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Background: The impact of pulmonary valve replacement (PVR) on major adverse clinical outcomes in patients with repaired tetralogy of Fallot (rTOF) is unknown. Objectives: The purpose of this study was to determine whether PVR is associated with improved survival and freedom from sustained ventricular tachycardia (VT) in rTOF. Methods: A PVR propensity score was created to adjust for baseline differences between PVR and non-PVR patients enrolled in INDICATOR (International Multicenter TOF Registry). The primary outcome was time to the earliest occurrence of death or sustained VT. PVR and non-PVR patients were matched 1:1 on PVR propensity score (matched cohort) and in the full cohort, modeling was performed with propensity score as a covariate adjustment. Results: Among 1,143 patients with rTOF (age 27 ± 14 years, 47% PVR, follow-up 8.3 ± 5.2 years), the primary outcome occurred in 82. The adjusted HR for the primary outcome for PVR vs no-PVR (matched cohort n = 524) was 0.41 (95% CI: 0.21-0.81; multivariable model P = 0.010). Full cohort analysis revealed similar results. Subgroup analysis suggested beneficial effects in patients with advanced right ventricular (RV) dilatation (interaction P = 0.046; full cohort). In patients with RV end-systolic volume index >80 mL/m2, PVR was associated with a lower primary outcome risk (HR: 0.32; 95% CI: 0.16-0.62; P < 0.001). There was no association between PVR and the primary outcome in patients with RV end-systolic volume index ≤80 mL/m2 (HR: 0.86; 95% CI: 0.38-1.92; P = 0.70). Conclusions: Compared with rTOF patients who did not receive PVR, propensity score-matched individuals receiving PVR had lower risk of a composite endpoint of death or sustained VT.

Over the past five decades, there have been multiple advances in the treatment of congenital heart defects, resulting in an increasing population of adults living with congenital heart disease (CHD). Despite improved survival, CHD patients often have residual haemodynamic sequelae and limited physiologic reserve and are at increased risk for acute decompensation with occurrence of arrhythmias, heart failure, and other medical conditions. Comorbidities occur more frequently and at an earlier age in CHD patients than in the general population. The management of the critically ill CHD patient requires an understanding of the unique aspects of congenital cardiac physiology as well as the recognition of other organ systems that may be involved. Certain patients may be candidates for mechanical circulatory support, and goals of care should be established with advanced care planning.

The American Heart Association and American College of Cardiology published practice guidelines for the management of adult congenital heart disease in 2018 and the European Society of Cardiology published analogous guidelines in 2020. Although there are broad areas of consensus between the 2 documents, there are important differences that impact patient management. This review discusses key areas of agreement and disagreement between the 2 guidelines, with discussion of possible reasons for disagreement and potential implications.

A novel approach to fabricating Nb3Sn SRF cavities and other RF components using an ultra-low-cost melt casting fabrication process has been investigated. This simple, low cost melt casting technique has the potential to be used to fabricate nearly any superconducting Nb3Sn structure using either the Bronze Route (BR), Internal Tin (IT), External Tin (ET) processes as well as normal conducting pure copper (Cu) cavities. Most of the heat treated samples that were examined using SEM/EDX seemed to show a reasonable correlation of Tc onset and transition width (ΔTc) of the ingots initial/starting Sn content to the desired stoichiometric Nb3Sn phase, where the lower starting Sn content coupons resulted in lower Tc's with broader transition widths and the higher starting Sn coupons resulted in higher Tc's with narrower ΔTc's. The best samples had Tc onsets ∼17–18 K and ΔTc's < 2 K. Two samples were further tested for RF surface resistance (Rs) and Quality Factor (Q) at JLAB. These RF measurements were performed at 7.4 GHz using a calorimetric technique and showed two transitions, one at ∼8 K and another at ∼14 K; values of Rs were at least two orders of magnitude higher than similar high quality Nb3Sn films directly deposited substrates by JLAB and with another superconducting transition close to the superconducting transition temperature of niobium. Substantial improvements in the processing variables ranging from higher quality of the initial ingots with higher Sn content, to better electro-polishing and reaction heat treatment regimens will be necessary to realize improved RF performance metrics.

Background In repaired tetralogy of Fallot (rTOF), abnormal left ventricular (LV) rotational mechanics are associated with adverse clinical outcomes. We performed a comprehensive analysis of LV rotational mechanics in rTOF patients using cardiac magnetic resonance (CMR) prior to and following surgical pulmonary valve replacement (PVR). Methods In this single center retrospective study, we identified rTOF patients who (1) had both a CMR ≤ 1 year before PVR and ≤ 5 years after PVR, (2) had no other intervening procedure between CMRs, (3) had a body surface area > 1.0 m2 at CMR, and (4) had images suitable for feature tracking analysis. These subjects were matched to healthy age- and sex-matched control subjects. CMR feature tracking analysis was performed on a ventricular short-axis stack of balanced steady-state free precession images. Measurements included LV basal and apical rotation, twist, torsion, peak systolic rates of rotation and torsion, and timing of events. Associations with LV torsion were assessed. Results A total of 60 rTOF patients (23.6 ± 7.9 years, 52% male) and 30 healthy control subjects (20.8 ± 3.1 years, 50% male) were included. Compared with healthy controls, rTOF patients had lower apical and basal rotation, twist, torsion, and systolic rotation rates, and these parameters peaked earlier in systole. The only parameters that were correlated with LV torsion were right ventricular (RV) end-systolic volume (r = − 0.28, p = 0.029) and RV ejection fraction (r = 0.26, p = 0.044). At a median of 1.0 year (IQR 0.5–1.7) following PVR, there was no significant change in LV rotational parameters versus pre-PVR despite reductions in RV volumes, RV mass, pulmonary regurgitation, and RV outflow tract obstruction. Conclusion In this comprehensive study of CMR-derived LV rotational mechanics in rTOF patients, rotation, twist, and torsion were diminished compared to controls and did not improve at a median of 1 year after PVR despite favorable RV remodeling.

Background Pregnant women with repaired tetralogy of Fallot (rTOF) are at increased risk of adverse outcomes. Although pre-pregnancy pulmonary valve replacement (PVR) may be considered in some women to attenuate risk, published data to support this practice are lacking. Our objective was to explore the impact of pre-pregnancy PVR on pregnancy outcomes in rTOF. Methods Women with rTOF and cardiovascular magnetic resonance imaging (CMR) before and after pregnancy were included if CMR studies were completed within 3 years of pregnancy. Subjects were compared according to presence (+) or absence (−) of PVR at pre-pregnancy CMR. Pregnancy outcomes (cardiovascular, obstetric, and fetal/neonatal) were documented. Results Of the 29 study women identified, 7 were PVR+ and 22 were PVR-. Post-pregnancy, the PVR- group demonstrated interval increase in indexed right ventricular end-diastolic volumes (RVEDVi) (157 ± 28 versus 166 ± 33 ml/m2, p = 0.003) and end-systolic volumes (RVESVi) (82 ± 17 versus 89 ± 20 ml/m2, p = 0.003) as compared with pre-pregnancy, but no significant change in RV ejection fraction, RV mass, or left ventricular measurements. In the PVR+ group, there were no interval changes in RV measurements pre-versus post pregnancy. Interval rate of change in RVESVi of PVR- exceeded PVR+ women (+3.7 ± 5.0 versus −2.2 ± 5.0 ml/m2/year, p = 0.03). Pregnancy outcomes did not differ in PVR+ versus PVR- women. Conclusions Pregnancy outcomes did not differ according to PVR status in our cohort. While RV volumes remained unchanged in PVR+ women, interval RV dilation was observed in PVR- women. Additional study of a larger population with longer follow-up may further inform clinical practice regarding pre-pregnancy PVR.

Individuals with single-ventricle congenital heart disease who are palliated to a Fontan circulation are at risk for heart failure and liver disease, with recurrent ascites being a potentially debilitating cause of late morbidity. Although ascites associated with heart failure or liver failure is usually characterized by a high serum-ascites albumin gradient (SAAG), we have observed multiple instances of ascites in Fontan patients with low SAAG, suggesting an inflammatory process. We present three cases in which recalcitrant ascites severely and adversely impacted the quality of life and describe our initial experience with intraperitoneal corticosteroids in this setting.

Background The Anatomic and Physiological (AP) classification system proposed in the 2018 American College of Cardiology/American Heart Association adult congenital heart disease (ACHD) guidelines assigns 2 dimensions to each patient: anatomic class (AnatC) and physiological stage (PhyS). This approach has not been tested in practice; we assessed interrater reliability and identified sources of disagreement. Methods Consensus definitions for AP categories were developed with input from 4 experts. Research assistants (RAs) assigned AnatC/PhyS for patients in the Boston ACHD Biobank, a prospectively enrolled cohort of ambulatory ACHD patients ≥18 years old seen between 2012 and 2019. Two (of 4) expert reviewers then independently assigned AnatC/PhyS for 41 patients. Interrater reliability was assessed with linearly weighted kappa (κω) for agreement between (1) experts and (2) an RA and an expert. Experts examined disagreements and identified sources of variability and areas requiring clarification. Results Interexpert agreement for AnatC was excellent, with agreement on 38/41 (92.7%) cases and κω 0.88 [0.75, 1.01]. Agreement for PhyS was less robust, with consensus on 24/41 cases (59.5%), κω 0.57 [0.39, 0.75]. Expert-RA agreement was lower for AnatC (κω 0.77 [0.60, 0.95]), whereas PhyS was similar to interexpert agreement (κω 0.53 [0.34, 0.72]). There was ambiguity in the definitions of (1) arrhythmia status, (2) cyanotic CHD, and (3) valve disease. Conclusions Although AnatC can be assessed reliably, that is not true for the PhyS part of the AP classification proposed in the 2018 American College of Cardiology/American Heart Association guidelines. Reliability of PhyS would be strengthened by more precise definitions readily interpretable in clinical practice.

Increasing numbers of women with repaired tetralogy of Fallot (rTOF) are reaching reproductive age and seek counseling regarding their cardiovascular risks related to pregnancy. Therefore, the aim of this study was to characterize changes in left ventricular (LV) strain in women with rTOF during pregnancy and in the postpartum period. Seventeen pregnancies in women with rTOF were included (mean age at repair = 3.2 years ± 5.5 years; mean age at delivery = 32.7 ± 4 years). Echocardiograms from three time periods were analyzed; baseline (prior to conception or in the first trimester), third trimester, and 4–6 weeks postpartum. Sixty-five percent of the patients had at least mild pulmonary regurgitation. Eight patients (47%) had undergone at least one pulmonary valve replacement. There were no changes in LV ejection fraction (EF) or circumferential strain across the three time periods. Significant differences were present in longitudinal strain within the three time points (p = 0.01). Postpartum strain decreased in magnitude compared to the third trimester value (− 17.7 ± 4.1 vs. − 21 ± 5, p-value = 0.003) but was not different when compared to baseline strain (− 17.7 ± 4.1 vs. − 19.4 ± 3.4; p-value = 0.15). In conclusion, in women with rTOF, changes in longitudinal strain were observed during pregnancy with a return to baseline after delivery; EF did not change. These findings provide evidence that pregnancy does not adversely impact LV mechanics in the short term in this potentially vulnerable patient population.

Repaired tetralogy of Fallot is one of the most common conditions managed by adult congenital heart disease providers. Recent comprehensive review articles and book chapters are devoted to this topic. The purpose of this article is to address several common clinical questions encountered in the management of patients with repaired tetralogy of Fallot. These answers are not intended to supplant Practice Guidelines.

Increasing numbers of women with congenital heart disease are undergoing pregnancy after transcatheter pulmonary valve replacement (TPVR). We present the course of 9 pregnancies in 7 women with TPVR, noting pre-pregnancy, antepartum, and postpartum gradients, as well as maternal cardiac, obstetric, and neonatal outcomes. (Level of Difficulty: Intermediate.), Graphical abstract, Increasing numbers of women with congenital heart disease are undergoing pregnancy after transcatheter pulmonary valve replacement (TPVR). We present…

Background Longitudinal clinical surveillance by transthoracic echocardiography (TTE) is an established practice in children with repaired tetralogy of Fallot (TOF). Non-Invasive Imaging Guidelines recommends a list of reporting elements that should be addressed during routine TTE in this population. In this study, we assessed the adherence to these recommendations. Methods This was a multi-center (n = 8) retrospective review of TTE reports in children ≤11 years of age who have had complete TOF repair. We included 10 patients from each participating center (n = 80) and scored 2 outpatient follow-up TTE reports on each patient. The adherence rate was based on completeness of TTE reporting elements derived from the guidelines. Results We reviewed 160 TTE reports on 80 patients. Median age was 4.4 months (IQR 1.5-6.6) and 3.6 years (IQR 1.3-6.4) at the time of complete surgical repair and first TTE report, respectively. The median adherence rate to recommended reporting elements was 61% (IQR 53–70). Of the 160 reports, 9 (7%) were ≥80% adherent and 40 (25%) were ≥70% adherent. Quantitative measurements of right ventricular outflow tract (RVOT), right ventricular (RV) size and function, and branch pulmonary arteries were least likely to be reported. Conclusions Overall adherence to the most recent published imaging guidelines for surveillance of children with repaired TOF patients was suboptimal, especially for reporting of RVOT, RV size and function, and branch pulmonary arteries. Further studies are needed to explore the barriers to adherence to guidelines and most importantly, whether adherence is associated with clinical outcomes.

The rationale for timing of pulmonary valve replacement (PVR) in patients with repaired Tetralogy of Fallot (rTOF) has focused on pre-PVR threshold values of indexed right ventricular end-diastolic volume (RVEDVi) that lead to normalization of right ventricular (RV) size after valve implantation. The goal of this study was to determine whether persistent RV dilation after PVR is associated with adverse clinical outcomes. Subjects with rTOF who underwent PVR and had a cardiac magnetic resonance (CMR) exam after valve implantation at a single center from 2001 to 2017 were included. The composite clinical outcome after PVR included: death, aborted sudden cardiac death, sustained ventricular tachycardia (VT), or NYHA class ≥3. In 189 rTOF subjects, the mean age at PVR was 23.5 ± 11.7 years, median follow-up was 6.0 years (IQR 3.4 to 8.7), and the primary outcome occurred in 14 subjects (7%). The 5- and 10-year event-free rates were 97% and 91%, respectively. Post-PVR RVEDVi was not associated with the composite outcome (p = 0.59). Independent predictors of the outcome were older age at PVR (hazard ratios [HR] 1.06; 95% confidence interval [CI] 1.02 to 1.11; p = 0.004), post-PVR lower RV ejection fraction (HR 0.91; 95% CI 0.86 to 0.97; p = 0.002), and post-PVR atrial tachyarrhythmia (HR 7.60, 95% CI 1.65 to 35.05, p = 0.009). Our study shows that post-PVR RV dilation as measured by CMR-derived RVEDVi was not associated with the composite adverse clinical outcome in this cohort. These findings challenge the validity of current guidelines for PVR, which are based on pre-procedural threshold values of RVEDVi aimed at achieving normal post-procedural RV volumes.

Background: Risk stratification in patients with repaired tetralogy of Fallot (rTOF) have focused on poor clinical outcomes while predictors of a benign clinical course have not been characterized. Objective: The goal of this study was to Identify cardiac magnetic resonance (CMR) markers of a good clinical course late after TOF repair. Methods: Clinical and CMR data from the International Multicenter TOF Registry (INDICATOR) were analyzed. The primary outcome was time to the earliest occurrence of a composite of death, aborted sudden death, and sustained ventricular tachycardia (VT). The secondary outcome was time to the earliest occurrence of atrial arrhythmia, nonsustained VT, and NYHA class >II. Multinomial regression was used to identify predictors of the 3-category outcome: (a) good outcome, defined as freedom from the primary AND secondary outcomes at age 50 years; (b) poor outcome, defined as presence of the primary outcome before age 50 years; and (c) intermediate outcome, defined as not fulfilling criteria for good or poor outcomes. Results: Among 1088 eligible patients, 96 had good outcome, 60 experienced poor outcome, and 932 had intermediate outcome. Patients were age 25.8±10.8 years at the time of the index CMR. Median follow-up was 5.8 years (IQR 3.0, 9.9) after CMR in event-free patients. By univariate analysis, smaller right ventricular (RV) end-systolic and end-diastolic volume index, smaller left ventricular end-systolic volume index, higher right and left ventricular ejection fraction, lower right and left ventricular mass index, and lower left ventricular mass/volume ratio were associated with good outcome. Multivariable modeling identified higher RV ejection fraction (OR 2.38 per 10% increase, P =.002) and lower RV mass index (OR 1.72, per 10 g/m2 decrease, P =.002) as independently associated with good outcome after adjusting for age at CMR. Classification and regression tree analysis identified important thresholds associated with good outcome that were specific to patients age ≥37 years at the time of CMR; these were RV ejection fraction ≥42% and RV mass index

Tricuspid regurgitation in patients with repaired tetralogy of Fallot is an important finding with a wide spectrum of primary and secondary etiologies. Moderate or severe tricuspid regurgitation is associated with a greater incidence of atrial tachyarrhythmias in these patients. It remains uncertain which patients with repaired tetralogy of Fallot may benefit from a tricuspid valve intervention at the time of pulmonary valve replacement.

Background American Heart Association and American College of Cardiology consensus guidelines introduce an adult congenital heart disease anatomic and physiological (AP) classification system. We assessed the association between AP classification and clinical outcomes. Methods and Results Data were collected for 1000 outpatients with ACHD prospectively enrolled between 2012 and 2019. AP classification was assigned based on consensus definitions. Primary outcomes were (1) all‐cause mortality and (2) a composite of all‐cause mortality or nonelective cardiovascular hospitalization. Cox regression models were developed for AP classification, each component variable, and additional clinical models. Discrimination was assessed using the Harrell C statistic. Over a median follow‐up of 2.5 years (1.4–3.9 years), the composite outcome occurred in 185 participants, including 49 deaths. Moderately or severely complex anatomic class (class II/III) and severe physiological stage (stage D) had increased risk of the composite outcome (AP class IID and IIID hazard ratio, 4.46 and 3.73, respectively, versus IIC). AP classification discriminated moderately between patients who did and did not suffer the composite outcome (C statistic, 0.69 [95% CI, 0.67–0.71]), similar to New York Heart Association functional class and NT‐proBNP (N‐terminal pro‐B‐type natriuretic peptide); it was more strongly associated with mortality (C statistic, 0.81 [95% CI, 0.78–0.84]), as were NT‐proBNP and functional class. A model with AP class and NT‐proBNP provided the strongest discrimination for the composite outcome (C statistic, 0.73 [95% CI, 0.71–0.75]) and mortality (C statistic, 0.85 [95% CI, 0.82–0.88]). Conclusions The addition of physiological stage modestly improves the discriminative ability of a purely anatomic classification, but simpler approaches offer equivalent prognostic information. The AP system may be improved by addition of key variables, such as circulating biomarkers, and by avoiding categorization of continuous variables.

The American Heart Association and American College of Cardiology published practice guidelines for the management of adult congenital heart disease in 2018 and the European Society of Cardiology published analogous guidelines in 2020. Although there are broad areas of consensus between the 2 documents, there are important differences that impact patient management. This review discusses key areas of agreement and disagreement between the 2 guidelines, with discussion of possible reasons for disagreement and potential implications.

Introduction: Cardiac magnetic resonance (CMR) derived biventricular global function index (BVGFI) is a new CMR parameter that integrates biventricular volumes, mass, and function using clinically available CMR parameters. The associations of BVGFI with clinical outcomes in repaired tetralogy of Fallot are unknown. Methods: Patients with repaired tetralogy of Fallot who had a CMR before the occurrence of a composite outcome of death, resuscitated sudden death, or sustained ventricular tachycardia were studied. BVGFI was calculated as the average of right and left GFI. GFI was defined as (ventricular stroke volume×100)/(ventricular mean cavity volume + total ventricular myocardial volume). Ventricular mean cavity volume was defined as ([end-diastolic + end-systolic volume]/2). Cox multivariable regression analysis and classification and regression tree methodology were used. Results: Of the 736 eligible subjects (mean age at CMR 25.4±14.5 years), with a median follow-up of 28 months, 55 subjects (7.4%) reached the composite outcome (46 deaths and 9 sustained ventricular tachycardia). Independent associations with the composite outcome were as follows: BVGFI P =0.004), right ventricular end-systolic volume index >85 mL/m 2 (hazard ratio, 3.25; P P =0.021), and age at repair >2.5 years (hazard ratio, 3.37; P Conclusions: BVGFI, a novel CMR-derived imaging biomarker combining biventricular volumes, mass, and function, may improve risk stratification for adverse clinical outcomes in patients with repaired tetralogy of Fallot.

Background: The Model for End-stage Liver Disease excluding INR (MELD-XI) is commonly used to identify patients with a Fontan circulation at increased risk of adverse events, However, this approach has not been evaluated in unselected ambulatory adults. Methods: We enrolled a cohort of 163 outpatients with a Fontan circulation aged ≥18-years in the Boston Adult Congenital Heart Disease Biobank from 2012 to 2018. Survival analysis was performed to assess the relationship between MELD-XI with both all-cause mortality and a composite outcome of mortality or non-elective cardiovascular hospitalization. Results: Mean age was 30.2±9.7 years, and 41.1% were women. Most had a lateral tunnel Fontan (62.6%). MELD-XI score averaged 10.6±2.1 (median = 13). Both creatinine and total bilirubin were ≤1.0 mg/dL in 94/163 (57.7%), translating to the lowest possible score. MELD-XI18. During follow-up of 3.2±2.2 years, the composite outcome occurred in 58 patients (35.6%), with 16 deaths (9.8%). Most deaths (n = 11, 68.8%) and composite outcomes (n = 39, 67.2%) occurred among patients with MELD-XI less than the median. MELD-XI score did not differ between those who did and did not have events (death: 10.8±2.2 vs. 10.6±2.1; p = 0.92; composite outcome: 10.6±2.2 vs. 10.7±2.1, p = 0.45). Likewise, survival analysis did not suggest an association between MELD-XI and either outcome. Conclusions: MELD-XI score does not appear to be associated with risk for adverse outcomes in an unselected cohort of outpatients with a Fontan circulation. Prior findings may reflect conditioning on a clinical referral for laboratory testing.

Highlights •Many patients with adult CHD will experience a progressive decline in cardiopulmonary function leading to advanced HF. •These high-risk patients present unique anatomic and physiological challenges that may not be amenable to repair. •For highly selected patients, mechanical circulatory support and heart transplantation offer excellent short- and long-term outcomes. •Prospective registries are needed to determine optimal timing of referral, risk factors for adverse outcomes, and strategies for preservation of end-organ function.

Background Validated protocols for diagnostic testing and management of pregnant women with cardiovascular disease (CVD) do not exist. Our objective was to establish a prospective standardized protocol for the clinical evaluation of pregnant women with CVD. Methods The Standardized Outcomes in Reproductive Cardiovascular Care (STORCC) initiative prospectively enrolled pregnant women with CVD into a standardized diagnostic testing and assessment protocol. Detailed cardiac and obstetric data were collected during the antepartum, intrapartum, and postpartum periods. Each woman was assigned a STORCC color code of perceived risk at a monthly multidisciplinary conference. Results In 250 pregnancies of 207 women with CVD, the standardized care protocol was followed in 136 and routine care in 114. The median age of the subjects was 32 years, and the most common form of heart disease was congenital heart disease (77%). Women enrolled in standardized care protocol had high compliance with second- and third-trimester visits (93%) and postpartum visits (76%). Maternal cardiac complications occurred in 10%. The STORCC cardiac and obstetric color codes predicted adverse outcomes within each respective category (P = .02, .01). Conclusions The STORCC protocol for prospective diagnostic testing and follow-up of pregnant women with CVD was successfully established, and compliance was high. The strength of a standardized testing and care protocol as well as detailed classification of labor and delivery characteristics allows for robust analyses into specific questions regarding testing protocols, and mode and timing of delivery.