Acute hemodynamic effects of increasing hemoglobin concentration in children with a right to left ventricular shunt and relative anemia.

The short-term effects of increasing hemoglobin concentration were evaluated at cardiac catheterization in seven children (aged 0.3 to 7.5 years) with a right to left ventricular shunt and relative anemia. Diagnoses were tetralogy of Fallot in six and L-transposition of the great vessels with ventricular septal defect and pulmonary stenosis in one. Before and 20 minutes after isovolumic partial exchange transfusion with 20 ml/kg packed red cells, the following variables were measured: hemoglobin, partial pressure of oxygen (PO2), oxygen consumption, oxygen saturation and pressure in the aorta, superior vena cava and right and left atria. After partial exchange transfusion, hemoglobin increased from 13.7 +/- 0.5 to 16.4 +/- 0.4 g/100 ml (p less than 0.001, mean +/- SEM). Aortic PO2 increased from 55.0 +/- 3.5 to 62.0 +/- 4.1 mm Hg (p less than 0.01) and aortic oxygen saturation increased from 84.3 +/- 2.3 to 90.9 +/- 1.3% (p less than 0.002). Effective pulmonary blood flow increased by 17% from 2.72 +/- 0.10 to 3.17 +/- 0.10 liters/min per m2 (p less than 0.01), and right to left shunt decreased by 59% from 1.44 +/- 0.29 to 0.59 +/- 0.10 liters/min per m2 (p less than 0.01). Systemic oxygen transport increased from 658 +/- 48 to 738 +/- 46 ml/min per m2 (p less than 0.002). After partial exchange transfusion, systemic vascular resistance increased from 15.9 +/- 1.1 to 20.0 +/- 1.4 units (p less than 0.01).(ABSTRACT TRUNCATED AT 250 WORDS)

Congenital pulmonary vein stenosis: structural changes in a patient with normal pulmonary artery wedge pressure.

A male infant is described who died at 13 months of age with stenosis of all extrapulmonary veins except the left upper vein. The pulmonary artery wedge pressure was normal, the first time this is reported in this condition. At autopsy, there were structural changes of the pulmonary arteries and veins in all lobes with or without pulmonary vein stenosis. Arterial changes-muscle extension, medial hypertrophy and decreased arterial size--analyzed quantitatively were found to be similar in all lobes. Venous medial hypertrophy was more marked in obstructed lobes. These anatomic changes are presumably due to fixed venous obstruction in the pulmonary lobes drained by stenotic veins and to high flow in the left upper lobe.

Accuracy of electrical impedance cardiography for measuring cardiac output in children with congenital heart defects.

This study determined whether noninvasive electrical impedance cardiography accurately measures systemic blood flow (cardiac output) in children with congenital heart defects. A total of 37 patients ranging in age from 2 to 171 months underwent complete right- and left-sided heart catheterizations that included simultaneous Fick and impedance measurement of cardiac output. Based on the diagnosis, 4 groups were formed consisting of a control group (n = 11) with no shunts, a group with intracardiac left-to-right shunting and an atrial septal defect (n = 7), another with a ventricular septal defect (n = 12) and an extracardiac left-to-right shunting with patent ductus arteriosus group (n = 7). Impedance values for systemic blood flow were compared with systemic and pulmonary blood flow obtained by the direct Fick method with measured oxygen consumption. The difference between impedance and Fick systemic blood flow was less than or equal to 5% in each of the 4 groups. The highest correlation between impedance and Fick systemic blood flow was with the atrial septal defect group (r = 0.89) and lowest with the ventricular septal defect and control (r = 0.69) groups. Fick pulmonary blood flow was significantly greater than impedance or Fick systemic flow in all 3 shunt groups. Impedance cardiography accurately measured systemic blood flow in children without shunts or valvular insufficiency. Likewise, systemic blood flow was accurately measured by impedance in the presence of intracardiac left-to-right shunts (atrial and ventricular septal defects) and extracardiac left-to-right shunts (patent ductus arteriosus).(ABSTRACT TRUNCATED AT 250 WORDS)

Radiologic and real time echocardiographic evaluation of the cyanotic newborn.

Prior to echocardiography, the recognition of serious heart disease in the cyanotic newborn or young infant could be extremely difficult. The profound hemodynamic changes taking place in the heart and lungs after birth influence the clinical manifestations of many cardiac disorders, and sometimes suggest the existence of a cardiac disorder when none is present. Real time echocardiography has revolutionalized the diagnosis of the cyanotic infant. If the reason for the infant's cyanosis or respiratory distress is not apparent from the history, physical examination, laboratory values, and chest radiograph; real time echocardiography should be performed to exclude or diagnose cyanotic congenital heart disease and persistent fetal circulation. This will prevent misdiagnosis in cyanotic infants and assure rapid and appropriate treatment.