MARCELO BISCEGLI JATENE
Projetos de Pesquisa
Unidades Organizacionais
Departamento de Cardio-Pneumologia, Faculdade de Medicina - Docente
Instituto do Coração, Hospital das Clínicas, Faculdade de Medicina
LIM/11 - Laboratório de Cirurgia Cardiovascular e Fisiopatologia da Circulação, Hospital das Clínicas, Faculdade de Medicina - Líder
Instituto do Coração, Hospital das Clínicas, Faculdade de Medicina
LIM/11 - Laboratório de Cirurgia Cardiovascular e Fisiopatologia da Circulação, Hospital das Clínicas, Faculdade de Medicina - Líder
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- 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.
- Suporte Circulatório como Ponte para Transplante Cardíaco Pediátrico(2016) CANEO, Luiz Fernando; MIANA, Leonardo A.; JATENE, Marcelo B.
- 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.
- Surgical Correction of Ascending Aortic Aneurysm Without Coronary Dilatation After Kawasaki Disease in a 3-Year-Old Child(2022) SANTOS, Romullo M.; MASSOTI, Maria Raquel B.; CORONEL, Monica Raquel Gonzalez; MAIA, Frederico Faria; MIANA, Leonardo Augusto; JATENE, Marcelo BiscegliKawasaki disease (KD) is an inflammatory condition that may affect genetically predisposed individuals in pediatric ages after infectious/ environmental triggering. An infrequent finding associated with KD is ascending aortic aneurysm during or after the acute phase of the disease. In this Multimedia presentation, we describe a three-year-old girl submitted to surgical treatment.
- 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 Insuficiência cardíaca congestiva e transplante cardíaco na criança(2016) AZEKA, Estela; RIBEIRO, Anna Christina; THOMAZ, Ana Maria; GUIMARãES, Vanessa; GALAS, Filomena Regina; HOROWITZ, Estela; SIQUEIRA, Adailson; BENVENUTI, Luiz; ZORZANELLI, Leina; JATENE, MarceloconferenceObject Stenting the Right Ventricular Outflow Tract as a Palliative Procedure in Patients with Tetralogy of Fallot(2014) PEDRA, Carlos; COSTA, Rodrigo; RIBEIRO, Marcelo; NASCIMENTO, Wanda; PEDRA, Simone; FURLANETTO, Glaucio; JATENE, Marcelo; ELIAS, Patricia; SANTANA, Virginia; FONTES, Valmir- Impact of Vacuum-Assisted Venous Drainage on Forward Flow in Simulated Pediatric Cardiopulmonary Bypass Circuits Utilizing a Centrifugal Arterial Pump Head(2020) GUIMARAES, Daniel Peres; CANEO, Luiz Fernando; MATTE, Gregory S.; CARLETTO, Luciana P.; POLICARPO, Valeria Camargo; CASTRO, Ana Vitoria C. X.; MIRANDA, Matheus H. C.; COSTA, Priscila S.; JATENE, Marcelo B.; CESTARI, Idagene; JATENE, Fabio B.Objective: To analyze the impact of vacuum-assisted venous drainage (VAVD) on arterial pump flow in a simulated pediatric cardiopulmonary bypass circuit utilizing a centrifugal pump (CP) with an external arterial filter. Methods: The simulation circuit consisted of a Quadrox-I Pediatric oxygenator, a Rotaflow CP (Maquet Cardiopulmonary AG, Rastatt, Germany), and a custom pediatric tubing set primed with Lactated Ringer's solution and packed red blood cells. Venous line pressure, reservoir pressure, and arterial flow were measured with VAVD turned off to record baseline values. Four other conditions were tested with progressively higher vacuum pressures (-20, -40, -60, and -80 mmHg) applied to the baseline cardiotomy pressure. An arterial filter was placed into the circuit and arterial flow was measured with the purge line in both open and closed positions. These trials were repeated at set arterial flow rates of 1500, 2000, and 2500 mL/min. Results: The use of progressively higher vacuum caused a reduction in effective arterial flow from 1490 +/- 0.00 to 590 +/- 0.00, from 2020 +/- 0.01 to 1220 +/- 0.00, and from 2490 +/- 0.0 to 1830 +/- 0.01 mL/min. Effective forward flow decreased with increased levels of VAVD. Conclusion: The use of VAVD reduces arterial flow when a CP is used as the main arterial pump. The reduction in the forward arterial flow increases as the vacuum level increases. The loss of forward flow is further reduced when the arterial filter purge line is kept in the recommended open position. An independent flow probe is essential to monitor pump flow during cardiopulmonary bypass.
- A model for preservation of thymocyte-depleted thymus(2023) DIAS, A. S.; DAMACENO-RODRIGUES, N. R.; GIMENEZ, T. M.; OLIVEIRA, P. M.; ZERBINI, M. C.; CARNEIRO-SAMPAIO, M.; FILHO, V. Odone; JATENE, M. B.; VASCONCELOS, D. M.; ROCHA, V.; NOVAK, E. M.DiGeorge syndrome is a disorder caused by a microdeletion on the long arm of chromosome 22. Approximately 1% of patients diagnosed with DiGeorge syndrome may have an absence of a functional thymus, which characterizes the complete form of the syndrome. These patients require urgent treatment to reconstitute T cell immunity. Thymus transplantation is a promising investigational procedure for reconstitution of thymic function in infants with congenital athymia. Here, we demonstrate a possible optimization of the preparation of thymus slices for transplantation through prior depletion of thymocytes and leukocyte cell lineages followed by cryopreservation with cryoprotective media (5% dextran FP 40, 5% Me2SO, and 5% FBS) while preserving tissue architecture. Thymus fragments were stored in liquid nitrogen at -196 & DEG;C for 30 days or one year. The tissue architecture of the fragments was preserved, including the distinction between medullary thymic epithelial cells (TECs), cortical TECs, and Hassall bodies. Moreover, depleted thymus fragments cryopreserved for one year were recolonized by intrathymic injections of 3 x 106 thymocytes per mL, demonstrating the capability of these fragments to support T cell development. Thus, this technique opens up the possibility of freezing and storing large volumes of thymus tissue for immediate transplantation into patients with DiGeorge syndrome or atypical (Omenn-like) phenotype.
- Parameters associated with outcome in pediatric patients with congenital heart disease and pulmonary hypertension subjected to combined vasodilator and surgical treatments(2019) THOMAZ, Ana Maria; KAJITA, Luiz J.; AIELLO, Vera D.; ZORZANELLI, Leina; GALAS, Filomena Regina B. G.; MACHADO, Cleide G.; BARBERO-MARCIAL, Miguel; JATENE, Marcelo B.; RABINOVITCH, Marlene; LOPES, Antonio AugustoManagement of pediatric pulmonary hypertension associated with congenital heart disease (PHT-CHD) is challenging. Some patients have persistently elevated pulmonary artery pressure (PAP) after cardiac surgery, an undesired condition that is difficult to predict. We investigated the value of clinical, hemodynamic, and histopathological data in predicting the outcome in a prospective cohort. Patients with PHT-CHD received sildenafil orally pre- and postoperatively for six months and then were subjected to a catheter study. Thirty-three patients were enrolled (age range = 4.6-37.0 months). Pulmonary vascular resistance (PVR) was 4.9 (range = 3.9-7.2) Wood units x m(2) (median with IQR). Twenty-two patients had a >= 20% decrease in PVR and pulmonary-to-systemic vascular resistance ratio (PVR/SVR) in response to inhaled nitric oxide (NO). The response was directly related to the degree of medial hypertrophy of pulmonary arterioles (P < 0.05) (morphometric analysis, intraoperative lung biopsy). Subsequently, five of the non-responders had a >= 30% increase in pulmonary blood flow in response to sildenafil (3.0 [2.0-4.0] mg/kg/day). Six months after surgery, PAP and PVR were significantly lower (P < 0.001 vs. baseline), even in seven patients with Heath-Edwards grade III/IV pulmonary vascular lesions (P = 0.018), but still abnormal in 12 individuals (>25 mmHg and >3.0 U x m(2), respectively). A preoperative PVR/SVR of >= 24% during NO inhalation and a wall thickness of arteries accompanying respiratory bronchioli of >= 4.7 (Z score) were identified, respectively, as risk and protection factors for abnormal postoperative hemodynamics (hazard ratio [95% CI] = 1.09 [1.01-1.18], P = 0.036; and 0.69 [0.49-0.98], P = 0.040, respectively). Thus, in PHT-CHD patients receiving oral sildenafil pre- and post-surgical repair of cardiac lesions, mid-term postoperative outcome is predictable to some extent.