The Fontan procedure is currently the palliative solution for children born with a univentricular heart. Despite an improved survival rate, many patients still suffer from complications, such as... Show moreThe Fontan procedure is currently the palliative solution for children born with a univentricular heart. Despite an improved survival rate, many patients still suffer from complications, such as heart failure. For this reason, both patients with and without heart failure are often treated with angiotensin-converting enzyme (ACE) inhibitors, although the effectiveness is unclear. In this thesis, we investigated various aspects of the Fontan circulation to assess not only the efficacy and side effects of ACE inhibition, but also the limitations of exercise capacity and the response of the circulation of these patients to acute volume loading tests. We showed that short-term ACE inhibition had no positive effects on exercise capacity, haemodynamics and cardiac function. It also appeared to have no negative effect on the Fontan circulation, apart from side effects during the titration period. Furthermore, we found that even relatively healthy young Fontan patients have reduced exercise capacity associated with diastolic dysfunction and aortic stiffness. Nevertheless, we found that paediatric Fontan patients are able to respond adequately to acute volume changes and they seem to adapt well to the Fontan circulation. Show less
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 is the result of several studies into the clinical and echocardiographic outcome of both open and hybrid surgical ventricular reconstruction for the treatment of ischemic cardiomyopathy... Show moreThis thesis is the result of several studies into the clinical and echocardiographic outcome of both open and hybrid surgical ventricular reconstruction for the treatment of ischemic cardiomyopathy. Additionally, predictors for a favorable outcome and important associated issues such as management and late outcome of functional mitral regurgitation and the use of LV ejection fraction as a selection criterium for indication for a implantable cardioverter defibrillator for the primary prevention ofventricular arrhythmias after surgical ventricular reconstruction were studied. In chapter 2 the early and late outcome of different types of open left ventricular reconstruction surgery by means of a meta-analysis are presented. Chapter 3 describesthe use of echocardiographic wall motion score index to predict mortality and functional results after surgical ventricular reconstruction for advanced ischemic heart failure. In chapter 4 the management of functional mitral regurgitation during left ventricular reconstruction is presented followed by a landmark analysis into the 10-year outcome of functional mitral regurgitation after left ventricular reconstruction. Chapter 5 discusses the use of the improved LV ejection fraction after SVR as an indication for a implantable cardioverter defibrillator for the primary prevention of ventricular arrhythmias after surgical ventricular reconstruction in heart failure patients. Chapter 6 discusses the early experience with a minimal-invasive hybrid transcatheter surgical ventricular reconstruction technique. First the technique of hybrid transcatheter left ventricular reconstruction is described. Followed by the preliminary results of this technique from 2 cardiac centres in the Netherland. Finally, the multicenter European results of hybrid less invasive reconstruction on clinical, functional and echocardiographic outcome are presented. Show less
Vasoplegia is a common complication after heart failure surgery, although the incidence differs for each surgical procedure type. It is associated with poor early and late survival rates. In the... Show moreVasoplegia is a common complication after heart failure surgery, although the incidence differs for each surgical procedure type. It is associated with poor early and late survival rates. In the vasoplegic survivors, renal function is compromised compared to non-vasoplegic patients even though the cardiac function is similar. Preoperative factors associated with an increased risk on vasoplegia (e.g. anemia and a higher thyroxine levels) and factors associated with an decreased risk (e.g. higher creatinine clearance, beta-blocker use, prior hypertension) could be used to assess the risk on vasoplegia preoperatively for specific heart failure procedures. Also, intraoperative factors that are associated with an increased risk of vasoplegia (intraoperative vasoresponsiveness, longer cross clamp -, cardiopulmonary bypass - and procedure times) could be used to estimate the risk on vasoplegia. Further research is necessary to verify whether these results can be extrapolated to other hospitals and to validate the proposed risk model. Show less
In this PhD thesis we have studied left ventricular systolic deformation with novel cardiovascular magnetic resonance (CMR) algorithm called feature-tracking in patients with acute myocardial... Show moreIn this PhD thesis we have studied left ventricular systolic deformation with novel cardiovascular magnetic resonance (CMR) algorithm called feature-tracking in patients with acute myocardial infarction (MI). We have shown that left ventricular strain with feature-tracking CMR offers an incremental value above conventional imaging parameters in the assessment of prognosis of patients with acute MI. In addition, left ventricular strain analysis confirmed a clear clinical benefit of early intravenous betablocker treatment in patients with acute MI. Moreover, we have explored the feasibility of novel automated 3D echocardiographic algorithm in the assessment of the aortic root anatomy in patients with severe aortic stenosis prior to transcatheter aortic valve replacement (TAVR). The 3D echocardiographic algorithm performed excellent against the gold standard multidetector row computed tomography to determine the aortic annulus size and to choose the correct TAVR prosthesis size; however, the accuracy was a bit lower in patients with severely calcified aortic valves. In addition, we have explored the advantages and limitations of different cardiac imaging techniques in the assessment of patients with TAVR, the role of multimodality imaging in patients with combined valvular heart disease and heart failure, and the role of myocardial fibrosis assessment with CMR in valvular heart disease. Show less
The aim of this thesis is to provide new insights on catheter ablation of VT in patients with prior MI. Improved understanding of the VT substrate in different types of MI, and in particular, in... Show moreThe aim of this thesis is to provide new insights on catheter ablation of VT in patients with prior MI. Improved understanding of the VT substrate in different types of MI, and in particular, in contemporary non-transmural reperfused infarctions, a critical reassessment of the value of non-inducibility as ablation endpoint and the development of new physiologically meaningful endpoints for substrate modification will hopefully contribute to optimize procedural outcomes in this patient population. In addition, recognition of the limitations of the technique will aid in patient selection and will help to consider alternative treatment options in some individuals. 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
Right ventricular (RV) dysfunction is important determinant of prognosis in patients at increased risk for development of RV overload. RV dysfunction in patients before and after surgical left... Show moreRight ventricular (RV) dysfunction is important determinant of prognosis in patients at increased risk for development of RV overload. RV dysfunction in patients before and after surgical left ventricular restoration is associated with a worse prognosis. RV overload in liver transplantation recipients was also associated with decreased survival. Furthermore, patients with congenital heart disease and an systemic RV are at increased risk for long-term complications caused by RV overload. No unique golden standard for the quantification of RV dysfunction exists. The combination of echocardiographic RV parameters yields additional prognostic information in heart failure patients undergoing surgical left ventricular restoration. In systemic sclerosis the electrical ventricular gradient can accurately detect RV overload and is consistent with other screening parameters in patients with limited organ involvement. Optimization of loading conditions can improve clinical status and RV function in patients with RV dysfunction. RV function can be improved in LVAD patients by optimizing the synergy between the LVAD and intrinsic cardiac function. Furthermore, interventions in complex congenital heart disease patients with pulmonary hypertension can improve RV loading conditions and true pulmonary flow and can improve RV loading conditions and clinical functioning. Show less
