Mitral valve transcatheter edge-to-edge repair as a bridge to treat aortic dissecting aneurysm in a case of Marfan syndrome: a case report.

Background: Marfan syndrome is an inherited disorder that manifests with various cardiovascular conditions. This case report discusses a patient with Marfan syndrome presenting with concurrent dissecting aortic aneurysm and acute mitral valve regurgitation (MR), exploring treatment strategies for this unique case. Case summary: A 57-year-old man diagnosed with Marfan syndrome presented with progressive dyspnoea and awareness of orthopnoea. Acute heart failure (HF) due to acute MR associated with chordae rupture was diagnosed. However, contrast-enhanced CT revealed the coexistence of a massive dissecting aortic aneurysm, indicating surgical intervention. The dissecting aortic aneurysm extended over a large area. Given the high risk of simultaneous surgery with the mitral valve, a staged approach was adopted. Mitral valve transcatheter edge-to-edge repair (MV-TEER) was performed as the initial step to reduce the perioperative HF risk, followed by a planned two-stage surgery for the dissecting aortic aneurysm. This strategy effectively facilitated surgical intervention for the dissecting aortic aneurysm in the chronic phase after MV-TEER. Discussion: Several reports showed the effectiveness of MV-TEER in cases of degenerative MR where surgical operation carries a high risk, but case report of MV-TEER in Marfan syndrome is rare. In recent years, the effectiveness of MV-TEER has also been reported as a 'bridge therapy' for heart transplantation. Mitral valve transcatheter edge-to-edge repair is considered a potential option to serve as a bridge to other invasive intervention.

Melody Valve Fracture Causing Mitral Stenosis: Novel Solution for Transapical Valve-in-Valve.

The use of Melody valves in the mitral position has been introduced in clinical practice. Stent fracture is a recognized complication of Melody valve implantation in the pulmonary position; however, reports in the mitral position are rare. We present the case of an 8-year-old boy in whom complete fracture of the proximal stent struts occurred, causing acute severe mitral stenosis, and in whom urgent hybrid transapical Melody valve implantation in the fractured Melody valve was performed successfully using a novel modified technique. This modification simplified the implantation, led to reduced time of the procedure, and minimized hemodynamic instability.

Good response to tolvaptan shortens hospitalization in patients with congestive heart failure.

Tolvaptan has been gradually spread to use as a potent diuretic for congestive heart failure in the limited country. However, the response to this aquaretic drug still is unpredictable. A total of 92 patients urgently hospitalized due to congestive heart failure and treated with tolvaptan in addition to standard treatment was retrospectively analyzed. Responder of tolvaptan treatment was defined as a patient with peak negative fluid balance greater than 500 mL/day, and clinical profiles were compared between 76 responders and 16 non-responders. Responders started to increase daily urine volume (UV) from Day 1 through Day 3. In contrast, non-responders showed no significant increase in daily UV from the baseline up to Day 5. Time between admission and tolvaptan administration was shorter in responders, even without statistical significance (3.3 vs. 4.6 days, p = 0.053). Multivariate analysis revealed that blood urea nitrogen (BUN) [cutoff: 34 mg/dL, odds ratio (OR) 9.0, 95% confidence interval (CI) 1.42-57.3, p < 0.01] and plasma renin activity (PRA) (cutoff: 4.7 ng/mL/h, OR 6.1, 95% CI 1.01-36.4, p < 0.01) at baseline were independent predictors for tolvaptan responsiveness. It suggests that renal perfusion may affect tolvaptan-induced UV. Finally, durations of stay in intensive care unit and total hospitalization were significantly shorter in responders (median: 6.0 vs. 13.0 days, p = 0.022; 15.0 vs. 25.0 days, p = 0.016, respectively). Responders of tolvaptan have lower BUN and renin activity at baseline, and shorten hospitalization period. Trial Registration The study was registered at University Hospital Medical Information Network Clinical Trials Registry (UMIN-CTR) with the identifier UMIN000023594. https://upload.umin.ac.jp/cgi-open-bin/ctr_e/ctr_view.cgi?recptno=R000024988.