Hematoma Subdural em Pediatria Diagnosticar e Tratar Precocemente

Introdução: O hematoma subdural agudo não traumático é uma entidade rara em Pediatria. A presença de sintomas neurológicos de instalação aguda associada a anti coagulação obriga à exclusão desta entidade. Caso clínico: Apresentamos o caso de uma criança, do sexo masculino, de sete anos de idade, com prótese mitral mecânica, medicada com varfarina, que recorreu ao serviço de urgência por cefaleias intensas e progressivas, associadas a alteração no estado de consciência e convulsões. A nível laboratorial o INR (International Normalized Ratio) era de 4,2. Foi admitida na Unidade de Cuidados Intensivos Pediátricos (UCIP) em coma com anisocoria. Iniciou ventilação mecânica, medidas anti-edema cerebral e antiepilépticos. O exame de imagem mostrou hematoma subdural agudo à esquerda, com desvio da linha média. Foi submetida a craniotomia descompressiva, 56 horas após o início da sintomatologia, com recuperação clínica e actualmente sem sequelas. Conclusão: Este caso clínico ilustra a importância da suspeição clínica de hematoma subdural em doentes anti coagulados, bem como a necessidade de optimização das condições cirúrgicas e da utilização das técnicas não invasivas na monitorização do nível de consciência.

Atherogenesis and atherosclerosis in primary antiphospholipid syndrome

ABSTRACT: In the late seventies the term “Haematological Stress Syndrome” defined some haematological abnormalities appearing in the course of acute and chronic disorders, such as raised plasma levels of fibrinogen (FNG) and factor VIII, reduced fibrinolytic activity and hyperviscosity. In the early nineties the “Membrane stress syndrome hypothesis” proposed the unification of the concepts of haematological stress syndrome with those of oxidation, inflammation and immune activation to explain the pathogenesis of the antiphospholipid syndrome (APS) Antiphospholipid antibodies, coagulation, fibrinolysis and thrombosis. This chapter investigated the occurrence of the “Haematological Stress Syndrome” and thrombosis in 144 participants positive for aPL detected by clotting and immune tests. Among the clotting assays for the detection of lupus anticoagulant, dilute Russell's viper venom time better correlated with a history of venous thrombosis than activated partial thromboplastin time (p<0.0002 vs p<0.009) and was the only test correlated with a history of arterial thrombosis (p<0.01). By regression analysis, serum levels of IgG anticardiolipin antibodies (aCL) associated with the number of venous occlusions (p<0.001). With regards to FNG and von Willebrand factor (vWF), the former rose by 36% (95% CI; 21%, 53%) and the latter by 50% (95% CI; 29%, 75%) at the first venous occlusion and remained unchanged after subsequent occlusions. At variance FNG rose by 45% (95% CI; 31%, 60%) per arterial occlusion and vWF by 27% (95% CI; 10%, 47%) per arterial occlusion throughout. The coagulation/fibrinolytic balance was cross-sectionally evaluated on 18 thrombotic PAPS patients, 18 subjects with persistence of idiopathic aPL and in healthy controls. Markers of thrombin generation prothrombin fragment 1+2 (F1+2), thrombin-antithrombin complex (TAT) and of fibrin turnover D-Dimer (D-D) were higher in thrombotic (p=0.006)and non-thrombotic subjects (p=0.0001) than in controls as were those of D-D (p<0.0001 and p=0.003 respectively). TAT levels did not differ. Gender analysed data revealed blunted tPA release (hence a negative venous occlusion test) in thrombotic females but neither in thrombotic males (p=0.01) nor in asymptomatic subjects of either sex. Also, in both patient groups females had higher mean PAI than males (p<0.0002) and control females (p<0.02). The activity of factor XIII (FXIIIa) was evaluated was evaluated in 29 patients with PAPS, 14 persistent carriers of aPL without thrombosis, 24 thrombotic patients with inherited thrombophilia, 28 healthy controls and 32 patients with mitral and aortic valve prosthesis as controls for FXIII only. FXIIIa was highest in PAPS (p=0.001), particularly in patients with multiple (n=12) than single occlusion (p=0.02) and in correlation with PAI (p=0.003) and FNG (p=0.005). Moreover FXIIIa was strongly associated with IgG aCL and IgG anti-2GPI (p=0.005 for both) in the PAPS group and to a lesser degree in the aPL group (FXIIIa with IgG aCL, p=0.02, with IgG anti-2GPI, p=0.04). Altogether these results indicate: 1) a differential relationship of aPL, vWF and FNG with venous and arterial thrombosis; 2) heightened thrombin generation, accelerated fibrin turnover and fibrinolysis abnormalities also in asymptomatic carriers of aPLs; 3) enhanced FXIIIa that may contribute to atherothrombosis via increased fibrin/fibrinogen cross-linking. Lipid profile, lipid peroxidation and anti-lipoprotein antibodies in thrombotic primary antiphospholipid syndrome. Given the atherogenic lipid profile of SLE, the same possibility was explored in PAPS by comparing high-density lipoprotein (HDL), low-density lipoprotein (LDL), total cholesterol (CHO), apolipoprotein AI (ApoAI), apolipoprotein B (ApoB), triglycerides (TG), anti-lipoprotein antibodies, beta-2-glycoprotein I complexed to oxidized low-density lipoprotein (oxLDL-2GPI) and C-reactive protein (CRP) in 34 thrombotic PAPS patients compared to 36 thrombotic patients with inherited thrombophilia (IT), to 18 subjects persistently positive for antiphospholipid antibodies (aPL) with no underlying autoimmune or non-autoimmune disorders and to 28 healthy controls. Average concentrations of HDL (p<0.0001), LDL (p<0.0001), CHO (p=0.0002), ApoAI (p=0.002) were lower in PAPS whereas average TRY was higher (p=0.01) than other groups. Moreover PAPS showed higher IgG anti-HDL (p=0.01) and IgG anti-ApoAI (p<0.0001) as well as greater average oxLDL-2GPI (p=0.001) and CRP (p=0.003). Within PAPS, IgG anti-HDL correlated negatively to HDL (p=0.004) and was an independent predictor of oxLDL-2GPI (p=0.009). HDL and ApoAI correlated negatively with CRP (p=0.001 and p=0.007, respectively). IgG anti-HDL may hamper the antioxidant and anti-inflammatory effect of HDL favouring low-grade inflammation and enhanced oxidation in thrombotic PAPS. Indeed plasma 8-epi-prostaglandin F2α (a very specific marker of lipid peroxidation) was significantly higher in 10 patients with PAPS than 10 age and sex matched healthy subjects (p=0.0002) and strongly related to the titre of plasma IgG aCL (r=0.89, p=0.0004). Hence oxidative stress, a major player in atherogenesis, also characterises PAPS. Nitric oxide and nitrative stress in thrombotic primary antiphosholipid syndrome. Oxidative stress goes hand in hand with nitrative stress and to address the latter plasma nitrotyrosine (NT, marker of nitrative stress), nitrite (NO2-) and nitrate (NO3-) were measured in 46 thrombotic PAPS patients, 21 asymptomatic but persistent carriers of antiphospholipid antibodies (PCaPL), 38 patients with inherited thrombophilia (IT), 33 patients with systemic lupus erythematosus (SLE) and 29 healthy controls (CTR). Average crude NT was higher in PAPS and SLE (p=0.01) whereas average plasma NO2- was lower in PAPS and average NO3- highest in SLE (p<0.0001). In PAPS, IgG aCL titer and number of vascular occlusions negatively predicted NO2-, (p=0.03 and p=0.001, respectively) whereas arterial occlusions and smoking positively predicted NO3- (p=0.05 and p=0.005). Moreover CRP (an inflammatory marker) positively predicted NT (p=0.004). Nitric oxide metabolites relates to type and number of vascular occlusions and to aPL titers, whereas nitrative stress relates to low grade marker) positively predicted NT (p=0.004). Nitric oxide metabolites relates to type and number of vascular occlusions and to aPL titers, whereas nitrative stress relates to low grade inflammation and both phenomena may have implications for thrombosis and atherosclerosis in PAPS Inflammation and immune activation in thrombotic primary antiphospholipid syndrome. To investigate inflammation and immune activation in thrombotic PAPS high-sensitivity CRP (hs-CRP), serum amyloid A (SAA), oxLDL-2GPI, CRP bound to oxLDL-2GPI (CRP-oxLDL-2GPI) (as inflammatory markers) neopterin (NPT) and soluble CD14 (sCD14) (as immune activation markers) were measured by ELISA in 41 PAPS patients, in 44 patients with inherited thrombophilia (IT) and 39 controls (CTR). Compared to other groups, PAPS presented with higher plasma concentrations of inflammatory, hs-CRP (p=0.0004), SAA (p<0.01), CRP-oxLDL-2GPI (p=0.0004) and immune activation markers, NPT (p<0.0001) and sCD14 (p=0.007). By regression analysis SAA independently predicted thrombosis number (p=0.003) and NPT independently predicted thrombosis type (arterial, p=0.03) and number (p=0.04). These data confirm that low-grade inflammation and immune activation occur and relate to vascular features of PAPS. Antiphosholipid antibodies, haemostatic variables and atherosclerosis in thrombotic primary antiphospholipid syndrome To evaluate whether IgG aCL titre, haemostatic variables and the lipid profile bore any relationship to the intima media thickness (IMT) of carotid arteries high-resolution sonography was applied to the common carotid (CC), carotid bifurcation (CB) and internal carotid (IC) of 42 aPL subjects, 29 with primary thrombotic antiphospholipid syndrome and 13 with persistence of aPL in the absence of any underlying disorder. The following were measured: plasma FNG, vWF, PAI, homocysteine (HC), CHO, TG, HDL, LDL, platelet numbers and aCL of IgG and IgM isotype. By multiple regression analysis, IgG aCL titre independently predicted IMT at all carotid segments examined (p always <0.005). Plasma FNG and HC independently predicted IMT at the CB (p=0.001 and p<0.0001, respectively) and IC (p=0.03 and p<0.0001, respectively). These data strongly support an atherogenic role for IgG aCL in patients with aPL in addition to traditional risk factors. The atherosclerosis hypothesis was investigated in an age and sex-matched case-double-control study including 49 thrombotic PAPS patients (18 M, 31 F, mean age 37 ± 11), 49 thrombotic patients for IT and 49 healthy subjects. Average IMT was always greater in PAPS than control patients (CC: p=0.004, CB: p=0.013, IC: p=0.001). By dividing participants into age tertiles the IMT was greater in the second (CC: p=0.003, CB: p=0.023, IC: p=0.003) and third tertiles (CC: p=0.03, CB: p=0.004, IC: p=0.007). Conclusion: Coagulation activation, fibrinolysis depression, hightened fibrin turnover, oxidative and nitrative stress in parallel with low grade inflammation and immune activation characterise thrombotic PAPS: all these are early atherogenic processes and contribute to the demonstrated premature atherosclerosis that should be considered a clinical feature of PAPS.

Efeitos da laqueação proximal dos vasos curtos e da fundoplicatura total sobre a microcirculação do fundo gástrico : estudo microvascular em cobaias

RESUMO: A operação de Nissen, por laparoscopia, é considerada a cirurgia antirefluxo mais adequada por ser a que melhor replica a fisiologia normal da válvula gastresofágica na maioria dos doentes com sintomas típicos de doença do refluxo gastresofágico (DRGE). São critérios técnicos o encerramento seguro dos pilares do diafragma e a criação de fundoplicatura completa (360 graus), curta (inferior a dois centímetros), lassa e sem tensão – desiderando para o qual a laqueação proximal dos vasos curtos gástricos é crucial. Realizei a operação de Nissen, por laparoscopia, em sessenta mulheres e quarenta homens com DRGE, sem mortalidade operatória, no Serviço de Cirurgia 6 do Hospital dos Capuchos, CHLC, EPE. Os cem doentes apresentavam média etária de 46 anos e queixas, com tempo de evolução entre 1 e 43 anos, de pirose (90%), regurgitação (80%), azia (73%), epigastralgias (54%). A endoscopia alta revelou esofagite de grau Savary-Miller 0-I (62%), II (23%), III (8%), IV (7%); hérnia de deslizamento (71%), hérnia paraesofágica (8%), sem hérnia (21%); a pHmetria de 24h diagnosticou padrão misto (38%), levantado (20%), deitado (20%), inconclusiva (22%) e a manometria diagnosticou EEI hipotónico (35%), peristálise esofágica normal (88%), hipomotilidade ligeira (5%) e foi omissa (7%). Hérnia hiatal, esofagite grave, ineficácia do controlo sintomático com inibidor da bomba de protões e desejo de descontinuidade terapêutica constituíram as indicações para tratamento cirúrgico. Por celioscopia, efetuei laqueação dos vasos curtos gástricos (70%), cruroplastia e fundoplicatura total (seda 2/0), curta (dimensão média 1,5-2 cm), lassa, sem tensão e sem calibração intraoperatória do esófago. A fundoplicatura de Nissen laparoscópica mostrou-se segura e eficaz no tratamento da DRGE. A sua idoneidade foi ainda comprovada pela normalização da pHmetria de 24 horas e da manometria pós-operatórias, com significado estatístico, num grupo de catorze voluntários assintomáticos. Em catamnese com recuo médio 30,7 meses 94% dos indivíduos persistem assintomáticos. Interrogando-me acerca das repercussões desta operação sobre a microcirculação do fundo gástrico coloquei, como premissa, a possibilidade de na operação de Nissen a laqueação dos vasos curtos poder induzir modificação no diâmetro arteriolar da parede do fundo gástrico. Para pesquisar a influência da laqueação dos vasos curtos gástricos e da fundoplicatura total sobre o calibre arteriolar da parede do estômago no cárdia, no fundo e na região dos vasos curtos gástricos, idealizei um Projeto de investigação experimental em cobaias. O Projeto foi desenvolvido no Centro de Investigação do Departamento de Anatomia da FCM-UNL. Para a sua realização obtive autorização da Comissão Científica e Pedagógica da FCM-UNL, requeri a acreditação como investigador à Direção Geral de Veterinária e, por recorrer à utilização de animais, submeti-o à Comissão de Ética da FCM-UNL, que o aprovou por unanimidade. Para limitar o número de animais utilizados ao mínimo necessário, calculei, por método estatístico, a quantidade de cobaias necessárias. Subdividindo-as num grupo de ensaio (GE), onde realizei a operação de Nissen, e num grupo de controlo (GC), onde apenas procedi a tração gástrica, defini e apliquei protocolos de anestesia, de cirurgia e de eutanásia, segundo os princípios dos 3R – Replacement, Reduction, Refinement da técnica de experimentação humana de Russell e Burch (1959) – uma estrutura ética amplamente aceite para a realização de experimentação científica humanizada com animais. A utilização das técnicas de estudo angiomorfológico permitiu-me analisar e descrever a anatomia normal, a vascularização arterial macroscópica, a microangioarquitetura, por microscopia eletrónica de varrimento de moldes de corrosão vascular, e a histologia da parede do estômago da cobaia. Procedi, também, à definição dos critérios morfológicos que considerei suscetíveis de validação deste modelo animal para o estudo proposto. Por razões académicas, foi necessário abreviar o Projeto encurtando, em cerca de dois anos, o prazo disponível para conclusão do estudo. Apreciando-o com o Gabinete de Análise Epidemiológica e Estatística do Centro de Investigação do CHLC, EPE, optou-se, perante a escassez de elementos após já terem sido recrutados 46 animais, por uma amostra, suplementar, de dimensão de conveniência de oito cobaias (quatro em cada grupo), condicionada pelo limite temporal universitário e pelo respeito pela dignidade dos animais. Neste subgrupo procedi, por microscopia eletrónica de varrimento, à medição dos calibres arteriolares nos moldes vasculares do cárdia, do fundo e da zona dos vasos curtos gástricos tanto no GC como no GE efetuando 469 medições no primeiro e 461 no último. Os dados foram enviados ao Centro de Investigação do CHLC, EPE que procedeu à sua análise estatística (ANOVA). A referida análise revelou que as arteríolas do plexo mucoso e as do plexo submucoso do cárdia, do fundo e da região dos vasos curtos gástricos, mostraram aumento de calibre no GE. O aumento foi, estatisticamente, significativo por ser superior a 50% do calibre do GC. Nos vasos curtos, a diferença foi mais pequena, mas persistiu sendo, estatisticamente, significativa. Os vasos retos dilataram na base, na sua emergência do plexo seroso, apenas no fundo gástrico. Na cobaia a operação de Nissen – fundoplicatura total com laqueação dos vasos curtos gástricos –, provocou vasodilatação arteriolar do fundo gástrico. Considero que essa vasodilatação constituiu acomodação à modificação introduzida e infiro que o mesmo possa acontecer no ser humano. Admito, assim, que também ocorra vasodilatação no ser humano, na sequência da laqueação dos vasos curtos gástricos, pela analogia microvascular entre as duas espécies e que essa vasodilatação corresponda, igualmente, a um mecanismo de adaptação arteriolar visando, por exemplo, suprir a perda incorrida pela laqueação. A associação experimental entre laqueação dos vasos curtos gástricos e realização de fundoplicatura total, que exerce aumento inerente de pressão sobre a JEG, não só não provocou défice da microcirculação do esófago distal ou do estômago proximal como desencadeou um mecanismo de vasodilatação fúndica que reforça o conceito de segurança da operação de Nissen para tratamento da DRGE. -------------- ABSTRACT: The laparoscopic Nissen operation is considered to be the most appropriate antirefluxsurgery because it suitably replicates the standard physiology of the gastroesophageal valve in most patients with typical symptoms of gastroesophageal reflux disease (GERD). The technical criteria includes the safe shutdown of the diafragmatic crura(cruroplasty) and the creation of a complete fundoplication (360 degrees), short (lesser than two inches), floppy and without tension – a goal for which the proximal ligation of the gastric short vessels is crucial. The laparoscopic Nissen operation was performed in sixty women and forty men with GERD, without any operative mortality, at the Surgical Department of the Hospital dos Capuchos, CHLC, EPE. The one hundred patients, averaged 46 years old, complained of heartburn (90%), regurgitation (80%) and upper abdominal pain (54 %). The endoscopy process revealed Savary-Miller esophagitis of grade 0-I (62%), II (23%), III (8%), IV (7%), sliding hernia (71%), paraesophageal hernia (8%) or no herniation (21%). The pHmetry/24h diagnosed mixed pattern (38%), raised (20%), lying (20%) or inconclusive (22%). The manometry diagnosed hypotensive LES (35%), normal esophageal peristalsis (88%), mild hypomotility (5%) and was absent (7%). Hiatal hernia, severe esophagitis, ineffective symptomatic control with proton pump inhibitor and request for treatment discontinuation were the signs for surgical action. A laparoscopic ligation of short gastric vessels (70%), cruroplasty and fundoplication (silk 2/0), short (average size 1.5–2 cm) and floppy, without tension and without intraoperative calibration of the esophagus were thus performed. The laparoscopic Nissen fundoplication behaved safe and effective in treating GERD. In a group of 14 asymptomatic volunteers its reputation was confirmed with statistical significance by normalization of postoperative pHmetry/24h and manometry. 94% of the individuals remained asymptomatic up to 30.7 months (average) in the follow-up. Interrogating myself about the impact of this operation on the microcirculation of the gastric fundus I put premised on the possibility of the ligation of the short gastric vessels in the Nissen procedure can induce changes in the arteriolar diameter in the Wall of the gastric fundus. To explore the influence of ligation of the short gastric vessels and the fundoplication at the arteriolar caliber of the cardia, the fundus and the region of the short vessels of the gastric wall, I designed a project of experimental research in guinea pigs with two interdependent components: one veterinary and another technical where I applied angiomorphological studies. The project was developed at the Research Centre of the Department of Anatomy FCMUNL. For its accomplishment I got permission from the Scientific and Pedagogical Committee of the FCM-UNL, I requested for accreditation as a researcher at the General Directorate of Veterinary and, by resorting to the use of animals I submitted it to the Ethics Committee of the FCM-UNL, which approved it unanimously. The guinea pigs were divided into two experimental groups: an experimental group (EG), in which the Nissen procedure was performed and a control group (CG) in which only a gastric traction was done. Protocols of anesthesia, surgery and euthanasia were applied according to the 3Rs – Replacement, Reduction, Refinement of the technique of human experimentation of Burch and Russell (1959) – a widely accepted ethical framework for conducting scientific experiments using animals humanely. Using histological and angiomorphological techniques, I performed the analysis and the description of the normal, macro and microvascular, anatomy of the guinea pig stomach and I defined the morphological criteria that I considered susceptible for validation of this animal model for the proposed study. By means of scanning electron microscopy I measured the arteriolar calibers of the vascular casts of the cardia, of the fundus and of the short gastric vessels in both CG and EG, making 469 measurements in the former and 461 in the latter. The data were sent to the Research Center of the CHLC which conducted the statistical analysis (ANOVA). The data were sent to the Centre for Research of the CHLC, EPE which proceeded to statistical analysis (ANOVA). This analysis revealed that the arterioles plexus of the mucosal and submucosal plexus of the cardia, fundus and region of the short gastric vessels, showed increased caliber in EG. The increase was statistically significant for being greater than 50% CG gauge. In the short gastric vessels, the difference was smaller, but persisted and statistically significant. Straight vessels were dilated at the base, on its emergence of the plexus serous only in the fundus. In the guinea pig, the Nissen procedure - complete fundoplication with ligation of the short gastric vessels - caused arteriolar vasodilation on the gastric fundus. I believe that this vasodilation constituted some accommodation to the modification introduced and infer that the same might happen in humans. I admit therefore that vasodilation also occurs in humans following the ligation of the short gastric vessels by microvascular analogy between the two species and that this vasodilation corresponds also to na adaptation mechanism arteriolar, for example, to compensate the loss incurred by ligation. The association of experimental ligation of the short gastric vessels with conducting complete fundoplication, which exerts increased pressure on the EGJ, not only did not cause a microcirculation deficit of the distal esophagus or proximal stomach as triggered a mechanism of fundic vasodilation which reinforces the security concept of the Nissen procedure for treatment of GERD.

Challenge in the management of infective endocarditis with multiple valvular involvement

We describe the case of a 41-year-old man with congenital heart disease and infective endocarditis (IE), who presented multiple vegetations attached to the pulmonary, mitral, and aortic valves. Three valve replacements were performed, but the patient developed an abscess at the mitral-aortic intervalvular fibrosa and died due to sepsis. We briefly discuss the indications for surgery in IE, emphasizing its role in the treatment of uncontrolled infection.

É a cardiomiopatia hipertrófica apical vista em uma amostra na cidade do Rio de Janeiro similar à encontrada no oriente?

OBJETIVO: Investigar a existência de diferença na forma de apresentação clínica e evolutiva da cardiomiopatia hipertrófica apical (CMHA), vista em uma amostra na cidade do Rio de Janeiro e a observada no oriente, onde foi descrita. MÉTODOS: Entre 156 pacientes com cardiomiopatia hipertrófica diagnosticados em nossa instituição, foram identificados 13 (8,34%) com a forma apical, sendo estudadas as suas manifestações clínicas, os meios de diagnóstico e a sua evolução. RESULTADOS: Eram 8 homens e 5 mulheres, com idades entre 19 a 75 anos, todos da raça branca, a maioria sintomática. O eletrocardiograma revelou ondas T gigantes em precordiais em 10 pacientes (76,92%), o ecocardiograma (ECO), a presença de hipertrofia apical em todos os casos, sendo em 10 (76,92%) restrita a ponta do ventrículo esquerdo (VE), em dois (15,4%) a ponta do ventrículo direito (VD) e, em 1 (7,68%), acometendo ambos os ventrículos. A cineventriculografia confirmou os achados do ECO. A evolução variou de 6 a 264 meses (x=95,4). Ocorreram dois óbitos (15,4%) em pacientes com o comprometimento associado ao VD, devido ao aumento significativo da dimensão dos átrios, ocorrência de fibrilação atrial, insuficiência valvar mitral e/ou tricúspide e tromboembolismo. Entre os vivos, a paciente com acometimento do VD evolui com restrição diastólica, e os restantes com lesão localizada do VE , 9 estão assintomáticos em uso de propranolol (8) ou amiodarona (1) e um permanece assintomático sem medicação. CONCLUSÃO: presentação, diagnóstico e evolução, porém notamos em 3 pacientes ( 23,08%) o acometimento da ponta do VD, não descrito no oriente, mas com péssimo prognóstico evolutivo.

Evolução intra-hospitalar e seguimento pós-alta de pacientes idosos atendidos com insuficiência cardíaca congestiva na unidade de emergência

OBJETIVO: Conhecer a evolução intra-hospitalar (IH) e pós-alta (PA) de uma população predominantemente idosa, com insuficiência cardíaca congestiva (ICC) na unidade de emergência (UE). MÉTODOS: Durante 11 meses, foram selecionados 57 pacientes consecutivos com ICC, atendidos em EU, com idade média de 69+15 (27 a 94) anos, sendo 39 (68,4%) homens. O diagnóstico de ICC baseou-se nos critérios de Boston. Avaliou-se a evolução IH e PA num período médio de 5,7+2,7 (1 a 12) meses, procurando-se identificar variáveis que se correlacionassem com a mortalidade e o mecanismo de morte, avaliado pelo sistema ACME . RESULTADOS: Oito (14%) pacientes faleceram na fase IH, sendo 7 por falência circulatória (FC), e 1 em pós-operatório (PO). Durante o seguimento ocorreram 9 (18,4%) óbitos, sendo 5 por FC, 2 mortes súbitas e 2 em PO (troca valvar mitral e ventriculectomia). A sobrevida dos pacientes que tiveram alta foi de 82% e 66%, aos 6 meses e 1 ano, respectivamente. Sódio sérico <135mEq/l (p= 0,004) e sexo feminino (p= 0,038) foram preditores independentes de mortalidade. CONCLUSÃO: Pacientes idosos com ICC atendidos em UE apresentam mortalidade IH e PA elevadas e alta taxa de reinternação hospitalar. A maioria morre por falência circulatória decorrente da progressão da ICC.

Cirurgia valvar em gestantes. Experiência em oito casos

OBJETIVO: Analisar, em gestantes, os resultados da cirurgia cardíaca e as conseqüências da circulação extracorpórea (CEC) nas gestantes e fetos. MÉTODOS: Estudo de 8 gestantes submetidas à cirurgia cardíaca valvar entre janeiro/86 e dezembro/96. Idade média de 31,4±8,9 anos, e idade gestacional entre 12 a 31 (média de 26,6±7,1) semanas. Em todas as pacientes foi realizada monitorização fetal. Durante a CEC manteve-se sempre o fluxo elevado e a temperatura acima de 34°C. Foram realizadas 4 cirurgias da valva aórtica e 4 da valva mitral, sendo duas reoperações. RESULTADOS: Não houve mortalidade materna. Ocorreram dois partos prematuros e em uma criança houve comprometimento neurológico. As demais crianças não apresentaram nenhum comprometimento em seu desenvolvimento. CONCLUSÃO: Os cuidados com o fluxo durante a CEC e a monitorização fetal no intra-operatório foram fundamentais para a obtenção de resultados bastante satisfatórios.

Avaliação morfo-funcional cardíaca em jovens normotensos, filhos de hipertensos. Estudo Doppler-ecocardiográfico prospectivo

OBJETIVO: Avaliar pelo Doppler-ecocardiograma alterações cardíacas, estrutural e/ou funcionais, que podem surgir nos jovens normotensos com história familiar de hipertensão arterial sistêmica (HAS). MÉTODOS: Estudo prospectivo realizado com 62 jovens normotensos entre 15 e 30 anos, sendo 32 filhos de hipertensos (grupo 1) e 30 filhos de normotensos (grupo 2) comparáveis quanto à pressão arterial, superfície corporal, freqüência cardíaca, idade e sexo. Após exame clínico, todos realizaram Doppler-ecocardiograma para avaliar as estruturas cardíacas e a função ventricular esquerda (sistólico e diastólica). RESULTADOS: A fração de encurtamento sistólico do ventrículo esquerdo (VE) foi significativamente maior no grupo 1 - valor médio de 38,03±4,95% - do que no grupo 2 - 34,7±4,48 % (p<0,01). O tempo de desaceleração mitral (TD) variou de 85 a 160ms - médio de 116,47±16,99ms - no grupo 1 e de 100 a 220ms - médio de 126,73±26,66ms - no grupo 2 (p<0,05). Houve correlação entre o índice de massa do VE e o diâmetro do átrio esquerdo (AE) no grupo 1 (r= 0,514, p<0,01). CONCLUSÃO: Os filhos de hipertensos, quando comparados com filhos de normotensos, exibem exacerbação da função sistólica do VE, semelhante ao que ocorre na fase inicial da HAS ou na HAS "borderline", mesmo que não haja hipertrofia do VE ou aumento dos níveis pressóricos. O TD mitral (mais curto no grupo 1) foi o único parâmetro de função diastólica do VE que diferiu nos 2 grupos analisados. A correlação entre o índice de massa do VE e o diâmetro do AE nos filhos de hipertensos sugere que o AE se modifica de acordo com as alterações funcionais e hemodinâmicas do VE.

Cholinergic stimulation with pyridostigmine, hemodynamic and echocardiographic analysis in healthy subjects

OBJECTIVE: Growing evidence suggests that sudden death after an acute myocardial infarction (AMI) correlates with autonomic nervous system imbalance. Parasympathomimetic drugs have been tested to reverse these changes. However, their effects on ventricular function need specific evaluation. Our objective was to analyze pyridostigmine's (PYR) effect on hemodynamic and echocardiographic variables of ventricular function. METHODS: Twenty healthy volunteers underwent Doppler echocardiographic evaluations, blood pressure (BP), and heart rate (HR) assessment at rest, before and 120 min after ingestion of 30 mg PYR or placebo, according to a double-blind, placebo-controlled, crossed and randomized protocol, on different days. RESULTS: PYR was well tolerated and did not cause alterations in BP or in ventricular systolic function. A reduction in HR of 10.9±1.3% occurred (p<0,00001). There was an A wave reduction in the mitral flow (p<0.01) and an E/A ratio increase (p<0.001) without changes in the other diastolic function parameters (p>0.05). CONCLUSION: PYR reduces HR and increases E/A ratio, without hemodynamic impairment or ventricular function change.

Dynamic three-dimensional reconstruction of the heart by transesophageal echocardiography

OBJECTIVE: To evaluate echocardiography accuracy in performing and obtaining images for dynamical three-dimensional (3D) reconstruction. METHODS: Three-dimensional (3D) image reconstruction was obtained in 20 consecutive patients who underwent transesophageal echocardiography. A multiplanar 5 MHz transducer was used for 3D reconstruction. RESULTS: Twenty patients were studied consecutively. The following cardiac diseases were present: valvar prostheses-6 (2 mitral, 2 aortic and 2 mitral and aortic); mitral valve prolapse- 3; mitral and aortic disease - 2; aortic valve disease- 5; congenital heart disease- 3 (2 atrial septal defect- ASD - and 1 transposition of the great arteries -TGA); arteriovenous fistula- 1. In 7 patients, color Doppler was also obtained and used for 3D flow reconstruction. Twenty five cardiac structures were acquired and 60 reconstructions generated (28 of mitral valves, 14 of aortic valves, 4 of mitral prostheses, 7 of aortic prostheses and 7 of the ASD). Fifty five of 60 (91.6%) reconstructions were considered of good quality by 2 independent observers. The 11 reconstructed mitral valves/prostheses and the 2 reconstructed ASDs provided more anatomical information than two dimensional echocardiography (2DE) alone. CONCLUSION: 3D echocardiography using a transesophageal transducer is a feasible technique, which improves detection of anatomical details of cardiac structures, particularly of the mitral valve and atrial septum.

Rheumatic carditis treated with high doses of pulsetherapy methylprednisolone. Results in 70 children over 12 years

PURPOSE: To report the result of patients treated with IV methylprednisolone divided into three groups and compare their follow-up during the last 12 years. METHODS: Seventy children with active rheumatic carditis (76 episodes) in heart failure Class III and IV (NYHA) were studied. The diagnosis was based on modified Jones' criteria. After ruling out infections and strongyloidiasis, treatment with IV methylprednisolone bolus was started three times a week until the laboratory tests became negative. Patients were divided into 3 groups, according to the time of hospital admittance: Groups 1, 2 and 3, comprising of 40, 18 and 12 children, respectively. RESULTS: Eighteen children in Group 1 (45%) were in their 1st attack: 2 series of pulsetherapy were used in 10 (25%), 3 in 9 (23%) and 4 in 21 (52%). In Group 2, 14 cases (77%) were in their 1st attack: 2 series were used in 7 (39%), 4 in 9 (50%) and 5 in 2 (11%). The echocardiogram showed a flail mitral valve in 12 (66%) of these patients (1 death occurred after mitral valvoplasty). In Group 3, 6 patients needed 5 or more series of pulsetherapy and a flail mitral valve was present in 5 (41%). One child underwent mitral valve replacement while still in the active phase, after 8 series of pulsetherapy, and another died. The number of patients who needed 5 or more series was significantly higher in Group 3. CONCLUSION: There were variations in the presentation and evolution of the cases during these 12 year. The established pulsetherapy protocol continues to be useful to treat severe cases.

Results of the surgical treatment of chronic atrial fibrillation

OBJECTIVE: Report clinical experience in surgical treatment of atrial fibrillation (AF) by Cox-maze procedure. METHODS: 61 patients underwent surgical treatment for AF. Two had primary AF and 59 AF secondary to heart disease (2 atrial septal defects, 57 mitral). Ages ranged from 20 to 74 years (mean = 49). There were 44 females (72%). The surgical technique employed was Cox 3 without cryoablation. The patients were follow-up in specific at patient clinics and underwent periodical ECG, exercise tests, echocardiogram and Holter monitoring. RESULTS: In-hospital mortality was 4.9% and late mortality 1.6%. A temporary pacemaker was used in 28 (46%) and a definitive in 7 patients (11.4%). On hospital discharge, AF remained in 17%; 63.9% had sinus rhythm, 6.9% atrial rhythm, 1.7% junctional rhythm, and 10.3% had pacemaker rhythm. In the last evaluation, AF was present in 19.5%; (70.5% sinus rhythm, 4% atrial rhythm, 2% atrial tachycardia, and 4% pacemaker rhythm). There was no report of thromboembolic episodes. Chronotropic response was considered adequate in 19%, intermediate in 29%, and inadequate in 42%. In Holter monitoring, the mean heart rate was 82±8 bpm, with a minimum of 57±7 bpm and maximum of 126±23 bpm, with supraventricular extrasystoles in 2.3±5.5% of the total heartbeats and ventricular extrasystoles in 0.8±0.5%. In the echocardiogram, the A wave was present in the left atrium in 87.5%. CONCLUSION: Maze procedure is effective and has acceptable surgical risk. Atrial or sinus rhythms remain stable with a small but remarkable frequency of atrial and ventricular arrhythmias. Left atrial contraction is present, although attenuated, as well as the chronotropic response to exercise.

Immediate and long term evolution of valve replacement in children less than 12 years old

OBJECTIVE: The aim of this work was the follow-up and evaluation of valve replacement in children under 12 years of age. METHODS: Forty-four children less than 12 years old were underwent valve replacement at INCOR-HCFMUSP between January 1986 and December 1992. Forty (91%) were rheumatic, 39 (88.7%) were in functional classes II or IV, 19 (43.2%) were operated upon on an emergency basis, and 6 (13.6%) had atrial fibrillation. Biological prostheses (BP) were employed in 26 patients (59.1%), and mechanical prostheses (MP) in 18 (40.9%). Mitral valves were replaced in 30 (68.7%), aortic valves in 8 (18.2%), a tricuspid valve in 1 (2.3%), and double (aortic and mitral) valves in 5 (11.4) of the patients. RESULTS: Hospital mortality was of 4.5% (2 cases). The mean follow-up period was 5.8 years. Re-operations occurred in 63.3% of the patients with BP and in 12.5% of those with MP (p=0.002). Infectious endocarditis was present in 26.3% of the BP, but in none of the cases of MP (p=0.049). Thrombosis occurred in 2 (12.5%) and hemorrhage in one (6.5%) of the patients with a MP. Delayed mortality occurred in 5 (11.9%) of the patients over a mean period of 2.6 years; four had had BP and one had a MP (NS). Actuarial survival and re-operation-free curves after 10 years were respectively, 82.5±7.7 (SD)% and 20.6±15.9%. CONCLUSION: Patients with MP required fewer re-operation, had less infectious endocarditis and lower late mortality rates compared with patients with bioprostheses. The former, therefore, appear to be the best valve replacement for pediatric patients.

Doppler echocardiographic study in adolescents and young adults with sickle cell anemia

OBJECTIVE: Anatomical and functional assessment of the heart through Doppler and echocardiography in patients with cell anemia (SCA). METHODS: Twenty-five patients with SCA and ages ranging from 14 to 45 years were prospectively studied in a comparison with 25 healthy volunteers. All of them underwent clinical and laboratory evaluation and Doppler echocardiography as well.The measurements were converted into body surface indices. RESULTS: There were increases in all chamber diameters and left ventricle (LV) mass of the SCA patients. It was characterised an eccentric hypertrophy of the left ventricle. The preload was increased (left ventricle end-diastolic volume) and the afterload was decreased (diastolic blood pressure, peripheral vascular resistance and end-systolic parietal stress ESPS). The cardiac index was increased due to the stroke volume. The ejection fraction and the percentage of the systolic shortening , as well as the systolic time intervals of the LV were equivalent. The isovolumetric contraction period of the LV was increased. The mitral E-septum distance and the end-systolic volume index (ESVi) were increased. The ESPS/ESVi ratio,a loading independent parameter, was decreased in SCA, suggesting systolic dysfunction. No significant differences in the diastolic function or in the pulmonary pressure occurred. CONCLUSION: Chamber dilations, eccentric hypertrophy and systolic dysfunction confirm the evidence of the literature in characterizing a sickle cell anemia cardiomyopathy.

Coronary bilateral ostial enlargement using the saphenous vein in a patient with syphilitic aortitis

A patient with tertiary syphilis presenting with bilateral coronary ostial lesions and aortic regurgitation underwent surgical reconstruction of the coronary ostia by the anterior approach with autogenous saphenous vein grafting and substitution of the aortic valve with a bovine bioprosthesis. The procedure was easily performed and had good outcomes both early and late. The rarity of the association of a lesion in both coronary ostia with aortic regurgitation in syphilis and the surgical technique employed are discussed.