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Background Pulmonary valve replacement in patients with congenital heart diseases and heart failure is challenging. Case summary Here, we describe a case of a patient who had surgical fallot repair with chronic heart failure. Investigations found severe biventricular dysfunction and enlargement due to chronic pulmonary regurgitation. The right ventricle outflow tract was tortuous and large with a diameter of 35 mm. Percutaneous pulmonary valve implantation (PPVI) was done after a challenging pre-stenting. A 32 mm Myval valve over-sized to 35 mm was used for PPVI, which yielded a good result. Discussion A 32 mm Myval valve is effective at extending the possibilities of PPVI in a large and tortuous right ventricle outflow tract not accessible for the other valves. [ABSTRACT FROM AUTHOR]

Aim: Ventricular arrhythmias (VAs) are the most common cause of death in patients with repaired Tetralogy of Fallot (rTOF). However, risk stratifying remains challenging. We examined outcomes following programmed ventricular stimulation (PVS) with or without subsequent ablation in patients with rTOF planned for pulmonary valve replacement (PVR). Methods: We included all consecutive patients with rTOF referred to our institution from 2010 to 2018 aged ≥18 years for PVR. Right ventricular (RV) voltage maps were acquired and PVS was performed from two different sites at baseline, and if non‐inducible under isoproterenol. Catheter and/or surgical ablation was performed when patients were inducible or when slow conduction was present in anatomical isthmuses (AIs). Postablation PVS was undertaken to guide implantable cardioverter‐defibrillator (ICD) implantation. Results: Seventy‐seven patients (36.2 ± 14.3 years old, 71% male) were included. Eighteen were inducible. In 28 patients (17 inducible, 11 non‐inducible but with slow conduction) ablation was performed. Five had catheter ablation, surgical cryoablation in 9, both techniques in 14. ICDs were implanted in five patients. During a follow‐up of 74 ± 40 months, no sudden cardiac death occurred. Three patients experienced sustained VAs, all were inducible during the initial EP study. Two of them had an ICD (low ejection fraction for one and important risk factor for arrhythmia for the second). No VAs were reported in the non‐inducible group (p <.001). Conclusion: Preoperative EPS can help identifying patients with rTOF at risk for VAs, providing an opportunity for targeted ablation and may improve decision‐making regarding ICD implantation. [ABSTRACT FROM AUTHOR]

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Introduction: The overshoot of the respiratory exchange ratio (RER) after exercise is reduced in patients with heart failure. Aim: The present study aimed to investigate the presence of this phenomenon in young patients with congenital heart disease (CHD), who generally present reduced cardiorespiratory fitness. Methods: In this retrospective study, patients with CHD underwent a maximal cardiopulmonary exercise testing (CPET) assessing the RER recovery parameters: the RER at peak exercise, the maximum RER value reached during recovery, the magnitude of the RER overshoot and the linear slope of the RER increase after the end of the exercise. Results: In total, 117 patients were included in this study. Of these, there were 24 healthy age-matched control subjects and 93 young patients with CHD (transposition of great arteries, Fontan procedure, aortic coarctation and tetralogy of Fallot). All patients presented a RER overshoot during recovery. Patients with CHD showed reduced aerobic capacity and cardiorespiratory efficiency during exercise, as well as a lower RER overshoot when compared to controls. RER magnitude was higher in the controls and patients with aortic coarctation when compared to those with transposition of great arteries, previous Fontan procedure, and tetralogy of Fallot. The RER magnitude was found to be correlated with the most relevant cardiorespiratory fitness and efficiency indices. Conclusions: The present study proposes new recovery indices for functional evaluation in patients with CHD. Thus, the RER recovery overshoots analysis should be part of routine CPET evaluation to further improve prognostic risk stratifications in patients with CHD. [ABSTRACT FROM AUTHOR]

One of the most common types of cyanotic congenital heart disease is Tetralogy of Fallot (ToF). Treatment has constantly increased since the first surgical repair in 1954. Excellent treatment having long-term survival (30 years survival ranges from 68.5% to 90.5%) is available for the ToF. However conventional and frequently required re-interventions include residual issues like right ventricular outflow tract obstruction, pulmonary regurgitation and (ventricular) arrhythmia. Right ventricular dysfunction might lead to longstanding pulmonary regurgitation and/or stenosis. It is important to perform pulmonary valve replacement or relief of pulmonary stenosis prior to irreversible right ventricular dysfunction, though determining optimal timing of pulmonary valve replacement is a problematic task due to various reasons. As seen in longstanding pulmonary regurgitation, the biological mechanisms underlying dysfunction of the right ventricle is often unclear. Various techniques of assessing the right ventricle are used to predict imminent dysfunction. The interventricular, ventriculo-arterial and atrioventricular interactions of right ventricle are not completely explained but play significant role in right ventricle performance. This review focuses on providing a brief overview of the history of ToF, describing the current strategies for treatment and describing the long-term survival, residual lesions and re-interventions following repair. Remaining related challenges and present condition of the art regarding these challenges are also illustrated. [ABSTRACT FROM AUTHOR]

Introduction: The overshoot of the respiratory exchange ratio (RER) after exercise is reduced in patients with heart failure. Aim: The present study aimed to investigate the presence of this phenomenon in young patients with congenital heart disease (CHD), who generally present reduced cardiorespiratory fitness. Methods: In this retrospective study, patients with CHD underwent a maximal cardiopulmonary exercise testing (CPET) assessing the RER recovery parameters: the RER at peak exercise, the maximum RER value reached during recovery, the magnitude of the RER overshoot and the linear slope of the RER increase after the end of the exercise. Results: In total, 117 patients were included in this study. Of these, there were 24 healthy age-matched control subjects and 93 young patients with CHD (transposition of great arteries, Fontan procedure, aortic coarctation and tetralogy of Fallot). All patients presented a RER overshoot during recovery. Patients with CHD showed reduced aerobic capacity and cardiorespiratory efficiency during exercise, as well as a lower RER overshoot when compared to controls. RER magnitude was higher in the controls and patients with aortic coarctation when compared to those with transposition of great arteries, previous Fontan procedure, and tetralogy of Fallot. The RER magnitude was found to be correlated with the most relevant cardiorespiratory fitness and efficiency indices. Conclusions: The present study proposes new recovery indices for functional evaluation in patients with CHD. Thus, the RER recovery overshoots analysis should be part of routine CPET evaluation to further improve prognostic risk stratifications in patients with CHD.

The outcomes of management of Tetralogy of Fallot (TOF) in children with only BT shunts have not been widely reported. Therefore, we present a 29-year-old man with complaints of progressive easy fatigability and effort intolerance. He was diagnosed with TOF in infancy and had both left- and right-sided BT shunts without corrective surgery. Examination revealed an asthenic young man with conjunctival plethora, cyanosis, digital clubbing, and hypertension. Chest X-ray, electrocardiography, and echocardiography revealed the typical anomalies of TOF. He has been referred for corrective surgery. Despite two previous BT shunts, the investigations still revealed the structural anomalies of TOF; this confirms BT shunt is not the definitive treatment but a palliative measure. Total corrective surgery remains the definitive treatment of TOF. Patients and their caregivers should be counseled on this and on the need for early corrective surgery.

A 12-year-old female with pulmonary artery stenosis developed pseudoaneurysms due to septic embolism, requiring close follow-up.

Tetralogy of Fallot (ToF) is the most common type of cyanotic congenital heart disease. Since the first surgical repair in 1954, treatment has continuously improved. The treatment strategies currently used in the treatment of ToF result in excellent long-term survival (30 year survival ranges from 68.5% to 90.5%). However, residual problems such as right ventricular outflow tract obstruction, pulmonary regurgitation, and (ventricular) arrhythmia are common and often require re-interventions. Right ventricular dysfunction can be seen following longstanding pulmonary regurgitation and/or stenosis. Performing pulmonary valve replacement or relief of pulmonary stenosis before irreversible right ventricular dysfunction occurs is important, but determining the optimal timing of pulmonary valve replacement is challenging for several reasons. The biological mechanisms underlying dysfunction of the right ventricle as seen in longstanding pulmonary regurgitation are poorly understood. Different methods of assessing the right ventricle are used to predict impending dysfunction. The atrioventricular, ventriculo-arterial and interventricular interactions of the right ventricle play an important role in right ventricle performance, but are not fully elucidated. In this review we present a brief overview of the history of ToF, describe the treatment strategies currently used, and outline the long-term survival, residual lesions, and re-interventions following repair. We discuss important remaining challenges and present the current state of the art regarding these challenges.

Introducción-objetivos: La situación Fallot tiene excelente supervivencia posquirúrgica, aunque presenta morbilidad tardía que ocasionalmente precisa reintervención: el 26% de nuestros pacientes seguidos en la unidad de cardiopatías congénitas del adulto han requerido reoperación. Analizamos estas cirugías con sus resultados. Métodos: Estudio retrospectivo (1991-2014), de 90 reoperaciones en 84 adultos con situación Fallot operada. Resultados: Edad media 33 ± 10 años, 59% varones, 2 ± 1 cirugías previas/paciente, clase funcional NYHA I-II: 46,4% y III-IV: 53,6%; antecedentes de arritmia en el 45%. Indicación quirúrgica principal: insuficiencia y/o estenosis pulmonar (76,7%), insuficiencia aórtica (11,1%), defectos septales residuales (7,8%) e insuficiencia tricuspídea (4,4%). Las reoperaciones precisaron circulación extracorpórea, evitando pinzado aórtico en el 34%. Técnicas quirúrgicas realizadas: a nivel pulmonar 81,1% (implante bioprótesis 68,9%), cierre de cortocircuito residual 50%, a nivel tricuspídeo 25,5% o aórtico 18,9%. Mortalidad hospitalaria: 4,4%. Seguimiento medio: 5,9 ± 6,2 años. Durante el seguimiento fallecieron 3 pacientes, 12 se reoperaron y 9 precisaron procedimientos percutáneos. La supervivencia a los 3 y 11 años fue, respectivamente, del 96 y el 90%. Actualmente, el 92,2% mantiene grado funcional I-II y el 82,3% está en ritmo sinusal. Comparando cardiorresonancias pre y postoperatorias observamos que la bioprótesis pulmonar significativamente reduce la insuficiencia pulmonar y los volúmenes del ventrículo derecho (p = 0,001), sin modificar su contractilidad. Conclusiones: Nuestra indicación principal para reoperar adultos con Fallot se localiza a nivel pulmonar y requiere implantar una bioprótesis. Realizamos estas reintervenciones con buena supervivencia, objetivando posteriormente mejoría clínica y reducción volumétrica ventricular derecha.

Introduction: There is a dearth of literature on tetralogy of fallot (TOF) in children in Sub-Saharan Africa. This study up aims to describe the prevalence, clinical profile and associated cardiac anomaly of children diagnosed with TOF documented over an eight year period in a tertiary hospital in South Western Nigeria. Methods: A prospective review of all consecutive cases of TOF diagnosed with echocardiography at the Lagos State University Teaching Hospital (LASUTH) between January 2007 and December 2014. Data were analyzed using SPSS version 20. Tables and charts were used to depict those variables. Descriptive statistic are presented as percentages or means and standard deviation. Means of normally distributed variables were compared using the student t-test and proportions using chi-square test. Skewed distribution were analyzed using appropriate non-parametric tests. Level of significance set at P

Background: Repair of tetralogy of fallot (TOF) is associated with diffuse myocardial fibrosis. Cardiac magnetic resonance imaging (CMR) can visualize the areas with myocardial fibrosis. Presence of fragmented QRS (fQRS) implies the presence of the underlying myocardial scar. Despite the strong association between fQRS and myocardial pathologies, the impact of fQRS with myocardial fibrosis in post-TOF correction is unknown. Objectives: Here, we evaluated the possible predictive role of fQRS in repaired TOF cases and its relationship with cardiac function. Patients and Methods: Thirty two patients with previous history of repaired TOF were enrolled. The extent of fQRS was evaluated according to the number of leads with fQRS. After electrocardiographic evaluation, the participants underwent CMR. Results: Results showed a significant relationship between the right ventricular (RV) systolic diameter and fQRS (P = 0.014). Also, an inverse linear relationship was found between the number of fQRS edges and RVEF (r = 0.77, P = 0.0001). The mean QRS duration in those with positive and negative fQRS was 132 mm and 115.8 mm (P = 0.0001). Furthermore, a linear correlation was observed between the number of edges and the percentage of scar tissue (r = 0.88, P = 0.001). However, no relevance between gender and fQRS was detected (P = 0.26), and the relationship between RV diastolic diameter and fQRS was not significant (P = 0.1). Thus, fQRS could be used as a marker of RV systolic dysfunction in patients with tetralogy of fallot. Conclusions: We suggested the fQRS as a surrogate indicator of RV dysfunction in repaired TOF patients and showed that diagnostic and prognostic information of the patients were available by fQRS. [ABSTRACT FROM AUTHOR]

Introducción: El cierre transauricular de la comunicación interventricular puede verse dificultado por tejido subvalvular. Presentamos una serie con desinserción parcial de valva anterior de la tricúspide para facilitar el cierre del defecto septal. Métodos: Estudio retrospectivo de 156 pacientes con comunicación interventricular (82 simple, 74 Fallot). En 20 casos (9 simple, 11 Fallot) se desinsertó parcialmente la válvula tricúspide (12,8%). Medias de edad y peso 10,6 meses y 7,13 kg, respectivamente. La valva anterior de la tricúspide fue desinsertada electivamente, facilitando el cierre del defecto con parche de dacrón en 19 pacientes y mediante sutura directa en uno. Posteriormente, se reimplantó la valva al anillo con una sutura independiente. Como procedimientos asociados: resección de bandas musculares medio-ventriculares (2), resuspensión valvular aórtica por insuficiencia (2), Alfieri mitral (1) y comisurotomía mitral (1). Resultados: Los tiempos medios de circulación extracorpórea fueron de 107 min (rango 61-153) y de isquemia 58 min (rango 31-99). No se detectó comunicación interventricular residual, insuficiencia tricuspídea ni insuficiencia aórtica en ecografía intraoperatoria. Un paciente falleció por sepsis y otro precisó marcapasos definitivo por bloqueo completo. El seguimiento medio es de 25 meses, sin defectos residuales. Conclusiones: La desinserción parcial de la valva anterior de la tricúspide en casos de dificultad para el cierre transauricular de la comunicación interventricular es útil. Ofrece una visón óptima del defecto, con buenos resultados y escasa morbilidad.

La tetralogía de Fallot es la cardiopatía congénita cianótica más frecuente en la población general, con una incidencia general de 0,1/1.000 nacidos vivos. El eje morfológico diagnóstico es el desplazamiento anterior izquierdo del septo infundibular -hacia la vía de salida ventricular derecha- durante el periodo de embriogénesis, lo que causa complejo de cabalgamiento aórtico, comunicación interventricular, estenosis subpulmonar e hipertrofia ventricular derecha. Sin intervención quirúrgica, su sobrevida al año alcanza 66% y sólo 10% a 15% en más de veinte años. La presentación clínica es variable y depende del grado de estenosis pulmonar; cuando ya es significativa en la etapa neonatal o en lactantes menores de tres a seis meses, pueden aparecer crisis de hipoxemia que requieren intervención médica o quirúrgica de urgencia. La corrección completa de la malformación ofrece buenos resultados de supervivencia durante décadas, si bien con las primeras técnicas -parche trans-anular y cierre del defecto interventricular- surgen problemas a largo plazo que pueden generar un riesgo adicional de morbimortalidad. La insuficiencia pulmonar severa, la presencia de dilatación ventricular derecha y el desarrollo de arritmias potencialmente fatales son problemas que se tornan importantes y que deben reconocerse a fin de valorar una reintervención temprana y reparar los defectos residuales inductores de arritmia. Las nuevas técnicas quirúrgicas tratan de conservar al máximo la integridad de la unión ventrículo-pulmonar y de la propia válvula, siempre y cuando la anatomía lo permita. Ello redunda, sin duda, en beneficio para el paciente ya que los problemas residuales descritos tienen menor significado clínico.The tetralogy of Fallot is the most frequent cyanotic congenital heart disease in the general population with a general incidence of 0.1/1000 live births. The morphologic diagnostic axis is the left anterior displacement of the infundibular ventricular septum - towards the right ventricle way out- during the embryogenic period that causes aortic override, ventricular septal defect, subpulmonary stenosis and right ventricular hypertrophy. Without surgical intervention, survival is 66% in 1 year and only 10% to 15% in > 20 years. Clinical presentation is variable and depends on the grade of pulmonary stenosis; when it is already significant in the neonatal period or in infants < 3 to 6 months, hypoxemic crisis that may require urgent medical or surgical intervention may appear. Complete surgical correction of the malformation offers good survival outcomes during decades, although with the first techniques -trans-anular patch and closure of the interventricular defect- there appear long term problems that may generate an additional mobimortality risk. Severe pulmonary insufficiency, presence of right ventricular dilation and development of potentially fatal arrhythmias are problems that become important and must be recognized in order to assess an early re-intervention and to repair residual defects inductors of arrhythmia. The new surgical techniques try to preserve as much as possible ventricular-pulmonary joint and the valve itself, if anatomy allows it. That will without doubt benefit the patient because the residual problems described have lesser clinical significance.

Background Percutaneous pulmonary valve implantation (PPVI) using Melody valve made of bovine jugular vein is safe and effective. However, infective endocarditis has been reported for unclear reasons. We sought to assess the impact of valvular substrates on selective bacterial adhesion. Methods Three valved stents (Melody valve, homemade stents with bovine and porcine pericardium) were tested in-vitro for bacterial adhesion using Staphylococcus aureus and Streptococcus sanguinis strains. Results Bacterial adhesion was higher on bovine jugular venous wall for S. aureus and on Melody valvular leaflets for S. sanguinis in control groups and significantly increased in traumatized Melody valvular leaflets with both bacteria (traumatized vs non traumatized: p = 0.05). Bacterial adhesion was lower on bovine pericardial leaflets. Conclusion Selective adhesion of S. aureus and S. sanguinis pathogenic strains to Melody valve tissue was noted on healthy tissue and increased after implantation procedural steps. [ABSTRACT FROM AUTHOR]

Tetralogy of Fallot is diagnosed in about 3.5% of patients who have congenital heart defects. In tetralogy of Fallot, ventricular septal defect is usually large because of malalignment of outlet septum. But the restrictive ventricular septal defect has been reported rarely & its association with dextrocardia and situs inversus is uncommon. We report all these findings in a 10-year-old boy and review the medical literature relevant to these combined conditions. [ABSTRACT FROM AUTHOR]

Abstract: Objective: To evaluate the utility of plasma BNP measurement in the long term follow-up of patients with surgically repaired tetralogy of Fallot (TOF). Methods: From 2002 to 2008, plasma BNP concentration was measured in 130 patients with TOF at the age of mean 16.1±7.1 years and mean 13.0±6.5 years after surgically repair. BNP levels were compared to age and gender-specific normal values, results of exercise testing, clinical, electrocardiographic, and echocardiographic data. Results: BNP was <200 pg/ml in all and elevated in 60% of patients. Higher values were found in females (p =0.001), in patients waiting for pulmonary valve replacement (p <0.001), and in NYHA class II compared to NYHA I patients (p =0.012) with an inverse correlation between BNP and exercise time (r =−0.59, p <0.001). BNP was correlated with right ventricular dilatation (r =0.29, p =0.005) and severity of both tricuspid (r =0.22, p =0.015) and pulmonary regurgitation (r =0.20, p =0.029). Longitudinal data revealed increasing BNP levels before (p =0.04) and a BNP decrease after pulmonary valve replacement (p =0.03), but no change in patients without surgery. Conclusions: In patients with surgically repaired TOF, plasma BNP is significantly correlated with right ventricular volume load. In clinical practice a considerable overlap limits the estimation of right ventricular volume load by BNP, but longitudinal evaluation helps to appoint the appropriate timing of pulmonary valve replacement. [ABSTRACT FROM AUTHOR]

Aims: To evaluate right ventricular (RV) volume and ejection fraction (EF) in adult normal subjects and repaired tetralogy of Fallot (ToF) with 3D trans-thoracic echocardiography (3DE) and a semi-automatic border detection algorithm. [ABSTRACT FROM PUBLISHER]

ROSTOCK, T., et al.: Radiofrequency Catheter Ablation of a Macroreentrant Ventricular Tachycardia Late After Surgical Repair of Tetralogy of Fallot Using Electroanatomic Mapping (CARTO). This case report describes a patient with a sustained monomorphic VT after surgical repair of a tetralogy of Fallot (TOF). In combination with the three-dimensional electroanatomic mapping system, CARTO, and conventional mapping techniques the VT was identified as a macro-reentrant tachycardia circling around the border between pulmonary graft and right ventricular outflow tract (RVOT). A y-shaped ablation line crossing this zone was created. The VT terminated during RF application and was not inducible again. This case underlines the use of a combined conventional and three-dimensional electroanatomic mapping technique can be helpful for catheter ablation of ventricular arrhythmias in TOF patients. (PACE 2004; 27[Pt. I]:801–804) [ABSTRACT FROM AUTHOR]

Background: The adult population of repaired tetralogy of Fallot is increasing and at risk of pre-mature death and arrhythmia. This study evaluates risk factors for adverse outcome and the effect of pulmonary valve replacement within a national cohort., Methods: A retrospective cohort study of 341 adult repaired tetralogy of Fallot (16-72 years) managed through a single national service was undertaken incorporating over 1200 patient-years of follow-up. Demographics, cardiopulmonary exercise testing, cardiac magnetic resonance, reintervention (including pulmonary valve replacement), and clinical events were analysed. The influence of these parameters on a primary outcome (death or arrhythmia) was evaluated., Results: Compared with an age-/gender-matched population, patients experienced a reduced survival, particularly males over 55 years (standardised mortality ratio : 6.12, 95% CI: 1.64-15.66, p = 0.004). Cox proportional hazards modelling identified increased indexed right ventricle (RV) end-diastolic volume (hazard ratio (HR): 2.86, 95% CI: 1.4-5.85, p = 0.004) and female gender (HR (male): 0.37, 95% CI: 0.14-0.98, p = 0.045) to be predictors significantly associated with the primary outcome. Pulmonary valve replacement undertaken at indexed RV end-diastolic volume = 145 ml/m2 reduced RV volumes and QRS duration but did not improve cardiopulmonary exercise testing nor NYHA class. Pulmonary valve replacement during cohort period was associated with increased risk of primary outcome (HR: 2.82, 95% CI: 1.36-5.86, p = 0.005)., Conclusions: Although the majority of adult tetralogy of Fallot were asymptomatic in NYHA 1, cardiopulmonary exercise testing revealed important deficits. Tetralogy of Fallot survival was reduced compared to the general population. Female gender and increasing RV end-diastolic volume predicted adverse events. Pulmonary valve replacement reduced RV volumes and QRS duration but did not improve primary outcome.

Background: The population of adults with tetralogy of Fallot (TOF) or pulmonary atresia with ventricular septal defect (PA/VSD) is growing and aging. Data regarding older patients are scarce. Prognostic outcome parameters in adults with TOF or PA/VSD ≥ 40 years were studied. Methods: This was a retrospective study of patients ≥ 40 years of age during the study period (January 2005–March 2018). Major adverse cardiac events (MACE) were a combined primary endpoint including death from any cause, prevented sudden cardiac death, pacemaker implantation, arrhythmia, and new-onset heart failure. Additionally, MACE II (secondary endpoint) was a combination of death from any cause and prevented sudden cardiac death. Results: 184 (58.7% female, mean age 45.3 ± 7.2 years) patients were included (159 (86.4%) TOF and 25 (13.6%) PA/VSD). During a median follow-up of 3.1 years (IQR: 0.6–6.5), MACE occurred in 35 and MACE II in 13 patients. On multivariable analysis, New York Heart Association class [HR: 2.1, 95% CI: 1.2–3.6, p = 0.009] emerged as an independent predictor for MACE, and age at corrective surgery [HR: 13.2, 95% CI: 1.6–107.1, p = 0.016] for MACE II. Conclusions: Adults with TOF or PA/VSD ≥ 40 years are burdened with significant morbidity and mortality. New York Heart Association class and age at corrective surgery were independent predictors of outcome. [ABSTRACT FROM AUTHOR]

Background: Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease (CHD). Abnormal aortic dimensions and elasticity parameters have been long described for corrected TOF (cTOF) together with left ventricular (LV) rotational abnormalities, but results are conflicting. The present study focuses on investigating LV rotational mechanics in cTOF, and possible correlation of these parameters with aortic elasticity. It was also aimed to be examined whether different surgical strategies have any effect on these results., Methods: The study involved 26 adult cTOF patients, from which 14 had palliative surgery first and a late total correction (pcTOF), while early total correction was the treatment of choice in 12 patients (etrTOF). Their results were compared to those of 37 age- and gender-matched healthy adults. Routine transthoracic two-dimensional Doppler echocardiography extended with assessment of aortic elastic properties and three-dimensional speckle-tracking echocardiography (3DSTE) was performed in all cTOF patients and controls., Results: Sixteen out of 26 cTOF patients showed normally directed LV rotational mechanics, while apical or basal LV rotations were in the same clockwise or counterclockwise directions in 7 and 3 cTOF cases, respectively (LV 'rigid body rotation', RBR). Significantly reduced LV apical rotation and twist could be demonstrated in all cTOF patients with preserved LV basal rotation regardless of previous procedure. pcTOF patients showed significantly reduced LV apical rotation as compared to that of etrTOF cases. Significant correlations could be demonstrated between LV apical rotation and aortic stiffness index (r=-0.55, P=0.03) and aortic distensibility (r=0.52, P=0.04)., Conclusions: Significant LV rotational abnormalities could be demonstrated in cTOF with the high prevalence of LV-RBR. pcTOF patients showed significantly reduced LV apical rotation as compared to that of etrTOF cases. Unexpected abnormal physiologic response of LV rotational mechanics to increased aortic stiffness can be detected in cTOF patients without LV-RBR., Competing Interests: Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at http://dx.doi.org/10.21037/cdt-20-365). The series “Current Management Aspects in Adult Congenital Heart Disease (ACHD): Part III” was commissioned by the editorial office without any funding or sponsorship. The authors have no other conflicts of interest to declare., (2021 Cardiovascular Diagnosis and Therapy. All rights reserved.)

Background: Aortopathy is a common phenomenon in tetralogy of Fallot (TOF). The current study was designed to detect left ventricular (LV) deformation abnormalities and its relation to aortic stiffness in corrected TOF (cTOF) using the novel three-dimensional (3D) speckle-tracking echocardiography (3DSTE). Detailed comparative analysis between patients with early palliation-late correction (pcTOF) and early total reconstruction (etrTOF) was also performed., Methods: The present study consisted of 28 cTOF patients (35.0±15.7 years, 11 males) from which 15 and 13 proved to be pcTOF and etrTOF, respectively. Their clinical parameters were compared to those of 39 matched healthy adults (35.5±6.0 years, 16 males)., Results: cTOF patients showed significantly lower global LV longitudinal, circumferential and area strains as compared to controls. In etrTOF patients, global LV 3D strain was higher than in controls. In pcTOF patients, all LV strains proved to be significantly lower as compared to those of etrTOF patients and controls. In all cTOF patients, several moderate correlations could be detected between LV strain parameters and aortic elastic properties., Conclusions: Significant LV deformational abnormalities could be demonstrated in cTOF patients. etrTOF patients have beneficial LV strain parameters as compared to those of pcTOF patients. LV strains show correlations with aortic elastic properties., Competing Interests: Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at http://dx.doi.org/10.21037/cdt-20-366). The series “Current Management Aspects in Adult Congenital Heart Disease (ACHD): Part III” was commissioned by the editorial office without any funding or sponsorship. The authors have no other conflicts of interest to declare., (2021 Cardiovascular Diagnosis and Therapy. All rights reserved.)