Tetralogy of Fallot or Pulmonary Atresia with Ventricular Septal Defect after the Age of 40 Years: A Single Center Study

Hock J, Schwall L, Pujol C, Hager A, Oberhoffer R, Ewert P, Tutarel O.J Clin Med. 2020 May 19;9(5):E1533. doi: 10.3390/jcm9051533.PMID: 32438748 Free article.


Take Home Points


  • Managing the older adult with ACHD is increasingly common in clinical practice but there is limited data available for this particular group
  • This was a retrospective analysis of patients aged >40 years old between 2005 and 2018
  • Combined primary endpoint was death, prevention of sudden cardiac death, aborted sudden cardiac death, pacemaker implant, arrhythmia or new-onset heart failure
  • 184 patients were identified who were aged > 40 years – 86% with tetralogy of Fallot and 14% with pulmonary atresia/VSD
  • Median follow up was 3.1 years (IQR 0.6-6.5 years)
  • The combined primary endpoint was defined as any cause death, prevented sudden death, implanted pacemaker, new onset heart failure or new arrhythmia – this was occurred in 19% (n=35).
  • The combined secondary end point was defined as all cause death or prevented sudden cardiac death – this occurred in 8% (n=13)
  • An acquired co-morbidity was present in over two thirds of patients (n=126) and nearly half of the cohort (n=72) had 2 or more co-morbidities
  • NYHA class (HR 2.1, 95% CI 1.2-3.6, p=0.009) and age were independent risk predictors of the primary outcome in multivariable analysis


Commentary from Dr. Damien Cullington (Liverpool, UK), section editor of ACHD Journal Watch:  Collective efforts to improve the care of patients with ACHD presents new challenges of the care of adults who are increasingly aged. Over the last few decades, these changes in demographics have resulted in the ‘new norm’ in most of our practices. It now means that acquired heart disease needs to be considered increasingly frequently when assessing symptoms. Often overlooked in a busy outpatient clinic, but closer consideration of risk factor modifications to avoid the development of acquired heart disease needs more of our attention i.e. weight management advice, blood pressure control, lipid modification, exercise advice – a non-exhaustive to-do list and this is just for starters!


There is limited data/analysis which has focused primarily on outcomes of the older adult with ACHD. The authors nicely present a table of other comparative analyses to their own (Table 1). Hock et al. identified 184 patients > 40 years old and followed up at the German Heart Centre over a 15-year period (mean age 45.3 ± 7.2 years). Surgical repairs were performed between 1958 and 2000 with a mean age of repair at 10 ± 7.8 yrs. The primary combined endpoint was classified as the occurrence of a major cardiovascular event and the secondary endpoint as either all cause death or aborted SCD. All patients had previously undergone total corrective repair – patients with a ‘palliative repair’ or who had not undergone surgery (n=15) were excluded from the final analysis (n-169) although it would have been of interest to the reader understand the outcomes of this minority cohort too. The baseline demographics of the cohort are shown in Table 2.


Table 1. Hock et al. study data vs comparable datasets



Table 2. Patient demographics at baseline


In 2005, 31 patients were aged >40 years old, increasing to 93 patients in 2017. Approximately 80% of patients included (n=149) were aged in the range 40-50 years. LV and RV function were qualitatively assessed – 78% had normal RV function and 92% normal LV function.


Patient co-morbidities are listed in Table 3. This cohort had high rates of co-incident co-morbidity – only about a third of patients had none (n=58). Of interest, 78 patients (n=42%) had at least one interventional procedure after their 40th birthday. Similarly, 48 patients (26%), had an EP procedure. Almost a third of patients (n=55) had experienced arrythmias – mostly atrial.


Table 3 – Co-morbidity prevalence

Primary and Secondary outcomes


During a median follow up of 3.1 years (IQR 0.6-6.5 years), 3 patients died. The primary endpoint occurred in 35 patients – death (n=3), prevented sudden cardiac death (n=10); pacemaker implant (n=9); new onset heart failure (n=8) and clinically significant arrhythmias (n=5).


Univariable and multivariable predictors of primary endpoint are shown in Table 4.


Table 4  – Univariable and multivariable analyses




This analysis of older patients with TOF or PA/VSD showed that acquired co-morbidities are common and likely to be increasingly so as the individual ages. Although co-morbidity of any kind was not an independent risk predictor of outcome, this is perhaps a reflection of the duration of follow up – some co-morbidities require decades to manifest pathologically. Assessment of primary prevention risk factors as part of a holistic strategy to modify risk needs to be made more systematically than is likely to be done at present – one hopes that modification will translate into better longer term outcomes. This is something which needs closer attention by all of us involved in the care of ACHD patients – too often this takes a backseat to our concentrated focus on the gross anatomy.