先天性肺静脈狭窄症

Common pulmonary vein atresia (CPVA) is an extremely rare and lethal congenital heart defect characterized by the absence of a functional connection between the common pulmonary vein chamber (CPVC) and both the left atrium and systemic venous system. We report a case of a full-term female infant who successfully underwent primary sutureless repair. The patient developed acute respiratory distress syndrome (ARDS) with severe cyanosis and refractory hypoxemia 12 min after birth and underwent emergent surgery at 15 h of life. Intraoperative findings confirmed CPVA with bilateral superior pulmonary vein stenosis. Sutureless anastomosis was performed, leaving a 2.5 mm interatrial communication. Due to severe pulmonary hypertension and high-dose vasoactive requirements, venoarterial extracorporeal membrane oxygenation (VA-ECMO) support was established immediately. ECMO was weaned on postoperative day 6, the patient was extubated on day 10, and discharged on day 43. Two-month follow-up showed unobstructed pulmonary venous flow without evidence of anastomotic stenosis. To our knowledge, this is the first report of successful primary sutureless repair of this cardiac malformation.

BACKGROUND: The treatment of primary mitral and secondary tricuspid regurgitation (MR, TR) in the setting of lung agenesis poses major technical challenges, and data on the feasibility of endoscopic tricuspid valve repair in adult congenital anatomy remain limited. CASE SUMMARY: We report the case of a 76-year-old male with severe primary MR due to Barlow's disease, torrential secondary TR, biatrial dilatation, persistent atrial fibrillation, and post-capillary pulmonary hypertension. Anatomical anomalies included dextrocardia, hypoplastic right lung, and scimitar vein syndrome. The patient had a history of surgical correction of an aortic coarctation at the age of 32 with residual stenosis but no proof of residual gradients in invasive assessment. Transcatheter repair was deemed unsuitable due to the massive dextrocardia, extensive leaflet pathology, and annular dilatation. The patient underwent endoscopic mitral and tricuspid repair with dual arterial and venous cannulation to avoid malperfusion of the upper body during extracorporeal circulation. Mitral valve repair included a surgical edge-to-edge repair, along with an implantation of an annuloplasty ring; secondary TR was repaired by ring annuloplasty too. Postoperatively, the patient experienced prolonged weaning and renal support. He was ultimately transferred to rehabilitation for uneventful recovery. Discharge echocardiography revealed good surgical results for the mitral valve and a residual moderate-to severe TR. DISCUSSION: The present case highlights that an endoscopic approach for the treatment of mitral and tricuspid valve pathologies can be used in selected patients with dextrocardia. Tricuspid annuloplasty may reduce but not abolish tricuspid regurgitation in a combination of primary, congenital, and secondary aetiologies.

Background: Pediatric pulmonary vein stenosis (PVS) is a progressive and recurrent condition associated with high morbidity and mortality. Long-term outcomes of individual pulmonary veins remain poorly described. Methods: All pediatric (<18 years) PVS cases diagnosed between September 2005 and January 2020 with a minimum follow-up of 5 years were included. Vein-specific outcomes were assessed using competing-risk survival analysis adjusted for patient death and vein atresia. Veins with recurrent disease maintaining patency for ≥3 years were analyzed longitudinally to characterize reintervention burden as a surrogate for disease trajectory. Results: A total of 244 affected veins in 107 patients were identified. The cumulative incidence of vein loss progressively increased during the first 3 years, followed by plateauing thereafter. Overall, 147 of 244 veins (60%) were lost within 3 years of diagnosis, including 75 veins lost secondary to patient death. Vein attrition was greatest during the first year following diagnosis. Ninety-seven veins remained patent for ≥3 years, including 73 with recurrent disease. Among these veins, the median reintervention rate decreased significantly from 2 (IQR: 1-4) during the first 3 years to 1 per year (IQR: 1-3) thereafter (p < 0.01) while maintaining continued patency. Conclusions: In pediatric PVS, the first 3 years after diagnosis represent a critical period of active disease with high rates of vein attrition. Veins maintaining patency beyond this period demonstrate a reduced reintervention rate, suggesting stabilization of disease progression. Aggressive surveillance and intervention during this therapeutic window may improve long-term vein and patient outcomes.

BACKGROUND: Bovine jugular vein graft is widely used for right ventricular outflow tract reconstruction in congenital heart disease, with a reported median time to conduit exchange exceeding 5 years in infants. The impact of severe postoperative infection on immune-mediated xenograft failure remains incompletely characterized. CASE SUMMARY: A 23-month-old boy with tetralogy of Fallot presented with critical obstruction of the distal pulmonary trunk 5 months after right ventricular outflow tract reconstruction with a 14-mm bovine jugular vein conduit. His postoperative course had been complicated by mediastinitis, which was successfully treated. Despite transcatheter intervention, progressive conduit distal stenosis ultimately required surgical reintervention. The explanted xenograft showed multinucleated giant cells, marked fibrosis, and extensive collagen degradation, consistent with an intense foreign-body inflammatory reaction. Following replacement with a 14-mm valved expanded polytetrafluoroethylene conduit, hemodynamics improved and remained stable on follow-up. DISCUSSION: We discuss the possible role of postoperative Staphylococcus epidermidis mediastinitis in accelerating xenograft degeneration through immune activation. Close surveillance of xenograft function and consideration of alternative conduits made of biologically inert materials may be warranted in high-risk settings. TAKE-HOME MESSAGES: Mediastinitis may function as an immunologically mediated trigger, potentially accelerating xenograft degeneration. For patients at an increased risk of inflammatory or infectious complications, expanded polytetrafluoroethylene conduits may represent a reasonable alternative.