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BACKGROUND: Ventricular arrhythmias and sudden death are recognized complications in tetralogy of Fallot. Electrophysiological studies (EPS) before pulmonary valve replacement (PVR), the most common reintervention in tetralogy of Fallot, could potentially inform therapy to improve arrhythmic outcomes. METHODS: A prospective multicenter study was conducted to systematically assess EPS with programmed ventricular stimulation in patients with tetralogy of Fallot referred for PVR from January 2020 to December 2021. A standardized stimulation protocol was used across all centers. RESULTS: A total of 120 patients were enrolled, mean age 39.2±14.5 years, 53.3% males. Sustained ventricular tachycardia was induced in 27 (22.5%) patients. When identifiable, the critical isthmus most commonly implicated (ie, in 90.0%) was between the ventricular septal defect patch and pulmonary annulus. Factors independently associated with inducible ventricular tachycardia were history of atrial arrhythmia (odds ratio, 8.56 [95% CI, 2.43-34.73]) and pulmonary annulus diameter >26 mm (odds ratio, 5.05 [95% CI, 1.47-21.69]). The EPS led to a substantial change in management in 23 (19.2%) cases: 18 (15.0%) had catheter ablation, 3 (2.5%) surgical cryoablation during PVR, and 9 (7.5%) defibrillator implantation. Repeat EPS 5.1 (4.8-6.2) months after PVR was negative in 8 of 9 (88.9%) patients. No patient experienced a sustained ventricular arrhythmia during 13 (6.1-20.1) months of follow-up. CONCLUSIONS: Systematically performing programmed ventricular stimulation in patients with tetralogy of Fallot referred for PVR yields a high rate of inducible ventricular tachycardia and carries the potential to alter management. It remains to be determined whether a standardized treatment approach based on the results of EPS will translate into improved outcomes. REGISTRATION: URL: https://clinicaltrials.gov/ct2/show/NCT04205461; Unique identifier: NCT04205461.
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Implantación de Prótesis de Válvulas Cardíacas , Insuficiencia de la Válvula Pulmonar , Válvula Pulmonar , Taquicardia Ventricular , Tetralogía de Fallot , Masculino , Humanos , Adulto Joven , Adulto , Persona de Mediana Edad , Femenino , Válvula Pulmonar/cirugía , Tetralogía de Fallot/cirugía , Implantación de Prótesis de Válvulas Cardíacas/efectos adversos , Estudios Prospectivos , Resultado del Tratamiento , Taquicardia Ventricular/diagnóstico , Taquicardia Ventricular/etiología , Taquicardia Ventricular/cirugía , Arritmias Cardíacas , Insuficiencia de la Válvula Pulmonar/etiología , Insuficiencia de la Válvula Pulmonar/cirugíaRESUMEN
Background Quality of Life (QoL) is a prognostic factor in heart failure (HF) of patients with acquired cardiac disease. The aim of this study was to determine the predictive value of QoL on outcomes in adults with congenital heart disease (ACHD) and HF. Methods and Results Quality of life of 196 adults with congenital heart disease with clinical heart failure (HF) (mean age: 44.3±13.8 years; 51% male; 56% with complex congenital heart disease; 47% New York Heart Association class III/IV) included in the prospective multicentric registry FRESH-ACHD (French Survey on Heart Failure-Adult with Congenital Heart Disease) was assessed using the 36-Item Short Form Survey (SF-36), a patient-reported survey. The primary end point was defined by all-cause death, HF-related hospitalization, heart transplantation, and mechanical circulatory support. At 12 months, 28 (14%) patients reached the combined end point. Patients with low quality of life experienced major adverse events more frequently (logrank P=0.013). On univariate analysis, lower score at physical functioning (hazard ratio [HR], 0.98 [95% CI, 0.97-0.99]; P=0.008), role limitations related to physical health (HR, 0.98 [95% CI, 0.97-0.99]; P=0.008), and general health dimensions of the SF-36 (HR, 0.97 [95% CI, 0.95-0.99]; P=0.002) were significantly predictive of cardiovascular events. However, after multivariable analysis, SF-36 dimensions were no longer significantly associated with the primary end point. Conclusions Patients with congenital heart disease with HF and poor quality of life experience severe events more frequently, making quality of life assessment and rehabilitation programs essential to alter their trajectory.
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Cardiopatías Congénitas , Insuficiencia Cardíaca , Adulto , Humanos , Masculino , Persona de Mediana Edad , Femenino , Calidad de Vida , Estudios Prospectivos , Cardiopatías Congénitas/complicaciones , Cardiopatías Congénitas/terapia , Insuficiencia Cardíaca/epidemiología , Insuficiencia Cardíaca/terapia , Insuficiencia Cardíaca/complicaciones , Sistema de RegistrosRESUMEN
Background: Pulmonary autograft reinforcement to prevent dilatation and subsequent neo-aortic valve regurgitation has been reported; however, data on long-term function of the neo-aortic valve after this modified Ross procedure are lacking. Our objective here was to assess long-term outcomes of the modified Ross procedure with autograft reinforcement using the reimplantation technique. Patients: The outcomes of 61 consecutive patients managed using the Dacron-conduit reinforced Ross procedure between 2009 and 2021 were reviewed. Most patients had a unicuspid or bicuspid aortic valve (n = 52; 85%), predominant aortic valve regurgitation (n = 42; 77%), and >30 mm dilatation of the ascending aorta (n = 33; 54%). A prior aortic valve procedure was noted in 47 patients (77%) patients, including 38 (62%) with surgical repair and 9 (15%) with balloon dilatation. The pulmonary autograft was reimplanted within a Dacron conduit with a median diameter of 25.6 mm (range, 20-30 mm) using the David valve-sparing aortic root replacement technique. Results: All patients survived. The median age at surgery was 16.8 years (range, 6-38 years). Neo-aortic valve replacement was required in 3 patients (4.9%; 95% CI, 0.34%- 12.7%) because of infective endocarditis, left ventricular false aneurysm, and leaflet perforation, respectively; the repeat procedure was done early in 2 of these patients (2 of 61; 3%). Six patients required right ventricular outflow conduit replacement, 5 by surgery and 1 percutaneously. The median duration of follow-up was 90 months (range, 10-124 months). The 5- and 10-year rates of reintervention-free survival were 84.3% (95% CI, 74%-95%) and 81.6% (95% CI, 72%-93%), respectively, and 5-year survival without aortic reintervention was 94.5% (95% CI, 88%-100%), with little change at 10 years. No patients experienced deterioration of initial neo-aortic valve function (ie, regurgitation or stenosis). Conclusions: Autograft reinforcement using the reimplantation technique allowed expansion of Ross procedure indications to all patients requiring aortic valve replacement and prevented neo-aortic root dilatation. Failures were uncommon. Long-term follow-up data showed stable neo-aortic valve function.
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AIMS: To investigate the utility of novel deep learning (DL) algorithms in recognizing transposition of the great arteries (TGA) after atrial switch procedure or congenitally corrected TGA (ccTGA) based on routine transthoracic echocardiograms. In addition, the ability of DL algorithms for delineation and segmentation of the systemic ventricle was evaluated. METHODS AND RESULTS: In total, 132 patients (92 TGA and atrial switch and 40 with ccTGA; 60% male, age 38.3 ± 12.1 years) and 67 normal controls (57% male, age 48.5 ± 17.9 years) with routine transthoracic examinations were included. Convolutional neural networks were trained to classify patients by underlying diagnosis and a U-Net design was used to automatically segment the systemic ventricle. Convolutional networks were build based on over 100 000 frames of an apical four-chamber or parasternal short-axis view to detect underlying diagnoses. The DL algorithm had an overall accuracy of 98.0% in detecting the correct diagnosis. The U-Net architecture model correctly identified the systemic ventricle in all individuals and achieved a high performance in segmenting the systemic right or left ventricle (Dice metric between 0.79 and 0.88 depending on diagnosis) when compared with human experts. CONCLUSION: Our study demonstrates the potential of machine learning algorithms, trained on routine echocardiographic datasets to detect underlying diagnosis in complex congenital heart disease. Automated delineation of the ventricular area was also feasible. These methods may in future allow for the longitudinal, objective, and automated assessment of ventricular function.
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Ecocardiografía , Aprendizaje Automático , Transposición de los Grandes Vasos/diagnóstico por imagen , Disfunción Ventricular Derecha/diagnóstico por imagen , Adulto , Operación de Switch Arterial , Estudios de Casos y Controles , Femenino , Humanos , Masculino , Sensibilidad y Especificidad , Transposición de los Grandes Vasos/fisiopatología , Transposición de los Grandes Vasos/cirugía , Disfunción Ventricular Derecha/fisiopatología , Disfunción Ventricular Derecha/cirugíaAsunto(s)
Cardiopatías Congénitas/terapia , Complicaciones Cardiovasculares del Embarazo/terapia , Adulto , Femenino , Cardiopatías Congénitas/diagnóstico , Cardiopatías Congénitas/fisiopatología , Humanos , Embarazo , Complicaciones Cardiovasculares del Embarazo/diagnóstico , Complicaciones Cardiovasculares del Embarazo/fisiopatología , Factores de RiesgoRESUMEN
BACKGROUND: The relationship between pulmonary arterial hypertension-specific drug therapy (PAH-SDT) and mortality in Eisenmenger syndrome (ES) is controversial. AIMS: To investigate outcomes in patients with ES, and their relationship with PAH-SDT. METHODS: Retrospective, observational, nationwide, multicentre cohort study. RESULTS: We included 340 patients with ES: genetic syndrome (n=119; 35.3%); pretricuspid defect (n=75; 22.1%). Overall, 276 (81.2%) patients received PAH-SDT: monotherapy (endothelin receptor antagonist [ERA] or phosphodiesterase 5 inhibitor [PDE5I]) 46.7%; dual therapy (ERA+PDE5I) 40.9%; triple therapy (ERA+PDE5I+prostanoid) 9.1%. Median PAH-SDT duration was 5.5 years [3.0-9.1 years]. Events (death, lung or heart-lung transplantation) occurred in 95 (27.9%) patients at a median age of 40.5 years [29.4-47.6]. The cumulative occurrence of events was 16.7% [95% confidence interval 12.8-21.6%] and 46.4% [95% confidence interval 38.2-55.4%] at age 40 and 60 years, respectively. With age at evaluation or time since PAH diagnosis as time scales, cumulative occurrence of events was lower in patients taking one or two PAH-SDTs (P=0.0001 and P=0.004, respectively), with the largest differences in the post-tricuspid defect subgroup (P<0.001 and P<0.02, respectively) versus patients without PAH-SDT. By multivariable Cox analysis, with time since PAH diagnosis as time scale, New York Heart Association/World Health Organization functional class III/IV, lower peripheral arterial oxygen saturation and pretricuspid defect were associated with a higher risk of events (P=0.002, P=0.01 and P=0.04, respectively), and one or two PAH-SDTs with a lower risk of events (P=0.009). CONCLUSIONS: Outcomes are poor in ES, but seem better with PAH-SDT. ES with pretricuspid defects has worse outcomes despite the delayed disease onset.
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Antihipertensivos/uso terapéutico , Presión Arterial/efectos de los fármacos , Complejo de Eisenmenger/complicaciones , Hipertensión Pulmonar/tratamiento farmacológico , Arteria Pulmonar/efectos de los fármacos , Adolescente , Adulto , Factores de Edad , Causas de Muerte , Distribución de Chi-Cuadrado , Niño , Progresión de la Enfermedad , Supervivencia sin Enfermedad , Complejo de Eisenmenger/mortalidad , Complejo de Eisenmenger/fisiopatología , Femenino , Francia , Humanos , Hipertensión Pulmonar/etiología , Hipertensión Pulmonar/mortalidad , Hipertensión Pulmonar/fisiopatología , Estimación de Kaplan-Meier , Estudios Longitudinales , Masculino , Persona de Mediana Edad , Análisis Multivariante , Modelos de Riesgos Proporcionales , Arteria Pulmonar/fisiopatología , Estudios Retrospectivos , Factores de Riesgo , Factores de Tiempo , Resultado del Tratamiento , Adulto JovenRESUMEN
OBJECTIVE: There is growing evidence that maternal mortality in pregnant women with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) is lower than that in available data. In order to evaluate this hypothesis, we collected data of pregnancies in women with PAH-CHD. METHODS: Women with PAH-CHD followed in seven French referral centres were retrospectively included from 1997 to 2015. All pregnancies were recorded. We collected data on maternal, obstetrical and neonatal outcomes. RESULTS: 28 pregnancies in 20 women (26±6â years old) with PAH-CHD were managed during this period. There were 18 complete pregnancies (≥20â weeks' gestation (WG)), 8 abortions and 2 miscarriages. Six (33%, 95% CI (11.9 to 54.3)) patients experienced severe cardiac events. The concerned women had lower resting oxygen saturation (79.6±4.1% vs 89.3±3.8%, p<0.01). The most common cardiac complications during the complete pregnancies were heart failure (n=4) and severe hypoxaemia (n=5). Heart failure was overall severe, requiring inotropic treatment in three patients, mechanical circulatory support in one and led to one maternal death (mortality=5.0% 95% CI (0.1 to 24.9)). Obstetrical complications occurred in 25% of pregnancies. Small for gestational age was diagnosed in 39% (7/18) of fetuses. 12/18 (67%) pregnancies were delivered by caesarean section, of which 10 in emergency for obstetrical reason. Prematurity was frequent (78%), but no neonatal death occurred. CONCLUSIONS: Outcome of pregnancy in women with PAH-CHD is better than previously reported, with only 5% maternal mortality in our cohort. However, because of the severity of heart failure and the high rate of neonatal complications, patients should still be advised against pregnancy.
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Cardiopatías Congénitas/complicaciones , Hipertensión Pulmonar/etiología , Complicaciones Cardiovasculares del Embarazo/etiología , Resultado del Embarazo , Embarazo de Alto Riesgo , Aborto Espontáneo/etiología , Adulto , Cesárea , Bases de Datos Factuales , Femenino , Edad Gestacional , Cardiopatías Congénitas/diagnóstico , Cardiopatías Congénitas/mortalidad , Cardiopatías Congénitas/terapia , Humanos , Hipertensión Pulmonar/diagnóstico , Hipertensión Pulmonar/mortalidad , Hipertensión Pulmonar/terapia , Recien Nacido Prematuro , Recién Nacido Pequeño para la Edad Gestacional , Nacimiento Vivo , Mortalidad Materna , Embarazo , Complicaciones Cardiovasculares del Embarazo/diagnóstico , Complicaciones Cardiovasculares del Embarazo/mortalidad , Complicaciones Cardiovasculares del Embarazo/terapia , Nacimiento Prematuro/etiología , Estudios Retrospectivos , Factores de Riesgo , Adulto JovenRESUMEN
BACKGROUND: The impact of prosthesis-patient mismatch (PPM) after aortic valve replacement (AVR) for aortic stenosis on exercise capacity remains controversial. The aim of this study was to analyze the long-term impact of PPM after mechanical AVR on maximal oxygen uptake (VO2max). METHODS: The study included 75 patients who had undergone isolated mechanical AVR for aortic stenosis with normal left ventricular (LV) function between 1994 and 2012. Their functional capacity was evaluated on average 4.6 years after AVR by exercise testing, including measurement of their VO2max, and by determining their New York Heart Association functional class and Short Form-36 score. Two groups were defined by measuring the patients' indexed effective orifice area (iEOA) by transthoracic echocardiography: a PPM group (iEOA < 0.85 cm2/m2) and a no-PPM group (iEOA ≥ 0.85 cm2/m2). RESULTS: PPM was present in 37.0% of the patients. The percentage of the predicted VO2max achieved was significantly lower in the PPM group (86.7 ± 19.5% vs 97.5 ± 23.0% in the no-PPM group; P = 0.04). Compared with the no-PPM group, the PPM group contained fewer patients in New York Heart Association functional class I and their mean Short Form-36 physical component summary score was significantly lower. The mean transvalvular gradient was significantly higher in the PPM group than in the no-PPM group (P < 0.001). Systolic and diastolic function and LV mass had normalized in both groups. CONCLUSIONS: PPM is associated in the long term with moderate but significant impairment of functional capacity, despite optimal LV reverse remodelling and normalization of LV systolic and diastolic function.
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Estenosis de la Válvula Aórtica , Válvula Aórtica , Implantación de Prótesis de Válvulas Cardíacas , Prótesis Valvulares Cardíacas/efectos adversos , Efectos Adversos a Largo Plazo , Válvula Aórtica/diagnóstico por imagen , Válvula Aórtica/patología , Válvula Aórtica/cirugía , Estenosis de la Válvula Aórtica/diagnóstico , Estenosis de la Válvula Aórtica/cirugía , Ecocardiografía/métodos , Análisis de Falla de Equipo/métodos , Prueba de Esfuerzo/métodos , Tolerancia al Ejercicio/fisiología , Femenino , Francia , Implantación de Prótesis de Válvulas Cardíacas/efectos adversos , Implantación de Prótesis de Válvulas Cardíacas/instrumentación , Implantación de Prótesis de Válvulas Cardíacas/métodos , Humanos , Efectos Adversos a Largo Plazo/diagnóstico , Efectos Adversos a Largo Plazo/etiología , Efectos Adversos a Largo Plazo/fisiopatología , Masculino , Persona de Mediana Edad , Función Ventricular Izquierda/fisiología , Remodelación VentricularRESUMEN
BACKGROUND: Despite serious long-term sequel, women with Fontan palliation have reached childbearing age. However there is paucity of data on the pregnancy outcomes and management of this condition. We aimed to determine the maternal and fetal outcomes of pregnancy in women with Fontan palliation. METHODS: This multicentric, retrospective study included women with Fontan circulation followed in 13 French specialized centers from January 2000 to June 2014. All pregnancies were reviewed, including miscarriages, abortions, premature and term births. We reviewed maternal and fetal outcomes. RESULTS: Thirty-seven patients had 59 pregnancies. Mean age was 27 ± 5 years at first pregnancy. There were 16 miscarriages (27%) and 36 live births with 1 twin pregnancy. Cardiac events occurred in 6 (10%) pregnancies, with no maternal death. The most common cardiac complication was atrial arrhythmia, which occurred in 3 patients. Hematological complications including thromboembolic/hemorrhagic events (n=3/7) occurred in 5 women antepartum (n=2/3), and 4 women postpartum (n=1/4). Two of the 3 thromboembolic events occurred in patients without anticoagulation. There was a high incidence of prematurity (n=25/36, 69%). Anticoagulation was associated with adverse neonatal outcome (OR=10.0, 95% CI [1.5-91.4], p<0.01). After a median follow-up of 24 months, there was no significant worsening of clinical status and thromboembolic disease noted. CONCLUSIONS: Pre-selected women can successfully complete pregnancy with Fontan circulation. There is an increase in cardiac and neonatal morbidity during pregnancy. Because thromboembolism could have a severe consequence on Fontan circulation, anticoagulation should be indicated during pregnancy and postpartum period.
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Cardiopatías Congénitas/epidemiología , Recien Nacido Prematuro , Complicaciones Cardiovasculares del Embarazo/epidemiología , Resultado del Embarazo/epidemiología , Adulto , Femenino , Francia/epidemiología , Humanos , Incidencia , Recién Nacido , Masculino , Embarazo , Estudios RetrospectivosRESUMEN
BACKGROUND: Ebstein's anomaly of the tricuspid valve often results in biventricular dysfunction and functional deterioration. Little is known about the relation between exercise capacity, disease severity and outcome in adults with Ebstein's anomaly. METHODS: Data on all patients with Ebstein's anomaly of the tricuspid valve who underwent cardiopulmonary exercise testing in our tertiary center were collected. The relation between exercise parameters, anatomic severity (Glasgow outcome score) and the combined end-point of death, non-elective hospitalization and surgical repair was studied using Cox regression analysis. RESULTS: A total of 51 adult patients fulfilled inclusion criteria (49% male, mean age 37.8±13.6 years). Mean peak oxygen uptake (peak VO2) was 63.2±18.7% of predicted, the slope of ventilation per unit of carbon dioxide output (VE/VCO2 slope) 37.4±11.4, heart rate reserve (HRR) 23.6±22.7 bpm. A significantly lower peak VO2 was found in patients with a higher Glasgow outcome score, higher cardiothoracic ratio and documented atrial shunt. Peak VO2 (HR for value <60% of predicted 3.47, 95% CI: 1.289.44, p=0.015) and HRR (HR for value <25 bpm 3.07, 95% CI: 1.247.61, p=0.016) were significant predictors of outcome, the former being the strongest on multivariable analysis. CONCLUSIONS: Reduced exercise capacity in patients with Ebstein's anomaly relates to severity of the underlyingdisease and is a strong and independent predictor of outcome. Cardiopulmonary exercise testing should be incorporated in the follow-up and risk stratification of patients with this relatively uncommon and challenging cardiac defect.
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Anomalía de Ebstein/diagnóstico , Anomalía de Ebstein/metabolismo , Consumo de Oxígeno/fisiología , Índice de Severidad de la Enfermedad , Válvula Tricúspide/anomalías , Válvula Tricúspide/metabolismo , Adulto , Anomalía de Ebstein/terapia , Femenino , Estudios de Seguimiento , Predicción , Humanos , Masculino , Persona de Mediana Edad , Oxígeno/metabolismo , Valor Predictivo de las Pruebas , Estudios Retrospectivos , Resultado del Tratamiento , Adulto JovenRESUMEN
BACKGROUND: Transposition of the great arteries (TGA) after atrial switch operation and congenitally corrected TGA (ccTGA) are commonly associated with impaired systemic right ventricular (RV) function and impaired prognosis. We aimed to investigate the value of indices of myocardial deformation on speckle-tracking echocardiography for quantifying ventricular function and their potential role in assessing ventricular-ventricular interaction and outcome in patients with a systemic RV. METHODS AND RESULTS: A total of 129 patients (87 with TGA and atrial switch and 42 with ccTGA, 71 men, age 35 ± 12 years) were investigated, and biventricular myocardial deformation was compared with findings in healthy subjects (n = 38, age 36 ± 10 years). Systemic ventricular longitudinal 2-dimensional (2D) peak systolic strain (RV 2D-LS) was significantly reduced compared with controls (-12.9 ± 3.6 and -15.4 ± 5.1 vs -21.0 ± 5.5 in TGAs, ccTGAs, and controls, P < .0001). Systemic and pulmonary 2D-LS were correlated in patients with TGA (r = 0.46, P < .0001) and ccTGA (r = 0.64, P < .0001), suggesting interventricular interaction, and this was confirmed when ejection fraction on magnetic resonance imaging was assessed (r = 0.53, P < .0001). More importantly, systemic 2D-LS (hazard ratio 1.31, P = .01) was related to adverse clinical outcome (symptomatic progression to New York Heart Association class ≥3, clinically relevant cardiac arrhythmia, or death) in patients with TGA and atrial switch independently of ejection fraction on cardiac magnetic resonance imaging, history of clinically relevant arrhythmia, or functional class. CONCLUSIONS: Global longitudinal systolic strain is significantly reduced in patients with a systemic RV, is related to subpulmonary ventricular function, and predicts adverse clinical outcome in adults with atrial switch TGA.