Project description:Coexistence of divided left atrium with tetralogy of Fallot is rare. Preoperative diagnosis of this rare association is difficult. We here report preoperative diagnosis of this rare combination. In addition, the patient also had coronary to left ventricle fistula.
Project description:BackgroundLittle is known regarding the effect of cardiopulmonary bypass (CPB) reoxygenation on cardiac function following tetralogy of Fallot repair. We hypothesized that hyperoxic reoxygenation would be more strongly associated with myocardial dysfunction in children with tetralogy of Fallot.MethodsWe investigated the association of perfusate oxygenation (PpO2) associated with myocardial dysfunction among children aged 6-72 months who underwent complete repair of tetralogy of Fallot in 2012-2018. Patients were divided into two groups: lower PpO2 group (≤ 250 mmHg) and higher PpO2 (> 250 mmHg) group based on the highest value of PpO2 during aortic occlusion. The odd ratio (ORs) and 95% confidence intervals (CIs) were estimated by logistic regression models.ResultsThis study included 163 patients perfused with lower PpO2 and 213 with higher PpO2, with median age at surgery 23.3 (interquartile range [IQR] 12.5-39.4) months, 164 female (43.6%), and median body mass index 15.59 (IQR 14.3-16.9) kg/m2. After adjustment for baseline, clinical and procedural variables, patients with higher PpO2 were associated with higher risk of myocardial dysfunction than those with lower PpO2 (OR 1.770; 95% CI 1.040-3.012, P = 0.035). Higher PpO2, lower SpO2, lower pulmonary annular Z-score, and longer CPB time were independent risk factors for myocardial dysfunction.ConclusionsAssociation exists between higher PpO2 and myocardial dysfunction risk in patients with tetralogy of Fallot, highlighting the modulation of reoxygenation during aortic occlusion to reduce cardiovascular damage following tetralogy of Fallot repair.Trial registrationClinical Trials. gov number NCT03568357. June 26, 2018.
Project description:Background: Tetralogy of Fallot (TOF) is one of the most common cyanotic congenital heart defects (CHDs). The patterns of fetal myocardial deformations in TOF have not been well-studied. This study aimed to assess biventricular myocardial deformations in fetuses with TOF compared with normal fetuses. Methods: A retrospective cohort study of fetuses with TOF and gestational age (GA)-matched controls was conducted at a single tertiary referral center from 2014 to 2020. All enrolled fetuses underwent detailed echocardiography, and four-chamber video-clips were recorded and analyzed offline for deformation assessment by using two-dimensional speckle tracking echocardiography (2D-STE). Comparisons for baseline characteristics, cardiac morphological measurements (ventricular, atrial, and great arterial diameters or ratios, global sphericity index), systolic function parameters [ejection fraction (EF), fractional area change (FAC)], and strain parameters [global longitudinal strain (GLS), global longitudinal strain rate in systole and diastole (GLSRs, GLSRd)] were performed between fetuses with TOF and GA-matched controls. Results: Fifty-two fetuses with TOF and 52 GA-matched controls were enrolled in this study. Fetuses with TOF exhibited similar left ventricular (LV) EF (58.51 ± 5.11% vs. 57.59 ± 5.38%, P = 0.16) and right ventricular (RV) FAC (43.64 ± 2.89% vs. 44.27 ± 3.04%, P = 0.25), compared to normal fetuses. While, in deformational analysis, TOF fetuses demonstrated significantly lower LV and RV GLS values (-22.57 ± 2.91% vs. -27.39 ± 4.38%, P < 0.001 for LV GLS; -24.27 ± 3.18% vs. -28.71 ± 4.48%, P < 0.001 for RV GLS). Both LV GLS (r = -0.518, P < 0.001) and RV GLS (r = -0.534, P < 0.001) were found negatively correlated with the aortic valve-to-pulmonary valve diameter ratio (AV:PV ratio). Z-scores of PV annulus and main pulmonary artery (MPA) also had positive correlation with LV and RV GLS, respectively. Conclusions: Decreased biventricular myocardial deformations can appear even in fetuses with TOF with normal systolic ventricular function. Both LV and RV GLS values are correlated with the severity of right ventricular outflow tract obstruction. It indicates 2D-STE may be a more sensitive tool to assess fetal cardiac function than the conventional echocardiographic methods.
Project description:A 3-dimensional printed model derived from a computed tomography dataset was obtained in a patient with tetralogy of Fallot and major aortopulmonary collaterals. The virtual and solid 3-dimensional heart models provided intuitive representation of such complex and highly individual pathologies and allowed comprehensive spatial conceptualization of the cardiac structures. (Level of Difficulty: Advanced.).
Project description:Thanks to advances in pediatric cardiology, most infants with tetralogy of Fallot (TOF) now survive into adulthood. This relatively new population of adult patients may face long-term complications, including pulmonary regurgitation (PR), right ventricular (RV) tract obstruction, residual shunts, RV dysfunction, and arrythmias. They will often need to undergo pulmonary valve (PV) replacement and other invasive re-interventions. However, the optimal timing for these procedures is challenging, largely due to the complexity of evaluating RV volume and function. The options for the follow-up of these patients have rapidly evolved from an angiography-based approach to the surge of advanced imaging techniques, mainly echocardiography, cardiac magnetic resonance (CMR), and computer tomography (CT). In this review, we outline the indications, strengths and limitations of these modalities in the adult TOF population.
Project description:AimsTo identify the correlates of focal scar and diffuse fibrosis in patients with history of tetralogy of Fallot (TOF) repair.Methods and resultsConsecutive patients with prior TOF repair underwent electrocardiogram, 24-h Holter, transthoracic echocardiography, exercise testing, and cardiac magnetic resonance (CMR) including cine imaging to assess ventricular volumes and ejection fraction, T1 mapping to assess left ventricular (LV) and right ventricular (RV) diffuse fibrosis, and free-breathing late gadolinium-enhanced imaging to quantify scar area at high spatial resolution. Structural imaging data were related to clinical characteristics and functional imaging markers. Cine and T1 mapping results were compared with 40 age- and sex-matched controls. One hundred and three patients were enrolled (age 28 ± 15 years, 36% women), including 36 with prior pulmonary valve replacement (PVR). Compared with controls, TOF showed lower LV ejection fraction (LVEF) and RV ejection fraction (RVEF), and higher RV volume, RV wall thickness, and native T1 and extracellular volume values on both ventricles. In TOF, scar area related to LVEF and RVEF, while LV and RV native T1 related to RV dilatation. On multivariable analysis, scar area and LV native T1 were independent correlates of ventricular arrhythmia, while RVEF was not. Patients with history of PVR showed larger scars on RV outflow tract but shorter LV and RV native T1.ConclusionFocal scar and biventricular diffuse fibrosis can be characterized on CMR after TOF repair. Scar size relates to systolic dysfunction, and diffuse fibrosis to RV dilatation. Both independently relate to ventricular arrhythmias. The finding of shorter T1 after PVR suggests that diffuse fibrosis may reverse with therapy.
Project description:BackgroundTetralogy of Fallot (TOF) is the most common cyanotic congenital heart defect. Although most infants nowadays undergo surgical repair approximately at the age of 6 months with excellent outcomes, older patients typically underwent a staged approach with an initial systemic-to-pulmonary Blalock-Taussig-Thomas (BTT) shunt reducing hypoxaemia, followed by surgical TOF repair at an older age (with takedown of the BTT shunt). Late complications related to the BTT shunt are rare.Case summaryWe report a case of recurrent haemoptysis late after classic BTT shunt and subsequent surgical TOF repair. Axial imaging indicated a stellate nodule with isotope accumulation in the right upper lung lobe, whereas bronchoscopy showed a rope-like structure in the bronchus to the right upper lung lobe. The patient underwent a lobectomy of the superior right lobe, with identification of what appeared to be the old suture around the BTT shunt. Anatomopathology confirmed diffuse necrotizing inflammation with erosion into the bronchus which eventually caused haemoptysis.DiscussionManagement of older patients with congenital heart disease requires a thorough knowledge of the anatomy and surgical history of the patient. Although late complications related to a BTT shunt are rare, a residual shunt, endocarditis, pseudoaneurysm, or chronic inflammation with haemoptysis may occur.
Project description:BackgroundTetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease. It is generally diagnosed and surgically repaired early in life, with good overall outcomes.Case presentationWe report the case of a patient incidentally diagnosed with paucisymptomatic TOF at the age of 56 years old, during investigations for carbon monoxide poisoning. The patient had a history of thyroidectomy, arterial hypertension, and four uncomplicated vaginal deliveries.ConclusionsThis case shows us that some patients with TOF can reach older ages without surgical correction. Late surgical repair should be meticulously decided on a case basis.
Project description:A 53-year-old woman was referred to our hospital with acute coronary syndrome. The coronary angiography demonstrated a single coronary artery. Culprit lesion was a subtotal occlusion of the proximal left anterior descending coronary artery, which was recanalized and treated with drug-coated balloon angioplasty. The patient recovered quickly after the procedure. A coronary computed tomography angiography visualized the left main coronary artery, which was passing between the ascending aorta and the pulmonary trunk and categorized this anomaly as R-II-B according to Lipton's classification, representing an extremely rare coronary anomaly. <Learning objective: ST-segment elevation myocardial infarction can present with atypical electrocardiographic manifestations. Percutaneous coronary intervention with drug-coated balloon may be considered in patients with relevant coronary stenosis in an anomalous coronary artery.>.