Focus on Paediatric Restrictive Cardiomyopathy: Frequently Asked Questions.
Mattia ZampieriChiara Di FilippoChiara ZocchiVera FicoCristina GolinelliGaia SpazianiGiovanni CalabriElena BennatiFrancesca GirolamiAlberto MarchiSilvia PassantinoGiulio PorceddaGuglielmo CapponiAlessia GozziniIacopo OlivottoLuca RagniSilvia FavilliPublished in: Diagnostics (Basel, Switzerland) (2023)
Restrictive cardiomyopathy (RCM) is characterized by restrictive ventricular pathophysiology determined by increased myocardial stiffness. While suspicion of RCM is initially raised by clinical evaluation and supported by electrocardiographic and echocardiographic findings, invasive hemodynamic evaluation is often required for diagnosis and management of patients during follow-up. RCM is commonly associated with a poor prognosis and a high incidence of heart failure, and PH is reported in paediatric patients with RCM. Currently, only a few therapies are available for specific RCM aetiologies. Early referral to centres for advanced heart failure treatment is often necessary. The aim of this review is to address questions frequently asked when facing paediatric patients with RCM, including issues related to aetiologies, clinical presentation, diagnostic process and prognosis.
Keyphrases
- heart failure
- left ventricular
- poor prognosis
- clinical evaluation
- intensive care unit
- emergency department
- long non coding rna
- cardiac resynchronization therapy
- left atrial
- atrial fibrillation
- primary care
- risk factors
- pulmonary hypertension
- catheter ablation
- drug induced
- ejection fraction
- combination therapy
- replacement therapy