Cardiovascular Morbidity and Mortality in Adult Patients With Repaired Aortic Coarctation

Meijs TA, Minderhoud SCS, Muller SA, de Winter RJ, Mulder BJM, van Melle JP, Hoendermis ES, van Dijk APJ, Zuithoff NPA, Krings GJ, Doevendans PA, Witsenburg M, Roos-Hesselink JW, van den Bosch AE, Bouma BJ, Voskuil M.J Am Heart Assoc. 2021 Nov 16;10(22):e023199. doi: 10.1161/JAHA.121.023199. Epub 2021 Nov 10.PMID: 34755532


Take Home Points:

  • Cardiovascular events are common in repaired Coarctation of aorta (21% in this cohort)
  • Arrhythmias and Aortic complications were most frequent cardiovascular events
  • Risk of cardiovascular events increased with age 0.8% at 20 years to ~ 8% at 70 years
  • Older age at repair and elevated LV mass index are associated with increased risk of cardiovascular events
  • All-cause mortality was 3.3 times higher than the matched Dutch general population





Commentary from Dr. Shailendra Upadhyay (Connecticut, USA), section editor of ACHD Journal Watch:

Coarctation of aorta (CoA) is one of the common CHDs. Long term issues include cardiovascular complications in majority, yet the knowledge of exact burden of cardiovascular disease in adult CoA is limited. Enrolled patients (n=920) cohort consisted of five tertiary referral centers in Netherlands, participants of the CONCOR prospective registry between 2002 and 2018 were included. All patients with surgical or trans-catheter CoA repair were included but univentricular circulation and transposition of the great arteries.


Cardiovascular events included: CAD, stroke or TIA, thoracic aortic complications (aneurysm or dissection), arrhythmia, heart failure hospitalization endocarditis and death.



Among 920 patients followed for a mean of 90.3 ± 5.1 years, 21% (191) developed cardiovascular events. Arrhythmia and aortic complications were most frequent cardiovascular events. A substantially higher incidence of events was observed in patients with the presence of a bicuspid aortic valve, mechanical heart valve (n= 60; 49 aortic, 5 mitral and 6 both), and/or Turner syndrome.


Overview of cardiovascular events



In univariate analysis, older age at CoA repair, bicuspid aortic valve increased BMI and elevated LV mass index [LVH defined on echocardiography as LV mass index > 115 G/m2 in Males and > 95 G/m2 in females] and were associated with increased risk, whereas female gender was associated with decreased risk. In multivariate analysis LV mass index and older age at repair (>10 years) were identified as independent risk factors for the occurrence of cardiovascular events.


Estimated early risk of cardiovascular events:



Factors associated with risk of cardiovascular events



LVH and Cardiovascular Events



Mortality occurred in 42 patients with 57% (24) attributed to cardiovascular events. Mortality was 3.3 times higher than the age, sex matched Dutch general population. It remained higher than general population even for patients with isolated CoA without other congenital heart defects or mechanical valves.


Overall Survival




Cardiovascular events are common after CoA repair. While aortic complications and arrhythmias are the most common cardiovascular events, late age at repair, and elevated LV mass index are independent risk factors. There is 3.3 fold mortality increase after CoA repair when compared to the matched general population. There data support preventive strategy implementations.