Biventricular noncompaction and mitral cleft/Biventrikuler "noncompaction" ve mitral kleft.
Noncompaction cardiomyopathy (NCC) is a rarely seen type of congenital cardiomyopathy. It is caused by the defective embryonic migration of myocardial fibers (1). It is frequently complicated with heart failure, arrhythmias and embolic events. In this report, we presented a case with biventricular noncompaction accompanying a cleft mitral valve.
A 20-year-old male patient was admitted to our clinic with exertional dyspnea, fatigue and palpitations for the last 5 months. Arterial blood pressure was 120/60 mmHg, temperature was 36.6[degrees]C, and heart rate was 96 bpm and regular. A 3/6 grade of systolic murmur was heard over apical region. A normal sinus rhythm, left axis deviation, and signs of left ventricular hypertrophy were seen on ECG. The transthoracic echocardiography (TTE) (Vivid 3, GE Medical Systems) revealed doublecontoured and hypertrabeculated ventricular myocardium involving both the ventricles. Deep trabeculations were present in noncompacted region. The ratio of noncompacted to compacted part was >2.0. Blood flow was detected inside deep trabeculations on color-Doppler echocardiography. Left ventricular apico-septal hypokinesia was also present. Left ventricular ejection fraction was 54%. The left atrium was larger than normal and moderate regurgitation flow through anterior mitral valve was seen. A cleft was suspected on anterior mitral leaflet in apical four-chamber and parasternal short-axis views, because the leaflet was seen as a two separate structure including a mitral regurgitation jet between two on color-Doppler echocardiography. Other valves were normal. For further anatomical investigation, transesophageal echocardiography (TEE) was performed (Vivid 3, GE Medical Systems). A cleft was present on anterior mitral leaflet with a moderate mitral regurgitation confirming the TTE findings (Fig. 1, Video 1, 2. See corresponding video/movie images at www.anakarder.com). No defect was seen in interatrial and interventricular septum. Deep trabeculations and blood flow in these deep trabeculations suggesting the noncompaction were observed in both ventricles (Video 3. See corresponding video/movie images at www.anakarder.com).
[FIGURE 1 OMITTED]
The NCC was defined by Chin and his colleagues in 1990 (1). This genetic cardiomyopathy is caused by a pause in interaction of myocardial fibers resulting in a disordered endomyocardial morphogenesis during embryonic survival (2). Echocardiographic diagnostic criteria are: after the exclusion of structural heart abnormalities; numerous, prominent trabeculations and deep intratrabecular recesses, intraventricular blood reaching recesses with color-Doppler, presence of two layers of compacted and non-compacted walls in parasternal short-axis, and non-compacted/compacted ratio > 2.0 (3). In the present case, all of these criteria were present. In some reports, magnetic resonance imaging is recommended. We didn't perform magnetic resonance imaging due to some technical problems and the fact that all criteria were present eliminating any suspicion.
The NCC can lead to serious clinical conditions such as heart failure, malignant arrhythmia and embolism (3, 4). Right ventricular involvement has been found in <50% of the cases, but the left ventricular involvement rate is 89%. It may also be biventricular (5).
Pediatric incidence of mitral cleft, a rarely seen valve anomaly, is 1:1340. Frequently the anterior leaflet of the mitral valve is involved. It is caused by incomplete expression of endocardial cushion (6). In our case, mitral cleft was also present. There are no established echocardiographic criteria for mitral cleft. In our case, the anterior mitral leaflet was constituted by two separate parts, resembling tricuspid mitral valve. Presence of moderate mitral regurgitation between these parts prompted us to define it as mitral cleft. The mitral cleft is frequently associated with other endocardial cushion defects, such as ostium primum atrial septal defect, atrioventricular septal defect (7). Until present, only one case with biventricular noncompaction and cleft mitral valve has been reported. There were also complete atrioventricular block and atrial septal aneurysm previously in that case, which was reported by Dagdeviren et al. (8). Besides, there is one other case of NCC, which was reported to be accompanying another valvular abnormality, Ebstein anomaly (9).
In a patient with cardiomyopathy with valvular abnormalities, the NCC should be kept in mind. The relation between NCC and valvular abnormalities needs to be delineated in further prospective studies.
Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
ZI performed echocardiographic studies. ZI, SC, OY, MU analyzed and interpreted the clinical data, and ZI was a major contributor to writing the manuscript. All authors read and approved the final version of the manuscript.
Zafer Isilak, Serkan Cay, Omer Yiginer, Mehmet Uzun
Department of Cardiology, Gulhane Military Medical Academy,
Haydarpasa Training Hospital, Istanbul-Turkey
Video 1. Transesophageal echocardiography showing anterior mitral leaflet cleft
Video 2. Color-Doppler echocardiography showing mitral regurgitation
Video 3. Trabeculations in both ventricles
(1.) Chin TK, Perloff JK, Williams RG, Jue K, Mohrmann R. Isolated noncompaction of left ventricular myocardium: A study of eight cases. Circulation 1990; 82: 507-13. [CrossRef]
(2.) Sedmera D, McQuinn T. Embryogenesis of the heart muscle. Heart Fail Clin 2008; 4: 235-45. [CrossRef]
(3.) Suvarna JC, Deshmukh CT, Hajela SA. Left ventricular noncompaction: A cardiomyopathy often mistaken. Indian J Med Sci 2009; 63: 303-7. [CrossRef]
(4.) Yuce M, Akkoyun C, Oylumlu M, Davutoglu V Cakici M, Sari I, et al. Spongy myocardium-observation of 23 cases. Anadolu Kardiyol Derg 2010; 10: 550-2.
(5.) Minardi G, Manzara C, Pulignano G, Pino GP, Pavaci H, Sordi M. Adult bi-ventricular noncompaction cardiomyopathy. Anadolu Kardiyol Derg 2010; 10: 188-90. [CrossRef]
(6.) Minardi G, Leonetti S, Bernardi L, Pulignano G, Pino PG, Boccardi L, et al. An isolated anterior mitral leaflet cleft: a case report. Cardiovasc Ultrasound 2010; 8: 26. [CrossRef]
(7.) Fraisse A, Massih TA, Kreitmann B, Metras D, Vouhe P Sidi D, et al. Characteristics and management of cleft mitral valve. J Am Coll Cardiol 2003; 42: 1988-93. [CrossRef]
(8.) Dagdeviren B, Eren M, Oguz E. Noncompaction of ventricular myocardium, complete atrioventricular block and minor congenital heart abnormalities: case report of an unusual coexistence. Acta Cardiol 2002; 57: 221-4. [CrossRef]
(9.) Baysan O, Yokusoglu M, Bugan B, Demirkol S. E-page original image. A case of Ebstein anomaly and biventricular noncompaction. Anadolu Kardiyol Derg 2010; 10: E26.
Address for Correspondence/Yazisma Adresi: Dr. Zafer Isilak
Gulhane Askeri Tip Akademisi Haydarpasa Egitim Hastanesi,
Kardiyoloji Klinigi, Istanbul-Turkiye
Phone: +90 216 542 34 80 Fax: +90 216 348 78 80
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|Title Annotation:||Case Reports/Olgu Sunumlari|
|Author:||Isilak, Zafer; Cay, Serkan; Yiginer, Omer; Uzun, Mehmet|
|Publication:||The Anatolian Journal of Cardiology (Anadolu Kardiyoloji Dergisi)|
|Date:||Jun 1, 2012|
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