Connolly, Heidi M.

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.

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.

Background Thoracic aortic aneurysm is common in patients with tetralogy of Fallot ( TOF ); the incidence of thoracic aortic dissection ( TAD ) is unknown, but generally considered to be uncommon. The purpose of this study was to determine incidence and risk factors for TAD in TOF patients. Methods and Results This work is a retrospective review of the National Inpatient Sample ( NIS ) database for cases of ascending TAD among all hospital admissions in adults with TOF , 2000–2014. Of 18 353 admissions in TOF patients, 11 (0.06%; 6 per 10 000 admissions) of these were TAD ‐related admissions. For the TAD ‐related admissions, mean age was 49.8±7.2 years; aortic surgical interventions were performed during 8 of the admissions, and overall in‐hospital mortality was 45% (5 of 11). Risk factors associated with TAD ‐related admission were age >60 years (odds ratio, 2.41; 95% CI, 1.23–3.25; P =0.013), male sex ( odds ratio, 6.91; 95% CI, 4.85–8.54; P <0.001), and hypertension (odds ratio, 1.74; 95% CI, 1.06–3.19; P =0.037). Conclusions This is the first population‐based study of TAD outcomes in patients with TOF , and it showed a low risk of TAD in this population. This has important clinical implication with regard to surveillance and surgical intervention. Given that prevalence of thoracic aortic aneurysm is higher in TOF patients compared with the general population; therefore, making clinical decisions based on outcomes data and practice guidelines derived from patients with degenerative and bicuspid aortic valve–related aortopathies may lead to overtreatment . Further studies are required to better understand the pathobiology of this disease in order to make evidence‐based recommendations for surveillance and treatment.

Background Available outcomes data for anticoagulation therapy in adults with congenital heart disease ( CHD ) provide assessment of global risk of this therapy for CHD patients (a heterogeneous population), but the risk of complications for the different CHD diagnoses is unknown. The purpose of the study was to describe the indications for anticoagulation, and the incidence and risk factors for major bleeding complication in adults with tetralogy of Fallot. Methods and Results We queried Mayo Adult Congenital Heart Disease (MACHD) database for tetralogy of Fallot patients (aged ≥18 years) that received anticoagulation, 1990–2017. Of 130 patients (42±14 years, 75 men [58%]), warfarin and direct oral anticoagulants were used in 125 (96%) and 5 (4%), respectively because atrial arrhythmias (n=109), mechanical prosthetic valve (n=29), intracardiac thrombus (n=4), pulmonary embolism (n=6), stroke (n=3), and perioperative anticoagulation (n=44). The median hypertension, abnormal renal or liver function; stroke; bleeding history or predisposition; labile international normalized ratio; elderly (>65 years); drug or alcohol use score for the entire cohort was 1 (0–2) and 27 (21%) had hypertension, abnormal renal or liver function; stroke; bleeding history or predisposition; labile international normalized ratio; elderly (>65 years); drug or alcohol use score ≥2. There were 14 minor bleeding events (1.6% per year) and 11 major bleeding events (1.3% per year) in 8 patients during median follow‐up of 74 months (856 patient‐years). Mechanical prosthesis (hazard ratio 1.78, CI 1.29–3.77, P =0.021) and hypertension, abnormal renal or liver function; stroke; bleeding history or predisposition; labile international normalized ratio; elderly (>65 years); drug or alcohol use score ≥2 (hazard ratio 1.41, CI 1.03–3.88, P =0.046) were risk factors for major bleeding events. All‐cause mortality was higher in patients with major bleeding events (n=6, 75%) compared with patients without major bleeding events (n=25, 21%), P =0.001. Conclusions Considering the heterogeneity of the CHD population, data from the current study may be better suited for clinical decision‐making in tetralogy of Fallot patients.

AbstractObjectiveTo review our experience with balloon testing prior to atrial septal defect (ASD) closure in adults with left ventricular (LV) diastolic dysfunction.BackgroundASD closure in patients with LV diastolic dysfunction may precipitate LV failure. Temporary ASD occlusion has been used in this scenario but data are limited.MethodsRetrospective review of 27 patients age ≥ 50 years undergoing temporary ASD balloon occlusion between 2000 and 2018 for suspected LV diastolic dysfunction or elevated LV end‐diastolic pressure (LVEDP).ResultsMedian age was 72 years (IQR 66.7; 75.2). Atrial fibrillation was seen in 58% of patients, hypertension in 58%, and coronary artery disease in 26%; 52% were females. Median ASD size was 13 mm (10; 18) and Qp/Qs 1.8 (1.6; 2.2). Median LVEDP was 14 mmHg (12; 22); pulmonary artery wedge pressure (PAWP) 12 mmHg (9; 16.5) and left atrial pressure (LAP) 13.5 mmHg (8; 16.3). After a median of 5 min (3; 10) of balloon occlusion, patients with baseline LVEDP ≥15 mmHg had more significant increases in LVEDP (9 [6; 12] vs. 2 mmHg [0.5; 5]; p = 0.03) and LAP/PAWP (10.5 [8.3; 16.3] vs. 1.5 mmHg [−1.5; 3]; p = 0.0003) than those with baseline LVEDP <15 mmHg. None of those with a baseline LVEDP <15 mmHg had a LAP/PAWP >15 mmHg during balloon testing compared to 92% of patients with a baseline LVEDP ≥15 mmHg.ConclusionLVEDP might be used to predict LAP post‐ASD closure. Comorbidities typically associated with LV diastolic dysfunction are common in these patients and should be considered in their management.