Congenital coronary artery anomalies in young patients: New perspectives for timely identification* Antonio Pelliccia MD

a , ,a

Institute of Sports Science, Department of Medicine, Italian National Olympic Committee, Rome, Italy vaila!le online " #e!ruary $%%&' Major coronary artery anomalies in a pediatric population: incidence and clinical importance Refers to: Journal of the American College of Cardiology, Volume 37, Issue 2, February 2001, ages !"3#!"7, (ulie ' Davis, #ran) Cecc*in, +*omas ,' (ones, Mic*ael ' -ortman
-D# ./&$ ,0 1

Article Outline
2 -revalence in t*e general population 2 Clinical identification 2 Mec*anism.s0 precipitating sudden deat* 2 Clinical management 2 References Sudden and une3pected cardiac deat*s in young su!4ects are rare, !ut tragic events in competitive at*letes assume *ig* pu!lic profile, and t*at unavoida!ly raise 5uestions regarding t*e underlying pat*ological causes and clinical strategies to prevent t*ese catastrop*es 6& and $7' 8arious pat*ological investigations *ave reported t*at several structural cardiovascular a!normalities are responsi!le for sudden deat* in young at*letes, !ut *ypertrop*ic cardiomyopat*y is t*e most common disease in 9'S' autopsy:!ased series, and rig*t ventricular cardiomyopat*y predominates in young Italian at*letes 6&, ;, <, =, ", > and ?7' Nevert*eless, congenital coronary artery anomalies .CC 0 are fre5uently found .in a!out $%@ of cases0, and t*ey represent t*e second most fre5uent disease responsi!le for at*letic field deat*s 6/, &%, &&, &$, &; and &<7' Specifically, t*e wrong sinus coronary artery origin, t*at is, t*e left main artery arising from t*e rig*t anterior sinus . AMC 0, and t*e rig*t coronary artery originating from t*e left sinus . RC 0, wit* a pro3imal course !etween aorta and pulmonary trun), are t*e most fre5uent occurrences of CC ' +*e article !y Davis et al' 6&=7 in t*is issue of t*e Journal descri!es t*e results of prospective ec*ocardiograp*ic identification of wrong sinus coronary anomalies in a large population of c*ildren and adolescents' +*eir contri!ution is remar)a!le, and it is

li)ely to generate new clinical interest regarding t*ese malformations, wit* special attention to t*e issues set fort* !elow'

Prevalence in the general population

-revalence of CC and, specifically, of t*e wrong aortic sinus coronary anomalies in t*e general population is still uncertain' -revious studies suggested t*at t*ese malformations occur in %'$@ to &@ of a large group of patients undergoing cardiac cat*eteriBation 6&"7' Davis et al' 6&=7 in t*e present article report a prevalence of %'&>@, derived from a population of $,;?? c*ildren and adolescents prospectively e3amined !y ec*ocardiograp*y' Cowever, t*eir study population comprised asymptomatic c*ildren and adolescents referred for cardiovascular investigation, and t*us cannot !e considered a true e3pression of t*e DnormalE population' +*e prevalence of wrong sinus coronary malformations in a large and unselected young population s*ould li)ely !e less t*an %'$@' +*is *ypot*esis is also supported !y results of our previous investigation: in a population of &,;"% asymptomatic competitive at*letes, routinely e3amined !y ec*ocardiograp*y, we found no anomalies of origin and course of ma4or coronary arteries, w*ic* is consistent wit* a prevalence of less t*an %'&@ 6&>7'

Clinical identification
Frong sinus coronary anomalies are rarely diagnosed or even suspected during lifeG t*is is !ecause of t*e scarcity of symptoms and inade5uacy of routine diagnostic testing 6? and &?7' -remonitory symptoms, suc* as c*est discomfort, atypical c*est pain, andHor syncope, are reported in less t*an ;%@ of patients 6/, &%, &&, &$, &;, &< and &/7' Symptoms are more li)ely to !e reported !y su!4ects wit* AMC , and t*ey usually occur in association wit* e3ercise, !ut rarely raise clinical suspicion for CC 6&$ and &;7' Indeed, w*en young individuals presenting wit* symptoms are evaluated wit* &$:lead resting and e3ercise electrocardiograp*y, isc*emic c*anges suggestive for CC are rarely detected' In a recent investigation descri!ing t*e anatomical and clinical profiles of young at*letes wit* wrong sinus origin of coronary arteries, Iasso et al' 6&/7 reported t*at all t*e resting &$: lead and e3ercise electrocardiograms .JCKs0 availa!le in t*e &$ su!4ects e3amined during life were wit*in normal limits' Indeed, a review of t*e literature encompassing t*e results of e3ercise electrocardiograms in &? young patients .L;= years0 wit* CC reveals t*at isc*emic c*anges were present in only four su!4ects .i'e', $$@0, including two w*o were already symptomatic 6&/7' Jc*ocardiograp*y, instead, *as t*e potential to address correct diagnosis, !ecause it provides good anatomic definition of t*e ostium and pro3imal epicardial course of coronary arteries' In &,;"% young at*letes prospectively evaluated !y ec*ocardiograp*y we were a!le to visualiBe t*e ostium and pro3imal epicardial course of AMC in />@ and RC in ?%@ of su!4ects 6&>7' Indeed, in young individuals presenting wit* symptoms or a!normal &$:lead JCK, ec*ocardiograp*y provided correct identification of wrong sinus origin of t*e coronary arteries, w*ic* was su!se5uently confirmed !y

coronary angiograp*y 6$%, $& and $$7' +*erefore, t*e present article !y David et al' 6&=7 is outstanding !ecause it demonstrates t*at prospective ec*ocardiograp*ic assessment is an efficient means to detect during life wrong sinus coronary malformations in a large population of c*ildren and adolescents' +*e present study emp*asiBes t*e diagnostic role of ec*ocardiograp*y, and it suggests t*at investigation of ostium and pro3imal course of coronary arteries s*ould !e a routine part of any ec*ocardiograp*ic study' F*en t*e origin of !ot* coronary arteries cannot !e identified !y transt*oracic ec*ocardiograp*y, eit*er transesop*ageal ec*ocardiograp*y or magnetic resonance imaging .MRI0 is recommended 6$;7' #inally, if none of t*e noninvasive tec*ni5ues are successful and suspicion for CC is still *ig*, coronary arteriograp*y is definitive' Cowever, it is unwarranted to generate ent*usiasm and e3pectation for easy noninvasive identification of wrong sinus coronary artery malformationsG false negative results are li)ely to occur wit* transt*oracic ec*ocardiograp*y, and t*e present study reports one of t*ese cases 6&=7' #alse negative results may !e caused !y a poor acoustic window or !y incorrect interpretation of t*e ec*ocardiograp*ic images' -revious studies *ave failed to address t*is 5uestion, and, at present, !ot* t*e sensitivity and t*e specificity of ec*ocardiograp*y for identifying CC in a large and unselected young population *ave still to !e defined'

Mechanism(s) precipitating sudden death

+*e mec*anism.s0 leading to sudden deat* in patients wit* CC are triggered !y myocardial isc*emia' Occurrence of isc*emia is suggested !y pat*ological evidence of acute myocardial damage .small infarcts0 andHor c*ronic in4uries .patc*y areas of myocardial fi!rosis0 in t*e area supplied !y t*e anomalous coronary artery 6&%, &&, &$ and &/7' Isc*emia is t*e conse5uence of anatomical malformations, including t*e acute angle ta)eoff of t*e anomalous vessel, wit* a narrowed slitli)e orifice t*at collapses in a valveli)e manner, t*ere!y limiting t*e !lood flow' Ot*er anatomical features responsi!le for isc*emia are t*e pro3imal intramural course of t*e anomalous vessel, w*ic* is s5ueeBed wit*in t*e aortic wall, and t*e compression of t*e anomalous vessel along its course !etween t*e aorta and t*e pulmonary artery, particularly during e3ercise' Cowever, in a pat*ology analysis t*at comparatively assessed t*e anatomical features of AMC or RC in &$ patients w*o died !y t*ese anomalies and in &? patients w*o died of unrelated causes, +aylor and colleagues 6&;7 found t*at neit*er t*e siBe of t*e ostium, t*e degree of angle ta)eoff, t*e lengt* of intramural aortic course, nor t*e presence of an ostial ridge was predictive for incidence of sudden deat*' F*atever t*e anatomic malformation, patients w*o died suddenly during e3ercise *ave usually done t*e same amount of e3ercise or even more strenuous e3ercise multiple times, wit*out symptoms or p*ysical impairment' It *as !een suggested, t*erefore, t*at isc*emia may !e caused !y sporadic spasm of t*e anomalous coronary artery induced !y endot*elial in4ury 6&<7' not*er mec*anism may !e t*e occurrence of ventricular tac*yarr*yt*mia, on t*e !asis of an electrically unsta!le myocardium' In a few patients !eing monitored wit* JCK at t*e time of t*eir sudden deat*, ventricular fi!rillation was t*e final event 6&% and &<7'

Clinical management
+imely identification of patients wit* wrong sinus coronary anomalies also raises t*e 5uestion of clinical management' +*oug* it is clear t*at not all individuals wit* suc* anomalies are at ris) of sudden cardiac deat* and t*at many patients *ave lived a full life and died of unrelated causes, it is also well )nown t*at t*ese malformations are among t*e most common reasons for sudden and une3pected deat* in young individuals, particularly during sporting activities 6;, <, =, >, ?, /, &%, &&, &$, &; and &<7' +*e paucity of reports descri!ing t*e clinical profile and efficacy of surgical approac* in patients wit* incidentally discovered CC ma)es t*e present recommendations for clinical management still controversial' #or simplicity, we may consider t*e most common instances: &' young patient .L;= years0 wit* une5uivocal diagnosis of wrong sinus coronary malformation, presenting wit* symptoms andHor s*owing evidence of myocardial isc*emia: In t*is case, t*e surgical approac* to repair t*is malformation is mandatory' $' young su!4ect .L;= years0 wit* incidental diagnosis of wrong sinus coronary anomaly, in t*e a!sence of symptoms or signs suggestive of myocardial isc*emia: In t*is case t*ere is uncertainty regarding t*e most appropriate t*erapeutic c*oice' In ma)ing t*is decision, we *ave to consider t*at precise mec*anism.s0 leading to isc*emia and sudden deat* are still un)nown and unpredicta!le, and t*ese patients cannot !e relia!ly stratified wit* regard to ris)' +*erefore, it seems reasona!le t*at t*ese young su!4ects s*ould not !e e3posed to t*e ris) of sudden deat*, and surgical repair of t*e coronary malformation s*ould !e considered' ttention s*ould !e paid to t*e level of t*e patientMs p*ysical activity, since t*e greatest incidence of sudden deat* occurs during *eavy p*ysical e3ertion' C*anging to a completely and c*ronic sedentary lifestyle in c*ildren and adolescents is not a realistic option, and t*e desire to participate in regular *eavy e3ercise or engage in competitive at*letics represents a furt*er indication for surgical repair' Ceavy e3ercise programs and competitive sports s*ould !e clearly discouraged in young patients wit* CC , at least until surgical correction is performed 6 $< 7' ;' n adult or older patient wit* incidental diagnosis of wrong sinus coronary malformations, in t*e a!sence of symptoms and induci!le isc*emia: In consideration t*at sudden deat* occurs at a young age, t*is occasional finding *as li)ely no clinical significance and most pro!a!ly needs no surgical t*erapy' Cowever, !ecause t*e magnitude of t*e ris) remains un)nown, individualiBation of t*e t*erapeutic c*oice is appropriate' #inally, t*is aut*or !elieves t*at it is now timely and appropriate to esta!lis* an international registry of prospectively identified young patients wit* CC in order to ac*ieve a !etter understanding of t*e clinical profile and t*e impact of surgical correction on t*e natural *istory of t*ese malformations'

eferences