Advanced Heart Failure in Special Population—Pediatric Age

Monda, Emanuele; Lioncino, Michele; Pacileo, Roberta; Rubino, Marta; Cirillo, Annapaola; Fusco, Adelaide; Esposito, Augusto; Verrillo, Federica; Fraia, Francesco Di; Mauriello, Alfredo; Tessitore, Viviana; Caiazza, Martina; Cesaro, Arturo; Calabrò, Paolo; Russo, Maria Giovanna; Limongelli, Giuseppe

doi:10.1016/j.hfc.2021.05.011

Cited by 7 publications

(5 citation statements)

References 51 publications

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“…HF is the leading cause of death in the majority of these adult patients, and more and more CHD are candidates for HTx [79] with beneficial long-term results [80]. However, LT-MCS is a valid option if a donor heart is not readily available [81,82].…”

Section: Heart Transplantationmentioning

confidence: 99%

Cardiac Surgery in Advanced Heart Failure

Hullin

Meyer

Yerly

et al. 2022

JCM

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Mechanical circulatory support and heart transplantation are established surgical options for treatment of advanced heart failure. Since the prevalence of advanced heart failure is progressively increasing, there is a clear need to treat more patients with mechanical circulatory support and to increase the number of heart transplantations. This narrative review summarizes recent progress in surgical treatment options of advanced heart failure and proposes an algorithm for treatment of the advanced heart failure patient at >65 years of age.

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Section: Heart Transplantationmentioning

confidence: 99%

Cardiac Surgery in Advanced Heart Failure

Hullin

Meyer

Yerly

et al. 2022

JCM

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“…Side effects of conventional medical treatment are not highly relevant in this setting, and in general all drugs are well tolerated. Hypotension and bradycardia are avoided by gradually titrating dosages and stopping it at the maximal tolerated dose ( 25 ).…”

Section: Discussionmentioning

confidence: 99%

Biallelic truncating variants in children with titinopathy represent a recognizable condition with distinctive muscular and cardiac characteristics: a report on five patients

Baban

Cicenia

Magliozzi

et al. 2023

Front. Cardiovasc. Med.

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BackgroundMonoallelic and biallelic TTN truncating variants (TTNtv) may be responsible for a wide spectrum of musculoskeletal and cardiac disorders with different age at onset. Although the prevalence of heterozygous TTNtv is relatively high in the general population, cardiac phenotyping (mainly cardiomyopathies, CMPs) in biallelic titinopathy has rarely been described in children.MethodsWe reviewed the medical records of pediatric patients with biallelic TTNtv and cardiac involvement. Clinical exome sequencing excluded pathogenic/likely pathogenic variants in major CMP genes.ResultsFive pediatric patients (four male) with biallelic TTNtv were included. Major arthrogryposis multiplex was observed in four patients; no patient showed intellectual disability. At a cardiac level, congenital heart defects (atrial and ventricular septal defects, n = 3) and left ventricular non-compaction (n = 1) were reported. All patients had dilated cardiomyopathy (DCM) diagnosed at birth in one patient and at the age of 10, 13, 14, and 17 years in the other four patients. Heart rhythm monitoring showed tachyarrhythmias (premature ventricular contractions, n = 2; non-sustained ventricular tachycardia, n = 2) and nocturnal first-degree atrio-ventricular block (n = 2). Cardiac magnetic resonance (CMR) imaging was performed in all patients and revealed a peculiar late gadolinium enhancement distribution in three patients. HyperCKemia was present in two patients and end-stage heart failure in four. End-organ damage requiring heart transplantation (HT) was indicated in two patients, who were operated on successfully.ConclusionBiallelic TTNtv should be considered when evaluating children with severe and early-onset DCM, particularly if skeletal and muscular abnormalities are present, e.g., arthrogryposis multiplex and congenital progressive myopathy. End-stage heart failure is common and may require HT.

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“…DCM, one important cause of heart transplantation in children ( 14 ), can cause sudden cardiac death in pediatric patients ( 15 ). The present study aimed to identify the clinical characteristics of pediatric patients with primary DCM without heart transplantation in an Asian population and the risk factors that predict mortality.…”

Section: Discussionmentioning

confidence: 99%

Clinical Profile and Risk Factors for Cardiac Death in Pediatric Patients With Primary Dilated Cardiomyopathy at a Tertiary Medical Center in China

et al. 2022

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AimWe sought to identify the clinical characteristics and risk factors for cardiac mortality in pediatric patients with primary dilated cardiomyopathy (DCM) in China.MethodsA total of 138 pediatric patients who were consecutively diagnosed with primary DCM from January 2011 to December 2020 were included. We assessed patients’ clinical symptoms and performed laboratory examinations, electrocardiography, and echocardiography.ResultsOf these patients, 79 (57%) had severe systolic dysfunction (left ventricular ejection fraction of < 30%), 79 (57.2%) developed DCM before 12 months of age, 62 (45%) were male, 121 (87.7%) presented with advanced heart failure (cardiac functional class III/IV), and 54 (39.1%) presented with arrhythmia. At a median follow-up of 12 months, the overall cardiac mortality rate was 33%, and 40 of 46 deaths occurred within 6 months following DCM diagnosis. A multivariate Cox regression analysis identified several independent cardiac death predictors, including an age of 12 months to 5 years [hazard ratio (HR) 2.799; 95% confidence interval (CI) 1.160–6.758; P = 0.022] or 10–15 years (HR 3.617; 95% CI 1.336–9.788; P = 0.011) at diagnosis, an elevated serum alanine aminotransferase (ALT) concentration (≥ 51.5 U/L) (HR 2.219; 95% CI 1.06–4.574; P = 0.031), and use of mechanical ventilation (HR 4.223; 95% CI 1.763–10.114; P = 0.001).ConclusionThe mortality rate of primary DCM without transplantation is high. Age, an elevated serum ALT concentration, and the need for mechanical ventilation predict mortality in patients with primary DCM, providing new insights into DCM risk stratification.

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Advanced Heart Failure in Special Population—Pediatric Age

Cited by 7 publications

References 51 publications

Cardiac Surgery in Advanced Heart Failure

Cardiac Surgery in Advanced Heart Failure

Biallelic truncating variants in children with titinopathy represent a recognizable condition with distinctive muscular and cardiac characteristics: a report on five patients

Clinical Profile and Risk Factors for Cardiac Death in Pediatric Patients With Primary Dilated Cardiomyopathy at a Tertiary Medical Center in China

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