Journal Geriatric Cardiology;9-2004,Coronary artery anomalies: prevalence and clinical profile in elderly patients by Gianluca Rigatelli,Giorgio Rigatelli ,, Mario Trivellato ... page 40

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Coronary artery anomalies: prevalence and clinical profile in elderly patients.

Gianluca Rigatelli ¹ , MD,FCCP, FSCAI, FESC, FACC, Giorgio Rigatelli ², MD, FSCAI, FESC, Mario Trivellato ³, MD, FACC, FSGC.
      ¹ E.C.V.T.R., Legnago, Verona, via T Speri 18, 37040 Legnago, verona, Italy, Fax: +39044220164, email: jackyheart@hotmail.com
      ² Department of Specialistic Medicine, “Mater Salutis” Legnago General Hospital, Legnago, Verona
      ³ Cardiology Unit, Geriatric Hospital, Padua, Italy

Running title: coronary artery anomalies

Address for correspondence to :
      Gianluca Rigatelli, MD, FANMCO, FASA, FSCAI, FESC, FACC
      EndoCardioVascular Therapy Research, Legnago, Verona
      Via Tito Speri, 18
      37040 Legnago, Verona, Italy
      phone: +39044220164
      E-mail: jackyheart@hotmail.com

Objectives . Although congenital heart diseases are uncommon in the elderly, coronary artery anomalies may be incidentally discovered in old age. We sought to determine the incidence and clinical features of coronary artery anomalies (CAAs) in patients over 65 years of age.

Patients and methods Medical records of patients undergoing coronary artery angiography in the years 1997-2002 at the Legnago General Hospital were retrospectively reviewed. The clinical profiles of all patients with CAAs and CAA subtypes were noted. Comparison between patients under and over 65 was performed. Data are given as mean standard deviation and as percentages.

Results. Sixty-six patients (1.21%, Female/Male 22/44, mean age 65.3±10.6 years) out of the 5450 who underwent coronary angiography in the years 1997-2002 at the Legnago General Hospital had CAAs. In most cases (63%, 41/66 patients), the patients were over 65. CAAs were discovered incidentally in these elderly patients while undergoing coronary artery angiography for dilated cardiomyopathy, ischemic heart disease, and valvular heart disease in 75% of the cases (30/41 patients) . Patients over 65 had more cardiac comorbidities and a higher incidence of coronary atherosclerosis.

Conclusions. The angiographic incidence of CAAs in elderly patients is increasing as the population ages and this occurrence calls for a wider knowledge of the anatomy and pathophysiology of CAAs among geriatric cardiologists. Elderly patients seem to present with lower risk coronary anomalies (separated origin of left anterior descending coronary artery and circumflex artery, origin of circumflex artery from the right sinus or the right coronary artery, double coronary artery) but have a higher risk profile compared to younger patients due to the frequency of cardiac comorbidities and superimposed coronary artery atherosclerosis.

Key words : coronary artery anomaly, congenital heart disease, coronary artery angiography.

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Introduction Coronary artery anomalies (CCAs) occured in 0.64 % to 5.6% of patients undergoing coronary angiography ^1-7. Although congenital heart diseases are uncommon in the elderly, CAAs are probably the most frequent forms of congenital heart diseases in old age, together with interatrial communications and bicuspid aorta. No data exist about their prevalence in the elderly, despite a number of case reports in Medline of patients with CAAs over 65 ^8-14 . Unfortunately, the clinical relevance of different coronary artery anomaly subtypes is unclear and in the elderly many factors such as the presence of concomitant ischemic heart disease, heart failure, and several other co-morbidities increase the confusion about their proper management and therapy. In this retrospective study the authors attempt to define the prevalence and clinical significance of CAAs in the elderly.

Patients and methods

Medical records of patients undergoing coronary artery angiography in the years 1997-2002 at the Legnago General Hospital were retrospectively reviewed. The clinical profiles of all patients with CAAs and CAA subtypes were documented. Calculations comparing patients <65 years and >65 were performed. Data are given as mean standard deviation and as percentages. Fisher’s exact or Chi-square test was used for data comparison. Statistical significance was set at p<0.05.

Results

Sixty-six patients (1.21%, Female/Male 22/44, mean age 65.3±10.6 years) of the 5450 consecutive patients who underwent coronary angiography had CAAs. In two thirds (63%, 41/66 patients), the patients were over 65 (Table I). Standard cardiovascular risk factors did not differ significantly in patients >65 and <65 years. CAAs were discovered incidentally in these patients whilst undergoing coronary artery angiography for dilated cardiomyopathy, silent ischemia, and valvular heart disease in 75% of the cases (30/41 patients) . In the other 11 patients coronary angiography was performed for CAA-related myocardial infarction in 2 patients, CAA-related non ST-elevation ACS in 8 , and for ventricular arrhythmias in the other 1 patient. Moreover, these patients over 65 had more cardiac comorbidities and a higher incidence of superimposed coronary atherosclerosis (table II). Twenty-one patients (%) required aggressive management: 6 patients undewent percutaneous revascularization, and 15 underwent surgical revascularization including those with combined valvular replacement.

Discussions

In our review of the literature, we can find sufficient data about the most frequent CAAs . The most frequent CAAs are usually clinically silent ⁵ and are unrelated per se to myocardial ischemia and sudden death, although they are often observed to have various degrees of atherosclerotic disease ^{5, 8, 15-16}.

Ectopic coronary origin from the ascending aorta ⁸, intercoronary communication ⁴, and separated origin of LAD and LCx ⁵, all cause no hemodynamic impairment and should be considered “benign”. In the same way, the ectopic origin of the LCx from the RCA or right sinus of Valsalva , probably the most frequent CAA, can be considered benign and is relevant only because it could be accidentally compressed during surgery for valve replacement.

Table 1. Incidence of most common CAAs in the authors’experience: comparison between > and < 65 years patients .

CAA : coronary artery anomaly; LAD: left anterior descending coronary artery; LCx: left circumflex coronary artery; LCA: left coronary artery; RCA: right coronary artery. Fisher's exact test has been used for percentages comparison. Significativity was set to p <0.05.

*Percentages refer to the whole case series of 5450 patients.

Table II. Related cardiac comorbidities in elderly patients in the authors’ series

ns : not significant

Dual descending coronary artery, classified as subtypes I to IV following Spindola-Franco classification ¹⁷, is not associated per se with unfavorable events in the absence of coronary atherosclerosis¹⁸; neither are myocardial bridges with score < 4 despite the possibility of episodic ischemia ¹⁹.

Some specific CAA subtypes, usually correlated to fixed or episodic myocardial ischemia in the absence of CAD, have a benign course. Some example are coronary artery fistulas which are clearly correlated to the development of myocardial ischemia ^20-21, the single coronary artery subtype R or L, I-II-III, A-P ²² and atresic coronary arteries normally correlated to congenital syndromes such as Rubeolla syndrome, Hurler’s syndrome and Friederich’s ataxia syndrome ^23-24 .

CAAs in origin, course and distribution have recently been suggested as the main cause of 5-35% of sudden deaths in young people ²⁵, whereas an acute take off angle and the presence of coronary ostial valve-like ridges have been proposed as an important cause of sudden death in the absence of CAD ^26-27 .

The L II-III B subtypes of single coronary artery are very rare conditions in which the whole coronary circulation is supported by an anomalous RCA originating from a normal LCA and usually passes between the pulmonary artery and aorta constituting a potential risk ^28-29.

Myocardial bridges with score ≥4 are likely to be prone to myocardial infarction and sudden death ^30-31: Coronary spasm or intravascular clotting are likely to be due to additional pathophysiological changes which are capable of exacerbating neurogenic or autocrine changes in the vessels.

Ectopic origin of LCA from the right sinus of Valsalva is differently significant depending on the origin and its relationship with the aorta and pulmonary artery: “septal” subtype is the most common finding, while “between” one was rare but often dangerous ³². Ectopic origin of the RCA from the left sinus of Valsalva, due to its pathway between the aorta and pulmonary arteries and its occlusion during the expansion of the aorta, is a potentially dangerous anomaly ^33-34, as well as all anomalous vessels with interarterial course.

The ectopic LCA origin from the pulmonary artery, which is recognized as affecting childhood survival in up to 90% of cases ³⁵ , is per se correlated to myocardial ischemia and volume afterload and is very rare in the elderly when this anomaly becomes a coronary fistula; the anomalous origin of the RCA from the pulmonary artery, which is extremely rare in the elderly, is usually asymptomatic, but can be related to sudden death ³⁶ .

The clinical significance of these CAAs seems to be enhanced by superimposed CAD, although there are no studies reporting real proof that these kinds of CAAs are more susceptible to CAD ³⁷. A management algorithm is presented in Figure 1.

Conclusions

The angiographic incidence of CAAs in elderly patients is growing due to the increased aging of the population. The association with cardiac and extra-cardiac comorbidities makes aged patients with CAAs, especially those patients with high risk CAAs or superimposed CAD, a complex clinical population. This occurrence calls for a wider knowledge of the anatomy and pathophysiology of CAAs not only among general adult cardiologists but also among geriatric cardiologists.

Figure 1: Proposed algorithm in managing CAAs in aged patients.AMI :acute myocardial infarct ; Atr : atresic ;Ect
PA : ectopic origin form the pulmonary artery; IVUS : intravascular ultrasound ; MyoBr : myocardial bridge ; MR :
magnetic resonance imaging; PCI : Ischemia; Sin CA : single coronary artery ; VT : Ventricular arrhythmias

Acknowledgements

The authors gratefully acknowledge the assistance and the invaluable competence of Mrs Anne Holdstock in the preparation of this article.

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