Factors associated with mortality or transplantation versus Fontan completion after cavopulmonary shunt for patients with tricuspid atresia



      Tricuspid atresia with normally related great vessels (TA) is considered the optimal substrate for the Fontan pathway. The factors associated with death or transplantation after cavopulmonary shunt (CPS) are underappreciated. We aimed to determine factors associated with CPS–Fontan interstage death/transplantation versus transition to Fontan in TA.


      A total of 417 infants younger than 3 months of age with TA were enrolled (January 1999 to February 2020) from 40 institutions into the Congenital Heart Surgeons' Society TA cohort. Parametric competing risk methodology was used to determine factors associated with the competing end points of death/transplantation without Fontan completion, and transition to Fontan.


      CPS was performed in 382 patients with TA; of those, 5% died or underwent transplantation without transition to Fontan and 91% transitioned to Fontan by 5 years after CPS. Prenatal diagnosis (hazard ratio [HR], 0.74; P < .001) and pulmonary artery band (PAB) at CPS (HR, 0.50; P < .001) were negatively associated with Fontan completion. Preoperative moderate or greater mitral valve regurgitation (HR, 3.0; P < .001), concomitant mitral valve repair (HR, 11.0; P < .001), PAB at CPS (HR, 3.0; P < .001), postoperative superior vena cava interventions (HR, 9.0; P < .001), and CPS takedown (HR, 40.0; P < .001) were associated with death/transplantation.


      The mortality rate after CPS in patients with TA is notable. Those with preoperative mitral valve regurgitation remain a high-risk group. PAB at the time of CPS being associated with both increased risk of death and decreased Fontan completion may represent a deleterious effect of antegrade pulmonary blood flow in the CPS circulation.

      Graphical abstract

      Key Words

      Abbreviations and Acronyms:

      AV (atrioventricular), BT (Blalock–Taussig), CHSS (Congenital Heart Surgeons' Society), CPS (cavopulmonary shunt), LV (left ventricular), MR (mitral valve regurgitation), MV (mitral valve), PA (pulmonary artery), Q1-Q3 (first quartile to third quartile), SVC (superior vena cava), TA (tricuspid atresia)
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      Linked Article

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          In the current issue of the Journal, Callahan and colleagues1 report their study of infants born with tricuspid atresia and normally related great arteries from the Congenital Heart Surgeons Society (CHSS) cohort. They focused on outcomes following cavopulmonary shunt (CPS) and examined factors associated with Fontan completion, death, or transplantation following CPS. They found that by 5 years after CPS, 5% of the patients died or underwent transplantation and 91% transitioned to Fontan. They determined that preoperative moderate or greater mitral regurgitation, concomitant mitral repair, a pulmonary artery band (PAB) at CPS, and postoperative superior vena cava interventions, including CPS takedown, were associated with death or transplantation.
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        • PDF