A modified perfusion protocol for pulmonary endarterectomy in a patient with a hematologic malignancy treated with a tyrosine kinase inhibitor

Tyrosine kinase inhibitors (TKI) are known to be highly effective in the treatment of various cancers with kinase-domain mutations such as chronic myelogenous leukemia. However, they have important side effects such as increased vascular permeability and pulmonary hypertension. In patients undergoing pulmonary endarterectomy with deep hypothermic circulatory arrest, these side effects may exacerbate postoperative complications such as reperfusion edema and persistent pulmonary hypertension. We report on a simple modification of the perfusion strategy to increase intravascular oncotic pressure by retrograde autologous priming and the addition of packed cells and albumin in a patient treated with a TKI.

Secondary left heart failure occurred during VA-ECMO assistance for severe residual pulmonary hypertension after pulmonary endarterectomy: a case report

Background In patients of chronic thromboembolic pulmonary hypertension undergoing pulmonary endarterectomy, veno-arterial extracorporeal membrane oxygenation (VA-ECMO) provides full haemodynamic support. However, during a rescue treatment of VA-ECMO for patients with difficulty weaning from cardiopulmonary bypass, a significantly increase left ventricular afterload through retrograde infusion of arterialized blood into the descending aorta may occur. Case presentation We report a 70-year-old man who suffered severe residual pulmonary hypertension following pulmonary endarterectomy for chronic thromboembolic pulmonary hypertension. Preoperative echocardiogram showed a dilated and poorly functioning right ventricle, as well as a small left heart with normal function (TAPES9.6 mm, LVEF64%, average E/E′11.94, lateral E′12.1 cm/s, tricuspid regurgitation velocity 2.5 m/s), while postoperative echocardiography revealed a significant decrease of whole ventricular function on postoperative day 1(TAPES4mm, LVEF28%, average E/E′15, lateral E′6.7 cm/s, tricuspid regurgitation velocity 4.1 m/s), indicating the patient developed severe secondary left ventricular dysfunction on the basis of right ventricular dysfunction, during VA-ECMO support. Then comprehensive measures were adopted, such as down-regulating VA-ECMO flow rate, adjusting respiratory parameters, using vasoactive drugs, as well as prostacyclin. Eventually, the pulmonary hypertension decreased to moderate degree, and the heart function improved gradually. Conclusions In the face of severe residual pulmonary hypertension and sencondary left ventricular dysfunction associated with VA-ECMO, comprehensive measures described above may facilitate recovery. ECMO flow titration to maintain relatively low flow rate is very important to not only maintain systemic perfusion, but also reduce left ventricular afterload and ensure pulsatile perfusion of pulmonary artery.

Right Ventricular Diastolic Performance in Patients With Chronic Thromboembolic Pulmonary Hypertension Assessed by Echocardiography

Background: There have been no systemic studies about right heart filling pressure and right ventricular (RV) distensibility in patients with chronic thromboembolic pulmonary hypertension (CTEPH). Therefore, we aimed to explore combinations of echocardiographic indices to assess the stages of RV diastolic dysfunction.Methods and Results: We recruited 32 healthy volunteers and 71 patients with CTEPH. All participants underwent echocardiography, cardiac catheterization (in patients with CTEPH), and a 6-min walk test (6MWT). The right atrial (RA) end-systolic area was adjusted for body surface area (BSA) (indexed RA area). RV global longitudinal diastolic strain rates (SRs) and RV ejection fraction (EF) were measured by speckle tracking and three-dimensional echocardiography (3D echo), respectively. All 71 patients with CTEPH underwent pulmonary endarterectomy. Of the 71 patients, 52 (73%) had decreased RV systolic function; 12 (16.9%), 26 (36.6%), and 33 (46.5%) patients had normal RV diastolic pattern, abnormal relaxation (stage 1), and pseudo-normal patterns (stage 2), respectively. The receiver operating characteristic curve analysis showed that the optimal cut-off values of early diastolic SR <0.8 s−1 and indexed RA area > 8.8 cm2/BSA had the best accuracy in identifying patients with RV diastolic dysfunction, with 87% sensitivity and 82% specificity. During a mean follow-up of 25.2 months after pulmonary endarterectomy, the preoperative indexed RA area was shown as an independent risk factor of the decreased 6MWT distance.Conclusions: Measuring early diastolic SR and indexed RA area would be useful in stratifying RV diastolic function.

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

<b>Background</b> 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 (PEA) for CTEPH remains unclear. We investigated sex−specific survival after PEA for CTEPH. <b>Methods</b> We included all patients who underwent PEA 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. <b>Results</b> 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 (HR: 1.36; 95% CI: 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. <b>Conclusions</b> In patients who underwent PEA 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.