Billie-Jean Martin

NEUROCOGNITIVE ABILITIES IN CHILDREN WHO HAVE UNDERGONE THE FONTAN OPERATION: THE ASSOCIATION BETWEEN HYPOPLASTIC LEFT HEART SYNDROME AND OUTCOMES

Successful palliation of patients with severe congenital heart disease has only become possible in recent decades. While children who have undergone single ventricle palliation are living longer, their neurocognitive outcomes are not well known. The objective of this study was to determine the

Tricuspid Valve Repair in Infancy Using Neochordae: Three-Dimensional Echocardiographic Imaging

Tricuspid regurgitation (TR) in infancy poses a surgical challenge. Both two- and three-dimensional echocardiography (3DE) can provide detailed information about the mechanism(s) of valve failure and insights into valve adaptation during follow-up. We report two patients who underwent tricuspid valve repair using Gore-Tex neochordae, repairs which were facilitated by and assessed with 3DE. Both infants had less than mild residual TR and no valve tethering at hospital discharge. Furthermore, follow-up 3DEs have helped to confirm valve competence, lack of tethering, and growth of the valve and valve apparatus.

Neonatal Tracheal and Intracardiac Repair in a High-Risk Premature Infant Requiring Preoperative ECMO Transport

Congenital tracheal stenosis is an uncommon malformation that portends a poor outcome in children who are symptomatic in the neonatal period. Over time, the management of significant tracheal disease has been consolidated at high-volume centers, and increasingly complex patients have undergone surgical repair. We present a premature newborn boy who was diagnosed with critical multi-level airway and cardiac disease who decompensated at a remote site, requiring extracorporeal membrane oxygenation support for transport. He underwent a complete repair including a slide tracheoplasty and was successfully discharged home, with no residual stenosis at follow-up.

A Unique Intracardiac Shunt: Identifying Complex Anatomy in Reoperative Adult Congenital Heart Disease:

Adult patients with repaired congenital heart disease are presenting with previously unseen types of residual lesions and consequences of prior repair. Patients with d-transposition of the great arteries repaired with atrial switch operations are returning with dysrhythmias and atrioventricular valve disease requiring intervention. We present the challenging case of a young adult with a residual shunt identified on preoperative three-dimensional transthoracic echocardiography, the precise anatomy of which was only characterized intraoperatively.

An Unusual Cause of Hemopericardium 22 Years After a Fontan Procedure

Late tamponade after cardiac operations is rare but reasonably well described. We report a case of exceedingly late tamponade secondary to a spontaneous coronary bleed 22 years after a Fontan operation, which was repaired with catheter intervention.

Hypoplastic Left Heart Syndrome is not Associated with Worse Clinical or Neurodevelopmental Outcomes Than Other Cardiac Pathologies After the Norwood–Sano Operation

There is evidence to suggest that patients undergoing a Norwood for non-HLHS anatomy may have lower mortality than classic HLHS, but differences in neurodevelopmental outcome have not been assessed. Our objective was to compare survival and neurodevelopmental outcome during the same surgical era in a large, well-described cohort. All subjects who underwent a Norwood–Sano operation between 2005 and 2014 were included. Follow-up clinical, neurological, and developmental data were obtained from the Western Canadian Complex Pediatric Therapies Follow-up Program database. Developmental outcomes were assessed at 2 years of age using the Bayley Scales of Infant and Toddler Development (Bayley-III). Survival was assessed using Kaplan–Meier analysis. Baseline characteristics, survival, and neurodevelopmental outcomes were compared between those with HLHS and those with non-HLHS anatomy (non-HLHS). The study comprised 126 infants (75 male), 87 of whom had HLHS. Five-year survival was the same for subjects with HLHS and those with non-HLHS (HLHS 71.8%, non-HLHS 76.9%; p = 0.592). Ninety-three patients underwent neurodevelopmental assessment including Bayley-III scores. The overall mean cognitive composite score was 91.5 (SD 14.6), language score was 86.6 (SD 16.7) and overall mean motor composite score was 85.8 (SD 14.5); being lower than the American normative population mean score of 100 (SD 15) for each (p-value for each comparison, <0.0001). None of the cognitive, language, or motor scores differed between those with HLHS and non-HLHS (all p > 0.05). In the generalized linear models, dominant right ventricle anatomy (present in 117 (93%) of patients) was predictive of lower language and motor scores. Comparative analysis of the HLHS and non-HLHS groups undergoing single ventricle palliation including a Norwood–Sano, during the same era, showed comparable 2-year survival and neurodevelopmental outcomes.