Leon M. Gerlis

Maternal Antibodies against Fetal Cardiac Antigens in Congenital Complete Heart Block

An immunologic basis for congenital heart block has been proposed previously. To investigate the association between congenital heart block and maternal antibodies capable of crossing the placenta, we used immunofluorescence to examine serum samples from 41 mothers and 8 affected children, together with serum from controls, for antibodies to fetal cardiac tissue. Twenty-one mothers (51 percent) had IgG antibody reactive with fetal heart tissue, as compared with only 9 of 94 controls (10 percent; P less than 0.001). Three of 8 affected babies, but none of 50 healthy babies, had similar antibodies. The antibodies reacted with all myocardial tissue and were not directed specifically to the conduction system. They also reacted with other fetal tissues and could be distinguished from nuclear and smooth-muscle autoantibodies. We also observed a higher occurrence of antibodies to cytomegalovirus, but not to Epstein-Barr virus, in these mothers. Autopsy specimens from babies with congenital heart block examined by immunoperoxidase staining showed deposition of immunoglobulin and complement components in all cardiac tissues. These findings strengthen the case implicating immune reactivity related to maternal antibody in the development of some but not all cases of congenital heart block.

Criss-Cross Atrioventricular Relationships Producing Paradoxical Atrioventricular Concordance or Discordance Their Significance to Nomenclature of Congenital Heart Disease

Two cases are described in which the systemic and pulmonary blood streams cross at atrioventricular level. One was examined pathologically, the other was diagnosed at cardiac catheterization by angiography. Both possessed situs solitus, but in one heart the right atrium was connected to a morphologic right ventricle on the left, whereas in the other it was connected to a left-sided morphologic left ventricle. In both cases transposition of the great arteries was present. The cases have been interpreted as representing rotation of embryonic d- and l-bulboventricular loops following septation.In attempting to describe these heart difficulties in terminology were encountered which were partially overcome by combining the concepts of concordant and discordant atrioventricular relationships with those of transposition and malposition. However, to give an accurate interpretation of the hearts, it proved necessary to include a term indicating rotation of the bulboventricular loop following septation. Thus the hearts were described as situs solitus, discordant (d-rotated) loop with d-transposition, and situs solitus, concordant (l-rotated) loop with l-transposition.

Persistent 5th aortic arch--a great pretender: three new covert cases.

Although persistence of the embryonic fifth aortic arch is considered a rare congenital malformation, analysis of the records of the cardiopathological collections of the Brompton and Killingbeck Hospitals reveals 6 examples in approximately 2000 specimens, an incidence amongst our material of 1 in 330. Since our review of the literature reveals only a further 13 described cases, we wonder if the condition may go unrecognized rather than being exceedingly rare. This possibility is further supported by the fact that 3 of our cases were incorrectly interpreted when initially described. In this report, we describe the details of 2 of these specimens and one other case that was not included in our earlier descriptions of this malformation. The first case had the persistent fifth arch as a conduit between the pulmonary and systemic circulations in the setting of aortic atresia with interruption of the aortic arch. The second case demonstrated a double lumen aortic arch, the fifth arch in this instance being an accessory systemic-to-systemic conduit. In the final case, the persistent arch was initially considered to represent an aorto-pulmonary window but review revealed an aortic to pulmonary conduit more in keeping with the presence of a fifth arch. We conclude that the powers of mimicry of this enigmatic structure may account for its apparent rarity.

Left ventricular bands. A normal anatomical feature.

Discrete delicate fibromuscular structures crossing the cavity of the left ventricle were identified on morphological examination in 329 (48%) of 686 hearts from patients of all ages with congenital heart disease, acquired heart disease, or normal hearts. These structures were also present in 151 (95%) of 159 hearts from animals of six species. Cross sectional echocardiographic findings compatible with these structures were obtained in 39 (21.7%) of 179 children reviewed retrospectively and in three of 800 (0.4%) adults studied prospectively. These structures appear to be a normal anatomical finding.

Anomalous subaortic position of the brachiocephalic (innominate) vein: a review of published reports and report of three new cases

Anomalous courses of the left innominate vein have rarely been described in anatomical specimens. Investigative techniques such as angiography and echocardiography have brought to light more instances of this anomaly. Three more cases identified by anatomical study are described. Earlier cases were reviewed to assess the type of associated cardiac malformations. Clinically, the abnormality is regarded as benign. When it is recognised during investigation it should alert the clinician to the possibility of associated malformations. Features commonly seen in tetralogy of Fallot--right aortic arch, ventricular septal defect, and right ventricular outflow obstruction--were common in patients with anomalous subaortic innominate veins.

Persistent fifth aortic arch. A report of two new cases and a review of the literature

Two new cases of persistent fifth aortic arch are described, one with a distal connexion to the pulmonary circulation and the other to the systemic circulation. The previously reported cases are reviewed and the advantages conferred clinically by persistence of this structure are noted.

Morphology and classification of complete atrioventricular defects.

Anatomical studies were made on 70 necropsied hearts with atrioventricular defects from patients with situs solitus and atrioventricular concordance, all having a common atrioventricular orifice. The arterial connections were concordant in 68 and were double outlet right ventricle in two; cases with arterial discordance (transposition) or single outlet of the heart were excluded. It proved possible to subdivide the hearts, depending on the morphology of the valve leaflets. Five leaflets were distinguished by the commissural pattern and their insertion to major papillary muscles. They were a posterior bridging leaflet, right and left lateral leaflets, and right and left anterior leaflets. Subdivision was made on the basis of the disposition of the anterior leaflets. In six hearts the left anterior leaflet was committed to the left ventricle and the right anterior leaflet to the right ventricle, the commissure between them being on the crest of the ventricular septum. In 39 hearts there was minimal bridging of the left anterior leaflet so that it extended between the anterior papillary muscle of the left ventricle and the medial papillary complex of the right ventricle. In eight hearts the right margin of the left anterior leaflet was attached to an apical papillary muscle, while in 17 hearts it was attached to the anterolateral papillary muscle of the right ventricle. As the bridging of the left anterior leaflet increased, so the size of the right anterior leaflet decreased, but in all hearts both leaflets were identified. These findings were compared with previous classifications of complete atrioventricular defects.

Calcification of Glutaraldehyde-Preserved Porcine and Bovine Xenograft Valves in Young Children

Eight children (mean age, 7.6 years) had xenograft prosthetic valves inserted. All developed evidence of prosthetic valve obstruction requiring reoperation 18 to 55 months after insertion (mean, 32 months). At operation, all the valves were found to be heavily calcified. There were two perioperative deaths. Of two patients who had a second xenograft valve inserted, one died suddenly 12 months later. Gross calcification of the xenograft was found at postmortem examination. The other patient had evidence of mild xenograft calcification at follow-up 19 months postoperatively. Four of the ten calcified xenograft valves were of bovine origin (Ionescu-Shiley type); in these the calcific obstruction occurred significantly earlier than in the six porcine (Hancock) valves.

Aortic valve damage caused by operative balloon dilatation of critical aortic valve stenosis.

Operative balloon dilatation of the aortic valve was performed in seven neonates with critical stenosis of the aortic valve. The procedure was followed by the development of severe aortic regurgitation in four patients. Necropsy was performed in three and revealed partial detachment of the right coronary cusp of the aortic valve. Damage to the valve leaflet caused by balloon dilatation was probably the result of using a balloon with a diameter that was too large in relation to the aortic valve ring diameter and of shearing forces created in the aortic wall by the contracting ventricle. The diameter of the inflated balloon should not be larger than the diameter of the aortic valve ring.

Cardiac malformations in spontaneous abortions

A detailed examination of 247 spontaneously aborted foetuses, under the age of 24 weeks gestation, showed 38 instances of cardiac malformation (15.4%). Very early specimens were not included. There was a bimodal relationship with maternal age and parity and a higher incidence in summer conceptions, but there was no apparent association with maternal blood groups. There were more male foetuses and they had proportionately more malformed hearts than the females. There was a marked inverse relationship with foetal age; in early foetuses, less than 25 mm, cardiac malformations were present in 68%. A wide variety of morphological abnormalities was encountered; these are listed. No cardiac malformations occurred in 72 threatened abortions which went to full-term delivery.