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Surgical outcomes in the treatment of patients with tetralogy of Fallot and absent pulmonary valve.
Eur J Cardiothorac Surg. 2007 Mar; 31(3):354-9; discussion 359.EJ

Abstract

OBJECTIVE

Tetralogy of Fallot and absent pulmonary valve (TOF/APV) is associated with significant pulmonary artery dilatation and airway compression. Treatment of infants presenting with respiratory symptoms early in life is associated with high mortality (20-60%). We aim to report our results and identify factors associated with survival and prolonged ventilation.

METHODS

We performed a retrospective review of 62 consecutive patients following repair of TOF/APV (1982-2006). Median age at repair was 1.4 years (1 day-35 years). Twenty patients required preoperative intubation.

RESULTS

Sixty-one patients underwent complete repair. Thirty-three patients underwent pulmonary artery plication (n=15) or reduction (n=18). The right ventricular outflow tract (RVOT) was reconstructed with valved conduit (n=31), bioprosthetic valve (n=18), monocusp (n=8), or transannular patch (n=4). There were three perioperative and five late deaths. All perioperative deaths were in neonates and before 1995. Five- and ten-year survival was 93+/-4% and 87+/-5%. Mean ventilatory requirements for neonates, infants, and children > or =1 year were 36+/-35, 8+/-8, and 2.6+/-2.4 days (p<0.0001). On multivariable analysis, significant factors associated with prolonged ventilation were neonates (p<0.0001) and preoperative mechanical ventilation (p=0.088). Eight airway reinterventions were needed in seven infants with persistent postoperative airway compromise, pulmonary artery suspension (n=4), innominate artery suspension (n=2), and lobectomy (n=2). Freedom from RVOT reoperation was 89+/-5% and 59+/-9% at 5 and 10 years. There were no significant risk factors for time-related survival or RVOT reoperation on multivariable analysis.

CONCLUSIONS

In contrast to children and adults with TOF/APV, neonates and small infants presenting with respiratory symptoms require prolonged ventilation and additional reinterventions for airway compression. Our current surgical approach which includes reduction and suspension of pulmonary arteries, reconstruction of a competent RVOT, and aggressive postoperative ventilatory management to relieve airway obstruction offers satisfactory outcomes.

Authors+Show Affiliations

The Cardiac Centre, Hospital for Sick Children and the University of Toronto, Toronto, Ontario, Canada. balsoufi@hotmail.comNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article

Language

eng

PubMed ID

17215132

Citation

Alsoufi, Bahaaldin, et al. "Surgical Outcomes in the Treatment of Patients With Tetralogy of Fallot and Absent Pulmonary Valve." European Journal of Cardio-thoracic Surgery : Official Journal of the European Association for Cardio-thoracic Surgery, vol. 31, no. 3, 2007, pp. 354-9; discussion 359.
Alsoufi B, Williams WG, Hua Z, et al. Surgical outcomes in the treatment of patients with tetralogy of Fallot and absent pulmonary valve. Eur J Cardiothorac Surg. 2007;31(3):354-9; discussion 359.
Alsoufi, B., Williams, W. G., Hua, Z., Cai, S., Karamlou, T., Chan, C. C., Coles, J. G., Van Arsdell, G. S., & Caldarone, C. A. (2007). Surgical outcomes in the treatment of patients with tetralogy of Fallot and absent pulmonary valve. European Journal of Cardio-thoracic Surgery : Official Journal of the European Association for Cardio-thoracic Surgery, 31(3), 354-9; discussion 359.
Alsoufi B, et al. Surgical Outcomes in the Treatment of Patients With Tetralogy of Fallot and Absent Pulmonary Valve. Eur J Cardiothorac Surg. 2007;31(3):354-9; discussion 359. PubMed PMID: 17215132.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Surgical outcomes in the treatment of patients with tetralogy of Fallot and absent pulmonary valve. AU - Alsoufi,Bahaaldin, AU - Williams,William G, AU - Hua,Zhongdong, AU - Cai,Sally, AU - Karamlou,Tara, AU - Chan,Chee Ching, AU - Coles,John G, AU - Van Arsdell,Glen S, AU - Caldarone,Christopher A, Y1 - 2007/01/09/ PY - 2006/09/01/received PY - 2006/11/14/revised PY - 2006/12/04/accepted PY - 2007/1/12/pubmed PY - 2007/7/12/medline PY - 2007/1/12/entrez SP - 354-9; discussion 359 JF - European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery JO - Eur J Cardiothorac Surg VL - 31 IS - 3 N2 - OBJECTIVE: Tetralogy of Fallot and absent pulmonary valve (TOF/APV) is associated with significant pulmonary artery dilatation and airway compression. Treatment of infants presenting with respiratory symptoms early in life is associated with high mortality (20-60%). We aim to report our results and identify factors associated with survival and prolonged ventilation. METHODS: We performed a retrospective review of 62 consecutive patients following repair of TOF/APV (1982-2006). Median age at repair was 1.4 years (1 day-35 years). Twenty patients required preoperative intubation. RESULTS: Sixty-one patients underwent complete repair. Thirty-three patients underwent pulmonary artery plication (n=15) or reduction (n=18). The right ventricular outflow tract (RVOT) was reconstructed with valved conduit (n=31), bioprosthetic valve (n=18), monocusp (n=8), or transannular patch (n=4). There were three perioperative and five late deaths. All perioperative deaths were in neonates and before 1995. Five- and ten-year survival was 93+/-4% and 87+/-5%. Mean ventilatory requirements for neonates, infants, and children > or =1 year were 36+/-35, 8+/-8, and 2.6+/-2.4 days (p<0.0001). On multivariable analysis, significant factors associated with prolonged ventilation were neonates (p<0.0001) and preoperative mechanical ventilation (p=0.088). Eight airway reinterventions were needed in seven infants with persistent postoperative airway compromise, pulmonary artery suspension (n=4), innominate artery suspension (n=2), and lobectomy (n=2). Freedom from RVOT reoperation was 89+/-5% and 59+/-9% at 5 and 10 years. There were no significant risk factors for time-related survival or RVOT reoperation on multivariable analysis. CONCLUSIONS: In contrast to children and adults with TOF/APV, neonates and small infants presenting with respiratory symptoms require prolonged ventilation and additional reinterventions for airway compression. Our current surgical approach which includes reduction and suspension of pulmonary arteries, reconstruction of a competent RVOT, and aggressive postoperative ventilatory management to relieve airway obstruction offers satisfactory outcomes. SN - 1010-7940 UR - https://www.unboundmedicine.com/medline/citation/17215132/Surgical_outcomes_in_the_treatment_of_patients_with_tetralogy_of_Fallot_and_absent_pulmonary_valve_ L2 - https://academic.oup.com/ejcts/article-lookup/doi/10.1016/j.ejcts.2006.12.001 DB - PRIME DP - Unbound Medicine ER -