National management guidelines recommend that patients with moderate and complex congenital heart disease (CHD) receive life-long cardiac care (LLCC), guided in adulthood by an adult congenital heart disease (ACHD) specialist. However, the percentage of adult CHD patients who receive such care is quite low. Inadequate knowledge regarding LLCC may contribute to care interruption. We, therefore, sought to determine the knowledge of adolescents and young adults regarding LLCC. In this multi-center study, we administered a survey to patients 13–20 years of age with surgically repaired congenital heart disease. We assessed the understanding of both their need for LLCC and awareness of the type of recommended care providers. A total of 290/302 (96%) patients approached in the outpatient clinic setting (10 centers) agreed to study participation; mean age was 16.3 ± 2.3 years; patients were 62% male. While the need for LLCC was recognized by 78% of subjects, only 37% understood that an ACHD specialist in adulthood should guide this care. Only 37% of respondents stated that their current cardiology team had spoken to them about LLCC, but 90% wished to learn more. A substantial number of adolescents and young adults with moderate and complex CHD lack adequate knowledge about LLCC, but most have a desire to learn more about the type of care they will require in adulthood. Transition education/assessment ensuring successful transfer to adult-oriented care for this population should emphasize the importance of LLCC.

The present review provides a selected choice of clinical trials and therapeutic advances in the field of cardiology in 2015. A new treatment option in heart failure will become available this year in Switzerland. In interventional cardiology, new trials have been published on the duration of dual antiplatelet therapy, the new stents with bioresorbable scaffold and the long-term results of TAVR in patients who are not surgical candidates or at high surgical risk. RegardingAF the BRIDGE trial provides new evidences to guide the management of patients during warfarin interruption for surgery. Recent publications are changing the paradigm of AF treatment by showing a major impact of the management of cardiometabolic risk factors. Finally, refined criteria for ECG interpretation in athletes have been recently proposed to reduce the burden of false-positive screening.

Background: Exercise capacity following Fontan surgery is often depressed. An inability to reduce pulmonary vascular resistance appropriately during exercise may contribute to this phenomenon. The aim of this study was to determine whether administration of iloprost, a selective pulmonary vasodilator, would improve exercise function after Fontan procedure.Methods: Double-blind, randomized, placebo controlled, crossover trial. Patients performed two cardiopulmonary exercise tests (CPX) separated by <1 month. A single nebulizer treatment (iloprost or placebo) was administered before each CPX.Results: 18 patients aged 12-49 (median 17) years were recruited. Mild throat discomfort developed in 10/18 patients during iloprost administration; all but 1 were able to complete treatment. No symptoms developed during placebo treatments (p < 0.001). Two additional patients did not complete CPX: one with atrial flutter; another with developmental issues that precluded adequate CPX. In the 15 remaining subjects oxygen pulse (a surrogate for forward stroke volume) at peak exercise was higher following iloprost (median increase 1.2 ml/beat; p < 0.001). Peak V-O2 also rose (median increase 1.3 ml/kg/min; p < 0.04). Nine patients had peak V-O2 >30 ml/kg/min; each of these patients had higher peak V-O2 following iloprost. Only 3/6 patients with peak V-O2 > 30 ml/kg/min had higher peak V-O2 following iloprost (p < 0.04).Conclusions: Iloprost improves the peak oxygen pulse and peak V-O2 of patients with Fontan physiology and appears to be particularly beneficial among patients with impaired exercise function. Treatment is associated with minor side effects. These findings support the concept of pulmonary vasodilator therapy in Fontan patients with limited functional capacity. (C) 2013 Elsevier Ireland Ltd. All rights reserved.

OBJECTIVES To determine the prevalence of aortic valve dysfunction, aortic dilation, and aortic valve and ascending aortic intervention in adults with coarctation of the aorta (CoA). BACKGROUND Aortic valve dysfunction and aortic dilation are rare among children and adolescents with CoA. With longer follow-up, adults may be more likely to have progressive disease. METHODS We retrospectively reviewed all adults with CoA, repaired or unrepaired, seen at our center between 2004 and 2010. RESULTS Two hundred sixteen adults (56.0% male) with CoA were identified. Median age at last evaluation was 28.3 (range 18.0 to 75.3) years. Bicuspid aortic valve (BAV) was present in 65.7%. At last follow-up, 3.2% had moderate or severe aortic stenosis, and 3.7% had moderate or severe aortic regurgitation. Dilation of the aortic root or ascending aorta was present in 28.0% and 41.6% of patients, respectively. Moderate or severe aortic root or ascending aortic dilation (z-score > 4) was present in 8.2% and 13.7%, respectively. Patients with BAV were more likely to have moderate or severe ascending aortic dilation compared with those without BAV (19.5% vs. 0%; P < 0.001). Age was associated with ascending aortic dilation (P = 0.04). At most recent follow-up, 5.6% had undergone aortic valve intervention, and 3.2% had aortic root or ascending aortic replacement. CONCLUSION In adults with CoA, significant aortic valve dysfunction and interventions during early adulthood were uncommon. However, aortic dilation was prevalent, especially of the ascending aorta, in patients with BAV.

Pulmonary hypertension includes heterogeneous diagnoses with distinct hemodynamic pathophysiologic features. Identifying elevated pulmonary vascular resistance (PVR) is critical for appropriate treatment. We reviewed data from patients seen at referral pulmonary hypertension clinics who had undergone echocardiography and right-side cardiac catheterization within 1 year. We derived equations to estimate PVR using the ratio of estimated pulmonary artery (PA) systolic pressure (PASP(Doppler)) to right ventricular outflow tract velocity time integral (VTI). We validated these equations in a separate sample and compared them with a published model based on the ratio of the transtricuspid flow velocity to right ventricular outflow tract VTI (model 1, Abbas et al 2003). The derived models were as follows: PVR = 1.2 x (PASP/right ventricular outflow tract VTI) (model 2) and PVR = (PASP/right ventricular outflow tract VTI) + 3 if notch present (model 3). The cohort included 217 patients with mean PA pressure of 45.3 +/- 11.9 mm Hg, PVR of 7.3 +/- 5.0 WU, and PA wedge pressure of 14.8 +/- 8.1 mm Hg. Just >1/3 had a PA wedge pressure >15 mm Hg (35.5%) and 82.0% had PVR >3 WU. Model 1 systematically underestimated catheterization estimated PVR, especially for those with high PVR. The derived models demonstrated no systematic bias. Model 3 correlated best with PVR (r = 0.80 vs r = 0.73 and r = 0.77 for models 1 and 2, respectively). Model 3 had superior discriminatory power for PVR >3 WU (area under the curve 0.946) and PVR >5 WU (area under the curve 0.924), although all models discriminated well. Model 3-estimated PVR >3 was 98.3% sensitive and 61.1% specific for PVR >3 WU (positive predictive value 93%; negative predictive value 88%). In conclusion, we present an equation to estimate the PVR, using the ratio of PASP(Doppler) to right ventricular outflow tract VTI and a constant designating presence of right ventricular outflow tract VTI midsystolic notching, which provides superior agreement with catheterization estimates of PVR across a wide range of values. (C) 2013 Elsevier Inc. All rights reserved.

BACKGROUND An increasing number of adults with congenital heart disease (CHD) require implantable cardioverter-defibrillators (ICDs), yet little is known about their impact on psychological wellbeing and sexual function.OBJECTIVE To assess shock-related anxiety in adults with CHD and its association with depression and sexual function.METHODS A prospective, multicenter, cross-sectional study was conducted on adult patients with CHD with (ICD+) and without (ICD) ICDs. The Florida Shock Anxiety Scale was administered to patients with ICD+ and the Beck Depression Inventory-II to all patients. Men competed the Sexual Health Inventory for Men, and women competed the Female Sexual Function Index.RESULTS A total of 180 adults with CHD (ICD+: n = 70; ICD : n = 110; median age 32 years [interquartile range 27-40 years]; 44% women) were enrolled. The complexity of CH D was classified as mild in 32 (18%), moderate in 93 (52%), and severe in 54 (30%) subjects. In ICD recipients, a high leve l of shock-related anxiety was identified (Florida Shock Anxiety Scale score 16; interquartile range 12-23.5), which was slightly higher than the median score for ICD recipients in the genera l population (P =.057). A higher leve l of shock-related anxiety was associated with poorer sexual function scores in both men (Spearman's p =.480; P <.001) and women (Spearman's p =.512; P <.01). It was also associated with self-reported depressive symptomatology (Spearman's p =.536; P <.001).CONCLUSIONS Adults with CHD and ICDs demonstrate a high level of shock-related anxiety, which is associated with lower sexual functioning scores in men and women. These results underscore the need for increased clinical attention related to ICD-related shock anxiety and impaired sexual function in this population.

Background— Pulmonary hypertension (PH) has diverse causes with heterogeneous physiology compelling distinct management. Differentiating patients with primarily elevated pulmonary vascular resistance (PVR) from those with PH predominantly because of elevated left-sided filling pressure is critical. Methods and Results— We reviewed hemodynamics, echocardiography, and clinical data for 108 patients seen at a referral PH clinic with transthoracic echocardiogram and right heart catheterization within 1 year. We derived a simple echocardiographic prediction rule to allow hemodynamic differentiation of PH attributed to pulmonary vascular disease (PH PVD , defined as pulmonary artery wedge pressure [PAWP]≤15 mm Hg and PVR>3 WU). Age averaged 61.3±14.8 years, μPAWP and PVR were 16.4±7.1 mm Hg and 6.3±4.0 WU, respectively, and 52 (48.1%) patients fulfilled PH PVD hemodynamic criteria. The derived prediction rule ranged from –2 to +2 with higher scores suggesting higher probability of PH PVD : +1 point for left atrial anterior–posterior dimension <3.2 cm; +1 for presence of a mid systolic notch or acceleration time <80 ms; –1 for lateral mitral E:e′>10; –1 for left atrial anterior-posterior dimension >4.2 cm. PVR increased stepwise with score (for –2, 0, and +2, μPVR were 2.5, 4.5, and 8.1 WU, respectively), whereas the inverse was true for pulmonary artery wedge pressure (corresponding μPAWP were 21.5, 16.5, and 10.4 mm Hg). Among subjects with complete data, the score had an area under the curve (AUC) of 0.921 for PH PVD . A score ≥0 had 100% sensitivity and 69.3% positive predictive value for PH PVD , with 62.3% specificity. No patients with a negative score had PH PVD . Patients with a negative score and acceleration time >100 ms had normal PVR (μPVR=1.8 WU, range=0.7–3.2 WU). Conclusions— We present a simple echocardiographic prediction rule that accurately defines PH hemodynamics, facilitates improved screening and focused clinical investigation for PH diagnosis and management.

The role of exercise testing to risk stratify patients with repaired coarctation of the aorta (CoA) is controversial. Concentric left ventricular (LV) hypertrophy, defined as an increase in the LV mass-to-volume ratio (MVR), is associated with a greater incidence of adverse cardiovascular events. The objective of the present study was to determine whether a hypertensive response to exercise (HRE) is associated with increased LVMVR in patients with repaired CoA. Adults with repaired CoA who had a symptom-limited exercise test and cardiac magnetic resonance imaging examination within 2 years were identified. A hypertensive response to exercise was defined as a peak systolic blood pressure >220 mm Hg during a symptom-limited exercise test. The LV mass and volume were measured using cardiac magnetic resonance by an investigator who was unaware of patient status. We included 47 patients (median age 27.3 years, interquartile range 19.8 to 37.3), who had undergone CoA repair at a median age of 4.6 years (interquartile range 0.4 to 15.7). Those with (n = 11) and without (n = 36) HRE did not differ in age, age at repair, body surface area, arm-to-leg systolic blood pressure gradient, gender, or peak oxygen uptake with exercise. Those with a HRE had a greater mean systolic blood pressure at rest (146 ± 18 vs 137 ± 18 mm Hg, p = 0.04) and greater median LVMVR (0.85, interquartile range 0.7 to 1, vs 0.66, interquartile range 0.6 to 0.7; p = 0.04) than those without HRE. Adjusting for systolic blood pressure at rest, age, age at repair, and gender, the relation between HRE and LVMVR remained significant (p = 0.001). In conclusion, HRE was associated with increased LVMVR, even after adjusting for multiple covariates.

Background: The exercise capacity of pts who have had Fontan surgery is often depressed. We hypothesized that an inability to reduce pulmonary vascular resistance appropriately during exercise contributes to this exercise intolerance, and that administration of an effective pulmonary vasodilator would improve exercise function after Fontan. Purpose: To determine whether treatment with iloprost, (ILO, a potent, selective, inhaled pulmonary vasodilator) improves exercise function in pts with Fontan. Methods: Double-blind, randomized, placebo controlled, crossover trial. All pts performed 2 symptom-limited cardiopulmonary exercise tests (CPX) on different days separated by <1 month. Nebulizer treatment was administered before each test; one contained ILO (5 mcg), the other placebo. Results: We recruited 18 Fontan pts aged 12-49 (median 18) yrs. Mild throat/chest discomfort developed in 10/18 pts during ILO administration; all but 1 were able to complete treatment. No symptoms were reported during placebo treatments (p<0.001). Two additional pts did not complete CPX: one was noted to be in recurrent atrial flutter, the other, with developmental issues, could not cooperate adequately for CPX. In the 15 subjects with analyzable CPX data, oxygen pulse (a surrogate for stroke volume) at peak exercise was significantly higher following ILO treatment (11.7±2.7 ml/beat vs. 10.4±2.1 ml/beat following placebo; p<0.001). Peak oxygen consumption (VO 2 ) trended higher after ILO compared to placebo (28.7±6.7 vs. 27.5±5.9 ml/kg/min; p=0.18). Nine pts had a peak VO 2 <30 ml/kg/min following placebo; each of these pts had a higher peak VO 2 following ILO (25.5±4.7 vs. 23.7±4.4 ml/kg/min; p<0.002). In contrast, only 3/6 pts with peak VO 2 >30 ml/kg/min on their placebo study had a higher peak VO 2 following ILO (p<0.04). Conclusion: ILO therapy improves peak VO 2 in subjects with compromised exercise function after Fontan palliation. The improvement is related to an increase in the oxygen pulse at peak exercise. The therapy appears to be associated with minor side effects. These findings support the concept of pulmonary vasodilator therapy in Fontan pts with limited functional capacity.

Background— Data regarding long-term outcomes after the arterial switch operation for D-transposition of the great arteries are scarce. Methods and Results— A single-institution retrospective cohort study was conducted to assess cardiovascular outcomes after an arterial switch operation between 1983 and 1999. Patients without follow-up visits within 3 years were contacted and secondary sources of information obtained. Overall, 400 patients, 154 (38.3%) with a ventricular septal defect, 238 (59.5%) with an intact septum, and 9 (2.3%) with a Taussig-Bing anomaly, were followed for a median of 18.7 years. In perioperative survivors, overall and arrhythmia-free survival rates at 25 years were 96.7±1.8% and 96.6±0.1%, respectively. Late mortality was predominantly a result of sudden deaths and myocardial infarction. At 25 years, 75.5±2.5% remained free from surgical or catheter-based reintervention. Freedom from an adverse cardiovascular event was 92.9±1.9% at 25 years. Independent predictors were a single right coronary artery (hazard ratio, 4.58; 95% confidence interval, 1.32-15.90), P=0.0166) and postoperative heart failure (hazard ratio, 6.93; 95% confidence interval, 1.57-30.62; P=0.0107). At last follow-up, the left ventricular ejection fraction was 60.3±8.9%, 97.3% had class I symptoms, and 5.2% obstructive coronary artery disease. Peak oxygen uptake was 35.1±7.6 mL/kg/min (86.1±15.1% predicted), with a chronotropic index <80% in 34.2%. At least moderate neoaortic and pulmonary regurgitation were present in 3.4% and 6.6%, respectively, and more than mild neoaortic and pulmonary stenosis in 3.2% and 10.3%. Conclusions— Long-term and arrhythmia-free survival is excellent after arterial switch operation. Although sequelae include chronotropic incompetence and neoaortic, pulmonary, and coronary artery complications, most patients maintain normal systolic function and exercise capacity.

OBJECTIVEThe objective of this study was to examine the relationship between exercise test data and mortality in patients who have had the Fontan procedure.DESIGNThe study was designed as a retrospective cohort study.SETTINGThe study was set in a tertiary care center.PATIENTSAll study participants were Fontan patients ≥16 years old who had cardiopulmonary exercise tests at our institution between November 2002 and March 2010. The first exercise test with adequate effort during the study period was retained for analysis. We enrolled 146 patients at a median age of 21.5 years (16.0-51.6); 15.8 years (1.2-29.9) after Fontan surgery.OUTCOME MEASURESThe outcome measures were exercise test data (peak oxygen consumption, peak heart rate, etc.); mortality.RESULTSPeak oxygen consumption averaged 21.2 ± 6.2 mL/kg/min, 57.1 ± 14.1% predicted. Follow-up data were collected 4.0 ± 2.0 years (range 0.3-7.7) after the exercise test. Sixteen patients (11%) died during follow-up; their peak oxygen consumption (16.3 ± 4.0 mL/kg/min) was significantly less than the survivors' (21.8 ± 6.2 mL/kg/min; P < .0001). Recursive partitioning and Cox proportional hazards modeling revealed that the hazard for death for patients with a peak oxygen consumption of <16.6 mL/kg/min was 7.5 (95% confidence interval: 2.6, 21.6; P < .0002) times that of patients with a higher peak oxygen consumption. Similarly, the hazard ratio for patients with peak-exercise heart rates of <122.5 bpm was 10.6 (3.0, 37.1; 0 < 0.0002). Data from exercise tests could also identify patients at increased risk for a combined morbidity/mortality end point.CONCLUSIONSIn adults with Fontan surgery, exercise test data can identify patients at increased risk of midterm morbidity and mortality.