Rochelson E, Richmond ME, LaPar DJ, Torres A, Anderson BR.
Semin Thorac Cardiovasc Surg. 2020 Feb 19. pii: S1043-0679(20)30033-2. doi: 10.1053/j.semtcvs.2020.02.018. [Epub ahead of print]
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Take Home Points:
- Despite significant improvements in the perioperative care of single ventricle patients, the risk for lifelong morbidity and mortality following the Fontan procedure persists.
- Neonates undergoing balloon atrial septostomy are at significant risk for Fontan failure later in life.
- Other patient characteristics and perioperative events were not associated with Fontan failure in this cohort.
Commentary from Dr. Charlotte Van Dorn (Rochester, MN), section editor of Pediatric Cardiology Journal Watch: This is a single center retrospective study to evaluate all patients undergoing a Fontan procedure. The objective of this study was to identify characteristics throughout a patient’s lifespan that might predict early Fontan failure (death, Fontan takedown, heart transplant listing before hospital discharge or <30 days postoperatively).
Methods: Data collected included perioperative patient care (stage I, stage II and stage III), patient and operative characteristics, as well as outcomes. Patients were excluded if they underwent a hybrid stage I or if they underwent stage I and/or stage 2 surgery elsewhere.
Results: A total of 191 patients met inclusion criteria with the most common anatomy being HLHS followed by tricuspid atresia. Relevant stage 1 perioperative characteristics including 8% undergoing balloon atrial septostomy; 2 of which required RF perforation; 56% underwent the Norwood procedure with 29% undergoing isolated shunt placement. Relevant stage II preoperative characteristics were notable for moderate or severe AV valve regurgitation in 12% and moderate or severe systemic ventricular systolic dysfunction in 5%. Most patients underwent a unilateral or bilateral Glenn procedure with approximately half of stage II patients also requiring a pulmonary arterioplasty. Post stage II median chest tube duration was 4 days and medial hospital LOS was 6 days. Prior to stage III, 12% had moderate or severe AV valve regurgitation by echocardiogram. The degree of valvar regurgitation and ventricular dysfunction was highly associated with pre-Stage 2 AV valve regurgitation and ventricular dysfunction. Approximately 50% of pre-Fontan patients required an intervention during their pre-Fontan cath with the most common intervention being coiling of collaterals. Of the Fontans performed, 56% were extracardiac conduits while the remaining were lateral tunnel procedures; 48% were fenestrated. Outcomes included operative deaths (6 patients), Fontan takedown (2 patients) and no patients listed for cardiac transplantation before discharge/30 days post-op.
A neonatal balloon atrial septostomy (BAS) was the only characteristic associated with early Fontan failure at an odds ratio of 8.5. This was not associated with pre-Stage 2 or pre-Fontan cardiac catheterization hemodynamics. No other perioperative characteristic was associated with Fontan failure in this cohort.
Discussion: In this cohort, the incidence of Fontan failure was low and only a single patient characteristic (neonatal BAS) was associated with failure. It is likely that BAS in this cohort represents the physiology of a restrictive atrial septum which has been previously reported to be associated with poorer.
Limitations: There is potential selection bias in that patients who died prior to the Fontan procedure or those not deemed good Fontan candidates were excluded from this cohort. This study is also limited by the small number of Fontan failures making regression analyses difficult.
Next Steps: A multicenter study of a larger single ventricle cohort, including patients undergoing Fontan procedure as well as those who died or were felt to be poor Fontan candidates are needed to better determine patient and perioperative characteristics contributing to the inability to undergo a Fontan or subsequent Fontan failure.