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The long-term outcome of adults with repaired total anomalous pulmonary venous connection (TAPVC) is poorly documented. Therefore, the present study aims to provide current clinical data on adult survivors with repaired TAPVC focusing on arrhythmia.

AbstractThe Patient Registry for Adolescents and Adults with Stable Fontan Circulation aims to describe a contemporary cohort of Fontan patients who could be eligible for a clinical trial investigating macitentan, an endothelin receptor antagonist. This international, non-interventional, multicentre, cross-sectional, observational registry enrolled patients with “stable” Fontan circulation ≥10 years following extra-cardiac conduit or lateral tunnel procedure. Main exclusion criteria were NYHA functional class IV, reoperation of Fontan circulation, or signs of disease worsening. Patient characteristics at enrolment are described; available data were collected during a single registration visit. Of the 266 screened patients, 254 were included in this analysis. At enrolment, median (interquartile range) age was 24 (20;30) years, 37%/63% of patients were from the USA/Europe, 54% were male, 54%/47% had undergone extra-cardiac conduit/lateral tunnel procedures, and 95% were in NYHA functional class I or II. History of arrhythmia was more common in older patients and patients with lateral tunnel; overall prevalence was 19%. Most laboratory values were within the normal range but mean creatinine clearance was abnormally low (87.7 ml/min). Angiotensin-converting enzyme inhibitors were used by 48% of patients and their use was associated with creatinine clearance <90 ml/min (p = 0.007), as was Fontan completion at an older age (p = 0.007). 53.4% of patients had clinical characteristics that could potentially meet an endothelin receptor antagonist trial’s eligibility criteria. The PREpArE-Fontan registry describes a cohort of patients who could potentially participate in an endothelin receptor antagonist trial and identified early subtle signs of Fontan failure, even in “stable” patients.

ObjectivePregnancy in women with aortic coarctation (CoA) has an estimated moderately increased risk (mWHO II–III) of adverse cardiovascular, obstetric or fetal events, but prospective data to validate this risk classification are scarce. We examined pregnancy outcomes and identified associations with adverse outcomes.MethodsPregnancies in women with CoA were selected from the worldwide prospective Registry of Pregnancy and Cardiac Disease (ROPAC, n=303 out of 5739), part of the European Society of Cardiology EURObservational Research Programme. The frequency of and associations with major adverse cardiac events (MACE) and hypertensive disorders (pregnancy-induced hypertension, (pre-)eclampsia or haemolysis, elevated liver enzymes and low platelets syndrome) were analysed.ResultsOf 303 pregnancies (mean age 30 years, pregnancy duration 39 weeks), 9.6% involved unrepaired CoA and 27.1% were in women with pre-existing hypertension. No maternal deaths or aortic dissections occurred. MACE occurred in 13 pregnancies (4.3%), of which 10 cases were of heart failure (3.3%). Univariable associations with MACE included prepregnancy clinical signs of heart failure (OR 31.8, 95% CI 6.8 to 147.7), left ventricular ejection fraction <40% (OR 10.4, 95% CI 1.8 to 59.5), New York Heart Association class >1 (OR 11.4, 95% CI 3.6 to 36.3) and cardiac medication use (OR 4.9, 95% CI 1.3 to 18.3). Hypertensive disorders of pregnancy occurred in 16 (5.3%), cardiac medication use being their only predictor (OR 3.2, 95% CI 1.1 to 9.6). Premature births were 9.1%, caesarean section was performed in 49.7% of pregnancies. Of 4 neonatal deaths, 3 were after spontaneous extreme preterm birth.ConclusionsThe ROPAC data show low MACE and hypertensive disorder rates during pregnancy in women with CoA, suggesting pregnancy to be more safe and better tolerated than previously appreciated.

AimsPatients with adult congenital heart disease (ACHD) are a potentially vulnerable patient cohort in case of COVID-19. Some cardiac defects may be associated with a poor COVID-19 outcome. Risk estimation in ACHD is currently based on expert opinion. The aim of this study was to collect clinical outcome data and to identify risk factors for a complicated course of COVID-19 in patients with ACHD.MethodsTwenty-five ACHD centres in nine European countries participated in the study. Consecutive patients with ACHD diagnosed with COVID-19 presenting to one of the participating centres between 27 March and 6 June 2020 were included. A complicated disease course was defined as hospitalisation for COVID-19 requiring non-invasive or invasive ventilation and/or inotropic support, or a fatal outcome.ResultsOf 105 patients with a mean age of 38±13 years (58% women), 13 had a complicated disease course, of whom 5 died. In univariable analysis, age (OR 1.3, 95% CI 1.1 to 1.7, per 5 years), ≥2 comorbidities (OR 7.1, 95% CI 2.1 to 24.5), body mass index of >25 kg/m2(OR 7.2, 95% CI 1.9 to 28.3) and cyanotic heart disease (OR 13.2, 95% CI 2.5 to 68.4) were associated with a complicated disease course. In a multivariable logistic regression model, cyanotic heart disease was the most important predictor (OR 60.0, 95% CI 7.6 to 474.0).ConclusionsAmong patients with ACHD, general risk factors (age, obesity and multiple comorbidities) are associated with an increased risk of complicated COVID-19 course. Congenital cardiac defects at particularly high risk were cyanotic lesions, including unrepaired cyanotic defects or Eisenmenger syndrome.

El objetivo de este estudio es analizar el valor pronóstico de la fracción aminoterminal del péptido natriurético cerebral (NT-proBNP) en pacientes con ventrículo derecho sistémico (VDs).

Introducción y objetivos:Evaluar el impacto del recambio de válvula pulmonar (RVP) en pacientes con tetralogía de Fallot reparada (TFr) en la evolución de los volúmenes y la función biventricular y en los eventos adversos.

Non-vitamin K antagonist (VKA) oral anticoagulants (NOACs) have several advantages over VKAs that render them an attractive option for adults with congenital heart disease (CHD). Efficacy and safety data specific to the adult CHD population are emerging. Herein, we synthesize the growing literature regarding NOACs in adults with CHD and attempt to identify subgroups for which it appears reasonable to extrapolate data from populations without CHD. Small observational studies suggest that NOACs are safe and effective in selected adults with CHD. NOACs are contraindicated in patients with a mechanical valve, in those with mitral or tricuspid valve stenosis with enlarged and diseased atria, with or without a mitral or tricuspid bioprosthesis, and after recent cardiac surgery (< 3 months). There is currently insufficient evidence to recommend NOACs in patients with a Fontan circulation or cyanotic CHD. Growing literature supports the use of NOACs in patients without CHD who have various forms of valvular heart disease. Therefore, when an indication for oral anticoagulation is established, it appears reasonable to consider a NOAC instead of a VKA in adults with CHD lesions analogous to isolated mitral regurgitation, tricuspid regurgitation, or aortic regurgitation or stenosis. The NOAC agent selected and the prescribed dose should be tailored according to bleeding risk, body weight, renal function, and comedications, especially antiepileptic drugs. The decision to initiate a NOAC should be shared between the patient and care provider. Large-scale research studies are required to further assess safety and efficacy in selected patient subgroups.

This study aimed to evaluate age at the first onset of cardiac complications and variation of frequency of complications between different congenital heart defects.

In adults with congenital heart disease (CHD) and atrial arrhythmias, recommendations for thromboprophylaxis are vague and evidence is lacking. We aimed to identify factors that influence decision‐making in daily practice. From the Swiss Adult Congenital HEart disease Registry (SACHER) we identified 241 patients with either atrial fibrillation (Afib) or atrial flutter/intraatrial reentrant tachycardia (Aflut/IART). The mode of anticoagulation was reviewed. Logistic regression models were used to assess factors that were associated with oral anticoagulation therapy. Compared with patients with Aflut/IART, patients with Afib were older (51 ± 16.1 vs 37 ± 16 years, P <.001) and had a higher CHA2DS2‐VASc (P <.001) and HAS‐BLED scores (P =.005). Patients with Afib were more likely on oral anticoagulation than patients with Aflut/IART (67% vs 43%, P <.001). In a multivariate logistic regression model, age [odds ratio (OR) 1.03 per year, 95%CI (1.01‐1.05), P =.019], atrial fibrillation [OR 2.75, 95%CI (1.30‐5.08), P =.007], non‐paroxysmal atrial arrhythmias [OR 5.33, 95%CI (2.21‐12.85)], CHA2DS2‐VASc‐Score >1 [OR 2.93, 95%CI (1.87‐4.61), P <.001], and Fontan palliation [OR 17.5, 95%CI (5.57‐54.97), P <.001] were independently associated with oral anticoagulation treatment, whereas a HAS‐BLED score >1 was associated with absence of thromboprophylaxis [OR 0.32, 95%CI (0.17‐0.60), P <.001]. In this multicenter study, age, type, and duration of atrial arrhythmias, CHA2DS2‐VASc and HAS‐BLED scores as well as a Fontan palliation had an impact on the use of thromboprophylaxis in adult CHD patients with atrial arrhythmias. In daily practice, anticoagulation strategies differ between patients with Afib and those with Aflut/IART. Prospective observational studies are necessary to clarify whether this attitude is justified.

A sofosbuvir/ledipasvir combination is part of a first-line treatment of hepatitis C. However, in patients concurrently treated with amiodarone, cardiac side effects have been described, resulting in an official warning in 2015 by the American Food and Drug Administration and the European Medicines Agency when combining those substances. This deprived numerous hepatitis C patients with concurrent cardiovascular problems of receiving this highly effective treatment. Here we present a treatment alternative with an elbasvir/grazoprevir regimen, based on our successful treatment of a patient under concurrent amiodarone therapy. Our observations indicate that patients treated with amiodarone can finally benefit from effective antiviral therapy.

El texto completo y el documento complementario de casos clínicos de la presente actualización se encuentran disponibles en: www.escardio.org/Guidelines/Clinical-Practice-Guidelines/2017-focused-update-on-dual-antiplatelet-therapy-dapt.

Zusammenfassung.Hintergrund:Heute erreichen bis zu 90 % der Patientinnen/Patienten mit angeborenem Herzfehler (AHF) das Erwachsenenalter. Um Betreuungsunterbrüche beim Übergang von der Pädiatrie in die Erwachsenenmedizin zu vermeiden, wurde am Inselspital Universitätsspital Bern eine pflegegeleitete Transitionssprechstunde (TS) eingeführt. Ziel:Es wurde exploriert, wie Adoleszente mit AHF und ein Elternteil den Transfer sowie die TS erlebten und welche Erwartungen sie hatten. Methode:Für die qualitative Studie mit interpretierendem phänomenologischem Ansatz wurden Einzelinterviews mit sieben Adoleszenten, sechs Müttern und zwei Vätern durchgeführt und in einem iterativen Prozess analysiert. Ergebnisse:Der Transfer Pädiatrie – Erwachsenenkardiologie im Rahmen der TS verlief für die Studienteilnehmenden mehrheitlich problemlos. Die TS erlebten sie positiv. Die Adoleszenten schätzten eine konstante Ansprechperson, die ihnen Wissen zum AHF vermittelte. Mütter und Väter begrüssten die Unterstützung ihrer Kinder im Alltag durch eine sachkundige, neutrale Fachperson. Im Übernehmen von Verantwortung für ihren AHF waren die Adoleszenten unterschiedlich weit. Den Müttern und den Vätern fiel die Abgabe von Verantwortung nicht leicht. Sie waren jedoch dazu bereit, wollten den Adoleszenten aber die nötige Zeit lassen, schliesslich selber verantwortlich zu handeln. Schlussfolgerungen:Die Transitionssprechstunde ist ein wichtiges Instrument, um Kontinuität in der Betreuung der Adoleszenten zu gewährleisten und ihren Eltern das Übergeben der Verantwortung an die Jugendlichen zu erleichtern.

At the beginning of the COVID-19 pandemic, professionals in charge of particularly vulnerable populations, such as adult congenital heart disease (ACHD) patients, were confronted with difficult decision-making. We aimed to assess changes in risk stratification and outcomes of ACHD patients suffering from COVID-19 between March 2020 and April 2021.

Fontan-associated liver disease is an increasing concern. Our aim was to assess prevalence and predictors of advanced liver fibrosis with a specific focus on utility of liver stiffness measurement by ultrasound transient elastography.

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. They have distinctive forms of heart failure and their cardiac disease can be associated with pulmonary hypertension, thromboemboli, complex arrhythmias and sudden death. Medical aspects that need to be considered relate to the long-term and multisystemic effects of single ventricle physiology, cyanosis, systemic right ventricles, complex intracardiac baffles and failing subpulmonary right ventricles. 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. Part III of the guidelines includes recommendations for the care of patients with complete transposition of the great arteries, congenitally corrected transposition of the great arteries, Fontan operations and single ventricles, Eisenmenger's syndrome, and cyanotic heart disease. Topics addressed include genetics, clinical outcomes, recommended diagnostic workup, surgical and interventional options, treatment of arrhythmias, assessment of pregnancy risk and follow-up requirements. The complete document consists of four manuscripts, which are published online in the present issue of The Canadian Journal of Cardiology. The complete document and references can also be found at www.ccs.caor www.cachnet.org.

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. They have distinctive forms of heart failure, and their cardiac disease can be associated with pulmonary hypertension, thromboemboli, complex arrhythmias and sudden death. Medical aspects that need to be considered relate to the long-term and multisystemic effects of single-ventricle physiology, cyanosis, systemic right ventricles, complex intracardiac baffles and failing subpulmonary right ventricles. Since the 2001 Canadian Cardiovascular Society Consensus Conference report on the management of adults with CHD, there have been significant advances in the understanding of the late outcomes, genetics, medical therapy and interventional approaches 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 executive summary is a brief overview of the new guidelines and includes the recommendations for interventions. The complete document consists of four manuscripts that are published online in the present issue of The Canadian Journal of Cardiology, including sections on genetics, clinical outcomes, recommended diagnostic workup, surgical and interventional options, treatment of arrhythmias, assessment of pregnancy and contraception risks, and follow- up requirements. The complete document and references can also be found at www.ccs.caor www.cachnet.org.

To identify risk factors associated with mortality in adult patients with Eisenmenger syndrome and to assess the effect of left ventricular dysfunction on mortality in these patients.

Objectives: The aim of this study was to determine the accuracy of six noninvasive Doppler methods for assessing invasively derived left ventricular diastolic pressure (LVDP). Background: To date, no studies have evaluated which of the various available Doppler methods are most reliable in determining LVDP in a consecutive patient population with different cardiac diseases. Methods: LVDP was estimated by the following Doppler methods (M): (1) M1—the peak mitral regurgitant flow velocity (peak MR), (2) M2—the mitral regurgitant velocity at the time of aortic valve opening (MRAVO), (3) M3—the aortic regurgitant end‐diastolic flow velocity (ARED), (4) M4—the ratio of the transmitral to mitral annular early diastolic velocity (ETM/EDTI), (5) M5—a transmitral flow velocity regression equation (RegrTM), and (6) M6—the difference of pulmonary venous and transmitral A wave duration (APV− ATMduration). For M1–M3, sphygmomanometric blood pressure was used to calculate LVDP. Results: In 101 patients, the regression coefficient, standard error of estimate, and mean difference with confidence limits between Doppler and catheter‐derived measurements were as follows: M1 (n = 46): r = 0.81 (P < 0.0001), 4.3 mmHg and 3.7 ± 12.0 mmHg; M2 (n = 47): r = 0.79 (P < 0.0001), 5.4 mmHg and 1.1 ± 11.2 mmHg; M3 (n = 20): r = 0.64 (P = 0.002), 7.8 mmHg and 4.6 ± 17.6 mmHg; M4 (n = 50): r = 0.62 (P < 0.0001), 5.6 mmHg and 0 ± 11.2 mmHg; M5 (n = 79): r = 0.24 (P = 0.03), 7.1 mmHg and −0.1 ± 16.8 mmHg; and M6 (n = 79): r = 0.22 (P = 0.05), 7.3 mmHg and 0 ± 14.4 mmHg, respectively. Conclusions: The Doppler measurement of mitral regurgitant jets is most accurate method to estimate left ventricular filling pressure noninvasively.

Aberrant origin of a pulmonary artery from the ascending aorta is an uncommon congenital vascular malformation with poor survival without surgery. In this case report, we describe the unusual late diagnosis of this congenital malformation in an otherwise asymptomatic young man presenting with mild hemoptysis. We review the natural and modified history of this defect and the relevant aspects of follow‐up in adult life.

Trisomy 21 (T21) is associated in 40–45% of cases with heart defects, most commonly shunt lesions. These defects, if not repaired, can lead to irreversible shunt-induced pulmonary hypertension (i.e. Eisenmenger syndrome [ES]). In ES patients, intracardiac repair is no longer possible, but selective pulmonary vasodilators may increase exercise capacity and improve prognosis. This study aimed to estimate the prevalence of cardiac defects and ES in adult T21 patients and to assess the impact of T21 on treatment modalities and outcome in ES patients. A questionnaire was sent to 6906 Swiss physicians inviting them to indicate the number of adults with T21 under their care (survey report). We also analyzed all adults with ES (with and without T21) included in the Swiss Adult Congenital HEart disease Registry (SACHER) and studied the impact of T21 on the use of selective pulmonary vasodilators and survival. In the survey, 348 physicians cared for 695 adult T21 patients. Overall, 24% of T21 survey patients were known to have a cardiac defect, one in four with a defect had developed ES and 13% of those with ES were on specific pulmonary vasodilators. In SACHER, ES was present in 2% of adults with congenital heart disease and selective pulmonary vasodilators were used in 68% of ES patients with T21. In SACHER, survival during follow-up was worse with higher nt-proBNP levels (hazard ratio [HR] = 1.15 per 1000 units, 95% confidence interval [CI] = 1.02–1.29) and lower left ventricular ejection fraction (HR = 1.07 per percent decrease, 95% CI = 1.01–1.13). Age at inclusion and T21 did not affect survival. The prevalence of cardiac defects in adults with T21 in Switzerland is half the prevalence in children. T21 is over-represented among adults with ES. Raised awareness of the therapeutic options for T21 patients with ES is warranted.