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(Circulation. 2001;103:325.)
© 2001 American Heart Association, Inc.

Images in Cardiovascular Medicine

Prenatal Diagnosis of Idiopathic Infantile Arterial Calcification

Jami C. Levine, MD; Juan Campbell, MD; Alan Nadel, MD

From Children’s Hospital (J.C.L., J.C.) and Brigham and Women’s Hospital and Harvard Medical School (A.N.), Boston, Mass.

Correspondence to Jami C. Levine, MD, Children’s Hospital, Department of Cardiology, 300 Longwood Ave, Boston, MA 02115.

A 33-year-old, healthy, G3 P1 woman had had a routine obstetrical ultrasound done at 19 weeks of gestation, which reportedly was normal. Because of decreased fetal activity, a repeat obstetrical ultrasound was performed at 32 weeks. That study showed polyhydramnios and a low biophysical profile, prompting transfer to a high-risk obstetrical center. The fetal echocardiogram, done on the same day, revealed a markedly abnormal heart. The heart looked structurally normal, but the aortic annulus, ascending aorta, transverse arch, descending aorta, main pulmonary artery, and coronary arteries were unusually echo-dense. Both ventricles were dilated, and biventricular function was severely depressed. There was a small pericardial effusion. There was almost no detectable antegrade flow across the pulmonary valve. Flow across the aortic valve was present but very low in velocity. Flow across the foramen ovale and the ductus arteriosus was bidirectional.

Figures 1 and 2 are from that fetal echocardiogram and illustrate dense calcification of the vessel walls. Note that the walls of the extracardiac vessels and coronary arteries have the same acoustic properties as that of the sternum. The diagnosis of probable idiopathic infantile arterial calcification was made. Steroids were administered in preparation for a premature delivery, but the fetus died 36 hours later, just before induction. Postmortem radiographs and fluoroscopy showed subtle evidence of aortic calcification. Figure 3 is from a histological specimen obtained at autopsy. This specimen illustrates the typical findings associated with infantile arterial calcification. There is calcium hydroxyapatite deposition in the internal elastic lamina as well as . . . [Full Text of this Article]

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