Potential for and timing of recovery in children with dilated cardiomyopathy

Fenton, Matthew; Horne, Philippa; Simmonds, Jacob; Neligan, Sophie L.; Andrews, Rachel; Burch, Michael

doi:10.1016/j.ijcard.2017.12.075

Cited by 12 publications

(12 citation statements)

References 24 publications

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“…Mortality and recovery can both be predicted from serial changes in NT-proBNP in childhood DCM. 10,29,30 Despite our data demonstrating a reduction NT-proBNP whilst on milrinone in our recovery group, few patients had complete serial measurements, and thus, this may simply reflect the natural disease course of this subgroup irrespective of treatment. Our results provide a basis from which future prospective studies could clarify the optimal duration of treatment and whether there is added benefit from further extended milrinone treatment beyond the point at which a child would typically be weaned.…”

Section: Discussioncontrasting

confidence: 59%

“…The Cardiac recovery from paediatric DCM has ranged from 16% to 41% in previous studies. [8][9][10][19][20][21][22] We elected to use long-term intravenous milrinone in combination with conventional heart failure therapies to maximise myocardial function recovery. This protocol, alongside differences in our inclusion criteria, study era, younger age at presentation and a higher proportion of patients with myocarditis, 20 will have contributed to the high rate of long-term recovery seen in our study.…”

Section: Discussionmentioning

confidence: 99%

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Long‐term milrinone therapy in children with dilated cardiomyopathy

et al. 2023

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Paediatric acute decompensated heart failure (ADHF) is a rare and serious condition affecting around 100 children per year in the United Kingdom and Ireland, the majority of whom have a dilated cardiomyopathy (DCM) phenotype. 1 The in-hospital mortality rate for the initial presentation of paediatric ADHF is 9%-11%, 2-4 and onethird will die or undergo heart transplantation within the first year. 1 The management of ADHF in children with DCM is challenging and heart transplantation is the only definitive treatment for

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Section: Discussioncontrasting

confidence: 59%

Section: Discussionmentioning

confidence: 99%

Long‐term milrinone therapy in children with dilated cardiomyopathy

et al. 2023

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“…Cardiomyopathy presents a high risk for sudden cardiac death in pediatric patients, and patients diagnosed as infants, children and young adolescents show worse prognosis than those diagnosed as adults [2]. A single-center study from America showed the transplant-free survival rate to be only 46% at 1 year, whereas registry data from the UK showed a transplant-free 1-year survival rate of 66% in children requiring hospitalization after an episode of heart failure [3].…”

Section: Discussionmentioning

confidence: 99%

Clinical features of Neonatal Cardiomyopathy

Sakurai

Nakano

Masumori

et al. 2019

Asp Biomed Clin Case Rep

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Background: Neonatal cardiomyopathy is a rare disease that ranges from being asymptomatic to abruptly lethal and is not well characterized [1]. We investigated the clinical features of five neonates with cardiomyopathy in our hospital to determine key clinical characteristics. Methods: We retrospectively reviewed the records of five newborns who were diagnosed with cardiomyopathy between January 2000 and December 2018. The primary evaluation included reasons for diagnosis, underlying diseases, therapy, and turning point. Results: Patients with hypertrophic cardiomyopathy (HCM) or left ventricular noncompaction (LVNC) were diagnosed on the basis of cardiac murmur, while the patient with dilated cardiomyopathy (DCM) was diagnosed on the basis of sucking failure. Underlying diseases included Noonan syndrome and LEOPARD syndrome. All patients had received β-blockers, and those with DCM and LVNC were also administered diuretics and angiotensin-converting enzyme inhibitors. The two patients with HCM underwent follow-up as out-patients. One patient with HCM died at 3 years old because of arrhythmia. The patient with DCM died due to heart failure 38 days after birth. The patient with LVNC exhibited severe heart failure after birth, requiring follow-up while considering heart transplantation. Conclusions: Noonan syndrome and LEOPARD syndrome, which is RAS/MAPK-related diseases, should be considered in patients diagnosed HCM. Because heart failure progresses rapidly in patients with neonatal DCM and those with LVNC, planned therapy should include consideration of heart transplantation. Case-1The baby was born by caesarean section at 38 weeks of gestation because of a breech presentation. The infant weighed was 2940 g at birth and her Apgar scores were normal. Her family histories were unremarkable. She was transferred to our hospital because of a cardiac murmur. Echocardiography on admission showed hypertrophy of the interventricular and posterior left ventricular walls (Fig-A). The pressure gradient at the left ventricular outflow tract

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“…Pediatric dilated cardiomyopathy patients can recover to normal LV size and function, particularly those with a smaller LVEDD zscore at diagnosis (9). We determined whether the LysoPA score could improve the LVEDD z-score for predicting the occurrence of LVRR.…”

Section: Lysopa Score and Prediction For Left Ventricular Reverse Remodelingmentioning

confidence: 99%

“…However, changes in NT-proBNP levels were only associated with adverse events in children with heart failure related to DCM (8). In addition, a small-scale study (9) identified some clinical predictors of LVRR in PDCM, such as higher ejection fraction, younger age, and smaller left ventricular end diastolic z-score. While the use of parameters from echocardiography would be a simple approach, they only reflect a limited aspect of cardiac physiology, and reproducibility of the measurement is relatively poor.…”

Section: Introductionmentioning

confidence: 99%

Higher Serum Lysophosphatidic Acids Predict Left Ventricular Reverse Remodeling in Pediatric Dilated Cardiomyopathy

Huang

You

et al. 2021

Front. Pediatr.

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Background: The prognosis of pediatric dilated cardiomyopathy (PDCM) is highly variable, ranging from death to cardiac function recovery. Left ventricular reverse remodeling (LVRR) represents a favorable prognosis in PDCM. Disturbance of lipid metabolism is associated with the change of cardiac function, but no studies have examined lipidomics data and LVRR.Methods: Discovery analyses were based on 540 targeted lipids in an observational, prospective China—AOCC (An Integrative-Omics Study of Cardiomyopathy Patients for Diagnosis and Prognosis in China) study. The OPLS-DA and random forest (RF) analysis were used to screen the candidate lipids. Associations of the candidate lipids were examined in Cox proportional hazards regression models. Furthermore, we developed a risk score comprising the significant lipids, with each attributed a score of 1 when the concentration was above the median. All significant findings were replicated in a validation set of the China-AOCC study.Results: There were 59 patients in the discovery set and 24 patients in the validation set. LVRR was observed in 27 patients (32.5%). After adjusting for age, left ventricular ejection fraction (LVEF), and left ventricular end-diastolic dimension (LVEDD) z-score, lysophosphatidic acids (LysoPA) 16:0, LysoPA 18:2, LysoPA 18:1, and LysoPA 18:0 were significantly associated with LVRR in the discovery set, and hazard ratios (HRs) were 2.793 (95% CI, 1.545–5.048), 2.812 (95% CI, 1.542–5.128), 2.831 (95% CI, 1.555–5.154), and 2.782 (95% CI, 1.548-5.002), respectively. We developed a LysoPA score comprising the four LysoPA. When the LysoPA score reached 4, LVRR was more likely to be observed in both sets. The AUC increased with the addition of the LysoPA score to the LVEDD z-score (from 0.693 to 0.875 in the discovery set, from 0.708 to 0.854 in the validation set) for prediction of LVRR.Conclusions: Serum LysoPA can predict LVRR in PDCM patients. When the LysoPA score was combined with the LVEDD z-score, it may help in ascertaining the prognosis and monitoring effects of anti-heart failure pharmacotherapy.

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Potential for and timing of recovery in children with dilated cardiomyopathy

Cited by 12 publications

References 24 publications

Long‐term milrinone therapy in children with dilated cardiomyopathy

Long‐term milrinone therapy in children with dilated cardiomyopathy

Clinical features of Neonatal Cardiomyopathy

Higher Serum Lysophosphatidic Acids Predict Left Ventricular Reverse Remodeling in Pediatric Dilated Cardiomyopathy

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