Handmade Bicuspid Valved Polytetrafluoroethylene Conduit for Right Ventricular Outflow Tract Reconstruction.

Background: Excellent outcomes of right ventricle to pulmonary artery conduits with polytetrafluoroethylene (PTFE) valves have been reported. The purpose of this study was to analyze the short-term results of our handmade PTFE bicuspid valved conduit (VC) for right ventricular outflow tract reconstruction. Methods: Between September 2019 and May 2023, bicuspid PTFE-VC was implanted in 17 patients at a median age of 2.5 years (range, 3 months to 13.6 years). The PTFE-VC was fashioned from a commercially available PTFE tube graft (14 mm in three patients, 16 mm in three patients, 18 mm in one patient, 20 mm in three patients, and 22 mm in seven patients) and 0.1 mm thick PTFE membrane for the leaflet material. Valve function was assessed by echocardiogram after the implantation. The conduit reoperation and the conduit dysfunction were analyzed. There were no early deaths, but there was one late death. Results: There were no postoperative in-hospital deaths. Follow-up echocardiograms were available for 14 of 17 patients. The median follow-up was 21 months (range, 7-49 months). Conduit stenosis was none or trivial in 11 patients while it was mild in two and moderate in one patient and severe in 0 patients. Conduit insufficiency was mild or trivial in all 14 patients. By the end of the study period, freedom from reoperation/reintervention was 100%. There were no episodes of aneurysmal dilatation of the conduit or endocarditis. Conclusions: Handmade bicuspid PTFE VC shows good short-term outcome, with no significant valve dysfunction and no reintervention. A longer follow-up is necessary to evaluate the long-term advantages of using the handmade bicuspid PTFE VC.

Redo-ALCAPA Repair With Left Subclavian Artery-to-Left Main Coronary Bypass for Left Coronary Atresia.

Different surgical techniques have been described for the primary repair of anomalous left coronary artery arising from the pulmonary artery (ALCAPA); however, very few techniques are described for management of coronary artery occlusion following ALCAPA repair. We present a case of a 7-year-old girl with left main coronary atresia status-post left coronary button transfer for ALCAPA in infancy. She underwent redo-sternotomy and left subclavian artery-to-left main coronary artery bypass plus mitral valve repair and had an uneventful postoperative course.

Giant Right Atrial Aneurysm in an Infant.

Right atrial aneurysm (RAA) is a rare congenital anomaly with a diverse clinical spectrum. We present a case of antenatal detection of a giant RAA. The infant had 3 episodes of staring spells presumed to be thrombo-embolic phenomena originating from the RAA. The infant underwent successful RAA resection with preservation of the right coronary artery that was displaced from its usual position due to invagination of the RAA in the subepicardial space of the right atrio-ventricular groove.

Primary Arterial Switch Operation for TGA/IVS and Regressed Left Ventricle: How and When to Use Left Ventricular Assist Device.

PURPOSE: Numerous attempts have been made to extend the boundaries of arterial switch operation (ASO) in children presenting late with transposition of great arteries with intact ventricular septum (TGA/IVS) and regressed left ventricle (rLV). Many children tolerate the delayed ASO uneventfully, whereas others need mechanical circulatory support (MCS) to sustain the systemic circulation while the left ventricle undergoes retraining. DESCRIPTION: In this article, we describe six consecutive children with TGA/IVS and rLV who underwent primary ASO. RESULTS: Three were managed medically, while three required MCS in the form of Centrimag left ventricular assist device (LVAD). All patients survived the operation and were discharged home in a stable condition. CONCLUSIONS: Primary ASO can be safely performed in children with TGA/IVS and rLV, provided the center has MCS options. Supporting the rLV with LVAD is feasible and can be achieved safely.

Use of Left Ventricular Assist Device After Arterial Switch Operation in Late Presenting D-Transposition of Great Arteries-A Technique for Retraining the Regressed Left Ventricle.

The preferred approach for patients with D-transposition of the great arteries with an intact ventricular septum (DTGA/IVS) is the arterial switch operation (ASO). In those patients presenting late, with regressed left ventricle (LV), successful LV preparation is of paramount importance to achieve this goal. We present a toddler with DTGA/IVS who underwent ASO followed by successful left ventricular retraining with postoperative left ventricular assist device support with CentriMag centrifugal pump.

Biventricular Repair in Interrupted Aortic Arch Type C With Aortic Atresia.

We report a case of interrupted aortic arch type C with aortic atresia and a ventricular septal defect with two well-developed ventricles, who underwent a successful single-stage biventricular repair with the modified Yasui procedure and arch reconstruction. Angiography done during conduit revision showed bilateral brachiocephalic trunks with high branching. The child is doing well six years after the initial operation.

Midterm outcome of primary arterial switch operation beyond six weeks of life in children with transposition of great arteries and intact ventricular septum.

BACKGROUND: We have previously reported our experience in primary arterial switch operation (ASO) in children more than six weeks with transposition of great arteries and intact ventricular septum (TGA/IVS). The upper age limit for performing an ASO in these children is not yet settled and reports regarding outcome of ASO in these children are few. In this prospective observational study, we report the midterm results of children with TGA-IVS older than six weeks undergoing primary ASO. METHODS: A total of 109 children aged more than 6 weeks with median age of 60 days (range 42-3,000 days), with regressed left ventricle underwent primary ASO. Extracorporeal membrane oxygenation was used in 20% (22 of 109) of them; 90.8% (99 of 109) of children who survived were prospectively followed, with a mean follow-up of 28 months (range 18-84 months). RESULTS: Two late deaths occurred, and survival in the remainder was estimated to be 98% at seven years. The incidence of aortic regurgitation (AR) was found to have a decreasing trend with freedom from AR approaching 100% by 34 months. The left ventricular shape and function returned to normal within one to three months following surgery. None of these children had any rhythm disturbances or evidence of myocardial ischemia. CONCLUSIONS: Primary ASO can be safely performed in children with regressed ventricle, irrespective of age with encouraging results. The midterm results of these children are comparable in terms of survival and freedom from complications associated with preserved ventricle.

Pediatric clear cell sarcoma of the kidney with cavoatrial thrombus.

Clear cell sarcoma of the kidney (CCSK) is a rare renal tumor. Only 4 cases of CCSK with vascular thrombus have been reported, and 2 of these were pediatric cases. One of the children had an intraatrial thrombus as well. We describe a 3-year-old boy who was diagnosed as having a Wilms tumor but did not respond to preresection chemotherapy. He underwent complete resection of the tumor under cardiopulmonary bypass. Histologic examination indicated that the tumor was a CCSK. The patient was then managed with appropriate chemotherapy and radiation therapy and is well 16 months after diagnosis.

How to do it: repair of anomalous left coronary artery from pulmonary artery (ALCAPA) with coronary button transfer and Lecompte maneuver.

An alternative technique of coronary button transfer and Lecompte maneuver for anomalous left coronary artery (ALCAPA) arising from left lateral pulmonary sinus is described. This technique was used by us successfully in four patients aged 6 months to 3.5 years, weighing from 4.7 to 16 kg. The importance of trapdoor technique and Lecompte maneuver is discussed.