Marfan's syndrome: pre-pubertal aortic rupture with left coronary artery aneurysms and fistulas / Síndrome de Marfan: ruptura aórtica prepubertal con fístulas y aneurismas de la arteria coronaria izquierda
West Indian med. j
;
61(9): 937-940, Dec. 2012. ilus
Article
in English
| LILACS
| ID: lil-694371
ABSTRACT
Aortic dissection and rupture occur in 20-40% of patients with Marfan's syndrome. This occurs predominantly in the third and fourth decade of life, contributing to the increased morbidity and mortality of this specific group of patients. This is the first known documented case report of pre-pubertal left coronary sinus rupture with left coronary artery aneurysms with fistulous communication to both the superior vena cava and right superior pulmonary vein, presenting with a continuous murmur.
RESUMEN
La disección y ruptura aórticas ocurren en 20-40% de los pacientes con el síndrome de Marfan. Esto ocurre predominantemente en la tercera y cuarta décadas de la vida, contribuyendo al aumento de la morbilidad y la mortalidad de este grupo específico de pacientes. Éste es el primer reporte de un caso documentado conocido de ruptura prepubertal del seno coronario izquierdo con aneurisma de la arteria coronaria izquierda, y comunicación fistulosa tanto con la vena cava superior como con la vena pulmonar superior derecha, acompañada de un soplo continuo.
Full text:
Available
Index:
LILACS (Americas)
Main subject:
Aortic Rupture
/
Pregnancy Complications, Cardiovascular
/
Pulmonary Veins
/
Sinus of Valsalva
/
Vena Cava, Superior
/
Coronary Aneurysm
/
Coronary Artery Disease
/
Arteriovenous Fistula
/
Aneurysm
/
Marfan Syndrome
Type of study:
Observational study
/
Prognostic study
Limits:
Adolescent
/
Child
/
Female
/
Humans
/
Pregnancy
Country/Region as subject:
English Caribbean
/
Jamaica
Language:
English
Journal:
West Indian med. j
Journal subject:
Medicine
Year:
2012
Type:
Article
Affiliation country:
Jamaica
Institution/Affiliation country:
Andrews Memorial Hospital/JM
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