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BackgroundPatients with Fontan circulation are known to be at high risk for developing atrial tachyarrhythmias (AAs). Our objective was to examine the efficacy and safety of amiodarone in the management of ATs in adult Fontan patients.MethodsPrimary outcomes of this single-centre, retrospective study included freedom from AAs and incidence of adverse effects of amiodarone on Fontan patients. Heart failure (HF) events and composite outcomes of death from any cause, Fontan revision and heart transplantation were evaluated as secondary outcomes. Predictors of HF and discontinuing amiodarone were also evaluated.ResultsA total of 61 patients (mean age 31.6±11.3 years, 40.9% female), who were treated with amiodarone in between 1995 and 2018, were included. AAs free survival at 1, 3 and 5 years were 76.2%, 56.9% and 30.6%, respectively. During a median follow-up of 50.5 months, 34 (55.7%) patients developed side effects, and 20 (32.8%) patients discontinued amiodarone due to side effects. Thyroid dysfunction was the most common side effect (n=26, 76.5%), amiodarone-induced thyrotoxicosis (AIT) (n=16, 27.1%) being most common thyroid dysfunction. Young age (age ConclusionsShort-term efficacy of amiodarone in Fontan physiology is acceptable. However, long-term administration is associated with a reduction of efficacy and a significant prevalence of non-cardiac side effects. AIT is associated with exacerbation of HF. The judicious use of amiodarone administration should be considered in this population.

PURPOSE To evaluate the diagnostic utility of the Look Locker inversion time (TI) sequence on cardiac magnetic resonance imaging in patients with suspected cardiac amyloidosis and to evaluate whether there are differences in the nulling pattern between amyloid types. MATERIALS AND METHODS A total of 144 patients with suspected cardiac amyloidosis who had undergone cardiac magnetic resonance imaging were included in this retrospective study. Sixty-four had cardiac amyloidosis (62.1±9.2 y, 70.3% male, 68.8% had light chain amyloid [AL], 18.8% had familial transthyretin amyloid caused by mutant genes [ATTRm], and 12.5% had wild-type transthyretin amyloid [ATTRwt]) and 80 did not have cardiac amyloidosis (61.3±13.3 y, 58.8% male). Time to myocardial and blood pool nulling on the Look Locker TI sequence was classified as normal if blood pool nulled before myocardium or abnormal if blood pool nulling was coincident with or after myocardial nulling. RESULTS The nulling pattern was abnormal in 26 patients with cardiac amyloidosis compared with none of the patients without cardiac amyloidosis (40.6% vs. 0.0%, P

BackgroundPatients on dialysis have impaired cardiac function, in part due to increased fluid volume and ventricular stress. Restored kidney function through transplantation reduces left ventricular volume in both systole and diastole. Paraoxonase 1 (PON1) is reduced in patients on dialysis, which may be related to their impaired cardiac function. We tested the hypothesis that change in PON1 is associated with changes in left ventricular (LV) end-volume and LV mass after kidney transplantation.MethodsPatients were studied before and 12 months after kidney transplantation. The control group was patients on dialysis not expected to receive a transplant in the next 12 months. Cardiac magnetic resonance imaging was used to measure LV end-diastolic and end-systolic volume and LV mass. PON1 was measured by arylesterase activity and by mass.ResultsPON1 mass and activity were not different between the groups at baseline. Both PON1 mass and activity were increased post-kidney transplantation (p2.7) (p=0.0146 and 0.0114 for diastolic and systolic, respectively) and with the change in hemoglobin (p=0.0042).ConclusionsPON1 mass and activity increase after kidney transplantation. The increase in PON1 mass is consistent with a novel relationship to the increase in hemoglobin and the decrease in LV end-systolic and end-diastolic volume.

Eisenmenger syndrome (ES) is considered a contraindication to pregnancy given the high risk of maternal mortality. A 35-year-old woman with ES presented at 12 weeks gestation (ga) despite counselling to avoid pregnancy (Figure 1). She was on tadalafil with a resting oxygen saturation of 91%.

The prevalence of congenital coronary anomalies is between 0.3% and 2%, depending on the definition used and the population studied [1,2,3]. Not all congenital coronary anomalies are correlated with negative outcomes. Only anomalous coronaries with an inter-arterial or intramural course, coronary fistulae, and anomalous left coronary artery from the pulmonary artery (ALCAPA) have been associated with an increased rate of cardiac events [4] . Classical definitions of “high-risk features” do not always correlate with necropsy studies [5]. Despite the uncertainty, stakes are high, as coronary anomalies are the secondmost prevalent cause of sudden cardiac death in young athletes [6]. Surgical series report good outcomes with surgical repair, but observational cohorts also report excellent outcomes [7,8]. Guidelines regarding themanagement of coronary anomalies provide indications for surgical repair, but they are different from the management paradigm practiced by most pediatric cardiologists [9,10] .

Background: Recommended thresholds for pulmonary valve replacement (PVR) in asymptomatic patients after tetralogy of Fallot (TOF) repair are continually decreasing. We studied the natural history versus outcomes after PVR in various categories of indexed right ventricular end-diastolic volume (RVEDVi). Hypothesis: We hypothesized that the benefit of PVR would not be outweighed by the risk at lower RVEDVi thresholds. Methods: Acquisition of all repeated measure datapoints and cross-sectional review in 2579 patients (born 1924 - 2011), including 7553 echos, 2579 MRI scans and all interventional data. Analysis was via parametric competing risks techniques and time-related regressions adjusted for repeated measures. Results: Survival was 95%, 81% and 73% at age 20, 40 and 60 years respectively. Freedom from PVR (N=516, mean age 25 years) was 87%, 66% and 45% at 20, 40 and 60 years after repair. Overall, survival after PVR was 98%, 95% and 87% at 1, 10 and 20 years. PVR-free survival in all 345 patients with MRI RVEDVi > 150 was 95% at 15 years. Comparative survival showed no survival advantage with PVR versus natural history for RVEDVi 150-160, 160-170, 170-180 (table); these categories had excellent PVR-free survival approaching 100% at 10 years. Patients with RVEDVi > 200 had late survival decrements with PVR or without. PVR resulted in a large and significant reduction in RVEDVi (mean 40 ml/m 2 , P 150 ml/m 2 . Conclusions: Lowering the RVEDVi threshold for PVR does not appear to offer a clear survival advantage and offers small differences (if any) to RV geometry, risks potential procedure-related morbidity and would mean intervening on many teenagers with repaired TOF who could otherwise anticipate intervention in later life.

Background: Vasodilator stress perfusion cardiovascular magnetic resonance imaging is a clinically useful tool for detection of clinically significant myocardial ischaemia in adults. We report our 5-year retrospective experience with perfusion cardiovascular magnetic resonance in a large, quarternary adult congenital heart disease centre. Methods: We reviewed all cases of perfusion cardiovascular magnetic resonance in patients referred from the adult congenital heart disease service. Dipyridamole stress perfusion cardiovascular magnetic resonance was undertaken on commercially available 1.5 and 3 T cardiovascular magnetic resonance scanners. Late gadolinium enhancement imaging was performed 8–10 minutes after completion of the rest perfusion sequence. Navigator whole-heart coronary magnetic resonance angiography was also performed where feasible. Results of stress cardiovascular magnetic resonance were correlated with complementary imaging studies, surgery, and clinical outcomes. Results: Over 5 years, we performed 34 stress perfusion cardiovascular magnetic resonance examinations (11 positive). In all, 84% of patients had further investigations for ischaemia in addition to cardiovascular magnetic resonance. Within a subgroup of 19 patients who had definitive alternative assessment of their coronary arteries, stress perfusion cardiovascular magnetic resonance demonstrated a sensitivity of 82% and specificity of 100%. Of the 34 studies, two were false negatives, in which the aetiology of ischaemia was extrinsic arterial compression rather than intrinsic coronary luminal narrowing. Coronary abnormalities were identified in 71% of cases who had coronary magnetic resonance angiography. Conclusion: Stress perfusion cardiovascular magnetic resonance is a useful and accurate tool for investigation of myocardial ischaemia in an adult congenital heart disease population with suspected non-atherosclerotic coronary abnormalities.

Objective The left ventricle (LV) is routinely assessed with cardiac magnetic resonance imaging (MRI) by using short-axis orientation; it remains unclear whether the right ventricle (RV) can also be adequately assessed in this orientation or whether dedicated axial orientation is required. We used phase-contrast (PC) flow measurements in the main pulmonary artery (MPA) and the ascending aorta (Aorta) as nonvolumetric standard of reference and compared RV and LV volumes in short-axis and axial orientations. Methods A retrospective analysis identified 30 patients with cardiac MRI data sets. Patients underwent MRI (1.5 T or 3 T), with retrospectively gated cine steady-state free-precession in axial and short-axis orientations. PC flow analyses of MPA and Aorta were used as the reference measure of RV and LV output. Results There was a high linear correlation between MPA-PC flow and RV–stroke volume (SV) short axis ( r = 0.9) and RV-SV axial ( r = 0.9). Bland-Altman analysis revealed a mean offset of 1.4 mL for RV axial and −2.3 mL for RV–short-axis vs MPA-PC flow. There was a high linear correlation between Aorta-PC flow and LV-SV short-axis ( r = 0.9) and LV-SV axial ( r = 0.9). Bland-Altman analysis revealed a mean offset of 4.8 m for LV short axis and 7.0 mL for LV axial vs Aorta-PC flow. There was no significant difference ( P = .6) between short-axis–LV SV and short-axis–RV SV. Conclusion No significant impact of the slice acquisition orientation for determination of RV and LV stroke volumes was found. Therefore, cardiac magnetic resonance workflow does not need to be extended by an axial data set for patients without complex cardiac disease for assessment of biventricular function and volumes.

A 71-year-old asymptomatic woman is found to have an incidental cardiac mass on transthoracic echocardiography. Cardiac magnetic resonance (CMR) findings are consistent with lipotamous hypertrophy of the inter-atrial septum. Given the characteristic appearances on CMR, biopsy or surgery was not indicated and the patient was managed conservatively.

Background: Do adult Fontans with RV-dominant single ventricle physiology (RV) have worse prognosis versus patients with LV-dominance (LV) or unseptated ventricles? Methods: 180 adult Fontans (median age 25) were reviewed. Endpoints relating to Fontan failure and exercise capacity were compared.

To investigate four-dimensional (4D) phase-contrast (PC) magnetic resonance (MR) in the evaluation of intracardiac shunts by simultaneous assessment of pulmonary (QP) and systemic (QS) flows in a pilot study and to compare results to through-plane two-dimensional (2D) PC MR.Institutional review board approval and written informed consent were obtained. Nineteen patients with suspected intracardiac shunts underwent cardiac MR at 1.5T. Assessments of QP and QS were performed using free-breathing retrospectively gated 2D PC gradient recalled echo (GRE; 1.6 × 1.6 × 5 mm(3)) imaging with one-dimensional through-plane velocity encoding gradient (venc = 150 cm/s) in consecutive measurements for the main pulmonary artery (MPA) and ascending aorta (AA), respectively. A prospectively triggered 4D PC GRE technique (2.4 × 1.8 × 3 mm(3)) with three orthogonal venc directions was also used with volume coverage of both MPA and AA.QP and QS assessed by 4D PC correlated with 2D PC acquisitions (r = 0.92 and r = 0.67 respectively; P .0001 for both) but demonstrated significant underestimation of individual flow volumes (-21.9 ± 12.2 mL; P .0001 and -10.7 ± 13.1 mL; P = .0023, respectively). Calculated QP:QS ratios demonstrated high correlation (r = 0.78; P .0001) and no significant differences between 4D PC and 2D PC acquisitions (-0.09 ± 0.24, P = .14). Image acquisition times for 2D PC assessment of QP and QS were 2.98 ± 0.52 and 2.84 ± 0.50 minutes, respectively (P = .038), whereas time to acquire 4D PC images was significantly longer, 18.75 ± 4.58 minutes (P .001).Four-dimensional PC MR imaging allows for accurate assessment of QP:QS ratios in the evaluation of intracardiac shunts while absolute flow volumes demonstrate offsets. Further refinement of the technique with improvement in acquisition times may be required before widespread clinical implementation.

Recommendations for the peripartum obstetric management of women with heart disease have included early induction of labour, shortening the second stage of labour during vaginal delivery, and low threshold for elective Caesarean section, although such techniques may result in complications. The objective of this study was to determine whether a less aggressive approach without routine preterm induction, shortening of the second stage, or Caesarean section adversely affects the mother or neonate.We examined peripartum obstetric management and its relationship with adverse maternal and neonatal outcomes in 1677 pregnancies: 559 in women with heart disease and 1118 in women without heart disease (control subjects). Logistic regression with propensity matching was used to compare outcomes in women with and without heart disease.Women with heart disease were more likely than control subjects to undergo induction of labour (P0.001). Induction of labour tended to be at term and for logistical reasons, not for the indication of maternal heart disease. Assisted vaginal deliveries were more common in women with heart disease (29% vs. 11%, P0.001) than in those without, and the second stage of labour was also more prolonged in women with heart disease. Rates of Caesarean section were similar in both groups (P = 0.66). A significant proportion of women with heart disease had unassisted vaginal deliveries. Invasive cardiac monitoring was rarely used. Adverse maternal cardiac events at delivery were rare (2% of pregnancies) and were not associated with mode of delivery. In multivariate analysis, maternal heart disease was not predictive of adverse neonatal events or third- or fourth- degree lacerations. Maternal heart disease was associated with postpartum hemorrhage, but this was not related to assisted delivery or prolonged second stage of labour.This large study has shown that in women with heart disease, avoidance of early induction of labour, rare use of Caesarean section for cardiac indications, and selective use of invasive monitoring produces safe obstetric outcomes.

Background In congenitally corrected transposition of the great arteries (cc-TGA), the morphologic right ventricle acts as the subaortic (systemic) ventricle, and deterioration of the ventricle over time is a well-known complication. The objective of this study was to characterize the right ventricle and explore factors that may be contributing to ventricular dilation and dysfunction and the relationship with exercise capacity. Methods This was a prospective study of adults with cc-TGA. All patients underwent cardiopulmonary stress testing, ventricular volume and fibrosis assessment by cardiac magnetic resonance imaging, and Velocity Vector Imaging strain echocardiography. Results Twenty-six patients were included (mean age, 38 ± 16 years; 54% women). Exercise capacity was significantly reduced in patients with cc-TGA compared with normal subjects (20.9 ± 6.0 vs 30.8 ± 9.2 mL/kg/min, P = .001). The majority of patients (61%) had right ventricular (RV) ejection fractions ≤ 40%. There was no evidence of fibrosis on cardiac magnetic resonance imaging. There was a significant difference in diastolic volumes among those with RV ejection fractions > 40% versus ≤ 40% (173 ± 29mL vs 233 ± 65 mL, P = .02) and moderate or severe versus no or mild tricuspid regurgitation (240 ± 80mL vs 190 ± 38mL, P = .04). RV apical longitudinal and mid free wall circumferential strain was decreased compared with these values in controls. Conclusions In this relatively "well" cc-TGA population, subaortic RV dilation, dysfunction, and exercise intolerance are a common problem. Significant systemic tricuspid atrioventricular valvular regurgitation is an important contributor to the problem. In this study, subaortic RV myocardial deformation parameters were found to be abnormal, suggesting that there is a failure of the ventricle to adapt to systemic pressures, and therapies to reduce afterload should be explored. Recurrent ischemia resulting in fibrosis likely does not contribute to RV dilation or dysfunction as demonstrated by the magnetic resonance imaging findings in this study.

Background MRI is deemed standard of reference for assessment of ventricular volumes and function. However, it remains unclear if the RV can also be adequately assessed in SAO orientation as used for assessment of LV function or requires dedicated axial-Cine imaging. Additional axial-Cine acquisition adds 10-15 min of scan time and thus has a substantial impact on clinical workflow and patient comfort.

Although survival of patients with repaired tetralogy of Fallot (TOF) into adulthood exceeds 90%, hemodynamic and electrophysiologic abnormalities contribute to substantial morbidity in this population. The rate of late complications such as exercise intolerance, heart failure, tachyarrhythmias, and death accelerates in the third decade of life. However, identifying predictors for adverse outcomes remains difficult, as the event rates for major outcomes, such as ventricular tachycardia (VT) and death are low. Small, single center studies have suggested that cardiac magnetic resonance (CMR) measures of ventricular size and function are independent predictors of major adverse clinical outcomes. However, the small number of outcome events and the single center study design limit general acceptance of these findings. A multicenter registry may allow identification of generalizable predictors of major adverse outcomes and will provide opportunities for robust analyses to address clinically relevant questions in this population.

A 41-year-old man was admitted with hemoptysis and a 10-year history of progressive exertional decline. He had a patent ductus arteriosus diagnosed in adult life that was unrepaired because of concomitant pulmonary hypertension. On examination, he had a sustained right ventricular impulse with a

Methods Steady-state free precession cine acquisitions across the aortic root in short axis in patients with a diagnosis of BAV from January 2006 to December 2008 were evaluated at our institution using a 1.5 T magnet. For each aortic root, end-systolic measurements were obtained in duplicate by a single observer from cusp-to-commissure (3 measurements: right, left and non-coronary cusps to opposite commissure respectively) and cusp-to-cusp (3 measurements: right-left, right-non and left-non coronary cusps) using CMR. Using TTE, measures of the aortic root were recorded from the parasternal long axis view. Aortic root asymmetry was defined by comparing the standard deviation (SD) of the average of the 3 measurements of an individual (SDi) to the SD of the entire population (SDp). Two groups were generated: symmetric (SDi SDp) aortic roots.

Background Summary: To examine the relationship between cardiovascular magnetic resonance imaging (CMR) parameters and pregnancy outcomes post Mustard palliation. Background: Impaired systemic ventricular function and presence of a systemic right ventricle (RV) are associated with adverse events in pregnancy. Women with transposition of the great arteries post atrial switch (Mustard procedure) are at risk of worsening arrhythmia and heart failure antenatally. Contemporary guidelines suggest that women with “more moderate systemic RV dysfunction” should be advised against pregnancy; however a threshold RV ejection fraction (EF) has not been defined as all studies to date have examined RV function using echocardiography alone. The CMR characteristics of women post Mustard palliation undergoing pregnancy and the relationship between CMR parameters and pregnancy outcomes have not yet been described. Methods A total of 17 consecutive women post Mustard procedure seen at 2 tertiary care centers who had undergone CMR within 2 years of pregnancy were included. Parameters of ventricular function were assessed by a single experienced reader using steady-state free-precession cine CMR acquired in the short-axis orientation. Adverse cardiovascular events (sustained arrhythmia, heart failure, stroke, cardiac arrest and/or urgent cardiac intervention), obstetric complications (eclampsia, pre-term labour, hemorrhage) and fetal/neonatal events (stillbirth/death, prematurity, low birthweight, intensive care unit admission) were recorded. Results

Background ARVC is a rare potentially life-threatening inherited disease of the desmosome. Right ventricular wall motion abnormalities combined with right ventricular dilatation or impaired function on CMR are diagnostic criteria for this disease. The role of LGE in this disease is still under debate. This study was performed to investigate the yield of LGE by CMR in patients referred with suspected ARVC. Methods

Background The most common cause of a systemic right ventricle is atrial redirection surgery (Mustard repair) in the setting of complete TGA. Decreased RVEF is common and is a predictor of morbidity and mortality. Cardiac magnetic resonance (CMR) cine imaging is the reference standard for assessment of right heart size and function. However, the optimal method of RV planimetry using CMR in patients with Mustard palliation for complete TGA remains unclear.

To examine the association between aortic dimensions on cardiovascular magnetic resonance imaging (CMR) and risk of adverse events related to pregnancy in women with coarctation of the aorta (CoA).