From the Guidelines
This 5-month-old female with extreme prematurity and multiple severe medical conditions requires a multidisciplinary approach in the PICU, prioritizing management of her pulmonary hypertension with targeted therapy, such as sildenafil or inhaled nitric oxide, to improve her survival to hospital discharge with favorable neurological outcome. The patient's history of Tetralogy of Fallot with further complications of pulmonary hypertension, acute on chronic hypoxemic hypercarbic respiratory failure, acute on chronic liver failure, and acute on chronic kidney failure necessitates careful consideration of her respiratory, cardiac, gastrointestinal, hepatic, and renal systems. For respiratory management, she needs mechanical ventilation with lung-protective strategies, targeting appropriate oxygen saturation (88-92%) to balance her pulmonary hypertension needs while avoiding oxygen toxicity, as recommended by the 2024 international consensus on cardiopulmonary resuscitation and emergency cardiovascular care science with treatment recommendations 1.
Key aspects of her management include:
- Pulmonary hypertension management with sildenafil (starting at 0.25-0.5 mg/kg every 8 hours) and potentially inhaled nitric oxide (10-20 ppm) if severe, as part of specific management strategies to avoid hypoxia and acidosis 1
- Fluid management to maintain euvolemia while avoiding fluid overload given her cardiac, renal, and respiratory compromise
- Liver failure management by avoiding hepatotoxic medications, monitoring coagulation parameters, providing vitamin K (1 mg IV daily), and considering ursodeoxycholic acid (10-15 mg/kg/day)
- Renal function monitoring with close attention to electrolytes, creatinine, and urine output, and adjusting medication doses based on renal function
- Optimized nutritional support with parenteral nutrition while her gut recovers from NEC, and careful advancement of enteral feeds when appropriate
- Infection prevention with judicious use of antibiotics only when clinically indicated
- Regular echocardiograms to monitor her cardiac repair and pulmonary pressures
The patient's complex condition requires careful coordination between neonatology, pediatric cardiology, gastroenterology, nephrology, and pulmonology to optimize outcomes in this critically ill infant, with a focus on survival to hospital discharge with favorable neurological outcome, as outlined in the study by 1.
From the FDA Drug Label
Methemoglobin concentrations increased during the first 8 hours of nitric oxide exposure. The mean methemoglobin level remained below 1% in the placebo group and in the 5 ppm and 20 ppm INOmax groups but reached approximately 5% in the 80 ppm INOmax group The efficacy of INOmax has been investigated in term and near-term newborns with hypoxic respiratory failure resulting from a variety of etiologies Inhalation of INOmax reduces the oxygenation index (OI= mean airway pressure in cm H2O × fraction of inspired oxygen concentration [FiO2]× 100 divided by systemic arterial concentration in mm Hg [PaO2]) and increases PaO2
The patient in question is a 5-month-old female born at 26 weeks premature with multiple medical complications, including acute on chronic hypoxemic hypercarbic respiratory failure and pulmonary hypertension.
- The NINOS study and CINRGI study show that inhaled nitric oxide can reduce the need for ECMO and improve oxygenation in term and near-term neonates with hypoxic respiratory failure.
- However, the patient's age and gestational age at birth are outside the range of the studied population.
- Key considerations for the use of nitric oxide in this patient include the potential for methemoglobinemia, particularly at higher doses.
- Given the patient's complex medical condition and the lack of direct evidence for the use of nitric oxide in a patient of this age and gestational age, a conservative approach would be to use nitric oxide with caution and close monitoring of methemoglobin levels and clinical response 2.
From the Research
Patient Condition and Management
The patient is a 5-month-old female with a complex medical history, including:
- Premature birth at 26 weeks
- Tracheoesophageal fistula status post-repair
- Recurrent necrotizing enteric colitis
- Tetralogy of Fallot with further complications of pulmonary hypertension
- Acute on chronic hypoxemic hypercarbic respiratory failure
- Acute on chronic liver failure
- Acute on chronic kidney failure
Respiratory Support and Management
The patient's respiratory failure can be managed with various modalities, including:
- Noninvasive respiratory support, such as heated humidified high-flow nasal cannula, continuous positive airway pressure, and bilevel positive airway pressure 3
- Invasive mechanical ventilation, which may be required in severe cases of acute respiratory failure 3, 4
- The choice of respiratory support depends on the severity of the patient's condition and the underlying cause of respiratory failure 4
Pediatric Intensive Care Unit (PICU) Admission and Management
The patient's admission to the PICU is guided by physiologic parameters and the need for close monitoring and interventions 5, 6
- The PICU provides a higher level of care than routine inpatient general pediatric care, with a focus on multidisciplinary subspecialty care 5
- The patient's management in the PICU will require close collaboration between pediatric intensivists, cardiologists, gastroenterologists, and other specialists to address her multiple medical problems and complications
Renal Replacement Therapy and Management
The patient's acute on chronic kidney failure may require renal replacement therapy, which can be managed in the PICU 7
- The choice of renal replacement therapy depends on the patient's underlying condition, the severity of kidney failure, and the availability of local expertise and resources 7