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A MIMICKER OF RHEUMATIC HEART DISEASE: CONGENITAL POLYVALVULAR DISEASE
2271 JACC March 21, 2017 Volume 69, Issue 11
FIT Clinical Decision Making A MIMICKER OF RHEUMATIC HEART DISEASE: CONGENITAL POLYVALVULAR DISEASE Poster Contributions Poster Hall, Hall C Friday, March 17, 2017, 3:45 p.m.-4:30 p.m. Session Title: FIT Clinical Decision‐Making: Prevention, Adult Congenital and Congenital Heart Disease Abstract Category: Congenital Heart Disease Presentation Number: 1169-427 Authors: Kiran Venkatesh, Benjamin Chen, Jennifer Walker, Theo Meyer, Mohammed Akhter, University of Massachusetts Medical School, Worcester, MA, USA
Background: A 31-year-old man presented with four months of dyspnea with an episode of significant dyspnea on exertion while at work. Upon presentation he was afebrile, normotensive and hypoxic with diffuse pulmonary edema on chest x-ray. He had been followed for chronic moderate mitral stenosis (MS), severe aortic stenosis (AS) with moderate aortic regurgitation, moderate tricuspid stenosis and mild pulmonic stenosis. Due to the polyvalvular nature of his disease, he carried the presumptive diagnosis of rheumatic heart disease (RHD). Despite slow interval worsening of valve gradients on yearly two dimensional transthoracic echocardiograms (TTE), this was his first episode in symptomatic heart failure (HF). He was not on chronic diuretics. His most recent TTE demonstrated a left ventricular ejection fraction of 60%, aortic valve (AV) peak pressure (PP) 85mmHg, AV mean gradient (MG) 45mmHg, AV area 0.7 cm2, mitral valve (MV) PP 32mmHg and MG of 100mmHg. An urgent TTE demonstrated significantly increased valve gradients across his AV (PP 160mmHg, MP 100mmHg) and MV (PP 53mmHg, MG 17mmHg). The presence of acute decompensated HF informed the decision to proceed with multivalvular surgical replacement. He underwent a cardiac computed tomography scan which was negative for any coronary artery disease nor coronary artery anomalies. In the operating room, the patient was found to have significant calcification of his MV and AV inconsistent with RHD. He received both a mechanical MV and AV replacement. Pathologic examination of the excised aortic and mitral valves demonstrated myxoid and calcific degeneration suggestive of chronic, non-rheumatic, valvular heart disease. Conclusions: As the incidence of RHD declines in the Western world, this case is a reminder that practitioners must be aware of alternative etiologies of polyvalvular disease, such as congenital heart disease (CHD). This is imperative as patients with CHD may require further workup and evaluation for other cardiac anomalies and/or systemic disease prior to valve replacement surgery.
FIT Clinical Decision Making A MIMICKER OF RHEUMATIC HEART DISEASE: CONGENITAL POLYVALVULAR DISEASE Poster Contributions Poster Hall, Hall C Friday, March 17, 2017, 3:45 p.m.-4:30 p.m. Session Title: FIT Clinical Decision‐Making: Prevention, Adult Congenital and Congenital Heart Disease Abstract Category: Congenital Heart Disease Presentation Number: 1169-427 Authors: Kiran Venkatesh, Benjamin Chen, Jennifer Walker, Theo Meyer, Mohammed Akhter, University of Massachusetts Medical School, Worcester, MA, USA
Background: A 31-year-old man presented with four months of dyspnea with an episode of significant dyspnea on exertion while at work. Upon presentation he was afebrile, normotensive and hypoxic with diffuse pulmonary edema on chest x-ray. He had been followed for chronic moderate mitral stenosis (MS), severe aortic stenosis (AS) with moderate aortic regurgitation, moderate tricuspid stenosis and mild pulmonic stenosis. Due to the polyvalvular nature of his disease, he carried the presumptive diagnosis of rheumatic heart disease (RHD). Despite slow interval worsening of valve gradients on yearly two dimensional transthoracic echocardiograms (TTE), this was his first episode in symptomatic heart failure (HF). He was not on chronic diuretics. His most recent TTE demonstrated a left ventricular ejection fraction of 60%, aortic valve (AV) peak pressure (PP) 85mmHg, AV mean gradient (MG) 45mmHg, AV area 0.7 cm2, mitral valve (MV) PP 32mmHg and MG of 100mmHg. An urgent TTE demonstrated significantly increased valve gradients across his AV (PP 160mmHg, MP 100mmHg) and MV (PP 53mmHg, MG 17mmHg). The presence of acute decompensated HF informed the decision to proceed with multivalvular surgical replacement. He underwent a cardiac computed tomography scan which was negative for any coronary artery disease nor coronary artery anomalies. In the operating room, the patient was found to have significant calcification of his MV and AV inconsistent with RHD. He received both a mechanical MV and AV replacement. Pathologic examination of the excised aortic and mitral valves demonstrated myxoid and calcific degeneration suggestive of chronic, non-rheumatic, valvular heart disease. Conclusions: As the incidence of RHD declines in the Western world, this case is a reminder that practitioners must be aware of alternative etiologies of polyvalvular disease, such as congenital heart disease (CHD). This is imperative as patients with CHD may require further workup and evaluation for other cardiac anomalies and/or systemic disease prior to valve replacement surgery.