Patient Education • Heart Health

Fetal AVSD with Tetralogy of Fallot Physiology

A reassuring, evidence-based guide to help parents navigate diagnosis, fetal stability, genetics, and the pathway of surgical repair.

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Orientation

Navigating this Journey Together

Our goal is to **clarify your baby's diagnosis**, explain why they are stable right now, outline prenatal monitoring, and establish a clear **surgical roadmap** for a healthy future.

Anatomy

Complete Atrioventricular Septal Defect

In a typical heart, the left and right sides are separated by walls (septa), with two separate valves controling flow.

An AVSD (Atrioventricular Septal Defect) means there is a **large single opening (hole)** in the center of the heart and a **single common valve** instead of two separate ones.

Anatomy

Tetralogy of Fallot & Valve Narrowing

The second component is Tetralogy of Fallot (TOF) physiology.

This means the pathway leading from the heart to the lungs is **narrowed (pulmonary valve stenosis)**. Because of this restriction, blood has to squeeze through a smaller opening to reach the lungs.

Physiology

A Natural, Protective Balance

Normally, a large hole in the center of the heart (AVSD) causes too much blood to flood the baby's lungs.

However, the **narrow pulmonary valve acts as a shield**, restricting this extra flow. This surprising balance **protects the baby's lungs** from overcirculation and fluid build-up during pregnancy.

Hemodynamic Interaction Balanced intrauterine blood flow
Stability

Safe and Stable in the Womb

The placenta performs all "breathing" and oxygenation for your baby during pregnancy, meaning the heart does not have to work hard to feed the lungs yet.

Because the **common valve does not leak (no significant regurgitation)**, your baby is at **very low risk for heart failure** or fluid build-up (hydrops) before birth.

Cardiovascular Stability Non-ductal dependent status expected
Genetics

Understanding the Chromosomal Link

Congenital heart disease is present in approximately 50% of patients with Down syndrome.

Specifically, an **AVSD carries a very strong association with Trisomy 21 (Down syndrome)**. Identifying this early helps our team coordinate dedicated neonatal and developmental care.

Diagnostics

Diagnostic Genetic Testing Options

While blood screenings (like cell-free DNA) are useful, a diagnostic **amniocentesis** is the gold standard for structural heart findings.

Amniocentesis analyzes the actual amniotic fluid to provide a **definitive chromosomal karyotype and microarray**, giving our team exact details to plan your baby's care.

Surveillance

Your Prenatal Monitoring Timeline

Frequent tracking ensures that if the narrowing changes or the valve develops leaks, we can adjust birth plans immediately.

Delivery Planning

Delivering at a Specialty Care Center

Because this is a complex heart condition, delivery should be planned at a **specialty care hospital (Level IV maternal facility)**.

This guarantees that an **on-site pediatric cardiothoracic surgery team** and a **Level III/IV Neonatal Intensive Care Unit (NICU)** are ready to evaluate your baby the moment they are born.

Postnatal Pathway

Your Baby's Surgical Timeline

Early correction allows the heart to grow normally, leading to **excellent long-term survival and active childhoods**.

Postnatal Surgery Timeline Usually performed within first 6 months
Clinical Summary

Your Care Plan at a Glance

Patient Counseling & Perinatal Record OpenMFM Standard Template
Diagnosis Fetal AVSD with Tetralogy of Fallot physiology; suspected Trisomy 21
Genetic Plan Offered referral to MFM/Genetics; Diagnostic Amniocentesis with chromosomal microarray
Surveillance Serial fetal echocardiograms every 4–6 weeks to monitor stenosis and valve competence
Delivery Target Full term (>37 weeks) at Level IV Maternity Center with Pediatric Heart Surgery on-site
Postnatal Plan Postnatal confirmatory echo; surgical repair anticipated between 4 and 6 months of age
Emergency Contact Contact MFM triage immediately for signs of preterm labor or decreased fetal movement
Perinatal Documentation Template Copy coordinates directly into EMR chart
Shared Decisions

Questions to Ask Your MFM Specialist

Stenosis Status
"Is there any sign that the narrowing is getting worse or becoming duct-dependent?"
Valve Competence
"Does the common heart valve show any signs of leakage on today's echo?"
Birth Timing
"Do we expect a standard term delivery, or does the heart layout suggest early delivery?"
Care Team Setup
"When can we schedule a prenatal consult with the pediatric cardiologist?"
Patient Question Guide Write down questions before your appointment
Disclaimer

Educational Information Only

This presentation is provided for **general educational purposes only** and does not constitute medical advice, diagnosis, or treatment.

Your personal health findings, laboratory outcomes, and fetal echocardiogram measurements determine your individual care plan. **Always consult your own Maternal-Fetal Medicine specialist** to make decisions about your pregnancy.

OpenMFM Educational Disclaimer Seek advice from your personal clinical team
Privacy

Protecting Your Health Information

All case studies, documentation templates, and clinical examples published on OpenMFM are **fully anonymized**.

No protected health information (PHI) is collected or stored. Your clinical discussions, genetic tests, and care timelines are confidential and **strictly protected by HIPAA regulations**.

Patient Confidentiality Compliant with federal privacy regulations
Evidence

Professional Guidance & Resources

Evidence-Based Medicine Guidance links open in a new tab
Partnership

We Are with You Every Step

A complex diagnosis is a roadmap, not a destination. With prenatal monitoring and a dedicated heart team, we are ready to guide your family toward a bright, active future.

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