Survival Outcomes of Two-Stage Intracardiac Repair in Large Ventricular Septal Defect and Trisomy 18

Survival Outcomes of Two-Stage Intracardiac Repair in Large Ventricular Septal Defect and Trisomy 18.

Nakai R, Fujioka T, Okamura K, Suzuki T, Nakao A, Kobayashi J, Tsuchiya K.

Pediatr Cardiol. 2021 Jan 29. doi: 10.1007/s00246-021-02546-9. Online ahead of print.

PMID: 33515091


Take Home Messages:

  • Patients with Trisomy 18 and congenital heart disease are offered surgical repair.
  • Cardiac surgical repair improves survival in patients with Trisomy 18.
  • For patients with Trisomy 18 and VSD, PAB followed by intracardiac repair (two-stage approach) offers improved survival.


Commentary from Dr. Luis Quinonez (Boston, MA, USA), section editor of Congenital Heart Surgery Journal Watch:

The authors present a retrospective review of their experience treating 18 patients (2 male, 16 female) with Trisomy 18 and VSD using a two-stage approach (2005-2019). Their approach was placing a PAB as a neonate or infant, followed months later by a complete intracardiac repair with PAB takedown. The gestational age and birth weight were 38 ± 2 weeks and 1861 ± 325 g. Pulmonary artery banding was placed after a median duration of 19.5 (range 6–194) days of life. The age and body weight at the time of intracardiac repair were 18.2 (7.6–50.7) months and 6.0 ± 1.0 kg. There was one hospital death.


Following intracardiac repair, CPR was performed in two patients. Other complications included pneumonia (n = 1), chylothorax (n = 1), pleural effusion (n = 1), and pneumothorax (n = 1). Two patients needed temporary peritoneal dialysis following VSD closure. Follow-up was 24.9 (3.9–173.0) months. The 1- and 5-year survival rates after VSD repair were 88.5% (95% confidence interval, CI 61.4–97.0) and 77.5% (95% CI 42.8–92.8). This survival was significantly better than a comparison group who was palliated with a PAB only. The authors provide details of 3 other cases who underwent complete repair as their first operation, and all died. The authors conclude that “two-stage ICR for large VSDs in patients with T18 improves their life expectancy. Offering palliative surgery during the early neonatal period followed by complete repair after sufficient weight gain is safe and improves survival.”



Many institutions offer cardiac surgical repair to patients with Trisomy 18. Any ethical discussion surrounding this practice is beyond the scope of this commentary. Neonates with Trisomy 18 and congenital heart disease pose many challenges related to associated comorbidities. They are often small for gestational age; may be premature; and may have associated airway disease, just to name a relevant few. The authors of the paper advocate for a two-stage repair of VSDs beginning with a PAB, followed later by complete intracardiac repair. The justification for such a practice seems mostly related the low weight of the patients in their cohort and the poor experience with complete repair (although only in 3 patients).


The authors have good follow-up survival outcomes using the two-stage approach, although morbidity was significant. Other experienced centers may advocate performing complete intracardiac repair from the outset, arguing that complete repair is feasible in a low weight neonate/infant and the morbidity and mortality of the single procedure is similar or less than a staged approach. The differences in approach (staged vs. complete repair) between centers will most likely be related to the comfort level of the surgeon in performing an intracardiac repair in a low birthweight neonate and the experience of the center in managing these small patients with multiple comorbidities. Regardless of the approach taken, the post-operative care of patients with Trisomy 18 can be challenging. Post-operative morbidities are significant, including the need for tracheostomy and gastrostomy. Whatever approach is taken, staged or not, the treating institution needs to commit expertise and resources to manage patients with Trisomy 18.