Pediatric Holt-Oram Syndrome Treatment & Management

Updated: Aug 05, 2015
  • Author: Poothirikovil Venugopalan, MBBS, MD, FRCPCH; Chief Editor: Syamasundar Rao Patnana, MD  more...
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Treatment

Medical Care

Acute-stage management in patients with Holt-Oram syndrome

Treatment is directed at the cardiac manifestations. Care may be necessary with exacerbations of heart failure, for interventional procedures, or for surgical correction of heart disease. Administer medications as dictated by the heart disease.

Establish a complete diagnosis, and counsel the patient and family.

Consultations

Consult with the following:

  • Pediatric cardiologist
  • Orthopedic specialist
  • Radiologist
  • Nuclear medicine specialist
  • Family physician
  • Occupational therapist
  • Physiotherapist
  • Psychologist
  • School teacher
  • Specialist nurse
  • Pharmacist
  • Dietitian

Transfer

Transfer may be necessary for further evaluation and surgical intervention.

Diet and activity

Patients with Holt-Oram syndrome may require dietary modification because of their specific cardiac abnormality.

No activity restrictions are required unless a specific cardiac abnormality indicates otherwise.

Outpatient monitoring

Regular follow-up is essential for both cardiac and skeletal conditions. The patient should be seen by orthopedic and occupational therapists, as well as by physiotherapists.

At each visit, the importance of prevention of deformity must be emphasized.

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Surgical Care

Cardiact defect

Treatment for the heart defect includes the following:

  • Appropriate surgical or nonsurgical correction of the heart defect is indicated and possible. A vast majority of atrial septal defects may be closed by transcatheter methods.
  • Palliative surgery, as indicated, if complete correction is not possible

Musculoskeletal defects

Treatment for musculoskeletal defects may include the following:

  • Take adequate measures to prevent acquired deformities and to treat existing deformities.
  • Surgical management of hand anomalies depends on the age, pattern, and degree of accompanying malformations of the upper limb.
  • Pollicization to improve function of index finger is recommended for patients with aplasia of the thumb. This is also recommended following amputation of a rudimental thumb.
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Long-Term Monitoring

 

 

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