- Email: [email protected]
The UCLA adult congenital heart disease program
1190
EDITORIALS
The UCLAAdult Congenital Heart DiseaseProgram JOSEPH K. PERLOFF, MD
1
igation of a patent ductus arteriosus in 1939 and “creation of a patent ductus” in 1944-the BlalockTaussig anastomosis-initiated one of the most successful rehabilitation programs that medicine has witnessed.ls2 Accordingly, long-term management of patients with congenital heart disease has come to include increasing numbers of postoperative patients while remaining concerned with natural survival.3 The quality of care provided by pediatric cardiologists from birth to maturity must be met with continuity care of equal quality .4,5In response to this need, an outpatient facility was established at UCLA in 1979, and 2 years later was formalized as the Adult Congenital Heart Disease Program that presently serves about 300 registered patients and bridges the gap between pediatrics and medicine (Tables I and II). Important outgrowths of the program are its teaching and research missions. The first necessity in establishing a formal program in continuity care is for the pediatric and medical cardiologists in policy-making positions to stand as enthusiastic advocates rather than passive supporters. At the UCLA Medical Center, Dr. William F. Friedman, Chairman, Department -of Pediatrics and Dr. Roberta G. Williams, Chief, Division of Pediatric Cardiology are enthusiastic advocates in the best sense. This editorial has 2 chief objectives: fir&, tounderscore the value of a continuity care Program, and second, to describe its operation to facilitate duplication elsewhere. The UCLA Program is staffed by 4 faculty persons-a medical cardiologist (Dr. John S. Child), a pediatric cardiologist [Dr. Gregory B. Wright), a cardiologist with an appointment in Medicine and Pediatrics (Dr. Joseph K. Perloffj and a Cardiovascular Nurse Clinical Specialist (Mary Canobbio, RN, MN). A medical and pediatric cardiologist represent the minimum staff necessary for the proper functioning of a continuity care program in congenital heart disease. During developmental years, the Program enjoyed the collaboration of Dr. Lee Benson (now at the Hospital for Sick Children, Toronto) and Dr. Alvin Chin (now at Children’s Hospital of Philadelphia]. Patients qualify for entry into the Adult Congenital Heart Disease Program when they reach age 18 years or when they are judged to have achieved appropriate psychologic and physical maturity. Some 18-year-old From the Departments of Medicine and Pediatrics and the Divisions of Adult and Pediatric Cardiology, UCLA Center for the Health Sciences. This editorial was supported in part by the Rosalind W. Alcott Bequest, the Streisand/American Heart Association Endowment and the Ethel Wilson Bowles Bequest.
patients are physically small and emotionally immature and are best cared for in a pediatric setting. Conversely, the transition is early for the 16- or 17-year-old who is fully grown or nearly so and who psychologically prefers to be considered as a young adult. The patient population is diverse, comprising those who have never undergone cardiac surgery and are either operable or inoperable, and those who have undergone cardiac surgery and either do not require further repair, have had palliation with the potential for corrective surgery, or are inappropriate for further operation. The most common source of referral is from UCLA pediatric cardiology. When a patient is considered ready for the transition to the adult setting, one of the Adult Congenital Heart Disease Program’s staff generally meets the patient and his or her pediatric cardiologist during a routine visit to the Pediatric Outpatient Clinic. This meeting is especially important when the patient has grown up under the care of one pediatric cardiologist who has empathetically shared the vicissitudes inherent in diagnostic studies and cardiac surgery. The sense of mutual attachment and affection, if not dependency, makes the transition an emotional experience that is more readily resolved by the reassurance provided in an informal meeting with the patient’s future cardiologist. It is also important for patients to know that they will still be seen in the same institution and that their pediatric cardiologist will be kept abreast of their progress. The remaining referrals are chiefly from area physicians who are either internists, pediatricians or cardiologists. Other patients are initially referred to a UCLA cardiac surgeon, are seen in collaboration as inpatients and then assigned for follow-up to the Adult Congenital Heart Disease Program. Organization of the Program’s outpatient clinic is one of its most important aspects. Appointments are made through a single secretary with whom patients become familiar and who is well briefed to respond to special patient needs. The same outpatient rooms and the same clinic nurses are assigned each week to provide a sense of familiarity. Examining rooms are located close to a conference room equipped with an oval table and chairs, x-ray view boxes and a blackboard. The Program discharges a major obligation in educating fellows, residents and nurse specialists who are the next generation of responsible professionals. A medical cardiac fellow and a pediatric cardiac fellow are regularly assigned to the outpatient facility together with a resident physician who rotates through the clinic as part of an outpatient training experience. Every effort is made to keep the scheduled number of
May 1, 1986
TABLE I Patients Who Have Not Undergone (Total 152, 53%)
Corrective
S::ronry I:nnRC%SS*rv nr deferred Functionally norma! bicuspid aortic va!ve Small perimembranous or trabecular VSD Mild pulmonic valve stenosis Ebstein’s anomaly Congenital complete heart block Restrictive VSD, mild AR Restrictive PDA (1 calcified) Situs inversus with dextrocardia Idiopathic dilatation of pulmonary trunk Congenital pulmonic valve regurgitation Pulmonary AV fistula Mild aortic valve stenosis Mild discrete subaortic stenosis Mild pulmonary artery stenosis Mild pulmonic valve stenosis with restrictive VSD L-TGA, mild systemic AV valve regurgitation L-TGA, mild pulmonic stenosis Mild coarctation of aorta Small perforated sinus of Valsalva aneurysm DOW, mild pulmonic stenosis
Surgery
12 9 9 8 6 4 4 4 3 3 3 2 2 2 2 2 2 1 1 1
Total Inoperable Pulmonary vascular disease Eisenmenger’s complex (VSD) ASD secundum Endocardial cushion defect Primary pulmonary hypertension Single ventricle PDA Truncus arteriosus Total anomalous pulmonary venous connection Fallot’s tetralogy, Potts anastomosis Fallot’s tetralogy, Blalock-Taussig anastomosis Aortopulmonary window DORV VSD coarctation of aorta Pulmonary atresia, VSD
80
Total
72
JDURNAL
OF CARDIOLOGY
TABLE II Patients Who Have Undergone (Total 135,47%)
visits and consultations at a numerical levei that permits time for relatively intense concentration on most if not all patients. Initial assessment is by a fellow or resident who then presents the patient to the group as a whole with one cardiologist assuming primary responsibility. Physical examinations are shared in the examining room with the help of a portable set of stethophones. A lively but focused discussion follows in the conference room where complex issues are addressed, clinical decisions are made and problems resolved. Dictated reports are designed to provide an educational experience for referring physicians, especially those in practice. The clinic is promptly responsive to needs for phlebotomy. The laboratory gives priority to processi.ng blood counts, and the clinic nurse makes arrangements for phlebotomy during the same outpatient visit. The clinical nurse specialist briefs new patients on the function of the Program. She is adept at providing counseling, assists in identifying patients whose emo-
Abbreviations
Volume
Correctivs
Extracardiac Coarctation of aorta PDA (not kept in registry) Intracardiac ASD secundum Fallot’s tetralogy VSD, perimembranous Aot-tic valve stenosis Pulmonic valve stenosis Fontan repair, single ventricle or tricuspid atresia Endocardial cushion defect Subaortic stenosis ASD secundum. pulmonic valve stenosis Ebstein’s anomaly Fallot’s tetralogy, pulmonary atresia Complete TGA, Mustard or Senning repair VSD perimembranous, ASD secundum VSD, coarctation of aorta L-TGA. left AV valve regurgitation VSD, PDA DORV, pulmonic stenosis Total anomalous caval connection to left atrium
57
1191
Surgery -___I__ 10
25 21 17 17 11 8 5 4 3 3 2 2 2 1 1 1 1 1 135
Total 18 8 8 8 5 5 3 2 2 1 1 1 -I 9
AR = aortic regurgitation; ASD = atrial septal defect; AV 5: atrioventricular; DORV = double-outlet right ventricle; L-TGA = congenitally corrected transposition of the great arteries; PDA = patent ductus arteriosus; VSD = ventricular septal defect.
foilow-up
THE AMERICAN
as in Table I.
_
tional needs require further help, arranges referrals to a clinical psychologist who is sensitive to the particular needs of our patient population, clarifies recommendations for infective endoearditis prophylaxis and diet, initiates inquiries regarding family planning and supervises nurse specialist trainees. The inpatient aspects of the Program dovetail with the outpatient facility. The nurse specialist coordinates the inpatient/outpatient interface. Patients who require hospitalization are seen in hospital by one of the cardiologists staffing the Program and by the clinical nurse specialist. Conversely, patients who enter the Program through inpatient referral to a cardiac surgeon are seen in hospital before discharge, and followup care arranged in collaboration with the surgeon and referring physician. Patients admitted for cardiac catheterization are studied in the adult laboratory under the supervision of one of the Program’s staff cardiologists (JSC). We provide genetic counseling for both female and male patients, contraceptive recommendations most appropriate for individual female patients, vocational counseling, and advice regarding insurability. An important service for female patients of childbearing age is the close coordination with the high-risk pregnancy group in the Department of Obstetrics and Cynecology. This arrangement is tailored to meet special needs and to provide a reassuring atmosphere, especially for the postoperative patient who confronts a long awaited pregnancy with poignant anticipation tempered by trepidation and uncertainty. We assume responsibility for medical care during gestation, labor and delivery, for fetal echocardiography and for arranging clinical evaluation of the newborn. In the truest sense, the family becomes the patient.
1192
EDITORIALS
Costs of operation were initially underwritten by a grant from the Ethel Wilson Bowles bequest which is gratefully acknowledged. Operational costs are relatively modest and are principally determined by our policy selectively to underwrite outpatient visits and laboratory work so that patient finances are no bar to continuity care. As the Program expanded, additional costs were met through endowments, especially the Rosalind W. Alcott Bequest. Importantly, the Program has developed into a lively area of clinical research prompted by a desire to address unresolved questions inherent in the particular patient population. The investigations generally require collaboration with colleagues in a number of other specialties, thus stimulating valuable interdisciplinary interchange. Hematologic collaboration is required for studies of erythropoiesis and coagulation abnormalities of cyanotic congenital heart disease. Studies of uric acid metabolism and renal function are done in collaboration with nephrologists. A pulmonary research laboratory is required for the investigation of oxygen uptake kinetics during the transition from rest to exercise. A study of myocardial metabolism in cyanotic patients before and after surgical repair requires access to positron emission tomography in the Division of Nuclear Medicine. Investigations of blood flow patterns after Fontan repair for tricuspid
atresia or single ventricle require the collaboration of cardiac surgeons as well as expertise in Doppler echocardiography. A major objective of the Adult Congenital Heart Disease Program is to provide an attractive model for other institutions. Continuity care is too important to be on an ad hoc basis, but instead should be formalized in a given hospital setting. At the University of Iowa, Dr. David Skorton, who was a fellow at UCLA, Dr. Ronald Lauer and Dr. Larry Mahoney are collaborating in a continuity care clinic. Dr. Nelson Schiller recently visited our hospital and is collaborating with Dr. Norman Silverman at the University of California, San Francisco. Future plans call for pooling of information from a number of centers in a computerized data base.
References 1. Engle MA, Perloff JK, eds. Symposium on postoperative congenital heart disease in adults. Am J Cardiol 1982;50:541-656. 2. Engle MA, Perloff JK, eds. Congenital Heart Disease after Surgery. New York: Yorke Medical Books, 1983. 3. Perloff JK. Pediatric congenital cardiac becomes a postoperative adult. The chanaine uooulation of congenital heart disease. Circulation 1973:47:606-619. 4. Engle MA, Adams PH, BGtsonC, Du Shane JW, Elliott LP, McNamara DG, Rashkind WJ, Talner NS. Resources for optimal long term care of congenital heart disease. Circulation 1971;44:A205-A219. 5. Perloff JK. Medical and pediatric cardiology: a contemporary overview. Am J Cardiol 1973;31:670-672. Y
“.
.
EDITORIALS
The UCLAAdult Congenital Heart DiseaseProgram JOSEPH K. PERLOFF, MD
1
igation of a patent ductus arteriosus in 1939 and “creation of a patent ductus” in 1944-the BlalockTaussig anastomosis-initiated one of the most successful rehabilitation programs that medicine has witnessed.ls2 Accordingly, long-term management of patients with congenital heart disease has come to include increasing numbers of postoperative patients while remaining concerned with natural survival.3 The quality of care provided by pediatric cardiologists from birth to maturity must be met with continuity care of equal quality .4,5In response to this need, an outpatient facility was established at UCLA in 1979, and 2 years later was formalized as the Adult Congenital Heart Disease Program that presently serves about 300 registered patients and bridges the gap between pediatrics and medicine (Tables I and II). Important outgrowths of the program are its teaching and research missions. The first necessity in establishing a formal program in continuity care is for the pediatric and medical cardiologists in policy-making positions to stand as enthusiastic advocates rather than passive supporters. At the UCLA Medical Center, Dr. William F. Friedman, Chairman, Department -of Pediatrics and Dr. Roberta G. Williams, Chief, Division of Pediatric Cardiology are enthusiastic advocates in the best sense. This editorial has 2 chief objectives: fir&, tounderscore the value of a continuity care Program, and second, to describe its operation to facilitate duplication elsewhere. The UCLA Program is staffed by 4 faculty persons-a medical cardiologist (Dr. John S. Child), a pediatric cardiologist [Dr. Gregory B. Wright), a cardiologist with an appointment in Medicine and Pediatrics (Dr. Joseph K. Perloffj and a Cardiovascular Nurse Clinical Specialist (Mary Canobbio, RN, MN). A medical and pediatric cardiologist represent the minimum staff necessary for the proper functioning of a continuity care program in congenital heart disease. During developmental years, the Program enjoyed the collaboration of Dr. Lee Benson (now at the Hospital for Sick Children, Toronto) and Dr. Alvin Chin (now at Children’s Hospital of Philadelphia]. Patients qualify for entry into the Adult Congenital Heart Disease Program when they reach age 18 years or when they are judged to have achieved appropriate psychologic and physical maturity. Some 18-year-old From the Departments of Medicine and Pediatrics and the Divisions of Adult and Pediatric Cardiology, UCLA Center for the Health Sciences. This editorial was supported in part by the Rosalind W. Alcott Bequest, the Streisand/American Heart Association Endowment and the Ethel Wilson Bowles Bequest.
patients are physically small and emotionally immature and are best cared for in a pediatric setting. Conversely, the transition is early for the 16- or 17-year-old who is fully grown or nearly so and who psychologically prefers to be considered as a young adult. The patient population is diverse, comprising those who have never undergone cardiac surgery and are either operable or inoperable, and those who have undergone cardiac surgery and either do not require further repair, have had palliation with the potential for corrective surgery, or are inappropriate for further operation. The most common source of referral is from UCLA pediatric cardiology. When a patient is considered ready for the transition to the adult setting, one of the Adult Congenital Heart Disease Program’s staff generally meets the patient and his or her pediatric cardiologist during a routine visit to the Pediatric Outpatient Clinic. This meeting is especially important when the patient has grown up under the care of one pediatric cardiologist who has empathetically shared the vicissitudes inherent in diagnostic studies and cardiac surgery. The sense of mutual attachment and affection, if not dependency, makes the transition an emotional experience that is more readily resolved by the reassurance provided in an informal meeting with the patient’s future cardiologist. It is also important for patients to know that they will still be seen in the same institution and that their pediatric cardiologist will be kept abreast of their progress. The remaining referrals are chiefly from area physicians who are either internists, pediatricians or cardiologists. Other patients are initially referred to a UCLA cardiac surgeon, are seen in collaboration as inpatients and then assigned for follow-up to the Adult Congenital Heart Disease Program. Organization of the Program’s outpatient clinic is one of its most important aspects. Appointments are made through a single secretary with whom patients become familiar and who is well briefed to respond to special patient needs. The same outpatient rooms and the same clinic nurses are assigned each week to provide a sense of familiarity. Examining rooms are located close to a conference room equipped with an oval table and chairs, x-ray view boxes and a blackboard. The Program discharges a major obligation in educating fellows, residents and nurse specialists who are the next generation of responsible professionals. A medical cardiac fellow and a pediatric cardiac fellow are regularly assigned to the outpatient facility together with a resident physician who rotates through the clinic as part of an outpatient training experience. Every effort is made to keep the scheduled number of
May 1, 1986
TABLE I Patients Who Have Not Undergone (Total 152, 53%)
Corrective
S::ronry I:nnRC%SS*rv nr deferred Functionally norma! bicuspid aortic va!ve Small perimembranous or trabecular VSD Mild pulmonic valve stenosis Ebstein’s anomaly Congenital complete heart block Restrictive VSD, mild AR Restrictive PDA (1 calcified) Situs inversus with dextrocardia Idiopathic dilatation of pulmonary trunk Congenital pulmonic valve regurgitation Pulmonary AV fistula Mild aortic valve stenosis Mild discrete subaortic stenosis Mild pulmonary artery stenosis Mild pulmonic valve stenosis with restrictive VSD L-TGA, mild systemic AV valve regurgitation L-TGA, mild pulmonic stenosis Mild coarctation of aorta Small perforated sinus of Valsalva aneurysm DOW, mild pulmonic stenosis
Surgery
12 9 9 8 6 4 4 4 3 3 3 2 2 2 2 2 2 1 1 1
Total Inoperable Pulmonary vascular disease Eisenmenger’s complex (VSD) ASD secundum Endocardial cushion defect Primary pulmonary hypertension Single ventricle PDA Truncus arteriosus Total anomalous pulmonary venous connection Fallot’s tetralogy, Potts anastomosis Fallot’s tetralogy, Blalock-Taussig anastomosis Aortopulmonary window DORV VSD coarctation of aorta Pulmonary atresia, VSD
80
Total
72
JDURNAL
OF CARDIOLOGY
TABLE II Patients Who Have Undergone (Total 135,47%)
visits and consultations at a numerical levei that permits time for relatively intense concentration on most if not all patients. Initial assessment is by a fellow or resident who then presents the patient to the group as a whole with one cardiologist assuming primary responsibility. Physical examinations are shared in the examining room with the help of a portable set of stethophones. A lively but focused discussion follows in the conference room where complex issues are addressed, clinical decisions are made and problems resolved. Dictated reports are designed to provide an educational experience for referring physicians, especially those in practice. The clinic is promptly responsive to needs for phlebotomy. The laboratory gives priority to processi.ng blood counts, and the clinic nurse makes arrangements for phlebotomy during the same outpatient visit. The clinical nurse specialist briefs new patients on the function of the Program. She is adept at providing counseling, assists in identifying patients whose emo-
Abbreviations
Volume
Correctivs
Extracardiac Coarctation of aorta PDA (not kept in registry) Intracardiac ASD secundum Fallot’s tetralogy VSD, perimembranous Aot-tic valve stenosis Pulmonic valve stenosis Fontan repair, single ventricle or tricuspid atresia Endocardial cushion defect Subaortic stenosis ASD secundum. pulmonic valve stenosis Ebstein’s anomaly Fallot’s tetralogy, pulmonary atresia Complete TGA, Mustard or Senning repair VSD perimembranous, ASD secundum VSD, coarctation of aorta L-TGA. left AV valve regurgitation VSD, PDA DORV, pulmonic stenosis Total anomalous caval connection to left atrium
57
1191
Surgery -___I__ 10
25 21 17 17 11 8 5 4 3 3 2 2 2 1 1 1 1 1 135
Total 18 8 8 8 5 5 3 2 2 1 1 1 -I 9
AR = aortic regurgitation; ASD = atrial septal defect; AV 5: atrioventricular; DORV = double-outlet right ventricle; L-TGA = congenitally corrected transposition of the great arteries; PDA = patent ductus arteriosus; VSD = ventricular septal defect.
foilow-up
THE AMERICAN
as in Table I.
_
tional needs require further help, arranges referrals to a clinical psychologist who is sensitive to the particular needs of our patient population, clarifies recommendations for infective endoearditis prophylaxis and diet, initiates inquiries regarding family planning and supervises nurse specialist trainees. The inpatient aspects of the Program dovetail with the outpatient facility. The nurse specialist coordinates the inpatient/outpatient interface. Patients who require hospitalization are seen in hospital by one of the cardiologists staffing the Program and by the clinical nurse specialist. Conversely, patients who enter the Program through inpatient referral to a cardiac surgeon are seen in hospital before discharge, and followup care arranged in collaboration with the surgeon and referring physician. Patients admitted for cardiac catheterization are studied in the adult laboratory under the supervision of one of the Program’s staff cardiologists (JSC). We provide genetic counseling for both female and male patients, contraceptive recommendations most appropriate for individual female patients, vocational counseling, and advice regarding insurability. An important service for female patients of childbearing age is the close coordination with the high-risk pregnancy group in the Department of Obstetrics and Cynecology. This arrangement is tailored to meet special needs and to provide a reassuring atmosphere, especially for the postoperative patient who confronts a long awaited pregnancy with poignant anticipation tempered by trepidation and uncertainty. We assume responsibility for medical care during gestation, labor and delivery, for fetal echocardiography and for arranging clinical evaluation of the newborn. In the truest sense, the family becomes the patient.
1192
EDITORIALS
Costs of operation were initially underwritten by a grant from the Ethel Wilson Bowles bequest which is gratefully acknowledged. Operational costs are relatively modest and are principally determined by our policy selectively to underwrite outpatient visits and laboratory work so that patient finances are no bar to continuity care. As the Program expanded, additional costs were met through endowments, especially the Rosalind W. Alcott Bequest. Importantly, the Program has developed into a lively area of clinical research prompted by a desire to address unresolved questions inherent in the particular patient population. The investigations generally require collaboration with colleagues in a number of other specialties, thus stimulating valuable interdisciplinary interchange. Hematologic collaboration is required for studies of erythropoiesis and coagulation abnormalities of cyanotic congenital heart disease. Studies of uric acid metabolism and renal function are done in collaboration with nephrologists. A pulmonary research laboratory is required for the investigation of oxygen uptake kinetics during the transition from rest to exercise. A study of myocardial metabolism in cyanotic patients before and after surgical repair requires access to positron emission tomography in the Division of Nuclear Medicine. Investigations of blood flow patterns after Fontan repair for tricuspid
atresia or single ventricle require the collaboration of cardiac surgeons as well as expertise in Doppler echocardiography. A major objective of the Adult Congenital Heart Disease Program is to provide an attractive model for other institutions. Continuity care is too important to be on an ad hoc basis, but instead should be formalized in a given hospital setting. At the University of Iowa, Dr. David Skorton, who was a fellow at UCLA, Dr. Ronald Lauer and Dr. Larry Mahoney are collaborating in a continuity care clinic. Dr. Nelson Schiller recently visited our hospital and is collaborating with Dr. Norman Silverman at the University of California, San Francisco. Future plans call for pooling of information from a number of centers in a computerized data base.
References 1. Engle MA, Perloff JK, eds. Symposium on postoperative congenital heart disease in adults. Am J Cardiol 1982;50:541-656. 2. Engle MA, Perloff JK, eds. Congenital Heart Disease after Surgery. New York: Yorke Medical Books, 1983. 3. Perloff JK. Pediatric congenital cardiac becomes a postoperative adult. The chanaine uooulation of congenital heart disease. Circulation 1973:47:606-619. 4. Engle MA, Adams PH, BGtsonC, Du Shane JW, Elliott LP, McNamara DG, Rashkind WJ, Talner NS. Resources for optimal long term care of congenital heart disease. Circulation 1971;44:A205-A219. 5. Perloff JK. Medical and pediatric cardiology: a contemporary overview. Am J Cardiol 1973;31:670-672. Y
“.
.