Frequency of Cardiovascular Involvement in Familial Amyloidotic Polyneuropathy in Brazilian Patients
Background:Familial amyloidotic polyneuropathy (FAP) is a rare disease diagnosed in Brazil and worldwide. The frequency of cardiovascular involvement in Brazilian FAP patients is unknown.Objective:Detect the frequency of cardiovascular involvement and correlate the cardiovascular findings with the m...
| Main Authors: | , , , , , , , |
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| Format: | Article |
| Language: | English |
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Sociedade Brasileira de Cardiologia (SBC)
2015-11-01
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| Series: | Arquivos Brasileiros de Cardiologia |
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| Online Access: | http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0066-782X2015002400503&lng=en&tlng=en |
| _version_ | 1828869279434407936 |
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| author | Márcia Cavalcanti de Campos Queiroz Roberto Coury Pedrosa Amanda Cardoso Berensztejn Basílio de Bragança Pereira Emília Matos do Nascimento Martha Maria Turano Duarte Pedro Paulo Pereira-Junior Marcia Waddington Cruz |
| author_facet | Márcia Cavalcanti de Campos Queiroz Roberto Coury Pedrosa Amanda Cardoso Berensztejn Basílio de Bragança Pereira Emília Matos do Nascimento Martha Maria Turano Duarte Pedro Paulo Pereira-Junior Marcia Waddington Cruz |
| author_sort | Márcia Cavalcanti de Campos Queiroz |
| collection | DOAJ |
| description | Background:Familial amyloidotic polyneuropathy (FAP) is a rare disease diagnosed in Brazil and worldwide. The frequency of cardiovascular involvement in Brazilian FAP patients is unknown.Objective:Detect the frequency of cardiovascular involvement and correlate the cardiovascular findings with the modified polyneuropathy disability (PND) score.Methods:In a national reference center, 51 patients were evaluated with clinical examination, electrocardiography (ECG), echocardiography (ECHO), and 24-hour Holter. Patients were classified according to the modified PND score and divided into groups: PND 0, PND I, PND II, and PND > II (which included PND IIIa, IIIb, and IV). We chose the classification tree as the statistical method to analyze the association between findings in cardiac tests with the neurological classification (PND).Results:ECG abnormalities were present in almost 2/3 of the FAP patients, whereas ECHO abnormalities occurred in around 1/3 of them. All patients with abnormal ECHO also had abnormal ECG, but the opposite did not apply. The classification tree identified ECG and ECHO as relevant variables (p < 0.001 and p = 0.08, respectively). The probability of a patient to be allocated to the PND 0 group when having a normal ECG was over 80%. When both ECG and ECHO were abnormal, this probability was null.Conclusions:Brazilian patients with FAP have frequent ECG abnormalities. ECG is an appropriate test to discriminate asymptomatic carriers of the mutation from those who develop the disease, whereas ECHO contributes to this discrimination. |
| first_indexed | 2024-12-13T05:47:14Z |
| format | Article |
| id | doaj.art-37334af2b87045f68288a27b59e32e95 |
| institution | Directory Open Access Journal |
| issn | 1678-4170 |
| language | English |
| last_indexed | 2024-12-13T05:47:14Z |
| publishDate | 2015-11-01 |
| publisher | Sociedade Brasileira de Cardiologia (SBC) |
| record_format | Article |
| series | Arquivos Brasileiros de Cardiologia |
| spelling | doaj.art-37334af2b87045f68288a27b59e32e952022-12-21T23:57:38ZengSociedade Brasileira de Cardiologia (SBC)Arquivos Brasileiros de Cardiologia1678-41702015-11-01105550350910.5935/abc.20150112S0066-782X2015002400503Frequency of Cardiovascular Involvement in Familial Amyloidotic Polyneuropathy in Brazilian PatientsMárcia Cavalcanti de Campos QueirozRoberto Coury PedrosaAmanda Cardoso BerensztejnBasílio de Bragança PereiraEmília Matos do NascimentoMartha Maria Turano DuartePedro Paulo Pereira-JuniorMarcia Waddington CruzBackground:Familial amyloidotic polyneuropathy (FAP) is a rare disease diagnosed in Brazil and worldwide. The frequency of cardiovascular involvement in Brazilian FAP patients is unknown.Objective:Detect the frequency of cardiovascular involvement and correlate the cardiovascular findings with the modified polyneuropathy disability (PND) score.Methods:In a national reference center, 51 patients were evaluated with clinical examination, electrocardiography (ECG), echocardiography (ECHO), and 24-hour Holter. Patients were classified according to the modified PND score and divided into groups: PND 0, PND I, PND II, and PND > II (which included PND IIIa, IIIb, and IV). We chose the classification tree as the statistical method to analyze the association between findings in cardiac tests with the neurological classification (PND).Results:ECG abnormalities were present in almost 2/3 of the FAP patients, whereas ECHO abnormalities occurred in around 1/3 of them. All patients with abnormal ECHO also had abnormal ECG, but the opposite did not apply. The classification tree identified ECG and ECHO as relevant variables (p < 0.001 and p = 0.08, respectively). The probability of a patient to be allocated to the PND 0 group when having a normal ECG was over 80%. When both ECG and ECHO were abnormal, this probability was null.Conclusions:Brazilian patients with FAP have frequent ECG abnormalities. ECG is an appropriate test to discriminate asymptomatic carriers of the mutation from those who develop the disease, whereas ECHO contributes to this discrimination.http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0066-782X2015002400503&lng=en&tlng=enNeuropatias Amilóides FamiliaresCardiomiopatiasPré-AlbuminaEletrocardiografia |
| spellingShingle | Márcia Cavalcanti de Campos Queiroz Roberto Coury Pedrosa Amanda Cardoso Berensztejn Basílio de Bragança Pereira Emília Matos do Nascimento Martha Maria Turano Duarte Pedro Paulo Pereira-Junior Marcia Waddington Cruz Frequency of Cardiovascular Involvement in Familial Amyloidotic Polyneuropathy in Brazilian Patients Arquivos Brasileiros de Cardiologia Neuropatias Amilóides Familiares Cardiomiopatias Pré-Albumina Eletrocardiografia |
| title | Frequency of Cardiovascular Involvement in Familial Amyloidotic Polyneuropathy in Brazilian Patients |
| title_full | Frequency of Cardiovascular Involvement in Familial Amyloidotic Polyneuropathy in Brazilian Patients |
| title_fullStr | Frequency of Cardiovascular Involvement in Familial Amyloidotic Polyneuropathy in Brazilian Patients |
| title_full_unstemmed | Frequency of Cardiovascular Involvement in Familial Amyloidotic Polyneuropathy in Brazilian Patients |
| title_short | Frequency of Cardiovascular Involvement in Familial Amyloidotic Polyneuropathy in Brazilian Patients |
| title_sort | frequency of cardiovascular involvement in familial amyloidotic polyneuropathy in brazilian patients |
| topic | Neuropatias Amilóides Familiares Cardiomiopatias Pré-Albumina Eletrocardiografia |
| url | http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0066-782X2015002400503&lng=en&tlng=en |
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