Repair of isolated partial anomalous pulmonary venous return (eg, Scimitar Syndrome)
CPT4 code
Name of the Procedure:
Repair of Isolated Partial Anomalous Pulmonary Venous Return (PAPVR) (eg, Scimitar Syndrome)
Summary
In this procedure, a surgeon corrects an abnormality where one or more of the pulmonary veins carrying blood from the lungs to the heart do not connect normally. These veins might drain into the right atrium instead of the left atrium, which can lead to various complications. The surgery reroutes the veins to ensure proper blood flow.
Purpose
This surgery addresses Scimitar Syndrome or other forms of partial anomalous pulmonary venous return. The goal is to correct the abnormal vein connections to improve blood circulation, reduce symptoms, and prevent complications such as heart failure or pulmonary hypertension.
Indications
- Symptoms such as difficulty breathing, recurrent respiratory infections, or fatigue.
- Evidence of abnormal pulmonary vein connections on diagnostic imaging.
- Right-sided heart enlargement or heart failure symptoms.
Preparation
- Patients may need to fast for several hours before the surgery.
- Preoperative assessments including blood tests, echocardiograms, and possibly cardiac catheterization.
- Discontinuation of certain medications as advised by the healthcare provider.
Procedure Description
- The patient is placed under general anesthesia.
- An incision is made in the chest, typically through a median sternotomy.
- The heart may be placed on a cardiopulmonary bypass machine.
- The surgeon identifies the anomalously draining pulmonary vein(s).
- The veins are disconnected from their abnormal location and re-implanted into the left atrium.
- The chest incision is closed, and the patient is moved to the recovery area.
Tools and technology include surgical instruments, cardiopulmonary bypass system, and diagnostic imaging aids.
Duration
The procedure typically takes 3-5 hours.
Setting
The surgery is performed in a hospital operating room.
Personnel
- Cardiothoracic surgeon
- Anesthesiologist
- Surgical nurses
- Perfusionist (for managing the bypass machine)
- Physician assistants or surgical technologists
Risks and Complications
Common risks include infection, bleeding, or reaction to anesthesia. Rare complications might involve abnormal heart rhythms, damage to heart structures, or blood clots. Postoperative monitoring and management are crucial to mitigate these risks.
Benefits
Successful repair can improve heart function, reduce symptoms, and prevent future complications. Benefits might be realized soon after recovery, usually within several weeks to months post-surgery.
Recovery
- Initial recovery in the intensive care unit (ICU) for close monitoring.
- Gradual transition to a regular hospital ward before discharge.
- Pain management with medication.
- Instructions on activity restrictions, wound care, and follow-up visits.
- Full recovery typically takes several weeks to months.
Alternatives
- Medical management of symptoms, although not curative.
- Less invasive catheter-based interventions in select cases. Pros and cons: Medical management may only provide symptom relief, while catheter-based interventions may not be suitable for all types of anomalous veins.
Patient Experience
Patients will be under general anesthesia during the surgery and won't feel any pain. Postoperatively, they might experience discomfort from the chest incision which is managed with pain medication. Patients can expect some limitations in physical activity during the initial recovery period, with gradual improvement in symptoms such as fatigue and breathlessness.
Pain management and comfort measures include medication, supportive care, and possibly physical therapy to enhance the recovery process.