Adult Tetralogy Of Fallot Research Articles

Pilot Study on Integrating 3D-Printed Models into Undergraduate Medical Education for Congenital Heart Disease

BackgroundThis study aimed to assess the feasibility and effectiveness of implementing 3D-printed heart models in the education of medical students in a Canadian Medical School. MethodsThree types of 3D-printed models – normal, unrepaired Tetralogy of Fallot (TOF) and surgically repaired adult TOF – were provided for medical students in their 2nd year of training after a 30-minute lecture. The repaired TOF model depicted a transannular patch, ventricular septal defect closure, and right ventricular outflow tract resection. Knowledge acquisition was measured by comparing pre-and post-session tests consisting of 5 questions. Learner satisfaction ratings were measured with questionnaires completed after the session. A statistical analysis was performed using the t-test. ResultsTwenty-two medical students participated in the study with 20 students submitting complete data for analysis. The students’ score on 5 questions improved after the 3D-printed model session. The average number of correct answers in the test was 2.1 ± 1.0 pre-session and 4.3 ± 0.9 post-session (P < 0.001) out of the possible total of 5. Participants reported a high level of satisfaction (86.4%), understanding (86.4%), and usefulness (90.9%) with the incorporation of 3D-printed models in the educational session. ConclusionsAn educational session which integrated 3D-printed anatomic models to teach congenital heart disease anatomy significantly enhanced knowledge outcomes. Learner assessment of the impact of the use of the 3D models was highly favourable.

Tetralogy of Fallot in adults.

Following surgical repair in infancy, a high proportion of children born with tetralogy of Fallot are surviving into adulthood, and it is important that these patients are monitored by an adult congenital heart disease specialist for problems and complications that can occur in the adult patient. To provide an overview on the management of adult patients with repaired tetralogy of Fallot and emphasise the need for specialist follow-up. Following surgical repair of tetralogy of Fallot, it is important to have lifelong surveillance in adulthood for complications such as pulmonary valve dysfunction. Modern imaging modalities have helped with monitoring and the early detection of problems. Cardiac surgery has been refined over the years, leading to improved longevity. Over the past 23 years, percutaneous interventions have been developed to deal with valve complications and the results have been promising. Healthy behaviour, such as regular exercise, and behaviours to minimise the risk of endocarditis should be encouraged. Regular follow-up with an adult congenital heart disease specialist is important. Patients wishing to undertake a pregnancy should be supervised through a high-risk pregnancy clinic.

A Case Series of Incidentally Detected Uncorrected Tetralogy Of Fallot in Adults

A Case Series of Incidentally Detected Uncorrected Tetralogy Of Fallot in Adults

4D flow MRI-derived energy loss and RV workload in adults with tetralogy of Fallot

PurposeTo assess flow energy loss (EL) pattern inside the pulmonary circulation in adult patients with repaired tetralogy of Fallot (TOF), particularly in TOF with pulmonary stenosis (PS) and pulmonary regurgitation (PR), as a cardiac workload parameter and its relationship to symptoms and major adverse cardiovascular events (MACE). MethodsProspectively, 51 consecutive TOF adults after intracardiac repair, who underwent four-dimensional flow magnetic resonance imaging, were enrolled. All of them had significant PR (PR regurgitant fraction >25 %). TOF patients who had already reached the conventional criteria were excluded. We defined MACE as the following: 1) fatal arrhythmias, 2) sudden cardiac death, 3) surgical pulmonary valvular repair (PVR), 4) right heart failure (HF) needing diuretics and/or hospitalization within 2 years. ResultsA total of 15 patients had MACE; 1) 10 patients underwent PVR within 2 years, 2) 2 patients had ventricular tachycardia, and 3) 6 patients developed right HF (overlapped). Right ventricular (RV) end diastolic volume index (EDVI), RV end systolic volume index (ESVI), average EL/cardiac output (CO), and diastolic EL/CO in patients with MACE were greater than ones without MACE. On a multivariate logistic analysis, the diastolic EL/CO ratio and RVEDVI had the highest odds with MACE in all TOF (odds ratio, 40.7 and 1.15. 95%CI, 1.83–905 and 1.02–13.0; p-value, 0.02 and 0.03). In sub-analysis within 29 patients with moderate PS with PR, and 10 patients with MACE showed higher diastolic EL/CO. Average and diastolic EL/CO negatively correlated with RV ejection fraction (EF) in this sub-analysis. ConclusionsHigh EL, particularly, high diastolic EL/CO, were the important factors for MACE in adult TOF. Higher diastolic EL/CO was also related to lower RV EF and deteriorated RV function in adult TOF with PS and PR. Right-sided EL can be a sensitive marker of excessive cardiac workload which integrates both afterload and preload in adult patients with TOF, beyond the RV size.

4D Flow Cardiovascular Magnetic Resonance-Derived Flow Biomarkers for Early Detection of Flow Abnormalities: A Multicohort New Zealand Based Study

Four-dimensional (4D) flow cardiovascular magnetic resonance (4D flow CMR) imaging has evolved as a versatile imaging technique for the assessment of cardiovascular three-dimensional time resolved haemodynamics. This study aimed to examine the utility and metrics derived from 4D flow CMR in different patient cohorts. Controls (n=26), adult tetralogy of Fallot (rToF; n=14), and left ventricular hypertrophy (LVH; n=10) patients were scanned at the Centre of Advanced MRI, University of Auckland. Quantification of flow and examination of between-group differences were performed. Intraclass correlation coefficients (ICC) were used to correlate 4D and 2D CMR. Forward flow rates averaged in the pulmonary veins were significantly greater in LVH than controls (79.11±54.24 mL/sec vs 32.58±35.98 mL/sec, respectively, p<0.05), as was the pulmonary venous atrial reversal peak velocity (–0.44±0.23 m/sec vs –0.13±0.27 m/sec, respectively, p<0.05). Mitral indices of net flow and peak velocity expressed no significant between-group differences. In the rToF group, quantification of pulmonary artery and branches demonstrated a good correlation with 2D CMR. Greatest correlation was between pulmonary artery backward flow (difference of s–2.50±6.91 mL/cycle, ICC 0.96). In relation to pulmonary valve regurgitation categorisation, 1 of 14 cases were differently categorised using 4D flow (ICC 0.91). Quantification of flow with 4D flow CMR was feasible, with a single acquisition introducing an array of flow metrics that may be valuable for the evaluation of various conditions. Larger scale investigations of 4D flow CMR are required, with wider-scale application in routine clinical workflows.

Right ventricular electromechanical dyssynchrony in adults with repaired Tetralogy of Fallot.

Electromechanical dyssynchrony, manifested by right bundle branch block and regional wall mechanical dysfunction, contributes to inefficient RV function in repaired Tetralogy of Fallot (ToF). This study aims to evaluate the synchronicity of multiple RV walls using two-dimensional multi-plane echocardiography (2D-MPE) in order to augment current understanding of the mechanisms behind RV dyssynchrony. Sixty-nine adult ToF patients [aged 33 (23-45) years; 61% male] and twenty-five matched healthy controls underwent deformational analysis of the RV lateral, anterior, inferior and septal walls following 2D-MPE acquisitions. RV synchronicity was assessed by the intra-RV deformation delay between each basal RV wall and mid-septal segment in addition to mechanical dispersion calculated across four, six and eight segments (MD). All RV wall-septum delays plus MD-4 and MD-6 indices were significantly greater in ToF patients compared to healthy controls (p < 0.001-0.03). In ToF patients, the lateral and anterior RV walls were last to reach peak deformation and anterior wall longitudinal strain was lower (p = 0.001). Post systolic shortening of at least one RV wall segment was identified in 19 (28%) ToF patients. Despite similar ECG characteristics, lateral and anterior wall-septum delays were significantly longer in patients with greater degrees of dyssynchrony (73 [37-108]ms vs. 37 [0-63]ms, p = 0.006; 91 [52-116]ms vs. 41 [1-69]ms, p = 0.013), although RV ejection fraction (RVEF) was not significantly lower. MD-4 and MD-8 indices displayed moderate negative associations with RVEF, strengthened by inclusion of lateral wall longitudinal strain (r = 0.64/0.65; p ≤0.01). RV dyssynchrony in ToF is characterised by electromechanical delays between the lateral, anterior and septal walls, with anterior wall dysfunction likely associated with surgical repair of the RV outflow tract. Prospectively, 2D-MPE may have an emerging role evaluating RV mechanical response to electrical resynchronisation therapy.

Tetralogy of Fallot in Adult – Uncorrected and Rare Presentation: A Case Report

Kular Simran Kaur, Mahesh L Gupta, Hemraj Singh Rajput and Cyril Sajan Author informationArticle notesCopyright and License information Department of Pharmacy, Sumandeep Vidyapeeth (Deemed to be University), Vadodara, Gujarat, INDIA Department of General Medicine, SBKS MI&RC, Dhiraj General Hospital, Sumandeep Vidyapeeth (Deemed to be University), Vadodara, Gujarat, INDIA Corresponding author. *Correspondence: Dr. Kular Simran Kaur

Abstract 14246: Right Atrial Dilation and Conduit Dysfunction is Associated With Significant Pulmonary Insufficiency in Tetralogy of Fallot: Assessment of a New Normative Dataset

Background: Right atrial (RA) size and dysfunction predicts adverse clinical outcomes in adult Tetralogy of Fallot (TOF); however limited normative or TOF data are published in children. We aimed to develop and validate 2-dimensional echocardiography normal values for RA size and function in children, by comparison with the RA of children with TOF and to determine the RA size and functional trajectory in TOF after initial repair. Methods: We prospectively imaged 156 healthy children, 13 days to 18 years, and measured RA area at end systole (RAAs), onset of ECG p wave (RAAp) and at end diastole (RAAd), to calculate total RA emptying fraction (EF) (RAAs - RAAd / RAAs), conduit EF (RAAs - RAAp / RAAs), and active EF (RAAp - RAAd /RAAs). Z-scores were developed using BSA indexing. We analysed studies at &lt;1, 3-4 and 6-7 years of age in 22 patients with TOF with sub-analysis according to degree of pulmonic regurgitation: none to mild (G1 n=7) vs. moderate or greater (G2 n=15). Results: RA area indexed to a best-fit power of BSA was homoscedastic. Normal RA conduit EF increased, and RA active EF decreased until a BSA ~1 m 2 before stabilizing, and was best fit by a linear spline function, node at BSA 1 m 2 (Figure 1). TOF RA was dilated early after repair and normalized by 4 years in G1 but not G2. Total RA EF and conduit EF, but not active EF were persistently reduced (Table 1). Conclusion: Using new z-score formulas for indexed RA size and EF, we demonstrate persistent RA dilation and conduit dysfunction in TOF is associated with significant pulmonary regurgitation. Future work will explore the potential utility of RA size and function in prediction of right heart normalization post-pulmonary valve replacement.

Health-Related Quality of Life After Single Ventricle Palliation or Tetralogy of Fallot Repair.

Single ventricle (SV) cardiac lesions and tetralogy of Fallot (TOF) are both common forms of cyanotic congenital heart disease. With advances in perioperative care and longitudinal follow-up, survival of these patients has dramatically improved and the majority survive to adulthood. This study compares health-related quality of life (HRQoL) of adult SV and TOF patients to each other and the general population. HRQoL of all surviving, non-transplanted SV and TOF patients 21years of age and older at our institution was assessed with the SF-36 questionnaire via phone. Additional data including demographic parameters and information related to comorbidities and healthcare utilization were also analyzed. Among 81 eligible SV patients and 207 TOF patients, 33 (41%) and 75 (36%) completed the SF-36 phone survey, respectively. The mean age of SV patients was 32 vs. 38 years in the TOF group (p=0.01). SV patients reported more hepatic, pulmonary, and renal comorbidities. TOF patients were more likely to complete advanced degrees and more likely to have children (p=0.03). SV physical functioning scores were worse compared to TOF. In other domains of the SF-36 questionnaire, SV and TOF scores were similar. Compared to the general population, both groups reported worse bodily pain and mental health, but other aspects of psychosocial and general health were comparable. Overall HRQoL is good for both SV and TOF patients through early and mid-adulthood. Some QoL metrics were modestly worse in the SV patients. While these patients may have some physical limitations, psychosocial wellbeing appears preserved.

Utility of Cardiac Magnetic Resonance Imaging in Predicting Atrial Arrhythmias in Repaired Tetralogy of Fallot

Arrhythmias are the leading cause of morbidity and mortality in repaired tetralogy of Fallot (TOF), and over 20% of these patients will develop a sustained atrial arrhythmia during their lifetimes. Cardiac magnetic resonance imaging (cMRI) is frequently performed in TOF, although its ability to identify patients at risk of atrial arrhythmias is uncertain. Adult TOF patients (n=175) with no history of atrial arrhythmia who underwent cMRI between 2003 and 2020 at a single tertiary care center were identified. Clinical characteristics and imaging findings were evaluated to identify a predilection for atrial arrhythmias using Kaplan-Meier survival analysis and log-rank testing. Multivariable Cox regression was used to determine independent predictors of atrial arrhythmias. Over a median follow-up of 3.6years, 29 patients (17%) developed atrial arrhythmias. Independent predictors of atrial arrhythmia included age (hazard ratio [HR] 1.06 per 1-year increase, 95% confidence interval [CI] 1.02 to 1.09, p=0.002), diabetes mellitus (HR 4.26, 95% CI 1.26 to 14.41, p=0.020), indexed right ventricular end-diastolic volume (RVEDVi), (HR 1.20 per 10-ml/m2 increase, 95% CI 1.05 to 1.39, p=0.010), and moderate or greater tricuspid regurgitation (TR) (HR 6.32, 95% CI 2.15 to 18.60, p=0.001). Utilizing Kaplan-Meier analysis, patients with at least mild right ventricular dilation (RVEDVi >100ml/m2, p=0.047) and greater than or equal to moderate TR (p <0.001) were found to be significantly more likely to develop atrial arrhythmias. In conclusion, cMRI can help to identify TOF patients at increased risk for atrial arrhythmia beyond standard clinical and imaging data by better quantifying RVEDVi and degree of TR.

Predictive factors of outcome in patients with Tetralogy of Fallot (TOF) after pulmonary valve replacement: preliminary data of our Centre experience (2010-2020)

Abstract Funding Acknowledgements Type of funding sources: None. Background TOF patients undergo multiple surgical procedures during their life. The improvement in surgical techniques and patient’s clinical management led to longer life expectancy, creating an adult TOF population that experiences specific adverse events, such as atrial/ventricular arrythmias, heart failure and sudden cardiac death. In selected patients, pulmonary valve replacement (PVR) is mandatory to reduce right ventricular (RV) volume; therefore, careful patients’ selection but also correct timing of PVR are crucial. Purpose Current guidelines are based on pre-PVR threshold values of indexed RV end-diastolic volume (RVEDVi) calculated with cardiac magnetic resonance (CMR), to achieve normalization of RV size after valve implantation, but recent studies demonstrated that post-PVR RV dilatation is not so tightly connected with adverse clinical outcomes. Therefore, our aim is to identify new possible predictive factors of post-PVR outcome to provide tailored PVR on the correct cohort of patients, at the correct time. Methods: The inclusion criteria of this retrospective and partially prospective cohort study were i) repaired TOF; ii) available CMR data pre- and post-PVR; iii) PVR performed between 2010 and 2020; iv) clinical and ECG available pre- and post-PVR; v) complete echocardiographic examination pre- and post-PVR. Multivariate analysis was applied. Primary outcomes were considered death, resuscitated death, atrial/ventricular arrhythmias. NYHA class was considered to evaluate patients’ clinical status. CMR Late Gadolinium Enhancement (LGE) score was calculated pre- and post-PVR. Results 22 patients were selected (12 males, median age ± SD 28,25 ± 11,2 years). After PVR, the only primary outcome observed was sustained ventricular tachycardia in 4 patients. Multivariate analysis showed that patients with later PVR, lower Fractional Area Change (FAC) at pre-PVR echocardiogram and higher LGE score post-PVR (LGE &amp;gt; 3) showed higher post-PVR RVEDVi. On the contrary, patients with class I NYHA post-PVR showed lower RVEDVi. Conclusion Our study demonstrates that lower age at PVR and careful patient’s selection, guided by echocardiographic and CMR findings, lead to greater benefit in terms of good volumetric recovery of the RV and better performance status post-PVR (NYHA class I) in TOF patients after PVR.

Right Ventricular Flow Vorticity Relationships With Biventricular Shape in Adult Tetralogy of Fallot.

Remodeling in adults with repaired tetralogy of Fallot (rToF) may occur due to chronic pulmonary regurgitation, but may also be related to altered flow patterns, including vortices. We aimed to correlate and quantify relationships between vorticity and ventricular shape derived from atlas-based analysis of biventricular shape. Adult rToF (n = 12) patients underwent 4D flow and cine MRI imaging. Vorticity in the RV was computed after noise reduction using a neural network. A biventricular shape atlas built from 95 rToF patients was used to derive principal component modes, which were associated with vorticity and pulmonary regurgitant volume (PRV) using univariate and multivariate linear regression. Univariate analysis showed that indexed PRV correlated with 3 modes (r = −0.55,−0.50, and 0.6, all p < 0.05) associated with RV dilatation and an increase in basal bulging, apical bulging and tricuspid annulus tilting with more severe regurgitation, as well as a smaller LV and paradoxical movement of the septum. RV outflow and inflow vorticity were also correlated with these modes. However, total vorticity over the whole RV was correlated with two different modes (r = −0.62,−0.69, both p < 0.05). Higher vorticity was associated with both RV and LV shape changes including longer ventricular length, a larger bulge beside the tricuspid valve, and distinct tricuspid tilting. RV flow vorticity was associated with changes in biventricular geometry, distinct from associations with PRV. Flow vorticity may provide additional mechanistic information in rToF remodeling. Both LV and RV shapes are important in rToF RV flow patterns.

Intracardiac Repair in Late Adolescent and Adult Tetralogy of Fallot - Early and Midterm Results from a Tertiary Care Centre.

IntroductionIn developing countries like India, it is common for late presentation of Tetralogy of Fallot (TOF) patients to a hospital as compared to that of developed countries. The objective of this study is to analyze the surgical outcome of TOF patients with age > 15 years.MethodsThis is a retrospective descriptive study of the surgical outcomes of 45 adult patients undergoing correction for TOF. Epidemiology, symptomology, and preoperative evaluation were performed.ResultsMost of the patients were male (33 [73%]). The median age was 21 years. A total of 42 (93.33%) patients had subaortic ventricular septal defect (VSD), while three (6.6%) patients presented with doubly committed VSD. The most common type of right ventricular outflow tract (RVOT) obstruction was combined infundibular and valvular types, accounting for 34 cases (75.5%). Six patients had infundibular RVOT obstruction, while three patients (6.6%) had predominantly valvular pulmonary stenosis. We performed trans-right atrial repair in 33 patients. Right atrium-pulmonary artery approach was used in five patients (11.1%). The most common postoperative complication was right bundle branch block, seen in 14 patients, with a mortality rate of 2% in the early postoperative period. We achieved excellent early and midterm survival results and significant improvement in functions and disease-free quality of life.ConclusionIntracardiac repair in adult TOF can be performed with low mortality, less residual RVOT obstruction, and need for revision of RVOT far less frequent by using the Jhajhria Infundibular Resection Adequacy Assessment technique (JIRAAT) to assess for adequacy of infundibular resection.

Flow vorticity relationships with right ventricular geometry in adult tetralogy of Fallot

Abstract Background Repaired tetralogy of Fallot adults (rToF) undergo right ventricular (RV) remodeling, in part due to volume overload of residual pulmonary regurgitation volume (PRV). Time-resolved phase-contrast cardiac magnetic resonance imaging (4D Flow MRI) enables the qualitative and quantitative measurement of altered blood flow patterns, including vorticity. Cardiac atlases allow for complex three-dimensional heart shapes to be expressed as morphometric scores. Those scores show the extent of geometrical shift and can help explore uncharted relationships between vorticity and architecture. Purpose We aimed to quantify vorticity, incorporating deep learning to enhance 4D Flow data, and correlate this with global cardiac parameters and morphometric scores. Methods 12 Adult rToF patients and 10 age-matched controls underwent 4D flow MRI and cine imaging. RV interventricular vorticity was calculated for outflow and inflow tracts. EDV, ESV and SV were computed from cines which were also used to build three-dimensional shape models. The biventricular models were projected onto an atlas generated from 95 rToF patients, and twenty-one principal component analysis shape modes were correlated with cardiac metrics and vorticity to identify global shape variations. Association between biventricular shape and vorticity was further analysed using multivariate multiple regression models. Results Strong correlation was found between PRV and the right ventricular outflow tract (RVOT) vorticity. PRV and RVOT vorticity both correlated with the same 3 shape modes (r=−0.55, −0.50 and 0.6 (p&amp;lt;0.05) respectively for PR and r=0.63, −0.82 and 0.60 (p&amp;lt;0.05) respectively for vorticity) i.e., the RV dilates with an increase in basal bulging, apical bulging and tricuspid annulus tilting with more severe regurgitation, as well as a smaller LV, and a paradoxical movement of the septum (Figure 1). However, RV vorticity correlated with 2 modes that did not correlate with PRV, (r=−0.62, −0.69, p&amp;lt;0.05). With higher vorticity the RV was longer, increased tilting of the tricuspid annulus and an increased basal bulge around the tricuspid area. The multivariate analysis model demonstrated that higher vorticity was associated with displacement of the pulmonary valve and change in the RVOT length and direction. A septal displacement towards the left ventricle was observed and increased apical flatness of the RV (Figure 1). Qualitatively, vorticity in rToF group was more heterogeneous than controls (Figure 2). Conclusions Vorticity is a novel marker based on the influence of blood motion providing new insight into early diagnosis and prognosis of cardiac disease. This is the first study to examine the relationships between vorticity and regional RV shape changes in rToF. Mode associations with vorticity were different to associations with PRV. More longitudinal studies are required for standardization of change in vorticity with the disease process. Funding Acknowledgement Type of funding sources: Foundation. Main funding source(s): The New Zealand heart foundation Mode variations and morphometric modelVorticity visualization and analysis

PULMONARY HYPERTENSION AS AN INITIAL PRESENTATION OF UNCORRECTED TETRALOGY OF FALLOT IN A 27-YEAR-OLD

TOPIC: Pulmonary Vascular Disease TYPE: Medical Student/Resident Case Reports INTRODUCTION: Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease (CHD) affecting children. It is characterized by ventricular septal defect (VSD), an overriding aorta, pulmonary stenosis or atresia, and right ventricular hypertrophy. Historically, severity of RV outflow obstruction determined the need for surgical intervention and only 3% of patients with unrepaired TOF reached the age of 40. With advances in early surgical repair and excellent surgical outcomes, survival 25 years after TOF repair has reached more than 94% in the United States and TOF is rarely left unrepaired, however, pulmonary arterial hypertension (PAH) continues to be a major complication in both repaired and unrepaired cases. CASE PRESENTATION: Case: A 27-year-old Vietnamese immigrant presented with complaints of hemoptysis, dyspnea, orthopnea, decreased exercise tolerance, and congestion. In the emergency department he was febrile, normotensive, non-tachycardic, cyanotic and hypoxic with an oxygen saturation of 79%. Investigations were significant for abnormal chest X-rays, leukocytosis to 19 and polycythemia to 20. He was treated for community-acquired pneumonia. Computed tomography angiogram of the chest showed the features of TOF with direct arterial communications to both lungs associated with the descending aorta. Echocardiogram showed an ejection fraction of 55%, an overriding aorta, a dilated aortic root (5cm), severely elevated pulmonary artery systolic pressure, VSD with right to left shunt, and tricuspid regurgitation velocity of 4.3m/s suggesting a Right Ventricle systolic pressure of 85 mmHg. He was diagnosed with unrepaired TOF with pulmonary valve atresia and major aortopulmonary collateral arteries (MAPCAs). Right and left heart catheterization showed a mean pulmonary artery pressure of 68 mmHg and pulmonary artery wedge pressure 14. He was diagnosed with PAH and was started on Bosentan therapy. His symptoms and exercise tolerance significantly improved. His follow-up visit saturation was 84%. DISCUSSION: Patients with TOF usually have systemic-pulmonary shunting. This increased flow leads to increased pressure and progressive endothelial dysfunction which results in arterial remodeling/PAH. Early surgical repair in CHD limits the amount of vascular remodeling and development of PAH. However, up to 15% of patients develop PAH even after surgical repair. There is a high mortality if untreated. CONCLUSIONS: In this case of TOF with MAPCAS and Eisenmenger syndrome, the endothelin receptor antagonist Bosentan was used with success. PAH can be the initial presentation of adult TOF. REFERENCE #1: John Willis Hurst, et al. Hurst's the Heart. 14th ed., vol. 2, New York, McGraw-Hill Medical, 2011, pp. 1380–1381. REFERENCE #2: https://onlinelibrary.wiley.com/doi/pdf/10.1002/clc.4960070807 REFERENCE #3: https://jamanetwork.com/journals/jamacardiology/fullarticle/2718499#:~:text=Tetralogy%20of%20Fallot%20%28TOF%29%20is%20the%20most%20common, or%20variation%20in%20population%20or%20health%20care%20systems. DISCLOSURES: No relevant relationships by Moises Cossio, source=Web Response No relevant relationships by Fernando Fuentes, source=Web Response No relevant relationships by Mohammad Nimer, source=Web Response

Pulmonary valve replacement in primary repair of tetralogy of Fallot in adult patients.

BackgroundAdults with unrepaired tetralogy of Fallot (ToF) are common in developing countries. Long-term overload of the right ventricle places adult patients at risk for postoperative right heart failure after primary repair, which contributes to morbidity and mortality. The effect of pulmonary valve replacement (PVR) in reducing postoperative morbidity and mortality in adults has never been validated.MethodsWe conducted a retrospective cohort study in adults (age ≥18 years) with ToF undergoing primary repair from January 2014 to December 2019 at our institution. Patients were divided into three groups according to techniques used to enlarge the right ventricle outflow tract (RVOT). Baseline variables and perioperative outcomes were collected. The primary endpoint was operative mortality. Secondary endpoints were incidences of right heart failure and stage 3 acute kidney injury (AKI).ResultsA total of 56 patients were enrolled (mean age 41.5±11.7 years, 30 females, 53.6%). They were divided into three groups designated as the following: TA-PVR group for trans-annular patch enlargement with PVR; TA group for trans-annulus patch enlargement without PVR; and group AP for annulus preservation. Four patients (7.1%) died postoperatively, all due to right heart failure. All twelve patients in the TA-PVR group survived. There was no significant difference in mortalities among groups. Ten patients (17.9%) developed right heart failure after surgery with no significant difference among groups. Three patients (5.4%) developed stage 3 AKI after surgery, none belonging to the TA-PVR group, however, not statistically significant.ConclusionsRight heart failure is a common complication after primary repair of adult ToF. Trans-annulus patch enlargement should be cautiously selected in this population. PVR with trans-annulus patch enlargement may be a promising technique to protect against postoperative right heart failure and mortality when annulus preservation is not feasible.

Tetralogy of Fallot: cardiac imaging evaluation.

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.

Prognostic implications of inferior vena cava haemodynamics in ambulatory patients with tetralogy of Fallot.

AimsRight atrial pressure (RAP) provides a composite measure of right ventricular diastolic dysfunction, right atrial compliance, and volume status, and these three variables are typically abnormal in adults with repaired tetralogy of Fallot (TOF). RAP is a well‐established prognostic metric in patients with pulmonary hypertension, and recent data suggest that RAP is associated with clinical outcomes in TOF. The purpose of this study was to determine the role of inferior vena cava (IVC) haemodynamics (size and collapsibility) for the assessment of RAP and its potential application for risk stratification and prognostication in the TOF population.Methods and resultsAdult TOF patients with echocardiographic assessment of IVC haemodynamics were divided into patients with (derivation cohort, n = 256) and without (validation cohort, n = 492) cardiac catheterization data. We assessed the correlation between IVC haemodynamics, RAP, and disease severity indices [arrhythmias, peak oxygen consumption (VO2), and heart failure hospitalization] in derivation cohort and compared it with the correlations in the validation cohort. IVC haemodynamics correlated with RAP (r = 0.52, P < 0.001), with disease severity indices {atrial arrhythmias [area under the curve (AUC) 0.81], ventricular arrhythmias [AUC 0.67], heart failure hospitalizations [AUC 0.78], and peak VO2 [r = 0.53]}, and with transplant‐free survival in the derivation cohort. Similar correlations between IVC haemodynamics, disease severity indices, and transplant‐free survival were also observed in the validation cohort.ConclusionsThese findings suggest that IVC haemodynamics can potentially be used for risk stratification and prognostication in TOF patients and can complement the current risk models that are based predominately on right ventricular volumes and systolic function.

PREOPERATIVE IMAGING PARAMETERS AND EARLY POSTOPERATIVE OUTCOMES IN ADULT PATIENTS WITH TETRALOGY OF FALLOT

Tetralogy of Fallot (TOF) is the most common form of cyanotic congenital heart disease with the majority of patients requiring additional surgical interventions in adulthood. We examined preoperative imaging in adult TOF patients to identify factors impacting early postoperative morbidity and

Left ventricular filling pressure in Tetralogy of Fallot: Correlation between invasive and noninvasive indices

BackgroundLeft heart filling pressures, as measured by pulmonary artery wedge pressure (PAWP), is associated with heart failure related mortality. Because of the prognostic importance of PAWP, several echocardiographic indices have been proposed for noninvasive assessment of PAWP. However, these indices have not been validated in the congenital heart disease population. The purpose of this study was to determine the correlation between echocardiographic indices of PAWP, and the effect of high PAWP on transplant-free survival in adults with tetralogy of Fallot (TOF). MethodsRetrospective study of adult TOF patients that underwent cardiac catheterization at Mayo Clinic, 1990–2017. We selected these pre-defined set of echocardiographic indices of LV diastolic function: mitral valve early velocity (E), mitral valve early and late velocity ratio (E/A), mitral valve deceleration time (DT), mitral annular tissue Doppler early velocity (e′), and left atrial volume index (LAVI). ResultsOf the echocardiographic indices analyzed among 213 patients (age 37 ± 14 years), only E velocity (β = 5.83, standard error = 1.52, p < 0.001) and LAVI (β = 0.14, standard error = 0.05, p = 0.007) correlated with PAWP. LAVI > 28 ml/m2 had sensitivity of 79% and specificity of 63% (AUC 0.712), and E velocity > 1.0 m/s had sensitivity of 66% and specificity of 89% (AUC 0.692), for detecting PAWP > 15 mmHg. LAVI > 28 ml/m2 was associated with reduced 10-year transplant-free survival (49% vs 90%, p < 0.001). ConclusionThis study supports the use of LAVI for noninvasive assessment of PAWP and for prognostication. Further studies are required to validate these results in a different population.