Walter Sobczyk

Transtelephonic echocardiography: Successful use in a tertiary pediatric referral center

Recent advances in computers and telecommunications have made transtelephonic echocardiography practical. Our institution is developing a network of transmissional echocardiographic sites at several hospitals in our referral region. We reviewed our initial experience to determine whether transmissional echocardiographic studies (1) gave an appropriate diagnostic impression compared with subsequent videotape review and (2) led to appropriate clinical management (i.e., transfer to a tertiary center or continuation of local care and follow-up). From Aug. 1, 1991, to May 31, 1992, we evaluated 47 transmissional studies (diagnoses: 24 normal, 8 patent ductus arteriosus, 6 ventricular septal defect, 2 pulmonary stenosis, 1 ventricular septal defect with interrupted aortic arch, and 6 miscellaneous). Of 47 studies, 39 (83%) were thought to give accurate diagnostic impressions compared with videotape review. Most inaccuracies were due to the selection and transmission of inconclusive information in an otherwise accurate diagnostic study. Only 1 (2%) of 47 studies resulted in an inappropriate clinical decision; a patients transfer for treatment of a potent ductus arteriosus was delayed 1 day because of an inconclusive transmissional study. We conclude that transmissional echocardiography is useful in the management of pediatric patients with suspected heart disease in a regional referral setting.

Quantitative assessment of ventricular function in sickle cell disease: effect of long-term erythrocytapheresis.

Previous studies on cardiac function in patients with sickle cell disease (SCD) demonstrated abnormalities of systolic and diastolic function including elevated left ventricular myocardial performance index (LVMPI) on chronic transfusion protocols. LVMPI has been validated as a useful and easy non‐invasive measure of overall cardiac function. Up to now, there are no reported studies on cardiac function in patients with SCD maintained on long‐term erythrocytapheresis (LTE).

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.

Cardiopulmonary stress testing in children with sickle cell disease who are on long-term erythrocytapheresis.

The purpose of the study was to assess the feasibility and safety of symptom-limited cardiopulmonary stress testing (CPST) in children with sickle cell disease (SCD), who are on long-term erythrocytapheresis. Maximal symptom-limited CPST was performed in 16 children with SCD who were maintained on long-term erythrocytapheresis and the exercise response in this patient cohort was compared with those of a healthy control population. All patients completed the CPST without any complications. Twelve patients with SCD had significant reduction in aerobic capacity [peak oxygen consumption (VO2) <80% predicted]. No patient demonstrated evidence of myocardial ischemia. In summary, symptom-limited CPST can be performed safely in a subgroup of children with SCD and can yield valuable clinical information.

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.

Catheter closure of autologous pericardial extracardiac Fontan fenestration

We describe two patients who required fenestration of an autologous pericardial extracardiac Fontan (APEF), short-medium term angiographic follow-up, and subsequent successful transcatheter closure of the fenestration.