Table 4.
Standard Dose | Mechanism of Action | Comments | |
---|---|---|---|
Diuretics | |||
1. Furosemide | 1 mg/kg dose BID up to max 6 mg/kg/day | Relieves congestive symptoms; useful in volume overload/fluid retention states; do not change the long-term outcomes | Aggressive use of diuretics can reduce the preload and may result in neurohormonal activation and fluid retention—a vicious cycle; patients refractory to usual oral dose of diuretics may need IV diuretics to relieve congestion |
2. Chlorothiazide | 10 mg/kg dose BID up to max 2 gm/day | ||
3. Metolazone | 0.1 mg/kg dose BID up to max 20 mg/day | ||
Digoxin | 3 to 5 mcg/kg dose BID | Increases inotropy; attenuates neurohormonal activation that results in decreased serum norepinephrine, improves baroreceptor function, decreases sympathetic nervous system activity | Very narrow toxic to therapeutic ratio; most common side effects are conduction disturbances (atrioventricular block); useful for atrial arrhythmia; reduces inter-stage mortality in infants with single ventricle CHD; excreted by the kidney so the dose must be decreased with renal insufficiency |
ACE inhibitors | |||
1. Captopril | 0.1 mg/kg dose TID up to max 2 mg/kg/dose | Decreases mortality and morbidity; blocks the conversion of angiotensin I to II and activates bradykinin and kallidin; causes vasodilation and natriuresis; reduces afterload | ACE inhibitors are beneficial in ISHLT HF stage B to D HF patients; not recommended for asymptomatic children with mild ventricular dysfunction, no recommendation for routine use in single ventricle CHD patients with RV as systemic ventricle; side effects include hypotension, and renal insufficiency |
2. Enalapril | 0.1 mg/kg dose BID up to max 0.5 mg/kg/day | ||
Beta-blockers | |||
1. Metoprolol | 0.1 mg/kg dose BID up to max 1 mg/kg dose | Decreases morbidity and mortality; Carvedilol has vasodilatory, antioxidant, antiproliferative and anti-apoptotic properties, reversing cardiac remodeling | Patients with ISHLT HF stage C and D; may be beneficial in children with HF due to CHD when LV is systemic ventricle; because of downregulation of β-2 receptor in children with HF due to dilated cardiomyopathy—a better option may be Metoprolol |
2. Carvedilol | 0.025 mg/kg/dose BID up to max 0.5 mg/kg/dose BID | ||
Aldosterone antagonist Spironolactone | 1 mg/kg dose BID up to max 200 mg/day | Decreases mortality and morbidity; improves endothelial dysfunction; suppresses vascular angiotensin conversion | Should be used with caution in patients with hyponatremia, renal insufficiency, hyperkalemia and hepatic disease; can cause gynecomastia |
BID = twice daily, TID = three times daily, max = maximum, LVEDP = left ventricular end-diastolic pressure.