Percutaneous transaortic occlusion of patent ductus arteriosus using a new versatile angiographic and delivery catheter.

We evaluated the usefulness of patent ductus arteriosus (PDA) occlusion using a new catheter that combines good angiographic properties, easy manipulation across the duct, documentation of aortic and pulmonary artery pressure, and coil delivery for percutaneous transaortic ductal occlusion. Thirty patients (13 females and 17 males) who met the clinical and echocardiographic criteria for the percutaneous closure of PDA were catheterized. The patients selected for this study had a small to moderate PDA with a conal or tubular shape, adequate aortic ampula, and normal or mildly elevated pulmonary artery pressure. The median age was 4.8 years (range, 10 months-20 years). Patients were catheterized by the transaortic approach using the new angiographic and coil delivery catheter specially designed for this procedure. A PDA was closed in every case. A single DuctOcclud pfm coil was delivered in 28 patients, and 2 patients had NitOcclud pfm coils for ductal occlusion. The screening time was measured and found to be 50% less than the measured screening time using other catheters in our laboratory. Only one catheter and one guidewire were used per procedure, with no need for exchange wires or the establishment of an arteriovenous loop. The procedure time was also reduced by 50%. A minimal residual shunt, demonstrated by color-flow Doppler mapping, was present in 2 patients. The new combined angiographic and coil delivery catheter is most suitable for transaortic PDA coil occlusion, significantly decreasing the procedure and screening time.

Cardiac manifestations in thiamine-responsive megaloblastic anemia syndrome.

Thiamine-responsive megaloblastic anemia (TRMA) syndrome is a rare autosomal recessive disorder defined by the occurrence of megaloblastic anemia, diabetes mellitus, and sensorineural deafness, responding in varying degrees to thiamine treatment. Other features of this syndrome gradually develop. We describe three TRMA patients with heart rhythm abnormalities and structural cardiac anomalies. Eight other reported TRMA patients also had cardiac anomalies. Recently, the TRMA gene, SLC19A2, was identified, encoding a functional thiamine transporter. Characterization of the metabolic defect of TRMA may shed light on the role of thiamine in common cardiac abnormalities.