Shone's syndrome diagnosed with echocardiography and confirmed at pathology.
The Shone's complex, defined by four cardiovascular defects such as a supravalvular mitral membrane, valvular mitral stenosis by a parachute mitral valve, subaortic stenosis, and aortic coarctation, is a rare entity, which occurs most frequently in its incomplete form. We report the case of a 19-year-old female patient who presented at the emergency room for progressively worsening dyspnoea, orthopnoea, fever, and productive cough, due to bronchopneumonia. Echocardiography revealed the co-existence of aortic coarctation with bicuspid aortic valves, mitral supravalvular ring, and dysplastic mitral valves producing severe mitral stenosis and severe pulmonary hypertension. Although wide spectrum antibiotics were administered from the first day of hospitalization, the patient developed severe sepsis and died. The components of the Shone's complex diagnosed by echocardiography were confirmed at pathology.
Popescu
Bogdan A
BA
'Prof. Dr. C. C. Iliescu' Institute of Cardiovascular Diseases, Bucharest, Romania. bogdan.a.popescu@gmail.com
Jurcut
Ruxandra
R
Serban
Marinela
M
Parascan
Liliana
L
Ginghina
Carmen
C
D000015:Abnormalities, Multiple / Q000000981:diagnostic imaging* / Q000473:pathology; D001017:Aortic Coarctation / Q000000981:diagnostic imaging* / Q000473:pathology; D017809:Fatal Outcome; D005260:Female; D006330:Heart Defects, Congenital / Q000000981:diagnostic imaging; D006801:Humans; D006976:Hypertension, Pulmonary / Q000000981:diagnostic imaging* / Q000209:etiology; D008943:Mitral Valve / Q000002:abnormalities / Q000000981:diagnostic imaging*; D008946:Mitral Valve Stenosis / Q000151:congenital / Q000000981:diagnostic imaging* / Q000473:pathology; D013577:Syndrome; D014463:Ultrasonography; D055815:Young Adult