Left ventricular diastolic heart failure in teenagers who underwent balloon aortic valvuloplasty in early infancy.
We report 4 teenage patients who, after successful palliation of severe aortic valve stenosis by balloon aortic valvuloplasty in early infancy, presented within the previous 3 years with significant left ventricular (LV) diastolic heart failure. All patients had remained asymptomatic until their teenage years and had normal or hyperdynamic LV systolic function on presentation, with limited residual aortic valve stenosis and regurgitation. All underwent echocardiography, cardiac catheterization, and cardiac magnetic resonance evaluation. One notable common feature in the 4 patients was the presence of a confluent layer of LV subendocardial hyperenhancement demonstrated by late gadolinium enhancement technique. Histopathology was available for 2 patients, which documented significant LV endocardial fibroelastosis. One patient who underwent endocardial fibroelastosis resection in conjunction with aortic valve replacement had late clinical improvement. In conclusion, this group of patients may represent an important emerging clinical entity and merits close clinical surveillance, with prospective assessment of diastolic function.
Robinson
Joshua D
JD
Department of Cardiology, Children's Hospital Boston, Boston, Massachusetts, USA.
Del Nido
Pedro J
PJ
Geggel
Robert L
RL
Perez-Atayde
Antonio R
AR
Lock
James E
JE
Powell
Andrew J
AJ
D000293:Adolescent; D001024:Aortic Valve Stenosis / Q000151:congenital* / Q000601:surgery; D002404:Catheterization; D003287:Contrast Media; D003971:Diastole; D018450:Disease Progression; D004452:Echocardiography; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D018487:Ventricular Dysfunction, Left / Q000209:etiology* / Q000601:surgery*
D003287:Contrast Media