Long-Term Outcome of Patients With Chronic Thromboembolic Pulmonary Hypertension

Abstract

C hronic thromboembolic pulmonary hypertension (CTEPH) is a rare complication of acute pulmonary embolism with uncertain prevalence, ranging from 0.4% to 9.1%. 1 Diagnosis is strongly associated with a history of acute venous thromboembolism (VTE). 2,3 It is caused by nonresolving fibrothrombotic obstructions of large pulmonary arteries, combined Background-Chronic thromboembolic pulmonary hypertension, a rare complication of acute pulmonary embolism, is characterized by fibrothrombotic obstructions of large pulmonary arteries combined with small-vessel arteriopathy. It can be cured by pulmonary endarterectomy, and can be clinically improved by medical therapy in inoperable patients. A European registry was set up in 27 centers to evaluate long-term outcome and outcome correlates in 2 distinct populations of operated and not-operated patients who have chronic thromboembolic pulmonary hypertension. Methods and Results-A total of 679 patients newly diagnosed with chronic thromboembolic pulmonary hypertension were prospectively included over a 24-month period. Estimated survival at 1, 2, and 3 years was 93% (95% confidence interval [CI], 90-95), 91% (95% CI, 87-93), and 89% (95% CI, 86-92) in operated patients (n=404), and only 88% (95% CI, 83-91), 79% (95% CI, 74-83), and 70% (95% CI, 64-76) in not-operated patients (n=275). In both operated and not-operated patients, pulmonary arterial hypertension-targeted therapy did not affect survival estimates significantly. Mortality was associated with New York Heart Association functional class IV (hazard ratio [HR], 4.16; 95% CI, 1. P=0.0065 and HR, 4.76; 95% CI, P=0.0021), increased right atrial pressure (HR, 1.34; 95% CI, 0.95-1.90; P=0.0992 and HR, 1.50; 95% CI, 1.20-1.88; P=0.0004), and a history of cancer (HR, 3.02; 95% CI, 1.36-6.69; P=0.0065 and HR, 2.15; 95% CI, 1.18-3.94; P=0.0129) in operated and not-operated patients, respectively. Additional correlates of mortality were bridging therapy with pulmonary arterial hypertension-targeted drugs, postoperative pulmonary hypertension, surgical complications, and additional cardiac procedures in operated patients, and comorbidities such as coronary disease, left heart failure, and chronic obstructive pulmonary disease in not-operated patients. Conclusions-The long-term prognosis of operated patients currently is excellent and better than the outcome of notoperated patients. with small-vessel arteriopathy in some patients. Both proximal and small-vessel obstruction increase pulmonary vascular resistance (PVR), leading to progressive pulmonary hypertension, right heart failure, and ultimately death. CTEPH can be cured by pulmonary endarterectomy (PEA) 4 and clinically improved by medical therapy. 5,6 Therefore, current guidelines recommend PEA as first-choice therapy, whereas the use of pulmonary arterial hypertension (PAH)-targeted drugs is restricted to inoperable patients or patients with persistent/recurrent pulmonary hypertension after PEA.7 Balloon pulmonary angioplasty, a new and promising treatment method for ...

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]. As 67 % of the Mexican patients were deemed operable, it is evident that surgical therapy will improve outcomes should it become available.…”

Sequential Hybrid Therapy With Pulmonary Endarterectomy and Additional Balloon Pulmonary Angioplasty for Chronic Thromboembolic Pulmonary Hypertension

“…Less experience with PEA (44 cases in 17 years) at our center compared with that in Europe and the United States can be a cause of higher mortality (5/44, 11.4%) 31. We excluded patients >75 years from the indication for PEA because the BPA program was initiated at our institution based on its lower mortality rate after BPA 13.…”

Risk factors detection in chronic thromboembolic pulmonary hypertension, a tool for risk quantification?

“…Nevertheless, it is necessary for understanding the pathophysiology of the development, progression of CTEPH in order to implement effective therapeutic and diagnostic strategies to decrease mortality rates (34). Studies show that the long-term prognosis of operated patients is better than the outcome of not operated patients (35).…”