ABSTRACT
El síndrome de tortuosidad vascular es un infrecuente trastorno de origen genético que cursa con tortuosidad y estenosis de las circulaciones pulmonar, sistémica y/o coronaria. Como consecuencia del tratamiento de las estenosis pulmonares puede producirse un cuadro de edema pulmonar conocido como síndrome de reperfusión pulmonar. Presentamos el caso de un adolescente con un síndrome de tortuosidad vascular que presentó un síndrome de reperfusión pulmonar tras la colocación de varios stents en la arteria pulmonar izquierda. Tras el procedimiento, el paciente desarrolló de forma inmediata un cuadro de edema agudo de pulmón, con deterioro severo de su estado clínico, qué llegó a precisar asistencia con oxigenación por membrana extracorpórea para su recuperación
Vascular tortuosity syndrome is a rare genetic disorder that causes tortuosity and stenosis of the pulmonary, systemic and / or coronary circulations. As a result of treatment of pulmonary stenosis, symptoms of pulmonary edema, known as lung reperfusion syndrome, may occur. The case is presented of an adolescent patient with vascular tortuosity syndrome who presented with a pulmonary reperfusion syndrome after multiple stent implants in the left pulmonary artery. After the procedure, the patient immediately developed an acute pulmonary edema with severe clinical deterioration, which required assistance with extracorporeal membrane oxygenation for recovery
Subject(s)
Humans , Male , Child , Reperfusion Injury/chemically induced , Reperfusion Injury/diagnosis , Reperfusion Injury/pathology , Coronary Circulation/genetics , Pulmonary Edema/diagnosis , Reperfusion Injury/complications , Reperfusion Injury/genetics , Coronary Circulation/physiology , Pulmonary Edema/complicationsABSTRACT
Vascular tortuosity syndrome is a rare genetic disorder that causes tortuosity and stenosis of the pulmonary, systemic and / or coronary circulations. As a result of treatment of pulmonary stenosis, symptoms of pulmonary edema, known as lung reperfusion syndrome, may occur. The case is presented of an adolescent patient with vascular tortuosity syndrome who presented with a pulmonary reperfusion syndrome after multiple stent implants in the left pulmonary artery. After the procedure, the patient immediately developed an acute pulmonary edema with severe clinical deterioration, which required assistance with extracorporeal membrane oxygenation for recovery.
