Rare Form of Right Ventricular Outflow Tract Obstruction
A 44-year-old woman had undergone repair of a perimembranous ventricular septal defect at age 9. She had been doing well medically until she developed fever, bloody diarrhea, nausea, vomiting, generalized abdominal pain, and a 10-lb weight loss shortly after undergoing a dental procedure. Blood cultures were positive for Gram-negative bacilli (Alcaligenes xylosoxidans), and intravenous antibiotics were initiated. Her symptoms improved, but repeat blood cultures remained positive. Bacterial endocarditis was suspected, warranting transthoracic and transesophageal echocardiography. The patient was transferred to The Cleveland Clinic for further evaluation and treatment.
Transesophageal echocardiography (Movies I and II) and cardiac magnetic resonance imaging (Movies III and IV) demonstrated a variant of “double-chamber right ventricle,” with resultant right ventricular outflow tract obstruction demonstrated by flow turbulence and increased flow gradient within the RV. Furthermore, a small vegetation was noted on the pulmonic valve.
The patient underwent open-heart surgery, which consisted of debridement of the bacterial vegetations from the pulmonic valve and right ventricle, resection of the right ventricular infundibular muscle, repair of the tricuspid valve with sutured commissuroplasty, and closure of a patent foramen ovale. On the most recent follow-up examination, she was doing well.
The online-only Data Supplement, which contains Movies I through IV, is available with this article at http://www.circulationaha.org.