CASE REPORTS

Familial Congenital Complete Heart Blockand Maternal Systemic Lupus Erythematosis

ROBIN B. WINKLER, M.D., AUDREY H. NORA, M.D.,AND JAMES J. NORA, M.D.

SUMMARY A family is reported in which two siblings had con-

genital complete heart block with resultant congestive heart failure,the father and paternal grandfather show adult-onset conductiondefects, and the mother has systemic lupus erythematosis. The in-teraction of heredity and environment is discussed in this context. A

FAMILIAL COMPLETE HEART BLOCK (CHB) hasbeen described by a number of observers since the report byMorquio' in 1901. Among those reported are thirteenfamilies with multiple cases of well-documented congenitalCHB2-14 using the classic criteria of Yater'5 and eliminatingthose with intracardiac defects. More recently, an associa-tion has been noted between congenital CHB and maternalconnective tissue disease (CTD), particularly systemic lupuserythematosis (SLE).'2 We would like to report a kindredrevealing a genetic-environmental interaction between theabove factors, which has not been noted in the medicalliterature. Previously reported cases of familial block will beanalyzed in the light of this new information.

Case Reports

The proband of our kindred (case 1, W.S.) prompted an

investigation which has revealed previously unrecognizedfactors in the death of a sibling and in surviving familymembers. Paternal transmission of CHB through threegenerations and maternal SLE were uncovered. The fivemost informative family members will be discussed (seefig. l).

Case 1. W.S., the proband (IV-2 in fig. 1), is a whitefemale infant. Fetal bradycardia at a rate of 70/min was

noted at 24 weeks gestation. At no time was the fetal heartrate normal. The infant was delivered by elective repeatCaesarean section at term with Apgar scores of 6 at both 1

and 5 minutes. Physical examination revealed a vigorousacyanotic infant with a left parasternal lift and a gradeII/VI harsh systolic murmur at the upper left sternal border.S, and S2 were normal for age. The liver edge was palpable 6cm below the right costal margin and the spleen edge 3 cmbelow the left costal margin. Electrocardiogram (ECG)showed complete heart block with atrial rate 150/min, ven-

tricular rate 68/min, and right atrial enlargement and rightventricular hypertrophy. Chest X-ray showed marked car-

review of the literature on familial complete heart block suggests thatso-called pure congenital-onset familial heart block, originally felt tobe genetic, may in fact have an important environmental component,specifically related to ongoing maternal factors such as systemiclupus erythematosis.

diomegaly and prominent pulmonary vascularity. Echocar-diogram showed normal anatomy and mild left atrialenlargement. A diagnosis of congenital CHB with con-

gestive heart failure was made, and the patient was treatedwith digoxin and furosemide. A gradual improvement in thecardiomegaly and visceromegaly was noted. The hospitalcourse was complicated by thrombocytopenia with a plateletcount of 40,000 at birth rising to 195,000 at 8 days of age.No bleeding diathesis was noted and no specific therapygiven.At 13 days of age a rash appeared on the head and within

one week became generalized (fig. 2). The lesions were an-

nular erythematous patches with central clearing. Skin biop-sy showed liquefaction degeneration of the basal zone, con-

sistent with discoid lupus erythematosis, but with negativeimmunofluorescence. Serum complement was normal at 54,and antinuclear antibody (ANA) speckled positive at a titerless than 1:64. A diagnosis of congenital lupus erythe-matosis was made. She was treated with topical steroidswith gradual resolution of the rash.

Case 2. B.S. (111-7 in fig. 1), mother of W.S. and T.S., is a28-year-old white female, now gravida 2 para 2 livingchildren 1. She has a history of hypothyroidism withoutthyroiditis since age 15 and currently takes synthroid 0.5 mgdaily. Since age 5 she has had intermittent arthralgias andmorning stiffness, and at 18 had an exacerbation of thesesymptoms accompanied by low grade fever. During her sec-

ond pregnancy (see case 1), ANA was negative. When thediagnosis of congenital lupus was made on W.S., furtherevaluation showed the following: ANA strongly positive at1:256; sedimentation rate 46; rheumatoid factor positive at1:1160; skin biopsy with immunofluorescence speckledpositive; and VDRL negative. A diagnosis of SLE withminimal clinical expression was made. As the patient was

adopted in infancy, her family history is unknown and no

relatives are available for study.Case 3. Two years prior to the birth of our proband, a

female sibling, T.S. (IV-1 in fig. 1), was delivered byemergency Caesarean section for fetal bradycardia at60-80/min. Previous fetal heart rate was not reported as ab-normal. Apgar scores were 1 at one minute and 5 at fiveminutes, and the infant was grossly meconium stained. Ex-amination revealed cyanosis, marked respiratory distress,

1103

From the Department of Pediatrics, University of Colorado MedicalCenter, 4200 East Ninth Avenue, Denver, Colorado.

Supported by Grant 05981 from the National Institutes of Health.Address for reprints: Robin B. Winkler, M.D., Department of Cardiology,

Children's Hospital Medical Center, Boston, Massachusetts 02115.Received July 5, 1977; revision accepted July 29, 1977.

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VOL 56, No 6, DECEMBER 1977

11

Ill

IV

ElNormal ECG

Conduction defect

Lupus erythematosus

FIGURE 1. Pedigree of "S' family

diffuse petechiae and ecehymoses, a grade I-II/VI systolicmurmur at the left sternal border, and massive hepato-splenomegaly. Laboratory studies revealed anemia, hypox-emia, mixed respiratory and metabolic acidosis, and dis-seminated intravascular coagulation. The chest X-rayshowed marked cardiomegaly with bilateral pulmonary in-filtrates and the ECG revealed complete heart block with anatrial rate of 120/min and a ventricular rate of 58/min.Despite vigorous medical intervention including mechanicalventilation, exchange transfusion, parenteral antibiotics andpressor agents, the infant died at 21 hours of age.

Postmortem examination revealed large amounts ofmeconium in the alveoli and terminal airways. The spleenand liver were severely enlarged with extensive extra-medullary hematopoiesis and iron deposition. Granulationtissue without acute infiltration of leukocytes was found sub-pericardially and there were scattered subendocardialcalcium deposits. The conduction system was not studied.Viral and bacterial cultures were negative and viral titers un-remarkable.Case 4. J.S. (111-6 in fig. 1), father of W.S. and T.S., is a

26-year-old white male whose past medical history is un-remarkable, with the exception of episodes of asympto-matic bradycardia at 40/min during his adult years. No ab-normal cardiac rhythms, heart murmurs, or syncopalepisodes were noted in childhood. The ECG currently showsa wandering atrial pacemaker with a rate varying from 47 to75/mmn, PR interval 0.16 sec and QRS duration of 0.06 sec.Physical examination is unremarkable.Case 5. A.S. (11- I in fig. 1), father of J.S., is a 50-year-old

white male who presented at age 41 with palpitations and ex-ertional dyspnea. He had no previous history of cardiac orcoronary artery disease or symptoms. On examination hehad a regular heart rate at 30/min with cannon waves in theneck veins. A grade II/VI systolic murmur was heard alongthe left sternal border. The ECG was highly variable, with attimes normal sinus rhythm, 2: 1 and 3: 1 atrioventricularblock and complete heart block. Conduction beyond theA-V node varied, including normal QRS, right bundle

branch block (RBBB) with left inferior hemiblock, and leftbundle branch block. Because of a history of pneumonia re-sponding to penicillin 5 months earlier, a presumptive diag-nosis of myocarditis was made and treatment with steroidsbegun. However, he returned one month later with near-syncopal attacks and complete heart block with a rate of23/min, and a permanent demand pacemaker was im-planted. Since that time he has done well on no medica-

FIGURE 2. Discoid lupusfirst appeared on theface ofthe probandat 13 days of age.

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FAMILIAL CHB AND LUPUS/Winkler et al.

tions, and intermittently overrides the pacemaker with nor-mal sinus rhythm and left bundle branch block.

Additional data on this kindred reveals the following (seefig. 1): III-1 and III-2, brothers of J.S. (III-6), arereportedly healthy and have normal ECGs. III-5, sister ofJ.S., is reportedly healthy, and shows ECG abnormalitiessimilar to those of her brother, i.e., wandering atrialpacemaker and rate varying from 47/min to 75/min. 1-1,father of A.S. (II-1), was not known to have heart diseasebut died suddenly in his early 50s. Patients 111-3 and III-4have not been examined.

Discussion

Familial conduction defects have been well documented inthe medical literature, with the available data ranging frompre-electrocardiographic clinical findings within an im-mediate family2 to studies involving over 250 members of asingle kindred"6 and His bundle electrograms, histo-pathology and intracellular electrophysiology.'7 The ma-jority of affected individuals involved have shown onset ofarrhythmias in the fourth decade of life or later, withprogressive changes and conduction defects distal to thebundle of His, such as right bundle branch block.

In 13 of the reported kindreds, however, heart block wascongenital. These cases were characterized by normal widthand configuration of the QRS complex, absence of progres-sive changes, and where postmortem studies were obtained,pathologic changes at the His bundle or between it and theA-V node with no involvement of more distal conductiontissue. These data have led Sarachek and Leonard"8 topostulate two types of familial heart block, the adult-onsetand congenital types, differing both in etiology and in ex-pression. They suggested that the mode of genetic transmis-sion for both familial types is autosomal dominant withvariable expressivity, although data in the congenital grouphave been felt by many authors to be inadequate for geneticanalysis.'8 'g In a collaborative international study,Michaelson and Engle20 reported that 13 of 14 families withcongenital complete heart block conformed to an autosomalrecessive mode of inheritance. In two families there werethree affected siblings.

Recently a newly recognized predisposing factor to con-genital heart block has come to light: maternal connectivetissue disease. Chameides et al." reported five children withcongenital CHB born to four mothers with definite SLE,and three other questionable cases. Included in his report isone family with two affected siblings without other knownconduction defects in the propositi or their relatives. McCueet al.'4 '5 reported 14 infants with congenital CHB of 11mothers with clinical and/or serological evidence of connec-tive tissue disease. Both authors suggest that maternal SLEmay be contributing to a heretofore unrecognized degree tothe population of patients with congenital CHB. In the latterstudy, in fact, 22 patients were screened to determine the 14with this association, suggesting a large contribution fromCTD.We postulate that the "SS" family represents the interac-

tion of two predisposing factors: genetic (paternallytransmitted conduction defects apparently of the adult-onsettype) and environmental (maternal SLE), resulting in the

heart failure with severe hepatosplenomegaly and thrombo-cytopenia. In the case of the second child, W.S., the presence

of maternal SLE is definite and there is strong evidence ofcongenital SLE in this patient.As the association was not looked for in the earlier case,

T.S., one can only postulate the presence of subclinical SLEin the mother at that time. The negative ANA obtained on

the mother following that pregnancy does not rule out thatpossibility, as serologic findings can vary widely for a givenpatient at different times in her obstetrical history.2" 22

McCue suggests that the transplacental transmission of ab-normal maternal antibodies suspected of damaging the fetalconduction system may occur "even prior to the develop-ment of clinical maternal SLE or CTD."'14 Hogg,25 in 1957,reported a case of congenital SLE and CHB in an infantwhose mother did not demonstrate findings of SLE until 11

months postpartum. In addition, this mother's medicalhistory includes symptoms suggestive of CTD antedatingboth pregnancies by several years. The postmortem findingsin T.S. (with the exception of meconium aspiration, the im-mediate cause of death) are markedly abnormal but non-

specific. They suggest severe intrauterine hypersplenism,possibly secondary to congestive heart failure, and representa more extreme case of the similar clinical findings whichoccurred in W.S. and also in a case reported by McCue. Un-fortunately, neither conduction system dissection nor im-munofluorescence were performed. The presence of suben-docardial calcific deposits suggests an inflammatory process

involving the conduction system, and we question whetherthe unusual epicardial pathology may also reflect a noninfec-tious inflammatory process, such as that seen in some cases

of lupus pericarditis.24 25

The importance of genetic-environmental interactions inthe production of congenital heart disease has beenrecognized for some time.26 A similar interaction has beenproposed in the etiology of adult-onset familial heart block,in which a genetic predisposition is manifested only in thepresence of the normal subendocardial fibrosis of aging. Wepropose such an interaction in the production of congenitalCHB in the "S" family. The environmental influence in thiscase is postulated to be the presence of abnormal maternalantibodies attacking a genetically vulnerable conductionsystem at a critical period in fetal development. The actionof antibody as a primary teratogen has been suggested in theetiology of structural cardiac anomalies27 as well as con-

genital CHB.'2lWallgren,6 in 1960, postulated the involvement of a

maternal immunological response to explain the presence ofcongenital CHB in all three live births to one mother with noconduction defects in other generations. However, he dis-missed this possibility as unlikely. A review of the reportedkindreds with familial congenital CHB, excluding cases withassociated intracardiac structural defects (table 1), revealsthat 9 out of 14 such kindreds show multiple involvementamong siblings without involvement in any other genera-tion. Included is the family reported by Chameides et al.'2with definite maternal SLE, the families reported byMcCue'4 with definite maternal SLE, and that reported byWright5 in 1959 where it is incidentally mentioned that themother of the three affected siblings died of SLE. A fifthkindred, reported by Aylward2 in 1928 with electrocardio-

birth of two infants with congenital CHB, which caused

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graphic co-nfirmation by Aitken211 in 1932, mentions the

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VOL 56, No 6, DECEMBER 1977

TABLE 1. Reported Cases of Familial Congenital Complete Heart BlockNumber of Other Conduction defects Maternal

Author affected sibs affected relatives other than CHB CTD

Aylward' (1928) 2 0 0 Mikulicz's syndromeWright5 (1959) 3 0 0 SLEWallgren GI (1960) 3 0 0Lynch7 (1961) 2 0 0Crittenden" (1964) 4 (?5) 0 0James" (1975) 2 0 0Chameides'2 (1976) 2 0 0 SLEMcCue'4 (1977) Case 2 2 0 0 SLE

Case 3 2 0 1° AV block SLE

Wallgren AJ3 (1937) 1 father, pat. aunt RBBBWendkos4 (1947) 2 father WPW/PATGazes' (1965) 1 mother, mat. aunt, LAD, RBBB,

mat. uncle, mat. cousin, 1c and 2° AV blockmat. grandfather

Veracochea'5 (1967) 1 father, brother LAD, 2° AV block"S" family 2 father, pat. grandfather, RBBB, LBBB, SLE

pat. aunt WAP, acquired CHBAbbreviations: CHB = complete heart block; CTD = connective tissue disease; SLE = systemic lupus erythematosus; RBBBright bundle branch block; LBBB = left bundle branch block; WPW = Wolff-Parkinson-White syndrome; PAT = paroxysmal

atrial tachyeardia; WAP = wandering atrial pacemaker.

presence of maternal Mikulicz's syndrome, now felt tobelong to the spectrum of connective tissue diseases andfrequently associated with SLE.21 22, 29 This raises the ques-tion of whether the other four single-generation familieswere also victims of an ongoing environmental influencesuch as maternal SLE.A review of the remaining five kindreds with multiple-

generation involvement (including the "S" family reportedhere) reveals yet another pattern. All of the families shownot only congenital CHB, but also other conduction defects(acquired CHB, RBBB, left anterior hemiblock, Wolff-Parkinson-White syndrome, wandering atrial pacemaker)usually associated with adult-onset type of familial block.These kindred could have adult-onset type familial blockwith variable expressivity or diverse and progressivepathology following some initial insult.30 31 A unifying con-cept is that these families may represent a genetic-environmental interaction such as that seen in the "S"family.A number of authors have observed the poor prognosis

associated with the familial form of congenital CHB as op-posed to the nonfamilial sporadic form.", 1' No explanationhas been put forth for this discrepancy. If, however, thisfamilial form does represent an immunological insult, onecan postulate a prognosis worse than that in isolatedidiopathic congenital CHB, based on evidence for involve-ment extending beyond a focal area of the conductionsystem. Certainly a portion of the isolated cases of con-genital CHB also reflect maternal CTD, but a higherproportion of these cases would be seen in multiply-affectedfamilies. In addition, the very presence of multiple involve-ment in such families may reflect a more severe response tothis immunological teratogen, producing greater morbidityand mortality in the affected individuals.

Acknowledgment

We would like to thank Dr. Antonio Martinez-Hernandez for his assistancein interpreting the pathological specimens in case 3.

References1.Morquio L: Sur une maladie infantile et familiale characterisee par des

modifications permanentes du pouls, des attaques syncopales et epilep-tiformes et la mort subite. Arch Med Enf 4: 467, 1901

2. Aylward RD: Congenital heart-block. Br Med J 1: 943, 19283. Wallgren AJ, Winblad S: Congenital heart-block. Acta Paediatr Scand

20: 175, 19374. Wendkos MH, Study RS: Familial congenital complete A-V heart block.Am Heart J 34: 138, 1947

5. Wright FS, Adams P, Anderson RC: Congenital atrioventricular dis-sociation due to complete or advanced atrioventricular heart block. Am JDis Child 98: 72, 1959

6. Wallgren G, Agoria E: Congenital complete A-V block in three sibs.Acta Paediatr Scand 49: 49, 1960

7. Lynch RJ, Engle MA: Familial congenital complete heart block. Am JDis Child 102: 210, 1961

8. Crittenden IH, Latta H, Ticinovich DA: Familial congenital heart block.Am J Dis Child 108: 104, 1964

9. Gazes PC, Culler RM, Taber E, Kelly TE: Congenital familial cardiacconduction defects. Circulation 32: 32, 1965

10. Veracochea 0, Zerpa F, Morales J, Hernandez 0, Waich S: Pacemakerimplantation in familial congenital A-V block complicated by Adams-Stokes attacks. Br Heart J 29: 810, 1967

11. James TN, McKone RC, Hudspeth AS: Familial congenital heart block.Circulation 51: 379, 1975

12. Chameides L, Truex R, Vetter V, Rashkind W, Noonan J: The associa-tion of maternal systemic lupus erythematosus with congenital heartblock. (abstr) Circulation 53 (suppl II): II-54, 1976

13. McCue CM, Mantakas ME, Tingelstad JB, Ruddy S: Congenital heartblock in newborns of mothers with connective tissue disease. (abstr)American Academy of Pediatrics 45th annual meeting, October 1976

14. McCue CM, Mantakas ME, Tingelstad JB, Ruddy S: Congenital heartblock in newborns of mothers with connective tissue disease. Circulation56: 82, 1977

15. Yater WM: Congenital heart block: Review of the literature; report of acase with incomplete heterotoxy. The electrocardiogram in dextrocardia.Am J Dis Child 38: 112, 1929

16. Lynch HT, Mohiuddin S, Moran J, Kaplan A, Sketch M, Zencka A,Runco V: Hereditary progressive atrioventricular conduction defect. AmJ Cardiol 36: 297, 1975

17. Amat-y-Leon F, Racki AJ, Denes P, TenEick RE, Singer DH, Bharati S,Lev M, Rosen KM: Familial atrial dysrhythmia with A-V block. Circula-tion 50: 1097, 1974

18. Sarachek NS, Leonard JJ: Familial heart block and sinus bradycardia.Am J Cardiol 29: 451, 1972

19. Waxman MB, Catching JD, Felderhof CH, Downar E, Silver MD, Ab-bott MM: Familial atrioventricular heart block: an autosomal dominanttrait. Circulation 51: 226, 1975

20. Michaelson M, Engle ME: Congenital complete heart block: an inter-national study of the natural history. Cardiol Clin 4: 85, 1972

21. Dubois EL: Lupus Erythematosis, ed 2. Los Angeles, University ofSouthern California Press, 1974, pp 373-379

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22. Harrison TR: Harrison's Principles of Internal Medicine, ed 8, edited byThorn GS. New York, McGraw-Hill, 1977, p 429

23. Hogg GR: Congenital acute lupus erythematosis associated with suben-docardial fibroelastosis. Am J Clin Pathol 28: 648, 1957

24. Brigden W, Bywaters EGL, Lassof MH, Ross IP: The heart in systemiclupus erythematosus. Br Heart J 22: 1, 1960

25. Griffith GC, Vural IL: Acute and disseminated lupus erythematosis. Cir-culation 3: 492, 1951

26. Nora JJ: Multifactorial inheritance hypothesis for the etiology of con-genital heart diseases. Circulation 38: 604, 1968

27. Nora JJ, Weishuhn EJ, Bourland BJ, Watson SC: Fluorescent antiheart

IgM and raised levels of serum IgM in newborns with congenital heartdiseases. Br Heart J 36: 167, 1974

28. Aitken JK: Congenital heart block. Lancet 2: 1375, 193229. Morgan WS: The probable systemic nature of Mikulicz's disease and its

relation to Sjogren's syndrome. N Engl J Med 251: 5, 195430. James TN, Spencer MS, Kloepfer JC: Adult-onset syncope with com-

ments on the nature of congenital heart block and the morphogenesis ofthe human atrioventricular septal junction. Circulation 54: 1001, 1976

31. Levy AM, Camm AJ, Keane JF: Multiple arrhythmias detected duringnocturnal monitoring in patients with congenital complete heart block.Circulation 55: 247, 1977

Truncus Arteriosus Communis

Unusual Case Associated with Transposition

PAOLO ANGELINI, M.D., ALFREDO LLOVET VERDUGO, M.D., JAIME PEY ILLERA, M.D.,

AND ROBERT D. LEACHMAN, M.D.

SUMMARY A child with truncus arteriosus communis, charac-terized by the posterior origin of an individualized pulmonary trunk ispresented. This relationship between the great arteries is unusual in

VARIETIES OF COMMON TRUNCUS have been pre-viously discussed in the literature.1'5 For classification pur-poses, the length of the main pulmonary trunk and its pointof origin from the common trunk have been most frequentlyutilized. It has been suggested' that the type of commontruncus, with a persisting segment of the pulmonary artery(type I of Collett and Edwards), is embryologically derivedfrom the partial failure of completion of truncal septation.

Depending upon the length of the main pulmonary arterysegment and its position with reference to the aortic portionof common trunk, it might be possible to identify truncusarteriosus in which the aorta and pulmonary artery rem-nants are in the position usually identified as transposition ofthe great arteries. This type of great vessel arrangement hasnot been reported in truncus arteriosus.The present case report is illustrative of what we believe to

be the simultaneous presence of "common truncus" andtransposition of the great vessels.

Case Report

A five-year-old child was admitted to Texas Children'sHospital for evaluation of congenital heart disease. He wasessentially asymptomatic, but known to have a complicatedheart anomaly from previous venous angiographic study.On physical examination the child was well developed

and had no signs of congestive heart failure. The bloodpressure was 90/60 in both arms. There was evidence of mildcardiomegaly with a right ventricular heave palpable at theleft lower parasternal area. A grade 2 systolic ejection mur-

From the Texas Heart Institute, Houston, Texas, and Escuela NacionalEnfermedades de Torax, Madrid, Spain.

Address for reprints: Dr. Robert D. Leachman, St. Luke's EpiscopalHospital, P.O. Box 20269, Houston, Texas 77025.

Received May 9, 1977; revision accepted July 6, 1977.

truncus arteriosus communis and the spatial orientation resembles thatseen in transposition of the great vessels. A brief discussion is pro-posed about a proper terminology in this type of complex anomaly.

mur began immediately following an ejection click and washeard best in the pulmonary area. The second heart soundwas single. No diastolic murmurs or sounds were heard. Theelectrocardiogram was interpreted as regular sinus rhythmwith evidence of right ventricular hypertrophy (fig. 1). ByX-ray examination, the heart was slightly enlarged, withoutselective chamber enlargement. The aortic arch was on theleft side. The vascular pedicle was narrow. The pulmonaryvascular shadows were large near the mediastinum, butsmall near the periphery of the lungs (fig. 2).

Heart catheterization data are presented in table 1.A large ventricular septal defect was seen in the angio-

grams below a single overriding semilunar valve. A singlearterial vessel of short length emerged from the heart anddivided into two vessels; one with the characteristics of anascending aorta and one with those of a pulmonary artery.The main pulmonary artery arose posteriorly from the com-mon trunk and had a 2 cm long undivided segment that wasobscured by the ascending aorta in the postero-anterior pro-jection and was seen to be completely posterior to the aortain the lateral projection (fig. 3).The final diagnosis was trunco-conal septal defect (com-

mon truncus arteriosus) with transposition of the dividedportion of the great vessels and pulmonary vascular obstruc-tive disease.

In view of the high pulmonary resistances (ratio ofpulmonary to systemic resistances equal to 0.78) this childwas not considered a suitable candidate for corrective sur-gery.

Discussion

In most anatomic specimens of common truncusarteriosus, type I of Collett-Edwards,2 the longer the mainpulmonary artery trunk, the more lateral and anterior is its

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R B Winkler, A H Nora and J J NoraFamilial congenital complete heart block and maternal systemic lupus erythematosis.

Print ISSN: 0009-7322. Online ISSN: 1524-4539 Copyright © 1977 American Heart Association, Inc. All rights reserved.

is published by the American Heart Association, 7272 Greenville Avenue, Dallas, TX 75231Circulation doi: 10.1161/01.CIR.56.6.1103

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