| Autor: |
Nair, Ashvin Krishna, Haranal, Maruti, Elkhatim, Ibrahim Mukhtar, Dillon, Jeswant, Chee Chin Hew, Sivalingam, Sivakumar |
| Předmět: |
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| Zdroj: |
Annals of Pediatric Cardiology; Jul-Sep2020, Vol. 13 Issue 3, p212-219, 8p |
| Abstrakt: |
Background: Absent pulmonary valve syndrome (APVS) is a variant of tetralogy of Fallot characterized by aneurysmal pulmonary arteries, which compresses the tracheobronchial tree, leading to respiratory symptoms. We report the mid term outcomes of surgical correction of patients with APVS. Subject and Methods: A total of 27 patients underwent surgery between 2001 and 2015, and they were followed up for a mean period of 6.4 ± 4.1 years. Out of the 27 patients, 14 (51.9%) were infants. The median age at repair was 9.8 months. Preoperative intubation was required in six patients (22.2%), and 11 patients (40.7%) had symptoms of respiratory distress. The pulmonary valve was replaced with a valved conduit in 15 patients (55.6%), monocusp valve in 6 patients (22.2%), and a transannular patch in 6 patients (22.2%). Reduction pulmonary arterioplasty was done in all patients. Results: The overall 10 year survival was 82.1%. There was 81.1% overall freedom from re intervention at 10 years. No statistically significant difference was found in 10-year survival (P = 0.464) and reoperation rates (P = 0.129) between valved conduit, monocusp, or transannular patch techniques. Older children had statistically significantly longer survival (P = 0.039) and freedom from re intervention (P = 0.016) compared to infants. Patients without respiratory complications had 100% 10 year survival and 93.3% freedom from reoperation at 10 years compared to 55.6% and 60.1%, respectively, for patients with respiratory complications. Conclusion: There has been improvement in surgical results for APVS over the years. However, it still remains a challenge to manage infants and patients with persistent respiratory problems. [ABSTRACT FROM AUTHOR] |
| Databáze: |
Complementary Index |
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