Papers by Christine Attenhofer
Journal of Cardiovascular Electrophysiology, 2017
Christoph Scharf reports grant support for scientific foundation from Medtronic, Boston Scientifi... more Christoph Scharf reports grant support for scientific foundation from Medtronic, Boston Scientific, Abbott, Biotronik; former proctor contract for Watchman (Atritech); stockholder and founder of Acutus. Other authors: No disclosures.
The American Journal of Cardiology
Our aim was to assess changes of right ventricular end-diastolic volumes (RVEDVi) and right ventr... more Our aim was to assess changes of right ventricular end-diastolic volumes (RVEDVi) and right ventricular ejection fraction (RVEF) in asymptomatic adults with repaired tetralogy of Fallot, with native right ventricular outflow tract and severe pulmonary regurgitation by serial cardiac magnetic resonance imaging (CMR). The study included 23 asymptomatic adults who underwent ≥3 CMR studies (total of 88 CMR studies). We compared changes in RVEDVi and RVEF between first and last study (median follow-up: 8.8 years, interquartile range: 6.3 to 13.1 years) and between all study pairs. Variability of measurements between study pairs (65 consecutive and 139 nonconsecutive CMR study pairs) were assessed using Bland-Altman analysis and intraclass correlation coefficients. On average, there were no significant changes of RVEDVi or RVEF over the study period (change in RVEDVi: +0.4 ± 17.8 ml/m2, change in RVEF: -1.0 ± 5.5%). Assessment of variability of measurements between study pairs demonstrated no systematic change in RVEDVi and RVEF between study pairs with limits of agreement within the range of previously published studies (RVEDVi -29.1 to +27.2 ml/m2; RVEF -11.5% to 10.2%). High intraclass correlation coefficients for RVEDVi (0.943, 95% CI 0.906 to 0.965, p <0.001) and RVEF (0.815, 95% CI 0.697 to 0.887, p <0.0001) indicate high reliability of reported measurements. In conclusion, in asymptomatic adults with repaired tetralogy of Fallot with native right ventricular outflow tracts and severe pulmonary regurgitation, CMR measurements of RV volumes and RVEF remain stable during follow-up with variability between CMR studies in individual patients, as expected for interobserver and interstudy variability. Measurements derived from a single CMR study or changes occurring between 2 CMR studies should be used with caution for clinical decision-making.
Sinus node disease and arrhythmias in the long-term follow-up of former professional cyclists
Swiss Medical Weekly, 2020
INTRODUCTION: Marfan syndrome (MFS) and related connective tissue disorders (CTDs) are increasing... more 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
European Heart Journal – Cardiovascular Imaging, 2017
Aims To determine the prevalence of baffle leaks in adults after atrial switch operations for tra... more 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: Thoracic radiotherapy (RT), especially with past technology, may affect the heart, bu... more Background: Thoracic radiotherapy (RT), especially with past technology, may affect the heart, but rarely leads to symptoms. In patients with symptomatic heart disease after RT, outcome seems to be dismal. Methods: In this observational descriptive study, clinical characteristics, findings of ECG, echocardiography, cardiac interventions and follow-up were analysed in patients with prior RT and symptomatic heart disease. The patients were identified in the echocardiography database during a ten year period. Results: There were 25 patients who had thoracic RT at a median age of 35 years (range: 9-59) for lymphoma (12 patients), breast cancer (9) or other cancer (4). At least likely inclusion of the heart in the target volume of previous RT was present in of 16 of 20 patients with detailed information on RT. Last follow-up was 24 years (range: 5-57) after RT at a median age of 56 years (range: 30-84). Symptoms (1 per patient) included: dyspnea (21 patients), angina (12) and/or heart failure (10). Three patients had prior myocardial infarction. The following disease was found: moderate valvular disease in 19 patients (76%), coronary artery disease in 12 (48%), abnormal ECG in 18 (72%), relevant conduction system disease in 9 (36%), restriction / constriction in 7 (28%), and pericardial effusion in 4 (16%). Cardiac surgery was necessary in 12 patients (death in 2 patients), percutaneous coronary interventions in 5 and pacemaker implantation in 3. Endocarditis occurred in 2 patients. During followup (21 ± 8 months), death occurred in 6 patients (24%) and was due to heart disease in 5 of them. Conclusions: If symptomatic heart disease develops in the long-term follow-up after RT, complex disease of valves, coronary arteries, conduction system, myocardium and pericardium is frequently observed. Cardiac interventions are often necessary; and heart disdisease may be a common cause of death in these patients. Careful assessment and evaluation of treatment options are needed in this patient group.
Journal of the American College of Cardiology, 2000
We sought to describe characteristics and outcome in adults with isolated ventricular noncompacti... more We sought to describe characteristics and outcome in adults with isolated ventricular noncompaction (IVNC). BACKGROUND Isolated ventricular noncompaction is an unclassified cardiomyopathy due to intrauterine arrest of compaction of the loose interwoven meshwork. Knowledge regarding diagnosis, morbidity and prognosis is limited. METHODS Echocardiographic criteria for IVNC include-in the absence of significant heart lesionssegmental thickening of the left ventricular myocardial wall consisting of two layers: a thin, compacted epicardial and an extremely thickened endocardial layer with prominent trabeculations and deep recesses. Thirty-four adults (age Ն16 years, 25 men) fulfilled the diagnostic criteria and were followed prospectively. RESULTS At diagnosis, mean age was 42 Ϯ 17 years, and 12 patients (35%) were in New York Heart Association class III/IV. Left ventricular end-diastolic diameter was 65 Ϯ 12 mm and ejection fraction 33 Ϯ 13%. Apex and/or midventricular segments of both the inferior and lateral wall were involved in Ͼ80% of patients. Follow-up was 44 Ϯ 40 months. Major complications were heart failure in 18 patients (53%), thromboembolic events in 8 patients (24%) and ventricular tachycardias in 14 patients (41%). There were 12 deaths: sudden in six, end-stage heart failure in four and other causes in two patients. Four patients underwent heart transplantation. Automated cardioverter/defibrillators were implanted in four patients. CONCLUSIONS Diagnosis of IVNC by echocardiography using strict criteria is feasible. Its mortality and morbidity are high, including heart failure, thrombo-embolic events and ventricular arrhythmias. Risk stratification includes heart failure therapy, oral anticoagulation, heart transplantation and implantation of an automated defibrillator/cardioverter. As IVNC is a distinct entity, its classification as a specific cardiomyopathy seems to be more appropriate.
Journal of the American College of Cardiology, 2002
Background Atdal natriuretic peptide (ANP) is a circulating hormone mainly of cardiac origin whos... more Background Atdal natriuretic peptide (ANP) is a circulating hormone mainly of cardiac origin whose biological actions include promotion of natriuresis and vasodilatation, inhibition of growth, and suppression of the renin-angiotensin and endothelin-1 (ET-1) systems. Transition T2238~C leading to the loss of the Scal restriction site in the atria/ natriuretic peptide (ANP) precursor gene and potentialy to the translation of ANP with two additional arginines, has been suggested to be associated with salt-sensitive hypertension. The aim of our study was to investigate whether there is an association between the Scal ANP gene polymorphism and extent of coronary artery disease among patients with significant coronary artery stenosis confirmed by means of angiography (at least one coronary artery with >50% lumen narrowing). Methods The study was performed in 847 consecutive, Caucasian patients: 719 males and 128 females (mean age 47±11 years) with significant coronary artery stenosis confirmed by elective coronary angiography. Screening for the T2238~C substitution was performed by polymerase chain reaction of genomic DNA, followed by Scal digestion and agarose gel electrophoresis.
Congenital Heart Disease, Sep 23, 2019
Ebstein's anomaly (EA) is predominantly a right-sided cardiomyopathy characterized by ventricular... more Ebstein's anomaly (EA) is predominantly a right-sided cardiomyopathy characterized by ventricular displacement of the tricuspid valve (TV) into the right ventricle (RV), a dilated TV annulus, enlarged and dysmorphic TV leaflets, as well as atrialization of the RV with right atrial (RA) enlargement. 1,2 Atrial septal defects (ASDs) and patent foramen ovales (PFOs) are frequently present in this complex malformation, 3-5 and atrial arrhythmias are often seen as well. 2,4-7 In patients with acquired heart disease, atrial arrhythmias are strongly associated
Praxis, 2018
Zusammenfassung. Das Wissen um kardiovaskuläre Veränderungen im Alter und deren therapeutische Op... more Zusammenfassung. Das Wissen um kardiovaskuläre Veränderungen im Alter und deren therapeutische Optionen ist wichtig. Es kann zur Hypertrophie des linken Ventrikels, zur diastolischen Dysfunktion, Herzklappenveränderungen und pulmonaler Hypertonie kommen. Im Alter entwickeln Patienten häufig eine arterielle Hypertonie. Bei über 100-Jährigen sind valvuläre Veränderungen häufig (Aortenstenose und Mitralinsuffizienz). Das Risiko einer koronaren Herzkrankheit beträgt 35 % für Männer und 24 % für Frauen. Im Alter sind Sinusknotendysfunktion und Vorhofflimmern gehäuft. 25 % aller Schlaganfälle sind kardiale Embolien bei Vorhofflimmern. Kardiale Interventionen bei Betagten werden zunehmend häufiger durchgeführt und beinhalten koronare kathetertechnische Revaskularisationen oder Klappeneingriffe (perkutaner Aortenklappenersatz oder MitraClip). Die optimale Therapie im Alter beinhaltet neben kardiovaskulären Interventionen auch Medikamente und eine Lebensstilmodifikation und dient vor allem d...
European Heart Journal, Dec 8, 2007
Significant brady-and tachyarrhythmias may occur in active endurance athletes. It is controversia... more Significant brady-and tachyarrhythmias may occur in active endurance athletes. It is controversial whether these arrhythmias do persist after cessation of competitive endurance training. Methods and results Among all 134 former Swiss professional cyclists [hereafter, former athletes (FAs)] participating at least once in the professional bicycle race Tour de Suisse in 1955-1975, 62 (46%) were recruited for the study. The control group consisted of 62 male golfers matched for age, weight, hypertension, and cardiac medication. All participants were screened with history, clinical and echocardiographic examination, ECG, and 24 h ECG. The time for the last bicycle race of FAs was 38 + 6 years. The mean age at examination was 66 + 6 years in controls and 66 + 7 years in FAs (P ¼ 0.47). The percentage of study participants with .4 h current cardiovascular training per week was identical. QRS duration (102 + 20 vs. 95 + 13 ms, P ¼ 0.03) and corrected QTc interval (416 + 27 vs. 404 + 18, P ¼ 0.004) were longer in FAs. There was no significant difference in the number of isolated atrial or ventricular premature complexes, or supraventricular tachycardias in the 24 h ECG; however, ventricular tachycardias tended to occur more often in FAs than in controls (15 vs. 3%, P ¼ 0.05). The average heart rate was lower in FAs (66 + 9 vs. 70 + 8 b.p.m.) (P ¼ 0.004). Paroxysmal or persistent atrial fibrillation or flutter was reported more often in FAs (P ¼ 0.028). Sinus node disease (SND), defined as bradycardia of ,40 b.p.m. (10 vs. 2%), atrial flutter (6 vs. 0%), pacemaker for bradyarrhythmias (3 vs. 0%), and/or maximal RR interval of .2.5 s (6 vs. 0%), was more common in FA (16%) than in controls (2%, P ¼ 0.006). Observed survival of all FAs was not different from the expected. Conclusions Among FAs, SND occurred significantly more often compared with age-matched controls, and there is trend towards more frequent ventricular tachycardias. Further studies have to evaluate prevention of arrhythmias with extreme endurance training, the necessity of regular follow-up of heart rhythm, and management of arrhythmias in former competitive endurance athletes.
Background In professional cyclists, typical changes include reversible dilatation of atria and l... more Background In professional cyclists, typical changes include reversible dilatation of atria and left ventricle (LV), LV hypertrophy but normal diastolic function. Data on long-term outcome are limited. Methods Of all 134 former Swiss professional cyclists (PC) participating !1Â in the professional bicycle race Tour de Suisse from 1955 to 1975, 62 (42%) were recruited for a prospective case control study. The PC and a control group of 62 golfers (matched for age, gender, hypertension, present physical activity) were screened [clinical examination, history, echocardiography, measurement of proBNP (normal ,227 pg/mL)]. Results The interval since the last bicycle race as PC was 38 (15-49) years. Average age at exam was equal in controls and PC (66+6 vs 66+7 years; P ¼ 0.73). Percentage of participants undergoing .4 h of endurance training per week was identical (P ¼ 0.72). Total kilometers (km) on the bicycle were higher in PCs with 311,000 (60,000-975,000) than in controls (2500 [0-120,000]; P , 0.0001). PC had larger atrial volume indices (P ¼ 0.002) and tended to have higher LV muscle mass indices (P ¼ 0.07). Multiple regression analysis identified the total number of bicycle km as an independent factor for LV muscle mass. For left atrial size, heart rate at rest, age, years since the last bicycle race and the current hours of endurance training were identified as independent predictors. Long axis function of both ventricles (systolic velocities of mitral and tricuspid annulus) was decreased in PC (P 0.04). There were signs of diastolic dysfunction with lower annular E 0 and A 0 velocities. ProBNP levels were comparable in both groups (P ¼ 0.21). Conclusion Among former PC, there seems to be incomplete cardiac remodelling with differences in systolic and diastolic function between former PCs and controls in the long time follow-up. Former high level endurance training may have a persisting impact on cardiac size and function.
International Archives of Cardiovascular Diseases, 2019
Introduction: The number of very old patients with cardiac disease is increasing due to the longe... more Introduction: The number of very old patients with cardiac disease is increasing due to the longer life expectancy. There are few data on incidence and outcome of percutaneous cardiac procedures in patients ≥ 90-years-old (nonagenarians). Aim of the study: To retrospectively analyse the spectrum and outcome of percutaneous cardiac procedures in nonagenarians at our center. Methods: The database at our center from 2005-2014 was searched for all percutaneous cardiac procedures, including coronary angiography with or without percutaneous coronary intervention (PCI), transcatheter aortic valve replacement (TAVR), percutaneous mitral valve repair by MitraClip (PMVR), balloon valvuloplasty of the aortic valve (ABVP) and radiofrequency ablation (RFA). Age at procedure, clinical data, type and findings at procedure, in-hospital outcome, survival and predictors for survival were analyzed. Results: Among 25,860 percutaneous cardiac procedures, 130 (0.5%) were performed in 93 nonagenarians including 48 women (mean age 91.7 ± 1.8 years at first procedure). Procedures included 85 coronary angiographies (45 including PCI), 25 TAVR, 11 ABVP, 6 PMVR (including one combined with TAVR), and 3 RFA. Overall survival after first procedure (n = 93) was 95% after 1 month, 78% after 1 year and 64% after 2 years. Left ventricular ejection fraction (LVEF) < 40%, history of heart failure, dyspnea NYHA III or IV, mitral valve insufficiency and history of atrial fibrillation were statistically significantly associated with worse estimated survival (p < 0.05, log-rank). These factors showed a strong collinearity in a multivariate survival analysis. Conclusion: Percutaneous cardiac procedures in nonagenarians represent 0.5% of our procedures and have an acceptable survival rate considering the overall life expectancy in this age group. Further studies are required to delineate optimal patient selection in this age group for an acceptable ratio of procedural risk and benefit regarding survival and quality of life.
Swiss medical weekly, Jan 9, 2017
In 2013, a prospective registry for adults with congenital heart disease (CHD) was established in... more In 2013, a prospective registry for adults with congenital heart disease (CHD) was established in Switzerland, providing detailed data on disease characteristics and outcomes: Swiss Adult Congenital HEart disease Registry (SACHER). Its aim is to improve the knowledge base of outcomes in adults with CHD. The registry design and baseline patient characteristics are reported. All patients with structural congenital heart defects or hereditary aortopathies, followed-up at dedicated adult CHD clinics, are asked to participate in SACHER. Data of participants are pseudonymised and collected in an electronic, web-based, database (secuTrial®). Collected data include detailed diagnosis, type of repair procedures, previous complications and adverse outcomes during follow-up. From May 2014 to December 2016, 2836 patients (54% male, mean age 34 ± 14 years), with a wide variety of congenital heart lesions, have been enrolled into SACHER. Most prevalent were valve lesions (25%), followed by shunt ...
Circulation, 2006
E bstein's anomaly is a rare congenital heart disorder occurring in Ϸ1 per 200 000 live births an... more E bstein's anomaly is a rare congenital heart disorder occurring in Ϸ1 per 200 000 live births and accounting for Ͻ1% of all cases of congenital heart disease. 2-6 This anomaly was described by Wilhelm Ebstein in 1866 in a report titled, "Concerning a very rare case of insufficiency of the tricuspid valve caused by a congenital malformation." 7,8 The patient was a 19-year-old cyanotic man with dyspnea, palpitations, jugular venous distension, and cardiomegaly. 7,8 At autopsy, Ebstein described an enlarged and fenestrated anterior leaflet of the tricuspid valve. The posterior and septal leaflets were hypoplastic, thickened, and adherent to the right ventricle. There was also a thinned and dilated atrialized portion of the right ventricle, an enlarged right atrium, and a patent foramen ovale 9 (Figure 1). By 1950, only 3 cases of this anomaly had been published. 8,10,11
Circulation, 1996
Background Although Doppler echocardiography can be used to estimate left ventricular filling pre... more Background Although Doppler echocardiography can be used to estimate left ventricular filling pressures (LVFPs) in patients in sinus rhythm, its utility in atrial fibrillation is unknown. Methods and Results An initial training population of 30 patients (17 men, 13 women; mean age, 69±9 years; range, 48 to 87 years) was studied. Measurements of LVFP were obtained simultaneously with pulsed Doppler recordings of mitral and pulmonary venous flow velocities and color M-mode recording of the flow propagation velocity of the mitral inflow. Measurements were averaged over 10 cardiac cycles. In addition, left atrial volume was derived from the apical four-chamber view. Significant relations were observed between LVFP and several parameters derived from the transmitral and pulmonary venous velocity and left atrial volume. The best relations were observed with the peak acceleration (PkAcc) of the mitral velocity ( r =.84), isovolumic relaxation time (IVRT) ( r =−.76), mean early (E) velocity...
CHEST Journal, 1994
Exercise-induced anaphylaxis (EIA) is a rare form of physical allergy. Although histamine release... more Exercise-induced anaphylaxis (EIA) is a rare form of physical allergy. Although histamine release is a feature of EIA, and histamine provocation of coronary spasm has been described, serious cardiac arrhythmias in EIA have not been reported. Exercise-induced anaphylaxis was diagnosed in a survivor of out-of-hospital cardiac arrest due to ventricular fibrillation after ECG signs of coronary spasm. Coronary artery disease was excluded. Ergonovine provocation induced coronary spasm in this patient. This is, to the authors&#39; knowledge, the first description of ventricular fibrillation in EIA, possibly due to coronary spasm.
Cardiology, 2000
Arrhythmogenic right ventricular cardiomyopathy is a rare heart muscle disease characterized by r... more Arrhythmogenic right ventricular cardiomyopathy is a rare heart muscle disease characterized by right and often left ventricular myocardial atrophy and fibrofatty replacement. Heart failure, arrhythmias and sudden death are characteristic complications. We observed a female in whom arrhythmogenic right ventricular cardiomyopathy was diagnosed due to presyncopes and dyspnea on exertion. A left ventricular thrombus was found echocardiographically, which disappeared with oral anticoagulation. Subsequently, however, extensive thrombus formation in the dilated akinetic right ventricle occurred which was resistant to combined treatment with heparin and oral anticoagulation. Thrombophilia screening showed a mutant prothrombin 20210A allele which is an inherited coagulopathy associated with increased plasma levels of prothrombin and increased risks of mainly venous thrombosis. The patient developed endstage biventricular heart failure and underwent heart transplantation within 3 months after thrombus formation in the right ventricle was diagnosed. In the explanted heart, the thrombus in the right ventricle was impressively large and calcified. In patients with unusual thrombus formation in the heart, coagulopathy may be associated and should be excluded.
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Papers by Christine Attenhofer