© 2004 by European Society of Cardiology
Copyright © 2003, The European Society of Cardiology
A rare case of left ventricular outflow obstruction
aNuovo Ospedale S.Giovanni di Dio, Cardiology Unit, Florence, Italy
bClinica Medica I, University of Florence, Florence, Italy
cVilla Maria Beatrice Clinic, Cardiac Surgery Unit, Florence, Italy
dDepartment of Human Pathology and Oncology, University of Florence, Florence, Italy
* Corresponding author. Via Cento Stelle n.3, 50137 Florence, Italy. Tel.: +39-055-613194. sergiominneci{at}hotmail.com
| Abstract |
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We report a patient with a mass originating from the anterior mitral valve leaflet causing severe left ventricular outflow tract obstruction. Noninvasive imaging provided the best diagnostic tools for diagnosis. Histological findings showed a very rare giant blood cyst of the mitral valve.
Keywords: cardiac mass; echocardiography; cardiac MRI
| 1. Case report |
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We evaluated a 44-year-old woman for exertional chest pain and dyspnea. The clinical history put in evidence of a familial ischemic heart disease only. Physical examination revealed a normal blood pressure and a harsh midsystolic murmur. The ECG showed QRS and ST criteria of left ventricular hypertrophy.
The echocardiographic examination showed concentric left ventricular hypertrophy and a spherical, poorly reflecting mass, 2 cm in diameter, in the left ventricle. The mass was adherent to the anterior mitral leaflet, with a systolic motion towards the aorta causing left ventricular outflow obstruction with a severe pressure gradient at Doppler examination (100 mmHg). There was moderate to severe mitral regurgitation caused by the anterior leaflet displacement (Fig. 1). MR imaging clearly depicted the mass, its relationship with the anterior mitral leaflet and the left ventricular systolic outflow obstruction on cine loop series. On spin echo T1 images the mass showed a homogeneous increased signal intensity indicating a bloody content and suggesting a cystic origin (Fig. 2).
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Cardiac catheterization provided no further specific information and the coronary arteries were normal.
Operative excision of the mass was performed through an aortotomy (Fig. 3) and a valvular bioprosthesis was inserted. Postoperative course was uneventful.
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The histological findings confirmed a smooth round mass, approximately 2 cm in diameter, attached to the ventricular surface of the anterior mitral leaflet. The mass looked like a cyst, thinly walled and containing a bloody fluid. Microscopically, the cystic wall was formed by hypocellular connective tissue lined on the inside by flattened cells. The leaflet consisted of dense collagen with many loose vascular lacunae (Fig. 4).
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| 2. Discussion |
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Blood cysts of the heart valves are congenital in origin and are most frequently a postmortem finding in fetuses and infants <6 months of age. Such a cyst was first reported by Elsasser in 1844.1 The cysts can vary in size: the small cysts (<2 mm in diameter) are generally asymptomatic, have no clinical relevance and disappear with age, probably as an involution after thrombosis of stagnant blood.2 The cysts rarely persist in elderly; in these cases they are larger (2–40 mm in diameter) and cause cardiac dysfunction.3–5 Various theories have been proposed to explain the development of blood cysts (progressive enlargement of vascular spaces, alteration during valve development, inflammation, vagal stimulation, anoxia and hemorrhagic diathesis) but there is no consensus. They may be unilocular, as in our case, or multilocular. Cardiac blood cysts are most commonly present in the atrioventricular valves and less often in the semilunar valves. Histologically, they are usually lined with flat endothelium-like cells on both sides and contain a bloody fluid; organized and calcified thrombi have sometimes been found. The natural history is not known. Conservative management in asymptomatic patients with a small cyst and echocardiographic follow up are suggested, while surgical resection should be performed for masses that interfere with cardiac function. We found two other reports of giant blood cysts of the mitral valve that were surgically treated.3,6
This case report confirms the fundamental role of echocardiography for the evaluation of intracardiac masses, as it supplies adequate information about tumor size, attachment and mobility, thus providing important information about the hemodynamic and functional consequences. Additional information about the nature of the lesion and its content is given by MR imaging.
Tumors of the mitral leaflets are very rare: fibroelastoma, a benign tumor, being the most commonly observed. Echocardiography most often allows a differential diagnosis.7
| References |
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- Elsasser C. Bericht uber die ereignisse in der gebasanstalt des Catherinen-Hospital im Jahre 1844. Med Correspondenzblatt (1844) 14:297.
- Minato H., Manabe T., Masaki H., Kawahara Y. Blood cyst of the pulmonary valve in an adult: report of a case and review of the literature. Hum Pathol (1997) 28:252–255.[CrossRef][Web of Science][Medline]
- Arnold I.R., Hubner P.J., Firmin R.K. Blood filled cyst of the papillary muscle of the mitral valve producing severe left ventricular outflow tract obstruction. Br Heart J (1990) 63:132–133.
[Abstract/Free Full Text] - Pelikan H.M.P., Tsang T.S.M., Seward J.B. Giant blood cyst of the mitral valve. J Am Soc Echocardiogr (1999) 12:1005–1007.[CrossRef][Web of Science][Medline]
- Abreu A., Galrinho A., Sa E.P., Ramos S., Martins Ap., Fragara J., et al. Hamartoma of the mitral valve with blood cysts: a rare tumor detected by echocardiography. J Am Soc Echocardiogr (1998) 11:832–836.[CrossRef][Web of Science][Medline]
- Ohmoto Y., Tsuchihashi K., Tamaka S., Shimamoto K., Iimura O. Giant endocardial blood cyst in left ventricle resected by transaortic valve approach. Chest (1993) 103(3):965–966.[CrossRef][Web of Science][Medline]
- Klarich K.Y., Enriquez-Sarano M., Gura G.M., Edwards W.D., Tajik A.J., Seward J.B. Papillary fibroelastoma: echocardiographic characteristics for diagnosis and pathologic correlation. J Am Coll Cardiol (1997) 30:784–790.[Abstract]
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