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Ann Thorac Surg 1995;60:970-976
© 1995 The Society of Thoracic Surgeons
Department of Pediatric Cardiac Surgery, Marie Lannelongue Hospital, Le Plessis-Robinson, France
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Abstract |
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 TopFootnotes Abstract IntroductionMaterial and MethodsResultsCommentReferences |
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Methods. Twenty-seven of 96 patients with univentricular heart and unobstructed pulmonary blood flow referred for surgical palliation were seen with systemic obstruction. Twenty-six were neonates with coarctation of the aorta in 21 and subaortic stenosis in 5. In 8 other patients, subaortic stenosis developed after initial pulmonary artery banding. Four different palliative procedures were performed: coarctation repair with pulmonary artery banding (group I, n = 15); Norwood or Damus-Kaye-Stansel or arterial switch operation (group II, n = 9); coarctation repair with pulmonary artery banding and bulboventricular foramen enlargement (group III, n = 2); and orthotopic heart transplantation with coarctation repair (group IV, n = 1).
Results. The mortality rate was 34.3% (n = 12) for all patients, 53.3% in group I, 33.3% in group II (p = 0.003 versus group I), and 50% in group III. Nine patients (8 in group I and 1 in group II) had development of subaortic stenosis and underwent a subsequent procedure: Damus-Kaye-Stansel operation in 5, arterial switch operation in 3, and bulboventricular foramen enlargement in 1. Three had a concomitant or subsequent Fontan procedure and 2, a bidirectional Glenn procedure. In group II, 1 patient underwent a subsequent Fontan procedure and another, a bidirectional Glenn anastomosis. Six of the 8 patients with subaortic stenosis after initial pulmonary artery banding underwent a second stage consisting of a Damus-Kaye-Stansel procedure (n = 3), bulboventricular foramen enlargement (n = 2), or creation of an aortopulmonary window (n = 1). Three had a concomitant Fontan procedure and 2, a bidirectional Glenn procedure. Actuarial 4-year survival was 65.5% ± 8.4% (70% confidence limits) for all patients; it was 40% ± 13.3% in group I and 66.6% ± 16.3% in group II (p < 0.05).
Conclusions. Initial management of patients with univentricular heart and systemic obstruction by Norwood-like procedures provides a better outcome. Success of the Fontan operation relies on the ability to provide timely relief of subaortic stenosis.
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Introduction |
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TopFootnotesAbstractIntroductionMaterial and MethodsResultsCommentReferences |
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Systemic obstruction by aortic arch obstruction, subaortic stenosis (SAoS), or a combination of both has been widely recognized as an important risk factor for a poor outcome in patients with single-ventricle physiology [1–3]. Although several methods of relief [4–11] have been tried, the outcome after a Fontan-type procedure is still unpredictable, probably because of the deleterious myocardial hypertrophy subsequent to SAoS [12, 13]. Subaortic stenosis can be present when the patient is first seen but can also occur after palliation and even after a Fontan operation [14–16]. It remains difficult to define which patients are at risk for SAoS when it is not initially present.
There are obvious categories of anatomic arrangements highly suggestive of development of SAoS, namely, patients with single ventricle, transposed great arteries, and aortic arch obstruction or patients with a neonatal bulboventricular foramen (BVF) area smaller than 2 cm2/m2 [17]. There are other anatomic situations in which, although the initial BVF area was large enough at the time of the initial palliative pulmonary artery banding (PAB), SAoS occurs with time. Therefore, to bridge these patients from birth to Fontan operation, it seems important and logical to manage them as optimally as possible by ensuring an unobstructed systemic pathway and avoiding any volume or pressure overloads. It is also important to preserve a stable pulmonary arterial bed, adequate atrioventricular valve function, and low pulmonary vascular resistance.
The initial clinical presentation is generally heart failure with torrential pulmonary blood flow. Pulmonary artery banding can provide adequate control of the heart failure, and if coarctation is present, coarctation repair is generally necessary. As awareness of the higher incidence of SAoS in these patients has increased, the initial surgical management has changed to a more aggressive approach, namely, a Norwood procedure or palliative arterial switch, as the first-stage operation [4–11]. This report reviews our experience with systemic obstruction in patients born with a single ventricle and compares the results of four different surgical approaches for neonates over a period of 14 years.
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Material and Methods |
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TopFootnotesAbstractIntroductionMaterial and MethodsResultsCommentReferences |
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After initial palliation, patients were followed by their referring pediatric cardiologists, and clinical progress and outcome were obtained by contacting them. Subaortic obstruction appeared secondarily in 19 other patients, 11 with and 8 without initial aortic arch obstruction. Therefore, a total of 35 patients (36.5%) with single-ventricle physiology and increased pulmonary blood flow were seen initially with or later had development of systemic obstruction. Patients with aortic stenosis or hypoplastic left heart syndrome were excluded.
Definitions and Morphology
The diagnosis was established by means of echocardiography and angiocardiography according to the method of Anderson and colleagues (Table 1
). Atrioventricular connection was concordant in all but 1 patient. Double-inlet left ventricle was demonstrated in 16 patients and absent atrioventricular valve connection was present on the right side in 8 and on the left side in 8. A common AV valve was found in 3. Great vessels were transposed in 28 patients and normally related in 7. The main ventricular chamber was of the left type in 26 patients and the right type in 9. None of the patients had abnormal pulmonary venous return.
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Surgical Techniques
Four surgical techniques were employed in neonates. Patients without evidence of SAoS at initial presentation were managed classically without attention given to the subaortic area.
PAB AND COARCTATION REPAIR (GROUP I).
Fifteen neonates had operation with this technique through a left thoracotomy. The aortic arch anatomy in each patient demonstrated an isthmic coarctation associated with a hypoplastic transverse arch. The aortic arch was repaired by an extended end-to-end anastomosis [18], and then PAB was performed to obtain a mean pressure of 20 mm Hg in the distal pulmonary artery. Seven of these patients already had a mild SAoS (mean gradient, 19 ± 5 mm Hg). The median age at operation was 15 days (range, 4 to 90 days), and the mean weight was 2.9 ± 0.6 kg.
NORWOOD-TYPE OPERATION OR PALLIATIVE ARTERIAL SWITCH (GROUP II).
Nine neonates (median age, 30 days, and mean weight, 3.2 ± 0.23 kg) with aortic coarctation and hypoplastic transverse arch (n = 4) or SAoS (n = 5) were managed according to this protocol. The palliative arterial switch, initially described by Freedom and colleagues [19] and then applied by the Melbourne group [9] to neonates with this condition, was performed in 4 patients with transposition of the great arteries [10]. Five other patients underwent a Norwood-like operation with the Lamberti modification [20] in the presence of transposed great arteries. In all these patients, the pulmonary arterial bed was supplied by a systemic-pulmonary shunt with a 4-mm Gore-Tex conduit. However, patients with an arterial switch operation also had a neo-pulmonary pathway left open.
COARCTATION REPAIR, PAB, AND ENLARGEMENT OF BVF (GROUP III).
Two patients 7 days and 7 months old underwent this procedure. The BVF was enlarged once through the aortic valve and once through a small ventriculotomy on the subaortic outlet chamber, the ventriculotomy being closed with a pericardial patch. After completion of the operation and weaning from bypass, the band was applied on the pulmonary artery and tighted to reach 20 mm Hg of mean pulmonary artery pressure.
ORTHOTOPIC HEART TRANSPLANTATION (GROUP IV).
One patient in this series was seen with coarctation, SAoS, and very poor ventricular function precluding all type of palliation. A matched heart was available, and he underwent orthotopic heart transplantation with reconstruction of the transverse aortic arch.
Follow-up
Survivors were followed by their referring pediatric cardiologists. Time of occurrence or recurrence of SAoS was recorded. Reoperation for relief of SAoS or a modified Fontan program was also noted. The latter was indicated when cyanosis was important. Repeat cardiac catheterization was performed whenever there was a clinical indication for entry into the Fontan program; otherwise, echocardiography with Doppler studies was used alone. The median follow-up was 32 months (range, 1 to 165 months).
Statistical Analysis
Early mortality rates were analyzed only in similar groups, namely, neonates with single ventricle, high pulmonary blood flow, with or without aortic coarctation, and without SAoS (groups I, II, and III). The patient in group IV and those with development of SAoS after PAB were not included in the statistical study. Survival was analyzed according to the Kaplan-Meier methods. Comparisons between survival curves were performed by the log-rank test. Comparisons between dichotomous variables were performed by 
2 test, and Student's t test was used to compare continuous variables. Ratios are expressed with 70% confidence limits (CL).
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Results |
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TopFootnotesAbstractIntroductionMaterial and MethodsResultsCommentReferences |
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. The courses of patients with initial systemic obstruction and those with later development of systemic obstruction are emphasized.
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). In group I, 3 patients died late after the first operation at different steps in the management program, and another patient had early takedown of a modified Fontan procedure. Therefore, the overall rate of failure of management defined as death or Fontan takedown in this group was 60% (70% CL, 43.3% to 75%) (p = 0.003 versus group II).
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Modified Fontan Program
Entry into a modified Fontan program was determined primarily by the clinical status of the patient and secondarily by the hemodynamic and angiographic assessments according to the criteria of Choussat and associates [21]. Five patients underwent a bidirectional Glenn anastomosis associated with another procedure to relieve SAoS. The mean age at this anastomosis was 13.5 months, and the mean postoperative arterial oxygen saturation was 75%. In this group of 5 patients there was one early death and one late death.
Six patients underwent a modified Fontan operation in association with various techniques to relieve SAoS without any deaths. Two of them had intraatrial baffle fenestration using a 3-mm hole in the atrial baffle. One patient, initially managed by aortic coarctation repair and PAB, underwent a palliative arterial switch operation with a modified B-T shunt 3 months later. This patient had a pulmonary pathway left patent in addition to the B-T shunt. When he was 1.5 years old, he underwent a modified Fontan operation, which required early takedown. The mean age at modified Fontan operation was 50 months, and the mean postoperative arterial oxygen saturation was 98%. In this group, there was no morbidity in terms of pleural effusion and ventricular dysfunction.
The overall actuarial survival at 4 years was 65.45% ± 8.4%. In group I it was 40% ± 13.3%, whereas in group II, it was 66.6% ± 16.3% (p < 0.05 versus group I at 12 months) (Fig 5).
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Comment |
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TopFootnotesAbstractIntroductionMaterial and MethodsResultsCommentReferences |
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When considering these patients, it can be very difficult to define reliable criteria predictive of the development of SAoS. The pressure gradient across the BVF might not be a reliable criterion because of ductal patency and hemodynamic instability of the patient. Consequently, Matitiau and colleagues [17] indexed the area of the BVF to the body surface area and proposed an initial index inferior to 2 cm2/m2 as a strong predictor of late SAoS. Others [9] have also proposed that if the ratio between the BVF and aortic valve areas is less than 1, the patient can be considered a candidate for potential development of SAoS.
On the basis of our experience, we have determined empirically that all neonates, regardless of BVF size, seen with single-ventricle physiology, unobstructed pulmonary blood flow, transposition of the great arteries, and aortic arch obstruction are prone to develop a secondary subaortic obstruction and therefore should undergo a more radical procedure. Patients with other anatomic arrangements should undergo PAB alone if they are free from SAoS. In the latter case, frequent careful observations of the subaortic area to detect the occurrence of an obstruction allow time for intervention. Indeed, of the 69 patients who underwent PAB for single ventricle and high pulmonary blood flow without aortic arch obstruction, only 8 had a secondary SAoS within a mean time of 48 months.
When dealing with neonatal palliation in these critically ill patients, several surgical options are available. The technique of aortic arch repair has previously been described [18]. If this procedure is chosen for use in these particular patients, it is important to make the extended end-to-end anastomosis as wide as possible to restore an unrestricted aortic arch. However, the high frequency of severe hypoplasia of the transverse arch may lead to incomplete relief of aortic obstruction. The addition of PAB will cause double obstruction to ventricular ejection. Four aggressive approaches that might prevent or relieve SAoS were used in this series. Orthotopic heart transplantation has been applied by Bailey and colleagues [26] in hypoplastic left heart syndrome and variants with good early and intermediate results. However, the shortage of available donors considerably limits this option.
Bulboventricular foramen enlargement, as described by Cheung and associates [7], corrects the site of restriction and provides a systolic-diastolic flow into the pulmonary vascular bed, which may preserve the pulmonary vascular reactivity. Although it is generally performed in older children, we used this technique in 1 neonate, who had recurrence of SAoS and underwent a DKS anastomosis. We believe that exposure of the subaortic area in neonates can be very difficult, and the necessary addition of a pulmonary artery band can promote recurrence of this stenosis. Moreover, adjustment of the band is relatively more difficult in patients after cardiopulmonary bypass because of unstable pulmonary vascular reactivity. Therefore, this technique is reserved for older children and patients with long-standing PAB in whom pulmonary valve function is questionable. We also chose this approach for 1 patient in whom the subaortic obstruction appeared after a Fontan operation. However, the SAoS is not always due to a restrictive BVF, but also in some cases to abnormal attachments of the atrioventricular valves. In these cases, BVF enlargement will not be effective.
We believe a Norwood-type intervention and the palliative arterial switch operation are the two most effective procedures for neonates. The neonatal palliative arterial switch operation initially seemed an attractive procedure because it theoretically avoided the need of a systemic-pulmonary shunt and the complex postoperative balance between systemic and pulmonary circulations. However, all patients in our series and 3 of 5 in the series reported by Karl and colleagues [9] needed a shunt, therefore converting them to a condition of single ventricle with multiple sources of pulmonary blood flow. In fact, 2 of our patients underwent Fontan procedures, one of which was taken down 2 days later. We think the main theoretical advantage of the arterial switch operation over the Norwood procedure should be a more physiologic coronary circulation because of the reimplantation of the coronary ostia in the adjacent sinuses of Valsalva. Finally, the neonatal Norwood- or DKS--type procedures with the Lamberti modification proved to be simpler than the other techniques while producing the same functional results. The single death in these patients was due to bleeding at the site of the anastomosis at the beginning of our experience.
On the other hand, when a DKS anastomosis or an arterial switch was performed after an initial conventional procedure, mortality seemed to be related to myocardial performance and type of vascular supply to the lungs. A modified B-T shunt resulted in death or failure of a Fontan operation in 3 of 4 patients, whereas a bidirectional Glenn anastomosis (n = 3) or a total cavopulmonary anastomosis (n = 6) resulted in one early and one secondary death in 9 patients. Therefore, the technique of vascular lung supply seems very important, particularly in these hypertrophied hearts. Indeed, it has been demonstrated by Chin and co-workers [27] that acute removal of ventricular overload in the presence of an unchanging ventricular mass results in geometric alterations that can impair diastolic ventricular performance after a Fontan operation by a mechanism of acute hypertrophic cardiomyopathy. Although these data were recorded in patients with shunts in whom the physiologic situation corresponded to an increased preload, one may anticipate that in patients with a pulmonary artery band and subaortic obstruction, ie, double obstruction to ventricular ejection, relief of subaortic obstruction associated with a modified B-T shunt will result in a physiologic situation of acute dilated cardiomyopathy.
Therefore, partial reestablishment of the pulmonary circulation in conjunction with the systemic circulation rather than in parallel with provision of the pulmonary blood flow by a bidirectional Glenn procedure has been advocated as an intermediate stage before the Fontan operation to obtain more progressive adaptation of the ventricular geometry. However, as reported by Gross and associates [28], a bidirectional Glenn anastomosis might not be an effective source of pulmonary blood flow in older children, and therefore a total cavopulmonary anastomosis should be performed. Our data support this hypothesis, and none of the patients who underwent relief of SAoS concomitant with total cavopulmonary anastomosis died. When there is a severe subaortic gradient, O'Leary [29], Di Donato [30], and their colleagues propose a staged approach consisting of BVF enlargement with PAB or a DKS anastomosis associated with a bidirectional Glenn anastomosis followed later by a Fontan operation. In these reports, the mean age at operation was between 5 and 10 years, and mortality rates ranged from 17% to 21%. We have chosen to perform the Fontan operation in younger children and when the subaortic gradient before relief was severe, the lateral cavocaval tunnel was fenestrated using a 3-mm hole in the baffle. However, depending on the team preference, an adjustable atrial septal defect will have the same advantages.
In conclusion, in accordance with Franklin and associates [31], we believe that coarctation repair in association with PAB should be abandoned in patients with single-ventricle physiology, high pulmonary blood flow, and aortic arch obstruction. A more radical Norwood-type operation provides better results in terms of survival and suitability for a Fontan procedure. When SAoS occurs secondarily after PAB, in patients without aortic arch obstruction and depending on their age and the duration of banding, a DKS operation or a BVF enlargement might be performed. In the latter instances, pulmonary blood flow is provided by a bidirectional Glenn anastomosis in children less than 18 months old and by a total cavopulmonary shunt in those older than 18 months. When the subaortic gradient before relief is higher than 50 mm Hg, the lateral cavocaval tunnel is fenestrated. Finally, when SAoS occurs after a Fontan operation, BVF enlargement seems to be the better option.
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Footnotes |
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TopFootnotesAbstractIntroductionMaterial and MethodsResultsCommentReferences |
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Address reprint requests to Dr Serraf, Department of Pediatric Cardiac Surgery, Marie Lannelongue Hospital, 133 avenue de la Résistance, 92350 Le Plessis-Robinson, France.
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TopFootnotesAbstractIntroductionMaterial and MethodsResultsCommentReferences |
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