Quick Fire Cases
Ryan P. O'Hara, PhD
Postdoc
Children's National Hospital
Ryan P. O'Hara, PhD
Postdoc
Children's National Hospital
Zeynep Eren, N/A
Research Trainee
Children's National Hospital
Arif Selcuk, MD
Fellow
Children's National Hospital
Menan Desai, MD
Cardiac Surgeon
Children's National Hospital
Sofia Hanabergh, MSc
Medical Illustrator
Children's National Hospital
Aybala Tongut, MD
Cardiac Surgeon
Children's National Hospital
Can Yerebakan, MD
Associate Chief of CV Surgery
Children's National Hospital
Yves d'Udekem, MD, PhD
Professor
Children's National Medical Center
Yue-Hin Loke, MD
Associate Professor
Children's National Medical Center
A 5-month-old boy, weighing 6.8 kg, with a prenatal diagnosis of transposition of the great arteries (TGA), ventricular septal defect (VSD), and pulmonary stenosis (PS), presented with cyanosis. His previous interventions included balloon atrial septostomy and patent ductus arteriosus stent placement. Echocardiography revealed dysplastic pulmonic valve leaflets and sub-pulmonary muscle narrowing the outflow. The aortic valve annulus was enlarged and there was a moderate peak pulmonary valve gradient. In the newborn period, the patient underwent balloon atrial septostomy and patent ductus arteriosus stent placement. Given the complex anatomy, the decision was made to perform the Nikaidoh procedure with RVOT reconstruction using an ascending aorta autograft and right atrial appendage (RAA).
Diagnostic Techniques and Their Most Important Findings:
Cardiovascular magnetic resonance (CMR) images were obtained prior to the procedure. The cardiac blood pool, great arteries, VSD, and PS were reconstructed using Mimics (Materialise) software, shown in Figure 1, and 3D printed using a Stratasys J55 printer. The patient-specific 3D model was used pre-operatively by the surgeon to assess the relative anatomies of the septum, VSD, pulmonary annulus, and atrioventricular valve. Pre-operative echocardiography showed normal biventricular systolic function and a moderate peak pulmonary valve gradient, with a large aortic valve annulus. Intraoperatively, the anatomy was confirmed, and the ascending aorta and pulmonary artery were transected for aortic root translocation. The RAA was harvested and reconstructed into a bicuspid valve, while the ascending aorta was used as an autograft for RVOT reconstruction. Postoperative echocardiography showed unobstructed left and right ventricular outflow tracts, no pulmonary valve insufficiency, and normal coronary artery flow. At four-month follow-up, echocardiography confirmed normal ventricular function with a moderate residual pulmonary gradient.
Learning Points from this Case: This case demonstrates a novel modification of the Nikaidoh procedure by using an ascending aorta autograft and RAA for RVOT reconstruction. Furthermore, the patient-specific 3D modeling approach enhanced the surgeon's understanding of the patient's unique cardiac anatomy, facilitating a successful outcome with this innovative surgical technique. Patient-specific 3D modeling allows for planning for cases like Nikaidoh, and in this case was an inspiration for the RVOT reconstruction. This case highlights how advanced 3D modeling and printing technologies can be leveraged to optimize surgical approaches and outcomes in challenging pediatric cardiac cases.
