Pulmonary Hypertension as a Complication of Pediatric Cancer

Abstract

Pediatric cancer is a life threatening disease known to create multi-organ complications that further compromise medical management affecting patient morbidity and mortality. Pulmonary hypertension (PH) is becoming more recognized as a complication of cancer and its therapies but has not been well characterized in pediatrics. Cancer pathophysiology can be uniquely set up to promote pulmonary vascular injury and remodeling that is similar to PH patients without cancer. This highlights the need to evaluate for PH clinically and with routine testing such as echocardiography during the course of a patient’s care even into adulthood. This review article will discuss the direct, indirect and therapy related aspects of cancer which can promote PH in these patients. This understanding is essential to target effective treatment options in a potentially fatal complication. Diagnostic and treatment algorithms are presented in relation to the most recent pediatric PH management guidelines.

BMPR2 variant may be related to pulmonary hypertension after lung irradiation

“…Pulmonary hypertension (PH) is a rare condition with a prevalence of 15–60 per million and is a general term for the abnormal elevation of pulmonary artery pressure (PAP), which leads to right heart failure 1 . Despite the recent approval of targeted drugs, PH still has a poor prognosis with a median survival of 7 years 2 .…”

“…Radiation also impairs pulmonary and cardiac function significantly. It affects various phases, including acute (cell injury and inflammation), intermediate (inflammation mainly from macrophages leading to more vascular changes), and chronic phases (pulmonary fibrosis) 1 . Adult survivors of pediatric cancer who received chest radiation therapy were reported to have a 25% frequency of echo‐estimated PH 1 .…”

“…It affects various phases, including acute (cell injury and inflammation), intermediate (inflammation mainly from macrophages leading to more vascular changes), and chronic phases (pulmonary fibrosis) 1 . Adult survivors of pediatric cancer who received chest radiation therapy were reported to have a 25% frequency of echo‐estimated PH 1 . The prevalence of elevated PAP reaches 31% in patients who receive high doses of irradiation (>30 Gy) 1 .…”

“…1 Adult survivors of pediatric cancer who received chest radiation therapy were reported to have a 25% frequency of echo-estimated PH. 1 The prevalence of elevated PAP reaches 31% in patients who receive high doses of irradiation (>30 Gy). 1 Although irradiation can cause postcapillary and precapillary PH, the pathological findings in our patient revealed reversible precapillary PH without any sign of postcapillary PH.…”

“…1 The prevalence of elevated PAP reaches 31% in patients who receive high doses of irradiation (>30 Gy). 1 Although irradiation can cause postcapillary and precapillary PH, the pathological findings in our patient revealed reversible precapillary PH without any sign of postcapillary PH. The possibility that this patient would have developed PH irrespective of irradiation or chemotherapy could not be completely excluded.…”