RESUMO
Tetralogy of Fallot (TOF) is a complex congenital heart defect that poses unique challenges for both mothers and their unborn children. This comprehensive review, aims to provide a holistic exploration of the maternal-fetal dynamics, treatment strategies, and psychological dimensions involved in navigating the path of TOF during pregnancy. It delves into the physiological changes that occur during pregnancy in TOF patients, including pulmonary regurgitation, right ventricular hypertrophy, and the overriding aorta. By understanding these dynamics, healthcare professionals can tailor treatment strategies to optimize maternal and fetal outcomes. The review further investigates the treatment strategies employed in managing TOF during pregnancy, encompassing medical interventions, cardiac monitoring, and multidisciplinary care. It explores the role of advanced imaging techniques, such as echocardiography and cardiac magnetic resonance imaging, in assessing TOF severity and guiding treatment decisions. The psychological factors influencing maternal adaptation, coping strategies, and the long-term implications on the child's psychological development are also examined. The integration of multidisciplinary approaches, including cardiac care, psychosocial support, and mental health interventions, can orchestrate a harmonious symphony of maternal-fetal well-being in the challenging journey of TOF pregnancies. Future research endeavours should continue to explore these dimensions, further refining treatment strategies and enhancing the understanding of TOF pregnancies for improved outcomes.
Assuntos
Insuficiência da Valva Pulmonar , Tetralogia de Fallot , Gravidez , Feminino , Humanos , Tetralogia de Fallot/cirurgia , Imageamento por Ressonância MagnéticaRESUMO
PURPOSE: Without neonatal screening in low middle-income countries like Pakistan, Tetralogy of Fallot (TOF) is a congenital heart disease which frequently remains untreated beyond infancy. The purpose of this study is to determine and assess outcomes and health related quality of life (HRQOL) in patients who undergo complete repair of TOF as adults. METHODS: 56 patients who underwent complete TOF repair after 16 years of age were included. Patient data was collected via retrospective chart review, and a semi structured interview along with Short-Form 36 (SF-36) questionnaire were used to assess HRQOL. RESULTS: 66.1% of patients were male with the mean age at surgery of 22.3 ± 6.00. All patients had a post-operative NYHA Classification of I or II, 94.6% had an ejection fraction of ≥ 50% and 28.6% showed small residual lesions in follow-up echocardiograms. 32.1% of patients suffered post-operative morbidity. For the quantitative assessment using SF-36 scores, patients showed good scores of median 95 (65-100). A major cause of delay to treatment was lack of consensus between treatments offered by doctors in different parts of Pakistan. There was a pattern of 'inability to fit in' among patients who had had late TOF repair, despite self- reported improved HRQOL. CONCLUSION: Our results indicate that even with a delayed diagnosis, surgical repair of TOF produces good functional results. However, these patients face significant psychosocial issues. While early diagnosis remains the ultimate goal, patients undergoing late repair should be managed in more holistic manner with attention to psychological impact of the disease as well.
Assuntos
Tetralogia de Fallot , Recém-Nascido , Humanos , Masculino , Adulto , Feminino , Tetralogia de Fallot/cirurgia , Qualidade de Vida/psicologia , Estudos Retrospectivos , Países em Desenvolvimento , Inquéritos e Questionários , Resultado do TratamentoRESUMO
The presence of persistent left superior vena cava to the left atrium connection without an innominate vein may give rise to technical challenges during intracardiac repair. In this report, the end-to-side anastomosis technique of the persistent left superior vena cava to the right superior vena cava is discussed in a patient with tetralogy of Fallot associated with persistent left superior vena cava draining directly into the left atrium. A successful end-to-side anastomosis between the persistent left superior vena cava and the right superior vena cava was performed and short-term anastomosis patency was documented via angiography.
Assuntos
Veia Cava Superior Esquerda Persistente , Tetralogia de Fallot , Malformações Vasculares , Criança , Humanos , Veia Cava Superior/cirurgia , Tetralogia de Fallot/diagnóstico por imagem , Tetralogia de Fallot/cirurgia , Átrios do Coração/cirurgia , Anastomose CirúrgicaRESUMO
We present a case of a female American Indian neonate born via a provider unattended home delivery. Her mother received limited prenatal care and the infant was not examined by a healthcare provider until day of life 10 when she presented to the emergency department for evaluation of a skin rash. She was found to have severe hypernatremic dehydration. She was subsequently diagnosed with tetralogy of Fallot, and this was the likely cause of her breastfeeding failure dehydration. The infant underwent careful correction of her electrolyte abnormalities and surgical repair of her cardiac defect on day of life 27. This case highlights the importance of comprehensive care during the prenatal and postpartum/newborn periods, especially in rural locations where access to care can be difficult.
Assuntos
Tetralogia de Fallot , Criança , Desidratação/diagnóstico , Desidratação/etiologia , Desidratação/terapia , Feminino , Humanos , Lactente , Recém-Nascido , Mães , Assistência Perinatal , Gravidez , Tetralogia de Fallot/complicações , Tetralogia de Fallot/diagnóstico , Tetralogia de Fallot/cirurgiaRESUMO
The objective of this study was to evaluate the safety and efficacy of combining transcatheter pulmonary valve replacement (TPVR) and electrophysiology (EP) procedures. A retrospective review was undertaken to identify TPVR and EP procedures that were concomitantly performed in the cardiac catheterization laboratory at University of Iowa Stead Family Children's Hospital from January 2011 to October 2019. Procedural and follow-up data were compared between patients who underwent TPVR and EP procedures in the same setting to those who received TPVR or EP procedure separately and that were similar in age and cardiac anatomy. A total of 8 patients underwent combined TPVR and EP procedures. One patient was excluded due to lack of adequate control, leaving seven study subjects (57% female; median age at time of procedure 16 years). The median follow-up time was 11.5 months (range 2-36 months). Patients who received combined TPVR and EP had shorter recovery times (combined: median 18.9 h; IQR 18.35-19.5 vs separate: median 27.98 h; IQR 21.42-39.25; p-value 0.031), shorter hospital length of stay (combined: median 27.5 h; IQR 26.47-31.4 vs separate: median 38.4 h; IQR 33.42-51.50; p-value 0.016), and a 51% reduction in total hospital charges (combined: median $171,640; IQR 135.43-219.22 vs separate: median $333,560 IQR 263.20-400.98; p-value 0.016). There were no significant differences in radiation dose or procedure time between the combined and control groups. The median radiation time for those who had the combination procedure was 30.5 min [IQR 29.6-47.9], and the median dose area product was 215 mGy [IQR 158-935]. In conclusion, combining TPVR and EP procedures is feasible, safe, and economically advantageous.
Assuntos
Cateterismo Cardíaco/métodos , Técnicas Eletrofisiológicas Cardíacas/métodos , Cardiopatias/cirurgia , Implante de Prótese de Valva Cardíaca/métodos , Valva Pulmonar/cirurgia , Adolescente , Adulto , Procedimentos Cirúrgicos Cardíacos , Criança , Terapia Combinada , Técnicas Eletrofisiológicas Cardíacas/economia , Feminino , Próteses Valvulares Cardíacas , Implante de Prótese de Valva Cardíaca/economia , Custos Hospitalares , Humanos , Tempo de Internação , Masculino , Insuficiência da Valva Pulmonar/cirurgia , Estudos Retrospectivos , Tetralogia de Fallot/cirurgia , Resultado do Tratamento , Adulto JovemRESUMO
BACKGROUND: Adults with repaired tetralogy of Fallot die prematurely from ventricular tachycardia (VT) and sudden cardiac death. Inducible VT predicts mortality. Ventricular scar, the key substrate for VT, can be noninvasively defined with late gadolinium enhancement (LGE) cardiovascular magnetic resonance but whether this relates to inducible VT is unknown. METHODS: Sixty-nine consecutive repaired tetralogy of Fallot patients (43 male, mean 40±15 years) clinically scheduled for invasive programmed VT-stimulation were prospectively recruited for prior 3-dimensional LGE cardiovascular magnetic resonance. Ventricular LGE was segmented and merged with reconstructed cardiac chambers and LGE volume measured. RESULTS: VT was induced in 22 (31%) patients. Univariable predictors of inducible VT included increased RV LGE (odds ratio [OR], 1.15; P=0.001 per cm3), increased nonapical vent LV LGE (OR, 1.09; P=0.008 per cm3), older age (OR, 1.6; P=0.01 per decile), QRS duration ≥180 ms (OR, 3.5; P=0.02), history of nonsustained VT (OR, 3.5; P=0.02), and previous clinical sustained VT (OR, 12.8; P=0.003); only prior sustained VT (OR, 8.02; P=0.02) remained independent in bivariable analyses after controlling for RV LGE volume (OR, 1.14; P=0.003). An RV LGE volume of 25 cm3 had 72% sensitivity and 81% specificity for predicting inducible VT (area under the curve, 0.81; P<0.001). At the extreme cutoffs for ruling-out and ruling-in inducible VT, RV LGE >10 cm3 was 100% sensitive and >36 cm3 was 100% specific for predicting inducible VT. CONCLUSIONS: Three-dimensional LGE cardiovascular magnetic resonance-defined scar burden is independently associated with inducible VT and may help refine patient selection for programmed VT-stimulation when applied to an at least intermediate clinical risk cohort.
Assuntos
Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Meios de Contraste , Gadolínio , Ventrículos do Coração/diagnóstico por imagem , Imageamento Tridimensional , Imageamento por Ressonância Magnética , Taquicardia Ventricular/diagnóstico por imagem , Tetralogia de Fallot/cirurgia , Adulto , Técnicas Eletrofisiológicas Cardíacas , Feminino , Ventrículos do Coração/patologia , Ventrículos do Coração/fisiopatologia , Humanos , Masculino , Pessoa de Meia-Idade , Miocárdio/patologia , Valor Preditivo dos Testes , Estudos Prospectivos , Fatores de Risco , Taquicardia Ventricular/etiologia , Taquicardia Ventricular/fisiopatologia , Resultado do TratamentoAssuntos
Potenciais de Ação , Bloqueio de Ramo/etiologia , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Frequência Cardíaca , Ventrículos do Coração/fisiopatologia , Tetralogia de Fallot/cirurgia , Função Ventricular Direita , Adulto , Bloqueio de Ramo/diagnóstico , Bloqueio de Ramo/fisiopatologia , Eletrocardiografia , Técnicas Eletrofisiológicas Cardíacas , Feminino , Ventrículos do Coração/diagnóstico por imagem , Humanos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Fatores de Risco , Tetralogia de Fallot/diagnóstico , Tetralogia de Fallot/fisiopatologia , Resultado do Tratamento , Adulto JovemAssuntos
Ablação por Cateter , Átrios do Coração/cirurgia , Taquicardia Ventricular/cirurgia , Tetralogia de Fallot/cirurgia , Potenciais de Ação , Idoso , Técnicas Eletrofisiológicas Cardíacas , Átrios do Coração/fisiopatologia , Frequência Cardíaca , Humanos , Masculino , Taquicardia Ventricular/diagnóstico , Taquicardia Ventricular/fisiopatologia , Fatores de Tempo , Resultado do TratamentoRESUMO
BACKGROUND: Tachyarrhythmias and bradyarrhythmias affect 20%-50% of adult patients with tetralogy of Fallot (TOF) and some of these patients will require cardiac implantable electronic devices (CIED) such as pacemaker and/or internal cardioverter defibrillator. METHODS: The Mayo Adult Congenital Heart Disease database was queried for patients with repaired TOF and history of CIED implantation, 1990-2017. The study objectives were: (1) determine the occurrence of device-related complications defined as lead failure, lead recall, device infection and lead thrombus; and (2) determine the occurrence and risk factors for defibrillator shock. RESULTS: There were 99 patients (age 46±14 years and 66 (66%) men) with CIED, and the CIEDs were 41 (41%) pacemakers and 73 (73%) defibrillators. Indication for defibrillator implantation was for primary prevention in 28 (38%) and secondary prevention in 45 (62%). Device-related complications occurred in 20 (20%) patients (lead failure 17, lead recall 4, device infection 12 and thrombus 3). Twenty-five per cent of all device infections occurred within 30 days from the time of device generator change. Annualised rates of appropriate and inappropriate shocks were 5.7% and 6.2%, respectively. The use of class III antiarrhythmic drug was protective against defibrillator shock. CONCLUSION: The current study provides useful outcome data to aid patient counselling and clinical decision-making. Further studies are required to explore ways to decrease the risk of postprocedural infection after device generator change, and to determine which patients will benefit from empirical antiarrhythmic therapy as a strategy to decrease incidence of inappropriate defibrillator shock.
Assuntos
Arritmias Cardíacas , Desfibriladores Implantáveis/efeitos adversos , Remoção de Dispositivo/efeitos adversos , Terapia por Estimulação Elétrica , Marca-Passo Artificial/efeitos adversos , Infecções Relacionadas à Prótese , Tetralogia de Fallot/complicações , Adulto , Arritmias Cardíacas/epidemiologia , Arritmias Cardíacas/etiologia , Arritmias Cardíacas/prevenção & controle , Arritmias Cardíacas/terapia , Desfibriladores Implantáveis/normas , Remoção de Dispositivo/métodos , Terapia por Estimulação Elétrica/efeitos adversos , Terapia por Estimulação Elétrica/instrumentação , Terapia por Estimulação Elétrica/métodos , Eletrocardiografia/métodos , Análise de Falha de Equipamento , Feminino , Humanos , Incidência , Masculino , Pessoa de Meia-Idade , Avaliação das Necessidades , Avaliação de Processos e Resultados em Cuidados de Saúde , Infecções Relacionadas à Prótese/epidemiologia , Infecções Relacionadas à Prótese/prevenção & controle , Estudos Retrospectivos , Tetralogia de Fallot/epidemiologia , Estados Unidos/epidemiologiaRESUMO
OBJECTIVES: This study sought to evaluate the influence of slow conducting anatomic isthmuses (SCAI) as dominant ventricular tachycardia (VT) substrate on QRS duration. BACKGROUND: QRS prolongation has been associated with VT in repaired tetralogy of Fallot. METHODS: Seventy-eight repaired tetralogy of Fallot patients (age 37 ± 15 years, 52 male, QRS duration 153 ± 29 ms, 67 right bundle branch blocks [RBBB]) underwent programmed stimulation and electroanatomic activation mapping during sinus rhythm. Right ventricular (RV) surface, RV activation pattern, RV activation time, conduction velocity at AI, and remote RV sites were determined. RESULTS: Twenty-four patients were inducible for VT (VT+); SCAI was present in 22 of 24 VT+ but only in 2 of 54 patients without inducible VT (VT-). Conduction velocity through AI was slower in VT+ patients (median of 0.3 [0.3 to 0.4] vs. 0.7 [0.6 to 0.9] m/s; p < 0.01) but conduction velocity in the remote RV did not differ between groups. In non-RBBB, QRS duration was similar in VT+ patients (n = 6) and VT- patients (n = 5), but RV activation within SCAI exceeded QRS offset in VT+ patients (37 ± 20 ms vs. -5 ± 9 ms, p < 0.01). In RBBB, both QRS duration and RV activation time were longer in VT+ patients (n = 18, 17 of 18 QRS > 150 ms) compared with VT- patients (n = 49, 27 of 49 QRS > 150 ms) (173 ± 22 ms vs. 156 ± 20 ms; p < 0.01; 141 ± 22 ms vs. 129 ± 21 ms; p = 0.04). In VT+ patients, QRS prolongation >150 ms (n = 17) was due to SCAI or blocked isthmus in 15 patients (88%) and 1 (6%). In contrast, in VT- patients, QRS prolongation >150 ms (n = 27) was due to enlarged RV or blocked isthmus in 10 patients (37%) and 8 (30%), but due to SCAI in only 1 (4%). After exclusion of a severely enlarged RV, a QRS duration >150 ms was highly predictive for SCAI/blocked AI (OR: 17; 95% CI: 3.3 to 84; p < 0.01). CONCLUSIONS: A narrow QRS interval does not exclude VT-related SCAI. In the presence of RBBB, SCAI further prolongs QRS duration. QRS duration >150 ms is highly suspicious for SCAI or isthmus block distinguishable by electroanatomic mapping.
Assuntos
Eletrocardiografia , Técnicas Eletrofisiológicas Cardíacas , Taquicardia Ventricular , Tetralogia de Fallot , Adulto , Estudos de Coortes , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Taquicardia Ventricular/epidemiologia , Taquicardia Ventricular/etiologia , Taquicardia Ventricular/fisiopatologia , Tetralogia de Fallot/complicações , Tetralogia de Fallot/epidemiologia , Tetralogia de Fallot/fisiopatologia , Adulto JovemAssuntos
Potenciais de Ação , Cateterismo Cardíaco/instrumentação , Cateteres Cardíacos , Técnicas Eletrofisiológicas Cardíacas/instrumentação , Sistema de Condução Cardíaco/fisiopatologia , Frequência Cardíaca , Taquicardia Ventricular/diagnóstico , Tetralogia de Fallot/cirurgia , Adulto , Estimulação Cardíaca Artificial , Humanos , Masculino , Valor Preditivo dos Testes , Taquicardia Ventricular/etiologia , Taquicardia Ventricular/fisiopatologia , Tetralogia de Fallot/complicações , Tetralogia de Fallot/diagnóstico , Tetralogia de Fallot/fisiopatologiaRESUMO
BACKGROUND: Electrical dyssynchrony and prolonged QRS duration are common in patients with repaired tetralogy of Fallot (ToF). It has been linked to increased risk of sudden cardiac death and right ventricular (RV) dysfunction. We investigated myocardial dyssynchrony using cardiac magnetic resonance imaging (CMR) and feature tracking analysis (FT) in this setting and compared it to myocardial deformation, conventional parameters of ventricular dysfunction and clinical parameters. METHODS AND RESULTS: Patients underwent standardized CMR investigations as part of a nationwide study. We prospectively assessed myocardial deformation and analysed regional wall motion abnormalities of the RV and the left ventricle (LV) using CMR-FT. The main measure of dyssynchrony was the maximal time difference (wall motion delay) of the regional strain as a parameter of mechanical biventricular dyssynchrony. In addition, clinical parameters and measures of cardiopulmonary exercise capacity were available. Overall 345 patients were included. Parameters of biventricular wall motion delay correlated significantly with global FT-strain parameters (pâ¯<â¯0.0001 for all imaging planes assessed). Furthermore, we found a significant correlation between circumferential RV motion delay and QRS duration (pâ¯=â¯0.006). Higher LV and RV wall motion delay parameters were also associated with lower peak oxygen consumption (pâ¯<â¯0.05) and a worse LV and RV ejection fraction (pâ¯<â¯0.02). CONCLUSIONS: Assessment of mechanical dyssynchrony is feasible using CMR-FT in ToF patients. Parameters of mechanical dyssynchrony correlate with electrical dyssynchrony, biventricular function and objective exercise capacity in this setting. Due to the weak degree of correlation, however, the clinical significance of these findings remains to be clarified by further studies.
Assuntos
Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Tolerância ao Exercício , Ventrículos do Coração , Imagem Cinética por Ressonância Magnética/métodos , Miocárdio/patologia , Complicações Pós-Operatórias , Tetralogia de Fallot/cirurgia , Disfunção Ventricular , Adolescente , Adulto , Procedimentos Cirúrgicos Cardíacos/métodos , Criança , Correlação de Dados , Técnicas Eletrofisiológicas Cardíacas/métodos , Feminino , Alemanha/epidemiologia , Testes de Função Cardíaca/métodos , Ventrículos do Coração/diagnóstico por imagem , Ventrículos do Coração/patologia , Ventrículos do Coração/fisiopatologia , Humanos , Masculino , Complicações Pós-Operatórias/diagnóstico , Complicações Pós-Operatórias/fisiopatologia , Estudos Prospectivos , Disfunção Ventricular/etiologia , Disfunção Ventricular/patologia , Disfunção Ventricular/fisiopatologiaRESUMO
BACKGROUND: Ventricular tachyarrhythmias are the most common cause of death in patients with repaired tetralogy of Fallot (TOF), but predicting those at risk remains a challenge. An electrophysiology study (EPS) has been proposed to risk stratify patients with TOF. OBJECTIVE: We sought to evaluate a perioperative EPS-guided approach to risk stratify patients with TOF undergoing pulmonary valve replacement (PVR) and guide concomitant cryoablation. METHODS: A prospective cohort study of patients with TOF undergoing an EPS at the time of PVR from 2006 to 2017 was conducted at 2 centers. Patients inducible at the time of pre-PVR had undergone concomitant cryoablation in addition to PVR. A repeat post-PVR EPS was performed in those initially inducible to guide implantable cardioverter-defibrillator (ICD) implantation. RESULTS: Of 70 patients who underwent a pre-PVR EPS, 34 (49%) had inducible sustained ventricular tachycardia (VT): 25 monomorphic VT and 9 polymorphic VT. Among patients undergoing cryoablation, 14 (45%) had inducible VT and underwent ICD implantation. During a mean follow-up period of 6.1 ± 3.2 years, 3 patients (21%) had appropriate ICD shocks for symptomatic VT. There was an average of 2.3 shocks (range 1-4 shocks), and the mean time to first shock post-device implantation was 3.6 years (range 2.9-4.3 years). Among patients with negative pre- or post-PVR EPS results, 2 had VT requiring radiofrequency ablation and/or subsequent ICD implantation. There were no arrhythmic deaths. CONCLUSION: A pre-PVR EPS identified patients with higher-risk TOF undergoing PVR. Despite empirical VT cryoablation at the time of PVR, a high percentage of patients remained inducible for VT. In this high-risk cohort, post-PVR EPS evaluation is important to identify patients at risk of VT despite cryoablation.
Assuntos
Procedimentos Cirúrgicos Cardíacos/métodos , Criocirurgia/métodos , Técnicas Eletrofisiológicas Cardíacas/métodos , Cuidados Pré-Operatórios/métodos , Valva Pulmonar/cirurgia , Taquicardia Ventricular/diagnóstico , Tetralogia de Fallot/cirurgia , Adulto , Feminino , Seguimentos , Humanos , Masculino , Estudos Prospectivos , Valva Pulmonar/diagnóstico por imagem , Taquicardia Ventricular/etiologia , Taquicardia Ventricular/fisiopatologia , Tetralogia de Fallot/complicações , Tetralogia de Fallot/diagnóstico , Resultado do Tratamento , Adulto JovemRESUMO
INTRODUCTION: ToF patients are at risk for ventricular deterioration at a relatively young age, which can be aggravated by AF development. Therefore, knowledge on AF development and its timespan of progression is essential to guide treatment strategies for AF. OBJECTIVE: We examined late postoperative AF onset and progression in ToF patients during long-term follow-up after ToF correction. In addition, coexistence of AF with regular supraventricular tachyarrhythmias (SVT) and ventricular tachyarrhythmias (VTA) was analyzed. METHODS AND RESULTS: ToF patients (N = 29) with AF after ToF correction referred to the electrophysiology department between 2000 and 2015 were included. All available rhythm registrations were reviewed for AF, regular SVT, and VTA. AF progression was defined as transition from paroxysmal AF to (longstanding) persistent/permanent AF or from (longstanding) persistent AF to permanent AF. At the age of 44 ± 12 years, ToF patients presented with paroxysmal (N = 14, 48%), persistent (N = 13, 45%) or permanent AF (N = 2, 7%). Age of AF development was similar among patients who either underwent initial shunt creation (N = 15, 45 ± 11 [25-57] years) or primary total ToF correction (N = 14, 43 ± 13 [26-66] years) (P = 0.785). AF coexisted with regular SVT (N = 18, 62%) and VTA (N = 13, 45%). Progression of AF occurred in 11 patients (38%) within 5 ± 5 years after AF onset despite antiarrhythmic drug class II (AAD, P = 0.052) or III (P = 0.587) usage. CONCLUSIONS: AF in our ToF population developed at a young age and showed rapid progression. Rhythm control by pharmacological therapy was ineffective in preventing AF progression.
Assuntos
Fibrilação Atrial/etiologia , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Frequência Cardíaca , Tetralogia de Fallot/cirurgia , Potenciais de Ação , Adulto , Idade de Início , Idoso , Antiarrítmicos/uso terapêutico , Fibrilação Atrial/tratamento farmacológico , Fibrilação Atrial/mortalidade , Fibrilação Atrial/fisiopatologia , Ablação por Cateter , Progressão da Doença , Técnicas Eletrofisiológicas Cardíacas , Feminino , Frequência Cardíaca/efeitos dos fármacos , Humanos , Estudos Longitudinais , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Fatores de Risco , Taquicardia Supraventricular/etiologia , Taquicardia Supraventricular/fisiopatologia , Taquicardia Supraventricular/cirurgia , Taquicardia Ventricular/etiologia , Taquicardia Ventricular/fisiopatologia , Taquicardia Ventricular/cirurgia , Tetralogia de Fallot/complicações , Tetralogia de Fallot/mortalidade , Tetralogia de Fallot/fisiopatologia , Fatores de Tempo , Resultado do Tratamento , Flutter Ventricular/etiologia , Flutter Ventricular/fisiopatologia , Flutter Ventricular/cirurgiaAssuntos
Procedimento de Blalock-Taussig , Ablação por Cateter , Modelos Anatômicos , Modelos Cardiovasculares , Modelagem Computacional Específica para o Paciente , Impressão Tridimensional , Taquicardia Ventricular/cirurgia , Tetralogia de Fallot/cirurgia , Técnicas Eletrofisiológicas Cardíacas , Feminino , Humanos , Pessoa de Meia-Idade , Tomografia Computadorizada Multidetectores , Taquicardia Ventricular/diagnóstico por imagem , Taquicardia Ventricular/fisiopatologia , Resultado do TratamentoAssuntos
Procedimentos Cirúrgicos Cardíacos , Sistema de Condução Cardíaco/fisiopatologia , Frequência Cardíaca , Taquicardia Ventricular/fisiopatologia , Tetralogia de Fallot/cirurgia , Técnicas de Ablação , Potenciais de Ação , Estimulação Cardíaca Artificial , Cardioversão Elétrica , Eletrocardiografia , Técnicas Eletrofisiológicas Cardíacas , Sistema de Condução Cardíaco/cirurgia , Hemodinâmica , Humanos , Masculino , Taquicardia Ventricular/diagnóstico , Taquicardia Ventricular/etiologia , Taquicardia Ventricular/terapia , Tetralogia de Fallot/complicações , Tetralogia de Fallot/fisiopatologia , Adulto JovemRESUMO
BACKGROUND: Electromechanical discoordination may contribute to long-term pulmonary right ventricular (RV) dysfunction in patients after surgery for congenital heart disease. We sought to evaluate changes in RV function after temporary RV cardiac resynchronization therapy. METHODS AND RESULTS: Twenty-five patients aged median 12.0 years after repair of tetralogy of Fallot and similar lesions were studied echocardiographically (n=23) and by cardiac catheterization (n=5) after primary repair (n=4) or after surgical RV revalvulation for significant pulmonary regurgitation (n=21). Temporary RV cardiac resynchronization therapy was applied in the presence of complete right bundle branch block by atrial-synchronized RV free wall pacing in complete fusion with spontaneous ventricular depolarization using temporary electrodes. The q-RV interval at the RV free wall pacing site (mean 77.2% of baseline QRS duration) confirmed pacing from a late activated RV area. RV cardiac resynchronization therapy carried significant decrease in QRS duration (P<0.001) along with elimination of the right bundle branch block QRS morphology, increase in RV filling time (P=0.002), pulmonary artery velocity time integral (P=0.006), and RV maximum +dP/dt (P<0.001), and decrease in RV index of myocardial performance (P=0.006). RV mechanical synchrony improved: septal-to-lateral RV mechanical delay decreased (P<0.001) and signs of RV dyssynchrony pattern were significantly abolished. RV systolic stretch fraction reflecting the ratio of myocardial stretching and contraction during systole diminished (P=0.001). CONCLUSIONS: In patients with congenital heart disease and right bundle branch block, RV cardiac resynchronization therapy carried multiple positive effects on RV mechanics, synchrony, and contraction efficiency.
Assuntos
Bloqueio de Ramo/terapia , Terapia de Ressincronização Cardíaca , Procedimentos Cirúrgicos Cardíacos , Contração Miocárdica , Tetralogia de Fallot/cirurgia , Disfunção Ventricular Direita/etiologia , Função Ventricular Direita , Adolescente , Fenômenos Biomecânicos , Bloqueio de Ramo/diagnóstico por imagem , Bloqueio de Ramo/etiologia , Bloqueio de Ramo/fisiopatologia , Cateterismo Cardíaco , Criança , Ecocardiografia Doppler de Pulso , Eletrocardiografia , Técnicas Eletrofisiológicas Cardíacas , Feminino , Hemodinâmica , Humanos , Masculino , Recuperação de Função Fisiológica , Tetralogia de Fallot/complicações , Tetralogia de Fallot/diagnóstico por imagem , Tetralogia de Fallot/fisiopatologia , Fatores de Tempo , Resultado do Tratamento , Disfunção Ventricular Direita/diagnóstico por imagem , Disfunção Ventricular Direita/fisiopatologiaRESUMO
BACKGROUND: Patients with tetralogy of Fallot (TOF) have increased risk of atrial arrhythmias. HYPOTHESIS: A measure of atrial dispersion, the P-wave vector magnitude (Pvm), can identify patients at risk for perioperative atrial flutter (AFL) or intra-atrial re-entrant tachycardia (IART) in a large TOF cohort. METHODS: We performed a blinded, retrospective analysis of 158 TOF patients undergoing pulmonary valve replacement between 1997 and 2015. History of AFL/IART was documented using electrocardiogram, Holter monitor, exercise stress test, implanted cardiac device, and electrophysiology study. P-R intervals, Pvm, QRS duration, and QRS vector magnitude were assessed from resting sinus-rhythm 12-lead electrocardiograms and identification of those with AFL/IART was determined. RESULTS: Fourteen patients (8.9%) were found to have AFL/IART. Pvm, QRS duration, and QRS vector magnitude significantly differentiated those with AFL/IART from those without on univariate analysis: 0.09 ± 0.04 vs 0.18 ± 0.07 mV, 161.3 ± 21.9 vs 137.7 ± 31.4 ms, and 1.2 (interquartile range, 1.0-1.2) vs 1.6 mV (1.0-2.3), respectively (P < 0.05 for each). The Pvm had the highest area under the ROC curve (0.88) and was the only significant predictor on multivariate analysis, with odds ratio of 0.02 (95% confidence interval: 0.01-0.53). P-R duration, MRI volumes, and right-heart hemodynamics did not significantly differentiate those with vs those without AFL/IART. CONCLUSIONS: In TOF patients undergoing pulmonary valve replacement, Pvm has significant value in predicting those with perioperative AFL/IART. These clinical features may help further evaluate TOF patients at risk for perioperative atrial arrhythmias. Prospective studies are warranted.
Assuntos
Flutter Atrial/etiologia , Implante de Prótese de Valva Cardíaca/efeitos adversos , Valva Pulmonar/cirurgia , Taquicardia Reciprocante/etiologia , Tetralogia de Fallot/terapia , Adolescente , Adulto , Flutter Atrial/diagnóstico , Flutter Atrial/fisiopatologia , Criança , Eletrocardiografia Ambulatorial , Técnicas Eletrofisiológicas Cardíacas , Teste de Esforço , Feminino , Humanos , Modelos Logísticos , Masculino , Análise Multivariada , Razão de Chances , Período Perioperatório , Valva Pulmonar/fisiopatologia , Estudos Retrospectivos , Fatores de Risco , Taquicardia Reciprocante/diagnóstico , Taquicardia Reciprocante/fisiopatologia , Tetralogia de Fallot/complicações , Tetralogia de Fallot/diagnóstico , Tetralogia de Fallot/fisiopatologia , Fatores de Tempo , Resultado do Tratamento , Adulto JovemRESUMO
BACKGROUND: Monomorphic ventricular tachycardia (MVT) is common in adults with repaired tetralogy of Fallot (TOF), and is associated with sudden cardiac death. Management of MVT is not defined, and results of catheter ablation (CA) are limited. AIMS: To evaluate long-term outcomes of MVT CA in repaired TOF. METHODS: Thirty-four patients (mean age 32±10.3 years; 59% male) with repaired TOF underwent CA for symptomatic MVT between 1990 and 2012 in our centre; direct-current ablation (DCA) was used in 6%, radiofrequency followed by DCA in 29% and radiofrequency alone in 65%. RESULTS: Right ventricular (RV) dysfunction was present in 35% and left ventricular (LV) dysfunction in 21%. Mean numbers of clinical and induced MVTs were 1 and 2, respectively. Mean VT rate was 225±95bpm. Ablation targeted a single site (range 1-2), which was RV outflow tract in 85%. Primary success, defined as ventricular tachycardia (VT) termination during CA and final non-inducibility, was obtained in 82%. Seven patients (21%) required redo ablation in the first 3 months (before 2004; DCA). No death related to CA occurred. Mean follow-up time was 9.5±5.2 years. Antiarrhythmic therapy was discontinued in 71%. There were two cases of sudden cardiac death and four VT recurrences. Freedom from death and arrhythmia recurrence was 94% at 5 years, 81% at 10 years and 70% at 20 years. Global survival was 91% at 20 years. Baseline LV ejection fraction<60% was significantly associated with ventricular arrhythmia recurrence (hazard ratio 16.4, 95% confidence interval 1.8-147; P=0.01). CONCLUSIONS: CA can safely address macroreentrant MVT in repaired TOF patients with an acceptable long-term rate of recurrence in this high-risk population. Anatomical classification of isthmuses with electroanatomical mapping provides reproducible endpoints for CA. Attention should be given to LV systolic function in risk assessment and selection of candidates for implantable cardioverter-defibrillator.