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Biomedical subjects

S M Yabek

Publications and source records attributed to S M Yabek.

At least 19 recordsLinked to original sources

Ventricular arrhythmias in children with an apparently normal heart.

Ventricular ectopy occurs frequently in normal children. In the presence of a normal heart, these arrhythmias, including asymptomatic, nonsustained ventricular tachycardia, carry a benign prognosis and are not associated with sudden, unexpected death. However, complex ventricular arrhythmias frequently indicate the presence of underlying cardiac disease; patients with such arrhythmias must undergo an appropriately thorough evaluation before decisions regarding prognosis and the need for therapy can be made.

Adolescent

Great vein and right atrial thrombosis in critically ill infants and children with central venous lines.

We performed a retrospective review of echocardiographic data files of infants and children hospitalized in the Newborn and Pediatric Intensive Care Units. Echocardiograms were examined to detect the presence and evolution of great vein and right atrial thrombosis in patients with central venous lines. Thirty-seven patients were identified over a five-year period. Echocardiograms were performed, not routinely, but in response to specific indications including catheter malfunction, thrombocytopenia, persistent chylothorax, bacterial or fungal sepsis, and superior vena cava syndrome. Fifteen of 37 patients died, 13 of them during the hospitalization in which the thrombus was discovered. Thrombolytic agents and surgery were used to treat selected patients, with mixed results. Two of the 22 survivors have significant disability related to the thrombus or complications arising from it. We conclude that great vein and/or right atrial thrombosis is a common complication of central venous catheterization in small infants and children; moreover, the morbidity and mortality relating to this complication is substantial.

Catheterization, Central Venous

Rate-adaptive cardiac pacing in children using a minute ventilation biosensor.

Chronotropic integrity is required for a normal cardiac output response to exercise. We evaluated a rate-adaptive ventricular demand pacemaker (Telectronics, META-MV) which uses minute ventilation as the sensed physiological variable for adjusting pacing rate, in seven young patients with a mean age of 11.4 years. All patients had clinically significant bradycardia related to complete heart block (n = 4) or sinus node dysfunction (n = 3). For the entire group, paced heart rates increased from 70 +/- 10 beats/min to 151 +/- 19 beats/min with exercise testing. The onset of rate adaptation took less than 30 seconds. Changes in paced rate were linearly related to workload, VO2 (5.9 to 20.7 mL/min/kg) and minute ventilation (8-65 L/min). The decline in pacing rate after exercise was related directly to the gradual decrease in minute ventilation and VO2. Our data show that minute ventilation closely and accurately reflects the metabolic demands of varying workloads in children and can be used to achieve physiological, rate-adaptive pacing.

Adult

Medical management of three asymptomatic infants with severe valvar aortic stenosis.

We report on the clinical course and serial hemodynamic studies of three patients with severe valvar aortic stenosis diagnosed in the neonatal period. None of the children were symptomatic in the first year of life. In each case, a conservative initial management approach was adopted. Between the time of initial study (mean age, 1.8 months) and the follow-up at 12-27 months of age (mean, 14.3 months), mean left ventricular systolic pressure decreased from 151 to 125 mmHg, the mean peak systolic pressure gradient across the aortic valve decreased from 61 to 33 mmHg, and the mean calculated aortic valve area index increased from 0.24 to 0.60 cm2/m2. One patient was operated on for symptoms that appeared at 14 months of age. The patient followed longest is now 5 years old, is growing well, has a normal electrocardiogram and an echo-predicted left ventricular systolic pressure of 128 mmHg. This experience suggests that not all asymptomatic neonates with severe valvar aortic stenosis require surgical intervention early in life. In some, the aortic valve orifice may increase in size with somatic growth and obviate the need for surgery in infancy or early childhood.

Aortic Valve

Cellular electrophysiologic responses of isolated neonatal and adult cardiac fibers to d-sotalol.

The short-term cellular electrophysiologic actions of d-sotalol on isolated neonatal and adult canine ventricular myocardium and Purkinje fibers were evaluated using standard microelectrode techniques. d-Sotalol, 10(-6) to 10(-4)M, had no effects on action potential amplitude, maximal diastolic potential or action potential upstroke velocity (Vmax) in any neonatal or adult preparation. In five adult myocardial preparations, d-sotalol produced concentration-dependent increases in action potential duration at 50% (APD50) and 90% (APD90) repolarization and effective refractory period. In six neonatal myocardial preparations, d-sotalol produced a biphasic response; APD50, APD90 and effective refractory period decreased at 10(-6) and 10(-5)M. At 10(-4)M, these values increased significantly but to a lesser extent compared with values in adults. In seven adult Purkinje fibers, d-Sotalol significantly increased APD50, APD90 and effective refractory period in a concentration-dependent manner. All six neonatal Purkinje fibers responded in a biphasic manner, with values for APD50, APD90 and effective refractory period being less than control at 10(-6)M and near control values at 10(-5)M. At 10(-4)M, these variables were significantly increased, but to a lesser extent than in adult preparations. Our data confirm the typical class III effects of d-sotalol in adult cardiac tissues. The shortening of repolarization and refractoriness at lower drug concentrations in developing cardiac tissues may relate to age-dependent differences in cellular ionic function and basic electrophysiology.

Action Potentials

Effects of flecainide on the cellular electrophysiology of neonatal and adult cardiac fibers.

The acute cellular electrophysiologic actions of flecainide acetate on isolated neonatal and adult canine ventricular myocardium and Purkinje fibers were evaluated with standard microelectrode techniques. Flecainide, 0.1 to 10.0 micrograms/ml, produced concentration-dependent decreases in action potential amplitude, overshoot, and phase O Vmax of adult ventricular myocardium and Purkinje fibers. The greatest effects were on Vmax. Neonatal action potential characteristics were affected to a lesser degree by flecainide. Flecainide had disparate effects on myocardial and Purkinje fiber repolarization and refractoriness. In adult ventricular myocardium, action potential duration (APD) and effective refractory period (ERP) increased progressively with drug concentration. APD and ERP were increased to a lesser degree in neonatal myocardium. In adult Purkinje fibers, APD decreased progressively with increasing flecainide concentrations. ERP decreased at 0.1 and 1.0 microgram/ml, but returned to control values at 10.0 micrograms/ml. APD and ERP of neonatal Purkinje fibers responded to a lesser degree. At faster stimulation frequencies (2 to 4 Hz), flecainide produced significant frequency-dependent decreases in Vmax in adult Purkinje fibers. Such use-dependency was not evident in neonatal fibers. These data indicate a significantly lower sensitivity of immature cardiac tissues to the electrophysiologic effects of flecainide.

Action Potentials

Effects of prostaglandin E1 and indomethacin on fetal and neonatal lamb mesenteric artery responses to norepinephrine.

The effects of prostaglandin E1 (PGE1) and indomethacin on isolated fetal and neonatal lamb mesenteric artery responses to norepinephrine were investigated. PGE1 (1.5 micrometer) significantly reduced vasoconstriction responses to 0.5 to 5 micrometer norepinephrine. Indomethacin (1 micrometer) markedly potentiated the constrictor effects of 0.5 to 10 micrometer norepinephrine. PGE1 prevented the potentiating effect of indomethacin. Neither PGE1 nor indomethacin altered basal muscle tension. These results suggest that endogenous PGs modify adrenergic responses in the isolated mesenteric arteries of preterm and newborn lambs.

Animals

Prostaglandin E1 infusion in the hypoplastic left heart syndrome.

Aortic infusion of prostaglandin E1 in a moribund infant resulted in marked clinical improvement. Appropriate studies were conducted and the diagnosis of hypoplastic left heart syndrome was made. Although the outcome was unaltered, the improved clinical condition allowed us to exclude potentially curable defects such as aortic coarctation and interruption.

Aorta

Partial anomalous pulmonary venous connection to the azygous vein with intact atrial septum.

A five-year-old girl with partial anomalous pulmonary venous connection to the azygous vein with intact atrial septum is reported. The clinical and roentgenographic features suggested the correct diagnosis. Surgical correction of this previously unreported defect was accomplished by creating an atrial septal defect and constructing a baffle to direct the blood flow from the azygous vein to the left atrium.

Azygos Vein

Accuracy of echocardiography in assessing left ventricular dimensions and volume.

The accuracy of determining left ventricular function from echocardiography was assessed in 26 children (group I) with cineangiographically-determined normal left ventricular volume (LVV) and 28 children (group II) with large left ventricular volumes. Conventional LV echo dimensions were compared to the cineangiographic LV anterior-posterior minor axis (LVmA) and LVV. Very good correlations were found in group I between LV end-diastolic echo dimensions (LVEDD) and cine LVmA (r = 0.91) and between LVEDD and LV end-diastolic volume (LVEDV) by cine in group I (r = 0.86). In group II correlations were less accurate between LVEDD and diastolic LVmA and between LVEDD and LVEDV. There was poor correlation between the cine and echo percent of shortening (r = 0.41) and velocity of circumferential fiber shortening (VCF) (r = 0.51). This study demonstrates that M-mode echocardiography is a very useful method for determining LV dimensions in children with normal LV volume, but is less accurate in children with left ventricular volume overload or with abnormal septal orientation or postoperative status after ventriculotomy.

Adolescent

Sinus node dysfunction in children, adolescents, and young adults.

Thirty children and young adults with electrocardiographic and clinical evidence of sinus node dysfunction (SND) are described. Ages ranged from 3 days to 25 years. Of these, 10 patients had inappropriate sinus bradycardia, 16 had periods of sinus arrest, and 9 had sinoatrial exit block. A total of 22 patients had significant heart disease and SND developed in 13 after cardiac surgery. Eight patients with SND had no obvious heart disease. Seven patients were symptomatic, having either syncopal (three) or near-syncopal episodes (four). The three with syncope have received permanent demand pacemakers. Although significant SND occurs most frequently in children with congenital heart disease, particularly following corrective cardiac surgery, it may occur in otherwise normal individuals.

Adolescent

Total anomalous pulmonary venous connection. Report of ten cases and review of the literature.

Ten cases of total anomalous pulmonary venous connection, all presenting at or below the age of eight weeks, are presented: three below the diaphragm, two to the coronary sinus, one to the right atrium, three to the left vertical vein, and one to the right superior vena cava. Six patients had other cardiac anomalies, and surgical mortality represented 75%. A review of the subject is presented.

Cardiac Catheterization