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Background. Congenital diaphragmatic hernia (CDH) is a malformation of the diaphragm that allows bowel to enter the thoracic cavity, resulting in pulmonary hypoplasia and pulmonary hypertension. Approximately 50% of CDH patients are treated with extracorporeal membrane oxygenation (ECMO). The optimal gestational age for delivery of term infants with CDH at high risk for requiring ECMO is not known. The goal of this study was to compare survival of infants with CDH receiving ECMO born early term (38 0/7-39 6/7 weeks&apos; gestation) with those born late term (40 0/7-41 6/7 weeks&apos; gestation). Changes in survival rates of term infants and the factors associated with these changes were assessed over the 25 years that ECMO has been available. Methods. Design. Retrospective cohort study of infants with CDH treated with ECMO. Data Sources. The Extracorporeal Life Support Organization registry of patients treated at active Extracorporeal Life Support Organization centers from April 1976 through June 2001. Analysis. Survival and clinical predictors of survival were compared between infants born early term (38 0/7-39 6/7 weeks&apos; gestation) and infants born late term (40 0/7-41 6/7 weeks&apos; gestation). Changes in survival rates over time and factors associated with survival were evaluated. Results. Among full-term infants with CDH treated with ECMO, late-term compared with early-term delivery was associated with improved survival (63% vs 53%). Among full-term survivors of ECMO, late-term infants spent less time on ECMO (181 vs 197 hours) and less time in the hospital (60 vs 67 days). In multivariate analysis, greater birth weight, higher 5-minute Apgar score, higher arterial pH and PC[O.sub.2] < 50 torr before ECMO, and absence of a prenatal diagnosis of CDH were associated with survival. Since the late 1980s, survival of infants with CDH requiring ECMO decreased from 63% to 52%. The decreased survival rate was associated with increased rates of prenatal diagnosis, early-term delivery, lower birth weight, longer ECMO runs, and more frequent complications on ECMO. Conclusions. Among term infants with CDH receiving ECMO, late-term delivery compared with early-term delivery is associated with improved survival, shorter ECMO duration, shorter hospital length of stay, and fewer complications on ECMO. These data suggest that, at least for the ~50% of CDH patients treated with ECMO, outcomes for infants with CDH may be improved by delay of elective delivery until 40 completed weeks of gestation. Pediatrics 2002;110:590-596; congenital diaphragmatic hernia, extracorporeal membrane oxygenation, survival, gestational age, Extracorporeal Life Support Organization Registry., ABBREVIATIONS. CDH, congenital diaphragmatic hernia; iNO, inhaled nitric oxide; ECMO, extracorporeal membrane oxygenation; ELSO, Extracorporeal Life Support Organization; LOS, length of stay; OR, odds ratio. Congenital diaphragmatic hernia (CDH), which [...]

Survival in infants with congenital diaphragmatic hernia (CDH) may depend on adequate lung volume and effective use of extracorporeal membrane oxygenation therapy (ECMO). Circulation of blood through an external oxygenator can be used in infants with abnormal lung development and function. Researchers reviewed the care of 90 infants with CDH, of whom about 60% survived. ECMO was highly effective in reducing high blood pressure in the lungs. Infants required a minimum of 45% predicted lung volume to survive. Infants with smaller lungs had inadequate oxygenation., Objectives: There is a wide variation in published mortality from congenital diaphragmatic hernia (CDH). The prevailing opinion is that this variation is related directly to the degree of pulmonary hypoplasia. Our aim was to test the hypothesis that other factors are important for outcome. The specific objectives of this study were: 1) to quantitate the degree of lung hypoplasia and pulmonary arterial wall thickness in infants eligible for, and treated with, extracorporeal membrane oxygenation (ECMO), using postmortem analysis of lung DNA, wet lung weight, lung volume, and vessel morphometrics; 2) to correlate the degree of lung hypoplasia and vascular changes with functional tests of oxygenation and estimated right ventricular systolic pressures (RVSP); 3) to determine the minimum lung volume necessary for survival; and 4) to determine contributory clinical factors as potential causes of death in ECMO-treated infants with CDH. Methodology. We retrospectively analyzed all 90 infants with CDH admitted consecutively over a 9-year period to a children&apos;s hospital with an ECMO program. Infants were categorized as lived or died, with or without ECMO. Indication for ECMO was an evolving process; however, in general, it was the therapy of last resort for pulmonary insufficiency. Clinically, the single best oxygenation index before ECMO or CDH repair while on conventional ventilation, and serial echocardiograms before, during, and after ECMO, were obtained. Twelve of 14 infants dying with ECMO and 6 of 12 without ECMO had postmortem examinations. Lung volume, DNA content, wet weights, and arterial wall thickness at the level of alveolar ducts were measured in both lungs. Postmortem morphometric findings were correlated with in vivo tests of cardiopulmonary function and contributory clinical factors in mortality. Results. Sixty-three percent of all infants with CDH and 61% of ECMO-treated infants lived. All infants with CDH requiring ECMO had elevated RVSP/systolic systemic blood pressure ratios before ECMO (0.98 [+ or -] 0.24). Eighty-eight percent of ECMO-treated infants with CDH decreased this ratio to [is less than] 0.5 within 14 days, regardless of lung size. However, infants dying with normal ratios still had increased arterial wall thickness and muscle in both lungs. In infants whose lung volume, DNA, and weight were [is greater than] 45% of values predicted for age-matched controls, the oxygenation index ranged from 4 to 29, significantly less than that in infants with values [is less than] 45% of predicted values (range, 25 to 133). We speculate that eight infants with lung volumes [is greater than] 45% of that for controls died from potentially preventable surgical and medical complications. Conclusion. A minimum lung volume of 45% of the value predicted from age-matched controls is required for survival in ECMO-treated infants. The RVSP/systolic systemic blood pressure ratio can be reduced with ECMO to [is less than] 0.5 in the majority of infants, even with lung volumes inadequate for survival. We speculate that 9% of infants with adequate lung volume were potentially survivable, but died of medical and surgical complications. Pediatrics 1998;101:289-295; lung volume, vessels, diaphragmatic hernia., ABBREVIATIONS. CDH, congenital diaphragmatic hernia; ECMO, extracorporeal membrane oxygenation; RVSP, right ventricular systolic pressure; V-V, veno-veno; OI, oxygenation index; SBP, systolic systemic blood pressure; FRC, functional residual capacity. The high [...]

Treatment with inhaled nitric oxide (INO) does not appear to reduce the death rate or the need for a specialized ventilator support called extracorporeal membrane oxygenation (ECMO) among newborns with congenital diaphragmatic hernia (CDH). Fifty-three newborns with CDH but no known heart disease who required ventilation were treated with either IN0 or 100% oxygen (control group). Forty-eight percent of the infants treated with IN0 and 43% of the control group died. Eighty percent of the group treated with IN0 and 54% of the control group required ECMO. Bleeding episodes within the brain, intestines and lungs were similar., Objective. We designed and conducted a randomized, double-masked, controlled multicenter study to determine whether inhaled nitric oxide (INO) in term and near-term infants with congenital diaphragmatic hernia (CDH) would reduce the occurrence of death and/or the initiation of extracorporeal membrane oxygenation (ECMO). Patients and Methods. Infants of 34 weeks gestation or more, [is less than]14 days of age with CDH, without known structural heart disease, requiring assisted ventilation for hypoxemic respiratory failure with two oxygenation indices (OIs) of 25 or more at least 15 minutes apart, were eligible for this trial. Infants were centrally randomized and then received masked treatment with 20 ppm NO or 100% oxygen as control. Infants with less than a full response to 20 ppm NO (increase in Pa[O.sub.2] [is greater than] 20 Torr) after 30 minutes were evaluated at 80 ppm NO/control study gas. Results. The 28 control and 25 treated infants enrolled by the 13 participating centers were not significantly different at randomization for any of the measured variables including prerandomization therapies and initial OIs (45.8 [+ or -] 16.3 for controls, 44.5 [+ or -] 14.5 for INO). Death at [is less than]120 days of age or the need for ECMO occurred in 82% of control infants compared with 96% of INO infants (ns). Death occurred in 43% of controls and 48% of the INO group (ns), and ECMO treatment was used for 54% of control and 80% of INO-treated infants. There was no significant improvement in Pa[O.sub.2] (A Pa[O.sub.2] 7.8 [+ or -] 19.8 vs 1.1 [+ or -] 7.6 Torr, ns) nor significant reduction in OI (-2.7 [+ or -] 23.4 vs 4.0 [+ or -] 14.8, ns) associated with INO treatment. Mean peak nitrogen dioxide ([NO.sub.2]) concentration was 1.9 [+ or -] 1.3 ppm and the mean peak methemoglobin was 1.6 [+ or -] 0.8 mg/dL. No infant had study gas discontinued for toxicity. There were no differences between the control and INO groups for the occurrence of intracranial hemorrhage, specific grades of intracranial hemorrhage, periventricular leukomalacia, brain infarction, and pulmonary or gastrointestinal hemorrhages. Conclusions. Although the immediate short-term improvements in oxygenation seen in some treated infants may be of benefit in stabilizing responding infants for transport and initiation of ECMO, we conclude that for term and near-term infants with CDH and hypoxemic respiratory failure unresponsive to conventional therapy, inhaled NO therapy as used in this trial did not reduce the need for ECMO or death. Pediatrics 1997;99:838-845;, ABBREVIATIONS. CDH, congenital diaphragmatic hernia; HFOV, high-frequency oscillatory ventilation; ECMO, extracorporeal membrane oxygenation; NO, nitric oxide; [NO.sub.2], nitrogen dioxide; EDRF, endothelium-derived relaxing factor; INO, inhaled nitric oxide; PPHN, persistent pulmonary [...]

Functional residual capacity (FRC) of the lungs may predict survivors of congenital diaphragmatic hernia (CDH). With CDH, a flaw in the development of the diaphragm allows the viscera to enter the chest cavity, compressing the lungs. Symptoms range from mild pulmonary insufficiency to severe respiratory failure. CDH can cause underdevelopment of the lungs. It would be helpful to determine the prognosis of infants before undertaking expensive and potentially futile therapies. Researchers measured FRC and airflow in 25 infants born at full term with CDH. Overall, 60% survived, including all seven infants requiring only mechanical ventilation. The remaining 18 infants required extracorporeal membrane oxygenation for severe respiratory failure. Fifteen of these infants required patches of synthetic material to repair the hernia, only two of whom survived. All infants who died had FRC measurements below 9 milliliters per kilogram. Only two survivors had FRC measurements this low., Objectives. To determine whether fatal pulmonary hypoplasia, as assessed by functional residual capacity (FRC), can be distinguished from other reversible causes of respiratory failure in infants with congenital diaphragmatic hernia (CDH). Methods. In the present study, 25 term neonates having CDH without other anomalies (mean birth weight [+ or -] SD, 3.25 [+ or -] 0.50 kg) were enrolled prospectively into a protocol evaluating pulmonary function. Lung compliance ([C.sub.L]) and FRC were measured before diaphragmatic repair and compared with the highest oxygenation index (01) and lowest [Paco.sub.], also obtained preoperatively. Pulmonary function assessment was repeated after diaphragm repair on postoperative days 3 and 7. [C.sub.L] was determined by esophageal manometry and pneumotachography, and FRC was determined by helium diluticln. Results. Fifteen infants (60%) survived to hospital discharge. Eighteen (72%) required extracorporeal membrane oxygenation (ECMO) for support, and of these, 8 (44%) survived. [Paco.sub.2] was similar preoperatively in infants grouped as survivors without ECMO, survivors with ECMO, and nonsurvivors. In nonsurvivors (all of whom received ECMO), the preoperative OI was significantly higher (51 [+ or -] 21), [C.sub.L] was less (0.11 [+ or -] 0.04 mL/cm of water per kg), and FRC was smaller (4.5 [+ or -] 1.0 mL/kg) than in the survivors who required ECMO (26 [+ or -] 18, 0.18 [+ or -] 0.08 mL/cm of water per kg, and 12 :t 5 ml/kg, respectively), as well as in the survivors without ECMO, (6 [+ or -] 2, 0.32 [+ or -] 0.16 mL/cm of water per kg, and 15.8 [+ or -] 4 mL/kg, respectively). The group surviving with ECMO had a higher OI than the infants surviving without ECMO. All nonsurviving infants had FRCs of less than 9.0 mL/kg preoperatively. In contrast, only 2 of the 15 survivors had preoperative FRCS less than 9 mL/kg. Conclusions. The results of this study suggest that preoperative assessment of FRC may predict fatal pulmonary hypoplasia in most infants with CDH. Pediatrics 1995;96:1117-1122; congenital diaphragmatic hernia, pulmonary function, lung volume., ABBREVIATIONS. CDH, congenital diaphragmatic hernia; [C.sub.L], pulmonary compliance; ECMO, extracorporeal membrane oxygenation; FRC, functional residual capacity; OI, oxygenation index; MAP, mean airway pressure; ANOVA, analysis of variance. Recent advances in [...]

The outcomes of children who have extracorporeal membrane oxygenation (ECMO) may depend on the indication for ECMO. ECMO is used in newborns who are unresponsive to other therapies for respiratory failure. The jugular vein or sometimes the carotid artery is used to shunt blood outside the body to a lung machine. Researchers evaluated outcomes at six and 12 months for children who had ECMO as newborns, of whom 21 had meconium aspiration syndrome (MAS) and 28 had congenital diaphragmatic hernia (CDH). All infants with MAS survived versus 68% of infants with CDH. At one year, all MAS infants could feed normally, whereas 36% of CDH infants required tube feeding. Gastroesophageal reflux had resolved in all MAS infants, whereas 43% of CDH infants still had symptoms. Seventeen percent of MAS infants had bronchopulmonary dysplasia versus half the CDH group. Only one child in the MAS group had abnormal muscle tone, versus three-fourths of the CDH group., Objective. Although extracorporeal membrane oxygenation (ECMO) has been responsible for the improved survival of infants with cardiorespiratory failure, its use over the last decade has raised concern as to the health of the survivors and the severity of neurodevelopmental sequelae. Though infants meeting ECMO criteria have a variety of reasons prompting the use of this therapy, most studies to date have simply reported outcome on the entire population that has survived without regard to the original nature of the child&apos;s illness. The purpose of this study was to determine the type and extent of health-related problems and neurodevelopmental sequelae in infants requiring ECMO therapy and the association of these findings with the infants&apos; primary diagnosis. Methods. Eighty-two neonates required ECMO therapy between May 1990 and December 1993. The most common diagnoses prompting ECMO therapy included 26% with meconium aspiration syndrome, 34% with congenital diaphragmatic hernia (CDH), 16% with persistence of the fetal circulation, and 9% with sepsis. Information concerning the hospital course was obtained through chart review, and the infants were seen at 6 and 12 months of age for medical and neurodevelopmental follow-up. Data were analyzed using descriptive statistics and Fisher&apos;s exact test, t-tests, and analysis of variance where appropriate. Assessment of hospital course and discharge data focused on the four main diagnostic groups, whereas follow-up data were further limited to the two most frequently encountered groups (meconium aspiration syndrome and CDH). Results. Overall survival was 79%. Significant differences in survival were noted based on primary diagnostic category. Those with CDH fared the worst, with an overall survival rate of 68% and a more complicated hospital course with a longer duration of ECMO. At discharge, the CDH group demonstrated a greater incidence of bronchopulmonary dysplasia, gastroesophageal reflux, feeding dysfunction, and hypotonia. No significant differences were noted in the incidence of intraventricular hemorrhage, cerebral infarction, extra-axial fluid collection, or seizures. Hearing loss was uncommon. During the first year of life, although no differences were noted in growth rate, infants in the CDH group continued to experience a higher incidence of gastroesophageal reflux (43%) and feeding dysfunction, with 36% of this group requiring tube feedings for nourishment. Although 40% of the entire ECMO population was diagnosed with bronchopulmonary dysplasia before initial discharge, by 1 year of age, 50% of those with CDH versus 17% of those with meconium aspiration syndrome continued to be clinically symptomatic. Although the ECMO population as a whole scored in the normal range developmentally, CDH infants had significantly lower motor and slightly lower cognitive scores at 1 year of age. Despite finding abnormal muscle tone in a high percentage of the entire ECMO population at discharge, most demonstrated resolution by 1 year of age. Of the CDH infants, however, 75% continued to evidence some degree of hypotonicity, which affected acquisition and quality of gross motor skills. Conclusion. Despite the impact that ECMO has had on the survival of infants with severe respiratory failure, the efficacy of ECMO cannot be assessed accurately without an analysis of the extent and morbidity in the surviving population. Most centers are reporting relatively low morbidity for the entire ECMO population. However, upon separating this population into primary diagnostic categories, we found that the CDH population encountered a greater number of neurodevelopmental, respiratory, and feeding abnormalities during the first year of life. The reasons for these differences are unclear but may be related to the severity of the primary illness itself or the variables associated with prolonged ECMO therapy. Stratifying outcome by primary diagnosis gives the health care provider more information to improve existing intervention techniques and to provide parents with more accurate counseling., ABBREVIATIONS. ECMO, extracorporeal membrane oxygenation; ELSO, Extracorporeal Life Support Organization; MAS, meconium aspiration syndrome; CDH, congenital diaphragmatic hernia; CHOP, Children's Hospital of Philadelphia; GER, gastro-esophageal reflux; BPD, bronchopulmonary dysplasia; MDI, [...]

Inhalation of nitric oxide (NO) appears to be effective in treating episodes of pulmonary hypertension in newborns with underdeveloped lungs after completion of ECMO therapy. NO dilates pulmonary blood vessels. Nine newborns with underdeveloped lungs due to diaphragmatic hernia in eight cases and lack of amniotic fluid in one case were studied. Prior to ECMO therapy, NO inhalation had no effect on oxygen content in the blood, but after ECMO therapy, NO treatment produced significant improvement in oxygenation. It is speculated that lung surfactant deficiency was responsible for the lack of effect prior to ECMO. The only two babies responding to NO prior to ECMO had been treated with surfactant. It is possible that the infants' surfactant systems matured during the time spent on ECMO ., Objective. We determined whether inhaled nitric oxide (NO) could improve systemic oxygenation in human neonates with hypoplastic lungs. Methods. A multicenter nonrandomized investigation was performed to study the efficacy of short-term NO inhalation. Inhaled NO was administered at 80 ppm to nine neonates without evidence of structural cardiac disease by echocardiography. Lung hypoplasia was due to congenital diaphragmatic hernia (CDH) in eight patients and to oligohydramnios in one patient. A total of 15 trials of NO inhalation were performed in these nine patients. Eight trials in seven patients were performed before extracorporeal membrane oxygenation ((ECMO); one patient had two trials) and seven trials were performed in five patients after decannulation from ECMO (two patients had two trials each). Results. NO inhalation before ECMO did not change postductal [Pao.sub.2] (42 [plus or minus] 3 mmHg vs 42 [plus or minus] 4 mmHg), oxygen saturation ([Spo.sub.2]; 89% vs 88%) or oxygenation index (31 plus or minus] 4 cm [H.sub.2]O/torr vs 31 [plus or minus] 4 cm [H.sub.2]O/torr) for the group. All patients required ECMO support, which lasted from 5 to 17 days (mean 9). After decannulation from ECMO, NO inhalation increased postductal [Pao.sub.2] from a median of 56 mm Hg (range 41 to 94) to a median of 113 mm Hg (range 77 to 326), P < .05. It decreased the oxygenation index from a median of 23 cm [H.sub.2]O/torr (range 11 to 70) to a median of 11 cm [H.sub.2]O/torr (range 4 to 21), P < .05. It increased [Spo.sub.2] from 91% to 96% (P

Objective. Despite new treatments, congenital diaphragmatic hernia (CDH) still has high mortality. The aim of this study was to identify echocardiographic predictors of outcome in newborns with an isolated CDH. Methods. We reviewed medical charts and echocardiograms of 40 newborns who presented with CDH in the first 24 hours of life, from 1992 to 1996. We compared the cardiac valves and great arteries diameters, left-ventricular volume and mass, Apgar scores, and modified McGoon index (the combined diameter of hilar pulmonary arteries, indexed to the descending aorta) of survivors and nonsurvivors. We performed Student&apos;s t test and multiple logistic regression analysis between the 2 groups. Results. Fourteen patients died 1 to 33 days after birth (median: 3 days), including 8 from progressive hypoxemia without operation; 26 have survived up to 5 years (median: 2 years) after successful operations. Nonsurvivors had significantly smaller diameters of right and left hilar pulmonary arteries, more frequent right-sided CDH, and lower mean Apgar scores at 1 and 5 minutes. The most significant prognostic factor was the modified McGoon index. A modified McGoon index [is less than or equal to] 1.3 predicted mortality with a sensitivity of 85% and specificity of 100%. Conclusion. Echocardiographic measurement of hilar pulmonary arteries, which may represent the adequacy of the pulmonary vascular bed, is a strong prognostic factor for newborns with congenital CDH. Pediatrics 2000;105: 1106-1109; congenital diaphragmatic hernia, echocardiography, pulmonary artery, prognosis, left ventricle., ABBREVIATIONS. CDH, congenital diaphragmatic hernia; LPA, left pulmonary artery; ECMO, extracorporeal membrane oxygenation; Pa[CO.sub.2], partial pressure of carbon dioxide; RPA, right pulmonary artery. The introduction of innovative treatments for congenital [...]

ABBREVIATIONS. CDH, congenital diaphragmatic hernia; RCT, randomized controlled trial; ECMO, exfracorporeal membrane oxygenation. The article of Harrison et al (1,2) is a major contribution to the literature despite its disappointing [...]