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J Thorac Cardiovasc Surg 2003;126:1367-1375
© 2003 The American Association for Thoracic Surgery
Surgery for congenital heart disease |
a Department of Pediatrics, Children’s Hospital of Wisconsin and Medical College of Wisconsin, Milwaukee, Wis, USA
b Division of Critical Care, Children’s Hospital of Wisconsin and Medical College of Wisconsin, Milwaukee, Wis, USA
c Division of Cardiology, Children’s Hospital of Wisconsin and Medical College of Wisconsin, Milwaukee, Wis, USA
d Department of Anesthesia, Children’s Hospital of Wisconsin and Medical College of Wisconsin, Milwaukee, Wis, USA
e Department of Surgery, Division of Cardiothoracic Surgery, Children’s Hospital of Wisconsin and Medical College of Wisconsin, Milwaukee, Wis, USA
f National Outcomes Center, Inc, Children’s Hospital of Wisconsin and Medical College of Wisconsin, Milwaukee, WisUSA
Read at the Eighty-second Annual Meeting of The American Association for Thoracic Surgery, Washington, DC, May 5-8, 2002.
Received for publication May 4, 2002; accepted for publication November 5, 2002.
* Address for reprints: Nancy S. Ghanayem, MD, Pediatric Critical Care, Children’s Hospital of Wisconsin, 9000 W Wisconsin Ave, Milwaukee, WI 53226, USA
nghanayem{at}aol.com
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Abstract |
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METHODS: Patients discharged before initiation of home surveillance (group A, n = 63) were compared with patients discharged with an infant scale and pulse oximeter (group B, n = 24). Parents maintained a daily log of weight and arterial oxygen saturation according to pulse oximetry and were instructed to contact their physician in case of an arterial oxygen saturation less than 70% according to pulse oximetry, an acute weight loss of more than 30 g in 24 hours, or failure to gain at least 20 g during a 3-day period.
RESULTS: Interstage mortality among infants surviving to discharge was 15.8% (n = 9/57) in group A and 0% (n = 0/24) in group B (P = .039). Surveillance criteria were breached for 13 of 24 group B patients: 12 patients with decreased arterial oxygen saturation according to pulse oximetry with or without poor weight gain and 1 patient with poor weight gain alone. These 13 patients underwent bidirectional superior cavopulmonary connection (stage 2 palliation) at an earlier age, 3.7 ± 1.1 months of age versus 5.2 ± 2.0 months for patients with an uncomplicated interstage course (P = .028). A growth curve was generated and showed reduced growth velocity between 4 and 5 months of age, with a plateau in growth beyond 5 months of age.
CONCLUSION: Daily home surveillance of arterial oxygen saturation according to pulse oximetry and weight selected patients at increased risk of interstage death, permitting timely intervention, primarily with early stage 2 palliation, and was associated with improved interstage survival. Diminished growth identified 4 to 5 months after the Norwood procedure brings into question the value of delaying stage 2 palliation beyond 5 months of age.
| See related editorial on page 1257.
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Despite improved early outcomes after stage 1 palliation (S1P) for hypoplastic left heart syndrome (HLHS), there continues to be a 10% to 15% incidence of late death before stage 2 palliation (S2P), creation of the bidirectional cavopulmonary connection.1,2 Recurrent, residual, or progressive lesions after S1P that have been linked to interstage death include restrictive atrial septal defect, neoaortic arch obstruction, systemic to pulmonary artery shunt stenosis, pulmonary artery distortion, coronary insufficiency, and atrioventricular valve insufficiency.3 Despite modifications of surgical technique and medical interventions, significant interim mortality continues to be associated with these anatomic lesions, which lead to progressive hypoxemia and impaired myocardial performance. In addition, hypovolemia and worsening hypoxemia related to common childhood illnesses are also associated with mortality among infants after S1P.4 To determine whether early identification of physiologic variances related to either anatomic changes or medical disease would reduce interstage mortality, we developed a home surveillance program for this at-risk population by enlisting parents to monitor daily arterial oxygen saturation via pulse oximetry (SpO2) and weight at home between S1P and S2P.
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Patients and methods |
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Discharge criteria
All patients were monitored with continuous pulse oximetry before discharge, with a target SpO2 of greater than 75% both awake and asleep. If necessary, patients were given oxygen to achieve this goal and discharged home with supplemental oxygen. If unable to take in a full diet (110-130 kcal/[kg · d]) orally, a gastrostomy tube was placed to provide caloric supplementation; 1 patient was discharged home with a nasogastric tube in place. Digoxin was prescribed for all patients. Afterload reduction with captopril and diuretic therapy with furosemide were initiated with weaning of inotropic support and used selectively for those patients demonstrating an elevated pulmonary to systemic flow ratio greater than 2.0 (
p/s > 2.0) in the early postoperative period. Medical therapy was adjusted on an outpatient basis at the discretion of the treating cardiologist. Cardiologists practicing at Children’s Hospital of Wisconsin followed up 93% of patients in group A and 67% of patients in group B.
Home management
Parents of group A patients received the usual discharge instructions, including directives to contact their children’s physician for respiratory or gastrointestinal illness, respiratory difficulties (tachypnea, accessory muscle use, nasal flaring, distress), and changes in perfusion (cool extremities, dusky or ashen appearance). Other than scheduled follow-up with the cardiologist every 2 to 4 weeks, no additional home surveillance was performed in group A. In addition to the usual discharge instructions, patients in group B were sent home with digital infant scales sensitive to changes of 10 g (Baby Checker Scale; Medela, McHenry, Ill) and pulse oximeters (Nellcor 200; Nellcor Puritan Bennett Inc, Pleasanton, Calif). Parents were asked to record weight and SpO2 in a daily log. Thresholds for parents to seek medical advice included resting SpO2 less than 70%, weight loss of 30 g or failure to gain 20 g of weight during 3 days. Patients who breached surveillance criteria underwent physician evaluation within 24 hours.
Data analysis
A prospective perioperative database containing demographic, surgical, hemodynamic, laboratory, and nutritional data for all patients who underwent S1P was reviewed. Patient-related variables assessed included age, sex, weights at birth and at S1P, diagnostic category, ascending aortic diameter, need for preoperative mechanical ventilation or inotropic support before S1P, and feeding method at time of hospital discharge. Operative variables included cardiopulmonary bypass time, deep hypothermic circulatory arrest time, total support time (defined as cardiopulmonary bypass time plus deep hypothermic circulatory arrest time), and shunt size. Hemodynamic variables compared during the first 48 postoperative hours included arterial and venous oxygen saturations, arteriovenous oxygen content difference, Qp/Qs, hemoglobin, mean blood pressure, central venous pressure, and anaerobic threshold. Variables assessed at the time of S2P included age, weight, and preoperative oxygen saturation. Outcome measures were operative survival after S1P and survival to S2P.
Interstage weights and SpO2 were obtained at periodic clinic visits for patients who were discharged home without home surveillance (group A, n = 57) and were compared with prospectively collected data from patients discharged with home monitoring equipment (group B, n = 24). Interstage SpO2 and weight were compared between the two groups. Fixed effects regression models were used to compare differences in SpO2 between groups A and B, as well as within group B for comparison of patients who did versus did not undergo any intervention as a result of home monitoring.
Statistical analysis was completed with SPSS advanced Models 9.0 (SPSS, Inc, Chicago, Ill) and STATA software (Stata Corporation, College Station, Tex). Descriptive statistics are presented as mean ± SD or percentage and count unless otherwise indicated. Median values are reported where appropriate. Variables were analyzed with 
2 statistics for categorical variables and analysis of variance techniques for continuous variables. Actuarial survival analysis was performed with Kaplan-Meier methods with log-rank comparison of cumulative survival by group. A polynomial regression equation was generated to plot patient growth between S1P and S2P (SPSS).
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p/s, hemoglobin, mean blood pressure, central venous pressure, and anaerobic threshold did not differ between groups during the first 48 hours.
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Mortality and interstage interventions in group b
All patients discharged with home monitoring of SpO2 and weight survived to S2P. Data obtained through home surveillance found 13 of 24 patients at increased risk: 12 patients with worsening hypoxemia from baseline and 1 patient with poor feeding and poor growth without worsening hypoxemia.
Outpatient detection of desaturation from baseline, as illustrated in Figure 2, was the predominant cause for intervention in group B and occurred 14 times in 12 of 24 home surveillance subjects. Parents of all but 3 patients sought medical attention at age less than 100 days (range 41-99 days, median 69 days) as the result of breach of home surveillance criteria. Two of 12 patients were hospitalized at 41 and 88 days of age with cough, congestion, hypoxemia, and need for frequent nasopharyngeal suctioning, consistent with bronchiolitis (1 had a positive culture for respiratory syncytial virus). These 2 patients each were seen a second time by the cardiologist for increased cyanosis without an associated respiratory illness, which led to a diagnostic cardiac catheterization and subsequent S2P at the ages of 113 and 108 days. Of the remaining 10 patients with increased interstage cyanosis detected at home, all underwent cardiac catheterization within 7 days of presentation (median age 78 days, range 59-182 days). Eight patients underwent catheterization at less than 100 days of age. Seven patients underwent subsequent S2P within 3 days of catheterization, and 1 patient underwent balloon angioplasty of the systemic to pulmonary artery shunt at 69 days of age with subsequent S2P performed at 129 days of age because of poor growth. Among the 7 patients who had subsequent S2P within 3 days of catheterization, 2 patients were found to have narrowing of the origin of the innominate artery from which the systemic to pulmonary shunt arose and 3 patients had distal shunt narrowing. Two patients without residual or recurrent anatomic lesions and without obstruction of shunt flow were thought to be favorable candidates for early S2P and underwent subsequent palliation at ages 79 and 90 days of age because of unacceptable cyanosis. Another of the 10 patients with increased interstage cyanosis was not found to have a recurrent or residual anatomic lesion at 59 days of age but was responsive to oxygen. He was thought to have medical lung disease and was discharged home with supplemental oxygen to undergo S2P 2.5 months later. One patient who had an elective preoperative diagnostic catheterization was admitted 2 weeks before scheduled S2P for earlier intervention related to worsening hypoxemia. In 8 of 24 home surveillance patients, interval hypoxemia led to S2P at less than 100 days of age.
Weight loss criteria were not violated by any patient, nor was any patient noted to have gastrointestinal losses. Of the 12 patients with increased interstage cyanosis, 4 (all less than 100 days old at 55, 69, 76, and 92 days) also had decreased feeding and poor weight gain reported. The patient who was seen at 55 days of age with poor feeding underwent gastrostomy tube placement for nutritional support. One patient with poor feeding and poor growth who did not have concomitant desaturations from baseline underwent cardiac catheterization followed by S2P with arch reconstruction at 113 days of age. No patient in group B underwent interventional catheterization for recurrent arch obstruction or surgical arch reconstruction before S2P; however, 6 of 24 patients (25%) underwent arch reconstruction at the time of S2P.
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Discussion |
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p/s combined with early detection of inadequate perfusion has resulted in improved S1P survival.5-7 Mortality before S2P remains high, and it is unclear whether improvements in S1P management have affected this high-risk interstage period.1,2 Even the optimal S1P patient continues to have physiologic risks, specifically parallel circulation, volume overload to the single ventricle, and cyanosis. The introduction of S2P as an intermediate step in single-ventricle palliation was a milestone that resulted in improved survival and allowed the practical application of the Fontan pathway to patients with HLHS. The benefits of S2P include relief of excess volume load and improvement in arterial saturation. The S2P procedure is well tolerated in this group of patients, with low mortality and short hospital stays. After S2P, patients are at substantially decreased risk of death, show improved growth, have better tolerance of concurrent illness, and are low-risk candidates for a completion Fontan operation.8-11 The optimal timing of S2P has not been determined. Although patients younger than 4 weeks tolerate S2P poorly, infants as young as 8 weeks with satisfactory preoperative hemodynamics have undergone S2P successfully.12 Earlier relief of volume overload and improvement in cyanosis may be especially important in the patient with a single right ventricle with limited ability to increase cardiac output and a tricuspid valve prone to development of insufficiency when exposed to prolonged volume or pressure overload. During the interstage period, patients are at risk for development of recurrent lesions, such as shunt stenosis or arch obstruction, that may be subtle and have important consequences. Jonas3 has previously described restrictive atrial septal defect, neoaortic arch obstruction, pulmonary artery distortion, and tricuspid valve insufficiency as anatomic abnormalities that may contribute to attrition after successful S1P. In a series of postmortem evaluations, Bartram and colleagues13 confirmed these findings, noting residual or recurrent lesions in 44 of 122 deaths (36%).
Any process that causes an imbalance in the oxygen supply-demand relationship will result in decreased arterial oxygen saturation in the patient with parallel circulation. An example of this is respiratory infection, which may result in pulmonary venous desaturation and worsening cyanosis because of impaired respiratory mechanics resulting from secretions, airway edema, and pneumonitis. This would support previous reports of viral illnesses as a cause of death in patients after S1P.4,13,14 In this study, of the 9 interstage deaths before implementing the home surveillance program, 4 (44%) patients had symptoms of a respiratory illness or dehydration. Gastroenteritis, a common illness in infants, may result in acute dehydration. In the patient with single-ventricle anatomy after S1P, the resulting increase in sympathetic tone will lead to further reduction of systemic flow as p/s increases. One additional patient who died after emergency S2P had evidence of a preoperative gastrointestinal pathologic condition that might have been identified through acute weight loss. Indeed, it was this patient who motivated us to develop a home surveillance program.
The primary goal of the home surveillance program was to develop a simple, reliable strategy to detect worsening systemic oxygenation and acute dehydration. Patients were discharged with infant scales, to identify dehydration through acute weight loss as well as growth failure through lack of weight gain, and with pulse oximeters, to identify worsening desaturation. These were devices with which the parents were already familiar and that were straightforward to use. The criteria for contacting a physician were determined by consensus as representing the physiologic limits beyond which survival would be in jeopardy and included SpO2 less than 70% and acute weight loss of 30 g or failure to gain 20 g during 3 days. Worsening desaturation was the most common indication for contacting a physician and occurred in 50% of the patients (12/24). No patient had acute dehydration or the clinical appearance of gastroenteritis, but isolated poor weight gain resulted in a gastrostomy tube in 1 patient and early S2P with repair of recurrent arch obstruction in another. Overall timing of S2P was earlier in group B than group A (5.6 ± 2.1 months vs 4.3 ± 1.6 months, P = .016). Of significant interest, though, is the intragroup analysis of group B patients. In 13 of 24 patients with evidence of increased vulnerability (54%), as detected by decreased SpO2 or slowed weight gain, progression to S2P occurred significantly earlier than in the remaining 11 patients in group B who had no detectable problems at home (3.7 ± 1.1 months vs 5.2 ± 2.0 months, P = .028). Additionally, the age at S2P of group B patients who did not breach surveillance criteria was not different from the age of S2P for group A patients (5.2 ± 2.0 months vs 5.6 ± 2.1 months).
A growth curve that included more than 1400 data points was developed for all the survivors to S2P. Unlike the growth curve of a healthy infant, who usually doubles the birth weight by 5 months of age, the patient with HLHS who has undergone S1P appears to have limited growth potential, with a plateau phase of weight gain after 150 days (Figure 3). This limited growth potential provides further evidence of the increased risk of the interstage period. Interestingly, although group A patients were older at the time of the S2P, they weighed the same as the patients in group B. The group A patients may have been subjected to a period of prolonged cyanosis and volume overload without the benefit or perhaps even the possibility of additional weight gain. This poor growth potential after 4 to 5 months calls into question the value of routinely delaying S2P beyond 5 months of age.
Previous studies have identified aortic atresia and smaller ascending aortic diameter as risk factors for late death after S1P.15-17 This anatomic subtype represents the most extreme form of HLHS, presumably with the lowest physiologic reserve. There were no differences in anatomic subtypes between groups, although there was a trend toward smaller ascending aortic size in the home surveillance group. Thus the survival advantage in group B was not based on favorable anatomy.
The data suggest that frequent monitoring of SpO2 and weight were useful in selecting patients at increased risk during the interstage period. In the patient with single-ventricle anatomy and parallel circulation, arterial saturation is a function, among other things, of hemoglobin, pulmonary venous saturation, p/s and total cardiac output. Therefore, diminished SpO2 may be particularly sensitive and will discriminate patients with anemia, respiratory infection, decreased total cardiac output, as well as decreased p/s. It is more difficult to develop a home surveillance strategy to reliably detect recurrent arch obstruction. Recurrent arch obstruction, however, might be identified through the more subtle symptom of slow weight gain.
The home surveillance program was associated with improved interstage survival; however, this study has important limitations. This study compared noncontemporary groups, although it should be noted that preoperative and postoperative management was uniform and postoperative hemodynamics did not differ between groups. Modification of surgical technique, more specifically implementation of continuous cerebral perfusion, resulted in shorter circulatory arrest time in the more recent cohort and may be implicated as a variable that improved interstage survival. We cannot rule out the impact of prenatal diagnosis on interstage survival. This study was neither blinded nor randomized, and therefore we cannot definitively conclude that lack of intervention in patients who breached surveillance criteria would have resulted in death. Overall progression along an institutional learning curve also probably contributed to improved outcomes in the home surveillance group. Despite these limitations, criteria selected to prompt examination were not different from those that would have triggered investigation in either group if identified at a routine clinic visit. In addition, it is important to note that the development of desaturation and poor weight gain occurred abruptly during the course of several days, a shorter interval than even reasonably spaced clinic visits.
The success of a home surveillance program requires dedicated family participation, as well as collaboration among multidisciplinary health care providers, which may be challenging for a given patient. Obtaining reliable data through appropriate equipment use is necessary for adequate assessment of physiologic variances and could prove to be a limitation. These patients frequently have a prolonged intensive care unit course, and parents become familiar with the concept and measurement of SpO2. We were impressed that parents came to identify episodes and recognize the significance of decreased saturation while their children were in the hospital. They appeared to be reassured when arterial saturations were in an acceptable range. The psychosocial impact of home SpO2 and weight monitoring was not formally evaluated. Given the risk of interstage death, however, we suspect that families have taken comfort in having objective data as an indicator of their children’s condition.
Improvements in S1P operative survival were, in our experience, the result of objective assessment of the patients’ circulatory status through improved physiologic monitoring. In this study improvement in interstage survival was also the result of continued collection of objective data through home surveillance to select patients at risk for interstage death. In this small series, we discriminated patients who were at increased risk for interstage death because of concurrent illness or residual or recurrent lesions. Early S2P was the primary strategy used to treat this subgroup of patients who appeared to be in jeopardy during the interstage period. It is clear from our experience and those of others that although S2P can be successfully accomplished in patients as young as 6 weeks, the postoperative course in these patients is prolonged relative to older patients. We must assume that the very young patient is at increased risk for death after S2P. Thus proposing early S2P for everyone, rather than reserving this therapy for those in whom the risk-benefit ratio is favorable, may in fact compound morbidity and mortality. Because growth appears to plateau between 4 and 5 months of age, we can conclude that S2P should be completed no later than 5 months of age. Continued data collection and follow-up are necessary to determine whether these short-term improvements will result in improved long-term outcomes. We continue to use home surveillance to discriminate patients at risk for interstage death.
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In this study, detection of home-monitored events, either decreased oxygen saturation or poor weight gain, led to earlier evaluation of these patients and frequently earlier performance of the superior cavopulmonary connection. There was no interstage mortality in the relatively small group of infants for whom home monitoring was used.
Dr Ghanayem, I have several questions. First, we recently reviewed risk factors for death after S1P in 158 infants during a 3.5-year period. In 46% of these infants there were additional risk factors, such as low birth weight (<2.5 kg); presence of additional cardiac defects at the time of S1P, such as interrupted aortic arch, moderate to severe tricuspid regurgitation, and anomalies of pulmonary or venous drainage; or presence of major extracardiac anomalies or genetic syndromes.
The interstage mortality in that series was approximately 10%, similar to your group A. In a multivariable analysis, the strongest predictors of interstage mortality were the presence of additional cardiac defects and the presence of an extracardiac anomaly or genetic syndrome. In your series, how many infants had either additional cardiac defects or extracardiac anomalies? And what was the impact of these associated anomalies on interstage mortality?
Second, it would appear that in group A several of the deaths occurred at a relatively early age, 50 to 70 days. These deaths appeared to occur before the age at which home monitoring events were detected in group B and before the age at which most group B patients underwent cavopulmonary connection. Do you attribute the lack of these early deaths and this decreased very early mortality to the home monitoring program, or does this simply represent increasing institutional experience with the management of these infants or some other change in management?
Third, an intriguing finding of your study is the decrease in growth velocity at approximately 5 months of age. This implies that there may be no advantage to delaying cavopulmonary connection even for children who are doing relatively well. What is your current policy for timing of cavopulmonary connection for children who have no home monitoring events and are otherwise doing well?
Fourth, the presence of aortic atresia, particularly with a diminutive ascending aorta, is a potential risk factor for coronary ischemia and interstage mortality. What was the incidence of very small ascending aorta, less than 2 mm, in your series? Was there any difference between group A and group B?
Finally, I was intrigued by the fact that many of your patients with recurrent arch obstruction undergo surgical intervention rather than balloon dilatation of the arch. What are your reasons for choosing surgical reconstruction rather than balloon dilatation?
I again congratulate you on your excellent study.
Dr Ghanayem. Thank you, Dr Gaynor, for your remarks. The presence of additional cardiac defects, such as preoperative moderate to severe tricuspid regurgitation, anomalous origin of the subclavian artery, or anomalies of pulmonary venous drainage, occurred in approximately 12% of the entire cohort, with no difference between the unmonitored and home-monitored groups. Among the interstage deaths in the earlier group, there was 1 patient with Turner syndrome; however, the incidence of extracardiac defects was not different between groups.
I do agree that an institutional learning curve has affected survival. Part of that learning curve is the appreciation that there are patients at risk for interstage death whom we have not been able to previously identify. In the unmonitored group, 4 of 9 interstage deaths occurred between 50 and 70 days, with the other 5 deaths occurring between 90 and 120 days. Although there was an absence of early deaths within the home-monitored group, there were several patients seen as a result of home-detected events for medical or surgical intervention between the ages of 50 and 70 days, with the earliest presentation prompting intervention occurring at 41 days. Also of significance was that the mean age for S2P was 111 days in patients with home-detected events versus 156 days in patients without home-detected events. What I believe we have learned, and successfully applied, is that there are changes in the health of these patients occurring during a short period of several days that would be missed by even frequent outpatient clinic evaluations and that recognition of desaturation, poor weight gain, and acute weight loss may better discriminate those patients at risk for interstage death, thus allowing earlier intervention.
The third question, regarding the timing of cavopulmonary connection, is one that we have contemplated as well. Because growth velocity appears to plateau at 4 to 5 months of age, why not advocate early S2P for everyone? It is clear from our experiences and those of others that although cavopulmonary connection can be successfully accomplished in patients as young as 6 weeks, the postoperative course these patients is prolonged relative to older patients. We must assume that the very young patient is at increased risk for mortality and morbidity after cavopulmonary connection, and this therapy thus should be reserved for those in whom the risk-benefit ratio is favorable (those who are struggling during the interstage period). Our current approach for timing of S2P depends on both growth and the degree of cyanosis. If growth velocity appears to plateau or the patient shows progressive hypoxemia with saturations consistently less than 70%, we will proceed with S2P. Alternatively, if growth is good and saturations remain acceptable, we will delay S2P until 5 to 6 months of age.
Although the presence of aortic atresia has previously been identified as a risk factor for coronary ischemia and interstage death, in our study it was not a risk factor for interstage death. The incidence of very small ascending aortas, less than or equal to 2 mm, was not different between groups: 40% for the group without home surveillance and 55% for the home-monitored group.
The incidence of recurrent arch obstruction was similar between groups. Only 3 patients in group A and 1 patient in group B had diagnoses made as a result of symptoms that were attributable to recurrent arch obstruction. Most of the patients with interventions for arch obstruction were symptom free, and the diagnosis was made during routine pre-S2P evaluation. When that has occurred, our approach has been to proceed with S2P and surgical arch revision, rather than performing balloon dilatation and delaying S2P until there is sufficient recovery from the interventional catheterization.
Dr Charles D. Fraser (Houston, Tex). I think that all of us who deal with patients with HLHS are forever frustrated by the interstage losses, and I think you are to be congratulated in tackling this problem. I wanted to ask you some practical questions about how you manage this program.
First, who pays for the monitors? Who pays for the pulse oximeters and the scales for the patients at home?
How do you handle non–English-speaking patients, of which many of us have a significant population?
Who collects the data and communicates with the families? I can imagine that having patients with HLHS at home with continuous pulse oximetry must generate a lot of telephone calls, so who handles all that?
Dr Ghanayem. Generally, health insurance companies have been willing to provide pulse oximeters and scales for these cyanotic infants at risk for poor growth. This has been facilitated by early correspondence to insurers, explaining the interstage risks and the importance of monitoring physiologic variances that are well-accepted indicators of infant health.
Some of the public aid patients have been more challenging in providing quality scales accurate to 10 g. We now have scales donated to our hospital, and these we loan to the families until the time of S2P.
Thus far we have had only one non–English-speaking family home with monitoring, and through a translator we were quite successful in training the family to monitor their child. In fact, this family successfully came to our attention during an acute illness as a result of home-detected desaturations.
Collection of the data has not been difficult, and parents have been keen about this project. We have developed fill-in-the-blank data sheets and have been pleasantly surprised and rewarded by the thoroughness with which the parents keep records. Parents are instructed to contact one of three nurses working in the cardiology clinic with concerns or if any aforementioned criteria are met.
Dr Thomas L. Spray (Philadelphia, Pa). This is an important area, but one concern that I have is that basically there weren’t any deaths in the second group. Therefore, how can you really say that your intervention changed the natural history of these patients? In the early group, concurrent illness and tricuspid regurgitation were the causes of death in most cases. But it’s not clear that those patients had events that would have been picked up by monitoring and could have been treated in a fashion that would have changed their outcome. I know you intervened early when you found problems, but does that really tell you that these patients would have gone on to interstage death?
Dr Ghanayem. Dr Spray, you correctly point out that patients were not randomly assigned to intervention versus no intervention if home-monitored events were detected. Therefore we cannot definitively say that the patients that breached home monitoring criteria benefited from intervention. However, the criteria used would likely prompt further investigation if spotted at a routine clinic visit, and we therefore suggest that home monitoring allowed early identification of well-accepted criteria for intervention. The patient with diminished arterial saturation, poor weight gain, or acute weight loss would be at greatly increased risk for death because of the limited physiologic reserve of this patient population.
Dr Frank A. Pigula (Pittsburgh, Pa). I congratulate you on a nice presentation. My question involves whether any anticoagulation regimen is used for these patients, either in the form of aspirin or low–molecular weight heparin. What you think the role of that may be in the interim management of these patients?
Dr Ghanayem. Dr Pigula, all patients receive aspirin unless they are more cyanotic than expected or we are concerned about shunt flow, in which case we will use subcutaneous low–molecular weight heparin injections for interstage anticoagulation.
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K. N. Fenton, K. Lessman, K. Glogowski, S. Fogg, and K. F. Duncan Cerebral Oxygen Saturation Does Not Normalize Until After Stage 2 Single Ventricle Palliation Ann. Thorac. Surg., April 1, 2007; 83(4): 1431 - 1436. [Abstract] [Full Text] [PDF]
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R. Delius Invited commentary Ann. Thorac. Surg., April 1, 2007; 83(4): 1436 - 1437. [Full Text] [PDF]
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O. Reinhartz, V. M. Reddy, E. Petrossian, M. MacDonald, J. J. Lamberti, S. J. Roth, G. E. Wright, S. B. Perry, S. Suleman, and F. L. Hanley Homograft Valved Right Ventricle to Pulmonary Artery Conduit as a Modification of the Norwood Procedure Circulation, July 4, 2006; 114(1_suppl): I-594 - I-599. [Abstract] [Full Text] [PDF]
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S. Tabbutt, T. E. Dominguez, C. Ravishankar, B. S. Marino, P. J. Gruber, G. Wernovsky, J. W. Gaynor, S. C. Nicolson, and T. L. Spray Outcomes After the Stage I Reconstruction Comparing the Right Ventricular to Pulmonary Artery Conduit With the Modified Blalock Taussig Shunt Ann. Thorac. Surg., November 1, 2005; 80(5): 1582 - 1591. [Abstract] [Full Text] [PDF]
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M. L. Skinner, L. A. Halstead, C. S. Rubinstein, A. M. Atz, D. Andrews, and S. M. Bradley Laryngopharyngeal dysfunction after the Norwood procedure J. Thorac. Cardiovasc. Surg., November 1, 2005; 130(5): 1293 - 1301. [Abstract] [Full Text] [PDF]
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C. L. Cua, R. R. Thiagarajan, R. Taeed, T. M. Hoffman, L. Lai, J. Hayes, P. C. Laussen, and T. F. Felt |
