Tetralogy of Fallot (TOF) is the most common type of cyanotic congenital heart disease. The physiologic consequences that accompany the disease increase the risk for perioperative complications, and mortality is increased in the mere 3% that survive into adulthood without correction. This case report highlights the anesthetic management of a rare case of uncorrected TOF in a 50-year-old female with two-pillow orthopnea, occasional palpitations, and exertional dyspnea who underwent a major gynecologic procedure. Thorough understanding and careful planning were performed to balance anesthesia, accounting for shifts brought about by the long-term hypoxia and decreased pulmonary blood flow, affording an uneventful perioperative course and recovery.

Tetralogy of Fallot (TOF) is the most common type of cyanotic congenital heart disease. The physiologic consequences that accompany the disease increase the risk for perioperative complications, and mortality is increased in the mere 3% that survive into adulthood without correction. This case report highlights the anesthetic management of a rare case of uncorrected TOF in a 50-year-old female with two-pillow orthopnea, occasional palpitations, and exertional dyspnea who underwent a major gynecologic procedure. Thorough understanding and careful planning were performed to balance anesthesia, accounting for shifts brought about by the long-term hypoxia and decreased pulmonary blood flow, affording an uneventful perioperative course and recovery.

Tetralogy of Fallot (TOF) is the most common type of cyanotic congenital heart disease. The physiologic consequences that accompany the disease increase the risk for perioperative complications, and mortality is increased in the mere 3% that survive into adulthood without correction. This case report highlights the anesthetic management of a rare case of uncorrected TOF in a 50-year-old female with two-pillow orthopnea, occasional palpitations, and exertional dyspnea who underwent a major gynecologic procedure. Thorough understanding and careful planning were performed to balance anesthesia, accounting for shifts brought about by the long-term hypoxia and decreased pulmonary blood flow, affording an uneventful perioperative course and recovery.

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

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

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

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