Michael R. Recto

Alemtuzumab (Campath-1H) Induction in a Pediatric Heart Transplant: Successful Outcome and Rationale for Its Use

Despite increasing clinical experience in adult transplantation, induction therapy with alemtuzumab (Campath-1H) has rarely been reported in pediatric solid-organ transplants, and has been limited to kidneys, intestine and multi-visceral organs. Basic science research and clinical observations reported from the adult experience suggest potential benefits of alemtuzumab in pediatric organ recipients. We report successful induction therapy with alemtuzumab and steroid-free maintenance therapy for cardiac transplantation in a teenager, and discuss its merits in this patient.

Midaortic Syndrome Presenting as Neonatal Hypertension

We describe a case of mid-aortic syndrome presenting as systemic hypertension in infancy and early childhood. Angiography of the descending and abdominal aorta is the diagnostic test of choice to confirm the diagnosis of mid-aortic syndrome. Severity of hypertension is one of the major factors in determining the timing of intervention. Because of variability in the anatomic extent of mid-aortic syndrome, management options need to be individualized.

Right Superior Vena Cava Draining Predominantly Into the Left Atrium Causing Cyanosis in a Young Child

During preoperative testing for tonsillectomy and adenoidectomy, an asymptomatic 4-year-old child was found to have clubbing, cyanosis (saturation of 78%), and polycythemia (hemoglobin 16.8 g/dl). By auscultation, he had a grade 1/6 systolic murmur. Electrocardiogram was normal. Chest x-ray revealed top normal heart size. Transthoracic echo and bubble study through a right arm vein were suggestive of right superior vena cava (SVC) draining predominantly into the left atrium. Transesophageal echocardiography (TEE) confirmed the diagnosis (Fig. 1); however, drainage of the right-sided pulmonary veins could not be defined. At cardiac catheterization, there was a 5-mmHg gradient on pullback from the SVC into the right atrium and an increase in oxygen saturation in the low SVC. SVC angiography demonstrated flow predominantly into the left atrium (Fig. 2) with anomalous drainage of the right upper pulmonary vein into the SVC (Fig. 2). At surgery, the innominate vein and inferior vena cava were cannulated for venous return. The right atrium was opened and a 3-mm tubular connection between the right atrium and SVC was closed. The SVC was divided above the entrance of the right superior pulmonary vein into the SVC and anastomosed to the superior aspect of the right atrial appendage [21]. The patient had an uneventful recovery, with a room air saturation of 99%. Anomalous drainage of the right SVC into the left atrium as an isolated anomaly is rare. Review of the literature documents at least 16 cases since it was first described by Wood [5] in 1956. Seven cases of biatrial drainage have also been reported [2]. Most of these patients were asymptomatic except for mild cyanosis. Although cyanosis appears to be well tolerated, surgical correction is recommended to decrease the risk of paradoxical emboli, which can result in systemic embolization, stroke, and brain abscess [2, 4]. The etiology of this anomaly has frequently been attributed to leftward and cephalic malposition of the right horn of sinus venosus [3]. Another proposed etiology is rightward deviation of atrial septum; however, this is unlikely because both the coronary sinus and the inferior vena cava would then be positioned to the left of the atrial septum [3]. This case illustrates the importance of using both echocardiography and cardiac catheterization to establish the diagnosis of this rare congenital malformation. After the diagnosis was established, the Correspondence to: M.R. Recto, email: mitch.recto@louisville.edu Fig. 1. Transesophageal echo (TEE) two-dimensional/color-flow image in the sagittal plane (long-axis view) demonstrating the superior vena cava (SVC) draining predominantly into the left atrium (LA). RA, right atrium. Pediatr Cardiol 25:163–164, 2004 DOI: 10.1007/s00246-003-9006-8

Thrombus Formation within the Right Ventricle-to-Pulmonary Artery Conduit (Sano Shunt) as a Cause of Acute Cyanosis in a Patient with Hypoplastic Left Heart Syndrome

We describe a case of thrombus formation within a right ventricle-to-pulmonary artery conduit (Sano shunt) in a patient with hypoplastic left heart syndrome with resulting acute cyanosis and hypotension requiring emergency balloon angioplasty.

Restrictive tunnel patent foramen ovale and left atrial hypertension in single-ventricle physiology: implications for stent placement across the atrial septum.

Our objective is to describe our approach to the management of patients with single-ventricle physiology and restrictive tunnel patent foramen ovale (TPFO) with unfavorable atrial septal morphology. We describe a series of five patients with single-ventricle physiology and restrictive TPFO and our experience with radiofrequency perforation (RFP), static balloon atrial septostomy (BAS), and stent implantation to create an alternative pathway for left atrial decompression. Between July 4, 2006, and July 10, 2007, five patients with single-ventricle physiology and restrictive TPFO were brought to the cardiac catheterization laboratory for decompression of a hypertensive left atrium. Four of five patients underwent RFP followed by static BAS and stent implantation across the newly created atrial communication. One patient had a stent placed across an existing TPFO. Unfortunately, stable stent position was not achieved in this case, and the patient required open atrial septectomy. In patients with single-ventricle physiology and a restrictive TPFO associated with left atrial hypertension, stent placement across the existing defect can result in unstable stent position. Using a RFP wire to create a new defect in the septum primum allows stable stent deployment across the atrial septum and achieves left atrial decompression.

IntraStent double-strut LD: Collapse and recoil following use in postoperative stenoses

We describe the use of the Intratherapeutics IntraStent in two postoperative patients, its collapse and recoil following deployment, and subsequent transcatheter correction. Cathet Cardiovasc Intervent 2002;56:254–261.