Isolated Left Ventricular Noncompaction Research Articles

Evaluation of isolated left ventricular noncompaction using cardiac magnetic resonance tissue tracking in global, regional and layer-specific strains

We used cardiac magnetic resonance tissue tracking (CMR-TT) to quantitatively analyze the global, regional and layer-specific strain of isolated left ventricular noncompaction (ILVNC). Combined with late gadolinium enhancement (LGE), we initially explored the effect of focal myocardial fibrosis on myocardial strain. CMR was performed in 63 patients with ILVNC and 52 patients without ILVNC (i.e., the control group). The ILVNC group was divided into an LGE(+) group (29 patients) and an LGE(−) group (34 patients) according to the presence or absence of late gadalinum enhancement (LGE). CVI42 software was used to measure global and regional (basal, middle, apical) radial strain (RS), circumferential strain (CS), longitudinal strain (LS), subendocardial LS and subepicardial LS. The basal–apical strain gradient was defined as the apical mean strain minus the basal mean strain. We then compared differences between these strain parameters. The subendocardial-subepicardial LS gradient was defined as the maximum subendocardial LS minus the subepicardial LS. Compared with the control group, the global and regional RS, CS, LS and the subendocardial, subepicardial LS of the ILVNC group were significantly diminished (P < 0.01). Compared with the LGE(−) group, the global and regional RS, CS, LS and the subendocardial, subepicardial LS of the LGE(+) group were significantly diminished (P < 0.05). In the ILVNC group, the basal–apical CS and LS gradient, and the subendocardial-subepicardial LS gradient were significantly lower than those in the control group (P < 0.01). There were significant differences in myocardial strain between patients with and without ILVNC. ILVNC revealed a specific pattern in terms of strain change. The myocardial strain of the cardiac apex and endocardium was significantly lower than that of the cardiac base and epicardium, respectively. Myocardial strain reduction was more significant in ILVNC patients with focal myocardial fibrosis.

Different Manifestations in Familial Isolated Left Ventricular Non-compaction: Two Case Reports and Literature Review

Left ventricular non-compaction (LVNC) is a form of cardiomyopathy characterized by prominent trabeculae and deep intertrabecular recesses which form a distinct “non-compacted” layer in the myocardium. It results from intrauterine arrest of the compaction process of the left ventricular myocardium. Clinical manifestations vary from asymptomatic to heart failure (HF), arrhythmias, or thromboembolic events. We present a case of mother and son diagnosed with isolated LVNC (ILVNC). A 4-years-old male patient, diagnosed at 3 months with ILVNC, and NYHA functional class IV HF, was admitted to the Emergency Institute for Cardiovascular Diseases and Transplantation of Targu Mures, Romania, for cardiologic reevaluation, and diagnosis confirmation. ILVNC was confirmed using echocardiography, revealing a non-compaction to compaction (NC/C) ratio of > 2.7. His evolution was stationary until the age of 8 years, when severe pneumonia caused hemodynamic decompensation, and he was listed for heart transplantation (HT). The patient underwent HT at the age of 11 years with favorable postoperative outcome. Meanwhile, a 22-years-old female patient, mother of the aforementioned patient, was also admitted to our institute due to severe fatigue, dyspnea, and recurrent palpitations with multiple implantable cardioverter defibrillator (ICD) shock delivery. Extensive medical history revealed that a presumptive ILVNC diagnosis was established when she was 11 years old. She was asymptomatic until 18 years old, when 3 months post-partum, she developed NYHA functional class III HF, and subsequently underwent ICD implantation. Her diagnosis was confirmed using multi-detector computed tomography angiography, which revealed a NC/C ratio of > 3.3. ICD adjustments were carried out with a favorable evolution under chronic drug therapy. The last evaluation, at 27 years old, revealed that she was in NYHA functional class II HF. In conclusion, ILVNC, even when familial, can present different clinical pictures and therefore requires different medical approaches.

İzole sol ventrikül noncompaction olan hastalarda miyokard dokusunun analizi

Aim: Videodensitometric myocardial texture analysis (VMTA) has been widely used to investigate left ventricular (LV) dysfunction in various cardiac disorders. Patients with isolated left ventricular noncompaction (IVNC) experience an undulating decline in LV function. The aim of this study was to assess the value of VMTA for evaluating deterioration of LV function in this patient group.Material and Methods: Twenty-two patients with IVNC (10 asymptomatic [preserved LV function], 12 symptomatic [LV dysfunction]) and 12 healthy controls were evaluated. Videodensitometry was used to record background-corrected mean gray levels (BC-MGL) for 2 regions of the mid-basal LV wall (the interventricular septum [IVS] and the posterior wall [PW]) at end-systole and end-diastole. The cyclic variation (CV) index for each region was calculated according to the formula, CV index % = ([BC-MGLend-diastole – BC-MGLend-systole] ÷ BC-MGLend-diastole) × 100. Results: The mean IVS-CV index in the symptomatic IVNC group (12.3 ± 4.9%) was significantly lower than the corresponding findings in the asymptomatic IVNC group (32.3 ± 14.8%, p &amp;lt; 0.05) and control group (36.6 ± 12.1%, p &amp;lt; 0.001). The mean PW-CV index in the symptomatic group (15.1 ± 5.5%) was also significantly lower than the corresponding values in the asymptomatic (27.0 ± 10.4%, p &amp;lt; 0.05) and control groups (28.8 ± 10.9%, p &amp;lt; 0.001). Conclusion: VMTA is a practical, useful adjunct to conventional echocardiography for assessing LV myocardium in patients with IVNC. Detection of reduced CV index values might predict the early stages of LV deterioration in this group.

Challenges and diagnosis of isolated left ventricular non-compaction: A case series of 4 patients with echocardiographic diagnosis of possible ILVNC

Isolated left ventricular non-compaction (ILVNC) is a rare, congenital, idiopathic cardiomyopathy that may present in adulthood. There is no true gold standard for the diagnosis of ILVNC. Two-dimensional echocardiography with colour Doppler is the modality of choice to diagnose the condition. However, the diagnosis should be confirmed with cardiac magnetic resonance imaging (CMRI), as well as either a positive family history, complications of ILVNC or confirmatory genetic testing.We describe the clinical and echocardiographic features in 4 patients, each with a possible diagnosis of ILVNC, in the setting of potential alternative aetiologies for heart failure. Approval to present these cases was obtained from the institutional ethics committee and the patients also provided consent. Sufficient transthoracic echocardiographic (TTE) evidence of ILVNC according to previously published criteria was found in all the cases, although it was not confirmed with CMRI.This case series highlights the importance of routine echocardiography in all patients who present with heart failure – irrespective of associated risk factors. We caution against over-diagnosis of ILVNC with TTE alone, and recommend the use of CMRI as a second-line diagnostic investigation. Screening of family members and prevention of complications of confirmed cases of ILVNC are important.

A rare distinct cardiomyopathy: Isolated left ventricular noncompaction

A rare distinct cardiomyopathy: Isolated left ventricular noncompaction

Efficacy of cardiac resynchronization therapy in patients with isolated ventricular noncompaction with dilated cardiomyopathy: a systematic review of the literature.

: This is a systematic review of current evidence regarding the efficacy of cardiac resynchronization therapy (CRT) on patients with dilated cardiomyopathy and isolated left ventricular noncompaction (IVNC). This systematic review was performed following Preferred Reporting Items for Systematic Reviews and Meta-analysis guidelines. Records were searched in Pubmed, Cochrane Library, Google Scholar, Biomed Central. We included only studies focused on ventricular noncompaction patients treated with CRT. Of 46 records screened, we included 14 studies involving a total of 70 patients. All studies showed a reduction of New York Heart Association class and an increase of the ejection fraction that ranges from 8 to 36% at follow-up after CRT. Analyzing the type of response to CRT, approximately 50% of the patients were classified as responders to the therapy and most of them were super-responders. In conclusion, CRT provides beneficial effects in terms of clinical status and left ventricular function on IVNC patients with heart failure. CRT responders seem to have a great left ventricular reverse remodeling supporting the theory that CRT is able to provide an additional benefit in the IVNC disease, improving the performance of IVNC segments, when paced.

Isolated left ventricular noncompaction: Report of a case from Ibadan, Nigeria

Isolated left ventricular noncompaction (ILVNC) is a form of cardiomyopathy caused by the intrauterine failure of the myocardium to compact (endomyocardial morphogenesis) resulting in multiple trabeculations of the left ventricular myocardium and deep intertrabecular recesses and thickened myocardium. The condition is relatively uncommon and is often complicated by heart failure, malignant arrhythmias, and thromboembolism. There have been a few reports of this condition from some parts of Africa (Djibouti, Sudan, and South Africa). To the best of our knowledge, there has not been previous report from Nigeria. The purpose of this paper is to report a case of ILVNC from Ibadan, Nigeria.

Quantification of left ventricular trabeculae using cardiac magnetic resonance imaging for the diagnosis of left ventricular non-compaction

Background Left ventricular non-compaction (LVNC) is an unclassified cardiomyopathy and there is no consensus in the diagnosis of LVNC. The aims of this study were to establish quantitative method to diagnose isolated LVNC (INC) using cardiac magnetic resonance (CMR) imaging and to suggest novel qualitative method to diagnose isolated LVNC. Methods This retrospective study included 145 subjects with moderate to severe trabeculation of LV myocardium [24 patients with isolated LVNC, 33 patients with non-isolated LVNC, 30 patients with dilated cardiomyopathy (DCM) with noncompaction, 27 patients with DCM and 31 healthy control subjects]. LVNC patients had to fulfill Petersen’s CMR criteria. LV ejection fraction, global LV volume, trabeculated LV volume, and number of segments with late gadolinium enhancement were measured. And most prominent noncompacted (NC), compacted (C), normal mid-septum, normal mid-lateral wall and apical trabeculation thickness on the end-diastolic frames of each long-axis slice was also measured. Results Results In the patients with isolated LVNC, the percentage of trabeculated LV volume was 1.4 times higher (42.6 ± 13.8) than in DCM (30.3 ± 14.3, p<0.0001) [YHC1], and 1.7 times higher than in controls (24.8 ± 7.1, p<0.0001). However, there were no significant differences between INC and DCMNC (47.1 ± 17.3, p = 0.210). And a value of percentage of trabeculated LV volume above 32% was predictive of isolated LVNC with a specificity of 90.3% (CI, 74.2-98.0%) and sensitivity of 79.2% (CI, 57.8-92.9%). A value of NC/septum over 1.1 was considered predictive for isolated LVNC with a specificity of 80.6% (CI, 62.5-92.5%) and sensitivity of 95.8% (CI, 78.9-99.9%). And a value of apex/C above 3.1 was considered predictive of isolated LVNC with a specificity of 93.5% (CI, 78.6-99.2%) and sensitivity of 87.5% (CI, 67.6-97.3%). Conclusions As a quantitative approach, a trabeculated LV volume above 32% of the total LV volume is diagnostic for isolated LVNC with high sensitivity and specificity. And as a qualitative approach, apex/C and NC/septum ratio could be useful for supplemental diagnostic criteria. Funding None.

Clinical usefulness of gated technetium-99m sestamibi myocardial perfusion single-photon emission computed tomography with phase analysis for the management of patients with isolated ventricular noncompaction

Gated Tc-99m sestamibi myocardial perfusion single-photon emission computed tomography (GMPS) with phase analysis provides information on myocardial perfusion, left ventricular (LV) function, and LV dyssynchrony. We present a case of isolated left ventricular noncompaction (IVNC) cardiomyopathy in which GMPS with phase analysis proved to be beneficial and reliable to monitor the long-term response to cardiac resynchronization therapy with defibrillator (CRT-D). The patient was an 84-year-old man with shortness of breath on minimal exertion (New York Heart Association class III) who had severe drug-refractory heart failure with hypotension and ventricular tachycardia. He was diagnosed with IVNC using echocardiography. At baseline, GMPS with phase analysis revealed a reduced ejection fraction (EF, 21%), large perfusion defects in the inferior and inferolateral walls, and severe LV dyssynchrony [histogram bandwidth (HBW) 120°]. Combination therapy with CRT-D and a titrated beta-blocker was initiated to induce LV reverse remodeling and reduce LV dyssynchrony. Two years after CRT-D implantation, GMPS with phase analysis showed marked improvement in LV function and LV dyssynchrony (EF 28%, HBW 36°). This case demonstrates that GMPS with phase analysis is an important and useful modality to evaluate LV function and LV dyssynchrony in IVNC patients undergoing CRT-D.<Learning objective: We experienced a rare case of heart failure with isolated left ventricular noncompaction (IVNC) treated with cardiac resynchronization therapy with defibrillator (CRT-D). We demonstrate that gated Tc-99m myocardial perfusion SPECT (GMPS) with phase analysis can simultaneously evaluate myocardial perfusion and left ventricular dyssynchrony to assess the indication and efficacy of CRT-D. This case demonstrates that GMPS with phase analysis is useful to monitor IVNC patients before and after CRT-D.>

Clinical Outcomes in Patients With Isolated Left Ventricular Noncompaction and Heart Failure

BackgroundWe prospectively evaluated long-term clinical outcomes of patients diagnosed with isolated left ventricular noncompaction (ILVNC) and heart failure from a sub-Saharan African population. Methods and ResultsPatients in this single-center study were followed at a tertiary care institution. Clinical follow-up was performed with the use of protocol-driven echocardiographic screening for ventricular thrombus every 4 months. Warfarin was maintained or initiated only if thrombus was detected with the use of echocardiography. Fifty-five patients were followed for 16.7 ± 5.9 (range 12–33) months. All individuals had left ventricular (LV) ejection fraction <50% (mean 29.6 ± 11.8%). Of the 55 patients, 7 (12.7%) died, and sudden cardiac death was the cause in 5 (71.4%). There were no differences in baseline clinical, echocardiographic, or electrocardiographic characteristics between survivors and nonsurvivors. Recurrent heart failure developed in 12 patients (21.8%); 1 patient developed a ventricular arrhythmia. No thromboembolic or major bleeding complications occurred in the 16 patients on warfarin; 1 episode of thromboembolism occurred in the 39 patients not on warfarin. Mean survival probability at 33 months was 0.64. ConclusionsSudden cardiac death was the most common cause of death in patients with ILVNC and heart failure. Recurrent heart failure occurred in 21.8% of patients. Development of LV thrombus and cardioembolism is uncommon in this population.

Abnormalities of myocardial perfusion and glucose metabolism in patients with isolated left ventricular non-compaction

BackgroundThe prevalence of myocardial perfusion and glucose metabolic abnormalities and their significance in patients with isolated left ventricular non-compaction (ILVNC) have not been well investigated. MethodsSeventeen ILVNC patients who underwent cardiac magnetic resonance (CMR) and 99mTc-sestamibi SPECT/fluorine-18 deoxyglucose (18F-FDG) PET imaging were included. Left ventricular non-compaction, regional wall motion abnormalities, left ventricular ejection fraction (LVEF), and delayed enhancement (DE) were estimated using CMR. Myocardial perfusion and metabolism were evaluated with SPECT/PET. ResultsNinety-five (32.9%) segments were considered non-compacted. DE was present in 52 (18.0%) segments and 10 (58.8%) patients. The rate of occurrence of DE was significantly higher in compacted segments than in non-compacted segments (22.7% vs 8.4%, P = .003). Myocardial perfusion abnormalities were present in 92 (31.8%) segments, of which 66 were perfusion/metabolism match and 26 were perfusion/metabolism mismatch. The rate of occurrence of perfusion abnormality was similar between compacted and non-compacted segments (32.0% vs 31.6%, P = .948), but it was significantly higher in segments with DE than in those without DE (51.9% vs 27.4%, P = .001). None of the imaging features alone (non-compaction, DE, perfusion abnormalities, match or mismatch) showed significant correlations with LVEF (all P > .05). ConclusionIn the current study, myocardial perfusion/metabolism mismatch and match were observed in both non-compacted and compacted myocardium in ILVNC patients. Further research is warranted to determine their pathologic and clinical significance.

Isolated left ventricular noncompaction: clinical profile and prognosis in 106 adult patients

This study was undertaken to evaluate the clinical course of isolated left ventricular noncompaction (ILVNC) and to identify the predictors for adverse outcomes in an adult cohort with ILVNC. Between March 2003 and April 2012, 106 adult patients diagnosed with ILVNC at Fuwai Hospital were included in this study. The medical history, electrocardiograms, and echocardiograms of these patients were retrospectively analyzed by chart review. Of these patients, 64 (60 %) were in New York Heart Association (NYHA) functional class III/IV and 84 (79 %) had systolic dysfunction (left ventricular ejection fraction (LVEF) <50 %). During a follow-up of 2.9 ± 2.1 years, 28 (26 %) patients died or underwent heart transplantation. The annual incidence of death or transplantation was 9.1 %. The determinants of death or heart transplantation included NYHA functional class III/IV (hazard ratio (HR) 4.52; 95 % confidence interval (CI) 1.57-13.04; P = 0.005), decreased left ventricular ejection fraction (HR 0.94; 95 % CI 0.90-0.97; P = 0.001), dilated left ventricular end-diastolic diameter (HR, 1.06; 95 % CI, 1.02-1.09; P = 0.001), increased left atrial diameter (HR 1.08; 95 % CI 1.03-1.14; P = 0.001), reduced systolic blood pressure (HR 0.96; 95 % CI 0.94-0.99; P = 0.003), the presence of pulmonary hypertension (HR 3.50; 95 % CI 1.63-7.51; P = 0.001), and right bundle branch block (HR 7.79; 95 % CI 2.56-23.76; P < 0.001). In conclusion, this study demonstrates that ILVNC is related to a high incidence of death or heart transplantation. Advanced heart failure, a dilated left heart with systolic dysfunction, reduced systolic blood pressure, pulmonary hypertension, and right bundle branch block predict adverse outcomes of ILVNC.

HEART's Original [症例] 10年の経過で心機能の増悪,正常化,増悪を呈した孤立性左室心筋緻密化障害の1例

HEART's Original [症例] 10年の経過で心機能の増悪,正常化,増悪を呈した孤立性左室心筋緻密化障害の1例

095: Segmental and global peak systolic longitudinal strain using speckle myocardial echocardiography in isolated left ventricular noncompaction. Description of 28 cases

Isolated left ventricular noncompaction (ILVNC) is a rare cardiomyopathy characterized by deep trabeculations in the ventricular wall which define recesses with the main chamber. The aim of our study was to compare segmental and global peak systolic longitudinal strain (pSSL) in ILVNC considering various left ventricular ejection fraction (LVEF). 28 patients (17-74 years old, mean age 46±16) with ILVNC were retrospectively enrolled and compared to 40 controls (group 1). Patients were divided into two groups according to LVEF group 2=LVEF>50% (n=15) and group 3 = LVEF<50% (n=13). pSSL was compared in each 17 left ventricular segments of the 3 groups. Number of non compacted segments was higher in group 3 than in group 2 (5.9±1.7 vs 4.6 ±1.3, p=0.008). Tissue Doppler Imaging E’ was lower in group 3 compared with group 1 (p<0.001) whereas E’ was not different between groups 1 and 2. Global pSSL (-15.75±4.8 and -22.18±1.9 p<0.001), basal mean (p<0.001), middle mean (p<0.001) and apex mean (p<0.001) were lower in patients with ILVNC compared to group 1. Basal mean (p=0.013), middle mean (p<0.001), apex mean (p<0.001) and global pSSL (-19.07±2.3 and -22.18±1.9 p<0.001) were lower in group 2 compared to controls. Basal pSSL of normally compacted basal segment in ILVNC were lower compared to basal pSSL in controls (septum p<0.001; lateral, p=0.009; inferior, p=0.008; anterior, p=0.009). There was a significant correlation between the number of non-compacted segments and the global pSSL (r=-0.418, p=0.027). Global left ventricular pSSL is reduced in patients with ILVNC even those with normal LVEF. Even in basal normally compacted segments, segmental correspondent pSSL is lower in patients with ILVNC compared to controls. This finding suggests that ILVNC is a diffuse left ventricular cardiomyopathy not only affected non compacted segments. Our results suggest that pSSL could be an early predictor of left ventricular systolic dysfunction in ILVNC.

Isolated Left Ventricular Noncompaction: What Do We Really Know?

Isolated left ventricular noncompaction (ILVNC) is a myocardial disorder thought to occur as a result of an arrest in embryogenesis. Recently, it has been classified as a genetic cardiomyopathy. Familial and sporadic forms of ILVNC have been documented. There is considerable genetic heterogeneity among familial forms, although sarcomeric mutations are the most common genetic mutations. Issues related to familial screening require careful consideration and future research. There is considerable controversy regarding several aspects relating to pathogenesis, diagnosis, and management of subjects with ILVNC. Differentiating some cases of ILVNC from normal variant myocardial architecture is challenging and requires future work. This review is intended to provide clinicians insight into several aspects of these challenging issues and offer some practical guidance in dealing with some of these difficult clinical scenarios.

Isolated Left Ventricular Noncompaction in Sub-Saharan Africa: A Clinical and Echocardiographic Perspective

Isolated left ventricular noncompaction (ILVNC) is a cardiomyopathy caused by intrauterine failure of the myocardium to compact. Common clinical complications are heart failure, arrhythmias, and cardioembolism. A paucity of data exists relating to clinical and echocardiographic features of ILVNC in Africans. This study is a single-center, prospective case-control study, whereby subjects attending a dedicated cardiomyopathy clinic were screened for and diagnosed with ILVNC, provided they had no other associated structural heart disease and fulfilled all the accompanying echocardiographic criteria: (1) end-systolic ratio of noncompacted layer to compacted layer >2, (2) presence of >3 prominent apical trabeculations, and (3) deep intertrabecular recesses that fill with blood from the ventricular cavity visualized using color Doppler ultrasound. Fifty-four subjects were identified, age 45.4±13.1 years (mean±SD), 95% confidence interval 3.6 to 10.2, 55.6% male, and 63.0% New York Health Association Class II, and prevalence of LVNC in our clinic was 6.9%, 95% confidence interval 3.6 to 10.2. Heart failure because of systolic dysfunction was the most common clinical presentation (53 subjects, 98.1%). Left ventricular end-diastolic diameter was 61.4±7.2 mm (mean±SD) and ejection fraction 26.7±11.9% (mean±SD). Common sites of noncompaction were the apical (100%), midinferior (74.1%), and midlateral (64.8%) walls. Right ventricular noncompaction occurred in 12 subjects (22.2%). Pulmonary hypertension was documented in 45 cases (83.3%). Right ventricular dilation was noted in 40 subjects (74.1%), while right ventricular function was depressed in 32 (59.3%). Tricuspid S' was 9.6±2.8 cm/s (mean±SD). No echocardiographic features suggestive of ILVNC were noted in a healthy control group of African descent. ILVNC in patients of African descent can be characterized by biventricular abnormality and pulmonary hypertension, in addition to isolated left-sided abnormality.

Comparison of cardiac magnetic resonance imaging features of isolated left ventricular non-compaction in adults versus dilated cardiomyopathy in adults

To compare cardiac magnetic resonance imaging (MRI) features between isolated left ventricular non-compaction (IVNC) and dilated cardiomyopathy (DCM) in adults. A consecutive series of 50 patients with IVNC from a single institution were reviewed. During the same period, 50 patients with DCM who had prominent trabeculations, who were matched for age, gender, and body surface area, were prospectively included. Left ventricular (LV) morphology and function were assessed using cardiac MRI. Compared with patients with DCM, patients with IVNC had a significantly lower LV sphericity index and end-diastolic volume index (LVEDVI) and a greater LV ejection fraction (LVEF), number of trabeculated segments, and ratio of non-compacted to compacted myocardium (NC/C ratio). There were no significant differences in stroke volume index, cardiac output, and cardiac index between the two patient groups. In patients with IVNC, the number of trabeculated segments and the NC/C ratio correlated positively with LVEDVI (r=0.626 and r=0.559, respectively) and negatively with LVEF (r=-0.647 and r=-0.521, respectively, p<0.001 for all). In patients with DCM, the number of non-compacted segments and the NC/C ratio had no correlation with either the LVEDVI (r=-0.082 and r=-0.135, respectively) or the LVEF (r=0.097 and r=0.205, respectively). There are demonstrable morphological and functional differences between IVNC and DCM at LV assessment using cardiac MRI. The occurrence of trabeculated myocardium might be due to a different pathophysiological mechanism.

Electrocardiographic Findings at Initial Diagnosis in Children with Isolated Left Ventricular Noncompaction

The aim of this study was to comprehensively evaluate electrocardiographic (ECG) findings of isolated left ventricular noncompaction (IVNC) patients at initial diagnosis and to explore the correlation between them and the clinical, echocardiographic, and magnetic resonance imaging (MRI) findings. Twenty-three patients diagnosed with IVNC by echocardiography and cardiac MRI between January 2006 and June 2010 were enrolled in this study. The patients were examined with standard ECG and 24-hour Holter ECG. For comparison purpose, ECGs of 50 healthy children of similar ages and demographic characteristics were taken. In 87% of patients, ECG abnormalities were found. The most frequently seen ECG findings were left ventricular hypertrophy, ST-segment depression, and negative T wave related to abnormal repolarization particularly in DII, DIII, and V(4-6) leads, as well as prolonged PR and QTc intervals. No ECG features or patterns were found that were specific to the disease. In contrast to adult patients, while no intraventricular conduction defects (particularly in the left bundle brach) were found in any of our patients, 13% had considerable bradycardia and one required a pacemaker. The Holter ECG recordings showed supraventricular tachycardia attacks in two patients and a short ventricular tachycardia attack in one. Patients whose echocardiograms and MRI showed left ventricular systolic dysfunction and left ventricular dilatation had signs of left ventricular hypertrophy and repolarization abnormality on their ECGs, but there was no significant difference in PR, QRS, and QTc intervals. Regardless of how frequently left ventricular hypertrophy and repolarization abnormalities are found on IVNC patients' initial ECGs, we think that they are not unique to the disease but are related to the severity of the cardiomyopathy.

Long-Term Follow-up of Patients With Isolated Left Ventricular Noncompaction - Role of Electrocardiography in Predicting Poor Outcome -

Abnormal baseline electrocardiograms (ECGs) are common in patients with isolated left ventricular noncompaction (IVNC). Whether certain electrocardiographic parameters are associated with a poor clinical outcome, however, remains elusive. The present study was therefore designed to comprehensively assess the predictive value of baseline ECG findings in patients newly diagnosed with IVNC. 74 patients diagnosed with IVNC were included in the analysis. During follow-up, 8 patients (11%) died of a cardiovascular cause or underwent heart transplantation (primary outcome measure). On univariate analysis, several variables, including repolarization abnormalities (ST segment elevation/depression, T-wave inversion) in the inferior leads (5-year estimator: 67.1 ± 10.7% vs. 98 ± 2.2%; P = 0.001), an increase in PQ- (hazard ratio (HR) 1.032, P=0.004) and QTc-duration (HR 1.037, P=0.001), were predictive of cardiovascular death or heart transplantation. On multivariate analysis, only PQ- and QTc-duration and the presence of repolarization abnormalities in the inferior leads remained significantly predictive of a poor outcome. PQ duration, QTc duration, and repolarization abnormalities in the inferior leads are independently predictive of a poor prognosis in IVNC. Further prospective studies are required to conclusively investigate the usefulness of baseline ECG parameters for risk stratification in patients with IVNC.

Isolated Left Ventricular Noncompaction in a Case of Sotos Syndrome: A Casual or Causal Link?

A 16-year-old boy affected by Sotos syndrome was referred to our clinic for cardiac evaluation in order to play noncompetitive sport. Physical examination was negative for major cardiac abnormalities and rest electrocardiogram detected only minor repolarization anomalies. Transthoracic echocardiography showed left ventricular wall thickening and apical trabeculations with deep intertrabecular recesses, fulfilling criteria for isolated left ventricular noncompaction (ILVNC). Some sporadic forms of ILVNC are reported to be caused by a mutation on CSX gene, mapping on chromosome 5q35. To our knowledge, this is the first report of a patient affected simultaneously by Sotos syndrome and ILVNC.