Right aortic arch with aberrant left subclavian artery and left sided superior vena cava

Case contributed by Dr René Pfleger

Presentation

Malaise, unintended weight loss, low grade fevers and night sweats for 6 weeks, onset shortly after acute respiratory infection (influenza-like illness).

Patient Data

Age: 50 years
Gender: Male

No acute abnormalities. No clear lymphadenopathy revealed. Spleen size near upper limit.
Right-sided aortic arch, vascular ring with aberrant left subclavian. Slight distension of upper third of esophagus.

Left-sided SVC, draining into coronary sinus (though hard to appreciate on the 3mm slices provided).

Dilated right ventricle. Implantable loop recorder in subcutaneous tissue of left chest.

Syndesmophytes, ankylosis of vertebral column and SI joints. Arthritic changes in both hips.

Incidental note of sub-centimeter non-calcified nodule in right thyroid lobe and small fat-containing right-sided inguinal hernia.

Diffuse syndesmophyitic ankylosis (in this case sparing LEFT not right side of thoracic vertebral column).

Ossification of spinal ligaments, joints and discs.

End-stage sacroiliitis with sacroiliac joint spaces not clearly discernible.

Case Discussion

A beautiful example of vascular anomalies in a patient with congenital heart disease.

Patient history positive for known ventricular septal defect (VSD) - perimembranous restrictive type - and patent ductus arteriosus Botalli (PSD), the latter been corrected in early childhood.

Blood cultures turned out to be positive for streptococcus spp, causing endocarditis adjacent to VSD, proven by trans esophageal echocardiography and treated by 6 weeks of antibiotics.

Coincidental skeletal findings are consistent with ankylosing spondylitis.

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