R. H. Anderson

Architecture of atrial musculature in humans.

OBJECTIVE--To investigate the gross arrangement of the principal muscular bundles of the two atria, and to suggest how it may contribute to contraction and spread of atrial excitation. DESIGN--A prospective analysis based on anatomical examination of adult human hearts. SETTING--A national heart and lung institute and a tertiary referral centre for cardiac disease. MATERIAL--9 normal postmortem human hearts. METHODS--Dissection of atrial muscles with macrophotography. RESUltS--The atrial walls consist of circumferential and longitudinal muscular bundles, the former being arranged at the base of the atria with the latter predominating in the parietal walls. The muscular bundles in the right atrium are larger than those in the left. The main muscles forming the right atrial wall are the terminal crest and terminal pectinate muscles. The terminal crest, the most obvious muscle, is arranged longitudinally with its pectinate muscles connecting to the musculature of the atrioventricular vestibule. No structure resembling the terminal crest is seen in the left atrium. Instead the left atrial wall is composed of intermingled series of muscles, chief of these being the interatrial band and the septoatrial bundle. The former is arranged circumferentially at the atrial base, while the latter is mainly longitudinal. The wall of the right atrium is not of uniform thickness because of the presence of the terminal crest and its pectinate muscles on its internal surface. By contrast, the left atrial wall is much more uniform and its average thickness is greater than that of the right atrium. The rim of the oval fossa is the most important muscular structure on the septal surface and is formed by the infolded atrial walls. The other principal muscles of the atria attach to it, so that the rim provides mechanical support for overall movement of the atrial walls. Comparison of the gross arrangement of the atrial musculature with earlier echocardiographic measurements showed that this arrangement of the muscle explains movement of the atrioventricular ring and overall atrial contraction, and provides a suitable substrate for preferential conduction. CONCLUSION--The anatomical features of the atrial musculature explain the known facts concerning atrial contraction and preferential conduction.

Ventricular myoarchitecture in tetralogy of Fallot.

BACKGROUND: Little attention has been paid to the architecture of the muscle fibres of the ventricular walls in congenitally malformed hearts. In this study the gross pattern of myocardial fibres in normal hearts was compared with that in cases of tetralogy of Fallot. METHODS AND RESULTS: After morphological examination nine specimens with tetralogy were dissected to study the ventricular myoarchitecture. Changes were found in the shape of the malformed ventricles. The ventricular walls were arranged in layers in all hearts. Superficial and deep layers were present in both ventricles, with the superficial layer showing a more oblique orientation in the specimens with tetralogy than in normal hearts. Modifications of muscle fibre that were related to the type of malformation were seen in the deep layer. A middle layer was present in the left ventricles of normal hearts and specimens with tetralogy: this showed a horizontal orientation in both groups. In contrast, a middle layer was found in the right ventricle only in specimens showing tetralogy. CONCLUSIONS: The malformed hearts showed modifications in ventricular shape, in the arrangement of muscle in the right ventricle, and in the overall myoarchitecture. These changes could well be the consequence of the same agent (or agents) that caused the structural defect.

Abnormal laterality and congenital cardiac anomalies. Relations of visceral and cardiac morphologies in the iv/iv mouse.

BackgroundIn the management of hearts with deranged laterality, it is essential that the left and right atrial chambers are correctly identified. There are two major approaches, which are based on venous connections or on the morphology of the atrial appendages, and there is no consensus as to which is the most useful. We used the ivliv mouse mutant, which is known to be pertinent to this problem, to evaluate the relations of cardiac defects with atrial, venous, and other visceral morphologies. Methods and ResultsThe morphology of the heart and other organs was examined in 275 ivliv mice using criteria based on abnormal laterality in humans. The arrangement of the atrial appendages was determined by morphological examination of the junction between the appendage and the venous component of the atrium. On this basis, 45.1% of cases were shown to have usual atrial arrangement, 50.2% had mirror imagery, 1.5% had right isomerism, and 3.3% had left isomerism. Every case of atrial isomerism had a cardiac lesion; the morphological types were similar to those seen in human cases. Of cases with either usual or mirror-image arrangement of the appendages, 33.2% had abnormal spleens, but only 3.1% had cardiac defects. Similarly, venous abnormalities were much more common (30.1%) than cardiac defects. ConclusionsStudy results endorse the importance of the morphology of atrial appendages in predicting cardiac abnormalities and point to the marked inconsistency of the arrangement of other organs, including the spleen and the connections of the systemic veins.

Left ventricular dysfunction in the fetus: relation to aortic valve anomalies and endocardial fibroelastosis.

OBJECTIVE--To examine the relation between a characteristic form of left ventricular dysfunction in the fetus and abnormalities of the aortic valve and endocardial fibroelastosis of the left ventricle. DESIGN--A retrospective study to examine the correlation between echocardiographic findings in the fetus and postnatal or necropsy findings. SETTING--Tertiary referral centre for fetal echocardiography. PATIENTS--Thirty fetuses showing a characteristic echocardiographic picture of left ventricular dysfunction. MAIN OUTCOME MEASURES--The relation between the prenatal echocardiographic features and the postnatal and necropsy findings. RESULTS--At presentation the size of the left ventricular cavity was normal or enlarged in all cases. The measurements of the orifice of the aortic root and mitral valve were either normal or small for the gestational age. The echocardiographic diagnosis made at presentation was critical aortic stenosis in all cases. At necropsy or postnatal examination the aortic valve was dysplastic and stenotic in 15 cases and the left ventricle had become hypoplastic in one of these. Aortic atresia was present in seven patients, three of whom had a hypoplastic left ventricle. In six patients the aortic valve was bicuspid although not obstructive. One of these patients had hypoplasia of the aortic arch and one had a hypoplastic left ventricle but in the remaining four patients endocardial fibroelastosis of the left ventricle was the only abnormality found. No follow up information was available in two. Of 26 patients for whom there was postmortem information, 24 had evidence of some degree of endocardial fibroelastosis of the left ventricle. Sequential observations showed that five cases developed into the hypoplastic left heart syndrome. CONCLUSIONS--This type of left ventricular dysfunction in the fetus is the result of an overlap of diseases, including primary left ventricular endocardial fibroelastosis, critical aortic stenosis, and the hypoplastic left heart syndrome.

The atrioventricular junctions in Ebstein malformation

OBJECTIVE To review the anatomical structure of the right atrioventricular junction, including the specialised atrioventricular conduction system, in hearts with Ebsteins malformation, to identify potential substrates for the abnormalities in conduction. METHODS Five heart specimens representing the morphological spectrum of Ebstein malformation were examined grossly and histologically. RESULTS On the endocardial surface, the atrioventricular junction was marked by a faint line in two hearts, and by a small ridge in the other three. Analysis of the right parietal junction in four hearts revealed only two accessory muscular atrioventricular connections. A plane of fibrofatty tissue separated atrial from ventricular myocardium in the right parietal junction in all hearts. The compact atrioventricular node was closer to the coronary sinus than usual. Accessory nodoventricular connections were present in four hearts, while accessory fasciculo-ventricular connections were found in one. The right bundle branch was hypoplastic or absent in four hearts. CONCLUSIONS In this small series, the parietal atrioventricular junction was better developed than previously thought. Structural abnormalities of the atrioventricular conduction system, however, were present. These may account for some of the conduction abnormalities frequently observed with the Ebstein malformation.

Morphological study of defects of the atrial septum within the oval fossa: implications for transcatheter closure of left-to-right shunt.

OBJECTIVE--To determine the anatomical variability of the oval fossa in cases of atrial septal defect and to find out which factors might make such defects suitable or unsuitable for closure by umbrella or clamshell devices. DESIGN--100 specimens with defects of the atrial septum within the oval fossa were studied, especially the position of the defects within the fossa; the area of the defect in relation to the total area of the oval fossa; the shape of the rims and flap valve of the oval fossa; and the anatomical variability in the eustachian and thebesian valves. RESULTS--The oval fossa was displaced to the mouth of the inferior caval vein in four cases; displaced to the mouth of superior caval vein in two cases; placed on the middle of the interatrial wall in 43; or placed slightly towards the inferior caval vein in 51. Because of their shape 29 of the hearts were considered to be unsuitable for transcatheter closure of the defect. This was because the defect was too large (16 hearts); the oval fossa was displaced to the mouth of the inferior caval vein (four hearts) or to the superior caval vein (two hearts); lacked its anterior rim (two hearts); lacked the posteroinferior rim (one heart); because a thick eustachian valve was displaced posteriorly forming a false posteroinferior rim (one heart); or because the strand of insertion of the floor of the oval fossa was too distant from the left atrial aspect (three hearts). CONCLUSION--68 hearts appeared to be ideal candidates for transcatheter closure; 3 would probably have been suitable; but 29 were unsuitable. These morphological variations might explain why the procedure has been unsuccessful in so many cases. Patients should be screened before any attempt is made at transcatheter closure.

Morphology of the sinus node in human and mouse hearts with isomerism of the atrial appendages.

BACKGROUND--The location of the sinus node is known to be at best abnormal, or at worst unknown, in patients with isomerism of the morphologically left atrial appendage. In contrast, the sinus node is known to be an excellent histological marker of the morphologically right appendage, being duplicated in those with right isomerism. The aim of the study was to investigate this condition further in fetal human and mouse hearts. METHODS--Serial histological sections of the area anticipated to contain the sinus node were studied in hearts with isomerism of the atrial appendages taken from 14 human fetuses and 13 iv/iv mice, using 12 mouse hearts with normally arranged or mirror imaged atrial chambers for controls. RESULTS--All hearts with isomerism of the right appendages (two human and four mouse) had bilateral sinus nodes. The cases with isomerism of the left appendages (12 human and nine mouse) showed absence of a recognisable sinus node except in four cases (19%) in which a small remnant of the node was found. In three of these cases, it was related postero-inferiorly to the superior cavoatrial junction. CONCLUSIONS--The concept of isomerism of the atrial appendages is endorsed by findings on the morphology of the sinus node, this being the most reliable histological criterion for existence of a morphologically right atrium. A small proportion of hearts with left isomerism had a structure resembling the sinus node, but it was hypoplastic and displaced postero-inferiorly, distant from its expected position had the hearts possessed an incompletely formed morphologically right appendage.

Atrioventricular septal defect in prenatal life.

In a series of more than 3500 pregnancies referred for fetal echocardiography, 29 cases of atrioventricular septal defect were detected in the fetus. There was a chromosomal anomaly in 14 of these cases, left atrial isomerism in 12, and right atrial isomerism in two. Complete heart block was found in 11 of the cases with left atrial isomerism. Many associated cardiac abnormalities were found, particularly in the fetuses with atrial isomerism; the most common were double outlet right ventricle or aortic arch anomalies. The prognosis was poor in all patients with atrioventricular septal defect detected prenatally. Fifteen pregnancies went to term but there are only four survivors. Two of those have trisomy 21, a further patient has inoperable defects, and only one remains well and is awaiting corrective surgery for a partial atrioventricular septal defect.

Bright-blood T(2)-weighted MRI has high diagnostic accuracy for myocardial hemorrhage in myocardial infarction: a preclinical validation study in swine.

Background— Myocardial hemorrhage after myocardial infarction (MI) usually goes undetected. We investigated the diagnostic accuracy of bright-blood T2-weighted cardiac MRI for myocardial hemorrhage in experimental MI. Methods and Results— MI was created in swine by occluding the left anterior descending (n=10) or circumflex (n=5) coronary arteries for 90 minutes followed by reperfusion for ⩽3 days (n=2), 10 days (n=7), or 60 days (n=6). MRI was performed at 1.5 T, using bright-blood T2-prepared steady-state free-precession, T2* and early (1 minute) and late (10–15 minutes) gadolinium enhancement (EGE, LGE, respectively) MRI. Left ventricular sections and histology were assessed for hemorrhage by an experienced cardiac pathologist blinded to the MRI data. Hypointense regions on T2-weighted and contrast-enhanced MRI were independently determined by 3 cardiologists experienced in MRI who were also blinded to the pathology results. Eighty ventricular pathological sections were matched with MRI (n=68 for EGE MRI). All sections with evidence of MI (n=63, 79%) also exhibited hyperintense zones consistent with edema on T2-weighted MRI and infarct on LGE MRI. Myocardial hemorrhage occurred in 49 left ventricular sections (61%) and corresponded with signal voids on 48 T2-weighted (98%) and 26 LGE-MRI (53%). Alternatively, signal voids occurred in the absence of hemorrhage in 3 T2-weighted (90% specificity) and 5 LGE MRI (84% specificity). On EGE MRI, 27 of 43 cases of early microvascular obstruction corresponded with hemorrhage (63% sensitivity), whereas 5 of 25 defects occurred in the absence of hemorrhage (80% specificity). The positive and negative predictive values for pathological evidence of hemorrhage were 94% and 96% for T2-weighted, 84% and 55% for LGE MRI, and 85% and 56% for EGE MRI. Conclusions— Bright-blood T2-weighted MRI has high diagnostic accuracy for myocardial hemorrhage.

Echocardiographic-anatomical correlations in aorto-left ventricular tunnel

OBJECTIVE--To investigate the echocardiographic, morphological, and histological appearances of aorto-left ventricular tunnel observed in four fetal hearts and compare the findings with those reported in older patients with the malformation. BACKGROUND--Previous studies have concentrated on clinical features of the malformation from birth to adult life and have speculated on either its embryological formation or its acquisition during late intrauterine life. The presentation of a large series of cases in fetal life is a unique opportunity to study the malformation at an early stage in its natural course. METHODS--A retrospective study was performed of four cases of aorto-left ventricular tunnel discovered among 872 cases of congenital abnormalities diagnosed at a tertiary centre for fetal echocardiography. Detailed echocardiographic and anatomical observations were made of the malformation as identified during fetal life. The precise anatomical arrangement was determined and compared with previous descriptions found in journals published in English. RESULTS--In fetal life, as after birth, the malformation is characterised by enlargement and hypertrophy of the left ventricle, enlargement of the aortic root, and free regurgitation at the level of the aortic valve. Anatomical abnormalities are found at the aortic ventriculoarterial and sinutubular junctions as well as in the intervening aortic wall. These are unrelated to necrosis, ischaemia, or the presence of mucopolysaccharides. CONCLUSIONS--The lesion is a developmental abnormality that should be reliably diagnosed by fetal echocardiography combined with colour flow Doppler echocardiography during the mid-trimester. The exact anatomical relations clarified by this study are pertinent to diagnosis and subsequent surgical correction.