Lung or heart/lung transplant surgery
Transplant surgery is normally indicated for patients who do not respond appropriately and are deteriorating despite being on the most powerful form of treatment, ie prostanoids (usually intravenous). In the case of pulmonary hypertension surgery can involve replacing the heart and lungs, or just the lungs. Both single and bilateral procedures have been performed, apparently with similar survival. Currently the vast majority of patients worldwide receive bilateral lungs.
In order to be listed for surgery patients must undergo a complex assessment procedure. Factors that are considered in this assessment include: age, life expectancy, other co-morbidities, psychological state and lifestyle. Not all PH patients are eligible for transplant. Transplant surgery can considerably improve quality of life and life expectancy. Risks include organ rejection, even long term, and infection. After transplant patients will be on anti-rejection therapy for the rest of their lives and must undergo periodic check-ups.
The reduced availability of donor organs is a very serious issue and this is a key limiting factor for surgery. Much remains to be done in terms of raising awareness to encourage organ donation. In some European countries, such as Austria, there is a so-called “opt-out” system whereby everyone is automatically a donor unless they explicitly say they do not wish to be. Easier access to transplant could be ensured by enacting more favourable donor legislation in Europe.
CTEPH is the only cause of severe pulmonary hypertension which is potentially curable without the need to resort to lung transplantation. Pulmonary endarterectomy (PEA) is the surgical procedure which removes the obstructing thromboembolic material, resulting in significant improvements (and in many cases normalisation) in right ventricular haemodynamics and function. Detailed pre-operative patient evaluation and selection, surgical technique and experience, and meticulous post-operative management are essential prerequisites for success after this intervention.
The selection of patients for surgery depends on the extent and location of the organized thrombi in relation to the degree of PH and taking into consideration age and co-morbidities. Proximal organized thrombi represent the ideal indication while more distal obstructions may prevent a successful procedure. Many people can be completely cured of with CTEPH with surgery. They will still need to undergo regular medical tests and patients will be on anticoagulant therapy for the rest of their lives.