Editorial
Balloon pulmonary angioplasty for chronic thromboembolic pulmonary hypertension

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare but potentially life-threatening disease of the pulmonary circulation [1]. The pathogenesis of CTEPH is not entirely clear. The most accepted scenario is that of aborted recanalisation of pulmonary arteries after a thromboembolic episode. While some post-embolic residua may persist in up to 50% of survivors of acute pulmonary embolism (aPE), only 0.5% to 2% will progress to CTEPH [2, 3]. This is believed to occur in the presence of significant redistribution of flow to remaining unoccluded pulmonary bed with resulting elevation of intravascular pressure and shear stress. Remodelling of initially patent pulmonary arterioles leads to an increase in pulmonary vascular resistance similar to that observed in left-to-right shunting in congenital heart disease. Progressive uncoupling of pulmonary and right ventricular elastance results in a fall of pulmonary flow, left ventricular preload, systemic blood pressure, and right ventricular (RV) coronary perfusion leading to right heart failure with severe functional disability and eventually to death.
Management of CTEPH requires precise differential diagnosis and qualification for surgical treatment by an experienced multidisciplinary team. Indeed, in operable patients pulmonary endarterectomy (PEA) is highly effective in restoring functional status and improving life expectancy. A surgical technique has been optimised and implemented worldwide by a group from San Diego – University of California [4]. Nevertheless, PEA performed in deep hypothermia and intermittent total cardiac arrest remains one of the most demanding cardiovascular interventions and is performed only in a limited number of highly dedicated centres. As an example, Papworth Hospital is the only centre performing PEA in the UK, while Marie-Lannelongue Hospital in Paris remains a referral centre for France for this type of surgery. Usually, individual cardiac surgeons are responsible for PEA in their centres, as the learning curve for this intervention has been well documented [5]. With growing experience of clinicians, radiologists, surgeons, and anaesthesiologists, an increasing proportion of patients with CTEPH may benefit from PEA despite distal, less accessible intravascular residua and/or advanced age and comorbidities. This is of paramount importance since the outcome of non-operated patients is drastically worse (Figure 1), despite identical baseline haemodynamic...


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