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Eur Heart J Case Rep
Eur Heart J Case Rep
ehjcr
European Heart Journal. Case Reports
2514-2119
Oxford University Press UK

39045524
10.1093/ehjcr/ytae345
ytae345
Case Report
AcademicSubjects/MED00200
Eurheartj/15
Eurheartj/17
Eurheartj/16
Eurheartj/18
Eurheartj/14
Eurheartj/31
Eurheartj/32
Eurheartj/35
Double-territory STEMI with cardiogenic shock in immune thrombocytopenic purpura with severe thrombocytopenia—a case report
Vijayachandra Yerramareddy Cardiology Division, Apollo Heart Institute, Apollo Hospitals, Chennai, Tamil Nadu, India

Wilson Antony Cardiology Division, Apollo Heart Institute, Apollo Hospitals, Chennai, Tamil Nadu, India

Sreeram Jayalakshmi Cardiology Division, Apollo Heart Institute, Apollo Hospitals, Chennai, Tamil Nadu, India

https://orcid.org/0000-0002-8305-7744
Mahesh Kumar Aishwarya Department of Medical Services, Apollo Hospitals, 21, Greams Lane, Off Greams Road, Thousand Lights, Chennai, Tamil Nadu 600006, India

Karanasos Antonios Handling Editor
Sohal Sumit Editor
Mattioli Maria Editor
Mantzouranis Emmanouil Editor
Corresponding author. Tel: 044 2829 5405, Email: draishwarya_m@apollohospitals.com
Conflict of interest: None declared.

7 2024
11 7 2024
11 7 2024
8 7 ytae34509 8 2023
22 12 2023
03 7 2024
23 7 2024
© The Author(s) 2024. Published by Oxford University Press on behalf of the European Society of Cardiology.
2024
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Abstract

Background

Myocardial infarction (MI) in a patient with immune thrombocytopenia is a rare scenario which is very challenging to manage.

Case summary

We present a rare case of a patient with immune thrombocytopenic purpura who developed double territory segment-elevation MI with cardiogenic shock. She had an extremely rare presentation with a fresh mobile thrombus in the aortic root which was trap-dooring the right coronary artery ostium and extending into the artery with an embolism into the distal left anterior descending artery. We managed this patient conservatively with excellent recovery owing to the dangerous location of the hanging thrombus, and severe thrombocytopenia.

Conclusions

Multidisciplinary approach is required for the management of MI in patients with pre-existing blood disorders, with therapy tailored to the patient's presentation and treatment requirements.

Acute myocardial infarction
Coronary thrombosis
Anti-platelets
Immune thrombocytopenic purpura
Case report
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pmcLearning points

To implement patient-specific personalized therapy for patients with idiopathic thrombocytopenic purpura presenting with myocardial infarction (MI).

To understand the role of medical management in patients with idiopathic thrombocytopenic purpura having MI.

Introduction

Idiopathic thrombocytopenic purpura (ITP) is an autoimmune haematologic condition characterized by rapid platelet breakdown and decreased platelet formation due to the action of autoantibodies on marrow megakaryocytes.1 Acute coronary syndrome (ACS) and segment (ST)-elevation myocardial infarction (STEMI) have been observed to be rare in ITP patients, which may be due to the protective effects of low platelet count.2 A paradoxical increase in thrombosis has also been reported.3 When ACS and STEMI do arise, the management becomes a difficult challenge, owing to the need for anticoagulation in the setting of high bleeding risk (HBR) imposed by the ITP. There are also no established guidelines for managing STEMI in the context of ITP.

Summary figure

Case presentation

A 40-year-old lady female with a history of hypertension, immune thrombocytopenia (ITP), s/p splenectomy, was recently hospitalized elsewhere for chest infection due to Influenza B. She had severe thrombocytopenia with bleeding manifestations (mild haemoptysis) and was managed there with corticosteroids. She now presented to our hospital with complaints of retrosternal chest discomfort, severe sweating, and giddiness. On admission, her blood pressure was 60/40 mm Hg, and her pulse was 102/min. Electrocardiogram (ECG) showed ST elevation in leads II, III, augmented vector foot, and V1–V6, and ST depression in leads I and augmented limb lead (Figure 1), suggestive of double territory inferior and anterior STEMI. Urgent bedside echocardiography showed good left ventricular (LV) systolic function with no regional wall motion abnormality (Supplementary material online, Video 1) with severe right ventricular (RV) dysfunction. There was no thrombus in the main pulmonary artery or main branches and there was no thrombus in RV, left atrium (LA), or left atrial appendage (LAA). The heart valves were normal with no vegetation. There was a thrombus in the aortic root in the right coronary sinus (RCS) at the right coronary artery (RCA) ostium with a ‘hanging thrombus’ appearance. (Figure 2), (Supplementary material online, Videos 2 and 3) There was no patent foramen ovale. Troponin I was positive with peak troponin of >10 ng/mL. There was no deep venous thrombosis of the lower limbs on Duplex scan. The patient had severe thrombocytopenia (platelet count, 40 000/μL). All other blood parameters were normal. In the background of recent history of severe thrombocytopenia with bleeding manifestations, and in view of bedside echocardiography findings suspicious of pulmonary thromboembolism in the setting of ECG findings suggestive of double territory STEMI, and in view of aortic root thrombus with a potential for catheterization-related mechanical dislodgement of thrombus and systemic embolism, it was decided to do a double rule-in/double rule-out computed tomography (CT) coronary and pulmonary angiography in a single shot, and this was done on an emergency basis as per our institutional protocol. CT angiogram revealed the presence of a thrombus in the aortic root in the RCS. There was a ‘hanging appearance’ of the thrombus at the RCA ostium with a mobile (protruding) component endoluminally within the aorta (Figure 3). CT also revealed complete long-segment thrombotic occlusion of the RCA with poor distal run-off. The terminal portion of the left anterior descending (LAD) artery was also occluded with a thrombus. Other coronary arteries were normal. CT pulmonary angiography did not reveal pulmonary embolism. A team approach involving haematology inputs was put into action. The option of systemic thrombolysis was not chosen in view of severe because of thrombocytopenia. An invasive approach of percutaneous coronary intervention (PCI) was not chosen as there was a significant risk of guide catheter-related cardioembolic stroke or systemic embolism from the hanging thrombus at the RCA ostium. The other concern was the difficulty in instituting dual antiplatelet therapy (DAPT) after stenting. The patient was managed with Heparin infusion (high Activated Partial Thromboplastin Time protocol: 12 U/kg/h after bolus of 16 U/kg) and single antiplatelet (Aspirin 75 mg), statin (Atorvastatin 40 mg), and corticosteroid (Dexamethasone 20 mg). She received IV infusion of Noradrenaline along with IV fluids initially for the management of cardiogenic shock. The haemodynamics improved gradually and the dosage was tapered over 72 h. Chest pain settled within 3 h and the ST elevations on ECG settled in 12 h. There was no recurrence of chest pain or ST changes. She had excellent clinical recovery with improved haemodynamics. A repeat echocardiogram performed after 3 days revealed similar findings as before (Supplementary material online, Video 4), but improvement in RV function was observed (Supplementary material online, Video 5).

Figure 1 Electrocardiogram on admission showing segment elevation in leads II, III, aVF, and V1–V6, and segment depression in leads I and aVL.

Figure 2 Echocardiography parasternal long axis view showed thrombus in aortic root in right coronary sinus near right coronary artery ostium.

Figure 3 CT Angiogram showing thrombus in the aortic root at right coronary sinus and ‘hanging thrombus’ from the right coronary artery ostium with normal left main coronary artery thrombotic occlusion of terminal left anterior descending.

It was decided to continue conservative management with a regular dose of low molecular weight heparin (LMWH Enoxaparin 40 mg subcutaneously twice daily [BD]), along with a single antiplatelet (Aspirin) and statin.

Echocardiography after 7 days of treatment showed no thrombus at the aortic root with normal RV and LV function. Platelet count improved during hospitalization, reaching 220 000/µL pre-discharge. There were no bleeding events or embolic episodes during the hospital stay. The patient was discharged on novel oral anticoagulant (NOAC) therapy with Apixaban (5 mg BD), Aspirin, and statin. She was asymptomatic at 1- and 4-month review. Table 1 represents the timeline of the patient’s presentation.

Table 1 Case presentation timeline

Past history	Known history of immune thrombocytopenic purpura. Patient was admitted to an outside hospital for influenza B infection and treated with corticosteroids	
Day 1 Presentation	Complaints of retrosternal chest discomfort with severe sweating and giddiness	
Clinical and investigatory findings	Double territory ST elevation myocardial infarction with aortic root thrombus with hanging thrombus from RCA ostium with thrombotic RCA occlusion, associated with thromboembolic occlusion of distal LAD	
Management	Conservative medical therapy	
Day 3	A repeat echocardiogram performed after 3 days revealed similar findings as before, but improvement in right ventricular function was observed	
Day 7	Echocardiography after 7 days of treatment showed no thrombus at aortic root with normal right and LV function. She did not have any embolic episodes during the hospital stay. She was discharged on novel oral anticoagulant therapy with Apixaban (5 mg BD) and single antiplatelet Aspirin	
Follow-up	The patient was asymptomatic at 1- and 4-month review	

Discussion

Idiopathic thrombocytopenic purpura is described as isolated thrombocytopenia (platelet count <100 000/uL) with normal white blood cells and haemoglobin in the presence of a widespread purpuric rash.4 The paradoxical increased rates of thromboembolic events in patients with ITP could be due to ITP-related factors and patient-related factors. Idiopathic thrombocytopenic purpura-related factors include presence of immature platelets, platelet microparticles, and increased inflammatory cytokines. Patient-related factors include advanced age, history of hypertension, diabetes mellitus, smoking, atrial fibrillation, and hyperlipidaemia. Therapies for ITP such as splenectomy, and immunoglobulin therapy also have a contributory role.3 Management of STEMI in patients with ITP is a challenge due to several factors. The options to manage STEMI in patients with ITP include primary percutaneous transluminal coronary angioplasty (PTCA) and stenting, plain balloon angioplasty (PBA), thrombolysis and conservative medical management, the choice depending on the clinical condition of the patient and platelet count.5–8 Percutaneous transluminal coronary angioplasty is the first line therapy in STEMI scenario if there are no contraindications.

In our case, CT coronary angiography clearly demonstrated not only the presence of aortic root ‘hanging thrombus’ but also showed ‘complete long segment occlusion of the RCA with poor distal run-off’ and ‘only terminal portion of the LAD artery occluded with thrombus’. Invasive option to revascularize the main culprit-vessel RCA which had long segment thrombotic occlusion was assessed to be fraught with two major limitations: First, adjunctive pharmacologies like intracoronary low-dose thrombolysis or systemic or intracoronary low-dose glycoprotein IIb/IIIa inhibitors would be associated with a high risk of bleeding in view of her thrombocytopenia of 40 000/µL (HBR scenario). Second, if any drug-eluting stents (DES) were to be used, DAPT strategy would be necessary for at least 4 weeks, and this was also fraught with a high risk of bleeding in view of her haematological condition. While PBA could be an option to avoid using DAPT, the chances of a good or even acceptable result with PBA were considered very low in view of the long segment thrombotic occlusion of the RCA. For the same reason, the chances of a good or even acceptable result with simple catheter thrombus aspiration were also considered very low. To add to it all, there was a very high risk of catheter-related dislodgement of thrombus from the aortic root with catastrophic consequences. The use of an embolic protection device during catheterization would prevent cerebrovascular accident but not other systemic embolisms. This consideration would be of value if the thrombus dislodgement during PCI was the only concern against invasive strategy; however, in our case, the bigger concern was the low likelihood of success with PBA and the inability to use DES in view of obvious pharmacological limitations discussed above.

Regarding other options of management, systemic thrombolysis for STEMI even with a tailored dose with a view for pharmaco-invasive strategy was contraindicated in view of her very low platelet count. Emergency Coronary artery bypass surgery was considered extremely high risk in view of the very low platelet count.

Considering all these factors, the risk-benefit assessment was in favour of ‘initial conservative strategy’ with the use of inotropic support, heparin, and single antiplatelet strategy (Aspirin), keeping the invasive option for bail-out scenario if there was no improvement.

Regarding Mechanical circulatory support (MCS), intra-aortic balloon pump (IABP) was not considered in this patient in view of the current evidence not showing any benefit with routine use in this scenario. Also, our patient had severe RV dysfunction with normal LV systolic function, a setting where IABP would not be of much benefit. Further, the use of IABP could sometimes worsen thrombocytopenia. The usual LV Impella would not be much useful in this case, unlike the RV Impella. Impella being a large bore access MCS device would be fraught with the dangers of access site bleeding in the scenario of very low platelet counts. Another big concern with LV Impella is that of dislodgement of the aortic root thrombus during insertion. The same bleeding concerns apply to the use of extracorporeal membrane oxygenation support. The patient’s haemodynamic status improved with inotropic support and ‘initial conservative strategy’.

Overall, the mortality of patients with ITP presenting with STEMI is higher than in the general population. A recent observational study, using multiple regression analysis, on patients with ITP hospitalized with myocardial infarction (MI) over a 15-year-period, demonstrated that stent placement, female gender, blood transfusions, platelet transfusion, 80 + age group, and higher Charlson's score were independent predictors of mortality in patients with ITP who have MI.9 Hence, it is crucial to tailor the treatment according to the individual patient’s needs and clinical situation and not to adopt a one-size-fits-all approach. According to a recent algorithm proposed by Swan et al.4 25 000/μL is regarded as the threshold for anti-platelet therapy in patients with a low bleeding risk and high mean platelet volume, who have not previously experienced ITP-related bleeding. Individuals at very high risk of both bleeding and thrombosis should be evaluated on a case-by-case basis, with multidisciplinary team involvement from Haematology and Cardiology, to assess the individual patient's risk: benefit ratio for anti-platelet therapy, with the threshold set at 50 000/μL.

To our knowledge, this is the first reported case of a double-territory STEMI in a patient with ITP. This is also the first reported case of aortic root thrombus with ‘hanging thrombus’ from RCA ostium with thrombotic RCA occlusion, associated with thromboembolic occlusion of distal LAD.

As for the differential diagnosis of thrombus in the aortic root, vegetation was ruled out in view of the absence of fever and all features of infective endocarditis. All the valve leaflets were normal without vegetation on echocardiogram. Tumour was also considered unlikely in view of the CT appearance and mobility. The presence of similar CT attenuation thrombus in aortic root, RCA, and terminal LAD also favoured thrombus as the possibility. In situ formation of aortic root thrombus in the RCS was considered as the possible source of RCA thrombus and the terminal LAD thrombus. Paradoxical hypercoagulability due to recent viral infection and steroid use was considered to be the causative factor for this thrombus in the aortic root; this Paradoxical hypercoagulability has been well reported in cases of ITP.10 We also evaluated for other causes of hypercoagulability in our patient: Lupus anticoagulant and anti-phospholipid antibody screening was negative.

Right coronary artery in situ thrombosis with extension of the thrombus into the aortic root is a less likely theoretical possibility. As for the alternate sources of thrombus, while Transthoracic echocardiography could miss LV or LA or appendage thrombus, CT angiography has higher sensitivity and a very low likelihood of missing them, thus ruling out other sources of cardiac thrombus with embolism to the aortic root. A pre-discharge 24-h Holter ECG was also done to rule out any arrhythmias like atrial fibrillation which could be suspected to be a source of LAA thrombus which could have embolized to aortic root.

After the initial recovery from the acute phase of STEMI, it was important to prevent systemic and cerebral embolism from the aortic root thrombosis, and anticoagulation was the only option. Low-dose LMWH was given initially, and NOAC was prescribed only at discharge. Full-dose NOAC + Aspirin vs. low-dose NOAC + Aspirin were the available options; as the patient’s platelet count improved to 220 000/µL at discharge, full-dose NOAC + Aspirin was prescribed, as suggested by the haematology team, with a provision to frequently monitor for side effects, as well as the platelet counts. She made a good clinical recovery with no episodes of bleeding till 4-month follow-up.

In clinical practice, one may encounter extremely rare scenarios which demand ‘different from the usual’ approaches. While STEMI with cardiogenic shock is a common clinical scenario, the association with ITP and severe thrombocytopenia, suggestive of paradoxical hypercoagulability setting in ITP, is very rare. The presentation with double-territory STEMI is even rarer. And the presence of aortic root—aortic sinus ‘hanging thrombus’ would be extremely rare. The usual invasive strategy of primary PCI could have been potentially catastrophic in this patient. This case highlights the role of emergency CT coronary angiography in such rare scenarios. This could be of use in the initial assessment of rare presentations of MI like in the setting of hypercoagulability. Not utilizing CT coronary angiography in our case could have led to a potentially catastrophic outcome, as there would not have been any possibility of engaging the RCA without thrombus dislodgement. We documented the total disappearance of aortic root thrombus by the 7th day of LMWH therapy on echocardiography. Our case management highlights the success of a tailored individualized approach in an extremely rare scenario with a good outcome.

Conclusion

ST-elevation MI in the context of immune thrombocytopenic purpura is very rare and challenging to manage. The conventional approaches of coronary intervention or thrombolysis are fraught with dangerous limitations of excessive bleeding risks and even mortality. Any abnormal or atypical presentation of STEMI in such scenarios should prompt good bedside echocardiography, and if needed, an urgent non-invasive CT-coronary angiography, to bring out any rare features of the disease which can have a tremendous impact on the strategy of management. This case brings out the importance of a multidisciplinary team approach in choosing nuanced patient-tailored effective and safe therapies in the management of such rare and atypical presentations. The case also highlights the dynamic component of the disease with regard to improved platelet counts (with use of steroids, etc.) which needs to be factored in to appropriately modify the pharmacotherapy regimen for better efficacy and safety.

Supplementary Material

ytae345_Supplementary_Data

Lead author biography

Dr. Y. Vijayachandra Reddy (Yerramareddy Vijayachandra), MD DM MRCP FACC FCSI FSCAI CCDS, is a Senior Consultant & Interventional Cardiologist at Apollo Main Hospitals, Chennai. He is well known in the field of Interventional Cardiology with extensive and versatile experience and range of professional work. He is a pioneer in transradial interventions and complex high-risk CHIP interventions including left-main, bifurcation and CTO PCIs. He is credited with pioneering work in acute MI interventions and his large body of PCI work in cardiogenic shock with unparalleled outcomes is well known. He is also versatile in coronary physiology and imaging, and facile with the gamut of PCI techniques involving Robotic PCI, Rotablation, IVL, Laser atherectomy etc.

Supplementary material

Supplementary material is available at European Heart Journal – Case Reports online.

Consent: The author(s) confirm that written informed consent has been obtained from the involved patient.

Funding: This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.

Data availability

The datasets used and/or analysed during the current study are available from the corresponding author on reasonable request.
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