Severe familial coronary artery spasm in 2 siblings: About 2 cases

Vasospastic angina is the spasm of coronary arteries causing transient myocardial ischemia. VSA is commonly managed with antispasmodic medications including calcium-channel blockers and nitrates. When vasospasm is refractory to conventional medications, unconventional treatment modalities may be used for symptomatic relief. Coronary artery spasm was observed in 2 sisters. Neither of them had significant atheromatous stenosis in the coronary arteries. The 22-year younger sister presented with rest angina in the early morning. The 32-year-old elder sister complained of rest and effort angina. Their coronary angiogram showed spontaneous spasm in the proximal segment of the left anterior descending coronary artery. The youngest one had resistant and recurrent coronary vasospasm involving different segments of the coronary tree causing myocardial infarction with total occlusion of the proximal segment in the left anterior descending coronary artery. Our patients presented with a lesser-known phenomenon called refractory VSA, where intermittent vasospasm continues despite being on a combination of 2 medications. The familial appearance of coronary artery spasm had been previously reported. Although it is not well understood, the underlying mechanism appears to involve a combination of endothelial damage and vasoactive mediators. Genetic factors such as human leucocyte antigen contribute to susceptibility to coronary spasm in some patients with VSA. Treatment for VSA is well documented; however, little data is available for refractory VSA.