A rare cause of congestive heart failure in a young woman

Transcription

1 A rare cause of congestive heart failure in a young woman MM. Gurzun, C. Beladan, BA. Popescu, M. Rosca, A. Calin, B. Radulescu, I. Marinica, V. Herlea, C. Ginghina Emergency Institute of Cardio-Vascular Disease C. C. Iliescu. Bucharest, Romania University of Medicine and Pharmacy, Bucharest, Romania

2 Incidence of advanced congestive heart failure Zannad et al., JACC, 1999

3 25 year old, woman Shortness of breath at rest Exertional dispnoea (1 month) Natural child birth (8 years before) a loud (3/6) systolic murmur (apex ->4-5 th intercostals space) signs of systemic and pulmonary congestion: pulmonary rales, jugular venous distention, ankle edema and hepatomegaly

4

5

6 MR etiology in adults/children Congenital Rheumatic Endocarditis Inflammatory uroheart Survey- Valvular disease, EHJ, 2003 Erez E, J Heart Valve Dis, 2003

7

8 Rheumatic valvular heart disease Trigger: microbial agent History: acute rheumatic fever Endocarditis Antiphospholipid Syndrome Commisural fussion Cronic inflamation Carcinoid syndrome Thickened valves and cordae Drug induced valvular heart disease (DIVHD) Calcification Congenital mitral regurgitation Veinot J., Cardiovascular Pathology 2006

9 Rheumatic valvular heart disease Previous healed episode of infective endocarditis Endocarditis Normal mitral valve Rheumatic mitral disease Congenital mitral disease Antiphospholipid Syndrome Suggestive clinical history Valvular echocardiographic morphology suggestive for endocarditis (vegetation, abcess, fistula) Carcinoid syndrome Microbiological diagnosis- blood cultures Drug induced valvular heart disease (DIVHD) Pathological examination of resected valvular tissue dg. gold standard. Congenital mitral regurgitation

10 Rheumatic valvular heart disease Primary Secondary (SLE-ANA) Endocarditis Antiphospholipid syndrome Anticardiolipin antibodies anti-ß 2 -glycoprotein I Lupus anticoagulant Antiphospholipid Syndrome Immunological injury Carcinoid syndrome Drug induced valvular heart disease (DIVHD) Valve masses (vegetations) Libman-Sacks endocarditis Valvular thickening Normal subvalvular apparatus Congenital mitral regurgitation Hojnik M, Circulation 1996

11 Rheumatic valvular heart disease carcinoid tumor SEROTONIN Endocarditis Flusing Diarrhea Bronhoconstriction Antiphospholipid Syndrome 5HT-2B receptor Carcinoid syndrome Pulmonary metastasis PFO lung Mitogenesis and GAG/colagen production Drug induced valvular heart disease (DIVHD) Plaque formation Congenital mitral regurgitation Valve/subvalvular thichening 5 HIAA urinary excretion

12 Rheumatic valvular heart disease Ergotamine, Endocarditis Methysergide Pergolide, Carbegoline, Bromocriptine MDMA Fenfluramine, Benfluorex Antiphospholipid Syndrome Carcinoid syndrome Drug induced valvular heart disease (DIVHD) Congenital mitral regurgitation DIVHD (definition) positive history of valvulopathic drug use. Cosyns B., Heart, 2012

13 Rheumatic valvular heart disease Congenital MR in adults rare % of mitral valve regurgitations 2% of mitral valve surgery Initial presentation in NYHA I-II. Endocarditis Antiphospholipid Syndrome Carcinoid syndrome Valvular Cleft 58% Retraction 2% Prolaps 40% Chordae Long 66% Short 13% Papilary muscle abnormality 5% Zegdi Z., EJCVS, 2008 Drug induced valvular heart disease (DIVHD) Congenital mitral regurgitation Jiang S., The Heart Surgery Forum, 2011

14 Indication for surgery in severe organic mitral regurgitation VHD Guidelines, 2007

15 Replacement/repair in organic MR Complex mitral valve repaire: Both leaflets augmentation Repair fesability % Chordae elongation Enriquez-Sarano M et al. Circulation 1995; Copyright American Heart Association

16 Histopathological examination of excised mitral valve tissue Microscopic images Haematoxylin Eosin stain

17 Severe congenital mitral regurgitation - unusual valvular morphology embryonic fetal Kruithof P.- Developmental biology, 2007 Butcher,Phill. Trans. R. Soc., 2007

18 Chronic decomp MR Severe congenital mitral regurgitation atypical pathophysiological evolution EF normal Acute MR EF Chronic comp MR EF Braunwald s Heart Disease, Eighth edition

19 Conclusion Congenital disease- cause of heart failure in a young, recently symptomatic adult Congenital MR- difficult preoperative etiological diagnosis (Histopathology of excised valve tissue) Atypical pathophysiological evolution Isolated endothelial cushion defect

20 A rare cause of congestive heart failure in a young woman