Rudolf J. Moene

Congenital mitral valve anomalies in transposition of the great arteries

In 165 hearts in transposition of the great arteries, including 16 with a ventricular septal defect and overriding pulmonary trunk and 5 with a so-called posterior transposition, the left ventricle was studied with emphasis on the morphology of the mitral valve. Distinct mitral valve anomalies were found in 36 cases (22 percent), and four categories of anomalies could be identified. Group A included 16 specimens with a cleft anterior mitral valve leaflet. The cleft was complete or partial. Partial clefts continued as a fibrous cord within the leaflet. In eight cases the cleft was situated posterior or lateral to the pulmonary ostium; in these cases the left ventricular outflow tract was not narrowed. Severe outflow tract stenosis was present in another eight cases in which the cleft was located anterior to the pulmonary ostium, usually in combination with a ventricular septal defect, and in four of these specimens there was straddling of the mitral valve. Group B included eight hearts with an abnormal size or position of the mitral valve, or both, the valve being hypoplastic or rotated clockwise, or both. Group C comprised seven hearts showing redundant left ventricular structures involving the mitral valve, among which were anomalous tissue strands, subpulmonary rings and redundant valve tissue. Group D included five specimens with deficient papillary muscles. It is concluded that an abnormal mitral valve is not unusual in hearts with transposition of the great arteries. The findings are notably important for those patients for whom anatomic surgical correction of the transposition is considered. In comparison with the venous baffle procedure, this operation makes greater demands on the structure of the mitral valve because the pressure in the left ventricle remains at systemic level. Thorough investigation of mitral valve anatomy and function is necessary before anatomic correction is considered.

Anatomic characteristics of ventricular septal defect associated with coarctation of the aorta

The morphologic characteristics of ventricular septal defect (VSD) and left ventricular outflow tract were studied in 45 hearts with VSD and coarctation of the aorta (C of A). Forty-one VSDs were classified into 1 of 4 categories. The first category includes 19 central muscular VSDs (43%), among which 3 hearts had the architecture of a spontaneously closed defect. The second category includes 10 perimembranous inlet defects (23%) with overlying tricuspid valve. The third category is formed by 8 perimembranous VSDs with leftward outlet septal malalignment (18%) and the fourth category includes 4 subarterial VSDs (8%) in which leftward malalignment of a deficient outlet septum results in subpulmonary localization of the defect. In addition to the main categories, 1 membranous, 1 perimembranous trabecular, 1 apical muscular and 1 doubly committed subarterial defects were identified. The left ventricular outflow tract was assessed as normal in only 2 cases. In 43 cases the left ventricular outflow tract was compromised by 1 or more of the following anomalies: anterolateral muscle bundle, anteroseptal twist, bicuspid aortic valve or a leftward malaligned outlet septum. These findings support the hypothesis of C of A being hemodynamically induced by anomalies of the left ventricular outflow tract. Previous studies have established that muscular, membranous and perimembranous inlet defects are prone to close spontaneously, in contrast to malalignment and subarterial VSDs. The present study shows that 70% of the VSDs belong to types characterized by a high incidence of spontaneous closure.

Morphology of ventricular septal defect in complete transposition of the great arteries

The morphologic characteristics of ventricular septal defect (VSD) was studied in 50 hearts with complete transposition of the great arteries. Except for 1 membranous and 1 subarterial defect, all VSDs could be classified into 1 of 4 categories. Group A included 14 specimens with a perimembranous defect extending into inlet and trabecular septum with rightward malalignment of the outlet septum; in this group aortic arch anomalies (6 hearts) and tricuspid valve abnormalities (7 hearts) were frequently present. Group B comprised 13 hearts with perimembranous defects extending into the inlet septum; 11 hearts in this group also had an anomalous mitral valve. Group C included 13 hearts with muscular inlet defects, with signs of spontaneous closure by marginal fibrosis in 10 cases. Group D included 8 hearts with muscular infundibular defects; in 6 of these hearts the outlet septum was malaligned to the left, causing different degrees of subpulmonary stenosis. A small second VSD was present in 5 hearts. Thus, the spectrum of VSD in transposition of the great arteries is different from that of isolated VSD.

Congenital right-sided pericardial defect with herniation of part of the lung into the pericardial cavity

Abstract This study describes an infant with congenital right-sided pericardial defect and herniation of part of the lung into the pericardial cavity. The patient presented with severe heart failure caused by a ventricular septal defect, a patent ductus arteriosus and mitral stenosis. During periods of deterioriation severe signs of superior vena caval obstruction were present, thought to be due to pressure of the herniated part of the lung on this vein. Chest roentgenograms showed a clearly demarcated radiolucent shadow in the superior mediastinum. The infant died at age 8 weeks because of intractable congestive heart failure. Necropsy revealed, in addition to the cardiovascular malformations, aplasia of the vermis cerebelli. The embryologie aspects of congenital pericardial defects are discussed.

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.

Pump Function of the Left Ventricle Evaluated from Pressure-Volume Loops

Evaluating the performance of the left ventricle and assessing contractility in a quantitative manner in the normal and failing heart constitute major problems in cardiology. The classical viewpoint has been to characterize the contractile behaviour of the heart in terms of quantities derived from cardiac muscle mechanics, i.e. force-velocity-length relations. Yet the application of these basic muscle concepts to the intact heart remains controversial (1).

Unclassified familial cardiomyopathy with ventricular dysrhythmia

SummaryThe case is described of a boy with some right ventricular dysplasia and episodes of ventricular tachycardia of left bundle branch block pattern who had symptoms from the age of 1 month. Angiography and cardiac biopsy demonstrated major involvement of the left ventricular myocardium. A sister of the patient presented at the age of 2 months with predominantly left ventricular cardiomyopathy; clinical signs and angiography suggested the presence of right ventricular dysplasia as well. She died suddenly at the age of 9 years. Her brother, now aged 14 years, is being treated with antiarrhythmic drugs. The hypothesis of this cardiomyopathy being a variant of “arrhythmogenic right ventricular dysplasia syndrome” is discussed.