[The persistence of fetal circulation syndrome].
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A 23 years old multiple pretransfused female with severe aplastic anemia (SAA) underwent bone marrow transplantation (BMT). Cyclophosphamide (200 mg/kg) was used for conditioning, donor buffy coat cells were given as rejection prophylaxis. Seven months after BMT regular menses started in the absence of exogenous hormonal manipulation and 21 months after BMT the patient became pregnant. Pregnancy was complicated by mumps in the 14th week. It was terminated at term by cesarean section. The normally developed newborn girl (3,450 gm) presented with a "persistent fetal circulation syndrome". After surgical correction of the patent ductus arteriosus Botalli the girl baby recovered quickly.
Extracorporeal membrane oxygenation is a pulmonary bypass procedure that has been employed in adults to provide temporary treatment for reversible acute pulmonary and cardiac insufficiency. The technology of membrane oxygenation has been used since 1977 in neonates with predictably fatal pulmonary failure due to respiratory distress syndrome, persistent fetal circulation or persistent pulmonary hypertension of the newborn, meconium aspiration syndrome, and congenital diaphragmatic hernia. The use of extracorporeal membrane oxygenation in older children with other pulmonary disorders has been limited. We report two cases of hydrocarbon aspiration involving petroleum-based products, both successfully treated with extracorporeal membrane oxygenation. A 15-month-old male infant who aspirated baby oil (light mineral oil) is particularly unusual owing to the generally expected low risk of aspiration with a hydrocarbon of such viscosity (greater than 60 Saybolt Universal Seconds). The second patient is a 16-month-old male infant who aspirated furniture polish (mineral seal oil). In both children severe intractable hypoxemia developed despite intensive ventilatory support, and they became candidates for alternative therapy. Extracorporeal membrane oxygenation provides a potentially life-saving option when a patient fails to respond to conventional therapy for hydrocarbon aspiration.
Ten patients clinically diagnosed as having perinatal aspiration syndromes were found to have pulmonary hypertension. These infants were either term or postmature babies and had the following characteristics: (1) systemic or suprasystemic levels of pulmonary artery pressure (range, 50 to 117 mm Hg); (2) a degree of pulmonary hypertension not related to the degree of aspiration evident on chest roentgenograms; (3) evidence of right-to-left shunting at the ductal or foramen ovale level; and (4) sustained severe hypoxemia despite 100% inspired oxygen concentration. The overall mortality for the group was 50%. Since these patients had marked clinical and physiologic similarities to patients previously reported as having the persistent fetal circulation syndrome (PFC), they were classified as having "PFC with aspiration." The existence of pulmonary hypertension should be suspected despite roentgenographic evidence of aspiration in any patient who also manifests the clinical characteristics of PFC because its treatment may alter the prognosis of such patients.
Extracorporeal membrane oxygenation (ECMO) is becoming an accepted therapeutic modality for newborn respiratory failure, but there is little information available regarding the prognostic determinants with this technique. One hundred thirty-five newborns treated with ECMO over a 4-year period were critically analyzed with regard to the influence that birth weight, gestational age, age at initiation of ECMO, best blood gases before ECMO, number of hours on ECMO, renal failure, intracerebral hemorrhage, and long-distance air transport had on survival. Infants with meconium aspiration and those undergoing long-distance transfer showed significant differences in blood gases before ECMO, with survivors having more normal pH and carbon dioxide tension values. Intracerebral hemorrhage and renal failure that developed during ECMO were grave prognostic signs, with few survivors in either group. These data show that ability to ventilate patients before ECMO, giving normal carbon dioxide tension and pH values, is an important prognostic sign in infants with meconium aspiration and undergoing long-distance transfer for ECMO, whereas renal failure and intracerebral hemorrhage are usually lethal complications of ECMO. Each center performing ECMO should continually reevaluate this invasive technique and its results and complications.
Prolonged continuous blood pressure (BP) and heart rate (HR) recordings from neonates of 35 to 42 weeks gestation were studied during and after ECMO. Data segments with significant deviation of BP were extracted for further analysis. The simultaneous changes in BP and HR were compared and the slope of the regression determined baroreflex sensitivity (BRS). Of 464 BP deviations, 98% produced curves with a negative slope consistent with the presence of a baroreflex. The average BRS was -1.0 +/- 0.8 bpm/mmHg (mean +/- S.D.) and curves were steeper during rising BP than falling BP (-1.1 +/- 0.9 beats/min per mmHg versus -0.9 +/- 0.6, P = 0.001). The baroreflex was more sensitive during ECMO than after ECMO to both rising BP (-1.0 +/- 0.5 beats/min per mmHg versus -0.7 +/- 0.5, P = 0.004) and falling BP (-1.0 +/- 0.6 beats/min/mmHg versus -0.7 +/- 0.5, P = 0.04). HR response curves obtained during different BP fluctuations on the same recording had varying threshold, consistent with acute resetting. One infant demonstrated chronic baroreceptor resetting over 3 days to a rise in resting BP. The near-term, critically ill neonate has an active baroreflex which is capable of resetting. ECMO was associated with accentuation of the baroreflex response.
The purpose of this study was to evaluate the significance and direction of shunts at the level of the foramen ovale or ductus arteriosus in full-term newborns with neonatal respiratory failure who were placed on extracorporeal membrane oxygenation (ECMO). A decrease in left ventricular dimension was expected when infants were placed on ECMO but did not occur. A left-to-right shunt was demonstrated at the ductal level in nine of 12 infants early in the course of ECMO before pulmonary resistance decreased. Presumably, the lack of change in the left ventricular dimension when infants were placed on bypass was due to a left-to-right shunt at the ductal level with ductal flow replacing the right heart output, being drawn into the bypass circuit.
To define the course of neonatal circulatory transition and to identify clinically relevant echocardiographic measurements in the diagnosis of persistent pulmonary hypertension, we prospectively studied 32 healthy term infants from 30 minutes to 24 hours after birth with color and quantitative Doppler echocardiography on the first day of life, and compared them with 33 term infants supported by mechanical ventilation for respiratory failure. Color Doppler imaging included measurements of cardiac output, left pulmonary artery flow, aortopulmonary pressure difference, ductal flow, left-to-right color-flow jet area of the ductus arteriosus, and ductal flow characteristics. In healthy infants the majority of measurable changes in cardiopulmonary hemodynamics had occurred by 8 hours after birth, although some degree of right-to-left ductal shunting was found up to 12 hours after birth. In the infants with respiratory failure, ductal flow and maximum aortopulmonary pressure difference measurements at 8, 12, and 24 hours showed a significant delay in ductal closure and a high incidence of persistent pulmonary hypertension, which correlated well with the severity of their respiratory failure. Factors such as aortopulmonary pressure difference, prolonged right-to-left shunting with decreased left pulmonary artery flow, and failure to develop a left-to-right ductal color-flow jet were found to be practical markers for assessing the course of neonatal circulatory transition in sick term infants.
A blinded, randomized, controlled study was designed to test whether multiple-dose surfactant therapy would improve pulmonary outcome in term infants with respiratory failure, resulting in a shortened period of extracorporeal membrane oxygenation (ECMO). Infants > or = 34 weeks of gestational age in severe respiratory failure and receiving ECMO were stratified by diagnosis and then randomly assigned to the treatment or the control group. Four doses of modified bovine lung surfactant extract (beractant) were administered to the surfactant group (n = 28), and an equal volume of air was administered to the control group (n = 28). Lung compliance was initially low in both groups; after treatment, values were higher with time in the surfactant group (F = 5.40, p = 0.026). The ECMO treatment period was significantly shorter in the surfactant group (mean +/- SD: 107 +/- 33 hours vs 139 +/- 54 hours for the control group; U = 232, p = 0.023). Tracheal aspirate concentrations of surfactant protein A were low in both groups, and then increased steadily to a higher level in the surfactant group (F = 2.58, p = 0.04). The overall incidence of complications after ECMO was decreased in the surfactant group (18% vs 46% for the control group; chi-square value = 5.004, p = 0.025). Radiographic scores, echocardiographic findings, incidence of intracranial or pulmonary hemorrhage and bronchopulmonary dysplasia, time to extubation, duration of oxygen therapy, and duration of hospitalization did not differ between the two groups. Beractant in this population improved pulmonary mechanics, increased surfactant protein A content in tracheal aspirate, decreased time on ECMO duration, and reduced disease complications.
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Congenital diaphragmatic hernia continues to be a critical problem in neonatal surgery. Despite the apparent simplicity of the anatomic defect, the physiology is complex, and survival remains uncertain. Surgical success has been achieved, but we recognize that the barrier to survival is pulmonary parenchymal and vascular hypoplasia as well as the complex syndrome of persistent fetal circulation. In many ways the problem of diaphragmatic hernia is as much of an enigma to today's physician-scientist as it was to Bochdalek in the nineteenth century. The treatment of respiratory distress after repair of congenital diaphragmatic hernia has brought out the most creative and innovative efforts of pediatric surgeons in both the laboratory and the intensive care unit.
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New trends in extracorporeal membrane oxygenation (ECMO) for respiratory failure in the newborn were reviewed. Following a decade of clinical research, ECMO is now the standard treatment for newborn respiratory failure when all other conventional less-invasive treatment options have been exhausted. As of July 2000, 15,525 newborns with respiratory failure treated with ECMO have been entered into the registry of the Extracorporeal Life Support Organization with an overall survival rate of 78%. The latest improvement in ECMO technology in this group of patients includes minimally invasive modes of vascular access through percutaneous approaches to minimize morbidity. However, with advances in modes of mechanical ventilation, including high-frequency ventilation and the introduction of inhaled nitric oxide, the use and necessity for ECMO have clearly diminished for newborn respiratory failure.
With the help of ECMO it is possible to save the lives of newborn infants suffering from severe respiratory distress syndrome not responding to conservative treatment. Using Bartlett's classic venous-arterial perfusion technique in ECMO the right arteria carotis communis had to be sacrificed. Thus, despite the life-saving character of this new method, the ligation of the carotid with all its possible complications had often been a major argument against using this therapy. We are now therefore trying to reconstruct the arteria carotis after decannulating the vessel after extracorporeal membrane oxygenation. In our 8 cases so far, post-op examinations showed no obstruction of blood flow in the vessel. No neurological deficiencies were recorded.
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High frequency oscillation (HFO) as rescue treatment for preterm infants with severe respiratory failure has been assessed and prognostic factors identified. Thirty six infants with a median gestational age of 27 weeks were studied. Immediately before transfer to HFO, the infants were receiving an inspired oxygen concentration of > or = 85% and/or a mean airway pressure of > or = 12 cm H2O and had a median alveolar-arterial oxygen gradient (A-aDO2) of 73.28 kPa (range 49.34-89.91). Seventeen infants subsequently died. Comparison of those 17 with the remaining 19 infants demonstrated that respiratory distress syndrome and persistent fetal circulation were associated with a significantly better outcome than pulmonary airleak. The A-aDO2 after two and six hours on HFO was significantly higher in those infants who survived compared with those who died. We conclude that a diagnosis of pulmonary airleak and failure to show early improvement in respiratory status indicate a poor prognosis when HFO is used as rescue treatment.
Mechanical assisted ventilation for neonatal respiratory failure is associated with residual lung disease. Because ECMO rests the lungs, it has been suggested that ECMO will prevent chronic lung disease in survivors. To determine whether or not ECMO survivors have evidence of pulmonary sequelae, we studied 19 infants who were treated with ECMO for neonatal respiratory failure. Ten infants still required supplemental oxygen or pulmonary medications or both to treat clinical lung disease during the first six months of life. Thoracic gas volume was normal. Pulmonary mechanics in ECMO survivors were compared with those of 13 preterm infants with BPD at similar age. We conclude that a significant proportion of ECMO survivors have residual abnormalities in pulmonary mechanics at 6 months of age. We speculate that neonatal lung injury due to meconium aspiration and other causes is a more important determinant of abnormal pulmonary sequelae than the method of treatment.
OBJECTIVE: Multiple platelet transfusions are invariably given to neonates on extracorporeal membrane oxygenation (ECMO), and no alternative to repeated transfusions exists. Before any alternatives, such as administration of thrombopoietic stimulators, could be contemplated, data regarding the number of platelet transfusions received by neonatal ECMO patients is needed, and the mechanisms that cause the thrombocytopenia of these patients must be better defined. As a step toward determining this, we analyzed the use of platelet transfusions in this group of neonates. We conducted a historic cohort study of neonates who were treated with ECMO to determine the number of platelet units received as a function of 1) days on ECMO, 2) medical diagnosis for which ECMO was instituted, and 3) type of ECMO used (venovenous [VV] vs venoarterial [VA]). METHODS: We reviewed the hospital records of all neonates who were admitted to the neonatal intensive care units at Shands Children's Hospital, Arnold Palmer Hospital for Children and Women, and Tampa General Hospital and treated with ECMO between January 1, 1995, and June 30, 2000. Data were expressed as the number of platelet transfusions versus number of days on ECMO, diagnosis for which ECMO was instituted, and type of ECMO used. RESULTS: Of the 234 ECMO patients, 81 were placed on VV, 138 were placed on VA, and 15 were converted from VV to VA. The average number of platelet transfusions received per day was 1.3 and varied by diagnosis and by type of ECMO. Neonates with meconium aspiration and sepsis required more platelet transfusions per day than neonates with other conditions. Infants who were converted from VV to VA required more transfusions per day (mean: 1.57) than did patients on VA (1.47) or VV (1.06). CONCLUSIONS: Platelet transfusions among neonates on ECMO are dependent of their medical diagnosis; they average 1.3 transfusions per day and are higher on VA than VV ECMO.