Pediatric idiopathic dilated cardiomyopathy: A single center experience

Azhar, Abdullah Zoheb

doi:10.4103/0976-9668.107279

Cited by 5 publications

(4 citation statements)

References 13 publications

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“…The overall survival rate in our study was 72.5% which is less than the 78% survival rate 78% reported by Azhar et al 10 and the remarkably higher survival rate of 86% (95% CI: 65–96) reported Abe et al 4 Klugman et al reported a 92% survival rate for acute myocarditis, and mortality was confined to those who presented with severe illness and required prompt extracorporeal membrane oxygenation (ECMO) and other complex therapies. 12 The reason for the comparatively lower survival rate in our study may be due to the delayed health-seeking behavior of parents in case of persistent symptoms in their children.…”

Section: Discussioncontrasting

confidence: 72%

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Myocarditis in Children: Impact of Early Presentation on Disease Outcomes at a Single Tertiary Center in Saudi Arabia

Albuali¹,

Yousef²,

Ghamdi³

et al. 2022

IJGM

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Purpose Myocarditis is the inflammation of the heart muscle and can be caused by a variety of infections, incendiary diseases, and pollutants. It is challenging for an emergency pediatrician to have a sufficiently high degree of suspicion for myocarditis to avoid diagnostic delay given the broad overlap of myocarditis symptoms with other disease processes. The study aimed to evaluate the impact of early presentation and diagnosis on the outcomes of acute myocarditis in children. Patients and Methods We performed a retrospective analysis of the complete records of 80 pediatric patients diagnosed with acute myocarditis between 2015 and 2019 at a single tertiary center in Saudi Arabia. Patients were two weeks to 14 years of age and were admitted to the pediatric intensive care unit (PICU) for various sequelae of myocarditis. Data from routine clinical and laboratory investigations were collected. Results The incidence of delayed presentation at the hospital after the onset of symptoms of myocarditis was 42.5%. A total of 22 (27.5%) patients expired during their hospital stay. There was marginally significant association of earlier presentation with in-hospital survival (80.4% vs 61.8%) and delayed presentation with higher proportion of in-hospital mortality (38.2% vs 19.6%, p=0.064). The rate of mechanical ventilator was also marginally significant in delayed presentation (p=0.068). Shock and multisystem organ failure were the significantly associated manifestations of delayed acute myocarditis presentations. Length of stay in PICU was also significant in delayed presentation group. The impact of presenting symptoms, ECG findings, and use of mechanical ventilator was not significantly associated with delayed presentation. Conclusion An earlier presentation may have a substantial impact on overall prognosis and length of PICU stay and may lead to comparatively lesser frequency of mechanical ventilation use.

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Section: Discussioncontrasting

confidence: 72%

“… 9 Only one single-center, retrospective study reported that most of the mortalities due to primary pediatric myocardial disease occurs in the first year of life, while the three-year survival rate was 78%. 10 …”

Section: Introductionmentioning

confidence: 99%

Myocarditis in Children: Impact of Early Presentation on Disease Outcomes at a Single Tertiary Center in Saudi Arabia

Albuali¹,

Yousef²,

Ghamdi³

et al. 2022

IJGM

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“…The incidence of DCM in the US is 13-times greater in infants than in older children and has a male predominance [8]. Similarly, over 50% of presentations were before 14 months of age but with a female predominance in Saudi Arabia [9] and 20% of cases were familial, which is consistent with global estimates of a hereditary aetiology in 20% to 35% of cases [10].…”

Section: Introductionmentioning

confidence: 59%

Digenic Inheritance of LAMA4 and MYH7 Mutations in Patient with Infantile Dilated Cardiomyopathy

et al. 2019

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Background and objectives: Dilated cardiomyopathy (DCM) is a rare cardiac disease characterised by left ventricular enlargement, reduced left ventricular contractility, and impaired systolic function. Childhood DCM is clinically and genetically heterogenous and associated with mutations in over 100 genes. The aim of this study was to identify novel variations associated with infantile DCM. Materials and Methods: Targeted next generation sequencing (NGS) of 181 cardiomyopathy-related genes was performed in three unrelated consanguineous families from Saudi Arabia. Variants were confirmed and their frequency established in 50 known DCM cases and 80 clinically annotated healthy controls. Results: The three index cases presented between 7 and 10 months of age with severe DCM. In Family A, there was digenic inheritance of two heterozygous variants: a novel variant in LAMA4 (c.3925G > A, p.Asp1309Asn) and a known DCM mutation in MYH7 (c.2770G > A; p.Glu924Lys). The LAMA4 p.Asp1309Asn variant was predicted to be likely pathogenic according to international guidelines. The other two families had no identifiable potentially deleterious variants. Conclusions: Inheritance of two genetic variants may have a synergistic or dose effect to cause severe DCM. We report of a novel p.Asp1309Asn variation associated with DCM. Targeted NGS is useful in the molecular diagnosis of DCM and to guide whole-family management and counselling.

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“…A large study from that USA showed that freedom from death or transplantation was 69% at 1 year, 61% at 2 years, 54% at 5 years and 46% at 10 years. 6 A study from Turkey found the mortality to be 5%, 16 a Saudi study reported that the survival rate over 3 years was 78% 13 and in Colombia, the mortality rate was 29.4%. 20 Compared with the published literature, it is clear that the mortality in the Sudanese population is relatively high.…”

Section: Discussionmentioning

confidence: 99%

Paediatric dilated cardiomyopathy in Khartoum state, Sudan: a prospective study

Elshazali

Abdalla²

2021

bmjpo

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BackgroundDilated cardiomyopathy (DCM) is a disease of the heart muscle that affects ventricular function; it is a debilitating disease that can lead to heart failure and death in children. There is a lack of good epidemiological data about paediatric DCM in Sudan or in sub-Saharan Africa.ObjectiveTo study the incidence, causes and outcome of DCM among children in Sudan.MethodsA prospective cross-sectional study that was conducted in three paediatric hospitals in Khartoum state, Sudan over the period of 6 months.ResultsDuring the study period, 55 children were seen with the diagnosis of DCM; The female: male ratio was 1.9:1. The incidence of DCM in Khartoum state was found to be 1.4 cases per 100 000 children per year, 10 children (18 %) were diagnosed in the first year of their life. Twenty-seven children (49 %) showed evidence of failure to thrive, being below the fifth percentile for weight. The most common cause of DCM was found to be postviral in 27 children (49%), followed by Idiopathic in 25 children (45%). The most common presentation was the shortness of breath in 53 children (97%), on echocardiography 26 children (47 %) showed evidence of severe left ventricular impairment and 23 children (42%) showed moderate ventricular impairment. Regarding the outcome, 8 children (15%) recovered, the condition of 11 children (20%) remained static and there were 36 deaths (65%).ConclusionThe incidence of DCM in Sudanese children was found to high with a higher mortality compared with middle-income and high-income countries; Improvement in the entire health system is needed to tackle this condition.

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Pediatric idiopathic dilated cardiomyopathy: A single center experience

Cited by 5 publications

References 13 publications

Myocarditis in Children: Impact of Early Presentation on Disease Outcomes at a Single Tertiary Center in Saudi Arabia

Myocarditis in Children: Impact of Early Presentation on Disease Outcomes at a Single Tertiary Center in Saudi Arabia

Digenic Inheritance of LAMA4 and MYH7 Mutations in Patient with Infantile Dilated Cardiomyopathy

Paediatric dilated cardiomyopathy in Khartoum state, Sudan: a prospective study

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