Cardiac Cases from the New England Journal

Kratka K…Loscalzo, J. Case Reprots — A Stormy Heart. N Engl J Med. 2023 Jan 5;388(1):71–78. PMID: 36599065

· One of the best ways to keep up with clinical cardiology (besides Hartford Hospital’s excellent cardiology grand rounds) is to read the N Engl J Med case reports on cardiac topics. You get to read interesting cases discussed by excellent clinicians. These are my notes from the cited case, although I inserted the comments on Doug Zipes, a friend of mine, and the comment on female sex and race.

· “A Stormy Heart” (Jan 5, 2023) describes a 68 year old woman (race not given) who presented after 2 years of palpatations with VT and a “relatively narrow QRS.”

· The narrow QRS suggests fasciluar ventricular tachycardia or “verapamil sensitive ventricular tachycardia” described by Doug Zipes over 40 years ago (Rinkenberger RL, Prystowsky EN, Heger JJ, Troup PJ, Jackman WM, Zipes DP.Effects of intravenous and chronic oral verapamil administration in patientswith supraventricular tachyarrhythmias. Circulation. 1980 Nov;62(5):996–1010. PMID: 7418184 ),

· So she was discharged on verapamil.

· Verapamil sensitive ventricular tachycardia would be an unusual diagnosis for this woman, since the usual age is aged 15–40 years.

· She soon returned with VT storm (diagnosed by 3 or more episodes of sustained VT in 24 hours or recurremnt VT within 5 mins of prior VT, or more ventricular beats than sinus beats over a 24 hour period.)

· A cardiac PET was done to evaluate possible cardiac sarcoid. PET detects sarcoid because sarcoid nodules are flurodeoxyglucose (FDG) avid.

· This showed a moderate perfusion defect of the cardiac base, anterior lateral and inferior lateral walls, but also mildly avid lymph node uptake.

· So, she underwent lymph node biopsy for sarcoid, but that was not diagnostic. The sensitivity of biopsy of a hot peripheral node for sarcoid is >90% so the negative biopsy is unusual.

· Nevertheless, she was presumptively treated for sarcoid with steroids.

· She again had VT so underwent a cardiac biopsy of the area deemed abnormal on prior studies.

· This showed cardiac infiltration of multinucleated giant cells diagnostic of giant cell myocarditis.

· Giant cell myocarditis is rare (0.13 / 100,000 individuals) and usually presents with acute-onset, fulminant heart failure and less frequently with ventricular arrhythmias.

· A more indolent course may be more common in the elderly (I am insulted that 68 is considered elderly).

· Besides VT giant cell myocarditis can present with high grade heart block.

· Interestingly, her race was not given. According to Google, women get sarcoid more often than men (women also get it more often later in life), and sarcoid is 2.5x more frequent in blacks than in whites.

· The paper discusses that biopsy for unexplained arrhythmia has a IIB recommendation (may be considered), but that diagnosing giant cell myocarditis requires a biopsy and that biopsies should be guided by non-invasive imaging.

I hope these notes are useful to you.