Division of Pediatric Cardiology, Department of Pediatrics, Mayo Clinic College of Medicine, Rochester, Minnesota 55905, USA.
Pediatr Pulmonol. 2010 Jun;45(6):614-8. doi: 10.1002/ppul.21231.
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.
慢性血栓栓塞性肺动脉高压(CTEPH)发生于复发性或慢性肺栓塞患者中,在青少年和年轻成人中是一种罕见但潜在致命的疾病。肺动脉血栓内膜切除术(PTE)是 CTEPH 患者的重要治愈性治疗方法。这种治疗方法的重要性可能在青少年和年轻成人中被低估和未充分利用。
我们对 2003 年至 2009 年在我院接受 PTE 治疗 CTEPH 的所有<24 岁患者进行了图表回顾(7 例患者,平均年龄 20.2 +/- 2.5 岁,范围 17-23 岁,平均 BMI 34 +/- 12 kg/m2)。所有患者均由一位外科医生进行手术。
无死亡病例。7 例患者中有 3 例在诊断 CTEPH 之前被误诊。右心室收缩压从术前的 61.3 +/- 19 mmHg 降至术后的 31.8 +/- 5 mmHg(P = 0.008)。所有患者均报告症状缓解,无患者出现 CTEPH 复发。
尽管在年轻患者中罕见,但复发性肺栓塞可能会产生严重后果。CTEPH 的潜在致命影响在青少年和年轻成人中可能被低估。在有指征的情况下,PTE 是一种具有低发病率和死亡率的确定性治愈性手术。