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Ann Thorac Surg 2006;82:2214-2220
© 2006 The Society of Thoracic Surgeons
a Division of Cardiothoracic Surgery, Cincinnati Childrens Hospital Medical Center, Cincinnati, Ohio
b Department of Surgery, University of Cincinnati, Cincinnati, Ohio
Accepted for publication June 28, 2006.
* Address correspondence to Dr Pearl, Cincinnati Childrens Hospital Medical Center, Division of Cardiothoracic Surgery, 3333 Burnet Ave, ML 2004, Cincinnati, OH 45229 (Email: jeffrey.pearl{at}cchmc.org).
Presented at the Forty-second Annual Meeting of The Society of Thoracic Surgeons, Chicago, IL, Jan 30Feb 1, 2006.
| Abstract |
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METHODS: A single-institution retrospective chart review was made of all 25 neonates with pulmonary atresia with intact ventricular septum from 1999 to 2005.
RESULTS: Mean age at first intervention was 3.1 ± 2.2 days, mean weight 3.3 ± 0.5 kg. Right ventricular hypoplasia varied: 20% normal, 16% mild, 28% moderate, 28% moderately severe or severe, 8% not classified. Median tricuspid valve z-score was 2.3 ± 2.6. First intervention was catheter-based therapy in 56% (14 of 25), operative in 36% (9 of 25), and no therapy in 2. Technically adequate valvotomy was achieved in 79% (11 of 14). Serious catheter-related complications occurred in 3 of 14 (21%). Only 5 of 14 (36%) with catheter-based therapy weaned from prostaglandins without surgery. Of these, 2 required surgery for cyanosis at 1 and 3 months. Surgery after catheter-based therapy consisted of right ventricular outflow patch in 36% (4 of 11), systemic to pulmonary shunt in 64% (7 of 11). Median time between catheter-based therapy and surgery was 8.5 days (range, 1 to 89). Only 3 of the 23 treated patients avoided operation during infancy. There was 1 early and 1 late death after operation after initial catheter-based therapy, and 1 late death after primary surgery alone during a mean follow-up of 33 months (range, 1.5 to 79).
CONCLUSIONS: Balloon valvotomy alone for pulmonary atresia with intact ventricular septum rarely obviates the need for an additional source of pulmonary blood floweither shunt or ductal stenting.
| Introduction |
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Early experience with PA-IVS involved RVOT patch or surgical valvotomy with or without shunt placement for those with reasonable RV and TV size [13]. Shunt alone for patients with a severely hypoplastic right ventricle or RV-dependent coronary circulation is preferred. Advances in and experience with catheter-based therapies (CBT), including balloon valvotomy, have led to pulmonary balloon valvotomy as initial palliation for PA-IVS in many centers. Catheter-based therapy, however, is not without complication and may also impact subsequent surgical management. This study examines whether initial balloon valvotomy has a significant impact on the need for early surgical intervention to relieve cyanosis and, furthermore, whether that impacts overall morbidity and mortality.
| Patients and Methods |
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Catheterization
Femoral vein and artery were cannulated with either a 4F or 5F sheath. Before valvotomy, a right ventriculogram was made to evaluate for RV-dependent coronary circulation and coronary stenosis. The pulmonary valve was punctured with either a stiff wire or radiofrequency ablation depending on infundibular size and preference of the interventionalist. Valvotomy was achieved by serial balloon dilations until the annulus was fully opened, as evidenced by no residual waist on the balloon. Right ventricle hemodynamics were measured before and after the procedure. Technically successful balloon dilation was defined as ability to puncture the valve with a wire and inflate the balloon to the size of the valve annulus. The decision for surgery after initial CBT was determined by either unsuccessful balloon therapy (n = 3) or failure to wean from prostaglandins or persistent cyanosis off prostaglandins (n = 6). One patient required ligation of a patent ductus arteriosus after balloon secondary to overcirculation, then subsequently required shunt placement.
Primary Surgery
Patients with severely hypoplastic RV without a well-formed infundibulum and a small TV or evidence of RV-dependent coronary circulation, or both, underwent shunt alone and are excluded from further analysis. For the remaining primary surgical patients, defined as group 1, surgical intervention consisted of Blalock-Taussig shunt or central shunt combined with an RVOT patch (n = 4) or surgical valvotomy (n = 1).
Statistics
Comparisons between treatments were made by unpaired Students t test and analysis of variance with a p value of 0.05 or less considered significant. Analyses were conducted using Statview 4.01 software (Abacus Concepts, Berkeley, California). Data are reported as the median (range) or mean ± SD.
| Results |
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The early death occurred in a female infant with Turners syndrome with a TV size of 10 mm, z-score 0.6, and tripartite RV. Initially balloon valvotomy was technically successful on DOL 2. After 8 days, she remained prostaglandin dependent; therefore, she underwent a central shunt. Her hospital stay was further complicated by a balloon atrial septostomy, open atrial septostomy, atrial dysrhythmias, and increased right atrial pressures. On DOL 47 she died of cardiac arrest of unknown etiology.
The late death occurred in a 3.0 kg neonate with a 9-mm TV, z-score of 1.7, who underwent attempted CBT at 2 days of age. Catheterization punctured the valve, allowing antegrade pulmonary blood flow, but the valve was unable to be dilated completely. Eight days later, the patient underwent a RVOT patch and ductus arteriosus ligation. Postoperatively, the patient required extracorporeal membrane oxygenation support, Blalock-Taussig shunt placement, and diaphragm plication. He was weaned from extracorporeal membrane oxygenation and discharged home on hospital day 40; he died at home secondary to unclear etiology on DOL 55.
Three serious complications directly from catheterization occurred in 2 of 14 patients, minor complications in 4 of 14 patients (29%). One patient, who underwent primary CBT followed by surgery and received prostaglandins for 14 days, was readmitted for a pyloromyotomy.
One unsuccessful catheterization patient had two serious complications. These occurred in a 10-day-old, 2.3-kg patient with a 15-mm TV, a z-score of 1.6, and Ebstein anomaly. Initial unsuccessful antegrade attempts at balloon valvotomy were followed by a retrograde attempt, which created a perivalvar channel complicated by tamponade and cardiac arrest. In this patient, an interventricular hemorrhage, necrotizing enterocolitis, and ischemia of the left lower extremity developed, resulting in left above-knee amputation. Because the patient had inadequate RV decompression and was ductal dependent for pulmonary blood flow, she was taken to the operating room 14 days after her initial intervention for RVOT patch, RV muscle resection, right atrial reduction, TV repair with annuloplasty, and partial atrial septal defect closure with fenestration. The patient was discharged on DOL 115.
The third serious complication also occurred in a patient with an unsuccessful catheterization attempt. This patients was a 1-day-old, 3.5 kg neonate with a TV annular size of 11.3 mm (z-score 0.8) but poor leaflet excursion, causing functional stenosis. The catheter attempt was complicated by perforation of the common iliac vein, retroperitoneal hematoma, and perinephric hematoma. Six days later, the patient went to the operating room for a Blalock-Taussig shunt, closed valvotomy, and patent ductus arteriosus ligation. At age 2 months, the patient required a balloon valvotomy.
In patients undergoing initial catheterization, average intensive care unit and hospital stay were 19.4 ± 20.3 days and 27 ± 26.4 days, respectively, and duration of prostaglandin infusion 8 ± 6.7 days. Patients requiring surgery after CBT averaged 11.3 ± 6.2 days of prostaglandin infusion. The TV size was 11.8 ± 3 mm (range, 9 to 15 mm), and TV z-score was 0.3 ± 1.7 (1.7 to 1.6) in patients who failed catheterization, not significantly different from patients who had successful balloon dilatation but still required surgery (TV size 10.0 ± 2.3 mm, TV z-score 1.2 ± 1.4), or from patients who had successful balloon dilatation and did not require further intervention before discharge (TV size 12.4 ± 3.7 mm, TV z-score 0.4 ± 1.3; Table 1).
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| Comment |
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Cheatham and colleagues [4] reported that the presence of tripartite RV, well-formed infundibulum, tricuspid annulus of 11 mm or greater, membranous atretic pulmonary valve, and annulus of 7 mm or greater were likely to indicate two-ventricle candidates and perhaps good candidates for CBT [4]. This group is not dissimilar to that with critical pulmonary stenosis, which does respond well to balloon valvotomy. That represents, however, only a small percentage of patients presenting with PA-IVS. In one of the largest studies published on PA-IVS, the Congenital Heart Surgeons Society reported 171 neonates: 18% had a one-ventricle repair, 32% had a two-ventricle repair, and 50% had incompletely separated pulmonary and systemic circulation at 3 years. A smaller TV z-score correlated with not receiving a two-ventricle repair [5]. This current study once again demonstrates that the majority of neonates presenting with PA-IVS do not have a mildly hypoplastic RV with a good infundibulum and near-normal TV size category and, hence, extrapolating the results of pulmonary balloon valvotomy for critical pulmonary stenosis may not be appropriate.
It is important to note that, in this study, the mean TV z-score of patients undergoing initial CBT was near normal. Patients taken for initial balloon therapy had less severe right ventricular hypoplasia and had reasonable potential as two-ventricle candidates (Fig 3). Despite this, few were able to avoid surgery in infancy, usually because of persistent cyanosis. These values are similar to those reported by Cheatham and coworkers [4].
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Morbidity and Mortality
The overall early mortality rate was low with either treatment strategy. Surprisingly, the group undergoing initial surgical therapy did not have any early mortality. The primary CBT group followed by surgery had 1 early and 1 late death. Prior attempt at CBT did appear to increase subsequent surgical risk. This finding has been reported by others as well [7, 8]. Although the causes for mortality are multifactorial, one can not discount the longer hospital stay and longer duration of prostaglandin infusion as potential contributing factors.
It is noteworthy that the severe catheter complications occurred in patients who in retrospect may not have been ideal candidates for CBT. One patient had moderate Ebsteins anomaly, which would be considered a relative contraindication to CBT currently. The other patient had an acceptable TV annular size but restricted motion of the leaflets, resulting in significant functional TV stenosis. According to the literature, if CBT is to be considered, the TV should have a z-score above 2.0 (from our data, greater than 1.0) and no more than mild Ebstein malformation [912].
Our results of pulmonary balloon valvuloplasty are not dissimilar to other published studies. In a study looking at laser perforation and balloon dilation as primary therapy for PA-IVS, Cheung and coworkers [13] reported that 14 of 15 attempts were achieved. One patient died secondary to tamponade from perforation, for a total of 3 deaths. Nine patients required a second balloon valvotomy. Only 6 of the original 15 were successfully weaned from prostaglandins, and 5 required a shunt [13].
The mortality and morbidity associated with CBT cannot be discounted. They must be compared with both the success for surgical intervention and the morbidity and mortality of surgery. A report of primary surgical intervention for PA-IVS from Boston Childrens Hospital demonstrated a 98% early and late survival in 47 patients. They concluded that overly aggressive attempts to create two-ventricle hearts may not be warranted because the results with the one-and-a-half ventricle or single ventricles leading to Fontan are quite good [14]. While our own results, consistent with reports from other centers, for surgical shunt placement for all diagnoses does not reach the 98% survival rate mentioned above, it is noteworthy that, for the small group of primary shunt in this series, there was no mortality.
Length of Stay
Patients undergoing primary surgical repair trended toward shorter average intensive care unit and hospital stay. A shorter duration of prostaglandin infusion is inherent with this approach. While technical success rates may be high with CBT, prolonged prostaglandin infusion may be required in some patients [11]. Persistent cyanosis even with a technically adequate valvotomy may be due to RV diastolic dysfunction. In this regard, surgical RVOT patch alone may also be inadequate without an additional source of pulmonary blood flow. With time, RV function and forward output may improve, making the shunt no longer necessary. The morbidity and increased hospital stay associated with prolonged prostaglandin infusion, however, must be balanced with that of surgery and potentially earlier discharge. Ductal stenting is a potential alternative for patients undergoing CBT who are unable to wean from prostaglandins.
Potential Benefits of CBT
Although balloon pulmonary valvotomy alone may not prevent surgery for most patients, delaying surgical intervention, especially cardiopulmonary bypass, past the first month of life may be beneficial. Combining balloon pulmonary valvotomy and surgical shunt placement can avoid cardiopulmonary bypass. In our series, however, in which ductal stenting was not routine, balloon valvotomy alone rarely avoided the need for surgery in the first month of life. Closed valvotomy and shunt does not require CPB either, and even an RVOT patch can be performed without CPB or with a brief period of warm CPB. The combination of an RVOT procedure, either valvotomy or patch, combined with shunt placement allows one intervention and excellent early results.
This is a small, retrospective, single-institution study. While pulmonary balloon valvotomy can play an important role in the management of PA-IVS, our initial experience did not demonstrate significant avoidance of early surgery. As with surgical valvotomy or RVOT patch alone, the majority of patients required an additional source of pulmonary blood flow even after successful balloon valvotomy. Although CBT may avoid neonatal CPB, it, too, is not without morbidity or mortality. Expanded application and careful analysis of ductal stenting as the additional source of pulmonary blood flow may provide a reasonable alternative to surgical shunt placement. Determination of approach should be based on scrutiny of the morbidity, mortality, and short- and long-term outcomes of the different approaches. Certainly team consultation and development of management guidelines based on TV and RV size and morphology are necessary to optimize management of this complex lesion.
| Discussion |
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DR MCLEAN: Thats mostly based on whether or not there is RV-dependent coronary circulation, whether or not it was thought that the ventricle would handle decompression.
DR FORBESS: So it was essentially independent of tricuspid valve size?
DR MCLEAN: Correct.
DR CRAIG J. BAKER (Los Angeles, CA): I was wondering if you could just comment, is there any decision tree or when you start weaning the prostaglandins after the catheter therapy? Our cardiologists kind of pick a number sometime around a week, and we try coming down and see if it works. Are there any echocardiographic criteria that you use, or do you wait a certain amount of time and then try it?
DR MCLEAN: I dont believe so. I think they try and wean prostaglandins off actually rather rapidly, because those that were able to come off prostaglandins were within a day or two of the initial catheter therapy.
DR BAKER: So they try early and see if they can come off?
DR MCLEAN: Yes.
DR CARL L. BACKER (Chicago, IL): I congratulate you on a nice study. Are you planning to present this at a cardiology meeting?
DR MCLEAN: No.
DR BACKER: This is very important information. We should "review" this with our cardiology colleagues by publishing it in The Annals of Thoracic Surgery. I would like to relate our own experience from when catheter-based therapy became popular. We actually had some neonates who had not just one interventional catheterization before surgery, but two. When the second was unsuccessful, they finally went to surgery.
DR MCLEAN: Thank you.
DR K. S. MURTHY (Hyderabad, India): Your retrospective studies belong to the different category, the aggressive interventionalist who is doing the ductal stenting. Are you considering this ductal stenting also in this catheter-based interventions, and will it change the approach for this patients management?
DR MCLEAN: Yes, definitely, improvements in ductal stenting would change this, because the majority of patients needed to undergo surgery because they were unable to be weaned from prostaglandin. The caveat to that is that the majority of these patients may not be candidates for it because they have tortuous ducts and are reverse-oriented. But definitely, if there is improvement with that and theyre able to be discharged with an open ductus, that would change it.
DR CARLO F. MARCELLETTI (Palermo, Italy): What type of surgery do you perform when either a balloon fails or you cannot do a balloon? Do you do an open valvotomy and a shunt? Do you always associate a shunt, or not?
DR MCLEAN: If the balloon has failed and the right ventricle outflow tract is not open, we will do a ventricular outflow tract patch; and then if they need a shunt, to shunt with that. When the valvotomy was successful but not sufficient to wean patients from prostaglandins, they all got shunts. We did not have any that we initially expanded the right ventricular outflow tract.
| Acknowledgments |
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| References |
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R. L. Hannan, J. A. Zabinsky, R. M. Stanfill, R. A. Ventura, A. F. Rossi, D. G. Nykanen, E. M. Zahn, and R. P. Burke Midterm Results for Collaborative Treatment of Pulmonary Atresia With Intact Ventricular Septum Ann. Thorac. Surg., April 1, 2009; 87(4): 1227 - 1233. [Abstract] [Full Text] [PDF] |
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