Treatment of pulmonary arterial hypertension secondary to chronic pulmonary thromboembolic disease is challenging and according to the established guidelines [4] for pulmonary thromboendarterectomy should be considered for patients with the following criteria: 1) NYHA functional class III or IV symptoms; 2) a preoperative pulmonary vascular resistance greater than 300 dynes.s.cm-5, or 800 dynes.s.cm-5 according to other authors [5]; 3) surgical accessible thrombus (in main, lobar, or segmental pulmonary arteries), as determined by the appropriate studies; and 4) no severe comorbidities. In addition, some centres have suggested that mean PAP preoperatively should be at least ≥ 40 mmHg.
Our patient clearly doesn't fill the first criteria since under treatment [6] with sildenafil 25 mg three times daily she has an excellent 6 MWT of 600 meters. However she is young and complains of exertional dyspnoea. Besides exercise echocardiography disclose seriously impaired pulmonary vascular vasodilator reserve. We admit that, despite clinical benignity, the patient still had severely limited pulmonary vasodilator reserve and we performed pulmonary angiography.
In face of the result of the exercise echo we decided to refer the patient to a centre with experience in pulmonary endarterectomy.
The presence or absence of this sign – right ventricular dilatation with exercise – should, in our opinion, be evaluated in patients with chronic pulmonary thromboembolic disease and pulmonary hypertension whenever there are uncertainties about the indication for surgical pulmonary endarterectomy. We think that this sign is probably ominous and should almost certainly reinforce the indication for surgery.
The acute morphologic changes in cardiac anatomy reflect the changes in right and left ventricular haemodynamics,. Echocardiography might be helpful in evaluating the acute hemodynamic response to pulmonary output increase that exercise elicits. The practical utility of this concept for clinical decision is demonstrated in this case report