2023 ASE Fetal Echocardiography Guideline Update: Progressive Fetal Cardiac Lesions

Summary

This presentation discusses the management and evaluation of progressive lesions in fetal cardiology, specifically addressing congenital heart defects that evolve over time into more severe forms. It highlights the importance of serial evaluations to monitor cardiovascular compromise, assess the need for prenatal interventions, and guide postnatal care. Key examples include critical aortic valve stenosis, Epstein's anomaly, and tetralogy of Fallot with absent pulmonary valve. The use of Doppler imaging, ventricular function assessment, and targeted follow-up are emphasized for better outcomes and accurate predictions in fetal cardiac care. The importance of prenatal counseling and surgical intervention options is also discussed.

Takeaways

• 😀 Progressive lesions in the fetus may appear mild initially but can evolve into severe heart disease over time, requiring serial evaluation.
• 😀 Regular fetal heart assessments are crucial for detecting cardiovascular compromise and planning appropriate interventions.
• 😀 Some fetuses with progressive heart lesions may benefit from fetal cardiac interventions such as fetoscopic laser therapy, balloon valvuloplasty, and pharmacotherapy.
• 😀 Epstein’s anomaly, a significant progressive lesion, can lead to severe right atrial and ventricular enlargement early in gestation, predicting poor postnatal outcomes.
• 😀 Disease-specific targeted assessments, as outlined in guidelines, help monitor the progression of common fetal heart lesions and guide follow-up evaluations.
• 😀 Follow-up imaging for fetal heart defects, like critical aortic valve stenosis, should include specific Doppler measurements and assessments of left and right ventricular function.
• 😀 Interventional procedures such as balloon valvuloplasty can improve outcomes for fetuses with critical outflow tract obstructions, although they may not always result in improved heart function.
• 😀 Research shows that serial echocardiographic indexes are reliable predictors of disease progression and are essential for prenatal management planning.
• 😀 Fetal heart lesions like tetralogy of Fallot with absent pulmonary valve can cause severe morbidity and mortality, with significant variability in clinical presentation.
• 😀 Accurate prenatal counseling based on comprehensive fetal heart and lung assessments helps guide families in making informed decisions about management and delivery options.

Q & A

What are progressive lesions in the context of fetal heart disease?

Progressive lesions refer to congenital heart defects in fetuses that initially appear mild but evolve into more severe structural and functional heart disease as fetal development progresses.

Why is it important to monitor fetuses with progressive lesions serially?

Serial monitoring is essential because fetuses with progressive lesions are at high risk for cardiovascular compromise. Close observation helps identify worsening conditions early, enabling timely interventions and postnatal care planning.

What are some examples of fetal cardiac interventions for progressive lesions?

Fetal cardiac interventions include fetoscopic laser photocoagulation therapy for high-output lesions, balloon valvuloplasty for valve obstructions, septoplasty for outlet obstructions, and transplacental pharmacotherapy for incessant arrhythmias.

What is Epstein's anomaly, and why is it considered a progressive lesion?

Epstein's anomaly involves the dysplastic tricuspid valve and can lead to severe right atrial and right ventricular enlargement. It is considered a progressive lesion because it worsens over time, leading to significant heart failure if left untreated.

How can Doppler assessments aid in evaluating progressive fetal lesions?

Doppler assessments help measure blood flow velocities, gradients, and directions across heart valves and vessels, which can identify abnormal blood flow patterns. These measurements are crucial for monitoring the progression and severity of heart defects in fetuses.

What role does the cardio-thoracic area ratio play in evaluating progressive lesions?

The cardio-thoracic area ratio is a key indicator of heart size relative to chest size. A progressive increase in this ratio often correlates with worsening cardiomegaly, which can predict a poor postnatal outcome.

How does serial evaluation help guide the management of a fetus with critical aortic valve stenosis?

Serial evaluations of a fetus with critical aortic valve stenosis provide essential information about the progression of the lesion, including changes in ventricular function and aortic valve flow. This data helps determine whether fetal interventions like balloon valvuloplasty are appropriate.

What was the outcome of the fetal intervention (balloon valvuloplasty) in the case of critical aortic valve stenosis?

Despite the balloon valvuloplasty, the left ventricular function did not improve significantly. The fetus eventually required a single ventricle procedure (Norwood procedure) due to worsening ventricular function and hypoplasia of the left ventricle.

What is the significance of MRI in assessing fetuses with congenital heart defects?

MRI is used to assess not only the heart but also the diaphragm and airway, especially in cases where airway obstruction might be present. It provides valuable insight into the potential effects of congenital heart defects on fetal lungs and respiratory function.

What are the main predictors of poor outcomes in fetuses with tetralogy of Fallot and absent pulmonary valve?

The main predictors of poor outcomes in these fetuses are right ventricular dysfunction and mediastinal shift caused by airway obstruction. Fluid trapping in the lungs due to airway compression also contributes to the severity of the condition.