ABSTRACT
Werner's syndrome (WS) is a genetic disorder presenting with premature senility. In the present study, we performed minimally invasive cardiac surgery (MICS)-aortic valve replacement (AVR) on a patient with Werner's syndrome who presented with aortic stenosis. The patient, a 49-year-old Japanese man, was brought to the emergency room with dyspnea during exercise. On echocardiography, severe aortic stenosis was found and surgery was planned. He had poorly controlled diabetes mellitus and underwent MICS-AVR to avoid the risk of sternal osteomyelitis, which resulted in a good outcome. The aortic valve had sclerotic changes and a genetic disease was suspected based on the onset of aortic stenosis at a young age, characteristic appearance, and various signs of aging. Genetic testing led to the diagnosis of WS.
Subject(s)
Aortic Valve Stenosis , Heart Valve Prosthesis Implantation , Werner Syndrome , Male , Humans , Middle Aged , Aortic Valve/diagnostic imaging , Aortic Valve/surgery , Werner Syndrome/complications , Werner Syndrome/diagnosis , Werner Syndrome/genetics , Treatment Outcome , Aortic Valve Stenosis/complications , Aortic Valve Stenosis/diagnostic imaging , Aortic Valve Stenosis/surgery , Heart Valve Prosthesis Implantation/adverse effectsABSTRACT
The patient was a 31-year-old pregnant woman who gave birth to her first child by vaginal delivery 7 years ago. She was diagnosed with Marfan's syndrome based on physical findings; however, the condition was not diagnosed before the onset. The patient developed acute aortic dissection at 28 weeks of pregnancy. A cesarean section was first performed to save the patient's life; then, a total hysterectomy was performed to prevent the risk of postpartum hemorrhage. Furthermore, aortic root replacement was performed using a temporary mechanical valve. The patient and her child have survived without any complications.
