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Tex Heart Inst J. 2010; 37(4): 445–448.
PMCID: PMC2929878

Superior Left Ventriclein Combination with Inferior Right Ventricle Presenting with Balanced Hemodynamics and Mild Symptoms in a Late Adolescent

Guangzhao Yang, MD, Qi Wang, MD, Jiong He, BS, and Ming Wu, MD

Abstract

We describe a case of superoinferior ventricles with an unusual superior location of the left ventricle and inferior location of the right ventricle. The patient was a 17-year-old boy who had been admitted for paroxysmal supraventricular tachycardia with pre-excitation syndrome. The cardiac anomaly was first recognized after admission, and the pre-excitation syndrome, eventually, was treated successfully by radiofrequency catheter ablation. This case featured a segmental set notation {S, D, S} with concordant atrioventricular and ventriculoarterial alignments. Persistent left superior vena cava was the only associated defect. To the best of our knowledge, there has been only 1 other reported case of superoinferior ventricles in which the left ventricle was in the superior position. In our case, the patient's hemodynamics were sufficiently normal that he had reached adolescence with only minor adverse events.

Key words: Adolescent, crisscross heart, heart defects, congenital/diagnosis/pathology, heart ventricles/abnormalities, persistent left superior vena cava

Superoinferior ventricles typically had been described as featuring a common spatial arrangement, with the right ventricle (RV) lying “upstairs,” superior to the left ventricle (LV). However, in 2003, a case of a 30-month-old boy with a superior morphologic LV was reported1 as a new variation of this congenital heart defect. Our present case is the first of this new variant to be identified in an adolescent. This anomaly was essentially unaccompanied by the additional structural defects that are frequent in superoinferior ventricles; therefore, this case might enrich our knowledge about this malformation and its essential features.

Case Report

A 17-year-old boy—born after a full-term pregnancy and possessed of an uneventful medical history—was admitted to our hospital in August 2006 because of an episode of syncope, preceded by chest discomfort and palpitations, which had resolved spontaneously.

On admission, he had normal test results for oxygen saturation and hemoglobin level (14.3 g/dL). An electrocardiogram revealed sinus rhythm, pre-excitation syndrome, and right-axis deviation with occasional isolated and coupled atrial or ventricular extrasystoles (Fig. 1). A transthoracic echocardiogram (Fig. 2) suggested superoinferior spatial configuration of the 2 ventricles and possibly a crisscross heart. The great arteries were found to be in D-configuration and normally crossed (Fig. 3). Cardiovascular magnetic resonance confirmed the superoinferior arrangement of the ventricle: that is, a superior morphologic LV and an inferior RV separated by a horizontal interventricular septum (Fig. 4). Cardiovascular magnetic resonance confirmed the presence of atrial situs solitus and normal atrioventricular alignment (Fig. 5). The mitral and tricuspid valves were normal. Concordant ventriculoarterial connections were also confirmed (Fig. 6). The right subclavian vein was found to drain into a persistent left superior vena cava, which then drained into the coronary sinus, without a right superior vena cava (Fig. 7).

figure 10FF1
Fig. 1 An electrocardiogram, before radiofrequency catheter ablation, shows pre-excitation and ventricular extrasystole.
figure 10FF2
Fig. 2 A transthoracic echocardiogram (4-chamber view) shows the morphologic left ventricle (LV) situated above the morphologic right ventricle (RV).
figure 10FF3
Fig. 3 A transthoracic echocardiogram shows the pulmonary artery position leftward and anterior to the ascending aorta, which indicates normal D-loop great arteries.
figure 10FF4
Fig. 4 A cardiovascular magnetic resonance image confirms the proper connection of the pulmonary artery (PA) to the morphologic right ventricle (MRV). An obviously muscular right ventricular outflow tract (RVOT) confirms the nature of the MRV chamber. ...
figure 10FF5
Fig. 5 A transverse cardiovascular magnetic resonance image shows that the left atrium (LA) lies to the left of the right atrium (RA). There is no muscular infundibulum between the mitral valve (MV) and the aortic valve (AV), which confirms the concordant ...
figure 10FF6
Fig. 6 A cardiovascular magnetic resonance image shows the normal position of the liver and the stomach. There is no muscular infundibulum between the mitral valve (MV) and the aortic valve (AV), which confirms the concordant connection of the morphologic ...
figure 10FF7
Fig. 7 A cardiovascular magnetic resonance image shows that the anomalous persistent left superior vena cava (PLSVC) drains into the coronary sinus (which does not appear in this image). Normal positions of liver and stomach are also shown.

The superoinferior ventricular arrangement was clearly seen: a superior morphologic LV and an inferior morphologic RV. Using a segmental approach, we described the arrangement as visceroatrial situs solitus, dextro-loop (D-loop) ventricular morphology, solitus normal of the great arteries {S, D, S}, and concordant ventriculoarterial and atrioventricular connections, with a left-sided apex.

The patient underwent a successful radiofrequency catheter ablation procedure and was left free of the pre-excitation syndrome (Fig. 8).

figure 10FF8
Fig. 8 This electrocardiogram shows disappearance of pre-excitation after radiofrequency catheter ablation.

Discussion

Our patient manifested superoinferior ventricles—that is, an over-and-under, rather than a side-by-side, relationship. The morphologic LV was situated above the morphologic RV. To the best of our knowledge, only 1 case of a similar ventricular arrangement has been reported in the medical literature, and that was in a 30-month-old child who had, in addition, multiple intracardiac deformities1: our case is the first to feature normal intracardiac morphology, which enabled the patient to reach his teenage years with only mild symptoms.

The superoinferior arrangement of the ventricles is generally believed to reflect an abnormality in rotation or tilting along the ventricular long axis, during cardiac morphogenesis. In the present case, the superoinferior ventricles, in our judgment, coexist with crisscross heart. Although crisscross heart and superoinferior ventricles often display similar morphologic defects and frequently coexist with each other, they are not exactly synonymous.2 Some investigators have even suggested that crisscross heart is an angiocardiographic illusion, rather than an anatomic fact.3 Cases of superoinferior ventricles almost never occur as an isolated presentation.4 Associated anomalies, such as pulmonary stenosis, ventricular septal defect, ventriculoarterial connection discordance, hypoplasia of the RV, and straddling atrioventricular valves, often present clinically in neonates as cyanosis, systolic murmur, or both.

These associated defects, complex and diverse in their presentation, can make it difficult to understand the morphogenesis of superoinferior ventricles. Essentially, the morphogenesis of the embryonic heart follows these steps: first, the situs of the atria is established (prelooping stage); then, the laterality of the atrioventricular valves and connections is irreversibly established by bulboventricular loop formation; and finally, the definitive formation of the apex occurs.4 The present case suggests that the malformation of superoinferior ventricles can occur at a later stage than does the establishment of the atrial and visceral situs or the bulboventricular loop. A suggested mechanism for the orientation of the superoinferior ventricles is abnormal rotation of the apical portions of the 2 ventricles along the cardiac long axis during apex formation.4 In our patient, the spatial configuration of the superior LV and inferior RV appears to have been caused by an abnormal counterclockwise rotation of concordant, D-loop ventricles.1

The only associated defect in this case, the persistent left superior vena cava, is the embryogenetic consequence of a persistent left anterior cardinal vein and the obliteration of the common cardinal vein and the proximal parts of the anterior cardinal veins on the right. In our patient, the persistent left superior vena cava was shown to drain into the coronary sinus. A persistent left superior vena cava that connects to the right atrium via an enlarged coronary sinus can alter left ventricular inflow obstruction and stretch the atrioventricular node and bundle of His. These cardiac disturbances are possibly responsible for the symptoms of chest discomfort, palpitations, and cardiac arrhythmia reported by our patient.

Cardiovascular magnetic resonance seems to be a useful noninvasive method for visualizing the segmental alignments and the topographic anatomy of individual segments of complex heart abnormalities, including superoinferior ventricles. In our patient, cardiovascular magnetic resonance showed the atrial situs solitus, normal D-loop great arteries, and concordance in both the ventriculoarterial and atrioventricular connections. The crisscross relationship5 of the atrioventricular connections was observed by echocardiography and confirmed by cardiovascular magnetic resonance. In deciding the D-loop handedness of the RV in this entity, we applied a simple and practical method of describing segmental connections that originally was suggested by Van Praagh and colleagues.5

Footnotes

Address for reprints: Ming Wu, MD, Department of Cardiothoracic Surgery, 2nd Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou 310009, PRC

E-mail: moc.liamtoh@gnimuwi

References

1. Porras D, Kratz C, Loukas M, van Doesburg NH, Davignon A, Van Praagh R. Superoinferior ventricles with superior left ventricle and inferior right ventricle: a newly recognized form of congenital heart disease. Pediatr Cardiol 2003;24(6):604–7. [PubMed]
2. Carminati M, Valsecchi O, Borghi A, Balduzzi A, Bande A, Crupi G, et al. Cross-sectional echocardiographic study of criss-cross hearts and superoinferior ventricles. Am J Cardiol 1987;59(1):114–8. [PubMed]
3. Freedom RM, Culham G, Rowe RD. The criss-cross and superoinferior ventricular heart: an angiocardiographic study. Am J Cardiol 1978;42(4):620–8. [PubMed]
4. Angelini P, Lopez A, Lufschanowski R, Nemeth MA, Flamm SD. Coronary arteries in crisscross heart. Tex Heart Inst J 2003;30(3):208–13. [PMC free article] [PubMed]
5. Van Praagh S, La Corte M, Fellows KE, Bossina K, Busch HJ, Keck EW, et al. Supero-inferior ventricles: anatomic and angiocardiographic findings in 10 postmortem cases. In: Van Praagh R, Takao A, editors. Etiology and morphogenesis of congenital heart disease. Mount Kisco (NY): Futura Publishing Co.; 1980. p. 317–78.

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