Pulmonary vascular complications of hereditary haemorrhagic telangiectasia.
Curr Opin Pulm Med
; 20(5): 421-8, 2014 Sep.
Article
em En
| MEDLINE
| ID: mdl-25032812
ABSTRACT
PURPOSE OF REVIEW The purpose of this study is to present the latest advances and recommendations in the diagnosis and treatment of pulmonary vascular complications associated with hereditary haemorrhagic telangiectasia (HHT) pulmonary arteriovenous malformations (PAVMs), pulmonary arterial hypertension (PAH), pulmonary hypertension associated with high output cardiac failure or liver vascular malformations, haemoptysis, haemothorax and thromboembolic disease. RECENT FINDINGS:
Transthoracic contrast echocardiography has been validated as a screening tool for PAVM in patients with suspected HHT. Advancements in genetic testing support its use in family members at risk as a cost-effective measure. Therapy with bevacizumab in patients with high output cardiac failure and severe liver AVMs showed promising results. PAH tends to be more aggressive in HHT type 2 patients.SUMMARY:
Patients suffering from this elusive disease should be referred to HHT specialized centres to ensure a standardized and timely approach to diagnosis and management.
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Malformações Arteriovenosas
/
Telangiectasia Hemorrágica Hereditária
/
Hipertensão Pulmonar
Tipo de estudo:
Guideline
/
Prognostic_studies
Limite:
Humans
Idioma:
En
Revista:
Curr Opin Pulm Med
Ano de publicação:
2014
Tipo de documento:
Article
País de afiliação:
Estados Unidos