Humans ; DiGeorge Syndrome/diagnostic imaging* ; Tetralogy of Fallot/surgery* ; Ultrasonography

Tetralogy of Fallot (TOF) in pregnancy is a rare occurrence which poses a high risk for detrimental effects on both mother and fetus. This paper reports a 21-year-old primigravid diagnosed with uncorrected TOF who had a successful caesarean section at 32 weeks of gestation. To address the hemodynamic challenges, the anaesthetic management involved the use of a minimally invasive hemodynamic monitor, controlled mechanical ventilation and a combined technique of intravenous anaesthesia using remifentanil and lumbar epidural anaesthesia using levobupivacaine.

Objective: To investigate the clinical and electrophysiological features of ventricular tachycardia (VT) in tetralogy of Fallot (TOF) patients post surgical repair (rTOF) and to analyze the therapeutic effect and prognosis of radiofrequency ablation of rTOF-VT. Methods: This is a retrospective study. Consecutive patients with rTOF-VT, who were treated in Fuwai Hospital from January 2015 to March 2020, were enrolled. All the patients underwent right ventricular voltage mapping following routine cardiac electrophysiological examination, followed by linear or homogenizing radiofrequency ablation based on the low-voltage substrate. The clinical features, 3-dimentional electrophysiological substrate mapping, radiofrequency ablation and long-term prognosis of the enrolled patients were analyzed. Acute ablation success was defined as completion of linear or homogenizing ablation or intraoperative evoked VT as destination of the procedure. Patients were followed up at 3 and 6 months post operation and every year thereafter. The endpoints were sudden cardiac death (SCD) and recurrence of ventricular tachycardia. Results: A total of 20 patients with rTOF-VT were enrolled including 14 males with an age of (35.8±11.8) years. The electrocardiogram identified 23 types of ventricular tachycardia, 19 of which were originated from right ventricular inflow tract outlet. The most common clinical manifestations were heart murmur (19 cases, 95%) and syncope (4 cases, 25%). Electroanatomical substrate mapping was performed in 20 patients and evidenced localized or diffuse scar or low-voltage area of right ventricle. Intraoperative electrophysiological tests provoked ventricular tachycardia in 6 patients (30%), including 5 patients with hemodynamics disturbance. The acute success rate of radiofrequency ablation was 95% (19/20). The follow-up time was (31.1±17.7) months and the recurrence rate of ventricular tachycardia was 30% during follow-up period and 5 cases received repeat radiofrequency ablation and there was no recurrent ventricular tachycardia during follow-up post repeat radiofrequency ablation. Conclusions: The voltage substrate mapping under sinus rhythm is a feasible mapping method for rTOF-VT. Linear or flaky radiofrequency ablation of the slow conduction zone is safe and effective treatment strategy, the recurrence rate after the first radiofrequency ablation is still high, and the effectiveness of repeat radiofrequency ablation is satisfactory in this patient cohort.
Adult ; Arrhythmias, Cardiac ; Catheter Ablation ; Electrocardiography ; Follow-Up Studies ; Humans ; Male ; Middle Aged ; Retrospective Studies ; Tachycardia, Ventricular/surgery* ; Tetralogy of Fallot/surgery* ; Treatment Outcome ; Young Adult

tetralogy of Fallot (control group; age range, 1 day to 6 months) and compared between the 2 groups. The type of final surgery was also evaluated in the hypoplastic RV group over a follow-up period of 3–8 years.RESULTS: The RV and LV volumes and lengths were successfully quantified in all 90 patients. The tricuspid valve annulus diameter could not be measured in cases showing muscular tricuspid atresia and double-inlet LV. The initial RV dimensions quantified by CT were significantly lower for the hypoplastic RV group than for the control group (p < 0.001). The types of final surgery performed in the hypoplastic RV group were univentricular repair in 46 patients, biventricular repair in 4 patients, or an indeterminate surgery in 7 patients.CONCLUSION: Initial RV dimensions in infants with CHD and a hypoplastic RV can be quantified by CT and are substantially smaller than those in infants with tetralogy of Fallot.]]>
Follow-Up Studies ; Heart Defects, Congenital ; Heart Ventricles ; Humans ; Infant ; Tetralogy of Fallot ; Tricuspid Atresia ; Tricuspid Valve

OBJECTIVE: To assess the accuracy and potential bias of computed tomography (CT) ventricular volumetry using semiautomatic three-dimensional (3D) threshold-based segmentation in repaired tetralogy of Fallot, and to compare them to those of two-dimensional (2D) magnetic resonance imaging (MRI). MATERIALS AND METHODS: This retrospective study evaluated 32 patients with repaired tetralogy of Fallot who had undergone both cardiac CT and MRI within 3 years. For ventricular volumetry, semiautomatic 3D threshold-based segmentation was used in CT, while a manual simplified contouring 2D method was used in MRI. The indexed ventricular volumes were compared between CT and MRI. The indexed ventricular stroke volumes were compared with the indexed arterial stroke volumes measured using phase-contrast MRI. The mean differences and degrees of agreement in the indexed ventricular and stroke volumes were evaluated using Bland-Altman analysis. RESULTS: The indexed end-systolic (ES) volumes showed no significant difference between CT and MRI (p > 0.05), while the indexed end-diastolic (ED) volumes were significantly larger on CT than on MRI (93.6 ± 17.5 mL/m² vs. 87.3 ± 15.5 mL/m² for the left ventricle [p < 0.001] and 177.2 ± 39.5 mL/m² vs. 161.7 ± 33.1 mL/m² for the right ventricle [p < 0.001], respectively). The mean differences between CT and MRI were smaller for the indexed ES volumes (2.0–2.5 mL/m²) than for the indexed ED volumes (6.3–15.5 mL/m²). CT overestimated the stroke volumes by 14–16%. With phase-contrast MRI as a reference, CT (7.2–14.3 mL/m²) showed greater mean differences in the indexed stroke volumes than did MRI (0.8–3.3 mL/m²; p < 0.005). CONCLUSION: Compared to 2D MRI, CT ventricular volumetry using semiautomatic 3D threshold-based segmentation provides comparable ES volumes, but overestimates the ED and stroke volumes in patients with repaired tetralogy of Fallot.
Bias (Epidemiology) ; Heart Defects, Congenital ; Heart Ventricles ; Humans ; Magnetic Resonance Imaging* ; Methods ; Retrospective Studies ; Stroke Volume ; Tetralogy of Fallot*

BACKGROUND: This study aimed to determine the effect of a multidisciplinary approach on the birth rate of fetuses with prenatally diagnosed congenital heart diseases (CHDs). METHODS: Among the fetuses of 724 gravidas who underwent fetal echocardiography in Samsung Medical Center from January 2013 to June 2017, 463 fetuses with normal cardiac structure, arrhythmia or simple left-to-right shunt were excluded, and the remaining 261 were included in the study. The subjects were subdivided into groups based on whether they were consulted multidisciplinarily, that is, consulted simultaneously by pediatric cardiologists, obstetricians and pediatric cardiac surgeons or not. They were also categorized based on the initial fetal echocardiogram results. RESULTS: Among the fetuses in the multidisciplinary group, 64.5% of the fetuses were given birth to, and the proportion was not different from that in the non-multidisciplinary group (68.6%, P = 0.48). The delivery rate in the multidisciplinary consultation group were 69.2% in the transposition of the great arteries group, 63.6% in the tetralogy of Fallot group, 68.8% in the pulmonary atresia or interrupted aortic arch group, 62.5% in the coarctation of aorta group, 60.0% in the atrioventricular septal defect group, 70.0% in the functional single ventricle group, and 55.6% in the hypoplastic left heart syndrome group; there were no significant differences between the 10 echocardiogram groups. However, when the subjects were categorized into Fontan repair group and biventricular repair group, the Fontan repair group showed a significant increase in the likelihood of delivery when a multidisciplinary approach was taken (P = 0.035). CONCLUSION: When a fetus was diagnosed with a CHD where Fontan repair should be considered, a multidisciplinary approach resulted in increased possibility of delivery.
Aorta, Thoracic ; Aortic Coarctation ; Arrhythmias, Cardiac ; Arteries ; Birth Rate ; Echocardiography ; Fetus ; Heart Defects, Congenital ; Heart Diseases ; Hypoplastic Left Heart Syndrome ; Parturition ; Prenatal Diagnosis ; Pulmonary Atresia ; Surgeons ; Tetralogy of Fallot

Child ; Child, Preschool ; Female ; Humans ; Infant ; Magnetic Resonance Imaging ; methods ; Male ; Pulmonary Valve Insufficiency ; diagnostic imaging ; Retrospective Studies ; Tetralogy of Fallot ; diagnostic imaging ; Ventricular Function, Right ; physiology

OBJECTIVE: To characterize the changes in right ventricular (RV) volume, volume load, and function measured with cardiac computed tomography (CT) over the entire time course of tetralogy of Fallot (TOF). MATERIALS AND METHODS: In 374 patients with TOF, the ventricular volume, ventricular function, and RV volume load were measured with cardiac CT preoperatively (stage 1), after palliative operation (stage 2), after total surgical repair (stage 3), or after pulmonary valve replacement (PVR) (stage 4). The CT-measured variables were compared among the four stages. After total surgical repair, the postoperative duration (POD) and the CT-measured variables were correlated with each other. In addition, the demographic and CT-measured variables in the early postoperative groups were compared with those in the late postoperative and the preoperative group. RESULTS: Significantly different CT-based measures were found between stages 1 and 3 (indexed RV end-diastolic volume [EDV], 63.6 ± 15.2 mL/m2 vs. 147.0 ± 38.5 mL/m2 and indexed stroke volume (SV) difference, 7.7 ± 10.3 mL/m2 vs. 32.2 ± 16.4 mL/m2; p < 0.001), and between stages 2 and 3 (indexed RV EDV, 72.4 ± 19.7 mL/m2 vs. 147.0 ± 38.5 mL/m2 and indexed SV difference, 5.7 ± 13.1 mL/m2 vs. 32.2 ± 16.4 mL/m2; p < 0.001). After PVR, the effect of RV volume load (i.e., indexed SV difference) was reduced from 32.2 mL/m2 to 1.7 mL/m2. Positive (0.2 to 0.8) or negative (−0.2 to −0.4) correlations were found among the CT-based measures except between the RV ejection fraction (EF) and the RV volume load parameters. With increasing POD, an early rapid increase was followed by a slow increase and a plateau in the indexed ventricular volumes and the RV volume load parameters. Compared with the preoperative data, larger ventricular volumes and lower EFs were observed in the early postoperative period. CONCLUSION: Cardiac CT can be used to characterize RV volume, volume load, and function over the entire time course of TOF.
Humans ; Multidetector Computed Tomography ; Postoperative Period ; Pulmonary Valve ; Pulmonary Valve Insufficiency ; Stroke Volume ; Tetralogy of Fallot ; Ventricular Function

OBJECTIVE: To evaluate flow pattern characteristics in the ascending aorta (AA) with four-dimensional (4D)-flow MRI and to determine predictors of aortic dilatation late after tetralogy of Fallot (TOF) repair. MATERIALS AND METHODS: This study included 44 patients with repaired TOF (25 males and 19 females; mean age, 28.9 ± 8.4 years) and 11 volunteers (10 males and 1 female, mean age, 33.7 ± 8.8 years) who had undergone 4D-flow MRI. The aortic diameters, velocity, wall shear stress (WSS), flow jet angle (FJA), and flow displacement (FD) at the level of the sinotubular junction (STJ) and mid-AA were compared between the repaired TOF and volunteer groups. The hemodynamic and clinical parameters were also compared between the aortic dilatation and non-dilatation subgroups in the repaired TOF group. RESULTS: The diameters of the sinus of Valsalva, STJ, and AA were significantly higher in the repaired TOF group than in the volunteer group (p = 0.002, p < 0.001, and p = 0.013, respectively). The FJAs at the STJ and AA were significantly greater in the repaired TOF group (p < 0.001 and p = 0.003, respectively), while velocities and WSS parameters were significantly lower. FD showed no statistically significant difference (p = 0.817). In subgroup analysis, age at TOF repair was significantly higher (p = 0.039) and FJA at the level of the AA significantly greater (p = 0.003) and mean WSS were significantly lower (p = 0.039) in the aortic dilatation group. FD were higher in the aortic dilatation group without statistical significance (p = 0.217). CONCLUSION: Patients with repaired TOF have an increased FJA, dilated AA, and secondarily decreased WSS. In addition to known risk factors, flow eccentricity may affect aortic dilatation in patients with repaired TOF.
Aorta ; Dilatation ; Female ; Hemodynamics ; Humans ; Magnetic Resonance Imaging ; Male ; Risk Factors ; Sinus of Valsalva ; Tetralogy of Fallot ; Volunteers

Child ; Child, Preschool ; Echocardiography ; methods ; Female ; Heart Ventricles ; diagnostic imaging ; pathology ; Humans ; Magnetic Resonance Imaging ; Male ; Tetralogy of Fallot ; diagnostic imaging ; pathology ; Ventricular Dysfunction, Right ; diagnostic imaging ; pathology