Long-Term Survival and Unnatural Deaths of Patients With Repaired Tetralogy of Fallot in an Asian Cohort

Abstract

Background— Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease in Taiwan. This study investigates the long-term survival and risks of TOF in an Asian cohort. Methods and Results— This study enrolled 819 consecutive patients with TOF (61.1% male), who received total correction between 1970 and 2002, as participants. Patient medical records were reviewed, and the survival status of those out of contact was confirmed by death records retrieved from the National Health database. The mean (±SD) patient age at cardiac repair was 6.5±7.6 years, and a prior shunt operation was performed in 119 (14.5%) of the patients. At cardiac repair, a transannular patch for right ventricle outlet reconstruction was required in 444 (54.2%) of the patients. After 13 808 patient-years of follow-up, the 30-year survival rate was 90.5%. The annual mortality rate increased from 0.123% in the initial 15 years after repair to 0.395% thereafter ( P <0.05). The presence of major aortopulmonary collateral arteries, older operative age, and previous shunt operation are independent risks of late cardiac deaths. Secondary to cardiac mortality, unnatural deaths (accident and suicide) accounted for 27.6% of late deaths, significantly higher compared with that of the general population (odds ratio, 2.18; P =0.028). Conclusions— In this Asian TOF cohort, except for a late decrease after 15 years, long-term survival after cardiac repair was satisfactory. Although cardiac death was the most common cause of late death, accidents or suicide may also be associated with late mortality, suggesting a potential role for psychosocial support.

Arrhythmic burdens in patients with tetralogy of Fallot: A national database study

“…2,3 The first cardiac repair of TOF was successfully performed in 1955 in the United States and in 1965 in Taiwan. 4,5 According to our institutional 30-year database comprising 819 TOF patients, acute surgical mortality was 3.3% and 30-year survival was 90.5%, which is considerably close to that reported in recent Western studies. [5][6][7] However, a substantial number of patients remain at risk for arrhythmias and sudden death during long-term follow-up.…”

“…4,5 According to our institutional 30-year database comprising 819 TOF patients, acute surgical mortality was 3.3% and 30-year survival was 90.5%, which is considerably close to that reported in recent Western studies. [5][6][7] However, a substantial number of patients remain at risk for arrhythmias and sudden death during long-term follow-up. 5,[8][9][10][11] The burden of arrhythmias in TOF patients varies with the duration of postoperative follow-up and the study population.…”

“…[5][6][7] However, a substantial number of patients remain at risk for arrhythmias and sudden death during long-term follow-up. 5,[8][9][10][11] The burden of arrhythmias in TOF patients varies with the duration of postoperative follow-up and the study population. Based on an adult cohort from the Alliance of Adult Research of Pediatric Cardiology, the arrhythmia burden was as high as 43%.…”

In‐Hospital Vital Status and Heart Transplants After Intervention for Congenital Heart Disease in the Pediatric Cardiac Care Consortium: Completeness of Ascertainment Using the National Death Index and United Network for Organ Sharing Datasets

“…Subsequent contemporary reports have been small and/or limited to single surgical centers. [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15] Hence, the Centers for Disease Control and Prevention has identified linkage of databases to assess the long-term outcomes of persons with CHD as filling a critical gap in knowledge. 16,17 As the oldest, and one of the largest, registries of CHD in the United States, the Pediatric Cardiac Care Consortium (PCCC) is uniquely placed to address the long-term outcomes of these patients.…”

Late arrhythmia in patients with repaired tetralogy of Fallot: who is at risk?

“…Patients who undergo repair of TOF present with a survival rate of approximately 90% after 30 years postoperatively [4,5]. Late mortality rate varies with age, however, it significantly rises at 25 years after the operation from 0.24%/y to 0.94%/y [6].…”