Refractory left main coronary vasospasm and electrical storm in a transgender woman: a case report

Coronary vasospasm is the aetiology in up to 46% of cases of myocardial infarction with non-obstructive coronary arteries. It may rarely precipitate ventricular tachyarrhythmias and cardiogenic shock.

A 56-year-old transgender woman on oestrogen therapy presented with worsening chest pain. They had known history of coronary artery disease and, a month prior, had undergone coronary angiography for syncope due to episodes of polymorphic ventricular tachycardia; this had shown severe left main vasospasm that responded to intracoronary nitroglycerin. They underwent implantable cardioverter-defibrillator placement and were discharged on high-dose vasodilators. At the time of their current presentation, labs were notable for high-sensitivity troponin T elevation to 375 ng/L and a negative toxicology screen. Electrocardiogram revealed anterolateral T-wave inversions, and transthoracic echocardiogram showed left ventricular ejection fraction of 25%–29% with diffuse mid-apical hypokinesis. With concern for recurrent vasospasm, intravenous nitroglycerin infusion was initiated. However, their course was complicated by electrical storm requiring venoarterial extracorporeal membrane oxygenation (ECMO) and microaxial flow pump placement. Coronary angiography with intravascular ultrasound re-demonstrated severe left main vasospasm. A stellate ganglion block was unsuccessful in preventing further ventricular tachyarrhythmias hence left main stenting was performed, allowing successful ECMO decannulation and microaxial flow pump removal. Oestrogen therapy was held at time of discharge.

Our case highlights that severe coronary vasospasm can trigger electrical storm due to transmural ischaemia despite optimal medical therapy. Such cases may require mechanical circulatory support and coronary revascularization. Further studies are needed to assess the role that oestrogen therapy plays in transgender women experiencing coronary vasospasm.