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Ann Thorac Surg 2002;74:1616-1620
© 2002 The Society of Thoracic Surgeons

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Original article: cardiovascular

Preoperative pulmonary venous obstruction affects long-term outcome for survivors of total anomalous pulmonary venous connection repair

^a Division of Pediatric Cardiac Surgery, The Cardiac Center at The Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania, USA
^b Division of Biostatistics and Epidemiology, The Cardiac Center at The Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania, USA
^c Division of Cardiology, The Cardiac Center at The Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania, USA

* Address reprint requests to Dr Gaynor, Division of Cardiothoracic Surgery, The Children’s Hospital of Philadelphia, 34th St and Civic Center Blvd, Suite 8527, Philadelphia, PA, 19104, USA.
e-mail: gaynor{at}email.chop.edu

Presented at the Poster Session of the Thirty-eighth Annual Meeting of The Society of Thoracic Surgeons, Fort Lauderdale, FL, Jan 28–30, 2002.

	Abstract

Top Abstract Introduction Patients and methods Results Comment References

 
BACKGROUND: Early outcomes after repair of total anomalous pulmonary venous connection continue to improve; however, little information is available concerning long-term functional status and quality of life.

METHODS: One hundred patients underwent isolated total anomalous pulmonary venous connection repair at The Children’s Hospital of Philadelphia between 1983 and 2001. Medical records were reviewed and a standardized questionnaire was administered to guardians of survivors.

RESULTS: Median age at repair was 15.5 days (range, 1 to 563 days). Overall hospital mortality was 14%, decreasing from 19% before 1995 to 5% after 1995. At 15 years after repair, actuarial survival was 84% and freedom from late death or reintervention for hospital survivors was 85%. At a median follow-up of 5.9 years (range, 0 to 17.7 years) 64% of guardians described their child’s overall health as excellent, 27% good, 9% fair, and 0% poor. With regard to school performance, 40% of children were characterized as above average, 29% average, 4% below average, and 27% were in special education classes or had repeated grades. By multivariable logistic regression, the presence of associated chromosomal or noncardiac syndromes and pulmonary venous obstruction were found to be significant factors with regard to parental assessment of both overall health and school performance.

CONCLUSIONS: The majority of children who undergo isolated total anomalous pulmonary venous connection repair can expect an excellent long-term functional outcome. Factors present before operation, such as pulmonary venous obstruction and associated anomalies, can influence overall health and school performance in the long term.

	Introduction

Top Abstract Introduction Patients and methods Results Comment References

 
Total anomalous pulmonary venous connection (TAPVC) is a relatively rare congenital cardiac anomaly accounting for less than 3% of congenital heart disease [1, 2]. Hospital mortality after surgical repair has steadily decreased during the 50 years since Muller reported the first successful operation for this defect [3–7]. During the past decade, operative mortality for simple TAPVC, defined as TAPVC without associated complex anomalies, has been reported to be between 0% and 8% [3, 8–11]. With hospital survival typically more than 95%, the focus of outcomes analyses must now shift beyond the early postoperative period to include long-term survival, need for reintervention, and quality of life.

The goal of this study was to identify and evaluate factors that appear to influence long-term functional outcome and quality of life for patients after correction of simple TAPVC. Two readily recognizable measures of a child’s general level of function and quality of life were used as outcomes in this study, school performance and overall health. This type of information is useful both as a prognostic guide for families and patients and as a baseline for comparison of results as new techniques and interventions develop.

	Patients and methods

Top Abstract Introduction Patients and methods Results Comment References

 
Operation, echocardiography, and autopsy databases were used to identify all patients with simple TAPVC admitted to the Children’s Hospital of Philadelphia between January 1983 and January 2001. Patients with associated patent foramen ovale, atrial septal defect, aortic coarctation, and patent ductus arteriosus were included because these lesions are commonly present in TAPVC. Patients with other cardiac lesions were excluded. A total of 104 patients with simple TAPVC were identified. Of these patients, four died before surgical intervention, leaving 100 patients in the cohort.

Patients with significant respiratory compromise were intubated and resuscitated before surgical intervention if possible. Extracorporeal membrane oxygenation was only used in 2 patients who could not be stabilized before operation. The approach to the surgical repair varied during the time period in question. Before 1995, the most common technique involved elevation of the cardiac apex out of the pericardial well to the right as the left atrium was anastomosed to the pulmonary venous confluence posteriorly. More recently, the most commonly used technique has included trans-septal access to the left atrium with exposure of and anastomosis to the pulmonary venous confluence from the right. Deep hypothermic circulatory arrest was used in all of the patients in this series.

Besides the approach to the confluence, another technical variable has been the use of a patch to repair or augment the atrial septum. The patients can be divided into four groups depending on the type of patch used. These groups include no atrial septal patch (n = 31), patch closure of an atrial septal defect (n = 24), intra-atrial baffle of pulmonary venous blood to the left atrium (typically in patients with TAPVC to the coronary sinus, n = 21), or patch augmentation of the left atrial/pulmonary venous confluence anastomosis (n = 21). In 3 patients the operative note did not clarify the purpose of the patch. Patch augmentation of the anastomosis involves suturing the leftward portion of the pulmonary venous confluence to the left atrium. A patch (typically pericardium or homograft) is then sutured to the rightward portion of the opened confluence up to the left atrium/confluence suture line. The patch is then extended along the atriotomy to the atrial septum and is used to close the atrial septal defect. Such a patch is designed to augment the size of the anastomosis and increase left atrial volume.

Data were gathered through review of hospital and office records. An attempt was made to contact all of the guardians of hospital survivors for completion of a telephone questionnaire focusing on medical and functional status. This questionnaire was modified from one used previously to evaluate children with hypoplastic left heart syndrome [12] and following the Fontan operation [13]. The study protocol and questionnaire were approved by the Children’s Hospital of Philadelphia Institutional Review Board, Committee for Protection of Human Subjects.

Statistical analyses
Data were analyzed for the 100 children receiving TAPVC repair between January 1983 and January 2001. Data analysis involved two distinct phases. Phase I consisted of basic descriptive analysis (central tendency, variability, correlation, and calculation of an actuarial survival curve for the group as a whole). Phase II consisted of fitting two separate logistic regression models with preoperative, intraoperative, and postoperative covariates to predict a measure of quality of life. The two quality of life measures used in the logistic equations were school performance and overall health, as reported by parents/guardians. In each case, model selection was made based on comparisons of nested models using both Wald statistics and log-likelihood difference values. Hypothesis-wise error rates were held constant at the = 0.02 level across the two analyses using Tukey, Ciminera, and Heyse’s adjustment for correlated end points [14]. All data were analyzed using STATA 7.0 (STATA Corporation, College Station, TX).

	Results

Top Abstract Introduction Patients and methods Results Comment References

 
Preoperative and perioperative data
The median age of the patients at operation was 15.5 days (range, 0 to 563 days). Sixty-five patients were neonates (30 days old or less) at the time of operation. Median weight was 3.4 kg (range, 1.5 to 10.7 kg). Median gestational age was 40 weeks (range, 30 to 41 weeks) with 19 patients delivered at 36 weeks or less. There were 68 males and 32 females. Median total deep hypothermic circulatory arrest time for repair was 37 minutes (range, 8 to 121 minutes, pump times unavailable for 2 patients). Median time to extubation and hospital length of stay for hospital survivors were 2 days (range, 0 to 50 days) and 12 days (range, 4 to 55 days), respectively.

Supracardiac drainage was present in 43 patients, cardiac level drainage in 17, infracardiac in 31, and mixed in 9 patients. There was evidence of preoperative pulmonary venous obstruction (PVO) by chest radiography, echocardiography, or cardiac catheterization in 35 patients, as opposed to 62 patients who had no evidence of obstruction. For 3 patients there was not enough information in the medical record to determine the presence or absence of PVO. Patients with PVO were significantly younger at the time of repair than patients without PVO (17.9 ± 30.3 days versus 66.0 ± 108.8 days, p = 0.02). The incidence of PVO and hospital mortality for the anatomic groups and the group as a whole are shown in Table 1. There were 7 patients with associated syndromes or noncardiac anomalies in the cohort. These included VACTERL syndrome (2), cat’s eye syndrome (2), ring chromosome 22 (1), tracheal stenosis (1), and isolated imperforate anus (1).

Overall hospital mortality was 14% with a significant decrease during the course of the study from 19% (12 of 62 patients) before 1995 to 5% (2 of 38 patients) since 1995 (p = 0.05). The perioperative deaths in the current era occurred in a patient with PVO and mixed TAPVC who required emergent operation due to hemodynamic instability, and a second patient who had multiple noncardiac anomalies.

Long-term outcomes
Median follow-up for hospital survivors was 5.9 years (range, 0 to 17.7 years). Actuarial survival 17 years after operation was 84% (95% CI, 75%–90%; Fig 1). The majority of the deaths occurred during the initial hospitalization with two late deaths occurring at 2 and 3 months after the initial operation. Each of these deaths occurred after readmission and reoperation for PVO. A total of 13 patients required reintervention for pulmonary venous obstruction, with three of these procedures occurring during the initial hospitalization. All of the reinterventions for recurrent or persistent PVO took place within 4 years of the first operation.

View larger version (10K): [in this window] [in a new window]   Fig 1. Actuarial survival for patients with total anomalous pulmonary venous connection after repair.

Parental/guardian responses to questions concerning the patient’s overall health and school performance are summarized in Tables 2 and 3. Response options for the question concerning the patient’s overall health were as follows: excellent, good, fair, or poor. School performance was first broken down by type of school (regular versus special education) and the response options for the parents’ perception of academic performance were as follows: above average, average, or below average. The responses to these questions were grouped for analysis as follows: "Better" outcomes included "Excellent" or "Good" for overall health assessment and "Above average" or "Average" in regular classes for school performance. "Worse" outcomes were "Fair" or "Poor" for overall health and "Below average" in regular classes, patients who repeated grades, or "Special education classes" for school performance. At the time of the study, 45 of the patients were old enough to attend kindergarten or beyond. Children in preschool were not included in the school performance analysis.

	Comment

Top Abstract Introduction Patients and methods Results Comment References

Long-term survival for patients with simple TAPVC has been reported to be in the 70% to 88% range at 10 to 15 years [3, 8, 9], which is consistent with the 84% 17-year survival rate in the current report. The majority of deaths occur early after operation with a very low late attrition rate beyond the first 6 months (Fig 1). There were only two deaths after the initial hospitalization, each of which followed reoperation for recurrent PVO at about 2 months after the initial operation.

Given the low hospital and long-term mortality rate for patients with simple TAPVC, the focus for outcomes analysis in the current era must shift to long-term functional status and quality of life measures. The questionnaire administered to the parents or guardians of surviving patients was intended to be a first step in such an analysis. Although providing insight only in broad strokes, these data suggest that the majority of TAPVC repair survivors can expect excellent or good overall health (91%; see Table 2). School performance is also average to above average in regular classes for 69% of these patients.

The multivariate logistic regression analysis yielded several results that are difficult to explain. For example, it is unclear why the use of a patch to close the atrial septal defect would improve long-term school performance. Also, although statistically borderline with p = 0.05, male children had a significantly better parental assessment of school performance (see Table 5.) The impact of associated chromosomal/noncardiac anomalies upon long-term outcome could have been predicted. However, the long-term influence of prerepair pulmonary venous obstruction is somewhat surprising. Patients with obstructed TAPVC would certainly be expected to have increased perioperative morbidity and mortality, but these long-term effects in survivors of the repair were unexpected. It is unclear if this finding is the result of the earlier age of operation for patients with obstructed TAPVC, the fact that these patients can be hemodynamically unstable before and occasionally after operation, or if they have long-term anatomic or functional sequelae of pulmonary venous obstruction.

There are multiple limitations to this study. It is a retrospective single center study, and thus the results may not be applicable to the population as a whole. Also, the study population was treated over a long period of time during which surgical and medical treatment for children with congenital heart disease has undergone considerable change. Deep hypothermic circulatory arrest was used in all of these patients, therefore no comparison between deep hypothermic circulatory arrest and continuous cardiopulmonary bypass is possible. However, the length of deep hypothermic circulatory arrest was included in the multivariable analysis and was not found to impact the parental questionnaire results.

Another potential problem is the use of questionnaire responses as an outcome variable. Clearly the use of a parental questionnaire introduced the possibility of parental bias in their answers. For example, do the parents of children who were more seriously ill as infants evaluate their child’s health status differently later in life? In addition, although this questionnaire has been used in several studies of this type [12, 13], it is not a validated instrument with recognized norms. Because of these limitations and the need for more in-depth information about this patient population, we have initiated follow-up studies including neurodevelopmental evaluation, exercise testing, echocardiography, and Holter monitoring.

In conclusion, most patients who undergo repair of simple TAPVC can expect an excellent long-term outcome with regard to survival, freedom from reintervention, and overall functional ability. Of the 45 patients old enough to attend school, 28 (62%) have had no reintervention, have good or excellent overall health, and are reportedly performing at or above average in regular schools. The presence of PVO or associated chromosomal/noncardiac anomalies at presentation are predictive of worse long-term outcome with regard to overall health and school performance. Further studies are needed to more accurately assess these long-term outcome measures, so as to provide a clearer picture of the prognosis for these patients.

	References

Top Abstract Introduction Patients and methods Results Comment References

 

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