Tetralogy of Fallot Publications (10730)


Tetralogy of Fallot Publications

Cardiol Young
Cardiol Young 2017 Jan;27(S1):S68-S74
Division of Cardiology,Nicklaus Children's Hospital,Miami,Florida,United States of America.

The prevalence of sudden cardiac arrest after surgery for CHD is primarily related to the complexity of the congenital defect and the presence of residual defects, especially ventricular dysfunction. Among all causes of death in patients having CHD, about 19% lead to sudden mortality. The specific risk factors associated with the various congenital defects are poorly understood. Read More

The lone exception is tetralogy of Fallot, largely due to its high prevalence and the historically high post-operative survival rate. In tetralogy of Fallot, historical, haemodynamic, and electrical features contribute to risk, and electrophysiologic testing may be helpful, particularly to rule out risk. An implantable cardioverter-defibrillator is highly effective for secondary prevention in most forms of CHD, and future advances will improve its role in primary prevention.

Cardiol Young
Cardiol Young 2017 Jan 12:1-5. Epub 2017 Jan 12.
Department of Pediatrics,University of California,San Francisco,California,United States of America.

Tetralogy of Fallot can be corrected with very low mortality at any age, even in neonates, but this does not necessarily mean that it should be corrected in the neonatal period. Although there are many advantages to early correction, a high proportion of these neonates have residual stenosis or pulmonary regurgitation that impairs ventricular function and may require further surgery or implantation of a pulmonary valve. Before we had the ability to correct this anomaly with low mortality in small children, a variety of palliative procedures had to be performed. Read More

Today, with better understanding of the anatomy of tetralogy of Fallot, we should consider what forms of palliation will increase growth of the right ventricular outflow tract in order to reduce the complications of very early surgery.

Cardiol Young
Cardiol Young 2017 Jan 12:1-5. Epub 2017 Jan 12.
Department of Pediatric Cardiology,National Cerebral and Cardiovascular Center,Osaka,Japan.

This study aimed to determine fetal echocardiographic features of tetralogy of Fallot in association with postnatal outcomes.
The Z-scores of the main and bilateral pulmonary arteries and the aorta were measured, and the following variables were calculated in 13 fetuses with tetralogy of Fallot: pulmonary artery-to-aorta ratio and main pulmonary artery cross-section ratio - the main pulmonary artery diameter squared divided by the sum of the diameter squared of the left and right pulmonary arteries. Fetuses were classified as having ductus arteriosus-dependent or ductus arteriosus-independent pulmonary circulation. Read More

We included two infants with pulmonary atresia and six infants with ductus-dependent pulmonary circulation, who underwent systemic-to-pulmonary shunt surgeries at ⩽1 month of age. The Z-scores of the main pulmonary artery and the pulmonary artery-to-aorta ratio in fetuses with ductus-dependent pulmonary circulation were lesser than those in fetuses with ductus independence, but not significantly. The main pulmonary artery cross-section ratio in fetuses with ductus dependence was significantly lesser (0.65±0.44 versus 1.56±0.48, p<0.005). Besides, the flow of the ductus arteriosus was directed from the aorta to the pulmonary artery in the ductus arteriosus-dependent group during the fetal period.
The main pulmonary artery cross-section ratio was the most significant variable for predicting postnatal outcomes in fetuses with tetralogy of Fallot.


Serum lactate and base deficit have been shown to be a predictor of morbidity and mortality in critically ill patients. Poor preoperative oxygenation appears to be one of the significant factors that affects early mortality in tetralogy of Fallot (TOF). There is little published literature evaluating the utility of serum lactate, base excess (BE), and oxygen partial pressure (PO 2 ) as simple, widely available, prognostic markers in patients undergoing surgical repair of TOF. Read More

This prospective, observational study was conducted in 150 TOF patients, undergoing elective intracardiac repair. PO 2 , BE, and lactate levels at three different time intervals were recorded. Arterial blood samples were collected after induction (T1), after cardiopulmonary bypass (T2), and 48 h (T3) after surgery in the Intensive Care Unit (ICU). To observe the changes in PO 2 , BE, and lactate levels over a period of time, repeated measures analysis was performed with Bonferroni method. The receiver operating characteristics (ROC) analysis was used to find area under curve (AUC) and cutoff values of various biomarkers for predicting mortality in ICU.
The patients who could not survive showed significant elevated lactate levels at baseline (T1) and postoperatively (T2) as compared to patients who survived after surgery (P < 0.001). However, in nonsurvivors, the BE value decreased significantly in the postoperative period in comparison to survivors (-2.8 ± 4.27 vs. 5.04 ± 2.06) (P < 0.001). In nonsurvivors, there was a significant fall of PO 2 to a mean value of 59.86 ± 15.09 in ICU (T3), whereas those who survived had a PO 2 of 125.86 ± 95.09 (P < 0.001). The ROC curve analysis showed that lactate levels (T3) have highest mortality predictive value (AUC: 96.9%) as compared to BE (AUC: 94.5%) and PO 2 (AUC: 81.1%).
Serum lactate and BE may be used as prognostic markers to predict mortality in patients undergoing TOF repair. The routine analysis of these simple, fast, widely available, and cost-effective biomarkers should be encouraged to predict prognosis of TOF patients.

J Cardiovasc Med (Hagerstown)
J Cardiovasc Med (Hagerstown) 2017 Jan 7. Epub 2017 Jan 7.
aCongenital Cardiac Surgery Unit bPediatric and Adult Congenital Heart Center cDepartment of Cardiac Surgery, IRCCS Policlinico San Donato, Milan, Italy.

The population of adults with congenital heart disease (ACHD) is increasing and aging, and a large percentage of this population is now over 65 years of age. For this reason, it is probable that acquired coronary artery disease (CAD) will become an important issue that needs to be addressed also in these patients. We retrospectively analyzed all ACHD patients who underwent surgery in our Institution with the aim to investigate the incidence of associated CAD and the results of surgical treatment. Read More

From January 2000 to December 2015, a total of 1154 ACHD underwent surgery in our center. Fifty patients (4.3%) were diagnosed with acquired CAD and required coronary artery bypass grafting. The mean age at surgery was 66 years (range 41-78 years). The primary diagnosis were atrial septal defect (n = 40 patients), Tetralogy of Fallot (n = 4 patients), ventricular septal defect (n = 2 patients), partial AV canal (n = 1), partial anomalous pulmonary venous drainage (n = 1), Ebstein's anomaly (n = 1), and subaortic stenosis (n = 1).
Hospital mortality was 2% (one patient). During a mean follow-up of 9 years (maximum follow-up: 15 years), seven patients died (14%). The actuarial survival was 83% at 5 years and 77% at 10 years. Freedom from reoperation for coronary artery bypass grafting or percutaneous coronary intervention was 88% at 5 years and 82% at 10 years.
Acquired CAD may coexist with congenital heart defects but the association is quite rare. It typically occurs later during adulthood and it is usually associated with atrial septal defect. Acquired CAD and congenital heart defects can be treated contemporarily with good early and late results.

Pacing Clin Electrophysiol
Pacing Clin Electrophysiol 2017 Jan 10. Epub 2017 Jan 10.
Electrophysiology Section, Cardiology Department, La Fe University Hospital, Valencia, Spain.

Information regarding suitability for S-ICD implant in Tetralogy of Fallot (ToF) population is scarce and needs to be further explored.
(i) to determine the proportion of patients with ToF eligible for S-ICD, (ii) to identify the optimal sensing vector in ToF patients, (iii) to test specifically the eligibility for sICD with right-sided screening, and (iv) to compare with the proportion of eligible patients in a non-selected ICD population.
We recruited 60 consecutive patients with ToF and 40 consecutive non-selected patients. Read More

Conventional electrocardiographic screening was performed as usual. Right-sided alternative screening was studied by positioning the left arm and right arm electrodes 1 cm right lateral to the xiphoid midline. The Boston Scientific ECG screening tool was utilized.
We found a higher proportion of patients with right-sided positive screening in comparison with standard screening (77±0.4% vs 67±0.4%; p<0.0001) and a trend to higher number of appropriate leads in right-sided screening (1.3±1vs 1.1±1ms; p = 0.07). Patients who failed the screening had a longer QRS duration and longer QT interval. Standard and right-sided screening showed a higher percent of positive patients in the control group compared to ToF patients (p<0.001).
Right-sided screening was associated with a significant 10% increase in S-ICD eligibility in ToF patients. When comparing with an acquired cardiomyopathies group, ToF showed a lower elegibility for S-ICD. The most appropriate ECG vector was the alternate vector in contrast to what is observed in the general population. This article is protected by copyright. All rights reserved.

Pediatr Cardiol
Pediatr Cardiol 2017 Jan 5. Epub 2017 Jan 5.
Division of Pediatric Cardiology, Memorial Hermann Children's Hospital, University of Texas Medical School at Houston, Houston, TX, USA.

Cardiac magnetic resonance imaging is an important tool to evaluate cardiac anatomy and ventricular size and function after repaired tetralogy of Fallot. Magnetic resonance tissue tagging is the gold standard for evaluation of myocardial strain. However, myocardial tagging strain requires tagged images to be obtained prospectively, during the scan and with limited temporal resolution. Read More

Cardiac magnetic resonance feature tracking is a new tool that allows the retrospective analysis of cine images. There is limited experience with cardiac magnetic resonance feature tracking strain analysis in children. The medical records of patients with repaired tetralogy of Fallot that had a cardiac magnetic resonance (CMR) study from December 2013 to June 2015 were reviewed. The control group included patients who underwent a CMR with normal cardiac anatomy and ventricular function. Global longitudinal, circumferential and radial strain parameters (2D and 3D) were obtained by retrospectively contouring cine images from ventricular short axis, two chamber and four chamber views using post-processing software (Circle CVi(42), Calgary, Canada). The correlation between conventional ventricular function parameters and ventricular strain was performed using Pearson's correlation. The mean age of tetralogy of Fallot and control subjects was 12.4 and 14.1 years, respectively. In patients after repaired tetralogy of Fallot, the mean left ventricular global 2D and 3D circumferential strains were -17.4 ± 2.9 and -10.1 ± 3, respectively. The mean indexed right ventricular end-diastolic volume was 135.4 cc m(2) ± 46 compared to 75.7 cc m(2) ± 17 in control subjects (P = 0.0001, CI 95%). Left ventricular global circumferential 3D strain showed a statistically significant difference in patients after TOF repair compared to normal subjects (-10.1 ± 3 vs. -14.71 ± 1.9, P = 0.00001). A strong correlation between left ventricular global circumferential 3D strain and right ventricular end-diastolic volume (P ≤ 0.0001) was noted. We found a strong correlation between left ventricular circumferential 3D strain and indexed right ventricular end-diastolic volume, as well as a strong correlation between left ventricular longitudinal 2D strain and right ventricular ejection fraction. Circumferential 3D strain may be a suitable tool to detect early abnormalities of ventricular myocardium even before the ejection fraction becomes compromised. Large-scale prospective studies are recommended.

Echocardiography 2017 Jan 3. Epub 2017 Jan 3.
Department of Pediatric Cardiology, Dr. Sami Ulus Obstetrics and Gynecology, Children's Health and Diseases Training and Research Hospital, Ankara, Turkey.

This study focuses on determining concomitant persistent left superior vena cava (SVC) in patients with congenital heart disease (CHD).
Between 2005 and 2012, a total of 2.663 patients with CHD, 88 (3. Read More

3%) of whom were diagnosed with persistent left SVC, were evaluated retrospectively. The demographic characteristics of patients, clinical and radiographic findings, echocardiography, cardiac catheterization, and angiography results obtained from the patients' records were reviewed.
The median age of the patients was 9.5 months, and 46 of the 88 (52.3%) patients were female. The most common concomitant CHD were ventricular septal defect, double outlet right ventricle (DORV), and tetralogy of Fallot (TOF). When the patients were compared according to their CHD, persistent left SVC frequency was significantly higher in those with DORV (P<.001), TOF (P=.04), patent ductus arteriosus (P=.01), and atrial septal defect (P=.03). Eighty-three of the 88 (94.3%) patients with persistent left SVC had right SVC, and 5 (5.7%) had absence of the right SVC. Twenty-seven of the 83 (32.5%) patients with double SVC had connected normal innominate vein. In all cases, right aortic arch association was seen in 14 (15.9%) patients. Eighty-four (95.4%) of the patients were diagnosed by echocardiography prior to catheter angiography. Persistent left SVC drained to the coronary sinus in all cases.
Increased awareness about the association of certain CHD with persistent left SVC and a careful echocardiographic examination can facilitate the diagnosis of persistent left SVC. In addition, precise prior diagnosis of persistent left SVC can prevent complications during surgery.

The growing adult population with surgically corrected tetralogy of Fallot (TOF) is at risk of arrhythmias and sudden cardiac death. We sought to investigate the contribution of right ventricular (RV) structural and electrophysiological remodelling to arrhythmia generation in a preclinical animal model of repaired TOF (rTOF).
Pigs mimicking rTOF underwent cardiac MRI functional characterisation and presented with pulmonary regurgitation, RV hypertrophy, dilatation and dysfunction compared with Sham-operated animals (Sham). Read More

Optical mapping of rTOF RV-perfused wedges revealed a significant prolongation of RV activation time with slower conduction velocities and regions of conduction slowing well beyond the surgical scar. A reduced protein expression and lateralisation of Connexin-43 were identified in rTOF RVs. A remodelling of extracellular matrix-related gene expression and an increase in collagen content that correlated with prolonged RV activation time were also found in these animals. RV action potential duration (APD) was prolonged in the epicardial anterior region at early and late repolarisation level, thus contributing to a greater APD heterogeneity and to altered transmural and anteroposterior APD gradients in rTOF RVs. APD remodelling involved changes in Kv4.3 and MiRP1 expression. Spontaneous arrhythmias were more frequent in rTOF wedges and more complex in the anterior than in the posterior RV.
Significant remodelling of RV conduction and repolarisation properties was found in pigs with rTOF. This remodelling generates a proarrhythmic substrate likely to facilitate re-entries and to contribute to sudden cardiac death in patients with rTOF.