Long-term mortality and cardiovascular burden for adult survivors of coarctation of the aorta.
Lee MGY, Babu-Narayan SV, Kempny A, Uebing A, Montanaro C, Shore DF, d’Udekem Y, Gatzoulis MA.
Heart. 2019 Mar 28. pii: heartjnl-2018-314257. doi: 10.1136/heartjnl-2018-314257. [Epub ahead of print]
Take Home Points:
- Large retrospective, single-centre analysis of the contemporary outcomes of patients following coarctation (CoA) repair.
- Initial cohort comprised 834 patients aged >16 years old.
- The median age of repair was 4 yrs. old (95% CI 1 month-15 years old).
- In 83% (n=690) repair was surgical and the remainder, percutaneously.
- 60% of surgical repairs were performed pre 1990 and 58% were end-to end anastomotic type.
- Median follow up 27 years. Deaths occurring >30 days (‘late’) after CoA repair occurred in 5% of patients (n=38).
- Almost a third (n=246) needed >an aortic arch intervention (n=234); 111 required an aortic valve intervention and 43 needed an ascending aortic intervention.
- In 59% of patients, (n=284/474) a residual pressure drop (>25mmHg) or repair site/diaphragm ratio < 70% was seen at latest follow up.
- A high burden of hypertension in the cohort – 70% of patients with available data were hypertensive (n=379/661) or prehypertensive (83/661).
- Survival in patients with a history of CoA was 99%, 88% and 65% at 30, 50 and 70 years of age – the standardised mortality ratio was 3.20 compared to age/sex matched normal subjects.
Commentary by Dr. Damien Cullington (Leeds to Liverpool), section editor of ACHD Journal Watch: Patients with coarctation of the aorta (CoA) are common attendees in the ACHD outpatient clinic and so they will continue to be so with ever increasing frequency as the congenital cardiac population ages. Although complications can certainly ensue, if patients are managed assiduously and in line with guidelines/good practice, many complications may be treated and potentially avoided. This was a large retrospective review from the UKs largest ACHD centre but variations in outcome will occur even between centres depending on the baseline complexity/comorbidity of the cohort and interventions performed and their ‘success’. Previously, dire outcomes (i.e. >25% mortality) within 3 decades of CoA repair have been reported. This study reappraises matters and contemporarily reports on current survival and other secondary outcomes.
The patient characteristics are shown in table 1. At latest follow up, 796 patients were alive – median age 33 years. The median age of CoA repair was 4 years old which is explained by a heterogeneous cohort of patients presenting at variable times with their CoA. Median age at surgical repair was relatively late compared to present day – only 36% (n=304) had CoA repair in the first year of life. A third of patients (n=276) were operated on in the very early surgical era (1947-79) which will impact on outcomes due to other covariates not accounted for in the analysis. End-to-end anastomotic repair was performed in 58% (n=397). Median follow up was 27 years (18-36 years) from repair.
Of those with available data (n=661), over half of patients (n=375) who were alive and under follow up had hypertension, albeit when measured in the clinic setting. Of all patients alive, over a third are prescribed antihypertensive medication (289/796) but it seems only a minority treated attain normotension.
38 patients have died with a median age of 46 years old (37-62 years). The commonest cause of demise was LV failure (n=10). Almost a third (n=11) of patients died due to non-cardiac or uncertain cause. Independent predictors of worse survival in the multivariable analysis were presence of VSD (HR 2.97 (95% CI, 1.21-7.27, p=0.017); LVOT obstruction HR 3.11 (95% CI, 1.01-9.52, p=0.047) and requirement for an ‘associated cardiac procedure’, HR 2.57 (95% CI, 1.00-6.57, p=0.048).
Overall, 39% (n=328) of patients required > 2 re-interventions on the arch e.g. for residual/re-coarctation, aneurysm repair or revision of previous surgery. The majority of patients in this re-intervention cohort (n=246) only required one further re-intervention and most were performed percutaneously (n=156).
Freedom from second arch re-intervention was 71% (95% CI:68-74) at 30 years and 47% (95% CI 37-57) at 60 years after CoA repair (Figure 2). Independent risk factors for re-intervention were patients with LVOT obstruction at the time of CoA repair, age <1 year old at time of repair, initial percutaneous repair and initial surgical patch repair.
At least one aortic valve intervention was required in 13% of the cohort (n=111). A bicuspid aortic valve was present in 58% of the cohort (n=485). Aortic valve replacement was required most commonly (n=62). Less frequently, ascending aortic intervention was required (n=43) –usually for root or ascending aortic dilation. Figure 3 shows freedom from aortic valve or ascending aortic intervention.
This analysis confirms, yet again, that patients even with a relatively simple ACHD lesions like CoA, are not fully ‘fixed’ and require structured, lifelong surveillance – preferably by ACHD teams versed in the complications that can ensue. Outcomes will inevitably reflect baseline complexity of anatomy, co-morbidities and the success and choice of initial intervention. Survival is much lower compared to age and sex matched normal subjects and whilst the survival and secondary outcomes described are ‘contemporary’, they probably will not reflect the long-term outcomes of present day practice.