The role of surgical therapy for pulmonary vein atresia in childhood.

UNLABELLED: The outcome for children with pulmonary vein atresia has been historically poor. This report describes clinical outcomes after surgical treatment of primary and secondary pulmonary vein atresia. All patients undergoing surgery for pulmonary vein atresia at Children's Hospital Boston from 1 January 2005 to 1 January 2009 were identified. The data available for analysis included demographic characteristics, findings based on cardiac catheterization and other imaging, clinical information, operative surgical intervention, and pulmonary vein histology. The study-specific information included the calculated number of atretic pulmonary veins as well as surgical interventions performed during the study period. The study end points included successful relief of pulmonary vein atresia, recurrence of pulmonary atresia or stenosis, overall pulmonary vein disease progression, and patient survival. A total of 16 patients with one or more pulmonary vein atresias were identified in the cardiac surgery registry database. Of these 16 patients, 5 had primary pulmonary atresia, and 11 had secondary atresia after repair of anomalous pulmonary venous drainage. Surgical recanalization of atretic pulmonary veins was achieved for more than 80% of the patients. At the most recent follow-up assessment, 7 (44%) of the 16 patients had recurrence of atresia. Five of the patients had development of new atresia in other previously healthy pulmonary veins. The median follow-up period was 4 months for primary and 17 months for secondary pulmonary vein atresia. The three main predictors of pulmonary vein disease progression after surgery were the presence of underlying lung disease (P = 0.036), prematurity (P = 0.035), and a history of recurrent pulmonary vein atresia. The overall mortality rate for the patients with primary or secondary pulmonary vein atresia was 56% at the most recent follow-up assessment (100% for the patients with primary vein atresia and 36% for the patients with secondary vein atresia). CONCLUSIONS: Surgical recanalization of pulmonary vein atresia is possible, but prognosis remains poor. Recurrence of atresia can take place as early as a few months in the presence of underlying lung disease, prematurity, or history of recurrent pulmonary vein atresia. Further research is needed to identify mechanisms for attaining and sustaining pulmonary vein patency after surgery.

Biventricular repair in patients with heterotaxy syndrome.

OBJECTIVE: Complex intracardiac and extracardiac anatomy is often confronted during biventricular repair in patients with heterotaxy syndrome. We examined factors affecting surgical outcomes in these patients. METHODS: Between January 1990 and July 2007, 371 patients received a diagnosis of heterotaxy syndrome; 91 (91/371, 24.5%) underwent biventricular repair. Left atrial isomerism was present in 73% (66/91) and right atrial isomerism in 10% (9/91), with indeterminate atrial anatomy in 17% (16/91). Median age at biventricular repair was 6.8 months (5 days to 22.3 years). Systemic venous anomalies were present in 75 patients, pulmonary venous anomalies in 26, and endocardial cushion defects in 36. Transposition complexes were present in 15 patients with atrioventricular discordance in 10; 8 underwent double switch, 2 received a physiologic repair, 2 underwent arterial switch, and 3 underwent the Rastelli operation. Other conotruncal anomalies included double-outlet right ventricle in 10 patients, tetralogy of Fallot in 3, and hemitruncus in 2. Separation of systemic from pulmonary venous return included intra-atrial baffling in 48 patients and extracardiac grafting in 2. Combined lesions were common, occurring in 99% (90/91). Statistical analysis with Kaplan-Meier and Cox proportional hazards models were performed. RESULTS: Average follow-up was 44.9 +/- 57.5 months (3 days to 189.3 months). Kaplan-Meier estimated survival was 93.4% at 10 years; unbalanced complete atrioventricular canal was the only risk factor for mortality (P = .006). Subsequent procedures were common with a 10-year freedom from reoperation or reintervention of 38% +/- 7.5%. Arrhythmias occurred in 36 (39.6%) patients; bradyarrhythmia in 27 (29.7%) and tachyarrhythmia in 15 (16.5%). Freedom from any arrhythmia was 53.9% +/- 6.7% at 10 years. CONCLUSIONS: Excellent survival for patients with heterotaxy undergoing biventricular repair can be expected, even for multiple, complex lesions. Reintervention is common, and arrhythmia is a long-term concern. This experience shows that patients with heterotaxy syndrome and complex cardiac anatomy can be considered for biventricular repair. Patients with unbalanced complete atrioventricular canal are a high-risk group for which selection criteria are particularly important.

Quantitative analysis of the degree of irreversible deformation of F cells and non-F cells and its relationship to cell density in sickle cell disease.

To study the effect of fetal hemoglobin (Hb F) on the formation of dense and irreversibly deformed cells in patients with sickle cell disease (SCD), we investigated the distribution of Hb F and Hb F cells (FC, cells having HbF) and the difference in the degree of irreversible deformation between FC and non-Hb F cells (NFC) in relation to their cell density. We used immunofluorescence to define FC and image analysis to quantify the degree of deformation. Blood samples from 16 patients with Hb F levels ranging from 4 to 24% were separated into less dense [top (T), d < 1.11 g/mL] and dense [bottom (B), d > or = 1.11 g/mL] fractions. We found higher percentages of Hb F and FC in top fractions, suggesting that FC are hydrated more than NFC. Quantitative shape analysis demonstrated that the irreversible deformation of FC in the top fraction [FC(T)] was not significantly different from that of NFC in the fraction [NFC(T)] (p = 0.163), but rather that the irreversible deformation of FC in the bottom fraction [FC(B)] was much milder than for NFC(B) (p < 0.001). The order of the degree of irreversible deformation was NFC(B) > FC(B) > NFC(T) = FC(T). These results clearly demonstrated that FC are less susceptible to both irreversible deformation and dehydration than NFC, suggesting that in circulation, the sickling process plays a major role in cell dehydration. Additionally, we found that the ratio of NFC(B) to FC(B) decreased as a patient's Hb F increased (r = 0.84) and that NFC(B) = FC(B) when Hb F was about 20%.