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Aortic root translocation with ventricular septal defect and pulmonary stenosis repair (ie, Nikaidoh procedure); with reimplantation of 1 or both coronary ostia

CPT4 code

Name of the Procedure:

Aortic Root Translocation with Ventricular Septal Defect and Pulmonary Stenosis Repair (Nikaidoh Procedure); with Reimplantation of One or Both Coronary Ostia

Summary

The Nikaidoh procedure is a complex heart surgery used mainly in children to correct congenital defects involving the heart's major blood vessels and chambers. This procedure relocates the aortic root and reconstructs the heart, including the repair of a hole between the heart's chambers and addressing any narrowing of the pulmonary valve or artery.

Purpose

The procedure addresses congenital heart conditions such as transposition of the great arteries, ventricular septal defect (VSD), and pulmonary stenosis (narrowing of the pulmonary valve or artery). It aims to ensure proper blood flow through the heart and major arteries and to ensure the heart functions efficiently.

Indications

  • Congenital heart defects such as transposition of the great arteries with ventricular septal defect (VSD) and pulmonary stenosis.
  • Symptoms like difficulty breathing, poor feeding, bluish skin (cyanosis), and growth delays in infants.
  • Ineffectiveness of other, less invasive treatments to correct these defects.

Preparation

  • Patients (or guardians) may be instructed to ensure fasting for a specific duration before the surgery.
  • Preoperative diagnostic tests such as echocardiograms, MRIs, or CT scans to evaluate the heart's structure.
  • Blood tests and thorough medical evaluations to determine overall fitness for surgery.
  • Adjustment or stoppage of certain medications as advised by the physician.

Procedure Description

  1. Anesthesia: The patient is put under general anesthesia.
  2. Incision: A chest incision is made to access the heart.
  3. Cardiopulmonary Bypass: The patient is connected to a heart-lung machine to maintain blood circulation and oxygenation during surgery.
  4. Aortic Root Translocation: The aortic root is detached and repositioned to the left ventricle.
  5. VSD Repair: The hole between the heart's lower chambers (ventricular septal defect) is closed.
  6. Pulmonary Stenosis Correction: The pulmonary valve or artery is widened to ease blood flow.
  7. Coronary Reimplantation: One or both coronary arteries are reattached to ensure proper blood supply to the heart.
  8. Closure: Incisions are closed, and the patient is gradually weaned off the heart-lung machine.

Duration

The procedure typically takes between 6 to 8 hours.

Setting

Performed in a hospital's specialized cardiac surgery operating room.

Personnel

  • Cardiothoracic surgeon
  • Anesthesiologist
  • Surgical nurses
  • Perfusionist (to manage the heart-lung machine)
  • Cardiologists (for pre- and post-operative care)

Risks and Complications

  • Common risks include bleeding, infection, or adverse reactions to anesthesia.
  • Specific complications may involve arrhythmias, coronary artery complications, or incomplete repair requiring additional surgeries.
  • Long-term risks can include heart valve dysfunction or heart failure.

Benefits

  • Improved heart function and circulation.
  • Alleviation of symptoms such as cyanosis and growth delays.
  • Enhanced quality of life and overall health.

Recovery

  • Initial recovery in the intensive care unit (ICU) for monitoring and support.
  • Gradual transfer to a regular hospital room as stability improves.
  • Total hospital stay ranges from 1 to 2 weeks.
  • Follow-up appointments for ongoing assessments.
  • Restrictions on physical activity to allow healing, with gradual return to normal activities over weeks to months.

Alternatives

  • Less invasive heart procedures like balloon angioplasty (in specific cases).
  • Other surgical repairs including Rastelli procedure or arterial switch operation.
  • Each alternative comes with its set of risks and benefits, dependent on the specific heart condition and patient characteristics.

Patient Experience

  • The patient may experience discomfort and soreness post-surgery.
  • Pain management through medications and comfort measures will be provided.
  • Limited physical activity initially; gradual increase as recovery progresses.
  • Emotional support and reassurance from healthcare providers and family are crucial during recovery.

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