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Treatment of pulmonary hypertension is determined by whether the PH is arterial, venous, hypoxic, thromboembolic, or miscellaneous. If it is caused by left heart disease, the treatment is to optimize left ventricular function by the use of medication or to repair/replace the mitral valve or aortic valve. [68]
If an echocardiogram is performed, marked thickening of the right ventricle will be seen, resulting from highly elevated pulmonary blood pressure. ACD is generally resistant to treatment. Babies who have persistent symptoms that are poorly relieved by standard therapies for neonatal pulmonary hypertension is commonly observed in ACD. [1]
A pulmonary artery wedge pressure being less than 15 mmHg (also measured by right heart catheterization) excludes post-capillary bed (in the veins distal to the capillary bed) pulmonary hypertension. Pulmonary arterial hypertension is a subgroup of pulmonary hypertension and is categorized as World Health Organization as group 1. [3]
For these disorders, the alveoli are typically impaired by inflammatory and fibrotic changes which can lead to dyspnea, diffuse infiltrates on chest radiographs, and abnormal pulmonary function tests. [3] Not all types of interstitial lung disease that occur in adults occur also in children, and vice versa.
Since the main causes of right ventricular hypertrophy is tricuspid regurgitation or pulmonary hypertension (discussed above), management involves treatment of these conditions. [3] Tricuspid regurgitation is typically treated conservatively by aiming to treat the underlying cause and following up the patient regularly. [ 12 ]
The inhaled vasodilator treprostinil (a synthetic prostacyclin which acts as a prostaglandin I2 analogue) is indicated in the treatment of pulmonary hypertension secondary to interstitial lung disease and is associated with improved exercise capacity as measured by a 6-minute walk test.
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