Chronic Thromboembolic Pulmonary Hypertension: Comorbidities, Treatment and Survival

Aadya Shrokowa^*
*Correspondence: Aadya Shrokowa, Department of Pulmonology, University of Bergamo, 24129 Bergamo, Italy, Email:

Keywords

Chronic thromboembolic pulmonary hypertension • Treatment • Survival • Comorbidities

Introduction

Chronic Thromboembolic Pulmonary Hypertension (CTEPH) is a rare but potentially life-threatening condition characterized by persistent pulmonary hypertension resulting from organized thromboembolic obstruction of the pulmonary arteries. Unlike acute pulmonary embolism, where clot dissolution and pulmonary vascular remodeling occur over time, CTEPH is characterized by the persistence of thromboembolic material, leading to progressive pulmonary vascular remodeling and right heart dysfunction. CTEPH shares similarities with other forms of pulmonary hypertension, such as Idiopathic Pulmonary Arterial Hypertension (IPAH), in terms of clinical presentation and hemodynamic abnormalities. However, CTEPH presents unique challenges in terms of diagnosis and management, as the underlying pathology involves chronic thrombotic obstruction rather than primary pulmonary vascular remodeling. The pathogenesis of CTEPH is multifactorial and may involve a combination of genetic, environmental and acquired risk factors. While acute pulmonary embolism is a major predisposing factor for CTEPH, other comorbidities such as chronic thromboembolic disease, hypercoagulable states, connective tissue disorders and underlying cardiopulmonary diseases may contribute to the development of CTEPH [1].

The diagnosis of CTEPH requires a high index of suspicion and a comprehensive evaluation, including imaging studies such as ventilation-perfusion (V/Q) scanning, Computed Tomography Pulmonary Angiography (CTPA) and pulmonary angiography. Right heart catheterization is essential for confirming the diagnosis and assessing the severity of pulmonary hypertension, as well as guiding treatment decisions. The treatment of CTEPH is complex and may involve a combination of medical therapy, Balloon Pulmonary Angioplasty (BPA) and Pulmonary Endarterectomy (PEA). Medical therapy includes anticoagulation, pulmonary vasodilators and targeted therapies for Pulmonary Arterial Hypertension (PAH). BPA is a minimally invasive procedure that can improve pulmonary hemodynamics by selectively dilating stenotic or occluded pulmonary arteries. PEA is the gold standard treatment for CTEPH, offering the potential for significant improvement in symptoms and survival by removing obstructive thromboembolic material from the pulmonary arteries. Despite advances in diagnosis and treatment, CTEPH remains associated with significant morbidity and mortality. Factors influencing survival in patients with CTEPH include the extent of pulmonary vascular obstruction, severity of pulmonary hypertension, presence of comorbidities, response to medical therapy and access to specialized care. Optimizing the management of CTEPH requires a multidisciplinary approach involving cardiologists, pulmonologists, cardiothoracic surgeons and specialized centers with expertise in pulmonary hypertension and thromboembolic disease [2].

Literature Review

The literature on Chronic Thromboembolic Pulmonary Hypertension (CTEPH) encompasses a wide range of topics, including its pathophysiology, diagnosis, treatment and outcomes. Studies have elucidated the complex interplay between acute pulmonary embolism and the development of CTEPH, highlighting the role of vascular remodeling, thrombus organization and unresolved pulmonary hypertension in the pathogenesis of the disease. Diagnostic modalities for CTEPH have evolved over the years, with ventilation-perfusion (V/Q) scanning, Computed Tomography Pulmonary Angiography (CTPA) and pulmonary angiography serving as key tools for identifying thromboembolic obstruction and assessing the extent of pulmonary vascular involvement. Advances in imaging technology and the development of novel biomarkers have improved the accuracy and efficiency of CTEPH diagnosis, facilitating timely intervention and management. Treatment options for CTEPH include medical therapy, Balloon Pulmonary Angioplasty (BPA) and Pulmonary Endarterectomy (PEA). Anticoagulation with vitamin K antagonists remains the cornerstone of medical therapy, aimed at preventing recurrent thromboembolic events and promoting thrombus resolution. Pulmonary vasodilators, such as riociguat and prostacyclin analogs, have been shown to improve hemodynamics and exercise capacity in patients with inoperable or residual CTEPH after PEA [3].

BPA has emerged as a promising therapeutic option for patients with inoperable or distal CTEPH lesions, offering the potential for selective dilation of stenotic or occluded pulmonary arteries and improvement in pulmonary hemodynamics. While BPA is less invasive than PEA and may be suitable for patients with distal disease or comorbidities precluding surgical intervention, its long-term efficacy and safety require further investigation. PEA remains the gold standard treatment for CTEPH, providing durable improvement in symptoms, exercise capacity and survival by removing obstructive thromboembolic material from the pulmonary arteries. However, PEA is technically challenging and requires expertise in cardiothoracic surgery and pulmonary hypertension management. Patient selection, surgical technique and perioperative care are critical factors influencing the outcomes of PEA in patients with CTEPH [4].

Discussion

The management of CTEPH requires a multidisciplinary approach involving collaboration between cardiologists, pulmonologists, radiologists, cardiothoracic surgeons and specialized centers with expertise in pulmonary hypertension and thromboembolic disease. Timely diagnosis and appropriate risk stratification are essential for optimizing treatment decisions and improving patient outcomes. While medical therapy and BPA offer valuable treatment options for patients with inoperable or residual CTEPH, PEA remains the treatment of choice for eligible patients, providing the potential for significant and durable improvement in symptoms and survival. Advances in surgical techniques, perioperative care and patient selection have led to improved outcomes following PEA, but challenges remain in identifying patients who will derive the greatest benefit from surgery [5]. Long-term follow-up and multidisciplinary care are essential components of CTEPH management, allowing for ongoing assessment of disease progression, treatment response and complications. Close monitoring of hemodynamic parameters, exercise capacity and quality of life can guide treatment adjustments and optimize outcomes in patients with CTEPH. Future research efforts should focus on improving our understanding of the pathophysiology of CTEPH, identifying novel therapeutic targets and optimizing treatment strategies to address the unmet needs of patients with this complex and heterogeneous disease. Collaborative multicenter studies, registries and clinical trials are essential for advancing the field of CTEPH and improving the care and outcomes of affected individuals [6].

Conclusion

In conclusion, Chronic Thromboembolic Pulmonary Hypertension (CTEPH) presents unique challenges in terms of diagnosis, treatment and prognosis. Advances in imaging technology, diagnostic modalities and treatment options have improved the management of CTEPH, but significant gaps remain in our understanding of the disease pathogenesis and optimal treatment strategies. A multidisciplinary approach involving collaboration between clinicians, researchers and specialized centers is essential for optimizing the care of patients with CTEPH and improving their long-term outcomes. Further research is needed to elucidate the underlying mechanisms of CTEPH, identify biomarkers for risk stratification and develop targeted therapies to address the complex pathophysiology of the disease. By advancing our understanding of CTEPH and translating research findings into clinical practice, we can improve the diagnosis, treatment and prognosis of this rare but potentially devastating condition, ultimately enhancing the quality of life and survival of patients affected by CTEPH.

Acknowledgment

None.

Conflict of Interest

No conflict of interest.

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