In Utero Management of Hydrops Fetalis Caused by Critical Aortic Stenosis

Abstract

Hydrops fetalis is rarely associated with congestive heart failure caused by obstructive left-sided heart lesions. There are rare cases of live born neonates with critical congenital valvar aortic stenosis and hydrops reported in the literature, all with fatal outcomes. This report describes, to the best of our knowledge, the first two newborns who were diagnosed prenatally to have hydrops fetalis caused by critical valvar aortic stenosis, who were treated prenatally with digoxin and who postnatally had successful percutaneous balloon aortic valvuloplasty. Both patients had not only left but right ventricular dysfunction. We speculate that right ventricular dysfunction was a contributing factor in the development of hydrops in these patients and in utero medical therapy with digoxin is associated with resolution of the hydrops before delivery.Hydrops fetalis is rarely associated with congestive heart failure caused by obstructive left-sided heart lesions. 1 " 4 There are rare cases of live born neonates with critical congenital valvar aortic stenosis and hydrops reported in the literature, 1 " 4 all with fatal outcomes. We describe, to the best of our knowledge, the first two newborns who were diagnosed prenatally to have hydrops fetalis caused by critical valvar aortic stenosis, who were treated prenatally with digoxin, and who postnatally had successful percutaneous balloon aortic valvuloplasty in the first hours of life. CASE REPORT 1A 3.2-kg male infant product of 35 weeks of gestation was diagnosed by fetal ultrasound to have hydrops fetalis with ascites, chest wall edema, and significant polyhydramnios. A fetal echocardiogram at 33 weeks of gestation revealed critical valvar aortic stenosis, severe mitral regurgitation with marked left atrial and left ventricular dilation, and biventricular systolic dysfunction. The fetal cardiac rhythm was regular. The mother was given digoxin intravenously to transplacentally digitalize the fetus and therapeutic digoxin levels were achieved and maintained. Repeat echocardiogram revealed some resolution of the ascites and improvement of biventricular systolic dysfunction. The Doppler estimate of the pretreatment aortic stenosis gradient was 20 mmHg and this increased to 36 mmHg after digoxin therapy. The baby was delivered vaginally 2 weeks later and required immediate intubation, intravenous dopamine, and abdominal paracentesis to relieve respiratory compromise from ascites. Evaluation revealed a grossly edematous infant with a regular heart rate at 160 beats per min and blood pressure of 60/43 mmHg. The first heart sound was normal and the second heart sound was single. There was a grade 2/6 systolic ejection murmur at the right upper sternal border and a grade 2/6 blowing pansystolic murmur at the apex. No diastolic murmurs were appreciated. An electrocardiogram revealed

Perinatal Management of Fetal Cardiac Anomalies in a Specialized Obstetric-Pediatrics Center

“…Most recent studies in this setting focus on the feasibility of prenatal diagnosis, [1][2][3][4][5] its cost, and effectiveness in a nonselected population, [6][7][8] whereas few deal with the particularities of the collaborative obstetric-pediatric management 9,10 and the outcome of the pregnancies with a high risk of perinatal hemodynamic compromise. 11 Our institution includes a fetal medicine unit, a neonatal intensive care unit, and a perinatal cardiology unit. This report presents the management of 229 pregnancies, in which a serious fetal cardiac disorder was detected by ultrasound during pregnancy.…”

Aortic Stenosis and Severe Mitral Regurgitation in the Fetus Resulting in Giant Left Atrium and Hydrops

“…These cardiac abnormalities were associated with hydrops in 11 fetuses. Fetuses with these findings have been described previously but only in isolated reports (22)(23)(24)(25). We contend that this is a unique complex of defects that should be thought of as a distinct clinical entity, which may be on the severe end of the mitral arcade spectrum (18).…”

Fetal aortic valvuloplasty may rescue fetuses with critical aortic stenosis and hydrops

“…Another reason could be the use of transplacental digoxin in all our patients undergoing FAV. Potential benefits of using transplacental digitalization have been reported previously in two studies 6,8 . Both reports describe the successful treatment of, altogether, three fetuses with CAS and hydrops without any other intrauterine treatment.…”