Abstract 18230: Wringing Pump or Roller Pump? Insights From Rotational Mechanics in Children With Left Ventricular Noncompaction Cardiomyopathy

Circulation ◽  
2015 ◽  
Vol 132 (suppl_3) ◽  
Author(s):  
Hythem Nawaytou ◽  
Putri Yubbu ◽  
Kelley Miller ◽  
Anirban Banerjee
Keyword(s):  
Systolic Function ◽  
Left Ventricular ◽  
Left Ventricular Noncompaction ◽  
Roller Pump ◽  
Ventricular Noncompaction ◽  
Apical Rotation ◽  
Rotational Mechanics ◽  
Lv Systolic Function ◽  
The Relationship ◽  
Lower Ejection Fraction

Introduction: Left ventricular noncompaction (LVNC) is a rare form of cardiomyopathy in children, characterized by abnormal endocardial myofiber arrangement. This may result in an altered pattern of LV rotation depicted by a “roller pump” like motion and described as rigid body rotation (RBR). This pattern, is characterized by the apex and the base rotating in the same direction. Hypothesis: To describe the LV rotational patterns in children with LVNC using speckle tracking echocardiography and detect the relationship between the different rotational patterns and measures of LV systolic function. Methods: We prospectively studied 19 children (age 0.9 to 17.8 years) with LVNC and compared them with age-matched controls. Apical and basal rotations, peak twist and peak torsion were analyzed as indices of systolic function. Peak apical recoil rate was measured as an index of diastolic function. Results: Children with LVNC had significantly lower apical rotation (6.26°± 3.09°, 8.59°± 2.19° p=0.01) than controls. In 7 out of 19 (36.8%) patients a pattern of RBR was detected. Twelve patients exhibited the typical “wringing” motion of the LV, where the apex rotated counterclockwise and the base clockwise. Patients with LVNC and RBR had significantly lower ejection fraction than patients with LVNC without RBR (49.1 ± 9.2% vs 60.3 ± 10.2%, p<0.05). There were no significant differences in the remainder of the systolic and diastolic indices between LVNC patients and controls. Conclusions: Children with LVNC can exhibit an abnormal LV rotational pattern characterized by RBR that is associated with impaired systolic function.

scholarly journals Reversible left ventricular noncompaction caused by hypertensive hydrocephalus: a pediatric case report

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Riko Kato ◽  
Hiromichi Taneichi ◽  
Shinya Takarada ◽  
Mako Okabe ◽  
Nariaki Miyao ◽  
...  
Keyword(s):  
Case Report ◽  
Systolic Function ◽  
Wide Spectrum ◽  
Left Ventricular ◽  
Left Ventricular Noncompaction ◽  
Pediatric Case ◽  
Posthemorrhagic Hydrocephalus ◽  
Ventricular Noncompaction ◽  
Vp Shunt ◽  
Lv Systolic Function

Abstract Background Left ventricular noncompaction cardiomyopathy (LVNC) is characterized by prominent ventricular trabeculations on cardiovascular imaging. Acquired reversible LVNC has not been reported in pediatrics without a genetic background. Case presentation A 9-year-old girl with a ventriculoperitoneal (VP) shunt for neonatal posthemorrhagic hydrocephalus was referred due to exacerbation of hydrocephalus caused by VP shunt dysfunction. Transthoracic echocardiography (TTE) revealed depressed left ventricular (LV) systolic function and thick prominent trabeculae in the LV, predominantly in the apex, suggesting LVNC. Following treatment with extraventricular drainage for hydrocephalus, prominent trabeculation of the LV was diminished on TTE within 3 months. Genetic testing using next-generation sequencing was performed, and no significant variants were identified. Conclusions We revealed for the first time a pediatric case of reversible LVNC without genetic predisposition. This case report provides valuable information on the pathogenesis of acquired LVNC and suggests that detailed evaluation is required to elucidate the diagnosis of this wide spectrum of etiologic–pathogenetic disorders.

scholarly journals Reduced Systolic Function and Not Genetic Variants Determine Outcome in Pediatric and Adult Left Ventricular Noncompaction Cardiomyopathy

2021 ◽  
Vol 9 ◽  
Author(s):  
Alina Schultze-Berndt ◽  
Jirko Kühnisch ◽  
Christopher Herbst ◽  
Franziska Seidel ◽  
Nadya Al-Wakeel-Marquard ◽  
...  
Keyword(s):  
Multivariate Analysis ◽  
Genetic Variants ◽  
Systolic Function ◽  
Left Ventricular ◽  
Left Ventricular Noncompaction ◽  
Ventricular Noncompaction ◽  
Noncompaction Cardiomyopathy ◽  
Pathogenic Variants ◽  
Lv Systolic Function ◽  
Left Ventricular Noncompaction Cardiomyopathy

Background: Left ventricular noncompaction cardiomyopathy (LVNC CMP) is a genetic cardiomyopathy. Genotype-phenotype correlation and clinical outcome of genetic variants in pediatric and adult LVNC CMP patients are still unclear.Methods: The retrospective multicenter study was conducted in unrelated index patients with LVNC CMP, diagnosed between the years 1987 and 2017, and all available family members. All index patients underwent next-generation sequencing for genetic variants in 174 target genes using the Illumina TruSight Cardio Sequencing Panel. Major adverse cardiac events (MACE) included mechanical circulatory support, heart transplantation, survivor of cardiac death, and/or all-cause death as combined endpoint.Results: Study population included 149 LVNC CMP patients with a median age of 27.8 (9.2–44.8) years at diagnosis; 58% of them were symptomatic, 18% suffered from non-sustained and sustained arrhythmias, and 17% had an implantable cardioverter defibrillator (ICD) implanted. 55/137 patients (40%) were ≤ 18 years at diagnosis.A total of 134 variants were identified in 87/113 (77%) index patients. 93 variants were classified as variant of unknown significance (VUS), 24 as likely pathogenic and 15 as pathogenic. The genetic yield of (likely) pathogenic variants was 35/113 (31%) index patients. Variants occurred most frequently in MYH7 (n=19), TTN (n = 10) and MYBPC3 (n = 8). Altogether, sarcomere gene variants constituted 42.5% (n = 57) of all variants. The presence or absence of (likely) pathogenic variants or variants in specific genes did not allow risk stratification for MACE.Reduced left ventricular (LV) systolic function and increased left ventricular end-diastolic diameter (LVEDD) were risk factors for event-free survival in the Kaplan-Meier analysis. Through multivariate analysis we identified reduced LV systolic function as the main risk factor for MACE. Patients with reduced LV systolic function were at a 4.6-fold higher risk for MACE.Conclusions: Genetic variants did not predict the risk of developing a MACE, neither in the pediatric nor in the adult cohort. Multivariate analysis emphasized reduced LV systolic function as the main independent factor that is elevating the risk for MACE. Genetic screening is useful for cascade screening to identify family members at risk for developing LVNC CMP.

A rare case of Ebstein’s anomaly with left ventricular noncompaction

2018 ◽  
Vol 27 (3) ◽  
pp. 208-209
Author(s):  
Christos Tourmousoglou ◽  
Harilaos Bogossian ◽  
Vlasis Ninios ◽  
Elias Ninios
Keyword(s):  
Rare Case ◽  
Systolic Function ◽  
Left Ventricular ◽  
Ebstein’S Anomaly ◽  
Left Ventricular Noncompaction ◽  
Ventricular Noncompaction ◽  
Ebstein's Anomaly ◽  
Congenital Cardiac Disease ◽  
Left Ventricular Noncompaction Cardiomyopathy ◽  
Apical Displacement

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.

Influence of cardiac shape on left ventricular twist

2010 ◽  
Vol 108 (1) ◽  
pp. 146-151 ◽  
Author(s):  
Bas M. van Dalen ◽  
Floris Kauer ◽  
Wim B. Vletter ◽  
Osama I. I. Soliman ◽  
Heleen B. van der Zwaan ◽  
...  
Keyword(s):  
Healthy Volunteers ◽  
Systolic Function ◽  
Left Ventricular ◽  
Lv Function ◽  
Total Study Population ◽  
Internal Dimension ◽  
Apical Rotation ◽  
Sphericity Index ◽  
And Gender ◽  
Lv Systolic Function

The dynamic interaction between subendocardial and subepicardial fibre helices in the left ventricle (LV) leads to a twisting deformation, which has an important role in LV function. This study sought to assess the influence of cardiac shape on LV twist in the normal and dilated human heart. The study comprised 45 dilated cardiomyopathy (DCM) patients and 60 for age- and gender-matched healthy volunteers. Speckle tracking echocardiography was used to determine basal and apical LV peak systolic rotation (Rotmax) and instantaneous LV peak systolic twist (Twistmax). LV sphericity index was calculated by dividing the LV maximal long-axis internal dimension by the maximal short-axis internal dimension at end-diastole. A parabolic relation between the sphericity index and apical Rotmaxor Twistmaxwas identified in the total study population ( R2= 0.56 and R2= 0.54, respectively; both P < 0.001) and healthy volunteers ( R2= 0.39 and R2= 0.25, respectively; both P < 0.001), whereas these relations were linear in DCM patients ( R2= 0.40 and R2= 0.43, respectively; both P < 0.001). In a multivariate analysis, LV sphericity index was the strongest independent predictor of apical Rotmaxand Twistmax. In conclusion, LV apical rotation and twist are significantly influenced by LV configuration. Taking the important function of LV twist into account, this finding highlights the vital influence of cardiac shape on LV systolic function.

scholarly journals Can left ventricular speckle tracking imaging be used in left ventricular non-compaction cardiomyopathy screening? A study of healthy paediatric athletes with and without echocardiographic criteria

2021 ◽  
Vol 22 (Supplement_1) ◽  
Author(s):  
D Dorobantu ◽  
CR Radulescu ◽  
N Riding ◽  
G Mcclean ◽  
C Adamuz ◽  
...  
Keyword(s):  
Speckle Tracking ◽  
Circumferential Strain ◽  
Systolic Function ◽  
Left Ventricular ◽  
P Value ◽  
Speckle Tracking Imaging ◽  
Medical Systems ◽  
Basal Rot ◽  
Apical Rotation ◽  
Rotational Mechanics

Abstract Funding Acknowledgements Type of funding sources: Other. Main funding source(s): The study was support by a contractual research partnership between the University of Bristol and Canon Medical Systems UK Background Left ventricular non-compaction (LVNC) is a rare cardiomyopathy, with hypertrabeculation often observed in athletes. In confirmed LVNC, LV systolic strain and rotational mechanics have been shown to be abnormal. Whether healthy athletes meeting echocardiographic LVNC criteria exhibit abnormal myocardial mechanics is not known. Purpose The aim of this study is to evaluate the prevalence of healthy paediatric athletes meeting the Jenni criteria for LVNC and how this relates to LV systolic function and rotational mechanics. Methods Professional athletes under 18 years undergoing comprehensive pre-participation screening (2014-2017) at two sports academies were included. Jenni criteria for LVNC were assessed from short axis LV views. Global and segmental peak systolic longitudinal (Sl) and circumferential strain (Sc), basal rotation (basal Rot) and apical rotation (apical Rot) were calculated using speckle tracking imaging. Results A total of 201 boys (11.9-18 years, median 15.1 years) were included, with diverse ethnicity (47.7% Arab, 28.5% Black, 21.8% White, and 2% other) and sports background (60% football, 21.2% athletics, 18.8% other). Of these n = 16 (8%) met the Jenni criteria for LVNC and were more likely to be of Black ethnicity than Arab or White (12.7% vs 4.4% or 9.5%). There were no differences in global, lateral or septal Sl, basal, mid or apical Sc, basal Rot or apical Rot between participants with or without Jenni criteria for LVNC (Table 1). Conclusions In healthy paediatric athletes, those meeting the criteria for LVNC (8%) do not have abnormal longitudinal, circumferential strain and rotational mechanics, compared to those without LVNC criteria. This finding supports the use of speckle tracking echocardiography as a tool in differentiating pathological changes reported in LVNC from exercise associated adaptations observed in athletes during preparticipation screening. LV mechanics Jenni criteria presence With Jenni criteria Median (IQR) Without Jenni criteria Median (IQR) p value Global LV Sl -18.6% (-19.1;-17.7) -18.6% (-19.7;-17.8) 0.7 Lateral Sl -18.3% (-18.8;-17.2) -18.5% (-19.8;-17.6) 0.5 Septal Sl -18.6% (-20.6;-17.3) -18.7% (-20.1;-17.7) 0.7 Basal LV Sc -23% (-24.6;-21.6) -23.3% (-25.7;-21.6) 0.7 Mid LV Sc -24.9% (-27.1;-23.2) -25.1% (-27.1;-22.5) 0.9 Apical LV Sc -27.8% (-32.3;-24.8) -26% (30.5;-22.4) 0.2 Basal Rot -4o (-4.7;-2.5) -3.8o (-5.3;-2.5) 0.9 Apical Rot 6.4o (5.2;7.1) 4.4 (2.9;7.1) 0.2

scholarly journals Biventricular Noncompaction Cardiomyopathy in a Patient Presenting with New Onset Seizure: Case Report

2012 ◽  
Vol 2012 ◽  
pp. 1-4 ◽  
Author(s):  
Oghenerukevwe Odiete ◽  
Ramanna Nagendra ◽  
Mark A. Lawson ◽  
Henry Okafor
Keyword(s):  
Right Ventricular ◽  
Systolic Function ◽  
Clinical Manifestations ◽  
Left Ventricular ◽  
Onset Seizure ◽  
Ventricular Noncompaction ◽  
Noncompaction Cardiomyopathy ◽  
Direct Current Cardioversion ◽  
Lv Systolic Function ◽  
New Onset

Ventricular noncompaction (VNC) of the myocardium is a rare genetic cardiomyopathy caused by a disorder during endocardial morphogenesis and could be accompanied by life-threatening complications. The major clinical manifestations of VNC are heart failure, arrhythmias, and embolic events. The left ventricle is the most commonly reported affected site, but a few cases of right ventricular involvement have also been reported. We report a case of biventricular noncompaction cardiomyopathy in a 31-year-old woman presenting with a new onset seizure. On the second day of her telemetry-monitored hospitalization, she suffered a witnessed ventricular fibrillation arrest requiring emergency direct-current cardioversion and induced hypothermia. Transthoracic echocardiography (TTE) showed isolated left ventricular (LV) noncompaction and depressed LV systolic function. Subsequent cardiac magnetic resonance imaging (MRI) revealed both left and right ventricular noncompaction. This unusual presentation highlights the importance of a complete and thorough evaluation of patients even when presenting with apparently noncardiac symptom(s).

Sign in / Sign up

Export Citation Format

Share Document