Spontaneous Dissection of Three Major Coronary Arteries Subsequent to Cystic Medial Necrosis

doi:10.1378/chest.116.5.1473

Chest

Volume 116, Issue 5, November 1999, Pages 1473-1475

https://doi.org/10.1378/chest.116.5.1473 Get rights and content

This case report describes the devastating consequences of spontaneous coronary dissection in a 36-year-old female patient. Surgical revascularization was attempted, but diffuse myocardial infarction developed. The patient was bridged to heart transplantation but died secondary to multiple organ failure. To our knowledge, this is the only reported case of spontaneous dissection of the three main coronary arteries due to severe cystic medial necrosis.

Section snippets

Case Report

A 36-year-old white woman experienced prolonged crushing precordial pain that started during light exercise. On admission to the hospital, acute anterior myocardial infarction was diagnosed and IV alteplase was administered. Cardiac enzymes were moderately increased (creatine kinase [CK]) max of 1,500 U/L, 13% MB fraction), and echocardiography revealed only a limited region of hypokinesia confined to the left ventricular apex. The patient was referred to a tertiary hospital 2 days later so

Discussion

Spontaneous dissection of the coronary arteries is a rare entity, mostly recognized postmortem in young victims of sudden death.¹ It is the result of an intramural hematoma in the media of the arterial wall that creates a false lumen. Expansion of this lumen through blood or clot accumulation leads to compression of the real lumen and myocardial ischemia. An intimal tear is only seldom observed. Most reported patients are apparently healthy, young to middle-aged women (78%; mean age, 40 years)²

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Cited by (39)

The presence of thrombus in spontaneous coronary artery dissection: A systematic review of autopsy findings
2022, American Heart Journal Plus: Cardiology Research and Practice
Spontaneous coronary artery dissection (SCAD) is an important cause of acute coronary syndrome in young women. There is no consensus on optimal treatment, though a conservative approach including antiplatelet agents is commonly used. We hypothesized that most cases of SCAD would not demonstrate true lumen thrombus in the dissected artery, suggesting that anti-platelet agents might not have a role in the treatment of SCAD.
We conducted a systematic review of the published literature through March 2022 to identify pathology images from individuals who died of SCAD. The images were independently reviewed by a pathologist to assess for the presence of thrombus and inflammatory cells.
We identified 40 cases from 34 publications with available pathology images and found only one case of true lumen thrombus. Additionally, we found that 53% of cases involved eosinophilic inflammation.
The role of antiplatelet agents in the treatment of SCAD should be re-evaluated. Further studies are needed to better understand the significance and treatment implications of eosinophilic inflammation.
Spontaneous Coronary Artery Dissection Associated With Ascending Aortic Dystrophy: A Tear for Fear
2015, Canadian Journal of Cardiology
A 36-year-old man with a recent history of surgery for an aortic valve ascending aortic aneurysm was admitted for non–ST-elevation myocardial infarction. Cardiac magnetic resonance imaging showed a limited necrotic lesion in the anterior wall. Coronary angiography showed no significant stenosis, but a mild distal dynamic tortuosity was seen on the left anterior descending artery. Optical coherence tomography analysis confirmed the absence of any significant atherosclerotic infiltration but revealed the presence of a localized short arterial dissection within the kinked zone. The patient was managed with a conservative medical approach. The subsequent evolution was uneventful.
Un homme de 36 ans ayant des antécédents de chirurgie récente d’un anévrisme de l’aorte ascendante et d’une réparation de la valve aortique a été admis en raison d’un infarctus du myocarde sans sus-décalage du segment ST. L’imagerie cardiaque par résonance magnétique a montré une petite lésion nécrotique de la paroi antérieure. L’angiographie coronarienne n’a montré aucune sténose significative, mais une légère tortuosité dynamique distale de l’artère interventriculaire antérieure a été observée. L’analyse en tomographie par cohérence optique a confirmé l’absence de toute infiltration athéroscléreuse significative, mais a révélé la présence d’une petite dissection artérielle localisée dans la zone tortueuse. Le patient a été pris en charge selon une approche médicale traditionnelle. L’évolution subséquente s’est déroulée sans incident.
Spontaneous coronary artery dissection
2014, International Journal of Cardiology
Citation Excerpt :
Although up to 80% of patients with a Marfan syndrome have coronary cystic medial necrosis, coronary dissections are more frequently due to extension of an aortic dissection [59,60]. Cystic medial necrosis has been described in 38% of SCAD, but only exceptionally a true pattern in non-Marfan patients has been associated with SCAD [7,61–64]. Loeys–Dietz syndrome and Neurofibromatosis type I are autosomal dominant disorders characterized by early mortality for dissection/rupture of the aorta and renal arteries, respectively.
Spontaneous coronary artery dissection (SCAD) is a relatively rare and unexplored type of coronary disease. Although atherosclerosis, hormonal changes during pregnancy and connective tissue disorders might represent a sufficiently convincing explanation for some patients with SCAD, the many remaining cases display only a weak relationship with these causes. While on one side the clinical heterogeneity of SCAD masks a full understanding of their underlying pathophysiologic process, on the other side paucity of data and misleading presentations hamper the quick diagnosis and optimal management of this condition. A definite diagnosis of SCAD can be significantly facilitated by endovascular imaging techniques. In fact, intravascular ultrasound (IVUS) and optical coherence tomography (OCT) overcome the limitations of coronary angiography providing detailed endovascular morphologic information. In contrast, optimal treatment strategies for SCAD still represent a burning controversial question. Herein, we review the published data examining possible causes and investigating the best therapy for SCAD in different clinical scenarios.
Acute myocardial infarction due to spontaneous postpartum multi-vessel coronary artery dissection
2014, Journal of Cardiology Cases
Citation Excerpt :
Immune system diseases such as systemic lupus erythematosus, type IV Ehler Danlos and Marfan's syndrome, hypersensitivity angiitis, sarcoidosis, Kawasaki disease, and rheumatoid arthritis with coronary arteritis can cause SCAD. Conraads and others found also a cystic medial necrosis and other defects in collagen metabolism in some patients [12,13]. The treatment depends on clinical presentation, myocardial ischemia, clinical stability of the patient, the number of vessels involved and the availability of coronary intervention services.
Spontaneous coronary artery dissection is a rare cause of myocardial infarction in young, otherwise healthy people. We present a case report of a 37-year-old woman, without cardiovascular risk factors, who survived a major acute myocardial infarction due to multi-vessel spontaneous coronary artery dissection which was complicated by cardiogenic shock in the third week postpartum. She fully recovered with medical therapy in combination with angioplasty.
<Learning objective: Acute myocardial infarction (AMI) during pregnancy and postpartum is a rare but often catastrophic event and spontaneous coronary artery dissection is the most frequent cause of AMI in this population. Because of the high mortality rate, it is important to recognize this entity in the early stage of presentation. Coronary angiography remains the golden standard and should be performed without hesitation in unstable patients.>
Spontaneous coronary dissection and its long-term prognostic implications in a cohort of 19 cases
2010, Revista Espanola de Cardiologia
La disección coronaria espontánea es una causa infrecuente de síndrome coronario agudo que afecta predominantemente a mujeres sin apenas factores de riesgo cardiovascular y cuya presentación varía desde la angina inestable hasta la muerte súbita. Los conocimientos de esta entidad se reducen a casos clínicos aislados, y la ausencia de grandes series hace que su tratamiento y sus implicaciones pronósticas no estén plenamente establecidos. Presentamos los datos de 19 casos en 18 pacientes atendidos en nuestro centro desde mayo de 1998 hasta enero de 2009, con una mediana [intervalo intercuartílico] de 3,8 [1,3-4,6] años de seguimiento. Una vez superada la fase aguda, el pronóstico fue favorable sin implicaciones funcionales. Una paciente presentó una recidiva en una coronaria diferente y otra paciente dio a luz sin complicaciones a los 3 años de la disección.
Spontaneous coronary artery dissection is a rare cause of acute coronary syndrome. It mainly affects women with no significant cardiovascular risk factors and its presentation varies from unstable angina to sudden death. Knowledge of the condition is based only on individual case reports and the lack of large case series means that its treatment and prognostic implications have not been fully established. We present data on 19 instances of spontaneous coronary artery dissection in 18 patients who were treated at our center between May 1998 and January 2009. The median follow-up period was 3.8 years (interquartile range: 1.3-4.6 years). Once the acute phase had passed, the prognosis was favorable and there were no implications for functioning. One patient presented with a relapse in another coronary artery and another patient gave birth without complications 3 years after the dissection.
Spontaneous coronary artery dissection of all major coronary arteries
2007, Canadian Journal of Cardiology
Spontaneous coronary artery dissection is a rare cause of myocardial infarction and sudden cardiac death. A 32-year-old man presented with effort angina. He had a positive treadmill exercise electrocardiogram test, and coronary angiography showed that he had dissection of all major coronary vessels. The left anterior descending coronary artery was completely blocked, probably secondary to dissection and subsequent occlusion. He was advised to undergo coronary artery bypass surgery, to which he did not agree; instead, he was treated by medication and followed up.
La dissection spontanée des artères coronaires est une cause rare d’infarctus du myocarde et de mort subite. Voici le cas d’un homme de 32 ans qui a consulté pour des douleurs angineuses à l’effort. Une épreuve d’effort sur tapis roulant s’est révélée positive, et la coronarographie a montré une dissection de toutes les grosses artères coronaires. L’artère interventriculaire antérieure gauche était complètement oblitérée, probablement à cause de la dissection et de l’obturation qui s’en est suivie. On a conseillé au patient de subir un pontage coronarien, mais il a refusé; il a donc été traité par des moyens médicaux et a fait l’objet de suivi.

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Chest

Selected ReportsSpontaneous Dissection of Three Major Coronary Arteries Subsequent to Cystic Medial Necrosis

Section snippets

Case Report

Discussion

Am Heart J

Chest

Spontaneous coronary artery dissection: a neglected cause of acute myocardial ischemia and sudden death

Heart

Cocaine-induced coronary-artery dissection

N Engl J Med

Selected Reports
Spontaneous Dissection of Three Major Coronary Arteries Subsequent to Cystic Medial Necrosis