Pre-Fontan Assessment Utilizing Combined Cardiac Catheterization and Cardiac MRI: Comparison to the Pre-Fontan CatheterizatioOutcomes in Adult Congenital Heart Disease Patients With Down Syndrome Undergoing a Cardiac Surgical Procedure.n.
Goldberg SW, Chalak C, Anderson BR, Elhoff J, Gaydos S, Lubert AM, Sassalos P, Gauvreau K, Gurvitz M.Ann Thorac Surg. 2025 Feb;119(2):398-405. doi: 10.1016/j.athoracsur.2024.09.037. Epub 2024 Oct 12.PMID: 39401550
Commentary by:
Dr. Damien Cullington (Liverpool, UK), section editor of ACHD Journal Watch:
Take-home Points:
- Patients with T21 had a longer median length of stay (7 days vs 5 days; P<0.001)
- Patients with T21 had longer duration of mechanical ventilation (12.5 hrs vs 6.7 hrs ; P<0.001). Requirement for NIV ventilation or re-intubation was greater in the T21 cohort (26% vs 4%; P0.001).
- Patients with T21 had a greater likelihood of postoperative infections (10% vs 2%; P=/0.035).
- Risk factors associated with extended length of stay for patients with T21 are pulmonary hypertension requiring medical therapy; history of immunodeficiency or at least moderate TR.
- Whilst surgery is associated with higher rates of complications and length of stay, the survival of selected patients with T21 vs without T21 was similar.
Introduction
Fuller understanding of the long-term prognosis of patients with T21 has resulted, over time, with a more proactive approach to repair congenital heart lesions in such patients. Studies have shown that cardiac surgical procedures can be performed on patients with T21 with similar mortality rates to patients without T21. Subsequently, there are increasing numbers of patients with T21 and repaired CHD – identically to their counterparts without T21, interventional or surgical redo procedures are needed.
The key purpose of this observational study was to compare the postoperative outcomes following cardiac surgery in teenaged and adult patients with T21 vs those without T21 and assess risk factors for greater morbidity and mortality.
Study design
A multicentre retrospective study assessing the surgical outcomes of teenage and adult patients with trisomy 21 (T21) compared to similar patients without T21. Patients were identified from 6 US congenital cardiac centres – Boston Children’s Hospital; Mott Childrens’ Hospital; Cincinnati Children’s Hospital; Morgan Stanley Children’s Hospital; Medical University of South Carolina and Texas Children’s Hospital.
Comparison of 121 patients undergoing surgery with T21 vs 121 patients without T21 between 2009-2018. Inclusion criteria were age >13 years and diagnosis of T21. Patient characteristics are shown in Table 1.
Table 1 – Patient characteristics of cohort.
Key Findings
- Patients with T21 vs without T21 were more likely to have a diagnosis of AVSD or Tetralogy of Fallot – (50% vs 12%; P <0.001) and (12% vs 0%; P< 0.001) respectively.
- Patients without T21 vs those with T21 were more likely to have isolated mitral or aortic valve disease – (4% vs 19%; P<0.001) and (2% vs 12%; P=0.024) respectively.
- Patients with T21 vs without T21 were more likely to have OSA (25% vs 3%; P<0.001).
- Patients with T21 vs without T21 were more likely to have endocrine disease (52% vs 3%; P<0.001).
- Post-operative outcomes are shown in Table 2
Table 2 – Post operative outcomes
The risk factors for prolonged hospital length of stay are shown in Table 3. Pulmonary hypertension requiring medical therapy, immunodeficiency and >moderate TR before surgery were the main risks identified in the multivariable analysis for predicting long length of stay (>10 days).
Table 3 – Risk factors for prolonged hospital length of stay in T21 cohort
Strengths
- Reassuring findings from a comparative dataset which bolster the argument that patients with T21 who undergo intervention can have favourable redo surgical outcomes in spite of their co-morbidities.
- Identifying risk factors which could lead to complications and longer length of stay is essential to help counsel patients and the families of patients with T21.
Limitations
- Retrospective design results in a selection bias to the cohort as this is a non randomised selection of patients put forward for surgical intervention. They comprise a cohort of T21 patients who may be inherently fitter for surgery which mitigates against complication and mortality. Understanding patient characteristics with T21 whom are turned down for surgery would be a useful insight.
- The median age in this T21 cohort was only 17.9 years and so may underestimate risk for older T21 patients.
Conclusion
Overall, this data provides reassurance and bolsters the argument that selected patients with T21 needing surgical intervention have favourable surgical outcomes in spite of their additional co-morbidities. For clinicians and surgeons who regularly provide care postoperatively for patients with T21, there are ‘known, known’ complications such as their likely extended length of stay. The analysis helps to focus on specific risk factors that are associated with potential complications post operatively. Future studies assessing older patients with T21 undergoing surgery would be useful.
