Computed tomography coronary angiography should be performed in all patients with Marfan Syndrome prior to aortic root replacement

Abstract

Dear Editor,Aortic dissection is the most common cause of death in the naturalhistory of patients with Marfan Syndrome [1]. To prevent death due toacute aortic dissection, indications for aortic root replacement are muchless restrictive in patients with Marfan Syndrome, compared to the gen-eral population [2,3]. The number of young Marfan patients undergoingaortic root surgery is steadily increasing [4]. With increasing numbers ofpatients in the operating theater comes an increasing prevalence ofperi-operative complications.In our clinic, three patients with Marfan Syndrome who had toundergo aortic root replacement were diagnosed with an aberrantcoronary artery, of whom only one pre-operatively. All three patientsunderwent aortic root replacement for progressive aortic root dilata-tion;withamaximumdiameterof50mm.Noneofthepatientsexperi-encedanycardiaccomplaints.Atthetimeofsurgery,twopatientswereon β-blockade, oneincombination withanangiotensin II (AT II)recep-tor blocker, and one patient was not on any medication, as β-blockadewas poorly tolerated, and the beneficial effects of ACE inhibitors andAT II receptor blockers had not yet been proven at the time. In allthree patients the ramus circumflexus (RCX) was found to arise fromthe right coronary artery (RCA).In patient A (male, 28 years old, surgery in 2008), the aberrantcoronary artery was diagnosed on transesophageal echocardiographyintheoperatingtheaterunderanesthesia(Fig.1A).Surgerywasabortedto perform computed tomography (CT) coronary angiography, afterwhichasuccessfulDavidprocedurewasperformed.Thebuttonholdingboth the RCA and the RCX was re-implanted in the commissurebetween the right- and the non-coronary cusps without complication.In patient B (female, 43 years old, surgery in 2009), the aberrant coro-nary artery had not been diagnosed pre-operatively, and was uninten-tionally sliced during surgical dissection of the right coronary button.Fortunately, the RCX could successfully be re-implanted at the locationof the non-coronary cusp. In retrospect, pre-operative transesophagealechocardiographydidrevealtheaberrantRCX,buthadbeenoverlooked(Fig. 1B). To prevent such major complications during future surgery,we introduced CT coronary angiography in all Marfan patients prior toaortic surgery. Therefore, in patient C (male, 31 years old, surgery in2013),anaberrant RCX arisingfromthe RCAwasfound on CT coronaryangiographypriortosurgery(Fig.2).Inthispatient,aorticrootreplace-mentwassuccessfullyperformed,withre-implantationofthecoronaryarteries.In the general population, about 1.1% of individuals have an aberrantcoronary artery [5]. These numbers could somewhat underestimate thereal prevalence, as malign coronary anatomy may result in suddencardiac death prior to diagnosis. Our population consisted of 54 patientswho underwent surgery between 2003 and 2013, in which threepatients (5.6%, CI 95% −0.6% to 11.8%) were diagnosed with an aberrantcoronary artery. This higher percentage could well be incidental, asabsolute patient numbers are small. Currently, no data have been pub-lishedindicatingthatpatientswithMarfanSyndromehaveahigherprev-alence of aberrant coronary anatomy. On the other hand, the chance of aMarfan patient undergoing aortic surgery during his/her lifetime is muchgreater than in the general population, increasing the relative risk to en-counter coronary anomalies in these patients.Current European guidelines recommend cardiovascularmagnetic resonance (CMR) in all Marfanpatients to evaluate the entireaorta.Moreover,ifevaluationofcoronaryarterydiseasepriortosurgeryis warranted, CT coronary angiography is recommended, as catheter