A 53-year-old Female with Sudden-Onset Chest Pain on a Background of Polyarteritis Nodosa
A 53-year-old previously healthy female patient presented with weakness in her lower extremities. She was initially suspected to have myasthaenia gravis and was started on pyridostigmine and prednisone. However, she subsequently developed sudden-onset chest pain and was admitted to the ED. An ECG was performed which showed ST-depression in leads I, aVL and V4-V6. Bloods also showed elevated cardiac troponin levels, leading to the diagnosis of NSTEMI. The patient's condition rapidly deteriorated soon after and she went into cardiogenic shock followed by cardiac arrest.
Despite rigorous resuscitation efforts, the patient's prognosis was deemed poor, leading to the decision to discontinue CPR. An autopsy was performed to determine the underlying cause of the cardiogenic shock and the examination revealed a transmural infarct throughout the entire left ventricle, as well as intimal thickening in the coronary arteries. Similar findings were observed in the kidney and GI arteries, consistent with a diagnosis of Polyarteritis Nodosa.
This patient’s ECG shows ST-depression in leads V4-V6 as well as in leads I & aVL. ST-elevation can also be seen in leads V1 & aVR.
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NSTEMI is diagnosed when there are ST-segment depressions and/or T-wave inversions on the ECG, along with elevated cardiac troponin levels.
References:
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When diagnosing MI (both STEMI & NSTEMI), use the universal definition of myocardial infarction —> this is the detection of rise and/or fall of cardiac biomarkers (hs-cTn is gold standard) with at least one value above the 99th percentile of the upper reference limit and at least one of the following:
symptoms of ischaemia
new or presumed new significant ST-segment-T wave(ST-T) changes or new left bundle branch block (LBBB)
development of pathological Q waves in the ECG
imaging evidence of new loss of viable myocardium or new regional wall motion abnormality
identification of an intracoronary thrombus by angiography.
References:
1) https://www.nice.org.uk/guidance/cg95/evidence/full-guideline-245282221
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Initial management of all patients who present with NSTEMI (and indeed for any patient presenting with Acute Coronary Syndrome) is as per the ACS Protocol set out in the Advanced LIfe Support (ALS) Guidelines:
300mg aspirin
GTN spray/sublingual (unless hypotensive or severe RV infarct suspected)
Supplemental oxygen titrated to appropriate target saturations (94-98% target sats. if Scale 1; 88-92% target sats. if Scale 2)
10mg morphine for pain relief as indicated
10mg metoclopramide if nausea/vomiting as indicated
References:
1) https://lms.resus.org.uk/modules/m15-v2-acute-coronary/11118/resources/chapter_4.pdf
2) https://www.nice.org.uk/guidance/cg95/evidence/full-guideline-245282221
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Polyarteritis nodosa (PAN) is a systemic vasculitis that primarily affects medium-sized arteries. It is characterised by inflammation and damage to the arterial walls (leading to impaired blood flow with hypoperfusion of the organ and resultant ischaemic damage). While coronary artery involvement in PAN is relatively rare, myocardial infarction can occur in some patients with this disease. Ongoing inflammation of the coronary arteries can result in damage to the blood vessels with exposure of tissue factor and activation of the coagulation cascade resulting in thrombus formation. If the thrombus grows large enough, it can significantly impair blood flow to the myocardium, eventually leading to ischaemia/infarction.
References:
2) https://emedicine.medscape.com/article/330717-overview#showall
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Yes this patient may have benefited from early PCI given the unstableness of her clinical condition.
PCI is only indicated in patients with NSTEMI in select circumstances. According to NICE guidelines, the indications for PCI are as follows:
1. Immediate angiography if the patient is unstable.
2. Consider angiography within 72 hours for intermediate/high-risk patients without contraindications.
3. Consider angiography for low-risk patients if ischemia occurs or is demonstrated.
4. Younger patients with low mortality risk may still benefit from early angiography.
If PCI is indicated, then the patient will require dual antiplatelet therapy with aspirin and either prasugrel or ticagrelor.
(Otherwise conservative management without early angiography is indicated for low-risk patients with NSTEMI).
References:
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Transmural ischaemia/infarction involves the entire thickness of the myocardial wall, whereas subendocardial ischaemia/infarction doesn’t involve the entire thickness of the myocardium (~2/3 of the wall is involved).
On ECG transmural ischaemia/infarction presents as ST-elevation, whereas subendocardial ischaemia/infarction presents as ST-depression or other non-specific changes (e.g. T-wave inversions).
References:
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This original article provides an in-depth analysis of a complex case involving a patient with polyarteritis nodosa (PAN) who experienced myocardial infarction and subsequent cardiogenic shock leading to death. The patient's myocardial infarction was attributed to the underlying pathology of PAN itself, which can result in coronary lesions such as stenosis, aneurysms, and spontaneous coronary dissection. These pathological changes create an environment conducive to thrombus formation, occlusion, and subsequent infarction.
The diagnosis of PAN in this case was established using the Japanese diagnostic criteria, considering clinical symptoms (myocardial infarction and polyneuritis), angiographic findings (bowel artery occlusion), and histopathological examination. The histological analysis revealed the involvement of medium and small caliber arteries, characteristic of PAN, showing marked intimal thickening and minimal cellular swelling.
The article also emphasises the challenges in distinguishing between myocarditis and myocardial infarction using cardiac magnetic resonance. In this case, the absence of a specific vascular territory affected by the infarct initially led to a suspicion of myocarditis. However, subsequent autopsy examinations excluded myocarditis and instead confirmed the presence of acute myocardial infarction with characteristics consistent with a 10 to 14-day evolution.
Furthermore, the authors hypothesise a potential vasospastic component in this case, given the patient's PAN-related vascular abnormalities and the possible triggering effect of pyridostigmine, a medication received prior to symptom onset. While lacking direct imaging evidence of coronary vasospasm, the patient's clinical presentation, timing of symptoms, and absence of occlusions or thrombi in the coronary arteries support this hypothesis.
This case study contributes to the growing understanding of coronary artery disease manifestations in patients with PAN, highlighting the potential role of coronary vasospasm in causing infarctions and underscoring the importance of recognising that myocardial infarctions in PAN can extend beyond a single vascular territory, resulting in significant haemodynamic consequences.
Source: https://doi.org/10.3389/fcvm.2022.1070378
Case Summary & Questions written by Dr Ahmed Kazie
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