Adult patients with congenital heart disease and a systemic right ventricle are prone to develop late complications such as rhythm and conduction disorders and heart failure. This thesis aims to... Show moreAdult patients with congenital heart disease and a systemic right ventricle are prone to develop late complications such as rhythm and conduction disorders and heart failure. This thesis aims to clarify the pathophysiology of this condition and to explore diagnostic and treatment options. Chapter 2 describes asymmetry and heterogeneity in cardiac autonomic innervation. Chapters 3 and 4 describe ambulatory ECG-derived measures of cardiac autonomic function in patients with a systemic right ventricle, and show that these measures are related to outcomes such as supraventricular arrhythmias. In chapter 5, echocardiographic measures of systemic right ventricular function are compared with cardiac MRI. Chapter 6 describes the clinical course of patients long after atrial switch correction. Chapter 7 describes the first results of treatment of systemic right ventricular failure with sacubitril/valsartan: a.o. a small but significant improvement in systemic right ventricular function was seen. Chapter 8 describes the experience of the application of eHealth smart technology in the titration of sacubitril/valsartan in the same cohort. Chapter 9 reports the first two cases of implantation of a ventricular assist device in patients with systemic right ventricular failure. This thesis may form the base for ongoing research to improve outcome in this vulnerable group. Show less
This thesis describes the additional value of advanced echocardiographic techniques in patients with primary cardiac disease, resulting in mitral regurgitation, and secondary cardiac disease caused... Show moreThis thesis describes the additional value of advanced echocardiographic techniques in patients with primary cardiac disease, resulting in mitral regurgitation, and secondary cardiac disease caused by a systemic disease. In this thesis we focussed on patients with mitral regurgitation, specifically primary MR and functional MR (FMR), and patients with Systemic Sclerosis (SSc). The general introduction provides a background of MR and cardiac involvement in SSc and how conventional 2-dimensional (2D) echocardiography is currently used. The potential role of 2D speckle tracking echocardiography and 3-dimensional (3D) echocardiography together with customized software to create 4-dimensional (4D) mitral valve models is introduced. This thesis aims to provide new insights in diagnosis, disease progression and risk stratification in patients with MR and patients with SSc with the implementation of advanced echocardiographic techniques. Show less
Although COPD was originally thought to merely affect the airways and lungs, the attention of research has been shifted towards the high prevalence of cardiovascular disease in patients with COPD.... Show moreAlthough COPD was originally thought to merely affect the airways and lungs, the attention of research has been shifted towards the high prevalence of cardiovascular disease in patients with COPD. Up to one third of deaths in patients with COPD can be attributed to a cardiovascular cause. In particular, a strong association between COPD and acute myocardial infarction (AMI) has been emphasized and the need for adequate risk stratification in this population has been recognized. Identification of patients at risk of adverse events after AMI is frequently performed with cardiac imaging. Echocardiography permits early assessment of left- and right ventricular size and function, as surrogates of cardiac damage in the acute phase. Emerging advanced echocardiographic techniques such as speckle tracking strain imaging enable characterization of myocardial mechanics, which have been associated with prognosis. Assessment of left- and right ventricular longitudinal strain in patient with COPD might provide better risk stratification as compared to conventional echocardiographic parameters. COPD is also a known risk factor for atrial fibrillation. Structural atrial remodeling, particularly of the right atrium, is suggested to be an important pathophysiologic substrate. Echocardiography may provide an additional tool in characterization of atrial structure and function, leading to targeted treatments. Show less
With the rising global health burden of aortic valve diseas and growing awareness of the consequences of severe AS, more patients are expected to be referred for aortic valve replacement.... Show moreWith the rising global health burden of aortic valve diseas and growing awareness of the consequences of severe AS, more patients are expected to be referred for aortic valve replacement. Multimodality imaging remains of paramount importance for proper patient selection, determining optimal timing of intervention and in the evaluation of therapy. For risk stratification and defining optimal timing of intervention, conventional and advanced echocardiography and computed tomography are crucial. Conventional echocardiography can be used to assess extra-aortic valvular cardiac damage in patients with severe AS. On top of conventional echocardiography, advanced echocardiography can provide additional insights. Left ventricular GLS by speckle tracking echocardiography has been suggested as a more sensitive marker of LV systolic dysfunction. LV GLS may be of help to define more optimal timing of intervention in asymptomatic patients with severe AS. Also, LV GLS may be of incremental value on top of conventional echocardiographic parameters for the assessment of cardiac injury. For the evaluation of prothesis function and durability after aortic valve implantation and detection of possible (late) complications, echocardiography is the mainstay imaging modality. Also, both conventional and advanced echocardiography can provide additional information on the effects of therapy on LV function and remodelling. Show less
Tricuspid regurgitation is a disorder in which the tricuspid valve does not close properly, causing backward flow of blood. It is a heterogeneous disease which can be classified based on the... Show moreTricuspid regurgitation is a disorder in which the tricuspid valve does not close properly, causing backward flow of blood. It is a heterogeneous disease which can be classified based on the underlying mechanism. The large majority of tricuspid regurgitation is secondary to conditions that cause tricuspid annulus dilation or leaflet tethering due to right ventricular or right atrial dilation. Part I focusses on the relationship between secondary tricuspid regurgitation and the right ventricle. Right ventricular dysfunction was demonstrated to be an important prognostic parameter in patients with secondary tricuspid regurgitation. A new staging system, incorporating right ventricular dysfunction and symptoms of right heart failure, may be valuable for risk stratification in patients with secondary tricuspid regurgitation. Patients who received tricuspid valve annuloplasty during left-sided valve surgery developed less right ventricular dilation and had less severe tricuspid regurgitation at long-term follow-up.Part II focusses on tricuspid regurgitation in specific patient populations: men and women, patients with atrial fibrillation and patients with obesity. An etiology-specific approach to detection and management of significant tricuspid regurgitation may be the most effective way to improve prognosis in all patients. Treatment of patients with tricuspid regurgitation remains challenging, but is developing rapidly with ongoing research. Show less
Severe secondary MR is known to be associated with a worse prognosis, whilst the effect of reducing MR on prognosis has still been unclear. The question remains what influences the prognosis of... Show moreSevere secondary MR is known to be associated with a worse prognosis, whilst the effect of reducing MR on prognosis has still been unclear. The question remains what influences the prognosis of these patients: is it the LV or the valve (i.e. the MR)? Characterization and risk-stratification of patients with secondary MR therefore remains challenging. Until recently the decision to intervene for secondary MR was based also on the LVEF. However, LVEF is subject to many limitations. Advanced echocardiography, such as speckle tracking echocardiography, from which LVGLS could be derived has shown to be of much more diagnostic and prognostic value in various valvular heart disease and is currently being implemented more in valvular heart disease guidelines. This supports the fact that LVGLS can detect LV dysfunction in an earlier stage and therefore guide physicians to refer patients for intervention before it is too late. Also the mitral valve geometry has an important role in the technical feasibility of intervention, but also could elaborate on which specific transcatheter intervention is more appropriate according to their targets (i.e. leaflets, annulus or sub apparatus). Additionally, multimodality imaging remains key in characterization and quantification of secondary MR and may help further risk-stratification. Show less
The use of LVEF in quantifying LV systolic dysfunction, is subject to a number of limitations, many of which can partially be overcome by speckle tracking echocardiography. The best-validated... Show moreThe use of LVEF in quantifying LV systolic dysfunction, is subject to a number of limitations, many of which can partially be overcome by speckle tracking echocardiography. The best-validated parameter of LV systolic function is LV GLS, for which ample evidence has accumulated to support its use in diagnosis and risk-stratification of various cardiac diseases affecting the LV. Speckle tracking echocardiography is currently transitioning from an experimental to a routine technique, and is recommended in several clinical scenarios, particularly in those where regular surveillance of LV systolic function has therapeutic implications.Furthermore, echocardiographic evaluation of patients with ischemic heart disease remains pivotal in both the acute setting of STEMI patients and at follow up. New advances in echocardiographic software enables for more comprehensive analysis of the LV. Application of this new data in addition to conventional parameters could provide more and better prognostic information. Using this strategy, patients after STEMI could be identified early for more aggressive and more targeted treatment, or identify patients at risk whom are in need of more intensive follow up. Also, echocardiography could be an additional tool to identify patients that may benefit from treatment strategies. Show less
In this thesis the prognostic value of the new echocardiographic techniques 'speckle tracking strain' and 'myocardial work' was evaluated in patients with hypertrophic cardiomyopathy and primary... Show moreIn this thesis the prognostic value of the new echocardiographic techniques 'speckle tracking strain' and 'myocardial work' was evaluated in patients with hypertrophic cardiomyopathy and primary mitral regurgitation. These parameters were indenpendently associated with outcome in these patients. Furthermore, the pathophysiology and hereditary component of primary mitral valve disease were discussed. Show less
The objectives of this thesis were to elucidate the pathogenesis of metabolic heart disease, evaluate the associated changes in myocardial structure and contractile function, and determine the long... Show moreThe objectives of this thesis were to elucidate the pathogenesis of metabolic heart disease, evaluate the associated changes in myocardial structure and contractile function, and determine the long-term prognostic implications of subclinical myocardial dysfunction on all-cause mortality. Show less
Functional mitral regurgitation (MR) – also referred to as secondary MR – is a disease condition which results from a combination of annular dilatation, papillary muscle displacement with increased... Show moreFunctional mitral regurgitation (MR) – also referred to as secondary MR – is a disease condition which results from a combination of annular dilatation, papillary muscle displacement with increased systolic leaflet tethering, and reduced closing forces, due to regional or global left ventricular (LV) remodelling.Functional MR is a common phenomenon and can be classified as either ischaemic or non-ischaemic, based on aetiology of LV remodelling. Regardless of aetiology, functional MR carries a poor prognosis.The primary step in the treatment of patients with functional MR consists of optimal medical and device therapy. In patients with persistence of MR despite optimal medical and device therapy, surgical treatment options can be considered. Over the past decades, many surgical treatment options have been developed, of which mitral valve repair by implantation of a restrictive mitral annuloplasty (RMA) ring forms the mainstay.In this thesis an integrated medico-surgical approach for patients with functional MR was examined, consisting of optimal medical and device therapy combined with RMA, and additional surgical interventions when indicated. The indication for each surgical intervention was determined after careful balancing of treatment options by the multidisciplinary Heart Team – consisting of heart failure specialists, interventional cardiologists, arrhythmia cardiologists and cardiac surgeons. Focus of this thesis was to determine (long-term) clinical and echocardiographic outcomes after this approach and to identify which patients are (un)likely to benefit from it. 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
This thesis has shown that significant lead-induced TR due to the mechanical presence of an RV-lead though the tricuspid valve was associated with worse long-term prognosis. CRT is one of the... Show moreThis thesis has shown that significant lead-induced TR due to the mechanical presence of an RV-lead though the tricuspid valve was associated with worse long-term prognosis. CRT is one of the main therapeutic breakthroughs in heart failure of the last decade but patients included in landmark trials do not completely mirror patients undergoing CRT in the clinical practice, who are usually older and have more frequently associated comorbidities such as renal dysfunction, diabetes or atrial fibrillation. This thesis shows a beneficial, although limited, effect of CRT also in elderly, in patients with diabetes and CKD stage 4, and therefore suggests that this therapy should not be withheld based on certain co-morbidities or on age alone. Furthermore, it shows that RBBB in patients referred to CRT and favourable RV-function improvement after CRT were associated with CRT outcomes. To improve clinical risk-stratification, this thesis proposed a CRT-SCORE using CRT-specific parameters and showed to be valuable in risk-estimation that may assist clinicians in counseling patients and guide clinical shared decision-making. Finally, novel approaches to optimize patient selection are presented in this thesis. SDI, a 3D-echocardiography LV-dyssynchrony measurement and T1-mapping, a novel CMR-technique to quantify diffuse myocardial fibrosis were significantly associated with CRT outcomes. Show less
