RESUMO
INTRODUCTION: The use of del Nido cardioplegia has increased in the adult cardiac surgery population. Centers have adopted the formula with a variety of circuit systems. This report includes our set up for delivering 1:4 (blood: crystalloid) del Nido cardioplegia. MATERIALS AND METHODS: A homemade circuit for cardioplegia administration was built with a pediatric reservoir, a roller pump, a coil cooler, a 3/16-inch circuit to administer and recycle cardioplegia, and two »-inch tubes to collect the patient's blood. TECHNIQUE: The circuit allows the perfusionist to collect the blood directly from the cardiopulmonary bypass arterial limb of the circuit, to precisely mix it with the crystalloid component of the del Nido cardioplegia solution already in the reservoir, and to administer the final solution under strictly controlled parameters. SUMMARY: We present a circuit design that can accurately measure and administer del Nido cardioplegia through the use of a roller pump and a pediatric reservoir. It simplifies and enhances the accuracy and efficiency of cardioplegic administration in our practice.
RESUMO
Spinal muscular atrophy with respiratory distress (SMARD 1) is a very rare autosomal recessive motor neuron disorder that affects infants and is characterized by diaphragmatic palsy, symmetrical distal muscular weakness, muscle atrophy, peripheral sensory neuropathy and autonomic nerve dysfunction. SMARD 1 is inherited as an autosomal recessive trait and the mutations have been identified in the gene encoding immunoglobulin µ-binding protein 2 (IGHMBP2), located on chromosome 11q13. It is considered a fatal form of infantile motoneuron disease and most of the patients dies within the first 13 months of life. We present a female child with genetically confirmed SMARD 1 displaying a mild phenotype and no severe signs of respiratory involvement, typically found in this form, up to 38 months despite a diaphragmatic palsy diagnosed at 6 months of age. Therefore, our clinical observation suggests that respiratory failure is not secondary, in any case, to the diaphragmatic palsy but other pathogenetic mechanisms might be involved.