Ann Thorac Surg 2005;79:2155-2158
© 2005 The Society of Thoracic Surgeons
Case report
Preoperative ECMO in Transposition of the Great Arteries With Persistent Pulmonary Hypertension
Sophie Jaillard, MDa,*,
Emre Belli, MDe,
Thameur Rakza, MDb,
Benoit Larrue, MDc,
Eric Magnenant, MDb,
Christian Rey, MDd,
Laurent Storme, MDb
a Services de Chirurgie Thoracique, CHRU de Lille, Lille, France
b Service de Medecine Neonatale, CHRU de Lille, Lille, France
c Service de Chirurgie Cardiaque, CHRU de Lille, Lille, France
d Service de Cardiologie Pediatrique, CHRU de Lille, Lille, France
e Service de Chirurgie Cardiaque, Hôpital Marie-Lannelongue, Le Plessis Robinson, France
Accepted for publication December 2, 2003.
* Address reprint requests to Dr Jaillard, Clinique de Chirurgie Thoracique, Hôpital Albert Calmette, Centre Hospitalier Régional Universitaire CHRU, 59037 Lille Cedex, France (E-mail: sjaillard{at}chru-lille.fr).
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Abstract
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Persistent fetal circulation in transposition of the great arteries results in severe persistent pulmonary hypertension, which increases the risk of early mortality. We report the case of a newborn with transposition of the great arteries and intact ventricular septum associated with pulmonary hypertension. After the failure of immediate balloon atrial septostomy and supportive therapy including inhaled nitric oxide, preoperative extracorporeal membrane oxygenation reversed pulmonary hypertension and ventricular insufficiency and preceded a safe, delayed, cardiac surgical procedure. Unlike the authors of the other few case reports on this subject, we recommend a preoperative stabilization period after discontinuation of extracorporeal membrane oxygenation to avoid left ventricular "deconditioning" and postoperative deterioration related to recurrent persistent pulmonary hypertension.
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Introduction
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Persistent pulmonary hypertension (PPHN) occurs in 1% to 3% of neonates with transposition of the great arteries (TGA) [1–3]. It results in a predominantly right-to-left shunt at the ductal level and poor interatrial blood mixing, both causing severe hypoxemia despite infusion of prostaglandin E1, and balloon atrial septostomy. This condition is associated with poor survival, unlike TGA alone, which usually has a favorable prognosis [4]. In a patient of ours, after failure of immediate balloon atrial septostomy and supportive therapy including inhaled nitric oxide (NO), preoperative extracorporeal membrane oxygenation (ECMO) reversed PPHN and ventricular dysfunction.
A full-term male neonate (birth weight, 3,250 g; Apgar score, 10) was referred to our neonatal intensive care unit 2 hours after birth because of cyanosis, respiratory distress, and systemic hypotension. On arrival, arterial oxygen saturation was 30% despite high-frequency oscillatory ventilation with 100% oxygen and inhaled NO (10 ppm). Admission pH was 7.23. Echocardiography revealed TGA with intact ventricular septum and a "narrow limited" atrial level shunt (1.7 mm). Prostaglandin E1 infusion (0.1 µg·kg–1·min–1) was started to maintain ductal patency. Balloon atrial septostomy, performed with bedside echocardiographic guidance, resulted in a large interatrial communication (5.5 mm).
Despite infusion of prostaglandin E1 and balloon atrial septostomy, a predominantly right-to-left ductal and atrial level shunt appeared, which was associated with severe biventricular dysfunction. Inhaled NO was increased to 25 ppm. Dopamine hydrochloride and dobutamine hydrochloride were infused at a rate of 5 and 20 µg·kg–1·min–1, respectively. Arterial oxygen saturation remained low (<50%), especially in the upper body. Thirty hours after admission, venoarterial ECMO was started because of clinical and biological markers of tissue hypoxia (lactate, 950 mg/L) and severe pulmonary hypertension. In our institution, venoarterial ECMO is usually preferred with systematic reconstruction of the right common carotid artery at the time of decannulation. Surgical cannulation of both the right common carotid artery (with a short cannula [tip < 1.5 cm] inserted in the artery) and the right jugular vein (with the tip in the midportion of the right atrium) was performed and allowed continuous flow within the circuit (Sofracob SA, Reventin-Vaugris, France; membrane oxygenator: Medtronic, Inc, Minneapolis, MN). Systemic anticoagulation (heparin sodium) was set to maintain Hemocron time between 160 and 180 seconds.
There was immediate improvement in the condition of the patient and a progressive decrease in pulmonary artery pressure. After 2 days of ECMO, a left-to-right ductal and atrial level shunt appeared. Bypass was continued until appropriate recovery of biventricular function and unidirectional left-to-right ductal shunting were observed on gradual weaning from ECMO, which was achieved after 6 days. Stable hemodynamic and respiratory status was maintained with mechanical ventilation, inhaled NO (10 ppm), and infusion of prostaglandin E1 (0.1 µg·kg–1·min–1). Pulmonary artery pressure was evaluated daily by echocardiography using ductus arteriosus gradient and aortic pressure (Table 1). An arterial switch operation was performed after the patient had shown stable vital signs for 7 days (Fig 1). Inhaled NO was stopped on postoperative day 2, and the infant was extubated on postoperative day 3. Pulmonary artery pressure was normal 3 weeks after operation. The infant was discharged on postoperative day 25 in good clinical condition. At the latest follow-up (10 months of age), neurological development was normal.
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Fig 1. Echocardiographic views on day 13 of life (after the preoperative stabilization period with extracorporeal membrane oxygenation): (A) Subcostal view of transposition of the great arteries with intact ventricular septum and (B) assessment of good left ventricular function with fractional shortening of 39% and ejection fraction of 0.69. (Ao = aorta; LV = left ventricle; LVd = left ventricular diastolic diameter; LVs = left ventricular systolic diameter; MPA = main pulmonary artery; RV = right ventricle; RVd = right ventricular diastolic diameter.)
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Comment
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The overall experience with the management of TGA with intact ventricular septum complicated by PPHN underlines the poor response to prostaglandin E1 infusion and balloon atrial septostomy and suggests that PPHN represents an additional risk factor for early mortality [2, 3]. Persistent pulmonary hypertension is characterized by sustained elevation of pulmonary vascular resistance and low pulmonary blood flow, causing severe hypoxemia and an extrapulmonary right-to-left shunt across the ductus arteriosus and the oval foramen. In TGA with PPHN, low pulmonary blood flow impairs left atrial filling and interatrial blood mixing even with a large interatrial communication. Right-to-left ductal shunting explains why arterial oxygen saturation is higher in the lower body than in the upper body. Inhaled NO has yielded encouraging results in this group of neonates [1, 5]. Other medications—sildenafil citrate, bosentan, beraprost-sodium are currently under consideration for use in these situations but require further evaluation.
Extracorporeal membrane oxygenation has emerged as an effective method of mechanical support after repair of congenital cardiac lesions in children with refractory cardiac failure. However, few infants with TGA have been treated with ECMO as preoperative support (Table 2) [1, 6]. In these previous cases, postoperative hemodynamic deterioration was observed and necessitated resumption of ECMO. However, operation was performed immediately after weaning from ECMO. The authors suggested that both pulmonary hypertension and left ventricular "deconditioning" resulting from preoperative total cardiopulmonary bypass were responsible for the postoperative deterioration. Although it stabilizes the circulation before operation, the use of ECMO may substantially reduce myocardial preload and result in left ventricular "deconditioning" [1]. To avoid postoperative cardiac failure, these authors speculated that ECMO should not exceed 3 days before the arterial switch operation and advocated progressive weaning from ECMO to allow gradual "reconditioning" of the myocardium for a few days after surgical intervention.
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Table 2. Management and Follow-Up of Neonates With Transposition of Great Arteries and Persistent Pulmonary Hypertension Treated by Preoperative Extracorporeal Membrane Oxygenationa
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Our observation does not support this hypothesis. Echocardiography did not show evidence of left ventricular "deconditioning" during the preoperative period. Especially, the shape of the septum was flat, and the ventricular mass did not decrease. Furthermore, neither clinical nor echocardiographic markers of postoperative cardiac failure were observed after operation despite 6 days of ECMO and 7 days between ECMO and the surgical procedure. We suggest that PPHN may prevent left ventricular "deconditioning." Indeed, echocardiography showed evidence of sustained high although infrasystemic pulmonary artery pressure during ECMO and the preoperative period. Persistent pulmonary hypertension is associated with vascular changes such as increased muscularization of the small pulmonary arteries, which require prolonged treatment [2]. We further speculate that immediate operation after ECMO can result in postoperative deterioration related to recurrent PPHN.
In conclusion, ECMO followed by an arterial switch operation was used successfully in a newborn with TGA and PPHN. We recommend careful echocardiographic monitoring of pulmonary hemodynamics to determine the optimal duration of the preoperative stabilization period.
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References
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- Luciani GB, Chang AC, Starnes VA. Surgical repair of transposition of the great arteries in neonates with persistent pulmonary hypertension Ann Thorac Surg 1996;61:800-805.[Abstract/Free Full Text]
- Kumar A, Taylor GP, Sandor GG, Patterson MW. Pulmonary vascular disease in neonates with transposition of the great arteries and intact ventricular septum Br Heart J 1993;69:442-445.[Abstract/Free Full Text]
- Wernovsky G, Mayer Jr JE, Jonas RA, et al. Factors influencing early and late outcome of the arterial switch operation for transposition of the great arteries J Thorac Cardiovasc Surg 1995;109:289-302.[Abstract/Free Full Text]
- Kirklin JW, Blackstone EH, Tchervenkov CI, Castaneda AR. Clinical outcomes after the arterial switch operation for transpositionPatient, support, procedural, and institutional risk factors. Congenital Heart Surgeons Society. Circulation 1992;86:1501-1515.[Abstract/Free Full Text]
- Chang AC, Wernovsky G, Kulik TJ, Jonas RA, Wessel DL. Management of the neonate with transposition of the great arteries and persistent pulmonary hypertension Am J Cardiol 1991;68:1253-1255.
- Trittenwein G, Fürst G, Golej J, et al. Preoperative ECMO in congenital cyanotic heart disease using the AREC system Ann Thorac Surg 1997;63:1298-1302.[Abstract/Free Full Text]
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Abstract
Introduction
