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BACKGROUND AND AIMS: Patients with repaired tetralogy of Fallot remain at risk of life-threatening ventricular tachycardia related to slow-conducting anatomical isthmuses (SCAIs). Preventive ablation of SCAI identified by invasive electroanatomical mapping is increasingly performed. This study aimed to non-invasively identify SCAI using 3D late gadolinium enhancement cardiac magnetic resonance (3D-LGE-CMR). METHODS: Consecutive tetralogy of Fallot patients who underwent right ventricular electroanatomical mapping (RV-EAM) and 3D-LGE-CMR were included. High signal intensity threshold for abnormal myocardium was determined based on direct comparison of bipolar voltages and signal intensity by co-registration of RV-EAM with 3D-LGE-CMR. The diagnostic performance of 3D-LGE-CMR to non-invasively identify SCAI was determined, validated in a second cohort, and compared with the discriminative ability of proposed risk scores. RESULTS: The derivation cohort consisted of 48 (34 ± 16 years) and the validation cohort of 53 patients (36 ± 18 years). In the derivation cohort, 78 of 107 anatomical isthmuses (AIs) identified by EAM were normal-conducting AI, 22 were SCAI, and 7 blocked AI. High signal intensity threshold was 42% of the maximal signal intensity. The sensitivity and specificity of 3D-LGE-CMR for identifying SCAI or blocked AI were 100% and 90%, respectively. In the validation cohort, 85 of 124 AIs were normal-conducting AI, 36 were SCAI, and 3 blocked AI. The sensitivity and specificity of 3D-LGE-CMR were 95% and 91%, respectively. All risk scores showed an at best modest performance to identify SCAI (area under the curve ≤ .68). CONCLUSIONS: 3D late gadolinium enhancement cardiac magnetic resonance can identify SCAI with excellent accuracy and may refine non-invasive risk stratification and patient selection for invasive EAM in tetralogy of Fallot.
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Imageamento Tridimensional , Taquicardia Ventricular , Tetralogia de Fallot , Humanos , Tetralogia de Fallot/cirurgia , Tetralogia de Fallot/diagnóstico por imagem , Masculino , Feminino , Adulto , Taquicardia Ventricular/diagnóstico , Taquicardia Ventricular/diagnóstico por imagem , Adulto Jovem , Meios de Contraste , Pessoa de Meia-IdadeRESUMO
A case of a newborn with tetralogy of Fallot, corpus callosum hypoplasia, and phenotypic features similar to DiGeorge syndrome. Chromosomal microarray analysis did not reveal any alterations. Whole exome sequencing and Sanger sequencing identified a de novo variant in the HIRA gene resulting in the loss of the start codon.
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Proteínas de Ciclo Celular , Síndrome de DiGeorge , Chaperonas de Histonas , Feminino , Humanos , Recém-Nascido , Masculino , Agenesia do Corpo Caloso/genética , Proteínas de Ciclo Celular/genética , Síndrome de DiGeorge/genética , Síndrome de DiGeorge/patologia , Sequenciamento do Exoma , Chaperonas de Histonas/genética , Fenótipo , Tetralogia de Fallot/genética , Fatores de Transcrição/genética , Adulto , LinhagemRESUMO
We present the case of a girl with complete tetraploidy who has survived to her present age of 4 years and 1 month. Infants with complete tetraploidy have been described to have a limited lifespan owing to complications. We report her characteristics, medical history, and development.
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BACKGROUND: The Alterra adaptive prestent is a novel self-expanding device designed to provide a landing zone for the 29 mm SAPIEN 3 valve to treat pulmonary regurgitation in patients with a right ventricular outflow tract that is too large for a balloon expandable valve alone. The mechanism of fixation for the Alterra prestent is radial force from the self-expanding stent frame, combined with a unique set of flared "tines" that protrude from both ends of the stent. AIMS, METHODS, AND RESULTS: In this report, we describe 6 patients who underwent uncomplicated transcatheter pulmonary valve replacement with an Alterra adaptive prestent and SAPIEN 3 valve and had surveillance chest computed tomography (CT) scans performed 1 day to 21 months after implant. In each patient, the CT scan demonstrated extravascular extension of a portion of the Alterra prestent, without clinical sequelae, but with extension into the ascending aorta in 1 patient and contact with the ascending aorta, left pulmonary vein, or left atrial appendage in 3 others. CONCLUSIONS: Surveillance CT imaging shows that the Alterra prestent can perforate the pulmonary artery and/or right ventricle. Although no sequelae were seen in these patients, prestent perforation has the potential to be clinically important. Implanters should be aware of this finding and its potential implications. As experience with the Alterra prestent grows, it will be important to further define the risk factors, incidence, and implications of this phenomenon.
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Implante de Prótese de Valva Cardíaca , Próteses Valvulares Cardíacas , Valor Preditivo dos Testes , Desenho de Prótese , Humanos , Masculino , Feminino , Resultado do Tratamento , Implante de Prótese de Valva Cardíaca/instrumentação , Implante de Prótese de Valva Cardíaca/efeitos adversos , Fatores de Tempo , Adulto , Cateterismo Cardíaco/instrumentação , Cateterismo Cardíaco/efeitos adversos , Valva Pulmonar/diagnóstico por imagem , Valva Pulmonar/cirurgia , Valva Pulmonar/fisiopatologia , Insuficiência da Valva Pulmonar/diagnóstico por imagem , Insuficiência da Valva Pulmonar/fisiopatologia , Insuficiência da Valva Pulmonar/cirurgia , Insuficiência da Valva Pulmonar/etiologia , Adulto Jovem , Angiografia por Tomografia Computadorizada , Adolescente , Artéria Pulmonar/diagnóstico por imagem , Artéria Pulmonar/fisiopatologia , Tomografia Computadorizada por Raios XRESUMO
OBJECTIVES: Cardiovascular magnetic resonance (CMR) imaging is routinely performed for assessing right ventricular (RV) systolic but not diastolic function. We aimed to investigate CMR-based assessment of RV diastolic function in pediatric patients with repaired tetralogy of Fallot (rTOF), compared to transthoracic echocardiography (TTE) measurements. METHODS: A total of 130 consecutive pediatric patients with rTOF who underwent clinically indicated CMR and same-day TTE were included. Forty-three controls were recruited. Phase-contrast images were used to measure trans-tricuspid valve flow velocities during early (E) and late diastolic (A) phases (measured in cm/s). Feature tracking of the tricuspid annulus was performed to derive early (e') and late diastolic (a') myocardial velocities (measured in cm/s). RV diastolic function was evaluated by E/A ratio, E/e' ratio, and E-wave deceleration time (measured in milliseconds). Regression analyses were utilized to identify potential variables associated with RV diastolic dysfunction (DD). The performance of CMR-derived parameters in diagnosing RV DD was assessed using receiver-operating characteristic analyses. RESULTS: Good agreement was found between CMR and TTE measurements (ICC 0.70-0.89). Patients with RV DD (n = 67) showed significantly different CMR-derived parameters including E and e' velocities, and E/A and E/e' ratio, compared to patients without DD (n = 63) (all p < 0.05). CMR-derived E and e' velocities and E/e' ratio were independent predictors of RV DD. E/e' of 5.8 demonstrated the highest discrimination of RV DD (AUC 0.76, sensitivity 70%, specificity 86%). CONCLUSIONS: CMR-derived parameters showed good agreement with TTE parameters in determining RV DD. CMR-derived E/e' was proved to be the most effective in identifying RV DD. CLINICAL RELEVANCE STATEMENT: This study demonstrated the feasibility and efficacy of CMR in assessing diastolic function in pediatric patients. RV DD was presented in over half of patients according to current TTE guidelines, highlighting the need for assessing RV diastolic function during follow-up. KEY POINTS: ⢠Routinely acquired cine and phase-contrast cardiovascular magnetic resonance (CMR) images yielded right ventricular (RV) diastolic parameters which demonstrated good agreement with transthoracic echocardiography (TTE) measurements. ⢠There was a high prevalence of RV diastolic function impairment in pediatric patients with repaired tetralogy of Fallot (rTOF). ⢠CMR is a reliable complementary modality of TTE for RV diastolic function evaluation.
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Diástole , Ecocardiografia , Tetralogia de Fallot , Disfunção Ventricular Direita , Humanos , Tetralogia de Fallot/cirurgia , Tetralogia de Fallot/diagnóstico por imagem , Tetralogia de Fallot/fisiopatologia , Masculino , Feminino , Criança , Ecocardiografia/métodos , Adolescente , Disfunção Ventricular Direita/diagnóstico por imagem , Disfunção Ventricular Direita/fisiopatologia , Disfunção Ventricular Direita/etiologia , Pré-Escolar , Função Ventricular Direita/fisiologia , Imagem Cinética por Ressonância Magnética/métodos , Imageamento por Ressonância Magnética/métodosRESUMO
BACKGROUND: Many patients with repaired tetralogy of Fallot require pulmonary valve replacement (PVR) due to significant pulmonary regurgitation (PR). Transcatheter PVR (TPVR) is an equally effective and less invasive alternative to surgical PVR but many native right ventricular outflow tracts (RVOTs) are too large for TPVR at time of referral. Understanding the rate of growth of the RVOT may help optimize timing of referral. This study aims to examine the longitudinal growth of the native RVOT over time in repaired tetralogy of Fallot (TOF). METHODS: A retrospective review of serial cardiac MRI cardiovascular magnetic resonance (CMR) data from 121 patients with repaired TOF and a native RVOT (median age at first CMR 14.7 years, average interval between the first and last CMR of 8.1 years) was performed to measure serial changes in RVOT diameter, cross-sectional area, perimeter-derived diameter, and length. RESULTS: All parameters of RVOT size continued to grow with increasing age but growth was more rapid in the decade after TOF repair (for minimum systolic diameter, mean increase of 5.7 mm per 10 years up to year 12, subsequently 2.3 mm per 10 years). The RVOT was larger with a transannular patch and in patients without pulmonary stenosis (p < 0.001 for both), but this was not associated with rate of growth. More rapid RVOT enlargement was noted in patients with larger right ventricular end-diastolic volume (RVEDV), higher PR fraction, and greater rates of increases in RVEDV and PR (p < 0.001 for all) CONCLUSIONS: in patients with repaired TOF, using serial CMR data, we found that RVOT size increased progressively at all ages, but the rate was more rapid in the first decade after repair. More rapid RVOT enlargement was noted in patients with a larger RV, more PR, and greater rates of increases in RV size and PR severity. These results may be important in considering timing of referral for transcatheter pulmonary valves, in planning transcatheter and surgical valve replacement, and in designing future valves for the native RVOT.
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Procedimentos Cirúrgicos Cardíacos , Valor Preditivo dos Testes , Insuficiência da Valva Pulmonar , Tetralogia de Fallot , Função Ventricular Direita , Humanos , Tetralogia de Fallot/cirurgia , Tetralogia de Fallot/diagnóstico por imagem , Tetralogia de Fallot/fisiopatologia , Estudos Retrospectivos , Adolescente , Fatores de Tempo , Criança , Adulto Jovem , Masculino , Feminino , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Resultado do Tratamento , Adulto , Insuficiência da Valva Pulmonar/fisiopatologia , Insuficiência da Valva Pulmonar/diagnóstico por imagem , Insuficiência da Valva Pulmonar/cirurgia , Insuficiência da Valva Pulmonar/etiologia , Estudos Longitudinais , Pré-Escolar , Imagem Cinética por Ressonância Magnética , Fatores Etários , Valva Pulmonar/diagnóstico por imagem , Valva Pulmonar/cirurgia , Valva Pulmonar/fisiopatologia , Lactente , Ventrículos do Coração/diagnóstico por imagem , Ventrículos do Coração/fisiopatologia , Ventrículos do Coração/cirurgia , Imageamento por Ressonância Magnética , Pessoa de Meia-IdadeRESUMO
BACKGROUND: The prognostic value of myocardial deformation parameters in adults with repaired tetralogy of Fallot (rTOF) has not been well-elucidated. We therefore aimed to explore myocardial deformation parameters for outcome prediction in adults with rTOF using cardiovascular magnetic resonance imaging (CMR). METHODS: Adults with rTOF and at least moderate pulmonary regurgitation were identified from an institutional prospective CMR registry. Left ventricular (LV) and right ventricular (RV) global strains were recorded in longitudinal (GLS), circumferential (GCS), and radial (GRS) directions. Major adverse cardiovascular events (MACE) were defined as a composite of mortality, resuscitated sudden death, sustained ventricular tachycardia (>30 seconds), or heart failure (hospital admission >24 hours). In patients with pulmonary valve replacement (PVR), pre- and post-PVR CMR studies were analyzed to assess for predictors of complete RV reverse remodeling, defined as indexed RV end-diastolic volume (RVEDVi) <110 mL/m2. Logistic regression models were used to estimate the odds ratio (OR) per unit change in absolute strain value associated with clinical outcomes and receiver operator characteristic curves were constructed with area under the curve (AUC) for select CMR variables. RESULTS: We included 307 patients (age 35 ± 13 years, 59% (180/307) male). During 6.1 years (3.3-8.8) of follow-up, PVR was performed in 142 (46%) and MACE occurred in 31 (10%). On univariate analysis, baseline biventricular ejection fraction (EF), mass, and all strain parameters were associated with MACE. After adjustment for LVEF, only LV-GLS remained independently predictive of MACE (OR 0.822 [0.693-0.976] p = 0.025). Receiver operator curves identified an absolute LV-GLS value less than 15 and LVEF less than 51% as thresholds for MACE prediction (AUC 0.759 [0.655-0.840] and 0.720 [0.608-0.810]). After adjusting for baseline RVEDVi, RV-GCS (OR 1.323 [1.094-1.600] p = 0.004), LV-GCS (OR 1.276 [1.029-1.582] p = 0.027) and LV-GRS (OR 1.101 [1.0210-1.200], p = 0.028) were independent predictors of complete remodeling post-PVR remodeling. CONCLUSION: Biventricular strain parameters predict clinical outcomes and post-PVR remodeling in rTOF. Further study will be necessary to establish the role of myocardial deformation parameters in clinical practice.
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AIMS: Slow conduction (SC) anatomical isthmuses (AIs) are the dominant substrate for monomorphic ventricular tachycardia (VT) in patients with repaired tetralogy of Fallot (rTF). This study aimed to evaluate the utility of automated propagational analysis for the identification of SC-AI in patients with rTF. METHODS AND RESULTS: Consecutive rTF patients undergoing VT substrate characterization were included. Automated isochronal late activation maps (ILAM) were obtained with multielectrode HD Grid Catheter. Identified deceleration zones (DZs) were compared with both SC-AI defined by conduction velocity (CV) (<0.5â m/s) and isthmuses of induced VT for mechanistic correlation. Fourteen patients were included (age 48; p25-75 35-52 years; 57% male), 2 with spontaneous VT and 12 for risk stratification. Nine VTs were inducible in seven patients. Procedure time was 140 (p25-75 133-180) min and mapping time 29.5 (p25-75 20-37.7) min, using a median of 2167 points. All the patients had at least one AI by substrate mapping, identifying a total of 27 (11 SC-AIs). Isochronal late activation maps detected 10 DZs mostly in the AI between ventricular septal defect and pulmonary valve (80%). Five patients had no DZs. A significant negative correlation between number of isochrones/cm and CV was observed (rho -0.87; P < 0.001). Deceleration zones correctly identified SC-AI (90% sensitivity; 100% specificity; 0.94 accuracy) and was related to VT inducibility (P = 0.006). Deceleration zones co-localized to the critical isthmus of induced VTs in 88% of cases. No complications were observed. CONCLUSION: Deceleration zones displayed by ILAM during sinus rhythm accurately identify SC-AIs in rTF patients allowing a safe and short-time VT substrate characterization procedure.
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Ablação por Cateter , Valva Pulmonar , Taquicardia Ventricular , Tetralogia de Fallot , Humanos , Masculino , Pessoa de Meia-Idade , Feminino , Tetralogia de Fallot/cirurgia , Frequência Cardíaca/fisiologia , Arritmias Cardíacas , Ablação por Cateter/efeitos adversosRESUMO
BACKGROUND AND AIMS: Right bundle branch block (RBBB) and resulting right ventricular (RV) electromechanical discoordination are thought to play a role in the disease process of subpulmonary RV dysfunction that frequently occur post-repair tetralogy of Fallot (ToF). We sought to describe this disease entity, the role of pulmonary re-valvulation, and the potential added value of RV cardiac resynchronization therapy (RV-CRT). METHODS: Two patients with repaired ToF, complete RBBB, pulmonary regurgitation, and significantly decreased RV function underwent echocardiography, cardiac magnetic resonance, and an invasive study to evaluate the potential for RV-CRT as part of the management strategy. The data were used to personalize the CircAdapt model of the human heart and circulation. Resulting Digital Twins were analysed to quantify the relative effects of RV pressure and volume overload and to predict the effect of RV-CRT. RESULTS: Echocardiography showed components of a classic RV dyssynchrony pattern which could be reversed by RV-CRT during invasive study and resulted in acute improvement in RV systolic function. The Digital Twins confirmed a contribution of electromechanical RV dyssynchrony to RV dysfunction and suggested improvement of RV contraction efficiency after RV-CRT. The one patient who underwent successful permanent RV-CRT as part of the pulmonary re-valvulation procedure carried improvements that were in line with the predictions based on his Digital Twin. CONCLUSION: An integrative diagnostic approach to RV dysfunction, including the construction of Digital Twins may help to identify candidates for RV-CRT as part of the lifetime management of ToF and similar congenital heart lesions.
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Terapia de Ressincronização Cardíaca , Tetralogia de Fallot , Disfunção Ventricular Direita , Humanos , Tetralogia de Fallot/diagnóstico por imagem , Tetralogia de Fallot/cirurgia , Ventrículos do Coração , Ecocardiografia , Terapia de Ressincronização Cardíaca/efeitos adversos , Bloqueio de Ramo/diagnóstico por imagem , Bloqueio de Ramo/etiologia , Bloqueio de Ramo/terapia , Disfunção Ventricular Direita/diagnóstico por imagem , Disfunção Ventricular Direita/etiologia , Disfunção Ventricular Direita/terapia , Simulação por ComputadorRESUMO
BACKGROUND: This study evaluated right ventricular (RV) volume, strain, and morphology using cardiac 4-dimensional computed tomography (4D-CT) to detect pulmonary hypertension (PH) in adults with repaired tetralogy of Fallot (TOF) scheduled for transcatheter pulmonary valve implantation (TPVI). METHODS AND RESULTS: Using cardiac 4D-CT data, we calculated RV strain in 3 different geometries and RV outflow tract (RVOT) mass in 42 patients with repaired TOF. We compared RV strain and RVOT mass between patients with and without PH. Receiver operating characteristic (ROC) analysis was conducted to evaluate the diagnostic performance of these measurements for identifying PH. Four-chamber (4ch) strain was significantly smaller for patients with (n=10) than without (n=32) PH (8.8±1.7% vs. 11.1±2.4%, respectively; P<0.01), whereas RVOT mass was significantly larger in the PH group (12.5±3.5 vs. 9.2±3.2 cm2; P<0.01). ROC analysis of the diagnostic performance revealed that the respective sensitivity and specificity was 70% and 84% (area under the curve [AUC]=0.784) for 4ch strain of 8.8%; 80% and 69% (AUC=0.766) for RVOT mass of 10.7 cm2; and 80% and 81% (AUC=0.844) for a 4ch strain/RVOT mass ratio of 0.97. CONCLUSIONS: RVOT mass and 4ch strain obtained from cardiac 4D-CT may be helpful for identifying PH in patients with repaired TOF.
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BACKGROUND: Tetralogy of Fallot (TOF) is the most common form of cyanotic congenital heart disease (CHD) worldwide. It accounts for 7% of CHD cases in Uganda and leads to fatal outcomes in the long term without surgery. Surgery is often delayed in developing countries like Uganda due to limited resources. OBJECTIVE: This study aimed to determine the early surgical outcomes of patients with TOF who underwent primary intracardiac repair at the Uganda Heart Institute (UHI) and to identify factors associated. METHODOLOGY: This retrospective chart review evaluated outcomes of primary TOF repair patients at UHI from February 2012 to October 2022. Patient outcomes were assessed from surgery until 30 days post-operation. RESULTS: Out of the 104 patients who underwent primary TOF repair at UHI, records of 88 patients (84.6%) were available for review. Males accounted for 48.9% (n = 43). The median age at the time of operation was 4 years (with an interquartile range of 2.5-8.0 years), ranging from 9 months to 16 years. Genetic syndromes were present in 5/88 (5.7%). These included 2 patients with trisomy 21, 2 with Noonan's, and 1 with 22q11.2 deletion syndrome. Early postoperative outcomes for patients included: residual ventricular septal defects in 35/88 (39.8%), right ventricular dysfunction in 33/88 (37.5%), residual pulmonary regurgitation in 27/88 (30.7%), residual right ventricular outflow tract obstruction in 27/88 (30.0%), pleural effusion in 24/88 (27.3%), arrhythmias in 24/88(27.3%), post-operative infections in 23/88(26.1%) and left ventricular systolic dysfunction in 9/88 (10.2%). Out of the children who underwent surgery after one year of age, 8% (7 children) died within the first 30 days. There was a correlation between mortality and post-operative ventilation time, cardiopulmonary bypass (CPB) time, aortic cross-clamp time, preoperative oxygen saturations, RV and LV dysfunction and the operating team. CONCLUSION: The most frequent outcomes after surgery were residual ventricular septal defects and right ventricular failure. In our study, the 30-day mortality rate following TOF repair was 8%. Deceased patients had lower pre-operative oxygen levels, longer CPB and cross-clamp times, longer post-operative ventilation, RV/LV dysfunction, and were more likely operated by the local team.
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Procedimentos Cirúrgicos Cardíacos , Complicações Pós-Operatórias , Tetralogia de Fallot , Humanos , Tetralogia de Fallot/cirurgia , Tetralogia de Fallot/mortalidade , Tetralogia de Fallot/diagnóstico , Masculino , Estudos Retrospectivos , Feminino , Uganda/epidemiologia , Pré-Escolar , Criança , Adolescente , Lactente , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Procedimentos Cirúrgicos Cardíacos/mortalidade , Resultado do Tratamento , Fatores de Tempo , Fatores de Risco , Complicações Pós-Operatórias/mortalidade , Medição de RiscoRESUMO
BACKGROUND: Patients with repaired Tetralogy of Fallot (rTOF) often develop pulmonary regurgitation (PR) and right ventricle (RV) dysfunction, experiencing increased mortality and morbidity rates in adulthood. Pulmonary valve replacement (PVR) timing to address PR is controversial. Cardiac Magnetic Resonance (CMR) is the gold standard for morpho-functional evaluation of complex cardiopathies. This study aims to identify CMR parameters predictive of adverse outcomes to help defining the best therapeutic management of rTOF patients. METHODS: 130 rTOF patients who underwent CMR (2006-2019) were enrolled in this retrospective single-center study. CMR, clinical, ECG and exercise data were analyzed. Univariate and multivariate analyses identified clinical and CMR parameters predictive of adverse outcomes both individually (e.g., death, arrhythmias, heart failure (HF), pharmacological therapy, QRS ≥ 160ms) and as composite outcome. RESULTS: Univariate analysis confirmed RV volumes and RV ejection fraction corrected for PR as adverse outcome predictors and identified interesting correlations: pulmonary artery bifurcation geometry and abnormal interventricular septum (IVS) motion with arrhythmias (p < .001; p = .037), HF (p = .049; p = .005), composite outcome (p = .039; p = .009); right atrium (RA) dimensions with the composite outcome and the outcomes individually (p < .001). The best predictive models by multivariate analysis included sex (male), RV and RA dilation for QRS ≥ 160ms, time form repair to CMR, age at TOF repair and IVS fibrosis for pharmacological therapy. CONCLUSIONS: Besides RV volumes, new adverse prognostic factors could guide rTOF therapeutic management: pulmonary arteries morphology, abnormal IVS motion, RV dysfunction, RA dilation. Perspective multicentric evaluation is needed to specify their effective role.
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Insuficiência Cardíaca , Insuficiência da Valva Pulmonar , Tetralogia de Fallot , Disfunção Ventricular Direita , Humanos , Masculino , Tetralogia de Fallot/diagnóstico por imagem , Tetralogia de Fallot/cirurgia , Estudos Retrospectivos , Imageamento por Ressonância Magnética , Insuficiência da Valva Pulmonar/diagnóstico por imagem , Insuficiência da Valva Pulmonar/etiologia , Insuficiência da Valva Pulmonar/cirurgia , Espectroscopia de Ressonância Magnética , Função Ventricular Direita , Disfunção Ventricular Direita/diagnóstico por imagem , Disfunção Ventricular Direita/etiologiaRESUMO
Tetralogy of Fallot is the most common cyanotic congenital heart disease. For decades, our institution has cared for humanitarian patients with late presentation of tetralogy of Fallot. They are characterized by severe right ventricular hypertrophy with consecutive diastolic dysfunction, increasing the risk of postoperative low cardiac output syndrome (LCOS). By right ventricular restrictive physiology, we hypothesized that patients receiving early postoperative beta-blockers (within 48 h after cardiopulmonary bypass) may have better diastolic function and cardiac output. This is a retrospective cohort study in a single-center tertiary pediatric intensive care unit. We included > 1-year-old humanitarian patients with a confirmed diagnosis of tetralogy of Fallot undergoing a complete surgical repair between 2005 and 2019. We measured demographic data, preoperative echocardiographic and cardiac catheterization measures, postoperative mean heart rate, vasoactive-inotropic scores, LCOS scores, length of stay, and mechanical ventilation duration. One hundred sixty-five patients met the inclusion criteria. Fifty-nine patients (36%) received early postoperative beta-blockers, associated with a lower mean heart rate, higher vasoactive-inotropic scores, and lower LCOS scores during the first 48 h following cardiopulmonary bypass. There was no significant difference in lengths of stay and ventilation. Conclusion: Early postoperative beta-blockers lower the prevalence of postoperative LCOS at the expense of a higher need for vasoactive drugs without any consequence on length of stay and ventilation duration. This approach may benefit the specific population of children undergoing a late complete repair of tetralogy of Fallot. What is Known: ⢠Prevalence of low cardiac output syndrome is high following a late complete surgical repair of tetralogy of Fallot. What is New: ⢠Early postoperative beta-blockade is associated with lower heart rate, prolonged relaxation time, and lower prevalence of low cardiac output syndrome. ⢠Negative chronotropic agents like beta-blockers may benefit selected patients undergoing a late complete repair of tetralogy of Fallot, who are numerous in low-income countries.
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Antagonistas Adrenérgicos beta , Tetralogia de Fallot , Humanos , Tetralogia de Fallot/cirurgia , Estudos Retrospectivos , Feminino , Masculino , Antagonistas Adrenérgicos beta/uso terapêutico , Pré-Escolar , Lactente , Débito Cardíaco/efeitos dos fármacos , Criança , Complicações Pós-Operatórias/epidemiologia , Baixo Débito Cardíaco/etiologia , Cuidados Pós-Operatórios/métodos , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Procedimentos Cirúrgicos Cardíacos/métodosRESUMO
OBJECTIVE: The objective of this meta-analysis was to assess the clinical utility of anomalous discoveries on cardiac magnetic resonance, particularly the right ventricular extracellular volume (RV-ECV), among individuals who underwent surgical repair for Tetralogy of Fallot (rTOF). METHODS: We conducted a systematic search of electronic databases including PubMed, Web of Science Core Collection, Cochrane advanced search, and EMBASE. Our analysis involved a comparison of ECV levels between rTOF patients and controls, as well as an evaluation of the predictive value of ECV for future adverse events. RESULTS: We identified 16 eligible studies that encompassed 856 rTOF patients and 283 controls. Our meta-analysis showed a significant increase in LV-ECV among rTOF patients compared to control subjects (MD = 2.63, 95%CI: 1.35 to 3.90, p < 0.0001, I2 = 86%, p for heterogeneity < 0.00001). Moreover, RV-ECV was found to be substantially higher in patients compared to LV-ECV. Our meta-analysis also revealed a significant association between RV-ECV and adverse events (HR = 1.15, 95% CI: 1.04 to 1.27, p = 0.005, I2 = 0%, p for heterogeneity = 0.62), while LV-ECV did not show any significant association with adverse events (HR = 1.12, 95% CI: 0.92 to 1.36, p = 0.16, I2 = 0%, p for heterogeneity = 0.46). CONCLUSION: The results of this meta-analysis on RV-ECV confirmed the presence of RV fibrosis as one of the prognostic factors in rTOF patients.
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Tetralogia de Fallot , Disfunção Ventricular Direita , Humanos , Tetralogia de Fallot/cirurgia , Imagem Cinética por Ressonância Magnética/métodos , Volume Sistólico , Imageamento por Ressonância Magnética , Fibrose , Função Ventricular DireitaRESUMO
OBJECTIVES: While significant evidence supports the benefits of normothermic cardiopulmonary bypass (NCPB) over hypothermic techniques, many institutions in developing countries, including ours, continue to employ hypothermic methods. This study aimed to assess the early postoperative outcomes of normothermic cardiopulmonary bypass (NCPB) for complete surgical repair via the Tetralogy of Fallot (TOF) within our national context. METHODS: We conducted this study in the Pediatric Cardiac Intensive Care Unit (PCICU) at the University Children's Hospital. One hundred patients who underwent complete TOF repair were enrolled and categorized into two groups: the normothermic group (n = 50, temperature 35-37 °C) and the moderate hypothermic group (n = 50, temperature 28-32 °C). We evaluated mortality, morbidity, and postoperative complications in the PCICU as outcome measures. RESULTS: The demographic characteristics were similar between the two groups. However, the cardiopulmonary bypass (CPB) time and aortic cross-clamp (ACC) time were notably longer in the hypothermic group. The study recorded seven deaths, yielding an overall mortality rate of 7%. No significant differences were observed between the two groups concerning mortality, morbidity, or postoperative complications in the PCICU. CONCLUSIONS: Our findings suggest that normothermic procedures, while not demonstrably effective, are safe for pediatric cardiac surgery. Further research is warranted to substantiate and endorse the adoption of this technique.
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Ponte Cardiopulmonar , Países em Desenvolvimento , Complicações Pós-Operatórias , Tetralogia de Fallot , Humanos , Tetralogia de Fallot/cirurgia , Masculino , Feminino , Lactente , Complicações Pós-Operatórias/epidemiologia , Pré-Escolar , Hipotermia Induzida , Resultado do Tratamento , Criança , Estudos Retrospectivos , Procedimentos Cirúrgicos Cardíacos/métodos , Unidades de Terapia Intensiva PediátricaRESUMO
Congenital diverticulum is an uncommonly detected cardiac lesion, especially in infancy. However, its association with cyanotic congenital heart disease is extremely rare. In the current work, we report a case diagnosed in the neonatal period with tetralogy of Fallot and pulmonary valve atresia associated with a large congenital diverticulum originating from the right ventricle.
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Divertículo , Ventrículos do Coração , Atresia Pulmonar , Tetralogia de Fallot , Humanos , Tetralogia de Fallot/complicações , Tetralogia de Fallot/diagnóstico por imagem , Atresia Pulmonar/complicações , Atresia Pulmonar/diagnóstico por imagem , Divertículo/complicações , Divertículo/diagnóstico por imagem , Ventrículos do Coração/diagnóstico por imagem , Ventrículos do Coração/anormalidades , Recém-Nascido , Ecocardiografia/métodos , Masculino , Anormalidades Múltiplas , Feminino , Diagnóstico DiferencialRESUMO
BACKGROUND: Mixed pulmonary disease with pulmonary regurgitation (PR) and stenosis (PS) in repaired tetralogy of Fallot (rTOF) can negatively impact ventricular health. Myocardial strain has been shown to be more sensitive at detecting occult ventricular dysfunction compared to right ventricular ejection fraction (RV EF). We hypothesize that rTOF patients with predominant PS will have lower RV global longitudinal strain (RV GLS) prior to and post-transcatheter pulmonary valve replacement (TPVR). METHODS: A retrospective cohort of rTOF patients who underwent cardiac magnetic resonance (CMR) and cardiac catheterization for right ventricular pressure (RVSP) measurement were analyzed at three time points: before valve implantation, at discharge and within 18 months post-TPVR. Patients were dichotomized into three groups based on RVSP: 0%-49%, 50%-74%, and >75%. RV GLS and left ventricular (LV) GLS by speckle tracking echocardiography (STE) were obtained from the apical 4-chamber using TomTec software (TOMTEC IS, Germany). RESULTS: Forty-eight patients were included. Every 14.3% increase in preimplantation RVSP above 28% was associated with an absolute magnitude 1% lower RV GLS (p = .001). Preimplantation RVSP when 75% or higher had 3.36% worse RV GLS than the lowest bin (p = .014). Overall, average RV strain magnitude was higher when preimplantation RVSP was less than 50% and had greater improvement over the three time points. Higher post implantation RVSP correlated with lower strain magnitude. CONCLUSION: Patients with significant PS (>50%) may benefit from earlier PVR and not depend solely on RV size and EF. Myocardial strain may be a more sensitive marker of function; however, larger, prospective studies are needed.
Assuntos
Insuficiência da Valva Pulmonar , Estenose da Valva Pulmonar , Valva Pulmonar , Tetralogia de Fallot , Disfunção Ventricular Direita , Humanos , Tetralogia de Fallot/complicações , Tetralogia de Fallot/cirurgia , Deformação Longitudinal Global , Valva Pulmonar/diagnóstico por imagem , Valva Pulmonar/cirurgia , Volume Sistólico , Estudos Retrospectivos , Função Ventricular Direita , Estenose da Valva Pulmonar/complicações , Estenose da Valva Pulmonar/cirurgia , Insuficiência da Valva Pulmonar/complicações , Insuficiência da Valva Pulmonar/diagnóstico por imagem , Insuficiência da Valva Pulmonar/cirurgia , Disfunção Ventricular Direita/complicações , Disfunção Ventricular Direita/diagnóstico por imagemRESUMO
Evaluation of the fetal heart involves two approaches. The first describes a screening protocol in which the heart is imaged in transverse planes that includes the four-chamber view (4CV), left and right outflow tracts, and the 3-vessel-tracheal view. The second approach is a fetal echocardiogram that requires additional cardiac images as well as evaluating ventricular function using diagnostic tools such as M-mode and pulsed Doppler ultrasound. Speckle tracking analysis of the ventricular and atrial endocardium of the fetal heart has focused primarily on computing longitudinal global strain. However, the technology enabling this measurement to occur has recently been adapted to enable the clinician to obtain numerous additional measurements of the size, shape, and contractility of the ventricles and atrial chambers. By using the increased number of measurements derived from speckle tracking analysis, we have reported the ability to screen for tetralogy of Fallot, D-transposition of the great arteries (D-TGA), and coarctation of the aorta by only imaging the 4CV. In addition, we have found that measurements derived from speckle tracking analysis of the ventricular and atrial chambers can be used to compute the risk for emergent neonatal balloon atrial septostomy in fetuses with D-TGA. The purpose of this review is to consolidate our experience in one source to provide perspective on the benefits of speckle tracking analysis to measure the size, shape, and contractility of the ventricles and atria imaged in the 4CV in fetuses with congenital heart defects.
Assuntos
Coração Fetal , Cardiopatias Congênitas , Contração Miocárdica , Ultrassonografia Pré-Natal , Humanos , Cardiopatias Congênitas/fisiopatologia , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/embriologia , Ultrassonografia Pré-Natal/métodos , Coração Fetal/diagnóstico por imagem , Coração Fetal/fisiopatologia , Contração Miocárdica/fisiologia , Ecocardiografia/métodos , Técnicas de Imagem por Elasticidade/métodos , Interpretação de Imagem Assistida por Computador/métodos , FemininoRESUMO
OBJECTIVES: This study was conducted to compare the efficacy of histidine-tryptophan-ketoglutarate (HTK) cardioplegia and cold blood cardioplegia (CBC), especially for postoperative right ventricular (RV) function after tetralogy of Fallot repair. DESIGN: Randomized, double-blinded, parallel-group, controlled clinical trial. SETTING: Ain Shams University Hospitals. PARTICIPANTS: A total of 100 children (1 to 5 years old) scheduled for tetralogy of Fallot repair. INTERVENTIONS: Patients were allocated (double-blinded 1:1 allocation ratio) to either the HTK group that received HTK cardioplegia (30 mL/kg via antegrade route) or the CBC group that received cold blood cardioplegia with blood-to-Ringer solution (4:1) in a volume of 20 mL/kg. MEASUREMENTS AND MAIN RESULTS: The HTK group showed a statistically significant reduction of the vasoactive inotropic score on admission to the pediatric intensive care unit (13.0 ± 4.1) in comparison to the CBC group (15.5 ± 5.4), with a p value of 0.011. However, after 24 hours, the vasoactive-inotropic score was comparable. Lactate level during the first 24 hours was 6.2 ± 0.7 mmol/L in the HTK group and 6.9 ± 0.4 mmol/L in the CBC group (p < 0.0001). Serial troponin measurements were lower in the HTK group (1.49 ± 0.45) compared to the CBC group (1.69 ± 0.18) at the first 72 hours postoperatively (p = 0.005). Postoperative echocardiographic assessment of RV function by means of tricuspid annular plane systolic excursion and myocardial performance index were better in the HTK group than in the CBC grpup (p < 0.05). CONCLUSIONS: HTK cardioplegia may offer better cardiac protection to pediatric patients undergoing tetralogy of Fallot repair than our institutional standard CBC with better recovery for the hypertrophic RV.
RESUMO
OBJECTIVES: Efficacy of dexmedetomidine (DEX) as a cardioprotective agent in Indonesian children undergoing classic tetralogy of Fallot (TOF) repair with cardiopulmonary bypass (CPB). DESIGN: A prospective, parallel trial using block randomization along with double-blinded preparation of treatment agents by other parties. SETTING: National Cardiovascular Center Harapan Kita, Indonesia. PARTICIPANTS: Sixty-six children with classic TOF scheduled for corrective surgery. No children were excluded. All patients had fulfilled the criteria for analysis. INTERVENTIONS: A total of 0.5 µg/kg bolus of DEX was added to the CPB priming solution, followed by 0.25 µg/kg/h maintenance during bypass. The placebo group used normal saline. Follow-ups were up to 30 days. MEASUREMENTS AND MAIN RESULTS: Troponin I was lower in the DEX group at 6 hours (30.48 ± 19.33 v 42.73 ± 27.16, p = 0.039) and 24 hours after CPB (8.89 ± 5.42 v 14.04 ± 11.17, p = 0.02). Within a similar timeframe, DEX successfully lowered interleukin-6 (p = 0.03; p = 0.035, respectively). Lactate was lower in the Dex group at 1, 6, and 24 hours after CPB (p < 0.01; p = 0.048; p = 0.035; respectively). Dexmedetomidine increased cardiac output and index from 6 hours after bypass, but vice versa in systemic vascular resistance. Reduction of vasoactive inotropic score was seen during intensive care unit monitoring in the Dex group (p = 0.049). Nevertheless, DEX did not significantly affect the length of ventilation (p = 0.313), intensive care unit stay (p = 0.087), and mortality (p > 0.99). CONCLUSIONS: Dexmedetomidine during CPB is an effective cardioprotective agent in TOF children having surgery. Postoperative mortality was comparable across groups.