CANDIDATE SELECTION AND LISTING
Timing for referral and listing is always challenging for lung
transplant candidates, particularly with any form of pulmonary
hypertension (PH). PH as an indication for lung transplanation
highlights the importance of interdisciplinary collaboration between the
referring pulmonary hypertension and transplant center teams. As a
general rule, patients should be considered for transplantation when
they are at high risk of death within 1 to 2 years or have unacceptable
quality of life, can survive the wait for organs and the surgery itself
and have a high chance of living a near normal life after lung
transplantation 7. Patients with severe forms of PH
like alveolar capillary dysplasia, pulmonary veno-occlusive disease
(PVOD), pulmonary capillary hemangiomatosis and pulmonary vein stenosis,
can progress rapidly to respiratory failure, making early referral vital8. Patients with IPAH or other forms of pulmonary
vascular disease that meet the following criteria should be referred for
evaluation: NYHA functional class III or IV, rapidly progressive
disease, and optimized use of targeted pulmonary hypertension treatment
(Table 1 ). Early referral gives the lung transplant e and
families the opportunity to address barriers identified during
evaluation. Contraindications to pediatric lung transplant include
conditions that predispose the patient to a poor post-transplant
outcome, such as poor adherence to therapy and concomitant organ failure
not suitable to transplant 8. Timing of listing should
take into consideration waiting time (patient’s blood type and size,
local policy regarding priority 9), center’s expertise
related to extracorporeal support 10 and the patient’s
disease trajectory. Listing should be considered when patients meet the
following criteria 7: NYHA functional class III or IV
without improvement after 3 months of combination therapy including
prostanoids, cardiac index < 2 liters/min/m^2, mean right
atrial pressure of > 15 mmHg. However, it has been noted
that pediatric patients often have preserved cardiac index and no
elevation of right atrial pressure even with advanced disease11. Furthermore, children often have preserved
exercise tolerance at a later stage of disease than adults making
exercise intolerance potentially too late a marker of severe disease12. Other risk factors that predict poor survival
include: significant hemoptysis, pericardial effusion, progressive right
heart failure, von Willebrand factor levels of > 240%,
elevated uric acid levels, and persistently elevated plasma levels of
brain natriuretic peptide > 180 pg/mL 8.