Fabry disease (FD) is an X-linked lysosomal storage disorder caused by mutations in the galactosidase A (GLA) gene that result in deficient galactosidase A enzyme and subsequent accumulation of glycosphingolipids throughout the body. The result is a multi-system disorder characterized by cutaneous, corneal, cardiac, renal, and neurological manifestations. Increased left ventricular wall thickness represents the predominant cardiac manifestation of FD. As the disease progresses, patients may develop arrhythmias, advanced conduction abnormalities, and heart failure. Cardiac biomarkers, point-of-care dried blood spot testing, and advanced imaging modalities including echocardiography with strain imaging and magnetic resonance imaging (MRI) with T1 mapping now allow us to detect Fabry cardiomyopathy much more effectively than in the past. While enzyme replacement therapy (ERT) has been the mainstay of treatment, several promising therapies are now in development, making early diagnosis of FD even more crucial. Ongoing initiatives involving artificial intelligence (AI)-empowered interpretation of echocardiographic images, point-of-care dried blood spot testing in the echocardiography laboratory, and widespread dissemination of point-of-care ultrasound devices to community practices to promote screening may lead to more timely diagnosis of FD. Fabry disease should no longer be considered a rare, untreatable disease, but one that can be effectively identified and treated at an early stage before the development of irreversible end-organ damage.

Yim J, et al. J Cardiovasc Imaging. 2023 Mar;31:e18. https://doi.org/10.4250/jcvi.2022.0120

Intracardiac stent migration is a rare but potentially life-threatening complication of venous interventions, often requiring surgical management. A 38-year-old patient underwent left common iliac vein endovascular intervention with an 18 × 80-mm self-expanding venous stent and presented 1 day later with palpitations and dyspnea. Imaging demonstrated stent migration across the tricuspid valve, causing severe regurgitation and nonsustained ventricular tachycardia. The stent was successfully retrieved using a dual-snare technique via bilateral femoral access under general anesthesia with transesophageal echocardiographic guidance and surgical backup. Postretrieval imaging revealed only mild residual tricuspid regurgitation. The patient recovered uneventfully. Venous stent migration into the tricuspid valve can be managed safely through a multidisciplinary, collaborative, transesophageal echocardiography-guided percutaneous approach, offering a minimally invasive alternative to surgery. Percutaneous retrieval of migrated venous stents is feasible and safe when performed in a hybrid environment by an experienced interventional, surgical, and imaging team. Early transition to a dual-snare technique with deflectable sheaths should be considered when a single-snare attempt risks injury to the tricuspid apparatus.

Atrial fibrillation (AF) and tricuspid regurgitation (TR) commonly coexist and are associated with adverse clinical outcomes. Whether restoration of sinus rhythm through catheter ablation improves TR severity has not been systematically quantified. We evaluated the association between AF catheter ablation and changes in moderate or greater TR. We performed a systematic review and meta-analysis of studies evaluating patients with AF undergoing catheter ablation and reporting paired preablation and postablation assessments of TR severity. MEDLINE, Embase, CENTRAL and Web of Science were searched from inception to 22 April 2026. The primary outcome was change in the prevalence of moderate or greater TR following ablation. Random-effects meta-analysis was performed using restricted maximum likelihood estimation. Meta-regression evaluated prespecified study-level modifiers, including sex proportion, mean age and baseline AF type. 17 studies comprising 2747 patients were included. AF catheter ablation was associated with a lower prevalence of moderate or greater TR (risk difference −0.21, 95% CI −0.33 to −0.09; pooled risk ratio 0.49, 95% CI 0.40 to 0.60), although heterogeneity was substantial (I²=98.3%) and the 95% prediction interval was wide (−0.74 to 0.31). Patients who maintained sinus rhythm had greater TR reduction than those with recurrent AF (risk difference −0.28, 95% CI −0.55 to −0.01). In meta-regression, higher proportions of female participants and persistent AF were associated with greater TR improvement, whereas mean left ventricular ejection fraction was not. Findings were consistent in sensitivity analyses restricted to studies at low-to-moderate risk of bias, observational studies and leave-one-out analyses. AF catheter ablation was associated with a significant reduction in moderate or greater TR, with greater improvement among patients maintaining sinus rhythm. These findings support prospective evaluation of rhythm control as a potential strategy for patients with moderate or greater TR and AF. CRD420251154277.