William W. Fox

Persistent pulmonary hypertension in the neonate: Diagnosis and management

PPHN should be recognized as a clinical condition associated with a number of pulmonary and systemic diseases. Present therapy has resulted in increased survival, but the aggressive methods required to produce improvement necessitate a clear understanding of the underlying pathophysiology in order to minimize sequelae.

Awake apnea associated with gastroesophageal reflux: A specific clinical syndrome

Fifteen infants with a specific clinical history including awake apnea were evaluated and compared with a control group of infants, using 24-hour studies of esophageal pH, nasal thermistor, impedance pneumography, and heart rate. Thirteen of the 15 children with awake apnea had clearly documented episodes of airway obstruction in associated with gastroesophageal reflux occurring at least twice during the study (mean 3.9 +/- 0.7, range 2 to 9). The control group did not show similar findings. All 15 children with awake apnea had frequent episodes of gastroesophageal reflux. Treatment with home monitoring and reflux precautions was successful in 10 of 15. Five children received therapy with urecholine hydrochloride because of continuing episodes of reflux-associated apnea. Two children subsequently required Nissen fundoplication, primarily for symptoms of severe esophagitis. Our data suggest that in children with awake apnea, the apnea is associated with gastroesophageal reflux. Medical management is usually successful, but fundoplication may be needed in refractory cases.

Physiologic factors affecting pulmonary artery pressure in infants with persistent pulmonary hypertension.

Indwelling pulmonary artery catheters were used for continuous monitoring of pulmonary artery pressure in ten infants with severe persistent pulmonary hypertention of the newborn. The labile nature of pulmonary artery pressure, with changes up to 50 mm Hg, was documented. Pulmonary artery pressure in the eight infants with suprasystemic pulmonary hypertension was analyzed at the time of maximum decrease in pressure (mean 36.1 mm Hg) and physiologic measurements were compared over an eight-hour period. During the study period when the infants were hyperventilated, as the Paco2 decreased from 48.9 to 28.3 mm Hg (P less than 0.02) the mean pulmonary artery pressure decreased by 36 mm Hg (P less than 0.001) to subsystemic pressure levels, and the mean AadeltaO2 decreased by 146 mm Hg (P less than 0.001). After the decrease in pulmonary artery pressure, patients were mechanically ventilated to maintain Paco2 in the range of 25 to 30 mm Hg until pulmonary hypertension gradually resolved in the six survivors.

Improved oxygenation and lung compliance withprone positioning of neonates

Fourteen intubated infants recovering from neonatal respiratory disease had arterial blood gases and lung mechanics measured in the supine position and in two variants of the prone position. Prone positioning resulted in significant increases in mean (+/- SEM) arterial oxygen tension (Pa(o2 70.4 +/- 2.5 to 81.1 +/- 4.4mm Hg), dynamic lung compliance (1.7 +/- 0.24 to 2.55 +/- 0.37 ml/cm H2O),and tidal volume (8.6 +/- 1.0 to 10.5 +/- 1.2 ml) when all prone values were compared to supine values. Prone positioning with the abdomen protruding freely, when compared to all supine values, was associated with significantly increased dynamic lung compliance and tidal volume. Values for prone-abdomen free were not significantly different from values for prone-abdomen restricted. This suggests that there are clinical benefits from prone positioning in neonates recovering from respiratory disease.

Improvement in Lung Mechanics as a Function of Age in the Infant with Severe Bronchopulmonary Dysplasia

Summary: Pulmonary function tests were performed in two groups of infants with bronchopulmonary dysplasia; a group less than 7 months of age with severe ventilator-dependent respiratory failure (Group A), and a group 7–22 months of age during resolution of their disease (Group B). Group A patients had significantly elevated minute volume, low specific compliance, elevated inspiratory and expiratory pulmonary resistance, and low functional residual capacity. Group B patients also demonstrated elevated minute volume, whereas specific compliance, inspiratory pulmonary resistance and functional residual capacity were within normal limits, and expiratory pulmonary resistance was only slightly above normal. With the exception of minute volume, the differences between the groups were significant (P < 0.05). Sequential studies of resistance and compliance over 4–5 months in two patients in the younger group demonstrated values that approached or achieved normal range. It is concluded that pulmonary mechanics improve with age in the infant with severe bronchopulmonary dysplasia.Speculation: The high minute ventilation demonstrated in children with branchopulmonary dysplasia results from increased dead space ventilation. With low compliance and high resistance the young infant cannot sustain the high minute ventilation required, and respiratory failure ensues, requiring mechanical ventilation. As the child grows, chest wall strength, compliance, and resistance improve, allowing the infant to sustain a high spontaneous minute ventilation, and thus to tolerate gradual reduction and eventual removal of mechanical ventilatory support.

Pulmonary physiotherapy in neonates:Physiologic changes and respiratory management

To investigate physiologic alterations in respiratory function associated with chest physiotherapy, arterial blood gases, respiratory patterns, lung mechanics, and functional residual capacity were measured in 13 neonates (weights 1.25 to 3.20 kg) during the control period, after vibration of the chest and suctioning, after hyperventilation, and two hours after suctioning. Compared to control values, mean PO2decreased significantly after suctioning to 43 mm Hg and increased significantly after hyperventilation to 78 mm Hg. There was a significant decrease in inspiratory resistance and a trend toward decrease in expiratory resistance after suctioning, with return to control levels after hyperventilation. Respiratory rate increased significantly after suctioning. Functional residual capacity, dynamic lung compliance, and tidal volume, as well as PCO2 and base excess, were not changed appreciably throughout the protocol. Because of potentially severe hypoxemia, this study suggests that suctioning and hyperventilation are not warranted on a routine basis in infants recovering from respiratory diseases.

The Clinical Profile of the Newborn with Persistent Pulmonary Hypertension Observations in 19 Affected Neonates

In 19 neonates with severe cyanosis, normal chest x-rays, anatomically normal hearts, and a high incidence of perinatal complications, the clinical course was characterized by variable sustained cyanosis. Cardiac catheteriza tion data showed high systemic or suprasystemic pulmonary artery pressure with right to left intracardiac shunting via the foramen ovale and ductus arteriosus. Arterial oxygen tension at an inspired oxygen concentration above 65 per cent was helpful in distinguishing these patients from those with congenital heart disease, and for predicting prognosis.

Effect of heat shielding on convective and evaporative heat losses and on radiant heat transfer in the premature infant.

Ten premature infants nursed on servocontrolled radiant warmer beds were studied in three environments designed to alter one or more factors affecting heat transfer (convection, evaporation, and radiation). In the control environment, infants were nursed supine on an open warmer bed. The second environment (walled chamber) was designed to reduce convection and evaporation by placing plastic walls circumferentially around the bed. In the third environment convection and evaporation were minimized by covering infants with a plastic blanket. Air turbulence, insensible water loss, and radiant warmer power were measured in each environment. There was a significant reduction in mean air velocity in the walled chamber and under the plastic blanket when compared to the control environment. A parallel decrease in insensible water loss occurred. In contrast, radiant power demand was the same for control and walled environments, but decreased significantly when infants were covered by the plastic blanket. This study suggests that convection is an important factor influencing evaporation in neonates nursed under radiant warmers. The thin plastic blanket was the most effective shield, significantly reducing radiant power demand.

Changes in pulmonary function during the diuretic phase of respiratory distress syndrome

To evaluate the relationship between improvement in pulmonary function and spontaneous diuresis in respiratory distress syndrome, nine premature infants requiring mechanical ventilation for RDS were studied at a mean age of 11.9 hours prior to the onset of diuresis, at onset of diuresis, at maximum urine output (mean age 44.9 hours), and at 24 hours after maximum urine output. Prior to diuresis functional residual capacity decreased from mean +/- SEM of 16.2 +/- 2 to 13.3 +/- 1.2 ml/kg, and dynamic lung compliance decreased from 2.5 +/- 0.3 to 1.8 +/- 0.3 ml/cm H2O (P less than 0.05), indicating that the respiratory disease was worsening. There was no significant change in alveolar-arterial oxygen gradient, peak inflating pressure, or rate of intermittent mandatory ventilation over this period. At the time of maximum urine output, however, FRC had increased 36% (P less than 0.05). CL had increased by 60% to 2.8 +/- 0.4 ml/cm H2O (P less than 0.025), AaDO2 had decreased from 246 +/- 27 to 184 +/- 30 torr (P less than 0.005), and PIP had decreased from 14.9 +/- 2.2 to 11.3 +/- 2.1 cm/H2O (P less than 0.05). On follow-up study 24 hours after maximum urine output, there was no further significant improvement in FRC, CL or PIP, but IMV rate and AaDO2 continued to decrease. These data show that the pulmonary function in RDS deteriorates until the onset of diuresis, after which it rapidly improves. This diuresis may represent the removal of excess lung liquid and seems necessary for improvement in RDS.

The diuretic phase of respiratory distress syndrome and its relationship to oxygenation

To determine the relationship between improvement in pulmonary function and diuresis in respiratory distress syndrome, ten consecutive premature infants requiring mechanical ventilation for severe RDS were studied. Every infant had a diuresis (output/intake greater than 80%), which began at 26 to 34 hours of life and which lasted for an additional 64-72 hours. The diuresis preceded significant improvement in AaDo2 and ventilator settings (IMV, PIP, PEEP) by 52 hours. There was a significant decrease in body weight among all study infants during the first four days of life despite an increase in fluid intake. This study suggests a relationship in RDS between improvement in oxygenation and removal of interstitial lung edema.