mri appearance of a double inlet and double outlet right ventricle with supero-inferior ventricular...

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Magnetic Resonance Imaging, Vol. 14, No. 9, pp. 1107- 1112. 1996 Copyright 0 1996 Elsevier Science Inc. Printed in the USA. All rights reserved 0730-725X/96 515.00 + .OO ELSEVIER l Case Report PI1 SO730-725X(96) 001151 MRI APPEARANCE OF A DOUBLE INLET AND DOUBLE OUTLET RIGHT VENTRICLE WITH SUPERO-INFERIOR VENTRICULAR RELATIONSHIP RUDOLF P. BEEKMAN,* FREDERIK J.A. BEEK,~ ERIC-JAN MEIJBOOM,~ AND ARNOLD C.G. WENINK$ Departments of tPediatric Cardiology and Radiology, Wilhelmina Children’s Hospital, Utrecht, *University Hospital of Maastricht, *Department of Anatomy and Embryology, University of Leiden, The Netherlands This patient was diagnosed with a double inlet and double outlet RV with supero-inferior ventricular relationship, ventricular inversion was diagnosed on the basis of left-handed topology of the RV, and the straddling of the right-sided mitral valve over an anterior VSD, with its tension apparatus extending into the outflow tract of the RV. MRI was found to be superior to color Doppler echocardiography and contrast ventriculography in the segmental analysis leading to a full understanding of this complex case. Copyright 0 1996 Elsevier Science Inc. Keywords: MRI; DIRV; DORV; Supero-inferior ventricular relationship; Ventricular inversion. INTRODUCTION Congenital malformations of the heart can be difficult to completely understand using color Doppler echocar- diography (CDE) or contrast ventriculography; mag- netic resonance imaging (MRI) has been very useful in this setting. We present an unusual case of supero- inferior ventricles, one of the malformations with twisted atrioventricular ( AV ) connections, ’ in which MRI was critical to the full understanding of the com- plex anatomy. MRI does not have the limitation of restricted insonation of ultrasound by bone and air, and in comparison with contrast ventriculography has the advantage of showing all structures in the field of view, allowing better evaluation of the spatial relationships of the components of the heart. PATIENT AND METHODS A 2-wk-old infant male presented with cyanosis and a murmur. He was not in cardiac failure. A double inlet and outlet ventricle was diagnosed by CDE. The ventricu- lar septum appeared to be horizontal. The inferior ventri- cle was smaller than the superior ventricle. The ventricu- lar septal defect (VSD) could not be precisely localized on two-dimensional echocardiography, and CDE was hampered by the relative lack of flow over the VSD (Fig. 1). Pulmonary artery banding was done to protect the patient’s pulmonary vasculature at 1 month. A segmental analysis of the heart including the localization of the VSD and precise characterization of the ventricles was necessary to determine subsequent management. To this end MR imaging was performed at 3 l/2 months. A complete segmental diagnosis of the heart includes a description of morphology at viscera-atrial, ventricular and arterial levels.* The first step is to establish viscero- atrial situs, to be based on pulmonary hilar morphology and that of the atria. Abdominal situs and veno-atrial connections are strong indicators. Then, based on the morphology of the ventricular mass, but irrespective of its position in the thorax, the type of AV connection is diagnosed. Similarly, irrespective of the relative positions of the great arteries, the type of ventriculo-arterial con- nection is established. Superimposed on this analysis, the modes of the connections can be specified and morpho- logic details and three-dimensional relationships have to be noted. In the present case, the underdevelopment of the inferior left ventricle (LV) and the remote relationship RECENED 12/29/1995; ACCEFTED: 412511996. P.O. Box 5800, 6202 AZ Maastricht, The Netherlands. Address correspondence to Rudolf P. Beekman, M.D., Email:[email protected] 1107

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Magnetic Resonance Imaging, Vol. 14, No. 9, pp. 1107- 1112. 1996 Copyright 0 1996 Elsevier Science Inc.

Printed in the USA. All rights reserved 0730-725X/96 515.00 + .OO

ELSEVIER

l Case Report

PI1 SO730-725X( 96) 001151

MRI APPEARANCE OF A DOUBLE INLET AND DOUBLE OUTLET RIGHT VENTRICLE WITH SUPERO-INFERIOR

VENTRICULAR RELATIONSHIP

RUDOLF P. BEEKMAN,* FREDERIK J.A. BEEK,~ ERIC-JAN MEIJBOOM,~ AND ARNOLD C.G. WENINK$

Departments of tPediatric Cardiology and Radiology, Wilhelmina Children’s Hospital, Utrecht, *University Hospital of Maastricht, *Department of Anatomy and Embryology, University of Leiden, The Netherlands

This patient was diagnosed with a double inlet and double outlet RV with supero-inferior ventricular relationship, ventricular inversion was diagnosed on the basis of left-handed topology of the RV, and the straddling of the right-sided mitral valve over an anterior VSD, with its tension apparatus extending into the outflow tract of the RV. MRI was found to be superior to color Doppler echocardiography and contrast ventriculography in the segmental analysis leading to a full understanding of this complex case. Copyright 0 1996 Elsevier Science Inc.

Keywords: MRI; DIRV; DORV; Supero-inferior ventricular relationship; Ventricular inversion.

INTRODUCTION

Congenital malformations of the heart can be difficult to completely understand using color Doppler echocar- diography (CDE) or contrast ventriculography; mag- netic resonance imaging (MRI) has been very useful in this setting. We present an unusual case of supero- inferior ventricles, one of the malformations with twisted atrioventricular ( AV ) connections, ’ in which MRI was critical to the full understanding of the com- plex anatomy. MRI does not have the limitation of restricted insonation of ultrasound by bone and air, and in comparison with contrast ventriculography has the advantage of showing all structures in the field of view, allowing better evaluation of the spatial relationships of the components of the heart.

PATIENT AND METHODS

A 2-wk-old infant male presented with cyanosis and a murmur. He was not in cardiac failure. A double inlet and outlet ventricle was diagnosed by CDE. The ventricu- lar septum appeared to be horizontal. The inferior ventri- cle was smaller than the superior ventricle. The ventricu-

lar septal defect (VSD) could not be precisely localized on two-dimensional echocardiography, and CDE was hampered by the relative lack of flow over the VSD (Fig. 1). Pulmonary artery banding was done to protect the patient’s pulmonary vasculature at 1 month. A segmental analysis of the heart including the localization of the VSD and precise characterization of the ventricles was necessary to determine subsequent management. To this end MR imaging was performed at 3 l/2 months. A complete segmental diagnosis of the heart includes a description of morphology at viscera-atrial, ventricular and arterial levels.* The first step is to establish viscero- atrial situs, to be based on pulmonary hilar morphology and that of the atria. Abdominal situs and veno-atrial connections are strong indicators. Then, based on the morphology of the ventricular mass, but irrespective of its position in the thorax, the type of AV connection is diagnosed. Similarly, irrespective of the relative positions of the great arteries, the type of ventriculo-arterial con- nection is established. Superimposed on this analysis, the modes of the connections can be specified and morpho- logic details and three-dimensional relationships have to be noted. In the present case, the underdevelopment of the inferior left ventricle (LV) and the remote relationship

RECENED 12/29/1995; ACCEFTED: 412511996. P.O. Box 5800, 6202 AZ Maastricht, The Netherlands. Address correspondence to Rudolf P. Beekman, M.D., Email:[email protected]

1107

1108 Magnetic Resonance Imaging l Volume 14, Number 9, 1996

Fig. 2. Axial 1. The ascending aorta is on the right with normal relationship to the pulmonary artery. The aorta de- scends on the left side.

Fig. 1. Ao = aorta; P = pulmonary artery trunk; M = mitral valve; T = tricuspid valve; VS = ventricular septum.

between the VSD and the aortic valve precluded the possibility of a biventricular repair.

Definitive preoperative evaluation was based on MRI and CDE performed at 2 yrs, which was essen- tially the same as at 3 l/2 months. Cardiac catheter- ization was performed to measure pressure in the

pulmonary artery. There was no pressure gradient within the right ventricle (RV). Contrast ventricu- lography, the gold standard in the pre-MRI era, was also performed, but the exact location of the VSD was better visualized by MRI. Accordingly, a total cave-pulmonary connection and a Damus-Kaye- Stensel procedure was performed at 2 yrs 2 months. The pulmonary artery was connected to the aorta, in case subaortic obstruction would develop.

MRI was performed by a Philips TS-II gyroscan operating at 0.5 Tesla (Philips, Eindhoven, The Netherlands). Spin-echo sequences were used with transverse, coronal, and sagittal sections (Figs. 2- 12).

(4 W

Fig. 3. Axial 2 and 3. Normal appearance of the atria. (A) The left-sided conus is muscular whereas the conus of the right- sided outflow tract (B) is attenuated.

Double inlet and outlet RV 0 R.P. BEEKMAN ET AL 1109

Fig. 4. Axial 4. The AV junction is univentricular and the two AV valves can be seen in one plane. Note the coarse trabecular pattern of the superior ventricle.

RESULTS

MRI jindings

Veno-atria1 connections. Situs solitus of the abdominal organs with normal venous connections of the atria.

Atria1 relationships. The atria are normally related, the atria1 appendages cannot be identified. The axes of the atria do not cross each other.

AV connections. The left-sided AV valve connects to the superior ventricle with RV morphology, and the right-sided AV valve connects to the superior ventricle for the greater part, and partly to the inferior ventricle which has LV morphology. Thus, there is a double inlet morphologically RV with straddling of the right-

Fig. 6. Axial 6. The inferiorly situated left ventricle. Part of the tension apparatus of the right-sided AV valve can be seen.

sided AV valve. The right-sided AV valve is more anterior than the left-sided AV valve, and extends to the outflow portion of the ventricle.

Ventricular relationships. The dominant ventricle is enlarged and superior to the inferior ventricle. The trabecular septum is aligned in the horizontal plane and the inlet septum extends to the crux of the heart. The most anterior part of the septum can be seen to extend from the left lateral to the right lateral wall of

Fig. 5. Axial 5. The horizontally aligned septum can be seen. The VSD lies anterior to to the right-sided AV valve.

Fig. 7. Coronal 1 (most anterior plane). Horizontal septum and both outflow tracts from the right ventricle.

1110 Magnetic Resonance Imaging 0 Volume 14, Number 9, 1996

(4 Fig. 9. Coronal 4. Normal appearance of the LA.

(W

Fig. 8. Coronal 2(A) and 3(A). Remote relationship of VSD to the great vessels.

the heart, which can be seen in the most anterior coro- nal section. The two adjacent posterior coronal sections depict the VSD in a median position with horizontal alignment. An axial section through the septum con-

Fig. 10. Sagittal 1 (extreme left>. The horizontal septum and enlarged right ventricle are seen.

Double inlet and outlet RV l R.P., @E~IAN ET AL. 1111

Fig. 11. Sagittal 2 (medial to Sag. 1). Outflow tract and pulmonary artery.

firms these findings, and demonstrates the almost flat- ness or lack of curvature of the septum. On coronal section the axis of the atria1 septum crosses the axis of the trabecular septum at an obtuse angle. There are coarse trabeculations and a circular muscular outlet portion under the pulmonary valve in the dominant superior ventricle suggesting right ventricular mor- phology. The outlet portion under the aorta lies to the right of pulmonary valve and has an attenuated muscu- lar cone with fibrous continuity with the left-sided AV valve. The VSD is remote from the ventriculo-arterial valves. The inferior ventricle is rudimentary with a deficient outlet portion, and has fine trabeculations sug- gesting left ventricular morphology.

The topology of the superior ventricle is left-handed or L-loop.

Ventriculo-arterial connections. The type of ventric- ulo-arterial connection is double outlet. Thus, there is a double outlet morphologically RV.

Great arterial relationship. The aortic orifice is to the right of the pulmonary orifice, and the ascending aorta is to the right of the pulmonary trunk.

DISCUSSION

This patient has a complex malformation, of which the diagnosis was further complicated by the unex- pected ventricular relationships. In general, the criss- cross heart, another of the malformations with twisted AV connections will present itself as a more complex malformation than that with supero-inferior ventricu- lar relationship. In the first case, a torsion of the ven- tricular mass may be thought to be the cause of the unexpected relationship, in the second case a more simple tilting of the ventricles has brought the ventric- ular septum in the horizontal plane.3 This does not mean that all criss-cross hearts would finally appear to be very complex when one would try, ideally in the patient or literally in the autopsy specimen, to reverse the twisting. Thus, a complex criss-cross heart may turn out to be no more than complete transposi- tion of the great arteries.’ Similarly, the more simple ventricular tilting, leading to a horizontal ventricular

Fig. 12. Sagittal 3. Posterior aspect of ascending aorta is in fibrous continuity with left-sided AV valve. The right-sided AV valve is more anterior than the left-sided valve and extends into the outflow portion of the dominant ventricle. Part of the aortic annulus is seen.

1112 Magnetic Resonance Imaging 0 Volume 14, Number 9, 1996

septum, may have taken place in a heart with initially rather complex morphology, as was the case in our patient.

The first speculation is that about the direction of the ventricular loop. The morphologically RV had left hand topology, which is usual in the setting of ventricular inversion.’ Readjustment of the ventricu- lar relationships would bring the ventricular septum in its more usual plane, and it would indeed make the morphologically RV left-sided. One would then expect the left-sided AV valve to be the tricuspid valve, the right-sided AV valve being the mitral valve, because valve morphology invariably follows that of the underlying ventricle.5X6

The next morphologic observation is that of the straddling of the right-sided AV valve, with its ten- sion apparatus extending into the outflow tract. This is pathognomonic for a straddling mitral valve,5,7 which supports the ventricular inversion as already diagnosed above. Moreover, the two papillary mus- cles seen in the inferior ventricle in Fig. 6 are those of a usual mitral valve.

These observations make it clear that the mere diagnosis of double inlet morphologically RV hemo- dynamically is correct, but does not completely de- scribe the case. As is found in the majority of such cases, there was straddling of the right-sided mitral valve over an anterior VSD.* In fact, double inlet to a morphologically RV can only be the result of sufficient override of a straddling mitral valve. More than 50% override of a straddling mitral valve was reported in only 2113 cases, whereas 9114 cases with a straddling tricuspid valve showed more than 50% override of that valve.’ Therefore, double inlet RV is rare. In the present case, we have been able to describe clinically such a heart in detail. This shows the power of MRI when used systematically.

REFERENCES

1. Yoo, S.J.; Sea, J.W.; Lim, T.H.; Park, I.S.; Hong, C.Y. Hearts with twisted atrioventricular connections: find- ings at MR imaging. Radiology 188:109-l 13; 1993.

2. Van Praagh, R. The segmental approach to diagnosis in congenital heart disease. In: J. Gore, (ed.). Birth Defects, Vol VIII. Baltimore: Williams & Wilkins Co.; 1972: pp. 4-23.

3. Anderson, R.H.; Macartney, F.J.; Shineboume, E.J.; Ty- nan, M. Abnormal positions and relationships of the heart. In: Paediatric Cardiology. London: Churchill Liv- ingstone; 1987: pp. 10.57-1072.

4. Van Mill, G.J.; Moulaert, A.J.; Harinck, E.; Wenink, A.C.G.; Oppenheimer-Dekker, A. Subcostal two-dimen- sional echocardiographic recognition of a criss-cross heart with discordant ventriculo-arterial connection. Pediatr. Cardiol. 3:319-323; 1982.

5. Wenink, A.C.G.; Gittenberger-de Groot, A.C.; Oppen- heimer-Dekker, A.; VanGifs, F.A.W.; Draulans-Noe, H.A.Y.; Moene, R.J. Septation and valve formation: similar processes dictated by segmentation. In: J.J. Nora, A. Takao (Eds) . Congenital Heart Disease: Causes and Processes. Armonk, NY: Futura Publishing Co.; 1984: pp. 513-529.

6. Wenink, A.C.G.; Gittenberger-de Groot, AC. Embryol- ogy of the mitral valve. Int. J. Cardiol. 11:75-84; 1986.

7. Wenink, A.C.G.; Gittenberger-de Groot, A.C. Strad- dling mitral and tricuspid valves: morphologic differ- ences and developmental backgrounds. Am. J. Cardiol. 49:1959-1971; 1982.

8. Becker, A.E.; Anderson, R.H.; Penkoske, P.A.; Zuber- buhler, J.R. Morphology of double inlet ventricle. In: R.H. Anderson, G. Crupi, L. Parenzan, (Eds). Double Inlet Ventricle: Anatomy, Diagnosis and Surgical Man- agement. Tunbridge Wells: Kent Castle House Publica- tions; 1987: pp. 36-71.