先天性肺静脈狭窄症

BACKGROUND: Uncorrected adults congenital heart disease (ACHD) may present with profound hypoxia and dual risks of thrombosis and bleeding, posing major challenges for definitive surgical repair. In such settings, right ventricular outflow tract (RVOT) stenting may provide a life-saving palliative option. CASE PRESENTATION: We report a 30-year-old woman with double-outlet right ventricle, subaortic ventricular septal defect, and severe infundibular pulmonary stenosis who suffered cardiac arrest due to profound hypoxia. Her clinical profile was complicated by polycythemia, upper extremity deep vein thrombosis, and active gastrointestinal bleeding. In light of prohibitive surgical risk, urgent RVOT stenting was undertaken, requiring multiple technical adaptations due to severe obstruction. Post-procedure, oxygen saturation improved to 92%, gastrointestinal bleeding ceased, and deep vein thrombosis regressed without anticoagulation. She subsequently completed phase II cardiac rehabilitation with improved functional capacity. CONCLUSION: Urgent RVOT stenting can serve as a feasible therapeutic option in high-risk ACHD patients, effectively restoring pulmonary blood flow while simultaneously stabilizing thrombotic and bleeding complications.

A six-year-old neutered male cat was presented for acute respiratory distress. Transthoracic echocardiography identified a left atrial membrane consistent with cor triatriatum sinister (CTS) and turbulent flow across a single fenestration. Additionally, it was suspected that the distal chamber may receive at least one pulmonary vein. Because respiratory signs improved with corticosteroids but not with a short diuretic trial, and because echocardiography suggested that the distal chamber might receive at least one pulmonary vein, cardiac computed tomography angiography was performed to further characterize the pulmonary venous anatomy and to differentiate CTS from pulmonary vein stenosis and other congenital vascular malformations. Cardiac computed tomography angiography confirmed left atrial subdivision with one interchamber orifice (5.33 mm) and demonstrated a pulmonary venous connection pattern in which three pulmonary veins connected to the proximal chamber, whereas the left cranial pulmonary vein connected to the distal chamber, which was continuous with the mitral valve. Adapting the human Lucas classification scheme, the anatomic configuration most closely resembled Lucas type C1a, and no congestive heart failure attributable to CTS was identified.

Pediatric pulmonary vein stenosis (PVS) is a rare, progressive condition associated with significant morbidity and mortality. While pulmonary venous decompressing collaterals (PVDC) have been described, their clinical relevance and impact on long-term outcomes remain unstudied. We aimed to characterize these collaterals and determine their association with outcomes and mortality in pediatric PVS. This was a single-center, retrospective cohort study of 64 pediatric patients (≤ 18 years) treated for PVS between 2017 and 2022. Patients with single-ventricle heart disease were excluded. PVDC were defined as contrast flow to a neighboring lobar or interlobar segment due to medial occlusion identified on serial angiograms. The primary outcome was PVS-related mortality analyzed using multivariable Cox proportional hazards models, adjusting for age and gestational age. PVDC were present in 39/64 (61%) patients. The presence of collaterals was significantly associated with lower all-cause (15% vs. 40%, p = 0.027) and PVS-related mortality (13% vs. 40%, p = 0.012). This benefit was most pronounced in the bilateral disease subgroup (45% of the cohort). In this group, patients with collaterals had significantly lower PVS-related mortality (15% vs. 78%, p = 0.002) and lower indexed pulmonary vascular resistance (iPVR 3.2 vs. 7.6 Wood unit x m2, p = 0.006). In the adjusted Cox model, collateral presence in bilateral PVS was strongly associated with improved survival (HR 0.09, 95% CI: 0.02-0.46, p = 0.003). In conclusion, pulmonary venous decompressing collaterals are a favorable adaptive mechanism in pediatric PVS. Their presence is associated with improved survival, particularly in high-risk patients with bilateral disease. These findings emphasize the importance of integrating collateral status into prognostic assessment and therapeutic decision-making.

Polytetrafluoroethylene (PTFE) grafts are the most widely used conduits for systemic-to-pulmonary artery shunts in pediatric patients with congenital heart disease requiring supplemental pulmonary blood flow, yet they carry well-documented risks of thrombosis, neointimal hyperplasia, and seroma formation. Saphenous vein homografts (SVH), available as cryopreserved or antibiotic-preserved preparations, have emerged as a biological alternative, but no prior meta-analysis has compared these conduit types. We systematically searched PubMed, Scopus, Web of Science, and Google Scholar from inception through 2026, identifying 16 studies (3 comparative, 13 single-arm, and 684 patients). Double-arm meta-analysis employed fixed-effect risk ratios (RR) with generalized linear mixed model sensitivity analysis; single-arm proportional meta-analysis used Freeman-Tukey double arcsine transformation. In the double-arm analysis (235 patients), SVH was associated with significantly lower early shunt thrombosis compared to PTFE [RR 0.29; 95% confidence interval (CI): 0.08-0.96; P = 0.04; I2 = 0.0%), confirmed by sensitivity analysis (odds ratio 0.25; P = 0.03). No significant differences were observed in overall mortality (RR 1.00; 95% CI: 0.44-2.24), infection (RR 0.68; 95% CI: 0.05-9.13), or catheter-based reintervention (RR 0.86; 95% CI: 0.52-1.42). Single-arm analyses showed similar early mortality (SVH 9% vs PTFE 11%) and stenosis rates (both 8%). GRADE certainty was very low to low for all outcomes. SVH is associated with significantly reduced early shunt thrombosis without increased mortality or reintervention; however, the low certainty of evidence underscores the need for adequately powered multicenter randomized trials with standardized perioperative protocols.

Heart valve abnormalities are present in 30% of congenital heart defects. Additionally, there are acquired conditions like rheumatic heart disease. They often require surgery, and in complex cases, valve replacement becomes unavoidable. The current valve replacement options, either mechanical, bioprosthetic, homografts, or autografts, lack key properties for pediatric patient needs: availability, durability, and the ability to grow. Our goal is to review 2 clinically relevant innovations: tissue-engineered heart valves (TEHVs) and partial heart transplantation (PHT). We conducted searches in electronic databases covering the past 25 years using the terms "partial heart transplantation," "tissue-engineered heart valves," and their combinations. Decellularized pulmonary homografts outperform cryopreserved homografts and bovine jugular vein conduits in pulmonary valve replacement, showing less stenosis and no infective endocarditis. The European Clinical Study for the Application of Regenerative Heart Valves trial confirmed decellularized pulmonary homograft safety, demonstrating 97.5% freedom from explantation at 5 years. Decellularized aortic homografts showed excellent 97.8% 5-year survival and low endocarditis rates, though increases in transvalvular gradient and regurgitation occurred. Partial heart transplantation uses living valves from donor hearts and accommodates somatic growth. Postoperative immunosuppression in PHT is required but may be limited, as children and valve tissues may possess immune privilege, as evidenced by limited valve injury even in failed orthotopic heart transplants. In a pilot study of 19 infants, with a median follow-up of 26 weeks, increases in annular diameter and leaflet length were observed. Both PHT and TEHVs are promising valve replacement options. While PHT provides a growth-adaptive capability, TEHVs hold the potential for off-the-shelf solutions with minimal immunogenicity.