From the Guidelines
This 8-year-old female with mild pulmonary hypertension and an estimated pulmonary arterial systolic pressure of 38 mmHg should undergo a comprehensive evaluation to determine the underlying cause, including echocardiography, chest imaging, pulmonary function tests, and possibly cardiac catheterization, as recommended by the 2015 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension 1. The initial step in managing this patient is to identify the underlying cause of the pulmonary hypertension, as it can result from various conditions such as congenital heart disease, respiratory disorders, or be idiopathic.
- The patient's symptoms, medical history, and risk factors for pulmonary hypertension should be thoroughly assessed.
- A comprehensive diagnostic workup, as described in the Circulation Research study 1, should be performed, including:
- Echocardiography to evaluate cardiac function and estimate pulmonary arterial pressure
- Chest imaging, such as a non-contrast CT scan or CT angiogram, to assess for underlying lung disease or thromboembolic disease
- Pulmonary function tests to evaluate lung function
- Cardiac catheterization to directly measure pulmonary arterial pressure and assess for any cardiac shunts
- The patient should be referred to a pediatric cardiologist or pulmonologist with expertise in pulmonary hypertension for proper evaluation and management.
- While waiting for specialist consultation, the patient should avoid strenuous physical activities but can continue normal daily activities.
- Regular follow-up is essential to monitor the patient's condition and adjust treatment as needed, as pulmonary hypertension can progress over time, and early intervention based on the underlying cause can prevent worsening of the condition and associated right heart failure.
From the Research
Patient Profile
- Age: 8 years old
- Female
- Mild pulmonary hypertension
- Estimated pulmonary arterial systolic pressure: 38 mmHg
Relevant Studies
- 2 studied the efficacy and tolerability of sildenafil in patients with moderate-to-severe pulmonary hypertension, showing improved exercise tolerance and decreased pulmonary artery pressures.
- 3 discussed the management of mild pulmonary hypertension in clinical practice, highlighting the need for consideration in diagnosis and treatment.
- 4 found that sildenafil improved clinical condition and reduced pulmonary vascular resistance in patients with primary and secondary pulmonary hypertension.
- 5 demonstrated that sildenafil produced a sustained benefit in patients with pulmonary hypertension associated with parenchymal lung and cardiac disease.
- 6 presented consensus recommendations for the treatment of children with pulmonary hypertension, including the use of PAH-specific medications and off-label drugs.
Key Findings
- Sildenafil has been shown to improve exercise tolerance and decrease pulmonary artery pressures in patients with pulmonary hypertension 2, 4, 5.
- The management of mild pulmonary hypertension requires consideration of diagnosis and treatment 3.
- PAH-specific medications and off-label drugs may be used in the treatment of children with pulmonary hypertension 6.
Treatment Options
- Sildenafil may be considered as a treatment option for mild pulmonary hypertension, given its efficacy in improving exercise tolerance and reducing pulmonary artery pressures 2, 4, 5.
- Other treatment options, such as PAH-specific medications and off-label drugs, may also be considered, particularly in children with pulmonary hypertension 6.