Repair of single ventricle with aortic outflow obstruction and aortic arch hypoplasia (hypoplastic left heart syndrome) (eg, Norwood procedure)
CPT4 code
Name of the Procedure:
Norwood Procedure, Repair of single ventricle with aortic outflow obstruction and aortic arch hypoplasia, Hypoplastic Left Heart Syndrome (HLHS) surgery
Summary
The Norwood procedure is a complex heart surgery designed to treat babies born with hypoplastic left heart syndrome (HLHS), a condition where the left side of the heart is severely underdeveloped. This procedure reconstructs the heart and its blood vessels to ensure proper blood flow throughout the body.
Purpose
This procedure addresses hypoplastic left heart syndrome (HLHS), a congenital heart defect. The primary goal is to reroute the blood flow in the heart, enabling adequate oxygenation and improving survival rates in newborns with this condition.
Indications
- Diagnosis of hypoplastic left heart syndrome (HLHS)
- Severe aortic outflow obstruction
- Hypoplasia of the aortic arch
- Newborns and infants typically within the first few weeks of life
Preparation
- Pre-surgery fasting as instructed by the healthcare team
- Adjustment or discontinuation of certain medications
- Comprehensive diagnostic tests, including echocardiography and cardiac catheterization
- Detailed physical examination and medical history review
Procedure Description
- General anesthesia administered to ensure the patient is unconscious and pain-free.
- The chest is opened via a midline incision to access the heart.
- The main pulmonary artery is connected to the aorta to redirect blood flow.
- A new aorta is constructed using parts of the existing aorta and the pulmonary artery.
- Placement of a shunt to direct blood to the lungs for oxygenation.
- Surgical closure and the patient is transferred to the intensive care unit for monitoring.
Duration
The procedure typically takes around 4-6 hours.
Setting
Performed in a specialized hospital with pediatric cardiac surgery capabilities, typically in an operating room.
Personnel
- Pediatric cardiac surgeon
- Pediatric anesthesiologist
- Surgical nurses
- Perfusionist (operates heart-lung machine)
- Pediatric cardiologist
Risks and Complications
- Bleeding
- Infection
- Blood clots
- Heart rhythm abnormalities
- Reduced oxygen delivery to body tissues
- Need for additional surgeries or procedures
- Mortality, particularly in high-risk cases
Benefits
- Improved heart function in babies with HLHS
- Enhanced oxygenation of the blood
- Increased survival rates and quality of life
- Potential for progressive surgical treatments leading to a more normal heart function over time
Recovery
- Intensive care unit (ICU) stay immediately after surgery
- Monitoring of heart function and vital signs
- Pain management with medications
- Gradual resumption of feeding and normal activities
- Follow-up appointments for echocardiograms and other tests
- Recovery period varies but typically involves several weeks in the hospital
Alternatives
- Heart transplantation: Generally considered when surgical management is not viable. Pros: Potentially definitive solution. Cons: Long waiting times, need for lifelong immunosuppressants.
- Palliative care: Focuses on comfort rather than cure. Pros: Non-invasive, supportive care. Cons: Does not address underlying heart defect.
Patient Experience
During the procedure, the patient is under general anesthesia and will not feel any pain. Post-operatively, the baby may experience discomfort managed with pain relief measures. Parents can expect a significant hospital stay, intensive care monitoring, and gradual recovery. Family support and detailed care instructions aid in transitioning back home.