Congenital isolated pulmonary valve incompetence: neonatal presentation and early natural history.
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Cardiac Catheterization↗
Biomedical subjects
W Berman
Publications and source records attributed to W Berman.
Pulmonary artery sling with severe tracheobronchial stenosis: repair in infancy by tracheal resection and pulmonary artery reimplantation.
Pulmonary artery sling is an unusual anomaly. The results of surgical therapy have been unsatisfactory in the past because of poor long-term left pulmonary artery patency and failure to address concomitant primary tracheobronchial stenoses. Refinement of suture materials and microsurgical techniques have allowed earlier surgical treatment of tracheal stenosis and have led to improved pulmonary artery patency rates. Intermediate results of primary one-stage repair with tracheal resection and left pulmonary artery reimplantation or translocation in early childhood have been promising. It remains to be seen whether late anastomotic tracheal stenosis will be a problem. This constitutes the first report of a case of one-stage repair with tracheal resection and left pulmonary artery reimplantation in an infant.
Anastomosis, Surgical↗
Nutritional issues in pediatric inflammatory bowel disease.
Malnutrition, characterized by weight loss, growth failure and micronutrient depletion, are prominent features of inflammatory bowel disease (IBD) in the pediatric age group. Accurate evaluation of the patient's nutritional status and appropriate nutritional support, whether enteral or parenteral, constitute integral parts of the management of the growing child with IBD. Over the past two decades, a number of studies have supported the potential use of nutritional therapy to induce remission and to control disease activity in symptomatic Crohn's disease. More recently, preliminary studies on the use of dietary supplements of marine-oil-derived omega-3 fatty acids have also indicated a beneficial effect in IBD patients. In parallel with these clinical trials, scientific research has recently focused on the concept that specific dietary alterations can modulate the immune response. Components of the diet that may have particular relevance to mucosal immunity and the pathogenesis of IBD include polyunsaturated fatty acids, nucleotides, and amino acids such as glutamine and arginine. Future research in the interactions between specific nutrients and the immune system will likely increase our understanding of the causes of IBD, as well as enhance the development of novel nutritional therapies for IBD patients.
Child↗
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↗
Cigarette smoking and neuroleptic-induced parkinsonism.
Several studies have reported an apparent protective effect of cigarette smoking for the risk of idiopathic Parkinson's disease (IPD). These observations are supported by neurochemical studies demonstrating enhancement of central dopaminergic neurotransmission by nicotine. We studied the prevalence and severity of neuroleptic-induced parkinsonism (NIP) in relation to cigarette smoking in a homogeneous sample of 130 psychiatric inpatients receiving long-term neuroleptic treatment. Despite the fact that smokers had significantly higher dosage of neuroleptics during the month prior to evaluation and longer exposure to medication, they presented with significantly less prevalence and severity of NIP than nonsmokers. These findings suggest that the inverse association between smoking and IPD may apply to NIP.
Antipsychotic Agents↗
Hemodynamic effects of ketamine in children undergoing cardiac catheterization.
Ketamine is used to supplement sedation during cardiac catheterization. We studied ketamine-induced circulatory changes in 28 acyanotic children (18 of whom had left-to-right shunts), aged 4-161 months (mean, 33 months). Oxygen consumption (VO2) was measured continuously. In the 18 patients with shunts, the pulmonary to systemic flow ratio fell slightly (2.3 +/- 1.1 to 1.8 +/- 0.4, p less than 0.05). In all patients, the ratio of pulmonary (PVR) to systemic vascular resistance (SVR) rose from 0.16 +/- 0.09 to 0.28 +/- 0.21, p less than 0.001. Ketamine increases VO2, heart rate, cardiac output, and pulmonary arterial pressure (PAP). The rise in PAP is more consistent than the rise in PVR; resistance changes were greatest in patients with elevated resting PVR (r = 0.54). Caution should be used in administering ketamine to selected subjects; moreover, ketamine can confuse interpretation of cardiac catheterization data, especially if VO2 is assumed and not measured.
Adolescent↗
Severe cardiac conduction abnormalities associated with atypical toxic shock syndrome.
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Adolescent↗
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↗
Liver transplantation in Virginia.
Survival rates ranging from 70-80% have been documented over the past three years in liver transplant patients at the Medical College of Virginia. Survival declined in patients with numerous complications of chronic liver disease and with significant deterioration in hepatic synthetic function. Early referral was a major factor contributing to survival.
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↗
Measurements of blood flow.
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Cardiac Output↗
Systemic oxygen transport in patients with congenital heart disease.
The physiology of oxygen delivery was studied in 118 stable patients from 3 months to 20 years old with congenital heart disease. During cardiac catheterization, oxygen consumption (VO2), arterial and venous blood gases and oxygen saturations (range 41% to 98%), hemoglobin concentration, diphosphoglycerate (2,3-DPG), and P50 levels were measured, and then cardiac output, systemic oxygen transport (SOT), arterial and venous oxygen contents, and the VO2/SOT ratio (fractional O2 extraction) were calculated. P50 averaged 31 mm Hg, compared with 27 mm Hg in 10 control children (p less than .01). The composite O2-hemoglobin dissociation curve in vivo was broad: Po2 varied from 37 to 65 mm Hg at 80% saturation. P50, 2,3-DPG, hemoglobin concentrations, and O2 saturation varied widely and inconsistently with Po2 and arterial and venous O2 content, but resulted in clustering of the arterial oxygen content near 165 +/- 23 (SD) ml/liter over a wide range of Po2 and hemoglobin concentrations. SOT varied in direct relation with flow (r = .82, p less than .001), but not with oxygen content, Po2, or P50. VO2 varied widely at normal or high levels of SOT, but decreased linearly at SOT levels below 400 ml/min/m2. Oxygen extraction varied inversely with venous O2 content, rising to about 50% and plateauing below venous contents of 100 ml/liter. O2 extraction did not correlate with Po2, arterial O2 content, or P50. These data suggest that: O2 saturation cannot be predicted or calculated accurately from measured Po2, but must be measured directly, 2,3-DPG, hemoglobin concentration, and P50 fluctuate to stabilize arterial oxygen content, SOT is determined primarily by cardiac output in subjects who are adapted chronically, O2 extraction rises, due to a fall in venous O2 content, to maintain VO2 as transport falls, below a critical level of SOT, O2 extraction ceases to rise and VO2 falls with further reduction in transport.
2,3-Diphosphoglycerate↗
Ouabain effects on oxygen physiology in anemic lambs.
We studied the effects of anemia and ouabain administration on cardiac function, oxygen physiology, and blood catecholamine levels in nine newborn lambs. We measured oxygen consumption continuously, along with traditional hemodynamic variables. Oxygen transport was calculated. Following baseline measurements, lambs were made anemic (mean hematocrit = 12%) by isovolemic exchange transfusion with Plasmanate, and measurements were repeated. Thereafter ouabain was administered as a 75 micrograms/kg bolus, followed by 30 min of infusion at a rate of 0.05 microgram/kg/min. Measurements were repeated at the conclusion of infusion. Anemia was associated with a heart rate related rise in left ventricular rate of pressure rise, a rise in oxygen consumption, an increase in cardiac output, a decrease in systemic vascular resistance, and a rise in fractional oxygen extraction (oxygen consumption/transport). Following ouabain administration, cardiac output, oxygen consumption, and stroke work fell, but left ventricular rate of pressure rise and the extraction ratio did not change. Serum levels of epinephrine and norepinephrine rose with sustained anemia and ouabain infusion. In this setting, ouabain effects are manifested primarily by alterations in oxygen transport and metabolism rather than by changes in traditional assessments of left ventricular contractile function.
Anemia↗
Noninvasive assessment of cardiopulmonary function in critically ill infants and children.
Echocardiography and Doppler echocardiography allow for the accurate noninvasive assessment of cardiac structure and function. Two-dimensional echocardiography accurately demonstrates both normal and abnormal intracardiac and great vessel anatomy and assists in differentiating cardiac from noncardiac causes of respiratory distress. M-mode echocardiography allows for documentation of cardiac chamber size and left ventricular function. Doppler echocardiography is used to measure cardiac output, assess AV and semilunar valve function, detect abnormal flow patterns within the heart and great vessels, and assist in the assessment of pulmonary artery pressures. This article discusses the uses of all these modalities as they apply to the critically ill infant and child.
Blood Pressure↗
Communicating hydrocephalus: a reversible complication of the Mustard operation with serial hemodynamics and long-term follow-up.
Severe superior vena caval (SVC) obstruction following the Mustard operation resulted in substantially increased intracranial pressure in 3 patients. A pathophysiological mechanism is postulated to explain the cerebral edema in 1 and the communicating hydrocephalus in 2. Following surgical relief of the obstruction in the latter 2, there was a decrease in both the rate of head growth and the ventricular size. It is of note that despite the angiographic appearance of azygous decompression, the pressure elevation from the SVC obstruction was transmitted to the brain. The long-term follow-up at three and four years of the 2 infants with communicating hydrocephalus suggests that aggressive treatment is compatible with a favorable outcome.
Brain Edema↗
Distinctive gastrointestinal anomaly associated with Coffin-Siris syndrome.
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Abnormalities, Multiple↗
Long-term follow-up of bronchopulmonary dysplasia.
We observed 10 children with bronchopulmonary dysplasia, evaluated initially by cardiac catheterization (mean age 18 months), for an average of 4.4 years. Age at last evaluation averaged 5.8 years; subjects reside in and around Albuquerque, N.M. (altitude 5000 ft). At initial cardiac catheterization, mean pulmonary artery pressure was 40 mm Hg, pulmonary vascular resistance index 8.9 units, and intrapulmonary shunt fraction was high; pulmonary wedge angiograms were normal. Over the period of follow-up the group has done poorly. Four of the 10 continue to receive home oxygen therapy, but none requires inotropic or diuretic therapy; four children have marked developmental or motor delays. Nine of 10 patients have abnormalities of respiratory function on spirometric testing. Four patients underwent recatheterization because of clinical indications; two had large atrial level left-to-right shunts not found on initial study. Reductions in pulmonary artery pressure (55 to 37 mm Hg) and pulmonary vascular resistance (11.9 to 7.8 units) occurred between the two studies in these four patients (average study interval 4.0 years); the still elevated levels of pressure and resistance fell further in response to 40% O2 administration. Pulmonary wedge angiograms were abnormal in each restudied patient. Although not uniformly bleak, the long-term outlook for children with severe bronchopulmonary dysplasia is diverse and guarded.
Blood Pressure↗