Byline: Sadia. Imtiaz, Ahmed. Saaedeldin, Nayef. Alqahtani, Majdy. Idrees
BACKGROUND: Chronic thromboembolic pulmonary hypertension (CTEPH) is uncommon but well-known sequel of venous thromboembolism (VTE). At present, it is the only potential curable subtype of pulmonary hypertension. The aim of this study is to describe the medically treated-CTEPH patients' characteristics in a single specialized PH center in Saudi Arabia. METHODS: This study presents demographic, clinical, physiological, and hemodynamic characteristics of medically treated-CTEPH patients in a single PH center, namely Prince Sultan Military Medical City, Riyadh, Saudi Arabia. Both incident and prevalent cases are included. RESULTS: A total of 20 patients with a confirmed diagnosis of CTEPH were included. Mean age at diagnosis was 43 years with a female preponderance of 75%. Most common presenting symptom was dyspnea (100%) followed by syncope (58%). At diagnosis, a mean of 15 [+ or -] 10 months had passed since symptoms onset. About 45% of patients were in WHO functional class IV. At baseline, mean 6-min walk distance was 354.3 meters. Overall, VTE was the most frequent risk factor identified (65% of all patients). Nearly 30% of patients had sickle cell disease. 13 out of 20 patients had radiographic (i.e., computed tomography [CT] pulmonary angiogram) features of chronic thromboembolism. About 75% of patients were found to have distal disease on radiographic imaging. At the time of diagnosis, 7 out of 20 (35%) patients demonstrated right ventricular failure on echocardiography. Mean tricuspid annular plane systolic excursion was 17.7 [+ or -] 1.20. Median NT-proBNP levels were found to be 688 pg/ml. Mean diffusing capacity for carbon monoxide was 74.8%. CONCLUSIONS: Diagnosis of CTEPH was established at a relatively younger age. Majority of patients had advanced but distal disease on radiographic imaging, not amenable to surgery.
Chronic thromboembolic pulmonary hypertension (CTEPH) is a debilitating disease with high morbidity and mortality if left untreated. Theoretically, it is considered as a consequence of incomplete thrombus resolution after an acute or recurrent pulmonary embolism which leads to organization and progressive fibrotic obliteration of proximal pulmonary arteries as well as vascular remodeling in distal vasculature. Consequently, this leads to rise in pulmonary vascular resistance (PVR) and pulmonary arterial pressure (PAP) culminating in right heart failure and death.
CTEPH is defined as precapillary PH (mean PAP >20 mmHg and pulmonary capillary wedge pressure [PCWP] [less-than or equal to]15 mmHg) in the presence of at least one mismatched segmental perfusion defect on ventilation/perfusion (V/Q) scan. As opposed to CTEPH, chronic thromboembolic disease (CTED) refers to patients with persistent symptoms and mismatched perfusion defects in the absence of resting PH on invasive hemodynamic study.
Incidence of CTEPH remains uncertain, however, large prospective cohort studies and international registries report an incidence of 0.56%-6.2%.,,, Recurrent pulmonary embolism, idiopathic venous thromboembolism (VTE), and right ventricular (RV) dysfunction at presentation are associated with higher risk of CTEPH.,,
Despite being a well-recognized complication of acute VTE, CTEPH remains underdiagnosed., Delayed referral, misdiagnosis, and lack of antecedent history of pulmonary embolism in a significant proportion of patients lead to delay...