A rare case of partial Shone complex in adulthood: coarctation-site methicillin-sensitive Staphylococcus aureus
Murat Akdoğan1, Burak Karaman1, Selen Münüsoğlu1
1Department of Cardiology, Etlik City Hospitalhttps://ror.org/01nk6sj42, Ankara, Türkiye.
Insights
This case details a rare adult Shone complex presentation with Staphylococcus aureus infection and aneurysm at the coarctation site. Recurrent infective endocarditis highlights the need for vigilant imaging and long-term follow-up in complex congenital heart disease.
Area of Science:
- Cardiology
- Infectious Diseases
- Medical Imaging
Background:
- Shone complex is a rare congenital cardiovascular anomaly with multiple left-sided obstructive lesions.
- Adult presentation is uncommon and can manifest atypically, especially with infective endocarditis.
- Infective endocarditis associated with coarctation of the aorta is particularly rare and clinically risky.
Purpose of the Study:
- To report a rare case of adult partial Shone complex complicated by infective endocarditis.
- To illustrate the diagnostic challenges and management of recurrent infections in complex congenital heart disease.
- To emphasize the importance of multimodality imaging and long-term follow-up.
Main Methods:
- Case report of a 25-year-old male with partial Shone complex.
- Diagnosis of methicillin-sensitive Staphylococcus aureus infective endocarditis confirmed by blood cultures.
- Multimodality imaging including echocardiography and positron emission tomography.
- Surgical repair followed by antibiotic therapy and long-term follow-up.
Main Results:
- The patient presented with fever, diagnosed with infective endocarditis, and had a saccular aneurysm at the coarctation site.
- Initial treatment involved antibiotics and surgical repair, but recurrent infection developed.
- Repeat treatment included antibiotics and long-term suppressive therapy, with persistent mitral regurgitation.
Conclusions:
- This case highlights a rare adult Shone complex with coarctation-site infection, aneurysm, and recurrent graft-associated infective endocarditis.
- Vigilant multimodality imaging is crucial for diagnosing and managing such complex cases.
- Early multidisciplinary evaluation and long-term follow-up are essential for adults with complex congenital heart disease and infectious complications.
Background:
Shone complex is a rare congenital cardiovascular anomaly characterized by multiple left-sided obstructive lesions. Adult presentation is uncommon and may result in atypical clinical manifestations, particularly when complicated by infective endocarditis. Coarctation of the aorta-associated infective endocarditis is especially rare and carries significant clinical risk.
Case Summary:
We report a 25-year-old male with partial Shone complex consisting of a parachute mitral valve, mitral stenosis, and coarctation of the aorta. The patient presented with persistent high-grade fever and was diagnosed with methicillin-sensitive Staphylococcus aureus infective endocarditis based on positive blood cultures. Transthoracic and transesophageal echocardiography revealed obstructive left-sided lesions, mitral valve pathology, and moderate aortic regurgitation. Positron emission tomography demonstrated intense hypermetabolic activity at the coarctation site, consistent with active infection and confirming a saccular aneurysm. The patient underwent prolonged intravenous cefazolin therapy followed by surgical repair. The early postoperative course was stable; however, seven months later he re-presented with recurrent fever. Repeat imaging demonstrated graft infection, vegetation on the prosthetic material, and a perforated mitral leaflet. A second course of intravenous antibiotics (cefazolin and rifampicin) was administered, followed by long-term suppressive oral therapy. Subsequent follow-up showed persistent moderate-to-severe mitral regurgitation with stable postoperative anatomy.
Conclusion:
This case highlights a rare adult manifestation of partial Shone complex complicated by coarctation of the aorta-site methicillin-sensitive Staphylococcus aureus infection, saccular aneurysm formation, and recurrent graft-associated infective endocarditis. The clinical course underscores the importance of vigilant multimodality imaging, early multidisciplinary evaluation, and long-term follow-up in adults with complex CHD, particularly when infectious complications are present.
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