Idiopathic Spontaneous Coronary Artery Dissection

Complex MONDO:0007385 Pathograph 4 Vascular disorder Idiopathic disease

Idiopathic spontaneous coronary artery dissection is a non-traumatic, non-iatrogenic coronary arteriopathy in which separation of the coronary arterial wall, often with intramural hematoma, narrows the true lumen and causes acute myocardial ischemia. It is distinct from atherosclerotic coronary artery disease and is classically recognized in younger or middle-aged women, including peripartum and postpartum presentations.

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2
Pathophys.
6
Phenotypes
4
Pathograph
1
Genes
4
Treatments
1
Trials
1
Deep Research
⚙

Pathophysiology

2
Coronary arterial wall hematoma and false lumen formation
A tear or hemorrhage within the coronary arterial wall creates a false lumen or intramural hematoma. Expansion of this wall hematoma compresses the true lumen, limits coronary blood flow, and can trigger myocardial ischemia or infarction.
vascular smooth muscle cell link endothelial cell link
blood vessel remodeling link ⚠ ABNORMAL blood coagulation link ⚠ ABNORMAL
coronary artery link tunica media link
Downstream
Coronary flow limitation Intramural hematoma or false-lumen expansion compresses the true lumen and reduces coronary perfusion.
Myocardial ischemia and infarction Reduced coronary perfusion can produce angina, myocardial infarction, arrhythmia, or cardiogenic shock.
Show evidence (2 references)
PMID:38390446 SUPPORT Human Clinical
"Spontaneous coronary artery dissection (SCAD) is a non-traumatic and non-iatrogenic separation of the coronary arterial wall."
Systematic review supports the core lesion as spontaneous coronary arterial-wall separation.
PMID:39742239 SUPPORT Human Clinical
"no clear data are available about antiplatelet treatment because of the supposed risk of intramural hematoma enlargement."
Review identifies intramural hematoma enlargement as a clinically relevant SCAD mechanism considered during management.
Nonatherosclerotic arteriopathy and vessel wall vulnerability
Many idiopathic SCAD presentations occur without plaque rupture, atherosclerotic stenosis, trauma, or instrumentation. A systemic nonatherosclerotic arteriopathy or extracellular matrix vulnerability is a common clinical framework, especially when extracoronary fibromuscular dysplasia, tortuosity, aneurysm, or additional dissections are found.
vascular smooth muscle cell link fibroblast link
extracellular matrix organization link ⚠ ABNORMAL blood vessel remodeling link ⚠ ABNORMAL
coronary artery link
Downstream
Coronary arterial wall hematoma and false lumen formation Vessel wall vulnerability lowers the threshold for spontaneous coronary wall separation.
Show evidence (2 references)
PMID:38089767 SUPPORT Human Clinical
"Spontaneous coronary artery dissection (SCAD), although in the majority of cases presents as an acute coronary syndrome (ACS), has different pathophysiology from atherosclerosis that influences specific angiography findings and enables most patients to be solved by optimal medical therapy rather..."
Review supports SCAD as a nonatherosclerotic pathophysiologic entity with different angiographic and management implications from plaque-mediated ACS.
PMID:37091648 SUPPORT Human Clinical
"The pathological substrates (fibromuscular dysplasia) and triggers (especially emotional stress) are commonly present in affected women."
Review supports the clinical framework of extracoronary arteriopathy and triggers predisposing to spontaneous dissection.
⬡

Pathograph

Use the checkboxes to hide or show graph categories. Hover nodes for evidence and cross-linked metadata.
Referential integrity issues (2):
  • Target 'Coronary flow limitation' (from 'Coronary arterial wall hematoma and false lumen formation') not found in named elements
  • Target 'Myocardial ischemia and infarction' (from 'Coronary arterial wall hematoma and false lumen formation') not found in named elements
Pathograph: causal mechanism network for Idiopathic Spontaneous Coronary Artery Dissection Interactive directed graph showing how pathophysiology mechanisms, phenotypes, genetic factors and variants, experimental models, environmental triggers, and treatments relate through causal and linked edges.
●

Phenotypes

6
Cardiovascular 3
Angina pectoris Angina pectoris (HP:0001681)
Show evidence (1 reference)
PMID:38298759 SUPPORT Human Clinical
"Approximately 48.5% of the patients were diagnosed with non-ST elevated myocardial infarction (NSTEMI), 36.8% with ST elevated myocardial infarction (STEMI), 3.41% with unstable angina, 0.56% with stable angina, and 0.11% were diagnosed with various types of arrhythmias."
Systematic review documents unstable and stable angina presentations among SCAD patients.
Myocardial infarction Myocardial infarction (HP:0001658)
Show evidence (1 reference)
PMID:37091648 SUPPORT Human Clinical
"Spontaneous coronary artery dissection (SCAD) is an under-recognized cause of acute coronary syndrome that predominantly affects women in adulthood and is the leading cause of acute myocardial infarction in pregnancy."
Review supports myocardial infarction as a major SCAD manifestation, especially in pregnancy-associated presentations.
Ventricular arrhythmia Ventricular arrhythmia (HP:0004308)
Show evidence (1 reference)
PMID:37091648 SUPPORT Human Clinical
"The most common clinical presentation is ST-segment elevation myocardial infarction (STEMI) or non-STEMI, followed by cardiogenic shock (∼2%), sudden cardiac death (0.8% in autopsy series), cardiac arrest, ventricular arrhythmias (∼5%), and Takotsubo syndrome."
Review explicitly lists ventricular arrhythmias among SCAD complications.
Constitutional 1
Chest pain Chest pain (HP:0100749)
Show evidence (1 reference)
PMID:39742239 SUPPORT Human Clinical
"Revascularization is recommended in the presence of high-risk features (such as left main or multivessel involvement, hemodynamic instability, recurrent chest pain, or ST elevation)."
Review recognizes recurrent chest pain as a clinically important SCAD feature used in acute management decisions.
Other 2
Coronary artery dissection Coronary artery dissection (HP:0006702)
Show evidence (1 reference)
PMID:38390446 SUPPORT Human Clinical
"Spontaneous coronary artery dissection (SCAD) is a non-traumatic and non-iatrogenic separation of the coronary arterial wall."
The disease definition directly supports coronary artery dissection as the diagnostic phenotype.
Cardiogenic shock Cardiogenic shock (HP:0030149)
Show evidence (1 reference)
PMID:37091648 SUPPORT Human Clinical
"The most common clinical presentation is ST-segment elevation myocardial infarction (STEMI) or non-STEMI, followed by cardiogenic shock (∼2%), sudden cardiac death (0.8% in autopsy series), cardiac arrest, ventricular arrhythmias (∼5%), and Takotsubo syndrome."
Review explicitly identifies cardiogenic shock among less common but severe SCAD presentations.
🧬

Genetic Associations

1
Rare connective-tissue genetic predisposition
Show evidence (1 reference)
PMID:37091648 SUPPORT Human Clinical
"The few cases with a precise genetic aetiology occur in the context of syndromic and non-syndromic connective tissue diseases."
Review supports rare genetic etiologies in connective-tissue disease contexts rather than a common Mendelian cause.
💊

Treatments

4
Conservative medical management
Action: supportive care MAXO:0000950
Hemodynamically stable patients are often managed conservatively because spontaneous healing of dissected segments is common, while invasive revascularization can be technically difficult.
Show evidence (2 references)
PMID:38298759 SUPPORT Human Clinical
"CONCLUSION: Our results highlight that conservative treatment should be the preferred method of treatment in patients with SCAD."
Systematic review supports conservative treatment as the preferred strategy for many SCAD patients.
PMID:39742239 SUPPORT Human Clinical
"Concerning its management, the preferred approach is conservative due to the high rates of spontaneous healing in the first months and the low rate of revascularization success (high complexity percutaneous coronary intervention (PCI) with dissection/hematoma extension risk)."
Review explains the rationale for conservative management: spontaneous healing and difficult revascularization.
Beta-blocker therapy for selected SCAD patients
Action: beta-blocker therapy Ontology label: beta-adrenergic antagonist therapy MAXO:0000187
Agent: Beta-Adrenergic Antagonist ↗
Beta-blockers are used as part of post-SCAD medical therapy, especially when ventricular dysfunction is present, while the optimal antiplatelet strategy remains uncertain in conservatively managed disease.
Show evidence (1 reference)
PMID:39742239 SUPPORT Human Clinical
"Medical therapy includes beta blockers in cases of ventricular dysfunction; however, no clear data are available about antiplatelet treatment because of the supposed risk of intramural hematoma enlargement."
Review supports beta-blocker use in selected SCAD medical therapy while noting uncertainty around antiplatelet treatment.
Percutaneous coronary intervention for high-risk SCAD
Action: coronary stent insertion MAXO:0009038
Percutaneous coronary intervention may be required when conservative management is unsafe because of ongoing ischemia, left main or multivessel involvement, hemodynamic instability, recurrent chest pain, or persistent ST elevation.
Show evidence (2 references)
PMID:39742239 SUPPORT Human Clinical
"Revascularization is recommended in the presence of high-risk features (such as left main or multivessel involvement, hemodynamic instability, recurrent chest pain, or ST elevation)."
Review supports revascularization for high-risk SCAD presentations.
PMID:39742239 SUPPORT Human Clinical
"The first choice is PCI; coronary artery bypass graft (CABG) is considered only if PCI is not feasible or too hazardous according to the operators' and centers' experience."
Review supports PCI as first-choice revascularization when an invasive strategy is needed.
Coronary artery bypass grafting for selected high-risk SCAD
Coronary artery bypass grafting is reserved for selected high-risk cases when PCI is not feasible or carries excessive procedural risk.
Show evidence (1 reference)
PMID:39742239 SUPPORT Human Clinical
"The first choice is PCI; coronary artery bypass graft (CABG) is considered only if PCI is not feasible or too hazardous according to the operators' and centers' experience."
Review supports CABG as a fallback revascularization strategy in selected high-risk SCAD cases.
🌍

Environmental Factors

2
Pregnancy, postpartum state, and hormonal exposures
Show evidence (2 references)
PMID:41776338 SUPPORT Human Clinical
"It is also associated with pregnancy, delivery, and the post-partum period."
Narrative review supports pregnancy, delivery, and postpartum association.
PMID:37091648 SUPPORT Human Clinical
"Oral contraceptives, post-menopausal therapy, and infertility treatments are recognized associated factors."
Review supports hormonal exposures as associated factors in SCAD.
Emotional stress
Show evidence (1 reference)
PMID:37091648 SUPPORT Human Clinical
"The pathological substrates (fibromuscular dysplasia) and triggers (especially emotional stress) are commonly present in affected women."
Review supports emotional stress as a recognized trigger in affected women.
🔬

Clinical Trials

1
NCT04850417 PHASE_IV NOT_RECRUITING
BA-SCAD is a randomized Phase 4 factorial trial evaluating beta-blocker use and antiplatelet duration in patients with spontaneous coronary artery dissection.
Target Phenotypes: Coronary artery dissection ↗ Myocardial infarction ↗
Show evidence (2 references)
clinicaltrials:NCT04850417 SUPPORT Human Clinical
"Using a factorial 2x2 design, patients will be randomized (1:1/1:1) to: 1) BB (yes/no) and 2) short AP regimen (1 month) vs prolonged dual AP therapy (DAPT) (12 months)."
ClinicalTrials.gov summary supports the randomized beta-blocker and antiplatelet treatment comparisons.
clinicaltrials:NCT04850417 SUPPORT Human Clinical
"A total of 600 SCAD patients will be randomized within 2 years (300 per arm in a factorial 2x2 design)."
ClinicalTrials.gov summary supports the planned randomized enrollment scale for BA-SCAD.
{ }

Source YAML

click to show 
name: Idiopathic Spontaneous Coronary Artery Dissection
creation_date: "2026-05-06T03:09:04Z"
updated_date: "2026-05-06T03:56:38Z"
description: >-
  Idiopathic spontaneous coronary artery dissection is a non-traumatic,
  non-iatrogenic coronary arteriopathy in which separation of the coronary
  arterial wall, often with intramural hematoma, narrows the true lumen and
  causes acute myocardial ischemia. It is distinct from atherosclerotic coronary
  artery disease and is classically recognized in younger or middle-aged women,
  including peripartum and postpartum presentations.
category: Complex
disease_term:
  preferred_term: idiopathic spontaneous coronary artery dissection
  term:
    id: MONDO:0007385
    label: idiopathic spontaneous coronary artery dissection
parents:
- Vascular disorder
- Idiopathic disease
synonyms:
- Spontaneous coronary artery dissection
- SCAD
progression:
- phase: Acute coronary syndrome presentation
  notes: >-
    SCAD most often enters clinical care as an acute coronary syndrome, with
    presentations that include ST-elevation myocardial infarction, non-ST
    elevation myocardial infarction, unstable angina, and less common acute
    complications.
  evidence:
  - reference: PMID:41776338
    reference_title: "Spontaneous coronary artery dissection: a clinically oriented narrative review."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Spontaneous coronary artery dissection (SCAD) is an important cause of acute coronary syndromes (ACS), with a higher incidence in younger female patients."
    explanation: Narrative clinical review supports the acute-coronary-syndrome presentation pattern and demographic enrichment.
  - reference: PMID:38298759
    reference_title: "Management and outcomes of spontaneous coronary artery dissection: a systematic review of the literature."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Approximately 48.5% of the patients were diagnosed with non-ST elevated myocardial infarction (NSTEMI), 36.8% with ST elevated myocardial infarction (STEMI), 3.41% with unstable angina, 0.56% with stable angina, and 0.11% were diagnosed with various types of arrhythmias."
    explanation: Systematic review quantifies the distribution of acute cardiac presentations in SCAD cohorts.
- phase: Follow-up and recurrence risk
  notes: >-
    Many acute dissections heal, but recurrence and major adverse cardiovascular
    events remain clinically important during follow-up.
  evidence:
  - reference: PMID:38298759
    reference_title: "Management and outcomes of spontaneous coronary artery dissection: a systematic review of the literature."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "The authors reported variable prevalence of MACE, recurrent SCAD up to 31%, ACS up to 27.4%, TVR up to 30%, repeat revascularization up to 14.7%, UA up to 13.3%, HF up to 17.4%, and stroke up to 3%."
    explanation: Systematic review supports clinically meaningful recurrence and adverse-event risk after the index event.
  - reference: PMID:39742239
    reference_title: "Spontaneous Coronary Dissection Review: A Complex Picture."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Eventually, SCAD follow-up is important, considering the risk of SCAD recurrence."
    explanation: Review explicitly supports ongoing follow-up because recurrent SCAD is a recognized concern.
pathophysiology:
- name: Coronary arterial wall hematoma and false lumen formation
  description: >-
    A tear or hemorrhage within the coronary arterial wall creates a false
    lumen or intramural hematoma. Expansion of this wall hematoma compresses the
    true lumen, limits coronary blood flow, and can trigger myocardial ischemia
    or infarction.
  cell_types:
  - preferred_term: vascular smooth muscle cell
    term:
      id: CL:0000359
      label: vascular associated smooth muscle cell
  - preferred_term: endothelial cell
    term:
      id: CL:0000115
      label: endothelial cell
  locations:
  - preferred_term: coronary artery
    term:
      id: UBERON:0001621
      label: coronary artery
  - preferred_term: tunica media
    term:
      id: UBERON:0002522
      label: tunica media
  biological_processes:
  - preferred_term: blood vessel remodeling
    modifier: ABNORMAL
    term:
      id: GO:0001974
      label: blood vessel remodeling
  - preferred_term: blood coagulation
    modifier: ABNORMAL
    term:
      id: GO:0007596
      label: blood coagulation
  evidence:
  - reference: PMID:38390446
    reference_title: "Spontaneous coronary artery dissection in women in the generative period: clinical characteristics, treatment, and outcome-a systematic review and meta-analysis."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Spontaneous coronary artery dissection (SCAD) is a non-traumatic and non-iatrogenic separation of the coronary arterial wall."
    explanation: Systematic review supports the core lesion as spontaneous coronary arterial-wall separation.
  - reference: PMID:39742239
    reference_title: "Spontaneous Coronary Dissection Review: A Complex Picture."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "no clear data are available about antiplatelet treatment because of the supposed risk of intramural hematoma enlargement."
    explanation: Review identifies intramural hematoma enlargement as a clinically relevant SCAD mechanism considered during management.
  downstream:
  - target: Coronary flow limitation
    description: Intramural hematoma or false-lumen expansion compresses the true lumen and reduces coronary perfusion.
    causal_link_type: DIRECT
  - target: Myocardial ischemia and infarction
    description: Reduced coronary perfusion can produce angina, myocardial infarction, arrhythmia, or cardiogenic shock.
    causal_link_type: DIRECT
- name: Nonatherosclerotic arteriopathy and vessel wall vulnerability
  description: >-
    Many idiopathic SCAD presentations occur without plaque rupture,
    atherosclerotic stenosis, trauma, or instrumentation. A systemic
    nonatherosclerotic arteriopathy or extracellular matrix vulnerability is a
    common clinical framework, especially when extracoronary fibromuscular
    dysplasia, tortuosity, aneurysm, or additional dissections are found.
  cell_types:
  - preferred_term: vascular smooth muscle cell
    term:
      id: CL:0000359
      label: vascular associated smooth muscle cell
  - preferred_term: fibroblast
    term:
      id: CL:0000057
      label: fibroblast
  locations:
  - preferred_term: coronary artery
    term:
      id: UBERON:0001621
      label: coronary artery
  biological_processes:
  - preferred_term: extracellular matrix organization
    modifier: ABNORMAL
    term:
      id: GO:0030198
      label: extracellular matrix organization
  - preferred_term: blood vessel remodeling
    modifier: ABNORMAL
    term:
      id: GO:0001974
      label: blood vessel remodeling
  evidence:
  - reference: PMID:38089767
    reference_title: "Contemporary review on spontaneous coronary artery dissection: insights into the angiographic finding and differential diagnosis."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Spontaneous coronary artery dissection (SCAD), although in the majority of cases presents as an acute coronary syndrome (ACS), has different pathophysiology from atherosclerosis that influences specific angiography findings and enables most patients to be solved by optimal medical therapy rather than percutaneous coronary intervention (PCI)."
    explanation: Review supports SCAD as a nonatherosclerotic pathophysiologic entity with different angiographic and management implications from plaque-mediated ACS.
  - reference: PMID:37091648
    reference_title: "Spontaneous coronary artery dissection: an unpredictable event."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "The pathological substrates (fibromuscular dysplasia) and triggers (especially emotional stress) are commonly present in affected women."
    explanation: Review supports the clinical framework of extracoronary arteriopathy and triggers predisposing to spontaneous dissection.
  downstream:
  - target: Coronary arterial wall hematoma and false lumen formation
    description: Vessel wall vulnerability lowers the threshold for spontaneous coronary wall separation.
    causal_link_type: INDIRECT_UNKNOWN_INTERMEDIATES
phenotypes:
- category: Cardiovascular
  name: Coronary artery dissection
  diagnostic: true
  description: Spontaneous dissection of an epicardial coronary artery is the defining lesion.
  phenotype_term:
    preferred_term: Coronary artery dissection
    term:
      id: HP:0006702
      label: Coronary artery dissection
  evidence:
  - reference: PMID:38390446
    reference_title: "Spontaneous coronary artery dissection in women in the generative period: clinical characteristics, treatment, and outcome-a systematic review and meta-analysis."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Spontaneous coronary artery dissection (SCAD) is a non-traumatic and non-iatrogenic separation of the coronary arterial wall."
    explanation: The disease definition directly supports coronary artery dissection as the diagnostic phenotype.
- category: Cardiovascular
  name: Chest pain
  description: Acute chest pain is the common presenting symptom because SCAD usually presents as acute coronary syndrome.
  phenotype_term:
    preferred_term: Chest pain
    term:
      id: HP:0100749
      label: Chest pain
  evidence:
  - reference: PMID:39742239
    reference_title: "Spontaneous Coronary Dissection Review: A Complex Picture."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Revascularization is recommended in the presence of high-risk features (such as left main or multivessel involvement, hemodynamic instability, recurrent chest pain, or ST elevation)."
    explanation: Review recognizes recurrent chest pain as a clinically important SCAD feature used in acute management decisions.
- category: Cardiovascular
  name: Angina pectoris
  description: Coronary lumen compression can produce ischemic angina.
  phenotype_term:
    preferred_term: Angina pectoris
    term:
      id: HP:0001681
      label: Angina pectoris
  evidence:
  - reference: PMID:38298759
    reference_title: "Management and outcomes of spontaneous coronary artery dissection: a systematic review of the literature."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Approximately 48.5% of the patients were diagnosed with non-ST elevated myocardial infarction (NSTEMI), 36.8% with ST elevated myocardial infarction (STEMI), 3.41% with unstable angina, 0.56% with stable angina, and 0.11% were diagnosed with various types of arrhythmias."
    explanation: Systematic review documents unstable and stable angina presentations among SCAD patients.
- category: Cardiovascular
  name: Myocardial infarction
  description: Flow-limiting SCAD can cause myocardial infarction when coronary perfusion is sufficiently compromised.
  phenotype_term:
    preferred_term: Myocardial infarction
    term:
      id: HP:0001658
      label: Myocardial infarction
  evidence:
  - reference: PMID:37091648
    reference_title: "Spontaneous coronary artery dissection: an unpredictable event."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Spontaneous coronary artery dissection (SCAD) is an under-recognized cause of acute coronary syndrome that predominantly affects women in adulthood and is the leading cause of acute myocardial infarction in pregnancy."
    explanation: Review supports myocardial infarction as a major SCAD manifestation, especially in pregnancy-associated presentations.
- category: Cardiovascular
  name: Ventricular arrhythmia
  description: Myocardial ischemia from SCAD can be complicated by ventricular arrhythmia.
  phenotype_term:
    preferred_term: Ventricular arrhythmia
    term:
      id: HP:0004308
      label: Ventricular arrhythmia
  evidence:
  - reference: PMID:37091648
    reference_title: "Spontaneous coronary artery dissection: an unpredictable event."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "The most common clinical presentation is ST-segment elevation myocardial infarction (STEMI) or non-STEMI, followed by cardiogenic shock (∼2%), sudden cardiac death (0.8% in autopsy series), cardiac arrest, ventricular arrhythmias (∼5%), and Takotsubo syndrome."
    explanation: Review explicitly lists ventricular arrhythmias among SCAD complications.
- category: Cardiovascular
  name: Cardiogenic shock
  description: Extensive ischemia or left main/proximal-vessel dissection can present with cardiogenic shock.
  phenotype_term:
    preferred_term: Cardiogenic shock
    term:
      id: HP:0030149
      label: Cardiogenic shock
  evidence:
  - reference: PMID:37091648
    reference_title: "Spontaneous coronary artery dissection: an unpredictable event."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "The most common clinical presentation is ST-segment elevation myocardial infarction (STEMI) or non-STEMI, followed by cardiogenic shock (∼2%), sudden cardiac death (0.8% in autopsy series), cardiac arrest, ventricular arrhythmias (∼5%), and Takotsubo syndrome."
    explanation: Review explicitly identifies cardiogenic shock among less common but severe SCAD presentations.
genetic:
- name: Rare connective-tissue genetic predisposition
  features: >-
    Most idiopathic SCAD is not explained by a single high-penetrance genetic
    diagnosis, but a small subset occurs in syndromic or non-syndromic
    connective-tissue disease contexts that imply inherited vessel-wall
    fragility.
  evidence:
  - reference: PMID:37091648
    reference_title: "Spontaneous coronary artery dissection: an unpredictable event."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "The few cases with a precise genetic aetiology occur in the context of syndromic and non-syndromic connective tissue diseases."
    explanation: Review supports rare genetic etiologies in connective-tissue disease contexts rather than a common Mendelian cause.
environmental:
- name: Pregnancy, postpartum state, and hormonal exposures
  effect: Predisposes to SCAD in susceptible patients
  evidence:
  - reference: PMID:41776338
    reference_title: "Spontaneous coronary artery dissection: a clinically oriented narrative review."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "It is also associated with pregnancy, delivery, and the post-partum period."
    explanation: Narrative review supports pregnancy, delivery, and postpartum association.
  - reference: PMID:37091648
    reference_title: "Spontaneous coronary artery dissection: an unpredictable event."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Oral contraceptives, post-menopausal therapy, and infertility treatments are recognized associated factors."
    explanation: Review supports hormonal exposures as associated factors in SCAD.
- name: Emotional stress
  effect: Triggers SCAD in susceptible patients
  evidence:
  - reference: PMID:37091648
    reference_title: "Spontaneous coronary artery dissection: an unpredictable event."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "The pathological substrates (fibromuscular dysplasia) and triggers (especially emotional stress) are commonly present in affected women."
    explanation: Review supports emotional stress as a recognized trigger in affected women.
epidemiology:
- name: Fibromuscular dysplasia association
  description: >-
    Fibromuscular dysplasia is a frequent associated extracoronary arteriopathy
    in SCAD cohorts and is clinically relevant because its presence supports a
    systemic vessel-wall vulnerability context rather than isolated
    plaque-mediated coronary disease.
  evidence:
  - reference: PMID:37091648
    reference_title: "Spontaneous coronary artery dissection: an unpredictable event."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "The pathological substrates (fibromuscular dysplasia) and triggers (especially emotional stress) are commonly present in affected women."
    explanation: Review supports fibromuscular dysplasia as a commonly present associated arteriopathy in affected SCAD patients.
diagnosis:
- name: Coronary angiography with selective intracoronary imaging
  description: >-
    Diagnosis is usually made by coronary angiography; optical coherence
    tomography or intravascular ultrasound may clarify intramural hematoma or an
    intimal flap when angiographic findings are uncertain and the procedure is
    clinically safe.
  results: Findings may include long smooth stenosis, contrast staining, multiple radiolucent lumens, or intramural hematoma.
  evidence:
  - reference: PMID:38089767
    reference_title: "Contemporary review on spontaneous coronary artery dissection: insights into the angiographic finding and differential diagnosis."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "So far, invasive coronary angiography remains the most important diagnostic tool in suspected SCAD."
    explanation: Review supports invasive coronary angiography as the central diagnostic test for suspected SCAD.
  - reference: PMID:39742239
    reference_title: "Spontaneous Coronary Dissection Review: A Complex Picture."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "the gold standard diagnostic exam for SCAD is an invasive coronary angiography (ICA) due to its increased sensitivity and disease characterization."
    explanation: Review supports ICA as the gold-standard diagnostic exam.
- name: Screening for extracardiac arteriopathy and connective-tissue disease
  description: >-
    After SCAD diagnosis, clinicians commonly evaluate for extracardiac
    arteriopathies and connective-tissue disease features, especially when FMD
    or other systemic vessel-wall abnormalities are suspected.
  evidence:
  - reference: PMID:39742239
    reference_title: "Spontaneous Coronary Dissection Review: A Complex Picture."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Furthermore, screening for extracardiac arthropathies or connective tissue diseases is recommended due to the hypothesized association with SCAD."
    explanation: Review supports screening for extracardiac arteriopathy/connective-tissue disease context after SCAD.
treatments:
- name: Conservative medical management
  description: >-
    Hemodynamically stable patients are often managed conservatively because
    spontaneous healing of dissected segments is common, while invasive
    revascularization can be technically difficult.
  treatment_term:
    preferred_term: supportive care
    term:
      id: MAXO:0000950
      label: supportive care
  evidence:
  - reference: PMID:38298759
    reference_title: "Management and outcomes of spontaneous coronary artery dissection: a systematic review of the literature."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "CONCLUSION: Our results highlight that conservative treatment should be the preferred method of treatment in patients with SCAD."
    explanation: Systematic review supports conservative treatment as the preferred strategy for many SCAD patients.
  - reference: PMID:39742239
    reference_title: "Spontaneous Coronary Dissection Review: A Complex Picture."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Concerning its management, the preferred approach is conservative due to the high rates of spontaneous healing in the first months and the low rate of revascularization success (high complexity percutaneous coronary intervention (PCI) with dissection/hematoma extension risk)."
    explanation: "Review explains the rationale for conservative management: spontaneous healing and difficult revascularization."
- name: Beta-blocker therapy for selected SCAD patients
  description: >-
    Beta-blockers are used as part of post-SCAD medical therapy, especially
    when ventricular dysfunction is present, while the optimal antiplatelet
    strategy remains uncertain in conservatively managed disease.
  treatment_term:
    preferred_term: beta-blocker therapy
    term:
      id: MAXO:0000187
      label: beta-adrenergic antagonist therapy
    therapeutic_agent:
    - preferred_term: Beta-Adrenergic Antagonist
      term:
        id: NCIT:C29576
        label: Beta-Adrenergic Antagonist
  evidence:
  - reference: PMID:39742239
    reference_title: "Spontaneous Coronary Dissection Review: A Complex Picture."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Medical therapy includes beta blockers in cases of ventricular dysfunction; however, no clear data are available about antiplatelet treatment because of the supposed risk of intramural hematoma enlargement."
    explanation: Review supports beta-blocker use in selected SCAD medical therapy while noting uncertainty around antiplatelet treatment.
- name: Percutaneous coronary intervention for high-risk SCAD
  description: >-
    Percutaneous coronary intervention may be required when conservative
    management is unsafe because of ongoing ischemia, left main or multivessel
    involvement, hemodynamic instability, recurrent chest pain, or persistent ST
    elevation.
  treatment_term:
    preferred_term: coronary stent insertion
    term:
      id: MAXO:0009038
      label: coronary stent insertion
  evidence:
  - reference: PMID:39742239
    reference_title: "Spontaneous Coronary Dissection Review: A Complex Picture."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Revascularization is recommended in the presence of high-risk features (such as left main or multivessel involvement, hemodynamic instability, recurrent chest pain, or ST elevation)."
    explanation: Review supports revascularization for high-risk SCAD presentations.
  - reference: PMID:39742239
    reference_title: "Spontaneous Coronary Dissection Review: A Complex Picture."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "The first choice is PCI; coronary artery bypass graft (CABG) is considered only if PCI is not feasible or too hazardous according to the operators' and centers' experience."
    explanation: Review supports PCI as first-choice revascularization when an invasive strategy is needed.
- name: Coronary artery bypass grafting for selected high-risk SCAD
  description: >-
    Coronary artery bypass grafting is reserved for selected high-risk cases
    when PCI is not feasible or carries excessive procedural risk.
  evidence:
  - reference: PMID:39742239
    reference_title: "Spontaneous Coronary Dissection Review: A Complex Picture."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "The first choice is PCI; coronary artery bypass graft (CABG) is considered only if PCI is not feasible or too hazardous according to the operators' and centers' experience."
    explanation: Review supports CABG as a fallback revascularization strategy in selected high-risk SCAD cases.
clinical_trials:
- name: NCT04850417
  phase: PHASE_IV
  status: NOT_RECRUITING
  description: >-
    BA-SCAD is a randomized Phase 4 factorial trial evaluating beta-blocker use
    and antiplatelet duration in patients with spontaneous coronary artery
    dissection.
  target_phenotypes:
  - preferred_term: Coronary artery dissection
    term:
      id: HP:0006702
      label: Coronary artery dissection
  - preferred_term: Myocardial infarction
    term:
      id: HP:0001658
      label: Myocardial infarction
  evidence:
  - reference: clinicaltrials:NCT04850417
    reference_title: Randomized Clinical Trial Assessing the Value of Beta-Blockers and Antiplatelet Agents in Patients With Spontaneous Coronary Artery Dissection. (The BA-SCAD Randomized Clinical Trial)
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Using a factorial 2x2 design, patients will be randomized (1:1/1:1) to: 1) BB (yes/no) and 2) short AP regimen (1 month) vs prolonged dual AP therapy (DAPT) (12 months)."
    explanation: ClinicalTrials.gov summary supports the randomized beta-blocker and antiplatelet treatment comparisons.
  - reference: clinicaltrials:NCT04850417
    reference_title: Randomized Clinical Trial Assessing the Value of Beta-Blockers and Antiplatelet Agents in Patients With Spontaneous Coronary Artery Dissection. (The BA-SCAD Randomized Clinical Trial)
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "A total of 600 SCAD patients will be randomized within 2 years (300 per arm in a factorial 2x2 design)."
    explanation: ClinicalTrials.gov summary supports the planned randomized enrollment scale for BA-SCAD.
notes: >-
  Fibromuscular dysplasia is represented in epidemiology and pathophysiology
  because the current Disease schema does not provide a dedicated disease-level
  comorbidities slot.
📚

References & Deep Research

Deep Research

1
Falcon ▸
Idiopathic Spontaneous Coronary Artery Dissection (SCAD): Disease Characteristics Research Report
Edison Scientific Literature 32 citations 2026-05-05T23:25:05.911807

Idiopathic Spontaneous Coronary Artery Dissection (SCAD): Disease Characteristics Research Report

Executive summary

Idiopathic spontaneous coronary artery dissection (SCAD) is a non-atherosclerotic cause of acute coronary syndrome (ACS) caused by separation within the coronary arterial wall that produces an intramural hematoma and/or intimal tear, compressing the true lumen and causing myocardial ischemia/infarction. Contemporary evidence supports conservative-first management for clinically stable patients due to high spontaneous healing rates, with selective revascularization for high-risk anatomy or ongoing ischemia; systematic screening for extracoronary arteriopathies—especially fibromuscular dysplasia (FMD)—is commonly recommended. Despite rapidly growing registry-based knowledge, randomized controlled trial (RCT) evidence remains limited, and many recommendations are based on expert consensus and observational studies. (morena2024advancesinthe pages 1-2, dang2024spontaneouscoronaryartery pages 6-6, smirnova2023spontaneouscoronaryartery pages 1-2, petrovic2024managementandoutcomes pages 1-2)

1. Disease information

1.1 Definition and overview

SCAD is described as a non-atherosclerotic, non-traumatic, non-iatrogenic separation of the coronary arterial wall resulting in a false lumen and/or intramural hematoma that compresses the true lumen and presents as ACS. (morena2024advancesinthe pages 1-2, smirnova2023spontaneouscoronaryartery pages 1-2, pender2025spontaneouscoronaryartery pages 1-2)

Abstract-supported quotes - “SCAD is caused by separation occurring within or between any of the three tunics of the coronary artery wall. This leads to intramural hematoma and/or formation of false lumen in the artery, which leads to ischemic changes or infarction of the myocardium.” (Frontiers review abstract) (dang2024spontaneouscoronaryartery pages 6-7) - “Spontaneous coronary artery dissection (SCAD) is a non-traumatic and non-iatrogenic separation of the coronary arterial wall.” (systematic review/meta-analysis abstract) (apostolovic2024spontaneouscoronaryartery pages 1-2)

1.2 Key identifiers (from retrieved evidence)

  • ICD-10-CM: I25.42 used to identify SCAD in U.S. administrative datasets. (mughal2022contemporarytrendsin pages 1-3, krittanawong2020recurrentspontaneouscoronary pages 1-2)
  • ICD-9-CM: 414.12 used to identify SCAD before the ICD-10 transition. (mughal2022contemporarytrendsin pages 1-3, krittanawong2020recurrentspontaneouscoronary pages 1-2)

Not confirmed in retrieved full texts: MeSH term(s), MONDO ID, Orphanet ID, OMIM entry specifically for “idiopathic SCAD” (these identifiers exist in external ontologies but are not present in the retrieved documents and are not inferred here). (mughal2022contemporarytrendsin pages 1-3, krittanawong2020recurrentspontaneouscoronary pages 1-2)

1.3 Synonyms / alternative names

  • Spontaneous coronary artery dissection (SCAD) (morena2024advancesinthe pages 1-2)
  • Non-atherosclerotic coronary artery dissection / non-iatrogenic coronary artery dissection (descriptive usage across reviews) (smirnova2023spontaneouscoronaryartery pages 1-2, pender2025spontaneouscoronaryartery pages 1-2)

1.4 Evidence provenance

The current understanding summarized here is derived largely from aggregated disease-level evidence: systematic reviews/meta-analyses, narrative reviews, and registry/administrative cohort analyses, with some mechanistic inference from intracoronary imaging and pathology discussions. (dang2024spontaneouscoronaryartery pages 6-6, petrovic2024managementandoutcomes pages 1-2)

2. Etiology

2.1 Disease causal factors (current mechanistic framing)

SCAD is thought to arise via two nonexclusive mechanisms: 1) “Inside–out”: an intimal tear allows blood to enter the arterial wall, forming a false lumen. 2) “Outside–in”: spontaneous hemorrhage (e.g., from vasa vasorum) causes intramural hematoma within the media, compressing the lumen. (rusali2025spontaneouscoronaryartery pages 3-5, pender2025spontaneouscoronaryartery pages 1-2)

2.2 Risk factors

Predisposing conditions (non-exhaustive)

  • Fibromuscular dysplasia (FMD): the most consistently associated extracoronary arteriopathy; prevalence among SCAD patients varies widely (in part due to screening modality/intensity). (dang2024spontaneouscoronaryartery pages 6-6, gori2023contemporaryreviewon pages 3-4)
  • Pregnancy/peripartum state (pregnancy-associated SCAD often more severe). (smirnova2023spontaneouscoronaryartery pages 1-2, gori2023contemporaryreviewon pages 3-4, apostolovic2024spontaneouscoronaryartery pages 1-2)
  • Inherited connective tissue disorders (rare; often in syndromic/non-syndromic connective tissue disease contexts). (smirnova2023spontaneouscoronaryartery pages 1-2, gori2023contemporaryreviewon pages 3-4)
  • Hypertension (frequent “traditional” risk factor reported in multiple reviews). (gori2023contemporaryreviewon pages 3-4)
  • Migraine and other neuropsychiatric conditions are reported in SCAD cohorts and may associate with recurrence risk. (dang2024spontaneouscoronaryartery pages 6-6, krittanawong2020recurrentspontaneouscoronary pages 1-2)

Precipitating triggers

Reviews report that emotional stress (more often in women) and physical stress/exertion (more often in men) commonly precede symptoms; stimulant exposure (e.g., cocaine/amphetamines) is also discussed as a precipitating factor in risk-factor reviews. (gori2023contemporaryreviewon pages 3-4)

2.3 Protective factors

No specific protective genetic variants or protective exposures were identified in the retrieved evidence set.

2.4 Gene–environment interactions

The retrieved evidence supports a conceptual model of arterial vulnerability (predisposition) + trigger (stress/hemodynamic/hormonal changes) but does not provide quantified gene–environment interaction estimates. (rusali2025spontaneouscoronaryartery pages 3-5, gori2023contemporaryreviewon pages 3-4)

3. Phenotypes

3.1 Clinical presentation

SCAD most commonly presents as ACS with chest pain and biomarker/ECG changes consistent with MI.

Abstract-supported quote - “Spontaneous coronary artery dissection (SCAD) represents a quite rare event but with potentially serious prognostic implications. Meanwhile, SCAD typically presents as an acute coronary syndrome (ACS).” (review abstract) (morena2024advancesinthe pages 1-2)

Frequencies / ranges reported in recent syntheses

  • Systematic review (n=1,801): NSTEMI ~48.5%, STEMI ~36.8%, unstable angina ~3.4%, stable angina ~0.56%. (petrovic2024managementandoutcomes pages 1-2)
  • In women of reproductive age meta-analysis (n=2,145): STEMI ~47.4% (pooled). (apostolovic2024spontaneouscoronaryartery pages 1-2)

Complications/severe presentations (examples)

A 2023 review reports cardiogenic shock (~2%), sudden cardiac death (~0.8% in autopsy series), and ventricular arrhythmias (~5%) among reported presentations/complications, and notes Takotsubo syndrome overlap. (smirnova2023spontaneouscoronaryartery pages 1-2)

3.2 Quality-of-life impacts

Post-event psychological morbidity is common; a 2024 narrative review highlights depression/anxiety/PTSD and recommends mental-health screening and peer support as part of follow-up care. (dang2024spontaneouscoronaryartery pages 6-6)

3.3 Suggested HPO terms (for knowledge base entry; not exhaustive)

  • Chest pain HP:0100749
  • Acute myocardial infarction HP:0001658
  • ST elevation HP:0031557 / Abnormal ST segment HP:0031425
  • Elevated cardiac troponin HP:0031195
  • Ventricular arrhythmia HP:0001663
  • Cardiogenic shock HP:0030144
  • Dyspnea HP:0002094
  • Nausea HP:0002018

(These HPO codes are provided as ontology suggestions; they were not explicitly enumerated in the retrieved articles.)

4. Genetic / molecular information

4.1 Current understanding

SCAD is generally not strongly inherited; familial cases are uncommon in the reviewed literature, and monogenic causes are considered rare and more often linked to connective tissue disorders. (gori2023contemporaryreviewon pages 3-4, smirnova2023spontaneouscoronaryartery pages 1-2)

A 2024 narrative review discusses reported genetic associations/case reports including PHACTR1/EDN1 locus, SMAD3 mutation cases, and variants in fibrillar collagens; however, the excerpted evidence does not provide a comprehensive gene list or variant-level frequencies. (dang2024spontaneouscoronaryartery pages 6-7)

4.2 When to consider genetic testing (practice-oriented guidance)

Genetic testing is described as low-yield and not routine, but may be considered in SCAD patients with recurrent SCAD, multivessel disease, extracoronary vascular abnormalities, or a family history/features suggestive of a heritable connective tissue disorder, with appropriate counselling and possible aortopathy/connective tissue gene panels. (dang2024spontaneouscoronaryartery pages 6-6)

4.3 Variants/modifier genes/epigenetics/chromosomal abnormalities

The retrieved evidence does not provide curated variant-level data (e.g., ACMG classifications, allele frequencies) or epigenetic/chromosomal abnormality findings specific to idiopathic SCAD.

5. Environmental information

5.1 Environmental/lifestyle factors

The evidence set primarily emphasizes stress-related triggers (emotional/physical) rather than classic lifestyle risk factors; traditional atherosclerotic risk factors are often less prevalent, though hypertension is frequently reported. (gori2023contemporaryreviewon pages 3-4)

5.2 Infectious agents

No specific infectious agent etiology was identified in the retrieved evidence.

6. Mechanism / pathophysiology

6.1 Causal chain (high-level)

Predisposing arteriopathy/hormonal milieu/stress exposure → arterial wall vulnerability → intramural bleeding and/or intimal disruption → intramural hematoma/false lumen → true-lumen compression → myocardial ischemia/infarction → ACS presentation and complications. (rusali2025spontaneouscoronaryartery pages 3-5, pender2025spontaneouscoronaryartery pages 1-2, smirnova2023spontaneouscoronaryartery pages 1-2)

6.2 Cellular processes and tissue injury (conceptual mapping)

  • Vessel wall injury leading to intramural hematoma with downstream ischemia is central to SCAD pathophysiology. (rusali2025spontaneouscoronaryartery pages 3-5, pender2025spontaneouscoronaryartery pages 1-2)

6.3 Suggested GO (biological process) and CL (cell type) terms (ontology suggestions)

  • GO:0001525 angiogenesis (vasa vasorum biology; conceptual)
  • GO:0007596 blood coagulation (intramural hemorrhage context; conceptual)
  • GO:0006954 inflammatory response (discussed as unclear/variable in reviews; conceptual) (rusali2025spontaneouscoronaryartery pages 3-5)
  • CL:0002131 vascular smooth muscle cell
  • CL:0002543 endothelial cell

(These are ontology suggestions; the retrieved evidence does not provide explicit GO/CL annotations.)

7. Anatomical structures affected

7.1 Primary structures

  • Coronary arteries (often LAD as culprit). (petrovic2024managementandoutcomes pages 1-2, apostolovic2024spontaneouscoronaryartery pages 1-2)

7.2 Secondary involvement/complications

  • Myocardium (ischemia/infarction); potential for heart failure, arrhythmias, cardiogenic shock. (smirnova2023spontaneouscoronaryartery pages 1-2, petrovic2024managementandoutcomes pages 1-2)

7.3 Suggested UBERON terms (ontology suggestions)

  • Coronary artery UBERON:0001621
  • Left anterior descending coronary artery (LAD) (UBERON term varies by subset; provide as “LAD coronary artery” mapping in your implementation)
  • Myocardium UBERON:0002349

8. Diagnostics

8.1 Imaging and diagnostic approach

  • Invasive coronary angiography (ICA) is emphasized as the diagnostic gold standard, with adjunctive intracoronary imaging (IVUS/OCT) improving diagnostic performance and clarifying mechanism. (morena2024advancesinthe pages 1-2, bollati2024spontaneouscoronarydissection pages 4-7)
  • Extracoronary vascular imaging: head-to-pelvis CT angiography is described as a method for FMD screening; MRA is an alternative but may have lower spatial resolution. (dang2024spontaneouscoronaryartery pages 6-6)

8.2 Angiographic classification (Saw types)

Recent reviews describe an ICA-based SCAD classification (Types 1–4) used in practice; Table/Figure evidence is available from a 2024 review, including examples of Types 1, 2A, 3, and 4 and management flow-chart context. (bollati2024spontaneouscoronarydissection pages 4-7, bollati2024spontaneouscoronarydissection media ffbb95a5, bollati2024spontaneouscoronarydissection media 8d0f25c2, bollati2024spontaneouscoronarydissection media 8c541a8a, bollati2024spontaneouscoronarydissection media 353fb04a, bollati2024spontaneouscoronarydissection media 94fd0b42, bollati2024spontaneouscoronarydissection media df824cb3)

8.3 Differential diagnosis

SCAD can mimic atherosclerotic ACS and requires careful angiographic interpretation; intracoronary imaging can help in ambiguous cases. (gori2023contemporaryreviewon pages 3-4)

9. Treatment

9.1 Acute management strategy (current practice pattern)

  • Conservative management is preferred in clinically stable SCAD because spontaneous healing is common, while PCI success is limited by technical challenges and risks of propagation/iatrogenic dissection. (bollati2024spontaneouscoronarydissection pages 4-7, petrovic2024managementandoutcomes pages 1-2)
  • Revascularization (PCI; CABG rarely) is reserved for high-risk presentations (e.g., left main/proximal multivessel, TIMI 0/1 flow, hemodynamic/electrical instability, ongoing ischemia). (bollati2024spontaneouscoronarydissection pages 4-7, bollati2024spontaneouscoronarydissection pages 7-8)

9.2 Medical therapy themes

  • Beta-blockers are commonly used; observational evidence associates beta-blockers with lower recurrence. (dang2024spontaneouscoronaryartery pages 6-6)
  • ACE inhibitor/ARB is recommended when LVEF is reduced after SCAD-related ACS. (dang2024spontaneouscoronaryartery pages 6-6)
  • Antiplatelet strategy in conservatively managed SCAD remains debated in reviews due to concern about intramural hematoma expansion; practice varies and RCT data are limited. (morena2024advancesinthe pages 1-2, bollati2024spontaneouscoronarydissection pages 4-7)
  • Statins are generally not routine unless indicated for dyslipidemia/atherosclerotic disease. (smirnova2023spontaneouscoronaryartery pages 1-2)

9.3 Cardiac rehabilitation and psychosocial care

Cardiac rehabilitation is described as safe and recommended for SCAD patients; low/moderate-intensity aerobic and low-resistance programs are favored, along with avoidance of high-intensity abrupt-movement activities, and psychological screening/support is emphasized. (dang2024spontaneouscoronaryartery pages 6-6)

9.4 Current clinical trials (real-world implementations of evidence generation)

  • NCT04850417: “Randomized Study of Beta-Blockers and Antiplatelets in Patients With Spontaneous Coronary Artery Dissection” (Phase 4; planned enrollment 600; Spanish Society of Cardiology). (clinical trial record) (dang2024spontaneouscoronaryartery pages 6-6)
  • NCT06955663: Exercise support/rehabilitation after SCAD (planned enrollment 120). (dang2024spontaneouscoronaryartery pages 6-6)
  • NCT04251039: RESPONSE observational study in SCAD patients undergoing complex PCI. (dang2024spontaneouscoronaryartery pages 6-6)

9.5 Suggested MAXO terms (ontology suggestions)

  • Conservative management / medical management (MAXO “medical therapy” class; implement with local MAXO mapping)
  • Percutaneous coronary intervention PCI
  • Coronary artery bypass grafting CABG
  • Cardiac rehabilitation
  • CT angiography screening for extracoronary arteriopathy

10. Prevention

10.1 Primary prevention

No established primary prevention is supported by RCT evidence in the retrieved set; prevention is largely framed as managing predispositions (e.g., blood pressure), avoiding extreme triggers, and individualized counselling. (dang2024spontaneouscoronaryartery pages 6-6, gori2023contemporaryreviewon pages 3-4)

10.2 Secondary/tertiary prevention

  • Recurrence risk mitigation: beta-blockers are associated with lower recurrence in observational data and are commonly used; follow-up and monitoring are emphasized. (dang2024spontaneouscoronaryartery pages 6-6)
  • Screening for FMD and other extracoronary vascular abnormalities is commonly recommended; FMD is associated with higher recurrence risk in review-level evidence. (dang2024spontaneouscoronaryartery pages 6-6)

11. Outcomes / prognosis

11.1 Mortality and major adverse cardiovascular events

A 2024 systematic review (13 observational studies, n=1,801) reported in-hospital mortality ~1.2% and follow-up mortality ~1.3%, with MACE including recurrent SCAD up to 31% across studies and other events (ACS, target vessel revascularization, HF, stroke) reported variably. (petrovic2024managementandoutcomes pages 1-2)

A 2024 meta-analysis in reproductive-age women reported pooled recurrent SCAD ~15.2% (95% CI 9.1–21.3). (apostolovic2024spontaneouscoronaryartery pages 1-2)

11.2 Recurrence and prognostic factors

  • Reviews report recurrence risk persists for years and may vary widely depending on definitions and cohorts; recurrence has been reported up to ~30% historically, with more recent prospective series reporting lower values in certain follow-up windows. (smirnova2023spontaneouscoronaryartery pages 1-2)
  • FMD is linked to higher recurrence risk (RR 2.02 in review summary). (dang2024spontaneouscoronaryartery pages 6-6)

12. Temporal development

12.1 Onset

SCAD often occurs in younger to middle-aged adults, with strong female predominance and particular relevance in peripartum settings. (smirnova2023spontaneouscoronaryartery pages 1-2, apostolovic2024spontaneouscoronaryartery pages 1-2)

12.2 Course

  • Early in-hospital period: risk of early extension/worsening in the first days is noted in reviews (reported ~5–10% in one review summary). (bollati2024spontaneouscoronarydissection pages 4-7)
  • Longer term: recurrence risk and chronic symptoms (e.g., persistent chest pain syndromes discussed in later reviews) can require ongoing follow-up care. (rusali2025spontaneouscoronaryartery pages 14-16)

13. Inheritance and population

13.1 Epidemiology

  • SCAD is commonly cited as accounting for ~1–4% of ACS. (gori2023contemporaryreviewon pages 3-4)
  • Administrative incidence trend analysis (U.S. National Inpatient Sample) reported rising crude incidence per 1,000,000 discharges from ~4.95 (2010) to ~14.81 (2017), identified using ICD-9/10 SCAD codes. (mughal2022contemporarytrendsin pages 1-3)

13.2 Demographics

  • Female predominance is consistently reported; many reviews cite ~80–90% of cases in women. (gori2023contemporaryreviewon pages 3-4, petrovic2024managementandoutcomes pages 1-2)

13.3 Inheritance pattern

The evidence supports mostly complex/polygenic susceptibility with rare monogenic syndromic cases, rather than a single Mendelian inheritance pattern for idiopathic SCAD. (smirnova2023spontaneouscoronaryartery pages 1-2, gori2023contemporaryreviewon pages 3-4)

14. Other species / natural disease

No evidence for naturally occurring SCAD as a defined veterinary disease entity was identified in the retrieved materials.

15. Model organisms

No SCAD-specific validated animal model descriptions were found in the retrieved evidence excerpts; current literature in this evidence set emphasizes human imaging/registry studies and connective-tissue disease genetics rather than experimental organism models. (dang2024spontaneouscoronaryartery pages 6-6, petrovic2024managementandoutcomes pages 1-2)

Visual evidence (angiographic types and management algorithm)

The following extracted visuals provide practical depictions of SCAD angiographic types and a management flow chart from a 2024 review; they can be used to support knowledge base UI elements and clinician-facing summaries. (bollati2024spontaneouscoronarydissection media ffbb95a5, bollati2024spontaneouscoronarydissection media 8d0f25c2, bollati2024spontaneouscoronarydissection media df824cb3)

Structured evidence table

Domain Compact summary Key figures / structured items
Definition & epidemiology SCAD is a non-atherosclerotic, non-traumatic, non-iatrogenic separation of the coronary arterial wall causing intramural hematoma and/or intimal tear, compression of the true lumen, myocardial ischemia, and ACS. It is increasingly recognized but still underdiagnosed. Women predominate, typically younger to middle-aged and often without classic atherosclerotic risk factors. (morena2024advancesinthe pages 1-2, singulane2025spontaneouscoronaryartery pages 1-2, smirnova2023spontaneouscoronaryartery pages 1-2, pender2025spontaneouscoronaryartery pages 1-2) ACS contribution: ~1–4% overall; ~22–43% of AMI/ACS in younger women depending on cohort/age definition; up to ~35% of MI/ACS in women <50 in some summaries. Sex: ~88–91% female. Typical age: mean ~49–50 years; reviews cite onset often 44–55 years. Culprit vessel: LAD most common (~50–51%). (morena2024advancesinthe pages 1-2, smirnova2023spontaneouscoronaryartery pages 1-2, mughal2022contemporarytrendsin pages 1-3, krittanawong2020recurrentspontaneouscoronary pages 1-2)
Risk factors / predisposition / triggers Strongest associated arteriopathy is fibromuscular dysplasia (FMD); other predispositions include inherited connective-tissue disorders, pregnancy/peripartum state, hormonal exposure, migraine, hypertension, and less commonly systemic inflammatory/autoimmune disease. Triggers differ by sex pattern in reviews: emotional stress commonly reported in women and physical stress/exertion in men; stimulant use (cocaine/amphetamines) also reported. (dang2024spontaneouscoronaryartery pages 6-7, rusali2025spontaneouscoronaryartery pages 3-5, smirnova2023spontaneouscoronaryartery pages 1-2, dang2024spontaneouscoronaryartery pages 6-6) FMD prevalence in SCAD: ~25%–86% across studies/screening strategies. Recurrence predictors: FMD RR 2.02 (95% CI 1.03–3.94) in review; migraine HR 3.4 and FMD HR 5.1 for recurrent SCAD in one cohort. Pregnancy-associated SCAD: <5–17% of SCAD overall in reviews/meta-analyses; postpartum clustering recognized. (dang2024spontaneouscoronaryartery pages 6-6, pender2025spontaneouscoronaryartery pages 1-2, krittanawong2020recurrentspontaneouscoronary pages 1-2)
Diagnostics & angiographic types Invasive coronary angiography (ICA) is the diagnostic gold standard in suspected SCAD; OCT and IVUS help confirm intimal tear, false lumen, and intramural hematoma and guide PCI when needed. Extracoronary vascular imaging (often head-to-pelvis CTA, with MRA alternative) is recommended to detect FMD/other arteriopathies. (morena2024advancesinthe pages 1-2, dang2024spontaneouscoronaryartery pages 6-7, dang2024spontaneouscoronaryartery pages 6-6, bollati2024spontaneouscoronarydissection pages 4-7, bollati2024spontaneouscoronarydissection pages 7-8) Saw/ICA angiographic types: Type 1 = classic radiolucent lumen/contrast staining; Type 2 = long diffuse smooth narrowing (most common; includes 2A/2B); Type 3 = focal stenosis mimicking atherosclerosis; Type 4 = distal vessel occlusion. Review cited Type 1 ~29% and Type 3 ~4%. ICA “golden rules”: gentle catheter handling, minimize injections, use nitro to distinguish spasm, consider OCT/IVUS. (morena2024advancesinthe pages 1-2, bollati2024spontaneouscoronarydissection pages 4-7, bollati2024spontaneouscoronarydissection media ffbb95a5)
Management, recurrence & outcomes Conservative therapy is preferred for clinically stable patients because spontaneous healing is common; PCI is reserved for ongoing ischemia, recurrent chest pain/ST elevation, hemodynamic/electrical instability, left main/proximal multivessel disease, or TIMI 0/1 flow. CABG is considered when PCI is not feasible/failed, especially left main or proximal disease. Beta-blockers are commonly used; ACEi/ARB for reduced LVEF; statins only for standard indications; antiplatelet strategy remains debated in conservatively managed SCAD. Cardiac rehabilitation and psychological support are recommended. (morena2024advancesinthe pages 1-2, smirnova2023spontaneouscoronaryartery pages 1-2, dang2024spontaneouscoronaryartery pages 6-6, bollati2024spontaneouscoronarydissection pages 4-7, bollati2024spontaneouscoronarydissection pages 7-8) Healing: angiographic healing in ~70–97% over months. Early extension/worsening: ~5–10% in first days. PCI technical failure/complexity: Mayo series failure 53%; European series 27% technical failure and 9% emergency CABG. Mortality: in systematic review, in-hospital ~1.2% and follow-up ~1.3%; 3-year mortality 0.8% in one review. Recurrence: often ~10–20% within ~4 years; systematic review reported recurrent SCAD up to 31% across studies; one cohort 10.6% over median 4.7 years. Rehab/screening uptake in meta-analysis: antiplatelet 92.1%, beta-blocker 78.0%, FMD screening 54.4%, rehab referral 70.2%. (smirnova2023spontaneouscoronaryartery pages 1-2, dang2024spontaneouscoronaryartery pages 6-6, bollati2024spontaneouscoronarydissection pages 4-7, bollati2024spontaneouscoronarydissection pages 7-8)
Coding identifiers & active trials Administrative studies identify SCAD using ICD-9-CM 414.12 and ICD-10-CM I25.42; iatrogenic coronary laceration/dissection is commonly excluded with ICD-9-CM 998.2 / ICD-10-CM I97.51 in database analyses. No explicit MONDO/Orphanet identifier was confirmed in the retrieved evidence. (mughal2022contemporarytrendsin pages 1-3, krittanawong2020recurrentspontaneouscoronary pages 1-2) Trial NCTs: NCT04850417 (BA-SCAD; randomized study of beta-blockers and antiplatelets in SCAD, phase 4, planned n=600); NCT06955663 (exercise support/rehabilitation after SCAD, planned n=120); NCT04251039 (RESPONSE observational study in SCAD patients undergoing complex PCI). (dang2024spontaneouscoronaryartery pages 6-6)

Table: This table condenses high-yield evidence on spontaneous coronary artery dissection, including definition, epidemiology, risk factors, diagnostics, management, outcomes, coding, and current trial identifiers. It is useful as a compact reference for building a structured disease knowledge base entry.

Recent developments and expert analysis (2023–2024 emphasis)

  • Registry-driven science has accelerated SCAD understanding, but reviews emphasize that RCT evidence is still scarce, prompting active trials such as BA-SCAD. (dang2024spontaneouscoronaryartery pages 6-6)
  • Reviews highlight system-level care gaps and practice variation, including incomplete FMD screening and variable rehabilitation referral; meta-analytic estimates suggest substantial heterogeneity across regions and eras. (petrovic2024managementandoutcomes pages 1-2)

URLs and publication dates (from retrieved sources)

  • Dang Q. et al. npj Cardiovascular Health (May 2024). https://doi.org/10.1038/s44325-024-00004-y (dang2024spontaneouscoronaryartery pages 6-6)
  • Petrović M. et al. Frontiers in Cardiovascular Medicine (Jan 2024). https://doi.org/10.3389/fcvm.2024.1276521 (petrovic2024managementandoutcomes pages 1-2)
  • Apostolović S. et al. Frontiers in Cardiovascular Medicine (Feb 2024). https://doi.org/10.3389/fcvm.2024.1277604 (apostolovic2024spontaneouscoronaryartery pages 1-2)
  • Smirnova A. et al. European Heart Journal Supplements (Apr 2023). https://doi.org/10.1093/eurheartjsupp/suad059 (smirnova2023spontaneouscoronaryartery pages 1-2)
  • Mughal M.S. et al. Expert Review of Cardiovascular Therapy (May 2022). https://doi.org/10.1080/14779072.2022.2080055 (mughal2022contemporarytrendsin pages 1-3)

Evidence gaps (important for knowledge base provenance)

  • MONDO/MeSH/Orphanet/OMIM identifiers were not extractable from the retrieved full texts; adding them will require direct ontology lookups outside this evidence set.
  • Variant-level genetics (ClinVar-style curation), epigenetic signatures, and experimental model organism evidence are not provided in the retrieved excerpts and remain incomplete here.

References

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  2. (dang2024spontaneouscoronaryartery pages 6-6): Quan Dang, Sonya Burgess, Peter J. Psaltis, Sarah Fairley, Jacqueline Saw, and Sarah Zaman. Spontaneous coronary artery dissection: a clinically oriented narrative review. npj Cardiovascular Health, May 2024. URL: https://doi.org/10.1038/s44325-024-00004-y, doi:10.1038/s44325-024-00004-y. This article has 14 citations.

  3. (smirnova2023spontaneouscoronaryartery pages 1-2): Alexandra Smirnova, Flaminia Aliberti, Claudia Cavaliere, Ilaria Gatti, Viviana Vilardo, Carmelina Giorgianni, Chiara Cassani, Alessandra Repetto, Nupoor Narula, Lorenzo Giuliani, Mario Urtis, Yukio Ozaki, Francesco Prati, Eloisa Arbustini, and Michela Ferrari. Spontaneous coronary artery dissection: an unpredictable event. European Heart Journal Supplements : Journal of the European Society of Cardiology, 25:B7-B11, Apr 2023. URL: https://doi.org/10.1093/eurheartjsupp/suad059, doi:10.1093/eurheartjsupp/suad059. This article has 11 citations.

  4. (petrovic2024managementandoutcomes pages 1-2): Milovan Petrović, Tatjana Miljković, Aleksandra Ilić, Mila Kovačević, Milenko Čanković, Dragana Dabović, Anastazija Stojšić Milosavljević, Snežana Čemerlić Maksimović, Milana Jaraković, Dragica Andrić, Miodrag Golubović, Marija Bjelobrk, Snežana Bjelić, Snežana Tadić, Jelena Slankamenac, Svetlana Apostolović, Vladimir Djurović, and Aleksandra Milovančev. Management and outcomes of spontaneous coronary artery dissection: a systematic review of the literature. Frontiers in Cardiovascular Medicine, Jan 2024. URL: https://doi.org/10.3389/fcvm.2024.1276521, doi:10.3389/fcvm.2024.1276521. This article has 17 citations and is from a peer-reviewed journal.

  5. (pender2025spontaneouscoronaryartery pages 1-2): Patrick Pender, Mithila Zaheen, Quan M. Dang, Viet Dang, James Xu, Matthew Hollings, Sidney Lo, Kazuaki Negishi, and Sarah Zaman. Spontaneous coronary artery dissection: a narrative review of epidemiology and public health implications. Medicina, 61:650, Apr 2025. URL: https://doi.org/10.3390/medicina61040650, doi:10.3390/medicina61040650. This article has 7 citations.

  6. (dang2024spontaneouscoronaryartery pages 6-7): Quan Dang, Sonya Burgess, Peter J. Psaltis, Sarah Fairley, Jacqueline Saw, and Sarah Zaman. Spontaneous coronary artery dissection: a clinically oriented narrative review. npj Cardiovascular Health, May 2024. URL: https://doi.org/10.1038/s44325-024-00004-y, doi:10.1038/s44325-024-00004-y. This article has 14 citations.

  7. (apostolovic2024spontaneouscoronaryartery pages 1-2): Svetlana Apostolović, Aleksandra Ignjatović, Dragana Stanojević, Danijela Djordjević Radojković, Miroslav Nikolić, Jelena Milošević, Tamara Filipović, Katarina Kostić, Ivana Miljković, Aleksandra Djoković, Gordana Krljanac, Zlatko Mehmedbegović, Ivan Ilić, Srdjan Aleksandrić, and Valeria Paradies. Spontaneous coronary artery dissection in women in the generative period: clinical characteristics, treatment, and outcome—a systematic review and meta-analysis. Frontiers in Cardiovascular Medicine, Feb 2024. URL: https://doi.org/10.3389/fcvm.2024.1277604, doi:10.3389/fcvm.2024.1277604. This article has 16 citations and is from a peer-reviewed journal.

  8. (mughal2022contemporarytrendsin pages 1-3): Mohsin S Mughal, Hafsa Akbar, Ikwinder P Kaur, Ali R Ghani, Hasan Mirza, Weiyi Xia, Mohammed Haris Usman, Mahboob Alam, and Tarek Helmy. Contemporary trends in the incidence of spontaneous coronary artery dissection (scad) – ethnic and household income disparities. Expert Review of Cardiovascular Therapy, 20:485-489, May 2022. URL: https://doi.org/10.1080/14779072.2022.2080055, doi:10.1080/14779072.2022.2080055. This article has 9 citations and is from a peer-reviewed journal.

  9. (krittanawong2020recurrentspontaneouscoronary pages 1-2): Chayakrit Krittanawong, Anirudh Kumar, Hafeez Ul Hassan Virk, Zhen Wang, Kipp W. Johnson, Bing Yue, and Deepak L. Bhatt. Recurrent spontaneous coronary artery dissection in the united states. International Journal of Cardiology, 301:34-37, Feb 2020. URL: https://doi.org/10.1016/j.ijcard.2019.10.052, doi:10.1016/j.ijcard.2019.10.052. This article has 28 citations and is from a peer-reviewed journal.

  10. (rusali2025spontaneouscoronaryartery pages 3-5): Andrei Constantin Rusali, Ioana Caterina Lupu, Lavinia Maria Rusali, and Lucia Cojocaru. Spontaneous coronary artery dissection unveiled: pathophysiology, imaging, and evolving management strategies. Jun 2025. URL: https://doi.org/10.20944/preprints202506.0355.v1, doi:10.20944/preprints202506.0355.v1.

  11. (gori2023contemporaryreviewon pages 3-4): Tommaso Gori, Luca Bergamaschi, Jarakovic Milovancev Cankovic Petrovic Bjelobrk Ilic Srdanov Kovacevic, M. Kovačević, M. Jarakovic, A. Milovančev, M. Čanković, M. Petrovic, M. Bjelobrk, A. Ilić, I. Srdanović, S. Tadić, D. Dabović, B. Crnomarković, N. Komazec, N. Dračina, S. Apostolovic, D. Stanojević, and V. Kunadian. Contemporary review on spontaneous coronary artery dissection: insights into the angiographic finding and differential diagnosis. Frontiers in Cardiovascular Medicine, Nov 2023. URL: https://doi.org/10.3389/fcvm.2023.1278453, doi:10.3389/fcvm.2023.1278453. This article has 14 citations and is from a peer-reviewed journal.

  12. (bollati2024spontaneouscoronarydissection pages 4-7): Mario Bollati, Vincenzo Ercolano, and Pietro Mazzarotto. Spontaneous coronary dissection review: a complex picture. Reviews in Cardiovascular Medicine, Dec 2024. URL: https://doi.org/10.31083/j.rcm2512448, doi:10.31083/j.rcm2512448. This article has 6 citations and is from a peer-reviewed journal.

  13. (bollati2024spontaneouscoronarydissection media ffbb95a5): Mario Bollati, Vincenzo Ercolano, and Pietro Mazzarotto. Spontaneous coronary dissection review: a complex picture. Reviews in Cardiovascular Medicine, Dec 2024. URL: https://doi.org/10.31083/j.rcm2512448, doi:10.31083/j.rcm2512448. This article has 6 citations and is from a peer-reviewed journal.

  14. (bollati2024spontaneouscoronarydissection media 8d0f25c2): Mario Bollati, Vincenzo Ercolano, and Pietro Mazzarotto. Spontaneous coronary dissection review: a complex picture. Reviews in Cardiovascular Medicine, Dec 2024. URL: https://doi.org/10.31083/j.rcm2512448, doi:10.31083/j.rcm2512448. This article has 6 citations and is from a peer-reviewed journal.

  15. (bollati2024spontaneouscoronarydissection media 8c541a8a): Mario Bollati, Vincenzo Ercolano, and Pietro Mazzarotto. Spontaneous coronary dissection review: a complex picture. Reviews in Cardiovascular Medicine, Dec 2024. URL: https://doi.org/10.31083/j.rcm2512448, doi:10.31083/j.rcm2512448. This article has 6 citations and is from a peer-reviewed journal.

  16. (bollati2024spontaneouscoronarydissection media 353fb04a): Mario Bollati, Vincenzo Ercolano, and Pietro Mazzarotto. Spontaneous coronary dissection review: a complex picture. Reviews in Cardiovascular Medicine, Dec 2024. URL: https://doi.org/10.31083/j.rcm2512448, doi:10.31083/j.rcm2512448. This article has 6 citations and is from a peer-reviewed journal.

  17. (bollati2024spontaneouscoronarydissection media 94fd0b42): Mario Bollati, Vincenzo Ercolano, and Pietro Mazzarotto. Spontaneous coronary dissection review: a complex picture. Reviews in Cardiovascular Medicine, Dec 2024. URL: https://doi.org/10.31083/j.rcm2512448, doi:10.31083/j.rcm2512448. This article has 6 citations and is from a peer-reviewed journal.

  18. (bollati2024spontaneouscoronarydissection media df824cb3): Mario Bollati, Vincenzo Ercolano, and Pietro Mazzarotto. Spontaneous coronary dissection review: a complex picture. Reviews in Cardiovascular Medicine, Dec 2024. URL: https://doi.org/10.31083/j.rcm2512448, doi:10.31083/j.rcm2512448. This article has 6 citations and is from a peer-reviewed journal.

  19. (bollati2024spontaneouscoronarydissection pages 7-8): Mario Bollati, Vincenzo Ercolano, and Pietro Mazzarotto. Spontaneous coronary dissection review: a complex picture. Reviews in Cardiovascular Medicine, Dec 2024. URL: https://doi.org/10.31083/j.rcm2512448, doi:10.31083/j.rcm2512448. This article has 6 citations and is from a peer-reviewed journal.

  20. (rusali2025spontaneouscoronaryartery pages 14-16): Andrei Constantin Rusali, Ioana Caterina Lupu, Lavinia Maria Rusali, and Lucia Cojocaru. Spontaneous coronary artery dissection unveiled: pathophysiology, imaging, and evolving management strategies. Jun 2025. URL: https://doi.org/10.20944/preprints202506.0355.v1, doi:10.20944/preprints202506.0355.v1.

  21. (singulane2025spontaneouscoronaryartery pages 1-2): Cristiane C. Singulane, Shuo Wang, Kelsey Watts, Macy E. Stahl, LeAnn Denlinger, Rachel Lloyd, Pranavi Pallinti, Lauren Preston, Mohamed Morsy, Odayme Quesada, Angela Taylor, Randy K. Ramcharitar, Mete Civelek, and Patricia F. Rodriguez-Lozano. Spontaneous coronary artery dissection (scad): unveiling the enigma of the unexpected coronary event. Current Atherosclerosis Reports, Aug 2025. URL: https://doi.org/10.1007/s11883-025-01328-5, doi:10.1007/s11883-025-01328-5. This article has 8 citations and is from a peer-reviewed journal.