Axel Rentzsch

Noninvasive assessment of liver fibrosis in patients with Fontan circulation using transient elastography and biochemical fibrosis markers

OBJECTIVE Studies have shown that patients with failing Fontan circulation may develop liver cirrhosis with its sequelae. Therefore, early detection of fibrosis is essential to identify patients at risk. Transient elastography has been evaluated for noninvasive staging of liver fibrosis in a multitude of studies. METHODS A total of 39 patients who underwent the Fontan procedure were included in the study. All patients underwent an abdominal ultrasound, transient elastography, and detailed laboratory testing. Conventional echocardiography assessment of inflow and outflow and ventricular function were performed. RESULTS Significant signs of liver fibrosis were found in 36 of 39 children according to the elastography method and in 28 of 39 children according to the measured biochemical fibrosis markers. The Spearman correlation coefficient between the liver stiffness measured with transient elastography and the time interval since the Fontan operation was highly significant (0.514, P = 0.001). CONCLUSION The present study shows that patients who undergo the Fontan procedure are at increased risk of developing liver fibrosis and liver cirrhosis. The risk increases with the age of the patient and the time interval since the Fontan procedure. The noninvasive measurement of liver fibrosis using transient elastography and fibrosis marker scores can be a useful tool to identify patients at risk and for noninvasive surveillance.

Assessment of interventricular and right- intraventricular dyssynchrony in patients with surgically repaired tetralogy of Fallot by two-dimensional speckle tracking

AIMS We aimed to assess interventricular and right-intraventricular dyssynchrony in patients after tetralogy of Fallot (TOF) repair by two-dimensional (2D) speckle tracking and to identify factors associated with dyssynchrony. METHODS AND RESULTS Forty-two patients after TOF repair with a mean age of 19.8 years and 42 age-matched healthy controls were studied. Longitudinal myocardial deformation (strain) and time-to-peak intervals were assessed by 2D speckle tracking and tissue Doppler imaging (TDI) in an apical four-chamber view. Dyssynchrony was defined as delay above 3 standard deviations of mean values in the control group. Magnetic resonance imaging (MRI) was performed for evaluation of ventricular function. Using 2D speckle tracking, 22 patients (52%) showed interventricular dyssynchrony and 16 (38%) had right-intraventricular dyssynchrony. The interventricular delay correlated significantly with right ventricular (RV) strain (r = 0.687, P < 0.001), RV systolic pressure (r = 0.535, P = 0.001), QRS duration (r = 0.466, P = 0.002), RV end-diastolic (r = 0.377, P = 0.018), and RV end-systolic volumes (r = 0.452, P = 0.004) as well as RV ejection fraction (r = -0.378, P = 0.018). Similarly, the right-intraventricular delay correlated significantly with RV strain (r = 0.534, P < 0.001), QRS duration (r = 0.428, P = 0.005), RV end-systolic volume (r = 0.34, P = 0.038), and RV systolic pressure (r = 0.413, P = 0.015). In multivariate regression analysis, reduced RV strain and prolonged QRS duration remained the main determinant factors predicting dyssynchrony. Moreover, 2D speckle tracking and TDI showed a significant correlation in the assessment of the interventricular (r = 0.738, P < 0.001) and right-intraventricular delay (r = 0.747, P < 0.001). CONCLUSION Interventricular and right-intraventricular dyssynchrony are detectable in patients after TOF repair by 2D speckle tracking. Reduced RV myocardial deformation and QRS prolongation are the main factors associated with the observed dyssynchrony.

Right and Left Ventricular Function and Mass in Male Elite Master Athletes: A Controlled Contrast-Enhanced Cardiovascular Magnetic Resonance Study.

Background— It is under debate whether the cumulative effects of intensive endurance exercise induce chronic cardiac damage, mainly involving the right heart. The aim of this study was to examine the cardiac structure and function in long-term elite master endurance athletes with special focus on the right ventricle by contrast-enhanced cardiovascular magnetic resonance. Methods and Results— Thirty-three healthy white competitive elite male master endurance athletes (age range, 30–60 years) with a training history of 29±8 years, and 33 white control subjects pair-matched for age, height, and weight underwent cardiopulmonary exercise testing, echocardiography including tissue-Doppler imaging and speckle tracking, and cardiovascular magnetic resonance. Indexed left ventricular mass and right ventricular mass (left ventricular mass/body surface area, 96±13 and 62±10 g/m2; P<0.001; right ventricular mass/body surface area, 36±7 and 24±5 g/m2; P<0.001) and indexed left ventricular end-diastolic volume and right ventricular end-diastolic volume (left ventricular end-diastolic volume/body surface area, 104±13 and 69±18 mL/m2; P<0.001; right ventricular end-diastolic volume/body surface area, 110±22 and 66±16 mL/m2; P<0.001) were significantly increased in athletes in comparison with control subjects. Right ventricular ejection fraction did not differ between athletes and control subjects (52±8 and 54±6%; P=0.26). Pathological late enhancement was detected in 1 athlete. No correlations were found for left ventricular and right ventricular volumes and ejection fraction with N-terminal pro-brain natriuretic peptide, and high-sensitive troponin was negative in all subjects. Conclusions— Based on our results, chronic right ventricular damage in elite endurance master athletes with lifelong high training volumes seems to be unlikely. Thus, the hypothesis of an exercise-induced arrhythmogenic right ventricular cardiomyopathy has to be questioned.

Assessment of left ventricular dyssynchrony and function using real-time 3-dimensional echocardiography in patients with congenital right heart disease.

BACKGROUND Patients after repair of congenital right heart disease (CRHD) may exhibit left ventricular (LV) dyssynchrony (LVD). However, the diagnosis of LVD is difficult and its reliability limited because current methods do not assess LVD of the whole LV simultaneously. The aim of the study was to assess LVD according to a novel global systolic dyssynchrony index (SDI) derived from real-time 3-dimensional echocardiography in patients after repaired CRHD. METHODS Two-dimensional echocardiography and real-time 3-dimensional echocardiography were performed in 30 patients after CRHD repair and in 30 matched healthy controls. Real-time 3-dimensional echocardiography data sets provided time-volume curves, and 2 global SDIs were derived from the dispersion of time to reach minimal systolic volume according to a 16- or 17-LV segment model. RESULTS Both SDIs were significantly elevated in the patient as compared with the control group (P < .001). A cutoff value for both SDIs was calculated and LVD defined as one of the SDIs exceeding cutoff. Left ventricular dyssynchrony was present in 5 (100%) of 5 patients with a LV ejection fraction (EF) <50% and 13 (52%) of 25 patients with preserved LVEF, thus being diagnosed in a total of 18 (60%) of 30 patients. Moreover, patients with LVD showed a significantly higher degree of pulmonary regurgitation (P = .01) with elevated right ventricular volumes and altered septal motion. Stepwise multivariate analysis identified LVEF (P = .005) and the degree of pulmonary regurgitation (P = .02) as independent predictors of LVD. CONCLUSIONS Left ventricular dyssynchrony can be detected in about 60% of patients after CRHD repair and is mainly due to significant pulmonary regurgitation resulting in an altered septal motion and systolic LV function.

Assessment of inter-atrial, inter-ventricular, and atrio-ventricular interactions in tetralogy of Fallot patients after surgical correction. Insights from two-dimensional speckle tracking and three-dimensional echocardiography

BACKGROUND We aimed to assess biatrial size and function, interactions on atrial and ventricular levels, and atrio-ventricular coupling in patients after tetralogy of Fallot repair. METHODS A total of 34 patients with a mean age of 20.9±9 years, and 35 healthy controls, underwent two-dimensional speckle tracking echocardiography for ventricular and atrial strain measurements and real-time three-dimensional echocardiography to assess ventricular and atrial volumes. RESULTS When compared with controls, tetralogy of Fallot patients had significantly reduced right atrial peak atrial longitudinal strain (p<0.01), right atrial peak atrial contraction strain (p<0.01), right atrial ejection fraction (p<0.01), left atrial peak atrial longitudinal strain (p<0.01), left atrial peak atrial contraction strain (p<0.05), and left atrial ejection fraction (p<0.01). In the tetralogy of Fallot group, left ventricular ejection fraction was negatively related to the right ventricular end-systolic volume normalised to body surface area (r=-0.62, p<0.01). An association was found in patients between the right atrial peak longitudinal strain and mean right ventricular strain (r=0.64, p<0.01). In patients, the left atrial peak longitudinal strain correlated negatively with right atrial end-diastolic volume normalised to body surface area (r=-0.67, p<0.01), whereas the left atrial ejection fraction correlated weakly with left ventricular ejection fraction (r=0.41, p<0.05). CONCLUSIONS In asymptomatic tetralogy of Fallot patients, biatrial dysfunction exists and can be quantified via two-dimensional speckle tracking echocardiography as well as real-time three-dimensional echocardiography. Different forms of interactions on atrial and ventricular levels are evident among such cohorts.

Right and Left Ventricular Function and Mass in Male Elite Master Athletes: A Controlled Contrast Enhanced CMR Study

Background— It is under debate whether the cumulative effects of intensive endurance exercise induce chronic cardiac damage, mainly involving the right heart. The aim of this study was to examine the cardiac structure and function in long-term elite master endurance athletes with special focus on the right ventricle by contrast-enhanced cardiovascular magnetic resonance. Methods and Results— Thirty-three healthy white competitive elite male master endurance athletes (age range, 30–60 years) with a training history of 29±8 years, and 33 white control subjects pair-matched for age, height, and weight underwent cardiopulmonary exercise testing, echocardiography including tissue-Doppler imaging and speckle tracking, and cardiovascular magnetic resonance. Indexed left ventricular mass and right ventricular mass (left ventricular mass/body surface area, 96±13 and 62±10 g/m2; P<0.001; right ventricular mass/body surface area, 36±7 and 24±5 g/m2; P<0.001) and indexed left ventricular end-diastolic volume and right ventricular end-diastolic volume (left ventricular end-diastolic volume/body surface area, 104±13 and 69±18 mL/m2; P<0.001; right ventricular end-diastolic volume/body surface area, 110±22 and 66±16 mL/m2; P<0.001) were significantly increased in athletes in comparison with control subjects. Right ventricular ejection fraction did not differ between athletes and control subjects (52±8 and 54±6%; P=0.26). Pathological late enhancement was detected in 1 athlete. No correlations were found for left ventricular and right ventricular volumes and ejection fraction with N-terminal pro-brain natriuretic peptide, and high-sensitive troponin was negative in all subjects. Conclusions— Based on our results, chronic right ventricular damage in elite endurance master athletes with lifelong high training volumes seems to be unlikely. Thus, the hypothesis of an exercise-induced arrhythmogenic right ventricular cardiomyopathy has to be questioned.

Left ventricular systolic dysfunction in asymptomatic Marfan syndrome patients is related to the severity of gene mutation: insights from the novel three dimensional speckle tracking echocardiography.

Background In asymptomatic Marfan syndrome (MFS) patients we evaluated the relationship between the types of fibrillin-1 (FBN1) gene mutation and possible altered left ventricular (LV) function as assessed by three-dimensional speckle tracking echocardiography (3D-STE). Methods and Results Forty-five MFS patients (mean age 24±15 years) and 40 age-matched healthy controls were studied. Genetic evaluation for the FBN1 gene was carried on 32 MFS patients. Gene mutation (n = 15, 47%) was classified as mild when the mutation resulted in nearly normally functioning protein, while mutations resulting in abnormally function protein were considered to be severe (n = 17, 53%). All patients and controls underwent 3D-STE for evaluation of LV function by an echocardiographer blinded to the results of the genetic testing. Compared to controls, MFS patients had significantly lower 3D-STE derived LV ejection fraction (EF, 57.43±7.51 vs. 62.69±4.76%, p = 0.0001), global LV longitudinal strain (LS, 14.85±2.89 vs. 17.90±2.01%, p = 0.0001), global LV circumferential strain (CS, 13.93±2.81 vs. 16.82±2.17%, p = 0.0001) and global LV area strain (AS, 25.76±4.43 vs. 30.51±2.61%, p = 0.0001). Apart from the global LV LS all these parameters were significantly lower in patients with severe gene mutation than in those with mild mutation (p<0.05). In the multivariate linear regression analysis only the type of mutation had a significant influence on the 3D-STE derived LVEF (p = 0.017), global CS (p = 0.005) and global AS (p = 0.03). Conclusions In asymptomatic MFS patients latent LV dysfunction can be detected using 3D STE. The LV dysfunction is mainly related to the severity of gene mutation, suggesting possible primary cardiomyopathy in MFS patients.

Right and Left Ventricular Function and Mass in Male Elite Master Athletes

Background— It is under debate whether the cumulative effects of intensive endurance exercise induce chronic cardiac damage, mainly involving the right heart. The aim of this study was to examine the cardiac structure and function in long-term elite master endurance athletes with special focus on the right ventricle by contrast-enhanced cardiovascular magnetic resonance. Methods and Results— Thirty-three healthy white competitive elite male master endurance athletes (age range, 30–60 years) with a training history of 29±8 years, and 33 white control subjects pair-matched for age, height, and weight underwent cardiopulmonary exercise testing, echocardiography including tissue-Doppler imaging and speckle tracking, and cardiovascular magnetic resonance. Indexed left ventricular mass and right ventricular mass (left ventricular mass/body surface area, 96±13 and 62±10 g/m2; P<0.001; right ventricular mass/body surface area, 36±7 and 24±5 g/m2; P<0.001) and indexed left ventricular end-diastolic volume and right ventricular end-diastolic volume (left ventricular end-diastolic volume/body surface area, 104±13 and 69±18 mL/m2; P<0.001; right ventricular end-diastolic volume/body surface area, 110±22 and 66±16 mL/m2; P<0.001) were significantly increased in athletes in comparison with control subjects. Right ventricular ejection fraction did not differ between athletes and control subjects (52±8 and 54±6%; P=0.26). Pathological late enhancement was detected in 1 athlete. No correlations were found for left ventricular and right ventricular volumes and ejection fraction with N-terminal pro-brain natriuretic peptide, and high-sensitive troponin was negative in all subjects. Conclusions— Based on our results, chronic right ventricular damage in elite endurance master athletes with lifelong high training volumes seems to be unlikely. Thus, the hypothesis of an exercise-induced arrhythmogenic right ventricular cardiomyopathy has to be questioned.

Right and Left Ventricular Function and Mass in Male Elite Master AthletesClinical Perspective: A Controlled Contrast-Enhanced Cardiovascular Magnetic Resonance Study

Background— It is under debate whether the cumulative effects of intensive endurance exercise induce chronic cardiac damage, mainly involving the right heart. The aim of this study was to examine the cardiac structure and function in long-term elite master endurance athletes with special focus on the right ventricle by contrast-enhanced cardiovascular magnetic resonance. Methods and Results— Thirty-three healthy white competitive elite male master endurance athletes (age range, 30–60 years) with a training history of 29±8 years, and 33 white control subjects pair-matched for age, height, and weight underwent cardiopulmonary exercise testing, echocardiography including tissue-Doppler imaging and speckle tracking, and cardiovascular magnetic resonance. Indexed left ventricular mass and right ventricular mass (left ventricular mass/body surface area, 96±13 and 62±10 g/m2; P<0.001; right ventricular mass/body surface area, 36±7 and 24±5 g/m2; P<0.001) and indexed left ventricular end-diastolic volume and right ventricular end-diastolic volume (left ventricular end-diastolic volume/body surface area, 104±13 and 69±18 mL/m2; P<0.001; right ventricular end-diastolic volume/body surface area, 110±22 and 66±16 mL/m2; P<0.001) were significantly increased in athletes in comparison with control subjects. Right ventricular ejection fraction did not differ between athletes and control subjects (52±8 and 54±6%; P=0.26). Pathological late enhancement was detected in 1 athlete. No correlations were found for left ventricular and right ventricular volumes and ejection fraction with N-terminal pro-brain natriuretic peptide, and high-sensitive troponin was negative in all subjects. Conclusions— Based on our results, chronic right ventricular damage in elite endurance master athletes with lifelong high training volumes seems to be unlikely. Thus, the hypothesis of an exercise-induced arrhythmogenic right ventricular cardiomyopathy has to be questioned.

Erste Ergebnisse aus der Multizentrischen Resynchronisationstherapie Studie bei Angeborenen Herzfehlern (CARE-CHD-Studie) im Rahmen des Kompetenznetzes für Angeborene Herzfehler (KNAHF)

Die Wirksamkeit der CRT bei AHF ist unklar. Fast alle publizierten Studien basieren auf retrospektiven Datenanalysen. Die erste multizentrische Studie wurde nach Einschluss von 55 Patienten beendet. Die ersten mittelfristigen Ergebnisse nach 12 Monate unter CRT werden hier prasentiert. Methode: 55 Patienten zwischen 2 und 60J. mit AHF aus 15 Zentren in Deutschland und Osterreich wurden erfolgreich eingeschlossen. Die Einschlusskriterien waren: AHF mit einer Insuffizienz der Systemkammer nach optimaler medikamentoser Therapie, EF der Systemkammer 120 ms und NYHA-II-IV. Die primaren Zielparameter waren eine Besserung der EF und Abnahme der QRS-Dauer. Sekundare Parameter waren NT-pro-BNP, Echo-Parameter, Belastbarkeit, SMWT, Lebensqualitat, Hospitalisation, Transplantation, Tod. Ergebnisse: Von den uns vorliegenden abgeschlossenen Visiten bis 12 Monate zeigte sich eine signifikante Abnahme des QRS-Komplexes (167,2 ± 30,5 vs. 154,2 ± 23,3 milliseconds, p= 0,024), eine Besserung der EF der Systemkammer (27,1 ± 10,6 vs. 33,7 ± 11%, p= 0,016), des 6-Minuten-Gehtests (457,8 ± 140,1 vs. 536,5 ± 144,4 m, p= 0,005), Systemkammer VTI (17,6 ± 4,7 vs. 22,7 ± 8,9 cm, p= 0,001), wobei der NT-pro-BNP statistisch unverandert blieb. 3 Patienten verstarben wahrend dieser Beobachtungszeit und bei 8 Patienten wurde die Studie aus unterschiedlichen Grunden abgebrochen. Die CRT scheint auch bei einer heterogenen Gruppe von Patienten mit AHF die systolische Funktion, Belastbarkeit und die elektromechanische Kopplung signifikant zu verbessern. Die Kriterien der Response fur die CRT sollen noch analysiert werden.