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Research Letters |

Noncompaction Cardiomyopathy Manifesting as Retinal Artery Occlusion FREE

Tan Jin-Poi, MBBChBAO; Ismail Shatriah, MD; Seng Loong Ng, MD; Yusof Zurkurnai, MD; Rohaizan Yunus, MD
[+] Author Affiliations

Author Affiliations: Departments of Ophthalmology (Drs Jin-Poi and Shatriah), Medicine (Drs Ng and Zurkurnai), and Radiology (Dr Yunus), School of Medical Sciences, Universiti Sains Malaysia, Kubang Kerian, Malaysia.


JAMA Ophthalmol. 2013;131(2):263-265. doi:10.1001/jamaophthalmol.2013.587.
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Published online

Noncompaction cardiomyopathy is a rare cardiomyopathy that affects both children and adults.1 It commonly manifests with heart failure, systemic embolism, and arrhythmia.1,2 We describe an adult patient with bilateral retinal embolism as the manifesting sign of noncompaction cardiomyopathy.

A 62-year-old nonsmoking man had bilateral sudden painless loss of vision 13 hours prior to his visit. He had no ophthalmic history and reported no significant medical illness. Visual acuity was hand motions OD and counting fingers OS. A relative afferent pupillary defect was present in the right eye. Dilated funduscopic examination showed the presence of a cherry-red spot with generalized pale edematous retina in the right eye (Figure 1A) and evidence of retinal edema involving the upper hemisphere in the left eye (Figure 1B). Visual field testing revealed a total field loss of the right eye and hemi-inferior field defect in the left eye (Figure 1C and D). Ocular massage, breathing in a mixture of carbon dioxide and oxygen, and an intravenous bolus injection of acetazolamide, 500 mg, were instituted in the emergency department. He was admitted on that day for systemic workup to assess the primary cause.

Place holder to copy figure label and caption
Graphic Jump Location

Figure 1. Fundus photographs and visual field testing results. A, Fundus photograph shows central retinal artery occlusion in the right eye. B, An embolus visible in the arteriole on the optic disc (arrow) causes hemicentral retinal artery occlusion in the left eye. Visual field testing shows field loss in the right (C) and left (D) eyes.

His blood pressure was 131/70 mm Hg and his heart rate was 78 beats/min. Systemic examination findings were significant for a pansystolic murmur at the mitral region that radiated to the axilla, displacement of the apex beat to the sixth intercostal space of the anterior axillary line, and presence of thrills.

The results of full blood cell count, urea, electrolytes, blood glucose level, serum fasting cholesterol level, clotting factors, thrombophilia screening, and cardiac enzyme levels were within the normal range. Electrocardiography showed high uptake in the ST segment in V4.

Transthoracic echocardiography revealed a dilated left ventricle of 60 mm with an ejection fraction of 60%. There was a concentric hypertrophy with prominent trabeculae seen on the left ventricular wall. The trabeculation thickness was more than twice the thickness of the underlying ventricular wall (Figure 2A). Color flow imaging demonstrated blood flow within the deep recess between the trabeculations (Figure 2B). Severe mitral regurgitation due to grade 1 anterior mitral valve prolapse was also present. Findings on the carotid Doppler study were normal, and no signs of carotid stenosis or plaques were observed.

Place holder to copy figure label and caption
Graphic Jump Location

Figure 2. Echocardiogram and color Doppler imaging. A, Echocardiogram shows prominent trabeculations in the left ventricle. Red arrow indicates intertrabecular recesses; yellow arrow, trabeculae. B, Color Doppler image shows the presence of intraventricular cavity blood flow up to the depth of the intertrabecular recesses. Green arrows indicate blood flow in the intertrabecular recesses; red arrow, noncompacted zone; and purple arrow, compacted zone.

A provisional diagnosis of noncompaction cardiomyopathy was made. Cardiac magnetic resonance imaging was recommended to further evaluate the cardiomyopathy, but the patient declined. Long-term oral warfarin sodium treatment was commenced to reduce the risk of systemic embolization. At 1 month after the attack, his visual acuity remained similar in the right eye and improved to 20/200 OS.

The embryonic arrest of compaction of myocardial fibers seen in noncompaction cardiomyopathy is most frequently observed in the left ventricle.3 The cardiomyopathy is diagnosed by echocardiography or magnetic resonance imaging.

Echocardiography shows trabeculations and deep intertrabecular recesses. Blood flow can be observed within the deep intertrabecular recesses, and the flow is in continuity with the left ventricular cavity. Noncompaction cardiomyopathy is diagnosed echocardiographically when the ratio of trabeculations to the thickness of the underlying ventricular wall is more than 2.

Magnetic resonance imaging shows a 2-layered wall structure comprising a thin compacted epicardium and a thick noncompacted myocardium. Our patient's echocardiograms are consistent with the diagnosis of noncompaction cardiomyopathy.

Strokes have been reported as a systemic thromboembolism that occurs in patients with noncompaction cardiomyopathy.4,5 The bilateral retinal artery occlusion seen in our patient is likely of a thromboembolic nature. We postulated that the microembolus observed in the left retinal arteriole originated from the heart. The noncompaction cardiomyopathy with relative blood stasis in the intertrabecular recess explains the most probable cause of this phenomenon.4 Thus, it is extremely important to highlight this rare cause of retinal artery occlusion that has resulted in devastating vision loss.

Correspondence: Dr Shatriah, Department of Ophthalmology, School of Medical Sciences, Universiti Sains Malaysia, Kubang Kerian 16150, Malaysia (shatriah@kck.usm.my).

Conflict of Interest Disclosures: None reported.

Pignatelli RH, McMahon CJ, Dreyer WJ,  et al.  Clinical characterization of left ventricular noncompaction in children: a relatively common form of cardiomyopathy.  Circulation. 2003;108(21):2672-2678
PubMed   |  Link to Article
Ergul Y, Nisli K, Demirel A,  et al.  Left ventricular non-compaction in children and adolescents: clinical features, treatment and follow-up.  Cardiol J. 2011;18(2):176-184
PubMed
Espinola-Zavaleta N, Soto ME, Castellanos LM, Játiva-Chávez S, Keirns C. Non-compacted cardiomyopathy: clinical-echocardiographic study.  Cardiovasc Ultrasound. 2006;4:35
PubMed   |  Link to Article
Mageshkumar S, Patil D, Samuel D, Muthukumar D. Unusual case of isolated biventricular non-compaction presenting with stroke.  J Postgrad Med. 2011;57(3):211-213
PubMed   |  Link to Article
Jiménez-Caballero PE. Juvenile stroke as the presenting symptom of a non-compaction cardiomyopathy [in Spanish].  Rev Neurol. 2010;51(8):509-510
PubMed

Figures

Place holder to copy figure label and caption
Graphic Jump Location

Figure 1. Fundus photographs and visual field testing results. A, Fundus photograph shows central retinal artery occlusion in the right eye. B, An embolus visible in the arteriole on the optic disc (arrow) causes hemicentral retinal artery occlusion in the left eye. Visual field testing shows field loss in the right (C) and left (D) eyes.

Place holder to copy figure label and caption
Graphic Jump Location

Figure 2. Echocardiogram and color Doppler imaging. A, Echocardiogram shows prominent trabeculations in the left ventricle. Red arrow indicates intertrabecular recesses; yellow arrow, trabeculae. B, Color Doppler image shows the presence of intraventricular cavity blood flow up to the depth of the intertrabecular recesses. Green arrows indicate blood flow in the intertrabecular recesses; red arrow, noncompacted zone; and purple arrow, compacted zone.

Tables

References

Pignatelli RH, McMahon CJ, Dreyer WJ,  et al.  Clinical characterization of left ventricular noncompaction in children: a relatively common form of cardiomyopathy.  Circulation. 2003;108(21):2672-2678
PubMed   |  Link to Article
Ergul Y, Nisli K, Demirel A,  et al.  Left ventricular non-compaction in children and adolescents: clinical features, treatment and follow-up.  Cardiol J. 2011;18(2):176-184
PubMed
Espinola-Zavaleta N, Soto ME, Castellanos LM, Játiva-Chávez S, Keirns C. Non-compacted cardiomyopathy: clinical-echocardiographic study.  Cardiovasc Ultrasound. 2006;4:35
PubMed   |  Link to Article
Mageshkumar S, Patil D, Samuel D, Muthukumar D. Unusual case of isolated biventricular non-compaction presenting with stroke.  J Postgrad Med. 2011;57(3):211-213
PubMed   |  Link to Article
Jiménez-Caballero PE. Juvenile stroke as the presenting symptom of a non-compaction cardiomyopathy [in Spanish].  Rev Neurol. 2010;51(8):509-510
PubMed

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