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Ann Thorac Surg 2009;87:833-840. doi:10.1016/j.athoracsur.2008.12.027
© 2009 The Society of Thoracic Surgeons
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Original Articles: Pediatric Cardiac

Adult Congenital Heart Surgery: Adult or Pediatric Facility? Adult or Pediatric Surgeon?

Brian E. Kogon, MDa,*, Courtney Plattner, BAa, Traci Leong, PhDb, Paul M. Kirshbom, MDa, Kirk R. Kanter, MDa, Mike McConnell, MDc, Wendy Book, MDd

a Division of Cardiothoracic Surgery, Emory University School of Medicine, Children's Healthcare of Atlanta, Atlanta, Georgia
d Division of Cardiology, Emory University School of Medicine, Children's Healthcare of Atlanta, Atlanta, Georgia
b Rollins School of Public Health, Children's Healthcare of Atlanta, Atlanta, Georgia
c Sibley Cardiology, Children's Healthcare of Atlanta, Atlanta, Georgia

Accepted for publication December 1, 2008.

* Address correspondence to Dr Kogon, Emory University, Children's Healthcare of Atlanta, Egleston, 1405 Clifton Rd, Atlanta, GA 30322 (Email: Brian_kogon{at}emoryhealthcare.org).


   Abstract
Top
 Abstract
Introduction
 Patients and Methods
 Results
 Comment
 References
 
Background: One of the current controversies in the field of adult congenital heart disease is whether patients should be cared for at an adult or pediatric facility and by an adult or pediatric heart surgeon. After transitioning our program from the children's hospital to the adult hospital, we analyzed our experience with each system.

Methods: Between 2000 and 2007, 303 operations were performed on adults (age ≥ 18 years) with congenital heart disease. One hundred eighty-five operations were performed in an adult hospital and 118 in a pediatric hospital. Forty-six operations were performed by an adult heart surgeon and 257 by a congenital heart surgeon.

Results: Mean age, coexisting medical problems, and preoperative risk factors were higher in both the adult hospital group and adult surgeon group compared with the respective pediatric groups. Mortality was similar at the adult and pediatric hospitals (4.3% versus 5.1%), but was markedly higher in the adult surgeon group compared with the pediatric surgeon group (15.2% versus 2.7%; p = 0.0008). By multivariate analysis, risk factors for mortality included older age at the time of surgery (p = 0.028), surgery performed at a children's hospital (p = 0.013), and surgery performed by an adult heart surgeon (p = 0.0004).

Conclusions: Congenital heart surgery can be performed in adults with reasonable morbidity and mortality. Caring for an anticipated aging adult congenital population with increasingly numerous coexisting medical problems and risk factors is best facilitated in an adult hospital setting. Also, when surgery becomes necessary, these adult patients are best served by a congenital heart surgeon.


   Introduction
Top
 Abstract
 Introduction
Patients and Methods
 Results
 Comment
 References
 
The increasing number of adult patients with congenital heart disease and the better survival of patients with complex disease into adulthood have increased the need for specific programs that are able to provide comprehensive care to these patients [1]. One of the current controversies in the field of adult congenital heart disease is whether these patients should be cared for at an adult or pediatric hospital and by an adult or pediatric heart surgeon.

Clearly, there are advantages and disadvantages of each system. The obvious benefit of the pediatric system is the familiarity with the pathophysiology of the cardiac disease by all of the cardiac team members. The benefits of the adult system include patient comfort issues, along with ancillary and consult service issues. Caring for adults in a children's hospital often creates awkward or uncomfortable social situations. More importantly, however, as the adult congenital cardiac population ages, they likely will develop more complex medical histories requiring the proximity of multiple adult consult and ancillary services. In this scenario, adult programs are not always equipped to deal with the range and complexity of adults with congenital heart disease, whereas pediatric programs cannot be expected to manage the many acquired adult diseases in a pediatric medical environment [1]. It is important to overcome the obstacles and provide these patients the best care as they transition into early adulthood and beyond.

During the last 7 years, we have slowly transitioned our program from the children's hospital to the adult hospital. In an effort to continue to provide optimal care for this patient population in the future, we analyzed our experience with each system.


   Patients and Methods
Top
 Abstract
 Introduction
 Patients and Methods
Results
 Comment
 References
 
After obtaining institutional review board approval, we retrospectively reviewed the records of adult patients with congenital heart disease who underwent cardiothoracic surgery. Between 2000 and 2007, 303 operations were performed. Patient outcomes were analyzed based on hospital location and surgeon. A risk factor analysis was also performed for mortality.

Patient Factors
Overall, the mean age at the time of surgery was 31 ± 13 years. Regarding sex distribution, there were 134 men and 169 women. Regarding race, there were 210 Caucasians and 66 African Americans. Sixteen patients were of other racial backgrounds and 11 were of unknown descent. Initial congenital diagnoses were categorized as follows: complex (n = 147), congenital valve disease (n = 65), septal defects (n = 64), thoracic vascular (n = 8), arrhythmias or dysrhythmias (n = 10), and other (n = 9; Table 1). Coexisting medical problems were present in 136 of 303 patients (45%), and are completely listed in Table 2. The Society of Thoracic Surgeons database–defined preoperative risk factors for mortality were present in 68 of 303 patients (22%) and are listed in Table 3.


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  		Table 1 Initial Diagnoses
 

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  		Table 2 Coexisting Medical Problems
 

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  		Table 3 Preoperative Society of Thoracic Surgery Database Defined Risk Factors for Mortality
 
Operative Factors
One hundred eighty-five operations were performed at the adult hospital, and 118 were performed at the children's hospital. Forty-six operations were performed by an adult heart surgeon and 257 by a congenital heart surgeon. The surgeon was designated by the majority of his or her surgical practice.

Operations were categorized by primary procedure as follows: valve repair or replacement (n = 179), complex open procedure (n = 17), septal defect (n = 39), thoracic vascular (n = 8), pacemaker or automatic implantable cardioverter-defibrillator (n = 44), heart failure (n = 10), and other (n = 6; Table 4). Of the open heart operations, 190 of 249 (76%) were redo sternotomies.


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  		Table 4 Adult Congenital Heart Disease—Major Procedure
 
To facilitate comparison, each operation was assigned a score from 1 to 6 based on the risk-adjusted congenital heart surgery (RACH) system. This method was designed to adjust for baseline risk differences and allow meaningful comparisons of in-hospital mortality for patients undergoing surgery for congenital heart disease [2]. Pacemaker procedures were assigned a RACH score of 1. The RACH score distribution among hospital location and surgeon is shown in Table 5.


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  		Table 5 Risk-Adjusted Congenital Heart Surgery Score Distribution Between Hospital and Surgeon
 
Statistics
In making comparisons between groups, statistical analyses were performed using the Mann-Whitney U test for continuous variables and {chi}2 test for categorical variables. In evaluating potential risk factors for mortality, a multiple regression analysis was performed.


   Results
Top
 Abstract
 Introduction
 Patients and Methods
 Results
Comment
 References
 
Throughout the transition, both the number of cases performed annually increased (14 to 63) and the percentage of cases performed annually at the adult hospital gradually increased (7% to 78%).

Comparisons
Comparisons were made between those patients who underwent surgery at the adult hospital and those who underwent surgery at the pediatric hospital (Table 6). Mean age (37 versus 22 years; p = 0.0001), presence of coexisting medical problems (62% versus 18%; p < 0.0001), and presence of Society of Thoracic Surgeons preoperative risk factors (34% versus 4%; p < 0.0001) were all higher in the adult hospital group compared with the pediatric hospital group. However, there was no difference in the RACH scores, and the mortality was similar between hospitals.


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  		Table 6 Comparison Between the Pediatric and Adult Hospital and Pediatric and Adult Surgeon
 
Comparisons were also made between those patients who underwent surgery by an adult cardiac surgeon and a pediatric cardiac surgeon (Table 6). Mean age (42 versus 28 years; p = 0.0001), presence of coexisting medical problems (72% versus 40%; p < 0.0001), presence of Society of Thoracic Surgeons preoperative risk factors (39% versus 19%; p = 0.006), and RACH score (2.38 versus 2.06; p = 0.024) were all higher in the adult surgeon group compared with the pediatric surgeon group. There was also a marked increase in mortality in the adult surgeon group compared with the pediatric surgeon group (15.2% versus 2.7%; p = 0.0008).

Mortality
Overall mortality was 14 of 303 (4.6%). Details of the mortalities are provided in Table 7, and the relationship between RACH score and mortality is provided in Table 8. A multivariate risk factor analysis was performed for mortality (<30 days; Table 9). Risk factors for mortality included older age at the time of surgery (p = 0.028), surgery performed at a children's hospital (p = 0.013), and surgery performed by an adult heart surgeon (p = 0.0004). Mortality was unaffected by prior operations, the presence of Society of Thoracic Surgeons database–defined risk factors for mortality, and RACH score.


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  		Table 7 Mortality Summary
 

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  		Table 8 Mortality Related to Risk-Adjusted Congenital Heart Surgery Score and Surgeon
 

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  		Table 9 Risk Factor Analysis for Mortality
 

   Comment
Top
 Abstract
 Introduction
 Patients and Methods
 Results
 Comment
References
 
With improvements in technology, operative technique, and critical care medicine, more and more children with congenital heart disease are surviving into adulthood. It is now estimated that the number of adults with congenital heart disease in the United States is more than 800,000, and that, for the first time, this number equals the number of children with congenital heart disease [3, 4].

With the emergence of this ever-growing adult congenital population, controversy has arisen regarding its care. Should these patients be cared for at an adult or pediatric facility? Should these patients be cared for by an adult or pediatric cardiac surgeon?

Bethesda Conference
In 2001, the American College of Cardiology convened the 32nd Bethesda Conference to study the needs of these patients and to invite expert participants to recommend changes that will improve these patients' care and access to the health-care system. The conference made recommendations regarding organization of care, workforce description and educational requirements, access to care, and special needs of adult patients with congenital heart disease (noncardiac surgery, reproductive issues, exercise and rehabilitation, and psychosocial issues) [4]. Although these recommendations were made for establishing a program, consensus was not established regarding the optimal setting.

International and National Programs
Canada, with its national health-care system, has established regional adult centers for congenital heart disease care. Although their system has grown to include 15 programs throughout the country, the mean reported surgical volumes (6 programs) are 33.8 cases per year, with only one program performing more than 75 cases per year [5–7]. The United Kingdom has also established adult centers for the grown-up congenital heart patient. Only 3 of 18 cardiac surgical centers performed more than 30 cases per year, and only 2 specialized units were fully equipped and staffed [8]. On the other hand, a Hungarian program has established an adult congenital heart program within a tertiary care facility of pediatric cardiology [9]. A recent multicenter analysis from Europe also suggests that the great majority of adult congenital heart disease cases are still being performed in pediatric centers [10].

In the United States, the Adult Congenital Heart Association clinic directory shows a total number of 57 programs. Of these programs, the mean number of operations performed per year is 44, with only 8 programs performing more than 75 cases per year [7]. These programs vary as to whether they function within a pediatric hospital or adult hospital. Information from a consortium of 37 freestanding children's hospitals showed 707 admissions for cardiac surgery in adults between July 2005 and June 2007. Median age at surgery was 26 years (range, 21 to 86 years). Adult cardiac surgery performed as a proportion of overall cardiac surgery at these pediatric hospitals varied from 0% to 11.1%. The majority of procedures were related to pacemaker or defibrillator implantation and semilunar valve surgery, rather than complex intracardiac repair or palliation. Comorbid conditions likely to require other subspecialty care was present in greater than 30% of patients [11].

Our Transition
With the pediatric hospital being consistently at capacity and an adult congenital population growing in number and complexity, we have chosen to transition our adult congenital program to the adult hospital with surgery being performed by a congenital heart surgeon.

Our rationale for the transition to the adult hospital is the following:

1 Presence of an enlarging complex population. Approximately 85% of children with significant congenital heart problems survive to adolescence, and it is predicted that admissions to specialized adult congenital heart disease units will increase in number and complexity [3, 8]. The profile of this patient population will also change with time, not only because of advancing age, but also with improved survival of patients with complex anomalies [4].
2 Presence of concomitant adult cardiac disease. It has been suggested that a dedicated unit is ideally located, and administered, within the directorate of adult cardiology. Acquired heart disease will occur more frequently as this population ages. Development of coronary arterial disease, or systemic hypertension, can cause new or worsening symptoms requiring treatment of both the congenital and acquired disorders. The combined congenital and acquired problems may require input from adult cardiologists [8].
3 Presence of comorbidities. A high proportion of patients have important noncardiac medical and surgical problems requiring specialist advice. The presence of other subspecialties on the same site, therefore, is highly desirable. The most common needs appear for noncardiac surgery, endocrine, rheumatologic, hepatologic, neurologic, orthopedic, and renal expertise [8, 12].

Our rationale for having the surgeries performed by a pediatric heart surgeon include the following:

1 Complexity of surgery. The majority of patients will have had one or more palliative or corrective operations in childhood. Few are curative, and most require long-term follow up, and often further surgery. It is suggested that surgeons who operate on children with similar conditions should perform most adult congenital heart surgery. A study of national practice patterns for management of adults with congenital heart disease has shown that in-hospital death rates for patients operated on by pediatric heart surgeons were lower than death rates for those operated on by nonpediatric heart surgeons [13]. Unique surgical problems exist in this group of patients, and surgeons who carry out operations on complex congenital heart disease need to be an integral part of these specialized units [8, 14].
2 Reoperative surgery. In one US center following more than 1,800 patients, 1,243 of whom had cardiac surgery, almost 50% patients had two or more operations and 23% had three or more operations. The need for reoperation again emphasizes the need for special surgical expertise in congenital heart disease [4].

Our Current Program
The core structure of our adult congenital heart disease program consists of three pediatric heart surgeons (one primarily) and two cardiologists (one pediatric cardiologist and one adult cardiologist). With the exception of the pediatric cardiologist, all physician members of the team have admitting privileges at the adult hospital.

For surgical patients, intraoperative transesophageal echocardiography is provided by the anesthesiologists. Postoperatively, the cardiac intensive care is provided jointly by the surgical team and an anesthesiology-based critical care team. The ward care is provided by the cardiac surgical service. For nonsurgical patients requiring admission, ward and intensive care is provided by the adult congenital cardiology service.

We have established educational programs for the residents, fellows, nurses, and ancillary services to improve familiarity with the pathophysiology of the adult congenital heart disease patients. After overcoming this issue, we are now realizing all of the benefits of having these patients at the adult facility. Although there are no formal practice patterns that dictate the flow of patients, territorial issues do not seem to be problematic. As our program grows and gains more visibility within the hospital, the adult cardiologists are more consistently referring their adult congenital patients through the system, and the adult cardiac surgeons are more consistently deferring surgery to their congenital surgical partners. Referrals from outside cardiologists and cardiac surgeons are also steadily increasing. Consultant services are becoming increasingly interested and involved in these unique patients as well. Almost exclusively, our current practice is to perform all of the operations, including those that involve complex single-ventricle physiology, at the adult hospital with a congenital cardiac surgeon.

Conclusions
The interest in adult congenital heart disease is increasing along with the patient population. Although recommendations have been made for establishing an adult congenital heart disease program, each individual institution varies, and programs have developed at both adult and pediatric hospitals with both adult and pediatric surgeons. However, as this population grows, it is anticipated that it will increase in age and complexity. Caring for an anticipated aging adult congenital heart disease population with increasingly numerous coexisting medical problems and risk factors is best facilitated in an adult hospital setting. Also, when surgery becomes necessary, these adult patients are best served by a congenital heart surgeon.


   References
Top
 Abstract
 Introduction
 Patients and Methods
 Results
 Comment
 References
 

  1. Calabro R, Sarubbi B, D'Alto M, Russo MG. Organization of care for adults with congenital heart disease G Ital Cardiol 2006;7:336-343.
  2. Jenkins K, Gauvreau K, Newburger J, Spray T, Moller J, Iezzoni L. Consensus-based method for risk adjustment for surgery for congenital heart disease J Thorac Cardiovasc Surg 2002;123:110-118.[Abstract/Free Full Text]
  3. Gatzoulis M, Webb G, Daubeny P. Diagnosis and management of adult congenital heart diseaseEdinburgh: Churchill Livingstone; 2003.
  4. Webb G, Williams R. 32nd Bethesda Conference: care of the adult with congenital heart disease J Am Coll Cardiol 2001;37:1161-1198.
  5. Canadian Adult Congenital Heart Networkhttp://www.cachnet.org/ 2001Last accessed July 1, 2008.
  6. Rosenberg HC, Webb G. Referral of young patients with congenital heart disease to adult centres. The Canadian Adult Congenital Heart Disease Network. Can J Cardiol 1996;12:600-602.[Medline]
  7. Adult Congenital Heart Associationhttp://www.achaheart.org/for_members/clinicdirectory/index.php 1996Last accessed July 1, 2008.
  8. Report of the British Cardiac Society Working Party. Grown-up congenital heart (guch) disease: current needs and provision of service for adolescents and adults with congenital heart disease in the UK. Heart 2002;88(Suppl 1):i1-i14.[Abstract/Free Full Text]
  9. Kiraly L, Temesvari A, Szekely A, et al. Adult congenital heart program in a tertiary care facility of pediatric cardiology Orv Hetil 2005;146:209-214.[Medline]
  10. Vida VL, Berggren H, Brawn WJ, et al. Risk of surgery for congenital heart disease in the adult: a multicentered European study Ann Thorac Surg 2007;83:161-168.[Abstract/Free Full Text]
  11. Mahle WT, Kirshbom PM, Kanter KR, Kogon BE. Cardiac surgery in adults performed at children's hospitals: trends and outcomes J Thorac Cardiovasc Surg 2008;136:307-311.[Abstract/Free Full Text]
  12. Dearani JA, Connolly HM, Martinez R, Fontanet H, Webb GD. Caring for adults with congenital heart disease: successes and challenges for 2007 and beyond Cardiol Young 2007;17(Suppl 2):87-96.[Medline]
  13. Karamlou T, Diggs BS, Person T, Ungerleider RM, Welke KF. National practice patters for management of adult congenital heart disease: operations by pediatric heart surgeons decrease in-hospital death Circulation 2008;118:2345-2352.[Abstract/Free Full Text]
  14. Webb G. Care of adults with congenital heart disease—a challenge for the new millennium Thorac Cardiovasc Surg 2001;49:30-34.[Medline]

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