Sickle cell disease related chronic thromboembolic pulmonary hypertension: challenging clinical scenario.

Sickle cell disease (SCD), a haemoglobinopathy characterized by chronic haemolysis with acute exacerbations and vascular occlusion episodes, may be complicated by pulmonary hypertension. The latter may be caused by chronic thromboembolic disease of pulmonary artery branches and its management is not well-defined. Herein, we present a case of SCD complicated by chronic thromboembolic pulmonary hypertension of subsegmental pulmonary artery branches successfully treated with endothelin receptor antagonists, orally administered prostacyclin analogs and balloon pulmonary angioplasty. This challenging case highlights the need for clinical awareness of chronic thromboembolic pulmonary hypertension as a specific and potentially curable form of pulmonary hypertension complicating SCD course that may necessitate combined pharmacologic and interventional management.

Balloon Pulmonary Angioplasty as a Treatment in Chronic Thromboembolic Pulmonary Hypertension: Past, Present, and Future.

PURPOSE OF REVIEW: Chronic thromboembolic pulmonary hypertension (CTEPH) is a major cause of precapillary pulmonary hypertension leading to right heart failure and death if left untreated. In addition to pulmonary endarterectomy, which is considered the standard of care, and specific drug therapy that is not however expected to offer relief from the mechanical component of the disease, the therapeutic options for CTEPH have expanded with the development of balloon pulmonary angioplasty (BPA). The purpose of this review is a better understanding and evaluation of BPA as a treatment option in CTEPH patients. RECENT FINDINGS: With the evolution of BPA almost all over the world, more centers outside Japan, which remains the leader for the technique, presented their results confirming the efficacy, safety, and feasibility of this procedure as a complementary strategy in the treatment algorithm of CTEPH. Summarizing, more data from all over the world confirm that BPA is a challenging but potentially effective intervention for the treatment of inoperable CTEPH patients. However, there is a need for standardization of the technique, and furthermore, large, international, multicenter randomized controlled trials comparing BPA with the other treatment modalities of CTEPH are imperative.

Balloon pulmonary angioplasty is a promising option in thalassemic patients with inoperable chronic thromboembolic pulmonary hypertension.

Pulmonary hypertension (PH), a serious disorder with a high morbidity and mortality rate, is known to occur in a number of unrelated systemic diseases. ß-Thalassaemia, among other haematological disorders, develop PH which is not an infrequent finding and worsens the prognosis. Haemolysis, iron overload and hypercoagulable state are among the main pathogenetic mechanisms. Haemoglobinopathies and congenital haemolytic anaemia constitute a unique patients population more predisposed to developing chronic thromboembolic pulmonary hypertension (CTEPH). Although pulmonary endarterectomy is accepted as the best treatment for CTEPH, surgery in these patients poses significant practical challenges given the distinct nature of the disease. As drug therapy is not expected to offer relief from the mechanical obstructions of pulmonary arteries and the use of specific pulmonary arterial hypertension drugs is not established in thalassaemia patients, the novel technique of balloon pulmonary angioplasty (BPA) may emerge as a new therapeutic option for patients with inoperable CTEPH and thalassaemia. We are reporting the case of a thalassaemic patient with a history of splenectomy suffering from progressive severe pulmonary hypertension related to chronic thromboembolic disease, who was successfully treated by BPA with substantial improvement.

Balloon Pulmonary Angioplasty in Patients With Thromboembolic Pulmonary Hypertension.

PURPOSE OF REVIEW: Chronic thromboembolic pulmonary hypertension (CTEPH) is the only potentially curable form of precapillary pulmonary hypertension. Although pulmonary endarterectomy (PEA) is the preferred management strategy, a significant number of CTEPH patients will have an inoperable disease. As drug therapy is not expected to offer relief from the mechanical component of the disease, the novel technique of balloon pulmonary angioplasty (BPA) has provided a new therapeutic option for patients with inoperable CTEPH. This review will discuss the contemporary use of BPA technique in inoperable CTEPH patients highlighting the effectiveness and safety of this therapeutic option. RECENT FINDINGS: Data supporting the role of BPA in inoperable CTEPH are limited to observational studies. However, these observational studies report consistent findings that BPA results in marked improvements in pulmonary hemodynamics and exercise capacity indicating its efficacy and safety as a treatment strategy in inoperable CTEPH patients. Summarizing, BPA is an emerging treatment option providing marked improvements in parameters affecting the outcome of CTEPH patients, but multicenter studies are needed to confirm the safety and the long-term efficacy of the procedure, before BPA can be recommended as an established treatment for CTEPH.