Ziel: Die partielle Ventrikulektomie (PLV) stellt eine chirurgische Therapieoption der terminalen Herzinsuffizienz dar. In dieser Arbeit wurde der Einfluss der PLV auf die kardiale Leistungsfähigkeit untersucht sowie eine Überlebensanalyse durchgeführt. Ferner sollte eine Aussage zum Verhalten der postoperativen linksventrikulären Funktion unter Belastung gemacht werden. Dabei wurden zwei Gruppen miteinander verglichen: 1. Patienten mit idiopathischer Kardiomyopathie (Gruppe ID) und 2. Patienten mit ischämischer Myopathie (Gruppe IS). Material und Methodik: Insgesamt wurden 75 Patienten, die sich im Zeitraum von 1995 bis 1999 in der Klinik für kardiovaskuläre Chirurgie der Charité einer PLV unterzogen hatten, berücksichtigt. Die postoperativen Untersuchungen erfolgten an 19 Patienten. Die linksventrikulären Funktionsparameter wurden mittels transthorakaler und transösophagealer Echokardiographie einschließlich Stressechokardiographie erhoben. Ergebnisse: In beiden Gruppen war postoperativ eine hochsignifikante Verbesserung der NYHA-Klassifikation zu registrieren. Die echokardiographischen Untersuchungen ergaben in beiden Gruppen eine Verbesserung der Ejektionsfraktion (EF) als Maß für die linksventrikuläre Funktion. Ferner war eine Verringerung des linksventrikulären enddiastolischen Durchmessers (LVEDD) und ein signifikanter Rückgang der Mitralinsuffizienz (MI) festzustellen. Die Stressechokardiographie zeigte für die Gruppe IS eine Steigerung der Kontraktilität unter Dobutamin mit tendenziell physiologischen Befunden. (signifikanter Anstieg von EF, cardiac index (CI), fractional area change (FAC) sowie Abnahme des linksventrikulären endsystolischen Volumenindex (LVESVI)). Für die Gruppe ID konnte dies nur bedingt gezeigt werden. Bei der Überlebensanalyse ergaben sich ebenfalls ein signifikant besseres Abschneiden der Gruppe IS, insgesamt lag die Überlebensrate hinsichtlich kardialer Todesursachen bei 69,9 % (1. Jahr) und 66,7 % (2. Jahr). Schlussfolgerungen: Beurteilend lässt sich feststellen, dass die überlebenden und zur Nachuntersuchung erschienenen Patienten mit einer Steigerung der körperlichen Belastbarkeit und Besserung der Ventrikelfunktionsparameter von der Operation profitieren konnten, wobei sich ein besseres Abschneiden der Gruppe IS zeigte. Dazu passend wies auch die stressechokardiographische Untersuchung vor allem für die Gruppe IS postoperativ auf myokardiale Reserven hin. Die hier aufgezeigten Unterschiede der Gruppen, die sich auch in den Überlebensraten widerspiegeln, machen die Bedeutung von guten Selektionskriterien deutlich, die in weiteren Studien noch differenzierter betrachtet werden müssen. Damit zusammenhängend kommt auch der Anpassung der mit der PLV kombinierten Eingriffe an die Ursachen der Myopathien besondere Wichtigkeit zu. / Aim: Partial left ventriculectomy (PLV) represents a surgical option for the treatment of end-stage-heart failure. The aim of this thesis was an investigation of the influence of PLV on LV-function and on clinical outcome and to analyse survival in a group of patients submitted to PLV: Also, post surgical left ventricular function under stress conditions was determined. Two groups served as a basis for comparison: 1. patients with idiopathic dilated cardiomyopathy (ID group) and 2. patients with ischemic myopathy (IS group). Methods: 75 patients undergoing PLV at the clinic for cardiovascular surgery at the Charité Berlin between 1995 and 1999 were considered. LV-function was determined by transthoracic and transesophageal echocardiography including stress echocardiography. Results: The NYHA-classification improved in both groups significantly after the surgery. The echocardiography showed a significant increase in ejection fraction (EF), a reduction of left ventricular enddiastolic diameter (LVEDD) and a significant decrease in mitral insufficiency (MI). The post surgical stress-echocardiography indicated a rise of left ventricular contractility under dobutamine with a tendency to physiological results (significant increase in EF, cardiac index (CI), fractional area change (FAC) and decrease in left ventricular enddiastolic volume index (LVEDVI)) in the IS group. Concerning the ID group, these results were only partially valid. Looking at the survival rates it turned out that they are significantly higher for the IS group than for the ID group. All in all, 1-year-survival was at 69,9 % and 2-year-survival was at 66,7 %. Conclusion: It can be concluded that survivors profited from the surgery which is shown by the improvement of physical capacity and left ventricular parameters. This applies especially to patients from the IS group, which conforms to the fact that also the post surgical stress echocardiography revealed myocardial reserves for this group. The differences between the groups which have been demonstrated in these investigations and which are also reflected in survival rates stress the importance of carefully chosen selection criteria and the need for further subtly differentiated research in this special field. Considering this, also the correlation between the intervention combined with PLV and the cause of the myopathy is of great importance.

A doença renal policística autossômica dominante (DRPAD) é uma das doenças hereditárias humanas mais comuns, apresentando uma prevalência de 1:543 a 1:4000 em diferentes populações. Esta enfermidade é essencialmente causada por mutação em um de dois genes: PKD1 (Polycystic Kidney Disease 1) ou PKD2. A maior parte dos casos decorre de mutações em PKD1. O tabagismo foi associado independentemente com progressão da doença renal na DRPAD. Em um estudo recente, contudo, tabagismo não influenciou a sobrevida renal nesta doença. Vários estudos também revelam que a deficiência em PKD1/Pkd1 ou PKD2/Pkd2 se associam a disfunção cardíaca. Para analisar os efeitos potenciais da exposição crônica ao tabagismo sobre os fenótipos renal e cardíaco associados à DRPAD, utilizamos como modelo experimental um animal cístico ortólogo fenotipicamente semelhante à DRPAD humana, o camundongo Pkd1flox/flox:Nestincre. Animais machos foram expostos ao tabagismo da concepção até a idade de 18 semanas. Os níveis séricos de ureia foram mais elevados em camundongos Pkd1flox/flox:Nestincre fumantes (CIF) que em Pkd1flox/flox:Nestincre não fumantes (CI) e em animais Pkd1flox/flox fumantes (NCF) que em Pkd1flox/flox não fumantes (NC). O índice cístico renal foi maior nos animais CIF que CI. A taxa de proliferação de células de revestimento cístico, mas não de apoptose, mostrou-se aumentada em camundongos CIF comparados a CI. As taxas de proliferação e apoptose de células tubulares renais foram mais altas nos rins CI que NC e nos rins CIF que NCF. Observamos, ainda, tais taxas mais elevadas em NCF que NC. Camundongos CIF desenvolveram maior índice de fibrose renal que CI. O mesmo efeito do tabagismo foi detectado em camundongos NCF, porém sua intensidade foi muito menor. As expressões renais de Il1b e Tgfb1 não diferiram significantemente entre os grupos. Os níveis renais de glutationa foram mais baixos em camundongos CIF que CI e em animais NCF que NC. A fração de ejeção do ventrículo esquerdo foi reduzida em camundongos CIF e CI em comparação a NC, mas não foi detectada diferença entre CIF e CI. Parâmetros cardíacos estruturais também se mostraram alterados em animais CIF comparados a CI e NCF. Interessantemente, strain circunferencial e a taxa de strain circunferencial foram menores em camundongos CIF que CI e NCF. A pressão arterial média foi mais baixa em animais CIF que CI hipertensos, atingindo níveis que não diferiram de NC e NCF. A taxa de apoptose em tecido cardíaco foi maior em animais CI que NC, mas não diferiu entre CIF e CI nem entre NCF e NC. A taxa de fibrose cardíaca foi mais elevada em camundongos CIF e CI que NC e NCF. Os grupos não diferiram quanto à sobrevida em 18 semanas, mas o peso corporal foi menor em animais CIF que CI em 16 semanas. Nossos achados indicam que a exposição crônica ao tabagismo desde a concepção agravou os fenótipos renal e cardíaco de camundongos císticos deficientes em Pkd1. Dadas as similaridades entre a DRPAD humana e o modelo animal ortólogo estudado, os efeitos deletérios do tabagismo observados devem provavelmente se aplicar a pacientes com DRPAD / Autosomal dominant polycystic kidney disease (ADPKD) is one of the most common human inherited diseases, with a prevalence of 1:543 to 1:4000 in different populations. This disease is essentially caused by mutation in one of two genes: PKD1 (Polycystic Kidney Disease 1) or PKD2. Most cases result from mutations in PKD1. Smoking has been independently associated with renal disease progression in ADPKD. In a recent study, however, smoking did not influence renal survival in this disease. Several studies also revealed that PKD1/Pkd1 or PKD2/Pkd2 deficiency are associated with cardiac dysfunction. To analyze the potential effects of chronic exposure to smoking on renal and cardiac phenotypes associated with ADPKD, we used an orthologous cystic animal phenotypically similar to human ADPKD as experimental model, the Pkd1flox/flox:Nestincre mouse. Male animals were exposed to smoking from conception to the age of 18 weeks. Serum urea nitrogen was higher in Pkd1flox/flox:Nestincre submitted to smoking (CYS) than in Pkd1flox/flox:Nestincre not submitted to smoking (CY) and in Pkd1flox/flox submitted to smoking (NCS) than in Pkd1flox/flox not submitted to smoking (NC). Renal cystic index was higher in CYS animals than CY. Proliferation rate of cyst-lining cells, but not apoptotic rate, was increased in CYS mice compared to CY. The proliferation and apoptotic rates of renal tubular cells were higher in CY kidneys than NC and in CYS kidneys than NCS. We also observed increase in such rates in NCS compared to NC. CYS mice developed a higher renal fibrosis index than CY animals. Although the same effect of smoking was detected in NCS mice, its intensity was much lower than in cystic animals. Renal expression of Il1b e Tgfb1 did not significantly differ among the groups. Renal levels of glutathione were lower in CYS mice than CY and in NCS animals than NC. Left ventricular ejection fraction was reduced in CYS and CY mice compared to NC, but no difference was detected between CYS and CY. Cardiac structural parameters were also altered in CYS animals in comparison to CY and NCS. Interestingly, circumferential strain and circumferential strain rate were lower in the CYS mice than CY and NCS. MAP was lower in CYS animals than in the hypertensive CY mice, reaching normotensive levels that did not differ from NC and NCS. The apoptotic rate in cardiac tissue was higher in CY animals than NC, but did not differ between CYS and CY and between NCS and NC. The rate of cardiac fibrosis was also higher in CYS and CY mice than in NC and NCS. Survival at 18 weeks did not differ among the groups but body weight was lower in CYS animals than CY at 16 weeks of age. Our findings show that chronic exposure to smoking since conception aggravated the renal and cardiac phenotypes of Pkd1-deficient cystic mice. Given the similarities between human ADPKD and the studied orthologous animal model, the observed deleterious effects of smoking are likely to apply to ADPKD patients

Die Ultraschalldiagnostik des Herzens ist eine der wichtigsten nichtinvasiven Diagnostikmethoden in der Kardiologie, auf vielen Gebieten der Herzchirurgie, der Anästhesiologie und der Kinderkardiologie. Ziel der vorliegenden Arbeit war die Darstellung der Möglichkeiten der Echokardiographie in der Diagnostik der Herzinsuffizienz mit Darstellung von Ischämie und Vitalität sowie der Therapieauswirkungen bei der ischämisch bedingten Herzinsuffizienz und der hämodynamischen Veränderungen. Ruhe und Stress Echokardiographie können in einem Untersuchungsgang ein großes Spektrum an Informationen über die Ruhe-Funktion, myokardiale Vitalität und Ischämie zu sammeln, die für die prognostische Einschätzung von Bedeutung sind. Die vorliegenden Untersuchungen ergaben eine regional unterschiedliche Wanddickenzunahme unter Dobutamin bei kardiovaskulär Gesunden in der 2D-Echokardiographie. Im Vergleich zu anderen Wandabschnitten zeigte die inferiore Wand eine reduzierte Wanddickenzunahme. Für die Interpretation in der Stress Echokardiographie sind diese Erkenntnisse von großer Bedeutung. Der Langzeitverlauf bei Patienten mit chronischer linksventrikulärer Dysfunktion wird durch klinische Faktoren, dem Grad der linksventrikulären Dysfunktion und wesentlich vom Vorhandensein ischämischen und/oder vitalen Myokards bestimmt. Die Kombination von Dipyridamol und Dobutamin zeigte in den ersten Untersuchungen eine erhöhte Sensitivität bei gleichbleibender Spezifität in der Vitalitätsdiagnostik bei chronisch ischämischer Herzkrankheit und reduzierter linksventrikulärer Funktion. Die Daten prospektiver Multicenter-Studien dokumentierten ein 1-Jahres Ereignis-Risiko von 2% bei Patienten mit einer negativen Stress-Echokardiographie und ergaben eine Verdopplung des Risikos bei einem positiven Testergebnis und eine Vervierfachung des Risikos, wenn der Test schon nach der ersten Dipyridamoldosis positiv ausfällt. Sowohl nach akutem Myokardinfarkt als auch bei chronisch hibernierendem Myokard mit reduzierter linksventrikulärer Funktion basiert der Nachweis vitalen Myokards mittels Kontrast-Echokardiographie auf der Demonstration einer intakten Mikroperfusion. In eigenen Untersuchungen konnte die Durchführbarkeit und gute diagnostische Genauigkeit der intravenösen Kontrast-Echokardiographie bei Patienten nach akutem Myokardinfarkt nach akuter perkutaner Koronar-Revaskularisation im Vergleich zur pharmakologischen Stress-Echokardiographie (Kombination aus niedrig dosiertem Dipyridamol und Dobutamin) und zur Myokardszintigraphie nachgewiesen werden. Die dreidimensionale Echokardiographie ermöglicht die plastische und räumliche Darstellung des Herzens und eine bessere topographische Zuordnung im Vergleich zur zweidimensionalen Echokardiographie. Besonders bei komplizierten pathologischen Veränderungen erleichtert die dreidimensionale Darstellung eine Einschätzung des jeweiligen Krankheitsbildes. Die Volumetrie der Herzhöhlen, die Bestimmung von Ejektionsfraktion und die Berechnung der linksventrikulären Masse, von Klappenöffnungsflächen oder intrakardialen Raumforderungen sind besonders bei pathologisch veränderten Zuständen und damit in geometrisch komplizierter Weise mit hoher diagnostischer Sicherheit möglich. Strukturveränderungen konnten sowohl bei sekundärer als auch bei primärer pulmonaler Hypertonie in Form von Wandverdickungen, Lumenreduktion, pathologischen Wandstrukturen und Einschränkung der systolisch-diastolischen Pulsatilität beschrieben werden. Es konnten derzeit bei Patienten mit pulmonaler Hypertonie im Vergleich zu einem Normalkollektiv verschiedene Befunde beschrieben werden: Wanddickenzunahme, Inhomogenität des Wandaufbaus, erhöhte Gefäßsteifigkeit und Plaques. Die Echokardiographie bleibt auch weiterhin ein Verfahren, das sich exzellent zur Verlaufbeobachtung bei den Patienten mit Koronarer Herzkrankheit und Herzinsuffizienz eignet und für die Diagnose und Therapieentscheidungen sowie für die Prognose entscheidende Informationen liefert. / The echocardiography is the most important non-invasive diagnostic tool in cardiology and in many fields of cardiac surgery, anaesthesiology and paediatric cardiology. The aim of this collection of studies was to demonstrate the importance of echocardiography for the diagnosis and guidance for therapeutic decision making in patients with heart failure and to demonstrate the ability of diagnosis of ischemia, viability, and hemodynamic assessment. Resting and stress echocardiography is a one-stop shop , which enables a wide range of information to be collected on resting function, myocardial viability, and induced ischemia, all of which are useful for prognostic stratification. Heterogeneity of left ventricular wall thickening can be induced or magnified by dobutamine infusion even in subjects without coronary artery disease, with the inferior wall showing a lack of hyperkinesis. Caution in aggressive dobutamine stress echocardiography reading, especially in the inferior wall, might be warranted. Large scale, multicentre, prospectively collected data could show that the 1-year risk of cardiac death is as low as 2% in patients with negative dipyridamole stress echocardiography: it doubles if the test is positive at e high dose, and is almost four times higher if it is positive at a low dose. When functional recovery after successful revascularization is considered as the postoperative gold standard in patients with severe heart failure, thallium scintigraphy had a higher sensitivity than dipyridamole or dobutamine alone, this sensitivity gap was filled with combined dipyridamole-dobutamine. The specificity of all forms of pharmacologic stress echo was better than thallium-201. In severe left ventricular ischemic dysfunction, myocardial viability, as assessed by low dose dipyridamole echo, is associated with improved survival in revascularized patients. The wall motion response during combined dipyridamole-dobutamine echocardiography was useful in the prediction of recovery of regional and global ventricular function after revascularization in patients short after acute myocardial infarction. Combined intravenous myocardial contrast echocardiography and pharmacological stress echocardiography was more accurate inn the diagnosis of stunned myocardium than myocardial scintigraphy alone. Dynamic three-dimensional echocardiography can provide objective three-dimensional images of the heart. Three-dimensional image reconstruction and volumetry were proven to have the potential to link morphological and hemodynamic diagnostic assessment of cardiac pathology. Three-dimensional transesophageal echocardiography could yield important additional clinical information in patients with heart tumors and improved the operative planning. Intravascular ultrasound imaging (IVUS) of the pulmonary arteries had been demonstrated as reliable method of quantifying lumen area and pulsatility. IVUS allowed the description and comparison of the morphologic changes of the pulmonary artery vessel with hemodynamic findings and endothelially mediated vasodilation in pulmonary hypertension. Echocardiography allows an integrated assessment of cardiac structure function and hemodynamics with important informations regarding the prognosis and therapeutic consequences.

Faktoren, die die funktionelle Erholung von hibernating myocardium nach aortokoronarem Venen Bypass (ACVB) beeinflussen, werden gegenwärtig untersucht. Von 3/2000 bis 3/2003 untersuchten wir prospektiv 41 Patienten mit einer linksventrikulären Ejektionsfraktion (LVEF) / Objectives: Factors that influence functional recovery of hibernating myocardium after coronary artery bypass grafting are at present under investigation. Methods: From 3/2000 to 3/2003 we prospectively analyzed 41 patients with ejection fraction (EF) 15% during DE in group I preoperatively; the increase was < 15% in group II. MRI hyperenhancement was measured with a mean of 16,7±11,6% of the left ventricle in group I compared to a mean of 27,4±14,4% in group II (p 19µm) in group I than in group II (< 17µm). Gene expression of pro-apoptotic genes such as BAK and BAX was lowered (0.5±0.1/0.8±0.1) compared to "normal" myocardium (1.0±0.1) (p

Introdução: O papel do sistema nervoso simpático (SNS) na remodelação ventricular esquerda na insuficiência aórtica crônica (IAo) é pouco conhecido. O aumento da atividade do SNS tem associação com remodelamento ventricular e mau prognóstico na insuficiência cardíaca (IC) não valvar, fazendo do seu bloqueio farmacológico importante conduta terapêutica. A despeito de similaridades na evolução clínica da IAo com IC não valvar, não há estudos com mensuração direta da atividade do SNS em IAo. Objetivo: Quantificar a atividade nervosa simpática muscular (ANSM) em pacientes com IAo importante em três situações clínicas, representativas da história natural dessa doença. Métodos: Trata-se de estudo transversal, unicêntrico, incluindo 30 pacientes com IAo importante que foram alocados em três grupos: (I) assintomáticos (n = 10, 70% homens, 37,4 ± 13,6 anos), (II) sintomáticos em pré-operatório de troca de valva aórtica (TVA) (n = 10, 70% homens, 42,2 ± 12,1 anos) e (III) - pós-operatório de TVA (n = 10, 80% homens, 41,2 ± 15,4 anos). Grupo controle formado por voluntários saudáveis sem doença cardíaca estrutural (n = 10) correspondentes para idade, sexo e IMC também foram avaliados. Variáveis clínicas, ecocardiográficas e BNP (peptídeo natriurético atrial) foram analisadas nos grupos. Apenas a pressão arterial sistólica era significativamente menor no grupo III. A ANSM foi mensurada utilizando a técnica padrão de microneurografia muscular (MM). A variável desfecho foi a Resumo média do número de espículas obtidas num registro contínuo de 10 minutos de MM. Resultados: Os grupos IAo não diferiram em relação às características demográficas, antropométricas e ecocardiográficas, assim como etiologia e BNP. A média de espículas obtidas pela MM, representativa da ANSM, nos grupos I, II, III e controle foi, respectivamente, 25,5 ± 4,1, 25,1 ± 3,6, 28,6 ± 6,5 e 15,6 ± 1,5 (p=0,001). Houve apenas diferença estatística entre os grupos IAo e o grupo controle. Conclusão: Houve aumento significativo da ANSM em pacientes com IAo importante associado ao remodelamento ventricular esquerdo em relação a indivíduos sem doença cardíaca estrutural. A ANSM foi similar em pacientes com IAo importante assintomáticos, sintomáticos e em pós-operatório de TVA. A participação da ação do SNS na IAo deve estar associada ao remodelamento ventricular, mas sem correlação com mudanças clínicas / Introduction: The role of sympathetic nervous system (SNS) in the left ventricle remodeling of severe aortic regurgitation (AR) remains poorly understood. The increase in SNS activity is associated with ventricular remodeling and poor prognosis in non-valvular heart failure (HF), making its pharmacological blockade an important therapeutic approach. Despite similarities in the clinical evolution of AR with non-valvular HF, there are no studies with direct measurement of SNS activity in AR. Aims: The present study aimed to quantify muscular sympathetic nervous activity (MSNA) in patients with severe AR in three clinical situations: asymptomatic, symptomatic before aortic valve replacement (AVR), and patients submitted to AVR. Methods: Thirty patients with severe AR were allocated to three groups: (I) asymptomatic patients (n=10, 70% men, age: 37.4 ± 13.6), (II) symptomatic patients before AVR (n=10, 70% men, age: 42.2 ± 12.1), and (III) patients submitted to AVR (n=10, 80% men, age: 41.2 ± 15.4). Healthy volunteers (n = 10) matched for age, sex, and BMI were also assessed. The AR groups did not differ in relation to etiology, demographic, anthropometric or echocardiographic data. Only systolic blood pressure was significantly lower in group III. MSNA was recorded using microneurography, with a spike per minute result. Results: The means of 10-minute recordings in groups I, II, III and control were 23.2 ± 6.4, 25.5 ± 4.1, 25.1 ± 3.6 and 15.6 ± 1.5, respectively (p=0.001). Only the AR and control groups differed from each other. Conclusions: AR is associated with relatively higher SNS activity, which is similar across different stages of the disease (asymptomatic, symptomatic and postoperative). The role of the SNS in AR must be associated with ventricular remodeling, but without correlation with clinical change

INTRODUÇÃO: A miocardiopatia não compactada (MNC) é caracterizada pela formação de trabéculas miocárdicas proeminentes com recessos intertrabeculares profundos no ventrículo esquerdo (VE). Os pacientes podem apresentar insuficiência cardíaca, tromboembolismo e/ou arritmia. Os avanços de métodos diagnósticos têm permitido um maior reconhecimento desta doença. Assim, o objetivo deste estudo foi analisar os achados do eletrocardiograma (ECG), Holter de 24hs e eletrocardiograma de alta resolução (ECGAR), e sua relação com ecocardiograma (ECO) transtorácico e pela ressonância magnética cardíaca (RMC) em pacientes com MNC. MÉTODOS: Foram analisados pacientes com diagnóstico de MNC pelo ECO e/ou RMC, em acompanhamento ambulatorial em nossa instituição. Esses pacientes foram submetidos à avaliação clínica, ECG de 12 derivações, Holter de 24hs e ECGAR. Os dados do ECG foram analisados por dois observadores e comparados com o grupo controle, saudáveis. RESULTADOS: Sessenta e seis pacientes com MNC (45,1 ± 17,3 anos, 56,1% do sexo masculino), e vinte controles (52,6 ± 19,2 anos, 30,0% do sexo masculino), foram incluídos neste estudo. Os pacientes apresentaram fração de ejeção do VE pelo ECO de 41,2 ± 9,4%, e pela RMC de 40,8 ± 16,9%. Os achados eletrocardiográficos mais frequentes foram alteração da repolarização ventricular (65,2%), sobrecarga do VE (24,2%) e extrassístoles ventriculares (EEVV) (19,7%); com índice de concordância interobservadores alto, entre todas as variáveis (p < 0,001), exceto sobrecarga biventricular. O grupo com fração de ejeção reduzida apresentou um maior número de casos com sobrecarga de átrio esquerdo e maior duração do QRS em relação ao grupo sem disfunção (p= 0,020; p=0,008, respectivamente). O grupo que apresentou realce tardio tem maior número de pacientes com bloqueio de ramo esquerdo/bloqueio divisional anterossuperior esquerdo e maior duração do QRS quando comparado ao grupo sem realce (p= 0,034; p=0,035, respectivamente). No Holter de 24 horas, 4,4% apresentou ritmo de fibrilação atrial e 18,2% taquicardia ventricular não sustentada (TVNS) relacionada com os diâmetros de átrio e ventrículos esquerdos pelo ECO (P=0,008, P=0,0048, respectivamente); houve correlação entre o aumento do volume diastólico final pela RMC e intervalo PR (r=0,414; p=0,007), EEVV (r=0,426; p < 0,005) e intervalo QRS (r=0,564; p < 0,001). Além disso, o realce tardio apresentou relação com TVNS (p=0,009), extrassístole atrial (p=0,002), EEVV (p=0,004), duração do QRS (p=0,002), pela análise do Holter de 24hs. O ECGAR estava alterado em 10,6% dos pacientes, sendo que destes 12,2% apresentavam disfunção ventricular. Houve correlação entre dilatação de cavidades esquerdas pelo ECO, com duração do QRS filtrado e Standard (STD) (r=0,577; p < 0,001), e relação entre QRS filtrado e realce tardio (p <0,001) e dilatação ventricular pela RMC com QRS filtrado e STD (p < 0,001 para ambos). Os pacientes que foram a óbito, no período de seguimento de 4 anos, tinham uma frequência maior de TVNS (p=0,006). CONCLUSÃO: Os pacientes com MNC apresentaram alterações significativas do ECG, ECGAR e Holter de 24hs, mais evidentes no grupo de pacientes com disfunção ventricular e realce tardio. Dessa forma, estes pacientes devem ser acompanhados mais frequentemente, pelo risco maior de desfecho clínico desfavorável ambulatory. / INTRODUCTION: Noncompacted cardiomyopathy (NCC) is characterized by the formation of prominent myocardial trabeculae with deep intertrabecular recesses in the left ventricle (LV). Patients may present with heart failure, thromboembolism and/or arrhythmia. Advances in diagnostic methods have allowed greater recognition of this disease. Thus, the aim of this study was to analyze the findings of electrocardiogram (ECG), 24-hour Holter and high resolution electrocardiogram (ECGAR), and its relationship with transthoracic echocardiogram (ECHO) and cardiac magnetic resonance imaging (MRI) in patients with MNC. METHODS: Patients with a diagnosis of NCC by ECGO and / or CMR were analyzed in an outpatient clinic at our institution. These patients underwent clinical evaluation, 12-lead ECG, 24-hour Holter, and ECGAR. ECG data were analyzed by two observers and compared to the healthy control group. RESULTS: Sixty-six patients with MNC (45.1 ± 17.3 years, 56.1% males), and twenty controls (52.6 ± 19.2 years, 30.0% males) were included in this study. The patients presented LV ejection fraction by ECHO of 41.2 ± 9.4%, and CMR of 40.8 ± 16.9%. The most frequent electrocardiographic findings were alteration of ventricular repolarization (65.2%), LV overload (24.2%) and ventricular extrasystoles (VEE) (19.7%); with a high interobserver agreement index among all variables (p < 0.001), except for biventricular overload. The group with reduced ejection fraction presented a higher number of cases with left atrial overload and longer duration of QRS compared to the group without dysfunction (p = 0.020, p = 0.008, respectively). The group that presented delayed enhancement had a higher number of patients with left bundle branch block / anterosuperior left divisional block and longer duration of the QRS when compared to the group without enhancement (p = 0.034, p = 0.035, respectively). In the 24-hour Holter, 4.4% presented atrial fibrillation and 18.2% non-sustained ventricular tachycardia (NSVT). This was related to left ventricular atrial and ventricular diameters (P = 0.008, P = 0.0048, respectively); (r = 0.414, p = 0.007), EEVV (r = 0.426, p < 0.005), and QRS interval (r = 0.564, p < 0.001) were correlated with the increase in final diastolic volume. In addition, the late enhancement was related to NSVT (p = 0.009), atrial extrasystole (p = 0.002), VLT (p = 0.004), QRS duration (p = 0.002), and 24h Holter analysis. The ECGAR was altered in 10.6% of the patients, of whom 12.2% had ventricular dysfunction. There was a correlation between left ventricular ECO dilatation with filtered QRS duration and standard (STD) (r = 0.577, p < 0.001), and the relationship between filtered QRS and late enhancement (p < 0.001) and ventricular dilation by MRI with filtered QRS and STD (p < 0.001 for both). Patients who died at the 4-year follow-up period had a higher frequency of NSVT (p = 0.006). CONCLUSION: Patients with NCC present significant ECG, ECGAR and Holter 24hs changes, most evident in the group of patients with ventricular dysfunction and late enhancement. Therefore, these patients should be monitored more frequently, due to the greater risk of unfavorable clinical outcome

Introdução: as mucopolissacaridoses (MPSs) são doenças lisossômicas de depósito, caracterizadas pela degradação enzimática deficiente dos glicosaminoglicanos (GAGs): ácido hialurônico, condroitin sulfato, dermatan sulfato, heparan sulfato e queratan sulfato. A classificação baseia-se na enzima comprometida, tendo sido descritos sete tipos com manifestações clínicas heterogêneas: MPS tipo I, II, III, IV, VI, VII e IX. O comprometimento cardiovascular é variável, porém a falência cardiopulmonar contribui significativamente para a morbidade e mortalidade. Lesões valvares esquerdas e a hipertrofia do ventrículo esquerdo são os achados mais citados, ainda que não haja concordância quanto à relação entre o comprometimento cardíaco e o tipo de MPS. Especula-se que o acometimento é mais grave em pacientes cujo defeito enzimático traz acúmulo do dermatan sulfato (MPS tipos I, II, VI e VII), visto que esse GAG predomina naturalmente em válvulas e vasos sanguíneos. Frente à perspectiva de tratamento específico dessas patologias através de reposição enzimática, torna-se fundamental conhecer o comprometimento cardiovascular inicial, para determinar com segurança o impacto destas terapêuticas sobre as crianças a elas submetidas. O propósito deste estudo foi caracterizar as alterações ecocardiográficas de pacientes pediátricos com MPSs, além de testar a associação entre o acúmulo de dermatan sulfato e a gravidade das lesões cardiovasculares. Métodos: foram analisados retrospectivamente os prontuários e os ecocardiogramas de 28 pacientes (15M: 13F) entre 2 e 14 anos (9 ± 3 anos), acompanhados no Ambulatório de Genética do Instituto da Criança de setembro de 2003 a novembro de 2005: 6 com MPS tipo I, 2 com tipo II, 7 com tipo III, 6 com tipo IV, 5 com tipo VI e 2 com tipo VII. No período estudado nenhum paciente realizava terapia de reposição enzimática. Um único ecocardiografista executou 53 avaliações, visto que 17 indivíduos submeteram-se a múltiplos exames, com intervalo de 10,3 ± 5,6 meses. Todos os ecocardiogramas foram realizados segundo as normas da Sociedade Americana de Ecocardiografia. Os pacientes foram analisados quanto aos aspectos clínicos e parâmetros xvi ecocardiográficos, sendo realizada em seguida a comparação entre o grupo que acumula (D+) e o que não acumula dermatan sulfato (D-). O grupo D+ incluiu os tipos I, II, VI e VII e o grupo D-, os tipos III e IV. O programa estatístico utilizado foi o Statistical Package for Social Sciency e os testes aplicados foram o Exato de Fisher e o de Correlação de Spearman, com um valor de p significativo 0,05. Resultados: 26 (93%) pacientes exibiram alterações ecocardiográficas ao exame final. No entanto, em apenas 16 (57%) havia registro de ausculta anormal e em 6 (21%) alguma queixa cardiovascular. Hipertrofia de septo e de parede posterior foram detectadas em 12 pacientes (43%) e em 5 (18%) ocorreu hipertrofia septal isolada. Somente 2 (7%) apresentaram dilatação ventricular. Em 22 casos foi possível avaliar a função diastólica de ventrículo esquerdo. Destes, 6 (27%) apresentaram disfunção de grau leve. Todos apresentaram função sistólica preservada. Detectou-se hipertensão pulmonar em 10 pacientes (36%). Quatro foram admitidos à Unidade de Terapia Intensiva e dois evoluíram a óbito, todos por agravamento de hipertensão pulmonar. Valva mitral normal foi o achado em 5 (17,8%) e espessamento sem disfunção em 6 (21,4%). Espessamento valvar com disfunção ocorreu em 17 pacientes (60,8%): 12 (42,8%) com insuficiência, 2 (7,2%) com estenose e 3 (10,8%) com dupla lesão. A valva aórtica foi normal em 5 (17,8%) e espessada sem disfunção em 13 (46,4%). Espessamento com disfunção ocorreu em 10 pacientes (35,8%): todos com insuficiência de grau leve ou moderado. Verificou-se forte associação entre o acúmulo de dermatan sulfato e a presença de: disfunção valvar mitral (p = 0,0003), disfunção valvar aórtica (p = 0,006) e hipertensão pulmonar (p = 0,006). Entre os 17 indivíduos com múltiplos exames, 14 (82%) mostraram piora ecocardiográfica justificada por: surgimento (4/14) ou agravamento (6/14) de lesões valvares, surgimento (5/14) ou progressão (6/14) da hipertrofia ventricular, desenvolvimento de disfunção diastólica (1/14) e de hipertensão pulmonar (4/14). Conclusões: as alterações ecocardiográficas em pacientes pediátricos com mucopolissacaridoses são freqüentes e têm caráter progressivo, enquanto os sinais e sintomas são escassos. Lesões valvares esquerdas, hipertrofia ventricular e hipertensão pulmonar foram os achados mais comuns, havendo associação significativa entre o acúmulo de dermatan sulfato e o comprometimento cardiovascular. Diferentemente do que é descrito em adultos, a hipertensão pulmonar foi a causa mais importante de óbito e não a disfunção sistólica de ventrículo esquerdo. / Introduction: mucopolysaccharidosis (MPSs) are lysosomal storage diseases, characterized by deficient enzymatic degradation of glycosaminoglycanes (GAGs): hyaluronic acid, chondroitin sulfate, dermatan sulfate, heparan sulfate and keratan sulfate. The classification is based on the defective enzyme and seven types with heterogeneous clinical manifestations have been described: MPS type I, II, III, IV, VI, VII and IX. The cardiovascular involvement is variable, but the cardiopulmonary failure contributes significantly towards the morbidity and mortality. Left valve lesions and left ventricle hypertrophy are the most commented findings, although there is still no agreement about the relationship between the heart involvement and the type of MPS. It is speculated that the lesions are more severe in patients whose enzymatic defect lead to the accumulation of dermatan sulfate (MPS types I, II, VI and VII), because this GAG prevails naturally in valves and blood vessels. Due to the perspective of specific treatment for the pathology through enzymatic replacement, it is essential to know the initial cardiovascular abnormalities to determine the impact of this therapeutics on pediatric patient. The purpose of this study was to characterize the echocardiographic alterations of the pediatric patients with MPSs, besides testing the association between the accumulation of dermatan sulfate and the severity of the cardiovascular lesions. Methods: the medical records and echocardiograms of 28 patients (15M: 13F) aged 2 to 14 (9 ± 3 years), seen at the Genetic Clinic between September 2003 and November 2005, were retrospectively analyzed: 6 with MPS type I, 2 with type II, 6 with type III, 7 with type IV, 5 with type VI and 2 with type VII. During the period of study no patient had enzymatic replacement. A single pediatric cardiologist executed 53 echocardiograms, since 17 individuals underwent multiple exams, with an interval of 10.3 ± 5.6 months. All the echocardiograms were performed according to the recommendations of the American Society of Echocardiography. Patients were analyzed according to both clinical and echocardiographic parameters, and then a comparison was made among the group that accumulates (D+) and the one that does not xviii accumulate dermatan sulfate (D-). The group D+ included the types I, II, VI and VII and the group D included types III and IV. The statistical program used was the Statistical Package for Social Science and the applied tests were the Fisher\'s exact test and the Spearman correlation, where a p-value < 0.05 was considered significant. Results: echocardiographic alterations were detected in 26 patients (93%), whereas 16 (57%) had abnormal auscultation, and only 6 (21%) presented cardiovascular complaint. Septum and posterior wall hypertrophy were diagnosed in 12 patients (43%) and five (18%) showed signs of isolated septal hypertrophy. Only 2 (7%) presented ventricular dilation. In 22 patients it was possible to evaluate the diastolic function of the left ventricle. Of these, 6 presented mild dysfunction. However, all patients had preserved systolic function. Pulmonary hypertension was detected in 10 patients (36%). 4 patients were admitted in the Intensive Care Unit and 2 died, due to aggravation of pulmonary hypertension. Normal mitral valve was found in 5 (17.8%) and thickening without dysfunction in 6 cases (21.4%). Valve thickening with dysfunction occurred in 17 (60.8%): 12 (42.8%) with regurgitation, 2 (7.2%) with stenosis and 3 (10.8%) with double lesion. The aortic valve was normal in 5 (17.8%) and thickened without dysfunction in 13 cases (46.4%). Thickening with dysfunction happened in 10 patients (35.8%): all with mild or moderate aortic regurgitation. A strong association was observed between accumulation of dermatan sulfate and presence of mitral valve dysfunction (p = 0.0003), aortic valve dysfunction (p = 0.006) and pulmonary hypertension (p = 0.006). Among 17 individuals with multiple exams, 14 (82%) had a worsening evolution justified by the appearance (4/14) or aggravation (6/14) of valve lesions, appearance (5/14) or progression (6/14) of ventricular hypertrophy, development of left ventricle diastolic dysfunction (1/14) and of pulmonary hypertension (4/14). Conclusions: echocardiographic alterations in pediatric patients with Mucopolysaccharidosis are frequent and have a progressive character. Left valve lesions, ventricular hypertrophy and pulmonary hypertension were the most common findings and there was association between accumulation of dermatan sulfate and cardiovascular involvement. Unlike in adults, pulmonary hypertension was the main cause of death, not left ventricle systolic dysfunction.

O objetivo da reperfusão mecânica ou medicamentosa em pacientes com infarto agudo do miocárdio (IAM) é a restauração do fluxo sangüíneo na artéria relacionada ao infarto, a fim de limitar a necrose miocelular e preservar a função contrátil, que são os maiores preditores de sobrevida após o IAM. Entretanto, seu sucesso é indicado pela reperfusão nos capilares miocárdicos e não pela recanalização da artéria relacionada ao infarto. A ecocardiografia com perfusão miocárdica em tempo real pode ser usada para avaliar a perfusão nos capilares miocárdicos e estimar o tamanho do infarto em vários estudos experimentais. O objetivo deste estudo foi determinar a área do miocárdio infartado e sua extensão transmural pela ecocardiografia com perfusão miocárdica em tempo real, em pacientes hospitalizados nos primeiros dias após primo infarto agudo do miocárdio, tendo como padrão de referência a ressonância magnética de perfusão. Prospectivamente, foram estudados 20 pacientes (12 homens), com idade média de 64,2 ± 13,3, dentro de 12 horas de reperfusão mecânica ou medicamentosa. Os exames de ecocardiografia e ressonância magnética foram realizados do segundo ao quinto dia de infarto. O contraste \"PESDA (perfluorocarbon-exposed sonicated dextrose albumin)\" foi administrado em veia periférica, na dose de 0,1ml/Kg, em infusão contínua. A análise ecocardiográfica foi feita por uma análise qualitativa (visual) denominada escala de cinza e por uma análise quantitativa, chamada imagem paramétrica. A área de infarto média foi de 3,03 ± 2,77 cm2 para a escala de cinza (r=0,97), de 3,36 ± 2,82 cm2 para a imagem paramétrica (r= 0,99) e de 3,42 ± 2,80 cm2 para a ressonância magnética. O porcentual médio da área de infarto pela ecocardiografia em escala de cinza foi de 15,22% ± 14,84% (r= 0,97), pela imagem paramétrica foi de 16,46% ± 14,41% (r= 0,99) e pela ressonância magnética foi de 16,76% ± 14,48%. A extensão transmural do infarto em cada segmento infartado pela escala de cinza,apresentou uma correlação menor (r=0,77) em relação à imagem paramétrica (r=0,93). Em conclusão, este estudo mostrou uma excelente correlação entre ecocardiografia com perfusão miocárdica em tempo real (sobretudo pela imagem paramétrica) e ressonância magnética nas medidas da área de infarto e seu porcentual, assim como na determinação de sua extensão transmural em pacientes internados por infarto agudo do miocárdio / The aim of mechanical or pharmacological reperfusion in patients with AMI is to restore blood flow through the infarct related artery in order to limit myocellular necrosis and ultimately preserve myocardial contractile function, which is still the most powerful predictor of survival after acute myocardial infarction. The success of reperfusion, however, is indicated by perfusion of myocardial capillaries rather than simply patency of the infarct related artery. Myocardial contrast echocardiography (MCE) can be used to assess myocardial capillary perfusion and ultimately the infarct size in animals. The aim of this study was to determine by MCE the transmural extent and infarct size using magnetic resonance imaging (MRI) as a gold standard. We prospectively studied 20 patients (12 men; mean age 64.2 ± 13.3) admitted for a first acute myocardial infarction, within 12 hours after mechanical or pharmacological reperfusion. The MCE and MRI were performed between the 3rd and 5th day of acute myocardial infarction (AMI). A suspension of 0.1 ml/kg of perfluorocarbon-exposed sonicated dextrose albumin (PESDA) ultrasound contrast agent was administered as a continuous infusion. The interpretation of perfusion images by MCE was assessed by qualitative analysis (visual- VIS) and a quantitative one called parametric image (PI). The average infarct area showed by VIS was 3.03 ± 2.77 cm 2, by PI was 3.36 ± 2.82 cm 2 and by MRI was 3.42± 2.80cm 2. The average percentage of infarct area by VIS was 15.22% ± 14.84%, by PI was 16.46% ± 14.41% and by MRI was 16.76% ± 14.48%. The transmural extent was calculated in each infarcted segment by VIS, with a worse correlation (r=0,77) than PI (r=0,93). In conclusion, this study showed that MCE, in special PI, correlates well with MRI in detecting infarct size, percentage of infarct size and transmural extent in patients with acute myocardial infarction

Introdução: As massas cardíacas (MC) podem ser tumores, trombos ou pseudotumores. A avaliação da vascularização poderá ser uma ferramenta adicional para o seu diagnóstico diferencial. Neste estudo, demonstrou-se o valor diagnóstico da ecocardiografia com perfusão na caracterização das MC or meio de análises qualitativas e quantitativas de perfusão. Métodos: Estudo prospectivo que envolveu 107 pacientes, classificados em quatro grupos: 33 trombos, 23 tumores malignos (TM), 24 tumores benignos (TB) e 6 pseudotumores; 21 pacientes foram excluídos por não terem diagnóstico definitivo confirmado. A avaliação de perfusão foi realizada pela ecocardiografia com perfusão em tempo real, utilizando contraste à base de microbolhas. Em um grupo selecionado de pacientes (32), o estudo foi complementado com dipiridamol para avaliação da reserva de fluxo da massa. A análise foi feita qualitativa e quantitativamente por dois observadores independentes. Na análise qualitativa, os parâmetros foram: intensidade da perfusão (escore 0 a 3), velocidade do repreenchimento microvascular (escore 0 a 2), padrão de perfusão central ou periférico (escore 0 a 2) e presença de áreas de necrose (escore 0 e 1). Os dois parâmetros de quantificação das massas foram: volume de sangue microvascular (A) e fluxo microvascular regional, que é o produto da velocidade de fluxo () e volume (A). Resultados: Na análise qualitativa, o padrão mais frequente para o grupo trombos foi: sem perfusão (81,9%), sem velocidade de perfusão (81,9%) e sem área de necrose (93,4%); nos tumores, predominou perfusão discreta (62,3%), com velocidade lenta (64,2%) e áreas de necrose (30,2%). Na análise qualitativa, a variação intraobservador para escore de perfusão e de velocidade foi de 20%, para áreas de necrose de 25% e para padrão de perfusão foi de 45%. Na análise quantitativa, o grupo trombos apresentou valores de A e Ax significativamente menores quando comparados ao grupo de tumores: Trombos: A = 0,08 (0,01-0,22dB); Ax = 0,03 (0,010,14dB/s-1); TM: A = 2,78 (1,31-7,0dB); Ax = 2,0 (0,995,58dB/s-1); TB: A = 2,58 (1,24-4,55dB); Ax = 1,18 (0,453,4dB/s-1). Quando comparados apenas os grupos de tumores com o uso de dipiridamol, os TM apresentaram volume sanguíneo microvascular (A) maiores: A = 4,18 (2,14-7,93dB); Ax = 2,46 (1,424,59dB/s-1), TB: A = 2,69 (1,11-4,26dB); Ax = 1,55 (0,555,50dB/s-1). Na análise com a curva ROC, a área sob a curva = 0,95, no parâmetro volume sanguíneo microvascular (A) < 0,65dB na ecocardiografia de perfusão com e sem uso de dipiridamol foi preditor para trombo, bem como o parâmetro fluxo sanguíneo microvascular (Ax) < 0,30dB/s-1, (área sob a curva = 0,94). Para distinguir entre TM de TB, o parâmetro volume sanguíneo microvascular (A), com o uso de dipiridamol > 3,28dB foi preditor de TM (área sob a curva = 0,75). Conclusão: O estudo ecocardiográfico para avaliação da perfusão das MC mostrou que a análise qualitativa é um método diagnóstico rápido e reprodutível para diagnosticar trombos. Os tumores cardíacos apresentam volume microvascular e fluxo sanguíneo regional maior se comparados com os trombos. O uso do dipiridamol foi útil na diferenciação entre os TM e TB / Background: Cardiac masses (CM) can be tumors, thrombi or pseudotumors. Evaluation of their vascularization might be an additional tool to perform a differential diagnosis. In the present study we demonstrated the diagnostic value of perfusion echocardiography for CM characterization, by qualitative and quantitative analyses of perfusion. Methods: We prospectively studied 107 patients, who were classified into 4 groups: 33 thrombus, 23 malignant tumors (MT), 24 benign tumors (BT) and 6 pseudotumors, of which 21 were excluded because no definitive diagnosis could be confirmed. Perfusion evaluation was performed by contrast echocardiography with real time perfusion imaging using microbubbles. A group of patients (32) was selected for a complementary study using dipyridamole to evaluate mass flow reserve. Qualitative and quantitative analyses were performed by two independent observers. Parameters for qualitative analysis were perfusion intensity (0-3 score), microvascular refilling velocity (0-2 score), central or peripheral perfusion pattern (0-2 score), and presence of areas of necrosis (0 or 1 score). The two parameters for quantification of masses were microvascular blood volume (A), and regional microvascular flow which is the product of blood flow velocity and vomume (A). Results: The most frequent pattern for the thrombi group in the qualitative analysis was absence of perfusion (81.9%), followed by no perfusion velocity (81.9%), and no areas of necrosis (93.4%), whilst among tumors there was predominance of discrete perfusion (62.3%), with slowed velocity (64.2%), and areas of necrosis (30.2%). Qualitative analysis, perfusion velocity showed intraobserver variability 20%, presence of areas of necrosis of 25% and perfusion pattern of 45%. In the quantitative analysis, the thrombi group was shown to have A and Ax values significantly smaller compared to the tumor group: Thrombi: A = 0.08 (0.01-0.22dB); Ax = 0.03 (0.010.14dB/s-1); MT: A = 2.78 (1.31-7.0dB); Ax = 2.0 (0.995.58dB/s-1); BT: A = 2.58 (1.24-4.55dB); Ax = 1.18 (0.453.4dB/s-1). When only the tumor groups with the use of dipyridamole were compared, MT was shown to have greater microvascular blood volume (A): A = 4.18 (2.14-7.93dB); Ax = 2.46(1.424.59dB/s-1), BT: A = 2.69 (1.11-4.265dB); Ax = 1.55 (0.555.50dB/s-1). Analysis of the ROC curve showed that an area of 0.95 for a microvascular blood volume of A < 0.65 dB predictive curve on perfusion echocardiography, both with and without dipyridamole, predicts thrombi, and so does a <0.30dB/s-1microvascular blood flow (Ax), area under curve = 0.94. In order to distinguish MT from BT, a >3.28dB microvascular blood volume (A) using dipyridamole was predictor of MT (area under curve = 0.75). Conclusion: The echocardiographic study to evaluate CM perfusion showed that qualitative analysis is reproducible diagnostic approach for diagnosing thrombi. Cardiac tumors show greater microvascular volume and regional blood flow when compared with thrombi. Dipyridamole quantitative stress mass perfusion was useful to differentiate MT from BT

Introdução: Previamente, demonstramos que o remodelamento histológico do miocárdio à época da correção da tetralogia de Fallot (TF) influenciou na função do ventrículo direito (VD) no pós-operatório (PO) precoce. O impacto da fibrose miocárdica na função ventricular no PO tardio ainda é desconhecido. O objetivo deste estudo foi avaliar ecocardiograficamente na mesma coorte de pacientes a função do VD no PO tardio, comparando com dados anteriormente obtidos por ecocardiografia convencional e morfometria miocárdica. Métodos: Estudamos 20 pacientes no PO da TF (tempo de seguimento = 96,6 ± 13,3 meses), 15 homens (75%), idade média no PO tardio (PO2) 128,3 ± 25,7 meses. As velocidades miocárdicas do VD diastólica precoce (e´), tardia (a\') e sistólica (S\') foram avaliadas pelo Doppler tecidual no pré-operatório, três dias após a cirurgia, entre 30º-90º dia e no PO2. Parâmetros convencionais, como a excursão sistólica do anel da valva tricúspide (TAPSE), variação fracional da área (FAC), volume do átrio direito indexado, pico da velocidade de enchimento diastólico precoce (E) do fluxo transvalvar tricúspide e da deformação miocárdica global e regional, strain longitudinal sistólico (GLS), strain rate sistólico (GLSRs) e o strain no pico do tempo de relaxamento isovolumétrico (GLSTRIV), foram analisados apenas no PO2. Também, nesta fase, realizamos a análise tridimensional da fração de ejeção, e dos volumes diastólico e sistólico finais do VD. Resultados: A velocidade a\' diminuiu nas avaliações iniciais e persistiu anormal no PO2 (RM ANOVA p < 0,001). Houve correlação negativa significante entre a velocidade e\' no PO2 e a fração de área de fibrose miocárdica (FIBR) (p = 0,02; r = -0,54), e correlação positiva entre FIBR e a relação E/e\' (p= 0,0002; r= 0,787). No PO2, o TAPSE (1,50 ± 0,19cm) foi reduzido e FAC normal (47,51± 7,56%). O valor do GLS global foi 18,48 ± 2,97%, com Z score < -2 em 16 pacientes e diferiu regionalmente no segmento médio do septo (Z score < -2 em 5 pacientes) e no segmento médio da parede lateral (Z score < -2 em 1 paciente). Houve correlação negativa entre FIBR e GLS no segmento médio septal (p = 0,0376; r = -0,493), entretanto sem influência no GLS global. No PO2, a insuficiência pulmonar residual foi moderada ou acentuada em 15 pac (75%), sem diferença quanto à FIBR miocárdica em relação ao grau leve (p = 0,58). Estavam aumentados os volumes indexados: diastólico final médio (89,5 ± 34,3ml/m²; Z score > 2DP em 12 pacientes) e sistólico (40,6 ± 9,1ml/m²; Z score > 2DP em 14 pacientes). A fração de ejeção média foi normal 51,8 ± 6,9% e não houve correlação com a FIBR. Conclusões: A avaliação ecocardiográfica tardia identificou alterações evolutivas e adaptativas das funções sistólica e diastólica do VD, com função sistólica preservada e função diastólica anormal e associada ao grau de FIBR avaliado em amostras operatórias; o estudo da deformação miocárdica revelou alterações globais e regionais, possivelmente relacionadas à arquitetura do miocárdio nessa malformação e às adaptações decorrentes da interposição de retalhos e suturas cirúrgicas; a avaliação pelo modo tridimensional correlacionou-se positivamente com as medidas obtidas no modo bidimensional; a insuficiência pulmonar foi lesão residual altamente prevalente / Introduction: We have previously demonstrated that the myocardial remodeling at the time of corrective surgery in tetralogy of Fallot (TF) patients influenced the right ventricular (RV) function in the early post-operative period (PO). The impact of myocardial fibrosis in late follow up (LFU) has not been investigated so far. Our objective was to analyze in the same cohort of patients in LFU, the RV function, comparing the obtained results with echocardiographic data from the early PO and with myocardial morphometry. Methods: 20 patients in the late FLU of TF correction were studied (time of follow up = 96.6 ± 13.3 months), 15 men (75%), mean age at LFU 128.3 ± 25.7months. The early (e\') and late (a\') diastolic and the systolic (S\') myocardial velocities were evaluated through tissue Doppler in the pre-operative period, three days after surgery, between the 30o-90o days and in LFU. We analyzed conventional echocardiographic parameters like the tricuspid annular plane systolic excursion (TAPSE), the fractional area change (FAC), the indexed right atrial volume, the peak early diastolic filling velocity (E) and of myocardial deformation: global longitudinal strain (GLS), global longitudinal systolic strain rate (GLSRs) and global longitudinal strain at the peak of the isovolumetric relaxation time (GLSTRIV) in the LFU. Also in LFU we analyzed by tridimensional echocardiography the ejection fraction and the final RV diastolic and systolic volumes. Results: The a\' velocity decreased in the initial evaluations and persisted abnormal in LFU (RM ANOVA, p < 0.001). There was a significant and negative correlation between e\' in LFU and the area fraction of myocardial fibrosis (FIBR) (p = 0.02; r = -0.54) and a positive correlation between FIBR and E/e\' ratio (p = 0.0002; r = 0.787). In the LFU TAPSE decreased (1.50 ± 0,19cm) and FAC was normal (47.51 ± 7.56%). The GLS value was 18.48 ± 2.97%, with Z score <- 2 SD in 16 patients, and was significantly different in the mid ventricular septum (Z score <- 2 in 5 patients) and in the mid segment of the lateral wall (Z score < -2 in 1 patient). There was a negative correlation between FIBR and GLS in the mid ventricular segment of the septum (p = 0.0376; r = -0.493), however without influence in GLS. In LFU pulmonary regurgitation was considered moderate or severe in 15 patients (75%), with no difference relative to the group with mild regurgitation regarding FIBR (p = 0.58). The final indexed RV volumes were increased: diastolic (89.5 ± 34.3ml/m2; Z score > 2SD in 12 patients) and systolic (40.6 ± 9.1ml/m2; Z score > 2SD in 14 patients). The mean RV ejection fraction was normal (51.8 ± 6.9%), and did not correlate with FIBR. Conclusions: The LFU echocardiographic evaluation identified evolutive and adaptative alterations in RV function, with preserved systolic and abnormal diastolic function, associated with the degree of FIBR assessed in myocardial samples; the study of myocardial deformation indexes revealed regional and global alterations, possibly related to the abnormal myocardial architecture specific for the cardiac malformation and/or to post-surgical adaptation to patches and sutures; the tridimensional echocardiography data correlated positively with those obtained through bidimensional echo; pulmonary regurgitation was a highly prevalent residual lesion