Fetal heart failure and hydrops fetalis may occur due to systemic arteriovenous fistula because of increased cardiac output. Arteriovenous fistula of the central nervous system, liver, bone or vascular tumors such as sacrococcygeal teratoma were previously reported to be causes of intrauterine heart failure. However, coronary arteriovenous fistula was not reported as a cause of fetal heart failure previously. It is a rare pathology comprising 0.2–0.4% of all congenital heart diseases even during postnatal life. Some may remain asymptomatic for many years and diagnosed by auscultation of a continuous murmur during a routine examination, while a larger fistulous coronary artery opening to a low pressure cardiac chamber may cause ischemia of the affected myocardial region due to steal phenomenon and may present with cardiomyopathy or congestive heart failure during childhood. We herein report a neonate with coronary arteriovenous fistula between the left main coronary artery and the right ventricular apex, who presented with hydrops fetalis during the third trimester of pregnancy.
Congestive heart failure and hydrops fetalis may occur due to cardiac or noncardiac causes during intrauterine life. About 26% of the fetuses with hydrops fetalis have cardiac pathologises and 41% of them are congenital heart diseases [
We herein present a case with CAVF leading to congestive heart failure and hydrops fetalis during intrauterine life.
A 22-year-old lady at the 26th week of pregnancy was referred to our clinic for fetal echocardiography; the indication was pericardial effusion detected during routine obstetric ultrasonography. She was previously healthy and this was her first pregnancy; her medical history was uneventful; however, her obstetric follow-up was not regular. Fetal echocardiography revealed pericardial, pleural, abdominal free fluid and skin edema. Intracardiac anatomy seemed to be normal despite poor echocardiographic images. However, cardiothoracic ratio was increased (heart was 1/2 times as large as the thoracic cavity), and significant holosystolic tricuspid regurgitation was observed; coronary sinus was found to be dilated (Figures
Four-chamber view of the fetal heart with increased cardiothoracic ratio.
Pulsed wave Doppler examination demonstrating significant tricuspid regurgitation.
Fetal echocardiographic appearance of dilated coronary sinus.
Postnatal echocardiographic examination demonstrating the large arteriovenous fistula connecting the left coronary artery with the apex of the right ventricle.
High-output congestive heart failure leading to hydrops fetalis may occur due to systemic arteriovenous fistula. Intrauterine heart failure and hydrops fetalis due to CAVF were not reported previously; however, systemic arteriovenous fistula involving other sites such as central nervous system, liver, bone or vascular tumors such as sacrococcygeal teratoma are known but rare causes of fetal heart failure [
On the other hand in many fetuses with nonimmune hydrops fetalis the real cause may not be identified; early echocardiographic examination or autopsy studies may be helpful in defining such abnormalities and the diagnosis may be difficult if they are not looked for. Common congenital heart diseases of postnatal life are usually tolerated during intrauterine life and do not cause intrauterine hydrops; however, intrauterine rhythm abnormalities, intrauterine infections, or congenital heart diseases associated with genetic abnormalities, structural malformations (Ebstein’s anomaly and premature closure of the foramen ovale), myocardiopathy, vascular obstruction (tumor, structural abnormalities, and fibroelastosis), vascular malformation, and hemangioma are other possible cardiac causes [
Fetal heart failure or hydrops fetalis usually does not cause any symptom in mother and is detected during routine obstetric ultrasonography, which was the case in our patient. Transplacental treatment of the fetal heart failure by giving inotropic agents to mother may be considered; however, once hydrops fetalis develops, the only option is the emergency delivery of the baby; we also proceeded in the same way.
Congenital coronary artery fistulas are defined as direct links between the coronary arteries and any of the four cardiac chambers. The dilated coronary arteries have no capillary system and rarely open into the coronary sinus, superior vena cava, pulmonary artery, or pulmonary vein [
Coronary AV fistula originates from the right coronary artery in 55–60% of the cases and 90% of them open into the right side of the heart (45% into the right ventricle, 25% into the right atrium, 15–20% into the pulmonary artery, and 7% into the coronary sinus). In Lowe and Sabiston’s series of 286 cases, right coronary artery was involved in 56%, left coronary artery was involved in 36%, and both coronary arteries were involved in 5% of the patients. The fistula opened into the right ventricle in 39%, right atrium in 33%, pulmonary artery in 20%, left atrium in 6%, and left ventricle in 2% of the cases [
Although the cardiac catheterization and coronary angiography are required for demonstration of the exact anatomy of the fistula and diagnosis of the additional cardiac anomalies, in recent years, technical developments in noninvasive diagnostic methods such as 2D and Doppler echocardiography provide sufficient information in CAVF. In our case, CAVF was diagnosed within the first hour of life by transthoracic echocardiography [
Treatment options are surgical or transcatheter closure of the fistula. Angiography was planned; however, the clinical condition of the patient was not suitable for any kind of intervention and he could stay alive for only 4 hours.
In conclusion, coronary arteriovenous fistula is a rare congenital heart disease which may cause intrauterine heart failure and must be considered in differential diagnosis of the fetuses with nonimmune hydrops fetalis. Presence of hydrops fetalis in routine obstetric ultrasonography is an indication for fetal echocardiography.
The authors declare that they have no conflict of interests regarding to the publication of this paper.