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Right arrow Congenital - cyanotic

Ann Thorac Surg 2006;82:2214-2220
© 2006 The Society of Thoracic Surgeons


Original Articles: Cardiovascular

Pulmonary Atresia With Intact Ventricular Septum: Initial Management

Kelly M. McLean, MDa,b, Jeffrey M. Pearl, MDa,b,*

a Division of Cardiothoracic Surgery, Cincinnati Children’s 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 Children’s 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 30–Feb 1, 2006.


    Abstract
 Top
 Abstract
 Introduction
 Patients and Methods
 Results
 Comment
 Discussion
 Acknowledgments
 References
 
BACKGROUND: Outcomes for pulmonary atresia with intact ventricular septum are suboptimal, while initial management remains controversial. This study was undertaken to determine the effect of catheter-based therapy on the need for early surgical intervention.

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 flow—either shunt or ductal stenting.


    Introduction
 Top
 Abstract
 Introduction
 Patients and Methods
 Results
 Comment
 Discussion
 Acknowledgments
 References
 
The goals of early palliation of pulmonary atresia with intact ventricular septum (PA-IVS) include the relief of cyanosis and ductal dependence by providing a reliable source of pulmonary blood flow, and the relief of right ventricular outflow tract (RVOT) obstruction to encourage forward flow and growth of right-sided structures when feasible. How this is accomplished may vary depending on anatomy and physiology, as well as physician and institutional bias. Initial evaluation of the degree of right-sided hypoplasia is valuable in defining which patients are potentially two- or one-and-a-half–ventricle candidates and which are obviously single-ventricle candidates. Although criteria exist to predict eventual two-ventricle repair, these criteria have not confirmed the best approach to achieve this. In infants with significant sinusoids and right ventricle (RV)-dependent coronary circulation, RV decompression is not only unwarranted, but may also be detrimental. Once the decision is made to either relieve cyanosis or promote RV and tricuspid valve (TV) growth by RV decompression, the possible approaches vary.

Early experience with PA-IVS involved RVOT patch or surgical valvotomy with or without shunt placement for those with reasonable RV and TV size [1–3]. 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
 Top
 Abstract
 Introduction
 Patients and Methods
 Results
 Comment
 Discussion
 Acknowledgments
 References
 
This was a retrospective review of all patients admitted to our institution with a diagnosis of PA-IVS. The study was approved by the Institutional Review Board and was granted waiver of consent. Between March 1999 and June 2005, 25 neonates were admitted to our hospital with PA-IVS. The treatment plan was based on discussion among surgeons, cardiologists, and intensivists taking into account RV and TV size and morphology and status of the coronary circulation. Patients were not randomly assigned to treatment, and no defined institutional protocol was followed for stratification. The diagnosis of PA-IVS was based on two-dimensional transthoracic echocardiography. Of the initial 25 patients (intent to treat = 25), 2 died before receiving any intervention. Of the remaining 23 patients, 4 were identified clearly as single-ventricle candidates and therefore not candidates for CBT. Of the remaining 19 patients, the initial intervention was surgical in 5 (group 1) and CBT in 14 (group 2). Three patients initially treated with CBT were found to not have true pulmonary atresia, demonstrating minimal antegrade flow on catheterization. Since the intention was to treat PA-IVS by catheterization, these patients remained in the study. No patient who initially underwent CBT was discovered to have previously undiagnosed RV-dependent coronary circulation. Tricuspid valve size, function, and anatomy, and RV size and partition were determined by echocardiography. Tricuspid valve annulus size was measured in the apical four-chamber view during diastole. The z-score was calculated as follows: (measured diameter – mean normal diameter) / standard deviation of mean normal diameter based on a body surface area normograms.

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 Student’s 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
 Top
 Abstract
 Introduction
 Patients and Methods
 Results
 Comment
 Discussion
 Acknowledgments
 References
 
Twenty-five newborns with the diagnosis of PA-IVS were seen at our institution between March 1999 and June 2005. At the time of initial treatment, the mean age was 3.1 ± 2.2 days (range, 1 to 10), mean weight was 3.3 ± 0.5 kg (2.2 to 4 kg), mean TV size was 9.0 ± 3.8 mm (2.5 to 15 mm), and TV z-score was –2.1 ± 2.6 (–9.0 to 1.6). The RV size was normal in 5 of 23; hypoplasia was mild in 4, moderate in 6, moderately severe in 2, severe in 4, and undefined in 2 (Fig 1). Two patients died before receiving treatment. The first patient had a TV z-score of –4 and a moderately hypoplastic, tripartite RV. On day-of-life (DOL) 3, diagnostic catheterization demonstrated RV-dependent coronary flow, absent main pulmonary artery, and absent right coronary artery. Because of his anatomy, the patient was considered not to be a good candidate for either CBT or surgery. Transplantation was offered but declined. Support was withdrawn on DOL 10. The second preintervention death was a patient with a severely hypoplastic RV and a TV z-score of –3.7 who had numerous other congenital anomalies. Although scheduled for a Blalock-Taussig shunt, the patient had necrotizing enterocolitis, abdominal wall cellulitis, and sepsis. The patient died on DOL 16 from multisystem organ failure.


Figure 1
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Fig 1. Treatment and outcomes for all 25 patients admitted with a diagnosis of pulmonary atresia with intact ventricular septum (PA-IVS). (D/C = discharged.)

 
Primary Catheter-Based Therapy
Fourteen patients with a mean age of 2.7 ± 2.6 days (range, 1 to 10), weight 3.3 ± 0.6 kg (2.2 to 3.9 kg), TV size 11.2 ± 2.9 mm (6.5 to 15 mm), and TV z-score –0.7 ± 1.4 (–3.6 to 1.6) underwent primary CBT. Of these patients, 3 of 14 attempts (21%) failed, 6 of 14 (43%) were successful but still required surgery before discharge, and 5 of 14 (36%) could be discharged home without further intervention. Of the 5 patients discharged home without surgical intervention, 2 returned for RVOT patch at 1 month and 3 months secondary to cyanosis. Therefore, CBT failed to adequately relieve cyanosis in 11 of 14 patients. Of the 11 patients who underwent early surgery after initial CBT, there was 1 early death after surgery and 1 late death.

The early death occurred in a female infant with Turner’s 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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Table 1. Comparison of Duration of Prostaglandin Infusion, Intensive Care Unit Stay, and Hospital Stay Between Group 1 and Group 2
 
Primary Surgical Therapy
Patients who were potential candidates for CBT who underwent primary surgery had a TV size 9.3 ± 3.2 mm and TV z-score –1.8 ± 2.2 Mean age at time of surgery was 3.1 ± 0.5 days, with no early mortality in this group (Fig 2). Surgery consisted of shunt in all 5, with an RVOT patch in 4 and a pulmonary valvotomy in 1. All patients were discharged home without further intervention. In this group, average intensive care unit and hospital stay were 6.6 ± 3.3 days and 11.2 ± 2.6 days, respectively, and duration of prostaglandin infusion was 3.0 ± 1.4 days (Table 2). There was no significant morbidity in group 1. Additionally, there were no early deaths for the 4 patients undergoing shunt alone for severe PA-IVS.


Figure 2
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Fig 2. Treatment and outcomes of the 9 patients with pulmonary atresia with intact ventricular septum undergoing primary surgical intervention. (CBT = catheter-based therapy; RVOT = right ventricular outflow tract; 2-V = two-ventricle.)

 

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Table 2. Comparison of Duration of Prostaglandin Infusion, Intensive Care Unit Stay, and Hospital Stay Between Patients Undergoing Primary Catheter-Based Intervention Separated by Outcome
 

    Comment
 Top
 Abstract
 Introduction
 Patients and Methods
 Results
 Comment
 Discussion
 Acknowledgments
 References
 
The initial tricuspid valve z-score can identify patients who are likely eventual two-ventricle circulation rather than single-ventricle candidates. Those considered based on RV size and morphology and TV size to be candidates for eventual two-ventricle circulation benefit from RV decompression to encourage growth of the right-sided structures. Often, however, neither RVOT patch nor pulmonary valvotomy are adequate to relieve cyanosis in the newborn period secondary to initial RV hypoplasia and RV diastolic dysfunction. An additional source of pulmonary blood flow, therefore, may be required to relieve cyanosis. Until recently, a surgically placed systemic to pulmonary artery shunt was the only reliable option. The advent and increased use of ductal stents may provide a less invasive alternative.

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].


Figure 3
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Fig 3. Scatter plot of tricuspid valve z-scores of all patients separated by initial treatment type. (Cath = catheterization; Surg = surgery.)

 
The success of CBT obviously varies with patient selection, anatomy, and aggressiveness of individual interventionalists. If success is defined as ability to be weaned from prostaglandins and discharged without operative intervention, then only 5 of 14 in our series obtained this, with 2 soon presenting again for surgery. The innovative combination of balloon valvotomy and ductal stent placement may redefine CBT success, as both cardiopulmonary bypass and surgery are avoided. Alternatively, the morbidity of catheterization and the cumulative morbidity and mortality of catheterization followed by surgery may not outweigh the risk of early surgery with a short period of cardiopulmonary bypass and no myocardial ischemia time. Closed surgical valvotomy at the time of shunt is a surgical option that can avoid the need for cardiopulmonary bypass, although outcomes have been less favorable than with RVOT patch [6].

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 Ebstein’s 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 [9–12].

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 Children’s 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
 Top
 Abstract
 Introduction
 Patients and Methods
 Results
 Comment
 Discussion
 Acknowledgments
 References
 
DR JOSEPH M. FORBESS (Dallas, TX): I may have missed it. With regard to the decision tree for the surgery alone pathway and the decision to open up the RV outflow tract, this was dictated by what again?

DR MCLEAN: That’s 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 don’t 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 patient’s 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 they’re 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
 Top
 Abstract
 Introduction
 Patients and Methods
 Results
 Comment
 Discussion
 Acknowledgments
 References
 
Kelly M. McLean, MD, was supported by the Ruth L. Kirschstein National Service Research Award (5T32-GM-008478-13).


    References
 Top
 Abstract
 Introduction
 Patients and Methods
 Results
 Comment
 Discussion
 Acknowledgments
 References
 

  1. Neiderhäuser U, Bauer EP, von Segesser LK, et al. Pulmonary atresia with intact ventricular septum: results and predictive factors of surgical treatment Thorac Cardiovasc Surg 1992;40:130-132.[Medline]
  2. Lewis AB, Wells W, Lindesmith GG. Evaluation and surgical treatment of pulmonary atresia and intact ventricular septum in infancy Circulation 1983;67:1318-1323.[Abstract/Free Full Text]
  3. de Leval M, Bull C, Stark J, Anderson RH, Taylor JFN, Macartney FJ. Pulmonary atresia and intact ventricular septum: surgical management based on a revised classification Circulation 1982;66:272-280.[Abstract/Free Full Text]
  4. Cheatham JP, Coe JY, Kugler JD, Fletcher SE, Tower AJ. Successful transcatheter perforation of the atretic pulmonary valve membrane in a newborn using the new Coe radiofrequency end hole catheter Cathet Cardiovasc Diagn 1998;45:162-166.[Medline]
  5. Hanley FL, Sade RM, Blackstone EH, Kirklin JW, Freedom RM, Nanda NC. Outcomes in neonatal pulmonary atresia with intact ventricular septum J Thorac Cardiovasc Surg 1993;105:406-427.[Abstract]
  6. Mi YP, Chau AKT, Chiu CSW, Yung TC, Lun KS, Cheung YF. Evolution of the management approach for pulmonary atresia with intact ventricular septum Heart 2005;91:657-663.[Abstract/Free Full Text]
  7. Ovaert C, Qureshi SA, Rosenthal E, Baker EJ, Tynan M. Growth of the right ventricle after successful transcatheter pulmonary valvotomy in neonates and infants with pulmonary atresia and intact ventricular septum J Thorac Cardiovasc Surg 1998;115:1055-1062.[Abstract/Free Full Text]
  8. Alwi M, Geetha K, Bilkis AA, et al. Pulmonary atresia with intact ventricular septum percutaneous radiofrequency-assisted valvotomy and balloon dilation versus surgical valvotomy and Blalock Taussig shunt J Am Coll Cardiol 2000;35:468-476.[Abstract/Free Full Text]
  9. Agnoletti G, Piechaud JF, Bonhoeffer P, et al. Perforation of the atretic pulmonary valve J Am Coll Cardiol 2003;41:1399-1403.[Abstract/Free Full Text]
  10. Bichell DP. Evaluation and management of pulmonary atresia with intact ventricular septum Curr Opin Cardiol 1999;14:60-66.[Medline]
  11. Gibbs JL, Blackburn ME, Uzun O, Dickinson DF, Parsons JM, Chatrath RR. Laser valvotomy with balloon valvoplasty for pulmonary atresia with intact ventricular septum: five years’ experience Heart 1997;77:225-228.[Abstract/Free Full Text]
  12. Wang J, Wu M, Chang C, Chen Y, Lue H. Outcome of transcatheter valvotomy in patients with pulmonary atresia and intact ventricular septum Am J Cardiol 1999;84:1055-1060.[Medline]
  13. Cheung Y, Leung MP, Chau AKT. Usefulness of laser-assisted valvotomy with balloon valvoplasty for pulmonary valve atresia with intact ventricular septum Am J Cardiol 2002;90:438-442.[Medline]
  14. Jahangiri M, Zurakowski D, Bichell D, del Nido PJ, Jonas RA. Improved results with selective management in pulmonary atresia with intact ventricular septum J Thorac Cardiovasc Surg 1999;118:1046-1055.[Abstract/Free Full Text]



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