Fetal Cardiology Featured Articles of August 2015

1. Variation in Prenatal Diagnosis of Congenital Heart Disease in Infants.

Quartermain MD, Pasquali SK, Hill KD, Goldberg DJ, Huhta JC, Jacobs JP, Jacobs ML, Kim S, Ungerleider RM.

Pediatrics. 2015 Aug;136(2):e378-85. doi: 10.1542/peds.2014-3783.

PMID: 26216324

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Abarbanell picture smallComment from Dr. Ginnie Abarbanell (Atlanta), section editor of Fetal Cardiology Journal Watch: How well are we doing with prenatal diagnosis of congenital heart disease (CHD)?  This robust study using data from the Society of Thoracic Surgeons  (STS) Congenital Heart Surgery Database evaluated this question.  31,374 patients from 91 participating centers across the United States from the years 2006-2012 were evaluated in regards to prenatal diagnosis of CHD.  The overall prenatal diagnosis rate for these 6 years was 34% and increased every year from 26% (2006) to 42% (2012). See figure 1.   There was variability in the prenatal diagnosis rate in regards to heart defect and geographical region.  Hypoplastic left heart syndrome had the highest detection rate (67%) and total anomalous pulmonary venous connection has the lowest detection rate (9.1%).   Heart defects that can be easily identified from a fetal 4 chamber cardiac view had a higher rate of diagnosis (56.7% vs. 32.2%). See Figure 2.   31.9 % of the overall cohort had an additional noncardiac anatomic abnormality, syndrome, or chromosomal abnormality.  There was significant variation between states in the rates of prenatal defects of CHD ranging from 11.8% to 53.4%.  See Figure 4.

Take home points:

  1. The rate of prenatal diagnosis of CHD is less than 50% in most regions.
  2. Prenatal diagnosis of heart defects that are not evident on the 4-chamber view is low.
  3. Approximately 30% of fetuses had an additional non-cardiac abnormality, syndrome or chromosomal abnormality.
  4. The authors conclude, “development of training opportunities for physicians and sonographers performing fetal imaging may improve the state of prenatal CHD detection in the United States.”

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2. The Natural History of Atrioventricular Valve Regurgitation Throughout Fetal Life in Patients with Atrioventricular Canal Defects.

Davey BT, Rychik J.

Pediatr Cardiol. 2015 Aug 4. [Epub ahead of print]

PMID: 26238793

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Comment from Dr. Ginnie Abarbanell (Atlanta), section editor of Fetal Cardiology Journal Watch:  This study from Children’s Hospital Philadelphia demonstrates that the degree of atrioventricular (AV) valve regurgitation in the fetus with an AV canal defect does not change remarkably throughout gestation.   43 fetuses with AV canal defects were evaluated by fetal echocardiograms at mid-gestational and late-gestational ages.  In 60% of fetuses there was no change in the degree of AV valve regurgitation.  14% had a decrease and 26% had an increase in AV valve regurgitation.  However, in those with an increase in AV valve regurgitation the increase was not hemodynamically significant  (i.e. from no regurgitation to mild regurgitation).    In a previously published article on this same cohort of patients with AV canal defects, researchers also found that 90% had no hemodynamic significant change in AV valve regurgitation from fetal to postnatal life.

Take home points:

  1. There is minimal progression in the AV valve regurgitation in fetuses with AV canal defects from mid-gestational age to post-natal life.
  2. There may not be a need for frequent follow-up fetal echocardiograms to evaluate AV valve regurgitation.
  3. The mid-gestation fetal echocardiogram “is a good predictor of long-term AV valve regurgitation, and therefore provides a good foundation for accurate individualized counseling and estimation of prognosis for families of these children when the diagnosis is made in fetal life.”

 

3. Variation in Prenatal Diagnosis of Congenital Heart Disease in Infants.

Quartermain MD, Pasquali SK, Hill KD, Goldberg DJ, Huhta JC, Jacobs JP, Jacobs ML, Kim S, Ungerleider RM.

Pediatrics. 2015 Aug;136(2):e378-85. doi: 10.1542/peds.2014-3783.

PMID: 26216324

Shaji Menon Portrait 12.15.14Comment from Dr. Shaji Menon (Salt Lake City, UT), section editor of Fetal Cardiology Journal Watch:  Prenatal diagnosis of congenital heart disease (CHD) may improve perioperative outcomes. This study evaluates the variation in prenatal diagnosis of CHD in United States using Society of Thoracic Surgeons Congenital Heart Surgery Database (2006–2012).  The study included 31374 patients from 91 Society of Thoracic Surgeons Congenital Heart Surgery Database participating centers across the United States. Prenatal detection of CHD increased from 26% in 2006 to 42% in 2012. There was significant geographic variation in rates of prenatal diagnosis across states (range 11.8%–53.4%, P < .0001). The CHDs identified on 4-chamber view had higher detection rates compared to those requiring outflow tract visualization (57% vs 32%, P < .0001).

Take home point:  Despite improvements in healthcare, rates of prenatal CHD detection in the United States remain low, with significant variability between states and across defect type. CHDs that can be diagnosed on a 4-chamber view is more likely to be diagnosed prenatally compared to those with abnormalities in the outflow tract.

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4. Serum Biomarkers of Inflammation, Fibrosis, and Cardiac Function in Facilitating Diagnosis, Prognosis, and Treatment of Anti-SSA/Ro-Associated Cardiac Neonatal Lupus.

Saxena A, Izmirly PM, Han SW, Briassouli P, Rivera TL, Zhong H, Friedman DM, Clancy RM, Buyon JP.

J Am Coll Cardiol. 2015 Aug 25;66(8):930-9. doi: 10.1016/j.jacc.2015.06.1088.

PMID: 26293764

Comment from Dr. Shaji Menon (Salt Lake City, UT), section editor of Pediatric Cardiology Journal Watch:  Cardiac manifestations of neonatal lupus (cardiac NL) include congenital heart block and cardiomyopathy. This study evaluated several candidate biomarkers for identification of maternal and fetal biomarkers associated with development and morbidity of cardiac NL in cases at risk for cardiac NL : C-reactive protein (CRP); NT-pro-B-type natriuretic peptide (NT-proBNP); troponin I; matrix metalloproteinase (MMP)-2; urokinase plasminogen activator (uPA); urokinase plasminogen activator receptor (uPAR); plasminogen; and vitamin D. Cord CRP, NT-proBNP, MMP-2, uPA, uPAR, and plasminogen levels were higher in cardiac NL-affected fetuses than in unaffected cases. These biomarkers were positively associated with a disease severity score derived from known risk factors for mortality in cardiac NL. Cord and maternal vitamin D levels were not significantly associated with cardiac NL, but average maternal vitamin D level during pregnancy was positively associated with longer time to postnatal pacemaker placement.

Take home point: The findings of this study support following CRP and NT-proBNP levels after birth to potentially monitor severity and progression of inflation mediated cardiac NL. MMP-2 and the uPA/uPAR/plasminogen cascade may provide novel therapeutic targets to decrease fibrosis. Although decreased vitamin D did not confer increased risk, given the positive influence on postnatal outcomes, maternal vitamin D levels should be optimized to reduce morbidity associated with cardiac NL.

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