NANA MIURA IKARI
Projetos de Pesquisa
Unidades Organizacionais
Instituto do Coração, Hospital das Clínicas, Faculdade de Medicina
14 resultados
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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- 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.
bookPart Cardiopatias congênitas no adulto(2018) TANAKA, Ana Cristina Sayuri; ZORZANELLI, Leína; IKARI, Nana Miura- Mechanical Circulatory Assistance in Children: Clinical Outcome(2023) CORBI, Maria Julia De Aro Braz; JATENE, Marcelo Biscegli; SIQUEIRA, Adailson Wagner Da Silva; GRAU, Claudia Regina Pinheiro Castro; TAVARES, Glaucia Maria Penha; IKARI, Nana Miura; AZEKA, EstelaBackground. In countries where organ donation is scarce, mortality in the pediatric heart transplant waiting list is high, and ventricular assist devices (VADs) are therapeutic alternatives in these situations. Berlin Heart EXCOR is currently 1 of the few VADs specific for children.Methods. This retrospective study includes pediatric patients who underwent Berlin Heart EXCOR placement in a Brazilian hospital between 2012 and 2021. Clinical and laboratory data at the time of VAD implantation and the occurrence of complications and outcomes (success as a bridge to transplant or death) were analyzed.Results. Eight patients, from 8 months to 15 years, were included: 6 with cardiomyopathy and 2 with congenital heart disease. Six were on Intermacs 1 and 2 on Intermacs 2. The most common complications observed were stroke and right ventricular dysfunction. Six were transplanted, and 2 died. Those submitted to transplant had a higher mean weight than those who died, with no statistically significant difference. The underlying disease had no impact on the outcome. The group undergoing transplant had lower brain natriuretic peptide and lactate values, but no laboratory variable showed a statistically significant difference in the outcome. Conclusion. A VAD is an invasive treatment with potentially serious adverse effects and is still poorly available in Brazil. However, as a bridge to transplant, it is a useful treatment for children in progressive clinical decline. In this study, we did not observe any clinical or laboratory factor at the time of VAD implantation that implied better outcomes.
conferenceObject CONGENITAL HEART DISEASE IN ADULTS - A NEW PROFILE INSPIRES A NEW PARADIGM(2021) DINARDI, Layara Fernanda Lipari; DEVIDO, Marcela; PEREIRA, Thiago Vicente; ROMANO, Gabriel Leiros; ZORZANELLI, Leina; THOMAZ, Ana Maria; TANAKA, Ana Cristina; SIQUEIRA, Adailson Vagner da Silva; IKARI, Nana Miura; AZEKA, Estela- Brain tuberculoma in pediatric heart transplant recipient(2023) OLIVEIRA, Pedro Carpini de; CORBI, Maria Julia de Aro Braz; SIQUEIRA, Adailson Wagner da Silva; NAVAJASEGARAN, Joshua; MESQUITA, Ana Sofia Silva; FRASSETTO, Fernando Pereira; JATENE, Marcelo Bisceglli; IKARI, Nana Miura; AZEKA, EstelaIntroductionHeart transplantation is the standard treatment for end-stage heart disease. Despite advances in the field, patients remain under risk of developing complications, including opportunistic infections, such as tuberculosis. We present the unprecedented case of cerebral tuberculoma in a 9-year-old heart transplant recipient. Case ScenarioA 9-year-old female child, who underwent heart transplantation in December 2020, was admitted to the emergency department in September 2021 due to headache and vomiting. She had normal vital signs and a mild left hemiparesis. Laboratory findings included lymphopenia and a low C Reactive Protein and brain images showed expansive lesions. A biopsy of the intracranial lesion was performed and anatomopathological analysis was compatible with tuberculoma. After the diagnosis was established, treatment protocol for neurotuberculosis was initiated, the patient had a satisfactory clinical evolution and was discharged 22 days after admission. DiscussionClinical manifestation of tuberculosis usually occurs up to 6 months after transplantation, the findings are commonly atypical and symptoms may be mild. We could not find in medical literature any description of the disease in a heart transplant recipient as young as the one presented in this case report. We documented great response to treatment, even though conventional antituberculosis therapy may interfere with immunosuppression. ConclusionPatients in the postoperative period following heart transplantation are at high risk for developing opportunistic infections such as tuberculosis, which may present with atypical symptoms. Therefore the clinician must have a high index of suspicion in order to make the correct diagnosis and promptly start treatment.
- Infective endocarditis in patent ductus arteriosus by bartonella sp: case report and literature review(2014) OLIVATO, Guilherme Benfatti; FAZZIO, Fernando Roberto De; GAZ, Marcus Vinicius Burato; SANTOS, Thaysa Moreira; PAIXAO, Milena Ribeiro; GOMES, Wilton Francisco; OLIVATO, Gustavo Benfatti; DUARTE, Daniel Renato Goncalves; TOSTA, Thais Juliano Garcia; IKARI, Nana Miura
conferenceObject ACTIVE SEARCH FOR 22q11.2 DELETION IN INFANTS WITH CONGENITAL HEART DISEASE UNDERGOING CORRECTIVE SURGERY: PRELIMINARY RESULTS(2015) GRASSI, Marcilia Sierro; KULIKOWSKI, Leslie Domenici; JACOB, Cristina Miuki Abe; DUTRA, Roberta Lelis; ZANARDO, Evelin; CECCON, Maria Esther Jurfest Rivero; KREBS, Vera Lucia Jornada; IKARI, Nana Miura; JATENE, Marcelo Biscegli; CARVALHO, Werther Brunow; CARNEIRO-SAMPAIO, Magda- Collaborative Quality Improvement in the Congenital Heart Defects: Development of the ASSIST Consortium and a Preliminary Surgical Outcomes Report(2017) CARMONA, Fabio; MANSO, Paulo Henrique; FERREIRA, Mariana Nicoletti; IKARI, Nana Miura; JATENE, Marcelo Biscegli; AMATO, Luciana; TURQUETTO, Aida Luiza; CANEO, Luiz FernandoObjective: ASSIST is the first Brazilian initiative in building a collaborative quality improvement program in pediatric cardiology and congenital heart disease. The purposes of this manuscript are: (a) to describe the development of the ASSIST project, including the historical, philosophical, organizational, and infrastructural components that will facilitate collaborative quality improvement in congenital heart disease care; (b) to report past and ongoing challenges faced; and (c) to report the first preliminary data analysis. Methods: A total of 614 operations were prospectively included in a comprehensive online database between September 2014 and December 2015 in two participating centers. Risk Adjustment for Congenital Heart Surgery (RACHS) 1 and Aristotle Basic Complexity (ABC) scores were obtained. Descriptive statistics were provided, and the predictive values of the two scores for mortality were calculated by multivariate logistic regression models. Results: Many barriers and challenges were faced and overcome. Overall mortality was 13.4%. Independent predictors of in-hospital death were: RACHS-1 categories (3, 4, and 5/6), ABC level 4, and age group (<= 30 days, and 30 days - 1 year). Conclusion: The ASSIST project was successfully created over a solid base of collaborative work. The main challenges faced, and overcome, were lack of institutional support, funding, computational infrastructure, dedicated staff, and trust. RACHS-1 and ABC scores performed well in our case mix. Our preliminary outcome analysis shows opportunities for improvement.