A Preliminary Study of Left Ventricular Rotational Mechanics in Children with Noncompaction Cardiomyopathy: Do They Influence Ventricular Function?

Nawaytou, Hythem; Montero, A.; Yubbu, Putri; Calderón-Anyosa, Renzo; Sato, Tomoyuki; O’Connor, Matthew J.; Miller, Kelley; Ursell, Philip C.; Hoffman, Julien I.E.; Banerjee, Anirban

doi:10.1016/j.echo.2018.02.015

Cited by 8 publications

(13 citation statements)

References 25 publications

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“…Bellavia et al reported that in adults, the value of LV rotation/torsion excessively decreased in patients with LVNC, whereas normal EF were retained when compared with those in controls [31]. Nawaytow et al reported that almost half of the children with LVNC exhibit reverse apical rotation, resulting in decreased LV torsion and untwist rate, which are associated with the degree of LVNC [34]. These previous studies might support that the deterioration of LV function occurs during the perioperative period, although LV systolic function was preserved preoperatively because of its unique structure.…”

Section: Discussionmentioning

confidence: 99%

Left Ventricular Noncompaction and Congenital Heart Disease Increases the Risk of Congestive Heart Failure

Hirono

Hata

Miyao

et al. 2020

JCM

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Background: Left ventricular noncompaction (LVNC) is a hereditary cardiomyopathy that is associated with high morbidity and mortality rates. Recently, LVNC was classified into several phenotypes including congenital heart disease (CHD). However, although LVNC and CHD are frequently observed, the role and clinical significance of genetics in these cardiomyopathies has not been fully evaluated. Therefore, we aimed to evaluate the impact on the perioperative outcomes of children with concomitant LVNC and CHD using next-generation sequencing (NGS). Methods: From Japanese probands with LVNC (25 males and 28 females) were enrolled and we screened 182 cardiomyopathy-associated genes in these patients using NGS. Results: The age at diagnosis of the enrolled patients ranged from 0 to 14 years (median: 0.3 months). A total of 23 patients (43.4%) were diagnosed with heart failure, 14 with heart murmur (26.4%), and 6 with cyanosis (11.3%). During the observation period, 31 patients (58.5%) experienced heart failure and 13 (24.5%) developed arrhythmias such as ventricular tachycardia, supraventricular tachycardia, and atrioventricular block. Moreover, 29 patients (54.7%) had ventricular septal defects (VSDs), 17 (32.1%) had atrial septal defects, 10 had patent ductus arteriosus (PDA), and 7 (13.2%) had Ebstein's anomaly and double outlet right ventricle. Among the included patients, 30 underwent surgery, 19 underwent biventricular repair, and 2 underwent pulmonary artery banding, bilateral pulmonary artery banding, and PDA ligation. Overall, 30 genetic variants were identified in 28 patients with LVNC and CHD. Eight variants were detected in MYH7 and two in TPM1. Echocardiography showed lower ejection fractions and more thickened trabeculations in the left ventricle in patients with LVNC and CHD than in age-matched patients with VSDs. During follow-up, 4 patients died and the condition of 8 worsened postoperatively. The multivariable proportional hazards model showed that heart failure, LV ejection fraction of < 24%, LV end-diastolic diameter z-score of > 8.56, and noncompacted-to-compacted ratio of the left ventricular apex of > 8.33 at the last visit were risk factors for survival. Conclusions: LVNC and CHD are frequently associated with genetic abnormalities. Knowledge of the association between CHD and LVNC is important for the awareness of clinical implications during the preoperative and postoperative periods to identify the populations who are at an increased risk of additional morbidity.

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Section: Discussionmentioning

confidence: 99%

Left Ventricular Noncompaction and Congenital Heart Disease Increases the Risk of Congestive Heart Failure

Hirono

Hata

Miyao

et al. 2020

JCM

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“…However, we found that patients with LVNC< 2 accounted for about half of the patient group in this research. Some researchers have suggested that technological advances in image resolution have led to a more intricate visualization of the left ventricular myocardium, resulting in overdiagnosis of LVNC [26]. The important question posed by this study was whether the high proportion of patients fulfilling the current diagnostic criteria for LVNC with an NC/C ratio < 2 was explained by a genuine congenital abnormality or an exaggeration of the normal trabeculation patterns.…”

Section: Discussionmentioning

confidence: 87%

Do children with left ventricular noncompaction and a noncompaction-to-compaction ratio < 2 have a better prognosis?

et al. 2020

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Background Ultrasonography is commonly used to diagnose left ventricular noncompaction (LVNC). A ratio of noncompacted to compacted myocardium (NC/C ratio) > >2 is often used to diagnose LVNC. However, a large proportion of patients with noncompact myocardium have NC/C < 2, and the prognosis of these patients have not been studied. Methods We included children diagnosed with LVNC between 0 and 15 years of age from January 2007 to December 2018. LVNC was diagnosed based on Stöllberger standard when over three trabeculae were found to be associated with the interventricular recesses. A maximal end systolic ratio of noncompacted to compacted layers was NC/C ratio. Outcomes for LVNC subjects with NC/C < 2 and NC/C > 2 were compared using Kaplan-Meier methods. Results There were 124 newly diagnosed LVNC cases, classified as isolated (i-LVNC, n = 47) or non-isolated (ni-LVNC, n = 77) LVNC and NC/C > 2 (n = 43) or < 2 (n = 81). The median (interquartile range) follow-up duration was 12 (3–30) months for all patients and 16 (6–36) months for survivors. Sixteen patients with i-LVNC died during follow-up. Patients with i-LVNC and NC/C > 2 had worse survival than those with NC/C < 2 (p = 0.022). Conclusions In conclusion, during a 12-month follow-up, patients with i-LVNC with NC/C < 2 had a benign prognosis and better outcomes than those with NC/C > 2, suggesting that the former could have a more active and routine lifestyle.

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“…Ratio of LV-RBR was significantly different between NCCM children and subjects with hypertrabecularization [19]. In another study, reverse LV apical rotation (Cw LV-RBR) was present in 39% of NCCM children [20].…”

Section: Noncompaction Cardiomyopathymentioning

confidence: 90%

“…Specificity and sensitivity of LV-RBR was 88% and 78% in differentiating NCCM from LV "hypertrabeculation", respectively [12]. Cw LV-RBR was detected in most NCCM cases [11,12,15,[18][19][20], which was found to be related to LV-EF [17]. Almost one-third of the relatives of NCCM patients showed LV-RBR, the ratio of Cw and CCw direction was the same [18].…”

Section: Noncompaction Cardiomyopathymentioning

confidence: 90%

“…According to the literature, NCCM is the most extensively examined LV-RBR-related disease, where its prevalence ranged from 26% to 100% (Table 1) [11][12][13][14][15][16][17][18][19][20][21]. van Dalen et al [12] assessed patients with dilated cardiomyopathy (DCM), healthy subjects and cases with prominent trabeculations, of whom expert opinion in NCCM defined some of them having NCCM, and found that LV-RBR was an objective, quantitative and reproducible functional criterion, and had a good predictive value in the diagnosis of NCCM.…”

Section: Noncompaction Cardiomyopathymentioning

confidence: 99%

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Prevalence of left ventricular ‘rigid body rotation’, the near absence of left ventricular twist (insights from the MAGYAR studies)

Nemes¹,

Kormányos²

2022

Rev. Cardiovasc. Med.

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Left ventricular (LV) twist is defined as the wringing motion of the LV around its long-axis during systole generated by rotation of the LV apex in a counterclockwise direction, as viewed from the apex, while the LV base moves in a clockwise direction. In several cases, the LV apex and base move in the same direction during ejection demonstrating a special condition called as LV 'rigid body rotation'. The present review aimed to summarize our knowledge about this rare but not fully understood entity demonstrating its theoretic pathophysiologic background, clinical significance, associated diseases, and reversibility based on available literature.

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A Preliminary Study of Left Ventricular Rotational Mechanics in Children with Noncompaction Cardiomyopathy: Do They Influence Ventricular Function?

Abstract: RAR is not a sensitive but is a specific indicator of complications in children with NCC. Therefore, RAR may have prognostic rather than diagnostic value. Premature untwisting of the left ventricle during ejection may be an even more worrisome indicator of LV dysfunction.

Cited by 8 publications

References 25 publications

Left Ventricular Noncompaction and Congenital Heart Disease Increases the Risk of Congestive Heart Failure

Left Ventricular Noncompaction and Congenital Heart Disease Increases the Risk of Congestive Heart Failure

Do children with left ventricular noncompaction and a noncompaction-to-compaction ratio < 2 have a better prognosis?

Prevalence of left ventricular ‘rigid body rotation’, the near absence of left ventricular twist (insights from the MAGYAR studies)

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