22q11.2 Deletion syndrome is associated with perioperative outcome in tetralogy of Fallot

Abstract

Objectives We sought to investigate the impact of 22q11.2 deletion on perioperative outcome in tetralogy of Fallot. Methods We conducted a retrospective review of patients with tetralogy of Fallot who underwent complete surgical reconstruction at The Children’s Hospital of Philadelphia between 1995 and 2006. Inclusion criteria included diagnosis of tetralogy of Fallot and known genotype. Fisher’s exact and Mann Whitney tests were used for categorical and continuous variables, respectively. Regression analysis was used to determine whether deletion status predicts outcome. Results We studied 208 subjects with tetralogy of Fallot, 164 (79%) without, and 44 (20%) with a 22q11.2 deletion (22q11.2DS). There were no differences in sex, race, gestational age, age at diagnosis, admission weight, and duration of mechanical ventilation. Presenting anatomy, survival, complications and re-operations were also comparable between patients with and without 22q11.2DS. Those with 22q11.2DS had more aortopulmonary shunts preceding complete surgical repair (21% vs. 7%, p= 0.02). This association was present after adjustment for presenting anatomy (stenosis, atresia, or absence of pulmonary valve and common atrioventricular canal) and surgical era. In addition, those with 22q11.2DS had longer cardiopulmonary bypass time (84 vs. 72 minutes, p=0.02), and duration of intensive care (6 days vs. 4 days, p=0.007). Conclusions Genotype affects early operative outcomes in tetralogy of Fallot resulting, in particular, in longer duration of intensive care. Future studies are required to determine factors contributing to such differences in this susceptible population.

Effect of Obesity and Underweight Status on Perioperative Outcomes of Congenital Heart Operations in Children, Adolescents, and Young Adults

“…In the current study, underweight patients underwent more complicated operations, suggesting more severe heart disease or at the very least an intrinsically higher risk of undesirable outcome. They were also more likely to have increased risk of genetic syndromes and anatomic abnormalities, which have also been shown in other studies to be associated independently with worse perioperative outcome 16,40,41 . Pre-operative symptom scores (e.g.…”

Trends in Pulmonary Valve Replacement in Children and Adults With Tetralogy of Fallot

“…Presence of a genetic syndrome was associated with younger age at PVR, longer hospital LOS, longer ICU LOS, and increased cost. The observed increased LOS and resource utilization is consistent with previous studies of perioperative outcomes in infants and younger children with 22q11.2 microdeletion 25,26 . TC-PVR was associated with reduced hospital LOS, but was not associated with significant reduction in cost compared with S-PVR.…”

Early postoperative remodelling following repair of tetralogy of Fallot utilising unsedated cardiac magnetic resonance: a pilot study

“…13 Briefly, subjects were fed 3 to 4 hours prior to the planned initiation of the procedure and fasted up until their cardiac MRI. Immediately prior to the procedure, electrocardiographic leads were placed, the patient was fed, swaddled in an infant blanket, and placed in a vacuum immobilizer (MedVac bag, CFI Medical Solutions/Contour Fabricators, Fenton, MI, USA).…”

“…5–10 Although perioperative outcomes have been described in tetralogy of Fallot, there is little information about the early ventricular adaptations, including pulmonary regurgitation, which occur immediately following surgical repair and may be important to understanding the changes which occur later in life and put the magnitude of these alterations into perspective. 11–13 The Feed and Sleep technique is an anesthesia-free method for performing cardiac MRI, and has been used in select patients with proven utility in the assessment of intra- and extra-cardiac anatomy in certain congenital heart defects. 14–16 …”