ABSTRACT
An 11-yr-old girl, born out of a consanguineous marriage presented with recurrent exertional syncope due to ventricular tachycardia. She had woolly hair, palmoplantar hyperkeratosis and mild cardiomegaly. Echocardiogram revealed mild left ventricular dysfunction. Features were consistent with Carvajal variant of Naxos disease, an arrhythmogenic cardiomyopathy with autosomal recessive inheritance.
Subject(s)
Arrhythmogenic Right Ventricular Dysplasia/diagnosis , Cardiomyopathies/diagnosis , Hair Diseases/diagnosis , Keratoderma, Palmoplantar/diagnosis , Cardiomyopathy, Dilated , Child , Female , HumansABSTRACT
OBJECTIVES: To describe the case selection, imaging considerations, technique, and results of catheter closure of atrial septal defects (ASD) with deficient inferior vena cava (IVC) rim. BACKGROUND: Transcatheter closure with Amplatzer septal occluder (ASO) has become standard treatment for most secundum ASDs. Defects with deficient IVC rim continue to be challenging to image and close in the catheterization laboratory. METHODS: Records of 12 patients with deficient IVC rim (<5 mm), who underwent catheter closure (April 2007 to June 2008) were reviewed. General anesthesia and transesophageal echo (TEE) guidance was used in all. The IVC rim was imaged at 70 degrees-90 degrees with retroflexion of the TEE probe, in addition to the conventional views. Devices 1-4 mm > maximal ASD size were selected. Deployment was accomplished either from the left atrium, left upper or from the right pulmonary veins. RESULTS: The median age was 5.5 (2.5-27) years and median weight was 19.5 (9-65) kg. The defects measured 16-32 mm and 18-36 mm septal occluders were used. The median fluoroscopic time was 13.1 (4.2-32.7) min. Initial device selection was revised in four patients. Two patients had residual flows at IVC margin. The device embolized to right ventricular outflow tract in one patient. This was retrieved, and a larger device was deployed. No other complications were observed immediately or on follow-up (median 6; range 1-14 months). CONCLUSIONS: Transcatheter closure of ASDs with deficient IVC rim is feasible under TEE guidance. The modified retroflexed view allows adequate imaging of IVC rim through TEE.
Subject(s)
Cardiac Catheterization , Echocardiography, Transesophageal , Heart Septal Defects, Atrial/therapy , Ultrasonography, Interventional , Vena Cava, Inferior/abnormalities , Adolescent , Adult , Cardiac Catheterization/adverse effects , Cardiac Catheterization/instrumentation , Child , Child, Preschool , Databases as Topic , Equipment Design , Feasibility Studies , Heart Septal Defects, Atrial/complications , Heart Septal Defects, Atrial/diagnostic imaging , Humans , Radiography, Interventional , Retrospective Studies , Tomography, X-Ray Computed , Treatment Outcome , Vena Cava, Inferior/diagnostic imaging , Young AdultABSTRACT
OBJECTIVES: To describe the case selection, technique and immediate and short-term results of balloon pulmonary valvotomy (BPV) in young infants with tetralogy of Fallot (TOF). BACKGROUND: Symptomatic young infants with TOF can either undergo corrective surgery or Blalock-Taussig (BT) shunt. Corrective surgery in early infancy is associated with significant morbidity and is not a realistic option in many centers. BT shunt carries the risk of branch pulmonary artery distortion and shunt occlusion. METHODS: Infants less than three months with a significant valvar pulmonary stenosis (with or without associated infundibular and annular component) and oxygen saturation
