Hybrid Management for Hypoplastic Left Heart Syndrome

Transcription

1 Pediatr Cardiol (2008) 29: DOI /s ORIGINAL ARTICLE Hybrid Management for Hypoplastic Left Heart Syndrome An Experience from Brazil Carlo B. Pilla Æ Carlos A. C. Pedra Æ Aldemir J. S. Nogueira Æ Marcelo Jatene Æ Luis Carlos B. Souza Æ Simone R. F. Pedra Æ Carlos Ferreiro Æ Claudia P. Ricachinevsky Æ Fernando A. Lucchese Received: 5 May 2007 / Accepted: 22 October 2007 / Published online: 13 December 2007 Ó Springer Science+Business Media, LLC 2007 Abstract Initial surgical reconstruction for hypoplastic left heart syndrome (HLHS) is associated with satisfactory outcomes only in a few referral centers. Moreover, there is a persistent high-risk period for sudden death while the patient waits for the next surgical procedure. The development of a less invasive approach, so-called hybrid, postponing a major surgery outside the neonatal period, might reduce the immediate and late surgical burden on these patients. This is a retrospective study of a contemporary series of patients with HLHS seen in two separate institutions. Patients with HLHS or its variants who underwent a hybrid management were included in the study. Data are described as the mean and standard deviation or absolute numbers and percentage, as appropriate. From January 2004 to June 2006, 15 patients (10 male; 5 ± 3.8 days old and 2.9 ± 0.5 kg) were included in the study. Ten had both mitral and aortic atresia; the ascending aorta and atrial septal defect measured 2.5 ± 1.4 and 4.9 ± 1.2 mm, respectively. There were six hospital survivors after stage I (mortality rate 60%). During the interstage period, all but one patient needed additional procedures. One patient died of bacterial meningitis 4 months after stage I. Four patients were submitted to stage II operation at 6.6 ± 0.5 months of age and one is waiting for the operation. All four required early reinterventions for C. B. Pilla (&) A. J. S. Nogueira C. P. Ricachinevsky F. A. Lucchese Complexo Hospitalar Santa Casa de Porto Alegre, Porto Alegre, RS, Brazil cbpilla@hotmail.com C. A. C. Pedra M. Jatene L. C. B. Souza S. R. F. Pedra C. Ferreiro Hospital do Coração da Associação Sanatório Sírio, São Paulo, SP, Brazil pulmonary artery stenosis. Only one was discharged home and was not yet submitted to the third stage. The hybrid approach for HLHS was associated with poor results in this early experience from two independent institutions in a developing country. This might have been related to infrastructure and technical problems, as well as our own learning curve. Institutions working under the same conditions might face similar problems during their initial experience. Keywords Congenital heart disease Stents Interventional cardiology Surgery Introduction Surgical approaches for the initial management for hypoplastic left heart syndrome (HLHS) are associated with satisfactory outcomes in a limited number of referral centers dealing with a large number of patients [10 12]. In addition, even when patients with this disease do well and survive the three steps toward a Fontan completion, cardiac sequelae are common and neurological outcomes are suboptimal, reflecting the need for multiple and prolonged cardiopulmonary bypass runs [9]. Cardiac transplantation is fraught with the limited availability of organ donors for neonates [3] and the need of lifetime immunosuppression and its attendant complications. Stenting of the arterial duct combined with banding of the pulmonary arteries and atrial septectomy or septostomy was introduced in the early 1990s for the initial palliation of this severe disease with satisfactory results [7]. This hybrid approach has been refined in the late 1990s and at the beginning of this decade [1]. Recently, it has been pushed to its limits: Cardiac surgeons and interventionalists have been working hand-in-

2 Pediatr Cardiol (2008) 29: hand not only during the initial palliation in phase I but also preparing the underlying anatomy during the comprehensive phase II operation for subsequent Fontan completion in the catheterization laboratory [6]. The encouraging initial results, reported even in poor candidates for the traditional surgical treatment, have underscored its potential application in high-risk patients [2]. In South America, surgical results for HLHS have been, in general, very disappointing with a high mortality rate for both the classic Norwood operation and, more recently, the Sano modification (nonpublished data). Because of this and the good initial outcomes of the hybrid procedure, some centers in Brazil decided to embark on this new management strategy. This article reports the results of an initial experience on the hybrid approach for HLHS in two referral centers for pediatric cardiology in Brazil. Materials and Methods Patient Selection From January 2004 to June 2006 a series of nonconsecutive patients with HLHS or its variants were selected for the hybrid phase I procedure among the two participating institutions (Porto Alegre and São Paulo). In the same period, some patients have still undergone a Norwood procedure according to their physicians preference. Informed consent was obtained from parents or guardians. Surgical and Interventional Techniques for Phase I After clinical stabilization in the intensive care unit using prostaglandins and mechanical ventilation in all patients, they were referred to the operating room for pulmonary artery banding, followed by ductal stenting under fluoroscopy using a portable C-arm. In all patients, invasive arterial blood pressure monitoring was obtained in the right radial artery; in some, another invasive arterial access was placed in the descending aorta via the umbilical artery or in the lower limbs. Prostaglandin infusion was discontinued at the beginning of the operation. If the atrial septal defect was deemed restrictive, a balloon atrial atrioseptostomy was performed just prior to or after the hybrid procedure, using the conventional femoral or a per-atrial approach and under fluoroscopic and transthoracic or transesophageal echocardiographic monitoring. Through a median sternotomy, both pulmonary arteries were dissected and exposed. They were banded using Gore-tex 1, silicone, or bovine pericardial bands according to the surgeon s preference (Fig. 1). They were empirically adjusted in order to achieve arterial oxygen saturation levels in the high 70s/ low 80s range. After both bands were properly adjusted, the right ventricular outflow tract or the main pulmonary artery was punctured just below or above the pulmonary valve, respectively, and a 6F or 7F regular sheath was secured in place using a purse string suture. Through the side arm of the sheath, repeat angiograms in lateral or steep left oblique views were performed to delineate the ductal anatomy and diameter and the adequacy of the pulmonary bands. The aortic arch features were also assessed, including the presence of a coarctation shelf and a stenotic distal aortic arch. In most patients, a premounted 19-mmlong balloon-expandable Genesis large stent (Cordis Corporation, Miami, FL) was implanted in the duct through the short sheath by the interventionalist. The final diameter of the stent depended on the size of the patient. A 7-mmdiameter stent was used for neonates under 1.5 kg, an 8- mm-diameter stent was used for kg neonates, and a 9 10-mm-diameter stent was used for neonates over 2.5 kg. Other stents were also employed: the 20-mm-long Bridge Assurant stent (Medtronic Inc., Minneapolis, MN), the 16- mm-long DoubleStrut LD stent (EV3, St. Paul, MN), the 17-mm-long Primus stent (EV3, St. Paul, MN), and the 20- mm-long self-expandable Protegé stent (EV3, St. Paul. MN). We aimed to place the proximal part of the stent just after the origin of the pulmonary arteries. In the case of malpositioning or if the stent did not fully cover a coarcted area, another stent was delivered, overlapping the first one. For those with aortic atresia, if there was a [20 mm Hg peak-to-peak pressure gradient between the upper and lower limbs associated with persistent electrocardiographic signs of myocardial ischemia, a 3-mm Gore-tex reverse shunt between the main pulmonary artery and the innominate artery was constructed [8]. The chest was then closed Fig. 1 Pulmonary trunk prior to stent implantation and after pulmonary artery branches bands positioned and secured

3 500 Pediatr Cardiol (2008) 29: using standard techniques and the patient was taken to the intensive care unit for routine management. Intravenous heparin infusion was maintained until oral feeding was reestablished. Subsequently, low doses (3 5 mg/kg) of aspirin were used for stent thrombosis prevention. Follow-up After Stage I (Interstage Period) Along with clinical evaluations, serial echocardiograms were performed every 1 3 weeks in order to check for right ventricular dysfunction, tricuspid valve regurgitation, atrial septal defect size, ductal stent patency, and pulmonary artery band adequacy. The patients were maintained on systemic vasodilators, digoxin, diuretics, and low-dose aspirin. There was a low threshold for additional cardiac catheterizations if there was a suspicion for a restrictive atrial septal defect, impaired retrograde flow to the aortic arch, or evidence for ductal stent obstruction. Routine diagnostic cardiac catheterization prior to the comprehensive phase II operation was performed at one of the two participating institutions (São Paulo). On this occasion, pressure tracings from the pulmonary arteries, atrial chambers, and across the ductal stent were obtained. Angiograms of the pulmonary arteries, the ductal stent, and the transverse aortic arch were done as well. At the other participating institution (Porto Alegre), a cardiac catheterization was deemed unnecessary and patients were referred for the phase II operation based solely on the echocardiographic findings. Comprehensive Phase II Operation In this operation, the ductal stent was removed by cutting the pulmonary artery and the descending aorta transversally. The removal was then performed in block or through peeling of the stent from the aortic wall. The neo-aorta was reconstructed using a homograft, either as a conduit or as a patch to enlarge the vessel. Initially, a modified hemi- Fontan type bidirectional cavopulmonary anastomosis (BCPA) was performed; this consisted of the placement of open surgical steel rings at the inferior vena cava right atrium junction and at the proximal part of the superior vena cava right pulmonary artery anastomosis. These rings were placed in order to work as radiopaque markers and retention spots to help positioning and implanting a covered stent at the time of Fontan completion in the catheterization laboratory. Later, we changed this approach to a standard BCPA, due to anticipated difficulties in performing a percutaneous Fontan completion in the future and to questions related to the integrity of the pulmonary arteries (see below). During the BCPA construction, the pulmonary arteries were surgically repaired if needed. The atrial septum was then removed, along with the interatrial stent, if present. The surgery was done under circulatory bypass, deep hypothermia, with a period of circulatory arrest and continuous cerebral low flow. In the intensive care unit, there was a low threshold to perform a cardiac catheterization if the patient exhibited signs of low cardiac output, hypoxemia, or high superior vena cava pressures despite optimization of medical therapy with inotropic support, nitric oxide, vasodilators, and diuretics. If stent implantation in the pulmonary arteries was required, coumadin was used aiming to prevent thrombus formation in the pulmonary circulation. Statistical Analysis Data were collected retrospectively through chart review. Values are expressed as the mean and standard deviation or absolute numbers and percentages, as appropriate. Results Between January 2004 and June 2006, 15 neonates (10 males) at a mean age of 5 ± 3.8 days and mean weight of 2.9 ± 0.5 kg underwent hybrid stage I procedures in the two participating institutions. Ten were managed at Porto Alegre. All but two were referred from and initially managed in local hospitals. Fourteen patients had typical HLHS; another patient had an atrioventricular septal defect with an imperforated left atrioventricular component and aortic atresia. The majority (10 patients; 66%) had functional mitral and aortic valve atresia. The ascending aorta measured 2.5 ± 1.4 mm and the atrial septal defect was 4.9 ± 1.2 mm. One patient had a very restrictive atrial septal defect measuring \1 mm (Table 1). Excluding the patient with a restrictive atrial septal defect, the arterial oxygen saturations of the patients were 88 ± 7% upon arrival at our centers. Seven had qualitatively mild-to-moderate right ventricular systolic dysfunction before the procedure, requiring inotropic support. Five (33%) required a balloon atrial septostomy immediately before or during the hybrid procedure. Pulmonary artery banding was performed with no technical difficulties. Balloon-expandable stents were used for ductal stenting in all but one patient, who had a self-expandable stent implanted. This patient required implantation of an additional stent (balloon-expandable) at the same procedure due to mal positioning of the self-expanding stent. None required the construction of a surgical shunt between the main pulmonary artery and the innominate artery. However, the patient with the very restrictive atrial septal

4 Pediatr Cardiol (2008) 29: Table 1 Patient characteristics Patient No. Institution Age (days) Gender Weight (kg) Cardiac morphology A aorta (mm) ASD (mm) 1 SC 2 Male 3.3 MS + AS SC 3 Male 3.8 MA + AA SC 2 Female 3 MA + AA SC 3 Male 2.9 MA + AA SC 12 Male 1.5 MA + AA SC 4 Male 2.7 MA + AA SC 10 Female 2.3 MS + AS SC 1 Male 2.9 MA + AA SC 1 Male 2.7 MS + AA 2 \1 10 SC 11 Female 3.4 AVSD 1.5 AVSD MA + AA 11 HC 8 Male 3.3 MA + AA HC 6 Male 2.8 MS + AS HC 3 Female 3 MA + AA HC 2 Male 2.9 MA + AA HC 2 Female 3.4 MS + AS Male 2.9 MA + AA (± 3.8) (66%) (± 0.5) -66% (± 1.4) (± 1.2) SC = Santa Casa, Porto Alegre; HC = Hospital do Coração, São Paulo; A Aorta = ascending aorta; ASD = atrial septal defect; MS = mitral stenosis; AS = aortic stenosis; MA = mitral atresia; AA = aortic atresia; AVSD = atrioventricular septal defect defect underwent surgical atrial septectomy on a brief run (10 min) of cardiopulmonary bypass. Hospital survival after the hybrid stage I procedure was 6 of 15 patients (40%). There were nine in-hospital deaths: four due to a persistent low cardiac output state unresponsive to pharmacological treatment; three due to acquired infection during the intensive care unit stay; one after a massive central nervous system hemorrhage in a 1.5-kg premature baby; and one due to sudden, unexplained death, just prior to hospital discharge (Table 2). During the interstage period, five out of the six surviving patients needed additional procedures (Table 3). All five patients underwent further interventions to enlarge the atrial septal defect. Stent implantation within the interatrial septum using premounted Palmaz-Genesis mm (Cordis) was required in two and a regular or static balloon septostomy in four patients (one patient had both types of procedures in separate settings) (Figs. 2A and 2B). Stenting the atrial septum was successfully performed after a radiofrequency transeptal approach in one patient (#14). In the other (#2), the stent migrated from the native atrial septal defect to the inferior vena cava, where it was implanted. Although all septostomies (static, dynamic, stenting) were effective in reducing the transatrial gradient to acceptable levels immediately after the procedure, all patients but one had some degree of obstruction within the interatrial septum before the phase II operation as determined by echocardiography. In two patients, the focus of the intervention was the ductal stent. In one (#13), there was a mm Hg retrograde gradient across the aortic arch associated with increasing tricuspid regurgitation and ventricular dysfunction. He underwent balloon dilation of the stents struts that were overriding the transverse arch, with gradient reduction to 5 mm Hg. Unfortunately, this patient died of an unrelated cause (bacterial meningitis) 3 months later. In the other patient (#6), there was a 30 mm Hg pressure gradient through the ductal stent requiring placement of an additional stent in the proximal uncovered portion of the duct and redilation of the distal part of the original stent (Figs. 3A and 3B). After the procedure, the gradient was eliminated. During the same procedure, the left pulmonary band was deemed loose (*20 mm Hg pressure gradient). This was considered a mild problem at the moment, not justifying a reoperation, and the patient was followed up without intervention. Currently, only one patient is still in the interstage period and four have approached the stage II operation (see below). Four patients have undergone the comprehensive stage II surgery at a mean age of 6.6 ± 0.5 months. There was significant difficulty removing the ductal stent in one patient (#14) using a peeling-off technique. Also in this patient, the stent within the atrial septum displayed significant fibrous ingrowth but offered no problem for complete atrial septectomy. In the others (#2, 3, 6), the segment of stented vessel was easily removed and replaced by an aortic homograft ranging in diameter from 14 to 21 mm. In those,

5 502 Pediatr Cardiol (2008) 29: Table 2 Technical aspects and mortality: Stage I procedures Patient No. BAS Stent diameter (mm) In-hospital mortality Cause of death 1 No 10 Yes LCO 2 No 10 No N/A 3 No 9 No N/A 4 Yes 10 Yes Infection 5 Yes 7 Yes CNS bleeding 6 No 9 No N/A 7 Yes 8 Yes LCO 8 No 10 Yes LCO 9 Yes 10 Yes LCO 10 No 8 No N/A 11 Yes 10 Yes Infection 12 No 9 Yes Infection 13 No 9 No N/A 14 No 10 No N/A 15 No 10 Yes Sudden death No 9.3 Yes LCO (66%) (± 1) (60%) (44%) BAS = balloon atrial septostomy; BE = balloon-expandable; SE = self-expandable; LCO = low cardiac output; N/A = not applicable; CNS = central nervous system Table 3 The interstage period Patient No. No. of additional procedures ASD enlargement/mode Stent intervention/type Current status 2 2 Yes BAS / stent No N/A Dead after stage II 3 1 Yes BAS No N/A Alive; after stage II 6 2 Yes BAS Yes Additional stent + original Dead after stage II stent redilation 10 None No N/A No N/A Alive; interstage 13 2 Yes BAS Yes Stent struts dilation Dead during interstage 14 1 Yes Stent No N/A Dead after stage II ASD enlargement Stent intervention Alive (83%) (33%) (33%) BAS = balloon atrial septostomy; N/A = not applicable the ascending aorta was anastomosed to the homograft in an end-to-side fashion and no evidence of poor coronary blood flow was observed in the recovery period. The pulmonary artery bands were removed and none of the vessels was judged to need plasty at the operation. In all four patients, the bands were made either of bovine pericardial or silicone strips. Shortly after surgery, all patients required interventions to the pulmonary arteries. In one case (#14), the left pulmonary artery was totally occluded and attempts at recanalizing the vessel in the catheterization laboratory on postoperative day 2 were unsuccessful. He subsequently died in the operating room due to disruption of the left pulmonary artery during intraoperative stent implantation. In another two patients (#2, #3), both pulmonary arteries were small and distorted. (Fig. 4). The patient (#2) had stents implanted in both pulmonary arteries on postoperative day 2 (Fig. 5). Subsequently, he required a right diaphragmatic plication and died 30 days after the phase II operation due to a massive hemothorax secondary to excessive oral anticoagulation. Another patient (#3) underwent balloon dilation of the right pulmonary artery and stent implantation in the left pulmonary artery on postoperative day 1. She was then taken back to the catheterization laboratory on postoperative day 6 due to unstable hemodynamics; balloon dilation of both pulmonary arteries and of the neo-aortic arch at the transition of the

6 Pediatr Cardiol (2008) 29: Fig. 2 A Interatrial stent implantation: A transesophageal echocardiographic view; B fluoroscopic view. RA = right atrium; LA = left atrium homograft to the descending aorta were successfully performed (Figs. 6A and B). This patient was discharged home and has been doing well 3 months after surgery. She has normal neurological and somatic development, arterial oxygen saturations in the low 80s, and satisfactory right ventricular function. The last patient (#6) had a standard BCPA done, instead of a modified hemi-fontan, and developed progressive cyanosis and superior vena cava syndrome shortly after extubation on postoperative day 1. On the following day, his condition worsened and he was taken to the catheterization laboratory where both pulmonary arteries and the BCPA had shown mild localized stenosis. Balloon angioplasty on both pulmonary arteries and at the BCPA was then successfully performed. However, the mean arterial pressure on the distal pulmonary arteries persisted quite high (*30 mm Hg) at the end of the Fig. 3 A Ductal tissue not covered by the stent and causing obstruction to the systemic outflow; B additional stent implanted in the arterial duct procedure, even with an adequate RV systolic function, suggesting the presence of increased pulmonary vascular resistance. Unfortunately, the patient died hours later due to unresponsive cyanosis and low cardiac output. Discussion The hybrid approach for the initial management of neonates with HLHS has been employed in the recent years in some centers, with evolving and encouraging results,

7 504 Pediatr Cardiol (2008) 29: Fig. 4 Pulmonary arteries hypoplastic and distorted after stage II operation Fig. 6 A Balloon redilation of a right pulmonary artery branch after the stage II operation; B balloon dilation of the neo-aortic arch after the stage II operation Fig. 5 Pulmonary arteries after stent implantation on both branches leading us to embark on this new management strategy. However, our results are worse than those that have been reported by other groups, possibly due to reasons discussed below. Even so, the outcomes presented here are better than those derived from our previous experiences with the traditional surgical procedures (unpublished data). In addition, we have learned some lessons that should be applied in subsequent cases. First and foremost, this series reflect the initial learning curve of two different and independent centers in the country. From the technical standpoint, phase I procedures were completed without major challenges. The majority of deaths in this series occurred during the recovery period of stage I procedures, with low cardiac output state being the commonest cause. This might have been related to the fact that ventricular dysfunction had been already present before the procedure in all four patients who exhibited this complication after the procedure. One of these patients had massive bleeding after removal of the pulmonary artery

8 Pediatr Cardiol (2008) 29: sheath in the operation room and immediately worsened the already compromised ventricular function; the other three had no such problems in the operation room but persisted with ventricular dysfunction despite optimal pharmacological treatment. The additional theoretical impairment of retrograde flow to the coronary arteries after ductal stent placement might have contributed to maintain a low cardiac output state in this group of patients. In this regard, whether the routine construction of a shunt between the main pulmonary artery and the innominate artery might improve coronary flow and decrease the risk of ventricular dysfunction is speculative [4]. Additionally, as we do not have prior large experience with postoperative care after the Norwood operation, the intensive care management might not have been as optimal as it should be. Infection was also a common cause of death in this series and reflects the need to improve our hospital infrastructure. Close surveillance during the interstage period with repeated echocardiographic assessments is mandatory in these patients. Progressive obstruction within the atrial septal defect and the ductal stent are common problems that need to be aggressively managed to avoid pulmonary hypertension due to overcirculation or venous congestion, retrograde coronary flow impairment, and its attendant right ventricular dysfunction. Therefore, it was not surprising that almost all patients underwent additional interventional procedures during the interstage period, with the majority having had atrial septal defect enlargement. The issue of which is the best method to provide an unobstructed flow through the atrial septum in patients with HLHS undergoing initial hybrid palliation remains to be determined with ongoing experience. Because such patients commonly exhibit a thick interatrial septum, it is unlikely that a standard balloon atrial septostomy or balloon dilation of the atrial septum (even using cutting balloons) will provide a long-lasting atrial septal defect. Also, blade septostomy might be very difficult and even dangerous in these patients because of the smal size of the left atrium. Stent implantation has been employed for this purpose in infants with good short-term results [8]. However, the observation that one of our patients showed progressive obstruction through the interatrial stent due to significant fibrous ingrowth was very disappointing. From the technical standpoint, it has not been clear whether the stenting the atrial septum through a radiofrequency-created new hole is better than stenting the native atrial septal defect. We feel that creating a new hole in a different portion of the septum offers more support for stent fixation, minimizing the risk of stent migration to the inferior vena cava, which could compromise the Fontan completion in the future. On the other hand, surgical atrial septectomy under a short run of cardiopulmonary bypass during phase I might be the best option for eliminating this problem, albeit going against the concept of providing the least invasive approach for the initial palliation of these patients. Attention should also be paid to the ductal stent during the interstage period. Uncovered ductal segments, neointimal proliferation, kinkings, and distortion of the adjacent transverse arch might occur after ductal stenting. However, it seems that these problems can be easily and effectively managed with additional stent implantation and/or balloon dilation, as seen in three patients in this series. Biodegradable stents might be a better option for ductal stenting in the future, as they might help the surgical team during the neo-arch construction at the stage II operation (see below). Although current technology is available, it is not suited for ductal stenting due to the too short period of degradation and the small stent diameter sizes. The stage II operation presented a great challenge to our surgical teams. Removal of the ductal stent was an issue in one patient (#14), probably related to the surgical technique. In the others, removal of the whole stented segment using a transversal incision and replacing it by a homograft was easy to accomplish, albeit associated with a mild residual gradient in one patient. Even so, we acknowledge that the presence of a nongrowing tissue in the systemic circulation might be problematic in the future. Our previous limited personal surgical experience with Norwood operations probably explains some of the difficulties encountered for aortic arch reconstruction. Distortions at the pulmonary arteries after the phase II operation were found in all in this series. This might be related to the material (bovine pericardial and silicone strips) and/or technique employed for banding. It has been proposed that the use of a Gore-tex tube for construction of the bands results in less scar tissue formation around the pulmonary arteries, minimizing the risk of distortions [7]. Also, the magnitude of flow restriction to the distal pulmonary artery might play a role on the functional status of the pulmonary arteries after the BCPA; one patient (#6) who has had a failed BCPA probably due to increased pulmonary vascular resistance, had a loose left pulmonary artery band for months and this might have contributed to the development of increased pulmonary vascular resistance on that lung. The use of percutaneous adjustable pulmonary artery bands might also help to minimize this problem, as the bands can be adjusted over time [5]. Moreover, as we move on trying to understand why the pulmonary arteries became so distorted and stenotic after the stage II operation, we speculate whether the additional suture lines on the right pulmonary artery, needed for the construction of the modified hemi-fontan, have an additional role on distorting that artery. This is stressed by the fact that the surgeons judged both pulmonary arteries of adequate size immediately before the BCPA construction. In fact, as for every standard BCPA, the right pulmonary artery is usually best visualized by the surgeon, leaving the

9 506 Pediatr Cardiol (2008) 29: left to be inspected only at its proximal portion. This is not a problem for a patient with a main pulmonary artery band, but it might be for one who has had both branches banded. This led us to ask if additional imaging for the left pulmonary artery, in the operating room, immediately after the BCPA construction, is needed. This would allow us to balloon dilate, implant a stent, or perform a pulmonary artery plasty at this very early moment. In conclusion, this experience reflects the initial results of the application of a hybrid approach for patients with HLHS in two cardiology centers in a developing country, as an alternative to the traditional surgical treatment. It is likely that other centers working under similar conditions will encounter similar problems. Even acknowledging that significant improvements in our infrastructure and intensive care management are necessary, we feel that refinements in the technique and materials for ductal stenting, pulmonary artery banding, and atrial septal defect enlargement are still required to achieve better and more reasonable outcomes. Also, the active participation of all involved in the care of these patients is required in order to develop real team work, which is crucial in this setting. References 1. Akintuerk H, Michel-Behnke I, Valeske K, et al. (2002) Stenting the arterial duct and banding of the pulmonary ateries: basis for combined Norwood stage I and II repair in hypoplastic left heart. Circulation 105: Bacha EA, Daves S, Hardin J, et al. (2006) Single-ventricle palliation for high-risk neonates: the emergence of an alternative hybrid stage I strategy. J Thorac Cardiovasc Surg 131(1): Bauer J, Thul J, Kramer U, et al. (2001) Heart transplantation in children and infants: short-term outcome and long-term followup. Pediatr Transplant 5: Caldarone CA, Benson LN, Holtby H, Van Arsdell GS (2005) Main pulmonary artery to innominate artery during hybrid palliation of hypoplastic left heart syndrome. J Thorac Cardiovasc Surg 130(4):e1 e2 5. Dias CA, Assad RS, Caneo LF, et al. (2002) Reversible pulmonary trunk banding. An experimental model for rapid pulmonary ventricular hypertrophy. J Thorac Cardiovasc Surg 124(5): Galantowicz M, Cheatham JP (2005) Lessons learned from the development of a new hybrid strategy for the management of hypoplastic left heart syndrome. Pediatr Cardiol 26: Gibbs JL, Wren C, Watterson KG, Hunter S, Hamilton JR (1993) Stenting of the arterial duct combined with banding of the pulmonary arteries and atrial septectomy or septostomy: a new approach to palliation of hypoplastic left heart syndrome. Br Heart J 69: Leonard GT, Justino H, Carlson KM (2006) Atrial septal stent implant: atrial septal defect creation in the management of complex congenital heart defects in infants. Congen Heart Dis 1: Mahle WT, Clancy RR, Moss E, Gerdes M, Jobes D, Wernovsky G (2000) Neurodevelopmental outcome and lifestyle assessment in school-aged and adolescent children with the hypoplastic left heart syndrome. Pediatrics 137: Mahle WT, Spray TL, Wernovsky G, Gaynor JW, Clark BJ (2000) Survival after reconstructive surgery for hypoplastic left heart syndrome: a 15-year experience from a single institution. Circulation 102(Suppl III):III-136 III McGuirk SP. Stickley J, Griselli M, et al. (2006) Risk assessment and early outcome following the Norwood procedure for hypoplastic left heart syndrome. Eur J Cardiothorac Surg 29(5): Stasik CN, Goldberg CS, Bove EL, Devaney EJ, Ohye RG (2006) Current outcomes and risk factors for the Norwood procedure. J Thorac Cardiovasc Surg 131(2):