BackgroundCardiac paragangliomas are extremely rare neuroendocrine tumours derived from neural crest cells that represent <2% of all paragangliomas. Approximately 35–40% of all paragangliomas... Show moreBackgroundCardiac paragangliomas are extremely rare neuroendocrine tumours derived from neural crest cells that represent <2% of all paragangliomas. Approximately 35–40% of all paragangliomas are associated with inherited syndromes such as mutation in the succinate dehydrogenase (SDH) enzyme.Case summaryA 44-year-old male with an SDH complex subunit D (SDHD) mutation was diagnosed with an intracardiac paraganglioma attached to the left main coronary artery. Multimodality imaging, including gallium dotatate positron emission tomography computed tomography, cardiac magnetic resonance imaging, and coronary computed tomography angiography (CCTA) confirmed the suspected intracardiac paraganglioma. During follow-up with a CCTA, the mass showed growth, and surgical removal was recommended to anticipate on the risk of compression of the left main coronary artery. Prior to surgery, coronary angiography was performed, which showed no coronary calcifications. The highly vascularized paraganglioma was visible near the left main and proximal left anterior descending artery. The intracardiac paraganglioma was successfully removed through a median sternotomy with cardiopulmonary bypass, without any complications. The post-operative course was uneventful, and histological examination confirmed the diagnosis of a paraganglioma.DiscussionIntracardiac paragangliomas in the vicinity of the left main coronary artery are rare, and surgical removal may be challenging. Therefore, screening and the use of multiple imaging modalities in patients with SDHD mutations prior to surgery is of major importance. Show less
Background Amiodarone is associated with a range of unwanted effects on pulmonary, thyroid, and liver function. However, the nephrotoxic side effect caused by renal phospholipidosis has hardly... Show moreBackground Amiodarone is associated with a range of unwanted effects on pulmonary, thyroid, and liver function. However, the nephrotoxic side effect caused by renal phospholipidosis has hardly received any attention up to now.Case summary This is a case of an 86-year-old Caucasian male with an acute on chronic kidney disease 4 months after the initiation of amiodarone. A renal biopsy demonstrated the intracellular accumulation of phospholipids that have previously been demonstrated in association with organ dysfunction because of amiodarone use. Serum creatinine levels subsequently improved from 388 to 314 & mu;mol/L after stopping amiodarone over the course of 2 months.Discussion In this case, a diagnosis of partially reversible acute on chronic kidney disease caused by lysosomal phospholipidosis due to amiodarone use was deemed highly likely. Lysosomal dysfunction leads to the accumulation of intra-lysosomal phospholipids (phospholipidosis). This accumulation is accompanied by progressive organ damage and dysfunction, including renal dysfunction, in rare instances. Guidelines advise regular surveillance for liver, lung, and thyroid toxicity during amiodarone treatment but do not mention the potential for renal toxicity. This case suggests that it might be prudent to include screening for renal toxicity in this surveillance. Show less
BackgroundPregnancy is a known trigger of novel and pre-existing supraventricular tachyarrhythmias. We present a case of a stable pregnant patient presenting with atrioventricular nodal reentry... Show moreBackgroundPregnancy is a known trigger of novel and pre-existing supraventricular tachyarrhythmias. We present a case of a stable pregnant patient presenting with atrioventricular nodal reentry tachycardia (AVNRT) and application of the ‘facial ice immersion technique’.Case summaryA 37-year-old pregnant woman presented with recurrent AVNRT. Due to unsuccessful attempts of conventional vagal manoeuvres (VMs) and refusal of pharmacological agents, we successfully performed a non-conventional VM with the ‘facial ice immersion technique’. This technique was applied successfully at repeated clinical presentation.DiscussionThe role of non-pharmacological interventions remains pivotal and may lead to desired therapeutical effects without the use of any costly pharmacological agents with possible adverse events. However, non-conventional VMs such as the ‘facial ice immersion technique’ are less commonly known but appear to be easy and a safe option for both mother and foetus in the management of AVNRT during pregnancy. Clinical awareness and understanding of treatment options are imperative in contemporary patient care. Show less
Neo(adjuvant) systemic treatment regimens containing anthracyclines such as doxorubicin cause a significant risk of heart failure. These regimens are one of the corner stones of osteosarcoma... Show moreNeo(adjuvant) systemic treatment regimens containing anthracyclines such as doxorubicin cause a significant risk of heart failure. These regimens are one of the corner stones of osteosarcoma treatment, and therefore several guidelines are in place to steer cardiotoxicity monitoring through baseline risk stratification and cardiac surveillance during and after completion of cancer therapy. Importantly, baseline risk stratification modules are dependent on age, prior cardiovascular disease and cardiovascular risk factors. Because the majority of osteosarcoma patients are below 30 years of age these criteria rarely apply and most patients are assigned to low or medium risk categories, whereas cardiovascular complications have profound impact on morbidity and mortality in this young population. Therefore, cardiac surveillance is very important in this group for timely detection of cardiotoxicity. Moreover, when severe cardiotoxicity that requires advanced heart failure treatment occurs, a cancer diagnosis has significant implications on treatment options, i.e. mechanical circulatory support and heart transplantation. These challenges are presented in this case of a patient without clinical risk factors admitted with cardiogenic shock requiring advanced heart failure treatment within 1 month after completion of doxorubicin containing chemotherapy for the treatment of high grade osteosarcoma. Show less
Grewal, N.; Yousef, D.; Palmen, M.; Klautz, R.; Eikenboom, J.; Wink, J. 2022
Introduction Extracorporeal circulation (ECC) in cardiac surgery is performed under systemic heparinization. Adequacy of heparin therapy and anticoagulation during ECC is assessed by activated... Show moreIntroduction Extracorporeal circulation (ECC) in cardiac surgery is performed under systemic heparinization. Adequacy of heparin therapy and anticoagulation during ECC is assessed by activated clotting time (ACT), although there are concerns regarding the reliability of this measure. The ACT can be affected by factors other than heparin anticoagulation. A novel factor that should be considered is the influence of a COVID-19 infection. More than half of the hospitalized COVID-19 patients develop coagulation abnormalities with dysregulated coagulation test results. Patients recently recovered from COVID-19 may still demonstrate some forms of coagulation disorder affecting the ACT. This case describes an inaccurate point-of-care ACT testing in a patient with previous COVID-19 infection undergoing cardiac surgery with ECC and the alternative coagulation testing performed. Case presentation A 77-years-old Caucasian male presented with symptomatic severe mitral valve regurgitation for which he underwent surgery. Medical history revealed a COVID-19 infection one month before surgery. Pre-operative hematological lab results were normal and baseline ACT during surgery was 100 s. To achieve an adequate ACT of > 400 s, multiple doses of heparin were needed and after administration of a triple dose (75,000 IE heparin in total) this adequate ACT was achieved. In the meanwhile we measured anti-Xa level and APTT, which were at adequate levels when ACT was still < 400 s. Discussion This case emphasizes the need of alternative methods for monitoring heparin therapy in case ACT does not respond adequately. Another point to highlight in this case is the poorly correlated relation between ACT and APTT and anti-Xa in light of the recent COVID-19 infection. Although studies have shown that COVID-19 infection can cause coagulopathy and altered hemostatic parameters, ACT has never been investigated in COVID-19 patient. Understanding the correlation between ACT, APTT and anti-Xa in COVID-19 patients is mandatory. Show less
Vonderen, J.J. van; Keus, J.M.H.; Schaik, J. van; Smiers, F.J.; Harkel, D.J. ten; Lopriore, E. 2021
Background In this case report, we describe a very rare case of severe limb ischemia due to an arterial embolus caused by an aneurysm of the oval foramen in a term-born infant that occurred in the... Show moreBackground In this case report, we describe a very rare case of severe limb ischemia due to an arterial embolus caused by an aneurysm of the oval foramen in a term-born infant that occurred in the first few hours after birth. Case presentation A newborn male Caucasian patient presented on the maternity ward with ischemia of the right foot. Ischemia was treated with nitroglycerin spray and low-molecular-weight heparin in therapeutic dosage. An aneurysm of the oval foramen was found during postnatal echocardiography screening. This was thought to be the source of an embolus causing limb ischemia. At birth and upon follow-up, no clotting disorders were found. A large part of the right forefoot was ischemic, leading to loss of digits 1, 2, and 3. No significant loss of function was found in the first year of life. Conclusion Severe limb ischemia can be caused by an embolus arising from an aneurysm of the oval foramen and can be treated with heparin. Show less
Background Progressive renal insufficiency is frequent in heart failure patients with a left ventricular assist device (LVAD). The optimal strategy for long-term dialysis in LVAD patients and its... Show moreBackground Progressive renal insufficiency is frequent in heart failure patients with a left ventricular assist device (LVAD). The optimal strategy for long-term dialysis in LVAD patients and its effect on quality-of-life in these patients remain to be determined.Case summary Our 55-year-old patient with pre-existing renal insufficiency received an LVAD as destination therapy because of advanced ischaemic heart failure. Six years after implantation, he developed end-stage renal disease for which peritoneal dialysis (PD) was initiated. Left ventricular assist device flow alterations during uttrafiltration did not cause clinical or technical problems. The patient's exercise capacity increased and quality-of-life improved. Over 7.5 years after LVAD implantation and 16 months after PD initiation, he died from encephalitis.Discussion Despite initial improvement, renal function often gradually decreases after LVAD implantation. Data on long-term renal replacement therapy in LVAD patients are limited. Haemodialysis is most commonly applied. Conceptually, however, PD has advantages over haemodialysis including less bloodstream infections, less haemodynamic shifts, and the comfort of the ambulant setting. This case illustrates that PD in an LVAD patient is feasible and improves quality-of-life. Key factors contributing to successful PD in LVAD patients may be a good right ventricular function and close cardiology-nephrology collaboration. Show less
Egorova, A.D.; Erven, L. van; Beeres, S.L.M.A.; Tops, L.F. 2021
Background Cardiac implanted electronic devices (CIED) have significantly improved the survival and quality of life in heart failure patients. Although implantable cardioverter-defibrillators (ICD)... Show moreBackground Cardiac implanted electronic devices (CIED) have significantly improved the survival and quality of life in heart failure patients. Although implantable cardioverter-defibrillators (ICD) and cardiac resynchronization therapy (CRT) have a major role in patients with moderate to severe heart failure symptoms, the role of these devices in patients with a left ventricular assist device (LVAD) is not yet well defined. The burden of CIED-related procedures in patients with an LVAD is high. The price of lead malfunctions and pocket complications requires creative approaches to tackle CIED-related issues in this patient population.Case summary Here, we describe the clinical course of a 67-year-old ventricular pacing dependent LVAD patient with an ICD indication based on recurrent monomorphic ventricular tachycardias and a CRT indication due to previous deterioration of (right-sided) heart failure in the absence of biventricular pacing. We were confronted with impending right ventricular lead failure and bilateral venous access problems due to chronic subclavian vein occlusion in a patient with a total of five transvenous leads, therapeutic anticoagulation, and pronounced thoracic collaterals. We sought for a creative solution to be able to deliver effective biventricular fusion pacing with the existing leads from two contralateral pulse generators resulting in biventricular fusion pacing. This provided the solution to deliver effective CRT.Discussion This case illustrates the complexity of care and CIED-related decision-making in pacing dependent LVAD patients, in particularly those with an ICD and CRT indication. Show less
Background Due to the malfunction of connective tissue, Marfan patients are at increased risk of aortic dissection. Uncomplicated acute type B dissection is usually managed with medical therapy.... Show moreBackground Due to the malfunction of connective tissue, Marfan patients are at increased risk of aortic dissection. Uncomplicated acute type B dissection is usually managed with medical therapy. Retrograde progression or new type A dissection is a relatively rare but often fatal complication that occur most frequently in the first 6 months after acute type B dissection.Case summary We present a 31-year-old male with Marfan syndrome and a recent uncomplicated type B dissection from the left subclavian to the right common iliac artery who underwent 4D flow magnetic resonance imaging (MRI). The dissection had a large proximal intimal tear just distal to the left subclavian artery (15 mm) and large false lumen (35 mm). Aortic blood flow just distal to the left subclavian artery (3.6 L/min) was split disproportionately into the true (0.8 L/min, 22%) and false lumen (2.8 L/min, 78%). 4D flow streamlines revealed vortical flow in the proximal false lumen. Increased wall shear stress was observed at the sinotubular junction (STJ), inner wall of the ascending aorta and around the subclavian artery. Two weeks after MRI, the patient presented with jaw pain. Computed tomography showed a type A dissection with an entry tear at the STJ for which an acute valve-sparing root, ascending and arch replacement was performed.Discussion Better risk assessment of life-threatening complications in uncomplicated type B dissections could improve treatment strategies in these patients. Our case demonstrates that besides clinical and morphological parameters, flow derived parameters could aid in improved risk assessment for retrograde progression from uncomplicated type B dissection to acute type A dissection. Show less
Nederend, M.; Erven, L. van; Zeppenfeld, K.; Vliegen, H.W.; Egorova, A.D. 2021
Background Patients with congenitally corrected transposition of the great arteries (ccTGA) are prone to the development of advanced atrio-ventricular block requiring chronic ventricular pacing.... Show moreBackground Patients with congenitally corrected transposition of the great arteries (ccTGA) are prone to the development of advanced atrio-ventricular block requiring chronic ventricular pacing. The morphological right ventricle (RV) often develops systolic dysfunction as it is unable to withstand the chronic pressure overload it is exposed to when supporting the systemic circulation.Case summary A 56-year-old woman with dextrocardia and complex ccTGA with a history of dual-chamber implantable cardioverter-defibrillator (DDD-ICD, high degree atrio-ventricular-block and syncopal ventricular tachycardia), presented with progressive heart failure and symptomatic atrial arrhythmias. She underwent a successful ablation and concomitant invasive haemodynamic evaluation of potential alternative/biventricular pacing modalities. During biventricular pacing, the QRS narrowed and the systemic RV intraventricular pressure (Dp/Dt) increased with 30%. She underwent a successful transvenous upgrade to cardiac resynchronization therapy (CRT). The electrocardiogram post-implantation showed biventricular capture and patient showed subjective and objective clinical improvement.Discussion Systemic RV dysfunction in ccTGA can be aggravated by chronic pacing-induced dyssynchrony, contributing to progression of heart failure in this patient group. Transvenous CRT is feasible in ccTGA anatomy and may be pursued in order to improve or preserve the functional status of pacing-dependent ccTGA patients. Invasive haemodynamic contractility evaluation can help assess the potential benefit of CRT in patients with complex anatomy. Show less
Art therapy is an often provided but little studied treatment option in mental health care.The effectiveness of art therapy in the treatment of anxiety was studied and its working mechanisms were... Show moreArt therapy is an often provided but little studied treatment option in mental health care.The effectiveness of art therapy in the treatment of anxiety was studied and its working mechanisms were explored. Outcomes of a randomised controlled trial (n=59) showed preliminary evidence of the effectiveness of art therapy. The treatment, consisting of three months of anthroposophic art therapy, showed major reduction of anxiety symptom severity in women, compared to a wait list condition. The therapy also improved quality of life and various aspects of self-regulation: higher HRV at rest (which may indicate an overall lower stress level), improvements in emotion regulation and improvements in daily executive functioning.The second part of this PhD research focused on case report methodology and the development of tools for research within this field, with and for therapists in clinical practice, and for education. A publication guideline for case reports was developed and positively evaluated in practice. An exemplary case report of a female client with anxiety was provided.The outcomes of this research support the indication of art therapy in the treatment of anxiety and justify further studying of the effectiveness and mechanisms of art therapy. Show less
Abbing, A.C.; Baars, E.W.; Van Haastrecht, O.; Ponstein, A.S. 2019
Anxiety is a major problem for many individuals, causing impairment in daily life. Art therapy is often deployed and although positive results are communicated in clinical practice, its... Show moreAnxiety is a major problem for many individuals, causing impairment in daily life. Art therapy is often deployed and although positive results are communicated in clinical practice, its effectiveness and working mechanisms have hardly been studied. Therefore, it is important to systematically describe the intervention process and to detect the working mechanisms to be able to evaluate them. Narrative case studies help to understand the intervention in more depth. A typical case file was selected for case reporting according to scientific (CARE & CARE-AAT) guidelines, with the aim to explore the therapeutic elements that contributed to the reduction of anxiety. The report describes the intervention process of a 54-year-old female, suffering from anxiety since childhood and diagnosed with panic disorder, agoraphobia, claustrophobia and hypochondria. After 14 sessions of anthroposophic art therapy, reduction of anxiety was shown, as well as improvements of emotion regulation and executive functioning. The client indicated that she became more tolerant and accepting towards her anxiety. She noted a softened attitude towards herself and her complaints, even one year after art therapy. The course of treatment suggests that aspects of emotion regulation and executive functioning were addressed through implicit learning processes in different art therapy assignments. Show less
