Systemic and Inflammatory Disease - Cases
Davide Margheri, MD
Radiology resident
University of Padua, Italy
Davide Margheri, MD
Radiology resident
University of Padua, Italy
Amalia Lupi, MD
Radiologist
University of Padua, Italy
Elisabetta Zanatta, MD
Rheumatologist
University of Padua, Italy
Alessandro Rossi
Radiology technician
University of Padua, Italy
Filippo Crimì, MD, PhD
Radiologist
University of Padua, Italy
Giulio Cabrelle, MD
Radiology resident
University of Padua, Italy
Emilio Quaia, MD, PhD
Professor/Radiologist
Institute of Radiology, Department of Medicine, University of Padua, Padua, Italy, Italy
Andrea Doria, MD
Rheumatology Professor
University of Padua, Italy
Alesisa Pepe, MD, PhD
Professor/Cardiologist/Radiologist
Institute of Radiology, Department of Medicine, University of Padua
Padova, Veneto, Italy
The present report describes the case of a 75-year-old woman affected by scleroderma, who was referred to the rheumatology Unit due to a worsening of the hemodynamic decompensation after a recent hospitalization for chronic pulmonary heart with severe pulmonary arterial hypertension (PAH). Rheumatological history revealed Raynaud's phenomenon for over 30 years and positivity to Extractable Nuclear Antigen (ENA) with specificity for anti-centromere antibodies (anti-CEN-P).
Diagnostic Techniques and Their Most Important Findings:
The transthoracic echocardiogram showed no significant involvement of the left ventricle. The patient underwent Cardiovascular Magnetic Resonance (CMR) (Siemens 1.5 T MAGNETOM Avanto Fit) with priority in order to assess potential involvement of the left ventricle for tailoring the therapy of the pulmonary hypertension. The compliance of the patients in the breath-holding was low due to the decompensated state. The CMR protocol included SSFP sequences for quantifying cardiac function and tissue characterization by TIRM T2w, MOLLI and T2p-SSFP sequences for T1- and T2-mapping, respectively, and late gadolinium enhancement (LGE) by PSIR after gadobutrol (0.2 ml/Kg). The CMR scan documented small volumes of the left chambers with a mild reduction of the global systolic ventricular function. We found severely dilated right chambers with a severe reduction of the global systolic ventricular function and a severe tricuspid insufficiency (Figure 1.A), signs of subacute myo-pericardial inflammation (Figures 2 and 3), multiple areas of biventricular replacement fibrosis by LGE (Figure 1. B-C), and an increased extracellular volume (ECV) (32%). Based on the segmental normal cut off value of our Institute by sex and age on 50 healthy subjects, we found elevated global (1024ms) and segmental T1 values and elevated global (54 ms) and segmental T2 values (Figure 3). Due to significant involvement of the left ventricle the patient started the endothelin inhibitor Macitentan in monotheraphy and no additive drugs for the severe PAH were used.
Learning Points from this Case:
This case represents an example of the huge role of the CMR in the management of the scleroderma patients [1-3]. Despite the transthoracic echocardiogram, CMR discovered a significant damage of the left ventricle thanks to the functional and the tissue characterization that avoided a potential dangerous combined therapy in the treatment of the PAH.