FREEDOM - Fetal CDH Study training modules

Fetal Risk Evaluation using Echocardiography, Ultrasound and MRI in congenital Diaphragmatic hernia for Outcomes Modeling (FREEDOM)

Fetal Echocardiography in CD

Apical 4 chamber view.

Long axis view with the LVOT

4 chamber with RV on top and LV on bottom

Short axis view

Fetal deformation analysis by Speckle Tracking Echocardiography

Deformation for the fetal LV - longitudinal (strain, strain rate, displacement, velocity) in an infant with CDH

Deformation for the fetal LV in short axis view (strain, strain rate, displacement, velocity) in an infant with CDH

Deformation for the fetal RV (strain, strain rate, displacement, velocity) in an infant with CDH

Fetal MRI

Short axis view at the level of the 4 chamber view in a fetus with CDH by fetal MRI.

Coronal axis view in a fetus with CDH by fetal MRI.

Volumetric analysis gave the following results (all in mL):

Right lung (4.95 mL observed), Left lung (1.39 observed), Total lung (6.34). Expected (Right: 9.39; Left: 8.08; Total: 17.70). O/E ratio of 35.8%

Another case of "severe" CDH by fetal MRI

Another case of "severe" CDH by fetal MRI showing the spleen herniating in the chest.

Fetal ultrasound

Data collection

Prenatal / Maternal characteristics

- Maternal age

- Maternal ethnicity

- Maternal co-morbidities (hypertension, diabetes, obesity, hypothyroidism), gestational age at diagnosis, fetal growth restriction.

- Exposure to Fetoscopic Endoscopic Tracheal Occlusion (FETO) procedure – gestational age at balloon placement and retrieval. Complication of FETO (premature rupture of membranes, preterm birth).

- Exposure to prenatal steroids – gestational age.

Fetal Echocardiography

- Gestational age at fetal echocardiography

- Indices of fetal LV dimensions: mitral valve, aortic valve, LV end-diastolic diameter at tip of mitral valve, LV length, estimated LV volume by Simpson’s disc method, LV end diastolic volume by STE analysis, LA area in apical view. LV diameter relative to the RV diameter in the short axis view. LV area relative to the RV area in the apical view.

- Indices of fetal LV performance: estimated ejection fraction by Simpson’s disc, LV shortening fraction by 2D (B-mode) measurement, LV deformation assessment by STE for both regional and global myocardial strain and strain rate in longitudinal and circumferential axis. STE allows for early detection of cardiac dysfunction in high-risk populations, and this technology applied to the fetal patients with CDH will heighten our understanding of their underlying cardiac mechanics. STE allows for the estimation of: a) ventricular ejection fraction b) segmental myocardial contribution to overall cardiac function and d) assessment of cardiac chamber dimensions and volumes.

- Indices of pulmonary vascular hypoplasia: McGoon index, largest main/right/left pulmonary arterial diameters. Evaluation of pulmonary artery Doppler if available. LV end systolic eccentricity index in the short axis fetal view.

- Indices of fetal RV performance and dimensions: RV diameter at the base, RV length, RV mid-cavity diameter, RA area and RV end diastolic area in apical view, RV fractional area change, RV deformation by speckle tracking echocardiography.

Fetal ultrasound

- Gestational age at fetal ultrasound

- Estimated fetal weight and percentile

- Resistive and pulsatility index of the umbilical artery.

- LHR and o/eLHR

- Presence of liver herniation, gastric herniation or other abdominal viscera herniation.

Fetal MRI

- Fetal total lung volume (o/eFTLV)

- Modified McGoon index

- Presence of abdominal viscera hernation and %Liver herniation (if present).

Postnatal parameters

- Demographics: sex, ethnicity, gestational age at birth, birth weight, mode of delivery, APGAR score, initial cord pH, mortality, date of surgery, type of defect (as per CDH surgical defect classification), duration of hospitalization, duration of respiratory support, oxygen / respiratory support at 30 and 56 days of life.

We will also evaluate the outcome of chronic lung disease based on two previously described definitions reported in the CDH population: respiratory support or oxygen use at 30 days 1 or ongoing respiratory support at 56 days or at earlier discharge 2. The later definition is based on the classification or moderate-to-severe chronic lung disease in infants born ≥32 weeks 2. Pulmonary vasodilator therapy includes: iNO, sildenafil or tadalafil, endothelin-receptor antagonist, prostacyclin analogs, prostaglandins infusion or milrinone. Postnatal inotropic/vasoactive medications include: epinephrine, norepinerphine, dobutamine, dopamine, vasopressin or levosimendan.

      1. Van Meurs K. Is surfactant therapy beneficial in the treatment of the term newborn infant with congenital diaphragmatic hernia? J Pediatr. 2004;145:312-316.

      2. Guslits E, Steurer MA, Nawaytou H, Keller RL. Longitudinal B-Type Natriuretic Peptide Levels Predict Outcome in Infants with Congenital Diaphragmatic Hernia. J Pediatr. 2021;229:191-198.e192.

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