CASE 11856 Published on 01.06.2014

Hypertrophic cardiomyopathy with apical aneurysm: a particular case without significant coronary obstruction



Case Type

Clinical Cases


Elisabetta Chiodi1, Maria Teresa Cannizzaro1, Bruna Malta2, Clorinda Montalto2, Marcello Natali2, Zairo Ferrante2, Nicola Murri Dello Diago2, Andrea Fiorencis3, Donato Mele3, Riccardo Righi1, Melchiore Giganti2, Giorgio Benea1.

1) Radiology Department,
2) Universitary Radiology Department
(Director: Ph Melchiore Giganti),
3) Cardiology Department,
Arcispedale Sant’Anna,
Cona (Ferrara), ITALY

58 years, male

Area of Interest Cardiac, Cardiovascular system ; Imaging Technique MR, CT-Angiography
Clinical History
A 58-year-old male patient, with a history of echocardiographic diagnosis of myocardial hypertrophy with mild nonspecific intraventricular gradient. ECG-Holter documents: 3 runs of non-sustained ventricular tachycardia (max. 12 ticks). Moreover, the patient had already had episodes of palpitations of variable duration.
Imaging Findings
We performed a cardiac MRI with SSFP sequences (Fig. 1), T1 and T2 (Fig. 2a-b) black-blood FAT SAT and without fat suppression, SPGR perfusion and delayed enhancement of IRGE post second scan planes in the short axis, 3 chamber, 2 chamber and 4 chamber for the evaluation of any areas of fibrosis. In SSFP images an evident left mid-ventricular hypertrophy (and lesser RV) with apical aneurysm aspect of the apex of the heart is recognizable. After contrast administration, the presence of an apical aneurysm within the extended delayed enhancement transmural distribution with apparently non-coronary obstruction was confirmed (Fig. 3). The patient refused coronary angiography and a coronary-CT was performed, documenting all significant stenoses in the coronaries (Fig. 4).
As part of the hypertrophic form, midventricular obstruction (MVO) was defined as midventricular gradient ≥30 mmHg with a simultaneous appearance of midventricular muscular apposition, creating an hourglass shape of the LV, on echocardiography or magnetic resonance imaging.
LV apical aneurysm was defined as a discrete, thin-walled dyskinetic or akinetic apex with a relatively wide communication to the LV cavity.
Myocardial fibrosis is present in the majority of patients with overt HCM, and corresponds with impairment in myocardial energy metabolism, and correlates with the severity of ventricular remodelling and dysfunction. [1-6, 8, 15]
Use of late Gadolinium imaging has become a well-accepted technique to depict focal myocardial fibrosis correlating well with the autopsy findings.
Myocardial fibrosis typically occurs in hypertrophied regions, with multiple patchy foci predominantly involved in the midventricular wall, junctions of the interventricular septum and the right ventricular free wall. In case of MVO, an apical aneurysm with transmural scar highlighted with the use of gadolinium may be present.
The location of scarring is related to decreased regional systolic wall thickening, which does not correspond to the perfusion territories of the epicardial coronary arteries.
There is an increasing body of evidence that the presence and extent of myocardial fibrosis - visualized by late Gadolinium imaging - in patients with HCM, is an independent predictor of adverse outcome (SCD, sustained ventricular tachycardia or fibrillation, and heart failure). [9-13]
Also, in patients with none or minimal symptoms, late Gadolinium imaging adds prognostic value to conventional criteria for risk stratification.
Moreover, myocardial enhancement is related not only to an increased risk for SCD, but also to the presence of progressive disease and prediction of systolic and diastolic dysfunction.
Extensive myocardial scarring is invariably associated with end-stage dilated HCM [14] and it is more extensive than in patients with dilated cardiomyopathy (DCM). [16]
Midventricular HCM is a rare variant of asymmetric HCM and it is characterized by hypertrophy occurring predominantly in the middle third of the LV wall and by systolic apposition of the midventricular wall. [1, 2, 7]
Midventricular HCM may be associated with an apical aneurysm caused by increased systolic pressures in the apex from midventricular obstruction.
The importance of this variant is its association with ventricular arrhythmia, myocardial necrosis, and systemic embolism.
These patients may present a gradient between the LV apical region and the remainder of the chamber.
This situation may turn into a non-contractile apical aneurysm (about 2% of HCM patients). Eventually, with apical thrombus formation.
Differential Diagnosis List
The final diagnosis was mid-ventricular hypertrophic cardiomyopathy with apical aneurysm.
Cardiac hypertrophy sport
Non-compaction disease
Final Diagnosis
The final diagnosis was mid-ventricular hypertrophic cardiomyopathy with apical aneurysm.
Case information
DOI: 10.1594/EURORAD/CASE.11856
ISSN: 1563-4086