エプスタイン病

OBJECTIVE: Cone reconstruction has become the preferred surgical technique for Ebstein's anomaly (EA). However, some patients aged ≥ 50 years are unsuitable for repair due to severe leaflet dysplasia, marked annular dilation, or impaired right ventricular function. This study evaluates the clinical outcomes of domestic bioprosthetic valve replacement in patients aged ≥ 50 years with EA. METHODS: A retrospective analysis was conducted on 15 consecutive patients aged ≥ 50 years with EA who underwent initial bioprosthetic tricuspid valve replacement at the Department of Cardiovascular Surgery, Affiliated Hospital of Southwest Medical University, from June 2019 to August 2024. Preoperative evaluations included electrocardiography, echocardiography, chest CT, and coronary angiography. Operative times, cardiopulmonary bypass time, aortic cross-clamp time, perioperative complications, and mortality were recorded. Follow-up assessed cardiac function, valve performance, and cardiac chamber dimensions. RESULTS: Among the 15 patients (11 females, 4 males; mean age 55.0 ± 4.2 years), cardiopulmonary bypass time was 127.9 ± 20.4 min and aortic cross-clamp time 69.5 ± 17.5 min. One patient died of cardiogenic shock on postoperative day 8. The remaining 14 were discharged alive. During follow-up (1-39 months), 13 patients (92.6%) of 14 survivors were followed; no late deaths or reoperations occurred. Symptomatic improvement was observed in 12 patients. No moderate or severe tricuspid regurgitation or bioprosthetic stenosis was detected. CONCLUSION: Bioprosthetic valve replacement appears feasible with acceptable early outcomes in patients aged ≥ 50 years with EA who are unsuitable for cone reconstruction, significantly improving symptoms in most patients. These preliminary findings require validation in larger studies with longer follow‑up.

The right atrium (RA) and atrialised RV (aRV) may be important in determining exercise capacity in Ebstein anomaly (EA). We performed exercise testing and resting/exercise cardiac magnetic resonance imaging (CMRI) to examine associations between RA and aRV and left heart volumes, cardiac index and exercise capacity (%VO2max). In 13 un-operated EA patients (median age 13.5 years), indexed end-diastolic volumes were calculated from resting CMRI; right atrium (RAi), atrialised RV (aRVi), functional RV (fRVEDVi), left atrium (LAi) and left ventricle (LVEDVi). Systolic (SCRsys) and diastolic (SCRdias) septal curvature were measured. Pulmonary artery and aortic flow were measured at rest and peak exercise, and cardiac index (CI) calculated. CIex was strongly positively associated with %VO2max (r = 0.74, p = 0.01). RAi and aRVi, but not fRVEDVi, were negatively associated with %VO2max, CIrest and CIex. fRVEF was positively associated with %VO2max, CIrest and CIex. LAi, LVEDVi and LVEF were associated with %VO2max, CIrest and CIex. SCRdias was strongly negatively associated with aRVi (r=-0.89, p < 0.01) and RAi (r=-0.73,p < 0.01) but not with fRVEDVi (r =-0.34,p = 0.28). In multivariable models LVEDVi had the most significant influence on %VO2max, CIrest and CIex. In EA, volumes of the true RA and aRV but not fRV are associated with exercise capacity and cardiac output at rest and on exercise. Maintaining LV diastolic volume is a key determinant of exercise capacity in EA.

Pediatric congenital right heart obstructive lesions encompass a spectrum of diseases that obstruct blood flow from the right atrium to the pulmonary artery. Right ventricular inflow obstructions include tricuspid valve abnormalities, such as Ebstein anomaly, tricuspid valve dysplasia, and tricuspid atresia. Right ventricular outflow obstructions include pulmonary valve stenosis, pulmonary atresia, and tetralogy of Fallot. Cardiac computed tomography angiography (CCTA) is a valuable tool for the diagnosis, treatment planning, and follow-up of these lesions. In this pictorial review, we highlight the diagnostic utility of CCTA in congenital right heart obstructive lesions, emphasizing its role in preoperative planning.

Primary tricuspid valve regurgitation includes congenital and acquired disorders, especially Ebstein anomaly and tricuspid valve dysplasia. Severe tricuspid regurgitation causes progressive right atrial and ventricular dilation, arrhythmias, exercise intolerance, impaired left ventricular filling, and eventual biventricular dysfunction. Some patients remain minimally symptomatic for decades despite progressive disease. Poor prognostic factors include severe cardiomegaly, ventricular dysfunction, cyanosis, and arrhythmias. Surgical indications include symptoms, declining exercise capacity, cyanosis, progressive right ventricular enlargement or dysfunction, recurrent arrhythmias, and, in selected asymptomatic patients, severe regurgitation with right ventricular dilation. The Cone repair procedure restores native valve competence, improves right and left ventricular interaction, promotes reverse remodeling, and has low operative risk in experienced centers. Current evidence supports individualized and earlier surgical repair, preferably during childhood and in specialized centers, to improve ventricular function, quality of life, and long-term survival.