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Eosinophilic myocarditis can result in endomyocardial fibrosis affecting both ventricles, leading to restrictive cardiomyopathy. Multimodality imaging is crucial for diagnosis, as demonstrated in this case of a patient presenting with symptoms of heart failure.
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Background: Fabry cardiomyopathy is characterized by left ventricular hypertrophy, myocardial fibrosis, arrhythmia, and premature death. Treatment with migalastat, an oral pharmacological chaperone, was associated with a stabilization of cardiac biomarkers and a reduction in left ventricular mass index, as measured by echocardiography. A recent study, using cardiac magnetic resonance (CMR) as the gold standard, found a stable course of myocardial involvement after 18 months of treatment with migalastat. Our study aimed to provide long-term CMR data for the treatment with migalastat. Methods: A total of 11 females and four males with pathogenic amenable GLA mutations were treated with migalastat and underwent 1.5T CMR imaging for routine treatment effect monitoring. The main outcome was a long-term myocardial structural change, reflected by CMR. Results: After migalastat treatment initiation, left ventricular mass index, end diastolic volume, interventricular septal thickness, posterior wall thickness, estimated glomerular filtration rate, and plasma lyso-Gb3 remained stable during the median follow-up time of 34 months (min.: 25; max.: 47). The T1 relaxation times, reflecting glycosphingolipid accumulation and subsequent processes up to fibrosis, fluctuated over the time without a clear trend. No new onset of late gadolinium enhancement (LGE) areas, reflecting local fibrosis or scar formation of the myocardium, could be detected. However, patients with initially present LGE showed an increase in LGE as a percentage of left ventricular mass. The median α-galactosidase A enzymatic activity increased from 37.3% (IQR 5.88-89.3) to 105% (IQR 37.2-177) of the lower limit of the respective reference level (p = 0.005). Conclusion: Our study confirms an overall stable course of LVMi in patients with FD, treated with migalastat. However, individual patients may experience disease progression, especially those who present with fibrosis of the myocardium already at the time of therapy initiation. Thus, a regular treatment re-evaluation including CMR is needed to provide the optimal management for each patient.
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OBJECTIVE: Transcatheter aortic valve implantation (TAVI) is an off-label procedure for selected patients at high surgical risk with native non- or mildly calcified aortic regurgitation (AR). Traditionally, self-expanding transcatheter heart valves (THV) have been favored over balloon-expandable THV's probably due to assumed better device fixation. We report a series of patients with native severe AR successfully treated with a balloon-expandable THV. METHODS: Between 2019 and 2022, 8 consecutive patients (5 male, 82 (interquartile range 80-85) years old, STS PROM 4.0 % (interquartile range 2.9-6.0), EuroSCORE II 5.5% (IQR 4.1-7.0) with non- or mildly calcified pure AR were treated with a balloon-expandable THV. All procedures were performed after heart team discussion and standardized diagnostic workup. Clinical endpoints were collected prospectively and included device success, procedural complications (according to VARC-2 definitions) and 1-month survival. RESULTS: Device success was 100% with no device embolization or migration. Two preprocedural nonfatal complications were reported (one access site complication that required stent implantation and one pericardial tamponade). Two patients required permanent pacemaker implantation for complete AV block. At discharge and at 30-day follow-up all patients were alive and no patient showed more than minimal AR. CONCLUSION: This series documents that treatment of native non- or mildly calcified AR with balloon-expandable THV is feasible, safe and offers favorable short-term clinical outcomes. Hence, TAVI with balloon-expandable THVs may offer a valuable treatment option in patients with native AR at high surgical risk.
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Aortic Valve Insufficiency , Heart Valve Prosthesis , Transcatheter Aortic Valve Replacement , Humans , Male , Aged, 80 and over , Catheters , HeartABSTRACT
Transcatheter Aortic Valve Implantation in Multivalvular Heart Disease Abstract. The prevalence of multivaluvular heart disease is high in patients undergoing transcatheter aortic valve implantation (TAVI). The most common combination is aortic valve stenosis (AS) and mitral regurgitation, followed by the combination of AS with a tricuspid regurgitation or mitral stenosis. Grading of multivalvular disease is challenging and can quickly lead to underestimation of the disease stage. Therefore, a profound knowledge of pathophysiologic interactions is essential, and the patient should always undergo multimodal evaluation. After a successful TAVI intervention, secondary heart valve defects may improve, deteriorate, or remain unchanged. Due to the still sparse scientific data in this field, the role of the heart team remains central to provide the patient with an individually adapted therapy plan.
