Noncompaction of the Right Ventricle

A 14-year-old boy with hypertrophic cardiomyopathy experienced ventricular fibrillation while running in his schoolyard. He was resuscitated and admitted to our hospital. Delayed enhancement cardiac magnetic resonance imaging revealed asymmetric ventricular septal hypertrophy and delayed enhancement of the ventricular septum. Monomorphic ventricular tachycardia (VT) was induced by double ventricular extrastimuli. During electroanatomical mapping, a low voltage area was found in the right ventricle, but none was found in the left ventricular endocardium. Pace-mapping from the anterior right ventricular outflow tract, i.e., the low voltage area, revealed delayed potentials, and pacing at this site generated an 85% morphological match to the induced VT. Endomyocardial biopsy of the right ventricular septum revealed differences in myocyte size and myocardial disarray, along with moderate interstitial fibrosis. A cardioverter defibrillator (ICD) was implanted. Genetic testing identified a novel heterozygous missense mutation in the cardiac myosin heavy chain gene.

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“…7) Isolated RV NCCMs have also been reported. [8][9][10][11] Our case was characterized by hypertrabeculation within the RV apex.…”

Section: Discussionmentioning

confidence: 79%

Aborted Sudden Cardiac Death in a Pediatric Patient with Hypertrophic Cardiomyopathy and a Novel MYH7 Mutation

Kogawa

Watanabe

Okumura

et al. 2016

Nichidai Igaku Zasshi

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“…This criterion was used in our series. It also has been suggested that a diagnosis of right NVM should be made associated with dilatation of the RV (21). In our series, three bilateral INVM had dilated ventricular lumen and only one right INVM had a normal volume.…”

Section: Discussionmentioning

confidence: 99%

Isolated Noncompaction of Ventricular Myocardium: a Magnetic Resonance Imaging Study of 11 Patients

et al. 2011

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ObjectiveTo retrospectively summarize the cardiac magnetic resonance imaging (CMRI) findings of isolated noncompaction of ventricular myocardium (INVM).Materials and MethodsEleven patients (M:F = 9:2; mean age, 35 years) were evaluated. Steady-state free precession (SSFP), fast spin echo (SE) sequence, SSFP cine imaging, and delayed enhanced inversion recovery spoiled gradient echo (IR-SPGR) sequence were used for showing abnormal myocardium, measuring ratio of noncompacted/compacted myocardium layers (NC/C ratio), and detecting myocardial viability. The left ventricle was divided into nine segments and a NC/C ratio > 2.3 in diastole was used as cutoff value in diagnosing left INVM. The right ventricle was assessed qualitatively.ResultsCardiac MRI indicated left INVM in seven patients, right INVM in one patient and biventricle INVM in three patients. Characteristic CMRI changes included prominent trabeculations, deep intertrabecular recesses and an increase in the NC/C ratio. The most frequently involved segments was left ventricular apex. Three patients had abnormal high signals within the trabecular structures on SE T2 weighted image. One ventricular aneurysm and one apical thrombus were also observed. Delayed enhancement was seen in six of nine patients with subendocardial and transmural patterns.ConclusionThere are CMRI features that might be characteristic for INVM.

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“…Right ventricular involvement in LVNC is poorly studied due to difficulty in differentiating the normal RV trabeculations from the pathological trabeculations [9]. However, dilatation of the right ventricle in addition to the hypertrabeculation can be considered for the diagnosis [10].…”

Section: Imagingmentioning

confidence: 99%

Unusual presentations of ‘‘left ventricular non compaction’’- A rare form of cardiomyopathy

Maheen¹,

MJ²

2021

J Med Sci Res.

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Left ventricular non-compaction is an overall rare form of non-ischemic cardiomyopathy. The true prevalence of left ventricular non compaction (LVNC) is unclear, but it has been reported in 0.014–0.05% of adults and even less in paediatric population [1]. We present the case series of the patients who presented with varied clinical presentation and diagnosed to have left ventricular non-compaction on imaging. We have reviewed the literature regarding diagnostic imaging criteria and imaging pitfalls of this condition.

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Noncompaction of the Right Ventricle

Cited by 29 publications

References 12 publications

Aborted Sudden Cardiac Death in a Pediatric Patient with Hypertrophic Cardiomyopathy and a Novel MYH7 Mutation

Aborted Sudden Cardiac Death in a Pediatric Patient with Hypertrophic Cardiomyopathy and a Novel MYH7 Mutation

Isolated Noncompaction of Ventricular Myocardium: a Magnetic Resonance Imaging Study of 11 Patients

Unusual presentations of ‘‘left ventricular non compaction’’- A rare form of cardiomyopathy

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