Pulmonary hypertension nosography: are all patients classifiable?

Abstract

A 73-year-old never smoking woman was admitted for exertional dyspnoea to the Respiratory Medicine Section of our hospital in June 2014. The patient's past clinical history revealed systemic arterial hypertension, persistent atrial fibrillation, non-insulin dependent diabetes, hypothyroidism, and hypercholesterolemia. She was taking in treatment with acenocoumarol and metformin. The patient had progressive exercise dyspnoea (NYHA class II) and ankle swelling since 2011. She had been diagnosed with interstitial lung disease with pulmonary hypertension (PAPs of 60 mmHg by transthoracic echocardiography) and hypoxemic respiratory insufficiency. She was referred to our hospital following further worsening of dyspnoea and reduction in exercise tolerance despite long-term oxygen therapy (1 L/min). At admission, she had dyspnoea on minor activities (modified Medical Research CouncilmMRC = 3, NYHA class III), cyanosis, increased respiratory rate (22 breaths/min), and ankle swelling. She had systemic pressure at lower limits of normality (105/ 65 mmHg) and a BMI of 28 (158 cm, 71 kg). At physical examination, fine crackles were audible at both lung bases. She had low (54 mmHg) arterial oxygen tension in oxygen therapy with 1 L/min with CO 2 tension of 50 mmHg, in a framework of compensated respiratory acidosis (HCO 3 -33 mmHg). No alterations of serum electrolytes were found. We got sufficient correction of hypoxia (PaO 2 63 mmHg) by increasing flow oxygen support at 3 L/min with a modest increase of hypercapnia (PaCO 2 54 mmHg). The chest radiograph showed increased cardiac size, bulging of the second arch on the left mediastinal border compatible with enlargement of the pulmonary artery common trunk (Fig. 1). No signs of pulmonary congestion were observed. The electrocardiogram showed atrial fibrillation with average ventricular rate of 77 beats per minute, intraventricular conduction delay, and right axial deviation. Transthoracic echocardiography showed severe tricuspidal regurgitation with TAPSE (tricuspid annular plane systolic excursion) of 15 mm, PAPs of 70 mmHg, right and left atrial enlargement, interventricular septum paradoxical movement, inferior vena cava dilatation with no change on inspiration, mild pericardial effusion, and normal size and wall thickness of the left ventricle, without alteration on the segmental kinesis. Cardiac ejection fraction was preserved (61%). No diastolic relaxation abnormalities were detected. E/A ratio [early transmitral flow (E) and the late flow with atrial contraction (A)] was 0.8 and the ratio E/e 0 [early filling velocity on transmitral Doppler (E) with the early relaxation velocity on tissue Doppler (e 0 )] was 7. Only mild mitral regurgitation was described. Calcification of the cusps of the aortic valve with normal opening was described. Aortic valve peak gradient was 22 mmHg.We decided to treat the patient with furosemide 20 mg iv TID, oxygen therapy (3 L/min) with a partial clinical