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Ann Thorac Surg 1997;63:1298-1302
© 1997 The Society of Thoracic Surgeons

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Original Article: Cardiovascular

Preoperative ECMO in Congenital Cyanotic Heart Disease Using the AREC System

Departments of Neonatology and Critical Care and Cardiac and Thoracic Surgery, University Hospital of Vienna, Vienna, Austria

Accepted for publication November 18, 1996.

	Abstract

Top Footnotes Abstract Introduction Material and Results Case Reports and Technical... Comment Acknowledgments References

 
Background. In cyanotic congenital heart disease, oxygen delivery is impaired either by reduced pulmonary perfusion or by limited entry of oxygenated blood into the systemic circulation. Additional impairment of oxygen delivery (eg, in pulmonary hypertension) leads to hypoxic cerebral damage. Preoperative extracorporeal membrane oxygenation enables oxygenation in otherwise untreatable cases.

Methods. In 3 neonates suffering from cyanotic congenital heart disease (1 with tricuspid atresia and 2 with transposition of the great arteries) with arterial desaturation despite application of prostaglandins, balloon atrioseptostomy, and eventually inhaled nitric oxide during intermittent positive-pressure ventilation with an inspired oxygen fraction of 1, oxygenation could only be established by means of preoperative extracorporeal membrane oxygenation. We used a venovenous single-lumen cannula tidal-flow extracorporeal membrane oxygenation system described by Chevalier and associates that has previously been used for extracorporeal lung support. In this system, called AREC (assistence respiratoire extra-corporelle), alternating clamps and a nonocclusive roller pump were used.

Results. All 3 survived.

Conclusions. We conclude that the AREC system enables sufficient preoperative oxygenation in patients with cyanotic congenital heart disease and hypoxia in spite of all conventional therapeutic means. This provides a stable preoperative condition for elective palliation or correction.

	Introduction

Top Footnotes Abstract Introduction Material and Results Case Reports and Technical... Comment Acknowledgments References

 
In cyanotic congenital heart disease (CCHD), oxygen delivery is impaired either due to decreased pulmonary perfusion (eg, in congenital heart defects with ductal-dependent pulmonary perfusion such as tricuspid atresia or due to decreased entry of oxygenated blood into the systemic circulation (eg, in transposition of the great arteries [TGA]). Additional events such as impaired oxygen uptake in persistent fetal circulation, respiratory insufficiency, acidosis, or sepsis might result in life-threatening conditions in children with CCHD.

Cerebral hypoxia before a corrective operation might aggravate the final outcome of children treated for CCHD [1–3]. Abnormal electroencephalographic tracings, retardation of brainstem maturation, increased jugular brain-type creatine kinase (creatine kinase-BB) levels, and cerebral infarction have been reported as posthypoxic sequelae in these children [2]. Neuropsychological testing revealed impaired cognitive as well as neuropsychological abilities at school age [1].

Thus, prevention of severe or sustained hypoxia in the preoperative course is the main important factor in determining long-term cerebral outcome. We report 3 cases of CCHD in which the entire spectrum of conventional intensive care therapies failed to reverse the hypoxic condition. Under these circumstances, we decided to introduce extracorporeal membrane oxygenation (ECMO) in the preoperative phase.

	Material and Results

Top Footnotes Abstract Introduction Material and Results Case Reports and Technical... Comment Acknowledgments References

 
Preoperative Extracorporeal Membrane Oxygenation in Cyanotic Congenital Heart Disease and the AREC System
Extracorporeal life support using venoarterial bypass in CCHD before cardiovascular operation has been reported previously [4]. Indications for ECMO were arterial saturations of less than 60% accompanied by hypotension and metabolic acidosis that did not respond to mechanical ventilation with 100% oxygen including paralysis and sedation, pharmacologic support with inotropes and vasodilators, or both. Venoarterial bypass was obtained by cannulation of the right carotid artery and internal jugular vein. The ECMO flow was adjusted to achieve arterial saturations of at least 90%. Extracorporeal membrane oxygenation was found to be useful in the preoperative management of children with CCHD and sustained hypoxia unresponsive to maximal conventional therapy.

Chevalier and associates [5] developed a venovenous single-lumen cannula extracorporeal lung support system in neonates called AREC (assistence respiratoire extra-corporelle) (Fig 1). This system uses a single-lumen cannula to avoid ligation of the carotid artery, alternating clamps that generate tidal flow, and a nonocclusive roller pump that avoids the use of a venous bladder. The AREC system has been used in neonates with severe respiratory failure despite maximal conventional treatment applying the usual ECMO criteria. Normalization of arterial carbon dioxide tension and marked improvement in arterial oxygen tension were achieved with a blood flow of 40% to 50% of the total cardiac output and by using apneic oxygenation [5].

View larger version (26K): [in this window] [in a new window]   Fig 1. . The assistence respiratoire extra-corporelle (AREC) system.

	Case Reports and Technical Remarks

Top Footnotes Abstract Introduction Material and Results Case Reports and Technical... Comment Acknowledgments References

 
PATIENT 1.
Tricuspid atresia, a ventricular septal defect, and a small subpulmonary outlet chamber with subpulmonary stenosis were diagnosed by echocardiography in a cyanotic male, term neonate (birth weight, 2,800 g). After intravenous administration of prostaglandin E₂ (0.1 µg • kg^-1 • min^-1) and volume substitution, arterial oxygen saturation (SaO₂) rose from 74% to 84%. during preparation for balloon atrioseptostomy the clinical condition deteriorated, and despite the performance of balloon atrioseptostomy and mechanical ventilation with 100% oxygen plus nitric oxide (up to 40 ppm), the SaO₂ fell to less than 40% and the ph to 6.99.

During pharmacologic resuscitation using bicarbonate and epinephrine, the AREC system was installed (Fig 2). A special double raceway tubing system (device and disposable material: Health Care Materials, Paris, France), a Jostra M8 Oxygenator (Jostra Company, Lund, Sweden), and a Jostra HEC 40 heat exchanger were filled with packed red cells and fresh frozen plasma containing 2 IU/mL heparin and buffered with trimetapham to a pH of 7.5.

View larger version (168K): [in this window] [in a new window]   Fig 2. . Roentgenogram of the AREC cannula in patient 1.

Within a few minutes SaO₂ increased up to 95%. at this time echocardiography showed the outlet chamber to be totally obstructed by subpulmonary artery stenosis. the ductus arteriosus was widely patent, without obvious flow into the pulmonary artery, due to marked elevation of the pulmonary vascular resistance.

During ECMO using the AREC system apneic oxygenation was performed using a conventional neonatal respirator (Babylog 8000; Dräger, Lübeck, Germany) with a respiratory rate of 7 breaths/min, an inspiration time of 0.6 seconds, a peak inspiratory pressure of 25 cm H₂o, and an end-expiratory pressure of 3 cm H₂o; the inspired oxygen fraction was 0.4 and the constant flow was 10 l/min.

Echocardiography after 11 hours in a stable clinical condition and with normal blood gas values showed restored pulmonary perfusion from the patent ductus arteriosus as well as through the subpulmonary stenosis. After 35 hours of ECMO a Blalock-Hanlon and Blalock-Taussig procedure was successfully performed with the patient on the AREC system throughout the operation. Only during the Blalock-Hanlon procedure was the pump flow interrupted for 2 minutes.

The baby was successfully weaned from the AREC system 2 days postoperatively, was extubated on day 10, and was discharged home on day 49.

One day after initiation of the AREC system a grade III intraventricular hemorrhage was diagnosed by ultrasound. We decided it was not a contraindication to the operation. During the following time the intracranial hemorrhage did not expand, but at the age of 4 months a ventriculoperitoneal shunt was necessary because of an increasing hydrocephalus. At the age of 13 months the child suffered from mild motor retardation.

PATIENT 2.
Transposition of the great arteries was diagnosed by echocardiography in a cyanotic female term neonate (birth weight, 3,700 g). While the patient was receiving a prostaglandin E₂ infusion, a balloon atrioseptostomy was carried out, resulting in increased arterial SaO₂ up to 82%.

However, SaO₂ decreased steadily over the next hours despite adequate filling pressures and ventilatory support and reached 67% after a further 20 hours. at this time inhaled nitric oxide (up to 60 ppm) was applied during intermittent positive-pressure ventilation with 100% oxygen, resulting in an increased SaO₂ up to 76%. four hours later the SaO₂ decreased again to 60%. there was no decrease in postnatal pulmonary vascular resistance; therefore, the surgeons decided to delay operation. using the above-mentioned single-lumen cannula, applied merely by percutaneous puncture, the arec system was introduced and SaO₂ increased to 96%. after 48 hours on the arec system, an arterial switch operation was performed. use of the arec system was continued until the intraoperative bypass was installed.

Six hours postoperatively venoarterial ECMO was necessary because of a low cardiac output. The right internal jugular vein was reused and the right common carotid artery was cannulated for venoarterial ECMO using a Biomedicus pump (Medtronic Biomedicus, Anaheim, CA). The patient was successfully weaned from ECMO 70 hours later. The patient was extubated on day 5 after the operation and discharged from the hospital on day 72. At 12 months, neuromotor development was normal except for a slight horizontal nystagmus.

PATIENT 3.
A male term neonate (birth weight, 3,420 g) was admitted after home delivery because of general cyanosis on day 2 of life. The initial SaO₂ was 55%, and echocardiography revealed tga. the baby was in bad condition due to multiorgan failure. prostaglandin E₂ administration accompanied by adequate volume therapy was started and balloon atrioseptostomy was performed, resulting in an SaO₂ of 74%; however, the baby showed recurrent decreases in SaO₂. eight hours after admission nitric oxide (20 to 40 ppm) was administered during intermittent positive-pressure ventilation with an inspired oxygen fraction of 1.0. this resulted in a transient improvement, but it was not possible to perform an operation because of sepsis and renal insufficiency as well as missing pulmonary vascular adaptation. fifty-five hours after admission, the SaO₂ decreased to less than 60% and arec treatment was initiated. for this baby we inserted a wire-reinforced percutaneously applicable cannula to avoid the risk of kinking (biomedicus 12f femoral venous kit; medtronic), using the technique reported in patient 1.

The SaO₂ immediately increased to 91%, but while on the arec system the patient required hemofiltration due to renal insufficiency. a hemofilter (minifilter plus; amicon) was installed parallel to the oxygenator. after 71 hours an arterial switch operation was successfully performed. the single-lumen cannula was used for venous drainage into the intraoperative bypass, and at the end of the operation the left carotid artery was cannulated. thus the intraoperative bypass was continued postoperatively as venoarterial ecmo, which was necessary because of impaired myocardial function. on the second postoperative day the baby was weaned from ecmo, 6 days later he was extubated, and on day 18 he was discharged from the pediatric intensive care unit. a ventriculoperitoneal shunt was necessary at the age of 7 weeks for enlargement of the ventricles (without previously recognized intraventricular hemorrhage). on follow-up examination at the age of 5 months, development appears normal.

	Comment

Top Footnotes Abstract Introduction Material and Results Case Reports and Technical... Comment Acknowledgments References

 
We report case studies of 3 babies with CCHD, all of them so seriously ill at admission that a definitive operation seemed to be impossible. In spite of the use of all conventional therapeutic techniques, fitness for operation could not be reached.

A dramatic worsening of the SaO₂ developed in the first baby during preparations for balloon atrioseptostomy. Spells provoked by manipulations with a heart catheter in newborns with tricuspid atresia have been previously reported [6]. We attempted to improve pulmonary perfusion by inhaled nitric oxide [7], but had no success. Oxygen saturation elevated quickly after the start of ECMO using the AREC system.

This baby revealed, after pharmacologic resuscitation, a wide patent ductus arteriosus without obvious flow into the pulmonary artery due to marked elevation of pulmonary vascular resistance. A similar condition, diagnosed by echocardiography has been described by Weinhaus and associates [8] as functional pulmonary atresia.

With the stable clinical condition provided by AREC therapy, pulmonary perfusion was restored. It has been previously reported that ECMO therapy leads to an improvement of pulmonary perfusion associated with decreased pulmonary vascular resistance as a result of elevation of SaO₂. This was confirmed by echocardiographic measurements of cardiovascular function during ECMO [9].

The second baby (with TGA) in our study suffered from insufficient SaO₂ in spite of adequate volume therapy, balloon atrioseptostomy, ventilatory support, and inhaled nitric oxide. It is known that an absence of postnatal pulmonary vascular adaptation means a high risk of permanent pulmonary hypertension, also in TGA [10]. Using AREC therapy it was possible to perform a successful switch operation, maintaining stable and sufficient SaO₂.

The third baby (home delivered, with TGA) was admitted on the second day of life already suffering from multiorgan failure (low cardiac output and oliguria). Balloon atrioseptostomy, prostaglandin E₂, adequate volume therapy, and ventilatory support did result in some improvement of SaO₂, but multiorgan failure made an operation impossible. Using AREC therapy we dealt with the multiorgan failure sufficiently that a successful switch operation could be performed 71 hours later.

In this report we demonstrate that preoperative extracorporeal life support by means of the AREC system represents an effective alternative for treating severe hypoxia refractory to conventional therapies in infants with CCHD. Such severe hypoxia might occur in association with sepsis and respiratory distress syndrome [4] as in patient 3 or as a result of persistent elevation of pulmonary vascular resistance, as in patient 1, which can lead to a negative response to balloon atrioseptostomy in TGA.

The venovenous AREC system used in our institution offers some important advantages over the venoarterial mode reported previously [11]. The main advantage is that the AREC system uses a single-cannula tidal-flow system that does not require carotid ligation and is thus less invasive and easier to place. Moreover, inadequate local cerebral perfusion after carotid ligation in infants suffering from extreme hypotension before ECMO cannulation is a risk factor for damage of the right hemisphere [12].

The AREC system, using the approach described, does not require ligation of the internal jugular vein, which would otherwise result in increased cerebral blood flow and enhanced cerebral oxygen consumption [13]. Compared with venoarterial ECMO, the AREC system requires less heparinization because the activated clotting time only needs to be around 150 to 180 seconds. The risk for raceway rupture or gas embolism is also lower [11]. The combination of the single-lumen tidal-flow system with alternating clamps and the nonocclusive pump makes the entire system safer, less invasive, and less complicated to monitor by the routine intensive care unit staff.

However, adequate systemic ventricular function is necessary for performing venovenous ECMO. On the other hand, improvement of hypoxic myocardial dysfunction has been demonstrated in infants with respiratory and circulatory compromise other than CCHD [11].

Once the AREC system is introduced as part of the preoperative arrangement it also can be used during transport to the operating room using the battery of the AREC 1 machine (Health Care Materials, Paris, France; see the report of patient 1). Furthermore, the child can remain on the AREC system during the Blalock-Taussig procedure for continuous adequate oxygenation. A short interruption is necessary only during atrioseptectomy.

If cardiopulmonary bypass during the operation or postoperative venoarterial ECMO is indicated, the single-lumen cannula can be used as venous source for the intraoperative bypass. (For atrial septal defect closure a short period of circulatory arrest is necessary.) Both babies with TGA were in need of postoperative circulation support by ECMO because left ventricular function was insufficient. Low cardiac output situations like that, which need ECMO after an arterial switch operation, were described recently [14].

Prolonged severe hypoxia has a high risk of secondary brain injury [1, 2], which ultimately leads to hypoxic neurologic sequelae in these children. We found twice an intraventricular hemorrhage, due in both cases to severe hypoxia before the installation of the AREC system. Concerning ECMO invasiveness, we weighed pros and cons very carefully before using the AREC system. It is possible that we could have prevented these sequelae by starting that therapy earlier.

The AREC system does not change the entire hemodynamics of congenital heart defects. There are exceptions: pulmonary arterial resistance, increased by hypoxia, is diminished, myocardial contractility (depressed by hypoxia) is improved by better oxygenation, and there are moderate fluctuations of right atrial pressure induced by tidal flow. In contrast to venoarterial ECMO, in the AREC system blood is not pumped from the right atrium to the systemic circulation, which may increase the risk of pulmonary edema (eg, in left ventricular insufficiency). Pulmonary hyperperfusion as a result of improved lung perfusion, eg, through a widely patent ductus arteriosus by decrease of pulmonal arterial resistance can be dealt with positive end-expiratory pressure increasing up to 12 cm H₂O in intermittent positive-pressure ventilation during use of the AREC system.

We think the main indication for use of the AREC system is quick improvement of oxygenation in children with congenital heart defects when high pulmonary arterial resistance in cyanotic heart defects, despite patent ductus arteriosus and all additional conventional means, prevents lung perfusion and venoarterial ECMO should be avoided. It must be decided for each special case whether an emergency palliative or corrective operation is more effective than an elective operation after preoperative stabilization.

We conclude that preoperative venovenous ECMO is a promising tool, in some desperate cases of CCHD, for preoperative stabilization and enables palliative or corrective operation on an elective basis. In the AREC system, venovenous ECMO is less invasive than venoarterial ECMO and offers several other advantages. However the system chosen has to be introduced early enough to keep preoperative hypoxias as short as possible.

	Acknowledgments

Top Footnotes Abstract Introduction Material and Results Case Reports and Technical... Comment Acknowledgments References

 
This work was supported by the Austrian Ministry of Science.

	Footnotes

Top Footnotes Abstract Introduction Material and Results Case Reports and Technical... Comment Acknowledgments References

 
Address reprint requests to Dr Trittenwein, Ebene 10, Intensivstation, Abteilung für Neonatologie und Intensivmedizin, Universitätskinderklinik Wien, Währinger Gürtel 18-20, A-1090 Wien, Austria.

	References

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2. Rossi RF, Ekroth R, Jansson K, Scallan N, Thompson RJ, Lincoln C. Brain type creatine kinase in relation to oxygen desaturation in the blood of children with congenital heart disease. Scand J Thorac Cardiovasc Surg 1990;24:75–7.[Medline]
3. Sunaga Y, Sone K, Nagashima K, Korume T. Auditory brain stem responses in congenital heart disease. Pediatr Neurol 1992;8:437–40.[Medline]
4. Hunkeler NM, Canter CE, Donze A, Spray TL. Extracorporeal life support in cyanotic congenital heart disease before cardiovascular operation. Am J Cardiol 1992;69:790–3.[Medline]
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6. Rao PS. Tricuspid atresia. In: Long WA, ed. Fetal and neonatal cardiology. Philadelphia: Saunders, 1990:525–40.
7. Winberg P, Lundell BP, Gustafsson LE. Effect of inhaled nitric oxide on raised pulmonary vascular resistance in children with congenital heart disease. Br Heart J 1994;71:282–6.[Abstract/Free Full Text]
8. Weinhaus L, Jureidini S, Nouri S, Conors RH. Functional pulmonary atresia: color flow recognition and treatment with extracorporeal membrane oxygenation. Am Heart J 1990;110:980–2.
9. Strieper MJ, Sharma S, Dooly KJ, Cornish JD, Clark RH. Effects of venovenous extracorporeal membrane oxygenation on cardiac performance as determined by echocardiographic measurements. J Pediatr 1993;122:950–5.[Medline]
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This Article Abstract Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Gregor Wollenek Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Trittenwein, G. Articles by Pollak, A. Search for Related Content PubMed PubMed Citation Articles by Trittenwein, G. Articles by Pollak, A.