GABRIEL SALICA, ALEJANDRA NARANJO, ENRIQUE ALONSO, CAROLINA GONZÁLEZ, FERNANDO RODRÍGUEZ
Hospital Ángel C Padilla.
(4000) San Miguel de Tucumán, Argentina. E-mail
Recibido 29-NOV-2019 – ACEPTADO después de revisión el 18-FEB-2019.
There are no conflicts of interest to disclose.
Coronary artery aneurysms are not frequent and diagnosis is made by coronary angiography. Many causes have been described as likely to provoke alterations in arterial structure such as atherosclerosis, inflammatory illnesses, congenital disorders and medical interventions. More common complications are cardiac arrest, rupture of coronary arteries and fistulas into the heart chambers. Their prognosis is uncertain as well as their management, when there are no coronary lesions to revascularize. We show a case with multiple coronary aneurysms and chronic stable angina during one year of follow-up.
Coronary artery aneurysms are rare, and most of them are detected as casual angiographic findings. They have been defined as the dilatation of an arterial segment exceeding the diameter of the adjacent segment by 1.5 times [1,2]. The reported incidence by autopsy is 0.15 at 4.9%, with the most frequently affected territories being the anterior descending artery (ADA) and the right coronary artery (RCA), with similar incidence in both territories .
In etiopathogenesis, it has been implied that atherosclerosis could be the main cause, followed by inflammatory diseases causing vasculitis such as Kawasaki disease, also considering a congenital origin, or as a consequence of interventionist procedures, mainly those in which coronary artery dissection occurs [1,4].
The most frequent complications are infarction, myocardial ischemia, although rupture and fistulae in cardiac chambers have also been reported [5,6,7]. Although the evolution of carriers of coronary artery aneurysms many not present complications in the mid and long term, evolution is influenced by the presence of non-revascularizable CAD; so it is complex to predict its evolution and for non-revascularizable cases, the best therapeutic strategy has not been defined yet .
Next, we present the case of a patient with multiple aneurysms in the coronary arteries, evolving with chronic stable angina.
Male, 61-year-old patient, construction worker, with history of HTN in discontinuous treatment with enalapril, that in 2007 mentions having suffered ischemic stroke, and in 2016 consulted several times due to angina and dyspnea in functional class (FC) II. Basal ECG showed sinus rhythm with no repolarization disorders, which during stress test presented ST-T segment rectification in the late recovery stages, with no accompanying angina. Echocardiogram did not show global or segmentary motility alterations with preserved ejection fraction (EF) (Figure 1).
Figure 1. Echocardiogram with preserved parietal motility.
In January 2017, myocardial perfusion (MP) test was made, yielding diaphragmatic attenuation in inferior side and homogeneous distribution of the radiotracer in the rest of the sides, with preserved EF. Due to persistence of angina, coronary angiography was scheduled, which showed giant aneurysm of the Left Coronary Trunk, aneurysmatic dilatation of the ADA middle and proximal segments, of its diagonal and septal branches, aneurysmatic dilatation of the Circumflex (Cx) artery, of its marginal branches, and finally, aneurysmatic dilatation of the RCA proximal and middle segments, with no angiographically significant stenosis (Figure 2).
Figure 2a, b y c. Coronary angiography showing in different projections,
aneurysmatic dilatation in several segments of the coronary tree.
Treatment with calcium channel blockers (CCB) enalapril and acenocoumarol was started. Before this diagnosis, a history of febrile exanthematous disease was investigated, indicating a relationship with Kawasaki disease, but with a negative result, and searching for arterial sectors searching for peripheral aneurysmatic disease did not yield pathological findings either.
In his evolution, the patient presented episodes of hypotension with the administration of CCB, so he was rotated to beta blockers (BB) that were well tolerated by him.
At one year of follow-up, the patient continues with a similar treatment, reporting pain with the same characteristics, which led him to quit his job, with no worsening of his FC. A new control echocardiogram did not show parietal motility alterations, with preserved ventricular function. Ergometer test was made, which was stopped with a low load due to typical precordial pain and anterior and lateral side depression. In 2018, a new myocardial perfusion was stopped at 600 Kg due to muscular fatigue, reaching 84% of coronary reserve, with homogeneous distribution of radiotracer in rest and in stress. Gated spect revealed global hypokinesis in a moderate degree after stress, with EF of 48%. Ambulatory electrocardiographic monitoring (Holter) showed infrequent ventricular and supraventricular ectopic activity with isolated forms and ventricular repolarization alterations with ST segment depression. Before these results and typical symptoms, trimetazidine was added to the treatment, with favorable evolution, with no symptoms and sub-maximal ergometer test sufficient and negative for ischemic heart disease.
DISCUSSION Coronary aneurysms are rare, and in most of cases they are casual findings [1,2]. In this case, coronary angiography (CAG) was made before persistence of precordial pain, and it was what allowed establishing the diagnosis.
Between the most frequent causes, Kawasaki disease has been involved, mainly in children younger than 5 years, according to data obtained in more than 500 children, followed for 20 years with CAG, which manifested that 25% could develop coronary aneurysms with 0.8% mortality, there was a possibility of regression of aneurysmatic lesions in more than 50% of cases per year, and rare ischemic compromise (2.2%) in adults . Kawasaki disease in adulthood is extremely rare, with very few published cases . The classical diagnosis of this disease is based on the presence of fever of 5 or more days of evolution, and 4 or more of the following clinical signs: skin alterations in limbs, polymorphic exanthema, bilateral nonexudative conjunctivitis, lips and oral cavity compromise, and cervical adenopathies, in patients with fever. If 4 of the previously described clinical signs are present, this diagnosis could be suspected, which will be confirmed if CAD is detected . The reported clinical case did not present any of the clinical signs for Kawasaki disease.
Coronary aneurysms have also been related to the presence of several coronary risk factors [4-8], hypertension, dyslipidemia, smoking, diabetes mellitus and family history of ischemic heart disease, which have been associated to atherosclerotic aneurysms that usually affect one or two coronary vessels , with collagen and elastic fibers degradation with internal and external elastic lamina disruption. The patient protagonist of this presentation, only had hypertension as associated risk factor.
Giant trunk aneurysms are infrequent, 0.1% , and in general have fistulae in cardiac chambers as a frequent complication, as well as heart failure symptoms, murmurs, or infectious endocarditis data [3-8]. The clinical case presented with trunk aneurysm, did not display any of these complications.
The main symptom reported by the patient was angina, having mentioned that coronary artery ectasia is capable of producing ischemia induced by exercise , by slowing of coronary flow, with a high microvascular resistance, probably due to the change in flow from laminar to turbulent in the ectasic segments, with greater sensitivity for coronary spasms , and possibility of thrombosis in ectasic areas by increase in platelet aggregation in this segment, and distal embolization, probably responsible for mild ischemic events that could manifest by post-stress myocardial perfusion .
Cannon et al, when studying the relationship between ventricular function and ST segment changes during exercise, found that 35% of patients with no electrocardiographic changes induced with exercise, presented regional motility alterations, evaluated by ventriculography with radionuclides, while those with ST ischemic changes during stress presented ventricular function impairment .
In the patient protagonist of this presentation, basal electrocardiogram, although displaying ventricular repolarization alterations, did not show changes or worsening of the previously described alterations during stress.
There are no clearly defined criteria to manage this pathology, maybe because of the low prevalence of this pathology. The American Association and the American College of Cardiology, for the management of adult patients with congenital heart diseases , consider that trunk aneurysms have class I indication (beneficial) to undergo surgical procedures, just as when these aneurysmatic dilatations are between the pulmonary artery and the aorta; likewise, it is considered that myocardial ischemia episodes by compression of adjacent giant aneurysm should be intervened to repair them. With aneurysms not considered giant (when the diameter is not ≥8 mm), there are no indications about them in the clinical guidelines of the referential scientific societies.
For the patient presented in this publication, carrier of trunk aneurysm present in other territories of the coronary tree, using nitrites could be harmful for the possibility of increasing myocardial ischemia , therapy with beta blockers (BB) is controversial, as some authors point out that they may cause vasospasm by lack of opposition to alpha-receptor action; on the contrary, other authors suggest using BB could be beneficial because of their negative chronotropic effect with less oxygen consumption by the myocardium . Also calcium channel blockers (CCB) would be a good option to attenuate coronary spasm, just like the combination of converting enzyme inhibitors and statins could be beneficial by decreasing the existing inflammatory and thrombotic component .
Antithrombotic therapy (anticoagulation and antiplatelet aggregation), associated to CCB or BB, has been proposed as the proper treatment for these patients, maintaining INR of 2-2.5. In the patient presented, a conservative treatment was chosen, initially with CCB and acenocoumarol, to change to BB during follow-up due to hypotension associated to CCB, with better tolerance.
In the 6-month follow-up and before the persistence of symptoms (angina and dyspnea) even with IRN in a therapeutic range, it was decided to add trimetazidine (TMZ), a drug that intervenes in metabolic modulation, by intervening in the metabolic cascade of myocardial ischemia, partially inhibiting elevated fatty acid oxidation during ischemia, favoring carbohydrates oxidation in a more efficient manner, allowing the oxygen available to be used preferentially in glycolysis, minimizing lactate accumulation and thus, tissue acidosis .
The TRIMPOL II study, included patients with CAD that received metoprolol and later, were randomized to receiving TMZ or placebo. In the TMZ arm, a significant improvement was observed with less vasodilators and decrease in the frequency of angina episodes . A meta-analysis that included 23 trials, with 1378 patients with chronic stable angina, showed that TMZ compared with placebo, as a single drug or in combination with conventional antianginal therapy, decreases the frequency of angina episodes, nitrates consumption per week, and more time until the onset of angina .
Coinciding with the clinical benefit reported above, the addition of TMZ was useful to improve the symptoms in the clinical case described. .
Coronary affection in multiple vessels, of the aneurysmatic type, in adults, is a rare pathology, with diverse sources: atherosclerotic, congenital and by inflammatory diseases, among them Kawasaki disease. Its most frequent clinical manifestation is ischemic heart disease, and coronary angiography is the diagnostic procedure by excellence.
Early detection would allow to optimize its management. With the disease already established, a close follow-up of these patients is necessary. Using therapy with metabolic modulation is an important strategy to take into account in cases in which coronary revascularization is not possible.
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