IntroductionLeft ventricular (LV) hypertrophy is the main feature of cardiac involvement in Anderson-Fabry disease (FD), but the right ventricle (RV) is also frequently affected. Previous studies... Show moreIntroductionLeft ventricular (LV) hypertrophy is the main feature of cardiac involvement in Anderson-Fabry disease (FD), but the right ventricle (RV) is also frequently affected. Previous studies failed to demonstrate an independent association between conventional parameters of RV performance and outcomes in FD. Nevertheless, if RV free wall strain (RV-FWS), assessed by 2D speckle tracking analysis, may provide a better prognostication is currently unknown.MethodsWe retrospectively evaluated the association between RV-FWS and the occurrence of cardiovascular events in a cohort of 56 patients with FD. The study endpoint comprises cardiovascular mortality, severe heart failure symptoms, new-onset atrial fibrillation and major arrhythmias requiring device implantation.ResultsReduced RV-FWS, defined by values lower than 23%, was found in 25 (45%) patients. During a median follow-up of 47 months, 16 (29%) patients met the study endpoint. A ROC-curve analysis confirmed the threshold of reduced RV-FWS (<23%) as the best cut-off for predicting cardiovascular events, but with a lower power compared to left-sided parameters. On univariable Cox regression analysis, RV-FWS, expressed as continuous variable, was significantly associated with the study endpoint (HR: 0.795, 95% CI: 0.710–0.889, p < 0.001). However, RV-FWS did not retain a significant association with outcomes, after adjustment for LV global longitudinal strain or indexed left atrial volume (p = 0.340 and p = 0.289 respectively).ConclusionsRV-FWS was not independently associated with the occurrence of cardiovascular events in FD, confirming previous observations that prognosis is mainly driven by the severity of LV cardiomyopathy. Show less
In hospitalized COVID-19 patients, myocardial injury and echocardiographic abnormalities have been described. The present study investigates cardiac function in COVID-19 patients 6 weeks post... Show moreIn hospitalized COVID-19 patients, myocardial injury and echocardiographic abnormalities have been described. The present study investigates cardiac function in COVID-19 patients 6 weeks post-discharge and evaluates its relation to New York Heart Association (NYHA) class. Furthermore cardiac function post-discharge between the first and second wave COVID-19 patients was compared. We evaluated 146 patients at the outpatient clinic of the Leiden University Medical Centre. NYHA class of II or higher was reported by 53% of patients. Transthoracic echocardiography was used to assess cardiac function. Overall, in 27% of patients reduced left ventricular (LV) ejection fraction was observed and in 29% of patients LV global longitudinal strain was impaired (> - 16%). However no differences were observed in these parameters reflecting LV function between the first and second wave patients. Right ventricular (RV) dysfunction as assessed by tricuspid annular systolic planar excursion (< 17 mm) was present in 14% of patients, this was also not different between the first and second wave patients (15% vs. 12%; p = 0.63); similar results were found for RV fraction area change and RV strain. Reduced LV and RV function were not associated with NYHA class. In COVID-19 patients at 6 weeks post-discharge, mild abnormalities in cardiac function were found. However these were not related to NYHA class and there was no difference in cardiac function between the first and second wave patients. Long term symptoms post-COVID might therefore not be explained by mildly abnormal cardiac function. Show less
Surgical repair of Tetralogy of Fallot (ToF) is usually performed in the first months of life with low early postoperative mortality. During long-term follow-up, however, both right (RV) and left... Show moreSurgical repair of Tetralogy of Fallot (ToF) is usually performed in the first months of life with low early postoperative mortality. During long-term follow-up, however, both right (RV) and left ventricular (LV) performances may deteriorate. Tissue Doppler imaging (TDI) and speckle tracking echocardiography (ST) can unmask a diminished RV and LV performance. The objective of the current study was to assess the cardiac performance before and shortly after corrective surgery in ToF patients using conventional, TDI and ST echocardiographic techniques. Thirty-six ToF patients after surgery were included. Transthoracic echocardiography including TDI and ST techniques was performed preoperatively and at hospital discharge after surgery (10 days to 4 weeks after surgery). Median age at surgery was 7.5 months [5.5-10.9]. Regarding the LV systolic function there was a significant decrease in interventricular septum (IVS) S ' at discharge as compared to preoperatively (pre IVS S ' = 5.4 +/- 1.4; post IVS S ' = 3.9 +/- 1.2; p < 0.001) and in global longitudinal peak strain (GLS) (pre = - 18.3 +/- 3.4; post = - 14.2 +/- 4.1; p = 0.003); but not in the fractional shortening (FS). Both conventional and TDI parameters showed a decrease in diastolic function at discharge. Tricuspid Annular Plane Systolic Excursion and RV S ' were significantly lower before discharge. When assessing the RV diastolic performance, only the TDI demonstrated a RV impairment. There was a negative correlation between age at surgery and postoperative LV GLS (R = - 0.41, p = 0.031). There seems to be an impairment in left and right ventricle performance at discharge after ToF corrective surgery compared to preoperatively. This is better determined with TDI and ST strain imaging than with conventional echocardiography. Show less
Chronic kidney disease (CKD) is a worldwide growing epidemic associated with an increased risk of cardiovascular morbidity and mortality. Heart failure is particularly frequent among patients... Show moreChronic kidney disease (CKD) is a worldwide growing epidemic associated with an increased risk of cardiovascular morbidity and mortality. Heart failure is particularly frequent among patients with CKD. Pressure and volume overload and non-hemodynamic factors associated with CKD induce left ventricular (LV) hypertrophy, reduce capillary density and increase myocardial fibrosis that lead to LV diastolic and systolic dysfunction. These processes have been proposed as important determinants of increased mortality in this population. The aim of this thesis was to evaluate cardiac mechanics using two-dimensional (speckle tracking) echocardiography and coronary computed tomography angiography in patients with CKD to define the prevalence and prognostic implications of cardiovascular diseases. In pre-dialysis and dialysis patients with preserved LV ejection fraction (LVEF), assessment of left ventricular global longitudinal strain (LV GLS) helps to identify patients with higher rates of heart failure hospitalization and all-cause mortality. When LVEF has already declined, patients within the lowest quartile of LV GLS showed worse prognosis compared to the other groups. The burden of cardiovascular diseases in patients with CKD is high and thorough assessment of cardiac mechanics with (advanced) echocardiographic techniques and coronary CTA helps in identifying patients with worse prognosis, who would potentially benefit of early treatment. Show less
Part I focuses on the potential role of 3-dimensional echocardiography. At first a clinical risk score model for prediction of outcome in patients undergoing TAVI is presented (Chapter 2). Second... Show morePart I focuses on the potential role of 3-dimensional echocardiography. At first a clinical risk score model for prediction of outcome in patients undergoing TAVI is presented (Chapter 2). Second the role of 3D-echocardiography is explored in depth in patients with mitral valve disease. Different non-invasive cardiac imaging modalities to evaluate mitral valve function and anatomy are described and the use of 3D-echocardiography is put into perspective (Chapter 3). We then evaluate the role of the latter to gain insights in patients with functional mitral regurgitation (Chapter 4), to select patients and guide procedures regarding percutaneous mitral valve repair using Mitra-Clip (Chapter 5) and to assess the effect of Mitra-Clip on the mitral valve (Chapter 6). In Part II we further elaborate the potential role of risk stratification by ECG and myocardial deformation imaging (strain), as surrogate markers of fibrosis. Surface ECG fragmentation in primary HCM is first evaluated (Chapter 7). The important future role of fibrosis imaging in valvular heart disease patients is then reviewed (Chapter 8). Finally the role of left atrial structure and function is evaluated in patients with mitral regurgitation (Chapter 9) and primary HCM (Chapter 10, 11). Show less
The presence of a decreased left ventricular (LV) function after myocardial infarction has demonstrated to be of considerable clinical importance. In this thesis, the role of 2D echocardiography to... Show moreThe presence of a decreased left ventricular (LV) function after myocardial infarction has demonstrated to be of considerable clinical importance. In this thesis, the role of 2D echocardiography to evaluate LV function in ischemic heart disease was investigated. In the first part of the thesis, recently introduced echocardiographic parameters to describe LV function were studied and their importance for prognosis after myocardial infarction was evaluated. In addition, in the second part of the thesis, the role for echocardiography in the decision making around advanced treatment options in heart failure such as cardiac resynchronization therapy (CRT) and cardiac surgery was explored. Show less
