Chronic thromboembolic pulmonary hypertension: anticoagulation and beyond

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare complication in pulmonary embolism (PE) survivors, characterized by chronic vascular occlusion and pulmonary hypertension. The identification and diagnosis of CTEPH requires a stepwise approach, starting with symptom evaluation, functional evaluation, screening imaging, and progressing to interventional hemodynamic assessment. On the backbone of anticoagulation, CTEPH management necessitates a multidisciplinary approach. Surgical pulmonary thromboendarterectomy (PTE) is the only potentially curative option. In nonoperable disease or residual disease after PTE, interventional balloon pulmonary angioplasty and/or pulmonary-vasodilator therapies can be offered, in collaboration with interventional and vascular pulmonary colleagues. As it is a disease that can cause high morbidity and mortality, CTEPH requires a high index of suspicion to diagnose and treat in patients following PE.

Proposition of quantitative parameters for pre- and early post-operative assessment in pulmonary thromboendarterectomy – An Indian prospective study

Introduction Chronic thromboembolic pulmonary hypertension results from the incomplete resolution of the vascular obstruction associated with pulmonary embolism. Symptoms are exertional dyspnoea and fatigue, and over a period of time, right ventricular dysfunction sets in. Pulmonary thromboendarterectomy is an effective surgical remedy for this condition. Our study is an initial post-operative experience of pulmonary thromboendarterectomy and we have also tried to formulate quantitative parameters for the prediction of the post-operative course in patients who are undergoing surgery. Methods Twenty patients with chronic thromboembolic pulmonary hypertension underwent pulmonary thromboendarterectomy between July 2017 and January 2020. Pre-operatively, each patient was subjected to the (i) 6-min walk test, (ii) pre-operative brain natriuretic peptide values and (iii) pulmonary artery systolic pressure. Following the surgery and subsequent discharge, the patients were followed up at intervals of 15 days, 1, 3, 6, 9 months and at 1 year. At one year post-operatively, the same three quantitative tests were performed on each subject. Results Post-operatively, the mean 6-min walk distance was 499.75 m as against 341.35 m pre-operatively ( p < 0.0001). Mean brain natriuretic peptide was 8.69 pm/l as against 47.58 pm/l pre-operatively ( p < 0.0001). Mean pulmonary artery systolic pressure was 22.25 as against 67.1 pre-operatively ( p < 0.0001). Conclusion 6-Min walk test, brain natriuretic peptide and pulmonary artery systolic pressure could be considered as useful predictors of the haemodynamic severity of disease and predict the post-operative outcome.

Reverse remodeling of tricuspid valve morphology and function in chronic thromboembolic pulmonary hypertension patients following pulmonary thromboendarterectomy: a cardiac magnetic resonance imaging and invasive hemodynamic study

Background To investigate changes in tricuspid annulus (TA) and tricuspid valve (TV) morphology among chronic thromboembolic pulmonary hypertension (CTEPH) patients before and 12 months after pulmonary thromboendarterectomy (PEA) and compare these findings to normal control subjects. Methods 20 CTEPH patients and 20 controls were enrolled in the study. The patients were examined with echocardiography, right heart catherization and cardiac magnetic resonance imaging prior to PEA and 12 months after. Results Right atrium (RA) volume was significantly reduced from baseline to 12 months after PEA (30 ± 9 vs 23 ± 5 ml/m2, p < 0.005). TA annular area in systole remained unchanged (p = 0.11) and was comparable to controls. The leaflet area, tenting volume and tenting height in systole were significantly increased at baseline but decreased significantly with comparable values to controls after 12 months (p < 0.005). There was correlation between the changes of right ventricular-pulmonary artery coupling and changes of TV tenting height (r = − 0.54, p = 0.02), TV tenting volume (r = − 0.73, p < 0.001) and TV leaflet area (− 0.57, p = 0.01) from baseline to 12 months after PEA. Tricuspid regurgitation jet area/RA area was significantly (p < 0.01) reduced from baseline (30 ± 13%) to 12 months after PEA (9 ± 10%). Conclusion In CTEPH patients selected for PEA, TV tenting height, volume and valve area are significantly increased whereas annulus size and shape are less affected. The alterations in TV morphology are fully reversed after PEA and correlates to improvements of right ventricular-pulmonary arterial coupling.

Chronic Thromboembolic Pulmonary Hypertension: A Comprehensive Review and Multidisciplinary Approach to Surgical Treatment

Chronic thromboembolic pulmonary hypertension (CTEPH) is an underdiagnosed and undertreated sequelae of acute pulmonary embolism. In this comprehensive review, we provide an introductory overview of CTEPH, highlight recent advances in its diagnostic imaging, and describe the surgical technique for pulmonary thromboendarterectomy (PTE), the only established curative treatment for CTEPH. We also discuss the emerging role of balloon pulmonary angioplasty, both independently and combined with PTE, for patients with inoperable, residual, or refractory pulmonary hypertension post PTE. Finally, we stress the importance of a specialized multidisciplinary team approach to CTEPH patient care and share our approach to optimizing care for these patients.

Prevalence of new coronavirus infection COVID-19 in patients undergoing pulmonary thromboendarterectomy (data from a single-center registry)

Aim. To assess the prevalence and clinical and functional features of a new coronavirus infection (COVID-19) in patients who underwent pulmonary thromboendarterectomy (PTE) using data from a single-center registry. Methods. This study included 127 patients with chronic thromboembolic pulmonary hypertension who underwent PTE from January 2016 to March 2020 and were included in a follow-up group. The follow-up after surgery was 6 or more months. The prevalence of COVID-19 and clinical and functional properties of the cardiorespiratory system were assessed in the study group. Results. The average follow-up period after PTE in the study group was 2.5 ± 0.9 years. 14 (11%) deaths not associated with COVID-19 were reported during this period. 5 cases of COVID-19 were detected among the remaining 113 patients. In one case, the infection was asymptomatic, while other patients had the typical clinical symptoms and developed bilateral polysegmental pneumonia. No patients required mechanical ventilation or died of COVID-19. All patients who underwent PTE were compliant with anticoagulation therapy and PAH-specific therapy for residual pulmonary hypertension. No adjustment of PAH-specific and anticoagulation therapy was required during COVID-19. Conclusion. The prevalence of COVID-19 in patients who underwent PTE was 4.4%, no deaths were recorded. Outpatient follow-up and timely informing of patients undergoing PTE allow keeping the COVID-19 morbidity and mortality in the studied group at the general population level. Evaluation of the impact of chronic anticoagulants and PAH-specific therapy on the outcome of COVID-19 deserves further research.