Korean Circ J.  2021 Apr;51(4):360-372. 10.4070/kcj.2020.0331.

Effect of Pulmonary Valve Replacement in the Repaired Tetralogy of Fallot Patients with Trans-annular Incision: More than 20 Years of Follow-up

• Kwak JG
• Shin HJ
• Bang JH
• Kim ER
• Lee JR
• Kim WH
• Bae EJ
• Song MK
• Kim GB

• KMID: 2514641
• DOI: http://doi.org/10.4070/kcj.2020.0331

Abstract

Background and Objectives
We reviewed the long-term outcomes after tetralogy of Fallot (TOF) repair with trans-annular incision; and evaluated the effectiveness of pulmonary valve replacement (PVR) on outcomes.
Methods
This was a retrospective review of clinical outcomes of 180 of 196 TOF patients who underwent total correction with trans-annular incision from 1991 to 1997 (PVR group: 81; non-PVR group: 99).
Results
The median age of the patients was 14.0 months (interquartile range [IQR], 10.7–19.8 months) at TOF repair. Ten in-hospital deaths (5.1%) occurred. During the followup, 81 patients underwent PVR at the median age of 13.5 years (IQR, 11.2–17.1 years). The patients in PVR group showed better outcomes than non-PVR group in overall survival rate (100% in PVR vs. 88.7% in non-PVR, p=0.007), in all adverse events (arrhythmia, neurologic complications, 95.5% in PVR vs. 74.6% in non-PVR, p=0.024) at 20 years. Age at TOF repair younger than 1 year (hazard ratio [HR], 2.265; p=0.01) and previous shunt history (HR, 2.195; p=0.008) were predictive for requiring PVR. During follow-up, 10 late deaths (5 sudden deaths) occurred in the non-PVR group, mainly due to ventricular arrhythmia and right ventricular failure; there was 1 late death (not a sudden death) in the PVR group.
Conclusions
Long-term survival after repair of TOF with trans-annular incision were acceptable. However, arrhythmias were frequently observed during 20 years of follow-up. The patient age <1 year at the time of TOF repair and shunt implantation prior to TOF repair were predictive factors for requiring PVR.

Keyword

Congenital heart disease; Tetralogy of Fallot; Pulmonary regurgitation; Pulmonary valve

Figure

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  Figure 1 Kaplan-Meier curves of (A) overall survival rate, (B) freedom from arrhythmia, (C) freedom from reoperation for PR, (D) freedom from adverse events (arrhythmia, neurologic events (stroke, seizure, hemorrhage).PR = pulmonary regurgitation; PVR = pulmonary valve replacement.

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  Figure 2 Predictive factors for PVR. Kaplan-Meier curves freedom from PVR (A) by prior shunt history and (B) age at TOF repair.PVR = pulmonary valve replacement; TOF = tetralogy of Fallot.

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  Figure 3 Kaplan-Meier curves for (A) overall-survival (B) Freedom from death, arrhythmia, reoperation or neurologic complication between PVR and non-PVR group.PVR = pulmonary valve replacement.

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