Evaluating Pulmonary Arterial Hypertension in Patients With Systemic Sclerosis

Evaluation of Lung Doppler Signals (LDS) in Detecting Pulmonary Arterial Hypertension in Patients With Systemic Sclerosis (SSc)

Doppler signals can be recorded from the lung parenchyma by means of a pulsed Doppler ultrasound system incorporating a special signal processing package- the Transthoracic Parametric Doppler (TPD) (Echosense Ltd., Haifa, Israel). Systemic sclerosis patients often develop pulmonary vascular disease leading to pulmonary hypertension. The TPD system may provide important insight into pulmonary blood vessels characteristics by the LDS (Lung Doppler Signals) signals that are related to pulmonary hypertension. The TPD performance in detecting PAH in SSc patients will be assessed in the study.

Inclusion Criteria:

1. Able and willing to give signed informed consent prior to enrollment
2. Male or female, ≥ 18 years of age
3. Diagnosis of SSc according to 2013 ACR/EULAR SSc Classification Criteria (van den Hoogen 2013): Score ≥ 9.
4. Patients with RHC data available from measurement within 3 weeks prior to TPD assessment
5. No change in or initiation of PAH specific therapy between the last RHC and TPD

Exclusion Criteria:

1. People unable or unwilling to give informed consent.
2. PCWP or LVEDP > 15 mmHg
3. Any PH etiology outside Group 1 (Dana Point, 2008)
4. Pregnant women
5. Patients having severe chest wall deformity