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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

10.1093/ehjcr/ytae451
ytae451
Case Series
AcademicSubjects/MED00200
Eurheartj/39
Eurheartj/41
Eurheartj/15
Eurheartj/17
Eurheartj/12
Clinical course of pregnancy-associated spontaneous coronary artery dissection: a case series
https://orcid.org/0000-0002-3048-0901
Würdinger Michael University Heart Center, Department of Cardiology, University Hospital Zurich, and University of Zurich, Zurich, Switzerland

Schweiger Victor University Heart Center, Department of Cardiology, University Hospital Zurich, and University of Zurich, Zurich, Switzerland

Rajman Katja University Heart Center, Department of Cardiology, University Hospital Zurich, and University of Zurich, Zurich, Switzerland

Di Vece Davide Internal Medicine B, University Medicine Greifswald, Greifswald, Germany

Gilhofer Thomas Department of Cardiology, Cantonal Hospital Winterthur, Winterthur, Switzerland

Ghadri Jelena R Center for Molecular Cardiology, Schlieren Campus, University of Zurich, Zurich, Switzerland

https://orcid.org/0000-0003-0287-4193
Templin Christian Internal Medicine B, University Medicine Greifswald, Greifswald, Germany
Center for Molecular Cardiology, Schlieren Campus, University of Zurich, Zurich, Switzerland
Private Hospital Bethanien, Swiss CardioVascularClinic (Swiss CVC), Zurich, Switzerland

Karamasis Grigoris Handling Editor
Kodaira Masaki Editor
Alahmad Yaser Editor
Spinthakis Nikolaos Editor
Corresponding author. Tel: +49 3834 86 80500, Email: christian.templin@med.uni-greifswald.de
Conflict of interest: C.T. has been supported by the H.H. Sheikh Khalifa bin Hamad Al-Thani Research Programme and received institutional grants from Abbott Vascular, Medtronic, SMT, as well as consulting grants from Biotronik, Microport, and Innova.

9 2024
11 9 2024
11 9 2024
8 9 ytae45111 10 2023
28 2 2024
19 8 2024
16 9 2024
© The Author(s) 2024. Published by Oxford University Press on behalf of the European Society of Cardiology.
2024
https://creativecommons.org/licenses/by-nc/4.0/ This is an Open Access article distributed under the terms of the Creative Commons Attribution-NonCommercial License (https://creativecommons.org/licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact reprints@oup.com for reprints and translation rights for reprints. All other permissions can be obtained through our RightsLink service via the Permissions link on the article page on our site—for further information please contact journals.permissions@oup.com.

Abstract

Background

Spontaneous coronary artery dissection (SCAD) is the most important cause of acute coronary syndromes during pregnancy and in the post-partum period and involves a spontaneous intimal tear or intramural haematoma of a coronary artery. Pregnancy-associated SCAD accounts for a minority of SCAD cases but is associated with a high rate of adverse events.

Case summary

We present a series of three cases with pregnancy-associated SCAD. All patients presented with acute coronary syndromes in the post-partum period, between 12 days and 5 months after delivery. They all had additional conditions that are associated with SCAD, such as fibromuscular dysplasia and migraine. The management of one patient was uncomplicated, however, the courses of the other two were characterized by adverse events. One presented after an out-of-hospital cardiac arrest, the other presented with multivessel SCAD and developed progression and recurrence of SCAD during follow-up. In conclusion, the patients could be successfully treated conservatively and were in good condition at their latest follow-ups.

Discussion

This case series highlights the wide range of clinical courses that could exist in pregnancy-associated SCAD, from a benign manifestation to a life-threatening condition. Importantly, those patients are at an increased risk for acute and late adverse events.

Spontaneous coronary artery dissection
SCAD
Pregnancy
Peripartum
Post-partum
Case series
Case report
Swiss Heart Foundation 10.13039/501100004362
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pmcLearning points

Spontaneous coronary artery dissection (SCAD) is the most important differential diagnosis of acute coronary syndromes during pregnancy and in the post-partum period.

The clinical course of pregnancy-associated SCAD has a wide spectrum, ranging from minor symptoms to life-threatening manifestations.

Specific obstetric considerations such as teratogenic effects of pharmaceuticals and radiation must be taken into account in the management of pregnancy-associated SCAD, and conservative therapy should be preferred.

Introduction

Spontaneous coronary artery dissection (SCAD) involves a spontaneous tear in the intimal layer of a coronary artery or a rupture of the vasa vasorum resulting in the development of an intramural haematoma. This causes luminal compression, which can lead to myocardial ischaemia and infarction.1 Spontaneous coronary artery dissection was first described in a 42-year-old woman with sudden cardiac death in 1931.2 Previously assumed to be a rare disease predominantly affecting pregnant women, it is nowadays recognized as a crucial differential diagnosis of acute coronary syndrome (ACS) in all age groups and both sexes.3,4

Only a minority of cases exhibit the classic angiographic appearance of a dissection membrane, and an angiographic classification has been introduced in the meantime: type 1 is characterized by the above-mentioned classic appearance with contrast dye staining of multiple lumina, type 2 describes a long diffuse and smooth stenosis, type 3 is defined as a focal or tubular stenosis,1 and type 4 is characterized by an abrupt vessel occlusion.5

Spontaneous coronary artery dissection is frequently associated with fibromuscular dysplasia (FMD) with a varying prevalence of 13–86% in SCAD patients.3,6 Fibromuscular dysplasia is defined as an idiopathic, non-atherosclerotic, non-inflammatory disease of the arterial wall that typically involves the renal, cervical, and visceral arteries. The two subtypes include the focal type with a single arterial stenosis and the more frequent multifocal type with sequential stenoses and a typical ‘string-of-beads’ pattern.7

Still, SCAD remains the most important cause of ACS in young women, especially during pregnancy and in the post-partum period.8 We present a series of three cases with post-partum SCAD to demonstrate the spectrum of different clinical courses of this disease.

Summary figure

Patient 1

A 36-year-old female patient presented with left-sided chest pain radiating to her left arm 12 days after the delivery of her fourth child. The patient had a history of mild gestational diabetes during pregnancy and migraine. On admission, the patient exhibited stable haemodynamics without signs of congestion. The electrocardiogram (ECG) demonstrated a Wellens type A pattern in the anterior leads (Figure 1A). Troponin T levels were elevated [peak hs-Troponin T 670 ng/L (<14 ng/L)].

Figure 1 Images for Patient 1. (A) ECG at admission demonstrating Wellens type A pattern in the anterior leads. (B) Coronary angiogram with spontaneous coronary artery dissection type 2B of the mid (arrow) to apical left anterior descending artery. (C) Magnetic resonance angiography with 3D reconstruction of the vertebral arteries demonstrating a ‘string-of-beads’ pattern of the left vertebral artery (arrow), consistent with the diagnosis of fibromuscular dysplasia.

The diagnosis of an ACS was made, and an urgent coronary angiography (CAG) was performed. Herein, SCAD type 2B of the mid to apical left anterior descending artery (LAD) with normal coronary flow [thrombolysis in myocardial infarction (TIMI) 3] distal to the lesion was diagnosed (Figure 1B). Conservative treatment with life-long aspirin, clopidogrel for one year, an angiotensin-converting enzyme (ACE)-inhibitor, and a betablocker was initiated.

Following an uneventful course, the patient was discharged after 9 days. The patient weaned her child according to the recommendation of her treating physicians based on an association of SCAD with breastfeeding. Screening for vasculopathies by magnetic resonance angiography demonstrated FMD of the vertebral arteries (Figure 1C). Her antihypertensive therapy was intensified by addition of a calcium-channel-blocker. The long-term outcome over the next five years was favourable without recurrence and with normal left ventricular ejection fraction (LVEF) despite minor residual akinesia of the apex.

Patient 2

A 39-year-old female patient was admitted after an out-of-hospital cardiac arrest due to ventricular fibrillation 17 days after the delivery of her second child. This was her fifth pregnancy, with a history of three habitual abortions. She experienced an acute migraine attack starting 2 days prior to the acute event. The spontaneous circulation was restored after nine minutes of resuscitation including three defibrillation shocks. On hospital admission, the patient presented with cardiogenic shock and pulmonary oedema. However, prompt administration of intravenous norepinephrine, epinephrine, and furosemide stabilized her haemodynamics and alleviated pulmonary congestion. The ECG displayed ST-segment elevations in leads III, aVR, and V1 (Figure 2A), prompting the need for emergency CAG.

Figure 2 Images for Patient 2. (A) ECG at admission with ST-segment elevations in leads III, aVR, and V1. (B) Coronary angiogram demonstrating spontaneous coronary artery dissection type 2B of the mid (arrow) to apical left anterior descending artery. (C) Second-look coronary angiography with spontaneous partial healing of the dissection (arrow) 5 days later.

Spontaneous coronary artery dissection type 2B of the mid to apical LAD was diagnosed (Figure 2B). The patient received a conservative treatment with aspirin and heparin until a planned second-look CAG.

The patient was successfully weaned from vasoactive drugs and extubated after a 24 h period of controlled temperature management. Biomarkers peaked on the day of admission [peak hs-Troponin T 2929 ng/L (<14 ng/L)]. The second-look CAG 5 days later already demonstrated partial healing of the SCAD lesion (Figure 2C). An angiography of the renal arteries did not reveal any evidence of FMD. She was discharged in a good condition the day after, receiving life-long aspirin, an ACE-inhibitor, and a betablocker. She weaned her child from breastfeeding. The further course was uneventful for four years of follow-up without recurrence and with a normal LVEF without regional wall motion abnormalities.

Patient 3

A 37-year-old female patient was admitted by ambulance after the diagnosis of an acute ST-segment elevation myocardial infarction (STEMI) with ST-segment elevations in both the anterior and inferior leads (Figure 3A). The patient was currently breastfeeding after the birth of her first child five months earlier. The pregnancy was induced by assisted fertilization.

Figure 3 Images for Patient 3. (A) ECG at admission with ST-segment elevations in both the anterior and inferior leads. (B) Multivessel spontaneous coronary artery dissection with the angiographic detection of a dissection type 2A (arrow) of the posterolateral branch of the right coronary artery with a normal vessel distal to the dissection (asterisk), abrupt stenosis of the posterior descending artery due to dissection type 4 (arrowhead), and (C) dissection type 2B of the left anterior descending artery (arrow). (D) Progression of spontaneous coronary artery dissection in the left anterior descending artery with complete stenosis (arrow) 2 days later and (E) improved flow (TIMI 2) after treatment by wire crossing. (F) Recurrence of SCAD one year later with a minor ischaemic area (arrow) in the anterolateral midventricular segment in cardiac magnetic resonance tomography, which (G) was attributable to the territory of the second diagonal branch (circle), as shown by coronary computed tomography. (H) Magnetic resonance angiography demonstrating an old dissection of the left vertebral artery (arrow) and (I) irregularities of the vertebral arteries (arrowhead), suggestive of fibromuscular dysplasia.

An emergency CAG confirmed a multivessel SCAD of the mid to apical LAD (type 2B), the posterior descending artery (type 4), and the posterolateral branch (type 2A) of the right coronary artery (Figure 3B and C). Her haemodynamics were stable, and she was pain-free at the end of the procedure. Conservative treatment with aspirin and heparin was initiated, and a second-look CAG was planned.

Two days later, the patient developed recurrent chest pain and anterior ST-segment elevations. An emergency CAG demonstrated the progression of SCAD with a complete occlusion of the mid LAD (Figure 3D). Revascularization of the LAD was attempted, and the coronary flow could be improved (TIMI 2) (Figure 3E) after wire crossing of the lesion without further coronary intervention. The further in-hospital course was uneventful with a peak of biomarkers after 4 days [peak hs-Troponin T 2766 ng/L (<14 ng/L)], and the patient was discharged 10 days after presentation. Transthoracic echocardiography showed a normal LVEF with apical akinesia. Her child was weaned from breastfeeding. Medical therapy consisted of life-long aspirin, clopidogrel for one year, and an ACE-inhibitor.

One year later, the patient experienced recurrence of SCAD in the first diagonal branch after sports, diagnosed by coronary computed tomography (Figure 3G). Non-invasive management was implemented due to spontaneous relief of pain, low cardiac biomarkers [peak hs-Troponin T 99 ng/L (<14 ng/L)], and only minor ischaemia in cardiac magnetic resonance tomography (Figure 3F). A betablocker was added to her established therapy. The patient had an uneventful cardiac course over the next seven years. However, the magnetic resonance angiography, which was performed following recurrent headaches, demonstrated an old dissection of the left vertebral artery and irregularities of both vertebral arteries, suggestive of FMD (Figure 3H and I).

Discussion

This case series describes three different clinical courses of pregnancy-associated SCAD. Spontaneous coronary artery dissection is the primary differential diagnosis of ACS during pregnancy and the post-partum period, accounting for nearly half of such cases. Most patients present either in the post-partum period (73%) or during the third trimester of pregnancy (21%).8 Pregnancy confers a threefold increased risk of SCAD compared to non-pregnant women of the same age.9,10 Women with pregnancy-associated SCAD experience worse outcomes compared to those with SCAD unrelated to pregnancy. They have higher incidences of cardiac arrest, cardiogenic shock, STEMI, and multivessel SCAD, along with higher in-hospital mortality rates.3

All above-mentioned patients had additional risk factors for SCAD besides pregnancy. Those are age > 30 years, preeclampsia, hypertension, diabetes, multiparity, infertility treatment, migraine, and FMD.3,9,10 Interestingly, all three cases were associated with migraine. Whether migraine is a particular strong risk factor in post-partum SCAD needs to be elucidated.

The pathophysiological interaction is unclear for many of these risk factors, however weakening of the vessel architecture following hormonal changes during pregnancy has been postulated.11 Fibromuscular dysplasia is very well known to be associated with SCAD, and screening for FMD is recommended in every patient.3 On the other hand, only a minority (2.7%) of FMD patients develop SCAD, as shown in the US Registry for FMD.12 Nevertheless, more intensive follow-up by a team involving both a high-risk obstetrician and a provider with expertise in FMD and a customized care plan, including planned caesarean section or facilitated second stage of labour, are recommended for patients with FMD.7

Coronary angiography is considered the gold standard for the diagnosis of SCAD due to its high spatial and temporal resolution. However, during pregnancy, the indication of CAG needs to be discussed on a case-by-case basis due to the risk of radiation damage to the unborn child. In the absence of ongoing ischaemia or haemodynamic instability, conservative treatment is preferred in patients with an established diagnosis of SCAD, since complications of percutaneous coronary interventions are frequent and spontaneous healing occurs in most patients.3,4

The long-term outcomes of SCAD are affected by a high rate of adverse cardiac events (up to 37% after five to seven years), mostly due to the recurrence of SCAD in different vessel segments.3,4 The optimal medical therapy is still under debate since no prospective data exist. Retrospectively, beneficial effects have only been reported for betablockers and an adequate blood pressure management.3,4

Many clinicians counsel for the discontinuation of breastfeeding after SCAD, as it has been associated with post-partum SCAD.3 Of note, some patients were reporting the onset of SCAD during lactation.11 Only small series investigated the risk of subsequent pregnancies. A series of eight patients with pregnancy after SCAD showed one recurrence after delivery.13 Therefore, some experts do not recommend subsequent pregnancies after SCAD, nevertheless more recent expert reviews have pushed this into perspective.14 It is recommended that women, who plan to become pregnant, should be referred to a tertiary centre equipped with an interdisciplinary team experienced in the management of SCAD.3,14

Conclusion

This case series highlights the wide range of different clinical courses that could exist in pregnancy-associated SCAD, from a benign manifestation to a life-threatening condition. Acute and late complications are frequent in this population, and a close and careful follow-up is essential in patients with pregnancy-associated SCAD.

Lead author biography

Michael Würdinger, MD, is a cardiologist in training at the University Hospital Zurich, Switzerland. He graduated from the University of Regensburg, Germany, and completed a specialization in internal medicine at the University Hospital Zurich. His clinical and research interests lie on interventional cardiology and coronary artery diseases, with a special focus on non-atherosclerotic acute coronary syndromes.

Consent: The authors confirm that consent was obtained from the patients in accordance with COPE guidelines.

Funding: This study was supported by the Swiss Heart Foundation.

Data availability

All data underlying this article are available in the article.
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