Clinical-radiological-pathological correlation in chronic thromboembolic pulmonary hypertension.
Tom VerbelenLaurent GodinasPeter DorfmüllerDeepa GopalanRobin CondliffeMarion DelcroixPublished in: European respiratory review : an official journal of the European Respiratory Society (2023)
Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare and potentially life-threatening complication of acute pulmonary embolism. It is characterised by persistent fibro-thrombotic pulmonary vascular obstructions and elevated pulmonary artery pressure leading to right heart failure. The diagnosis is based on two steps, as follows: 1) suspicion based on symptoms, echocardiography and ventilation/perfusion scan and 2) confirmation with right heart catheterisation, computed tomography pulmonary angiography and, in most cases, digital subtraction angiography. The management of CTEPH requires a multimodal approach, involving medical therapy, interventional procedures and surgical intervention. This clinical-radiological-pathological correlation paper illustrates the diagnostic and therapeutic management of two patients. The first had chronic thromboembolic pulmonary disease without pulmonary hypertension at rest but with significant physical limitation and was successfully treated with pulmonary endarterectomy. The second patient had CTEPH associated with splenectomy and was considered unsuitable for surgery because of exclusive subsegmental lesions combined with severe pulmonary hypertension. The patient benefited from multimodal treatment involving medical therapy followed by multiple sessions of balloon pulmonary angioplasty. Both patients had normalised functional capacity and pulmonary haemodynamics 3-6 months after the interventional treatment. These two examples show that chronic thromboembolic pulmonary diseases are curable if diagnosed promptly and referred to CTEPH centres for specialist treatment.
Keyphrases
- pulmonary hypertension
- pulmonary artery
- pulmonary arterial hypertension
- computed tomography
- pulmonary embolism
- heart failure
- end stage renal disease
- atrial fibrillation
- newly diagnosed
- ejection fraction
- healthcare
- chronic kidney disease
- optical coherence tomography
- randomized controlled trial
- peritoneal dialysis
- minimally invasive
- coronary artery
- drug induced
- palliative care
- magnetic resonance imaging
- respiratory failure
- early onset
- pain management
- mental health
- inferior vena cava
- chronic pain
- coronary artery disease
- prognostic factors
- mesenchymal stem cells
- stem cells
- intensive care unit
- coronary artery bypass grafting
- dual energy
- percutaneous coronary intervention