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Ann Thorac Surg 1999;67:1403-1411
© 1999 The Society of Thoracic Surgeons

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Original Articles

Ventricular morphology and coronary arterial anatomy in hearts with isomeric atrial appendages

^a National Heart and Lung Institute, London, England, UK

Accepted for publication November 9, 1998.

Address reprint requests to Dr Uemura, Department of Cardiovascular Surgery, National Cardiovascular Center, 5-7-1 Fujishirodai, Suita, Osaka 565-8565, Japan
e-mail: huemura{at}hsp.ncvc.go.jp

	Abstract

Top Abstract Introduction Material and methods Results Comment Acknowledgments References

 
Background. Knowledge of the precise anatomy can be advantageous when striving to improve surgical results in patients with visceral heterotaxy.

Methods. We studied the ventricular mass, and its coronary arterial supply, in 125 specimens with isomeric right and 58 with isomeric left appendages.

Results. The situation in which each atrium connected to its own ventricle was the most common arrangement in hearts with isomeric left appendages. The pattern with both atriums connecting to the same ventricle was more frequently seen in those with isomeric right appendages. Concordant ventriculoarterial connections were seen in only 4% of cases with isomeric right appendages, but were seen in 45% of those with isomeric left appendages. Abnormal patterns in branching of the coronary arteries were commonly associated with abnormal ventricular architecture. The morphologically right or left ventricular arteries were frequently lacking in those hearts with a dominant ventricle and a rudimentary and incomplete ventricle. A solitary coronary artery was seen in 13%.

Conclusions. Recognition of these abnormalities is of clinical importance if optimal surgical strategies are to be established for patients with visceral heterotaxy.

	Introduction

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When striving to improve surgical outcomes in patients with isomerism and visceral heterotaxy, it is vital to choose the optimal operative strategy. Definitive repairs [1–4] are only likely to be achieved with acceptable rates of mortality if the anatomic structure of the heart is favorable. Although much attention has been paid to the well-recognized abnormalities in systemic and pulmonary venoatrial connections known to accompany isomerism [4–6], there are few detailed morphologic descriptions concerning the spectrum of ventricular morphology [4, 7]. In the present study, therefore, we have examined the makeup of the ventricular mass, concentrating particularly on the patterns of the coronary arteries feeding the ventricular myocardium.

	Material and methods

Top Abstract Introduction Material and methods Results Comment Acknowledgments References

 
We investigated 183 hearts diagnosed by postmortem inspection as having isomeric right or left atrial appendages. All these hearts were previously described in terms of the morphologic features of the atrial chambers and the precise venoatrial connections [5]. All the hearts came from patients known to have had visceral heterotaxy. Within the heart itself, however, the unifying feature was isomerism of the atrial appendages. Based on the morphologic criterion of the extent of the pectinate muscles within the appendages, relative to the atrial vestibules, all hearts could be categorized as showing isomerism of the morphologically right or morphologically left appendages (Fig 1).

View larger version (99K): [in this window] [in a new window]   Fig 1. Isomerism of the atrial appendages is diagnosed on the basis of the extent of the pectinate muscles within the appendages relative to the atrial vestibules. In right isomerism (A), the pectinate muscles encircle the vestibules, but are confined anteriorly (B) in those with left isomerism (* = crux of the heart; = os of appendage.)

	Results

Top Abstract Introduction Material and methods Results Comment Acknowledgments References

 
Ventricular morphology
Hearts with each atrium connected to its own ventricle (biventricular atrioventricular connections) were more common in the group of hearts with isomeric left than in those with isomeric right appendages (p = 0.003) (Table 1 ). Among these hearts, left-hand topology of the ventricular mass (l-ventricular loop) (Fig 2) was more frequently seen in those with isomeric right appendages (p < 0.001). In contrast, in those with both atrial chambers connected predominantly to the same ventricle (Fig 3 ), or the rarer hearts in which one atrioventricular connection was absent, no statistical difference was observed in the relationship of rudimentary and incomplete ventricles relative to the dominant ventricle, to the specific type of univentricular atrioventricular connection, nor to the structure of the atrioventricular valves.

View larger version (150K): [in this window] [in a new window]   Fig 2. In hearts with each atrium connected to its own ventricle (biventricular atrioventricular connections), ventricular topology of the ventricular mass is described as right- and left-hand patterns, corresponding to d- and l-ventricular loop, respectively. The heart shown on the upper panels has isomeric arrangement of the left atrial appendages, whereas the heart shown on the lower panels has isomeric right appendages.

View larger version (76K): [in this window] [in a new window]   Fig 3. In hearts with both atrial chambers connected predominantly to the same ventricle (double inlet ventricle), the dominant ventricle was of morphologically left (A), or of morphologically right (B) type. In rare instances, there was a solitary and morphologically indeterminate ventricle (C).

The atrioventricular junctions were most frequently guarded by a common atrioventricular valve in hearts with isomeric right appendages (Table 2 ), irrespective of the presence of biventricular or univentricular connections, whereas separate right and left valves were more frequent in those with isomeric left appendages (p < 0.001). The right-sided component of the common atrioventricular valve was imperforate in one heart with isomeric right appendages, and the left-sided one was imperforate in another with isomeric left appendages, both hearts having right-hand topology.

Ventriculoarterial connections and arterial pathways
Concordant ventriculoarterial connections were less frequent, and absence of any connection between the ventricle and the pulmonary trunk was more frequent, in hearts with isomeric right than in those with isomeric left appendages (p < 0.001) (Table 3 ). The aorta was frequently positioned anterior to the pulmonary trunk in those with isomeric right appendages, whereas the posterior ("normal") position was common in the setting of isomeric left appendages (p < 0.001) (Table 4 ). Concordant ventriculoarterial connections were correlated to the "normal" position of the aorta relative to the pulmonary trunk, whereas an anterior aorta was more frequently associated with absence of any connection between the pulmonary trunk and the ventricles (p < 0.001). Reflecting these arrangements, the subarterial infundibulums were often of abnormal pattern, particularly in the group of hearts with isomeric right appendages, in which subpulmonary obstruction was also very frequent (Table 3). In contrast, either subaortic or aortic obstruction was seen with a relatively high incidence in the group of hearts with isomeric left appendages. Abnormal arborization of the pulmonary arterial tree was seen in 8 hearts with isomeric right, and in 1 with isomeric left appendages (Table 4).

View larger version (57K): [in this window] [in a new window]   Fig 4. Coronary arterial branching and orifices. (A) Diagram of the coronary arteries in the normal heart and that of the most common pattern in complete transposition. (B) In hearts with the balanced morphologically left and right ventricles. (C) In hearts with a dominant morphologically right ventricle with a rudimentary and incomplete left ventricle. (D) In hearts with a dominant morphologically left ventricle with a rudimentary and incomplete right ventricle. (E) In hearts with a solitary and indeterminate ventricle. Arrows indicate unusual origins and courses of the major coronary arteries. Patterns in hearts with left-hand topology were described by mirror image projection. (LV = morphologically left ventricle; RV = morphologically right ventricle; indetV = solitary and indeterminate ventricle; PT = pulmonary arterial trunk; AIA = anterior interventricular artery; PIA = posterior interventricular artery; LVA = left ventricular arteries; RVA = right ventricular arteries.).

In the 6 hearts with a solitary and indeterminate ventricle, it was not possible to identify interventricular arteries because there was no muscular ventricular septum. A solitary coronary arterial system was seen in 3 hearts, and two major coronary arteries were present in the other 3 hearts. These ventricular arteries were distributed symmetrically in all except 1 specimen (Fig 4E).

	Comment

Top Abstract Introduction Material and methods Results Comment Acknowledgments References

 
Appropriate introduction of palliative surgical procedures has markedly improved the rates of early survival in patients with isomerism and visceral heterotaxy [1,10,11]. In addition, concomitant improvements and innovations in surgical technique have increased the number of patients undergoing either bypass of the right heart or biventricular repair [1–3].

The crucial factor in achieving successful anatomic repair is obviously the morphology of the ventricles themselves. The majority of our hearts with right isomerism had one dominant and one rudimentary ventricle, or else a solitary and indeterminate ventricle, most often with the atrioventricular junctions guarded by a common valve. These hearts are unlikely to be suitable for anatomic repair, at least at present. Such hearts accounted for more than half of those with isomeric right appendages, but also made up one quarter of those with isomeric left appendages. Although neither clinical volumetry of the ventricles, nor physiologic information were available in our series, as judged morphologically we deemed that the majority of hearts with each atrium connected to its own ventricle possessed balanced ventricles. In respect to the arrangement of such balanced ventricles, we are unable to account for the random nature of right-hand and left-hand patterns in hearts with isomeric right appendages, as opposed to the predominance of right-hand patterns in those with isomeric left appendages.

Combined with the frequent presence of a common atrioventricular valve, as well as the grossly abnormal ventriculoarterial connections (see below), the features of the ventricular mass militate against successful anatomic repair in the majority of cases with isomeric right appendages. When all anatomic features are considered, only 6 hearts of 125 (5%) with right isomerism could reasonably have been considered potentially suitable for anatomic biventricular repair. In the group of hearts with isomeric left appendages, in contrast, biventricular surgical repair would have been feasible, at least on the basis of anatomy, in 35 of 58 cases (60%). Lest it be thought that these figures are compounded by the autopsy nature of this series, these incidences are comparable (no significant difference by ² test) to those seen in the patients with isomeric atrial appendages investigated at the National Cardiovascular Center in Osaka, Japan, during the last decade. In that clinical series, 7 of 60 patients with isomeric right appendages undergoing a definitive procedure (12%) were successfully corrected by anatomic biventricular repair. Functional biventricular repair, in which the morphologically right ventricle was placed to support the systemic circulation, leaving the left ventricle for the pulmonary circulation, was achieved in a further 2 patients with isomeric right appendages (3%). In 34 of 48 patients having isomeric left appendages (71%), however, an anatomic repair was the definitive option. In the remaining patients, 51 with isomeric right and 14 with isomeric left appendages, total cavopulmonary connection was the chosen surgical option because of the impossibility of achieving anatomic repair.

Such right heart bypass, which would have represented the only surgical choice in 119 of our specimens with isomeric right appendages (95%), and in 23 with isomeric left appendages (40%), would not have demanded the same stringent anatomic requirements within the ventricles so as to create postoperatively a noncyanotic circulation. Moderate or severe subaortic stenosis, which might have produced undesirable hypertrophy of the ventricular mass for a Fontan-type procedure [12], was present in 8 with isomeric right appendages (7% of 119 cases), and 5 with isomeric left appendages (22% of 23). The functional aspects of the ventricular morphology, rather than morphologic aspects, are more likely to represent the problem in these instances [13, 14]. In this respect, therefore, it should be noted that the incidence of hearts with a dominant morphologically left ventricle and a rudimentary and incomplete right ventricle was low in the settings of either isomeric right or left appendages.

Precise knowledge concerning the coronary arterial circulation is self-evidently of major importance. We have discussed the morphologic features of the cardiac veins previously, and emphasized their surgical implications [15]. To the best of our knowledge, no precise description has yet been given for the coronary arterial anatomy in the setting of isomeric atrial appendages. The surgical implications of the observed anatomy can be considered from three viewpoints. The first concern is the frequency of anterior courses of the major coronary arteries relative to the arterial trunks. Surgical incisions for widening the ventricular outflow tracts would have been limited in the subaortic and subpulmonary regions in a quarter and a seventh, respectively, of all 163 hearts in which we investigated the coronary arterial anatomy. The precise courses of the coronary arteries should, of course, always be evaluated before embarking on such surgical strategies. The second potential problem is an intramural course of the coronary arteries within the aortic wall. In 2 cases of 3 with such abnormal origin, the arterial switch procedure would have been ruled out because of the presence of either severe pulmonary stenosis or atresia. In the remaining heart, however, because significant subaortic stenosis would have required some surgical intervention, the abnormal coronary arterial pattern could have posed surgical problems had a switch been contemplated. In this particular case, nonetheless, with double inlet to the morphologically right ventricle, a Norwood type procedure would have been the option of surgical choice [16] rather than the arterial switch procedure. The third potential problem is the high incidence of a solitary coronary artery, much higher than expected in normally structured hearts. In this setting, it is crucial to avoid accidental obstruction of the solitary stem and malperfusion within the arterial system. This might be caused by injury, mechanical compression, or air embolization of the solitary coronary artery. These happenings, of course, would be of much more significance in patients with a solitary artery, because they would produce greater ischemia of the ventricular mass than if occurring in hearts with two coronary arteries arising from the aorta. Such ischemia, even if only transient, would be potentially deleterious, particularly in the critical period occurring immediately after extensive surgery.

To achieve anatomic repair, the morphologically left ventricle, of a suitable size to support the systemic circulation, must also be connected to the aortic valvar orifice. The abnormal ventriculoarterial connections seen in many of the hearts in our series would have been sufficient to invalidate simple intraventricular rerouting. The pulmonary orifice, of course, can be also used in anatomic repair if a concomitant switch procedure is carried out at the arterial level. This option, however, would also have been ruled out in many of our hearts because of the frequent finding of obstructions within the subpulmonary pathway, particularly in hearts with isomeric right appendages. In those with isomeric left appendages, obstructions to the subaortic outflow tract, as well as at the level of the aortic arch, are also of clinical importance. The incidence in our present series was comparable to that found in previous studies [6, 17]. Direct enlargement of the subaortic tract [18], or construction of dual outlets partially bypassing the obstructive lesion [19], can be used so as to create an adequate outlet from the morphologically left ventricle. When the aorta is anterior to the pulmonary trunk, the connection of the pulmonary trunk to the ventricle was often narrow between the ventriculo-aortic junction and a common or a solitary atrioventricular valve. In hearts with pulmonary atresia, the ventriculo-infundibular fold was almost always prominent between the attachment of the atrioventricular valve and the aortic orifice, producing a solitary and subaortic infundibulum. In contrast, when the aorta was located posterior to the pulmonary trunk, the aortic orifice was squeezed between the pulmonary arterial connection to the ventricle and the atrioventricular junction. In our series, however, there were no hearts with the morphologically tricuspid and the mitral valves showing subaortic obstruction. This, presumably, is because the posteriorly located aorta in such circumstances wedges between the atrioventricular valves, as seen in the normal heart [20].

Information regarding the conduction system is obviously another aspect of concern. Knowledge concerning the arrangement of the atrial appendages is crucial in predicting the location of the sinus nodes [5]. The orientation of the atrioventricular nodes and their conducting bundles, in contrast, is most likely related to the ventricular structure. To avoid atrioventricular block when achieving either plasty to the atrioventricular valves or intraventricular baffling, it is essential to recognize precisely the structural features of the ventricle. Although the number of articles describing morphologic findings is still small [1], it is known that conduction via a posterior node is the rule in hearts with biventricular atrioventricular connections and right-hand topology, whereas an anterior node or a sling is expected in those with left-hand topology, the bundles themselves penetrating from the junctions at the site of the crest of the muscular ventricular septum. In hearts with a dominant right ventricle and an incomplete left ventricle, the atrioventricular node usually gives rise to a conducting bundle at the posterior crest of the muscular ventricular septum. In all other circumstances, an anomalous node gives rise to an anterior course penetrating bundle. When the ventricular mass is formed by a solitary and indeterminate chamber, the location of the conduction tissue cannot reliably be predicted.

The spectrum of ventricular structures in hearts with isomeric atrial appendages, including the outflow tracts, is obviously of major surgical import. Precise diagnoses of these features are needed in each patient so as to determine the optimal surgical strategy. It is the very diversity in morphology, nonetheless, that complicates the choice. The awareness of the potential anatomic spectrums should help us to avoid overlooking clinically significant abnormalities.

	Acknowledgments

Top Abstract Introduction Material and methods Results Comment Acknowledgments References

 
We express our special thanks to those who granted us access to the hearts described in our present study, particularly Mr William A. Devine of the Children’s Hospital of Pittsburgh, Pennsylvania, and Dr Audrey Smith of the Royal Liverpool Children’s Hospital in Liverpool, UK. The work was supported by grants from the British Heart Foundation together with the Joseph Levy Foundation.

	References

Top Abstract Introduction Material and methods Results Comment Acknowledgments References

 

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