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Right arrow Congenital - cyanotic

Eur J Cardiothorac Surg 2006;29:659-665
© 2006 Elsevier Science NL

Risk factors for early death and reoperation following biventricular repair of pulmonary atresia with intact ventricular septum

Jonah Odim * , Hillel Laks, Thomas Tung

Division of Cardiothoracic Surgery, Department of Surgery, David Geffen School of Medicine at UCLA, 10833 Le Conte Avenue, CHS 62-226B Los Angeles, CA 90095, USA

Received 28 June 2005; received in revised form 6 January 2006; accepted 25 January 2006.

* Corresponding author. Tel.: +1 310 206 9520; fax: +1 310 825 7473. (Email: jodim{at}mednet.ucla.edu).


   Abstract
Top
 Abstract
1. Introduction
 2. Methods
 3. Results
 4. Discussion
 References
 
Objective: Since a functional right ventricle is desirable when repairing pulmonary atresia with intact ventricular septum, we sought to determine the factors that portend a successful biventricular repair in these children. Methods: A review of operative records at UCLA between 1982 and 2001 revealed 56 patients diagnosed with pulmonary atresia with intact ventricular septum that underwent either a partial (n = 26) or complete biventricular repair. Kaplan–Meier survival curves with log rank statistics were used to evaluate the influence of demographic, technical, and anatomic factors on survival and need for reoperation. Results: Five-year actuarial survival following biventricular repair was 91.5%. Non-Caucasian race (p = 0.011) and omission of palliative right ventricular outflow tract obstruction (RVOTO) relief (p = 0.042) were risk factors for early death. All patients with adequate follow-up required reoperation with median duration of 6.9 years. The most common cause of early reoperation (<1 year) was wound infection (35.3%) while pulmonary valve replacement (58.8%) constituted the majority of late reoperations. Risk factors for wound infection included female sex (p = 0.011) and use of a synthetic transannular patch (p = 0.085). The most significant risk factor for early pulmonary valve replacement was the use of a monocusp pericardial valve in the pulmonary annular position (p = 0.003). Conclusions: Excellent survival follows biventricular repair of pulmonary atresia with intact ventricular septum if RVOTO relief is performed in conjunction with initial palliation. Although most patients eventually require reoperation, avoidance of synthetic transannular patches and monocusp pericardial pulmonary valves may reduce the incidence of wound infection and delay need for pulmonary valve replacement.

Key Words: Cyanotic congenital heart disease • Biventricular repair


   1. Introduction
Top
 Abstract
 1. Introduction
2. Methods
 3. Results
 4. Discussion
 References
 
Pulmonary atresia with intact ventricular septum (PA-IVS) is a rare condition, comprising only 1–3% of all congenital heart defects [1]. Morphologic heterogeneity regarding right ventricular hypoplasia prevents any unique optimal surgical solution for all patients [2–6]. Rather, tailored intervention based on anatomic and physiologic presentation yields the best results [2–10]. Patients with severest disease have few options today except univentricular repair or transplantation as the right ventricle is too underdeveloped for re-establishment of reliable ventricular-arterial antegrade flow [10]. Additionally, many of these patients have a coronary circulation dependent on suprasystemic right ventricular pressures, limiting the ability to decompress this pulmonary pump [2,9]. With moderate degrees of hypoplasia, biventricular repair becomes an option [10]. A functional right ventricle in the circuit provides the theoretical advantage of preventing systemic venous hypertension and diminished forward flow with increases in pulmonary vascular resistance [10].

Although biventricular repair is usually possible with mild right ventricular hypoplasia, the viability of this repair in neonates with moderate hypoplasia depends on future growth of the ventricular cavity and tricuspid valve in response to re-establishing right ventricle to pulmonary artery continuity [10–14].

Since 1985, treatment of patients encountered at our institution with PA-IVS follows an algorithm dictated by severity of right ventricular hypoplasia, graded as mild, moderate, or severe [10,13]. In the absence of right ventricle-dependent coronary circulation (RVDCC), patients with mild or moderate hypoplasia are treated with intention of eventually achieving a biventricular repair. A pathway towards one-ventricle repair is selected for those with severe hypoplasia or RVDCC. With this standard approach, 56 patients over 20 years achieved either a partial or completed biventricular repair. To assess potential risk factors, we retrospectively evaluated the postoperative course of these patients to identify any characteristics that predicted either a favorable or adverse outcome.


   2. Methods
Top
 Abstract
 1. Introduction
 2. Methods
3. Results
 4. Discussion
 References
 
2.1 Patient population
Among 106 patients with PA-IVS treated at UCLA between 1982 and 2001, 56 achieved either a complete (30) or partial biventricular repair (26). A partial biventricular repair is a biventricular repair and a bidirectional Glenn, partially off-loading the right ventricle, with or without an adjustable atrial septal defect. For the other 50 patients, 23 underwent a single ventricle repair and 2 received heart transplants, 9 died before definitive repair, 8 were lost to follow-up and 8 are currently awaiting definitive repair. Half of the 56 patients undergoing biventricular repair were male, and 44 of 56 (78.6%) were of Caucasian descent. The route to biventricular physiology yielded two patient groups. For 6 of the 56 patients (10.7%), re-establishment of right ventricle to pulmonary artery continuity with a transannular patch (2), pulmonary valvotomy (3), or both (1) was conducted in conjunction with a systemic to pulmonary artery shunt in the neonatal period. Extensive muscle from the right ventricular infundibulum was resected in three of these children. One of these infants died perioperatively. In the remaining five, biventricular repair was ultimately achieved at a later date with closure of systemic to pulmonary artery shunts via coil embolization (2), spontaneous obliteration demonstrated by echocardiography (2), or surgical ligation (1). In this last patient, an atrial septal defect required surgical closure.

2.2 Dimensions of the right ventricle
The size of the tricuspid valve and right ventricular cavity in relation to the companion left ventricle classifies patients with PA-IVS into three groups at UCLA: mild, moderate, and severe right ventricular hypoplasia [10,13]. Patients with mild hypoplasia have right ventricle size greater than or equal to two-thirds of normal predicted size, which correlates to tripartite anatomy and tricuspid Z-scores between 0 and –2. Moderate hypoplasia indicates a right ventricle that is one-third to two-thirds of normal size, usually consistent with a pulmonary outflow tract of adequate maturity to perform effective pulmonary valvotomy and Z-scores between –2 and –4. A severely hypoplastic right ventricle is below one-third of expected size, with Z-score below –4.

These estimates of right ventricular dimensions are based on preoperative two-dimensional, transthoracic echocardiograms and were available for 97 of the 106 patients. In PA-IVS, the accompanying LV is usually of normal size and this is the visual yardstick used to assess RV hypoplasia. In 43 of 56 patients, tricuspid valve orifice diameters were quantified, and were used in conjunction with the patient's body surface area to determine tricuspid Z-scores from published nomograms [2]. Among 30 patients undergoing a completed biventricular repair, hypoplasia was classified as mild in 16 (53.3%), moderate in 13 (43.3%), and severe in 1 (3.3%). This last patient had a tricuspid Z-score of –2.5, which indicated a milder degree of hypoplasia than estimates from the ventricular cavity dimensions. Among 26 patients undergoing a partial biventricular repair, hypoplasia was mild in 4 (15.4%), moderate in 21 (80.8%), and severe in 1 (3.8%).

2.3 Right ventricle to coronary fistulae and right ventricular coronary dependence
Right ventricle to coronary fistulae was identified by review of coronary angiograms and ventriculograms available for all patients. Although a spectrum of fistulae covering of broad range of physiologic significance existed, for the purposes of statistical analysis, their presence was treated identically among all patients. Right ventricle-dependent coronary circulation was deemed present when a major portion left ventricle perfusion was supplied exclusively from the right ventricle. This circumstance occurred with proximal left coronary stenoses and concomitant right ventricle to left coronary fistulae, extensive right ventricle to coronary fistulae or myocardial perfusion scans indicating reversible defects. Fifteen of the 56 patients had right ventricle to coronary fistulae (26.7%) without evidence of RVDCC.

2.4 Procedures
The technical details of procedures achieving a biventricular repair at UCLA are described elsewhere [10,13]. The operative repair strategy remodels the right ventricle and its outflow tract through multiple coordinated surgical interventions. When possible, the right ventricle is enlarged by resection of excess trabeculations and muscular subpulmonic obstruction (n = 46, 82.1%). The tricuspid valve is made competent by annuloplasty, cordal repair, commissurotomy, or replacement (n = 13 for all repairs, 23.2%) if necessary. One of four strategies is utilized to reconstruct the right ventricular outflow tract: use of an appropriately sized aortic or pulmonary homograft for right ventricular outflow tract (RVOT) reconstruction with pericardial patch closure of the infundibulotomy if necessary (n = 9, 16.1%), use of an over-sized (relative to the child normal valve size) bioprosthetic valve into the outflow tract usually below the level of the true pulmonary annulus (n = 17, 30.3%) with pericardial or Gore-Tex patch closure of the transannular incision, a pericardial transannular patch with construction of a monocusp pericardial valve (n = 8, 14.3%) and a significant fraction of patients (35.7%) had no attempts pulmonary valve replacement or reconstruction. Briefly, we construct a pericardial monocusp valve using a ‘cusp’ fashioned from fresh autologous pericardium for the single leaflet with overlying glutaldehyde-treated autologous pericardium for transannular augmentation. At the time of biventricular repair, small atrial septal defects (ASD) are closed primarily though the majority of patients required creation of an adjustable ASD. Forty-five of 56 patients (80.4%) had creation of adjustable interatrial communications.

In patients with small ventricles or those with depressed right ventricular function, a bidirectional Glenn was added for partially unloading the right pump (n = 26, 46.4%). Patients with each ventricle ejecting into its respective great artery after closure of all systemic to pulmonary artery communications attain a complete biventricular repair (n = 30). Those patients fulfilling these conditions except for the presence of a cavopulmonary anastomosis, with or without adjustable ASD, have a partial biventricular repair (n = 26). The decision to add a bidirectional Glenn following biventricular repair and an adjustable ASD in children with greater than mild RV hypoplasia is made when, despite optimal adjustment of the interatrial communication, the right atrial pressures remain in the high teens and/or there is evidence of important desaturation (<80%) from right to left shunting across the atrial communication. The majority of patients required placement of a thin-walled Gore-tex sheet serving as a pericardial membrane substitute to reduce morbidity from later sternal reentry for re-operation.

2.5 Study design and statistical analysis
The outcome of biventricular repair was assessed by patient survival and freedom from reoperation. Potential risk factors that were analyzed included demographic traits (gender, race), measures of cardiac anatomy (degree of right ventricular hypoplasia, right ventricle to coronary sinusoids), methods of preoperative palliation (systemic to pulmonary artery shunting ± re-establishment of right ventricle to pulmonary arterial continuity), and surgical techniques used during biventricular repair. The influence of each of these variables was determined using Kaplan–Meier survival curves compared with log rank statistics. A Cox proportional hazards regression model was used for the multivariable analysis of significant variables [15]. Patient follow-up ranged from 1 day to 17.6 years, with a mean of 5.2 ± 0.6 years and a median of 3.5 years. Eleven patients out of 106 were lost to follow-up. In certain instances, {chi} 2 analysis was also performed by splitting patients into those with and without a risk factor and then constructing 2 x 2 contingency tables. Odds ratios were calculated from these tables by dividing the product A x D by B x C, where A and B represent patients positive for the studied outcome in each subgroup, and C and D are those negative for the outcome.

The postoperative hospital course was summarized by five continuous outcomes: days of intravenous inotropic support, days of intravenous vasodilator use, days until final extubation, days until removal of all thoracic drainage catheters, and total hospital days. The previously described potential risk factors were also studied for influence on these postoperative measures through segregation of patients into subgroups having and lacking each risk factor. The means for each subgroup were calculated, and the groups were compared using the Student's t-test with unequal variance. p-values less than 0.05 were considered significant.

All statistical analyses were performed using the SPSS software package for Windows (Chicago, IL, USA). All values are expressed as mean ± standard error of the mean (SEM). This study received approval from the UCLA institutional review board for patient-oriented outcomes research.


   3. Results
Top
 Abstract
 1. Introduction
 2. Methods
 3. Results
4. Discussion
 References
 
3.1 Mortality following biventricular repair is early and predicted by non-Caucasian race and failure to re-establish right ventricle to pulmonary artery continuity during palliation
The actuarial survival following biventricular repair was 91.5% with maximum follow-up of 17 years. Four of the 56 patients died within 25 days of the repair. There were no late deaths. Right heart failure was the most common cause of death (Table 1 ). By Kaplan–Meier analysis, predictors of early death included non-Caucasian race (log rank p = 0.011, Fig. 1A) and omission of palliative efforts to re-establish right ventricle to pulmonary artery continuity prior to definitive repair (log rank p = 0.042, Fig. 1B). Only 1 of 44 Caucasian patients (2.2%) died following repair compared to 3 of 12 patients (25%) of non-Caucasian background (odds ratio 14.3, p = 0.007). Patients without prior restoration of right ventricle to pulmonary artery continuity were at higher risk, with an odds ratio of 8.1 compared to those patients with this form of palliation (2.5% vs 17.6%, p = 0.044).


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  		Table 1. The profiles of the four patients who died following biventricular repair for PA-IVS
 

Figure 1
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  		Fig. 1. Early mortality from either partial or biventricular repair of pulmonary atresia with intact ventricular septum is predicted by both (A) non-Caucasian racial background (log rank p = 0.011) and (B) omission of a procedure to re-establish right ventricle to pulmonary artery continuity during prior palliation (log rank p = 0.042).

 
Neither birthweight and age at repair nor ratio of actual to predicted weight at date of surgery (predicted weight estimated from growth curves) influenced mortality. Other potential risk factors that had no effect on survival were the severity of right ventricular hypoplasia, the presence of right ventricle to coronary fistulae, omission of an adjustable interatrial communication or pulmonary valve replacement at the time of biventricular repair, and incorporation of a bidirectional Glenn.

Patient follow-up (N = 106) ranged from 1 day to 17.6 years, with a mean of 5.2 ± 0.6 years and a median of 3.5 years. No patient in the study group of 56 was lost to follow-up.

The 10-year actuarial survival for all PA-IVS patients was 86.3% over the same period. Twelve of the 16 deaths occurred within the first year of life, with 7, within the first month. Although all patients received palliative intervention, 9 of these 16 patients died before definitive repair. Eight of these nine died in hospital.

3.2 All PA-IVS patients require further surgery following biventricular repair
All patients undergoing either partial or complete biventricular repair required additional operations following definitive repair (median duration to reoperation was 6.9 years, Fig. 2 ). Within the first 2 years, the most common indication for reoperation was intervention for infectious complications in seven patients. Five of these seven patients required sternal wound debridements for wound infection within 42 days of repair. In four cases, wound infection required removal of in-dwelling synthetic snares adjusting the atrial septal defect and Gore-tex pericardial membrane substitutes. One other patient had chronic wound drainage eventually treated with soft tissue and superficial sternal debridement within the first year. The last patient developed an empyema from infection of a chronic pleural effusion. This patient was treated with thoracotomy and decortication.


Figure 2
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  		Fig. 2. Patients with pulmonary atresia with intact ventricular septum all require eventual reoperation after biventricular repair with median duration of 6.9 years. In the early postoperative period, infections are the major cause of reoperation (dotted line), whereas pulmonary valve replacements are the predominant late indication for reoperation (gray line).

 
The most common late indication for reoperation was replacement of the pulmonary valve. All patients with adequate follow-up needed a pulmonary valve replacement with a median duration of 8.1 years. Late pulmonary valve replacement defined the overall need for reoperation among these patients, 2 years or more after definitive repair (Fig. 2). However, pulmonary valve replacement was accompanied by other operations including reconstruction of the right ventricular outflow tract with myomectomy and transannular patch (n = 6), tricuspid valve replacement (n = 4), and takedown of prior cavopulmonary anastomoses (n = 3). In most instances, late pulmonary valve replacement was part of a larger effort to either relieve the right ventricle outflow tract of further obstruction or protect the RV from volume overload. No patient required later conversion to a single ventricle system following either partial or complete biventricular repair.

3.3 Use of a synthetic transannular patch and female gender place patients at risk for reoperation for infectious complications
By Kaplan–Meier analysis, female gender is the strongest predictor of reoperation for infectious complications after biventricular repair (log rank p = 0.011, Fig. 3A). All seven patients requiring reoperation for infection were female. Actuarial risk for reoperation for infection at 2 years for female patients was 42.9%. Compared to the males in the study, females with PA-IVS had a similar number of palliative procedures performed (1.5 vs 1.3, p = NS) in addition to comparable systemic oxygenation (79% vs 83%, p = NS), operative weight (11.0 kg vs 10.6 kg, p = NS), and age (32 months vs 29 months, p = NS). A greater fraction of females had mild right ventricular hypoplasia (50% vs 21.4%, p = 0.026), but tricuspid Z-scores were not significantly different between males and females (–2.7 vs oe2.4, p = NS). The distribution of synthetic transannular patches was similar between males (n = 6) and females (n = 4). RVOT reconstruction with Dacron or Gore-Tex as opposed to biologic material (pericardium or homograft) yielded more postoperative infections (log rank p = 0.085, Fig. 3B). Only 4 of 46 patients (8.6%) with transannular patches constructed from biological materials developed infectious complications necessitating surgery. A nearly identical number of infections (n = 3, 30%) occurred in the smaller group of patients receiving a synthetic transannular patch.


Figure 3
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  		Fig. 3. Infections requiring reoperation after partial or complete biventricular repair of pulmonary atresia with intact ventricular septum are related to (A) female gender (log rank p = 0.011), and with lesser significance (log rank p = 0.085) (B) use of a synthetic transannular patch to close the right ventricular outflow tract.

 
3.4 Pericardial monocusp valve reconstruction of the right ventricular outflow tract causes an earlier need for pulmonary valve replacement after biventricular repair
Among strategies for reconstruction of the right ventricular outflow tract, use of an over-sized bioprosthestic valve in the pulmonary position and insertion of a valved homograft had equivalent durability. However, the need for future pulmonary valve replacement was no different from patients judged to require no valvular reconstruction during biventricular repair. By contrast, fashioning a pericardial monocusp valve for pulmonary competence failed to maintain long-term competence for each patient with at least 5 years of follow-up (Fig. 4 ). Patients with a monocusp valve placed during biventricular repair required pulmonary valve replacement within 5 years. The use of a pericardial monocusp valve halved the median duration to pulmonary valve replacement seen with other forms of RVOT management (4.3 years vs 9.0 years, log rank p = 0.003).


Figure 4
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  		Fig. 4. Use of a pericardial monocusp valve to reconstruct the right ventricular outflow tract leads to early reoperation for pulmonary valve replacement when compared to other strategies (log rank p = 0.003).

 
3.5 The degree of right ventricular hypoplasia determines the acuity of care after biventricular repair
Patients with a mild grade of right ventricular hypoplasia experienced a more benign postoperative hospital course following biventricular repair (Table 2 ) than those with moderate or severe hypoplasia. Specifically, they required significantly less intravenous vasodilatory support (1.9 days vs 3.6 days, p = 0.04) and thoracic drainage (4.0 days vs 6.2 days, p = 0.03). In addition, they showed trends towards needing less intravenous inotropic support (3.7 days vs 5.7 days, p = 0.12) and mechanical ventilation (3.1 days vs 7.6 days, p = 0.10). These patients left the hospital in half the time of patients with moderate or severe hypoplasia (8.9 days vs 15.5 days, p = 0.03).


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  		Table 2. The influence of right ventricular hypoplasia and type of repair on postoperative intravenous inotrope and vasodilator need, requirements for mechanical ventilation, thoracic drainage, and length of hospital stay
 
The majority of patients (80%) with mild hypoplasia underwent complete biventricular repair. Segregating this study group into those undergoing partial from complete biventricular repair demonstrated similar differences in these postoperative outcomes (Table 2).

Three, 13 and 23 infants had RV sinusoids and coronary fistulae in the mild, moderate and severe RV hypoplasis groups, respectively. There were 15 infants with RVDCC, confined to the severe RV hypoplasia subset alone.

In the entire cohort of 106 patients with pulmonary atresia and intact ventricular septum, only five children presented with additional Ebstein's malformation of the tricuspid valve. In the subset, one patient achieved a complete biventricular repair and another patient successfully underwent partial biventricular repair. A third patient died following initial palliation and two remaining patients await further intervention following recent palliation.


   4. Discussion
Top
 Abstract
 1. Introduction
 2. Methods
 3. Results
 4. Discussion
References
 
Excellent results following biventricular repair are attainable in properly selected patients with PA/IVS. The 91.5% 5-year actuarial survival in this subset compares favorably to other series [2,6–10] and in no instance did biventricular repair require conversion to a one-ventricle physiology. Four deaths that occurred in these 56 patients were early within 25 days of repair, and were disproportionately distributed among those without prior relief of right ventricular outflow tract obstruction (RVOTO) and those of non-Caucasian racial background. Though the degree of right ventricular hypoplasia was not a risk factor for death in this study, mild hypoplasia did predict a more benign postoperative course regarding the need for inotropic drugs, vasodilators, mechanical ventilation, thoracic drainage, and length of hospitalization. The effect on mortality from relief of RVOTO probably relates to right ventricular growth and remodeling potential. Restoration of right ventricle to pulmonary artery continuity during palliation is an absolute requirement for future growth of a hypoplastic right ventricle [2]. In its absence, the dimensions of the right ventricle and tricuspid valve relative to body size actually decrease as the patient ages [2]. The hypertrophied, suprasystemic right ventricle in patients with PA-IVS exhibits histologic evidence of disordered microcirculation [14] and without relief of the outflow tract obstruction, these microvascular anomalies remain at the time of definitive repair.

Persistence of right ventricular sinusoids and a suprasystemic-pressured right ventricle may also contribute to abnormal postoperative hemodynamics. Therefore, patients without prior outflow tract relief may have stunted right ventricular growth and development in addition to micro- and macrocirculatory coronary anomalies. These factors may place these children at substantial risk of death at the time of biventricular repair.

While this retrospective study was not designed to address the 1.5 ventricle repair (partial biventricular repair), the rationale for this approach over a Fontan configuration when a complete biventricular repair was not possible was the physiologic benefit of lower systemic venous pressure (and corresponding potential salutatory effects on cerebral, coronary, hepatic and splanchnic circulatory beds), the maintenance of sinus rhythm, prevention of right heart blood stasis and subsequent thrombo-embolism, improved exercise tolerance and naturally pulsatile pulmonary blood flow.

Although non-Caucasian race is another risk factor for mortality in this series, explanations for this understudied finding are elusive. While the incidences of different congenital heart defects do vary among races when epidemiologic databases are studied [16,17] no data exists to support race-related difference in mortality for these defects achieving definitive repair in PA/IVS most often is not the patient's last surgical intervention. Within the first 2 years in this series, patients were reoperated for infectious complications. These complications related to sternal wound infections early postoperatively or shortly after discharge. In later years, pulmonary valve replacement for either insufficiency and/or residual or recurrent RVOTO predominated. Although the incidence of late reintervention after biventricular repair of PA-IVS is unknown, patients with Tetralogy of Fallot (TOF) and pulmonary atresia have a high probability of future reconstruction of the right ventricular outflow tract following definitive repair [15,18]. The actuarial probability of future RVOT intervention approaches 57% at 10 years [18]. Valveless reconstruction of the right ventricular outflow tract regularly leads to pulmonary regurgitation [19,20], which in experimental models and certain clinical settings is well tolerated. However, some patients progress to right ventricular dilatation with heart failure or arrhythmias [21–25].

The greater need for revision and reoperation when pulmonary atresia complicates TOF may stem from the poorly developed pulmonary arterial bed [17]. In PA-IVS, poor capacity and function of the hypoplastic right ventricle further compounds this problem. Reconstruction of the outflow tract in our population of PA-IVS patients with either an over-sized bioprosthetic value or homograft provides at least 7 years of clinically adequate pulmonary valve function. If a competent pulmonary valve is deemed necessary, our results favor either an over-sized bioprosthetic valve or a valved homograft over a pericardial monocusp valve. Monocusp valves had a high rate of incompetence, and in most instances, required replacement by 5 years postoperatively. Although the short-term competence of monocusp valves is accepted, outcome results fail to support an adequate long-term result. These pericardial monocusp valves prove useful in early surgical RVOT management permitting further growth and development of the right ventricle and later bridging to an adult-sized bioprosthetic device if RVOT reoperation is necessary in these growing children and adolescents.

Female gender and the use of a synthetic transannular patch at definitive repair put children with PA/IVS at greater risk for infection despite the additional finding that most of the entire cohort had an adjustable ASD with retained polyethylene snare and a pericardial membrane substitute of thin-walled Gore-Tex. As with the influence of race on mortality in this series, the gender predilection for sternal wound infections requires further study since an explanation is not readily apparent. Levels of oxygen saturation, severity of disease, and number of prior sternotomies did not differ between the sexes. The association of prosthetic patch material with infection is intriguing given the recognized higher rate of graft infections seen when prosthetic rather than autologous material is used for vascular reconstruction. Furthermore, since each patient in the study received a Gore-Tex pericardial substitute (in anticipation of future sternal re-entry) this finding is not simply attributable to the presence of a foreign body in the mediastinum. Indeed, Gore-Tex pericardial substitutes have not been associated with a higher incidence of sternal wound infections, and simply placing a foreign body into a tissue space does not by itself predispose to infection, as substantiated by experience with Marlex mesh implantation for hernia repairs. Although intuitively attractive as a risk factor, the use of a synthetic patch on the heart does not directly explain sternal infections unless it serves as a nidus for chronic or episodic bacteremia.

In conclusion, this retrospective study of biventricular repairs performed over a twenty-year period demonstrates that re-establishment of right ventricle to pulmonary artery continuity during palliation is critical for prevention of early mortality The repairs are nominally definitive, as patients will require future procedures, which in the short-term reflected infectious issues in our experience and in the long-term relate to RVOT reoperation and pulmonary valve replacement. A potential risk factor for infection among these children is use of a synthetic transannular patch during repair. This finding, along with the risk posed by female gender on infection and non-Caucasian race on mortality, will require further evaluation with an accumulated larger experience. Data regarding long-term ventricular function in these children with PA and IVS undergoing univentricular, partial ventricular or complete biventricular repairs is sparse though Mi [26] recently demonstrated impairment of RV and LV regional longitudinal myocardial function late after biventricular repair using tissue Doppler echocardiography when compared to an age-matched control group.


   References
Top
 Abstract
 1. Introduction
 2. Methods
 3. Results
 4. Discussion
 References
 

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Y. Hirata, J. M. Chen, J. M. Quaegebeur, W. E. Hellenbrand, and R. S. Mosca
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