Usefulness of perioperative transoesophageal echocardiography during paediatric cardiac surgery.

BACKGROUND: Paediatric transoesophageal echocardiography probes allow perioperative evaluation during paediatric congenital heart disease surgery. AIM: To assess the usefulness of perioperative transoesophageal echocardiography in evaluating the severity of residual lesions, based on the type of congenital heart disease repaired in paediatric patients. METHODS: A retrospective analysis was conducted on paediatric patients who underwent open-heart surgery at our tertiary centre over a four-year period. Perioperative transoesophageal echocardiography studies were performed, and residual lesions were classified as mild, moderate or severe. RESULTS: Overall, 323 procedures involving 310 patients with a median age of 13.8 (0.07-214.4) months and a median weight of 8.2 (2-96) kg at intervention were enrolled in the study. Twenty-one (6.5%) residual lesions led to immediate reintervention: severe right ventricular outflow tract obstruction (n=12); severe aortic regurgitation (n=3); superior vena cava stenosis (n=2); moderate residual ventricular septal defect (n=2); severe mitral regurgitation (n=1); and severe mitral stenosis (n=1). Three (0.9%) neonates had ventilation difficulties caused by the transoesophageal echocardiography probe having to be removed, but experienced no sequelae. CONCLUSION: Perioperative transoesophageal echocardiography is a safe procedure, providing information on severe residual lesions, leading to the immediate revision of several paediatric congenital heart disease cases.

Advancing paediatric cardiac imaging: a comprehensive analysis of the feasibility and accuracy of a novel 3D paediatric transoesophageal probe.

Aims: Pediatric transoesophageal echocardiography (TOE) probes have remained two-dimensional (2D) limiting their use compared to adults. While critical in pediatrics for interventions and post-surgery assessments, technological advancements introduced a three-dimensional (3D) pediatric TOE probe. This study assessed the new 3D pediatric TOE probe (GE 9VT-D) for feasibility, handling, and imaging quality. Methods and results: At Children's Hospital of Toulouse, 2-month prospective study enrolled children undergoing TOE with the new probe. All imaging modalities were rated by 2 operators using a 5-point Likert-type scale from 1 (very poor) to 5 (very good) quality. Forty-five children, median age 3.7 (range: 2 months-14.7 years) median weight 7.8 kg (range: 4.3-48 kg) underwent 60 TOEs: 25% pre-surgery, 45% post-surgery, 28% during percutaneous procedures, and 2% in intensive care. Probe handling was "very easy" in all cases without adverse events. The median score of 2D, 2D colour, pulsed Doppler and 3D were noted 5 out of 5 and continuous Doppler and 3D colour 4 out of 5. The 3D image quality remained consistent irrespective of the patient weighing above or below 7.8 kg (p = 0.72). Postoperative TOEs identified two cases needing further interventions, emphasizing its value in evaluating surgical outcomes and also for guiding percutaneous interventions. Conclusion: Our comprehensive evaluation demonstrates that the new 3D pediatric TOE probe is feasible and provides high-quality imaging in pediatric patients. The successful integration of this novel probe into clinical practice has the potential to enhance diagnostic accuracy and procedural planning, ultimately optimizing patient outcomes in pediatric cardiac care.

Case Report: Successful Cerebral Revascularization and Cardiac Transplant in a 16-Year-Old Male With Syndromic BRCC3-Related Moyamoya Angiopathy.

Background: BRCC3/MTCP1 deletions are associated with a rare familial moyamoya angiopathy with extracranial manifestations. Case: We report the case of an adolescent male presenting with progressive and symptomatic moyamoya angiopathy and severe dilated cardiomyopathy caused by a hemizygous deletion of BRCC3/MTCP1. He was treated for renovascular hypertension by left kidney homograft and right nephrectomy in infancy and had other syndromic features, including cryptorchidism, growth hormone deficiency, and facial dysmorphism. Due to worsening of the neurological and cardiac condition, he was treated by a direct superficial temporal artery to middle cerebral artery bypass to enable successful cardiac transplant without cerebral damage. Conclusions: BRCC3-related moyamoya is a devastating disease with severe heart and brain complications. This case shows that aggressive management with cerebral revascularization to allow cardiac transplant is feasible and efficient despite end-stage heart failure.