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Congenital cardiac anomalies in myelomeningocele patients
Iman Moeini Naghani1, Taraneh Hashemi Zonouz1, Shima Shahjouei1
1Department of Neurosurgery, Children's Hospital Medical Center, Tehran University of Medical Science, Tehran, Iran.
Insights
Congenital heart defects are uncommon in patients with myelomeningocele (MMC). Echocardiography is recommended for female MMC patients with skeletal anomalies and suspicious cardiac exams to detect potential heart defects.
Area of Science:
- Pediatric Cardiology
- Developmental Biology
- Clinical Genetics
Background:
- Myelomeningocele (MMC) is a complex congenital anomaly often associated with other malformations.
- The incidence of congenital heart disease (CHD) in MMC patients varies and appears more prevalent with skeletal deformities.
Purpose of the Study:
- To evaluate the occurrence of cardiac anomalies in pediatric patients diagnosed with myelomeningocele.
- To investigate the correlation between myelomeningocele characteristics, skeletal malformations, and the presence of congenital heart disease.
Main Methods:
- A cohort of 75 myelomeningocele patients underwent clinical examination, electrocardiography, and echocardiography.
- Data collected included patient demographics, MMC location, orthopedic deformities, neurological deficits, and radiographic findings.
Main Results:
- Cardiac anomalies were identified in only two out of 75 patients (2.7%) via echocardiography.
- Both affected patients were female and presented with severe scoliosis, rib deficiencies, and vertebral anomalies.
Conclusions:
- Congenital heart defects are not highly prevalent in myelomeningocele patients.
- Echocardiography is advised for female MMC patients with skeletal anomalies and suggestive clinical findings to rule out associated cardiac defects.
Objective:
Myelomeningocele may be isolated but more frequently is associated with other anomalies. Congenital heart disease occurs with different incidence rate in myelomeningocele which is observed more frequently with skeletal malformations.
Methods:
This study was undertaken in the Children's Hospital Medical Center between 2010 to 2012 to evaluate 75 myelomeningocele patients for cardiac anomalies, with electrocardiography and echocardiography in addition to clinical examination of the cardiopulmonary system. Demographic information, myelomeningocele location and characteristics, orthopedic deformities, neurological deficits and radiographic findings were studied besides cardiologic assessments.
Results:
The ages of the patients ranged from 1 day to 4 years. The myelomeningocele locations were lumbosacral, lumbar and sacral area in most cases. Physical examination of the heart was abnormal in 6 children, but echocardiography revealed cardiac anomalies in only two children. Both children were female patients with severe scoliosis, multiple rib deficiencies and associated vertebral anomalies.
Conclusion:
Congenital heart defects are not very common in MMC patients. Female patients with suspicious clinical examinations for cardiac anomalies and associated rib and vertebral anomalies are advised to be investigated by echocardiography to rule out associated cardiac anomalies.
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