Case presentation
A 37-year-old female patient with petechia and purpura from 7 months ago
was diagnosed with leukocytoclastic vasculitis confirmed by biopsy.
Despite receiving anti-inflammatory treatments, she was admitted to our
hospital due to the gradual progression of constitutional symptoms,
pancytopenia, and new pulmonary and renal involvement. Diffuse petechia
and purpuric lesions were detected, especially in the lower limb. In
Table 1, initial lab data results are shown.
Splenomegaly was detected in the abdominal ultrasound. Computed
tomography (CT) of the chest showed consolidations in the left upper
lobe. Bone marrow aspiration and biopsy showed 40% cellularity with
increased erythroid precursors. Megaloblastic changes in both erythroid
and myeloid precursors were detected as well. According to these
findings, myelodysplastic syndrome secondary to leukocytoclastic
vasculitis was the main reason for megaloblastic changes. Megaloblastic
anemia was also considered a second differential diagnosis. Due to the
history of VSD in childhood, a tans thoracic echocardiogram was done.
Multiple mobile masses in RVOT and highly mobile masses at the atrial
side of the tricuspid valve and the ventricular side of the pulmonary
valve were seen (Figure 1, video). A turbulent flow in RVOT with a peak
gradient of about 80 mmHg was detected at the hypertrophied muscle
bundle site (Figure 1, video). A transesophageal echocardiogram for
further evaluation was performed. The muscle bundle in the right
ventricle (RV) was divided it into two chambers: A proximal chamber on
the side of the RV inflow and a distal chamber on the side of the
pulmonary valve. Many masses were attached to both the muscle bundle and
along the distal chamber involving RV endothelium and pulmonary valve
(Figure, video). Multiple masses and destruction of the tricuspid valve
leaflets resulted in severe regurgitation (Figure, video).
The patient was diagnosed with DCRV and IE. After consultation with the
cardiac surgeon and infectious disease specialist, empiric antibiotics
therapy was initiated, and the patient underwent cardiac surgery.
Pulmonary and tricuspid valve replacement with bioprosthesis valves was
performed. Although VSD was not found in the echocardiogram, probably
due to high RV pressure, surgery proved it and was repaired with a
pericardial patch. The patient’s condition improved after surgery and
antibiotics therapy, and she was discharged with normal lab results. The
patient was in good clinical and echocardiographic condition and free of
any major event in the one-year follow-up.