Prenatal Diagnosis of Right Coronary Artery to Right Atrium Fistula: A Case Report and Review of Literature

Introduction: Coronary artery fistulas (CAF) are rare anomalies which constitute approximately 0.2-0.4% of all congenital heart defects. As most of the patients are asymptomatic, there is often delay in diagnosis until later age. Reports of prenatal diagnosis have been limited. Catheter or surgical...

Full description

Saved in:
Bibliographic Details
Published in:Pediatric cardiology 2019-01, Vol.40 (1), p.244
Main Authors: Bogarapu, Soujanya, Albaro, Cecilia A, Jantzen, David, Plunkett, Mark, Hasselman, Ty.E, Shah, Jitendra J
Format: Article
Language:English
Subjects:
Catheterization
Congenital heart disease
Diagnostic imaging
Electrocardiogram
Electrocardiography
Genetic disorders
Heart
Infants (Newborn)
Obstetrics
Pregnant women
Vascular fistula
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:Introduction: Coronary artery fistulas (CAF) are rare anomalies which constitute approximately 0.2-0.4% of all congenital heart defects. As most of the patients are asymptomatic, there is often delay in diagnosis until later age. Reports of prenatal diagnosis have been limited. Catheter or surgical intervention may be recommended at diagnosis as serious complications may occur. Objective: We report a prenatally diagnosed case of CAF draining to the right atrium (RA) with serial follow-up echocardiograms during fetal life. Surgical repair was required on the third day of life. Case Description: The patient was referred at 23 weeks of gestation for fetal echocardiogram due to "tricuspid regurgitation" on initial screening obstetric ultrasound. Diagnosis of CAF to the RA was suspected and confirmed with follow-up imaging. Follow-up fetal echocardiograms at 26, 29, 32, and 36 weeks gestation demonstrated progressive enlargement of the right coronary artery (RCA), RA, and right ventricle (RV) without signs of overt heart failure. Baby was born uneventfully at 39 weeks gestation by induced vaginal delivery. Postnatal echocardiogram confirmed the presence of CAF. RCA was markedly dilated with a mildly restrictive fistulous connection to the RA. Initially RV and RA were both mildly dilated with normal RV function. However, by day three of life, progressive RV dysfunction, worsening tricuspid regurgitation, and acute ST segment changes on electrocardiogram prompted early surgical repair. Initial surgical repair consisted of closure of fistula from the RA side. Due to residual fistula flow, external ligation of the fistula proximal to entry into the right atrium was performed. Improvement in RA size and RV function was noted immediately and normalized over the next week. Postoperative course was uncomplicated, except for transient supraventricular tachycardia. Anticoagulation was achieved with enoxaparin initially and later was transitioned to aspirin as cardiac catheterization and angiography later showed no evidence of residual shunt across the fistula. Postoperative electrocardiograms have remained normal without T wave or ST segment abnormalities. Conclusion: CAF can be diagnosed prenatally and may progressively enlarge during fetal life. Coronary steal from myocardium may lead to ventricular dysfunction prenatally and in early neonatal period. Successful obliteration of fistula either surgically or by catheter technique can improve the outcome. Also as noted in
ISSN:0172-0643