Echocardiographic Assessment of Left Ventricular Function in Type 1 Gaucher's Disease
There is predominate opinion among physicians managing type 1 Gauchers' disease (GD) that cardiac involvement is not an issue in these patients. In order to follow this hypothesis, we prospectively investigated 15 adult imiglucerase-treated type 1 GD patients by echocardiography, Doppler, and t...
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| Format: | Article |
| Language: | English |
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Wiley
2010-01-01
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| Series: | Advances in Hematology |
| Online Access: | http://dx.doi.org/10.1155/2010/820843 |
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| author | Mirta Koželj Samo Zver Vesna Zadnik |
| author_facet | Mirta Koželj Samo Zver Vesna Zadnik |
| author_sort | Mirta Koželj |
| collection | DOAJ |
| description | There is predominate opinion among physicians managing type 1 Gauchers' disease (GD) that cardiac involvement is not an issue in these patients. In order to follow this hypothesis, we prospectively investigated 15 adult imiglucerase-treated type 1 GD patients by echocardiography, Doppler, and tissue Doppler echocardiography. This was a case-controlled study with 18 matched healthy volunteers. The obtained data was correlated with the levels of NT-proBNP (brain natriuretic peptide). None of the GD patients had clinical signs of heart disease. In 3 of the 15 patients, we observed echocardiographic signs of aortic and mitral valve calcification. The left ventricular systolic function was within normal limits. Compared to the control group, there was no statistically significant difference observed in the most sensitive indices of left ventricular diastolic function, parameter Em (P=.095), and E/Em ratio (P=.097), as demonstrated by tissue Doppler echocardiography. However, there was a positive correlation between the E/Em ratio and NT-proBNP plasma levels (P=.009). In conclusion, the prospective echocardiographic study of type 1 GD patients did not validate any left ventricular dysfunction. But, the E/Em ratio showed a strong statistical correlation with the most sensitive indicators of heart failure, NT-proBNP. Research on larger groups of patients and the usage of even more sensitive methods as strain-rate imaging will be necessary to confirm eventual myocardial involvement in GD patients. |
| format | Article |
| id | doaj-art-637ea721eb764d9aa31cbcce1ec6e40c |
| institution | Kabale University |
| issn | 1687-9104 1687-9112 |
| language | English |
| publishDate | 2010-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Advances in Hematology |
| spelling | doaj-art-637ea721eb764d9aa31cbcce1ec6e40c2025-02-03T05:52:39ZengWileyAdvances in Hematology1687-91041687-91122010-01-01201010.1155/2010/820843820843Echocardiographic Assessment of Left Ventricular Function in Type 1 Gaucher's DiseaseMirta Koželj0Samo Zver1Vesna Zadnik2Department of Cardiology, University Medical Centre Ljubljana, Zaloška 7, 1525 Ljubljana, SloveniaDepartment of Haematology, University Medical Centre Ljubljana, Zaloška 7, 1525 Ljubljana, SloveniaInstitute of Oncology, Zaloška 2, 1525 Ljubljana, SloveniaThere is predominate opinion among physicians managing type 1 Gauchers' disease (GD) that cardiac involvement is not an issue in these patients. In order to follow this hypothesis, we prospectively investigated 15 adult imiglucerase-treated type 1 GD patients by echocardiography, Doppler, and tissue Doppler echocardiography. This was a case-controlled study with 18 matched healthy volunteers. The obtained data was correlated with the levels of NT-proBNP (brain natriuretic peptide). None of the GD patients had clinical signs of heart disease. In 3 of the 15 patients, we observed echocardiographic signs of aortic and mitral valve calcification. The left ventricular systolic function was within normal limits. Compared to the control group, there was no statistically significant difference observed in the most sensitive indices of left ventricular diastolic function, parameter Em (P=.095), and E/Em ratio (P=.097), as demonstrated by tissue Doppler echocardiography. However, there was a positive correlation between the E/Em ratio and NT-proBNP plasma levels (P=.009). In conclusion, the prospective echocardiographic study of type 1 GD patients did not validate any left ventricular dysfunction. But, the E/Em ratio showed a strong statistical correlation with the most sensitive indicators of heart failure, NT-proBNP. Research on larger groups of patients and the usage of even more sensitive methods as strain-rate imaging will be necessary to confirm eventual myocardial involvement in GD patients.http://dx.doi.org/10.1155/2010/820843 |
| spellingShingle | Mirta Koželj Samo Zver Vesna Zadnik Echocardiographic Assessment of Left Ventricular Function in Type 1 Gaucher's Disease Advances in Hematology |
| title | Echocardiographic Assessment of Left Ventricular Function in Type 1 Gaucher's Disease |
| title_full | Echocardiographic Assessment of Left Ventricular Function in Type 1 Gaucher's Disease |
| title_fullStr | Echocardiographic Assessment of Left Ventricular Function in Type 1 Gaucher's Disease |
| title_full_unstemmed | Echocardiographic Assessment of Left Ventricular Function in Type 1 Gaucher's Disease |
| title_short | Echocardiographic Assessment of Left Ventricular Function in Type 1 Gaucher's Disease |
| title_sort | echocardiographic assessment of left ventricular function in type 1 gaucher s disease |
| url | http://dx.doi.org/10.1155/2010/820843 |
| work_keys_str_mv | AT mirtakozelj echocardiographicassessmentofleftventricularfunctionintype1gauchersdisease AT samozver echocardiographicassessmentofleftventricularfunctionintype1gauchersdisease AT vesnazadnik echocardiographicassessmentofleftventricularfunctionintype1gauchersdisease |