Dilated cardiomyopathy and severe heart failure. An update for pediatricians

Bottner, Paola Caviedes; Fernández, Tamara Córdova; Valenzuela, Marcos Larraín; Romero, Pablo Cruces

doi:10.5546/aap.2018.eng.e421

Cited by 9 publications

(7 citation statements)

References 44 publications

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“…His medications on admission included NPH insulin, aspirin, atorvastatin, furosemide, and metoprolol. We estimated his subjective metabolic equivalent of tasks ≤4, revised cardiac risk index (Lee criteria) of >11%, and glomerular filtration rate of 21,518 mL/min/1.73m 2 .…”

Section: Case Descriptionmentioning

confidence: 99%

“…Congestive heart failure (CHF) is a major health burden, affecting 40 million people globally, and dilated cardiomyopathy (DCM) is one of its leading causes. 1,2 The number of patients with CHF presenting for surgery continues to rise, and anesthesiologists are responsible to provide safe perioperative care for patients with low ejection fraction (EF). 3 The risk of perioperative mortality and morbidity in patients with CHF is considerably high demonstrating the need for optimal perioperative management in these patients.…”

Section: Introductionmentioning

confidence: 99%

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<p>Selective Spinal Anesthesia in a Patient with Low Ejection Fraction Who Underwent Emergent Below-Knee Amputation in a Resource-Constrained Setting</p>

Mulugeta¹,

Zemedkun²,

Getachew³

2020

LRA

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Patients with congestive heart failure have a high risk of perioperative major adverse cardiac events and death. The major perioperative goal of management in patients with low ejection fraction is maintaining hemodynamic stability. Evidence is scarce on the safety of a certain anesthetic technique for patients with heart failure. In this report, we present a 48-year-old man with ischemic dilated cardiomyopathy and low-output congestive heart failure (estimated ejection fraction of 27%) who underwent emergent below-knee amputation under selective spinal anesthesia without any apparent complications. We believe that selective spinal anesthesia can be a useful alternative anesthetic technique in patients with low ejection fraction undergoing emergent lower limb surgery. We showed evidencebased and customized anesthetic management of a high-risk patient with the available equipment and resources. This report will hopefully show the contextual challenges of the perioperative care of critically ill patients in resource-constrained settings.

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Section: Case Descriptionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

<p>Selective Spinal Anesthesia in a Patient with Low Ejection Fraction Who Underwent Emergent Below-Knee Amputation in a Resource-Constrained Setting</p>

Mulugeta¹,

Zemedkun²,

Getachew³

2020

LRA

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“…Co-occurrence with myocarditis, often of viral origin, has also frequently been described in these patients, possibly acting in a synergistic manner with the structural defect caused by the genetic disorder [ 7 ]. When diagnosed in childhood, DMCs manifest mainly as rapidly progressive and severe diseases, as the consequent systolic and/or diastolic myocardial dysfunction ultimately results in heart failure, requiring mandatory organ transplant [ 8 ]. The majority of patients are diagnosed with heart failure within the first year of age.…”

Section: Introductionmentioning

confidence: 99%

Homozygous TNNI3 Mutations and Severe Early Onset Dilated Cardiomyopathy: Patient Report and Review of the Literature

Sorrentino

Gabbiato

Canciani

et al. 2023

Genes

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The TNNI3 gene encodes for the cardiac isoform of troponin I, a pivotal component of the sarcomeric structure of the myocardium. While heterozygous TNNI3 missense mutations have long been associated with autosomal dominant hypertrophic and restrictive cardiomyopathies, the role of TNNI3 null mutations has been more debated due to the paucity and weak characterization of reported cases and the low penetrance of heterozygous genotypes. In recent years, however, an increasing amount of evidence has validated the hypothesis that biallelic TNNI3 null mutations cause a severe form of neonatal dilated cardiomyopathy. Here, we expand the case series reporting two unrelated patients afflicted with early onset dilated cardiomyopathy, due to homozygosity for the p.Arg98* TNNI3 variant, which had thus far been documented only in heterozygous patients and apparently healthy carriers, and the recurrent p.Arg69Alafs*8 variant, respectively. A review of previously reported biallelic TNNI3 loss-of-function variants and their associated cardiac phenotypes was also performed.

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“…During the last two decades, substantial research and progress were made resulting in a shift in focus from disease treatment to disease prevention and etiology-driven personalized approach. This approach has improved substantially the prognosis of DCM (1,7,8).…”

Section: Introductionmentioning

confidence: 99%

The Genetic Pathways Underlying Immunotherapy in Dilated Cardiomyopathy

Kadhi

Mohammed

Nemer

2021

Front. Cardiovasc. Med.

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Heart failure (HF) is a global public health threat affecting 26 million individuals worldwide with an estimated prevalence increase of 46% by 2030. One of the main causes of HF and sudden death in children and adult is Dilated Cardiomyopathy (DCM). DCM is characterized by dilation and systolic dysfunction of one or both ventricles. It has an underlying genetic basis or can develop subsequent to various etiologies that cause myocardium inflammation (secondary causes). The morbidity and mortality rates of DCM remains high despite recent advancement to manage the disease. New insights have been dedicated to better understand the pathogenesis of DCM in respect to genetic and inflammatory basis by linking the two entities together. This cognizance in the field of cardiology might have an innovative approach to manage DCM through targeted treatment directed to the causative etiology. The following review summarizes the genetical and inflammatory causes underlying DCM and the pathways of the novel precision-medicine-based immunomodulatory strategies to salvage and prevent the associated heart failure linked to the disease.

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Dilated cardiomyopathy and severe heart failure. An update for pediatricians

Cited by 9 publications

References 44 publications

<p>Selective Spinal Anesthesia in a Patient with Low Ejection Fraction Who Underwent Emergent Below-Knee Amputation in a Resource-Constrained Setting</p>

<p>Selective Spinal Anesthesia in a Patient with Low Ejection Fraction Who Underwent Emergent Below-Knee Amputation in a Resource-Constrained Setting</p>

Homozygous TNNI3 Mutations and Severe Early Onset Dilated Cardiomyopathy: Patient Report and Review of the Literature

The Genetic Pathways Underlying Immunotherapy in Dilated Cardiomyopathy

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