Myocardial infarction in a 21-year-old patient: Spontaneous coronary artery dissection
BRAIAN A. CARDINALI RÉ, FEDERICO J. OLGIATI,
LUCIA M. ORTIZ,
DIEGO ECHAZARRETA, MARCELO PORTIS
Servicio de Cardiología. Hospital San Juan de Dios.
(1900) La Plata, Buenos Aires, Argentina E-mail
Recibido 27-OCT-2019 – ACEPTADO después de revisión el 21-DICIEMBRE-2019.
There are no conflicts of interest to disclose.
A 21-year-old male patient consulted for precompetitive evaluation with a history of consulting 4 years before for precordial pain of anginal characteristics. The electrocardiogram displayed previous anterior myocardial necrosis, which led to the suspicion of previous coronary event.
Additional studies were made, corresponding to ischemic cardiomyopathy, including coronary cineangiography, which showed chronic lesion compatible with spontaneous dissection of the anterior descending artery.
Based on these findings, myocardial viability was investigated, which was negative, so pharmacological treatment and implantable cardioverter defibrillator were implemented because of increased risk of sudden death.
Spontaneous coronary artery dissection is an infrequent cause of acute coronary event, with a prevalence of 1-2.4 per 1000 acute coronary syndromes , but it is not a rare disease.
It consists of the separation of two of the three layers of the arterial wall , to create a false lumen, the progression of which may impair distal coronary flow and produce ischemia, with types of presentation that range from the elevation of cardiac biomarkers to ST-segment modifications without the presence of ischemic symptoms, up to the episode of sudden cardiac death .
This clinical case is about a young, 21-year-old male patient, with no cardiovascular risk factors, who consulted due to a precompetitive evaluation, in whom because of a diagnostic suspicion, additional diagnostic tests were requested.
Male, 21-year-old patient with no cardiovascular risk factors or family history, but who did have a personal history of recently diagnosed hyperthyroidism, who attended a cardiology office as an outpatient for testing before starting a high-performance physical activity (CrossFit).
During his interview, the patient reported not presenting dyspnea, angina, palpitations or syncope. He reported that 4 years before consulting, he suffered precordial pain in rest, oppressive, not radiating, of 6/10 intensity, of twelve hours of evolution, about which he had not consulted and that yielded spontaneously.
The patient appeared to be in a good general state, with all his vital signs within normal parameters. As positive data, mitral regurgitation murmur was auscultated with 2/6 intensity, radiating into the armpit, which increased its intensity after handgrip maneuver. Electrocardiogram (ECG) was performed, displaying sinus rhythm, 80 beats per minute, anterior sequela and isolated premature ventricular contractions.
Following the anamnesis and the ECG findings compatible with chronic ischemic event, the following additional tests were requested:
Transthoracic echocardiography: Left ventricular diastolic diameter (LVDD) 69 mm, left ventricular systolic diameter (LVSD) 53 mm, left ventricular ejection fraction (by Simpson’s method - LVEF) 32%, diastolic dysfunction I, with akinesia and hyperechoic lesion in apical cap and middle segments of the anterior and antero-septal sides. Organized apical thrombus.
Coronary cineangiography (Figure 1). Anterior descending artery with chronic subocclusive lesion in the middle third, which could be secondary to spontaneous dissection; the rest with no angiographically significant lesions.
Diagnosis. Chronic ischemic heart disease secondary to spontaneous dissection of anterior descending artery.
Management. Viability was assessed for a possible myocardial revascularization.
The SPECT scan of myocardial perfusion (Figure 2) showed anterior and antero-septal fixed defect before the developed stress and the double product reached. Permanent dilatation of the LV chamber. Severely deteriorated LVEF in rest and post-stress. No evidence of viability by this method. MRI showed extension of myocardium in risk: 30%; defect intensity, summed stress score: 15 – reversibility score: 0. Figure 3 highlights compromised early and late enhancement.
Essentially, it was cardiomyopathy of ischemic origin with severe global deterioration of systolic function and extensive sequela in the territory of the anterior descending artery and extensive non-transmural but significant compromise, of parietal thickness with no evidence of viability.
Evolution. Faced with the lack of viability proven by two methods, the decision was made to optimize pharmacological treatment for ischemic heart disease and heart failure with aspirin 100 mg/day, atorvastatin 10 mg/day, enalapril 10 mg/day, acenocoumarol 4 mg/day, propranolol 240 mg/day, eplerenone 25 mg/day, dismissing the possibility of revascularization.
Because of the 24 h Holter with very frequent premature ventricular contractions and salvoes of nonsustained ventricular tachycardia (NSVT) of 8 beats, added to the basal risk of sudden death of the patient, it was decided to implement implantable cardioverter defibrillator (ICD) as primary prevention.
Figure 1. Coronary cineangiography showing anterior descending artery with chronic subocclusive lesion in the middle third, secondary to probable spontaneous coronary artery dissection.
Figure 2. SPECT scan with no evidence of myocardial viability.
Figure 3. Magnetic resonance imaging highlighting early and late enhancement of the compromised myocardium.
The spontaneous dissection of coronary arteries is a very frequent entity, although currently it should not be considered a rare disease , which should always be suspected in young patients who present acute coronary syndrome, but with no classical cardiovascular risk factors.
Since the first report of spontaneous coronary dissection in 1931, numerous cases have been published, many as a result of autopsies after an episode of sudden death.
It presents more frequently in young women, generally in relation to hormone changes, during peripartum, or else because of hormone treatments. In other cases, it is attributed to little prevalent diseases  as lupus , sarcoidosis, Kawasaki, Marfan and Ehler-Danlos.
However, in recent years, different authors have reported on spontaneous dissection within a scenario of acute coronary syndrome .
The knowledge of this entity is mainly reduced to isolated clinical cases or series with a reduced number of cases, which makes its treatment and prognosis not fully established .
Some authors consider that the indication should be surgery; others favor endovascular management ; and in some cases pharmacological treatment is chosen. There is no general consensus as to which therapeutic option is the most convenient one.
The pathophysiology is not completely proven and it has been related to the inflammatory atherosclerotic plaque rupture, an unlikely cause in this patient given his youth .
Factors related to spontaneous coronary artery dissection are: hypertension, cocaine consumption, coronary spasm by increase in vessel shear stress, vasculitis, treatment with cyclosporine, compressive forces secondary to intramyocardial trajectories of coronary arteries, and even exhausting physical exercise . Also, it is necessary to mention the existence of a possible inherited factor that would explain the familial cases described .
The generalization of the hemodynamic test of coronary arteries in the management of ACS increased the number of diagnosed cases, with the visualization of an intimal flap appearing as a radiotransparent line that separates the double lumen along with indirect signs of dissection, such as contrast agent retention in the false lumen. Less frequently, it is possible to find arterial narrowing with no evidence of flap, when there is no contrast material flow in the false lumen .
In case of doubt, intravascular echography , even in the initial stages of the implementation of optical coherence tomography , may help to achieve the definitive diagnosis, mainly when it is necessary to verify before the percutaneous intervention, that the guidewire is in the true lumen. Coronary CT may equally help to clarify the diagnosis in a noninvasive manner, mainly when there is suspicion of associated complications .
The treatment guidelines depend on the location of the dissection, its accessibility and extension, as well as the severity of ischemia and stability of the patient. The therapeutic choice should be adopted individually .
The prophylactic treatment with an implantable cardioverter defibrillator (ICD) does not reduce overall mortality in patients in high risk who recently had myocardial infarction . Although therapy with ICD is associated to a reduction in the rate of death by arrhythmia, it is offset by an increase in the rate of death by nonarrhythmic causes.
Spontaneous dissection of coronary arteries is a very infrequent entity, which should be suspected in young men with no classical factors of cardiovascular risk and who present acute coronary syndrome. The treatment to follow is not standardized, so series with a larger number of patients and randomized studies are required.
The clinical case presented once again proves the significance of a proper precompetitive clinical assessment in presumably healthy young people.
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