Pregnancy-related spontaneous coronary dissection revealing a fibromuscular dysplasia: a case report
Rahma Kallel, Rania Hammami, Aymen Dammak, Leila Abid, Imed Frikha, Samir Kammoun
Corresponding author: Rania Hammami, Cardiology Department, Hedi Chaker Hospital, Faculty of Medicine of Sfax, Sfax, Tunisia
Received: 18 Jun 2020 - Accepted: 02 Apr 2024 - Published: 15 Jul 2024
Domain: Cardiology
Keywords: Coronary artery dissection, spontaneous, peripartum period, fibromuscular dysplasia, case report
©Rahma Kallel et al. PAMJ Clinical Medicine (ISSN: 2707-2797). This is an Open Access article distributed under the terms of the Creative Commons Attribution International 4.0 License (https://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Cite this article: Rahma Kallel et al. Pregnancy-related spontaneous coronary dissection revealing a fibromuscular dysplasia: a case report. PAMJ Clinical Medicine. 2024;15:28. [doi: 10.11604/pamj-cm.2024.15.28.24397]
Available online at: https://www.clinical-medicine.panafrican-med-journal.com//content/article/15/28/full
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Pregnancy-related spontaneous coronary dissection revealing a fibromuscular dysplasia: a case report
Pregnancy-related spontaneous coronary dissection revealing a fibromuscular dysplasia: a case report
Rahma Kallel1, Rania Hammami1,&, Aymen Dammak2, Leila Abid1, Imed Frikha2, Samir Kammoun1
&Corresponding author
Spontaneous coronary artery dissection (SCAD) is a serious pathology. The mechanism is poorly understood. The diagnosis has largely benefited from the development of intra-coronary imaging, but the treatment is still not consensual. We report the case of a 32-year-old woman with a history of aspirin allergy, who presented with an ST elevation acute coronary syndrome, one month after delivery. The coronary angiography showed a spiroid dissection of the right coronary and an ulceration of the left main coronary. We discovered a lesion in the left external iliac artery consistent with fibromuscular dysplasia, but renal arteries were spared. The management of this patient was challenging regarding allergy antecedents, the antiplatelet treatment should be extended for a long time, considering fibromuscular dysplasia. Even though hormonal impregnation during the peripartum period may explain SCAD, the systematic etiologic investigation was very contributive to adapting the management and the follow-up of the patient.
Spontaneous coronary artery dissection (SCAD) is an uncommon cause of acute coronary syndrome. It often affects young women, and it is responsible for non-negligible morbidity and mortality [1]. Through a spontaneous coronary artery dissection, with specially superposed predisposing and stressor factors, we propose to understand the physio-pathological mechanism, the etiologies, and the management of this entity.
Patient information: we report the case of a 32-year-old multigravida woman, with a history of aspirin allergy, who presented to the emergency room, one month after delivery, for breathlessness.
Clinical findings: at the interrogation, the patient reported intense chest pain during labor, which persisted a few hours later. The physical examination was normal apart from tachycardia.
Diagnostic assessment: the electrocardiogram showed Q waves in inferior leads. The chest radiography was unremarkable. The troponin was negative. Cardiac echography revealed a dilated left ventricle, globally hypokinetic with inferior akinesia, and a deteriorated systolic function with a left ventricle ejection fraction (LVEF) = 35%.
Diagnosis: we thought about cardiomyopathy of the peripartum, but the cardiac MRI showed an akinetic inferior wall with a late subendocardial enhancement. The coronary angiography showed a spiroid dissection of the right coronary beginning at the proximal segment with an aneurismal lesion in the middle segment and an image of addition in the left main related to an ulcerated plaque (Figure 1). The spontaneous coronary artery dissection was retained.
Therapeutic interventions: the angioplasty of the right coronary has been discussed. Considering antecedents of allergy to aspirin, the large size of the lesion, and the clinical stability of the patient, we opted for a medical treatment. The patient was treated with clopidogrel, we stopped heparin fearing an extension of the intra-parietal hematoma and we started heart failure treatment. The desensitization to aspirin was tried but failed twice. We performed many explorations in search of the etiology of SCAD. The echo-Doppler of the supra-aortic trunks as well as the CT scan of renal arteries were normal, but we discovered a fibromuscular dysplasia of the left external iliac artery. The immunologic and thrombophilia investigations were negative. The specialized clinical exam and the biological test did not reveal arguments in favor of an underlying chronic inflammatory pathology.
Follow-up and outcome of interventions: the control coronary angiography practiced 6 months later revealed the healing of the ulcerated lesion in the left coronary trunk and a partial improvement of the right coronary lesion (Figure 2). The echocardiography practiced at one year objectified a decrease in the size of the left ventricle with an improvement of the systolic function LVEF at 45%, but the inferior wall was akinetic with no viability on dobutamine echocardiography. Thus, we continue the medical treatment.
The patient's perspective: the patient appreciated the efforts of the clinical team and was happy to regain her sight. She promised to continue taking the treatment for heart failure and to avoid any new pregnancies.
Informed consent: the patient gave her informed written consent.
Spontaneous coronary dissection is a non-traumatic or iatrogenic separation of artery walls, creating a wrong lumen [1]. When relying on angiography, the incidence of spontaneous coronary dissection is variable, going from 0.07 to 1.1% [2,3]. It will be about 3 to 4% according to studies based on data from tomography with optical coherence (OCT) [2,4,5]. Thirty percent of SCAD occur in peripartum. The peak of occurrence coincides with the first two weeks of post-partum, but SCAD could happen even many months after delivery, especially in women still breastfeeding [6-8]. The differential diagnosis of Meadows syndrome is sometimes difficult because of the clinical context and the consequences on the systolic function of the left ventricle. In our case, the cardiac MRI had a great impact, the sub-endocardial localization of late enhancement was in favor of an ischemic cardiomyopathy, but during pregnancy, gadolinium could not be used, and then it will be difficult to make the true diagnosis. As a matter of fact, during pregnancy hormone changes especially Estrogen and progesterone surges and hemodynamic variations with > 50% increase in cardiac output result in the weakening of the aortic wall which may extend to coronary [1,9]. During labor, the increase of intra-abdominal pressures could increase vascular shear stresses, leading to dissection.
The sophistication of etiologic investigation, in particular the systematic research of fibromyodysplasia has allowed us to better detect this pathology which has turned out to be a predisposing arteriopathy to coronary dissection. Saw et al. mentioned in a series of 168 patients with SCAD that 72% of cases were linked to fibromyodysplasia [10]. For retaining fibromyodysplasia as the etiology of coronary dissection, it is necessary to find at least another localization of the disease outside coronaries [11]. In our patient, the aneurismal aspect of the right coronary was evocative of the disease, but the artery pressure and kidney function were normal, and thoracic cerebral and abdominal imaging didn´t reveal other vascular localizations. Given the spontaneous tendency to the healing of lesions, the conservative strategy is generally proposed in stable patients under medical treatment with a normal coronary flow despite the dissection [8,12]. The anti-platelet treatment has shown useful in reducing thrombotic risk; however, some authors suggested an extension of intra-parietal hematoma with heparin therapy. Some teams stop heparin as soon as the angiographic confirmation of coronary dissection [1].
In patients with persistent chest pain, ischemia, or hemodynamic instability, percutaneous revascularization could be tried; but the rate of technical success is inferior to atherosclerosis lesions. This rate has not exceeded 30% according to Saw et al. review [10], it was 65% in the series of Tweet et al. [13]. The technical difficulties are multiple, such as the risk of passage of coronary guide through the false lumen, the risk of extension of dissection in the anterograde and retrograde direction, and the choice of stent. In our patient, we opted for medical treatment because of the clinical stability, the extent of the lesion, and the impossibility of prescribing aspirin resulting in a high risk of stent thrombosis.
Spontaneous coronary artery dissection is a challenging diagnosis condition with no clear consensual management. The differential diagnosis with peripartum myocarditis or a Taku-Tsubo syndrome is sometimes difficult, and the cardiac MRI is very contributive. Through our case of SCAD within a young woman in the peripartum period, the methodical investigation revealed fibromyodysplasia. Thus, the etiologic investigation must be systematic to adapt the management to the pathology rather than the lesions.
The authors declare no competing interests.
Rania Hammami, Rahma Kallel: patient management, data collection. Rahma Kallel: manuscript drafting. Manuscript revision: Rania Hammami. All authors revised critically the paper and approved the final version of the manuscript.
Figure 1: (A, B) the coronary angiography: spiroid dissection of the right coronary and an image of addition in the left main related to an ulcerated plaque
Figure 2: (A, B) the control coronary angiography practiced 6 months later: healing of the ulcerated lesion in the left coronary trunk and a partial improvement of the right coronary lesion
- Yip A, Saw J. Spontaneous coronary artery dissection-A review. Cardiovasc Diagn Ther. 2015;5(1):37-48. PubMed | Google Scholar
- Mortensen KH, Thuesen L, Kristensen IB, Christiansen EH. Spontaneous coronary artery dissection: a Western Denmark Heart Registry study. Catheter Cardiovasc Interv Off J Soc Card Angiogr Interv. 2009;74(5):710-7. PubMed | Google Scholar
- Vanzetto G, Berger-Coz E, Barone-Rochette G, Chavanon O, Bouvaist H, Hacini R et al. Prevalence, therapeutic management and medium-term prognosis of spontaneous coronary artery dissection: results from a database of 11,605 patients. Eur J Cardio-Thorac Surg Off J Eur Assoc Cardio-Thorac Surg. 2009;35(2):250-4. PubMed | Google Scholar
- Hill SF, Sheppard MN. Non-atherosclerotic coronary artery disease associated with sudden cardiac death. Heart Br Card Soc. 2010;96(14):1119-25. PubMed | Google Scholar
- Hering D, Piper C, Hohmann C, Schultheiss HP, Horstkotte D. Prospective study of the incidence, pathogenesis and therapy of spontaneous, by coronary angiography diagnosed coronary artery dissection. Z Für Kardiologie. 1998;87(12):961-70. PubMed | Google Scholar
- Vrints CJM. Spontaneous coronary artery dissection. Heart Br Card Soc. 2010;96(10):801-8. PubMed | Google Scholar
- Rajab TK, Khalpey Z, Kraemer B, Resnic FS, Gallegos RP. Recurrent post-partum coronary artery dissection. J Cardiothorac Surg. 2010;5:78. PubMed | Google Scholar
- Alfonso F, Paulo M, Lennie V, Dutary J, Bernardo E, Jiménez-Quevedo P et al. Spontaneous coronary artery dissection: long-term follow-up of a large series of patients prospectively managed with a « conservative » therapeutic strategy. JACC Cardiovasc Interv. oct 2012;5(10):1062-70. PubMed | Google Scholar
- Manalo-Estrella P, Barker AE. Histopathologic findings in human aortic media associated with pregnancy. Arch Pathol. 1967;83(4):336-41. PubMed | Google Scholar
- Saw J, Aymong E, Sedlak T, Buller CE, Starovoytov A, Ricci D et al. Spontaneous coronary artery dissection: association with predisposing arteriopathies and precipitating stressors and cardiovascular outcomes. Circ Cardiovasc Interv. oct 2014;7(5):645-55. PubMed | Google Scholar
- Michelis KC, Olin JW, Kadian-Dodov D, d´Escamard V, Kovacic JC. Coronary artery manifestations of fibromuscular dysplasia. J Am Coll Cardiol. 2014;64(10):1033-46. PubMed | Google Scholar
- Tweet MS, Eleid MF, Best PJM, Lennon RJ, Lerman A, Rihal CS et al. Spontaneous coronary artery dissection: revascularization versus conservative therapy. Circ Cardiovasc Interv. 2014;7(6):777-86. PubMed | Google Scholar
- Tweet MS, Hayes SN, Pitta SR, Simari RD, Lerman A, Lennon RJ et al. Clinical features, management, and prognosis of spontaneous coronary artery dissection. Circulation. 2012;126(5):579-88. PubMed | Google Scholar