Premature Coronary Atherosclerosis Linked to A Primary Antiphospholipid Syndrome: A Case Report
Akiel Rody, MD *1, Amabile Nicolas, MD, Phd 1, Mami Zoheir, MD 1
1. Institut Mutualiste Montsouris, Paris, France.
Corresponding Author: Akiel Rody, Institut Mutualiste Montsouris, Paris, France.
Copy Right: © 2023, Akiel Rody, This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Received Date: May 24, 2023
Published Date: June 01, 2023
Abstract
We report the case of a young female without cardiovascular risk factors presenting for acute myocardial infarction. The diagnosis was confirmed by endovascular imaging. She was found to have atherosclerotic disease secondary to antiphospholipid syndrome. This case sheds light on premature atherosclerosis, it also highlights importance of endovascular imaging in non-conclusive results.
Keywords: Premature coronary atherosclerosis, antiphospholipid syndrome, intracoronary optical coherence tomography.
Abbreviations
APLS: Antiphospholipid syndrome.
MRI: Magnetic resonance imaging.
CT: Computed tomography.
LAD: Left anterior descending artery.
LM: Left main artery.
LCX: Left circumflex artery.
OCT: Optical coherence tomography.
CCU: Coronary care unit.
TTE: Transthoracic Echocardiography.
NST-ACS: Non ST elevation acute coronary syndrome.
ACS: Acute coronary syndrome.
PCI: Percutaneous coronary intervention.
HDL: High-density Lipoprotein.
LDL: Low-density Lipoprotein.
TTE: Transthoracic echocardiogram.
CBC: Complete blood count.
BUN: Blood urea nitrogen.
CRP: C reactive protein.
BNP: Brain natriuretic peptide.
History of Presentation
A 30 years old female, with no known classical cardiovascular risk factors, presented to our Emergency Department for three episodes of retrosternal, oppressive chest pain at rest, of twenty-four hours’ duration.
She reported having similar episodes of chest pain previously upon exertion for the last four months. No medical workup was done.
Her physical examination was unremarkable, her vital signs were: 115/75 mmHg, 64 bpm, 36.8ºC, Oxygen saturation 99%.
Past Medical History
The patient is known to have an obstetric primary Antiphospholipid syndrome (unexplained death of a morphologically normal fetus at 11weeks of gestation, and positive B2GP1 antibodies, followed by an internist), iron deficiency anemia related to heavy menstruations, and migraine.
Investigations
ECG showed negative T waves in anterior and septal leads, she had elevated troponins (580 ng/L, with the upper limit being 34 ng/mL) on her lab panel (CBC, D-dimers, BUN, Creatinine, Electrolytes, NT-proBNP, CRP, Liver function tests were unremarkable).
Transthoracic echocardiogram found a non-dilated, non-hypertrophic left ventricle with moderate anteroseptal and inferoseptal basal and mid hypokinesis, a preserved LVEF, no major valvular abnormalities, no pericardial fluid. Chest X-ray was unremarkable.
After a whole night in the CCU, a coronary CT was done and revealed a severe ostial and proximal LAD atheromatous lesion (> 90% of luminal obstruction, figure 1), thus an urgent coronary angiogram was then performed, and confirmed the aforementioned result (video 1).
Video 1: Coronary angiogram showing proximal LAD lesion. https://youtube.com/shorts/fi0yLyjQtTU?feature=share
Aiming to better understand the mechanism of the stenosis, an OCT was done and the atherosclerotic nature of the lesion was demonstrated (video 2).
Video 2: OCT showing atheromatous nature of the LAD lesion with plaque erosion.
https://youtube.com/shorts/rbNR6RwVwK4?feature=share
On day three, a repeated TTE showed the absence of wall motion abnormalities. Lab tests done to screen for Dyslipidemia and Diabetes came back negative.
Screening for peripheral artery disease was done via an arterial lower limb and carotid ultrasounds and came back with no abnormal results.
Video 3: Coronary angiogram showing stent deployed.
https://youtube.com/shorts/7ky2jqK27OU?feature=share
Differential diagnosis
Myopericarditis, NST-ACS, coronary dissection.
Management
On day one, the patient was admitted to the CCU, symptom-free, with appropriate myopericarditis and NST- ACS medications (Aspirin 1 g IV Q 8 hour, Atorvastatin 40 mg/ day, Enoxaparin 60 mg each 12 hours, Bisoprolol 1.25 mg / day, Ramipril 1.25 mg / day, Trinitrine 5 mg/ day).
The next day, in the catheterization lab, and after having a clear diagnosis, an Everolimus (4*12 mm) drug eluting stent was deployed successfully covering from the distal LM to proximal LAD, and a kissing balloon technique done with the LCX. The final result was then controlled by another OCT run, Ticagrelor 90 mg each 12 hours was added to her treatment.
The patient was then retransferred to the CCU for further management and observation.
A staff meeting was held the day after with Internists for case discussion, and optimal management.
On day four, the patient had fully recovered and had no active complaints. Thus, she was discharged and instructed to follow up as an outpatient.
Discussion
Antiphospholipid syndrome is an autoimmune disorder characterized by venous or arterial, thrombosis and/or pregnancy morbidity (e.g. placental insufficiency, fetal death after 10 weeks’ gestation), associated with a persistent laboratory evidence of antiphospholipid antibodies.
Premature coronary artery disease is defined as the occurrence of symptomatic obstructive coronary atherothrombotic lesions before the age of 45 years [1].
Atherosclerosis is a complex inflammatory disease involving aberrant immune and tissue healing, that commences in childhood but manifests clinically later in life. However, the presence of Inflammation can aggravate atherosclerosis via multiple mechanisms, leading sometimes to what is called "Premature atherosclerosis" in patients with chronic inflammatory/immune conditions [2].
Premature atherosclerosis has been found in Antiphospholipid syndrome patients more frequently than in the general population despite having the same incidence of traditional Framingham risk factors [3,4].
Cross-sectional data suggest that Antiphospholipid syndrome (among other inflammatory disorder) is linked to premature atherosclerosis[5]. The prevalence of coronary premature atherosclerosis in Primary antiphospholipid syndrome is not well defined, but some data suggest that asymptomatic coronary atherosclerosis is around 15% of the patients, and myocardial infarction (including thrombotic mechanism) is around 1.2% of the patients [6].
The underlying mechanisms are not yet clearly understood, but available data suggests that antiphospholipid antibodies exert proinflammatory and procoagulant effects on the endothelium: Among these, β2GPI seems to have a role by forming a β2GPI-oxidized-LDL complex that is linked to autoimmune-mediated atherogenesis [7], and, on the other hand, a described cross-reactivity between Anticardiolipin and antibodies to oxidized LDL, HDL, and Apo A1 suggests a potential interaction mechanism by this latter with serum lipoproteins [7].
Besides that, Anticardiolipin antibodies and anti-ß2GPI may contribute to LDL oxidation by inhibition of Paraoxonase (an enzyme attached to HDL, that normally prevents oxidation of LDL, contributing for the protective effects of HDL) [8].
Finally, the autoimmune inflammatory response leads to proliferation of macrophages, and of endothelial and vascular myocytes culminating in the production of matrix-degrading proteases and tissue factor eventually leading to plaque rupture and thrombi formation [8].
In our case, coronary atherosclerosis was a lesser considered diagnosis. Intracoronary thrombus (that is caused by her hypercoagulable state), and spontaneous coronary artery dissection which may be more prevalent in this scenario (Spontaneous coronary artery dissection has been reported to account for nearly a quarter of cases of ACS in women ≤50 years old [9]), were more likely to be the first differential diagnoses.
But the performed OCT showed atherosclerotic lesion with plaque erosion (figure 2), and confirmed the absence of a significant thrombotic material (which could be a more frequent diagnosis considering her APLS), as well as the absence of any coronary dissection, and the decision of stenting was taken on the spot.
Although the indication of stenting was(and still) obvious, it is noteworthy to state that antiphospholipid patients who undergo PCI tend have worse long-term clinical outcomes, driven by higher rates of revascularization, caused by in-stent restenosis, and/or accelerated atherosclerosis in non-stented arteries, and extreme cautious should be sought in the follow-up of these patients [10]. As for the medical treatment, literature review showed that coronary atherosclerosis(in antiphospholipid patients) treatment should be the same used in the general population, with the possibility of adding Hydroxychloroquine but without evidence based benefits [6].
Finally, and based on the absence of regular cardiovascular risk factors, and the presence of primary Antiphospholipid syndrome, and due to the atherosclerotic nature of the lesion, we considered the primary antiphospholipid syndrome as being the sole risk factor responsible for the premature atherosclerosis and the resulting myocardial infarction.
Follow-up
The patient was seen at one month, and four months after her myocardial infarction in follow up visits. She was asymptomatic, in a good health status, and compliant with her medications. Another visit was scheduled two months after with a stress echocardiogram.
Conclusion
This Patient suffered from myocardial infarction due to premature atherosclerosis, linked solely to her primary antiphospholipid syndrome. This coronary entity was not primarily evoked when patient had chest pain episodes four months ago, nor at the time of her myocardial infarction.
Thus, we found it important to shed light on this entity, so it can be considered among clinician’s differential diagnoses when similar clinical scenarios are faced, and similar patient status is of concern.
Hence, in patients with antiphospholipid syndrome, premature atherosclerosis could be responsible for lethal cardiac complications. This entity should be screened for, and treated appropriately to improve patient outcomes.
On the other hand, we think that any coronary atherosclerosis diagnosed at an early age without cardiovascular risk factors, should prompt screening for conditions associated with premature atherosclerosis such as Antiphospholipid syndrome.
Finally, we highlight the role of intracoronary imaging when encountering discrepancy between angiographic findings and clinical settings.
References
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