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Isolated ventricular noncompaction syndrome in a nigerian male: case report and review of the literature.

Bhattacharya IS, Dweck M, Gardner A, Jones M, Francis M - Cardiol Res Pract (2010)

Bottom Line: Isolated ventricular non-compaction cardiomyopathy (IVNC) is a rare, morphologically distinct primary genetic cardiomyopathy, which is now gaining prominence as an important differential diagnosis in patients presenting with cardiac failure.We describe a case report of a Nigerian male with facial dysmorphism presenting with cardiac failure.This is followed by a review of the literature with focus on the diagnosis of this condition, which may be difficult especially in non-Caucasian populations.

View Article: PubMed Central - PubMed

Affiliation: Cardiology Unit, Victoria Hospital, Kirkcaldy, Fife KY2 5AH, Scotland.

ABSTRACT
Isolated ventricular non-compaction cardiomyopathy (IVNC) is a rare, morphologically distinct primary genetic cardiomyopathy, which is now gaining prominence as an important differential diagnosis in patients presenting with cardiac failure. We describe a case report of a Nigerian male with facial dysmorphism presenting with cardiac failure. This is followed by a review of the literature with focus on the diagnosis of this condition, which may be difficult especially in non-Caucasian populations.

No MeSH data available.


Related in: MedlinePlus

CMR measurements of the compacted (8 mm) and non-compacted (21 mm) layers of the myocardium.
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fig3: CMR measurements of the compacted (8 mm) and non-compacted (21 mm) layers of the myocardium.

Mentions: He had a normal full blood count, urea, creatinine and electrolytes, liver function and thyroid function tests. He was HIV negative and his antinuclear factor was negative. His electrocardiogram showed a sinus tachycardia with voltage criteria for left ventricular hypertrophy. Troponin and CK levels were normal. His chest X-ray showed cardiomegaly with upper-lobe diversion. Echocardiographic images were suboptimal in quality but showed a dilated left ventricle, and severe global impairment of LV systolic function. Cardiac MRI (CMR) revealed similar findings but in addition demonstrated deep ventricular recesses and hypertrabeculation of the lateral wall extending down towards the apex (Figure 2). Unusually the true apex was not involved. The ratio of the compacted to noncompacted myocardium was 2.6 in diastole (Figure 3). Ejection fraction was 15% and there was no late gadolinium enhancement.


Isolated ventricular noncompaction syndrome in a nigerian male: case report and review of the literature.

Bhattacharya IS, Dweck M, Gardner A, Jones M, Francis M - Cardiol Res Pract (2010)

CMR measurements of the compacted (8 mm) and non-compacted (21 mm) layers of the myocardium.
© Copyright Policy - open-access
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC2913813&req=5

fig3: CMR measurements of the compacted (8 mm) and non-compacted (21 mm) layers of the myocardium.
Mentions: He had a normal full blood count, urea, creatinine and electrolytes, liver function and thyroid function tests. He was HIV negative and his antinuclear factor was negative. His electrocardiogram showed a sinus tachycardia with voltage criteria for left ventricular hypertrophy. Troponin and CK levels were normal. His chest X-ray showed cardiomegaly with upper-lobe diversion. Echocardiographic images were suboptimal in quality but showed a dilated left ventricle, and severe global impairment of LV systolic function. Cardiac MRI (CMR) revealed similar findings but in addition demonstrated deep ventricular recesses and hypertrabeculation of the lateral wall extending down towards the apex (Figure 2). Unusually the true apex was not involved. The ratio of the compacted to noncompacted myocardium was 2.6 in diastole (Figure 3). Ejection fraction was 15% and there was no late gadolinium enhancement.

Bottom Line: Isolated ventricular non-compaction cardiomyopathy (IVNC) is a rare, morphologically distinct primary genetic cardiomyopathy, which is now gaining prominence as an important differential diagnosis in patients presenting with cardiac failure.We describe a case report of a Nigerian male with facial dysmorphism presenting with cardiac failure.This is followed by a review of the literature with focus on the diagnosis of this condition, which may be difficult especially in non-Caucasian populations.

View Article: PubMed Central - PubMed

Affiliation: Cardiology Unit, Victoria Hospital, Kirkcaldy, Fife KY2 5AH, Scotland.

ABSTRACT
Isolated ventricular non-compaction cardiomyopathy (IVNC) is a rare, morphologically distinct primary genetic cardiomyopathy, which is now gaining prominence as an important differential diagnosis in patients presenting with cardiac failure. We describe a case report of a Nigerian male with facial dysmorphism presenting with cardiac failure. This is followed by a review of the literature with focus on the diagnosis of this condition, which may be difficult especially in non-Caucasian populations.

No MeSH data available.


Related in: MedlinePlus