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Marfan syndrome with acute abdomen: a case report.

Zencirci B - Int J Gen Med (2010)

Bottom Line: In addition, according to the echocardiographic examination, Ebstein's anomaly, mitral valve prolapse, pulmonary hypertension, and inferior deficiency of mitral, aorta, and tricuspid valves were present.The patient was planned to be operated on with the prediagnosis of acute abdomen.Taking into consideration the pathologies that may accompany MFS and the probable future complications, the patients must be closely monitored during anesthesia applications and required measures should be taken beforehand.

View Article: PubMed Central - PubMed

Affiliation: Kahramanmaras Sutcu Imam University Medical Faculty, Department of Anesthesiology and Reanimation Kahramanmaras, Turkey.

ABSTRACT

Introduction: Marfan syndrome is an autosomal dominant connective tissue disorder characterized by a combination of clinical manifestations in different organ systems. Patients with Marfan syndrome (MFS) whose lifetimes are extended may be encountered as acute abdomen (appendicitis) cases apart from the obligatory reasons and emergencies arising naturally out of their disease, as in the case reported.

Case presentation: In a 28-year-old Turkish male, arachnodactyly, pectus excavatum, kyphoscoliosis and, according to pulmonary roentgenogram, a density increase in the left apical field were detected. In addition, according to the echocardiographic examination, Ebstein's anomaly, mitral valve prolapse, pulmonary hypertension, and inferior deficiency of mitral, aorta, and tricuspid valves were present. The patient was planned to be operated on with the prediagnosis of acute abdomen.

Conclusion: Taking into consideration the pathologies that may accompany MFS and the probable future complications, the patients must be closely monitored during anesthesia applications and required measures should be taken beforehand.

No MeSH data available.


Related in: MedlinePlus

Ebstein’s anomaly; a portion of the right ventricle is atrialized and as a result, the functional right ventricle is small. Reproduced with permission from Brickner ME, Hillis LD, Lange RA. Congenital heart disease in adults: second of two parts. N Engl J Med. 2000;342:334–342.10 Copyright © 2000 Massachusetts Medical Society. All rights reserved.
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f5-ijgm-3-359: Ebstein’s anomaly; a portion of the right ventricle is atrialized and as a result, the functional right ventricle is small. Reproduced with permission from Brickner ME, Hillis LD, Lange RA. Congenital heart disease in adults: second of two parts. N Engl J Med. 2000;342:334–342.10 Copyright © 2000 Massachusetts Medical Society. All rights reserved.

Mentions: Ebstein’s anomaly includes an abnormal tricuspid valve and “atrialized” right ventricle (Figure 5), that is, the septal leaflets and often the posterior leaflets of the tricuspid valve are displaced into the right ventricle. But the anterior leaflet is usually malformed, excessively large, and abnormally attached or adherent to the right ventricular free wall. Thus, a portion of the right ventricle is “atrialized” in that it is located on the atrial side of the tricuspid valve, and the remaining functional right ventricle is small. Eighty percent of patients with Ebstein’s anomaly have an interatrial communication (atrial septal defect or patent foramen ovale) through which right-to-left shunting of blood may occur.9,10


Marfan syndrome with acute abdomen: a case report.

Zencirci B - Int J Gen Med (2010)

Ebstein’s anomaly; a portion of the right ventricle is atrialized and as a result, the functional right ventricle is small. Reproduced with permission from Brickner ME, Hillis LD, Lange RA. Congenital heart disease in adults: second of two parts. N Engl J Med. 2000;342:334–342.10 Copyright © 2000 Massachusetts Medical Society. All rights reserved.
© Copyright Policy
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC2990397&req=5

f5-ijgm-3-359: Ebstein’s anomaly; a portion of the right ventricle is atrialized and as a result, the functional right ventricle is small. Reproduced with permission from Brickner ME, Hillis LD, Lange RA. Congenital heart disease in adults: second of two parts. N Engl J Med. 2000;342:334–342.10 Copyright © 2000 Massachusetts Medical Society. All rights reserved.
Mentions: Ebstein’s anomaly includes an abnormal tricuspid valve and “atrialized” right ventricle (Figure 5), that is, the septal leaflets and often the posterior leaflets of the tricuspid valve are displaced into the right ventricle. But the anterior leaflet is usually malformed, excessively large, and abnormally attached or adherent to the right ventricular free wall. Thus, a portion of the right ventricle is “atrialized” in that it is located on the atrial side of the tricuspid valve, and the remaining functional right ventricle is small. Eighty percent of patients with Ebstein’s anomaly have an interatrial communication (atrial septal defect or patent foramen ovale) through which right-to-left shunting of blood may occur.9,10

Bottom Line: In addition, according to the echocardiographic examination, Ebstein's anomaly, mitral valve prolapse, pulmonary hypertension, and inferior deficiency of mitral, aorta, and tricuspid valves were present.The patient was planned to be operated on with the prediagnosis of acute abdomen.Taking into consideration the pathologies that may accompany MFS and the probable future complications, the patients must be closely monitored during anesthesia applications and required measures should be taken beforehand.

View Article: PubMed Central - PubMed

Affiliation: Kahramanmaras Sutcu Imam University Medical Faculty, Department of Anesthesiology and Reanimation Kahramanmaras, Turkey.

ABSTRACT

Introduction: Marfan syndrome is an autosomal dominant connective tissue disorder characterized by a combination of clinical manifestations in different organ systems. Patients with Marfan syndrome (MFS) whose lifetimes are extended may be encountered as acute abdomen (appendicitis) cases apart from the obligatory reasons and emergencies arising naturally out of their disease, as in the case reported.

Case presentation: In a 28-year-old Turkish male, arachnodactyly, pectus excavatum, kyphoscoliosis and, according to pulmonary roentgenogram, a density increase in the left apical field were detected. In addition, according to the echocardiographic examination, Ebstein's anomaly, mitral valve prolapse, pulmonary hypertension, and inferior deficiency of mitral, aorta, and tricuspid valves were present. The patient was planned to be operated on with the prediagnosis of acute abdomen.

Conclusion: Taking into consideration the pathologies that may accompany MFS and the probable future complications, the patients must be closely monitored during anesthesia applications and required measures should be taken beforehand.

No MeSH data available.


Related in: MedlinePlus