Fetal pulmonary atresia with ventricular septal defect: Features, associations, and outcome in fetuses with different pulmonary circulation supply types.

OBJECTIVES: To assess features and outcome in fetuses with pulmonary atresia with ventricular septal defect (PA-VSD). METHODS: Fetuses with PA-VSD were prospectively enrolled and grouped on the basis of the pulmonary blood supply, including type A (only arterial duct [DA]), type B (both DA and major aortopulmonary collateral arteries [MAPCAs] present), and type C (MAPCAs only). The echocardiography features, associated chromosomal/genetic malformations, and postnatal outcome were compared among the three groups. RESULTS: Fifty-five fetuses with PA-VSD were enrolled. The presence of confluent PAs varied, with the highest displaying rate in type A and lowest rate in type C (100% vs 41.1%). The intrapericardial pulmonary arteries in all groups were hypoplastic but smaller in types B and C than in type A (P < .05). Deletion of 22q11.2 and right aortic arch were more frequently observed in types B and C than in type A. At the end of the study, overall survival rates in type C were lower than those in type A (22.1% vs 77.3%). CONCLUSION: There are great differences in the size of pulmonary arteries, associated genetic malformations, and perinatal outcomes among fetuses with PA-VSD. These results could be used for family counseling and surgical planning.

Ventricular septal defect with normally connected and with transposed great arteries.

The morphologic characteristics of 50 hearts with ventricular septal defect (VSD) and ventriculoarterial (VA) discordance were compared with those of 105 hearts with VSD and VA concordance. Of the 7 VSD types in VA concordant hearts, the 3 that occur most frequently--central muscular (26%), perimembranous with leftward malalignment of the outlet septum (18%) and perimembranous with overriding posterior artery (15%)--were absent in VA discordant hearts. Of the 6 with VA discordance, 2 principal VSD types (perimembranous with rightward outlet septum malalignment [25%] and muscular infundibular with leftward outlet septum malalignment [15%]) did not occur in VA concordant specimens. Thus, 4 VSD types occurred with VA concordance as well as with VA discordance: subarterial (12% concordance, 3% discordance); perimembranous inlet (15%, 27%); muscular inlet (10%, 27%); and membranous (4%, 3%). Of these 4 common groups, perimembranous inlet VSD with VA concordance was accompanied by tricuspid valve anomalies; however, mitral valve anomalies complicated this type of VSD with VA discordance. It is concluded that in hearts with VA concordance vs hearts with VA discordance the spectrum of VSD and ventricular morphology is different.

Of clefts, commissures, and things.

A full understanding of the morphology of atrioventricular septal defects ("endocardial cushion defects," "atrioventricular canal malformations") demands knowledge of the exact nature of three specific breaches in the skirt of valve leaflet tissue guarding the atrioventricular junction. One of these is the space between the left ventricular components of the two leaflets that are enclosed in both the right and left ventricles (the bridging leaflets). Traditionally described as a cleft, it has been suggested more recently that this gap functions as a commissure. The second space is that which is found anteriorly and superiorly in the so-called Rastelli type A malformation. This is also called a cleft in a common anterior leaflet, but it has been suggested that it too is a commissure. The final breach is that produced by the surgeon when he divides the free-floating superior bridging leaflet if repairing the so-called Rastelli type C malformation with a one-patch technique. It is generally agreed that this breach be considered a division. To adjudicate the nature of the other breaches, it is necessary to compare them with commissures in atrioventricular and arterial valves and with the isolated cleft that exists in the aortic leaflet of a normal mitral valve. These considerations show that the gap between the left ventricular components of the bridging leaflets functions as a commissure even though it is not supported by a papillary muscle. This would not be expected, since the commissural attachments of the leaflets are in the left and right ventricle, respectively. The gap seen anteriorly in the Rastelli type A malformation is also a commissure, being supported in typical fashion by the medial papillary muscle of the right ventricle.

Anatomical-embryological correlates in atrioventricular septal defect.

Recent embryological studies have supported the consideration that the ventricular septum is multifocal in origin. These data have also provided excellent correlation of the morphology of malformed hearts with their embryology. In particular, atrioventricular septal defect correlates accurately with these observations on ventricular septation. Many of the names given to atrioventricular septal defect (for example ostium primum, persistent atrioventricular canal, endocardial cushion defect) indicate attempts at correlating the anatomy with embryology. None of these has been very convincing. In the light of this uncertainty, this review considers briefly the anatomy of the malformation and its ontogeny, and presents a hypothesis of the development of atrioventricular septal defect. Although there is almost always a communication above the atrioventricular valves, the malformation lies in the ventricular, not the atrial septum. Hearts with inlet septal defect without interatrial communication represent one end of the spectrum of anomalies, and those with common atrioventricular orifice, in which Fallot's tetralogy or single outlet heart may be associated, mark the other end. The outflow tract malformations are not randomly associated, but are points in a huge range of cardiac malformations.

[Surgical treatment of a single ventricle. Apropos of 4 operated cases]. / Traitement chirurgical du ventricule unique. A propos de 4 cas opérés

A surgical operation with a "curative" aim has been performed on four patients with a lone ventricle. It consisted of a IA type in 3 cases and IC type in one. There were one operative death and three long-term survivals. The operation consisted essentially in: a delicate step of partition of the lone cavity and the treatment of the often combined, and sometimes complex, heart malformation. The trauma to the conduction pathways is one of the major dangers of this type of operation and should be avoided or decreased by the detection during operation of the His bundle. Operation should be indicated in badly tolerated forms. Stenosis of the infundibulo-pulmonary pathway interferes with the development of a hypertensive pulmonary arteriolitis, seems to be a favourable element for surgical correction. In the absence of such stenosis, one should perform a banding early enough or discuss the indication for an intracardiac operation in relation with the pulmonary artery resistance.

[Atrio-ventricular canal. Apropos of 47 operated cases]. / Le canal atrio-ventriculaire. A propos de 47 cas opérés

The authors present their experience of this "bad reputation surgery". The operations performed ranged from pulmonary artery banding to complete reparation of A and B forms. They insist on the value of the assessment during operation of the quality of valvular repair.