Isolated noncompaction of ventricular myocardium, sometimes referred to as spongy myocardium, is a rare congenital cardiomyopathy. Spongy myocardium results from an arrest in normal endomyocardial embryogenesis. The clinical symptoms and electrocardiographic findings of this disorder can be showed variously. The diagnosis is usually made by echocardiography. We report a case of isolated noncompaction of the ventricular myocardium in a 20-year-old male patient initially presented with syncope caused by ventricular tachycardia, who was diagnosed with echocardiography, cardiac MRI and endomyocardial biopsy.
Adult* ; Biopsy ; Cardiomyopathies ; Diagnosis ; Echocardiography ; Electrocardiography ; Embryonic Development ; Female ; Humans ; Isolated Noncompaction of the Ventricular Myocardium ; Magnetic Resonance Imaging ; Male ; Myocardium* ; Pregnancy ; Syncope ; Tachycardia, Ventricular* ; Young Adult

Isolated noncompaction of ventricular myocardium, sometimes referred to as spongy myocardium, is a rare congenital cardiomyopathy. Spongy myocardium results from an arrest in normal endomyocardial embryogenesis. The clinical symptoms and electrocardiographic findings of this disorder can be showed variously. The diagnosis is usually made by echocardiography. We report a case of isolated noncompaction of the ventricular myocardium in a 20-year-old male patient initially presented with syncope caused by ventricular tachycardia, who was diagnosed with echocardiography, cardiac MRI and endomyocardial biopsy.
Adult* ; Biopsy ; Cardiomyopathies ; Diagnosis ; Echocardiography ; Electrocardiography ; Embryonic Development ; Female ; Humans ; Isolated Noncompaction of the Ventricular Myocardium ; Magnetic Resonance Imaging ; Male ; Myocardium* ; Pregnancy ; Syncope ; Tachycardia, Ventricular* ; Young Adult

OBJECTIVE: To retrospectively summarize the cardiac magnetic resonance imaging (CMRI) findings of isolated noncompaction of ventricular myocardium (INVM). MATERIALS AND METHODS: Eleven 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. RESULTS: Cardiac 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. CONCLUSION: There are CMRI features that might be characteristic for INVM.
Adolescent ; Adult ; Female ; Heart Aneurysm/complications/diagnosis ; Heart Ventricles/pathology ; Humans ; Isolated Noncompaction of the Ventricular Myocardium/complications/*diagnosis ; *Magnetic Resonance Imaging ; Male ; Middle Aged ; Myocardium/pathology ; Young Adult

OBJECTIVETo analyze the incidence of coronary artery disease (CAD) and outcome of patients with left ventricular noncompaction (LVNC).

METHODSFifty-one patients with LVNC evaluated by echocardiography and/or cardiac magnetic resonance (CMR) from January 2006 to August 2010 were retrospectively reviewed. Coronary angiography or MDCT was performed for detecting coronary artery disease. Predictors of the cardiac events were analyzed by Cox regression analysis.

RESULTSThere were 31 LVNC patients without CAD and 20 LVNC patients with CAD including single vessel coronary disease in 9 cases, double vessel coronary disease in 3 cases, three vessel coronary disease in 5 cases and left main coronary disease in 3 cases. Coronary artery bypass graft and percutaneous coronary intervention (PCI) were performed in 4 patients. Compared to LVNC patients without CAD, mean age (P = 0.008), incidence of hypertension (65.0% vs. 19.4%, P = 0.001), diabetes mellitus (40.0% vs. 12.9%, P = 0.026) and hyperlipidemia (55.0% vs. 25.8%, P = 0.035) were significantly higher while NT-proBNP level was significantly lower (P = 0.049) in LVNC patients with CAD. Incidence of major cardiac events was similar in LVNC patients with or without CAD. LogNT-proBNP is the independent prognostic factor for adverse cardiac events in patients with LVNC (HR 3.993, 95%CI 1.140 - 13.988, P = 0.030).

CONCLUSIONSCoronary artery disease is common in patients with LVNC and associated with traditional risk factors for CAD. Poor prognosis is associated with increased NT-proBNP but not with CAD in this patient cohort.

Adolescent ; Adult ; Aged ; Cardiomyopathies ; complications ; diagnosis ; pathology ; Female ; Heart Ventricles ; pathology ; Humans ; Incidence ; Isolated Noncompaction of the Ventricular Myocardium ; complications ; diagnosis ; pathology ; Male ; Middle Aged ; Prognosis ; Retrospective Studies ; Young Adult

PURPOSE: Isolated noncompaction of the ventricular myocardium(INVM) can present as heart failure or arrhythmias in a child. It is a rare disorder, characterized by prominent trabecular meshwork and deep intertrabecular recesses. We still know little about the diagnosis, symptoms, and clinical outcomes of INVM. METHODS: We included in our study 6 patients who showed ventricular noncompaction on echocardiography. Patients were diagnosed as INVM were excessively prominent trabeculations with deep intertrabecular recesses were found on echocardiography. Patients who had other complex heart lesions such as pulmonary atresia with intact ventricular septum in addition to ventricular noncompaction, were excluded. RESULTS: Age at presentation ranged from 1 day 7 years, with follow up being as long as 6 years. Symptoms at initial presentation were heart murmur, paroxysmal supraventricular tachycardia, cyanosis, feeding intolerance, ventricular tachycardia, and cardiomegaly at fetal screening. Prominent trabeculations and intertrabecular recesses were observed at left ventricular apex in all six patients. All patients were alive at last follow-up. One patient showed WPW syndrome on electrocardiography. Echocardiography revealed decreased systolic function in 4 patients, and decreased systolic and diastolic function in 1 patient. One patient is currently asymptomatic. CONCLUSION: Six patients were diagnosed with INVM with various symptoms at initial presentation. Echocardiography is the most important tool in the diagnosis of INVM due to its morphological characteristics. INVM can rarely be the cause of long term systolic dysfunction, and early detection by echocardiographic screening may be beneficial.
Arrhythmias, Cardiac ; Cardiomegaly ; Child ; Cyanosis ; Diagnosis ; Echocardiography ; Electrocardiography ; Follow-Up Studies ; Heart ; Heart Failure ; Heart Murmurs ; Humans ; Isolated Noncompaction of the Ventricular Myocardium* ; Mass Screening ; Pulmonary Atresia ; Tachycardia, Supraventricular ; Tachycardia, Ventricular ; Trabecular Meshwork ; Ventricular Septum ; Wolff-Parkinson-White Syndrome

OBJECTIVETo investigate the clinicopathologic manifestations of non-compaction of ventricular myocardium (NVM).

METHODSClinical data, electrocardiograms, echocardiography images and pathologic changes were studied in five cases of non-compaction of ventricular myocardium.

RESULTSThe patient's ages ranged from 29 to 57 years old, all were males. Abnormal electrocardiograms were obtained in all of the 5 cases. Among them, 3 were diagnosed using echocardiography. Histopathologic examination showed that there were abnormally coarse muscle trabeculation and deep recesses, interlacing in arrangement, over the inner wall of the heart chambers. The compacted myocardium became thinning down gradually from the base to the apex of the heart. The non-compacted myocardium bundles locating close to the endocardium were coarse and orderless in arrangement, nuclei were irregular and abnormal, nevertheless, the arrangement and appearance of the muscle bundles near by the pericardium part were essentially normal and the cell nuclei were evenly distributed.

CONCLUSIONThere are no specific clinical manifestations obtained in patients with non-compaction of ventricular myocardium, however, the pathologic changes are characteristic and a clinical diagnosis can be made by using echocardiography.

Adult ; Cardiomyopathy, Dilated ; diagnostic imaging ; pathology ; Cardiomyopathy, Hypertrophic ; diagnostic imaging ; pathology ; Diagnosis, Differential ; Electrocardiography ; Heart Ventricles ; pathology ; Humans ; Isolated Noncompaction of the Ventricular Myocardium ; diagnostic imaging ; pathology ; Male ; Middle Aged ; Myocardium ; pathology ; Ultrasonography

PURPOSE: Diagnostic criteria for left ventricular non-compaction (LVNC) are still a matter of dispute. The aim of our present study was to test the diagnostic value of two novel diagnostic cardiac magnetic resonance (CMR) parameters: proof of non-compact (NC) myocardium blood flow using T2 sequences and changes in geometry of the left ventricle. MATERIALS AND METHODS: The study included cases with LVNC and controls, from a data base formed in a period of 3.5 years (n=1890 exams), in which CMR protocol included T2 sequences. Measurement of perpendicular maximal and minimal end diastolic dimensions in the region with NC myocardium from short axis plane was recorded, and calculated as a ratio (MaxMinEDDR), while flow through trabecula was proven by intracavital T2-weighted hyperintensity (ICT2HI). LVNC diagnosis met the following three criteria: thickening of compact (C) layer, NC:C>2.3:1 and NC>20%LV. RESULTS: The study included 200 patients; 71 with LVNC (35.5%; i.e., 3.76% of CMRs) and 129 (64.5%) controls. MaxMinEDDR in patients with LVNC was significantly different from that in controls (1.17±0.08 vs. 1.06±0.04, respectively; p < 0.001). MaxMinEDDR >1.10 had sensitivity of 91.6% [95% confidence intervals (CI) 82.5–96.8], specificity of 85.3% (95% CI 78.0–90.0), and area under curve (AUC) 0.919 (95% CI 0.872–0.953; p < 0.001) for LVNC. Existence of ICT2HI had sensitivity of 100.0% (95% CI 94.9–100.0), specificity of 91.5% (95% CI 85.3–95.7), and AUC 0.957 (95% CI 0.919–0.981; p < 0.001) for LVNC. CONCLUSION: Two additional diagnostic parameters for LVNC were identified in this study. ICT2HI and geometric eccentricity of the ventricle both had relatively high sensitivity and specificity for diagnosing LVNC.
Adolescent ; Adult ; Aged ; Aged, 80 and over ; Area Under Curve ; Case-Control Studies ; Female ; Gadolinium/chemistry ; Heart Ventricles/diagnostic imaging ; Heart Ventricles/pathology ; Humans ; Isolated Noncompaction of the Ventricular Myocardium/diagnosis ; Magnetic Resonance Imaging ; Male ; Middle Aged ; ROC Curve ; Young Adult

PURPOSE: Isolated noncompaction of the ventricular myocardium(INVM) is one of the unclassified cardiomyopathies that is characterized by numerous, excessively prominent trabeculations, and deep intertrabecular recesses. We performed this study to evaluate the clinical features of INVM in children. METHODS: The medical records of 10 patients with INVM were reviewed. We analyzed the clinical manifestations, hemodynamics, pattern of inheritance, and long-term prognosis of INVM in children. RESULTS: Age at diagnosis was 45+/-53 months(1 day-14 years) with follow-up lasting as long as 78 months. Most INVM was asymptomatic on diagnosis. Associated cardiac anomalies were noted in six patients(ventricualr or atrial septal defect, patent ductus arteriosus with mitral valve prolapse, or mitral valve cleft). Depressed or flat changes of T wave in lead II, III and aVF were observed on electrocardiography. Various arrhythmia including WPW syndrome with paroxysmal, supraventricular tachycardia, third-degree atrioventricular block, and familial sick sinus node dysfuction were observed. The degree of trabeculation in INVM was significantly prominent from level of mitral valve to apex compared to age-matched control. Familial recurrences were noted in two patients. The systolic function of the left ventricle was decreased in 20% of patients during the follow-up period, but systemic embolism or ventricular tachycardia was not observed. CONCLUSIONS: INVM is not a rare disorder. The cardiac function may be deteriorated in children as well as adults during long-term follow up. Thus early diagnosis and long-term follow-up must be done. So, the nation-wide multicenter clinical study would be mandatory to evaluate the incidence, long-term prognosis, and establishment of objective diagnostic criteria of INVM.
Adult ; Arrhythmias, Cardiac ; Atrioventricular Block ; Cardiomyopathies ; Child ; Diagnosis ; Ductus Arteriosus, Patent ; Early Diagnosis ; Electrocardiography ; Embolism ; Follow-Up Studies ; Heart Septal Defects, Atrial ; Heart Ventricles ; Hemodynamics ; Humans ; Incidence ; Isolated Noncompaction of the Ventricular Myocardium* ; Medical Records ; Mitral Valve ; Mitral Valve Prolapse ; Prognosis ; Recurrence ; Sinoatrial Node ; Tachycardia, Supraventricular ; Tachycardia, Ventricular ; Wills ; Wolff-Parkinson-White Syndrome

We describe a case of new-onset heart failure in a patient in whom cardiac CT enabled the non-invasive diagnosis of isolated non-compaction and associated functional abnormalities of the left ventricle with the concomitant evaluation of coronary arteries. This case highlights the utility of cardiac CT for the morphological and functional evaluation of the heart as a single imaging modality.
Aged ; Cardiac-Gated Imaging Techniques ; Contrast Media/diagnostic use ; Diagnosis, Differential ; Disease Progression ; Echocardiography ; Electrocardiography ; Heart Failure/*diagnosis ; Humans ; Image Interpretation, Computer-Assisted ; Iohexol/analogs & derivatives/diagnostic use ; Isolated Noncompaction of the Ventricular Myocardium/*diagnosis ; Magnetic Resonance Imaging ; Male ; *Tomography, X-Ray Computed