Effect of surgical repair on respiratory mechanics in congenital diaphragmatic hernia

To determine whether surgical repair of congenital diaphragmatic hernia (CHD) results in improvement in respiratory mechanics, we measured respiratory system compliance in nine patients (five survivors and four nonsurvivors) before and after operation. In all nine infants, CHD was diagnosed within 6 hours of life, and surgical repair was through an abdominal approach after a period of stabilization. Measurements were made noninvasively, using the passive expiratory flow-volume technique. In only one of the nine infants did compliance immediately improve after surgical repair, and in another it showed no change. Both of these infants survived, with an uneventful postoperative course. In the remaining seven infants, however, postoperative compliance immediately decreased to 10% to 77% from the preoperative value. The four infants with more than 50% decrease in compliance died with increasing hypoxemia and acidosis. These results suggest that respiratory mechanics in CHD, far from improving, frequently deteriorate as a result of repair of the hernia. The role of urgent surgery in this malformation should be reevaluated.     

Extracorporeal membrane oxygenation and the management of congenital diaphragmatic hernia

Uniform survival in infants with congenital diaphragmatic hernia (CDH) remains an elusive goal. Those that qualify for extracorporeal membrane oxygenation (ECMO) represent a subgroup that has a predicted mortality of 90%. This report summarizes an experience over 5 years of offering ECMO to all CDH infants with an oxygen index > 40 for 2 h. No infants were excluded on the basis of a minimum PaO₂ or other criteria. Thirty-eight infants were treated and 24 survived (63%). Patients were grouped into survivors and nonsurvivors. There was no difference in the average worst pre-ECMO PaO₂ or PaCO₂, while the pH values were less in those that died (P <.01). The average best pre-ECMO PaO₂ of 113 mm Hg in survivors was significantly different from that of 44 mm Hg observed in nonsurvivors (P <.05). Of 24 survivors, 14 would have died as a result of exclusion if a minimum pre-ECMO PaO₂ of 50 mm Hg had been required. Pre-ECMO CDH repair was performed in 15 patients with 3 deaths; repair was performed during ECMO in 17 patients with 6 deaths. In all patients except 2, ECMO could be discontinued within several hours of repair. No improvement in lung function was seen in 5 patients at 10 to 14 days and bypass was discontinued without repair. Dynamic lung compliance and end-tidal CO₂ as well as the ability to be weaned from ECMO were used as measures of lung function. A heparin/Carmeda bonded circuit and reduced heparin were used successfully in 3 patients to treat bleeding complications. It is concluded that all infants should be resuscitated with ECMO. Survival in this series was signficantly increased over historical controls treated without ECMO (P <.01). The timing of CDH repari did not appear to affect survival. With over 37% of patients still dying from this disease, future investigation is necessary to define the role fo therapies such as fetal surgery and neonatal lung transplantation as well as to improve current therapy.     

Prolonged preoperative stabilization using high-frequency oscillatory ventilation does not improve the outcome in neonates with congenital diaphragmatic hernia

Although delayed repair with preoperative stabilization using high-frequency oscillatory ventilation (HFOV) has been advocated in neonates with congenital diaphragmatic hernia (CDH), improved survival has not been reported. We compared survival between neonates undergoing delayed repair after prolonged stabilization for more than 48 h using HFOV and those undergoing immediate repair following short stabilization using HFOV in subjects with high-risk CDH. Patient selection was made by two criteria: fetuses with the lung/thorax transverse area ratio (L/T) below 0.26 and neonates who presented with respiratory distress significant enough to require intubation within 6 h after delivery. Selection of the strategies differed by era and medical center. Stabilization failed in 11 of 18 patients with delayed repair and only 7 patients (38.9%) in this group survived, but 18 of 23 patients (78.3%) in the immediate-repair group survived (P < 0.05). Although lower values of Apgar scores, best postductal PO₂ (BPtDPO₂), gestational weeks, and L/T and more frequent patch repair were observed in the delayed than in the immediate repair group, significant differences in survival among the subpopulations were also observed in the prenatally diagnosed group, including the group with L/T < 0.15 and the group that required ECMO, the group with the best preductal PO₂ (BPrDPO₂) ≥ 100 torr, and the group with BPtDPO₂ < 100 torr. These results indicate that prolonged stabilization for more than 48 h using HFOV does not improve survival in patients with high-risk CDH.     

Experience with delayed repair of congenital diaphragmatic hernia during extracorporeal membrane oxygenation in a European center

In high-risk patients with congenital diaphragmatic hernia (CDH), we conducted a strategy of delayed repair following preoperative stabilization, including ECMO if necessary. From January 1991 to July 1992, preoperative ECMO treatment was delivered to 6 out of 14 high-risk patients with CDH. In this study, we report our experience with this policy of preoperative stabilization in six ECMO-treated patients in whom the diaphragmatic defect was repaired on ECMO. In all patients bleeding complications occurred. Three of the six ECMO-treated patients died, in two cases due to recurrent persistent pulmonary hypertension. Overall mortality was 43%.Conceptual aspects of our approach are discussed. Since the goals of our policy were not achieved, adjustments and renovations of the management protocol are discussed.On behalf of the ECMO Group     

Fetal diaphragmatic hernia: prenatal evaluation of lung hypoplasia and effects of immediate operation

The outcome of fetuses with diaphragmatic hernia (CDH) has been reported to be related to the severity of lung hypoplasia. As an index of pulmonary hypoplasia, we attempted to measure the lung-thorax transverse area ratio (L/T) using ultrasonic echography in eight fetuses with left-sided CDH. Two cases with L/T more than 0.28 (controls: 0.52±0.04) were transported postnatally and recovered after early operation without episodes of persistent fetal circulation. Elective surgical repair was performed in six infants immediately after cesarean delivery at 35–37 weeks' gestation. In three cases with L/T between 0.21 and 0.24 who recovered with no complications, surgical reduction of the abdominal organs improved arterial blood gases and high-frequency oscillation ventilation (HFOV) was fully effective for respiratory management. In three with L/T between 0.11 and 0.17, extracorporeal membrane oxygenation (ECMO) was required from the 1st to the 12th postoperative day despite HFOV. Although two infants died of combined cardiovascular anomalies and airway bleeding caused by prolonged HFOV, respectively, one infant with minimal L/T survived. Measurement of L/T may help to predict the outcome of fetuses with CDH and to determine the indications for various treatments including immediate operation after cesarean delivery, HFOV, and ECMO. Offprint requests to: S. Kamata     

Congenital diaphragmatic hernia: experience without extracoporeal membrane oxygenation

While extracorporeal membrane oxygenation (ECMO) is used in the management of congenital diaphragmatic hernia (CDH), its value is questioned. The charts of all newborn infants who presented in respiratory distress due to CDH over the past 27 years were reviewed. Inborn versus outborn, year of repair, use of ultrasound (US), and the predictive value of various parameters was evaluated. Fisher's exact test and logistic regression tests were used to analyze data. There were 81 patients, 43 males and 38 females. Repair occurred after stabilization without ECMO; 65 patients survived (80%). Apgar scores at 1 min (P=0.03) and 5 min (P=0.005), best postductal PaO₂ (BPDPaO₂) (P=0.02), and type of repair (P=0.01) were predictive of outcome. There was no difference in survival between inborn and outborn patients or over the years of review (P=0.29). Forty-six patients had documented prenatal US scans, with no obvious impact on outcome. Thus, survival of CDH patients without ECMO is comparable to the best results reported with ECMO, suggesting that the costs and associated morbidity of ECMO may not justify its use for such patients. Apgar scores, BPDPaO₂, and type of repair are good predictors of outcome. Accepted: 9 March 2001     

ECMO in newborn infants: The Melbourne experience

At the Royal Children's Hospital, Melbourne, extracorporeal membrane oxygenation (ECMO) has been used in the treatment of newborn infants with life-threatening respiratory or cardiac failure since May 1989. The main indications for the use of ECMO are that the disease is reversible, the surviving infant is likely to be normal and there is an 80% likelihood of death without ECMO. Sixteen of 22 (73%) newborn infants have survived at least 6 months after ECMO. Fourteen of 16 (87.5%) infants receiving ECMO (who did not have a congenital diaphragmatic hernia) were functionally normal survivors; the other two infants died. Two of six infants with congenital diaphragmatic hernia who received ECMO were discharged and survived to have normal neurological and respiratory function at 6 month follow up. These results are similar to results from other centres internationally. It would appear that ECMO is a useful therapy for near-term newborn infants with otherwise fatal cardiorespiratory failure.     

Extracorporeal membrane oxygenation and conventional medical therapy in neonates with persistent pulmonary hypertension of the newborn: a prospective randomized study

Thirty-nine newborn infants with severe persistent pulmonary hypertension and respiratory failure who met criteria for 85% likelihood of dying were enrolled in a randomized trial in which extracorporeal membrane oxygenation (ECMO) therapy was compared with conventional medical therapy (CMT). In phase I, 4 of 10 babies in the CMT group died and 9 of 9 babies in the ECMO group survived. Randomization was halted after the fourth CMT death, as planned before initiating the study, and the next 20 babies were treated with ECMO (phase II). Of the 20, 19 survived. All three treatment groups (CMT and ECMO in phase I and ECMO, phase II) were comparable in severity of illness and mechanical ventilator support. The overall survival of ECMO-treated infants was 97% (28 of 29) compared with 60% (6 of 10) in the CMT group (P less than .05).     

Congenital diaphragmatic hernia

Posterolateral congenital diaphragmatic hernia (Bochdalek's hernia, CDH) that presents in the neonatal period is often manifest as life-threatening respiratory distress due to mechanical compression of the cardiopulmonary system by herniated viscera. Most patients can be supported through surgical reduction and repair with standard neonatal techniques. Postoperatively, many develop severe, refractory persistent pulmonary hypertension of the newborn (PPHN) after a variable period of adequate oxygenation. Despite aggressive ventilatory and pharmacologic maneuvers, a significant number of these infants develop fatal hypoxia and acidosis. At our institution, extracorporeal membrane oxygenation (ECMO) has been used as a therapy of last resort in 12 moribund infants with PPHN and respiratory failure after repair of CDH. There were 7 survivors. All long-term survivors [6] were totally weaned from ventilatory support; all met the criteria for nonsurvival as defined by Bohn et al. [2]. Eight patients (4 survivors) had no PaO₂ greater than 100 torr postoperatively. ECMO can relieve the hypoxic and metabolic stimuli that aggravate pulmonary hypertension and provide cardiopulmonary support. It is the treatment of choice for infants who are deteriorating in the face of maximum conventional therapy after surgical correction. Lesions that were formerly denoted nonsurvivable can be effectively treated. It is urged that this therapy be used whenever necessary to sustain the life of an infant who has survived operation, regardless of whether the patient has demonstrated a honeymoon period, PaO₂ greater than 100 torr, or any other factor conventionally thought to delineate survivable from nonsurvivable lesions. Such practices may exclude patients with survivable lesions from life-saving therapy.     

Extracorporeal membrane oxygenation in infants with congenital diaphragmatic hernia: a systematic review of the evidence.

AIM: The aim of this study was to evaluate the evidence supporting the use of extracorporeal membrane oxygenation (ECMO) in infants with congenital diaphragmatic hernia (CDH) and severe respiratory failure. METHODS: Medline, Embase, ISI Current Contents and Biosis databases were searched using a defined strategy. Case reports and opinion articles were excluded. We performed: 1) a systematic review of non randomised studies comparing mortality when ECMO was not available with a period when ECMO was available. Mortality was classified as "early" (before hospital discharge) and "late" (after discharge). Patients were classified as "ECMO" and "non-ECMO" candidates according to criteria reported by the authors; 2) a meta-analysis of randomised controlled trials (RCTs) comparing ECMO and conventional mechanical ventilation (CMV). Differences in mortality are reported as relative risk (RR) and 95 % confidence intervals. RESULTS: A) SYSTEMATIC REVIEW: 658 studies and 21 (2043 patients) fulfilled the entry criteria. Both early (RR 0.60 [0.51-0.70]; p < 0.001) and late mortality (RR 0.63 [0.53-0.73]; p < 0.001) were significantly lower when ECMO was available than when ECMO was unavailable. This difference in mortality was observed in "ECMO candidates" (RR 0.46 [0.32-0.68]; p < 0.001) but not in "non-ECMO candidates" (RR 0.80 [0.58-1.10]; p = 0.17). B) META-ANALYSIS: 3 trials comparing ECMO and conventional ventilation were identified which included 39 infants with CDH. The early mortality was significantly lower with ECMO compared to CMV (RR 0.73 [95 % CI 0.55-0.99]; p < 0.04), however, late mortality was similar in the two groups (RR 0.83 [0.66-1.05]; p = 0.12). CONCLUSIONS: Non randomised studies suggest a reduction in mortality with ECMO. However, differences in the indications for ECMO and improvements in other treatment modalities may contribute to this reduction. The meta-analysis of RCTs indicates a reduction in early mortality with ECMO but no long-term benefit. A large RCT in infants with CDH and severe respiratory failure is warranted.     

Entry criteria for extracorporeal membrane oxygenation In neonates with congenital diaphragmatic hernia treated with high-frequency oscillatory ventilation

Although respiratory management with high-frequency oscillatory ventilation (HFOV) has generally been used for neonates with congenital diaphragmatic hernia (CDH), entry criteria for extracorporeal membrane oxygenation (ECMO) based on data from patients who underwent HFOV have not yet been reported. To establish entry criteria for ECMO in such patients, we retrospectively studied 36 neonates with CDH treated by HFOV in our institutions between 1986 and 1994. From the admission records, preductal and postductal arterial blood gas data and HFOV ventilation conditions for 72 h after admission were extracted. Oxygenation index (01) and alveolar-arterial oxygen gradient (A-aD02) time interval combinations were calculated. Patients were divided into two groups: candidates for ECMO (n = 22) who underwent ECMO (n = 18) or died without ECMO (n = 4); and non-candidates (n = 14), who survived without ECMO. Blood gas data in patients placed on ECMO were comparable to those in patients who died without ECMO: mean pre- and postductal OI for 4 h > 30, postductal A-aD02 ≥620 mmHg for 4 h, postductal A-aD02 ≥580 mmHg for 8 h, and postductal A-aD02 ?550 mmHg for 12 h showed better sensitivity with a specificity of more than 90% compared to entry criteria that had previously been used in our institutions: a postductal OI >40 for 4 h and postductal A-aDO₂ ≥610 mmHg for 8 h. In addition, a combination of preand postductal OI >30 for 4 h indicated a sensitivity of 95.5% and a specificity of 92.9%.     

Infant pulmonary function in a randomized trial of fetal tracheal occlusion for severe congenital diaphragmatic hernia

Congenital diaphragmatic hernia (CDH) carries a high mortality risk secondary to pulmonary hypoplasia and respiratory failure. In experimental animals, fetal tracheal occlusion (TO) induces lung growth and morphologic maturation. We measured indicators of pulmonary function in 20 infants who were enrolled in a randomized trial of fetal TO as treatment for severe CDH [nine with conventional treatment (controls); 11 with TO]. We hypothesized that TO would improve lung function. At birth, the TO group had a lower mean gestational age (30.8 +/- 2.0 versus 37.4 +/- 1.0 wk; p=0.0002). All infants required assisted ventilation. Mortality did not differ between groups (64 versus 78%, TO and control, respectively; p=0.64). We measured respiratory mechanics at four study points: 1) first 24 h, 2) before CDH operative repair (5.9 +/- 2.2 d), 3) immediately after repair (7.0 +/- 2.2 d), and 4) before elective extubation (32.5 +/- 16.1 d). We calculated perioperative oxygenation index and alveolar-arterial oxygen difference to assess efficiency of pulmonary gas exchange. Data were analyzed by univariate and repeated measures techniques. Respiratory system compliance (Crs) was low. The rate of increase in Crs over the four study points was greater in the TO group than in control subjects. Crs in the TO group was significantly greater at study 2 (0.28 +/- 0.12 versus 0.17 +/- 0.04 mL.cm H2O(-1).kg(-1); p=0.02) and study 4 (0.93 +/- 0.45 versus 0.51 +/- 0.16 mL.cmH2O(-1).kg(-1); p=0.02). oxygenation index did not differ between groups, but alveolar-arterial oxygen difference was lower in the TO infants. We conclude that fetal TO for severe CDH results in modest improvements in neonatal pulmonary function that are of questionable clinical significance.     

Use of inhaled nitric oxide in the new born period: results from the European inhaled nitric oxide registry

Aims: The aim of this study was to present data relating to the use of inhaled nitric oxide (iNO) in newborn infants included in the European Inhaled Nitric Oxide Registry. Methods: Demographic, clinical and therapeutic data from seven European centres are reported. Univariate analyses were performed to identify factors associated with acute response to iNO and survival without extra corporeal membrane oxygenation (ECMO). Results: A total of 112 newborn infants received iNO, with 40% being less than 34 weeks gestational age. The commonest indication for iNO was secondary pulmonary hypertension. Acute response to iNO was more common in infants with a higher oxygenation index (median OI 32.7 vs 22.6, p = 0.040), although acute response did not predict survival without ECMO. Infants who survived without ECMO had a lower OI prior to therapy (median OI 24 vs 43, p = 0.009), were commenced on a higher starting dose (median dose 20 ppm vs 10 ppm p = 0.013) and received a lower maintenance dose (median dose 10 vs 17 ppm, p = 0.027) than those who died or received ECMO. Conclusion: Collating and reporting data about iNO therapy in neonates across a number of European centres using a web‐based system is feasible. These data may be used to monitor the clinical use of iNO, identify adverse effects, generate research hypotheses and promote high standards in the clinical use of iNO.     

Is preoperative risk assessment of newborns with congenital diaphragmatic hernia possible by means of chest X-rays?

Congenital diaphragmatic hernia (CDH) of the newborn constitutes an emergency. The overall mortality exceeds 50%. Touloukian and Markowitz described a preoperative X-ray scoring system for predicting outcome following repair of CDH. We reviewed the preoperative X-ray of 33 newborns with CDH according to this system. All infants had to be operated within 24 h of age because of serious respiratory distress. Unlike Touloukian and Markowitz, we were not able to identify the survivors by this scoring system. A number of arguments are presented which may contribute to an explanation for this discrepancy. We conclude from our series and that of Touloukian and Markowitz that a preoperative pneumothorax and a diaphragmatic defect which cannot be closed primarily seem to constitute the only reliable unfavorable signs in emergency cases.Offprint requests to: R. van Dijk Azn     

Phospholipid and surfactant protein A concentrations in tracheal aspirates from infants requiring extracorporeal membrane oxygenation.

To test the hypothesis that infants with severe respiratory failure and the need for extracorporeal membrane oxygenation (ECMO) are surfactant deficient, we measured the amount of surfactant phospholipids, disaturated phosphatidylcholine, surfactant protein A, and protein in tracheal aspirates from 22 infants, who received ECMO therapy for respiratory failure with meconium aspiration syndrome (n = 18) or pneumonia (n = 4). Tracheal suction material was obtained in a standardized way every 4 hours during the period of ECMO treatment and pooled for 24-hour periods. During ECMO, mean total phospholipid, disaturated phosphatidylcholine, and surfactant protein A values in tracheal aspirates increased and protein values decreased significantly, predominantly during the 72-hour period before infants were weaned from ECMO. Of the 22 infants, 14 had an increase in tracheal aspirate phospholipid values of more than 200% and were found to need a shorter period of ECMO support (p less than 0.005) and post-ECMO ventilatory support (p less than 0.025) than did the eight infants with stationary or only moderate increases in tracheal aspirate phospholipid values, three of whom had pneumonia. We conclude that infants with respiratory failure who require ECMO treatment often have surfactant deficiency. We speculate that surfactant treatment might decrease the need for or the duration of ECMO support.     

The immediate and long-term outcomes of newborns with congenital diaphragmatic hernia

In spite of the innovations in the management of newborns with congenital diaphragmatic hernia (CDH) presenting with respiratory distress at birth, mortality and ongoing morbidity still remain high. This is a retrospective analysis of newborns with CDH to determine the immediate and long-term outcomes among survivors. Medical records of newborns with CDH and respiratory distress at birth between January 1993 and March 2002 were reviewed retrospectively. There were 45 newborns, 29 males and 16 females. Eleven newborns (24%) died during the period of preoperative stabilization, 9 from pulmonary hypoplasia and 2 with complex anomalies who were not resuscitated. Surgery was performed in 34 newborns (76%). Three died postoperatively from severe pulmonary hypoplasia and pulmonary hypertension. Eleven newborns (24%) had sepsis from coagulative-negative staphylococci. Thirty-one of 43 newborns (72%) with isolated CDH were discharged home. Twenty-seven of 31 survivors (87%) had adverse long-term outcome and 2 late deaths were from pulmonary complications. Twenty-nine of 43 newborns (67%) with isolated CDH survived. The principal determinant of survival was pulmonary hypoplasia. Eighty-seven percent of survivors have associated morbidity including ongoing pulmonary, nutritional and neuro-developmental problems. Nevertheless preoperative stabilization and delayed surgery have been a satisfactory mode of management.     

Doppler flow patterns through the ductus arteriosus in patients with congenital diaphragmatic hernia.

We investigated Doppler flow patterns through the ductus arteriosus (DA) of 13 newborn infants with congenital diaphragmatic hernia (CDH) using color Doppler ultrasonography (CDUS). Patterns were classified into 3 types: left-to-right (L-R), bidirectional (BD) and right-to-left (R-L) shunting. Among the 13 patients examined, 3 showed L-R shunting, 5 BD shunting and 5 R-L shunting. Patients with L-R shunting showed significantly better levels of PaO2 and AaDO2 than those with other Doppler flow patterns. However, there were no differences in clinical findings between patients with BD and R-L shunting. All patients with L-R shunting survived after CDH repair without pre-operative stabilization including Lipo-PGE1 (LPE) administration. Four of the 5 patients with BD shunting survived, but only one of the 5 patients with R-L shunting survived after CDH repair following administration of LPE. It was suggested that Doppler flow patterns through the DA may be useful for predicting prognoses and selecting suitable treatment for CDH.     

Improving survival of neonates with isolated congenital diaphragmatic hernia

Despite advances in neonatal care including prenatal diagnosis, conventional ventilation, surfactant, high frequency oscillation, nitric oxide and extracorporeal membrane oxygenation (ECMO) the diagnosis of CDH is reported to carry a high mortality rate. Pulmonary hypoplasia and persistent pulmonary hypertension are major factors that contribute to death. In an effort to improve the survival of these infants a protocolized approach was adopted. In summary, this involves antenatal use of steroids if CDH antenatally diagnosed, sedation and muscle relaxation following tracheal intubation, administration of surfactant, gentle ventilation with permissive hypercapnia, trial of nitric oxide, preoperative ECMO for those infants failing therapy and delayed repair of the CDH. With the use of this protocolized approach the survival of infants with isolated CDH was 96 % for inborn and 81 % for outborn infants.     

Neonatal extracorporeal membrane oxygenation: practice patterns and predictors of outcome in the UK

OBJECTIVE: To review the UK neonatal extracorporeal membrane oxygenation (ECMO) service and identify predictors of outcome. DESIGN: Retrospective review of the national cohort. Patients and interventions: 718 neonates received ECMO for respiratory failure between 1993 and 2005. Measurements and results: Diagnoses were: 48.0% meconium aspiration syndrome (97.1% survivors), 15.9% congenital diaphragmatic hernia (CDH; 57.9% survivors), 15.9% sepsis (62.3% survivors), 9.5% persistent pulmonary hypertension (79.4% survivors), 5.6% respiratory distress syndrome (92.5% survivors) and 5.1% congenital lung abnormalities (24.3% survivors). The overall survival rate of 79.7% compared favourably with the worldwide Extracorporeal Life Support Organization (ELSO) Registry. Over the period of review, pre-ECMO use of advanced respiratory therapies increased (p<0.001), but ECMO initiation was not delayed (p = 0.61). The use of veno-venous (VV) ECMO increased (p<0.001) and average run time fell (p = 0.004). Patients treated with VV ECMO had a survival rate of 87.7% compared with 73.4% in the veno-arterial (VA) ECMO group; only 42.4% of those needing conversion from VV to VA ECMO survived. In non-CDH neonates, lower birth weight, lower gestational age, older age at ECMO and higher oxygenation index (OI) were associated with increased risk of death. In CDH neonates, lower birth weight and younger age at ECMO were identified as risk factors for death. CONCLUSION: The UK neonatal ECMO service achieves good outcomes and with overall survival rate reaching 80% compares favourably with international results. Advanced respiratory therapies are used widely in UK ECMO patients. Identification of higher OI and older age at ECMO as risk factors in non-CDH neonates reinforces the importance of timely referral for ECMO.     

Extracorporeal membrane oxygenation after stage I reconstruction for hypoplastic left heart syndrome.

OBJECTIVE: Although extracorporeal membrane oxygenation (ECMO) is an acceptable strategy for children with refractory cardiac dysfunction after cardiac surgery, its role after stage I reconstruction for hypoplastic left heart syndrome and its variants is controversial. Our objective is to describe the outcome of "nonelective" ECMO after stage I reconstruction. DESIGN: Retrospective case series. SETTING: Pediatric cardiac intensive care unit. PATIENTS: Infants placed on ECMO after stage I reconstruction from January 1998 to May 2005. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Of the 382 infants who underwent stage I reconstruction during the study period, 36 (9.4%) required ECMO in the postoperative period. There were 22 infants with hypoplastic left heart syndrome. Indications for ECMO included inability to separate from cardiopulmonary bypass in 14 and cardiac arrest in 22. Fourteen infants (38.8%) survived to hospital discharge. Nonsurvivors had longer cardiopulmonary bypass time (150.1 +/- 70.0 mins vs. 103.9 +/- 30.0 mins, p =. 01). 9/14 infants (64%) supported with ECMO> than 24 hrs after stage I reconstruction survived while only 5/22 infants (22%) requiring ECMO< 24 hrs of stage I reconstruction survived (p =. 02). Of note, all five infants diagnosed with an acute shunt thrombosis were early survivors. Mean duration of ECMO was 50.1 +/- 12.5 hrs for survivors and 125.2 +/- 25.0 for nonsurvivors (p =. 01). 7/14 early survivors are alive at a median follow-up of 20 months (2-78 months). CONCLUSIONS: In our experience, ECMO after stage I reconstruction can be life saving in about a third of infants with otherwise fatal conditions. It is particularly useful in potentially reversible conditions such as acute shunt thrombosis and transient depression of ventricular function.