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Redux valvular surgery with coronary artery bypass graft in familial hypercholesterolemia.

Jalel Z, Sobhi M, Skander BO, Adel K - Ann Pediatr Cardiol (2014)

Bottom Line: An intensive cholesterol-lowering therapy and LDL aphaeresis in association with surgery may be useful.She was readmitted for acute coronary syndrome.Three coronary artery bypass grafting was performed with saphenous veins with positive results.

View Article: PubMed Central - PubMed

Affiliation: Department of Cardiovascular and Thoracic, Rabta Hospital, Medical University of Tunis, Tunis, Tunisia.

ABSTRACT
Familial hypercholesterolemia (FH) is a dominantly inherited disorder caused by mutation at the locus for the low-density lipoprotein (LDL) receptor and is frequently associated with premature coronary artery disease and aortic valve involvement. The surgical treatment of these complications is accompanied by a high degree of risk, even in skillful hands. An intensive cholesterol-lowering therapy and LDL aphaeresis in association with surgery may be useful. The case of a 12-year-old girl, with a medical history of familial hypercholesterolemia is reported here, operated two years previously for valvular aortic stenosis; Ross intervention was done. She was readmitted for acute coronary syndrome. Three coronary artery bypass grafting was performed with saphenous veins with positive results.

No MeSH data available.


Related in: MedlinePlus

Stenosis of both subclavian arteries
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Figure 4: Stenosis of both subclavian arteries

Mentions: We report the case of a 12-year-old girl; she had a medical history of FH like her brother with tendon xanthomas of the upper and lower extremities [Figure 1]. She was operated two years previously for aortic stenosis associated with mitral valve regurgitation grade 3 without coronary artery disease [Figure 2]. She had a Ross intervention with mitral annuloplasty with positive results. Currently, she was admitted in emergency for acute coronary syndrome with negative ST and positive troponin I with initially stable hemodynamic constants. A transthoracic echocardiography was done which showed a dilated left ventricle with an ejection fraction of 61%, mitral regurgitation grade 2, absence of aortic insufficiency, and a good functioning of a Contegra tube in pulmonary position. The patient was directly sent to the catheterization laboratory where angiography showed stenosis of the distal left main trunk, subocclusive stenosis of the proximal circumflex coronary, a stenosis of the second and distal parts of the interventricular coronary, and proximal subocclusive stenosis of the right coronary artery [Figure 3]; it also showed stenosis of both left and right subclavian arteries [Figure 4]. She was hospitalized in the surgical intensive care unit and antianginal treatment was initiated. Her hospital course was complicated by the deterioration of hemodynamic status with several outbreaks of left heart failure related to mitral regurgitation grade 3-4 with a low ejection fraction of 35%, requiring the introduction of vasoactive drugs in gradually high doses.


Redux valvular surgery with coronary artery bypass graft in familial hypercholesterolemia.

Jalel Z, Sobhi M, Skander BO, Adel K - Ann Pediatr Cardiol (2014)

Stenosis of both subclavian arteries
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3959067&req=5

Figure 4: Stenosis of both subclavian arteries
Mentions: We report the case of a 12-year-old girl; she had a medical history of FH like her brother with tendon xanthomas of the upper and lower extremities [Figure 1]. She was operated two years previously for aortic stenosis associated with mitral valve regurgitation grade 3 without coronary artery disease [Figure 2]. She had a Ross intervention with mitral annuloplasty with positive results. Currently, she was admitted in emergency for acute coronary syndrome with negative ST and positive troponin I with initially stable hemodynamic constants. A transthoracic echocardiography was done which showed a dilated left ventricle with an ejection fraction of 61%, mitral regurgitation grade 2, absence of aortic insufficiency, and a good functioning of a Contegra tube in pulmonary position. The patient was directly sent to the catheterization laboratory where angiography showed stenosis of the distal left main trunk, subocclusive stenosis of the proximal circumflex coronary, a stenosis of the second and distal parts of the interventricular coronary, and proximal subocclusive stenosis of the right coronary artery [Figure 3]; it also showed stenosis of both left and right subclavian arteries [Figure 4]. She was hospitalized in the surgical intensive care unit and antianginal treatment was initiated. Her hospital course was complicated by the deterioration of hemodynamic status with several outbreaks of left heart failure related to mitral regurgitation grade 3-4 with a low ejection fraction of 35%, requiring the introduction of vasoactive drugs in gradually high doses.

Bottom Line: An intensive cholesterol-lowering therapy and LDL aphaeresis in association with surgery may be useful.She was readmitted for acute coronary syndrome.Three coronary artery bypass grafting was performed with saphenous veins with positive results.

View Article: PubMed Central - PubMed

Affiliation: Department of Cardiovascular and Thoracic, Rabta Hospital, Medical University of Tunis, Tunis, Tunisia.

ABSTRACT
Familial hypercholesterolemia (FH) is a dominantly inherited disorder caused by mutation at the locus for the low-density lipoprotein (LDL) receptor and is frequently associated with premature coronary artery disease and aortic valve involvement. The surgical treatment of these complications is accompanied by a high degree of risk, even in skillful hands. An intensive cholesterol-lowering therapy and LDL aphaeresis in association with surgery may be useful. The case of a 12-year-old girl, with a medical history of familial hypercholesterolemia is reported here, operated two years previously for valvular aortic stenosis; Ross intervention was done. She was readmitted for acute coronary syndrome. Three coronary artery bypass grafting was performed with saphenous veins with positive results.

No MeSH data available.


Related in: MedlinePlus