Congenital heart block and widespread fibrosis due to maternal lupus erythematosus.

Abstract

We here report the clinical and pathological findings of a newborn infant with complete heart block who died 25 minutes after birth. Extensive fibrosis was found in many organs, including the heart, liver, and kidneys. The mother suffered from systemic lupus erythematosus and it seems probable that the maternal disease was responsible for the infant's condition. Case ReportMaternal history. In 1949 the mother (age 22) developed the characteristic cutaneous rash of lupus erythematosus. The clinical diagnosis was supported by the histological appearance of a skin biopsy. The erythrocyte sedimentation rate ranged between 68-100 mm./hr., and the white cell count between 3000-4000/ c.mm. Since that time her general health has been good, the skin lesions have varied in severity, and the leucopenia and high sedimentation rate have persisted. LE cells were not found in the peripheral blood, and the Wassermann reaction was negative. Her blood group was 0 Rh positive.Previous pregnancies. In 1947 her first child was born at 34 weeks' gestation after surgical induction (male, birthweight 2-3 kg.). In 1953 her second child was born at term (female, 2 * 8 kg.). The delivery and subsequent development of both infants were normal. In 1957 her third child was born by spontaneous vertex delivery at term (female, 2-8 kg.). At birth the baby had a rash on the face and scalp, which was characteristic of chronic discoid lupus erythematosus. The rash cleared spontaneously over the next few months. Present pregnancy. In December 1962 the mother (age 37) became pregnant for the fourth time. During the first three months of the pregnancy she took chloroquine 250 mg. once or twice each day because of an exacerbation of the skin lesions. At 36 weeks' gestation it was noted that the foetal heart rate was slow but regular. The bradycardia persisted and a clinical diagnosis was made of intrauterine heart block. This was confirmed by foetal ECG which showed the rate to be 45 beats/minute. Received March 8, 1966. Delivery. Labour commenced at 38 weeks' gestation after spontaneous rupture of the membranes. There was moderate hydramnios and the liquor was stained with meconium. The labour lasted 3j hours. When the cervix was fully dilated, the head was rotated by hand from the occipito-posterior position and delivered after an easy pull with Wrigley's forceps.Infant. The infant (male, 2 * 5 kg.) had gross oedema of the head and trunk. In contrast the abdomen and limbs were not swollen. There were numerous purple patches on the face, scalp, and upper chest, which were similar to blotch haemorrhages commonly seen in newborn infants with severe anaemia, and the liver and spleen were both enlarged (3-4 cm. below the costal margin). The scrotum was darkly pigmented.At birth, the infant had normal muscle tone and reflex irritability and, though he did not gasp or cry, he immediately began regular respiratory movements. The ECG showed complete heart block, atrial rate 168 beats/ min., and ventricular rate 45 beats/min. (Fig.). His chest, which initi...

Maternal Anti-Ro and Anti-La Antibody–Associated Endocardial Fibroelastosis

“…18,19 The presence of EFE was assumed to be attributable to prolonged bradycardia and cardiac failure. However, the results from our study suggest an alternative hypothesis.…”

Congenital complete heart block and maternal connective tissue disease

“…CCHB associated with structural heart disease has got a far worse prognosis compared to isolated CCHB both pre and postnatally. The association between isolated CHB and maternal connective tissue disease was described by Hull et al in the late 1960s [5]. This association was shown to be due to the circulating anti SSA/Ro and anti SSB/La antibodies in most of the mothers of these affected infants.…”

Congenital complete heart block in the newborn associated with maternal systemic lupus erythematosus and other connective tissue disorders.

“…We can find only one report in the UK (Hull et al, 1966), but we believe it is more common here than generally appreciated and we wish to report a further 5 cases, with survival in 4, to show that this is part of a spectrum of abnormalities which may be associated with maternal connective tissue disease.…”