Calcification of the fetal heart—four case reports and a literature review

Non-atherosclerotic coronary artery disease associated with sudden cardiac death

“…IACI is a genetically inherited autosomal recessive condition characterised by extensive arterial calcification and stenosis of large and medium-sized arteries 43. It is caused by homozygous or heterozygous mutations in the ectonucleotide pyrophosphatase/phosphodiesterase 1 ( ENPP1 ) gene 44 .…”

A Tale of Two Sisters: Idiopathic Arterial Calcification of Infancy in Siblings

“…The incidence is unknown, but approximately 180 cases have been reported to date and many cases are presumed to go undiagnosed or unreported. Most reported cases of IACI have been diagnosed at autopsy [5], Antenatal diagnosis of IACI by fetal echocardiography has also been reported [5,6]. Dystrophic calcification in IACI results in decreased vessel compliance, with resultant hypertension, ischemia, myocardial infarction and cardiac failure [7].…”

“…Dystrophic calcification in IACI results in decreased vessel compliance, with resultant hypertension, ischemia, myocardial infarction and cardiac failure [7]. Fetal heart failure can result in polyhydramnios, fetal hydrops, and demise during the early half of the last trimester of pregnancy [5,6]. Coronary artery involvement can be lethal within the first 6 months.…”

Prenatal Diagnosis of Complete Trisomy 9 With a Novel Sonographic Finding of Heart Calcification

“…10 Antenatal detection of massive calcification of the fetal heart is rare, as only 23 cases have been reported to date. [10][11][12][13][14][15][16][17][18][19] Of these 23 cases, 5 had evidence of infection: 1 case had toxoplasma 13 ; 1 case had cytomegalovirus 13 ; 1 case had a maternal history of varicella exposure 12 ; 1 case had herpes simplex virus type II infection 18 ; and the fifth was a liveborn child with findings suggestive of an intrauterine infection, although no infectious agent could be identified. 10 Two of the remaining 18 cases had a maternal history of cocaine use, and both were live births.…”

“…19 One case had calcification at the outflow tract, which led to suspicion of idiopathic calcification of infancy. 13 Two cases had a complete fetal heart block with maternal connective tissue disease, and immunologic mechanisms were thought to be the cause of myocardial damage and calcification in these fetuses. 17,19 In 4 cases, no cause for the abnormal calcification was found.…”