Holt-Oram Syndrome Treatment & Management

Updated: May 05, 2016
  • Author: Craig T Basson, MD, PhD; Chief Editor: Yasmine Subhi Ali, MD, FACC, FACP, MSCI  more...
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Treatment

Medical Care

A clinical evaluation can usually be performed in an outpatient setting, but inpatient studies and surgical treatment may be necessary. Admit patients for cardiovascular testing and surgical intervention.

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.

No specific medications are indicated for this condition. However, antibiotic prophylaxis and anticoagulation may be required, depending on the severity of congenital heart disease.

Transfer may be required for further diagnostic evaluation and surgical intervention.

Consultations

Obtain consultations with the following specialists:

  • Cardiologist
  • Geneticist
  • Cardiothoracic surgeon
  • Orthopedic surgeon

Diet and activity

No special diet is required. Limit activity if heart failure or persistent cardiac sequelae are present.

Prevention

No known causative environmental factors are described; therefore, no particular deterrent is available.

Long-term monitoring

Follow up with patients with significant congenital heart disease at least annually. Periodic follow-up for cardiac conduction disease is warranted in all affected individuals.

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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.

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