Development of a double-chambered right ventricle after repair of tetralogy of Fallot

Abstract

Objectives: We sought to determine the frequency, etiology and progressive nature of midcavity obstruction in patients after primary repair of tetralogy of Fallot (TOF).

Background: Midcavity obstruction (double-chambered right ventricle [DCRV]) represents a significant portion of reoperations in patients who have had TOF repair. This group is still poorly defined.

Methods: A retrospective review of clinical, echocardiographic and catheterization data for all patients with TOF who later underwent reoperation for DCRV was performed.

Results: Between 1973 and 1995, 552 children <2 years of age underwent primary TOF repair (median age 6.7 months). Long-term follow-up (median 50 months) was available in 308 children. Of these, 17 children subsequently developed DCRV requiring reoperation. The median age at initial operation was 7.9 months. During a median follow-up interval of 43.2 months, murmur intensity increased in all patients, and the average subpulmonary gradient at catheterization increased from 24+/-10 to 80+/-27 mm Hg in seven children (p = 0.002) and at Doppler echocardiography from 14+/-16 to 89+/-18 mm Hg in five children (p = 0.002). Before reoperation, 6 of the 17 children were symptomatic. During the operation (median age 55.4 months), obstruction was relieved by incision of hypertrophied anomalous muscle bundles in all 17 patients, with prominent fibrosis noted in 8 patients. Excessive septal and parietal hypertrophy was noted in one child. No new transannular patches were required. Recurrent obstruction has reappeared in 3 of these 17 children during follow-up.

Conclusions: DCRV is a medium-term complication of TOF repair in infants, with a minimal incidence of 3.1% (95% CI 1.8% to 4.9%). The condition is progressive and is due to anomalous muscle bundle hypertrophy or fibrosis, or both, which may represent displaced insertion of a moderator band. Further reobstruction does occur; continued careful follow-up is therefore essential.