No description available.

Die chirurgische Mitralklappenrekonstruktion (MKR) ist der konservativen Therapie bei signifikanter Mitralklappeninsuffizienz (MI) überlegen. Bisher fehlen sensitive präoperative Parameter zur Detektion latenter linksventrikulärer Funktionsstörungen. Aufgrund der pathophysiologisch bedingten Nachlastreduktion und Vorlaststeigerung bei MI bergen die konventionell verwendeten Ejektionsindices, Ejektionsfraktion (EF) und Fractional Area Change (FAC), die Gefahr der Überschätzung der effektiven Pumpfunktion des linken Ventrikels (LV). Der dopplersonographisch erhobene Tei Index gilt als ein Marker globaler myokardialer Funktion. Ziel der vorliegenden Studie war es, mit dem Tei Index (bzw. dem Myokardialen Performance Index, MPI) einen sensitiveren präoperativen Parameter zur Prädiktion der postoperativen linksventrikulären Funktion zu finden. Hierzu wurden im Rahmen einer prospektiven klinischen Studie 130 Patienten mit signifikanter MI am Herzzentrum Leipzig entsprechend den ASE/SCA Leitlinien vor und nach kardiopulmonaler Bypass-Operation mittels transösophagealer echokardiographischer (TEE) Bildgebung untersucht. Die Quantifizierung der MI erfolgte durch Messung der Vena contracta (VC). Die FAC wurde in der transgastrischen midpapillären kurzen Achse und die EF im midösophagealen Zwei- sowie Vier-Kammer-Blick erfasst. Die Zeitintervalle zur Berechnung des Tei Index wurden im tiefen transgastrischen und midösophagealen Vier-Kammer-Blick erfasst. Eine statistische Korrelation zwischen präoperativen Tei Index und postoperativer EF und FAC konnte zur Validierung unserer Hypothese nicht detektiert werden. Folgend kann der Tei Index nicht als Prädiktor der effektiven linksventrikulären Funktion vor MKR gewertet werden.

The general purpose of this thesis is to establish the ability of Speckle Tracking Echocardiography (STE) in assessing left atrial (LA) response to pressure and volume overload respectively in aortic stenosis (AS) and mitral regurgitation (MR), and to evaluate its accuracy in predicting LA and right ventricular (RV) fibrosis in patients with end-stage heart failure (HF) undergoing heart transplantation (HTx). I demonstrated that assessment of left ventricular (LV) long axis systolic velocity and amplitude of excursion is more sensitive than simple determination of ejection fraction (EF) for revealing the beneficial impact of MR surgery on overall LV systolic performance. Severe symptomatic AS is associated with LA enlargement and compromised mechanical function with a high incidence of peri-operative atrial fibrillation (AF). Valve replacement reverses these abnormalities and regains normal atrial function, a behaviour which is directly related to the severity of pre-operative LV outflow tract obstruction. Early identification of LA size and function disturbances, as shown by myocardial strain measurements might contribute to better patient’s recruitment for a safe valve replacement. In late stage HF patients, the right ventricle is enlarged, with reduced systolic function due to significant myocardial fibrosis. RV free wall myocardial deformation is the most accurate function measure that correlates with the extent of RV myocardial fibrosis and functional capacity. In patients with preserved EF, severe MR masks LV and LA myocardial dysfunction and correlates with symptoms and post-operative cavity function instability. Three months after MVR, the underlying myocardial disturbances are unmasked suggesting that most pre-operative measurements are subject to loading conditions. Finally LA volume and PALS remain the main predictors of post-operative AF, thus should be used for stratifying surgical risk. STE has been shown to accurately determine the severity of impairment of LA myocardial function shown by suppressed PALS which was the strongest predictor of the presence and extent of fibrosis, over and above other structure and function parameters. These findings may assist in better stratifying patients with end stage HF and identifying particularly those requiring HTx.

No description available.

Les analyses des fonctions ventriculaires gauche (VG) et droite (VD) et des pressions de remplissage sont des éléments importants dans le contexte des défaillances cardio-circulatoires graves car elles ont des conséquences diagnostiques et pronostiques avec un impact sur les prises de décisions thérapeutiques. Toutefois, l'évaluation de la fonction myocardique en cas de choc reste difficile pour des raisons physiopathologiques et technologiques. Les paramètres de fonction ventriculaire longitudinale (FVL) pourraient avoir un intérêt dans ce domaine car ils permettent une évaluation directe d'une composante majeure de la mécanique ventriculaire. Ces paramètres ont été évalués chez les défaillances chroniques stables et ont montré leurs intérêts diagnostiques et pronostiques. Ils restent très peu étudiés dans les contextes aigues et graves. / Despite advances in management and therapies, cardiogenic shock remains a clinical challenge with high mortality rates.The analysis of left and right ventricular functions and filling pressures are important in this context because they had diagnostic and prognostic consequences with impact in therapeutic decisions. Nevertheless, the assessment of myocardial function remains difficult for physiopathologic and technical reasons. The parameters of longitudinal ventricular function (LgVF) could have an interest in this context because they permit a direct assessment of a major component of ventricular mechanics whereas ejection fraction remains a global evaluation. These indices were assessed in chronic and stable heart failure patients and were found to have prognostic and diagnostic interests. Though, they were not evaluated in the context of acute and severe cardio-circulatory failures.

Introdução: O exame ecocardiográfico direcionado realizado por médicos intensivistas e emergencistas pode ser uma ferramenta valiosa para a avaliação e manejo do estado hemodinâmico de crianças gravemente doentes. O objetivo do presente estudo foi avaliar o aprendizado de médicos pediatras de setores de emergência submetidos a um programa de treinamento teórico e prático supervisionado em ecocardiografia de emergência. Método: Treinamento com componente teórico e prático foi realizado por dois ecocardiografistas experientes e teve como alvo 16 médicos intensivistas/emergencistas pediátricos. O programa incluiu análises qualitativas de função do ventrículo esquerdo (VE) e ventrículo direito (VD), avaliação de derrame pericárdio/tamponamento cardíaco, regurgitação valvar e avaliação de variação respiratória de veia cava inferior através do índice de distensibilidade da veia cava inferior (dVCI), além de medidas objetivas de fração de ejeção (FE) e índice cardíaco (IC). As aulas práticas foram realizadas na unidade de terapia intensiva pediátrica do Instituto da Criança do Hospital das Clínicas e cada aluno realizou 24 exames direcionados de ecocardiograma durante o treinamento. Os alunos foram avaliados de forma prática, sendo os resultados dos exames realizados pelos alunos comparados com os exames realizados pelo ecocardiografista experiente. As avaliações ocorreram após 8, 16 e 24 exames práticos. Resultados: A taxa de concordância entre o aluno e ecocardiografista na análise subjetiva da função do VE foi de 81,3% na primeira avaliação, 96,9% na segunda avaliação e 100% na terceira avaliação (p < 0,001). Para o dVCI, observou-se uma concordância de 46,7% na primeira avaliação, 90,3% na segunda avaliação e 87,5% na terceira avaliação (p = 0,004). As médias das diferenças entre os alunos e ecocardiografista na medida de fração de ejeção e índice cardíaco foram 7% e 0,56 L/min/m2, respectivamente, após a terceira etapa do treinamento. Conclusões: O treinamento proposto demonstrou ser suficiente para capacitar os médicos pediatras na análise de função do VE de forma subjetiva e também para as medidas objetivas de dVCI, FE e IC. Este modelo de curso pode facilitar o desenho de outros treinamentos médicos em ecocardiografia de emergência e auxiliar na implementação destes cursos de forma isolada ou inserido em programas de residência médica, gerando melhoria nas habilidades técnicas dos médicos dos setores de emergência e desta forma culminando com melhor cuidado dos pacientes criticamente enfermos / Background: Focused echocardiographic examinations performed by intensivists and emergency room physicians can be a valuable tool for diagnosing and managing the hemodynamic status of critically ill children. The aim of this study was to evaluate the learning curve achieved using a theoretical and practical training program designed to enable pediatric intensivists and emergency physicians to conduct targeted echocardiograms. Methods: Theoretical and practical training sessions were conducted with 16 pediatric intensivist/emergency room physicians. The program included qualitative analyses of the left ventricular (LV) and right ventricular (RV) functions, evaluation of pericardial effusion/cardiac tamponade and valvular regurgitation and measurements of the distensibility index of the inferior vena cava (dIVC), ejection fraction (EF) and cardiac index (CI). The practical training sessions were conducted in the intensive care unit; each student performed 24 echocardiograms. The students in training were evaluated in a practical manner, and the results were compared with the corresponding examinations performed by experienced echocardiographer. The evaluations occurred after 8, 16 and 24 practical examinations. Results: The concordance rates between the students and echocardiographers in the subjective analysis of the LV function were 81.3% at the first evaluation, 96.9% at the second evaluation and 100% at the third evaluation (p < 0.001). For the dIVC, we observed a concordance of 46.7% at the first evaluation, 90.3% at the second evaluation and 87.5% at the third evaluation (p=0.004). The means of the differences between the students\' and echocardiographers\' measurements of the EF and CI were 7% and 0.56 L/min/m2, respectively, after the third stage of training. Conclusions: The proposed training was demonstrated to be sufficient for enabling pediatric physicians to analyze subjective LV function and to measure dIVC, EF and CI. This training course should facilitate the design of other echocardiography training courses that could be implemented in medical residency programs to improve these physicians\' technical skills and the care of critically ill patients

Objetivo: O principal propósito do estudo foi pesquisar a disfunção ventricular esquerda subclínica em pacientes com lúpus eritematoso sistêmico juvenil (LESJ) através da técnica de speckle-tracking bidimensional. Foi investigada ainda uma possível correlação entre o comprometimento da deformação miocárdica e o SLEDAI-2K (Systemic Lupus Erithematosus Disease Activity Index 2000), bem como a presença de fatores de risco cardiovascular, tanto tradicionais como ligados à doença. Métodos: 50 pacientes assintomáticos do ponto de vista cardiovascular e 50 controles saudáveis (14,74 vs. 14,82 anos, p=0.83) foram avaliados pelo ecocardiograma convencional e pelo speckle-tracking bidimensional. Resultados: Apesar da fração de ejeção normal, os pacientes apresentaram redução de todos os parâmetros de deformação miocárdica longitudinal e radial, quando comparados aos controles: strain de pico sistólico longitudinal [-20,3 (-11 a -26) vs. -22 (-17,8 a -30.4) %, p < 0,0001], strain rate de pico sistólico longitudinal [-1,19 ± 0,21 vs. -1,3 ± 0,25 s-1, p=0,0005], strain rate longitudinal na diástole precoce [1,7 (0,99 a 2,95) vs. 2 (1,08 a 3,00) s-1 , p=0,0034], strain de pico sistólico radial [33,09 ± 8,6 vs. 44,36 ± 8,72%, p < 0,0001], strain rate de pico sistólico radial [1,98 ± 0,53 vs. 2,49 ± 0,68 s-1, p < 0,0001] e strain rate radial na diástole precoce [-2,31 ± 0,88 vs. -2,75 ± 0,97 s-1, p=0,02]. O strain de pico sistólico circunferencial [-23,67 ± 3,46 vs. - 24,6 ± 2,86%, p=0,43] e o strain rate circunferencial na diástole precoce [2 (0,88 a 3,4) vs. 1,99 (1,19 a 3,7) s-1, p=0,88] foram semelhantes em pacientes e controles. Apenas o strain rate de pico sistólico circunferencial [-1,5 ± 0,3 vs. -1,6 ± 0,3 s-1, p=0,036] mostrou-se reduzido no LESJ. Uma correlação negativa foi identificada entre o strain de pico sistólico longitudinal e o SLEDAI-2K (r = - 0,52; p < 0,0001) e também o número de fatores de risco cardiovascular por paciente (r = -0,32, p=0,024). Conclusões: Foi evidenciada disfunção sistólica e diastólica subclínica de ventrículo esquerdo no LESJ através da técnica de speckle-tracking bidimensional. A atividade da doença e a exposição aos fatores de risco cardiovascular provavelmente contribuíram para o comprometimento da deformação miocárdica nesses pacientes / Objectives: The main purpose of the study was to investigate left ventricular (LV) subclinical systolic and diastolic dysfunction in childhood-onset systemic lupus erythematosus (c-SLE) patients using two-dimensional speckletracking (2DST) echocardiography. We also interrogated possible correlations between impairment of myocardial deformation and the SLE Disease Activity Index 2000 (SLEDAI-2K), as well as the presence of traditional and disease-related cardiovascular risk factors (CRFs). Method: A total of 50 asymptomatic patients and 50 controls (age 14.74 vs. 14.82 years, p = 0.83) were evaluated by standard and 2DST echocardiography. Results: Despite a normal ejection fraction (EF), there was reduction in all parameters of LV longitudinal and radial deformation in patients compared to controls: peak longitudinal systolic strain [-20.3 (-11 to -26) vs. -22 (-17.8 to -30.4)%, p < 0.0001], peak longitudinal systolic strain rate [-1.19 ± 0.21 vs. -1.3 ± 0.25 s-1, p = 0.0005], longitudinal strain rate in early diastole [1.7 (0.99-2.95) vs. 2 (1.08-3.00) s-1, p = 0.0034], peak radial systolic strain [33.09 ± 8.6 vs. 44.36 ± 8.72%, p < 0.0001], peak radial systolic strain rate [1.98 ± 0.53 vs. 2.49 ± 0.68 s-1, p < 0.0001], and radial strain rate in early diastole [-2.31 ± 0.88 vs. -2.75 ± 0.97 s-1, p = 0.02]. Peak circumferential systolic strain [- 23.67 ± 3.46 vs. -24.6 ± 2.86%, p=0.43] and circumferential strain in early diastole [2 (0,88 a 3,4) vs. 1,99 (1,19 a 3,7) s-1, p=0.88 ] were similar between patients and controls, although peak circumferential systolic strain rate [-1.5 ± 0.3 vs. -1.6 ± 0.3 s-1, p = 0.036] was reduced in c-SLE. Further analysis of patients revealed a negative correlation between LV peak longitudinal systolic strain and SLEDAI-2K(r= -0.52, p < 0.0001) and also between LV PLSS and the number of CRFs per patient (r = -0.32, p = 0.024). Conclusions: 2DST echocardiography has identified subclinical LV deformation impairment in c-SLE patients. Disease activity and cumulative exposure to CRFs contribute to myocardial compromise

Die Diagnostik von angeborenen Herzfehlern, und vor allem die häufig lebenslange postoperative Verlaufskontrolle dieser Patienten, verlangt nach einem möglichst schonenden und dennoch zuverlässigen bildgebenden Verfahren. In der Regel wird hierzu die Echokardiographie eingesetzt, die jedoch insbesondere bei älteren Patienten aufgrund eines unzureichenden Schallfensters Schwierigkeiten bereitet. Für diese Patienten bietet sich die Magnetresonanztomographie (MRT) als alternatives bildgebendes Verfahren an. Ziel dieser Untersuchung war es, nach der Evaluation zweier funktioneller MRT-Methoden, der Flussmessung im Phantomversuch und der Ventrikelfunktionsanalyse im Vergleich zur 3D-Echokardiographie, verschiedene MR-Methoden an 6 verschiedenen Patientenkollektiven mit verschiedenen angeborenen Herzfehlern unkorrigiert und operativ korrigiert anzuwenden. 1. Bei Patienten mit Aortenisthmusstenose zeigte sich, dass neben der guten Korrelation der Abschätzung des Druckgradienten über einer Stenose oder Re-Stenose mit Hilfe der MR-Flussmessung, auch die drei-dimensionale Rekonstruktion aus der MR-Angiographie (MRA) zusätzliche Informationen liefert. 2. In der postoperativen Verlaufskontrolle von Patienten mit Fallott´scher Tetralogie ergab die Analyse der mit der MRT ermittelten Funktionsparameter Regurgitationsfraktion über der Pulmonalklappe und rechtsventrikulärer Volumina eine Korrelation zwischen der QRS-Dauer und dem rechtsventrikulären enddiastolischen Volumen (RV-EDV), was auf eine mechanoelektrische Interaktion bei der Genese ventrikulärer Arrhythmien hinweist. 3. Die MRT war in der Lage bei Patienten mit kongenital korrigierter Transposition der grossen Gefässe alleine aufgrund der Analyse der Morphologie die Diagnose dieser Fehlbildung zu stellen und in der Mehrzahl der Fälle Begleitfehlbildungen aufzudecken. 4. Mit Hilfe der MR-Flussmessung konnten bei Patienten nach arterieller und atrialer switch Operation zur Korrektur einer kompletten Transposition der grossen Gefässe sowohl Stenosen im Vergleich zur invasiven Herzkatheteruntersuchung sicher quantifiziert werden als auch unphysiologische Flussverhältnisse, die möglicherweise das Auftreten von Stenosen in den Pulmonalarterien oder dem venösen "baffle" begünstigen, erkannt werden. 5. Bei der seltenen Ebsteinanomalie erwies sich die MRT als zuverlässige Methode insbesondere die pathologische Anatomie der Trikuspidalklappe zu detektieren, als auch die Ventrikelfunktion zu quantifizieren. 6. Nach Fontanoperation erwies sich die MRT mit der Ventrikelfunktionsanalyse und Flussmessung ebenfalls als geeignete Methode, um pathologische Flussverhältnisse im Operationsgebiet zu detektieren und ihren möglichen Einfluss auf die ventrikuläre Funktion zu analysieren. In der Primärdiagnostik und insbesondere in der postoperativen Verlaufskontrolle von angeborenen Herzfehlern stellt die MRT somit unter Einsatz der MR-Flussmessung und Ventrikelfunktionsanalyse eine zuverlässige nicht-invasive Methode dar, die auch im Vergleich zur Herzkatheteruntersuchung einige Vorteile aufweist. / The diagnostics of congenital heart disease, and especially the need for frequent lifelong postoperative follow-up examinations, require an imaging modality as gentle and reliable as possible. The method commonly used for this purpose ? echocardiography - is associated with some disadvantages, especially in older patients with an insufficient acoustic window. For these patients magnetic resonance imaging (MRI) is an alternative imaging modality. The aim of this study was to evaluate two functional MR methods, flow measurement with a flow phantom and ventricular function analysis in comparison to 3D echocardiography. These methods were performed in 6 different groups of patients with uncorrected or surgically corrected congenital heart diseases. 1. Beside a good correlation between the estimated pressure gradients in the area of stenoses and re-stenoses in patients with coarctation with the aid of MR flow measurements, three-dimensional reconstruction from MR angiography (MRA) data supplies additional information. 2. The analysis of parameters such as regurgitant fraction over the pulmonary valve and right ventricular volumes derived by MRI in the postoperative follow-up of patients with tetralogy of Fallot showed a correlation to QRS duration which indicates a mechanoelectrical interaction as one etiology of arrhythmias in these patients. 3. The diagnosis of a congenitally corrected transposition of the great arteries could be made only by anatomical evaluation of the MR images. In the majority of patients all additional anomalies could be detected by MRI. 4. With the use of MR flow measurements as compared to gradients measured invasively by cardiac catheterization it was possible to reliably quantify stenoses. Furthermore, unphysiologic flow patterns, which could favor the development of pulmonary artery or "baffle" stenoses, could be detected. 5. MRI could be reliably used to detect the abnormal morphology of the tricuspid valve in patients with Ebstein anomaly and to quantify ventricular function in these patients. 6. Furthermore, after Fontan operation MRI was a suitable method to detect pathologic flow patterns at the operation site and to analyze their potential effects on ventricular function. MRI using the techniques of flow measurements and ventricular function analysis is a reliable non-invasive tool in the primary diagnostic procedure and especially in the postoperative follow-up of congenital heart disease. It has advantages even in comparison with the invasive method of cardiac catheterization.

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

Introdução: Dos mecanismos fisiopatológicos à estratificação de risco e manejo, ainda persistem hoje muitas lacunas e debates sobre a miocardiopatia não compactada (MNC). Recentemente, a ressonância magnética cardiovascular (RMC) vem sendo amplamente utilizada para aumentar a precisão do diagnóstico de MNC em pacientes com alta probabilidade clínica pré-teste, com valor prognóstico e alta relevância na tomada de decisões clínicas. Objetivo: Este estudo teve como objetivo caracterizar o mapeamento T1 e o volume extracelular (VEC) miocárdico por RMC em pacientes com MNC e investigar como esses marcadores teciduais relacionam-se com a fração de ejeção do ventrículo esquerdo (FEVE) e arritmias ventriculares (AV). Métodos: Foram recrutados prospectivamente 36 pacientes com MNC e 18 controles saudáveis para realizarem uma RMC com mapeamento T1 entre julho de 2013 e setembro de 2016. O VEC foi avaliado apenas para os segmentos do ventrículo esquerdo sem áreas de fibrose macroscópica pela técnica do realce tardio (RT), objetivando-se investigar a presença de fibrose miocárdica intersticial difusa. Para avaliar as diferenças entre os parâmetros de RMC nos pacientes e controles, foram usados o teste t entre as amostras pareadas (Wilcoxon) e um modelo de regressão linear foi construído para investigar a relação entre a FEVE e os achados clínicos e de imagem (inclusive o VEC). Resultados: Os pacientes com MNC apresentaram maiores valores de T1 nativo (1.024 ± 43ms versus 995 ± 22ms, p = 0,01) e VEC (28,0 ± 4,5% vs. 23,5 ± 2,2%, p < 0,001) em relação aos controles. Apenas o VEC foi associado independentemente com a FEVE (beta = -1,3, p = 0,003) na regressão multivariada. Houve uma interessante tendência para a terapia betabloqueadora modificar positivamente a relação entre ECV e LVEF (beta = 4,1, intervalo de confiança de 95%, -0,6 a 8,8), porém com p alto (0,08). Além disso, entre pacientes com MNC e RT ausente (negativo), AV foram associadas com maior VEC (27,7% em pacientes com AV vs 25,8% em pacientes sem AV, p = 0,002). Conclusão: Nos pacientes com MNC, a caracterização tecidual miocárdica por mapeamento T1 sugere uma expansão extracelular por fibrose intersticial difusa no miocardio sem fibrose focal pelo RT, o que foi associada à disfunção ventricular e AV. Tais achados podem dar suporte a um potencial valor do mapeamento T1 no refino da estratificação de risco de pacientes com MNC / Background: From pathophysiological mechanisms to risk stratification and management, much debate and discussion persist regarding non-compaction cardiomyopathy (NCC). Recently, cardiovascular magnetic resonance (CMR) imaging has been widely used to more accurately diagnose NCC in patients with high clinical pre-test probability, with prognostic value and high relevance in the clinical decision making process. Purpose: This study aimed to characterize myocardial T1 mapping and extracellular volume (ECV) fraction by cardiovascular magnetic resonance (CMR), as well as investigate how these tissue markers relate to left ventricular ejection fraction (LVEF) and ventricular arrhythmias (VA) in patients with NCC. Methods: We prospectively recruited 36 patients with NCC and 18 controls to perform a cardiovascular magnetic resonance (CMR) with T1 mapping between July 2013 and September 2016. ECV was quantified in LV segments without late gadolinium enhancement (LGE) areas to investigate diffuse myocardial fibrosis. Differences in CMR parameters between patients and controls were assessed using t-test or Wilcoxon rank-sum test, and a linear regression model was built for LVEF to test the association with ECV and clinical characteristics. Results: Patients with NCC had higher native T1 (1024±43ms vs. 995±22ms, p=0.01) and expanded ECV (28.0±4.5% vs. 23.5±2.2%, p < 0.001) compared to controls. ECV was independently associated with LVEF (beta=-1.3, p=0.003). There was a trend for beta-blocker therapy to modify the relationship between ECV and LVEF (beta=4.1, 95% confidence interval, 0.6 to 8.8, p=0.08). Moreover, among patients without LGE, VA were associated with higher ECV (27.7% with VA vs 25.8% without VA, p=0.002). Conclusion: In NCC patients, tissue characterization by T1 mapping suggests an extracellular expansion by diffuse fibrosis in myocardium without LGE, which was associated with myocardial dysfunction and VA. These findings lend support to the potential role of T1 mapping in refining NCC risk stratification