Citations

Background Premature coronary artery disease ( CAD ) is common in patients with coarctation of aorta ( COA ), but there are limited data about any direct relationship (or lack thereof) between COA and CAD . We hypothesized that atherosclerotic cardiovascular disease risk factors, rather than COA diagnosis, was the primary determinant of CAD occurrence in patients with COA . Methods and Results This is a retrospective study of 654 COA patients and a control group of 876 patients with valvular pulmonic stenosis and tetralogy of Fallot to determine prevalence and independent risk factors for CAD . There was no evidence of a difference in the unadjusted CAD prevalence between the COA and control groups (7.8% versus 6.3%, P =0.247), but premature CAD was more common in COA patients (4.4% versus 1.8%, P =0.002). In the analysis of a propensity‐matched cohort of 126 COA and 126 control patients, there was no evidence of a difference in overall CAD prevalence (6.3% versus 5.6% versus P =0.742) and premature CAD prevalence (4.8% versus 3.2%, P =0.518). The multivariable risk factors for CAD were hypertension (odds ratio [ OR ] 2.14; 95% CI 1.36–3.38), hyperlipidemia ( OR 3.33; 95% CI 2.02–5.47), diabetes mellitus ( OR 1.98; 95% CI 1.31–3.61), male sex ( OR 2.05; 95% CI 1.33–3.17), and older age per year ( OR 1.06; 95% CI 1.04–1.07). Conclusions After adjusting for atherosclerotic cardiovascular disease risk factors, we did not find evidence of a difference in CAD risk between the patients with COA and other patients with congenital heart disease.

Background The purpose of this study was to compare the incidence of pregnancy‐related adverse outcomes ( PRAO ) between patients with versus without hemodynamically significant right ventricle outflow tract ( RVOT) . Methods and Results This was a retrospective cohort study of all pregnant patients with isolated RVOT lesions undergoing evaluation at the Mayo Clinic, 1990 to 2017. Hemodynamic significance was defined as ≥moderate pulmonary/conduit stenosis (≥3 m/s) and/or ≥moderate regurgitation. Patients with concomitant significant left heart disease were excluded. PRAO was defined as cardiovascular, obstetric, and/or neonatal complications occurring during the pregnancy through 6 weeks postpartum. A total of 224 pregnancies in 114 patients with RVOT lesions were identified; 38 pregnancies occurred in 24 patients with hemodynamically significant RVOT . Forty‐eight (21%) pregnancies ended in spontaneous abortion. Of the 173 completed pregnancies, median gestational age at delivery was 38 (35–40) weeks and median birth weight 2965 (2065–4122) g. Seven pregnancies (4%) were complicated by cardiovascular events, 14 (8%) by obstetric complications, with adverse neonatal outcomes occurring in 38 (22%). There were no maternal deaths. The incidence of spontaneous abortion and PRAO were similar in both the RVOT and hemodynamically significant RVOT groups. As an isolated condition, Tetralogy of Fallot–pulmonary atresia was associated with spontaneous abortion and neonatal complications. Conclusions The risk of cardiovascular complications was low in patients with isolated RVOT lesions, and hemodynamically significant RVOT lesions were not associated with either cardiovascular complications or PRAO . Further studies are required to explore the factors responsible for PRAO in patients with Tetralogy of Fallot–pulmonary atresia.

Background A previous study reported histologic abnormalities in the pulmonary artery ( PA ) of patients with tetralogy of Fallot ( TOF ). However, the potential effect of these anatomical findings on PA vascular function has not been studied. We hypothesized that TOF patients had abnormal PA vascular function, and that PA vascular function was associated with exercise capacity. Methods and Results This a study of adult TOF patients who had cardiac magnetic resonance imaging and echocardiogram (on the same day) at Mayo Clinic, 2002–2015. In order to test the study hypothesis, we compared PA elastance index ( PAE i) between 207 TOF patients and a referent group of 8 subjects without structural heart disease. PAE i was calculated as a quotient of PA systolic pressure and cardiac magnetic resonance imaging–derived right ventricular stroke volume. Mean age was 33±13 and 36±4 years in the TOF and referent groups respectively. TOF patients had higher PAE i compared with the referent group (0.62±0.12 versus 0.48±0.08 mm Hg/ mL /m 2 ; P =0.001). There was a good correlation between PAE i and peak oxygen consumption (adjusted R 2 =0.73; r =0.85; P <0.001). After multivariate adjustment for potential confounders, PAE i was independently associated with peak oxygen consumption (adjusted R 2 =0.69; r =0.83; P <0.001). Conclusions The high PA elastance in the TOF group may be attributed to abnormal PA vascular function. The association between PAE i and exercise intolerance suggests that PA vascular dysfunction may contribute to exercise intolerance, which is an important clinical problem in this population. Further studies are required to validate our findings and explore potential therapies to improve PA vascular function in this population.

Background In Fontan patients with atrial arrhythmias (AA), non-vitamin K antagonist oral anticoagulants(NOACs) have a class III recommendation according to the Pediatric & Congenital Electrophysiology Society (PACES)/Heart Rhythm Society (HRS) guideline in 2014, due to lack of data on outcomes as opposed to evidence of harm. To address this gap in data, we investigated the safety and efficacy of NOACs in adults with a Fontan circulation in a worldwide study. Methods This is an international multicentre prospective cohort study, using data from the NOTE ( n on-vitamin K antagonist o ral anticoagulants for t hrombo e mbolic prevention in patients with congenital heart disease) registry. The study population comprised consecutive adults with a Fontan circulation using NOACs. Follow-up took place at 6 months and yearly thereafter. The primary endpoints were thromboembolism and major bleeding. Secondary endpoint was minor bleeding. Results From April 2014 onward, 74 patients (mean age 32±10 years (range 18–68), 54% male) with a Fontan circulation using NOACs were included. During a median follow-up of 1.2 (IQR 0.8–2.0) years, three thromboembolic events (2.9 per 100 patient-years (95% CI 0.7 to 7.6)) and three major bleedings (2.9 per 100 patient-years (95% CI 0.7 to 7.6)) occurred in five atriopulmonary Fontan and one total cavopulmonary connection Fontan patients with AA. Fifteen patients experienced minor bleeding episodes (15.8 per 100 patient-years (95% CI 9.1 to 25.2)). In patients (n=37) using vitamin K antagonists (VKAs) prior to the initiation of NOAC, annual incidence of historical thromboembolic events and major bleeding were 2.4% (95% CI 0.4% to 7.4%) (n = 2) and 1.2% (95% CI 0.7% to 5.1%) (n = 1), respectively. Conclusions In this review of the largest Fontan cohort using NOACs with prospective follow-up, NOACs appear to be well tolerated and their efficacy and safety during short-term follow-up seem comparable to VKAs. Longer term data are required to confirm these promising short-term results.

AbstractObjectiveTo assess the association between elevated ventricular‐end diastolic pressures (VEDP) and pulmonary artery wedge pressure (PAWP) on long‐term survival in adult Fontan patients.BackgroundThe impact of ventricular filling pressures on long‐term survival in adults post‐Fontan palliation is unknown.MethodsWe included 148 adult Fontan patients (age ≥ 18 years) without atrioventricular valve prosthesis or pulmonary vein stenosis undergoing arterial and venous catheterization between December 1999 and November 2017. VEDP was defined as ≥12 mmHg and PAWP as >12 mmHg based on optimal cut‐offs for prediction of mortality on receiver‐operator curves (AUC 0.63 and 0.66, respectively).ResultsMean age was 31.3 ± 9.2 years and 48.6% of patients were females. Most common congenital defects were tricuspid atresia (36.4%) and double‐inlet left ventricle (28.3%); 59.5% patients had atriopulmonary Fontan connections. Mean VEDP was 11.5 ± 4.7 mmHg and PAWP 10.6 ± 4.5 mmHg (correlation coefficient .76). During a follow‐up of 6.0 ± 4.8 years (median 5.4, IQR 1.4–9.4), there were 45 deaths (30.4%). Overall survival was lower in patients with VEDP ≥ 12 compared to those with VEDP < 12 mmHg (p = .02). Similarly, survival was lower in patients with PAWP>12 compared to patients with PAWP ≤ 12 mmHg (p < .0001). In the multivariate model, PAWP was an independent predictor of death (HR 1.1 per mmHg, 95% CI 1.02–1.15, p = .009) whereas VEDP was not (HR 1.1 per mmHg, 95% CI 1.0–1.13; p = .08).ConclusionPAWP but not VEDP was independently associated with long‐term overall mortality in adult Fontan patients. Perhaps PAWP rather than VEDP should be used in the risk stratification of these patients.

Background Turner syndrome ( TS ) is the most common sex chromosome abnormality in women and is associated with increased morbidity and mortality. We describe long‐term outcomes in a large cohort of patients with TS . Methods and Results Retrospective review of patients with TS followed at Mayo Clinic Rochester from 1950 to 2017 was performed. Clinical, imaging, surgical, and genetic data were analyzed. Survival analysis was performed with the Kaplan–Meier method using age‐ and sex‐matched Olmsted County residents as the reference group. The study cohort comprised 317 patients with TS . Average age at diagnosis was 9 (range, 2–12) years, genetic testing was performed in 202 (64%), and pure monosomy X was present in 75 (37%). Congenital heart disease occurred in 131 (41%), with the most frequent lesions being bicuspid aortic valve (n=102, 32%) and coarctation of the aorta (n=43, 14%). Ascending aortic dilation was common, with mean aortic root size index 2 cm/m 2 , and aortic dissection occurred in 6 (2%) patients. The average follow‐up was 11 (range, 2–26) years, yielding 3898 patient‐years, and during this period 46 (14%) patients died; mean age at the time of death was 53±17 years. Patients with TS had reduced survival compared with the control group (82% versus 94% at 30 years; P <0.001), and the leading causes of death were cardiovascular disease, liver disease, and malignancy. Conclusions Patients with TS have reduced survival compared with age‐matched controls, and cardiovascular disease is the major cause of death. Further studies are required to determine if targeted cardiovascular risk factor modification will result in improved survival in this population.