Left Ventricular Noncompaction Cardiomyopathy in Pediatric Patients: A Case Series of a Clinically Heterogeneous Disease

Gupta, Umang; Makhija, Pooja

doi:10.1007/s00246-016-1566-5

Cited by 7 publications

(12 citation statements)

References 34 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Baseline characteristics. A total of 32 patients with echocardiographic diagnosis of LVNC were recruited between February 2008 and July 2020, with the median age being 11.5 (6)(7)(8)(9)(10)(11)(12)(13)(14)(15) years, and the proportion of males acquiring to 53%. Patients were followed prospectively for the median of 4.02 (IQR 0.48-10.14) years.…”

Section: Resultsmentioning

confidence: 99%

“…Clinical parametersTotal n=32 Age, cohort median , yrs (IQR)11.5[6][7][8][9][10][11][12][13][14][15] Age 1yr, n(%) 1 (3%) Age >1 and  10 yrs, n(%) 10 (31%) Age >10 and < 18 yrs, n(%) 21 (66%) Positive family history, n (%) Nodal rhythm, n (%) 2 (6%) WPW, n (%) 1 (3%) RBBB, n (%) 1 (3%) LBBB, n (%) 0 (0%) LV overload, n (%)4 = 2.3 -6.24, n (%) 24 (82%) NC/C = 1.2 -2.1, n (%) 5 (17%) Pharmacological treatment , n (%) 22 (69%) Beta-blockers, n (%) 9 (28%) ACE-I, n (%) 19 (59%) Furosemide, n (%) 1 (3%) Spironolaktone, n (%) 16 (50%) Acetylsalicylic acid, n (%) 3 (9%) Acenokumarol, n (%) 1 (3%) Salbutamol, n (%) 4 (13%) Other procedures, n (%) 6 (19%) Electrophysiological study , n (%) 1 (3%) RF ablation , n (%) 1 (3%) Pacemaker, n (%) 2 (6%) LVAD, n (%) 1 (3%) W/L for HTx, n (%) 1 (3%) Death, n (%) 1 (3%) HF -heart failure; SCD -sudden cardiac death; CTR -cardiothoracic ratio; NC/C -noncompaction to compaction layer ratio; EF -ejection fraction; RBBB -right bundle branch block;, LBBB -left bundle branch block; EPSelectrophysiology study; ablation RF -radiofrquency ablation; LVAD -left ventricular assist device; LVNC -left ventricular noncompaction cardiomyopathy; HCM -hypertrophic cardiomyopathy;, DCM -Dilated cardiomyopathy; WPW -Wolff-Parkinson-White syndrome;, AVNRT -Atrioventricular nodal reentry tachycardia.…”

mentioning

confidence: 99%

See 1 more Smart Citation

Spectrum of Clinical Features and Genetic Profile of Left Ventricular Noncompaction Cardiomyopathy in Children

Paszkowska¹,

Mirecka-Rola²,

Piekutowska‐Abramczuk³

et al. 2021

Preprint

View full text Add to dashboard Cite

Background: Left ventricular noncompaction (LVNC) is a genetically determined cardiomyopathy, that occurs following a disruption of endomyocardial morphogenesis. The purpose of this study was to identify the clinical characteristics and genetic profile of children with LVNC. Methods: From February 2008 to July 2020, a total of 32 children (median 11.5 years) with LVNC were prospectively enrolled and followed up for the median of 4.02 years. Diagnosis was made based on characteristic features of LVNC in echocardiography and cardiovascular magnetic resonance (CMR). Patients’ clinical symptoms, family history, ECG, Holter ECG and genetic tests were also evaluated. Results: The most common presenting symptom was heart failure (31% of children). ECG abnormalities were noted in 56% of patients. The most prominent features were ventricular arrhythmias, sinus bradycardia and paroxysmal third-degree atrioventricular block. Most of the patients (94%) met the criteria for LVNC and CMR confirmed this diagnosis in 82% of cases. The molecular etiology was found in 53% of children. Conclusion: Although heart failure and arrhythmias were very frequent in our study group, thromboembolic events and genetic syndromes were rare. For accurate and reliable assessment of children with LVNC, it is necessary to get to know their family history and detailed clinical profile.

show abstract

Section: Resultsmentioning

confidence: 99%

mentioning

confidence: 99%

Spectrum of Clinical Features and Genetic Profile of Left Ventricular Noncompaction Cardiomyopathy in Children

Paszkowska¹,

Mirecka-Rola²,

Piekutowska‐Abramczuk³

et al. 2021

Preprint

View full text Add to dashboard Cite

show abstract

“…The spectrum of arrhythmias is very wide, with the most common one being ventricular arrhythmia. Left ventricular (LV) systolic dysfunction, arrhythmias, and blood stasis in the recesses of the myocardium predispose to thromboembolic events [6][7][8]. The basic diagnostic examination in LVNC is echocardiography.…”

Section: Introductionmentioning

confidence: 99%

Spectrum of Clinical Features and Genetic Profile of Left Ventricular Noncompaction Cardiomyopathy in Children

Paszkowska

Mirecka-Rola

Piekutowska‐Abramczuk

et al. 2021

Cardiogenetics

View full text Add to dashboard Cite

Background: Left ventricular noncompaction (LVNC) is a genetically determined cardiomyopathy that occurs following a disruption of endomyocardial morphogenesis. The purpose of this study was to identify the clinical characteristics and genetic profile of children with LVNC. Methods: From February 2008 to July 2020, a total of 32 children (median 11.5 years) with LVNC were prospectively enrolled and followed up for a median of 4.02 years. Diagnosis was made based on characteristic features of LVNC in echocardiography and cardiovascular magnetic resonance (CMR). Patients’ clinical symptoms, family history, ECG, Holter ECG, and genetic tests were also evaluated. Results: The most common presenting symptom was heart failure (31% of children). ECG abnormalities were noted in 56% of patients. The most prominent features were ventricular arrhythmias, sinus bradycardia, and paroxysmal third-degree atrioventricular block. Most of the patients (94%) met the criteria for LVNC and CMR confirmed this diagnosis in 82% of cases. The molecular etiology was found in 53% of children. Conclusion: Although heart failure and arrhythmias were very frequent in our study group, thromboembolic events and genetic syndromes were rare. For the accurate and reliable assessment of children with LVNC, it is necessary to get to know their family history and detailed clinical profile.

show abstract

“…Left ventricular noncompaction (LVNC) is a rare cardiomyopathy of both familial and sporadic origin [ 1 , 2 ]. Familial inheritance is more common in the pediatric population and demonstrates various penetrance and presentation [ 3 ].…”

Section: Introductionmentioning

confidence: 99%

“…LVNC is caused by arrest of embryonic endomyocardial morphogenesis leading to the characteristic finding of prominent trabeculations and intertrabecular recesses within the LV cavity [ 2 ]. Presentation ranges from incidental diagnosis to patients presenting with cardiogenic shock, thromboembolism, arrhythmia, and sudden death [ 1 , 4 , 5 ]. We report a case of LVNC in a previously healthy 7-year-old male who presented with chronic abdominal pain, vomiting, anorexia, and fatigue complicated by dyspnea, acute liver injury, and shock concerning acute decompensation of chronic heart failure.…”

Section: Introductionmentioning

confidence: 99%

Acute on Chronic Heart Failure Secondary to Left Ventricular Noncompaction

Capin¹,

Capone

Taylor

2020

Case Reports in Pediatrics

View full text Add to dashboard Cite

Left ventricular noncompaction (LVNC) is a rare cardiomyopathy characterized by hypertrabeculations and intertrabecular recesses most often seen in the left ventricle (LV). The patient may be asymptomatic or present with heart failure, arrhythmia, and sudden death. We discuss a previously healthy 7-year-old male who presented to the Emergency Department (ED) multiple times over a three-week period. His complaints evolved over the course of his illness, initially presenting with fatigue and suicidal ideation, followed by diffuse abdominal pain. Prior to his ICU admission, he had been discharged from the ED twice, due to well appearance and reassuring lab findings. He returned to the ED a final time with severe venous congestion and cardiogenic shock with acute hepatic injury. Echocardiogram revealed LV apical hypertrabeculation with a severe dilated cardiomyopathy and biventricular failure along with a large thrombus in the left ventricular cavity. Congestive heart failure and anticoagulation therapy was initiated, and the patient went on to biventricular assist device (BiVAD) placement and cardiac transplant. Although LVNC is rare, pediatric heart failure does present to the general pediatrician and has high morbidity and mortality. The presenting symptoms can be obscure and pose a challenge to pediatricians. This case report and review will assist in familiarizing the general pediatrician with pediatric heart failure presentation, treatment, and course.

show abstract

Left Ventricular Noncompaction Cardiomyopathy in Pediatric Patients: A Case Series of a Clinically Heterogeneous Disease

Cited by 7 publications

References 34 publications

Spectrum of Clinical Features and Genetic Profile of Left Ventricular Noncompaction Cardiomyopathy in Children

Spectrum of Clinical Features and Genetic Profile of Left Ventricular Noncompaction Cardiomyopathy in Children

Spectrum of Clinical Features and Genetic Profile of Left Ventricular Noncompaction Cardiomyopathy in Children

Acute on Chronic Heart Failure Secondary to Left Ventricular Noncompaction

Contact Info

Product

Resources

About