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Comparison of Pre- and Postnatally Diagnosed Coronary Artery Fistulae: Echocardiographic Features and Clinical
Andrea Pisesky1, Lynne E Nield1, Joanna Rosenthal2
1Labatt Family Heart Center, Department of Pediatrics, The Hospital for Sick Children, Toronto, Ontario, Canada.
Insights
Coronary artery fistulae (CAFs) detected prenatally often resolve before birth, but some require intervention due to potential cardiogenic shock. Postnatal surveillance is crucial for managing these congenital heart conditions.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Congenital Heart Disease
Background:
- Coronary artery fistulae (CAFs) are uncommon abnormal connections between coronary arteries and heart chambers/vessels.
- Limited data exists on clinical outcomes for CAFs, particularly those diagnosed prenatally.
Approach:
- A multicenter retrospective cohort study analyzed CAF cases from 2002-2016.
- Compared clinical characteristics and outcomes between prenatal and postnatal CAF diagnoses.
- Conducted a scoping literature review of prenatal CAFs.
Key Points:
- Prenatal CAFs were often large and associated with structural defects/genetic conditions.
- Half of prenatal CAFs resolved spontaneously before birth; one required neonatal intervention.
- Postnatal CAFs rarely presented with severe symptoms, with most interventions for large shunts.
Conclusions:
- Most CAFs, whether prenatal or postnatal, have a favorable prognosis and often do not require intervention.
- Prenatal diagnosis necessitates careful perinatal planning and postnatal surveillance due to risks like cardiogenic shock.
- While many prenatal CAFs resolve, ongoing monitoring is essential for infants with these conditions.
Background:
Coronary artery fistulae (CAFs) are abnormal connections of a coronary artery to a cardiac chamber or vessel. There is a paucity of data regarding clinical outcomes, especially when detected prenatally.
Methods:
This was a multicenter retrospective cohort study of all CAF cases from 2002 to 2016. Clinical characteristics and outcomes were compared between the prenatal and postnatal cohorts. A scoping literature review of prenatal CAFs was completed.
Results:
CAFs were diagnosed prenatally in 12 (median, 23 weeks' gestation; interquartile range, 17-36 weeks' gestation) and postnatally in 94 (median, 2.8 years; interquartile range, 0-15 years) cases. Structural heart defects were present in five (42%) prenatal and 19 (20%) postnatal cases (P = .011) and genetic conditions in five (42%) and 14 (15%), respectively (P = .001). CAFs were considered large in 12 (100%) prenatal versus 14 (15%) postnatal cases (P < .001). The CAF distribution was similar between cohorts: 39 (67%) from the left and 19 (33%) from the right coronary artery, with the most common exit sites being the main pulmonary artery 54 (51%), right ventricle 30 (28%), and right atrium 12 (11%). Of prenatal cases, all large at presentation, none progressed, six (50%) resolved by birth, and one (8%) underwent elective neonatal ligation. Of postnatal cases, one presented in cardiogenic shock, and no other case had ventricular dysfunction, arrhythmias, or ischemic changes. Nine (10%) with large shunts underwent intervention (seven percutaneous, two surgical), of whom three were symptomatic. Two (17%) prenatal and two (2%) postnatal cases had coronary abnormalities, two with normal results on stress perfusion imaging. Postnatal death occurred in two (17%) prenatal and four (4%) postnatal cases (P = .05). Of the total 36 prenatal cases reported in the literature, including the 12 cases in the present series, 10 (28%) had clinical symptoms at birth, including three (8%) with cardiogenic shock, and 19 (53%) underwent intervention.
Conclusions:
Prenatally and postnatally encountered CAFs are associated with a good prognosis for most, with many not requiring intervention. Although half of the prenatal CAFs resolved prenatally, given the risk for cardiogenic shock at birth and heart failure in early infancy, appropriate perinatal planning and postnatal surveillance is warranted.
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