Table 2. Clinical Characteristics That Suggest the Need
for Referral for Lung Transplantation in Children
With PH With Progressive Decline Despite Optimal
Therapy
• Progressive deterioration in World Health Organization (WHO) or New York Heart
Association (NYHA) functional class III or IV during escalating therapy over 2 serial
assessments at least 3 months apart
• Progressive hemodynamic deterioration noted at serial cardiac catheterization at least 3
months apart, independent of functional class change
• Inability to tolerate maximal medical therapy (WHO functional class IIIa or IIIb)
• Worsening right ventricular function (moderate or greater) regardless of other
parameters
• Life-threatening complications (e.g., recurrent hemoptysis or recurrent syncope) that
progress despite medical therapy or creation of a right-to-left shunt
• Development of secondary liver or kidney dysfunction (if reversible) or if requires
multi-organ transplant
• Development of worsening quality of life as determined by family
• Known or suspected pulmonary capillary hemangiomatosis or pulmonary veno-
occlusive disease or similar diseases such as alveolar capillary dysplasia
• Progressive pulmonary vein stenosis not responding to medical or procedural
interventions
Treatment
