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(Circulation. 2001;103:269.)
© 2001 American Heart Association, Inc.

Clinical Investigation and Reports

Ventricular Preexcitation in Children and Young Adults

Atrial Myocarditis as a Possible Trigger of Sudden Death

Presented in part at the 72nd Scientific Sessions of the American Heart Association, Atlanta, Ga, November 7–10, 1999.

Cristina Basso, MD, PhD; Domenico Corrado, MD; Lino Rossi, MD; Gaetano Thiene, MD

From the Departments of Pathology (C.B., L.R., G.T.) and Cardiology (D.C.), University of Padua Medical School, Padua, Italy.

Correspondence to Gaetano Thiene, MD, FESC, FEACTS, Istituto di Anatomia Patologica, Via A. Gabelli 61, 35121 Padova, Italy. E-mail cardpath{at}ux1.unipd.it

Background—Sudden death (SD) in ventricular preexcitation (VP) syndrome is believed to be the result of atrial fibrillation with rapid ventricular response over the accessory pathway. Previous reports are anecdotal and often lack autopsy validation.

Methods and Results—Prevalence and clinicopathological features of VP were investigated in a series of 273 SDs in children and young adults (aged 35 years). Site of accessory atrioventricular (AV) connection was predicted by 12-lead ECG. Right and left AV ring together with the sinoatrial and AV septal junction were studied in serial histological sections. Ten patients (3.6%; male, mean age 24±7 years) had VP: 8 had Wolff-Parkinson-White (WPW) and 2 had Lown-Ganong-Levine (LGL) syndrome. Six patients had previous symptoms, and SD occurred at rest in all but 1. Pathological substrates of LGL consisted of AV-node hypoplasia and right-sided atrio-Hisian tract, respectively. In the 8 WPW patients, 10 total accessory AV pathways consisting of ordinary myocardium were found (7 left lateral, 2 right posterolateral, and 1 septal). These pathways were close to the endocardium (mean distance, 750±530 µm) and 310±190 µm thick. In 4 WPW patients (50%), isolated acute atrial myocarditis was found, which was polymorphous in 1 and lymphocytic in 3.

Conclusions—VP accounted for 3.6% of SD in young people and was not preceded by warning symptoms in 40%. A left accessory pathway was the most frequent substrate, and its subendocardial location supports the feasibility of catheter ablation. Isolated atrial myocarditis may act as a trigger of paroxysmal atrial fibrillation that leads to SD.

Key Words: atrium • death, sudden • myocarditis • pathology • Wolff-Parkinson-White syndrome

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