Balloon pulmonary angioplasty – efficient therapy of chronic thromboembolic pulmonary hypertension in the patient with advanced sarcoidosis – a case report

Abstract

BackgroundApproximately a quarter of patients with advanced sarcoidosis develop pulmonary hypertension (PH), which affects their prognosis. We report unusual case of confirmed chronic thromboembolic pulmonary hypertension (CTEPH) in a patient with stage IV sarcoidosis successfully treated with balloon pulmonary angioplasty (BPA).Case presentationA 65 years old male with a history of colitis ulcerosa, and pulmonary sarcoidosis diagnosed in 10 years before, on long term oral steroids, with a history of deep vein thrombosis and acute pulmonary embolism chronically anticoagulated was referred to our center due to severe dyspnea. On admission he presented WHO functional class IV, mean pulmonary artery pressure (mPAP) in right heart catheterization (RHC) was elevated to 54 mmHg. Diagnosis of CTEPH was definitely confirmed with typical V/Q scan, and with selective pulmonary angiography (PAG) completes by intravascular imagining (intravascular ultrasound, optical coherent tomography). The patient was deemed inoperable by CTEPH team and two sessions of BPA with multimodal approach resulted in significant clinical and haemodynamical improvement to WHO class II and mPAP decrease to 27 mmHg.ConclusionsBalloon pulmonary angioplasty, rapidly developing method of treatment of inoperable CTEPH patients, is also extremely useful therapeutic tool in complex PH patients.

Sarcoidosis-associated pulmonary hypertension due to pulmonary arteries stenosis – a case report

“…In situ thrombosis and venous thromboembolic disease (VTE) are other mechanisms of intrinsic pulmonary artery obstruction described in sarcoidosis patients [ 13 , 21 , 32 , 36 ]. VTE is more frequent in sarcoidosis compared to the general population, thus chronic thromboembolic pulmonary hypertension (CTEPH) as the cause of SAPH has to be also considered [ 23 , 32 , 33 , 37 , 38 ]. Several recently published studies have reported a twofold to fourfold increased risk of VTE in sarcoidosis patients compared to controls [ 36 , 38 ].…”

“…In case of active disease, corticosteroid and immunosuppressive treatment, are recommended [ 31 , 41 ]. Otherwise, balloon pulmonary angioplasty, which is currently recommended for the therapy of inoperable chronic thromboembolic pulmonary hypertension, or pulmonary artery stenting performed in PH expert centre may be considered [ 23 , 42 – 44 ]. In case of failure or insufficient response to treatment and deterioration of lung function, early referral for lung or lung and heart transplantation is recommended.…”

“…Direct vascular involvement in SAPH patients is seen rarely, and it may present with occlusion of pulmonary arteries mostly due to coexisting venous thromboembolic disease, or external obstruction of large pulmonary arteries caused by markedly enlarged mediastinal/hilar lymph nodes, and/or with fibrotic tissue localized in hilar/mediastinal region [ 13 , 14 , 20 – 23 ]. Complete occlusion of pulmonary arteries is extremely rare, nevertheless it has to be considered in the differential diagnosis of severe PH in the course of sarcoidosis.…”

Sarcoidosis associated pulmonary hypertension: an update

“…Rarely but importantly, mechanical interventions for nodal compression or fibrosing mediastintis including stenting of the PA [60] or vein [26] (often associated with a unilateral pleural effusion) may be required, however, these come with considerable risk. Pulmonary endarterectomy [61] and BPA [62] may be needed for associated CTEPH. Some patients with SAPH require lung transplantation, where although severe SAPH transplants are high risk and require veno-arterial extracorporeal membrane oxygenation support, longer term survival is not impacted by a pretransplant severe PH phenotype [63 ▪▪ ].…”

Epidemiology, Pathogenesis, and Clinical Approach in Group 5 Pulmonary Hypertension

“…Moreover, sarcoidosis is associated with increased risk of venous thromboembolism, which carries associated risk of developing CTEPH in some patients ( 97 , 98 ). Sarcoidosis can be associated with mediastinal and hilar lymphadenopathy that can cause extrinsic compression of pulmonary arteries (and venous stenosis) or fibrosing mediastinitis, resulting in SAPH.…”