What is the most rare vascular cardio disease in female childbearing age?

Most Rare Vascular Cardiovascular Disease in Females of Childbearing Age

Spontaneous coronary artery dissection (SCAD) is the most rare vascular cardiovascular disease in females of childbearing age, particularly occurring in the peripartum period, with significant implications for morbidity and mortality. 1

Epidemiology and Prevalence

• SCAD accounts for up to 4% of all acute coronary syndromes but represents 20-35% of acute coronary syndromes in women under 60 years of age 1
• Over 90% of SCAD cases occur in women, disproportionately affecting young to middle-aged women without traditional cardiovascular risk factors 1
• Peripartum SCAD is particularly rare but represents a significant cause of pregnancy-associated acute coronary syndromes 2, 1
• Cardiomyopathies, including SCAD, are rare but represent severe causes of cardiovascular complications in pregnancy 2

Pathophysiology

• SCAD involves spontaneous formation of an intramural hematoma within the coronary artery wall, with or without an intimal tear 1
• The condition is predominantly caused by a combination of predisposing arteriopathies and precipitating stressors 1
• Pregnancy-related hormonal changes contribute to structural alterations in the collagen of vessel walls, increasing vulnerability to dissection 2
• Thrombi and dissections occur more frequently in the peripartum period than before delivery 2

Risk Factors and Associations

• Fibromuscular dysplasia (FMD) is present in up to 72% of SCAD patients and represents the strongest association 1
• Connective tissue disorders including systemic lupus erythematosus and Marfan syndrome predispose to weakened arterial walls 1
• Pregnancy and the peripartum period represent significant risk factors 1, 3
• Intense emotional or physical stress, including labor and delivery, can trigger SCAD in predisposed individuals 1

Clinical Presentation

• Patients typically present with chest pain, acute coronary syndrome symptoms, or sudden cardiac death 3, 4
• SCAD should be suspected in any young woman presenting with acute coronary syndrome without traditional risk factors 1
• Presentation is often as ST-elevation myocardial infarction in approximately 49% of cases 5
• Some cases may be asymptomatic and discovered incidentally 6

Diagnosis

• Diagnosis is confirmed by coronary angiography, potentially supplemented by intracoronary imaging (OCT or IVUS) 1, 7
• Modern imaging techniques such as optical coherence tomography, intravascular ultrasound, and coronary angiography have contributed to earlier diagnosis 4
• Angiographically, SCAD may present with the classic "flap" and multiple lumens, or as an intramural hematoma without intimal rupture 7

Management Considerations

• Conservative management is preferred for stable patients, as revascularization attempts can worsen dissections 1
• Beta-blockers are strongly recommended for long-term management as they reduce recurrence risk 1
• Percutaneous coronary intervention (PCI) is associated with high rates of complications (35%) and technical failure 5, 3
• Coronary artery bypass grafting (CABG) may be preferable over PCI in peripartum SCAD to avoid complications or sudden cardiac death 3
• High-risk pregnant patients should be managed by interdisciplinary teams in specialized centers 8

Prognosis and Follow-up

• In-hospital mortality is generally low regardless of initial treatment 5
• Long-term prognosis requires careful monitoring as SCAD can recur, particularly in women 5
• Estimated 10-year rate of major adverse cardiac events (death, heart failure, myocardial infarction, and SCAD recurrence) is 47% 5
• Regular follow-up is essential due to the risk of recurrence 4

SCAD represents a unique challenge in cardiovascular medicine due to its rarity, predilection for young women without traditional risk factors, and its association with pregnancy. Early recognition, appropriate imaging, and careful management decisions are crucial to improve outcomes in this vulnerable population.