**5. Conclusion**

calculation of trabeculated LV mass using MRI could help also in the diagnosis of ILVNC, with trabeculated LV mass of > 20% of the total LV mass having the highest sensitivity and specificity

**Figure 2.** Apical 2 chamber view showing prominent trabeculations in the apex and inferior walls consistent with Iso‐

Computed tomography scan could be used to diagnose ILVNC and has high spatial resolution. Prominent trabeculations as well as deep intertrabecular recesses are typically seen [103]. Contrast ventriculography could also be used but is invasive. PET scan could show decreased myocardial flow reserve in noncompacted areas as well as microcirculatory dysfunction in both compacted and noncompacted myocardium but this has limited utility in establishing the diagnosis [92, 110]. To date, Echocardiography and MRI remain the most common

Management of patients with ILVNC involves treating heart failure, protection from sudden cardiac death, anticoagulation to prevent thromboembolic events, and screening of family members. β-blockers, angiotensin converting enzyme inhibitors and angtiotensin receptor blockers are used and have been reported to improve symptoms and the LVEF [111]. Antico‐ agulation with coumadin is recommended in all patients, even if they don't have atrial fibrillation. Electrophysiology testing to predict the risk of sudden cardiac death has not

(93.7%) in diagnosing ILVNC [109].

modalities used for diagnosing ILVNC.

*4.7.3. Other imaging modalities*

lated Left Ventricular Non-Compation.

152 Cardiomyopathies

**4.8. Therapy**

Cardiac Sarcoidosis, arrhythmogenic right ventricular dysplasia and isolated left ventricular noncompaction are rare forms of cardiomyopathy that affect young patients and put them at risk of sudden cardiac death. Early recognition and treatment is important. In the absence of clear guidelines to prevent sudden cardiac death in this young population, the clinician should use current knowledge, clinical judgment and expertise when treating these patients. Advan‐ ces in diagnostic imaging as well as genetic testing will help early diagnosis and identification of affected family members.
