William W. Miller

Red-Cell 2,3-Diphosphoglycerate Levels in Subjects with Chronic Hypoxemia

Recently, Benesch et al.1 , 2 and Chanutin and Curnish3 demonstrated that the affinity of a hemoglobin solution for oxygen may be decreased by its interaction with organic phosphates. Both 2,3-diph...

The effects of deoxygenation of adult and fetal hemoglobin on the synthesis of red cell 2,3-diphosphoglycerate and its in vivo consequences

Patients over 1 month of age with arterial oxygen pressures of less than 60 mm Hg were found to have elevated red cell 2,3-diphosphoglycerate (2,3-DPG) levels and blood with a decreased affinity for oxygen. The increase in 2,3-DPG was proportional to the degree of hypoxemia. In patients under 1 month of age this relationship was not observed. Red cells from adults, but not newborns, showed rapid increases in 2,3-DPG when incubated under nitrogen. Adult, but not fetal, deoxyhemoglobin was shown to facilitate in vitro synthesis of 2,3-DPG by binding this organic phosphate and relieving the product inhibition of 2,3-DPG mutase. Throughout a wide range change in oxygen affinity as measured by the P(50) is linear with respect to the 2,3-DPG concentration; a change of 430 mmumoles of 2,3-DPG/ml of red blood corpuscles (RBC) resulting in a change of the P(50) of 1 mm Hg. It appears that the 2,3-DPG of the adults red cells responds rapidly to metabolic and environmental influences and in turn effects metabolism and the cellular environment. Many of these effects are not shared by the red cells of the newborn.

Transposition of the Great Arteries Results of Palliation by Balloon Atrioseptostomy in Thirty-one Infants

A nonsurgical method of creating atrial septal defects using a balloon catheter has been reviewed, and important procedural and technical details have been presented. The results of balloon atrioseptostomy in 31 infants with transposition of the great arteries have been reported and compared with those from the surgical creation of atrial septal defects. All 31 patients survived balloon atrioseptostomy without complications; 26 of them (84%) had effective immediate palliation, and 22 (71%) are long-term survivors. These results demonstrate that effective palliation of transposition of the great arteries, with or without associated ventricular septal defect, can be provided rapidly and safely by balloon atrioseptostomy. All patients with transposition and a large ventricular septal defect should have balloon atrioseptostomy and may also need pulmonary artery banding. For some of these patients banding may be postponed safely for many months, may occur spontaneously, or may be avoided completely.Atrioseptostomy by the balloon catheter technique requires neither sedation nor general anesthesia, and obviates the need for thoracotomy and cardiotomy. The rapidity with which it can be performed makes it particularly advantageous for the desperately hypoxic newborn with transposition of the great arteries who may be considered too sick to withstand major surgery.

Sensorineural hearing loss associated with Kawasaki disease.

In five children who met the diagnostic criteria for Kawasaki Disease, sensorineural hearing loss developed in association with the acute illness. The children, aged 7 months to 13 years, had deficits ranging from mild to profound bilateral sensorineural hearing loss. There were no associated neurologic abnormalities, and immunologic investigations and magnetic resonance imaging failed to reveal a cause. Treatment regimens differed among the children, but none had high salicylate levels (greater than 20 mg/dl) or received other ototoxic medications. Antiinflammatory therapy was not obviously beneficial in any case, and four of the children have persistent hearing deficits. We conclude that auditory involvement may be a complication of Kawasaki disease; screening of clinically affected children should be considered.

Comparison of Oxygen Poisoning of the Lung in Cyanotic and Acyanotic Dogs

Abstract Cyanosis was produced in dogs by the surgical creation of large intracardiac venoarterial shunts. Eight cyanotic and 10 acyanotic dogs were exposed, awake and unrestricted, to 1 atmosphere of 98 per cent to 100 per cent oxygen for two days. In each of the two groups of animals, a similar frequency and extent of pulmonary oxygen damage was found, as judged by the production of respiratory distress, atelectasis, alveolar edema and hemorrhage and abnormal surface tension of whole-lung extracts. Cyanosis did not protect against pulmonary injury from 100 per cent oxygen at 1 atmosphere for two days.

Hemodynamic effects of arteriovenous oxygenation with a small-volume artificial extracorporeal lung

Summary Extracorporeal perfusion, with a small-volume artificial lung interposed between a single femoral artery and vein, has proved capable of achieving highly significant increases in arterial pO 2 and pH, with a comparable decrease in arterial pCO 2 . The use of this technique in normal and hypoxic puppies does not result in any significant changes in cardiac output, cardiac work, or central venous pressure. Transitory changes in arterial blood pressure are of short duration, although a slight increase in pulse pressure persists. These data suggest that prolonged clinical use of this device could be expected to produce favorable changes in blood gas levels without adverse cardiovascular changes.

Ascending Aorta-Right Pulmonary Artery Anastomosis: Clinical Experience with Thirty-five Patients with Cyanotic Congenital Heart Disease

The results of ascending aorta-right pulmonary artery anastomosis in 35 patients are reported. The patients ranged in age from 18 hours to 12 years; 16 were less than 12 months old. Seventeen had tetralogy of Fallot, six had pulmonary atresia and a ventricular septal defect, and eight had tricuspid atresia with hypoplasia of the right ventricle. In six infants under 4 months of age with tricuspid atresia, an atrial septal defect was made by the balloon catheter technique prior to the shunt operation. Four patients initially required digitalis for left heart failure. Three patients died in the postoperative period. The remaining 32 patients have been followed up to 3 years and have done well and maintained adequate shunts.

Transposition of the Great Arteries with Ventricular Septal Defect Palliation by Atrial Septostomy and Pulmonary Artery Banding

Infants with transposed great arteries need atrioseptostomy for adequate intracardiac mixing of the systemic and pulmonic circulations. Those with ventricular septal defect and pulmonary artery hypertension, with or without other associated lesions, usually require further surgical palliation. Postseptostomy measurements of pulmonary artery pressures at systemic levels were obtained in ten infants with transposition of the great arteries and ventricular septal defects. Three had, in addition, patent ductus arteriosus, and two of these had aortic coarctation (one preductal with hypoplasia of the aortic arch). One had transposition of the great arteries, ventricular septal defect, and tricuspid atresia. Four of the patients had surgical septectomy; the remaining seven had balloon atrioseptostomy. All had pulmonary artery banding; the three ductus arteriosus lesions were ligated; and both coarctations were repaired (one with reconstruction of the aortic arch). The only operative death was in one of the patients with transposition of the great arteries and ventricular septal defect only. When the interatrial communication is adequate in patients with transposition, ventricular septal defect, and pulmonary artery hypertension, pulmonary vascular obstructive changes may be prevented by pulmonary artery banding and surgical correction of the associated lesions.

Intrapericardial teratoma associated with pericardial effusion: An acute surgical problem in infancy

Summary An intrapericardial teratoma associated with pericardial effusion was found in a 3 month old male member of a triple birth. The mass lesion was demonstrated by angiography and was successfully removed.

Changes in Pulmonary Vascular Resistance Following Closure of Ventricular Septal Defects

In five patients with pulmonary arterial hypertension (PAH) associated with ventricular septal or endocardial cushion defects, measurements of pulmonary vascular resistance (PVR) were made up to seven days after surgical closure. Before operation, the ratio of mean pulmonary arterial pressure [See Equation in PDF File] to mean systemic arterial pressure [See Equation in PDF File] averaged 0.77. Catheters placed at operation were used to measure pressure in the pulmonary artery, a systemic artery, and right and left atria. Cardiac output was determined by dye dilution. Controlled mechanical ventilation maintained a normal alveolar ventilation and arterial PO2. Postoperative [See Equation in PDF File], [See Equation in PDF File], and PVR were initially elevated, averaging 42 mm Hg, 0.57, and 1,132 dyne sec cm-5, respectively. These values decreased to 29, 0.38, and 459 in three to seven days in the four survivors, but rose in the single death. With unassisted ventilation and 100% 02, or with acidosis (pH 7.21), [See Equation in PDF File] and PVR increased as much as +177% and 570%, respectively. These studies show the relative pulmonary vasoconstricting effect of systemic acidosis and of spontaneous unassisted breathing with 100% O2. In patients with markedly elevated, but reversible, hyperkinetic pulmonary vascular resistance, the beneficial effects of prolonged mechanical ventilation enabled the pulmonary circulation to return toward normal.