Holt-Oram Syndrome Treatment & Management
- Author: Craig T Basson, MD, PhD; Chief Editor: Park W Willis IV, MD more...
Medical Care
- Evaluation can usually be performed in an outpatient setting, but inpatient studies and surgical treatment may be necessary.
- Patients with advanced heart block may require a permanent pacemaker.
- Surgical therapy can be used to correct cardiac defects or to possibly improve limb function.
Surgical Care
- Most cardiac lesions such as ASD and VSD are amenable to complete surgical correction if pulmonary hypertension or ventricular failure has not developed. Several percutaneous transcatheter devices that can be placed to occlude the septum are in US Food and Drug Administration–approved clinical trials and may be nonsurgical options in the future.
- Septal defects without hemodynamically significant shunts do not require correction.
- Children with severe limb anomalies can be referred to orthopedic surgeons for consideration of procedures such as pollicization of the fifth digit (to improve upper limb function).
- Children with severe limb shortening may benefit from prostheses.
Consultations
- Cardiologist
- Geneticist
- Cardiothoracic surgeon
- Orthopedic surgeon
Diet
No special diet is required.
Activity
Limit activity if heart failure or persistent cardiac sequelae are present.
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