Two-dimensional transthoracic echocardiography (TTE) provides a noninvasive assessment of pulmonary artery pressures. TTE can also identify left ventricular dysfunction or pericardial effusion to assess for causes of elevated pulmonary artery pressures other than PH.
The cardiologist interpreting the TTE confirms that there is no evidence of right ventricular enlargement, right ventricular hypertrophy, right atrial enlargement ,functional tricuspid regurgitation, or a midsystolic notch on the pulmonary artery Doppler flow tracing.) These findings makes PH less likely.
Early PH is difficult to detect in patients with ILD because its symptoms are the same as those of the underlying ILD: progressive dyspnea on exertion and exercise impairment with an insidious onset.
Right heart catheterization is usually reserved only for those patients in whom the severity of PH on echocardiogram is not explained by the severity of their underlying lung disease.
Diagnosis of PH on right heart catheterization would be confirmed by elevated mean pulmonary artery pressure > 25 mm Hg at rest and exclusion of both left ventricular dysfunction and pericardial disease.
Thus, it appears at this stage in diagnosis that this patient likely has SSc-associated ILD, with no evidence of PH. Lung disease in the setting of SSc is associated with a worse prognosis than that seen in patients without pulmonary involvement.
Summary and Clinical Applicability
Early diagnosis and definition of the severity of disease is crucial in treating SSc. Treatment should be initiated as soon as diagnosis is made to achieve a better prognosis. A schedule for re-evaluation and repeated testing should be developed, with the aim of halting disease progression, and if possible, reversing disease-related changes.