Quality of life and functional status after pulmonary endarterectomy for chronic thromboembolic pulmonary hypertension: A Swedish single-center study.

Little is known about long-term quality of life (QOL) and functional status after pulmonary endarterectomy (PEA) for chronic thromboembolic pulmonary hypertension (CTEPH). We investigated QOL and functional status late after PEA. All patients who underwent PEA for CTEPH 1993-2020 at one Swedish center were included. Baseline characteristics and data from right heart catheterization, 6-min walk test, and Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) were obtained from patient charts and national registers. The RAND 36-Item Health Survey was sent by post, and Karnofsky Performance Status (KPS) was evaluated by telephone. A total of 110 patients were included. The survey was completed by 49/66 (74%) patients who were alive in 2020. In all domains except for bodily pain, QOL was slightly lower than that of an age-matched reference population. The KPS score was obtained from 42/49 (86%) patients; of these, 31 patients (74%) had a KPS score of ≥80% (able to carry on normal activity). All 42 patients were able to live at home and care for personal needs. The median postoperative CAMPHOR scores were: 4 for symptoms, 4 for activity, and 2.5 for QOL. We observed that QOL after PEA approached the expected QOL in a reference population and that CAMPHOR scores were comparable to those of a large UK cohort after PEA. Functional status improved when assessed late after PEA. Three-quarters of the study population were able to conduct normal activities at late follow-up. Our findings suggest that many patients enjoy satisfactory QOL and high functional status late after PEA.

Association of residual pulmonary hypertension with survival after pulmonary endarterectomy for chronic thromboembolic pulmonary hypertension.

This study investigated whether residual pulmonary hypertension (PH), defined as early postoperative mean pulmonary artery pressure (mPAP) of ≥30 mmHg, after undergoing pulmonary endarterectomy (PEA) for chronic thromboembolic pulmonary hypertension (CTEPH) was associated with long-term survival. All patients who underwent PEA for CTEPH at two Scandinavian centers were included in this study. Baseline characteristics and vital statuses were obtained from patient charts and national health-data registers. The patients were then categorized based on residual PH measured via right heart catheterization within 48 h after undergoing PEA. Crude and weighted flexible parametric survival models were used to estimate the association between residual PH and all-cause mortality and to quantify absolute survival differences. From 1992 to 2020, 444 patients underwent surgery. We excluded 6 patients who died on the day of surgery and 12 patients whose early postoperative pulmonary hemodynamic data was unavailable. Of the total study population (n = 426), 174 (41%) and 252 (59%) patients had an early postoperative mPAP <30 and ≥30 mmHg, respectively. After weighting, there was a significant association between residual PH and all-cause mortality (hazard ratio: 2.49; 95% confidence interval [CI]: 1.60-3.87), and the absolute survival difference between the groups at 10 and 20 years was -22% (95% CI: -32% to -12%) and-32% (95% CI: -47% to -18%), respectively. A strong and clinically relevant association of residual PH with long-term survival after PEA for CTEPH was found. After accounting for differences in baseline characteristics, the absolute survival difference at long-term follow-up was clinically meaningful and imply careful surveillance to improve clinical outcomes in these patients. Early postoperative right heart catheter measurements of mPAP seem to be helpful for prognostication following PEA for CTEPH.

Sex and survival following pulmonary endarterectomy for chronic thromboembolic pulmonary hypertension: a Scandinavian observational cohort study.

Studies have suggested sex-related survival differences in chronic thromboembolic pulmonary hypertension (CTEPH). Whether long-term prognosis differs between men and women following pulmonary endarterectomy for CTEPH remains unclear. We investigated sex-specific survival after pulmonary endarterectomy for CTEPH. We included all patients who underwent pulmonary endarterectomy for CTEPH at two Scandinavian centers and obtained baseline characteristics and vital statuses from patient charts and national health-data registers. Propensity scores and weighting were used to account for baseline differences. Flexible parametric survival models were employed to estimate the association between sex and all-cause mortality and the absolute survival differences. The expected survival in an age-, sex-, and year of surgery matched general population was obtained from the Human Mortality Database, and the relative survival was used to estimate cause-specific mortality. A total of 444 patients were included, comprising 260 (59%) men and 184 (41%) women. Unadjusted 30-day mortality was 4.2% in men versus 9.8% in women (p = 0.020). In weighted analyses, long-term survival did not differ significantly in women compared with men (hazard ratio: 1.36; 95% confidence interval: 0.89-2.06). Relative survival at 15 years conditional on 30-day survival was 94% (79%-107%) in men versus 75% (59%-88%) in women. In patients who underwent pulmonary endarterectomy for CTEPH, early mortality was higher in women compared with men. After adjustment for differences in baseline characteristics, female sex was not associated with long-term survival. However, relative survival analyses suggested that the observed survival in men was close to the expected survival in the matched general population, whereas survival in women deviated notably from the matched general population.

[Chronic thromboembolic pulmonary hypertension may be cured with surgery]. / Kronisk lungembolism kan botas med kirurgi.

Chronic thromboembolic pulmonary hypertension is a subtype of pulmonary hypertension that affects 0,5-4% of patients who have had a pulmonary embolism. Emboli in the pulmonary arteries lead to fibrosis, obstruction and remodeling of the pulmonary arteries which causes increased pulmonary arterial blood pressure and increased pulmonary vascular resistance. Cardiac ultrasound is used as initial screening in order to identify high pulmonary artery pressures and right heart dysfunction. Lung scintigraphy, computed tomography, and pulmonary angiography are used to verify and describe disease severity. Surgical treatment entails circulatory arrest with endarterectomy starting proximal in the pulmonary arteries and continuing distally throughout the pulmonary arterial tree. Removal of obstruction leads to decreased pulmonary artery pressure and pulmonary vascular resistance, resulting in improved quality of life and near normal life expectancy.

Life expectancy after pulmonary endarterectomy for chronic thromboembolic pulmonary hypertension: a Swedish single-center study.

Pulmonary endarterectomy is the guideline recommended treatment for chronic thromboembolic pulmonary hypertension, in addition to life-long anticoagulation therapy. The aim was to analyze long-term relative survival after pulmonary endarterectomy for chronic thromboembolic pulmonary hypertension. We included all patients who underwent pulmonary endarterectomy for chronic thromboembolic pulmonary hypertension at Karolinska University Hospital between 1997 and 2018 (n = 100). We obtained baseline characteristics and vital status from patient charts and national health-data registers. The expected survival from the general Swedish population matched by age, sex, and year of surgery was obtained from the Human Mortality Database. The relative survival was used as an estimate of cause-specific mortality. The mean age of the patients was 62 years and 39% were women. Most patients were severely symptomatic (95% in New York Heart Association functional class III-IV), and mean preoperative systolic/diastolic (mean) pulmonary artery pressure was 78/27 (45) mmHg. The mean and maximum follow-up time was 7.2 and 22.1 years, respectively. Early (30-day) mortality was 7%. The 15-year observed, expected, and relative survival was 55% (95% confidence interval, 40%-68%), 71%, and 77% (95% confidence interval, 56%-95%), respectively. The 15-year relative survival conditional on 30-day survival was 83% (95% confidence interval, 60%-100%). Although the life expectancy following pulmonary endarterectomy was shorter compared to the general population, the difference was small in those who survived the operation and the early postoperative period. Patients with chronic thromboembolic pulmonary hypertension who are surgical candidates should undergo pulmonary endarterectomy to improve prognosis.

Midterm results of surgery for adults with congenital heart disease centralized to a Swedish cardiothoracic center.

OBJECTIVE: The surgical management of adults with congenital heart disease (ACHD) offers a great challenge, with a large number of anomalies with complex pathophysiology necessitating specific treatments. Pre- and postoperative morbidity has been relatively high, and the influencing factors are not completely identified. We sought to evaluate the incidence and predictors of postoperative complications following surgery for ACHD centralized to a Swedish cardiothoracic center. DESIGN: Between April 2003 and May 2012, 191 consecutive patients with ACHD underwent 192 surgical procedures at our department. Pre-, intra-, and postoperative data were prospectively entered in a clinical database and retrospectively reviewed. Multivariate analysis was used to identify determinants of postoperative complications as a composite end point. RESULTS: The 30-day mortality was 0.5%. Overall survival was 98.3% ± 1.0 at 1 year and 98.3% ± 1.0 at 5 years postoperatively. Repeat sternotomy had to be performed in 94 patients (49%). New onset atrial fibrillation or flutter was the most prevalent (13%, n = 17/135) postoperative complication. Independent risk factors for major postoperative complications were age (odds ratio [OR] 1.81/10 year increment, P = 0.001; 95% confidence interval [CI] 1.29-2.53), reduced (<50%) systemic left ventricle ejection fraction (OR 3.61, P = 0.031; 95% CI 1.13-11.6), and the duration of cardiopulmonary bypass (OR 3.34/60 minute increase, P < 0.001; 95% CI 2.03-5.49). CONCLUSIONS: Our present data suggest that surgery in ACHD can be performed in centralized units with an excellent early and midterm survival. The incidence of postoperative complications was relatively low consisting mainly of supraventricular arrhythmias. In our opinion, ACHD surgery should be performed in centralized units with experienced surgeons in a dedicated multidisciplinary team for optimized postoperative management.