Quantitative metrics of the LV trabeculated layer by cardiac CT and cardiac MRI in patients with suspected noncompaction cardiomyopathy.

OBJECTIVES: To compare cardiac computed tomography (CCT) and cardiac magnetic resonance (CMR) for the quantitative assessment of the left ventricular (LV) trabeculated layer in patients with suspected noncompaction cardiomyopathy (NCCM). MATERIALS AND METHODS: Subjects with LV excessive trabeculation who underwent both CMR and CCT imaging as part of the prospective international multicenter NONCOMPACT clinical study were included. For each subject, short-axis CCT and CMR slices were matched. Four quantitative metrics were estimated: 1D noncompacted-to-compacted ratio (NCC), trabecular-to-myocardial area ratio (TMA), trabecular-to-endocardial cavity area ratio (TCA), and trabecular-to-myocardial volume ratio (TMV). In 20 subjects, end-diastolic and mid-diastolic CCT images were compared for the quantification of the trabeculated layer. Relationships between the metrics were investigated using linear regression models and Bland-Altman analyses. RESULTS: Forty-eight subjects (49.9 ± 12.8 years; 28 female) were included in this study. NCC was moderately correlated (r = 0.62), TMA and TMV were strongly correlated (r = 0.78 and 0.78), and TCA had excellent correlation (r = 0.92) between CMR and CCT, with an underestimation bias from CCT of 0.3 units, and 5.1, 4.8, and 5.4 percent-points for the 4 metrics, respectively. TMA, TCA, and TMV had excellent correlations (r = 0.93, 0.96, 0.94) and low biases (- 3.8, 0.8, - 3.8 percent-points) between the end-diastolic and mid-diastolic CCT images. CONCLUSIONS: TMA, TCA, and TMV metrics of the LV trabeculated layer in patients with suspected NCCM demonstrated high concordance between CCT and CMR images. TMA and TCA were highly reproducible and demonstrated minimal differences between mid-diastolic and end-diastolic CCT images. CLINICAL RELEVANCE STATEMENT: The results indicate similarity of CCT to CMR for quantifying the LV trabeculated layer, and the small differences in quantification between end-diastole and mid-diastole demonstrate the potential for quantifying the LV trabeculated layer from clinically performed coronary CT angiograms. KEY POINTS: • Data on cardiac CT for quantifying the left ventricular trabeculated layer are limited. • Cardiac CT yielded highly reproducible metrics of the left ventricular trabeculated layer that correlated well with metrics defined by cardiac MR. • Cardiac CT appears to be equivalent to cardiac MR for the quantification of the left ventricular trabeculated layer.

When amiodarone-induced thyroiditis meets cardiomyopathy with excessive trabeculation: a case report.

Introduction: Amiodarone is a potent antiarrhythmic medication used to treat life-threatening ventricular arrhythmias; however, its well-established adverse effect is a thyroid disorder. Amiodarone-induced thyroiditis (AIT), a clinical entity involving two types with different etiopathology and treatment approaches, may occur at the beginning or even several years after amiodarone treatment discontinuation. The toxicity profile of amiodarone becomes especially important in young patients with lifelong cardiac disorders, which are often refractory to other antiarrhythmic drugs. Herein, we report the first case of non-sustained ventricular tachycardia (NSVT), an unusual presentation of type II AIT, in a young male patient who was previously diagnosed with left ventricular cardiomyopathy with excessive trabeculation. Case report: A 36-year-old male non-athlete presented with tiredness during regular follow-up. Continuous electrocardiographic monitoring (cECG) revealed NSVT, whereas echocardiography and cardiac magnetic resonance imaging detected discrete structural and functional changes that could not fully explain the observed cECG report. Conversely, an unmeasurably low thyrotropin level on admission and previous exposure to amiodarone pointed the diagnostic pathway in the direction of the thyroid gland. Elevated free thyroxine and undetectable autoantibody titers with unremarkable sonographic findings raised clinical suspicion of type II AIT. Scintigraphic imaging with 99mTc-2-methoxyisobutylisonitrile (sestamibi) revealed decreased thyroid uptake; hence, prednisone was introduced for treatment. Clear improvements in both biochemical and electrocardiographic parameters were observed after immunomodulatory treatment of type II AIT in this young patient with cardiomyopathy and excessive trabeculation. Conclusion: Treatment of reversible causes of cardiac rhythm abnormalities such as type II AIT should be considered before choosing other treatment modalities, particularly in patients with structural cardiac disorders. The importance of a multidisciplinary approach in complex cases such as the one reported, thus, cannot be emphasized enough.

Genetic Basis of Left Ventricular Noncompaction.

BACKGROUND: Left ventricular noncompaction (LVNC) is the third most common pediatric cardiomyopathy characterized by a thinned myocardium and prominent trabeculations. Next-generation genetic testing has led to a rapid increase in the number of genes reported to be associated with LVNC, but we still have little understanding of its pathogenesis. We sought to grade the strength of the gene-disease relationship for all genes reported to be associated with LVNC and identify molecular pathways that could be implicated. METHODS: Following a systematic PubMed review, all genes identified with LVNC were graded using a validated, semi-quantitative system based on all published genetic and experimental evidence created by the Clinical Genome Resource (ClinGen). Genetic pathway analysis identified molecular processes and pathways associated with LVNC. RESULTS: We identified 189 genes associated with LVNC: 11 (6%) were classified as definitive, 21 (11%) were classified as moderate, and 140 (74%) were classified as limited, but 17 (9%) were classified as no evidence. Of the 32 genes classified as definitive or moderate, the most common gene functions were sarcomere function (n=11; 34%), transcriptional/translational regulator (n=6; 19%), mitochondrial function (n=3; 9%), and cytoskeletal protein (n=3; 9%). Furthermore, 18 (56%) genes were implicated in noncardiac syndromic presentations. Lastly, 3 genetic pathways (cardiomyocyte differentiation via BMP receptors, factors promoting cardiogenesis in vertebrates, and Notch signaling) were found to be unique to LVNC and not overlap with pathways identified in dilated cardiomyopathy and hypertrophic cardiomyopathy. CONCLUSIONS: LVNC is a genetically heterogeneous cardiomyopathy. Distinct from dilated or hypertrophic cardiomyopathies, LVNC appears to arise from abnormal developmental processes.

Continuous Arrhythmia Monitoring in Pediatric and Adult Patients With Left Ventricular Noncompaction.

Patients with left ventricular noncompaction (LVNC) are at risk of clinically significant arrhythmias and sudden death. We evaluated whether implantable loop recorders could detect significant arrhythmias that might be missed in these patients during annual Holter monitoring. Selected pediatric and adult patients with LVNC who consented to implantable loop recorder placement were monitored for 3 years (study duration, 10 April 2014-9 December 2019). Fourteen subjects were included (age range, 6.5-36.4 yr; 8 males). Of 13 patients who remained after one device extrusion, one underwent implantable cardioverter-defibrillator placement. Four patients (31%) had significant arrhythmias: atrial tachycardia (n=2), nonsustained ventricular tachycardia (n=1), and atrial fibrillation (n=1). All 4 events were clinically asymptomatic and not associated with left ventricular ejection fraction. In addition, a high frequency of benign arrhythmic patterns was detected. Implantable loop recorders enable continuous, long-term detection of important subclinical arrhythmias in selected patients who have LVNC. These devices may prove to be most valuable in patients who have LVNC and moderate or greater ventricular dysfunction.

Correlation between left ventricular fractal dimension and impaired strain assessed by cardiac MRI feature tracking in patients with left ventricular noncompaction and normal left ventricular ejection fraction.

OBJECTIVES: To investigate the correlation between the extent of excessive trabeculation assessed by fractal dimension (FD) and myocardial contractility assessed by cardiac MRI feature tracking in patients with left ventricular noncompaction (LVNC) and normal left ventricular ejection fraction (LVEF). METHODS: Forty-one LVNC patients with normal LVEF (≥ 50%) and 41 healthy controls were retrospectively included. All patients fulfilled three available diagnostic criteria on MRI. Cardiac MRI feature tracking was performed on cine images to determine left ventricular (LV) peak strains in three directions: global radial strain (GRS), global circumferential strain (GCS), and global longitudinal strain (GLS). The complexity of excessive trabeculation was quantified by fractal analysis on short-axis cine stacks. RESULTS: Compared with controls, patients with LVNC had impaired GRS, GCS, and GLS (all p < 0.05). The global, maximal, and regional FD values of the LVNC population were all significantly higher than those of the controls (all p < 0.05). Global FD was positively correlated with the end-diastolic volume index, end-systolic volume index, and stroke volume index (r = 0.483, 0.505, and 0.335, respectively, all p < 0.05), but negatively correlated with GRS and GCS (r = - 0.458 and 0.508, respectively, both p < 0.001). Moreover, apical FD was also weakly associated with LVEF and GLS (r = - 0.249 and 0.252, respectively, both p < 0.05). CONCLUSION: In patients with LVNC, LV systolic dysfunction was detected early by cardiac MRI feature tracking despite the presence of normal LVEF and was associated with excessive trabecular complexity assessed by FD. KEY POINTS: • Left ventricular global strain was already impaired in patients with extremely prominent excessive trabeculation but normal left ventricular ejection fraction. • An increased fractal dimension was associated with impaired deformation in left ventricular noncompaction.

Clinical characteristics and pregnancy outcomes of pregnant women with left ventricular non-compaction / 中华妇产科杂志

Objective:To investigate the clinical characteristics and pregnancy outcomes in pregnant women with left ventricular non-compaction (LVNC).Methods:The clinical data of seven pregnant women with LVNC from January 2011 to December 2021 in Beijing Anzhen Hospital,Capital Medical University were retrospectively analyzed, including age, gestational age of symptom first occured, LVNC history, clinical symptoms, New York Heart Association (NYHA) cardiac function class, echocardiography, blood brain natriuretic peptide (BNP), treatment and the maternal and fetal outcomes.Results:Five cases were diagnosed before pregnancy, of which there were three women with medication; one case diagnosed in the month of pregnancy; one case diagnosed at 36 weeks of gestation. NYHA cardiac function was grade Ⅰ in four cases and grade Ⅱ in three cases before or during the first trimester of pregnancy. Of the five pregnant women who underwent echocardiography, there were one case of left ventricular insufficiency, three cases of mild left ventricular dysfunction and one case of normal left ventricular function before or during the first trimester of pregnancy. Of the five pregnant women to the second and third trimester of pregnancy, there were one case of grade Ⅳ, one case of grade Ⅲ, two cases of grade Ⅱ-Ⅲ and one case of grade Ⅱ in NYHA class ; three cases of left ventricular insufficiency, two cases of normal left ventricular function by echocardiography four cases had cardiac symptoms at 15-24 weeks of gestation and were treated with medication. In four cases, blood BNP increased to 214-1 197 ng/L during pregnancy, and were 89-106 ng/L after termination of pregnancy. There were 4 cases with arrhythmia. Indications for termination of pregnancy: LVNC complicated with heart failure in two cases, LVNC complicated with decreased cardiac function and threatened preterm birth in one case, complicated with pregnancy at full term in two cases, LVNC complicated with severe pulmonary hypertension in one case, and left ventricular dysfunction in one case. Cesarean section in four cases in the third-trimester, in one case in the second-trimester, and forceps curettage in two cases were taken. Two full-term infants,two preterm infants were born without LVNC.Conclusions:Women diagnosed with LVNC and low left ventricular ejection fraction before pregnancy are more prone to decreased cardiac function during pregnancy. Carrying out pregnancy risk assessment and strengthening the multi-disciplinary team management of high risk factors in pregnancy are conducive to achieve good pregnancy outcomes.

Mirror syndrome with noncompaction cardiomyopathy in the mother and fetus. Case report / Síndrome en espejo con miocardiopatía no compactada en la madre y el feto. Reporte de caso

Objective: To report the case of a pregnant woman with mirror syndrome associated with non-compaction cardiomyopathy in the mother and the fetus, in which antenatal medical treatment provided to the mother resulted in a favorable perinatal maternal outcome. Case presentation: A 16-year old primigravida with 33 weeks of gestation referred from a Level I institution to a private Level IV center in Medellín, Colombia, because of a finding of fetal hydrops on obstetric ultrasound. During hospitalization, the patient showed clinical and ultrasonographic signs of heart failure (dyspnea, edema and hypoxemia), with the diagnosis of hydrops fetalis (mirror syndrome) also confirmed. Diuretic treatment with furosemide was initiated in the mother, with subsequent improvement of the maternal condition as well as of the fetal edema. During the subacute postpartum period in the hospital, the presence of non-compaction cardiomyopathy was confirmed on cardiac nuclear magnetic resonance imaging in both the mother and the newborn. After discharge in adequated condition, they were included in the cardiovascular follow-up program for heart failure and congenital heart disease, respectively. Conclusion: A case of mirror syndrome associated with maternal and fetal non-compaction cardiomyopathy is presented. There is a limited number of reports on mirror syndrome due to cardiac anomalies (maternal and fetal), with weak treatment descriptions, pointing to the need for research in this area. It would be important to consider the diagnosis of non-compaction cardiomyopathy in fetuses with hydrops unrelated to isoimmunization or cardiac dysfunction, and approach these cases from a multi-disciplinary perspective.

Objetivo: reportar el caso de una gestante con síndrome en espejo asociada a miocardiopatía no compactada, tanto en la madre como el feto, en los que el tratamiento médico antenatal en la madre llevó a un resultado materno perinatal favorable. Presentación del caso: se describe el caso de una primigestante de 16 años, con 33 semanas de embarazo, remitida desde una institución de primer nivel de atención a una institución privada de cuarto nivel en la ciudad de Medellín, Colombia, por presentar feto con hidropesía en ultrasonido obstétrico de control. Durante la hospitalización, la paciente presentó signos clínicos y ecocardiográficos de falla cardiaca (disnea, edema e hipoxemia), a la vez que se confirmó el diagnóstico de Hydrops fetalis (síndrome en espejo). Se instauró tratamiento diurético con furosemida en la madre, logrando mejoría del cuadro materno y del edema fetal. En el puerperio mediato hospitalario se confirmaron la presencia de miocardiopatía no compactada en la resonancia magnética nuclear cardiaca, tanto de la madre como del recién nacido. Ambos egresaron en adecuadas condiciones y fueron vinculados al programa de seguimiento cardiovascular: falla cardiaca y de cardiopatía congénitas, respectivamente. Conclusión: se presenta un caso de síndrome en espejo asociado a miocardiopatía no compactada materna y fetal. Es limitado el número de reportes de síndrome en espejo por anomalías cardiacas (maternas y fetales) y pobre la descripción de los tratamientos realizados que surgen como temas a investigar. Sería importante considerar el diagnóstico de MNC en fetos con hidropesía no asociados a isoinmunización y con disfunción cardiaca, así como su atención por equipos multidisciplinarios.

Síndrome en espejo con miocardiopatía no compactada en la madre y el feto. Reporte de caso / Mirror syndrome with noncompaction cardiomyopathy in the mother and fetus. Case report

Objetivo: reportar el caso de una gestante con síndrome en espejo asociada a miocardiopatía no compactada (MNC), tanto en la madre como el feto, en los que el tratamiento médico antenatal en la madre llevó a un resultado materno perinatal favorable. Presentación del caso: se describe el caso de una primigestante de 16 años, con 33 semanas de embarazo, remitida desde una institución de primer nivel de atención a una institución privada de cuarto nivel en la ciudad de Medellín, Colombia, por presentar feto con hidropesía en ultrasonido obstétrico de control. Durante la hospitalización, la paciente presentó signos clínicos y ecocardiográficos de falla cardiaca (disnea, edema e hipoxemia), a la vez que se confirmó el diagnóstico de Hydrops fetalis (síndrome en espejo). Se instauró tratamiento diurético con furosemida en la madre, logrando mejoría del cuadro materno y del edema fetal. En el puerperio mediato hospitalario se confirmaron la presencia de miocardiopatía no compactada en la resonancia magnética nuclear cardiaca, tanto de la madre como del recién nacido. Ambos egresaron en adecuadas condiciones y fueron vinculados al programa de seguimiento cardiovascular: falla cardiaca y de cardiopatía congénitas, respectivamente. Conclusión: se presenta un caso de síndrome en espejo asociado a miocardiopatía no compactada materna y fetal. Es limitado el número de reportes de síndrome en espejo por anomalías cardiacas (maternas y fetales) y pobre la descripción de los tratamientos realizados que surgen como temas a investigar. Sería importante considerar el diagnóstico de MNC en fetos con hidropesía no asociados a isoinmunización y con disfunción cardiaca, así como su atención por equipos multidisciplinarios.

ABSTRACT Objective: To report the case of a pregnant woman with mirror syndrome associated with noncompaction cardiomyopathy in the mother and the fetus, in which antenatal medical treatment provided to the mother resulted in a favorable perinatal maternal outcome. Case presentation: A 16-year old primigravida with 33 weeks of gestation referred from a Level I institution to a private Level IV center in Medellín, Colombia, because of a finding of fetal hydrops on obstetric ultrasound. During hospitalization, the patient showed clinical and ultrasonographic signs of heart failure (dyspnea, edema and hypoxemia), with the diagnosis of hydrops fetalis (mirror syndrome) also confirmed. Diuretic treatment with furosemide was initiated in the mother, with subsequent improvement of the maternal condition as well as of the fetal edema. During the subacute postpartum period in the hospital, the presence of non-compaction cardiomyopathy was confirmed on cardiac nuclear magnetic resonance imaging in both the mother and the newborn. After discharge in adequated condition, they were included in the cardiovascular follow-up program for heart failure and congenital heart disease, respectively. Conclusion: A case of mirror syndrome associated with maternal and fetal non-compaction cardiomyopathy is presented. There is a limited number of reports on mirror syndrome due to cardiac anomalies (maternal and fetal), with weak treatment descriptions, pointing to the need for research in this area. It would be important to consider the diagnosis of non-compaction cardiomyopathy in fetuses with hydrops unrelated to isoimmunization or cardiac dysfunction and approach these cases from a multidisciplinary perspective.

Effect of trabeculated myocardial mass on left ventricle global and regional functions in noncompaction cardiomyopathy.

BACKGROUND: Left ventricular (LV) noncompaction cardiomyopathy is a rare cardiomyopathic subtype that has been recognized in recent years and is being diagnosed at an increased rate. There is no consensus regarding the diagnosis of the disease, and increased trabeculation rates that meet the existing diagnostic criteria may even be present in healthy asymptomatic people. This indicates that differentiating criteria for diagnosis are needed. AIM: To examine the increase in myocardial trabeculation and the change in left ventricular global and regional functions. METHODS: This retrospective study included 65 patients (28 females, 37 males) diagnosed with LV noncompaction cardiomyopathy who underwent cardiac magnetic resonance imaging between January 2011 and August 2016 and had a noncompacted/compacted myocardial thickness ratio of over 2.3 in more than one segment in the left ventricle. The distribution and ratios of trabeculations in apical, midventricular, and basal regions were examined in short-axis images obtained from cardiac magnetic resonance. In addition, by using short-axis cine images, regional ejection fraction (EF) and global EF were calculated using the Simpson method in the left ventricle at apical, basal, and midventricular levels. RESULTS: While the number of trabeculated segments were similar at the apical (3.2 ± 1.0) and midventricular levels, a statistically significant level of involvement was not observed at the basal level (0.4 ± 0.9) (P > 0.05). The highest noncompacted/compacted (trabeculation) ratio was observed at the apical level (3.9 ± 1.4), while this ratio was higher at the anterior (59%-89.4%) and lateral (62%-84.8%) segments (P > 0.05). Global EF was positively correlated with apical, midventricular, and basal regional EF (P < 0.05). However, there was no significant correlation between regional EF and the number of trabeculated segments or trabeculation ratio in all three regions; nor was there a significant correlation between regional EF and the number of trabeculated segments or trabeculation ratio in the entire LV (P > 0.05). CONCLUSION: No global or regional relationship was observed between LV dysfunction and trabeculation rate or the number of trabeculated segments. This limits the usefulness of change in LV functions in the differentiation between normal and pathological trabeculation.

Diagnóstico prenatal de no compactación del miocardio ventricular: presentación de un caso / Prenatal diagnosis of noncompaction of the ventricular myocardium: A case report / Prenatal diagnosis of noncompaction of the ventricular myocardium: A case report

RESUMEN El 'miocardio esponjoso' o cardiopatía no compactada es una rara miocardiopatía congénita de etiología desconocida, que resulta de un fallo en la embriogénesis en el proceso evolutivo de la trabeculación miocárdica normal. Los hallazgos ecocardiográficos característicos de esta enfermedad consisten en múltiples trabeculaciones miocárdicas y profundos recesos intertrabeculares que se comunican con la cavidad ventricular izquierda, derecha o ambas. Se presenta un caso de esta miocardiopatía que afecta el ventrículo derecho, en feto de 31 semanas de gestación, cuya madre era portadora asintomática de dicha patología. Se presenta las imágenes ecográficas prenatales, incluyendo la evaluación mediante la técnica de deformación miocárdica (strain), haciendo énfasis en la importancia de la historia clínica del paciente, las posibles etiologías y el diagnóstico diferencial con otras entidades.

ABSTRACT "Spongy cardiomyopathy" or noncompaction heart disease is a rare congenital cardiomyopathy of unknown etiology which results from a failure in embryogenesis in the evolutionary process of normal myocardial trabeculation. The characteristic echocardiographic findings of this disease consist of multiple myocardial trabeculations and deep intertrabecular recesses that communicate with the left or right ventricular cavity or both. We present a case of this cardiomyopathy that affects the right ventricle, in a fetus of 31 weeks gestation, whose mother was an asymptomatic carrier of said pathology. Prenatal ultrasound images are presented, including evaluation using the strain (myocardial deformation) technique, emphasizing the importance of the patient's medical history, possible etiologies and differential diagnosis with other entities.

Left Ventricular Noncompaction Detected by Cardiac Magnetic Resonance Screening: A Reexamination of Diagnostic Criteria.

In a previous cross-sectional screening study of 5,169 middle and high school students (mean age, 13.1 ± 1.78 yr) in which we estimated the prevalence of high-risk cardiovascular conditions associated with sudden cardiac death, we incidentally detected by cardiac magnetic resonance (CMR) 959 cases (18.6%) of left ventricular noncompaction (LVNC) that met the Petersen diagnostic criterion (noncompaction:compaction ratio >2.3). Short-axis CMR images were available for 511 of these cases (the Short-Axis Study Set). To determine how many of those cases were truly abnormal, we analyzed the short-axis images in terms of LV structural and functional variables and applied 3 published diagnostic criteria besides the Petersen criterion to our findings. The estimated prevalences were 17.5% based on trabeculated LV mass (Jacquier criterion), 7.4% based on trabeculated LV volume (Choi criterion), and 1.3% based on trabeculated LV mass and distribution (Grothoff criterion). Absent longitudinal clinical outcomes data or accepted diagnostic standards, our analysis of the screening data from the Short-Axis Study Set did not definitively differentiate normal from pathologic cases. However, it does suggest that many of the cases might be normal anatomic variants. It also suggests that cases marked by pathologically excessive LV trabeculation, even if asymptomatic, might involve unsustainable physiologic disadvantages that increase the risk of LV dysfunction, pathologic remodeling, arrhythmias, or mural thrombi. These disadvantages may escape detection, particularly in children developing from prepubescence through adolescence. Longitudinal follow-up of suspected LVNC cases to ascertain their natural history and clinical outcome is warranted.

Cardiovascular magnetic resonance based diagnosis of left ventricular non-compaction cardiomyopathy: impact of cine bSSFP strain analysis.

BACKGROUND: Investigation of the myocardial strain characteristics of the left ventricular non-compaction (LVNC) phenotype with cardiovascular magnetic resonance (CMR) feature tracking. METHODS: CMR cine balanced steady-state free precession data sets of 59 retrospectively identified LVNC phenotype patients (40 years, IQR: 28-50 years; 51% male) and 36 healthy subjects (39 years, IQR: 30-47 years; 44% male) were evaluated for LV volumes, systolic function and mass. Hypertrabeculation in patients and healthy subjects was evaluated against established CMR diagnostic criteria. Global circumferential strain (GCS), global radial strain (GRS) and global longitudinal strain (GLS) were evaluated with feature-tracking software. Subgroup analyses were performed in patients (n = 25) and healthy subjects (n = 34) with normal LV volumetrics, and with healthy subjects (n = 18) meeting at least one LVNC diagnostic criteria. RESULTS: All LVNC phenotype patients, as well as a significant proportion of healthy subjects, met morphology-based CMR diagnostic criteria: non-compacted (NC): compacted myocardial diameter ratio > 2.3 (100% vs. 19.4%), NC mass > 20% (100% vs. 44.4%) and > 25% (100% vs. 13.9%), and NC mass indexed to body surface area > 15 g/m2 (100% vs. 41.7%). LVNC phenotype patients demonstrated reduced GRS (26.4% vs. 37.1%; p < 0.001), GCS (- 16.5% vs. -20.5%; p < 0.001) and GLS (- 14.6% vs. -17.1%; p < 0.001) compared to healthy subjects, with statistically significant differences persisting on subgroup comparisons of LVNC phenotype patients with healthy subjects meeting diagnostic criteria. GCS also demonstrated independent and incremental diagnostic value beyond each of the morphology-based CMR diagnostic criteria. CONCLUSIONS: LVNC phenotype patients demonstrate impaired strain by CMR feature tracking, also present on comparison of subjects with normal LV volumetrics meeting diagnostic criteria. The high proportion of healthy subjects meeting morphology-based CMR diagnostic criteria emphasizes the important potential complementary diagnostic value of strain in differentiating LVNC from physiologic hypertrabeculation.

Unconventional 2:1 Ventricular Pacing in a Neonate with Congenital Heart Block and Biventricular Noncompaction.

Congenital complete heart block with concomitant biventricular noncompaction cardiomyopathy has been reported once previously. Although not universal, when restrictive physiology is present, impaired diastolic filling may pose a distinct challenge to pacing during the neonatal period. We present the case of a neonate with congenital complete heart block and biventricular noncompaction that resulted in severe diastolic dysfunction and atrioventricular dyssynchrony. We intentionally used 2:1 ventricular pacing to provide atrioventricular synchrony with every paced beat, and this resulted in hemodynamic and clinical improvement. This unconventional pacing technique may be beneficial in other neonates who have complete heart block and diastolic dysfunction.

Isolated Non-Compacted Myocardium: Should We Consider the Presence of Extrasystoles as the Initial Manifestation?

Ventricular non-compaction occurs due to failure in myocardial morphogenesis during the fetal period. Patients can have heart failure, as well as systemic complications due to thromboembolism and cardiac arrhythmias. Early diagnosis is essential. We present the case of an asymptomatic 49-year-old woman who initially manifested ventricular extrasystoles and heart failure with reduced ejection fraction and a myocardial noncompaction diagnosis

A rare case of Ebstein's anomaly with left ventricular noncompaction.

Ebstein's anomaly is a rare congenital cardiac disease characterized by apical displacement of the tricuspid valve with decreased right ventricular function. Left ventricular noncompaction is a cardiomyopathy characterized by the presence of numerous and prominent trabeculations together with deep intertrabecular recesses in a portion of the ventricular wall, principally at the lateral and apical level. We describe the very rare case of a patient living for 78 years with a benign form of Ebstein's anomaly and left ventricular noncompaction cardiomyopathy with slightly reduced systolic function.

Left ventricular noncompaction in a Para athlete / Miocárdio não compactado em paratleta

ABSTRACT The left ventricular noncompaction is a congenital cardiomyopathy characterized by the presence of abnormal trabeculations in the left ventricle. The present study describes the case of a 14-year-old female Para athlete, who plays goalball. She was asymptomatic, with history of congenital nystagmus and mild visual impairment, who presented nonspecific electrocardiographic abnormalities during pre-competition screening. Cardiac magnetic resonance imaging showed left ventricular non-compaction (non-compacted to compacted layer ratio equal to 2.5) and mild biventricular systolic dysfunction. Initially, the patient was excluded from sports participation and clinical follow-up was performed every three months. Patient remained asymptomatic during the one-year follow-up, with no history of unexplained syncope, marked impairment of systolic function or significant ventricular arrhythmias at the exercise stress test. Finally, she was released for competitive goalball participation and clinical follow-up was continued every 6 months. There is no consensus regarding the eligibility criteria for sports participation in cases of left ventricular non-compaction. Thus, it is prudent to individualize the decision regarding practice of sports, as well as to consider participation in competitive sports for asymptomatic individuals and with no disease repercussions.

RESUMO O miocárdio não compactado é uma cardiomiopatia congênita caracterizada pela presença de trabeculações anormais no ventrículo esquerdo. O presente estudo descreve o caso de uma paratleta de goalball, 14 anos, sexo feminino, assintomática, com história pessoal de nistagmo congênito e leve deficiência visual, que apresentou alterações eletrocardiográficas inespecíficas durante avaliação pré-participação. A ressonância magnética cardíaca evidenciou presença de não compactação miocárdica (relação entre camada não compactada/camada compactada igual a 2,5) e disfunção sistólica biventricular leve. Inicialmente, a paciente foi afastada da prática de esportes, e o seguimento clínico foi realizado a cada 3 meses. A paciente permaneceu assintomática durante o período de 1 ano de seguimento, sem história de síncope inexplicada, comprometimento significativo da função sistólica ou taquiarritmias ventriculares importantes ao teste de esforço. Por fim, ela foi liberada para prática competitiva de goalball, e o seguimento clínico foi mantido a cada 6 meses. Não há consenso quanto aos critérios de elegibilidade para a prática esportiva nos casos de miocárdio não-compactado. Assim, é prudente individualizar a decisão quanto a prática esportiva, bem como considerar a participação em esportes competitivos para indivíduos assintomáticos e sem repercussões da doença.