Biventricular Noncompaction Cardiomyopathy in an Adult with Unique Facial Dysmorphisms: Case Report and Brief Review
Left ventricular noncompaction (LVNC) is a rare cardiomyopathy that is believed it to arise from an arrest in embryonic endomyocardial development. More recent studies suggest that it can be acquired later on in life sporadically. It may be accompanied by life-threatening complications, which are mo...
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| Format: | Article |
| Language: | English |
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Wiley
2015-01-01
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| Series: | Case Reports in Cardiology |
| Online Access: | http://dx.doi.org/10.1155/2015/831341 |
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| author | Gaurav Rao James Tauras |
| author_facet | Gaurav Rao James Tauras |
| author_sort | Gaurav Rao |
| collection | DOAJ |
| description | Left ventricular noncompaction (LVNC) is a rare cardiomyopathy that is believed it to arise from an arrest in embryonic endomyocardial development. More recent studies suggest that it can be acquired later on in life sporadically. It may be accompanied by life-threatening complications, which are most commonly heart failure, arrhythmias, and thromboembolic events. We report a case of biventricular noncompaction cardiomyopathy in a 36-year-old man presenting for the first time with clinical heart failure as well as atrial arrhythmia. Transthoracic echocardiography (TTE) revealed LVNC with depressed ejection fraction (EF). Cardiac magnetic resonance imaging (MRI) further revealed a left atrial appendage thrombus as well as right ventricular noncompaction involvement. His physical exam was unique for a characteristic facial dysmorphisms pattern and developmental delays reminiscent of the earliest descriptions of LVNC in the pediatric population and it was rarely described in adult patients. This unique presentation underscores the importance of a better understanding of the genetics and natural course of LVNC. This will help us to elucidate the uncertainty surrounding its clinical management, discussed in a brief review of the literature following the case. |
| format | Article |
| id | doaj-art-fb280986593847bfad780b6c95b703f6 |
| institution | Kabale University |
| issn | 2090-6404 2090-6412 |
| language | English |
| publishDate | 2015-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Cardiology |
| spelling | doaj-art-fb280986593847bfad780b6c95b703f62025-02-03T05:44:53ZengWileyCase Reports in Cardiology2090-64042090-64122015-01-01201510.1155/2015/831341831341Biventricular Noncompaction Cardiomyopathy in an Adult with Unique Facial Dysmorphisms: Case Report and Brief ReviewGaurav Rao0James Tauras1Division of Internal Medicine, Montefiore Medical Center, Albert Einstein College of Medicine, Bronx, NY 10467, USADivision of Cardiology, Weiler Hospital, Albert Einstein College of Medicine, Bronx, NY 10461, USALeft ventricular noncompaction (LVNC) is a rare cardiomyopathy that is believed it to arise from an arrest in embryonic endomyocardial development. More recent studies suggest that it can be acquired later on in life sporadically. It may be accompanied by life-threatening complications, which are most commonly heart failure, arrhythmias, and thromboembolic events. We report a case of biventricular noncompaction cardiomyopathy in a 36-year-old man presenting for the first time with clinical heart failure as well as atrial arrhythmia. Transthoracic echocardiography (TTE) revealed LVNC with depressed ejection fraction (EF). Cardiac magnetic resonance imaging (MRI) further revealed a left atrial appendage thrombus as well as right ventricular noncompaction involvement. His physical exam was unique for a characteristic facial dysmorphisms pattern and developmental delays reminiscent of the earliest descriptions of LVNC in the pediatric population and it was rarely described in adult patients. This unique presentation underscores the importance of a better understanding of the genetics and natural course of LVNC. This will help us to elucidate the uncertainty surrounding its clinical management, discussed in a brief review of the literature following the case.http://dx.doi.org/10.1155/2015/831341 |
| spellingShingle | Gaurav Rao James Tauras Biventricular Noncompaction Cardiomyopathy in an Adult with Unique Facial Dysmorphisms: Case Report and Brief Review Case Reports in Cardiology |
| title | Biventricular Noncompaction Cardiomyopathy in an Adult with Unique Facial Dysmorphisms: Case Report and Brief Review |
| title_full | Biventricular Noncompaction Cardiomyopathy in an Adult with Unique Facial Dysmorphisms: Case Report and Brief Review |
| title_fullStr | Biventricular Noncompaction Cardiomyopathy in an Adult with Unique Facial Dysmorphisms: Case Report and Brief Review |
| title_full_unstemmed | Biventricular Noncompaction Cardiomyopathy in an Adult with Unique Facial Dysmorphisms: Case Report and Brief Review |
| title_short | Biventricular Noncompaction Cardiomyopathy in an Adult with Unique Facial Dysmorphisms: Case Report and Brief Review |
| title_sort | biventricular noncompaction cardiomyopathy in an adult with unique facial dysmorphisms case report and brief review |
| url | http://dx.doi.org/10.1155/2015/831341 |
| work_keys_str_mv | AT gauravrao biventricularnoncompactioncardiomyopathyinanadultwithuniquefacialdysmorphismscasereportandbriefreview AT jamestauras biventricularnoncompactioncardiomyopathyinanadultwithuniquefacialdysmorphismscasereportandbriefreview |