Pulmonary endarterectomy in a 12‐year‐old boy with multiple comorbidities

Abstract

A 10-year-old boy, with multiple comorbidities presented with fever, exertional dyspnea, fatigue and an obliterated brachiocephalic and inferior caval vein. Chronic thromboembolic pulmonary hypertension (CTEPH) was diagnosed. Nadroparine, antibiotics and supplemental oxygen were successfully started. Aged 12 years, supplemental oxygen was permanently needed with progressive exertional dyspnea and fatigue. In the country of residence the patient was considered as inoperable. The right ventricle was severely dilated, hypocontractile and hypertrophic. Mean pulmonary artery pressure (mPAP) was 79 mmHg and cardiac output 2.2 L/min. Pulmonary endarterectomy was uneventful. Four days later, mPAP was 33 mmHg and cardiac output 6.4 L/min. Three months later the boy restarted his education without supplemental oxygen. Six months after surgery right ventricular size and function and mPAP (14 mmHg) were normal. We demonstrated that pulmonary endarterectomy in young aged children is feasible and well-tolerated, even in the presence of severe co-morbidities. CTEPH should be an important diagnostic consideration in symptomatic children with a known hypercoaguable state, a history of thrombo-embolism or venous catheter placement, and/or a diagnosis of pulmonary hypertension. Hesitating to refer children for surgical consideration, or attempting to treat them by medication, only postpones the single potentially curable treatment and may worsen their prognosis.

Successful Pulmonary Endarterectomy after Acute Pulmonary Embolism and Reversal of Acute Cor Pulmonale in an 11-Year-Old Boy with Nephrotic Syndrome

“…The successful management of our patient by PEA required a multi-disciplinary team, including cardiac surgeons, hematologists, cardiac intensivists, and cardiologists. Furthermore, this case, along with other cases reported in the literature [ 13 , 14 , 15 , 16 , 17 , 18 , 19 ], demonstrate that young age is not a contraindication for PEA, and this may be the single most effective treatment for acute cor pulmonale due to massive PE when the thrombus is not resolved with standard anticoagulation and anti-thrombolytic therapy.…”

“…To our knowledge, this is the first case of PEA in a child developing acute cor pulmonale due to massive PE associated with NS. Prior published reports have demonstrated that PEA is feasible and well tolerated to relieve acute PE secondary to different comorbidities [ 13 , 14 , 15 , 16 , 17 , 18 , 19 ]. Madani et al have shown that after PEA, there was a significant improvement in hemodynamics, RV function, and the functional status of patients with minimal postoperative complications and low perioperative mortality, similar to that reported for adults with CTEPH, with the notable exception being a higher rate of re-thrombosis in pediatric patients [ 19 ].…”

Chronic Thromboembolic Pulmonary Hypertension in a Child With Sickle Cell Disease

“…Our patient was noted to have unexplained PH on routine screening echocardiography in the setting of a chronic hypercoagulable state and recent RA thrombus associated with a central venous catheter, and his diagnosis was confirmed by lung VQ scan and right heart catheterization. While a recently published case report described a 12-year-old with CTEPH successfully treated with PTE, that patient had severe comorbidities including paraplegia, and he was found to have factor V Leiden and antiphospholipid antibodies during his hypercoagulable workup (11).…”

ERS statement on chronic thromboembolic pulmonary hypertension

“…While CTEPH is a rare disease in adults, it is quite exceptional in the paediatric population. There are very little data published on chronic thromboembolism and only few case reports/series with CTEPH [208][209][210]. This important knowledge gap should be addressed by international prospective registries.…”