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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 focal intimal proliferation and thickening of the vessel walls.

View larger version (167K): [in this window] [in a new window]   Figure 1. Off-axis view of ascending aorta (Ao) showing origin of coronary arteries (CA). Ascending aorta and walls of coronary arteries are unusually bright, suggesting severe calcification. Calcium deposition creates increased acoustic shadowing around coronary vessels.

View larger version (172K): [in this window] [in a new window]   Figure 2. Long-axis image of aorta and aortic arch. There is dense calcification from aortic valve to bifurcation of femoral arteries. Ao indicates ascending aorta; Dao, descending aorta; and RPA, right pulmonary artery in cross section.

View larger version (124K): [in this window] [in a new window]   Figure 3. Cross-section of left subclavian artery. Marked subintimal calcification and fibrosis with significant luminal narrowing. Hematoxylin-eosin staining at x25 magnification. Ca indicates calcium.

Despite extensive investigations of maternal factors, fetal calcium metabolism, and histopathology, the pathogenesis of this disease is still not well understood. The diagnosis is usually made postnatally, although there are rare reports of prenatal diagnosis by ultrasound. Most affected individuals die in the first 6 months, although there have been reports of spontaneous resolution of calcification as well as isolated reports of successful medical therapy.

Footnotes

The editor of Images in Cardiovascular Medicine is Hugh A. McAllister, Jr, MD, Chief, Department of Pathology, St Luke’s Episcopal Hospital and Texas Heart Institute, and Clinical Professor of Pathology, University of Texas Medical School and Baylor College of Medicine.

Circulation encourages readers to submit cardiovascular images to the Circulation Editorial Office, St Luke’s Episcopal Hospital/Texas Heart Institute, 6720 Bertner Ave, MC1-267, Houston, TX 77030.

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				A. M. Nagar, V. Hanchate, A. Tandon, H. Thakkar, and N. G. Chaubal Antenatal Detection of Idiopathic Arterial Calcification With Hydrops Fetalis J. Ultrasound Med., June 1, 2003; 22(6): 653 - 659. [Full Text] [PDF]

This Article Extract Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Request Permissions Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Levine, J. C. Articles by Nadel, A. Search for Related Content PubMed PubMed Citation Articles by Levine, J. C. Articles by Nadel, A. Related Collections Echocardiography