Raising the bar to ultradisciplinary collaborations in management of chronic thromboembolic pulmonary hypertension

Abstract

Chronic thromboembolic pulmonary hypertension is an underdiagnosed and potentially fatal subgroup of pulmonary hypertension, if left untreated. Clinical signs include exertional dyspnea and non-specific symptoms. Diagnosis requires multimodality imaging and heart catheterization. Pulmonary endarterectomy, an open heart surgery, is the gold standard treatment of choice in selected patients in specialized centers. Targeted medical therapy and balloon pulmonary angioplasty can be effective in high-risk patients with significant comorbidities, distal pulmonary vascular obstructions, or recurrent/persistent pulmonary hypertension after pulmonary endarterectomy. Currently, there is a limited number of data regarding novel coronavirus-2019 infection in patients with chronic thromboembolic pulmonary hypertension and the changing spectrum of the disease during the pandemic. Challenging times during this outbreak due to healthcare crisis and relatively higher case-fatality rates require convergence; that is an ultradisciplinary collaboration, which crosses disciplinary and sectorial boundaries to develop integrated knowledge and new paradigms. Management strategies for the "new normal" such as virtual care, preparedness for further threats, redesigned standards and working conditions, reevaluation of specific recommendations, and online collaborations for optimal decisions for chronic thromboembolic pulmonary hypertension patients may change the poor outcomes.

Keywords: Balloon pulmonary angioplasty; chronic thromboembolic pulmonary hypertension; pulmonary endarterectomy; pulmonary thromboembolism; targeted medical therapy.

Figure 1. A V/Q scintigraphy image showing diffuse perfusion defects in right lung middle and lower lobe.

Figure 2. Pre and postoperative dual energy computed tomography scans showing preoperative occlusions of the pulmonary artery branches, the endarterectomy material and the significant improvement after surgical treatment.

Figure 3. Operation materials of the patient.

Figure 4. Algorithm for the diagnosis of chronic thromboembolic pulmonary hypertension. CT: Computed tomography; CTEPH: Chronic thromboembolic pulmonary hypertension; PH: Pulmonary hypertension; V/Q: Ventilation/perfusion. Adapted from. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT) and Chronic thromboembolic pulmonary hypertension (CTEPH): Updated Recommendations from the Cologne Consensus Conference

Figure 5. Intraoperative images showing essential points of the operation and some significant landmarks.

Figure 6. Levels of pulmonary endarterectomy material. Type 1 disease describes the obstructive material present in main pulmonary arteries. In this group, a special group in which only one of the main pulmonary arteries is obstructed and the remaining lung is not perfused is called Type I C. Type 2 disease is characterized by intimal thickening and fibrosis and the presence of organized thrombus starting at the lobar artery level. In Type 3 disease, fibrosis starts from the distal segmental arteries, and intimal webs, and thickening of the intima with or without clots are seen. Surgical interventions are required for each segmental artery. Type 4 disease is the group that clot starts at the sub-segmental level and is considered advanced distal disease.

Figure 7. Pathological images of pulmonary arteries; (a) Pulmonary arterial Wall exhibiting fragmentation and areas of myxoid degeneration (H-E, x40) and (b) Foci of myxoid degeneration in the intima and media of pulmonary artery (H-E, x100).

Figure 8. Ultradisciplinary algorithm for the treatment of chronic thromboembolic pulmonary hypertension. BPA: Balloon pulmonary angioplasty; CTEPH: Chronic thromboembolic pulmonary hypertension; PH: Pulmonary hypertension. Adapted and modified from 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT) and Chronic thromboembolic pulmonary hypertension (CTEPH): Updated Recommendations from the Cologne Consensus Conference.