Chronic Thromboembolic Pulmonary Hypertension Successfully Treated in a
Six Year Old with Asthma
Abstract
Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare but
serious, sequala of acute pulmonary embolism. Symptoms can be subtle and
non-specific and the prognosis is poor if severe pulmonary hypertension
(PH) and right ventricular dysfunction are present. While PH-targeted
therapies are often used, there is only one FDA approved therapy, and
only for disease that is deemed inoperable. The greatest chance for
potential cure and long-term survival is surgical pulmonary
endarterectomy. We report a 6-year-old male with a history of asthma and
two unprovoked deep venous thromboses who presented with syncope. Chest
x-ray showed cardiomegaly and an echocardiogram showed severe PH with
severely decreased right ventricular (RV) function.
Ventilation-perfusion scan showed mismatched perfusion defects involving
the right lower lobe, and CT of the chest showed right lower lobe
subsegmental pulmonary thrombus, suspicious for chronic thromboembolism.
Given his clinical presentation and the severity of his right
ventricular dysfunction, he was started on ambrisentan, sildenafil and
subcutaneous treprostinil. Bilateral pulmonary endarterectomy was
performed with resection of level 2 to 3 disease and he was successfully
weaned off all PH therapy. Four months post-op, he is clinically
asymptomatic and his echo shows normal RV function without PH. In
conclusion, CTEPH is a rare but likely underdiagnosed disease process in
pediatrics. Clinicians should have a high index of suspicion for at risk
patients with unexplained dyspnea. Even if right ventricular dysfunction
and severe PH are present, surgical pulmonary endarterectomy can be
performed successfully in young children.