Chronic Thromboembolic Pulmonary Hypertension: Experience from a Single Center in Mexico

Abstract

Introduction Chronic thromboembolic pulmonary hypertension (CTEPH) is characterized by precapillary pulmonary hypertension secondary to vaso-occlusive pulmonary vasculopathy and is classified as Pulmonary Hypertension Group 4. The aim of this study is to report the clinical experience of CTEPH in Mexico. Methods Consecutive patients diagnosed with CTEPH were identified from the Registro de Pacientes con Hipertension Pulmonar del Instituto de Seguridad y Servicio Social de los Trabajadores del Estado (REPHPISSSTE) registry between January 2009 and February 2014. Right heart catheterization was not routinely performed prior to August 2010 in the work-up of CTEPH. Results We identified 50 patients with CTEPH; their median age was 63 years and 58 % were female. Patients had multiple associated co-morbidities and moderate hemodynamic impairment. All patients were treated with anticoagulation. Despite surgical evaluation for pulmonary endarterectomy (PEA), only one patient underwent PEA given the lack of infrastructure for post-operative care and lack of insurance for this procedure. Most of the patients were treated with sildenafil, bosentan, or both, with increasing use of rivaroxaban and sildenafil in recent years. The overall survival of the cohort was similar to that reported in other international registries, despite the limitations of care imposed by drug availability and surgical feasibility. Conclusion This is the first report on the CTEPH experience in Mexico. It highlights the similarity of patients in the REPHPISSSTE registry to those in international registries as well as the challenges that clinicians face in a resource-limited setting.

Sex-specific cardiopulmonary exercise testing indices to estimate the severity of inoperable chronic thromboembolic pulmonary hypertension

“…Although patients with CTEPH are a heterogeneous group with respect to their hemodynamic status and surgical accessibility of pulmonary thromboemboli, resulting in demarcation as operable or inoperable patients, they also present with many similarities at diagnosis, suggesting a common underlying disease process. 26 In line with previous registries from the US, Canada, Europe, Japan, and Mexico, 5 , 26 – 30 CTEPH was almost equally frequent in men and women in their 6th decade of life, approximately 63 years, which is comparable to our cohort. The slight female predominance observed in this study is also consistent with the findings from the international registry.…”

Chronic Thromboembolic Pulmonary Hypertension: A Worldwide View of How Far We Have Come

“…With a median follow up of 20 months, the mortality rate in the Mexican registry reported by Al-Naamani et al [11] was 28 %, which is consistent with the outcomes reported in other registries in developed countries that are enriched with more resources. Examination of the survival curve for the cohort diagnosed via right heart catheterization discloses a 2-year survival of about 80 %, consistent with the survival rate reported in the international (European countries and Canada) registry for non-operated patients, but more than 10 % lower compared to operated patients [12].…”

“…In this issue of Lung, Al-Naamani et al report on the experience of a CTEPH cohort registry (REPHPISSSTE registry) in two medical centers in Mexico [11]. Out of the 45 patients who were evaluated for operability (whether retrospectively by chart review or prospectively), a total of 29 patients (64 %) were deemed operable.…”

A survey on pulmonary thromboendarterectomy in the Asia-Pacific region

“…Indeed, the challenge of attracting potential candidates for PEA surgery in a resource-limited setting was recently highlighted in the first report on the CTEPH experience in Mexico, where only one patient in fifty could undergo PEA surgery. 7 Of note, PEA surgery was already being performed in many centers that did not have heart/lung transplantation or ECMO capabilities, and this indicates a lack of agreement among Asia-Pacific surgeons regarding the necessity for such resources.…”