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Shone's anomaly: operative results and late outcome
S F Bolling1, M D Iannettoni, M Dick
1Sections of Thoracic Surgery, University of Michigan Medical School, Ann Arbor.
Insights
Shone's anomaly, a complex congenital heart defect, has a poor prognosis. While initial surgeries for left heart obstruction have no mortality, subsequent operations increase risk, particularly due to mitral valve disease.
Area of Science:
- Cardiology
- Congenital Heart Disease
- Pediatric Cardiac Surgery
Background:
- Shone's anomaly is a rare congenital cardiac malformation characterized by multiple left heart obstructions.
- These obstructions include supravalvar mitral ring, parachute mitral valve, subaortic stenosis, and coarctation.
- The prognosis for patients with Shone's anomaly is generally poor.
Purpose of the Study:
- To evaluate the operative results and long-term outcomes of patients with Shone's anomaly.
- To analyze the spectrum of anatomical diagnoses and surgical interventions required.
- To identify factors influencing mortality and morbidity in this patient population.
Main Methods:
- Retrospective review of 30 consecutive patients diagnosed with Shone's anomaly between 1966 and 1989.
- Detailed analysis of anatomical diagnoses, surgical procedures performed, and patient follow-up data.
- Assessment of operative mortality, late mortality, and long-term morbidity.
Main Results:
- The majority of patients presented with multiple obstructive lesions, with 19 having all four classic components.
- A total of 84 operative procedures were performed on 28 patients, with 18 requiring multiple interventions.
- While the first operation had no mortality, mortality increased to 24% after the second operation, primarily due to severe mitral valve disease.
Conclusions:
- Shone's anomaly necessitates complex surgical management involving multiple procedures.
- Mitral valve disease is a critical factor influencing mortality, especially in patients undergoing reoperations.
- Despite surgical challenges, survivors experienced no late or sudden deaths, though morbidity such as congestive heart failure can persist.
Abstract:
Shone's anomaly, a congenital cardiac malformation, consists of multiple levels of left heart obstruction including supravalvar mitral ring, parachute mitral valve, subaortic stenosis, and coarctation. The prognosis for patients with Shone's anomaly is poor. To assess operative results and late outcome, we reviewed the records of 30 consecutive patients seen with Shone's anomaly at our institution between 1966 and 1989. Anatomical diagnoses in these patients were supravalvar mitral ring (22 patients), mitral valve abnormalities including parachute mitral valve, fused chordae, or single papillary muscle (26 patients), subaortic gradients (26 patients), and coarctation (29 patients). Nineteen patients had all four lesions. Other common defects were bicuspid aortic valve (19 patients) and ventricular septal defect (20). Two patients were treated medically. The other 28 patients required 84 operative procedures with 18 patients undergoing more than one procedure. Operations included coarctation repair (28 patients), mitral valve repair or replacement (11), ventricular septal defect closure (8), subaortic resection (8), and complex left ventricular outflow tract reconstruction or bypass (4). Age at first operation ranged from 7 days to 7 years (median age, 3 months). There were no operative deaths at the first operation. However, mortality rose to 24% (4/17) after the second operation. All operative deaths were secondary to severe mitral valve disease. The survivors have been followed from 1 to 16 years (mean follow-up, 6 +/- 1 years). There were no late or sudden deaths. Morbidity has included stroke (1), gastrointestinal bleeding (2), permanent heart block (1), and persistent congestive heart failure (6).(ABSTRACT TRUNCATED AT 250 WORDS)