Asunto(s)
Complejo de Eisenmenger/complicaciones , Defectos del Tabique Interatrial/complicaciones , Hipertensión Pulmonar/complicaciones , Diagnóstico Tardío , Complejo de Eisenmenger/diagnóstico , Complejo de Eisenmenger/tratamiento farmacológico , Complejo de Eisenmenger/patología , Electrocardiografía , Resultado Fatal , Insuficiencia Cardíaca/complicaciones , Insuficiencia Cardíaca/diagnóstico , Defectos del Tabique Interatrial/diagnóstico , Defectos del Tabique Interatrial/tratamiento farmacológico , Defectos del Tabique Interatrial/patología , Humanos , Hipertensión Pulmonar/diagnóstico , Hipertensión Pulmonar/tratamiento farmacológico , Hipertensión Pulmonar/patología , Masculino , Persona de Mediana Edad , Vasodilatadores/uso terapéuticoRESUMEN
The term Eisenmenger Syndrome is used to refer to any systemic to pulmonary congenital communication causing pulmonary vascular obstructive disease (PVOD) severe enough as to produce bidirectional or reversed shunt. Once established, the PVOD deteriorates the quality of life and limits the survival of the patients with congenital heart disease. In the last decade, there has been a significant advance in the knowledge of the pathobiology of PVOD. Potentially important pathologic processes include: genetic abnormalities, hemodynamically-induced changes associated to shear stress, endothelial abnormalities, dysfunction of potassium channels, and extracellular matrix alterations. Other processes such as--in situ--thrombosis, angiogenesis, and cellular apoptosis may also be involved. The medical management of this condition has improved. The role of phlebotomy and long-term oxygen therapy is now better defined. Furthermore, as a result of a better knowledge in pathobiology, new and promising forms of pharmacological treatment have appeared, including prostacyclin analogs, such as Epoprostenol (Flolan) for continuous intravenous use and Trepostinil sodium (Remodulin) for continuous subcutaneous administration.
Asunto(s)
Complejo de Eisenmenger/patología , Complejo de Eisenmenger/terapia , HumanosRESUMEN
Altered endothelial anti-thrombotic properties have been observed in primary and secondary pulmonary hypertension. In the Eisenmenger syndrome, correlations of these abnormalities with the clinical status and occurrence of chronic intravascular coagulation (CIC) have not been confirmed. The purpose of this study was to investigate whether the occurrence of CIC, as determined by circulating levels of D-dimer is associated with changes in endothelial markers in Eisenmenger patients; and to identify variables that correlate with the severity of clinical presentation. Twenty-one patients were enrolled (ages, 4-52 years). Plasma levels of D-dimer, tissue plasminogen activator (t-PA), thrombomodulin, and von Willebrand factor antigen (vWF:Ag) were measured with enzyme-linked immunosorbent assay. Univariate and multivariate analyses were used to differentiate patients with stable (group 1, N=12) from those with unstable disease (group 2, N=9). Increased t-PA (p<0.0001) and vWF:Ag (p=0.001) and decreased thrombomodulin (p<0.0001) were associated with increased D-dimer levels (p=0.0201) in patients. Group 2 had a higher prevalence of affected women (p=0.0242), lower arterial oxygen saturation, and higher t-PA levels compared with group 1 (p<0.0001, discriminant analysis). t-PA and vWF:Ag correlated positively in group 2 (r=0.71, p=0.0309), but not in group 1 (r=0.25, p=NS). Two patients in group 2 but none in group 1 had episodes of pulmonary arterial thrombosis. Endothelial dysfunction is associated with evidence of CIC and correlates to some extent with the severity of symptoms in these patients.