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Background: General interest and incidence are increasing in wild-type transthyretin amyloidosis (ATTRwt) in recent time. As patient population increases, further knowledge of the management of the frequently encountered interacting cardiac comorbidities is requested to improve treatment of ATTRwt patients. Case summary: A 73-year-old male ATTRwt patient presented to the outpatient clinic (Day 0) with dyspnoea, leg swelling, and palpitations. At diagnosis, 3 years prior to presentation, he exhibited only minor signs of ATTRwt. At Day 0, clinical examination revealed atrial fibrillation and mild peripheral oedema. Anticoagulant and symptomatic treatment with beta-blocker and diuretics was initiated, and the patient was planned for sub-acute direct cardioversion, and the patient was discharged with a Holter monitor to outpatient care. At Day 7, analysis of the monitoring demonstrated spontaneous conversion to sinus rhythm and, unexpectedly, episodes of high-rate self-remittent sustained monomorphic ventricular tachycardia (VT) and frequent ventricular ectopic beats. At Day 8, a sub-acute coronary angiography was performed which revealed a significant proximal left anterior descending artery stenosis which was treated with percutaneous coronary intervention (PCI) and subsequently an internal defibrillator was implanted. Following visits at 1- and 3-month post-PCI at the outpatient clinic revealed no VT and suppression of ventricular ectopic beats. Discussion: The case illustrates some of the frequently encountered cardiac comorbidities (e.g. atrial fibrillation, ventricular arrhythmia, and ischaemic heart disease) associated with ATTRwt. A high level of suspicion is warranted to identify treatable cardiac conditions [atrial fibrillation, atrioventricular (AV) block, and ischaemic heart disease] and to uncover potentially fatal cardiac conditions in patients with ATTRwt.
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BACKGROUND: A subvalvular annuloplasty is often used for aortic valve repair in patients with isolated aortic regurgitation with aortic annulus dilatation. Our aim was to characterize and compare annulus geometry and dynamics of the Dacron ring and suture annuloplasty and compare it with the native aortic annulus under standardized conditions. METHODS: We randomized 29 pigs of 80 kg into a Dacron ring group, a suture annuloplasty group and a native control group. The assessment was performed using sonomicrometry crystals for evaluation of dynamic geometry, and pressure measurements and echocardiography to evaluate valve performance. RESULTS: Aortic annulus area (AAA) was significantly reduced in the Dacron and Suture group compared with the Native group. Expansibility was similar and within normal physiologic limits in all three groups (Native: 12%±7%; Dacron: 11%±3%; Suture: 10%±4%). The largest segmental expansion was observed at the right coronary sinus (RC) in the Native and Dacron group but in the Suture group there was no significant difference between segments. The aortic annulus was primarily oval in systole and became more circular in diastole in the Native and Dacron group, however, in the Suture group, the sphericity remained relatively unchanged throughout the cardiac cycle. CONCLUSIONS: This study is the first to describe and compare detailed segmental geometry of the Dacron ring and suture annuloplasty in a standardized porcine model. The two annuloplasties effectively downsized the aortic annulus, while expansibility was maintained. Each annuloplasty had its own geometrical characteristics, but the Dacron ring was more similar to the native aortic annulus than the suture annuloplasty. This study suggests that the Dacron ring offers a more physiological and standardized support by mimicking the geometry and dynamics of the native aortic annulus and thus is a preferable choice over the suture annuloplasty for valve-sparing aortic root procedures.
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PURPOSE: With new repair techniques of the aortic root and valve emerging, a detailed understanding of the dynamics of the aortic annulus and valve is required for optimal results. The objective of this study was to characterize geometrical changes and force distribution of the native porcine aortic annulus throughout the cardiac cycle. METHODS: Measurements were performed in an acute 80 kg porcine model (n = 7) using sonomicrometry crystals in the aortic annulus for evaluation of geometry and dynamics, annular force transducer evaluating force distribution, and pressure measurements and echocardiography evaluating valve performance. RESULTS: Overall, segmental force distribution and geometrical changes differed between different segments of the aortic annulus. The highest force development was found at the left/right interleaflet triangle (2.87 ± 2.1 N) and the largest segmental expansion was observed at the right-coronary and left-coronary sinus. The aortic annulus changed configuration throughout the cardiac cycle and became more oval in systole. CONCLUSIONS: This study is the first to describe detailed segmental dynamics and force distribution of the native aortic annulus in a porcine model in vivo. The heterogenous behavior of the aortic annulus suggests that different segments demand different support for repair of the aortic root and valve.
