Usefulness of Magnetic Resonance Imaging Evaluation of Congenital Left Ventricular Aneurysms

doi:10.1016/j.amjcard.2007.02.094

The American Journal of Cardiology

Volume 100, Issue 2, 15 July 2007, Pages 310-315

https://doi.org/10.1016/j.amjcard.2007.02.094 Get rights and content

Congenital left ventricular (LV) aneurysm is a rare malformation of unknown cause that is often associated with a poor prognosis. This study was undertaken to evaluate the usefulness of cardiac magnetic resonance imaging in patients with congenital LV aneurysms and to determine the relation between clinical manifestations and the morphologic and functional characteristics of the aneurysms. Among the 26 consecutive patients with congenital LV aneurysms included, the anomalies involved the apex or free wall in 15 and the submitral myocardium in 11. Cardiac magnetic resonance provided detailed anatomic and functional assessment of the aneurysms in all patients. Compared with submitral aneurysms, apical or free-wall aneurysms were larger (24 ± 29 vs 3 ± 2 ml/m², p = 0.02), were more frequently associated with scar tissue by myocardial delayed enhancement imaging (71% vs 0%, p = 0.03), and tended to be more commonly associated with symptoms (53% vs 18%, p = 0.08). Aneurysm volume but not location correlated with LV size (r = 0.735, p <0.0001) and the ejection fraction (r = 0.774, p <0.0001). Apical or free-wall aneurysms were resected in 5 patients, with no mortality. There was 1 death after aortic valve replacement in a patient with type B Niemann-Pick disease, which was not clearly related to the LV aneurysm. The remaining 25 patients are alive at a median age of 13.5 years. In conclusion, the experience with this cohort illustrates that cardiac magnetic resonance is well suited for the morphologic and functional evaluation of congenital LV aneurysms.

Section snippets

Subjects

We searched the electronic records of all CMR examinations performed at Children’s Hospital Boston from September 1998 to September 2006 for the diagnosis of congenital LV aneurysm or diverticulum. Patients with traumatic or acquired aneurysms were excluded. In patients who had cardiac surgery, the aneurysms were known preoperatively. The following demographic and clinical data were abstracted from the patients’ medical records: date of birth, gender, date and age at CMR, presenting symptoms,

Results

The database search identified 26 patients with congenital LV aneurysms (n = 25) or diverticulum (n = 1). Table 1 details the clinical and CMR findings of the study patients. Age at CMR ranged from 1 day to 48 years (median 13.5 years), with a male/female ratio of 1.6. The aneurysms involved the LV apex in 15 patients (58%), including the LV free wall in 4 and an apical diverticulum in 1, and were confined to the submitral myocardium in 11 (42%), including 2 patients with 2 aneurysms each.

Discussion

The results of this study demonstrate the utility of CMR in the evaluation of patients with congenital LV aneurysms and expand the previously published clinical characteristics of this entity. To our knowledge, this is the largest case series of this entity reported from 1 center.

In a recent review of the published research, Ohlow⁴ identified 411 cases published since the anomaly was first described in 1816. Most case series, including recently published ones, report high rates of mortality and

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Characteristics and outcome of congenital left ventricular aneurysm and diverticulum: Analysis of 809 cases published since 1816
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Citation Excerpt :
All retrieved studies were examined to eliminate potential duplicates or overlapping data. An article was included if it reported about congenital LVA or LVD [5–7,9–293]. The 116 cases reported of Chen et al. [294] could not be included in our analysis because it was not possible to retrieve this article despite many efforts.
Congenital left ventricular aneurysm (LVA) or diverticulum (LVD) is rare cardiac anomalies. We aimed to analyse the clinical characteristics and outcome in all ever published patients.
MEDLINE, Web of science, Google and EMBASE, and reference lists of relevant articles were searched for publications reporting on LVA or LVD patients.
We identified 809 patients published since 1816 [354 (49.1%) LVA, 453 (50.6%) LVD, 2 (0.3%) both]. Mean age at diagnosis was 34.1 ± 27 (LVA) and 29.7 ± 27.6 years (LVD; p = 0.05). 48.9% were male. LVA was larger (38.7 ± 22.5 mm versus 31.4 ± 21.2 mm; p = 0.002) and frequently found in submitral location (33% versus 4.9%; p < 0.001), LVD was frequently located at the LV-apex (61.2% versus 28.7%; p < 0.001). LVD was often associated with cardiac (34.2% versus 11%; p < 0.001) or extracardiac anomalies (32.7% versus 3%; p < 0.001). LVA patients presented more frequently with ventricular tachycardia/fibrillation (18.1% versus 13.1%; p = 0.01), the incidences of rupture (4% versus 4.5%; p = 0.9), syncope (8.3% versus 5.1%; p = 0.1), and embolic events (4.9% versus 3.6%; p = 0.4) at presentation were not different between LVA and LVD.
Mean follow-up was 56.3 ± 43 months. Cardiac death occurred more frequently in the LVA group (11.5% versus 5.0%; p = 0.05) at a median age of 0.8 [LVA] and 2.5 [LVD] years. The leading cause of cardiac death was congestive heart failure in the LVA-group (50.0% versus 0.0%; p = 0.01), and rupture in the LVD-group (75.0% versus 27.3%; p = 0.04).
LVA and LVD are distinct congenital anomalies with different clinical and morphological characteristics. The prognosis of LVA is significantly worse during long-term follow-up.
Clinical significance of left ventricular apical aneurysms in hypertrophic cardiomyopathy patients: The role of diagnostic electrocardiography
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The diagnosis of HCM was based on echocardiographic documentation of a hypertrophied, non-dilated LV in the absence of another cardiac or systemic disease that could account for the hypertrophy [11]. A LVAA was defined as a discrete thin-walled dyskinetic or akinetic segment of the most distal portion of the chamber with a relatively wide communication to the LV cavity [6,12–14]. Twelve-lead ECG was reviewed during the evaluation.
Some patients with hypertrophic cardiomyopathy (HCM) develop left ventricular apical aneurysm, leading to serious cardiovascular complications. The aims of this study were to identify the incidence and clinical course of HCM patients with apical aneurysms in Japan, and to evaluate the role of electrocardiography (ECG) as a screening test to detect apical aneurysms in HCM patients.
In a retrospective, single center analysis of a population of 247 HCM patients, 21 patients (8.5%) had left ventricular apical aneurysms. Their mean age was 60 ± 14 years (range: 23–77 years) at study entry. Over 4.7 ± 3.3 years of follow-up, 10 patients (47.6%) experienced an adverse clinical event (annual event rate: 10.1%/y), including five implantable cardioverter-defibrillator (ICD) implantations for ventricular tachycardia/ventricular fibrillation (VT/VF), an appropriate discharge of ICD for VT/VF, and four nonfatal thromboembolic strokes. Two patients developed systolic dysfunction (ejection fraction <50%). No sudden cardiac death or progressive heart failure was detected. Fourteen patients showed ST-segment elevation (≥1 mm) in V3 through V5 of ECG. In four patients, progression of the ST-segment elevation was recognized. When the ST-segment elevation was used to identify apical aneurysms in HCM patients, the sensitivity was 66.7%, and the specificity was 98.7%.
Apical aneurysms in HCM patients in Japan are not rare, and are associated with serious cardiovascular complications. The early diagnosis of apical aneurysms can be achieved by serial ECG.
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View all citing articles on Scopus

: This study was supported in part by the Higgins Family Noninvasive Cardiac Imaging Research Fund.

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Congenital heart diseaseUsefulness of Magnetic Resonance Imaging Evaluation of Congenital Left Ventricular Aneurysms

Section snippets

Subjects

Results

Discussion

Am Heart J

J Thorac Cardiovasc Surg

Chest

Chest

Am J Cardiol

Am J Cardiol

Am J Cardiol

J Thorac Cardiovasc Surg

Cardiac diverticulum and omphalocele: Cantrell’s pentalogy or syndrome

Cardiol Young

Congenital left ventricular aneurysms and diverticula: definition, pathophysiology, clinical relevance and treatment

Cardiology

A giant congenital left ventricular diverticulum simulating an aneurysm

Eur Heart J

Images in cardiologyCongenital foramen of the left pericardium masquerading as left ventricular aneurysm

Heart

Congenital cardiac left ventricular aneurysm with pericardial effusion: early prenatal diagnosis and intervention

J Ultrasound Med

Left ventricular apical aneurysm as a consequence of diffuse type congenital nonfamilial supravalvular aortic stenosis in a 30-year-old female

Int Heart J

Clinical surgical experience of congenital submitral left ventricular aneurysm

Thorac Cardiovasc Surg

Congenital heart disease
Usefulness of Magnetic Resonance Imaging Evaluation of Congenital Left Ventricular Aneurysms