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Incomplete information on characteristics of prosthetic heart valves (PHV) may lead to inappropriate choices for PHV implantation (patient-prosthesis-mismatch) or erroneous interpretation of PHV function after implantation. No single and easy accessible source provides all relevant information on PHV. The aim of this study was to provide a comprehensive overview of available data for the majority of PHVs and annuloplasty rings. Information was collected by reviewing articles published on www.pubmed.org up to December 2014 and by written contact to all PHV manufacturers. Four areas of interest were defined: (1) PHV image, (2) in vivo transvalvular gradients, (3) effective orifice area (EOA) calculators and (4) PHV dimensions. Available information was classified as complete (all categories), partial (two or three categories) or minimal (one category). 108 PHV (including homografts) and 34 annuloplasty rings systems were identified. The information on PHV was complete, partial or minimal in 19.5, 61.0 and 19.5% of PHV, respectively. In 91.6% a picture of the valve could be obtained, whereas normative data for transvalvular gradients and EOA calculators were available in 63.0 and 25.0% of all PHV, respectively. The available data was summarized on a new open access webpage ( www.valveguide.ch ). There is a lack of accessible data on PHV dimensions, normal transvalvular gradients and effective orifice area calculators, although such information is of crucial importance for proper PHV assessment.

Left ventricular noncompaction (LVNC) is characterized by a two-layered myocardium consisting of a noncompacted inner and a compacted outer layer. The ratio of the thicknesses of these two layers is a major diagnostic criterion, which is, however, often difficult to apply in clinical practice.Transthoracic echocardiography was performed in 41 patients with LVNC, 41 patients with moderate or severe aortic valve stenosis (AVS), and 41 age-matched normal controls. The maximal systolic thicknesses of "noncompacta" and "compacta" were measured in standard short-axis views at the apical or midventricular level, in the segment with most prominent recesses (in patients with LVNC) or trabeculation (in patients with AVS and controls).The mean maximal systolic thickness of noncompacta was 1.8 ± 0.4 cm in patients with LVNC compared with 0.2 ± 0.1 cm in controls and 0.6 ± 0.02 cm in patients with AVS (P.0001). The mean maximal systolic thickness of compacta was lower in patients with LVNC (0.5 ± 0.1 cm) compared to controls (1.2 ± 0.2 cm; P.0001) and patients with AVS (1.6 ± 0.06 cm; P.0001). The maximal systolic thickness of compacta was ≤8.1 mm in patients with LVNC compared with8.1 mm (P.0001) in controls and8.1 mm in patients with AVS (P.0001). The ratio of the maximal systolic thickness of the indexed basal septum to that of the compacta was ≥0.64/m(2) in patients with LVNC compared to ≤0.62/m(2) in controls and ≤0.96/m(2) in patients with AVS.Maximal systolic compacta thickness8 mm is specific for LVNC and allows the differentiation of LVNC from normal hearts as well as those with myocardial thickening due to AVS. This observation may be particularly useful as an additional diagnostic criterion for preventing the overdiagnosis of LVNC.

Aims Supravalvar aortic stenosis is a rare form of left ventricular outflow tract obstruction that is often progressive in childhood. Little data are available on outcomes in the adult population. Our aim was to define cardiac outcomes in adults with supravalvar aortic stenosis. Methods and results This is a multicentre retrospective study of cardiac outcomes in adults (≥18 years) with supravalvar aortic stenosis. We examined: (i) adverse cardiac events (cardiovascular death, myocardial infarction, stroke, heart failure, sustained arrhythmias, and infective endocarditis) and (ii) the need for cardiac surgery in adulthood. One hundred and thirteen adults (median age at first visit 19 years; 55% with Williams–Beuren syndrome; 67% with surgical repair in childhood) were identified. Adults without Williams–Beuren syndrome had more severe supravalvar aortic stenosis and more often associated left ventricular outflow tract obstructions ( P < 0.001). In contrast, mitral valve regurgitation was more common in patients with Williams–Beuren syndrome. Eighty-five per cent of adults (96/113) had serial follow-up information (median follow-up 6.0 years). Of these patients, 13% (12/96) had an adverse cardiac event and 13% (12/96) had cardiac operations (7 valve repair or replacements, 4 supravalvar aortic stenosis repairs, 1 other). Cardiac surgery was more common in adults without Williams–Beuren syndrome ( P = 0.007). Progression of supravalvar aortic stenosis during adulthood was rare. Conclusion Adults with supravalvar aortic stenosis remain at risk for cardiac complications and reoperations, while progression of supravalvar aortic stenosis in adulthood is rare. Valve surgery is the most common indication for cardiac surgery in adulthood.

Advances in surgical and medical treatment of children born with congenital heart disease have led to a growing number of adult survivors, particularly to a growing number of adults with complex congenital heart disease. Childhood mortality has continuously decreased over the last few decades and mortality has shifted almost entirely to adulthood. However, most patients are not cured and many remain at risk of premature death. The extent of excess mortality among individual congenital disease entities is not well defined. In this article we outline the current demographics of adults with selected congenital heart lesions. Based on these contemporary patient cohorts, we delineate future changes in patient demographics. A better understanding of these trends may help in the optimal planning of future resource allocation for medical care and optimal planning of multicenter research, for this novel and growing population of young, chronically ill adults.

This editorial refers to ‘Preoperative thresholds for mid-to-late haemodynamic and clinical outcomes after pulmonary valve replacement in tetralogy of Fallot’[†][1], by J.P. Bokma et al ., on page 829. Tetralogy of Fallot (TOF) is the most common cyanotic heart defect (CHD), accounting for 3–5% of all infants born with a CHD.1 Survival prospects without intervention are bleak, and

Purpose of review Adult survivors with congenital heart disease (CHD) are a novel group of adults with chronic disease. Due to the nature of their surgical repair, the majority is not cured. This review focuses on changing demographics and mortality of adults with CHD, available knowledge and data on end-of-life (EOL) and palliative care in this patient group and outlines needs for future clinical practice and research. Recent findings Recent findings underscore that survival to adulthood has become the rule for the majority of patients born with CHD. Many remain at increased risk of premature death from their disease. Chronic circulatory failure has been identified as one of the leading causes of death and, thus, patients may benefit from incorporation of palliative care principles in their management. Recent studies show that this practice has not yet been widely adopted. Nonetheless a survey of adult outpatients has shown that many patients prefer to be involved in EOL decisions early in the course of their disease. Summary More research is needed to learn how we can best implement principles of palliative care in the growing population of adult survivors with CHD. This includes improved communication about EOL issues and a more comprehensive approach for symptom control.

Background: Echocardiographic speckle tracking strain has gained clinical importance. However, the comparability of measurements between different software systems is not well defined. Methods: In 47 healthy subjects left ventricular (LV) two-dimensional (2D) peak strain and time to peak strain (TTP) generated by EchoPAC (2DS) and velocity vector imaging (VVI) were compared. For each type of strain (longitudinal [LS], circumferential [CS], and radial strain [RS]) we compared global, anatomical level and segmental values. Results: When comparing 2DS to VVI, Pearson correlation coefficients (r) of global LS, CS, and RS were 0.68, 0.44, and 0.59, respectively (all P < 0.05). Correlation of global TTP was higher: 0.81(LS), 0.80 (CS), and 0.68 (RS), all P < 0.01. Segmental peak strain differed significantly between 2DS and VVI in 8/18 (LS), 17/18 (CS), and 15/18 (RS) LV segments (P < 0.05). However, segmental TTP significantly differed only in 5/18 (LS), 7/18 (CS), and 4/18 (RS) of LV segments. Similar strain gradients were found for both systems: apical strain was higher than basal and midventricular strain in LS and CS, with a reversed pattern for RS (P < 0.05). Conclusion: TTP strain as well as strain gradients were comparable between VVI and 2DS, but most peak strain values were not. The software-dependency of peak strain values must be considered in clinical application. Further studies comparing the diagnostic and prognostic accuracy of strain values generated by different software systems are mandatory. (Echocardiography 2011;28:539-547)

No published studies have evaluated the role of cardiac magnetic resonance (CMR) imaging for the assessment of Ebstein anomaly. Our objective was to evaluate the right heart characteristics in adults with unrepaired Ebstein anomaly using contemporary CMR imaging techniques. Consecutive patients with unrepaired Ebstein anomaly and complete CMR studies from 2004 to 2009 were identified (n = 32). Volumetric measurements were obtained from the short-axis and axial views, including assessment of the functional right ventricular (RV) end-diastolic volume (EDV) and end-systolic volume. The volume of the atrialized portion of the right ventricle in end-diastole was calculated as the difference between the total RVEDV and the functional RVEDV. The reproducibility of the measurements in the axial and short-axis views was determined within and between observers. The median value derived from the short-axis and axial views was 136 ml/m(2) (range 59 to 347) and 136 ml/m(2) (range 63 to 342) for the functional RVEDV, 153 ml/m(2) (range 64 to 441) and 154 ml/m(2) (range 67 to 436) for the total RVEDV, 49% (range 32% to 46%) and 50% (range 40% to 64%) for the functional RV ejection fraction, respectively. The axial measurements demonstrated lower intraobserver and interobserver variability than the short-axis approach for all values, with the exception of the intraobserver functional RVEDV and interobserver total RVEDV for which the limits of agreement and variance were not significantly different between the 2 views. In conclusion, measurements of right heart size and systolic function in patients with Ebstein anomaly can be reliably achieved using CMR imaging. Axial imaging appeared to provide more reproducible data than that obtained from the short-axis views.

In patients with heart failure from acquired cardiomyopathy, respiratory and skeletal muscle weakness is common and is an independent predictor for adverse events. Despite a different underlying pathology, many young adults with congenital heart disease (CHD) develop a syndrome comparable to heart failure from acquired cardiomyopathy and may be at risk for a similar skeletal muscle weakness.To assess respiratory and skeletal muscle strength in adults with complex CHD.Respiratory and skeletal muscle function was assessed in 51 adults; 41 with complex CHD (16 tetralogy of Fallot, 11 univentricular anatomy with Fontan operation and 14 with subaortic right ventricles) and 10 controls. Maximal inspiratory (MIPs) and expiratory (MEPs) pressures, handgrip strength, lung volumes and aerobic capacity (peak VO₂) were measured. Results In patients with CHD (age 34 ± 13 years), average% predicted MIPs, MEPs and handgrip strength were lower than in controls (77 ± 27% vs 106 ± 28%, 85 ± 32% vs 116 ± 41% and 72 ± 15% vs 93 ± 14%, respectively, p ≤ 0.01). There was no significant difference in muscle weakness between CHD subgroups. In 39% of patients with CHD, the handgrip strength, and in 22%, respiratory muscle strength was70% predicted. These patients had a significantly lower peak VO₂ (50 ± 12% vs 64 ± 14% predicted, p=0.008).Respiratory and skeletal muscle weakness is common in young adults with complex CHD and similar to that found in older adults with advanced heart failure from acquired heart disease.

Objectives We sought to determine cardiac outcomes in young adults with complete transposition of the great arteries (TGA) after the arterial switch operation (ASO). Background Although cardiac outcomes in the pediatric population with TGA after ASO have been well described, outcomes in the adult population have not to our knowledge been studied. Methods We determined late survival in all operative survivors with TGA after ASO performed before 1991 at our local pediatric referring hospital. In the subset of adults (n = 65) followed in our adult congenital cardiac clinic, we examined cardiac outcomes in adulthood. Results Survival of the 132 infants discharged from hospital after ASO was 97% (70% confidence interval [CI]: 95.0% to 98.1%) at 20 years. In the 65 patients (mean age 21 ± 3 years, 62% male) followed at our institution, 17% (11 of 65) had at least 1 clinically significant cardiac lesion, including ventricular dysfunction, valvular dysfunction, or arrhythmias. Residual lesions were more common in those who had had cardiac reinterventions in childhood (odds ratio: 10.7, 95% CI: 2.1 to 55). In adulthood, 5 patients (8%) had arrhythmia requiring treatment and 7 patients (11%) required reinterventions (5 reoperations and 2 pacemaker implantations). Intervention for aortic valve regurgitation and aortic root dilation were not observed. Exercise capacity was reduced in most adults (82%) after ASO. Conclusions Although most adults after ASO are well, and few have residual defects, there are subgroups, particularly those who needed further cardiac intervention in childhood, who are at higher risk for ventricular and valve dysfunction and arrhythmias.

With advances in pediatric cardiology and cardiac surgery, the population of adults with congenital heart disease (CHD) has increased. In the current era, there are more adults with CHD than children. This population has many unique issues and needs. Since the 2001 Canadian Cardiovascular Society consensus conference report on the management of adults with CHD, there have been significant advances in the field of adult CHD. Therefore, new clinical guidelines have been written by Canadian adult CHD physicians in collaboration with an international panel of experts in the field. The present introductory section is a summary of the epidemiology and scope of adult CHD in Canada, the structure of the Canadian health care system and adult congenital cardiac health services in Canada. The recommendations for antibiotic prophylaxis and genetic evaluation in this population are included. The complete document consists of four manuscripts, which are published online in the present issue of The Canadian Journal of Cardiology, including sections on genetics, outcomes, diagnostic workups, surgical and interventional options, treatment of arrhythmias, assessment of pregnancy and contraception risks, and follow-up recommendations. The complete document and references can also be found at www.ccs.ca or www.cachnet.org.

BACKGROUND: Little is known on the performance of newly initiated carotid artery stenting (CAS) programs. The safety of the procedure is being questioned following the publication of the EVA-3S trial, a study criticized for the limited interventional experience required to enroll patients. METHODS: Within a newly started academic CAS program, patient data and outcomes were collected prospectively. The outcomes of the first 100 consecutive patients treated are reported. A CAS-fellowship-trained interventionalist was involved in all procedures. All patients underwent clinical assessment by a neurologist before and after the procedure, and serial ECG and cardiac enzymes were routinely obtained. Primary outcome measures included 30-day major adverse events (MAE), defined as death, stroke, or myocardial infarction, while on follow-up deaths and ipsilateral strokes were added. RESULTS: Between July 2003 and November 2006, 92 patients had a single internal carotid artery treated, while 7 underwent staged bilateral CAS. In one patient, the procedure was aborted prior to lesion treatment. The 30-day MAE rate per procedure was 1.9% (one major and one minor stroke). By a mean follow-up of 16 months (range 2-42 months), one patient had died of refractory heart failure, while one patient had a minor ipsilateral stroke and three had minor contralateral strokes, corresponding to total MAE per patient of 4%. The rate of any stroke or death was 7%. The rate of restenosis >or=50% per lesion by ultrasound was 3.8%. CONCLUSION: This single center experience suggests that it is safe to start a CAS program following dedicated fellowship.

Le syndrome de Marfan est la maladie congenitale du tissu conjonctif la plus frequente. Il est cause par des mutations du gene FBN1 codant la fibrilline-1, un composant des microfibrilles qui forment les fibres elastiques du tissu conjonctif. Etant donne que les fibres elastiques sont omnipresentes dans l’organisme, le syndrome de Marfan peut affecter plusieurs organes.

Beim Ehlers-Danlos-Syndrom handelt es sich um eine heterogene Gruppe seltener genetischer Erkrankungen des Bindegewebes. Fur den autosomal-dominant vererbten vaskularen Subtyp typisch sind eine dunne, durchschimmernde Haut, eine Blutungsneigung bereits durch leichte Traumata sowie eine Fragilitat des Bindegewebes.

Context: Aberrant cellular oxygen sensing is a leading theory for development of pheochromocytoma (PHEO) and paraganglioma (PGL). Objective: The objective of the study was to test the hypothesis that chronic hypoxia in patients with cyanotic congenital heart disease (CCHD) increases the risk for PHEO-PGL. Design/Setting/Participants: We investigated the association between CCHD and PHEO-PGL with two complementary studies: study 1) an international consortium was established to identify congenital heart disease (CHD) patients with a PHEO-PGL diagnosis confirmed by pathology or biochemistry and imaging; study 2) the 2000–2009 Nationwide Inpatient Survey, a nationally representative discharge database, was used to determine population-based cross-sectional PHEO-PGL frequency in hospitalized CCHD patients compared with noncyanotic CHD and those without CHD using multivariable logistic regression adjusted for age, sex, and genetic PHEO-PGL syndromes. Results: In study 1, we identified 20 PHEO-PGL cases, of which 18 had CCHD. Most presented with cardiovascular or psychiatric symptoms. Median cyanosis duration for the CCHD PHEO-PGL cases was 20 years (range 1–57 y). Cases were young at diagnosis (median 31.5 y, range 15–57 y) and 7 of 18 had multiple tumors (two bilateral PHEO; six multifocal or recurrent PGL), whereas 11 had single tumors (seven PHEO; four PGL). PGLs were abdominal (13 of 17) or head/neck (4 of 17). Cases displayed a noradrenergic biochemical phenotype similar to reported hypoxia-related PHEO-PGL genetic syndromes but without clinical signs of such syndromes. In study 2, hospitalized CCHD patients had an increased likelihood of PHEO-PGL (adjusted odds ratio 6.0, 95% confidence interval 2.6–13.7, P < .0001) compared with those without CHD; patients with noncyanotic CHD had no increased risk (odds ratio 0.9, P = .48). Conclusions: There is a strong link between CCHD and PHEO-PGL. Whether these rare diseases coassociate due to hypoxic stress, common genetic or developmental factors, or some combination requires further investigation.

Congenital heart defects are the most common birth defects. Major advances in open-heart surgery have led to rapidly evolving cohorts of adult survivors and the majority of affected women now survive to childbearing age. The risk of cardiovascular complications during pregnancy and peripartum depends on the type of the underlying defect, the extent and severity of residual haemodynamic lesions and comorbidities. Careful individualized, multi-disciplinary pre-pregnancy risk assessment and counselling, including assessment of risks in the offspring and estimation on long-term outcomes of the underlying heart defect, will enable informed decision making. Depending on the estimated risks, a careful follow-up plan during pregnancy as well as a detailed plan for delivery and postpartum care can reduce the risks and should be made by the multi-disciplinary team.

Background: Surgical palliation of hypoplastic left heart syndrome (HLHS) has only been possible for the past few decades. Prior to this, without heart transplantation, HLHS was universally fatal in infancy. The oldest survivors of palliated HLHS are only now entering adulthood and limited data are available regarding their welfare. Methods: For this multi-center, cross-sectional, observational study, 6 adult congenital heart disease (ACHD) centers contributed data regarding all HLHS patients aged >18 years followed at their institution. HLHS was defined as a dominant right ventricle (RV) and diminutive left ventricle with a combination of mitral valve disease (stenosis [MS] or atresia [MA]) and aortic valve disease (stenosis [AS] or atresia [AA]). Patients with single RV physiology without hypoplasia of left heart inlet and outlet structures were excluded. All available clinical data, including cardiac imaging, cardiac catheterization results and exercise tests were reviewed. Results: The study included 53 HLHS adults (65% male) with mean age 21.8±3.6 years. Underlying cardiac anatomy was AA&MA (n=21, 41%), AS&MS (n=19, 37%), AS&MA (n=10, 20%), and AA&MS (n=1, 2%). Stage 1 Norwood was completed at age 6.0±4.4 days, Glenn shunt at age 10.8±8.7 months and Fontan at age 3.3±2 years. Stage 1 cardiopulmonary bypass and circulatory arrest times were 94±46 and 59±2 minutes respectively. The mean duration of follow-up in an ACHD center was 3.4±2.5 years. After age 18 years, major adverse events had occurred in 15/53 patients (28%). These mutually exclusive events were: death (n=3, 6%), transplant (n=1, 2%), protein losing enteropathy (n=2, 4%), stroke (n=2, 4%), symptomatic ventricular tachycardia (n=1, 2%), heart failure requiring admission for intravenous therapy (n=3, 6%) and major cardiac surgery (n=3, 6% [aortic valve replacement n=1, tricuspid valve replacement n=2]). Conclusions: Young adults with a Fontan palliation for HLHS are at high risk of major adverse cardiac events. It is essential that close attention is paid to successful transition of this vulnerable population into adult care and that these patients remain under vigilant specialist follow-up through adult life.

Aims To determine the prevalence of baffle leaks in adults after atrial switch operations for transposition of the great arteries, as these may predispose to paradoxical embolic events, particularly in patients with transvenous pacemaker or defibrillator leads. Methods and results We routinely perform contrast echocardiography with agitated saline in all patients after atrial switch operations. For this study, we analysed patients who had saline contrast echocardiography between 2010 and 2012. The presence of baffle leaks and the severity of right-to-left shunting were assessed. We compared baseline characteristics and oxygen saturation at rest and during exercise between patients with and without baffle leaks. A total of 65 patients (56 Senning and 9 Mustard repair) without previously known baffle leaks were included (mean age 32 ± 8 years, 77% males). Right-to-left shunting was identified in 42 patients (65%) and occurred without provocation manoeuvres in 88%. There were no differences in baseline characteristics, echocardiographic findings, or exercise capacity between patients with and without baffle leaks, except for lower oxygen saturation at peak exercise in those with baffle leaks (29% had oxygen saturations below 90% at peak exercise compared to none without baffle leaks, P = 0.011). Four patients with baffle leaks had previous implantation of transvenous pacemaker leads; one of them had suffered a stroke. Two other patients with baffle leaks had a history of potential embolic stroke. Conclusions Because of the high prevalence of baffle leaks in adults after atrial switch operations, we propose routine screening with agitated saline contrast, particularly prior to implantation of transvenous pacemaker or defibrillator leads.

BACKGROUND: Echocardiographic quantification of mitral regurgitation (MR) can be challenging if the valve geometry is significantly altered. Our aim was to compare the quantification of MR by the recently developed real time three-dimensional (3D) volume color flow Doppler (RT-VCFD) method to the conventional two-dimensional (2D) echocardiographic methods during the MitraClip procedure. METHODS: Twenty-seven patients (mean age 76 ± 8 years, 56% male) were prospectively enrolled and severity of MR was assessed before and after the MitraClip procedure in the operating room by 3 different methods: (1) by integrative visual approach by transesophageal echocardiography, (2) by transthoracic 2D pulsed-wave Doppler-based calculation of aortic stroke volumes (SV) and mitral inflow allowing calculation of regurgitant volume, and (3) by transthoracic 3D RT-VCFD-based calculation of regurgitant volume. RESULTS: We found moderate agreement between the integrative visual approach and the 3D RT-VCFD method for assessment of MR severity before (κ = 0.4, P

Marked advances in the diagnosis and treatment of congenital heart disease (CHD) and other pediatric-onset cardiac conditions have significantly increased survival, such that the majority of patients are now expected to reach adulthood. However, childhood mortality will not completely disappear and there continues to be a subgroup of patients with complex heart disease who will die in childhood. In addition, CHD, particularly of moderate to great complexity, remains a chronic medical condition associated with premature mortality in adulthood. Patients with congenital and pediatric heart disease have end-of-life (EOL) care considerations unique from those of adult patients dying of acquired heart disease. Though EOL considerations certainly vary between neonates, children and adolescents, and adults, an emphasis on communication and interdisciplinary care is recommended in order to improve advance care planning and EOL care for this deserving group of patients.

Progress in management of congenital heart disease has shifted mortality largely to adulthood. However, adult survivors with complex congenital heart disease are not cured and remain at risk of premature death as young adults. Thus, our aim was to describe the evolution and mortality risk of adult patient cohorts with complex congenital heart disease.Among 12,644 adults with congenital heart disease followed at a single center from 1980 to 2009, 176 had Eisenmenger syndrome, 76 had unrepaired cyanotic defects, 221 had atrial switch operations for transposition of the great arteries, 158 had congenitally corrected transposition of the great arteries, 227 had Fontan palliation, and 789 had repaired tetralogy of Fallot. We depict the 30-year evolution of these 6 patient cohorts, analyze survival probabilities in adulthood, and predict future number of deaths through 2029.Since 1980, there has been a steady increase in numbers of patients followed, except in cohorts with Eisenmenger syndrome and unrepaired cyanotic defects. Between 1980 and 2009, 308 patients in the study cohorts (19%) died. At the end of 2009, 85% of survivors were younger than 50 years. Survival estimates for all cohorts were markedly lower than for the general population, with important differences between cohorts. Over the upcoming two decades, we predict a substantial increase in numbers of deaths among young adults with subaortic right ventricles, Fontan palliation, and repaired tetralogy of Fallot.Anticipatory action is needed to prepare clinical services for increasing numbers of young adults at risk of dying from complex congenital heart disease.

The advent of open heart surgery has dramatically improved survival in children born with congenital heart disease (CHD). Over the last few decades, mortality has shifted almost entirely to adulthood. This has led to a growing number of adult survivors, particularly to a growing number of adults with complex congenital heart disease. Most patients are however not cured, and many remain at risk for cardiovascular complications and premature death. As most surgical repair techniques were invented only a few decades ago, the cohorts of adult survivors are still emerging and will continue to change in number and age distribution over the upcoming decades.

Grown-up congenital heart disease is a rapidly growing field in adult cardiology with specific needs.

Impaired left ventricular (LV) myocardial deformation is associated with adverse outcome in patients with severe aortic stenosis (AS). The aim of this retrospective study was to assess the impact of transcatheter aortic valve implantation (TAVI) on the recovery of myocardial mechanics and the influence of postprocedural aortic regurgitation (AR).Speckle-tracking echocardiography was used to assess multidirectional myocardial deformation (longitudinal and circumferential strain) and rotational mechanics (apical rotation and twist) before and at midterm follow-up after TAVI. Predictors of myocardial recovery, defined as a ≥20% relative increase in the magnitude of global longitudinal strain compared with baseline, were examined.Sixty-four patients (median age, 83 years; interquartile range, 77-86 years) with severe AS and high surgical risk (mean European System for Cardiac Operative Risk Evaluation score, 20 ± 13%) were evaluated. Overall, LV longitudinal deformation was impaired at baseline compared with controls. At 5 ± 3 months after TAVI, LV longitudinal deformation had significantly improved only in the group of patients with baseline LV ejection fractions (LVEF) ≤ 55%: global longitudinal strain from -9.7 ± 3.7% to -11.8 ± 3.2% (P = .05), longitudinal strain rate from -0.44 ± 0.14 sec(-1) to -0.57 ± 0.16 sec(-1) (P = .001), and early diastolic strain rate from 0.38 ± 0.17 sec(-1) to 0.49 ± 0.18 sec(-1) (P = .01). In patients with normal LVEFs, LV twist was supraphysiologic at baseline and normalized after TAVI (from 16.1 ± 6.9° to 11.9 ± 6.2°, P = .004). In patients with baseline LVEFs ≤ 55%, circumferential deformation was impaired before TAVI and improved after TAVI. Baseline LVEF (odds ratio, 0.56 per 10% increment; P = .02) and global longitudinal strain (odds ratio, 0.65 per absolute 1% increment; P.001) were significant predictors of myocardial recovery. LV mass, volumes, and longitudinal strain failed to favorably remodel in patients with post-TAVI important AR (defined as new mild post-TAVI AR or moderate or severe post-TAVI AR [either preexisting or new AR]).TAVI restores LV function toward more physiologic myocardial mechanics in both normal- and depressed-LVEF groups. Patients with lower systolic function derive the most benefit in terms of longitudinal reverse remodeling. Postprocedural AR adversely affects LV structural and functional remodeling.

We report the case of a young patient with repaired Ebstein’s anomaly who developed severe tachycardia-induced cardiomyopathy and a large apical thrombus as a consequence of sustained atrial flutter with a 2:1 conduction. In spite of a dramatic course in hospital with prolonged mechanical resuscitation and extracorporeal membrane oxygenation, she survived and made a rapid and full recovery. This remarkable case underlines that atrial arrhythmias, the most common complication in adults with congenital heart disease, may have devastating outcomes when timely recognition is missed and treatment delayed—thus, emphasizing the importance of good patient education.

A 47-year-old man with a history of systemic hypertension and type 2 diabetes mellitus was admitted to our centre in cardiogenic shock. He experienced rapidly progressive shortness of breath for the past 3 weeks. The ECG on admission showed atrial flutter with 2:1 conduction and a ventricular rate of 142 b.p.m. ( Panel A ). Transoesophageal echocardiography prior to cardioversion ( Panels B and C , …

Due to impressive improvements in surgical repair options, even patients with complex congenital heart disease (CHD) may survive into adulthood and have a high risk of end-stage heart failure. Thus, the number of patients with CHD needing heart transplantation (HTx) has been increasing in the last decades. This paper summarizes the changing etiology of causes of death in heart failure in CHD. The main reasons, contraindications, and risks of heart transplantation in CHD are discussed and underlined with three case vignettes. Compared to HTx in acquired heart disease, HTx in CHD has an increased risk of perioperative death and rejection. However, outcome of HTx for complex CHD has improved over the past 20 years. Additionally, mechanical support options might decrease the waiting list mortality in the future. The number of patients needing heart-lung transplantation (especially for Eisenmenger’s syndrome) has decreased in the last years. Lung transplantation with intracardiac repair of a cardiac defect is another possibility especially for patients with interatrial shunts. Overall, HTx will remain an important treatment option for CHD in the near future.

Most adults with congenital heart disease (CHD) are interested in discussing matters related to advance care planning (ACP) early in the disease course, yet few such conversations actually occur. We aimed to evaluate factors that impact these discussions between patients and adult CHD providers.Two hundred adult CHD outpatients completed a survey that included factors that might impact ACP discussions with their doctors. In parallel, forty-eight providers within the Canadian Adult Congenital Heart Network completed a similar online survey. Responses were compared between the groups.Most providers (85%) worried that they were unable to reliably estimate life expectancy and believed that patients were not ready for end-of-life discussions if their estimated life expectancies were beyond 5 years (63%) or beyond 10 years (79%). In contrast, only 24% of patients, independent of disease complexity, thought they were not ready to talk about ACP. Most providers (83%) reported that greater certainty about patients' prognoses would help them discuss ACP. Patients thought that such discussions were best facilitated when they had trust in their doctors (85%) and believed their doctors are good at taking care of patients with CHD (78%).Despite the fact that challenges to prognostication exist, discussions about ACP should not be reserved for patients with a severely reduced life expectancy. Most patients want these discussions regardless of the complexity of their disease. The trusting and close patient-doctor relationship in adult CHD, often evolving over many years, may provide an excellent platform from which to initiate such discussions.

A 56-year-old male was referred for percutaneous treatment of multiple-symptomatic persistent pulmonary arterio-venous fistulae (PAVFs). The patient's history included surgical repair of a secundum-type atrial septal defect with closure of right lung PAVFs in 1961, repeat transcatheter radiofrequency ablations for recurrent episodes of intra-atrial re-entrant tachycardia, and implantation of a transvenous dual-chamber pacemaker for sick sinus syndrome in 2002. Over the …

Percutaneous closure of the patent foramen ovale (PFO) is a widely used procedure in patients with paradoxical embolism. Whether or not implantation of a PFO closure device alters cardiac chamber anatomy and in turn affects valvular function is unclear.Out of 334 patients who underwent PFO closure between 2002 and 2010, a total of 196 received an Amplatzer septal occluder and were retrospectively analyzed. Nineteen patients (9.7%) were excluded due to an incomplete follow-up. Thus, 177 patients with a mean age of 51 ± 13 years remained for analysis. Clinical and echocardiographic examinations were performed before and 6 month after PFO closure.At follow-up, significant residual shunt (20 microbubbles) was present in only 11 patients (6.2%). Newly developed or worsened aortic regurgitation (AR) was noted in 16 patients (9%), whereas in 2 patients (1%) a previously documented AR had disappeared. In 33 patients (19%), mitral valve regurgitation (MR) developed or worsened, while in 10 patients (5.6%) a previously documented MR was no longer present at follow-up. In 44 patients (25%), tricuspid regurgitation (TR) had developed at follow-up, while in 5 patients (3%) a previously documented TR was no longer visible echocardiographically.Implantation of an Amplatzer septal occluder is a safe and effective procedure. However, it can induce or worsen valvular regurgitation in almost half of the patients. Although the degree of regurgitation was generally mild, it is likely that implanted devices alter cardiac chamber structure.

Isolated left ventricular noncompaction is a rare form of primary cardiomyopathy. Although increasingly diagnosed, data on the outcomes are limited. To define the predictors of adverse outcomes, we performed a retrospective analysis of a prospectively defined cohort of consecutive patients (age >14 years) diagnosed with left ventricular noncompaction at a single center. The baseline characteristics included presentation with a cardiovascular complication (i.e., decompensated heart failure, systemic embolic event, or sustained ventricular arrhythmia). The primary end point was survival free from cardiovascular death or transplantation. The predictors of survival were evaluated using the Kaplan-Meier method and Cox proportional hazards analysis. A total of 115 patients were included, 77% of whom were symptomatic at diagnosis. Compared to the asymptomatic patients, the symptomatic patients were significantly older and had larger left ventricular cavities and worse left ventricular ejection fraction. Of the 115 patients, 49 (43%) presented with a cardiovascular complication. During a median follow-up of 2.7 years (range 0.1 to 19.4), none of the asymptomatic patients died or underwent transplantation compared to 31% (27 of 88) of the symptomatic patients (p = 0.001). The major determinants of cardiovascular death or transplantation were presentation with a cardiovascular complication (hazard ratio 20.6, 95% confidence interval 4.9 to 87.5, p

The ninth author's name was spelled incorrectly. The correct name is: Beatrice Amann-Vesti. Information was missing from the affiliation. The correct affiliation is: Cardiology, Angiology, and Neurology, Cardiovascular Center, University Hospital Zurich, Switzerland

Congenital heart disease (CHD) is a chronic illness. Few adults with CHD are cured and those with disease of moderate or great complexity remain at risk of premature death. Current adult CHD guidelines recommend that providers encourage their patients to complete advance directives. We evaluated the prevalence of completed advance directives by and the preference for information about life expectancy of outpatients at a large adult CHD program. Two hundred patients with CHD (52% men, 35 ± 15 years old, range 18 to 79, 81% with disease of moderate or great complexity) completed a survey that assessed knowledge of advance directives and nature of and preferences for advance care planning. Only 5% of patients reported that they had completed advance directives; 56% had never heard of them. However, most patients (87%) reported that they would prefer to have an advance directive available if they were dealing with their own dying and were unable to speak for themselves. Patients who had formally identified substitute decision makers (n = 34) were typically older (47 ± 16 vs 33 ± 13 years, p0.001) and more likely to have partners (30% vs 6%, p0.001). Most patients (70%) reported that they wanted general information about the average life expectancy for patients with their heart condition. In conclusion, in contrast to recommendations from published guidelines, advance care planning documents are infrequently completed by outpatients. Health care providers caring for patients with CHD should educate their patients about advance directives and assist them in preparing formal end-of-life-planning documents.

Adults with congenital heart disease represent a growing patient population. Notwithstanding dramatic improvements in survival and life expectancy over recent decades, many of these patients remain at risk of premature death from progressive heart disease and would benefit from the principles of palliative and end-of-life care. Data on end-of-life care in this patient group are, however, lacking. We report a retrospective study of 48 patients with congenital heart disease who died while admitted to our hospital (mean age at death 37 ± 14 years). We describe circumstances of death, end-of-life discussions, and the provision of end-of-life care. The majority of patients had complex congenital heart disease and were considered to be in the end stage of their disease. Despite this, only a minority of patients had documented end-of-life discussions prior to their terminal admission and most received continuing aggressive medical treatment up to their demise. Advanced palliative and end-of-life care strategies should be developed for and provided to this group of patients, with the dual aims of reduction of unwarranted therapies and enhancement of the quality of death and dying.

Echocardiographic speckle tracking strain has gained clinical importance. However, the comparability of measurements between different software systems is not well defined.In 47 healthy subjects left ventricular (LV) two-dimensional (2D) peak strain and time to peak strain (TTP) generated by EchoPAC (2DS) and velocity vector imaging (VVI) were compared. For each type of strain (longitudinal [LS], circumferential [CS], and radial strain [RS]) we compared global, anatomical level and segmental values.When comparing 2DS to VVI, Pearson correlation coefficients (r) of global LS, CS, and RS were 0.68, 0.44, and 0.59, respectively (all P0.05). Correlation of global TTP was higher: 0.81(LS), 0.80 (CS), and 0.68 (RS), all P0.01. Segmental peak strain differed significantly between 2DS and VVI in 8/18 (LS), 17/18 (CS), and 15/18 (RS) LV segments (P0.05). However, segmental TTP significantly differed only in 5/18 (LS), 7/18 (CS), and 4/18 (RS) of LV segments. Similar strain gradients were found for both systems: apical strain was higher than basal and midventricular strain in LS and CS, with a reversed pattern for RS (P0.05).TTP strain as well as strain gradients were comparable between VVI and 2DS, but most peak strain values were not. The software-dependency of peak strain values must be considered in clinical application. Further studies comparing the diagnostic and prognostic accuracy of strain values generated by different software systems are mandatory.

A 66-year-old man with Kartagener's syndrome, situs inversus totalis, and recurrent supraventricular tachycardia and a 49-year-old man with atrial fibrillation and drug-refractory rapid ventricular rate response were referred for catheter ablation. In the first case, the mirrored anatomy of the right atrium was reconstructed using three-dimensional electroanatomical mapping, which guided successful ablation of a typical atrioventricular nodal reentrant tachycardia. In the second case, computerized tomography showed the presence of left atrial isomerism with interruption of the inferior vena cava and azygous continuation into the superior vena cava, guiding advancement of the ablation catheter for access into the positionally right atrial ablation site. These cases illustrate the role of imaging in patients with unusual anatomy of the cardiac chambers and major blood vessels guiding optimal catheter access for catheter ablation.

Tumoren im und am Herzen sind insgesamt eine Raritat, am haufigsten sind Metastasen. Die meisten primaren Herztumoren sind benigne. Maligne Tumoren sind nochmals seltener, gehen dafur jedoch meistens mit einer infausten Prognose einher, da die Therapieoptionen ausert limitiert sind.

Objectives The objectives of this study were to examine: 1) B-type natriuretic peptide (BNP) response to pregnancy in women with heart disease; and 2) the relationship between BNP levels and adverse maternal cardiac events during pregnancy. Background Pregnancy imposes a hemodynamic stress on the heart. BNP might be a useful biomarker to assess the ability of the heart to adapt to the hemodynamic load of pregnancy. Methods This was a prospective study of women with structural heart disease seen at our center. Serial clinical data and plasma BNP measurements were obtained during the first trimester, third trimester, and after delivery (>6 weeks). Results Seventy-eight pregnant women were studied; 66 women with heart disease (age 31 ± 5 years), and 12 healthy women (age 33 ± 5 years). During pregnancy, the median peak BNP level was higher in women with heart disease compared with control subjects (median 79, interquartile range 51 to 152 pg/ml vs. median 35, interquartile range 21 to 43 pg/ml, p < 0.001). In women with heart disease, those with subaortic ventricular dysfunction had higher BNP levels (p = 0.03). A BNP >100 pg/ml was measured in all women with events during pregnancy (n = 8). Sixteen women had increased BNP levels during pregnancy but did not have clinical events. None of the women with BNP ≤100 pg/ml had events. BNP ≤100 pg/ml had a negative predictive value of 100% for identifying events during pregnancy. Conclusions Many pregnant women with heart disease have increased BNP levels during pregnancy. Incorporating serial BNP levels in into clinical practice can be helpful, specifically in adjudicating suspected adverse cardiac events during pregnancy. © 2010 American College of Cardiology Foundation.

The right ventricular (RV) systolic function is important for decision making in adults with repaired tetralogy of Fallot (rTOF). Our aim was to assess regional RV systolic function and its impact on global RV systolic function by echocardiography.In 101 adults with rTOF, regional RV systolic function on echocardiography was compared to 50 individuals with structurally normal hearts. In patients with rTOF, the impact of echocardiographic measures of regional RV systolic function on global RV-ejection fraction as measured by cardiac magnetic resonance imaging (CMR-RVEF) was determined.Compared to normals, patients with rTOF had impaired systolic function of the RV outflow tract. Patients with rTOF and normal CMR-RVEF compensate this loss of RV outflow tract function with increased contractions of the RV-body, measured as fractional area change on short axis (30±12% versus 19±7%, p0.0001) and on 4-chamber views (42±7% versus 38±8%, p=0.06). In contrast, patients with rTOF and abnormal global CMR-RVEF showed significantly lower systolic function of the RV-body compared to normal controls (fractional area change on 4-chamber view: 31±6% versus 38±8%, p0.0001). A simple regression model, incorporating fractional shortening of RV outflow tract and fractional area change on 4-chamber view allows accurate echocardiographic estimation of the CMR-derived RVEF.Patients with repaired tetralogy of Fallot have markedly different regional systolic RV-function compared to normals, even when the global RV systolic function is preserved. The systolic function of the RV outflow tract and of the RV body are both important determinants of global systolic RV function in these patients. Their prognostic significance needs to be determined.