Document Detail

Isolated left ventricular noncompaction with a congenital aneurysm presenting with recurrent embolism.
Jump to Full Text
MedLine Citation:
PMID:  22787529     Owner:  NLM     Status:  PubMed-not-MEDLINE    
Abstract/OtherAbstract:
Isolated left ventricular noncompaction (LVNC) is a rare disorder caused by embryonic arrest of compaction. LVNC is sometimes associated with other congenital cardiac disorders; however, there have been few reports of its coexistence with a left ventricular aneurysm. A 40-year-old woman was admitted to our hospital for renal infarction. She had a history of embolic cerebral infarction 10 years ago. Transthoracic echocardiography showed prominent trabeculae and deep intertrabecular recesses which are filled with blood from the left ventricular (LV) cavity. A thrombus in the akinetic apical wall was confirmed by contrast echocardiography. Using cardiac computed tomography and magnetic resonance imaging, we rejected a possible diagnosis of suspicion of coronary artery disease. She was diagnosed LVNC with a thrombus in apical aneurysm. Here, we report the first patient in Korea known to have LVNC accompanying LV congenital aneurysm presenting with recurrent embolism.
Authors:
Jong-Hwa Ahn; Jin-Sin Koh; Jeong Rang Park; Mi Jung Park; Ji Hyun Min; Sang Young Cho; Eun Ju Lee; Wan Chul Kim; Kye Hwan Kim
Related Documents :
8006659 - Isolated hemiataxia after supratentorial brain infarction.
12044649 - The administration of complement component c9 augments post-ischemic cerebral infarctio...
7491649 - Measurement of infarct size using mri predicts prognosis in middle cerebral artery infa...
8452759 - Hemorrhagic transformation after cerebral ischemia: mechanisms and incidence.
16722979 - Is tee useful in patients with small subcortical strokes?
16002219 - Hydroxysafflor yellow a protects rat brains against ischemia-reperfusion injury by anti...
23305309 - The role of optical coherence tomography in clarifying the mechanisms for dobutamine st...
2930359 - Cardiac contusion. the effect on operative management of the patient with trauma injuries.
18971099 - Incremental value of n-terminal pro-brain natriuretic peptide over left ventricle eject...
Publication Detail:
Type:  Journal Article     Date:  2012-06-25
Journal Detail:
Title:  Journal of cardiovascular ultrasound     Volume:  20     ISSN:  2005-9655     ISO Abbreviation:  J Cardiovasc Ultrasound     Publication Date:  2012 Jun 
Date Detail:
Created Date:  2012-07-12     Completed Date:  2012-10-02     Revised Date:  2013-05-30    
Medline Journal Info:
Nlm Unique ID:  101477138     Medline TA:  J Cardiovasc Ultrasound     Country:  Korea (South)    
Other Details:
Languages:  eng     Pagination:  103-7     Citation Subset:  -    
Affiliation:
Department of Internal Medicine, Gyeongsang National University Hospital, Jinju, Korea.
Export Citation:
APA/MLA Format     Download EndNote     Download BibTex
MeSH Terms
Descriptor/Qualifier:
Comments/Corrections

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

Full Text
Journal Information
Journal ID (nlm-ta): J Cardiovasc Ultrasound
Journal ID (iso-abbrev): J Cardiovasc Ultrasound
Journal ID (publisher-id): JCU
ISSN: 1975-4612
ISSN: 2005-9655
Publisher: Korean Society of Echocardiography
Article Information
Download PDF
Copyright © 2012 Korean Society of Echocardiography
open-access:
Received Day: 26 Month: 2 Year: 2012
Revision Received Day: 03 Month: 4 Year: 2012
Accepted Day: 15 Month: 5 Year: 2012
Print publication date: Month: 6 Year: 2012
Electronic publication date: Day: 25 Month: 6 Year: 2012
Volume: 20 Issue: 2
First Page: 103 Last Page: 107
ID: 3391626
PubMed Id: 22787529
DOI: 10.4250/jcu.2012.20.2.103

Isolated Left Ventricular Noncompaction with a Congenital Aneurysm Presenting with Recurrent Embolism
Jong-Hwa Ahn, MD1
Jin-Sin Koh, MD1
Jeong Rang Park, MD1
Mi Jung Park, MD2
Ji Hyun Min, MD1
Sang Young Cho, MD1
Eun Ju Lee, MD1
Wan Chul Kim, MD1
Kye Hwan Kim, MD1
1Department of Internal Medicine, Gyeongsang National University Hospital, Jinju, Korea.
2Department of Radiology, Gyeongsang National University Hospital, Jinju, Korea.
Correspondence: Address for Correspondence: Jeong Rang Park, Department of Internal Medicine, Gyeongsang National University Hospital, 79 Gangnam-ro, Jinju 660-702, Korea. Tel: +82-55-750-8058, Fax: +82-55-755-9078, parkjrang@gmail.com

INTRODUCTION

Isolated left ventricular noncompaction (LVNC) is a congenital cardiomyopathy caused by a defect in endomyocardial morphogenesis.1) It is characterized by prominent trabeculation and deep intertrabecular recesses, resulting in thickened myocardium consisting of 2 layers-compacted and noncompacted myocardium.2) LVNC is occasionally combined with other congenital cardiac diseases3-6) and genetic cardiomyopathies.7) Its clinical manifestations include heart failure, thromboembolism, ventricular tachyarrhythmia, and sudden death.8) The risk of embolic complications was mostly associated with impaired systolic function.9) There were only a few cases about coexisting left ventricular aneurysm.10-12) We report a rare case of LVNC associated with left ventricular (LV) aneurysm, presenting with recurrent embolism.


CASE

A 40-year-old woman was admitted to our hospital for abdominal and lower back pain lasting for 10 days. In 1999, she had an embolic stroke of the right middle cerebral artery territory; however, after 2007, she was lost to follow-up and discontinued all medications, including antiplatelet and anticoagulant therapy. She did not smoke or drink alcohol. She had none of the classic cardiovascular risk factors such as diabetes or hypertension nor any family history of cardiomyopathy or heart failure. Her chest radiography and laboratory data, including B-type natriuretic peptide and cardiac enzymes levels, were unremarkable. An electrocardiogram revealed nonspecific ST changes and intraventricular conduction delay. Abdominal computed tomography, performed because of her abdominal pain, revealed left renal infarction. To evaluate for an embolic source, transthoracic and transesophageal echocardiography were performed. Two-dimensional echocardiography showed prominent trabeculations at the interventricular septum and the lateral wall. Color Doppler imaging revealed deep intertrabecular recesses filled by blood flowing from the ventricular cavity (Fig. 1). The ratio of maximal thickness of the noncompacted to compacted layers in the lateral wall was greater than 2 at end-systole. The 4-chamber and 2-chamber views defined the LVNC in the mid-septal and inferior walls and apical wall thinning (Fig. 2). Because wall motion of the apex was akinetic at end-systole and end-diastole, we confirmed the diagnosis as an apical aneurysm. Additionally, thrombus in the apical aneurysm was shown by contrast echocardiography (Fig. 3). The LV ejection fraction by modified Simpson's rule was 48% (LV end-diastolic volume 89 mL and LV end-systolic volume 49 mL) and the ratio of early diastolic mitral inflow velocity to early diastolic mitral septal annular velocity (E/E') was 7.62. We performed coronary computed tomography angiography and cardiac magnetic resonance imaging (MRI) to differentiate the aneurysm from post-infarction aneurysms. On computed tomography, there was no coronary stenosis (Fig. 4). Cardiac MRI showed two-layered appearance of trabeculated and compacted myocardium, it revealed a thin compacted layer preserving contractility and an apical aneurysm with akinetic motion at end-diastole and end-systole (Fig. 5). No late gadolinium enhancement was observed. We therefore diagnosed her with left renal infarction caused by LVNC, coexisting with an LV aneurysm. She was prescribed warfarin and has followed up uneventfully to date.


DISCUSSION

LVNC is a rare congenital cardiomyopathy characterized by multiple prominent trabeculations with deep intertrabecular recesses.1) An arrest of compaction of the developing myocardium is strongly suggested as the probable mechanism of LVNC.1), 9) Recently, the American Heart Association classified LVNC as a primary genetic cardiomyopathy.13) In contrast, the European Society of Cardiology considers LVNC to be an "unclassified cardiomyopathy".14) Multiple diagnostic criteria for LVNC have been proposed on the basis of echocardiography and cardiac MRI findings. The echocardiographic criteria suggested by Jenni et al.15) have become widely accepted. They are as follows: 1) thickened myocardium with a 2-layered structure consisting of a thin compacted epicardial layer [C] and a much thicker, noncompacted endocardial layer [N] or trabecular meshwork with deep endomyocardial spaces (N/C ratio > 2.0 at end-systole); 2) predominant location of the pathology in the mid-lateral, mid-inferior, and apical areas; 3) color Doppler evidence of deep intertrabecular recesses filled with blood from the LV cavity; and 4) absence of coexisting cardiac abnormalities (in isolated LVNC). There have been many reports of coexistent congenital cardiac disorders, including atrial septal defect, ventricular septal defect, pulmonary stenosis, anomalous pulmonary venous connection, Ebstein's anomaly, and a bicuspid aortic valve.3-6) However, only a few cases of LVNC with LV aneurysm have been reported.10-12) The mechanism of aneurysm is uncertain. Sato et al.10) proposed impaired microcirculation of noncompacted and compacted layers as the mechanism of aneurysm formation in LVNC. However, in our patient, the epicardial coronary arteries appeared normal on coronary computed tomography angiography and neither perfusion defects nor delayed enhancement were seen on cardiac MRI. We therefore thought that our patient's aneurysm might be congenital rather than degenerative change of LVNC.

The classical triad of complications with LVNC is heart failure, ventricular arrhythmia, and systemic embolic events.8) Because there are limited data regarding treatment of this condition, it is recommended that clinical complications be managed according to the current guidelines for each clinical complication. Our patient presented with 2 embolic events: stroke and renal infarction. The prevalence of systemic embolic events in patients with LVNC varied in reports. Based on the high rate of embolic events reported in long-term follow-up data, Oechslin et al.8) recommended anticoagulant therapy for these patients, independent of ventricular systolic function. However, Oechslin and Jenni9) recently recommended anticoagulation therapy for patients with impaired systolic function (LV ejection fraction < 40%) because deep intertrabecular recesses and slow blood flow might increase the risk of thrombus formation. Our patient had a thrombus in an apical LV aneurysm. We believed that the apical thrombus was the embolic source of her presentation with renal infarction and that the apical aneurysm with slow blood flow was a risk factor for recurrent embolic events. Therefore, we suggest that anticoagulation therapy might be needed in patients with LVNC with coexisting LV aneurysm, even in the absence of systolic dysfunction or atrial fibrillation.

In conclusion, we described a rare case of LVNC with LV aneurysm presenting as recurrent thromboembolic events. We believe that careful evaluation of LVNC patients for coexisting heart abnormalities such as aneurysms is essential for making the best clinical decisions for their management.


References
1. Chin TK,Perloff JK,Williams RG,Jue K,Mohrmann R. Isolated noncompaction of left ventricular myocardium. A study of eight casesCirculationYear: 1990825075132372897
2. Ritter M,Oechslin E,Sütsch G,Attenhofer C,Schneider J,Jenni R. Isolated noncompaction of the myocardium in adultsMayo Clin ProcYear: 19977226319005281
3. Burke A,Mont E,Kutys R,Virmani R. Left ventricular noncompaction: a pathological study of 14 casesHum PatholYear: 20053640341115892002
4. Ichida F. Left ventricular noncompactionCirc JYear: 200973192619057090
5. Attenhofer Jost CH,Connolly HM,Warnes CA,O'leary P,Tajik AJ,Pellikka PA,Seward JB. Noncompacted myocardium in Ebstein's anomaly: initial description in three patientsJ Am Soc EchocardiogrYear: 20041767768015163943
6. Betrián Blasco P,Gallardo Agromayor E. Ebstein's anomaly and left ventricular noncompaction associationInt J CardiolYear: 200711926426517067700
7. Biagini E,Ragni L,Ferlito M,Pasquale F,Lofiego C,Leone O,Rocchi G,Perugini E,Zagnoni S,Branzi A,Picchio FM,Rapezzi C. Different types of cardiomyopathy associated with isolated ventricular noncompactionAm J CardiolYear: 20069882182416950194
8. Oechslin EN,Attenhofer Jost CH,Rojas JR,Kaufmann PA,Jenni R. Long-term follow-up of 34 adults with isolated left ventricular noncompaction: a distinct cardiomyopathy with poor prognosisJ Am Coll CardiolYear: 20003649350010933363
9. Oechslin E,Jenni R. Left ventricular non-compaction revisited: a distinct phenotype with genetic heterogeneity?Eur Heart JYear: 2011321446145621285074
10. Sato Y,Matsumoto N,Yoda S,Inoue F,Kunimoto S,Fukamizu S,Tani S,Takayama T,Tokai K,Kasamaki Y,Saito S,Uchiyama T,Koyama Y. Left ventricular aneurysm associated with isolated noncompaction of the ventricular myocardiumHeart VesselsYear: 20062119219416715195
11. Ionescu CN,Turcot D. Left ventricular noncompaction and aneurysm revealed by left ventriculographyCatheter Cardiovasc IntervYear: 2011 [Epub ahead of print].
12. Yun H,Zeng MS,Jin H,Yang S. Isolated noncompaction of ventricular myocardium: a magnetic resonance imaging study of 11 patientsKorean J RadiolYear: 20111268669222043150
13. Maron BJ,Towbin JA,Thiene G,Antzelevitch C,Corrado D,Arnett D,Moss AJ,Seidman CE,Young JB. American Heart AssociationCouncil on Clinical Cardiology, Heart Failure and Transplantation CommitteeQuality of Care and Outcomes Research and Functional Genomics and Translational Biology Interdisciplinary Working GroupsCouncil on Epidemiology and PreventionContemporary definitions and classification of the cardiomyopathies: an American Heart Association Scientific Statement from the Council on Clinical Cardiology, Heart Failure and Transplantation Committee; Quality of Care and Outcomes Research and Functional Genomics and Translational Biology Interdisciplinary Working Groups; and Council on Epidemiology and PreventionCirculationYear: 20061131807181616567565
14. Elliott P,Andersson B,Arbustini E,Bilinska Z,Cecchi F,Charron P,Dubourg O,Kühl U,Maisch B,McKenna WJ,Monserrat L,Pankuweit S,Rapezzi C,Seferovic P,Tavazzi L,Keren A. Classification of the cardiomyopathies: a position statement from the European Society Of Cardiology Working Group on Myocardial and Pericardial DiseasesEur Heart JYear: 20082927027617916581
15. Jenni R,Oechslin E,Schneider J,Attenhofer Jost C,Kaufmann PA. Echocardiographic and pathoanatomical characteristics of isolated left ventricular non-compaction: a step towards classification as a distinct cardiomyopathyHeartYear: 20018666667111711464

Article Categories:
  • Case Report

Keywords: Left ventricular noncompaction, Left ventricular aneurysm.

Previous Document:  Perforated Mitral Valve Aneurysm in the Posterior Leaflet without Infective Endocarditis.
Next Document:  Multiple Fistula Emptying into the Left Ventricle through the Entire Left Ventricular Wall.