Pulmonary thromboendarterectomy in adolescents and young adults

Chronic thromboembolic pulmonary hypertension (CTEPH) occurs in patients with recurrent or chronic pulmonary embolism, and is a rare but potentially devastating disease in adolescents and young adults. Pulmonary thromboendarterectomy (PTE) is an important curative therapy for patients with CTEPH. The importance of this treatment may be under-appreciated and under-utilized in adolescents and young adults.

We performed a chart review of all patients <24 years of age who had PTE performed for CTEPH at our institution from 2003-2009 (seven patients, average age 20.2 +/- 2.5 years, range 17-23 years, average BMI 34 +/- 12 kg/m(2)). All patients had their operations performed by a single surgeon.

There were no deaths. Three of the seven patients were incorrectly diagnosed prior to the diagnosis of CTEPH. Right ventricular systolic pressure decreased from 61.3 +/- 19 mmHg pre-operatively to 31.8 +/- 5 mmHg post-operatively (P = 0.008). All patients reported symptomatic relief, and no patient has had recurrence of CTEPH.

Although rare in the young patient, recurrent pulmonary emboli can have devastating consequences. The potentially lethal implications of CTEPH may be under-appreciated in adolescents and young adults. When indicated, PTE is a definitive, curative procedure with low morbidity and mortality.