Chronic Thromboembolic Pulmonary Hypertension

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Retrieved

2021-01-23

Source

Orphanet

Trials

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Genes

PAH, BMPR2, CPB2, HLA-B, EDN1, FOXO1, SLC6A4, TGFBR1, TGFB1, TERC, TBX1, SPP1, AGT, CCL2, REN, PTGIS, PTGIR, PPIA, PECAM1, PDGFRB, THBD, PDE5A, TNF, NPPB, MIR759, MIR942, MIR940, MIRLET7B, COPD, EXD2

PAH, BMPR2, CPB2, HLA-B, EDN1, FOXO1, SLC6A4, TGFBR1, TGFB1, TERC, TBX1, SPP1, AGT, CCL2, REN, PTGIS, PTGIR, PPIA, PECAM1, PDGFRB, THBD, PDE5A, TNF, NPPB, MIR759, MIR942, MIR940, MIRLET7B, COPD, EXD2, PHAX, NOX4, NOX1, ADAMTS13, PDPN, CTPP, ROCK2, ADIPOQ, PROM1, PAFAH1B1, NCAM1, NOS3, CRP, F5, F3, F2R, EPHA2, DGCR, ACE, CDH5, FCGR3A, CD40LG, CD34, CD6, CASP3, DST, BCL2, F8, FCGR3B, NFKBIL1, KDR, ALB, MUC6, MPO, MMP2, MGP, MAA, CXCL10, MTOR, ICAM1, HTR2A, HP, HLA-DPB1, HIF1A, HGF, MIR3148

Drugs

Ambrisentan ( AMBRISENTAN MYLAN, LETAIRIS, VOLIBRIS ), Beraprost sodium ( DORNER ), Bosentan ( STAYVEER, TRACLEER )

Ambrisentan ( AMBRISENTAN MYLAN, LETAIRIS, VOLIBRIS ), Beraprost sodium ( DORNER ), Bosentan ( STAYVEER, TRACLEER ), Elafin, Iloprost ( VENTAVIS ), Lisuride hydrogen maleate, Methyl 4,6-diamino-2-[1-(2-fluorobenzyl)-1H-pyrazolo[3,4-b]pyridine-3-yl]-5-pyrimidinyl(methyl)carbamate, Riociguat ( ADEMPAS ), Selexipag ( UPTRAVI ), Sildenafil citrate ( MYSILDECARD, REVATIO ), Sitaxentan sodium ( THELIN ), Terguride, Treprostinil diethanolamine (oral use) ( ORENITRAM ), Treprostinil sodium ( Trepulmix ), Treprostinil sodium (inhalation use) ( TYVASO, REMODULIN )

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Chronic thromboembolic pulmonary hypertension (CTEPH) is characterized by the persistence of thromboemboli in the form of organized tissue obstructing the pulmonary arteries. The consequence is an increase in pulmonary vascular resistance (PVR) resulting in pulmonary hypertension (PH) and progressive right heart failure.

Epidemiology

The true prevalence is unknown: CTEPH is a rare disease but recent reports suggest that it is underdiagnosed.

Clinical description

Patients commonly present with progressive dyspnea on exertion with or without signs of right heart dysfunction including fatigue, palpitations, syncope, or edema. A period between the initial event (acute embolism) and the development of clinical signs is common and may last from a few months to many years. However, up to 60% of patients have no history of acute pulmonary embolism.

Etiology

The pathophysiology of CTEPH is assumed to be associated with restricted flow through the pulmonary arteries, initially related to vascular obliteration caused by unresolved thromboemboli and subsequently to progressive vascular remodeling in unobstructed vessels. The underlying causes of the disease remain unknown.

Diagnostic methods

Diagnosis is suspected on the presence of mismatched segmental perfusion defects on ventilation-perfusion scanning. When CTEPH is suspected, pulmonary angiography and high-resolution CT scan are required to confirm the diagnosis and to assess operability. Pulmonary angiography is always performed in conjunction with diagnostic right heart catheterization, which is mandatory to confirm the diagnosis of PH and to determine the degree of hemodynamic impairment.

Management and treatment

If there is a good correlation between the PVR and the anatomical obstruction (evaluated by pulmonary angiography), pulmonary endarterectomy (PEA) must be proposed. Indeed, PEA is the treatment of choice whenever possible as it can restore near-normal cardiorespiratory function. In other cases, vasodilator and antiproliferative treatments, and lung or heart-lung transplantation represent treatment alternatives. However, further randomized trials are needed to assess the efficacy of medical therapies for some patients: 1) those with inoperable CTEPH due to distal lesions, 2) before PEA (therapeutic bridge) in patients considered as ``high risk'' due to extremely poor hemodynamics, and 3) in patients with persistent pulmonary hypertension after surgery. Indeed, several medical agents including bosentan, sildenafil, iloprost, treprostinil and epoprostenol have been evaluated in CTEPH, but none of them is approved for the treatment of CTEPH.