Outcomes of total anomalous pulmonary venous drainage repair in neonates and the impact of pulmonary hypertension on survival.
JTCVS Open
; 12: 335-343, 2022 Dec.
Article
en En
| MEDLINE
| ID: mdl-36590732
ABSTRACT
Background:
Mortality after repair of total anomalous pulmonary venous drainage (TAPVD) in neonates has remained high. Analysis of risk factors may help identify therapeutic targets to improve survival.Methods:
Retrospective analysis of all neonates who underwent simple TAPVD repair.Results:
Between 1973 and 2021, 175 neonates underwent TAPVD repair, at a median age of 6 days (interquartile range, 2-15 days) and a mean weight of 3.2 ± 0.6 kg. TAPVD was supracardiac in 42.3% of the patients (74 of 175), cardiac in 14.3% (25 of 175), infracardiac in 40% (70 of 175), and mixed type in 3.4% (6 of 175), with obstruction in 65.7% (115 of 175). Pulmonary hypertension (PHT) crisis occurred in 12% (21 of 175). Early mortality was 9.7% (17 of 175) and late mortality was 5.1% (8 of 158), with most deaths occurring within 1 year (75%; 6 of 8). Survival was 86.5% (95% CI, 80.3%-90.8%) at 1 year and 85.8% (95% CI, 79.6%-90.3%) at 5, 10, 15, and 20 years. Survival was lower in patients with obstructed TAPVD, patients with emergent surgery, and those with PHT crisis. PHT crisis (hazard ratio [HR], 4.93; 95% CI, 1.95-12.51; P = .001), urgency of surgery (HR, 2.51; 95% CI, 1.11-5.68; P = .027), and higher pulmonary artery pressure-to-systemic blood pressure percentage ratio (HR, 1.06; 95% CI, 1.01-1.11; P = .026) were identified as risk factors for mortality. Histopathological analysis of 17 patients (9.7%; 17 of 175) showed signs of pulmonary arterial hypertension with media hypertrophy in 58.8% (10 of 17).Conclusions:
Mortality after TAPVD repair occurred mainly within the first year of life. Urgency of surgery and persistent PHT appears to be risk factors for mortality. Lung biopsy might be useful for identifying patients at risk and guiding newer treatment modalities.
ECMO, extracorporeal membrane oxygenation; HI, hemodynamic index; HR, hazard ratio; IQR, interquartile range; MAP, mean systemic arterial pressure; PAH, pulmonary arterial hypertension; PAP, mean pulmonary arterial pressure; PHT, pulmonary hypertension; TAPVC; TAPVD; TAPVD, total anomalous pulmonary venous drainage; TAPVR; neonatal repair; pulmonary arterial hypertension; total anomalous pulmonary venous connection
Texto completo:
1
Colección:
01-internacional
Base de datos:
MEDLINE
Tipo de estudio:
Prognostic_studies
/
Risk_factors_studies
Idioma:
En
Revista:
JTCVS Open
Año:
2022
Tipo del documento:
Article
País de afiliación:
Australia