Lutembacher Syndrome in a Child with Chronic Tonsillopharyngitis
Background: Lutembacher syndrome (LS) is a condition where congenital atrial septal defect (ASD) and acquired rheumatic mitral stenosis (MS) co-occur, seen mainly in adult population. We report LS incidentally detected in a child, while seeking medical care for unrelated problem. Clinical Descriptio...
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| Main Authors: | , |
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| Format: | Article |
| Language: | English |
| Published: |
Wolters Kluwer Medknow Publications
2025-01-01
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| Series: | Indian Pediatrics Case Reports |
| Subjects: | |
| Online Access: | https://journals.lww.com/10.4103/ipcares.ipcares_168_24 |
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| Summary: | Background:
Lutembacher syndrome (LS) is a condition where congenital atrial septal defect (ASD) and acquired rheumatic mitral stenosis (MS) co-occur, seen mainly in adult population. We report LS incidentally detected in a child, while seeking medical care for unrelated problem.
Clinical Description:
A 13-year-old boy presented with chronic throat pain and was planned for tonsillectomy. During pre-anesthetic checkup, a cardiac murmur was detected and sent for cardiac evaluation. He was otherwise asymptomatic in the past. On examination, he was underweight with stable vitals, without edema, cyanosis, clubbing, or engorged neck veins. Auscultation revealed normal S1, wide split S2 with A2 >P2, and short ejection systolic in pulmonary area.
Management and Outcome:
Routine laboratory investigations were normal. Electrocardiogram showed sinus rhythm, with normal PR and QT intervals. QRS axis – Right axis, normal “P” axis, incomplete RBB (V1–V3 leads) with right ventricle dominant forces. Echocardiography showed a 20 mm ASD, along with MS, thickened anterior mitral leaflet, thickened and fixed posterior mitral leaflet, and chordal thickening, consistent with rheumatic MS, thereby confirming LS. The child was started on regular penicillin prophylaxis, and was advised tonsillectomy.
Conclusion:
This case creates awareness among pediatricians regarding the possible occurrence of LS in children having a chronic source of streptococcal infection like tonsillopharyngitis. Knowledge about this condition and astute echocardiographic evaluation can identify this condition. |
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| ISSN: | 2772-5170 2772-5189 |