Publications by authors named "Matthias Greutmann"

98 Publications

Effect of oxygen therapy on exercise performance in patients with cyanotic congenital heart disease: Randomized-controlled trial.

Int J Cardiol 2021 Nov 29. Epub 2021 Nov 29.

Clinic of Pulmonology, University Hospital of Zurich, Switzerland; Center for Human Integrative Physiology, and Zurich Center for Interdisciplinary Sleep Research, University of Zurich, Switzerland. Electronic address:

Background: Patients with unrepaired cyanotic congenital heart disease (CHD) suffer from aggravated hypoxemia during exercise. We tested the hypothesis that supplemental oxygen improves exercise performance in these patients.

Methods: In this randomized, sham-controlled, single-blind, cross-over trial cyanotic CHD-patients underwent four cycle exercise tests to exhaustion, while breathing either oxygen-enriched (FiO 0.50, oxygen) or ambient air (FiO 0.21, air) using incremental (IET) or constant work-rate (CWRET) exercise test protocols (75% of maximal work rate achieved under FiO 0.21). Pulmonary gas-exchange, electrocardiogram, arterial blood gases, oxygen saturation (SpO), cerebral and quadriceps muscle tissue oxygenation (CTO and QMTO) by near-infrared spectroscopy were measured.

Results: We included seven patients with cyanotic CHD (4 Eisenmenger syndrome, 3 unrepaired cyanotic defects, 4 women) median (quartiles) age 36 (32;50) years, BMI 23 (20;26) kg/m and SpO at rest 87 (83;89) %. When comparing supplemental oxygen with air during exercise, maximal work-rate in IET increased from 76 (58;114) watts to 83 (67;136) watts, median difference 9 (0;22) W (p = 0.046) and CWRET-time increased from 412 s (325;490) to 468 s (415;553), median increase 56 (39;126) s (p = 0.018). In both IET and CWRET SpO was significantly higher and ventilatory equivalent for carbon dioxide significantly lower at end-exercise with oxygen compared to air, whereas CTO and QMTO did not significantly differ.

Conclusions: Patients with cyanotic CHD significantly improved their exercise performance, in terms of maximal work-rate and endurance time along with an improved arterial oxygenation and ventilatory efficiency with supplemental oxygen compared to air.
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http://dx.doi.org/10.1016/j.ijcard.2021.11.066DOI Listing
November 2021

Impact of a structured patient education programme on early diagnosis of prosthetic pulmonary valve endocarditis.

Cardiol Young 2021 Nov 10:1-6. Epub 2021 Nov 10.

Department of Cardiology, University Heart Center, University of Zurich, Zurich, Switzerland.

Background: Infective endocarditis is a major threat after prosthetic pulmonary valve replacement. Early diagnosis may improve outcomes.

Methods: A structured patient education programme for prevention and early diagnosis of infective endocarditis was developed at our institution since 2016. Time delay between onset of symptoms of prosthetic pulmonary valve endocarditis and its diagnosis (defined as initiation of appropriate high-dose intravenous antibiotic treatment) was compared for patients presenting before (cohort 1) and after (cohort 2) initiation of the patient education programme.

Results: Between 2008-2019, 26 patients (median age 24.9, range: 16.8-62.0 years, 73% male) were diagnosed with prosthetic pulmonary valve endocarditis, 13 patients (cohort 1) before (1.7 cases/year) and 13 patients (cohort 2) after June 2016 (3.7 cases/year). There were no differences in baseline characteristics or clinical presentation between the study cohorts. Overall, the median delay between onset of symptoms and diagnosis of infective endocarditis was 6 days (range: 0-133 days) with a significantly longer delay among patients in cohort 1, compared to cohort 2 (25 days, range: 5-133 days versus 3 days, range: 0-13 days, p < 0.0001). A delay of >7 days was documented in 11/13 patients (85%) in cohort 1 as compared to 1/13 (8%) in cohort 2 (p < 0.001). Need for urgent valve replacement or permanent deterioration of prosthetic valve function was higher in cohort 1, compared to cohort 2 (11/13, 85% versus 5/13, 39%; p = 0.041).

Conclusions: Prosthetic pulmonary valve endocarditis is increasingly recognised. A structured patient education programme may improve early diagnosis and clinical outcomes.
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http://dx.doi.org/10.1017/S1047951121004510DOI Listing
November 2021

Reduced Systolic Function and Not Genetic Variants Determine Outcome in Pediatric and Adult Left Ventricular Noncompaction Cardiomyopathy.

Front Pediatr 2021 3;9:722926. Epub 2021 Sep 3.

Department of Pediatric Cardiology, Charité - Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität zu Berlin, Berlin Institute of Health, Berlin, Germany.

Left ventricular noncompaction cardiomyopathy (LVNC CMP) is a genetic cardiomyopathy. Genotype-phenotype correlation and clinical outcome of genetic variants in pediatric and adult LVNC CMP patients are still unclear. The retrospective multicenter study was conducted in unrelated index patients with LVNC CMP, diagnosed between the years 1987 and 2017, and all available family members. All index patients underwent next-generation sequencing for genetic variants in 174 target genes using the Illumina TruSight Cardio Sequencing Panel. Major adverse cardiac events (MACE) included mechanical circulatory support, heart transplantation, survivor of cardiac death, and/or all-cause death as combined endpoint. Study population included 149 LVNC CMP patients with a median age of 27.8 (9.2-44.8) years at diagnosis; 58% of them were symptomatic, 18% suffered from non-sustained and sustained arrhythmias, and 17% had an implantable cardioverter defibrillator (ICD) implanted. 55/137 patients (40%) were ≤ 18 years at diagnosis. A total of 134 variants were identified in 87/113 (77%) index patients. 93 variants were classified as variant of unknown significance (VUS), 24 as likely pathogenic and 15 as pathogenic. The genetic yield of (likely) pathogenic variants was 35/113 (31%) index patients. Variants occurred most frequently in (n=19), ( = 10) and ( = 8). Altogether, sarcomere gene variants constituted 42.5% ( = 57) of all variants. The presence or absence of (likely) pathogenic variants or variants in specific genes did not allow risk stratification for MACE. Reduced left ventricular (LV) systolic function and increased left ventricular end-diastolic diameter (LVEDD) were risk factors for event-free survival in the Kaplan-Meier analysis. Through multivariate analysis we identified reduced LV systolic function as the main risk factor for MACE. Patients with reduced LV systolic function were at a 4.6-fold higher risk for MACE. Genetic variants did not predict the risk of developing a MACE, neither in the pediatric nor in the adult cohort. Multivariate analysis emphasized reduced LV systolic function as the main independent factor that is elevating the risk for MACE. Genetic screening is useful for cascade screening to identify family members at risk for developing LVNC CMP.
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http://dx.doi.org/10.3389/fped.2021.722926DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8447880PMC
September 2021

Neurocognitive functioning in young adults with congenital heart disease: insights from a case-control study.

Cardiol Young 2021 Jul 23:1-8. Epub 2021 Jul 23.

Child Development Centre, University Children's Hospital Zurich, Zurich, Switzerland.

Background: While there is evidence that cognitive impairment of children with congenital heart disease (CHD) may persist into adolescence, little is known about the spectrum of neurocognitive functioning of young adults with this disorder. The aim of this study was to assess neurocognitive functioning in a population of young adults with different types of CHD.

Methods: Cross-sectional cohort study in young adults with CHD and a group-matched healthy control group. We assessed neurocognitive and general intellectual functioning with a comprehensive battery of standardised neuropsychological tests. In addition to task-based assessments, questionnaire data of executive dysfunctions in everyday life were measured with the Behaviour Rating Inventory of Executive Function - Adult Version.

Results: A total of 67 patients (55% men) with CHD and 55 healthy controls (51% men) were included for analysis. Mean age at assessment was 26.9 (3.68) and 26.0 (3.32) years, respectively. The CHD group performed poorer in the domains of Executive Functions, Memory, Attention & Speed, and general intellectual functioning. Patients with a CHD of severe complexity were more affected than patients with simple or moderate complexity. Behaviour Rating Inventory of Executive Function - Adult Version scores indicated that patients' self-rated deficits in behaviour regulation in everyday life was higher compared with healthy controls.

Conclusion: Our findings indicate lower neurocognitive functioning in young adults with a CHD, particularly in those with severe defect complexity. In view of the potentially enhanced risk for cerebrovascular and neurodegenerative disease in this patient group as reported in the literature, systematic longitudinal monitoring of cognitive functioning is recommended.
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http://dx.doi.org/10.1017/S1047951121002705DOI Listing
July 2021

Risk stratification of adults with congenital heart disease during the COVID-19 pandemic: insights from a multinational survey among European experts.

Open Heart 2021 04;8(1)

Center for Congenital Heart Disease, Cardiology, Inselspital University Hospital Bern, University of Bern, Bern, Switzerland

Objective: Adults with congenital heart disease (ACHD) may be at a higher risk of a fatal outcome in case of COVID-19. Current risk stratification among these patients relies on personal experience and extrapolation from patients with acquired heart disease. We aimed to provide an expert view on risk stratification while awaiting results from observational studies.

Methods: This study was an initiative of the EPOCH (European Collaboration for Prospective Outcome Research in Congenital Heart disease). Among nine European countries (Austria, Belgium, Denmark, France, Germany, Italy, the Netherlands, Spain and Switzerland), 24 experts from 23 tertiary ACHD centres participated in the survey. ACHD experts were asked to identify ACHD-specific COVID-19 risk factors from a list of potential outcome predictors and to estimate the risk of adverse COVID-19 outcomes in seven commonly seen patient scenarios.

Results: 82% of participants did not consider all ACHD patients at risk of COVID-19 related complications. There was a consensus on pulmonary arterial hypertension, Fontan physiology and cyanotic heart disease as risk factors for adverse outcomes. Among different ACHD scenarios, a patient with Eisenmenger syndrome was considered to be at the highest risk. There was a marked variability in risk estimation among the other potential outcome predictors and ACHD scenarios.

Conclusions: Pulmonary arterial hypertension, Fontan palliation and cyanotic heart disease were widely considered as risk factors for poor outcome in COVID-19. However, there was a marked disparity in risk estimation for other clinical scenarios. We are in urgent need of outcome studies in ACHD suffering from COVID-19.
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http://dx.doi.org/10.1136/openhrt-2020-001455DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8061557PMC
April 2021

COVID-19 in Adults With Congenital Heart Disease.

J Am Coll Cardiol 2021 04;77(13):1644-1655

Division of Cardiology, Medical University of South Carolina, Charleston, South Carolina, USA.

Background: Adults with congenital heart disease (CHD) have been considered potentially high risk for novel coronavirus disease-19 (COVID-19) mortality or other complications.

Objectives: This study sought to define the impact of COVID-19 in adults with CHD and to identify risk factors associated with adverse outcomes.

Methods: Adults (age 18 years or older) with CHD and with confirmed or clinically suspected COVID-19 were included from CHD centers worldwide. Data collection included anatomic diagnosis and subsequent interventions, comorbidities, medications, echocardiographic findings, presenting symptoms, course of illness, and outcomes. Predictors of death or severe infection were determined.

Results: From 58 adult CHD centers, the study included 1,044 infected patients (age: 35.1 ± 13.0 years; range 18 to 86 years; 51% women), 87% of whom had laboratory-confirmed coronavirus infection. The cohort included 118 (11%) patients with single ventricle and/or Fontan physiology, 87 (8%) patients with cyanosis, and 73 (7%) patients with pulmonary hypertension. There were 24 COVID-related deaths (case/fatality: 2.3%; 95% confidence interval: 1.4% to 3.2%). Factors associated with death included male sex, diabetes, cyanosis, pulmonary hypertension, renal insufficiency, and previous hospital admission for heart failure. Worse physiological stage was associated with mortality (p = 0.001), whereas anatomic complexity or defect group were not.

Conclusions: COVID-19 mortality in adults with CHD is commensurate with the general population. The most vulnerable patients are those with worse physiological stage, such as cyanosis and pulmonary hypertension, whereas anatomic complexity does not appear to predict infection severity.
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http://dx.doi.org/10.1016/j.jacc.2021.02.023DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8006800PMC
April 2021

Infective endocarditis: prevention and antibiotic prophylaxis.

Swiss Med Wkly 2021 02 28;151:w20473. Epub 2021 Feb 28.

University Heart Center, Department of Cardiology, University Hospital of Zurich, Zurich, Switzerland.

The Swiss societies of Infectious Diseases, Pediatric Cardiology and Cardiology and the Pediatric Infectious Disease Group of Switzerland present the current update on infective endocarditis prophylaxis in a joint initiative. The major focus of the revised recommendations is a comprehensive prevention campaign for all patients at risk for infective endocarditis. Antibiotic prophylaxis is recommended only for individuals at high risk. Within this high-risk group there is a ranking order, and the conditions are presented accordingly. Antibiotic prophylaxis is no longer recommended for patients with unrepaired ventricular septal defects and patent ductus arteriosus. Recommendations for antibiotic prophylaxis for the prevention of infective endocarditis are categorized in dental and non-dental interventions.
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http://dx.doi.org/10.4414/smw.2021.20473DOI Listing
February 2021

Clinical outcome of COVID-19 in patients with adult congenital heart disease.

Heart 2021 Mar 8. Epub 2021 Mar 8.

Departament of Cardiology, University Heart Center, University Hospital Zurich, Zurich, Switzerland.

Aims: Patients 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.

Methods: Twenty-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.

Results: Of 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/m (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).

Conclusions: Among 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.
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http://dx.doi.org/10.1136/heartjnl-2020-318467DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7944416PMC
March 2021

Dental Medicine and Infective Endocarditis: Current guidelines for antibiotic prophylaxis and recommendations for daily clinical practice.

Swiss Dent J 2021 Mar;131(3):245-251

Klinik für Oral Health & Medicine, Universitäres Zentrum für Zahnmedizin Basel UZB und Universität Basel.

Surgical interventions in the dental practice as well as interventions in the ear, nose and throat area, the skin, the respiratory, gastrointestinal or urogenital tract can lead to transient bacteremia. As a result, an infectious endocarditis (IE) may occur. Overall, this is a rare occurrence, but it is associated with high morbidity and mortality. Individuals with a history of IE, implanted valve prostheses and certain congenital heart defects are at the highest risk for IE. In dental medicine, there is a controversial debate as to whether bacteremia can occur already by chewing or brushing the teeth. Antibiotic prophylaxis for prevention of endocarditis should only be given to those patients who are at the highest risk for IE. This article discusses novelties and Swiss recommendations for antibiotic prophylaxis for highrisk patients for IE in the dental practice. In this context, antibiotic prophylaxis has gained importance for dentists, which is also reflected by the fact that the recommendations on the new endocarditis card are limited solely to dental interventions.
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March 2021

Brain volumes in adults with congenital heart disease correlate with executive function abilities.

Brain Imaging Behav 2021 Oct 30;15(5):2308-2316. Epub 2021 Jan 30.

Children's Research Center, University Children's Hospital Zurich, Zurich, Switzerland.

Congenital heart disease is the most common birth defect, and patients are at risk for neurodevelopmental impairment and brain abnormalities. Yet, little is known about the link between brain volumes and cognitive function in adults with congenital heart disease. Forty-four patients and 53 controls between 18 and 32 years underwent brain magnetic resonance imaging and cognitive testing, assessed with an intelligence quotient and executive function global score. Associations between brain volumes and cognitive function were calculated using linear models. Cognitive function in patients was within the normal range (intelligence quotient: 97.74 (10.76)). Total brain volume was significantly smaller in patients compared to controls (1067.26 (113.53) vs 1113.04 (97.88) cm, P < 0.01), irrespective of cardiac factors (heart defect complexity, cyanosis, cardiopulmonary bypass: all P > 0.4). After adjusting for total brain volume, only corpus callosum volume remained significantly smaller (P = 0.03). Smaller total brain volume was associated with poorer overall executive functioning (P = 0.02) and inhibition (P < 0.01), in both patients and controls. The association between total brain volume and overall executive functioning was moderated by parental socioeconomic status (lower socioeconomic status was associated with a stronger association between brain volume and EF; interaction P = 0.03). In adults with congenital heart disease, despite normal intelligence quotient, brain volume alterations persist into adulthood and are related to executive functioning, in particular inhibitory control. Adults coming from low socioeconomic background and with altered brain volumes are especially vulnerable and should thus be followed-up during adulthood to ensure optimal social and educational support.
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http://dx.doi.org/10.1007/s11682-020-00424-1DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8500877PMC
October 2021

Altered white matter microstructure is related to cognition in adults with congenital heart disease.

Brain Commun 2021 28;3(1):fcaa224. Epub 2020 Dec 28.

Child Development Center, University Children's Hospital Zurich, Zurich, Switzerland.

Adults with congenital heart disease are at risk for persisting executive function deficits, which are known to affect academic achievement and quality of life. Alterations in white -matter microstructure are associated with cognitive impairments in adolescents with congenital heart disease. This study aimed to identify microstructural alterations potentially associated with executive function deficits in adults with congenital heart disease. Diffusion tensor imaging and tract-based spatial statistics were conducted in 45 patients (18 females) and 54 healthy controls (26 females) aged 18-32 years. Fractional anisotropy of white matter diffusion was compared between groups and correlated with an executive function score, derived from an extensive neuropsychological test battery. Patients showed widespread bilateral reduction in fractional anisotropy ( < 0.05, multiple comparison corrected) compared to controls. Lower fractional anisotropy was driven by patients with moderate and severe defect complexity (compared to controls:  < 0.001). Executive function scores were lower in patients ( < 0.05) and associated with lower fractional anisotropy in the left superior corona radiata and the corticospinal tract (corrected  < 0.05). Our findings confirm alterations of white matter microstructure in adults with congenital heart disease, mainly in those patients of moderate to severe complexity. These alterations are associated with impairments in executive functioning. A better understanding of the neurocognitive deficits may help counselling and care of patients with congenital heart disease across their lifespan and have the potential to improve their outcome and quality of life.
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http://dx.doi.org/10.1093/braincomms/fcaa224DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7811757PMC
December 2020

Thrombocytopaenia in cyanotic CHD.

Cardiol Young 2021 Mar 2;31(3):429-434. Epub 2020 Dec 2.

Department of Cardiology, University Heart Center, University Hospital Zurich, Zurich, Switzerland.

Introduction: Thrombocytopaenia is common in adults with cyanotic heart disease. Our aim was to explore potential mechanisms for thrombocytopaenia in these vulnerable patients.

Methods: Adults with cyanotic heart defects were identified from our clinical database. Haemoglobin levels, platelet counts, and resting oxygen saturations were determined at baseline and during follow-up. Associations between patient characteristics and cardiac physiology with these parameters at baseline and during follow-up were analysed using regression models. Survival estimates were determined by the Kaplan-Meier method.

Results: We included 79 patients (mean age 32.2 ± 12.4, 48 (61%) Eisenmenger syndrome, 20 (25%) Down syndrome). Mean oxygen saturation was 84.1 ± 5.9%; 38 (48%) had thrombocytopaenia. There was a strong inverse correlation between platelet count and haemoglobin level (R = -0.655, R2 = 0.429, p < 0.0001) and a weaker but significant positive correlation between platelet count and oxygen saturation (R = 0.345, R2 = 0.119, p = 0.002). There was a significant inverse correlation between decrease in platelet count and increase in haemoglobin level during follow-up (R = -0.401, R2 = 0.161, p = 0.001) but not to changes in oxygen saturation (R = 0.043, R2 = 0.002, p = 0.726). Survival estimates were lower for patients with thrombocytopaenia at baseline (log-rank test p < 0.0001).

Conclusions: Our findings suggest a direct inverse correlation between platelet counts and haemoglobin levels in adults with cyanotic heart disease. Further studies are required to explore the mechanisms of thrombocytopaenia in cyanotic heart disease and its potential role as an independent marker of risk.
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http://dx.doi.org/10.1017/S1047951120004230DOI Listing
March 2021

Pregnancy outcomes in women with aortic coarctation.

Heart 2020 Oct 29. Epub 2020 Oct 29.

Department of Cardiology, Erasmus MC, Rotterdam, The Netherlands

Objective: Pregnancy 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.

Methods: Pregnancies 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.

Results: Of 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.

Conclusions: The 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.
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http://dx.doi.org/10.1136/heartjnl-2020-317513DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7873427PMC
October 2020

Recommendations for advance care planning in adults with congenital heart disease: a position paper from the ESC Working Group of Adult Congenital Heart Disease, the Association of Cardiovascular Nursing and Allied Professions (ACNAP), the European Association for Palliative Care (EAPC), and the International Society for Adult Congenital Heart Disease (ISACHD).

Eur Heart J 2020 11;41(43):4200-4210

Center for Congenital Heart Disease, University Hospital Inselspital, Department of Cardiology, University of Bern, Freiburgstrasse 15, 3010 Bern, Switzerland.

Survival prospects in adults with congenital heart disease (CHD), although improved in recent decades, still remain below expectations for the general population. Patients and their loved ones benefit from preparation for both unexpected and predictable deaths, sometimes preceded by a prolonged period of declining health. Hence, advance care planning (ACP) is an integral part of comprehensive care for adults with CHD. This position paper summarizes evidence regarding benefits of and patients' preferences for ACP and provides practical advice regarding the implementation of ACP processes within clinical adult CHD practice. We suggest that ACP be delivered as a structured process across different stages, with content dependent upon the anticipated disease progression. We acknowledge potential barriers to initiate ACP discussions and emphasize the importance of a sensitive and situation-specific communication style. Conclusions presented in this article reflect agreed expert opinions and include both patient and provider perspectives.
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http://dx.doi.org/10.1093/eurheartj/ehaa614DOI Listing
November 2020

Safety and efficacy of non-vitamin K antagonist oral anticoagulants for prevention of thromboembolism in adults with systemic right ventricle: Results from the NOTE international registry.

Int J Cardiol 2021 01 14;322:129-134. Epub 2020 Aug 14.

Adult Congenital Heart Disease Unit, Department of Cardiology, Monaldi Hospital, Naples, Italy.

Background: Patients with systemic right ventricle (sRV), including transposition of great arteries (TGA) after atrial switch procedure and congenitally corrected transposition of great arteries (ccTGA), may require anticoagulation for thromboembolism (TE) prevention. In the absence of data on non-vitamin K antagonist oral anticoagulants (NOACs), vitamin K antagonists (VKAs) remain the agent of choice. We investigated the safety, efficacy and feasibility of NOACs treatment in adults with sRV in a worldwide study.

Methods: This is an international multicentre prospective study, using data from the NOTE registry on adults with sRV taking NOACs between 2014 and 2019. The primary endpoints were TE and major bleeding (MB). The secondary endpoint was minor bleeding.

Results: A total of 76 patients (42.5 ± 10.0 years, 76% male) with sRV (74% TGA, 26% ccTGA) on NOACs were included in the study. During a median follow-up of 2.5 years (IQR1.5-3.9), TE events occurred in 3 patients (4%), while no MB episodes were reported. Minor bleeding occurred in 9 patients (12%). NOAC treatment cessation rate was 1.4% (95%CI:0.3-4%) during the first year of follow-up. All the patients with TE events had a CHADS-VASc score ≥ 2 and impaired sRV systolic function at baseline. The total incidence of major events during follow-up was significantly lower compared to historical use of VKAs or aspirin before study inclusion (1.4% (95%CI:0.29-4%) vs 6,9% (95%CI:2.5-15.2%); p = .01).

Conclusions: In this prospective study, NOACs appear to be well-tolerated, with excellent efficacy and safety at mid-term in patients with sRV.
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http://dx.doi.org/10.1016/j.ijcard.2020.08.034DOI Listing
January 2021

Marfan syndrome and related connective tissue disorders in the current era in Switzerland in 103 patients: medical and surgical management and impact of genetic testing.

Swiss Med Wkly 2020 03 3;150:w20189. Epub 2020 Apr 3.

Cardiovascular Centre, Klinik Im Park, Zurich, Switzerland.

Introduction: Marfan syndrome (MFS) and related connective tissue disorders (CTDs) are increasingly recognised. Genetic testing has greatly improved the diagnostic outcome/power over the last two decades. In this study we describe a multicentre cohort of adults with MFS and related CTDs, with a particular focus on results from genetic testing.

Methods: All patients with MFS and related CTDs were identified from the databases of five centres in the canton of Zurich. Echocardiographic and clinical findings including systemic Marfan score, use of medication and genetic results were retrospectively analysed. MFS was diagnosed using the revised Ghent criteria (including FBN1 genetic testing if available); other CTDs (Loeys-Dietz syndrome) were diagnosed by genetic testing only.

Results: A cohort of 103 patients were identified (62 index patients, 41 relatives of family members): 96 patients with MFS and 7 patients with other CTD, 54 males (52%), median age 23 years (range 1–75). The median systemic Marfan score was 5 (range 0–18). Only 40 patients (40/103, 39%) fulfilled criteria for systemic involvement (≥7 points). A history of aortic dissection was present in 14 out of 103 patients (14%). Echocardiographic data were available for all: aortic root enlargement (Z-score ≥2 in adults, Z-score ≥3 in children) was found in 49 patients (48%) and mitral valve prolapse in 64 (62%). Genetic testing had been performed in 80 patients (78%); FBN1 mutations were present in 69 patients (86%); other pathogenic mutations could be identified in seven patients (9%); no disease-causing mutation was found in four patients, three of them fulfilling the Ghent criteria of MFS. Of the mutation-positive patients, 33 had a systemic score of ≥7 and 43 had a systemic score of ≥5. Revised Ghent criteria were fulfilled in 70 patients: in 69 patients with FBN1 mutations and 1 patient with another CTD. Recommended treatment (beta-blocker, angiotensin receptor blocker) was taken by 63% of patients.

Conclusions: In this cohort a high percentage of patients fulfilling the revised Ghent criteria for MFS underwent genetic testing, often leading to or confirming the diagnosis of MFS. Other CTDs could be discriminated best by genetic testing. With respect to the diagnosis of MFS and related CTDs, the usefulness of the systemic score is limited, showing the importance of genetic testing, which enabled definitive diagnosis in 95% of tested patients. Patient education on medical treatment still has to be improved. (Trial registration no: KEK-ZH-Nr. 2013-0241).
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http://dx.doi.org/10.4414/smw.2020.20189DOI Listing
March 2020

Structural brain abnormalities in adults with congenital heart disease: Prevalence and association with estimated intelligence quotient.

Int J Cardiol 2020 05 24;306:61-66. Epub 2020 Feb 24.

Child Development Centre, University Children's Hospital Zurich, Steinwiesstrasse 75, 8032 Zurich, Switzerland; Children's Research Centre, University Children's Hospital Zurich, Steinwiesstrasse 75, 8032 Zurich, Switzerland. Electronic address:

Background: Little is known about the prevalence of structural brain abnormalities and cognitive functioning in the growing population of patients with adult congenital heart disease (ACHD). Thus, our aim was to assess structural abnormalities on brain magnetic resonance imaging (MRI) and their association with intelligence quotient (IQ) in ACHD patients.

Methods: Cross-sectional study in ACHD patients and healthy controls as comparison group. Brain MRI was performed on a 3 T MR scanner, and inspection of structural abnormalities was performed blinded to ACHD or control status. IQ was estimated using the vocabulary and matrix reasoning subtests from the Wechsler Adult Intelligence Scale, Fourth Edition.

Results: A total number of 67 (55% males) ACHD patients and 55 (51% males) controls were included (mean age 26.9 and 26.0 years respectively). Abnormalities on brain MRI were detected in 29 of 46 (63%) ACHD patients and in none of the controls. Abnormalities consisted of focal infarction or atrophy, white matter lesions, microhemorrhages, and global atrophy. Mean estimated IQ was significantly lower in ACHD patients than in controls (98.51 versus 104.38; 95% CI: -10.09 to -1.66; P value = 0.007). Comparison between patients with and without cerebral abnormalities revealed no significant difference in estimated IQ.

Conclusion: Our findings indicate a high prevalence and wide spectrum of structural brain abnormalities in ACHD patients. Furthermore, this population is at a higher risk of impaired intellectual functioning than healthy controls. However, the present study could not establish a statistically significant association between MRI findings and estimated IQ.

Clinical Trial Registration: ClinicalTrials.gov ID: NCT04041557; URL: https://clinicaltrials.gov/ct2/show/NCT04041557?term=NCT04041557&rank=1.
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http://dx.doi.org/10.1016/j.ijcard.2020.02.061DOI Listing
May 2020

Simple cardiac shunts in adults: atrial septal defects, ventricular septal defects, patent ductus arteriosus.

Heart 2020 02 7;106(4):307-314. Epub 2020 Jan 7.

Cardiology, University Hospital Zurich, Zurich, Switzerland.

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http://dx.doi.org/10.1136/heartjnl-2019-314700DOI Listing
February 2020

Percutaneous Valve Interventions in the Adult Congenital Heart Disease Population: Emerging Technologies and Indications.

Can J Cardiol 2019 Dec 24;35(12):1740-1749. Epub 2019 Oct 24.

Toronto Congenital Cardiac Centre for Adults, Division of Cardiology, University of Toronto, Mount Sinai Hospital, University Health Network, Toronto, Ontario, Canada.

Adult survivors with congenital heart disease are not cured and residual cardiac valve lesions are common and contribute substantially to long-term morbidity. Given the increased risk of reoperations in patients with previous cardiac surgery, percutaneous treatment options have been developed. Initially percutaneous therapies focused on right ventricular outflow tract lesions, but they have now expanded to include mitral and aortic valve interventions. Although some of these procedures, such as balloon valvuloplasty of pulmonary valve stenosis and percutaneous pulmonary valve replacement, have become standard of care, there are many new and evolving technologies that will likely become important treatment strategies over the coming decade. The key for success of these transcatheter valve procedures is the careful evaluation of the patient's individual anatomy and physiology and a multidisciplinary assessment involving cardiologists specialized in adult congenital heart disease, specialized imagers, cardiac surgeons, and interventionalists. Because many of these percutaneous interventions are relatively new, long-term outcomes are not yet well defined, dictating the need for careful and structured long-term observational studies on outcomes of these novel procedures, which will allow refining the indications of a specific intervention and to improve its technical aspects. The aim of this article is to provide an overview of common valve lesions in the adult congenital heart disease population and to discuss treatment options and strategies with a specific focus on percutaneous options.
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http://dx.doi.org/10.1016/j.cjca.2019.10.019DOI Listing
December 2019

Myocardial blood flow and cardiac sympathetic innervation in young adults late after arterial switch operation for transposition of the great arteries.

Int J Cardiol 2020 01 13;299:110-115. Epub 2019 Jul 13.

Department of Nuclear Medicine, Cardiac Imaging, University Hospital Zurich, Raemistrasse 100, 8091 Zurich, Switzerland. Electronic address:

Background: The arterial switch operation (ASO) for repair of transposition of the great arteries (TGA) requires transection of the great arterial trunks and re-implantation of the coronary arteries into the neoaortic root resulting in cardiac sympathetic denervation which may affect myocardial blood flow (MBF) regulation. The aims of the present study were to evaluate sympathetic (re-)innervation in young adults after ASO and its impact on MBF.

Methods: Twelve patients (age 22.5 ± 2.6 years) after ASO for TGA in the neonatal period and ten healthy controls (age 22.0 ± 1.7 years) were included. Positron emission tomography (PET) was used for measuring cardiac sympathetic innervation with [C]meta-hydroxyephedrine (mHED) and MBF with [O]HO PET at rest, during adenosine stimulation, and during sympathetic stimulation with cold pressor test. Cold pressor-induced MBF response capacity was calculated as maximal global MBF over peak rate-pressure product multiplied by 10'000.

Results: Global [C]mHED uptake was significantly lower in patients compared to controls (7.0 ± 2.3 versus 11.8 ± 2.1%/min, p < 0.001). Global MBF was lower in patients compared to controls at rest and during adenosine-induced hyperemia (0.66 ± 0.08 versus 0.82 ± 0.15 ml/min/g, p = 0.005; 2.23 ± 1.19 versus 3.36 ± 1.04 ml/min/g, p = 0.030, respectively). Interestingly, MBF during cold pressor test did not differ between patients and controls (0.99 ± 0.20 versus 1.07 ± 0.16 ml/min/g, p = 0.330). However, cold pressor-induced MBF response capacity was significantly lower for patients as compared to controls (1.09 ± 0.35 versus 1.44 ± 0.39 ml/g/10,000 mmHg, p = 0.040).

Conclusions: With only partial sympathetic re-innervation of the coronary arteries, maximal dilator capacity of the coronary microvasculature and cold pressor-induced MBF response capacity remain substantially impaired in young adults after ASO compared to healthy controls.
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http://dx.doi.org/10.1016/j.ijcard.2019.07.041DOI Listing
January 2020

Non-vitamin K antagonist oral anticoagulants in adults with a Fontan circulation: are they safe.

Open Heart 2019;6(1):e000985. Epub 2019 Jun 3.

Cardiology, UCLA, Los Angeles, California, USA.

Background: In Fontan patients with atrial arrhythmias (AA), non-vitamin K antagonist oral anticoagulants(NOACs) have a class III recommendation according to the Pediatric & Congenital Electrophysiology Society (PACES)/Heart Rhythm Society (HRS) guideline in 2014, due to lack of data on outcomes as opposed to evidence of harm. To address this gap in data, we investigated the safety and efficacy of NOACs in adults with a Fontan circulation in a worldwide study.

Methods: This is an international multicentre prospective cohort study, using data from the NOTE (on-vitamin K antagonist ral anticoagulants for hrombombolic prevention in patients with congenital heart disease) registry. The study population comprised consecutive adults with a Fontan circulation using NOACs. Follow-up took place at 6 months and yearly thereafter. The primary endpoints were thromboembolism and major bleeding. Secondary endpoint was minor bleeding.

Results: From April 2014 onward, 74 patients (mean age 32±10 years (range 18-68), 54% male) with a Fontan circulation using NOACs were included. During a median follow-up of 1.2 (IQR 0.8-2.0) years, three thromboembolic events (2.9 per 100 patient-years (95% CI 0.7 to 7.6)) and three major bleedings (2.9 per 100 patient-years (95% CI 0.7 to 7.6)) occurred in five atriopulmonary Fontan and one total cavopulmonary connection Fontan patients with AA. Fifteen patients experienced minor bleeding episodes (15.8 per 100 patient-years (95% CI 9.1 to 25.2)). In patients (n=37) using vitamin K antagonists (VKAs) prior to the initiation of NOAC, annual incidence of historical thromboembolic events and major bleeding were 2.4% (95% CI 0.4% to 7.4%) (n = 2) and 1.2% (95% CI 0.7% to 5.1%) (n = 1), respectively.

Conclusions: In this review of the largest Fontan cohort using NOACs with prospective follow-up, NOACs appear to be well tolerated and their efficacy and safety during short-term follow-up seem comparable to VKAs. Longer term data are required to confirm these promising short-term results.
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http://dx.doi.org/10.1136/openhrt-2018-000985DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6560660PMC
February 2021

Patterns of Incidence Rates of Cardiac Complications in Patients With Congenital Heart Disease.

Can J Cardiol 2018 12 29;34(12):1624-1630. Epub 2018 Sep 29.

Department of Cardiology, University Hospital of Basel, University of Basel, Basel, Switzerland. Electronic address:

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

Methods: The analysis included participants of the Swiss Adult Congenital Heart Disease Registry (SACHER). For this study, cardiac complications up to the time of inclusion in SACHER were analysed. Complications included atrial fibrillation, atrial flutter, supraventricular tachycardia, ventricular tachycardia, complete heart block, heart failure, stroke, endocarditis, myocardial infarction, and pulmonary hypertension. Incidence rates (IR; incidence rate per 1000 patient-years) for different age categories and diagnosis groups were analysed.

Results: Of 2731 patients (55% male, mean age 34 ± 14 years, 92,349 patient-years), a total of 767 (28%) had experienced at least 1 cardiac complication. The majority of complications (550; 72%) occurred in adulthood (> 18 years). Apart from perioperative stroke (IR: 1.77 in age group ≤ 4 years) and complete heart block (IR: 2.36 in age group ≤ 4 years), IR were much lower in childhood (IR < 1 for all complications between 5 and 17 years). Incidence of cardiac complications increased during adult life with highest IR for atrial fibrillation and atrial flutter in the age group ≥ 50 years (IR: 17.6 and 9.7, respectively). There were important variations of the distribution of complications among different diagnosis groups.

Conclusions: Cardiac complications are frequent in congenital heart disease. Apart from perioperative stroke and complete heart block, IR are low in childhood but the incidence increases during adult life. These data underscore the need of lifelong follow-up and may help for better allocation of resources maintaining follow-up.
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http://dx.doi.org/10.1016/j.cjca.2018.09.010DOI Listing
December 2018

The impact of trisomy 21 on treatment modalities and outcome in adults with congenital heart disease in Switzerland.

Pulm Circ 2019 Jan-Mar;9(1):2045894018811147. Epub 2018 Oct 19.

1 Center for Congenital Heart Disease, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland.

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.
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http://dx.doi.org/10.1177/2045894018811147DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6287318PMC
October 2018

Predictors of quality of life in young adults with congenital heart disease.

Eur Heart J Qual Care Clin Outcomes 2019 04;5(2):161-168

Department of Psychosomatics and Psychiatry, University Children's Hospital Zurich, Steinwiesstrasse 75, Zurich, Switzerland.

Aims: The aim of this study was to identify medical and psychosocial risk factors for impaired health-related quality of life (HRQoL) and poor psychological adjustment (PA) in young adults with congenital heart disease (CHD).

Methods And Results: A group of 188 patients (43% females, ages 18-30 years) with various types of CHD and 139 age-matched healthy controls (57% females) completed questionnaires assessing HRQoL, PA, social support, significant life events in the past year, education level, civil status, and employment status. Medical variables were retrieved from the patients' hospital records. Patients reported worse physical HRQoL than controls but similar mental HRQoL and PA. Female CHD patients showed worse physical and mental HRQoL and poorer PA than males. In CHD patients, a lower educational level and lower physical exercise capacity predicted lower physical HRQoL, but complexity of CHD was not related to HRQoL or PA. Less social support was associated with lower mental HRQoL and poorer PA in CHD patients.

Conclusion: Young adults with CHD have impaired physical HRQoL but normal mental HRQoL and PA. Lower physical exercise capacity, female sex, less social support, and lower educational level predict an unfavourable quality of life and PA. This subgroup of patients should be monitored more closely and provided with special psychosocial care to improve long-term outcome.
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http://dx.doi.org/10.1093/ehjqcco/qcy046DOI Listing
April 2019

Pulmonary artery dilatation in congenital heart disease: Size doesn't matter.

Int J Cardiol 2019 02 23;277:235-236. Epub 2018 Aug 23.

Adult Congenital Heart Disease Program, University Heart Center, Zurich, Switzerland.. Electronic address:

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http://dx.doi.org/10.1016/j.ijcard.2018.08.063DOI Listing
February 2019

Anticoagulation practices in adults with congenital heart disease and atrial arrhythmias in Switzerland.

Congenit Heart Dis 2018 Sep 22;13(5):678-684. Epub 2018 Jul 22.

Department of Cardiology, University Hospital of Basel, University of Basel, Switzerland.

Background: 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.

Methods: 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.

Results: Compared with patients with Aflut/IART, patients with Afib were older (51 ± 16.1 vs 37 ± 16 years, P < .001) and had a higher CHA DS -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)], CHA DS -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].

Conclusions: In this multicenter study, age, type, and duration of atrial arrhythmias, CHA DS -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.
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http://dx.doi.org/10.1111/chd.12627DOI Listing
September 2018

Editorial commentary: The caveats of cardiac imaging in Ebstein anomaly.

Trends Cardiovasc Med 2018 08 20;28(6):410-411. Epub 2018 Feb 20.

Cardiovascular Center Zurich, Klinik im Park, Switzerland. Electronic address:

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http://dx.doi.org/10.1016/j.tcm.2018.02.007DOI Listing
August 2018

Mid-term Outcome of 100 Consecutive Ross Procedures: Excellent Survival, But Yet to Be a Cure.

Pediatr Cardiol 2018 Mar 12;39(3):595-603. Epub 2018 Jan 12.

Pediatric Heart Centre, University Children's Hospital Zurich, Zurich, Switzerland.

The Ross procedure offers excellent short-term outcome but the long-term durability is under debate. Reinterventions and follow-up of 100 consecutive patients undergoing Ross Procedure at our centre (1993-2011) were analysed. Follow-up was available for 96 patients (97%) with a median duration of 5.3 (0.1-17.1) years. Median age of the patient cohort was 15.2 (0.04-58.4) years with 76 males. 93% had underlying congenital aortic stenosis. Root replacement technique was applied in all. The most common valved conduits used for reconstruction of the right ventricular outflow tract were homografts (66 patients) and bovine jugular vein (Contegra) graft (31 patients). Additional procedures included Ross-Konno procedure (14%), resection of subaortic stenosis/myectomy (11%) and reduction plasty of the ascending aorta (25%). One patient died within the first 30 days (1%). Late deaths occurred in 4 patients (4%) 0.5-4.5 years postoperatively: causes included pulmonary hypertension due to endocardial fibroelastosis (2), subarachnoid haemorrhage (1) and sudden cardiac death (1). Five-year survival was 93.6 (95% CI 88.1-99.1)%. Moderate or severe aortic (autograft) regurgitation needing reoperation occurred in 8 patients with a 5-year freedom from autograft reoperation of 98.5 (95.6-100)%. Five-year freedom from reintervention (surgery or catheter based) on the right ventricular outflow tract conduit was 91.5 (85.5-96.5)%. Univariate predictors of this reinterventions were smaller graft size (p = 0.03) and use of a Contegra graft (p = 0.04). Ross procedure can be performed with low mortality and good survival in the long term. Most of the reinterventions are related to the neo-right ventricular outflow tract and may be partly attributed to the lack of growth. While the Ross Procedure remains an invaluable option for aortic valve disease in children, new solutions for the neo-pulmonary valve as well as for the less often occurring problems on the autograft are needed.
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http://dx.doi.org/10.1007/s00246-017-1798-zDOI Listing
March 2018
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