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Ann Thorac Surg 2002;73:1786-1793
© 2002 The Society of Thoracic Surgeons
a Section of Thoracic and Cardiovascular Surgery, Children’s Hospital at Oklahoma University Medical Center, Oklahoma City, Oklahoma, USA
b Section of Pediatric Cardiology, Children’s Hospital at Oklahoma University Medical Center, Oklahoma City, Oklahoma, USA
* Address reprint requests to Dr Knott-Craig, Section of Thoracic and Cardiovascular Surgery, University of Oklahoma Health Sciences Center, 920 Stanton Young Blvd, Room WP2230, Oklahoma City, OK 73104, USA
e-mail: ckc{at}ouhsc.edu
Presented at the Forty-eighth Annual Meeting of the Southern Thoracic Surgical Association, San Antonio, TX, Nov 8–10, 2001.
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Abstract |
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Methods. Since 1994, 8 severely symptomatic neonates and young infants underwent biventricular repair by one surgeon. Six had Ebstein’s anomaly and 2 had physiologically similar pathology with severe tricuspid valve dysplasia, cyanosis, and gross cardiomegaly. One Ebstein patient (2 months old) had undergone a Starnes operation elsewhere. Weight of the patients at operation ranged from 2.1 to 6.4 kg (mean 2.7 kg). Five patients had either anatomical (n = 3) or functional (n = 2) pulmonary atresia. Severe (4/4) tricuspid regurgitation was present in all except 1 (Starnes operation), and cardiothoracic ratio exceeded 0.85 in all patients. Echocardiography severity scores were >1.5 in 6 (grade 4/4) and 1.3 in 1 (grade 3/4). Repair consisted of tricuspid valve repair, reduction atrioplasty, relief of right ventricular outflow tract obstruction, partial closure of atrial septal defect, and correction of all associated cardiac defects. Technique of tricuspid valve repair evolved over time: 3 had Danielson-type repairs, 3 had DeVega-type repairs, and 2 had complex repairs.
Results. One patient died in hospital: a 2.1 kg patient with tricuspid dysplasia, anatomical pulmonary atresia, and hypoplastic pulmonary arteries. The other 7 patients are all in functional class I and in sinus rhythm. Although 3 patients had symptomatic tachyarrhythmias before surgery, no child has experienced SVT after discharge. At recent echocardiography 4 patients had mild tricuspid regurgitation, and 2 had mild-moderate (2/4) tricuspid regurgitation. Three patients are now 7 years old, 2 are almost 2 years old, and the remaining 2 patients are 1 year old.
Conclusions. Surgical repair of the severely symptomatic neonate with Ebstein’s anomaly is feasible and safe. The repair appears durable and with good medium-term outcome.
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Introduction |
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However, when EA becomes clinically evident in the neonatal period the condition is often fatal [12–15]. In the severely symptomatic neonate EA seems to be associated with more complex morphology and pathophysiology [12, 13, 16] and continues to have a dismal prognosis despite various surgical interventions both conservative and aggressive [12, 16]. Currently attempts to palliate EA in the neonate involve converting the heart to a single-ventricle physiology [17]. Predictors of fetal and neonatal death in patients with EA are well documented (Table 1) [12, 13, 18, 19].
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Material and methods |
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Patients with ventricular discordance or other complex intracardiac anatomy are not included. Six patients were neonates. One 2-month-old infant was transferred to us from out of state having previously undergone a Starnes single-ventricle palliation [17]; this ventilator-dependent infant sustained three cardiac arrests during the transfer to Oklahoma City in the 24 hours before her biventricular repair. The final patient, a 4-month-old infant from out of state, was cyanotic with an associated conotruncal ventricular septal defect (VSD) and in severe congestive heart failure. One other neonate with EA developed a pneumothorax and died within 3 hours of birth and is therefore not included. One additional neonate with a less severe form of EA was successfully treated conservatively.
Patients weighed 2.1 to 3.8 kg at the time of operation (Table 2), except for the older infant who weighed 6.4 kg. Except for the patient who had previously undergone a reduction atrioplasty and Starnes palliation, all patients had a cardiothoracic ratio exceeding 0.85. Six patients had an echocardiographic severity score of greater than 1.5:1 (grade 4/4) and one had a severity score of 1.3:1 (grade 3/4) [12]. (Echocardiographic severity score is the ratio of the sum of the areas of the right atrium and atrialized portion of the right ventricle to the sum of the areas of the functional right ventricle, the left atrium, and left ventricle.)
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Perioperative management
The perioperative management of these critically ill neonates is fully described in our previous report [20]. The patients are immediately paralyzed and heavily sedated to minimize pulmonary vascular resistance and reduce mean ventilating pressures. Dopamine or isoproterenol and prostaglandin infusions are started immediately to maintain cardiac output and ductal patency. Echocardiograms are regularly done to evaluate function and document a reduction in pulmonary vascular resistance (as evidenced by the start of prograde flow through the pulmonary valve). It is important that the ventilating tidal volumes used are sufficient to overcome the cardiomegaly present (10 to 15 mL/kg body weight) in order to reexpand the lungs and thereby reduce pulmonary vascular resistance. Although the lungs initially appear hypoplastic, the pulmonary arterial and parenchymal architecture are both generally normal [21]. The oxygen saturations typically improve from the mid 70s to the mid 80s within 2 to 3 days. Inhaled nitric oxide may be useful both preoperatively and postoperatively [20, 22]. We used iNO in the last 2 patients in our series (it was not available to use before this).
Postoperatively, a similar strategy is used—often the oxygen saturations are in the mid 70s for the first 2 to 3 days after operation, especially if the functional right ventricle is small or the tricuspid valve repair is less than ideal. The philosophy of patience is crucial to achieving a successful outcome. We think it important to open the right pleural cavity to prevent tamponade and to leave a drain in the peritoneal cavity to prevent ascites accumulation and to reduce mean airway pressures.
Surgical repair
Reduction atrioplasty
An aggressive reduction right atrioplasty is done to correct the gross cardiomegaly present and allow more room for the lungs to ventilate. This usually takes the form of an elliptical resection of right atrium free wall. It is useful to mark out the course of the right coronary artery with a marking pen prior to arresting the heart to ensure that it is not injured during the resection and subsequent repair. This was probably responsible for the death in patient 6.
Correction of all associated cardiac defects
The infundibulum forms a dominant portion of the functional right ventricle in EA, and as such needs to be carefully conserved if a right ventricular outflow tract (RVOT) patch or pulmonary valve replacement is necessary (patients 3, 5, 6). If pulmonary atresia is present and the tricuspid valve repair is less than ideal, a small homograft or other valved conduit may help to unload the dysfunctional small right ventricle (patients 5 and 6). When functional pulmonary atresia or stenosis is present, calibrating or dilating the pulmonary valve through the opened right atrium is not only helpful (patient 2) but also provides important information about the orientation of the tricuspid valve opening.
The surgical anatomy may be deceptive and more complex when a VSD is present because the left ventricle may be ejecting through the VSD into the pulmonary artery through the infundibular chamber, which may not be functionally connected to the right atrium, or to the inlet or trabecular parts of the right ventricle. This obstruction at the os infundibuli level within the functional right ventricle may be caused by abnormal muscle bundles, muscularization, and tethering of the anterior leaflet of the tricuspid valve or malorientation of the tricuspid valve opening [3, 4, 16, 23, 24]. In this situation the repair of the tricuspid valve may be much more complex (patient 8). In addition the pulmonary vascular resistance may be higher than anticipated and the addition of a bidirectional Glenn anastomosis (hemi-Fontan) to the Ebstein repair may be disastrous. In patient 5 the hemi-Fontan needed to be taken down 3 days after the repair for this very reason.
Fenestrated ASD closure
In the presence of a small, poorly functioning right ventricle and in the neonatal context of elevated pulmonary vascular resistance, leaving an intra-atrial communication is essential. We believe that the atrial septal defect (ASD) should be closed but fenestrated, leaving an opening of about 3 mm. The size of the fenestration should however be inversely proportional to the effectiveness of the tricuspid valve repair. In older infants with a competent pulmonary valve, the right ventricle may be alternatively unloaded by adding a hemi-Fontan connection (bidirectional Glenn anastomosis) to the repair (patients 5 and 8) [25]. This 11/2 ventricle repair is usually well tolerated in older infants. At most recent follow-up the ASD fenestrations had closed spontaneously in all but 1 patient. This patient is only 15 months old and we expect this to close spontaneously too.
Tricuspid valve repair
The surgical repair has evolved over time as the anatomy is better appreciated and variations in the morphology are encountered. In the majority of those patients with EA who survive to adulthood before surgical intervention is warranted, the effective orifice of the tricuspid valve is directed toward the RVOT (Fig 1,
"Y" arrow) [3, 4, 23]. This is also the case in many of the neonates—certainly in 2 of our first 3 patients. The anterior leaflet is usually large, sail-like, and freely mobile. This allows a simple lateral annuloplasty to be done with the goals being to reduce the annulus from about 23 mm to about 13 to 14 mm and creating a competent monocusp valve. In typical EA this is easily achieved using the modified Danielson technique of placing a pledgetted suture at the anteroposterior commissure and bringing this down to the coronary sinus (Fig 2)
thus creating a "double orifice" valve, the inferior (lateral) orifice containing the atrialized right ventricle, which may then be closed by plicating it vertically. It is important to bring this suture down to the coronary sinus or even to the ventricular septum deeper within the cavity of the right ventricle so that the plane of the valve is closer to the junction between the atrialized and functional RV and not closer to the anatomical annulus, as this would stretch out the chordae of the anterior leaflet and so limit the sail-like anterior leaflet from properly coapting. If the large anterior leaflet still does not coapt well with the ventricular septum, a pledgetted suture from the anterior papillary muscle to the ventricular septum may be used to correct this (Fig 3
[Gordon K. Danielson, personal communication]), similar to the technique used by Sebening [11].
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In the more severe forms of EA in the neonate the orifice of the tricuspid valve is directed away from the outflow tract toward the apex of the right ventricle (Fig 1, "X"). This may be caused by the superior portion of the anterior leaflet having a distal linear attachment to muscle, or by the midportion of the anterior leaflet being tethered down to muscle at the junction between the trabecular and outlet portions of the functional RV. In addition, the commissure between the anterior and septal leaflets may be imperforate or patent only through small fenestrations [3], and this may represent the only access to the functional RV from the atrium; the posterior leaflet on the other hand may be reasonably well developed and mobile. Clearly, doing any type of posterior (lateral) annuloplasty in this situation would create near atresia of the tricuspid valve. In these circumstances detaching the entire anterior and posterior leaflets from the annulus (Fig 3, dashed line A–B), freeing the leaflets from their muscularized attachments and reducing the annulus in size posteriorly (Fig 4), and then reattaching the leaflets to the smaller annulus not only corrects the defect but also effectively changes the orientation of the tricuspid valve back to the outflow tract and the functional RV. The annulus is effectively narrowed by a clockwise rotation, while the valve is reorientated by an anticlockwise rotation relative to the annulus (Fig 5, patient 8). Fenestrating the antero-septal commissure and leaflet further prevents tricuspid stenosis (Fig 3, dashed line C–D; patient 8). At some point it may be necessary to enlarge the anterior leaflet with a strip of pericardium, although we have not yet needed to do this.
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Results |
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Three patients are now 7 years old, 1 is 2 years old, and the rest are 17 months, 15 months, and 1 year old (Table 2).
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Comment |
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Recently we published our results with successful biventricular repair in 3 neonates [20], and subsequently have had the opportunity to successfully operate on several more neonates both in our institution and around the country (not included in this manuscript). We believe that our philosophy of care, rather than the details of the surgical technique, has been central to our modest success in a small series of patients. Maintaining cardiac output by fenestrating the ASD closure, minimizing oxygen requirements by prolonged postoperative anesthesia, maintaining postoperative hematocrit greater than 42 and tolerating lower oxygen saturations for 5 to 10 days (as in the postoperative care of neonatal tetralogy of Fallot or post-Norwood patients) all are critically important in allowing the dysfunctional small right ventricle to recover and assume an increasing workload and cardiac output in these critically ill neonates.
The surgical techniques we have used are not new or complicated; they are well documented in the literature in the exceptional contributions of pioneers such as Danielson [7, 30], Chauvaud and Carpentier [8, 9], and Sebening [11]. The clinical presentations and writings of Anderson [3, 4, 31, 32] and Thiene [16, 23] have greatly enhanced our understanding of the morphology of EA. Furthermore the concept that the poor prognosis is related to fetal pulmonary hypoplasia resulting from gross cardiomegaly restricting the development of the lungs, as in neonates with diaphragmatic hernias, appears to be wrong; neonates with EA seem to have normal pulmonary vasculature and parenchyma [21]. We have come to realize that there is no perfect repair for all neonates with EA; because the morphology is so variable, the surgeon needs to be familiar with all the techniques outlined above and be prepared to be more or less aggressive depending on the anatomy. The preoperative echocardiogram is often misleading in our experience because the entire anterior leaflet cannot be visualized in a single view; it may appear to be freely mobile yet be well tethered down in its most superior part. If this is not detected at operation and a lateral annuloplasty repair is done, a successful outcome is unlikely.
The indications for urgent intervention based on the fetal or neonatal echocardiogram are sound (Table 1) [12, 13, 33, 34]: in the fetus, echocardiographic severity scores grade 4, especially associated with a small intra-atrial communication, predict perinatal death without surgery. In the neonate, a cardiothoracic ratio greater than 0.85, echocardiographic severity score grade 4, or grade 3 associated with cyanosis, and severe tricuspid regurgitation all predict neonatal death without surgery. In our experience, 2 other neonates fitting these criteria were initially referred to our center from out of state for surgery; in both cases the referral was canceled 3 to 4 days postnatal because the neonates seemed to be "doing great." Neither survived the neonatal period. Neonates and young infants fulfilling the criteria outlined in Table 1 should probably undergo surgical repair irrespective of their clinical condition.
In summary, biventricular repair of Ebstein’s anomaly in the critically ill neonate is feasible and the repair seems durable, at least through 7 years of follow-up. The absence of arrhythmias postoperatively is encouraging and may argue for the earlier repair of EA as a means of possibly preventing tachyarrhythmias in EA patients. As experience is gained and more complex repairs are successfully completed, a more aggressive attitude to critically unstable neonates may be warranted; that could include intrauterine diagnosis, elective delivery, and placement on extracorporeal membrane oxygenation, followed later by biventricular repair.
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Discussion |
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What I am curious about and would like you to comment on is the right ventricular outflow tract issues related to these babies because so many of them have significant pulmonary hypertension and functional, if not truly anatomic, pulmonary outflow problems even as severe as atresia. So I am wondering what do you add to your repair in these babies related to their right ventricular outflow tract? Are you giving them pulmonary conduits, are you giving them monocusps? Perhaps you could describe that part of the repair as well.
DR KNOTT-CRAIG: Thank you very much, Ross. The management of the right ventricular outflow tract is critical to the care of these patients. If one is forced to operate within the first two or three days, it is more difficult to assess the adequacy of the pulmonary valve and the right ventricular outflow tract; it is helpful to delay surgery until you see echocardiographic evidence of prograde flow through the pulmonary valve. Furthermore, in Ebstein’s anomaly, the infundibulum forms a major part of the functional right ventricle, and is critical to the forward flow of blood; therefore, one needs to minimize the incisions in the right ventricle. In patients with associated pulmonary atresia, one should achieve a reasonably competent valve in the pulmonary position, particularly if the tricuspid valve repair is less than ideal. We have used small pulmonary homografts in two patients.
DR IRVING L. KRON (Charlottesville, VA): Chris provided me the manuscript and, by the way, it is very well written and has excellent illustrations. How much postoperative tricuspid regurgitation or TEE would you accept? What are the typical saturations postoperatively? You leave a fenestration; you have clearly some shunting going on. What situations would you accept postoperatively? Thank you.
DR KNOTT-CRAIG: Those are both excellent questions. Postoperatively, four had mild tricuspid regurgitation and two had mild to moderate tricuspid regurgitation (one patient had a valve replacement). One of the latter, in fact, had a heart catheterization after which the tricuspid regurgitation was downgraded to mild from mild-to-moderate. Very early postoperatively, most patients have moderate regurgitation by echo.
Second, one needs to be patient in the early postoperative period; saturations typically in the first 24 to 48 hours are in the mid-70% range. These generally improve over 2 or 3 days; however, by the time the children go home, they are fully saturated. The saturations are inversely proportional to the size of the interatrial fenestration. The size of the fenestration depends on the surgeon’s assessment of the tricuspid valve repair—the better the repair, the smaller the fenestration that is left.
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References |
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