Standard cardiovascular monitoring: early signs are tachycardia and hypotension; hypoxia occurs early only due to intracardiac shunts or as a late sign
Prophylaxis for postoperative PHT:
Maintain adequate analgesia and sedation (fentanyl 1 mcg/kg IV before painful stimuli), consider paralysis, normothermia, normal pH, aim paCO2 30-35 mmHg, paO2>75 mmHg in non-cyanotic lesion, prevent hyper- and hypoinflation, minimise intrathoracic pressures, consider milrinone infusion, consider iNO
Therapy for acute PHT crisis:
Increase FiO2 to 1.0: O2 is the best pulmonary vasodilator
Support cardiac output:resuscitation if required, dopamine (5-10 mcg/kg/min), dobutamine (5-10 mcg/kg/min), adrenaline (0.02-0.1 mcg/kg/min), milrinone (0.25-0.75 mcg/kg/min) as a PDE3 inhibitor increases cAMP → PVR vasodilation
NO donator: increased cGMP → vasodilation: commence NO 20 ppm, SNP (0.5-4 mcg/kg/min), GTN (0.5-5 mcg/kg/min)
Prostacyclin (= prostaglandin I2 = epoprostenol): increased cAMP → vasodilation, commence infusion (5-15 ng/kg/min), or nebulised; half-life: 3 min., can increase PBF and promote pulmonary oedema
Surfactant in neonates: promotes lung expansion, commence stat dose of poractant alpha (200 mg/kg)
Sildenafil: (PDE5 inhibitor → increased cGMP), test dose 0.1 mg/kg, then increase slowly to maximum 2 mg/kg q4hr. FDA recommends against the use in sildenafil in chronic PHT in children, as lower doses were not effective, higher doses increased mortality. The implication its use in PICU is unclear.
Prostacyclin
Bosentan (1 mg/kg BD, increase to 2 mg/kg BD after 4 weeks)
Lung transplantation
References:
[1] Pediatr Crit Care Med. 2010 Mar;11(2 Suppl):S79-84: Steinhorn: Neonatal pulmonary hypertension
[2] Pediatr Crit Care Med. 2010 Mar;11(2 Suppl):S27-9: Taylor et al: Fundamentals of management of acute postoperative pulmonary hypertension
[3] Pediatr Crit Care Med. 2010 Mar;11(2 Suppl):S23-6: Mullen: Diagnostic strategies for acute presentation of pulmonary hypertension in children: particular focus on use of echocardiography, cardiac catheterization, magnetic resonance imaging, chest computed tomography, and lung biopsy
[4] Pediatr Crit Care Med. 2010 Mar;11(2 Suppl):S15-22: Bronicki et al: Pathophysiology of right ventricular failure in pulmonary hypertension
[5] Pediatr Crit Care Med. 2010 Mar;11(2 Suppl):S30-6: Barr et al: Inhaled nitric oxide and related therapies
[6] Pediatr Crit Care Med. 2010 Mar;11(2 Suppl):S41-5: Ivy: Prostacyclin in the intensive care setting
[7] Pharmacotherapy. 2010 Jul;30(7):728-40: Buckley et al: Inhaled epoprostenol for the treatment of pulmonary arterial hypertension in critically ill adults
All Marc’s PICU cardiology FOAM can be found on PICU Doctor and can be downloaded as a handy app for free on iPhone or Android. A list of contributors can be seen here.
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Pulmonary hypertension
Marc Anders. Pulmonary hypertension, Don't Forget the Bubbles, 2013. Available at:
https://doi.org/10.31440/DFTB.3731
Definition: systolic PAP >35 mmHg or mean PAP >25 mmHg, clinically if systolic PAP:systolic BP <0.5
Diagnosis:
Physiology:
Increase in RV afterload → RV volume and pressure increase → RV systolic dysfunction (→ TR) and diastolic dysfunction (→ RV diastolic HTN → increased right to left shunt if exists → hypoxia) → reduced RV output → reduced LV filling → reduced CO and reduced coronary artery perfusion pressure → RV ischemia and ventricular interdependence → RV systolic dysfunction
Neonatal PHT:
Incidence 2:1000, most common due to MAS, RDS, pneumonia, also idiopathic or in congenital diaphragmatic hernia
Postoperative PHT:
Prophylaxis for postoperative PHT:
Maintain adequate analgesia and sedation (fentanyl 1 mcg/kg IV before painful stimuli), consider paralysis, normothermia, normal pH, aim paCO2 30-35 mmHg, paO2>75 mmHg in non-cyanotic lesion, prevent hyper- and hypoinflation, minimise intrathoracic pressures, consider milrinone infusion, consider iNO
Therapy for acute PHT crisis:
Therapy for chronic PHT:
References:
[1] Pediatr Crit Care Med. 2010 Mar;11(2 Suppl):S79-84: Steinhorn: Neonatal pulmonary hypertension [2] Pediatr Crit Care Med. 2010 Mar;11(2 Suppl):S27-9: Taylor et al: Fundamentals of management of acute postoperative pulmonary hypertension [3] Pediatr Crit Care Med. 2010 Mar;11(2 Suppl):S23-6: Mullen: Diagnostic strategies for acute presentation of pulmonary hypertension in children: particular focus on use of echocardiography, cardiac catheterization, magnetic resonance imaging, chest computed tomography, and lung biopsy [4] Pediatr Crit Care Med. 2010 Mar;11(2 Suppl):S15-22: Bronicki et al: Pathophysiology of right ventricular failure in pulmonary hypertension [5] Pediatr Crit Care Med. 2010 Mar;11(2 Suppl):S30-6: Barr et al: Inhaled nitric oxide and related therapies [6] Pediatr Crit Care Med. 2010 Mar;11(2 Suppl):S41-5: Ivy: Prostacyclin in the intensive care setting [7] Pharmacotherapy. 2010 Jul;30(7):728-40: Buckley et al: Inhaled epoprostenol for the treatment of pulmonary arterial hypertension in critically ill adultsAll Marc’s PICU cardiology FOAM can be found on PICU Doctor and can be downloaded as a handy app for free on iPhone or Android. A list of contributors can be seen here.
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