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1881

Clinical Progress Series

Congenital Heart Disease in AdultsA New Cardiovascular Subspecialty

Joseph K. Perloff, MD

Advances in diagnostic methods and in thesurgical and medical care of infants andchildren with congenital heart disease have

resulted in one of the most successful rehabilitationprograms that medicine has witnessed.1'2 In theUnited States alone, there are presently between500,000 and 600,000 adults with congenital heartdisease. Each year, about 20,000 open operations forcongenital heart disease (an approximate minimum)are performed in the United States (personal com-munication, 1990, Dr. Peter Einstein, Assistant Pro-fessor of Surgery and Pediatrics, University of Ten-nessee). Of an estimated 25,000 infants currentlyborn with congenital malformations of the heart andcirculation, over 85% will reach adulthood. Theheavy investments in time and resources needed toaccomplish these results should now be matched witha similar dedication to the adolescent and adult withcongenital heart disease. Potential subspecialty pro-fessional resources (1989 figures) include 884 board-certified pediatric cardiologists, too few to cope withthe patient load of adults with congenital heartdisease even if all 884 were committed to do so, and12,119 board-certified medical cardiologists, only asmall minority of whom have knowledge of, interestin, or responsibility for adults with congenital cardiacmalformations.23 Congenital heart disease in adultsis still a largely unrecognized subspecialty but isemerging as a discipline that requires special expertise.The adult congenital heart disease patient popula-

tion includes those who have never undergone car-diac surgery, those who have undergone cardiacsurgery and require no further operation, those whohave had palliation with or without anticipation ofreparative surgery, and those who are inoperableapart from organ transplantation. The following re-marks deal broadly with what this new area ofcardiovascular interest presently comprises: multidis-ciplinary facilities for comprehensive care, survivalpatterns (natural and postoperative), medical consid-erations, surgical considerations, and postoperativeresidua and sequelae.

From the Divisions of Cardiology, Departments of Medicineand Pediatrics and the Adult Congenital Heart Disease Center,UCLA School of Medicine, Los Angeles, Calif.Address for correspondence: Joseph K. Perloff, MD, UCLA

School of Medicine, Division of Cardiology, 47-123 CHS, LosAngeles, CA 90024.

The seminal contributions of Maude Abbott,4Robert Gross,5 Helen Taussig and Alfred Blalock,6and Clarence Crafoord7 set the stage. The postwarintroduction of cardiac catheterization was a majorstep forward, and the development of extracorporealcirculation in the early to mid 1950s was destined tomake virtually all congenital malformations of theheart accessible to the skills of cardiac surgeons.8Survival patterns have been affected, often pro-foundly. Congenital heart disease should thereforebe considered not only in terms of age of onset butalso in terms of the age range that survival nowpermits.9"10 Long-term management remains con-cerned with natural survival but is increasingly con-cerned with the growing numbers of postoperativepatients who, with few exceptions, continue to needmedical surveillance.11 Unoperated adults experi-ence improved longevity and well-being owing torefinements in the medical management of hemato-logic disorders, renal function, urate metabolism,pulmonary physiology, infective endocarditis, elec-trophysiological abnormalities, pregnancy, and non-cardiac surgery.12 The ideal cardiac surgical objec-tive-cure in the literal sense-is rarely achieved.10Accordingly, operations leave behind a broad rangeof residua and sequelae that may require prolonged,if not indefinite, medical attention.1' Uninterrupted,long-term care is essential if the concerns inherent inthis new and increasing patient population are to beaddressed.9"10

Multidisciplinary Facilities for the ComprehensiveCare of Adults With Congenital Heart DiseaseSpecialized tertiary medical facilities for the care

of adults with congenital heart disease were begun inthe 1960s at the Toronto General Hospital (now theToronto Hospital) and evolved there into the Con-genital Heart Disease Centre.2 An analogous facilitywas developed in 1975 at the National Heart HospitalLondon (now the Royal Brompton and NationalHeart Hospitals).2 Development of a similar facilityat the Mayo Clinic (1988) is indebted to the experi-ence at the National Heart Hospital London. TheUCLA Adult Congenital Heart Disease Center be-gan in 1978, and the University of Iowa Adolescent/Adult Congenital Heart Disease Clinic, which beganin 1981, is indebted to the UCLA experience.2 Over6,000 adults with congenital heart disease are cur-

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1882 Circulation Vol 84, No 5 November 1991

rently registered in these five specialized centers inCanada, Great Britain, and the United States.2 Anumber of other teaching hospitals are following suit.A first necessity is to recognize the seminal role of

pediatric cardiologists and pediatric cardiac sur-geons, without whose efforts congenital heart diseasein adults would not be a matter of importance.Relevant to this topic are the staff, the laboratories,the criteria for patient entry, the referral sources, theoutpatient and inpatient management, the multidis-ciplinary consultants, and the educational, training,and research commitments of the specialized facili-ties for adults with congenital heart disease.1,2The staff reflects the current consensus that pa-

tients are best managed, at least in the foreseeablefuture, by collaboration between pediatric and med-ical cardiologists with the assistance of cardiovascularnurse specialists and physician assistants. Collaborat-ing cardiac surgeons must be equipped to deal withboth congenital and acquired cardiovascular diseasesand with surgical problems that are often unique tothe adult with congenital heart disease. Dedication totask is a collaborative effort, especially in the settingof a university hospital in which intellectual inter-change, teaching, and research are as paramount asoptimal patient care.

Criteria for patient entry into the specialized facil-ities are based on age and psychological and physicalmaturity. Entry may begin at age 12 years, recogniz-ing adolescent medicine as a bridge between thepreadolescent patient and the adult. Alternatively,admission may begin at ages 17 or 18 years, depend-ing on maturity.

Referrals reflect the notion that tertiary centersare not designed to compete with practicing physi-cians or community hospitals but instead to offerservices difficult, if not impossible, to duplicate. Animportant source of referrals is pediatric cardiologyat the parent institution, but significant and growingnumbers of referrals are from internists, pediatri-cians, family practitioners, or cardiologists within oroutside the immediate geographic area (state, out-of-state, and abroad).There is a mounting consensus that outpatients are

more appropriately seen in clinics for adults (i.e., thatadult care is best provided in an adult setting).Advanced planning of diagnostic studies is obligatoryfor patients who are referred to the outpatient facil-ity from long distances. The number of patients peroutpatient clinic largely determines the amount oftime available for sharing information between staffand trainees, underscoring the educational mission ofthe facility including teaching of medical students,house officers (pediatric and medical), and pediatricand medical cardiac fellows.

Inpatients include admissions for cardiac or non-cardiac surgery, for labor and delivery, for the med-ical management of arrhythmias or heart failure, orfor the management of noncardiac medical disorders.Inpatients constitute an important part of the educa-tional experience of house staff and fellows. The

clinical nurse specialist coordinates the inpatient/outpatient interface.

Noninvasive, catheterization, and angiographiclaboratories for adults with congenital heart diseasemust meet the standards established by pediatriccardiology laboratories. Adults are best studied inlaboratories that are designed for adults, but qualityshould not be compromised for expediency or design.Noncardiac consultants should be formally incor-

porated into the adult congenital heart disease facil-ity, including specialists in hematology, nephrology,urate metabolism, pulmonary medicine, electrophys-iology, genetics and epidemiology, gynecology andobstetrics, psychiatry, anesthesiology, pathology, so-cial services, insurability, and vocational counsel-ing.12 The objective is to have ready access to con-sultants who have gained experience in the specificproblems associated with congenital heart disease inadults.Adult congenital heart disease facilities assume a

major role in the education and training of fellows,residents, nurse specialists, and physician assistantswho will become the next generation of responsibleprofessionals.1-3 If the medical and pediatric spe-cialty boards placed more emphasis on congenitalheart disease in adults, the percent of interestedfellows and training program directors would in-crease accordingly. The cardiology specialty boardscan play pivotal roles in encouraging fellowship pro-grams to adjust their curricula. If passing the boardsrequired an appropriate level of knowledge of con-genital heart disease in adults, the training programsare likely to take cognizance.2 For medical cardiacfellows, formal rotations (inpatient and outpatient)including a rotation in pediatric cardiology should bepart of their experience. Anticipated professionalrequirements dictate the need for specialized fellow-ship training designed for cardiologists who wish toqualify as career experts in the evolving field ofcongenital heart disease in adults.2 Educational re-sponsibilities extend to community physicians, tovisiting physicians from other institutions, and, im-portantly, to the patients themselves. Stratification ofpatient care is a major issue.2 The base of thepyramid is the primary care physician (general pedi-atrician, general physician). The next level (stratum)is the cardiologist (pediatric, medical) who providesboth consultative and primary care in the community.Tertiary care depends on regional specialized centersthat provide experience and expertise even with rareand complex lesions. It is not yet clear how large apopulation is needed to serve a tertiary care center.A commitment to research is prompted by a desire

to address unresolved questions intrinsic to the adultcongenital heart disease population. The investiga-tions generally require collaboration with colleaguesin a number of other specialties, thus promotingvaluable interdisciplinary interchange. A researchbase must be provided for cardiac fellows interestedin careers in academic medicine.



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Perloff Congenital Heart Disease in Adults 1883

TABLE 1. Common Defects With Expected Adult Survival

Bicuspid aortic valve (functionally normal, stenotic, incompetent)Coarctation of the aortaPulmonary valve stenosisAtrial septal defect secundumPatent ductus arteriosus

Natural Survival PatternsNatural survival includes malformations that do

not require operation, those that remain amenable tooperation in adulthood, and those that are inopera-ble except for organ transplantation.1,2 Managementof adults with congenital heart disease must take intoaccount acquired disorders of the heart and circula-tion and noncardiac illnesses that may coexist andmodify the physiological expressions of the basiccongenital cardiac malformation. Natural survivalpatterns, in order of clinical importance, includecommon defects with expected adult survival (Table1), uncommon defects with expected adult survival(Table 2), and common defects with exceptionaladult survival (Table 3).

Survival Patterns After Surgery or InterventionalCatheterization

An understanding of prognosis after cardiac sur-gery or interventional catheterization requires knowl-edge of the preoperative congenital malformation,the nature and effects of the therapeutic interven-tion, and the postoperative or postinterventionalresidua and sequelae.112 Success is measured notonly by the length of survival but by the quality of lifeand the need for reoperation. Patients who under-went cardiac surgery decades ago benefited from theanatomic repairs but often suffered from the delete-rious effects of what would now be considered inad-equate myocardial protection.' Prosthetic materi-als-valves, patches, and conduits-that were stateof the art decades ago have been superceded by many

TABLE 2. Uncommon Defects With Expected Adult Survival

Situs solitus with dextrocardiaSitus inversus with dextrocardiaCongenital complete heart blockCongenitally corrected transposition of the great arteriesCongenital mitral regurgitationEbstein's anomaly of the tricuspid valveUhl's anomalyDiscrete subaortic stenosisCongenital pulmonary valve regurgitationPrimary pulmonary hypertensionAtrial septal defect (primum, sinus venosus)Lutembacher's syndromeAneurysm of a sinus of ValsalvaCoronary arteriovenous fistulaPulmonary arteriovenous fistulaAnomalous course of a coronary artery

TABLE 3. Common Defects With Exceptional Adult Survival

Ventricular septal defectFallot's tetralogy

generations of improved devices and materials. Latesurvival after cardiac surgery or interventional cath-eterization relates to repair or replacement of con-genitally malformed cardiac valves; intra-atrial re-pairs including atrial septal defects, Mustard orSenning venous switch operations for complete trans-position of the great arteries; intraventricular surgerythrough a right atrial incision or through a ventricu-lotomy (Fallot's tetralogy, for example); central arte-rial surgery (patent ductus arteriosus, coarctation ofthe aorta, sinus of Valsalva aneurysms) and partial orcomplete caval to pulmonary artery circulations(Glenn or Fontan procedures).'

Medical ConsiderationsCyanotic Congenital Heart Disease: HematologicManagement, Renal Function, and Urate Metabolism

Cyanotic patients with erythrocytosis have beenclassified as "compensated" or "decompensated."'13"14Those with compensated erythrocytosis establishequilibrium hematocrits in iron-replete states.Symptoms attributable to hyperviscosity14 are usu-ally mild or absent when hematocrit levels are lowerthan 65% and absent, mild, or moderate even athigh hematocrit levels, occasionally 70% or more.Phlebotomy for relief of hyperviscosity symptoms isrequired rarely, if at all. Patients with decompen-sated erythrocytosis fail to establish equilibriumconditions and manifest unstable, rising hematocritlevels and recurrent, moderate to severe symptomsattributable to hyperviscosity.14 Erythrocyte pro-duction is not regulated; feedback inhibition fails tooccur. Symptomatic hyperviscosity is common,prompting therapeutic phlebotomy that temporarilyrelieves symptoms but depletes iron stores. Becausesymptomatic hyperviscosity in an iron-replete stateseldom occurs with hematocrit levels of lower than65%,14 these symptoms in patients with hematocritlevels lower than 65% are almost always due to irondeficiency; phlebotomy aggravates rather than alle-viates the symptoms.

TABLE 4. Common Acyanotic and Cyanotic Malformations ThatPermit Survival to Childbearing Age

AcyanoticAtrial septal defect (secundum)Patent ductus arteriosusPulmonary valve stenosisCoarctation of the aorta

Aortic valve diseaseCyanotic

Fallot's tetralogy

Malformations are listed in descending order of prevalenceamong women.



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In our experience, adults with cyanotic congenitalheart disease are at little or no increased risk ofstroke, even if the hematocrit level is above 65% andeven if the erythrocytosis is decompensated (irondeficient).1,13,14 Cerebrovascular accidents in cyanoticadults are usually associated with excessive, injudi-cious phlebotomies or with the use of aspirin oranticoagulants that reinforce the intrinsic hemostaticdefects (see below) and cause intracranial bleed-ing.1,14 Phlebotomy is not recommended for adultpatients with compensated erythrocytosis, includingthose with hematocrit levels in the range of 70%, aslong as symptoms attributed to hyperviscosity14 aremild or absent. Phlebotomy is recommended in pa-tients with marked to severe hyperviscosity symptomsand with hematocrit levels of 65% or greater, pro-vided dehydration is not the cause. Dehydration istreated by volume replacement, not phlebotomy. Acomparatively safe, simple outpatient method forphlebotomy in adults involves removal of 500 ml ofblood over 30 to 45 minutes followed by quantitativevolume replacement, generally with isotonic saline,less commonly with salt-free dextran in 5% dextroseif patients have congestive heart failure.When iron is administered in symptomatic iron-

deficient patients with inappropriately low hemato-crit levels, the dose should be small (325 mg ferroussulfate per day). Hematocrit levels tend to risequickly so the erythrocyte response must be moni-tored closely. Patients should be cautioned to avoidover-the-counter preparations that contain iron.A significant increase in red cell mass can lead to

inaccuracies in routine laboratory determinations ofhematocrit. Hematocrit levels must be based onautomated blood counts, because microhematocritcentrifugation methods result in plasma trappingwith falsely elevated levels.1'15Home oxygen administration has sometimes been

advocated, especially during sleep.15-19 From thehematologic and respiratory points of view, there ispresently insufficient evidence that oxygen is useful inadults with cyanotic congenital heart disease, and thedrying effect on nasal mucous membranes tends toincrease the risk of epistaxes (see below).

Hemostasis is abnormal in cyanotic congenitalheart disease.1'14-19 For the most part, bleeding ten-dencies are mild and are characterized by easy bruis-ing, petechial hemorrhages in the skin and mucousmembranes, epistaxis, gingival bleeding, and hemop-tysis. Occasionally, however, epistaxis and hemopty-sis can be copious and infuriatingly recurrent. Plate-let counts are generally in the low range of normal,but when the increased blood volume is taken intoaccount, the total circulating platelet mass is closer tonormal than platelet concentrations imply. Inherentabnormalities in platelet function20 are reinforced byaspirin or nonsteroidal anti-inflammatory agents. Inaddition, abnormalities of the intrinsic and extrinsiccoagulation systems with elevations of prothrombintime and activated partial thromboplastin time, re-spectively, and specific deficiencies of a number of

coagulation factors have been reported.16 Aspirin,oral anticoagulants, and heparin are ill-advised be-cause the inherent risk of stroke is low, because thesedrugs have no demonstrated efficacy in reducing thatnegligible risk, but instead may significantly aggra-vate the existing hemostatic defects and increase therisk of bleeding.

Serious hemorrhage may occur during surgery oraccidental trauma.21 Phlebotomy has been shown toimprove hemostasis temporarily in some erythrocy-totic patients. Preoperative phlebotomy is thereforeselectively used to reduce the hematocrit level to justbelow 65%.1 Phlebotomized units are reserved forpotential autologous transfusions.

Renal function and urate metabolism are oftenabnormal in adults with cyanotic congenital heartdisease.22,23 High plasma uric acid levels are second-ary to inappropriately low renal fractional uric acidexcretion rather than to urate overproduction.22 En-hanced urate reabsorption is believed to be caused byrenal hypoperfusion reinforced by a high filtrationfraction. Accordingly, hyperuricemia serves as amarker of abnormal intrauterine hemodynamics.22Renal histopathology is characterized by enlarged,hypercellular glomeruli, basement membrane thick-ening, focal interstitial fibrosis, tubular atrophy, andhyalinization of afferent and efferent arterioles.24

Arthralgias are relatively common in erythrocytoticadults with cyanotic congenital heart disease, butacute gouty arthritis is relatively infrequent despiteelevated uric acid levels, an observation similar tothat in other forms of secondary hyperuricemia.25 Ifcolchicine is used to treat acute gouty arthritis, caremust be taken to avoid the dehydrating effects ofvomiting and diarrhea. Nonsteroidal anti-inflamma-tory agents may then be considered but should beused cautiously because of the intrinsic hemostaticdefects. Uricosuric agents are not routinely advisedbut can be efficacious in patients with hyperuricemiaand recurrences of gouty arthritis.'

Cyanotic Congenital Heart Disease: Dynamics ofOxygen Uptake and Control of Ventilation

Exercise may significantly increase the degree ofvenoarterial mixing and materially influence the dy-namics of 02 uptake and ventilation.26-28 Patientswith cyanotic congenital heart disease exhibit markedabnormalities in achieving a new steady state for Vo2after the onset of exercise.26 Those with right-to-leftshunts have greater increases in ventilation duringexercise than do normal subjects; breathlessness onexertion may be a prominent clinical complaint un-associated with heart failure.27 Accordingly, assigninga New York Heart Association functional class ismisleading (see below).

Infective Endocarditis: Risks and ProphylaxisWith the advent of intracardiac surgery and pros-

thetic devices, the clinical and bacteriological profileof infective endocarditis changed significantly.1'2'229Some operations (for example, ligation of a patent



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ductus arteriosus) eliminate the risk altogether,whereas other operations (shunts, prosthetic valvesor conduits) materially increase the risk. However,certain general principles still prevail, namely, thattwo major causes predispose to infective endocardi-tis: a susceptible cardiac or vascular substrate and asource of bacteremia. Prophylaxis includes bothchemotherapeutic (antimicrobial) and nonchemo-therapeutic measures. Chemotherapeutic prophy-laxis is based on the cardiac lesion, the source ofpotential bacteremia, and the absence or presence ofthe history of antibiotic sensitivity.3031 Nonchemo-therapeutic prophylaxis includes day-to-day oral hy-giene, skin care, nail care, and in females, avoidanceof certain contraceptive devices.1,2 The spongy, frag-ile gums of patients with cyanotic congenital heartdisease are a special concern. Appointments withdentists or oral hygienists should be made twiceyearly for teeth and gum prophylaxes. Meticulousskin care is important, especially in adolescents withacne. Young adults with cyanotic congenital heartdisease are prone to develop acne, and pustules maybe distributed beyond the face.

Pregnancy and Congenital Heart Disease: The Motherand the FetusThe common congenital malformations of the

heart and circulation in which natural (unoperated)survival of women into childbearing age can beanticipated are listed in Table 4 in approximate orderof prevalence among women. However, the postop-erative cardiac female now constitutes an importantcategory of pregnancy and heart disease and repre-sents a growing patient population.1"32 Successfulcardiac surgery improves fertility and stabilizes thepregnancy in patients whose heart disease had re-duced their sexual and ovarian functions. Womenwho were physiologically ill equipped to bear chil-dren or previously would not, in all probability, havereached reproductive age are now presenting forobstetric and cardiac care after reparative surgery forcongenital heart disease.",32 Although the expectantmother's cardiac reserve is reduced by the hemody-namic burden of pregnancy, the reduction can almostalways be countered by addressing the factors thatencroach on circulatory reserve. Maternal mortalityhas been said to vary directly with functional class,but in the presence of congenital heart disease, thereare two important caveats: 1) cardiac symptoms onwhich the New York Heart Association functionalclasses were originally based are more relevant toacquired than to congenital heart disease, and 2) inthe presence of certain congenital cardiac malforma-tions, childbearing imposes such a formidable threatto maternal survival that pregnancy is proscribed orshould be interrupted irrespective of functional class.A strong consensus favors spontaneous vaginal

delivery.'32 Labor in the lateral decubitus positionattenuates the hemodynamic fluctuations associatedwith major uterine contractions in the supine posi-tion. Cesarean section is reserved for cephalopelvic

disproportion or for preterm labor in women takingoral anticoagulants. Because the probability ofthromboembolism increases during the postpartumperiod, patients with lesions susceptible to paradox-ical embolization (ostium secundum atrial septaldefect, for example) require meticulous leg care, useof elastic support stockings, and early ambulation.1"33The need for antibiotic prophylaxis during routine

delivery in pregnant cardiac patients is controver-sial.1"2 It has been argued that prophylaxis is unnec-essary in uncomplicated vaginal deliveries becausebacteremia is neither a natural nor an inevitableoccurrence.2 However, it can also be argued that it isnot prudent to assume that delivery will be uncom-plicated, and delivery with an episiotomy is not,strictly speaking, uncomplicated.

Maternal congenital heart disease exposes the fetusto immediate risks that threaten its intrauterine viabil-ity and to remote risks that express themselves ascongenital and developmental malformations.",2'34'35Immediate risks are chiefly determined by the func-tional class of the mother (with the qualificationsmentioned earlier), by maternal cyanosis, and by anti-coagulants. Remote risks to the fetus take the form ofgenetic parental transmission, teratogenic effects ofcertain cardiac drugs, and the harmful effects of certainenvironmental toxins and environmental exposures.

Exercise and Athletics Before and After Surgery orInterventional Catheterization

Consideration must be given to 1) the type, inten-sity, and duration of exercise; 2) the risk of bodycollision inherent in a given type of athletic activity,especially in patients receiving anticoagulants; 3) thetraining program (conditioning) required for a givensport; 4) the emotional response (stress) that theathlete experiences in anticipation of or during aparticular sporting event; and 5) the risk of injuryeither to the athlete or to spectators if the athleticactivity provokes loss of consciousness.'136,37 Twocategories of exercise are recognized, isotonic andisometric, but there is often a continuum between thetwo, with most physical activity incorporating bothisotonic and isometric components.36 Relevant to thisdiscussion are the type and severity of a given con-genital malformation and the presence, type, andsuccess of surgery. As a rule, the malformation underconsideration is clinically overt (congenital aorticstenosis, for example) but an occult aberrant coro-nary artery coursing between the aorta and rightventricular outflow tract may first announce itself byexercise-induced angina pectoris, myocardial infarc-tion, or sudden death.38 Pulmonary vascular diseaseis an important independent variable epitomized byprimary pulmonary hypertension in which syncope oneffort is a striking feature that often heralds suddendeath.1'2 In Fallot's tetralogy, isotonic exercise aug-ments the right-to-left shunt and results in subjectivebreathlessness chiefly caused by the response of therespiratory center to the sudden changes in blood gas



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composition and pH, not by heart failure. After aright ventriculotomy, exercise may induce ventriculararrhythmias.39 The Fontan operation results in acirculation in series without a subpulmonic ventricle;exercise performance may improve significantly butcardiac indexes in response to isotonic exercise sel-dom increase more than twofold.40,41

Insurability, Employability, and PsychosocialConsiderationsAs newer and more successful methods of treat-

ment are applied and as the long-term benefits ofthese therapeutic advances are realized, patients arelikely to enjoy higher access to insurance.1,2,42,43 Mostyoung adults who have undergone successful repairof congenital heart lesions are eligible for some formof life insurance but health insurance is more prob-lematic. It has been roughly estimated that in theUnited States, one third of adults with congenitalheart disease have inadequate or no health insuranceeven though they may be physically capable of work-ing.2 Constraints imposed by fee-for-service insur-ance plans (no coverage for preexisting illness) makethese plans unattractive, especially for patients whoanticipate further surgery or cardiac catheterizationor for those who require frequent ambulatory evalu-ations or diagnostic testing. The future of healthmaintenance organizations and independent practiceassociations is uncertain, but there is a trend towardthese prepaid medical care providers. The currentheightened interest and concern in the funding ofhealth care in the United States44 would do well toinclude congenital heart disease in adults, a patientpopulation that not only is increasing year by year butalso generally has the educational achievements andphysical capabilities for productive employment.2 In-novations such as statewide insurance pools, employ-er-based health care systems, and national healthinsurance should be explored so that these deservingand productive members of the community can ob-tain adequate medical care.2There are advocacy groups for patients with cystic

fibrosis and Down's syndrome; why not for patientswith congenital heart disease? If funding for the careand study of adults with congenital heart diseasewere secure, support for specialized care facilitieswould improve, more cardiologists would be inter-ested, and a manpower shift might take place.The opportunities for employment of adults with

congenital cardiac defects are influenced by educa-tion, type of cardiac lesion, job discrimination, car-diac surgery, and often by the reluctance of anemployer to accept the health insurance responsibil-ities involved in hiring.1,2,43 In certain occupations,the safety of others is in the hands of a singleindividual (bus drivers and airline pilots, for exam-ple). Job discrimination is one of the most importantfactors affecting employment opportunities for thesepatients. The smaller the company to which theapplication is made, the greater is the reluctance ofemployers to hire someone with a thoracotomy scar

or a preexisting cardiac disorder. The National Re-habilitation Act of 1973 prevents job discriminationagainst the handicapped by almost all employers with10 or more employees. The Vocational Rehabilita-tion Act of 1920, strengthened by amendments, offersa wide range of services but these services are signif-icantly underused, especially by cardiac patients.There are special, if not unique, psychosocial con-

cerns in patients who have experienced dramatic andsometimes traumatic diagnostic and therapeutic in-terventions during key developmental phases of theirlives.1,2'45-47Nevertheless, most patients with congen-ital heart disease function psychologically within nor-mal range, although low self-esteem, insecurity, andfeelings of vulnerability are sometimes matters ofconcern.44 Parental knowledge, understanding, andattitude largely determine the patients' and the par-ents' perceptions of the congenital heart disease andsignificantly affect psychological adjustments. Theadult with congenital heart disease faces tangibleproblems in the work force (see above), in dating,marriage, and parenthood. Cyanosis is believed toimpair intellectual function, although the degree ofimpairment is generally mild and may be overesti-mated by IQ tests that depend on gross motorfunction at a young age.47

Surgical ConsiderationsOperation or reoperation in adults with congenital

heart disease often involves special surgical concernspeculiar to older patients.12 These concerns musttake into account the congenital cardiac malforma-tion per se (previously operated or unoperated)together with acquired cardiac and noncardiac dis-eases of adulthood. In cyanotic adults confrontingtheir initial operation, hematologic disorders (seeabove) and aortopulmonary collaterals are matters ofconcern. In adults who have undergone reparativesurgery, the chief concerns at the time of reoperationare prosthetic materials such as conduits and valves.Concerns that apply to both unoperated patients andpatients undergoing reoperation include pulmonaryvascular disease, ventricular function, myocardialprotection (cardioplegia), blood salvage techniques,the risk of infective endocarditis, the residua andsequelae of previous cardiac surgery, and coexistingacquired heart disease, the incidence of which varieswith age.

Interventional cardiac catheterization is now thepreferred primary treatment or an adjunct to thesurgical treatment of increasing numbers of pediatricand adult patients with congenital malformations ofthe heart and circulation.1,24849 Corrective or repar-ative interventional catheterization procedures ad-dress pulmonary valve stenosis, recoarctation of theaorta, patent ductus arteriosus, and in selected pa-tients, ostium secundum atrial septal defect. Pallia-tive interventions can be either in lieu of surgery or asadjuncts to surgery. Therapeutic cardiac catheteriza-tion, like cardiac surgery, has three principle objec-tives: preservation of or improvement in cardiac



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function, an increase in longevity, and maintenanceof or improvement in the quality of life.When adults with congenital heart disease require

noncardiac surgery, perioperative risks can be re-duced, often appreciably, when problems inherent inthis patient population are anticipated.1'2 Uncompli-cated situs inversus with dextrocardia may go unrec-ognized until an illness requiring surgery brings thepatient to attention. Symptoms are likely to be mis-construed and diagnostic conclusions incorrect un-less the mirror-image visceral positions are known. Inacute appendicitis, the abdominal pain is in the leftlower quadrant, and the pain of biliary colic is in theleft upper quadrant. Noncardiac surgery in an adultwith a functionally normal or mildly stenotic orincompetent bicuspid aortic valve imposes a risk thatis essentially that of infective endocarditis. Fibrocal-cific obstruction of a congenital bicuspid aortic valveaccounts for approximately one half of the cases ofsurgically important pure aortic stenosis in adults,50the immediate risks of noncardiac surgery are deter-mined by the degree of obstruction, the functionaladequacy of the after-loaded left ventricle, and thepresence and degree of acquired coronary arterydisease. Patients with hemodynamically significantcongenital bicuspid aortic regurgitation confront car-diac surgery with risks determined by left ventricularfunction and susceptibility to infective endocarditis.In patients with primary pulmonary hypertension,even minor noncardiac surgery incurs risks that causetrepidation",2; the stress of a surgical procedure cantrigger circulatory demands that are not adequatelymet. The corollary to these remarks is that evenminor elective surgery in patients with primary pul-monary hypertension is, with few exceptions, to beavoided. Ostium secundum atrial septal defect inasymptomatic young adults with normal pulmonaryarterial pressure imposes comparatively little riskduring noncardiac surgery but with two qualifica-tions. In response to hemorrhage, systemic resistancerises and venous return diminishes, a combinationthat sometimes appreciably augments the left-to-right intra-atrial shunt. The second concern is therisk of paradoxical emboli from leg veins becausethrombi carried by the inferior vena cava tend tostream across the atrial septal defect into the sys-temic circulation.1"32 In cyanotic adults with congen-ital heart disease, the incidence of cholecystitis (cal-cium bilirubinate stones) is increased. Biliary colicsometimes becomes manifest years after intracardiacsurgery has eliminated the cyanosis. Hemostasis incyanotic adults can be improved by preoperativephlebotomy (see earlier discussion) with the phlebot-omized units stored for potential autologous transfu-sion. Intravenous lines, infusions, and drugs in cyan-otic patients must be managed with special care. Anair filter should be inserted into the intravenous line.In the adult with uncorrected Fallot's tetralogy whorequires noncardiac surgery, meticulous intraoper-ative and postoperative monitoring of blood pressureis important because a sudden fall in systemic resis-

TABLE 5. Residua After Reparative Surgery for CongenitalHeart Disease

ElectrophysiologicalValvularVentricularChamber morphologyChamber massChamber functionMyocardial connective tissue

VascularAnatomic (morphological) vascular anomalies or defectsElevated resistance and/or pressures; systemic, pulmonary

NoncardiovascularDevelopmental abnormalitiesSomatic defectsMedical disorders

tance may precipitate intense cyanosis and occasion-ally death or a sudden rise in systemic resistance mayabruptly and dangerously depress systemic bloodflow.12 Continuous monitoring with a pulse oximeteris useful. Cyanotic patients with elevated pulmonaryresistance confront noncardiac surgery with risksinherent in the cyanosis per se, in addition to theformidable risks of pulmonary vascular disease. Anexample is tubal ligation in a woman with Eisen-menger's complex. Prosthetic mechanical valves com-plicate the management of subsequent noncardiacsurgery. The immediate intraoperative and perioper-ative concern is anticoagulation, in addition to andapart from the risk of infective endocarditis. Ventric-ular function is an important variable in the long-term management of patients after surgery for con-genital heart disease, and the adequacy of ventricularfunction (left, right, or single ventricle) is a majordeterminant of risk during noncardiac surgery inadulthood. Adults with congenital heart disease notonly have their preoperative or postoperative cardiacmalformations but also have cardiovascular and non-cardiac diseases that are acquired with age.

Postoperative Residua and SequelaeResidua after reparative surgery for congenital

heart disease (Table 5) are represented by cardiac,vascular, or noncardiovascular abnormalities that areintentionally left behind at the time of reparativesurgery."2"1 With few exceptions, these residua areobligatory, that is, they do not result from surgeryhaving fallen short of its goal, at least in a technicalsense.51 By contrast, sequelae are alterations or dis-orders that are intentionally incurred, occasionally orinvariably, at the time of reparative surgery and arelooked upon as necessary and acceptable conse-quences of operation (Table 6). Complications areunintentional aftermaths of reparative surgery thatrange in severity from inconsequential to fatal. Thefocus here is not on complications, although compli-cations and sequelae may imperceptibly merge.



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TABLE 6. Sequelae of Reparative Surgery for Congenital HeartDisease

ElectrophysiologicalAtriotomy

Intra-atrial repairIntraventricular repair

VentriculotomyIncision siteIntracardiac repair

Native valvesLeft ventricular or right ventricular outflow repairLeft ventricular or right ventricular inflow repair

Prosthetic materialsPatchesValvesConduits

Myocardial and endocardial sequelae

Electrophysiological residua are represented byabnormalities that are, with some exceptions, inher-ent components of certain congenital cardiac malfor-mations.12 These residua persist- sometimes harm-lessly, sometimes not so harmlessly -after reparativesurgery. Residual abnormalities of cardiac valvesafter reparative surgery fall into three general cate-gories': 1) congenitally malformed cardiac valves thatare functionally normal and do not require attentionduring reparative surgery, 2) intrinsically normalcardiac valves that are rendered incompetent be-cause of the physiological stress imposed by thecongenital malformation that prompted surgical re-

pair, and 3) residually incompetent or stenotic con-

genitally malformed cardiac valves that do not lendthemselves to complete repair. Residual ventricularabnormalities after reparative surgery are repre-sented by disorders that are permanent, such as theintrinsic morphology of the ventricle, or disordersthat change with the passage of time, such as alter-ations in chamber mass and function. The develop-ment of increased ventricular mass and its regressionafter reparative surgery are important properties ofthe myocardium.1'52-54 Vascular residua after pri-mary repair of congenital cardiac malformations con-sist of anatomic anomalies or defects or elevatedresistance and/or pressure in the systemic or pulmo-nary circulation.' A more than casual relation existsbetween aortic root disease and bicuspid aorticvalves, and on rare occasions the natural history or

postoperative history of bicuspid aortic stenosis isdramatically punctuated by dissecting aneurysm.',55The younger the patient at the time of successfulcoarctation repair, the more likely is the long-termnormalization of postoperative systemic blood pres-

sure.5657 The preoperative status of the pulmonaryvascular bed, especially the resistance vessels, is a

major determinant of the presence and degree ofresidual postoperative pulmonary vascular disease.Early operation sets the stage for normal develop-ment of the pulmonary vascular bed and reduces the

probability that preoperative alterations will betranslated into the undesirable postoperative residuaof increased muscularity of small pulmonary arteries,intimal hyperplasia, and a reduction in the number ofintra-acinar vessels.58 Noncardiac residua can beimportant long-term concerns after reparative sur-gery; these include developmental abnormalitiessuch as mental deficiency, residual somatic defectssuch as dysmorphism or limb abnormalities, preoper-ative medical or psychosocial disorders, and healedbrain abscess that can serve as a focus of a seizuredisorder.1 Cataracts and deafness persist as residuaafter division of a patent ductus in children with therubella syndrome.55

Sequelae of reparative surgery for congenital heartdisease involve electrophysiological mechanisms, na-tive cardiac valves, prosthetic materials, myocardium,and endocardium (Table 6).' Electrophysiologicalsequelae follow intra-atrial repairs, and intraventric-ular repairs via a right atrial incision or through aright ventriculotomy. Sequelae involving native car-diac valves occur after left ventricular or right ven-tricular outflow repairs or left ventricular or rightventricular inflow repairs. Postoperative or post-balloon angioplasty aortic regurgitation as a sequel ofrepair of congenital bicuspid aortic stenosis is a casein point.12 Reconstruction of the right ventricularinflow valve (in Ebstein's anomaly, for example) isnecessarily followed by sequelae that are intrinsic tothe basic congenital tricuspid malformation, even ifcompetence is established. Prosthetic materials rep-resent a special category of sequelae after reparativesurgery for congenital heart disease.' Patches areoften devoid of concern but prosthetic valves andconduits result in sequelae that vary in significanceaccording to the physical and hemodynamic charac-teristics of the materials. There is a fine line betweensequelae and complications.

ConclusionCongenital heart disease in adults has emerged as

a special area of cardiovascular interest, a newsubspecialty.1X2 The accrued benefits have beengratifying but uninterrupted, long-term care is es-sential if the concerns inherent in this increasingpatient population are to be addressed. As GeorgeP. Elliott wrote in the Ametican Scholar, "In sciencegenerally to solve one set of problems may be tocreate or discover a whole new set, and of noscience ... is this more true than of medicine."'59

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KEY WORDS * congenital heart disease * Clinical Progress Series



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J K PerloffCongenital heart disease in adults. A new cardiovascular subspecialty.

Print ISSN: 0009-7322. Online ISSN: 1524-4539 Copyright © 1991 American Heart Association, Inc. All rights reserved.

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