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Noncompaction Cardiomyopathy with Charcot-Marie-Tooth Disease.

Eltawansy SA, Bakos A, Checton J - Case Rep Cardiol (2015)

Bottom Line: There is a high incidence of arrhythmia and embolic complications.The treatment usually consists of the medical management, defibrillator placement, and lifelong anticoagulation.Heart transplantation will be the last resort.

View Article: PubMed Central - PubMed

Affiliation: Internal Medicine Department, Monmouth Medical Center, Long Branch, NJ 07740, USA.

ABSTRACT
We report a case of a 53-year-old female presenting with a new-onset heart failure that was contributed secondary to noncompaction cardiomyopathy. The diagnosis was made by echocardiogram and confirmed by cardiac MRI. Noncompaction cardiomyopathy (also known as ventricular hypertrabeculation) is a newly discovered disease. It is considered to be congenital (genetic) cardiomyopathy. It is usually associated with genetic disorders and that could explain the genetic pathogenesis of the non-compaction cardiomyopathy. Our case had a history of Charcot-Marie-Tooth disease. There is a high incidence of arrhythmia and embolic complications. The treatment usually consists of the medical management, defibrillator placement, and lifelong anticoagulation. Heart transplantation will be the last resort.

No MeSH data available.


Related in: MedlinePlus

Echocardiogram.
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Related In: Results  -  Collection


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fig1: Echocardiogram.

Mentions: We report a case of a 53-year-old Caucasian female who started to experience shortness of breath on exertion few months before presentation to us. She was admitted to a hospital in another state as she was on a trip due to worsening shortness of breathing. She was initially diagnosed as a case of congestive heart failure and was given furosemide and she improved. According to her medical records, she had a CT scan of the chest with angiography to exclude pulmonary embolism and it was negative. She was not known for any congenital heart disease, diabetes mellitus, or hypertension. She had a history of Charcot-Marie-Tooth disease with neuropathy. Her surgical history included uterine fibroid embolization 10 years ago and history of cat scratch disease 20 years ago. She went back home and was still taking oral furosemide. She was single and was working in a software company. She denied history of smoking, alcohol, or drug dependence. Review of system was negative apart from exertional dyspnea, orthopnea, and nocturnal dyspnea. Chronic foot pain with weakness and high arched foot was secondary to the history of Charcot-Marie-Tooth disease. By examination, body mass index was 29.84 kg/m2. By auscultation, there was a holosystolic murmur grade 2/6 in the lower left sternal border. There were no rales, rhonchi, or wheezes on auscultation at the time of presentation to us. The patient was then referred by her primary physician to the cardiologist office. EKG was done and showed sinus bradycardia with heart rate of 54/minute. There were nonspecific ST-T wave changes. Patient had an echocardiogram (Figure 1) showing left ventricle moderately dilated with severely reduced systolic function.


Noncompaction Cardiomyopathy with Charcot-Marie-Tooth Disease.

Eltawansy SA, Bakos A, Checton J - Case Rep Cardiol (2015)

Echocardiogram.
© Copyright Policy - open-access
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC4477189&req=5

fig1: Echocardiogram.
Mentions: We report a case of a 53-year-old Caucasian female who started to experience shortness of breath on exertion few months before presentation to us. She was admitted to a hospital in another state as she was on a trip due to worsening shortness of breathing. She was initially diagnosed as a case of congestive heart failure and was given furosemide and she improved. According to her medical records, she had a CT scan of the chest with angiography to exclude pulmonary embolism and it was negative. She was not known for any congenital heart disease, diabetes mellitus, or hypertension. She had a history of Charcot-Marie-Tooth disease with neuropathy. Her surgical history included uterine fibroid embolization 10 years ago and history of cat scratch disease 20 years ago. She went back home and was still taking oral furosemide. She was single and was working in a software company. She denied history of smoking, alcohol, or drug dependence. Review of system was negative apart from exertional dyspnea, orthopnea, and nocturnal dyspnea. Chronic foot pain with weakness and high arched foot was secondary to the history of Charcot-Marie-Tooth disease. By examination, body mass index was 29.84 kg/m2. By auscultation, there was a holosystolic murmur grade 2/6 in the lower left sternal border. There were no rales, rhonchi, or wheezes on auscultation at the time of presentation to us. The patient was then referred by her primary physician to the cardiologist office. EKG was done and showed sinus bradycardia with heart rate of 54/minute. There were nonspecific ST-T wave changes. Patient had an echocardiogram (Figure 1) showing left ventricle moderately dilated with severely reduced systolic function.

Bottom Line: There is a high incidence of arrhythmia and embolic complications.The treatment usually consists of the medical management, defibrillator placement, and lifelong anticoagulation.Heart transplantation will be the last resort.

View Article: PubMed Central - PubMed

Affiliation: Internal Medicine Department, Monmouth Medical Center, Long Branch, NJ 07740, USA.

ABSTRACT
We report a case of a 53-year-old female presenting with a new-onset heart failure that was contributed secondary to noncompaction cardiomyopathy. The diagnosis was made by echocardiogram and confirmed by cardiac MRI. Noncompaction cardiomyopathy (also known as ventricular hypertrabeculation) is a newly discovered disease. It is considered to be congenital (genetic) cardiomyopathy. It is usually associated with genetic disorders and that could explain the genetic pathogenesis of the non-compaction cardiomyopathy. Our case had a history of Charcot-Marie-Tooth disease. There is a high incidence of arrhythmia and embolic complications. The treatment usually consists of the medical management, defibrillator placement, and lifelong anticoagulation. Heart transplantation will be the last resort.

No MeSH data available.


Related in: MedlinePlus