RESUMEN
Hereditary hemorrhagic telangiectasia (HHT), also called Osler-Weber-Rendu syndrome, is an autosomal dominant genetic disease that affects the vasculature of numerous organs. The prevalence of HHT is estimated to be between 1.5 and 2 persons per 10,000. While there is still much to learn about this condition, there is an increasing understanding its underlying pathophysiology, genetic basis, presentations, and management. Recognizing that the clinical manifestations of HHT can involve a number of organ systems will provide clinicians with a higher index of suspicion for the disease. This early diagnosis and genotyping can greatly reduce mortality for a patient with HHT through appropriate screening for complications. This review will focus on the gastrointestinal manifestations of HHT and how these can dictate treatment and prognosis.
Asunto(s)
Enfermedades Gastrointestinales/diagnóstico , Telangiectasia Hemorrágica Hereditaria/diagnóstico , Animales , Biopsia , Endoscopía Gastrointestinal , Enfermedades Gastrointestinales/epidemiología , Enfermedades Gastrointestinales/genética , Enfermedades Gastrointestinales/terapia , Predisposición Genética a la Enfermedad , Humanos , Técnicas de Diagnóstico Molecular , Fenotipo , Examen Físico , Valor Predictivo de las Pruebas , Prevalencia , Pronóstico , Factores de Riesgo , Telangiectasia Hemorrágica Hereditaria/epidemiología , Telangiectasia Hemorrágica Hereditaria/genética , Telangiectasia Hemorrágica Hereditaria/terapia , Tomografía Computarizada por Rayos XRESUMEN
Coronary artery aneurysms are abnormal dilations of arterial segments, in some cases associated with underlying atherosclerosis. Although affected patients can be asymptomatic, some are at risk of plaque rupture, dissection, and other complications. Investigations into the optimal management of these vascular malformations are ongoing, because no consensus exists regarding when and how best to intervene. We present the case of a 58-year-old man whose large left main coronary artery aneurysm we ligated and removed during 5-vessel coronary artery bypass grafting. This distal aneurysm was at the trifurcation level of the patient's left anterior descending and left circumflex coronary arteries. In addition, we discuss considerations about left main coronary artery aneurysms and their treatment.