IntroductionCongenital heart disease is an abnormality in the structure or cardiocirculatory function, occurring from birth, even if diagnosed later. It can result in intrauterine death in childhood or in adulthood. Accounted for 6% of infant deaths in Brazil in 2007.ObjectiveTo estimate underreporting in the prevalence of congenital heart disease in Brazil and its subtypes.MethodsThe calculations of prevalence were performed by applying coefficients, giving them function rates for calculations of health problems. The study makes an approach between the literature and the governmental registries. It was adopted an estimate of 9: 1000 births and prevalence rates for subtypes applied to births of 2010. Estimates of births with congenital heart disease were compared with the reports to the Ministry of Health and were studied by descriptive methods with the use of rates and coefficients represented in tables.ResultsThe incidence in Brazil is 25,757 new cases/year, distributed in: North 2,758; Northeast 7,570; Southeast 10,112; South 3,329; and Midwest 1,987. In 2010, were reported to System of Live Birth Information of Ministry of Health 1,377 cases of babies with congenital heart disease, representing 5.3% of the estimated for Brazil. In the same period, the most common subtypes were: ventricular septal defect (7,498); atrial septal defect (4,693); persistent ductus arteriosus (2,490); pulmonary stenosis (1,431); tetralogy of Fallot (973); coarctation of the aorta (973); transposition of the great arteries (887); and aortic stenosis 630. The prevalence of congenital heart disease, for the year of 2009, was 675,495 children and adolescents and 552,092 adults.ConclusionIn Brazil, there is underreporting in the prevalence of congenital heart disease, signaling the need for adjustments in the methodology of registration.

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OBJETIVO: Este trabalho relata a experiência no manuseio da crise hipertensiva pulmonar (CHP) refratária, com o uso do óxido nítrico inalatório (NOi) no pós-operatório imediato do transplante cardíaco (TC) ortotópico. MÉTODOS: De outubro/1997 a fevereiro/2002 foram realizados 31 TC em adultos, sendo incluídos pacientes com RVP < 6uW, ou < 2,5uW na prova farmacológica. Após o período de circulação extracorpórea (CEC) (M=101±21 minutos), todos fizeram uso de dobutamina, dopamina e milrinona; entretanto, frente à ausência de resposta adequada e CHP, administrou-se NOi em doses crescentes de 20 a 40ppm. Foram utilizados registros das pressões através de cateter no átrio esquerdo e no tronco da artéria pulmonar, gasometria arterial seriada e ecocardiograma transtorácico (ETT). O tempo médio de morte encefálica (ME) do doador foi de 16±5,1horas.RESULTADOS: Cinco pacientes (1 mulher), com idade média de 42 anos, fizeram uso de NOi por apresentarem critérios de CHP, todos com sinais de baixo débito cardíaco. O tempo médio de CEC foi de 150,8±34,3 minutos, a média sistólica arterial pulmonar foi de 87mmHg (75-115) e a PO2 média de 60mmHg (FiO2 100%), sendo evidenciada dilatação moderada a severa do ventrículo direito no ETT. Foi administrado NOi durante uma média 35h (6-96), iniciando-se desmame após estabilização hemodinâmica. O tempo médio de ME do doador foi de 27,6±5,5horas, a internação hospitalar média foi de 63 dias (17-145), uma morte ocorreu por sepse no 17º PO e os demais pacientes estão em CF I (NYHA). CONCLUSÕES: O tempo de CEC superior a 120 minutos e tempo de ME do doador superior a 20 horas sugerem fatores de risco para CHP no período pós-operatório imediato do TC. O NOi é uma ferramenta útil no manuseio desta complicação, refratária ao tratamento convencional no POI de pacientes submetidos ao TC.
OBJECTIVE: This study reviews our experience with the use of nitric oxide inhalation (NOi) in the treatment of the refractory pulmonary hypertensive crisis (PHC) during the immediate post-operative period (IPOP) of heart transplantation (HT). METHOD: Between Oct/97 and Feb/02, 31 HT in adults were performed. Among them were patients with Pulmonary Vascular Resistance (PVR) greater than 6 uW, or patients with PVR greater than 2,5 uW during the pharmacological test. All patients were weaned from the CPB (M=101±21 minutes) in use of dobutamine, dopamine and milrinone. In the absence of an adequate response to these drugs and with the evidence of PHC, NOi was given, beginning with 20ppm and increasing, when necessary, up to 40ppm. The recording of the left atrium and pulmonary artery pressures, serial arterial blood gases samples and trans-thoracic echocardiograms (TTE) were performed. The donor's brain death (BD) time mean was 16±5,1 hours. RESULTS: In five patients (1 female) (mean age = 42 years) NOi was employed, due to low cardiac output signs and evidence of PHC and right ventricular dysfunction. The mean CPB time was 150,8±34,3 min. The pre-NOi mean PAP was 8...

The perspective of the integrated health system has a network of care with multiple integration dimensions among subsystems as nuclear representation, relating the clinical aspects and governance to the representations and collective values. The normative integration aims to ensure coherence between the system of representations and values of society simultaneously with the interfaces of clinical and functional integration. It builds a bridge with governance, which allows, through their skills, management of all system components, encouraging cooperation, communication and information, in order to ensure the population under their responsibility to access excellence services, exceeding their expectations. The integration of care consists of a durable coordination of clinical practices for those who suffer from health problems in order to ensure continuity and full range of the required professional services and organizations, coordinated in time and space, in accordance with the available knowledge. It is possible to establish the type of health equipment for each level of care for patients with congenital heart diseases. This strategy intends to offer timely care in appropriate moments and places, efficiently, operating cooperatively an interdependently, with ongoing exchange of its resources. Thus, situational integration establishes the system connection with the assessment environment that proposes to carry out value judgment, guided by an objective worldview, about an intervention or any of its components, in order to objectify the decision making.

Objetivo: Demonstrar a viabilidade na feitura de túnel cava inferior-cava superior com retalho da parede atrial direita, evitando o emprego de material protético. Casuística e Métodos: Foram operados 2 pacientes nos quais se empregou a técnica de anastomose cavo-pulmonar total, sem uso de material protético. O primeiro caso, A.L.M., masc, 4 anos, 15 kg, era portador de atresia tricúspide (EP), com comunicação interventricular (CIV) restritiva. O segundo caso, M.E.N.O., fem, 15 anos, 47 kg, tinha doença de Ebstein. O controle pós-operatório dos pacientes foi feito com ecocardiograma e cateterismo cardíaco. As operações foram realizadas com o emprego de circulação extracorpórea (CEC), e cardioplegia sangüínea como método de proteção miocárdica. A canulação das cavas foi o mais distal possível. A tunelização foi realizada com retalho de tecido atrial direito, suturado ao septo interatrial, deixando-se o seio coronariano e a comunicação interatrial (CIA) para a esquerda. Resultados: Ambos os pacientes evoluíram, sem complicações, na UTI. O primeiro apresentou derrame pleural discreto à direita, e o segundo mantém-se em estimulação artificial (VVI,R). Conclusões: A tunelização intra-atrial para anastomose cavo-pulmonar total pode ser realizada sem o uso de material protético, evitando-se os riscos advindos do seu emprego (calcificação, retração, embolização).
The authors describe two cases (one tricuspid atresia without TGA or pulmonary stenosis, but with a restrictive IVC: the second of an Ebstein disease) of total cavo-pulmonary anastomosis using only the tissue of the atrial wall, avoiding any prosthetic material. Both cases were done under cardiopulmonary bypass and cold blood cardioplegia. A flap of the right atrial wall was used to re-direct the blood flow. Pleural effusion in one patient and complete AV block in the other were the complications found. They conclude that right intra-atrial tunneling for total cavo-pulmonary anastomosis, avoiding the use of any foreign material is feasible, thus diminishing the possible late risks (calcification, embolization, retraction) of prosthetic materials