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Eur J Cardiothorac Surg 2003;23:991-995
© 2003 Elsevier Science NL

Right ventricle to pulmonary artery conduit has a favorable impact on postoperative physiology after Stage I Norwood: preliminary results

Nemours Cardiac Center, Alfred I,. duPont Hospital for Children, 1600 Rockland Road, Wilmington, DE 19899, USA

Received 14 February 2003; received in revised form 27 February 2003; accepted 3 March 2003.

^* Corresponding author. Tel.: +1-302-651-6600; fax: +1-302-651-5345
e-mail: cpizarro{at}nemours.org

	Abstract

Top Abstract 1. Introduction 2. Material and methods 3. Results 4. Discussion References

 
Objective: Although significant progress has been made in the perioperative management of neonates with hypoplastic left heart syndrome (HLHS), early survival has plateaued. Moreover, low but important interstage mortality remains unsolved. With a systemic to pulmonary artery shunt, the combination of significant diastolic runoff into the pulmonary circulation, a large volume load on the single ventricle and precarious coronary perfusion result in a delicate physiologic state. In order to minimize these detrimental features, a right ventricle to pulmonary artery (RV to PA) conduit was used as the source of pulmonary blood flow in patients undergoing Stage I Norwood for HLHS. Methods: Prospective data collection in 15 consecutive patients who underwent Stage I Norwood with an RV to PA conduit. Results: Mean age at surgery was 2.5±2 days (range 1–8), mean weight was 2.9±0.3 kg (range 2.2–3.6) and mean gestational age was 37 weeks (range 35–40). Anatomic diagnosis was HLHS in all patients, aortic atresia was present in ten. Mean ascending aortic size was 2.9±0.9 mm (range 1.5–5). Two patients had moderate atrioventricular valve regurgitation and a genetic syndrome and/or congenital anomaly was present in five patients. Thirteen patients received a 5-mm polytetrafluoroethylene RV to PA conduit, and a 4-mm conduit was used in two. Mean circulatory arrest time was 55±6 min. Postoperatively, mean diastolic blood pressure at 1, 8 and 24 h were 47±7, 46±3 and 43±6 mmHg, respectively. Median time to extubation was 23 h (range 9–96) and was less than 24 h in ten patients. Median intensive care unit and hospital stay were 5 days (range 2–19) and 10 days (6–22), respectively. Early mortality was 1/15 (6%). At a mean follow-up of 10.8±3.4 months, 12 patients underwent stage II, and three patient have completed the Fontan. Conclusion: RV to PA conduit eliminated diastolic runoff into the pulmonary vascular bed resulting in a higher diastolic blood pressure. This physiology appears to be associated with a more stable postoperative course and improved hospital survival.

Key Words: Hypoplastic left heart syndrome • Norwood procedure • Congenital • Pediatric • Cardiac surgery • Single ventricle

	1. Introduction

Top Abstract 1. Introduction 2. Material and methods 3. Results 4. Discussion References

 
In the current era the surgical reconstruction for HLHS involves a three-staged operative strategy in which the initial procedure remains the most challenging. The physiology of the initial stage is characterized by the presence of significant diastolic runoff into the pulmonary vasculature away from coronary perfusion and a persistent volume load on the single ventricle. This physiology requires a delicate balance between the systemic and pulmonary resistances in order to provide the appropriate systemic perfusion to meet metabolic demands [1].

In order to modulate these detrimental features, the use of a right ventricle to pulmonary artery conduit (RV/PA) was revisited. This report describes the preliminary experience with the use of an RV to PA conduit in a group of neonates undergoing Stage I Norwood reconstruction for HLHS, while no other aspect of the surgical technique or perioperative management has been altered.

	2. Material and methods

Top Abstract 1. Introduction 2. Material and methods 3. Results 4. Discussion References

 
Prospective data collection was performed in all patients with HLHS who underwent the Stage I Norwood procedure with the use of an RV to PA conduit between June 2001 and May 2002. A total of 15 patients were identified and constitute the focus of this report. Data collection included demographic, preoperative, operative and postoperative variables, as well as follow-up information obtained during office visits.

The anatomic diagnosis of HLHS was based on two-dimensional echocardiography and required the presence of aortic valve atresia or hypoplasia, a hypoplastic or absent left ventricle and a ductus arteriosus-dependent systemic circulation with retrograde flow in the aortic arch.

The surgical procedure consisted of atrial septectomy, association of the aortic root with the proximal main pulmonary artery, augmentation of the ascending aorta and aortic arch with cryopreserved pulmonary homograft and placement of a right ventricle to pulmonary artery non-valved polytetrafluoroethylene tube graft. Three patients received a 4-mm conduit and the remaining 12 patients received a 5-mm conduit, depending on the patient's size. One end of this conduit was anastomosed to the central orifice of an oval-shaped pulmonary artery patch, which was used to patch-close the distal main pulmonary artery. The other end was anastomosed directly to an infundibulotomy in the right ventricular free wall (Fig. 1) .

View larger version (100K): [in this window] [in a new window]   Fig. 1. Stage I Norwood procedure with a right ventricle to pulmonary artery conduit as the source of pulmonary blood flow.

Data collection was performed following the guidelines of the local Institutional Review Board.

	3. Results

Top Abstract 1. Introduction 2. Material and methods 3. Results 4. Discussion References

 
The mean patient weight was 2.9±0.3 kg (range 2.2–3.6) and three patients were less than 2.5 kg. The mean age at the time of surgery was 2.5±2 days (range 1–8), and mean gestational age was 37 weeks (range 35–40). There was no appreciable weight change between birth weight and weight at surgery. Associated non-cardiac diagnosis or chromosomal anomaly was present in five patients and included meconium aspiration, necrotizing enterocolitis with Gram-negative sepsis, omphalocele, club feet and Turner's syndrome.

Distribution of anatomic subtypes was as follows: three had aortic atresia with mitral atresia, five had aortic atresia with mitral stenosis/hypoplasia and four had aortic stenosis with mitral stenosis/hypoplasia. Two patients had unbalanced complete common atrioventricular canal defect with aortic atresia and another neonate had double outlet right ventricle with mitral atresia. The size of the ascending aorta ranged between 1.5 and 5 mm with a median value of 2.9 mm.

Echocardiographic assessment demonstrated significant atrioventricular valve regurgitation in two patients, and flow acceleration across the foramen ovale with a velocity greater than 2 m/s in three patients. A circumflex coronary artery arising from the left pulmonary artery was present in one patient, an aberrant right subclavian artery in one, and a restrictive ductus arteriosus with an estimated peak gradient of 36 mmHg by echocardiography unchanged by prostaglandin infusion in another.

Measures to balance the systemic to pulmonary circulation ratio were utilized in nine patients preoperatively and included inspired carbon dioxide or nitrogen delivered via a mechanical ventilatory circuit or a tent when patients were breathing spontaneously. Median preoperative PaO₂ was 43 mmHg (range 25–56). The lowest value was in a patient with meconium aspiration treated with prolonged ventilatory support including supplemental oxygen in the perioperative period. A foreign patient who presented with multiorgan dysfunction and necrotizing enterocolitis underwent bilateral branch pulmonary artery banding as part of resuscitative effort prior to a successful Stage I Norwood reconstruction.

The mean duration of cardiopulmonary bypass and deep hypothermic circulatory arrest was 93±14 min (range 75–129) and 55±6 min (range 41–64), respectively. Three patients received extracorporeal circulatory support (ECCS) in the postoperative period. Support was initiated in the operating room in two patients. One of these patients had severe neoaortic valve insufficiency probably due to destabilization of the semilunar valvular apparatus by a right ventriculotomy adjacent to the valve annulus. Despite attempts to reestablish neoaortic valve competency, the patient could not be weaned from ECCS. The second patient was a 2.7-kg newborn product of a 37-week gestation with intrauterine growth retardation who had several episodes of ventricular fibrillation immediately after weaning from cardiopulmonary bypass, and was supported with ECCS for a few hours until ventricular irritability abated. The third patient was a 2.5-kg premature newborn in whom ECCS was initiated on the second postoperative day after apnea precipitated bradycardia and cardiovascular deterioration unresponsive to mechanical ventilation. This patient was successfully weaned off support within 24 h. The patient with severe neoartic regurgitation who could not be weaned from ECCS, accounted for an early mortality of 1/15 (6%).

The median intensive care unit and hospital stay were 5 days (range 2–19) and 10 days (range 6–22), respectively. Median duration of mechanical ventilatory support was 23 h (range 9–96), including ten patients who received mechanical ventilatory for less than 24 h. Postoperative maneuvers to balance Q_p/Q_s (inspired CO₂ or N₂) were used on a 2.6-kg patient product of a 35-week pregnancy who received a 5-mm conduit and exhibited signs of significant circulatory imbalance towards the pulmonary circulation. On the contrary, the remaining patients exhibited a tendency towards hypoxemia (mean PaO₂ values of 32±5.6, 33.2±4.6 and 33.6±2.4 at 8, 24 and 48 h after surgery, respectively). These PaO₂ values, although lower than the ones commonly observed in patients who received a modified Blalock–Taussig shunt, were appropriate to meet the metabolic requirements and were associated with signs of good peripheral perfusion, brisk urine output, and metabolic alkalosis.

The hemodynamic profile of these patients was characterized by a mean diastolic blood pressure above or equal to 40 mm with a pulse pressure ranging between 21 and 30 mmHg. (Fig. 2) .

View larger version (19K): [in this window] [in a new window]   Fig. 2. Graphic representation of the systolic and diastolic blood pressure curves in the perioperative period of patients undergoing Stage I Norwood with an RV to PA conduit. Time 0 is a preoperative determination. Disch, discharge.

View larger version (39K): [in this window] [in a new window]   Fig. 3. Pulsed Doppler spectral display of the waveforms generated when sampling the distal arch/proximal descending aorta after Stage I Norwood with an RV to PA conduit. Flow below the baseline represents antegrade flow and flow above the baseline represents retrograde flow. Electrocardiographic tracing provides relationship with the cardiac cycle. The picture demonstrates a minimal amount of flow reversal during diastole.

Hemodynamic parameters prior to hemifontan at a mean age of 4.2±1 months are shown in Table 1. The angiographic evaluation demonstrated appropriate growth and development of the pulmonary vascular tree and showed no evidence of ventricular wall motion abnormality or tricuspid regurgitation (Fig. 4) .

View larger version (75K): [in this window] [in a new window]   Fig. 4. AP and lateral angiographic images of the RV to PA conduit and pulmonary vascular architecture at the time of second stage palliation (hemifontan).

	4. Discussion

Top Abstract 1. Introduction 2. Material and methods 3. Results 4. Discussion References

 
The significant improvement in the perioperative management of patients with HLHS has led to a marked increase in hospital survival for Stage I Norwood in recent times [3–5]. This achievement has been the result of refinement in surgical technique and perfusion strategies, but most importantly the lessons learned from the management of these patients who have a unique and challenging physiology characterized by a delicate equilibrium between systemic, pulmonary and coronary circulation.

Although the initial surgical efforts included the use of a conduit between the right ventricle and the pulmonary artery to provide a controlled amount of pulmonary blood flow, it was largely due to technical constraints (large size, dacron conduit, etc.) and concerns of the effect of a ventriculotomy on right ventricular function, that this source of pulmonary blood flow was replaced by a systemic to pulmonary artery shunt (classical BT, modified BT, central shunt) [6]. Once this type of reconstruction was adopted and used routinely it became evident that having both circulations connected at the arterial level allowed for a significant runoff from the systemic into the pulmonary circulation during diastole, resulting in a further volume load on the single ventricle, a lower diastolic blood pressure and a change in the pattern of coronary perfusion [7,8].

This physiology has proven to be fragile and despite various innovative strategies utilized to aid in the perioperative management of these patients [9–12], the hospital mortality has remained significant and the issue of interim death before completing the second stage continues to be important [13,14].

Recently, improved outcomes after Stage I Norwood with a right ventricle to pulmonary artery conduit have been reported in surgical series which avoided a period of circulatory arrest and myocardial ischemia [15,16]. However, the real contribution of the RV/PA to these results could not be determined due to the simultaneous introduction of several technical modifications, including the use of selective cerebral and myocardial perfusion, arch reconstruction with autologous material, continuous low flow cardiopulmonary bypass, and so on.

As in most series, the patients in this cohort showed signs of a large Q_p/Q_s in the preoperative period, which prompted the use of ventilatory or pharmacologic intervention in order to balance both circulations [9–11]. However, in the postoperative period they demonstrated a remarkable circulatory stability, which was associated with virtually no interventions used to balance the systemic and pulmonary circulations, the presence of generous systemic perfusion and appropriate oxygen delivery. As a matter of fact, rather than using manipulations to reduce the amount of pulmonary blood flow, these patients usually received some oxygen supplementation in the initial postoperative period while pulmonary vascular resistance decreased and Q_p/Q_s increased.

From the hemodynamic standpoint, the elimination of diastolic runoff into the pulmonary circulation resulted in a diastolic blood pressure which was consistently over 40 mmHg throughout the entire postoperative period. This was associated with a normal systolic blood pressure and an appropriately narrower pulse pressure when compared to the similar measurements reported in patients who received a Blalock–Taussig shunt as part of a Norwood procedure [17]. Moreover, the elimination of the diastolic runoff also resulted in a lower Q_p/Q_s, and a reduction of the volume load on the single ventricle. All these conditions can have a favorable impact on coronary perfusion and be responsible at least in part for the clinically apparent beneficial effect of this surgical modification.

Echocardiographic assessment of the flow characteristics using time–velocity integrals obtained by Doppler pulsed wave interrogation in the distal aortic arch demonstrated the absence of flow reversal (from aorta into the pulmonary artery) during diastole. This observation is in contrast with the findings described by Rychik et al., in a group of patients after Stage I Norwood with a modified Blalock–Taussig shunt, where diastolic flow reversal provided a significant contribution towards effective pulmonary blood away from systemic and coronary perfusion [2]. This observation reaffirms the concept that the use of an RV to PA conduit can result in a more favorable physiology by virtue of reducing the volume load on the single ventricle and providing a higher diastolic pressure, which could have a favorable impact on coronary perfusion.

The progressive improvement in hospital survival after Stage I Norwood reconstruction has initiated a shifting of the attention to the issue of interim mortality. A variety of factors have been implicated, including the presence of residual lesions, arrhythmias and perhaps more importantly, the abnormal coronary flow patterns and diminished coronary reserve exhibited by patients with post Norwood physiology [14]. It seems reasonable to believe that the potential change in physiology and most importantly coronary perfusion introduced by use of the RV to PA conduit as part of Stage I Norwood reconstruction could eliminate the physiologic substrate for these events and contribute to a significant reduction in interim mortality.

In this series, the use of an RV to PA conduit provided the time necessary and the appropriate regulation of pulmonary blood flow during early infancy, allowing for pulmonary vascular maturation to occur in preparation for the second-stage palliation. Largely due to the development of progressive hypoxemia, the second stage was performed earlier, and the mean age of these patients at the time of hemifontan was generally at 4–5 months as opposed to the usual 6 months. However, even in the younger patients, the length of time after the initial palliation has been sufficient to allow for the necessary conditions to successfully perform the hemifontan followed by a Fontan completion at a usual age of 12–15 months.

These data suggest that the RV to PA conduit eliminates the diastolic runoff into the pulmonary circulation and results in a higher diastolic blood pressure which could have a favorable influence on coronary perfusion. These effects are associated with postoperative circulatory stability and it is possible they could potentially have a beneficial impact on the interim mortality.

4.1. Limitations
The main limitation of this review is the small number of patients. Although it would be useful to have a control group for comparison, the small number of the patients available who received an RV to PA conduit results in a significant lack of power for a meaningful statistical analysis.

	Acknowledgments

 
We thank Karen O'Neil, Lisa Elliot and Jane Vetter for their valuable assistance in the acquisition of the echocardiographic data.

	Footnotes

 
Presented at the 16th Annual Meeting of the European Association for Cardio-thoracic Surgery, Monte Carlo, Monaco, September 22–25, 2002.

	References

Top Abstract 1. Introduction 2. Material and methods 3. Results 4. Discussion References

 

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This Article Abstract Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Christian Pizarro Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Pizarro, C. Articles by Norwood, W. I. Search for Related Content PubMed PubMed Citation Articles by Pizarro, C. Articles by Norwood, W. I. Related Collections Congenital - acyanotic Congenital - cyanotic