Cardiology Letters 2017, 26(1):47-51

Pulmonary hypertension associated with systemic sclerosis - too many grey zones

Luknár M¹, Lesný P¹, Tuchyňová A², Rovenský J², Malinová J³, Gonçalvesová E¹

¹ Centrum pre pľúcnu hypertenziu, Oddelenie zlyhávania a transplantácie srdca, Národný ústav srdcových a cievnych chorôb, Bratislava

² Národný ústav reumatických chorôb, Piešťany

³ Lekárská fakulta Univerzita Komenského v Bratislave, Slovenská republika

Background: Pulmonary arterial hypertension (PAH) is an important cause of death in systemic sclerosis (SSc). Diagnosis of PAH is based on strict arbitrary hemodynamic criteria. Borderline conditions with pulmonary artery pressures in grey zones are not rare in SSc patients. The diagnostic, prognostic, and therapeutic significance of these findings has not been satisfactorily clarified. Correct diagnostic conclusion has a major impact on appropriate treatment decisions.

Aim: To describe the results of diagnostic work-up in patients with SSc in a single centre referred for clinical or echocardiographic suspicion of PAH with special regard to borderline conditions.

Patients and methods: 104 patients (pts) with SSc were referred by rheumatologists to a PAH centre. Cardio­ vascular examination including echocardiography was performed. 57 pts (7 men) with suspicion of PAH underwent a complete PAH diagnostic algorithm including right heart catheterization. Median age was 61 (39-80 years). Estimated systolic pulmonary artery pressure <50 mm Hg or no tricuspid regurgitation was detected in 15 pts: however, catheterization was performed due to unexplained dyspnea.

Results: PAH was diagnosed in 23 (40%) pts who underwent right heart catheterization. Group II pulmonary hypertension due to left ventricular diastolic dysfunction was diagnosed in 12 (21%) pts. In 22 (39%) patients, no pulmonary hypertension was detected. Mean pulmonary arterial pressure of 21-24 mm Hg was present in 7 (32%) pts who did not meet the pulmonary hypertension criteria. Pulmonary wedge pressure of 12 to 15 mm Hg was present in 7 (30%) PAH patients. Of 15 pts with estimated systolic pulmonary pressure of <50 mm Hg or no tricuspid regurgitation on echo, 2 (13%) were diagnosed with PAH and 5 (33%) with group II pulmonary hypertension. Complications of right heart catheterization were rare.

Conclusion: Prevalence of PAH in a risk group of patients with SSc referred for clinical or echocardiographic suspicion of PAH is relatively high. However, borderline hemodynamic conditions in patients with dyspnea in context of SSc are frequent. Borderline situations require a correct interpretation and detailed evaluation as treatment strategy and patient outcome depend on a thorough diagnosis.

Keywords: pulmonary hypertension; systemic sclerosis; borderline pressures

Published: January 1, 2017  Show citation