European Journal of Heart Failure 2006 8(7):760-763; doi:10.1016/j.ejheart.2006.01.017
© 2006 European Society of Cardiology
Non-compaction cardiomyopathy associated with myocardial bridging: A frequently overlooked or misdiagnosed cardiomyopathy
Dursun Aras*,
Omac Tufekcioglu,
Serkan Topaloglu,
Ozcan Ozeke,
Kumral Ergun,
Ali Yildiz,
Bulent Deveci and
Sule Korkmaz
Turkiye Yuksek Ihtisas Hospital, Department of Cardiology, Ankara, Turkey
* Corresponding author. Ballibaba Sokak, No: 52/3, Kucukesat, 06700, Ankara, Turkey. Tel.: +90 312 4302825; fax: +90 312 4175315. E-mail address: sevgidursun06{at}yahoo.com (D.Aras).
 |
Abstract
|
|---|
Isolated ventricular non-compaction (IVNC) is an unclassified
cardiomyopathy which occurs due to a morphogenetic abnormality
involving an arrest of compaction of the loose myocardial meshwork
during fetal ontogenesis. Despite recent advances in knowledge,
diagnosis remains problematic because of its similarity to other
diseases of the myocardium and endocardium. In this report,
we describe a case of IVNC and myocardial bridging. The patient
had been misdiagnosed with apical hypertrophic cardiomyopathy
2years earlier. The correct diagnosis was established by transthoracic
echocardiography and confirmed by cardiac catheterization and
angiocardiography.
Key Words: Non-compaction cardiomyopathy • Myocardial bridging • Apical cardiomyopathy
Received June 15, 2005; Revised October 4, 2005; Accepted January 30, 2006
|
1. Introduction
|
|---|
Isolated ventricular non-compaction (IVNC) is an unclassified
cardiomyopathy, which occurs due to a morphogenetic abnormality
involving an arrest of compaction of the loose myocardial meshwork
during fetal ontogenesis
[1,
2]. Despite recent advances in knowledge,
diagnosis of IVNC remains problematic due to its similarity
to other diseases of the myocardium and endocardium such as
dilated cardiomyopathy
[3] or apical hypertrophic cardiomyopathy
(ACM)
[4,
5]. It is thought that this cardiomyopathy type has
a higher prevalence than previously reported, possibly due to
increased detection rates following improvements in cardiac
imaging procedures and increasing awareness of this disease.
|
2. Case report
|
|---|
A 45-year-old male patient, who had received a diagnosis of
ACM at another hospital 2years earlier, was admitted to our
institution with worsening heart failure. He was a non-smoker
and had no significant history of alcohol use. There was no
family history of cardiomyopathy. He had New York Heart Association
class III heart failure symptoms. On physical examination, his
blood pressure was 110/70mmHg and heart rate was 90beats/min.
Cardiac auscultation revealed a 2/6 holosystolic murmur at the
cardiac apex. Examination of the chest detected bi-basilar rales
in both lung fields. There was bilateral pretibial oedema. Mild
cardiomegaly was observed on chest X-ray. A surface electrocardiogram
showed first degree atrioventricular block with left bundle
branch block morphology. A transthoracic echocardiogram showed
a moderately enlarged left ventricle (LV) (end-diastolic diameter
of 59mm) and severely depressed LV systolic function with an
ejection fraction of 20%. All cardiac valves appeared normal
and there were no findings of any coexisting congenital lesion.
However, prominent trabeculations with deep intertrabecular
recesses (
Figs. 1 and
2A) were identified in the apex and mid
segments of the LV inferior, posterior and lateral walls. The
ratio of the non-compacted to the compacted layer measured at
end-systole was greater than two. The colour Doppler study showed
multiple deep intertrabecular recesses filled with blood from
the ventricular cavity (
Fig. 1). Since these echocardiographic
findings were found to be consistent with previously reported
criteria of IVNC
[6], the diagnosis of ACM was changed to IVNC.
Coronary angiography, performed to rule out coronary artery
pathology, revealed a significant systolic milking effect in
the distal part of the left anterior descending coronary artery
(LAD) with an angiographically normal vessel in diastole, consistent
with myocardial bridging (
Fig. 3). LV angiography showed the
presence of extraordinary hypertrophy of trabeculae within the
LV, especially in the inferolateral wall (
Fig. 2B). Since IVNC
can be familial, the patient's first-degree relatives were screened
by echocardiography as a precaution; however, no abnormalities
were found. The patient was discharged on conventional heart
failure therapy including aspirin, ramipril, sprinolactone,
furosemide and carvedilol. Although warfarin was thought to
be indicated, it was not prescribed due to the patient's refusal.
At 6months' follow-up, the patient had an uneventful clinical
course with no changes in functional capacity.
View larger version (67K):
[in this window]
[in a new window]
[Download PowerPoint slide]
|
Fig. 1 Two-dimensional echocardiogram in parasternal short-axis view at the mid-ventricular level showing prominent, numerous trabeculations in the left ventricular inferior, posterior, and lateral wall with deep intertrabecular recesses (A). Colour Doppler echocardiogram demonstrating flow within deep intertrabecular recesses in continuity with the left ventricular cavity (B).
|
|
View larger version (84K):
[in this window]
[in a new window]
[Download PowerPoint slide]
|
Fig. 2 Apical four-chamber view of the left ventricle showing the typical prominent trabeculations (arrows) in the left ventricular apicolateral wall (A). Left ventricular angiogram in left anterior oblique projection demonstrating an abnormally prominent trabecular zone and deep intertrabecular recesses (arrows) in the inferolateral wall (B).
|
|
View larger version (81K):
[in this window]
[in a new window]
[Download PowerPoint slide]
|
Fig. 3 Coronary angiography showing myocardial bridging of the left anterior descending artery in diastole (A) and systole (B). During systole, a significant reduction in the coronary diameter (arrow in B) with a "milking effect" may be noted.
|
|
|
3. Discussion
|
|---|
IVNC, first described by Chin et al. in 1990
[1], is a rare
and unclassified congenital cardiomyopathy
[2] that is thought
to be related to arrest of myocardial development, resulting
in persistent multiple prominent ventricular trabeculations
and deep intertrabecular recesses
[1,
7]. Non-compacted myocardium
was first described in association with other congenital abnormalities,
such as obstruction of the right or left ventricular outflow
tracts, complex cyanotic congenital heart disease, and coronary
artery anomalies
[7,
8]. In these patients with "spongy myocardium",
the recesses represent "persisting sinusoids" that fail to regress
during ontogenesis due to persistent pressure overload. These
recesses communicate with the ventricular cavity and the coronary
arteries
[7,
9]. In contrast, IVNC is considered to be a congenital
anomaly caused by arrest of the compaction process in the loose
myocardial meshwork during fetal ontogenesis. These intertrabecular
recesses, covered with endothelial cells, are filled with blood
from the ventricular cavity but there is no evidence of communication
with the epicardial coronary artery system
[7-12].
The diagnosis of IVNC is often missed or delayed because of lack of knowledge about this uncommon disease and its similarity to other diseases of the myocardium such as ACM [3-5,9]. Abnormalities of the resting ECG are found in the majority of patients with INVM but findings are nonspecific and include left ventricular hypertrophy, repolarization changes, inverted T waves, ST segment changes, axis shifts, intraventricular conduction abnormalities, and AV block [9]. However, ACM is characterized by giant negative T waves (–1.0 to –4.2mV) in the left precordial leads (V4 or V5) of the resting ECG [10]. Currently, transthoracic echocardiography is the most useful noninvasive diagnostic test for the diagnosis of IVNC [12,13]. The characteristic echocardiographic findings include multiple, prominent myocardial trabeculations and deep intertrabecular recesses which communicate with the LV cavity. IVNC is diagnosed when the above criteria are satisfied and coexisting cardiac lesions, such as semilunar valve obstruction and coronary artery anomalies, are excluded [6,9].
Treatment for non-compaction of the ventricular myocardium focuses on the three major clinical manifestations: heart failure, arrhythmias, and systemic embolic events. Standard medical therapy for systolic and diastolic ventricular dysfunction is warranted. Prevention of embolic complications is also an important management issue, and several authors have recommended long-term prophylactic anticoagulation for all patients with ventricular non-compaction whether or not a thrombus has been found [9,14,15].
Myocardial bridging, a congenital coronary anomaly, is characterized by systolic compression of an intramyocardial segment of an epicardial coronary artery and is most commonly localized in the middle segment of the LAD [16]. Although the condition is generally considered benign, it has been associated with myocardial ischaemia, myocardial infarction, depressed LV function, myocardial stunning, ventricular tachycardia, conduction disturbances, and sudden death [17,18]. Myocardial bridging is generally detected as an isolated abnormality, however, several reports have shown an association with hypertrophic cardiomyopathy [18,19]. Since patients with IVNC may be misdiagnosed as ACM, as in our case, the coexistence of IVNC and MB, which has not been reported previously, should be considered.
|
References
|
|---|
- Chin T.K., Perloff J.K., Williams R.G., Jue K., Mohrmann R. Isolated noncompaction of left ventricular myocardium: a study of eight cases. Circulation (1990) 82:507–513.[Abstract/Free Full Text]
- Richardson P., McKenna W., Bristow M., et al. Report of the 1995 World Health Organization/International Society and Federation of Cardiology Task Force on the definition and classification of cardiomyopathies. Circulation (1996) 93:841e2.
- Tufekcioglu O., Aras D., Cagli K., Ozeke O., Sahin O. Imaging of isolated ventricular non-compaction with dynamic cardiomyoplasty. Eur J Echocardiogr (2005) 6:219–220.[Free Full Text]
- Celik S., Gorgulu S., Gurol T., Dagdeviren B., Eren M., Tezel T. Myocardial noncompaction: two cases and review. Anadolu Kardiyol Derg (2003) 3:48–53.[Medline]
- Aras D., Tufekcioglu O., Topaloglu S., et al. Preserved systolic function with isolated left ventricular noncompaction in an elderly patient. Eur J Echocardiogr (2006) 7:71–74.[Abstract/Free Full Text]
- Agmon Y., Connolly H.M., Olson L.J., Khandheria B.K., Seward J.B. Noncompaction of the ventricular myocardium. J Am Soc Echocardiogr (1999) 12:859–863.[CrossRef][Web of Science][Medline]
- Dusek A., Osadal B., Duskova M. Postnatal persistence of spongy myocardium with embryonic blood supply. Arch Pathol (1975) 99:312–317.[Web of Science][Medline]
- Lauer R.M., Fink H.P., Petry E.L., Dunn M.I., Diehl A.M. Angiographic demonstration of intramyocardial sinusoids in pulmonary-valve atresia with intact ventricular septum and hypoplastic right ventricle. N Engl J Med (1964) 271:68–72.[Web of Science][Medline]
- Weiford B.C., Subbarao V.D., Mulhern K.M. Noncompaction of the ventricular myocardium. Circulation (2004) 109:2965–2971.[Free Full Text]
- Sakamoto T., Tei C., Murayama M., Ichiyasu H., Hada Y. Giant T wave inversion as a manifestation of asymmetrical apical hypertrophy (AAH) of the left ventricle. Echocardiographic and ultrasono-cardiotomographic study. Jpn Heart J (1976) 17:611–629.[Medline]
- Angelini A., Melacini P., Barbero F. Evolutionary persistence of spongy myocardium in humans. Circulation (1999) 99:247S. [Abstr].
- Jenni R., Oechslin E., Schneider J., Jost C.A., Kaufmann P.A. Echocardiographic and pathoanatomical characteristics of isolated left ventricular non-compaction: a step towards classification as a distinct cardiomyopathy. Heart (2001) 86:666–671.[Abstract/Free Full Text]
- Lin M.L., Connelly K., Prior D. An unusual cause of heart failure identified by echocardiography in an octogenarian. Eur J Heart Fail (2005) 7:99–102.[Abstract/Free Full Text]
- Ritter M., Oechslin E., Sutsch G., Attenhofer C., Schneider J., Jenni R. Isolated noncompaction of the myocardium in adults. Mayo Clin Proc (1997) 72:26–31.[Abstract]
- Oechslin E.N., Attenhofer Jost C.H., Rojas J.R., Kaufmann P.A., Jenni R. Long-term follow-up of 34 adults with isolated left ventricular noncompaction: a distinct cardiomyopathy with poor prognosis. J Am Coll Cardiol (2000) 36:493–500.[Abstract/Free Full Text]
- Mohlenkamp S., Hort W., Ge J., Erbel R. Update on myocardial bridging. Circulation (2002) 106:2616–2622.[Free Full Text]
- Noble J., Bourassa M.G., Petitclerc R., Dyrda Y. Myocardial bridging and milking effect of the left anterior descending coronary artery: normal variant or obstruction? Am J Cardiol (1976) 37:993–999.[CrossRef][Web of Science][Medline]
- Atmaca Y., Ozdol C., Pamir G., Kilickap M., Oral D. Successful surgical resection of a muscular bridge in a patient with nonobstructive hypertrophic cardiomyopathy. Angiology (2002) 53:225–227.[CrossRef][Web of Science][Medline]
- Saygi S., Turk O.U., Ozdogan O., Zoghi M. Apical hypertrophic cardiomyopathy coexistent with a myocardial bridging. Anadolu Kardiyol Derg (2004) 4:370–371.[Medline]
CiteULike 
Connotea 
Del.icio.us What's this?
Top
Abstract
1. Introduction