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2.
J Am Coll Cardiol ; 75(8): 857-866, 2020 Mar 03.
Artigo em Inglês | MEDLINE | ID: mdl-32130920

RESUMO

BACKGROUND: Early in the prevention and treatment of bioprosthetic valve thrombosis (BPVT), anticoagulation is effective, but the long-term outcome after BPVT is unknown. OBJECTIVES: The goal of this study was to assess the long-term outcomes of patients with BPVT treated with anticoagulation. METHODS: This analysis was a matched cohort study of patients treated with warfarin for suspected BPVT at the Mayo Clinic between 1999 and 2017. RESULTS: A total of 83 patients treated with warfarin for suspected BPVT (age 57 ± 18 years; 45 men [54%]) were matched to 166 control subjects; matching was performed according to age, sex, year of implantation, and prosthesis type and position. Echocardiography normalized in 62 patients (75%) within 3 months (interquartile range [IQR]: 1.5 to 6 months) of anticoagulation; 21 patients (25%) did not respond to warfarin. Median follow-up after diagnosis was 34 months (IQR: 17 to 54 months). There was no difference in the primary composite endpoint between the patients with BPVT and the matched control subjects (log-rank test, p = 0.79), but the former did have a significantly higher rate of major bleeding (12% vs. 2%; p < 0.0001). BPVT recurred (re-BPVT) in 14 (23%) responders after a median of 23 months (IQR: 11 to 39 months); all but one re-BPVT patient responded to anticoagulant therapy. Patients with BPVT had a higher probability of valve re-replacement (68% vs. 24% at 10 years' post-BPVT; log-rank test, p < 0.001). CONCLUSIONS: BPVT was associated with re-BPVT and early prosthetic degeneration in a significant number of patients. Indefinite warfarin anticoagulation should be considered after a confirmed BPVT episode, but this strategy must be balanced against an increased risk of bleeding.

4.
Int J Cardiol ; 306: 49-55, 2020 May 01.
Artigo em Inglês | MEDLINE | ID: mdl-32145939

RESUMO

BACKGROUND: Previous studies have described echocardiographic indices of right ventricular (RV) diastolic function in patients with tetralogy of Fallot (TOF) but these indices have not been validated against invasive hemodynamic data. The purpose of this study was to determine echocardiographic predictors of severe RV diastolic dysfunction, and the impact of severe RV diastolic dysfunction on transplant-free survival. METHODS: Cohort study of TOF patients that underwent non-simultaneous cardiac catheterization and echocardiogram at Mayo Clinic. Based on prior studies we selected these indices for assessment: tricuspid E/A, E/e', deceleration time, pulmonary artery forward flow, dilated inferior vena cava (IVC), and hepatic vein diastolic flow reversal (HVDFR). RV diastolic function classes (normal, mild/moderate and severe dysfunction) were created using arbitrary cut-off points of the median values of right ventricular end-diastolic pressure (RVEDP) and right atrial pressure (RAP) for the cohort. RESULTS: Among 173 patients (age 40 ± 13 years), 68 patients were classified as normal (RVEDP≤14 and RAP≤10), 37 as mild/moderate dysfunction (either RVEDP>14 or RAP>10), and 69 as severe dysfunction (RVEDP>14 and RAP>10). Of the indices assessed, dilated IVC had the best sensitivity of 95% (area under the curve [AUC] 0.689) while HVDFR had the best specificity of 69% (AUC 0.648) for detecting severe RV diastolic dysfunction. Severe RV diastolic dysfunction was an independent risk factor for death/transplant (hazard ratio 2.83, p = 0.009). CONCLUSION: Severe RV diastolic dysfunction, as defined by invasive hemodynamic indices, was associated with poor prognosis. Echocardiographic indices can identify these high risk patients, and hence improve risk stratification in clinical practice.

5.
Circ Heart Fail ; 13(2): e006651, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-32059629

RESUMO

BACKGROUND: Coarctation of aorta (COA) results in chronic left ventricular (LV) pressure overload and subsequently leads to LV diastolic dysfunction and heart failure over time. The goal of COA intervention is to prevent these complications. The timing of COA interventions is based on the presence of these COA severity indices: doppler mean COA gradient, systolic blood pressure, upper-to-lower-extremity SBP gradient, aortic isthmus ratio, presence of collaterals, and exercise-induced hypertension. Although these indices are physiologically intuitive, the relationship between these indices and LV diastolic dysfunction and exertional symptoms has not been studied. The purpose of this study was to evaluate the association between the indices of COA severity and LV diastolic function and symptoms. METHODS: In this cross-sectional study, multivariate linear and logistic regression analyses were used to assess the correlation between indices of COA severity, LV diastolic function (average e' and E/e'), and exertional symptoms (NYHA II-IV and peak oxygen consumption). RESULTS: Of all the COA indices analyzed in 546 adult COA patients, aortic isthmus ratio had the strongest correlation with e' (ß [95% CI]: 3.11 [2.02-4.31]; P=0.014) per 1 cm/second; E/e' (-13.4 [-22.3 to -4.81]; P=0.009) per 1 unit; peak oxygen consumption (4.05 [1.97-6.59] per 1% change, P=0.019), and NYHA II to IV symptoms (odds ratio, 2.16 [1.65-3.18]; P=0.006). CONCLUSIONS: Of all the COA severity indices stipulated in the guidelines, aortic isthmus ratio had the strongest correlation with LV diastolic function and exertional symptoms. As LV diastolic dysfunction typically precede heart failure symptoms, we anticipate that the results of this study will improve and simplify patient selection for COA intervention and potentially improve long-term outcomes.

6.
Cardiol Young ; 30(1): 19-23, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31910919

RESUMO

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.

7.
JACC Cardiovasc Imaging ; 13(2 Pt 1): 357-369, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-30878438

RESUMO

OBJECTIVES: This study sought to determine the prevalence of reduced contractility and uncompensated wall stress in patients with aortic stenosis (AS) with preserved or reduced left ventricular ejection fraction (LVEF) and their impact on survival. BACKGROUND: LVEF in AS is determined not only by contractility but also by loading conditions. METHODS: Patients with first diagnosis (time 0) of severe AS (aortic valve area [AVA]≤1 cm2) with prior echo study (-3±1 years) were identified. Contractility was evaluated by plotting midwall fractional shortening (mFS) against circumferential end-systolic wall stress (cESS), stratified by LVEF of 60% at time 0. The temporal changes (from -3 years to time 0) and prognostic value of LVEF, contractility, and wall stress were assessed. RESULTS: Of 445 patients, 290 (65%) had LVEF ≥60% (median: 66% [interquartile range {IQR}: 63% to 69%]) and 155 patients (35%) had LVEF <60% (median: 47% [IQR: 34% to 55%]). Median AVA was 1.27 cm2 (IQR: 1.13 to 1.43 cm2) at -3 years and 0.90 cm2 (IQR: 0.83 to 0.96 cm2) at time 0. Decreased contractility was already present at -3 years (49 [17%] vs. 59 [38%]; LVEF ≥60% vs. <60%; p < 0.001) and became more prevalent at time 0 (69 [24%] vs. 106 [68%]; p < 0.001). Overall, wall stress was well controlled in both groups at -3 years (1 [0%] vs. 12 [8%]; p < 0.001) but deteriorated over time in patients with LVEF <60% (time 0: 0 [0%] vs. 26 [17%]; p < 0.001). During a median follow-up of 3.4 years, LVEF <60%, decreased contractility and high wall stress were associated with worse survival (p < 0.01 for all). Decreased contractility remained incremental to LVEF in patients with LVEF ≥60% (p < 0.01), but less so when LVEF was <60% (p = 0.11). CONCLUSIONS: In patients with severe AS, LVEF <60% is associated with a poor prognosis, being linked with decreased contractility and/or high wall stress. Decreased contractility is also present in a subset of patients with LVEF ≥60% and provides incremental prognostic value. These abnormalities already exist before AVA reaches 1.0 cm2.

8.
Int J Cardiol ; 299: 136-139, 2020 01 15.
Artigo em Inglês | MEDLINE | ID: mdl-31351788

RESUMO

BACKGROUND: Pulmonary valve replacement (PVR) is associated with improvement in symptoms and right ventricular remodeling in patients with tetralogy of Fallot (TOF). There are limited population-based data about outcomes after PVR. We therefore hypothesized a temporal increase in annual volume of PVR and decrease in in-hospital mortality after PVR in the United States. METHODS: We reviewed the National Inpatient Sample (NIS) for PVR performed in adults (>18 years) with TOF, 2000-2014. The primary outcome was trends in admissions for PVR, in-hospital mortality after PVR, and age at time of PVR. In order to assess trends, we divided the study period into tertiles: early era (2000-2004), mid era (2005-2009) and late era (2010-2014). RESULTS: There were 18,353 admissions in adults with TOF diagnosis, of which PVR was performed in 1230 (6.7%), and 90 (7.3%) were transcatheter PVRs. The median age at PVR was 34 years and in-hospital mortality was 1.5%. Comparisons by study era showed temporal increase in the proportion of admissions for PVR (3.7% vs 6.3% vs 9.6%, p < 0.001), decrease in in-hospital mortality (4.1% vs 1.2% vs 0.8%, p = 0.002), and a decrease in age at the time of PVR (35.8 vs 33.8 vs 31.0 years, p < 0.001). CONCLUSIONS: The proportion of admissions for PVR increased while in-hospital mortality and age at time of PVR decreased over time. A younger age at the time of PVR highlights important concerns and knowledge gaps about the cumulative lifetime risk of reinterventions and prosthetic valve endocarditis. Further studies are required to address these knowledge gaps.

9.
Can J Cardiol ; 35(12): 1784-1790, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31732195

RESUMO

BACKGROUND: Although there are robust data about the pathophysiology and prognostic implications of left ventricular (LV) systolic dysfunction in patients with acquired heart disease, similar prognostic data about LV systolic dysfunction are sparse in the tetralogy of Fallot (TOF) population. The purpose of this study was to perform a meta-analysis of all studies that assessed the relationship between LV ejection fraction (LVEF) and cardiovascular adverse events (CAEs) defined as death, aborted sudden death, or sustained ventricular tachycardia. METHODS: We used random-effects models to calculate hazard ratios (HRs) and 95% confidence intervals (CIs). RESULTS: Of the 1,809 citations, 7 studies with 2,854 patients (age 28 ± 4 years) were included. During 5.6 ± 3.4 years' follow-up, there were 82 deaths, 17 aborted sudden cardiac deaths, and 56 sustained ventricular tachycardia events. Overall, CAEs occurred in 5.1% (144 patients). As a continuous variable, LVEF was a predictor of CAE (HR 1.29, 95% CI, 1.09-1.53, P = 0.001) per 5% decrease in LVEF. Similarly, LVEF < 40% was also a predictor of CAE (HR 3.22, 95% CI, 2.16-4.80, P < 0.001). CONCLUSIONS: LV systolic dysfunction was an independent predictor of CAE, and we observed a 30% increase in the risk of CAE for every 5% decrease in LVEF, and a 3-fold increase in the risk of CAE in patients with LVEF <40% compared with other patients. These findings underscore the importance of incorporating LV systolic function in clinical risk stratification of patients with TOF and the need to explore new treatment options to address this problem.

11.
Obstet Gynecol ; 134(4): 882, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31568353
12.
Am Heart J ; 218: 1-7, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31648061

RESUMO

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.

14.
Congenit Heart Dis ; 14(6): 1157-1165, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31545019

RESUMO

INTRODUCTION: Mechanisms and risk factors for cerebrovascular accidents (CVAs) in Ebstein's anomaly (EA) are not well understood; hence, we aimed to clarify these in a large cohort of EA patients. METHODS: Patients with a confirmed diagnosis of EA were retrospectively reviewed. Baseline characteristics were compared between patients with and without a prior history of CVA using logistic regression modeling. Cox regression analysis was used to identify predictors of CVA following initial evaluation. CVA incidence from birth and following tricuspid valve surgery were estimated using the Kaplan-Meier method. RESULTS: Nine hundred sixty-eight patients (median age 21.1 years, 41.5% male) were included, in which, 87 patients (9.0%) had a history of CVA (54 strokes, 33 transient ischemic attacks; 5 associated with brain abscesses) prior to their initial evaluation. The odds of atrial septal defect/patent foramen ovale (odds ratio [OR] 4.91; 95% CI 2.60-21.22; p = .0002) and migraines/headaches (OR 2.38; 95% CI 1.40-4.04; p = .0013) but not atrial arrhythmias (OR 0.75; 95% CI 0.44-1.30; p = .31) were significantly higher among patients with prior CVA following multivariable adjustment. Seventeen patients experienced CVA following initial evaluation; no examined variables including atrial arrhythmias (HR 2.38; 0.91-6.19; p = .076) were predictive of CVA risk. The 10-year, 50-year, and 70-year incidences of CVA were 1.4%, 15.9%, and 23.5%, respectively, with paradoxical embolism heavily implicated. CONCLUSION: Patients with EA are at substantive risk for CVA. Histories of migraines/headaches and interatrial shunts should prompt concern for paradoxical embolic CVAs. This has significant implications for all patients with atrial-level shunting.

16.
Am J Cardiol ; 124(8): 1293-1297, 2019 Oct 15.
Artigo em Inglês | MEDLINE | ID: mdl-31439278

RESUMO

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.

17.
Methodist Debakey Cardiovasc J ; 15(2): 138-144, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31384377

RESUMO

Ebstein's anomaly is a malformation of the tricuspid valve with myopathy of the right ventricle (RV) that presents with variable anatomic and pathophysiologic characteristics, leading to equally variable clinical scenarios. Medical management and observation is often recommended for asymptomatic patients and may be successful for many years. Tricuspid valve repair is the goal of operative intervention; repair also typically includes RV plication, right atrial reduction, and atrial septal closure or subtotal closure. Postoperative functional assessments generally demonstrate an improvement or relative stability related to degree of RV enlargement, RV dysfunction, RV fractional area change, and tricuspid valve regurgitation.


Assuntos
Procedimentos Cirúrgicos Cardíacos , Fármacos Cardiovasculares/uso terapêutico , Anomalia de Ebstein/terapia , Insuficiência da Valva Tricúspide/terapia , Valva Tricúspide/efeitos dos fármacos , Valva Tricúspide/cirurgia , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Fármacos Cardiovasculares/efeitos adversos , Anomalia de Ebstein/complicações , Anomalia de Ebstein/diagnóstico por imagem , Anomalia de Ebstein/fisiopatologia , Hemodinâmica/efeitos dos fármacos , Humanos , Recuperação de Função Fisiológica , Resultado do Tratamento , Valva Tricúspide/anormalidades , Valva Tricúspide/fisiopatologia , Insuficiência da Valva Tricúspide/tratamento farmacológico , Insuficiência da Valva Tricúspide/etiologia , Insuficiência da Valva Tricúspide/fisiopatologia , Função Ventricular Direita/efeitos dos fármacos
18.
Ann Thorac Surg ; 108(5): 1410-1415, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31323213

RESUMO

BACKGROUND: There are limited data on the incidence of prosthetic valve dysfunction (PVD) after pulmonary valve replacement (PVR). The purpose of this study was to determine the longevity of bioprosthetic valves in the pulmonary position and the factors associated with bioprosthetic valve longevity in adults with congenital heart disease. METHODS: This retrospective review of adults with bioprosthetic PVR was conducted at the Mayo Clinic in Rochester, Minnesota from 1990 to 2017. The study end point was PVD, defined as peak velocity greater than 4 m/s, severe prosthetic regurgitation, or both. For the purpose of this study we assessed bioprosthetic valve longevity by using 3 different indices: (1) cumulative incidence of PVD, (2) incidence density of PVD, and (3) time to PVD. RESULTS: There were 807 bioprosthetic PVRs in 573 patients. PVD occurred in 267 (33%) prostheses. Time to PVD was 12.6 ± 3.9 years, the incidence of PVD was 3.2 (95% confidence interval [CI], 3.0 to 3.4) cases per 100 prosthesis-years, and the 15-year cumulative incidence was 48% (95% CI, 43%- to 53%). No difference in prosthesis longevity by type of prosthesis implanted was observed. The multivariate risk factors for PVD were a history of atrial fibrillation (hazard ratio [HR], 1.36; 95% CI, 1.08 to 2.54; P = .014), and greater than moderate right ventricular dysfunction (HR, 1.21, 95% CI, 1.01 to 1.48; P = .049). Postoperative anticoagulation with a vitamin K antagonist was associated with a lower risk of PVD (HR, 0.83; 95% CI, 0.61 to 0.92; P = .038). CONCLUSIONS: The limited longevity of bioprosthetic valves poses significant concerns about the cumulative lifetime risk of reinterventions. Prospective studies are required to determine if interventions such as treatment of atrial fibrillation and postoperative anticoagulation will delay the occurrence of PVD.

19.
Can J Cardiol ; 35(7): 914-922, 2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-31292091

RESUMO

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.

20.
Am J Cardiol ; 124(5): 803-807, 2019 09 01.
Artigo em Inglês | MEDLINE | ID: mdl-31272701

RESUMO

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.


Assuntos
Procedimentos Cirúrgicos Cardíacos/mortalidade , Causas de Morte , Tetralogia de Fallot/mortalidade , Tetralogia de Fallot/cirurgia , Centros Médicos Acadêmicos , Adulto , Fatores Etários , Procedimentos Cirúrgicos Cardíacos/métodos , Bases de Dados Factuais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Minnesota , Valor Preditivo dos Testes , Prognóstico , Modelos de Riscos Proporcionais , Estudos Retrospectivos , Medição de Risco , Fatores Sexuais , Estatísticas não Paramétricas , Análise de Sobrevida , Tetralogia de Fallot/diagnóstico por imagem , Resultado do Tratamento
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