Gender-based disparities in outcomes of coronary bifurcation stenting in patients undergoing percutaneous coronary intervention: a systematic review and meta-analysis.

INTRODUCTION: Around 15-20% of lesions necessitating percutaneous coronary interventions (PCI) are attributed to coronary bifurcation lesions. We aim to study gender-based differences in PCI outcomes among bifurcation stents. METHODS: 3 studies were included after thorough systematic search using MEDLINE (EMBASE and PubMed). CRAN-R software using the Metabin module was used for statistical analysis. Pooled odds ratios (OR) were calculated using the random effect model and the Mantel-Haenszel method, with a 95% confidence interval (CI) used to determine statistical significance. Heterogeneity was assessed using Higgins I2. RESULT: Women exhibited a higher risk of in-hospital mortality (OR 0.67, 95% CI 0.58-0.76, I2 = 0%, P < 0.0001), post-procedural bleeding (OR 0.53, 95% CI 0.47-0.6, I2 = 0%, P < 0.0001) and post-procedure stroke (OR 0.72, 95% CI 0.52-1.0, I2 = 0%, P < 0.06) as compared to men. However, there were no significant differences in terms of myocardial infarction (OR 0.84, 95% CI 0.22-3.27, I2 = 49.4%, P < 0.80) and cardiac tamponade (OR 0.63, 95% CI 0.06; 5.72, I2 = 0%, P < 0.6821) in both groups. CONCLUSION: Our study reveals a noteworthy increase in in-hospital mortality in women, which could be attributed to a higher rate of major bleeding, advanced age, increased co-morbidities, and complex pathophysiology of the lesion in comparison to men. Further studies are required to gain a better understanding of the precise mechanisms thus enhancing procedural outcomes.

Short-Term Outcomes of Cardiac Arrhythmias Among COVID-19 Patients: A Propensity Matched National Study.

The risk of arrhythmia is high in patients with COVID-19. The current literature is limited in understanding the clinical impact of arrhythmias and the extent of healthcare utilization in COVID-19 patients. The Nationwide In-patient Sample Database (NIS) from 2019 to 2020 was queried to identify COVID-19 patients who developed arrhythmias vs those without. Multivariate regression for adjusted odds ratios (aOR) and propensity score matching (PSM) were done to compare outcomes among both cohorts. A total of 1,664,240 patients (weighted) were hospitalized with COVID-19 infection, 380,915 (22.89%) of whom were diagnosed with an arrhythmia. After propensity matching COVID-19 with arrhythmias had higher rates of in-hospital mortality (22.4% vs 13.5%, P < 0.001), acute kidney injury (PSM 39.4% vs 35.7%, P < 0.001), acute heart failure (AHF) (18.2% vs 12.6%, P < 0.001), acute stroke (0.76% vs 0.57%, P < 0.001), cardiogenic shock (1.38% vs 0.5%, P < 0.001), cardiac arrest (5.26% vs 2.3%, P < 0.001) acute myocardial infarction (AMI) (12.8% vs 7.8%, P < 0.001), intracerebral hemorrhage (0.63% vs 0.45%, P < 0.001), major bleeding (2.6% vs 1.8%, P < 0.001) and endotracheal intubation (17.04% vs 10.17% < 0.001) compared to arrhythmias without COVID-19. This cohort also had lower odds of receiving interventions such as cardiac pacing (aOR 0.15 95% Cl 0.13-0.189 P < 0.001), cardioversion (aOR 0.43 95% CI 0.40-0.46, P < 0.001), and defibrillator (aOR 0.087 95% Cl 0.061-0.124, P < 0.001) compared to arrhythmia patients without COVID-19. Cardiac arrhythmias associated with COVID-19 resulted in longer length of hospital stay and higher total costs of hospitalizations. Arrhythmias associated with COVID-19 had worse clinical outcomes with an increased rate of in-hospital mortality, longer length of hospital stay, and higher total cost. These patients also had lower odds of receiving interventions during the index hospitalization.

A Propensity-Matched National Analysis of Transcatheter Aortic Valve Implantation Outcome in Patients With Gastrointestinal Bleeding.

Gastrointestinal (GI) bleeding is often observed in severe aortic stenosis, which can be attributed to the presence of arteriovenous malformations and von Willebrand's factor deficiency. GI is one of the most common complications in patients who underwent transcatheter aortic valve implantation (TAVI). The outcome of the TAVI procedure with GI bleeding is unknown. We performed an International Classification of Diseases, Tenth Revision-based national cohort analysis using the national readmission database from 2016 to 2020. We compared cardiovascular outcomes, mortality, and readmission rates of patients with TAVI who developed GI bleeding compared with those who had no GI bleeding. A total of 320,353 hospitalizations with TAVI were identified from the year 2016 to 2020. Patients with TAVI with GI bleeding were 6,193.37 and without GI bleeding were 314,160. The median age of the patients with TAVI with GI or without GI bleeding was 80. GI bleed patients had statistically significantly higher readmission rates at 30, 90, and 180 days and they had higher odds of in-hospital mortality (adjusted odds ratio [aOR] = 6.35; 95% confidence interval [CI]: 5.37 to 7.52; p <0.0001), acute kidney injury (aOR = 5.22; 95% CI:4.75 to 5.74; p <0.0001), stroke (aOR = 2.83; 95% CI 2.05 to 3.91 p:0.0001, postprocedural bleeding (aOR: 1.76; 95% CI: 1.35 to 2.30; p:0.0001), cardiac tamponade (aOR = 2.54; 95% CI 1.86 to 3.49; p <0.0001), use of mechanical circulatory support (aOR: 5.33; 95% CI:4.13 to 6.86; p <0.0001), and heart failure (aOR:1.73; 95%CI: 1.54 to 1.94; p <0.0001). The total cost of hospitalization and length of stay was higher in the GI bleed group. Patients with TAVI with GI bleeding have worse clinical outcomes and higher in-hospital mortality and readmission rates compared with patients with no GI bleeding.

Investigating the Complications and Causes of Failure of the AngioVac System: A Post-Marketing Surveillance From the MAUDE Database.

Background Aspiration thrombectomy devices, such as the AngioVac, allow the removal of thrombus, especially in patients with contraindications to anticoagulation use. The AngioVac was approved by the U.S. Food and Drug Administration to remove fresh, soft thrombi or emboli during extracorporeal bypass for up to six hours. Real-world data on the most common modes of failure and complications associated with the AngioVac are unavailable. Methods The Manufacturer and User Facility Device Experience database was queried for reports of the AngioVac device failure and adverse events from April 2013 to March 2022. Categorical variables were described as numbers, and all statistical calculations were performed with IBM SPSS Statistics, version 27.0 (IBM Corp., Armonk, NY). Results A total of 115 events were reported during the study period. After the exclusion of duplicate reports, the final cohort included 93 reports. The most common mode of failure for the AngioVac was physical damage of the device, with 13 reports (14%). The most common vessels associated with events were the superior vena cava and inferior vena cava, occurring in 23 reports (24.7%). The most common adverse clinical events were pulmonary embolism (PE), occurring in 33 reports (35.5%), and perforation, occurring in 16 reports (17.2%). Other less frequent adverse outcomes were arrhythmias, stroke, and foreign body device embedment. There were 45 deaths reported with the use of the AngioVac. Conclusions Aspiration thrombectomy devices provide promising efficacy; however, physicians should be aware of known adverse outcomes, even if they are infrequent. Based on this analysis, PE and vessel perforation were the most common adverse outcomes. Furthermore, the most common mode of failure was secondary to physical damage of the device.

The Prevalence of Cardiovascular Complications and Causes of AngioJet Failure: A Post-Marketing Surveillance Study Based on the MAUDE (Manufacturer and User Facility Device Experience) Database.

Background and objective Aspiration thrombectomy devices, such as the AngioJet Solent Omni (Boston Scientific Corporation, Marlborough, MA) have been approved by the US FDA for the treatment of thrombi in peripheral arterial disease, venous disease, and AV fistulas. However, there is a dearth of real-world data on the most common modes of failure and complications associated with the AngioJet Solent Omni. In this study, we aimed to address this scarcity of data. Methods The MAUDE (Manufacturer and User Facility Device Experience) database was queried for reports of device failure and adverse events spanning the period from October 2012 to December 2021. Results A total of 499 events were reported during the study period. After the exclusion of duplicate reports, the final analysis included 450 reports. The most common mode of failure was catheter breakage/kinking during suction thrombectomy with 137 reports (30%). The most common vessel associated with events was the superficial femoral artery or vein, which was documented in 82 reports (18.2%). The most common adverse clinical outcome was the embedding of a piece of the device in the patient, which occurred in seven reports (1.6%). There were seven (1.6%) events of death reported during the period studied. Conclusions Based on our findings, theAngioJet Solent Omni device provides promising results; however, it is important to evaluate device safety. It is associated with complications including device embedment, catheter breakage/kinking, and death, and these adverse events are linked to patient characteristics and risk factors.

Determinants and Prognostic Implications of Left-Heart Filling Pressures in Tetralogy of Fallot.

BACKGROUND: Elevated pulmonary artery wedge pressure (PAWP) is the hallmark of left-heart failure and is responsible for heart failure symptoms and mortality. Although PAWP typically correlates with right atrial pressure (RAP), it is primarily dependent on left-heart myocardial properties and volume status. As right-heart disease can occur in the absence of left-heart disease in patients with tetralogy of Fallot (TOF), we hypothesized that RAP was the primary determinant of PAWP in this population. METHODS: A cohort study of adults with TOF that underwent right-heart catheterization at Mayo Clinic Rochester (1990 to 2017) to determine the relationship among RAP, PAWP, and mortality. RESULTS: Among 213 patients (male 105; age 37 ± 14 years), the mean PAWP was 14 ± 5 mm Hg, and RAP was 11 ± 5 mm Hg. RAP was the strongest predictor of PAWP (ß = 0.68, standard error = 0.06, P < 0.001), independent of left-heart disease and atherosclerotic cardiovascular risk factors. The patients with high PAWP also had normal tissue Doppler velocities, suggesting normal left-ventricular myocardial properties. PAWP was an independent predictor of death/transplant (hazard ration [HR] 1.11, 95% confidence interval [CI], 1.03-1.20, P = 0.004). However, when RAP was incorporated into the regression model, RAP (and not PAWP) became the independent predictor of outcomes (HR 1.14, 95% CI, 1.06-1.22, P = 0.001). CONCLUSIONS: The current study showed that RAP was the primary determinant of PAWP and accounts, to some extent, for the mortality in patients with TOF and high PAWP. The data provide new insight in the pathophysiology of disease progression for symptomatic patients with TOF.

Feasibility, reproducibility and accuracy of electrical velocimetry for cardiac output assessment in congenital heart disease.

BACKGROUND: Noninvasive cardiac output assessment is important for prognostication in patients with heart failure. Electrical velocimetry (EV), an impedance cardiography technique, can be used for noninvasive cardiac output assessment. The purpose of this study was to determine the feasibility, reproducibility and accuracy of cardiac output assessment by EV in adults with congenital heart disease (CHD). METHODS: Cross-sectional study of CHD patients that had simultaneous cardiac output assessment by Fick and EV (using Cardiotronic monitor, Osypka Medical). We divided the cohort into: Group 1 patients (n = 54) had hemodynamic assessment at rest only, while Group 2 patients (n = 7) had assessment both at rest and peak exercise. RESULTS: EV cardiac output assessment was feasible in 100% of the patients. There was good correlation between Fick-derived and EV-derived cardiac index (r = 0.89, p < 0.001) in Group 1. Among 26 patients in Group 1 that underwent cardiac output assessment pre- and post-intervention, there was no difference in the strength of correlation of Fick and EV cardiac output pre- and post-intervention (p-interaction 0.244) indicating good reproducibility of the technique. There was also modest correlation between Fick-derived and EV-derived cardiac index at rest (r = 0.68, p = 0.032), and peak exercise (r = 0.62, p = 0.055), in Group 2. CONCLUSION: In this study, we demonstrated the feasibility and accuracy of EV cardiac output assessment in adults with CHD. We also demonstrated, for the first time, that EV cardiac output assessment was reproducible under different loading conditions, and that EV can be used for the assessment of cardiac output augmentation at peak exercise.

Incidence and outcomes of prosthetic valve endocarditis in adults with tetralogy of Fallot.

BACKGROUND: The risk of endocarditis varies with CHD complexity and the presence of prosthetic valves. The purpose of the study was therefore to describe incidence and outcomes of prosthetic valve endocarditis in adults with repair tetralogy of Fallot. METHODS: Retrospective review of adult tetralogy of Fallot patients who underwent prosthetic valve implantation, 1990-2017. We defined prosthetic valve endocarditis-related complications as prosthetic valve dysfunction, perivalvular extension of infection such abscess/aneurysm/fistula, heart block, pulmonary/systemic embolic events, recurrent endocarditis, and death due to sepsis. RESULTS: A total of 338 patients (age: 37 ± 15 years) received 352 prosthetic valves (pulmonary [n = 308, 88%], tricuspid [n = 13, 4%], mitral [n = 9, 3%], and aortic position [n = 22, 6%]). The annual incidence of prosthetic valve endocarditis was 0.4%. There were 12 prosthetic valve endocarditis-related complications in six patients, and these complications were prosthetic valve dysfunction (n = 4), systemic/pulmonary embolic events (n = 2), heart block (n = 1), aortic root abscess (n = 1), recurrent endocarditis (n = 2), and death due to sepsis (n = 1). Three (50%) patients required surgery at 2 days, 6 weeks, and 23 weeks from the time of prosthetic valve endocarditis diagnosis. Altogether three of the six (50%) patients died, and one of these deaths was due to sepsis. CONCLUSIONS: The incidence, complication rate, and outcomes of prosthetic valve endocarditis in tetralogy of Fallot patients underscore some of the risks of having a prosthetic valve. It is important to educate the patients on the need for early presentation if they develop systemic symptoms, have a high index of suspicion for prosthetic valve endocarditis, and adopt a multi-disciplinary care approach in this high-risk population.

Effect of right ventricular pacing on left ventricular systolic function in patients with Tetralogy of Fallot.

BACKGROUND: Prevalence of pacemaker-induced cardiomyopathy (PICM) in adults with congenital heart disease is unknown. Tetralogy of Fallot (TOF) is a common diagnosis in the adult congenital heart disease population, and the purpose of this study was to determine association between frequent right ventricular (RV) pacing and temporal decrease in left ventricular ejection fraction (LVEF) from pre-implantation to 2-years post-implantation (LVEFpost-pre) in TOF patients. METHODS: We studied TOF patients that received RV leads only (N = 51) and a reference group of 7 patients with atrial pacing or biventricular pacing. We defined PICM as a ≥10% decrease in LVEF resulting in LVEF <50%. Linear regression was used to assess relationship between frequent RV pacing (≤20%, 21-40%, >40%) and LVEFpost-pre. RESULTS: PICM occurred in 2 (4%) of 51 patients in RV pacing group. LVEFpost-pre was +3% (95% confidence interval [CI] 0% to +5%) in the reference group and -4% (95% CI -11% to +2%) in RV pacing group. No significant difference occured in LVEFpost-pre between the reference group (LVEFpost-pre +3%) vs RV pacing ≤20% (LVEFpost-pre +1%) vs RV pacing 21-40% (LVEFpost-pre -3%) vs RV pacing >40% (LVEFpost-pre -5%), p = 0.318. There was also no association between frequent RV pacing and LVEFpost-pre, R 2 = 0.307, p = 0.10. CONCLUSION: PICM occurred in 4% of TOF patients receiving RV pacing, and there was no association between frequent RV pacing and temporal decline in LVEF. Further studies are required to determine the long-term impact of RV pacing in the TOF population, and explore optimal treatment strategies.

Impact of atrial arrhythmia on survival in adults with tetralogy of Fallot.

BACKGROUND: Atrial arrhythmia is a late complication after tetralogy of Fallot (TOF) repair, but arrhythmia outcomes data are limited. OBJECTIVES: The purpose of the study was to describe atrial arrhythmia presentations, outcomes of antiarrhythmic therapy, and impact of arrhythmia on transplant-free survival. METHODS: We reviewed the MACHD (Mayo Adult Congenital Heart Disease) Registry and identified 113 patients (age 49 ±â€¯13 years) with documented arrhythmia, and 302 patients without history of arrhythmia, 1990-2017. We classified arrhythmias into atrial fibrillation and atrial flutter/tachycardia based on the rhythm on the first abnormal electrocardiogram. RESULTS: At the time of first documented arrhythmia, 58(51%) had atrial fibrillation while 55(49%) had atrial flutter/tachycardia. Of the 113 patients, 14(12%) received rhythm control with class I/III antiarrhythmic drugs (AAD), 79(70%) had direct current cardioversion, 9(8%) received rate control with class II/IV AAD, and 11(10%) received only anticoagulation. Successful cardioversion occurred in 100(89%) patients, and arrhythmia recurrence rate was 16 per 100 patient-years. The multivariate risk factors for death and/or heart transplant were atrial fibrillation (HR 1.94, CI 1.10-3.15, P = .031) and older age (HR 1.63, CI 1.12-2.43, P = .019) per 5 year increment. CONCLUSIONS: Atrial fibrillation, but not atrial flutter, was associated with reduced survival in our repaired TOF cohort. Further studies are required to determine if more aggressive antiarrhythmic therapy will improve survival in patients with atrial fibrillation.

Prevalence and in-hospital mortality during arrhythmia-related admissions in adults with tetralogy of Fallot.

BACKGROUND: Although outcomes of arrhythmia diagnosis have been described in ambulatory tetralogy of Fallot (TOF) patients, these have not been studied in hospitalized patients. The purpose of this study was to determine the prevalence and in-hospital mortality due to arrhythmias in TOF patients based on a review of the National Inpatient Sample database. METHODS: Admissions in adult TOF patients (2000-2014) were categorized as arrhythmia-related admission (ARA) or non-arrhythmia-related admission (NRA) based on arrhythmia diagnostic codes. RESULTS: Of 18,353 admissions, 5071 (27.6%) were ARA. The most common arrhythmias were atrial fibrillation (15.5%), atrial flutter (8.4%) and ventricular tachycardia (8.2%), and the prevalence of overall ARA as well as specific arrhythmia types increased over time. In-hospital mortality for ARA was 5.4%, and decreased over time. Arrhythmia diagnosis was an independent predictor of in-hospital mortality (odds ratio [OR] 1.63, 1.34-2.01, p = 0.001). Similarly, atrial fibrillation (OR 1.49, 1.18-1.89, p = 0.001) and ventricular tachycardia (OR: 2.01, 1.55-2.98, p = 0.001) were independent predictors of in-hospital mortality. Compared to small bed-size hospital, ARA in large hospital bed-size hospital was associated with a lower in-hospital mortality (OR 0.71, 0.53-0.96, p = 0.03). CONCLUSION: Atrial fibrillation was the most common arrhythmia in hospitalized TOF patients, and arrhythmia diagnosis (specifically atrial fibrillation and ventricular tachycardia) was an independent predictor of in-hospital mortality, while admission to a large bed-size hospital was associated with a lower risk of in-hospital mortality. Further studies are required to determine if a more proactive approach to arrhythmia management in the ambulatory TOF population will reduce hospitalizations and mortality.

Assessment and Implications of Right Ventricular Afterload in Tetralogy of Fallot.

Patients with tetralogy of Fallot (TOF) have abnormal right ventricular (RV) afterload because of residual or recurrent outflow tract obstruction, often with abnormal pulmonary artery (PA) vascular function. The purpose of this study was to determine if RV afterload was independently associated with death and/or heart transplant in patients with TOF. This is a retrospective study of TOF patients that underwent cardiac catheterization for clinical indications at Mayo clinic between 1990 and 2015. Invasively measured RV systolic pressure (RVSP) was used to define RV afterload. To explore clinical utility for echocardiographic estimates of invasive data, correlations between invasive and Doppler-derived indices of RV afterload were examined. Among 266 patients with TOF (age 35 ± 14 years, TOF-pulmonary atresia 117 [44%]), RVSP was 72 ± 28 mm Hg, PA systolic pressure 45 ± 19 mm Hg, mean PA pressure 27 ± 10 mm Hg, pulmonary vascular resistance 4.2 ± 3.1 WU, and PA wedge pressure 14 ± 5 mm Hg. Over a mean follow up of 12.9 years, there were 35 deaths and 4 heart transplants. Invasively measured RVSP (hazard ratio 1.25, 95% confidence interval 1.12 to 1.37; p <0.001) and TOF-pulmonary atresia (hazard ratio 1.18, 95% confidence interval 1.08 to 1.41; p = 0.023) were independent risk factors for death and/or transplant. Doppler-derived RVSP was well-correlated with invasive RVSP (r = 0.92, p <0.001), and was also independently associated with the combined end point. RVSP, a composite measure of RV afterload, is independently prognostic in patients with TOF, and can be reliably assessed using Doppler echocardiography. Further study is required to test whether interventions to reduce RVSP can improve outcomes in patients with TOF.

Usefulness of Right Ventricular Volumetric and Afterload Indices for Risk Stratification in Patients With Tetralogy of Fallot.

Right ventricular (RV) volume overload due to chronic pulmonary regurgitation is the common mechanism for hemodynamic deterioration after tetralogy of Fallot (TOF) repair. As a result, RV volumetric indices are used for clinical risk stratification in this population. Since RV afterload is a determinant of RV hemodynamic performance, we hypothesized that afterload-adjusted RV volumetric indices will have a better correlation with disease severity compared with RV volumetric indices alone in patients with TOF. Cross-sectional study of adults with previous TOF repair that received care at Mayo Clinic, 2002-2015. We defined disease severity as atrial arrhythmia and/or impaired exercise capacity. We created afterload-adjusted RV volumetric indices by indexing these indices to RV systolic pressure (RVSP) as follows: RV end-diastolic volume (RVEDVi)/RVSP, RV end-systolic volume (RVESVi)/RVSP, and RV ejection fraction (RVEF)/RVSP. The RV volumetric indices were: RVEDVi 141 ± 43 ml/m2, RVESVi 79 ± 38 ml/m2, and RVEF 44 ± 10%, and RVSP was 48 ± 9 mm Hg. RVESVi was the only RV volumetric parameter that was associated with disease severity (odds ratio [OR] 1.13, 95% confidence interval [CI] 1.01 to 1.32, p = 0.041) with area under the curve (AUC) of 0.612. In contrast RVEF/RVSP (OR 0.73, 95% CI 0.38 to 0.92, p = 0.037, AUC 0.649), and RVESVi/RVSP (OR 1.28, 95% CI 1.14-1.55, p = 0.008, AUC 0.798) were associated with disease severity. Compared with RV volumetric indices alone, the combined RV volumetric and afterload indices had better correlation with disease severity as measured by AUC. Afterload-adjusted RV volumetric indices had better correlation with disease severity, and may potentially improve risk stratification in this population.

Assessment of Right Ventricular-Pulmonary Arterial Coupling in Chronic Pulmonary Regurgitation.

BACKGROUND: We hypothesized that noninvasively measured right ventricular (RV) to pulmonary arterial (RV-PA) coupling would be abnormal in chronic pulmonary regurgitation (PR) even in the setting of normal RV ejection fraction, and that RV-PA coupling indices would have a better correlation with peak oxygen consumption (VO2) compared with RV systolic indices alone. METHODS: This was a retrospective study of 129 adults (repaired tetralogy of Fallot [TOF] n = 84 and valvular pulmonic stenosis [VPS] with previous intervention n = 45) with ≥ moderate native PR and RV ejection fraction > 50%. The 84 TOF patients were propensity matched with 84 patients with normal echocardiogram (control); age 28 ± 7 years and male sex n = 39 (46%). RV-PA coupling was measured according to fractional area change (FAC)/RV systolic pressure (RVSP) and tricuspid annular plane systolic excursion (TAPSE)/RVSP. RESULTS: RV systolic function indices were similar between TOF and control groups (FAC 43 ± 6% vs 41 ± 5% [P = 0.164] and TAPSE 22 ± 5 mm vs 24 ± 6 mm [P = 0.263]). However, RV-PA coupling was lower in the TOF group (FAC/RVSP 1.10 ± 0.29 vs 1.48 ± 0.22 [P < 0.001]; TAPSE/RVSP 0.51 ± 0.15 vs 0.78 ± 0.11 [P < 0.001]) because of higher RV afterload (RVSP 42 ± 3 mm Hg vs 31 ± 3 mm Hg [P = 0.012]). FAC/RVSP (r = 0.61; P < 0.001) and TAPSE/RVSP (r = 0.69; P < 0.001) correlated with peak VO2 especially in the patients with impaired exercise capacity whereas FAC and TAPSE were independent of peak VO2. Similar comparisons between VPS and control groups showed no difference in TAPSE and FAC between groups, but lower FAC/RVSP and TAPSE/RVSP in the VPS group. CONCLUSIONS: There is abnormal RV-PA coupling in chronic PR, and noninvasively measured RV-PA coupling might potentially be prognostic because of its correlation with exercise capacity.

Mechanism and Risk Factors for Death in Adults With Tetralogy of Fallot.

One of the goals of lifelong care in adults with tetralogy of Fallot (TOF) is early identification and treatment of patients at high risk for adverse events. Clinical risk stratification tools are critical for achieving this goal. We reviewed the Mayo Adult Congenital Heart Disease database and identified 465 TOF patients (age 37 ± 14 years, men 223 [48%]) seen at Mayo Clinic Rochester between 1990 and 2017. The aim was to determine the risk factors for death and/or heart transplant through a comprehensive analysis of 8 groups of variables (demographics, co-morbidities, medications, heart rhythm, echocardiography, cardiac magnetic resonance imaging, cardiac catheterization, and cardiopulmonary exercise test data) using univariable and multivariable Cox proportional hazard models. The end point of death and/or transplant occurred in 57 (12%) patients during a follow-up of 13.6 ± 8.2 years, yielding an event rate of 0.9% per year. Independent risk factors were age >42 years, atrial fibrillation, ≥moderate QRS fragmentation, left ventricular ejection fraction <50%, right ventricular end-diastolic pressure >16 mm Hg, and left ventricle end-diastolic pressure >16 mm Hg. There is nearly a twofold increase in the risk of death and/or transplant per unit increase in number of risk factors (hazard ratio 1.92, 95% confidence interval 1.62 to 2.27, p <0.001). In conclusion, the current study provides risk stratification indices based on a comprehensive risk model of all clinical variables in an unselected TOF population. Further studies are required to determine whether interventions targeted at modifying these risk factors will alter the annual event rate.

Long-term outcomes in adults with repaired tetralogy of Fallot and pulmonary atresia.

BACKGROUND: There are limited outcome data in adults with tetralogy of Fallot and pulmonary atresia. The purpose of this study was to describe re-operations and all-cause mortality in adults with tetralogy of Fallot and pulmonary atresia. METHODS: Retrospective review of adults with repaired tetralogy of Fallot and pulmonary atresia who received care at the Mayo Adult Congenital Heart Disease Clinic, 1990-2016. All-cause mortality was calculated as events per 100 patient-years from the time of first presentation to the Adult Congenital Heart Disease Clinic. RESULTS: Of the 221 patients, the age at initial tetralogy of Fallot repair was 6 (5-13) years, and the age at first presentation to the clinic was 27 - 8 years. All patients had at least one right ventricular to pulmonary artery conduit re-operation. There were 31 deaths (14%) at mean age of 41 - 14 years. The causes of death were end-stage heart failure (n = 17), sudden cardiac death (n=9), post-operative death after cardiac surgery (n = 2), sepsis with multi-system organ failure (n = 2), and unknown (n = 1). All-cause mortality rate was 1.7 per 100 patient-years. The risk factors for all-cause mortality were older age (>12 years) at the time of repair (hazard ratio 1.41, 95 confidence interval 1.06-2.02, p = 0.033), non-sustained ventricular tachycardia (hazard ratio 1.36, 95 confidence interval 1.17-2.47, p = 0.015), and left ventricular ejection fraction <50% (hazard ratio 1.39, 95 confidence interval 1.08-2.31, p = 0.031). CONCLUSION: Based on a review of 221 adults with repaired tetralogy of Fallot and pulmonary atresia, all patients had re-operations and all-cause mortality rate was 1.7 events per 100 patient-years. The current study provides important outcomes data for risk stratification in adults with tetralogy of Fallot and pulmonary atresia.

Role of Doppler echocardiography for assessing right ventricular cardiac output in patients with atrial septal defect.

BACKGROUND: Although Doppler echocardiography is routinely used to assess left ventricle cardiac output, there are limited data about the feasibility of Doppler echocardiography for right ventricular (RV) cardiac output assessment in patients with left-to-right shunt. The purpose of the study was to determine the correlation between Doppler-derived and Fick-derived RV cardiac index (CI), and the interobserver correlation in Doppler-derived RV CI assessment. METHODS: Retrospective study of patients (age ≥18 years) with unrepaired atrial septal defect who underwent cardiac catheterization and echocardiography (within 3 days), 2004-2017. RV CI was calculated using the hydraulic orifice formula: [.785 × (right ventricle outflow tract diameter)2  × right ventricular outflow tract (RVOT) time velocity integral × heart rate]/body surface area. RESULTS: A total of 128 patients (age 52 ± 17 years; female 88 [69%]) met the inclusion criteria. There was a modest correlation between Doppler-derived and Fick-derived RV CI (r = .57, P < .001), and the mean difference between Doppler-derived and Fick-derived RV CI was -.3 (95% confidence interval of agreement, -.8 to +.9) L/min/m2 . There was also a modest correlation between Doppler-derived RV CI from observer #1 and observer #2 (r = .62, P < .001), and the mean difference between Doppler-derived RV CI from observer #1 and observer #2 was -.2 (95% confidence interval of agreement, -.9 to +.6). CONCLUSIONS: The current study demonstrated a modest correlation between Doppler-derived and Fick-derived RV cardiac output, and a modest interobserver correlation in Doppler-derived RV cardiac output assessment. Further studies are required to validate these results and to explore other potential applications such as in patients with chronic pulmonary regurgitation.

Coronary artery disease in adults with tetralogy of Fallot.

BACKGROUND: There are limited data about outcomes of coronary artery disease (CAD) in adults with repaired tetralogy of Fallot (TOF). The purpose of this study was to describe the prevalence and treatment of CAD in adults with TOF, and the impact of CAD on long-term survival. METHODS: Retrospective review of MACHD database for adults with repaired TOF who underwent aortic root/selective coronary angiogram, 1990-2017. Patients were categorized into three groups: (1) No CAD defined as normal coronary angiogram; (2) Mild CAD defined as ≤50% stenosis in all vessels; and, (3) Significant CAD defined as >50% stenosis in any vessel. RESULTS: We identified 105 (23%) of 465 TOF patients that had angiograms; mean age 47 ± 12 years. The prevalence of mild CAD and significant CAD was 19% (20 patients) and 15% (16 patients), respectively. Of these 16 patient with significant CAD, 9 (56%), 3 (19%), and 4 (24%) patients received guideline directed medical therapy, percutaneous coronary intervention, and coronary artery bypass grafting, respectively. Significant CAD was an independent risk factor for mortality (HR: 2.03, 95% CI 1.64-4.22, P = .022) after adjustment for differences in age, and prevalence of atrial fibrillation and renal dysfunction. CONCLUSIONS: Based on a review of a selected cohort of 105 TOF patients, the prevalence of mild CAD and significant CAD was 19% and 15%, respectively. Significant CAD was an independent risk factor for mortality. There is need for more research to determine optimal noninvasive diagnostic strategies and optimal patient selections and methods for revascularization.

Right ventricular and pulmonary vascular function indices for risk stratification of patients with pulmonary regurgitation.

BACKGROUND: We hypothesized that echocardiographic indices of right ventricular to pulmonary artery (RV-PA) coupling were comparable to cardiac magnetic resonance imaging (CMRI)-derived RV volumetric indices in predicting disease severity in chronic pulmonary regurgitation (PR). METHODS: Patients with ≥ moderate PR (2003-2015) with and without prior CMRI scans were enrolled into the study cohort and validation cohort, respectively. Endpoint was to determine the association between noninvasive RV-PA coupling indices (tricuspid annular plane systolic excursion/right ventricular systolic pressure [TAPSE/RVSP] and fractional area change [FAC]/RVSP ratio) and markers of disease severity, and compared this association to that of CMRI-derived RV volumetric indices and markers of disease severity (peak oxygen consumption [VO2 ], NT-proBNP and atrial and/or ventricular arrhythmias). RESULTS: Of the 256 patients in the study cohort (age 33 ± 6 years), 187 (73%) had tetralogy of Fallot (TOF) while 69 (27%) had valvular pulmonic stenosis (VPS). TAPSE/RVSP (r = 0.73, P < .001) and FAC/RVSP (r = 0.78, P < .001) correlated with peak VO2 . Among the CMRI-derived RV volumetric indices analyzed, only right ventricular end-systolic volume index correlated with peak VO2 (r = -0.54, P < .001) and NT-proBNP (r = 0.51, P < .001). These RV-PA coupling indices were tested in the validation cohort of 218 patients (age 37 ± 9 years). Similar to the study cohort, TAPSE/RVSP (r = 0.59, P < .001) and FAC/RVSP (r = 0.70, P < .001) correlated with peak VO2 . TAPSE/RVSP (but not FAC/RVSP) was also associated with arrhythmia occurrence in both the study cohort and validation cohorts. CONCLUSION: Noninvasive RV-PA coupling may provide complementary prognostic data in the management of chronic PR. Further studies are required to explore this clinical tool.