of them were re-transplanted on the early postoperative period with high mortality (66.6%). One patient was submitted to simultaneous re-transplantation and kidney transplantation after 13.9 yrs of follow-up and one of patient was successful re-transplanted after 10 yr of follow-up. The post-operative immunosuppression regimen was double immunosuppression and polyclonal anti-thymocyte serum induction therapy. The actuarial survival was 80%, 71%, and 61% at 1, 5, and 10 yr, respectively. CONCLUSION: Heart transplantation has been a promising option for children. The use of double immunosuppression with polyclonal anti-thymocyte serum induction therapy combined with surveillance of acute rejection with non-invasive tests may provide favorable clinical outcome.
conferenceObject GOING AGAINST THE ODDS - A CASE OF SUCCESS IN A PREGNANT PATIENT WITH COMPLEX CONGENITAL HEART DISEASE(2021) KORMANN-MOREIRA, Mylena Cristina; MARTINS, Ana Vitoria Vitoreti; LEMOUCHE, Stephanie Ondracek; SANTANA, Marcela Devido; TANAKA, Ana Cristina Sayuri; ZORZANELLI, Leina; THOMAZ, Ana Maria; IKARI, Nana Miura; AVILA, Walkiria SamuelbookPart Estenose pulmonar(2016) ZORZANELLI, Leina; IKARI, Nana Miura- Cytogenomics Investigation of Infants with Congenital Heart Disease: Experience of a Brazilian Center(2022) GRASSI, Marcilia Sierro; MONTENEGRO, Marilia; ZANARDO, Evelin Aline; PASTORINO, Antonio Carlos; DORNA, Mayra Barros; KIM, Chong; JATENE, Marcelo; MIURA, Nana; KULIKOWSKI, Leslie; CARNEIRO-SAMPAIO, MagdaBackground: Some syndromes have specific and easily recognizable features, while others may be more complex to identify and may present different phenotypic manifestations, for example. An etiological diagnosis is important to understand the nature of the disease, to establish the prognosis and to start the treatment, allowing the inclusion of patients in society and reducing the financial cost of such diseases. Objective: The initial proposal of this study was cytogenetic screening for the detection of the 22q11.2 deletion syndrome in consecutive newborns and infants with congenital heart disease using the multiplex ligation-dependent probe amplification (MLPA) technique. Therefore, throughout our research, other genomic alterations were identified in these cardiac patients. Thus, our objective was extended to investigate these other cytogenetic alterations. Methods: We investigated 118 neonates with congenital heart diseases born consecutively during one year using the MLPA technique. Results: The MLPA technique allowed the detection of 22q11.2DS in 10/118 patients (8.5%). Other genomic alterations were also identified in 6/118 patients (5%): 1p36 del, 8p23 del (2 cases), 7q dup, 12 dup and 8q24 dup. Conclusion: This study highlights the relevance of detecting genomic alterations that are present in newborns and infants with congenital cardiac diseases using cytogenomic tools.
- Mucopolysaccharidosis type VI: case report with first neonatal presentation with ascites fetalis and rapidly progressive cardiac manifestation(2020) HONJO, Rachel Sayuri; VACA, Evelyn Cristina Nunez; LEAL, Gabriela Nunes; ABELLAN, Deipara Monteiro; IKARI, Nana Miura; JATENE, Marcelo Biscegli; MARTINS, Ana Maria; KIM, Chong AeBackground The Mucopolysaccharidosis type VI (MPS VI), also known as Maroteaux-Lamy syndrome (OMIM 253200) is an autosomal recessive lysosomal disorder, caused by the deficiency of the enzyme N-acetylgalactosamine 4-sulfatase (also known as arylsulfatase B) due to mutations of the ARSB gene. Cardiologic features are well recognized, and are always present in MPS VI patients. Generally, the onset and the progression of the cardiologic symptoms are insidious, and just a few patients have developed a rapidly progressive disease. Cardiac involvement in MPS VI is a common and progressive feature. For MPS patients, cardiac evaluations are recommended every 1 to 2 years, including blood pressure measurement, electrocardiography and echocardiography. However, congestive heart failure and valvular surgical repair are not frequently seen, and if so, they are performed in adults. Here we report on an atypical MPS VI case with ascites fetalis and a rapidly progressive cardiac disease. Case presentation A 6-month-old Brazilian male, only child of a Brazilian healthy non-consanguineous couple. During pregnancy, second trimester ultrasonography observed fetal ascites and bilateral hydrocele. Physical exam at 6 months-old revealed a typical gibbus deformity and MPS was suspected. Biochemical investigation revealed a diagnosis of MPS type VI, confirmed by molecular test. Baseline echocardiogram revealed discrete tricuspid regurgitation and a thickened mitral valve with posterior leaflet prolapse, causing moderate to severe regurgitation. The patient evolved with mitral insufficiency and congestive heart failure, eventually requiring surgical repair by the first year of age. Conclusions We report the first case of MPS VI whose manifestations started in the prenatal period with fetal ascites, with severe cardiac valvular disease that eventually required early surgical repair. Moreover, in MPS with neonatal presentation, including fetal hydrops, besides MPS I, IVA and VII, clinicians should include MPS VI in the differential diagnosis.
- Heart Transplantation in Pediatric Population and in Adults With Congenital Heart Disease: Long-term Follow-up, Critical Clinical Analysis, and Perspective for the Future(2014) AZEKA, E.; JATENE, M.; GALAS, F. R. B.; TANAMATI, C.; PENHA, J.; BENVENUTI, L.; MIURA, N.; JUNIOR, J. O. C.Background. Heart transplantation is a treatment option for children as well as for adults with congenital heart disease. Objective. To report the experience of a tertiary center with heart transplant program in pediatric population and in adults with congenital heart disease. Patients and Methods. The study consisted of the evaluation of pediatric as well as adult patients undergoing heart transplantation for congenital heart disease. We evaluated the following indication and complications such as renal dialysis, graft vascular disease, tumors and survival. Results. From October 1992 to November 2013, 134 patients had transplantation, and there were 139 transplantations and 5 retransplantations. The immunosuppression regimen is based on calcineurin inhibitors and cytostatic drugs. The type of heart disease indicated for transplantation was cardiomyopathies in 70% and congenital heart disease in 30%. Of these 134 patients, 85 patients were alive. Actuarial survival is 77.4%, 69.6%, 59.3% at 1, 5, and 10 years after transplantation. Three patients underwent renal transplantation, 1 patient is in renal dialysis, and 8.2% of patients had post-transplant lymphoproliferative disease. Two patients had retransplantation for graft vascular disease; 1 of them required a simultaneous kidney transplant and died 30 days after the procedure and 1 patient is clinically well 2 years after retransplantation. Conclusion. Heart transplantation in children and in adults with congenital heart disease is a promising therapeutic option and enables long-term survival for these patients.
- Pregnancy in Women with Complex Congenital Heart Disease. A Constant Challenge(2019) AVILA, Walkiria Samuel; RIBEIRO, Veronica Martins; ROSSI, Eduardo Giusti; BINOTTO, Maria Angelica; BORTOLOTTO, Maria Rita; TESTA, Carolina; FRANCISCO, Rossana; HAJJAR, Ludhmilla Abraao; MIURA, NanaBackground: The improvement in surgical techniques has contributed to an increasing number of childbearing women with complex congenital heart disease (CCC). However, adequate counseling about pregnancy in this situation is uncertain, due to a wide variety of residual cardiac lesions. Objectives: To evaluate fetal and maternal outcomes in pregnant women with CCC and to analyze the predictive variables of prognosis. Methods: During 10 years we followed 435 consecutive pregnancies in patients (pts) with congenital heart disease. Among of them, we selected 42 pregnancies in 40 (mean age of 25.5 +/- 4.5 years) pts with CCC, who had been advised against pregnancy. The distribution of underlying cardiac lesions were: D-Transposition of the great arteries, pulmonary atresia, tricuspid atresia, single ventricle, double-outlet ventricle and truncus arteriosus. The surgical procedures performed before gestation were: Fontan, Jatene, Rastelli, Senning, Mustard and other surgical techniques, including Blalock, Taussing, and Glenn. Eight (20,0%) pts did not have previous surgery. Nineteen 19 (47.5%) pts had hypoxemia. The clinical follow-up protocol included oxygen saturation recording, hemoglobin and hematocrit values; medication adjustment to pregnancy, anticoagulation use, when necessary, and hospitalization from 28 weeks, in severe cases. The statistical significance level considered was p < 0.05. Results: Only seventeen (40.5%) pregnancies had maternal and fetal uneventful courses. There were 13 (30.9%) maternal complications, two (4.7%) maternal deaths due to hemorrhage pos-partum and severe pre-eclampsia, both of them in women with hypoxemia. There were 7 (16.6%) stillbirths and 17 (40.5%) premature babies. Congenital heart disease was identified in two (4.1%) infants. Maternal and fetal complications were higher (p < 0.05) in women with hypoxemia. Conclusions: Pregnancy in women with CCC was associated to high maternal and offspring risks. Hypoxemia was a predictive variable of poor maternal and fetal outcomes. Women with CCC should be advised against pregnancy, even when treated in specialized care centers.
article 5 Citação(ões) na Scopus Tratamento cirúrgico da persistência do canal arterial na população adulta(2011) JATENE, Marcelo Biscegli; ABUCHAIM, Decio Cavalet Soares; TIVERON, Marcos G.; TANAMATI, Carla; MIURA, Nana; RISO, Arlindo; AUIK, Edrnar; LOPES, Antonio Augusto; MARCIAL, Miguel BarberoObjective: To analyze 34 patients submitted to surgical treatment of patent arterial duct with age beyond 18 years old. Methods: Retrospective data collected from patient's charts with more than eighteen years old, submitted to surgical correction of patent arterial duct between 1997 and 2008 at Instituto do Coracao da Faculdade de Medicina da Universidade de Sao Paulo. Results: The mean age was 28.7 (18 a 53) years and 22 (64.7%) were female. The more prevalent symptom was dyspnea (76.5%). Left lateral thoracotomy was used in 33 (97.1%); the DA was sectioned and sutured in 25 (73.5%) cases and one patient needed cardiopulmonary bypass support. There were eight (23.5%) calcified arterial duct and 12 (35.3%) previous treatment with transcatheter devices were performed. The complication rate was 32%, with one (2.9%) permanent vocal cord palsy. Two (5.8%) patients had residual shunt less than 2mm. Transient left cord voice palsy was observed in 3 (8.8%) The procedure improves functional class (P < 0.0001) and no mortality was observed. Conclusion: In this series, the surgical treatment of patent arterial duct in adults could be done without mortality and low incidence of complications.conferenceObject HEART TRANSPLANTATION IN PEDIATRIC AND ADULT WITH CONGENITAL HEART DISEASE: CURRENT STATUS.(2017) COUTINHO, K.; SIQUEIRA, A.; BENVENUTI, L.; MIURA, N.; JATENE, M.; AZEKA, E.- Heart Retransplantation for Coronary Allograft Vasculopathy in Children: 25 Years of Single -Center Experience(2020) AZEKA, Estela; WALKER, Thomas; SIQUEIRA, Adailson Wagner da Silva; PENHA, Juliano; MIANA, Leonardo; CANEO, Luiz Fernando; MASSOTI, Maria Raquel; TANAMATI, Carla; MIURA, Nana; JATENE, Marcelo Biscegli