Dilated cardiomyopathy

Causes and palliative care considerations

Definition and prognosis

Dilated cardiomyopathy (DCMO) refers to dilation and systolic dysfunction of the ventricles (predominantly the left ventricle) with or without congestive cardiac failure.

About a third of patients with DCMO improve, a third go on to develop chronic congestive heart failure and a third recover. Poor prognosis is associated with:

  • Congenital DCMO
  • DCMO related to storage diseases
  • Post viral myocarditis in patients whose EF remains < 30% for more than 6 months

Palliative care considerations

DCMO is exacerbated by intercurrent infections with or without recovery to previous baseline or lower baseline and very few patients in South Africa have access to the possibility of cardiac transplantation. In addition, disease modifying drugs such as Entresto may modify the disease trajectory but are only available in certain centres of the country. In progressive and intractable cardiomyopathy, admissions become closer together and may increase in length and some patients get stuck in hospital on inotropes.

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Psychosocial and spiritual considerations

Parents/caregivers with children with DCMO experience a significant burden of care, especially when there are recurrent hospital admissions. They may also feel a sense of guilt when a child is diagnosed with a genetic disorder or congenital DCMO. There is a possibility of multiple losses with inherited conditions, leading to complex grief.

Causes

Most common causes of DCMO in children include:

Congenital DCMO

  • Familial
  • Associated with other neuro-muscular conditions e.g. Duchenne’s and Beckers Muscular Dystrophy
  • Metabolic and endocrine Storage diseases
  • Aberrant Left Coronary artery syndrome

Acquired DCMO

  • Post myocarditis: viral, bacterial, HIV
  •  Drugs e.g. Chemotherapy (Anthracyclines, cyclophosphamide)
  • Rheumatic heart disease

Ideopathic DCMO

  • Unknown cause

Symptom control

 

Symptoms in DCMO depend on which ventricles are involved:

Left ventricular Failure Right ventricular failure
Dyspnoea RUQ pain (hepatic congestion)
Orthopnoea Anasarca
Chronic cough or wheeze Gastric congestion- impaired absorption of meds (esp diuretics)
Fatigue Anorexia, Nausea (squashed stomach syndrome)
Poor concentration/confusion Palpitations
Syncope
Angina Chest pain
Limb weakness (strokes)
Oliguria/anuria (With renal failure)
  • For management of cardiorespiratory symptoms please see section 2 page x.
  • Continue treating cardiac failure for as long as benefit > burden
  • Absorption of medications (esp diuretics) may be impaired with gastric congestion- short term IV anti-diuretics may provide the necessary boost to resolve this problem and once again allow for oral medication administration
  • ?note on anti-coagulation in patients with LV thrombus
  • Consider alternative routes of administration in patients where IV access is difficult
    • Subcut (morphine for dyspnoea) and Midazolam for anxiety
    • Nebulized Lasix (pulmonary congestion)
    • Rectal Diazepam for seizures

Terminal care

 

  • In patients who are actively dying medications not contributing to symptom control should be discontinued
  • Continue meds whose discontinuation may result in suffering : eg Calcium replacement (tetany)
  • Dietary (eg salt) and fluid restrictions should also be stopped in dying children so that they may feed for comfort and enjoyment in their last days

 

Ethical considerations

 

Withdrawal or withholding of inotropes in patients who are no longer responding to these or requiring protracted admissions

  • Use first prolonged admission to introduce this possibility and include this as part of advance care planning
  • Do not prolong or postpone dying in patients who are heart transplant candidates where illness is so severe that patient is unlikely to survive to transplant and where ECMO or cardiac assist devices are not available
  • ?Stopping of pacemakers
  • DNAR orders
    • CPR is seldom successful in patients with DCMO and is an indication of severe disease (?exceptions)

References

  1. Venugopalan P. Pediatric Dilated Cardiomyopathy: Medscape; 2019 [cited 2021 3 February 2021]. Available from: https://emedicine.medscape.com/article/895187-overview.