CHDFNigeria
Acyanotic CHD comprises heart defects that typically do not result in cyanosis because there is no significant oxygen deprivation in systemic circulation. In these conditions, there is usually a left-to-right shunt, where oxygen-rich blood is recirculated to the lungs rather than the body.
In acyanotic CHD, oxygenated blood flows abnormally from the left side of the heart to the right side due to defects such as holes in the septum or malformations that obstruct blood flow. This left-to-right shunt increases blood flow to the lungs, causing pulmonary congestion but not necessarily affecting the oxygen saturation of the blood going to the rest of the body.
Common Types of Acyanotic CHD
1. Ventricular Septal Defect (VSD): A hole between the ventricles causes oxygen-rich blood to mix with oxygen-poor blood, leading to increased blood flow to the lungs.
2. Atrial Septal Defect (ASD): A hole between the atria results in a similar left-to-right shunt, often leading to pulmonary over-circulation.
3. Patent Ductus Arteriosus (PDA): The ductus arteriosus fails to close after birth, allowing oxygenated blood from the aorta to flow back into the pulmonary artery.
4. Coarctation of the Aorta: Narrowing of the aorta leads to increased workload on the left ventricle, potentially causing hypertrophy.
5. Aortic and Pulmonary Valve Stenosis: These are conditions where the aortic or pulmonary valves are narrowed, obstructing blood flow and increasing heart workload.
Clinical features and presentation
While acyanotic CHDs may not cause cyanosis, symptoms like shortness of breath, poor weight gain, difficulty in breathing, repeated chest infections, and fatigue are common. Presentation is based on the defect type and flow gradient and direction across the defect.
Diagnosis and Investigations
Acyanotic CHDs are diagnosed through a combination of clinical assessment and imaging techniques as with cyanotic:
1. Echocardiography: The primary tool for visualizing heart defects and assessing blood flow patterns.
2. Pulse Oximetry: Useful in identifying reduced oxygen saturation in cyanotic CHD. Most children have oxygen saturation level above 92%.
3. Cardiac MRI or CT: Provides detailed structural images for complex cases.
4. Chest X-ray: Used to assess heart size and pulmonary blood flow.
5. Electrocardiogram (ECG): Evaluates electrical activity, which may show signs of hypertrophy or other abnormalities.
Early diagnosis and monitoring are critical to managing CHD effectively, as timely interventions can prevent complications.
Complications
Untreated ACHDs: delay in treatment of acyanotic defects can cause pulmonary hypertension, heart failure, and arrhythmias due to the strain on the heart and lungs.
Post operative complication: In catheter device closure, procedure may fail or device even dislodged, requiring the traditional Open-Heart Surgery. Following cardiac surgery, complication can include: prolonged hospitalization, infection, arrythmias (e.g bradycardia), heart block, surgical wound infection or even death.
Treatment
Treatment is based on the defect and severity, ranging from deliberate delay (with close follow up of the child until when they are mature, better decision can be made or child is best fit for the correction) to immediate corrective intervention or surgery. For example, a small VSD may close on its own, whereas severe cases may require surgical closure or catheter-based intervention.
Please visit the Nigeria Open-Heart Registry https://www.nigeriaheartregistry.com/ for information about centres performing paediatric OHS and their rates. Unfortunately cost of surgery varies and will require you to contact hospital directly for this. Typically, price is usually around 10-15 million naira as of 2025.
What to Expect After Treatment
- Recovery: With early surgery, many children live into adulthood (61–85% survival at 20 years post-Fontan). Multiple surgeries are needed, and exercise capacity may be limited.
- Follow-Up: Lifelong care with a pediatric or adult congenital heart specialist is essential. Regular echocardiograms, ECGs, and check-ups monitor heart function.
- Complications: Risks include arrhythmias, stroke (if shunts remain), heart failure, or need for heart transplantation. Preventive measures for bacterial endocarditis are required lifelong.
- Support: Join support groups (e.g., American Heart Association, Mended Little Hearts) for emotional and practical support.
With treatment, many children with ACHD can thrive.
How to Help Your Child
- Act Quickly: Seek immediate medical help if you notice cyanosis, fast breathing, or poor feeding.
- Follow Medical Advice: Attend all appointments and follow medication or surgery plans.
- Keep Records: Document your child’s diagnosis, surgeries, medications, and doctor contacts for easy reference.
- Stay Informed: Ask your cardiologist about the Acyanotic CHD, treatment plans, and long-term care.
- Seek Support: Connect with other families through support groups or organizations like the National Organization for Rare Disorders (NORD) and Congenital Heart Disease Foundation of Nigeria (CHDFNigeria). Follow CHDFNigeria on all platforms for up-to-date information about your child’s condition.
