International Journal of Cardiology, 36 (1992) 361-363 0 1992 Elsevier Science Publishers B.V. All rights resewed

CARD10

0167-5273/92/$05.1X3

15211

Brief Reports

Discordance between abdominal and atria1 arrangement of complex congenital heart disease S. Radhakrishnan,

in a case

Mandeep Singh and R. Bajaj

Deppartment of Cardiology, Sanjay Gandhi Post Graduate Institute of Medical Sciences. Lucknow, India (Received

19 December

1991; revision

accepted

2 March

1992)

The report describes a rare case of a complex congenital heart disease wherein the splenic status and the relationship of the suprarenal abdominal great vessels strongly suggested right isomerism, but atrial morphology demonstrated usual atrial arrangement. It emphasises the increasing recognition that the arrangement of the abdominal organs need not accurately reflect atrial arrangement. Key words:

Abdominal

situs; Atrial situs; Asplenia

Introduction Determination of atrial arrangement is the basis to the diagnosis of congenital cardiac malformations. Many correlates exist to predict atrial arrangement noninvasively. These include splenic status, lung lobaLion, bronchial morphology [l] and the interrelationship of the suprarenal abdominal great vessels [2]. It is now recognized that splenic status is unreliable as a guide to atria1 arrangement [3]. We report an unusual case of a complex congenital heart disease with usual atrial arrangement where the abdominal arrangement, including relationship of the great vessels, suggested right isomerism. Case Report This 5-yr-old boy, presented with progressive cyanosis since birth. Cardiovascular examination revealed that the apex was on the left side. There was a

Corrqxmdence ro: S. Radhakrishnan. M.D., Dept. of Cardiology. SGPGIMS. P.O. Box 375. Raebareilly Road, Lucknow 226 001. India.

short ejection systolic murmur at the left parasternal area and a widely split second sound. Investigations revealed polycythemia; the peripheral blood film showed Howell Jolly bodies. The electrocardiogram showed right axis deviation, right ventricular hypertrophy, monomorphic QRS complexes from VZ V, and changing P wave axis from - 60 ’ to + 60 ’ in the frontal plane. A tomogram showed normal bronchial arrangement. Ultrasound examination of the abdomen revealed that the spleen, along with the splenic artery and vein, were absent and the liver was central. Short axis cuts of the suprarenal segment of the great vessels revealed that the inferior caval vein and the abdominal aorta were both to the right of the spine (Fig. 1A). The inferior caval vein was anterior to the abdominal aorta. In the long axis view, it was possible to visualize both great vessels in the same cut with the inferior caval vein opening into the right-sided atrium after receiving the hepatic veins (Fig. 1B). There were large interatrial communications across the oval fossa and the “ostium primurn”, a single atrioventricular valve committed to both ventricles, and a large posterior ventricular septal defect. Both great arteries orginated from the morphologically right ventricle with the aorta anterior and to the right.

362

Fig. 1A. Short axis scan of the suprarenal segment of abdominal great vessels. Both great vessels are seen on the right of the spine (SP) with the inferior caval vein (IV0 directly anterior to the aorta (AO).

Fig. IB. Long axis scan in the same patient right-sided

showing the inferior caval vein receiving the hepatic veins (HV) before atrium (RA). A0 = aorta: IVC = inferior caval vein.

Cardiac catheterization and angiography confirmed the cardiac anatomy. Separate injections into the atrial appendages showed the usual atria1 arrangement (Fig. 2A, B). Discussion .In the present case, right isomerism was strongly suspected when the peripheral blood smear showed

Fig. 2A. Direct

Fig. 2B. Direct

injection

injection

into the right-sided

opening

into the

Howell Jolly bodies and the abdominal ultrasound showed absence of the spleen, together with the splenic vein and artery. The diagnosis of right isomerism was further strengthened when asymmetry in the arrangement of suprarenal aorta and inferior caval vein was found. The study by Huhta et al. [2] showed that if the aorta and inferior caval vein run together at the level

atria1 appendage in the lateral view. The broad junction suggestive of morphologically right appendage.

with the body of the atrium

into the left-sided atrial appendage in the lateral view demonstrating the hooked narrow junction with the body of atrium typical of morphologically left appendage.

appearance

is

and its

3fl.3

of the tenth thoracic vertebra, then atrial isomerism is almost certainly present unless some other conditions, like examphalos, exist to disturb this arrangement. The splenic status has also been considered a specific marker for intracardiac abnormalities. Thus, absence of spleen has been equated with a constellation of findings suggestive of right isomerism, and multiple spleens considered a strong pointer to left isomerism. Recently these views have been challenged. In a necropsy study by Anderson et al. [3] it was shown that the splenic status did not reflect accurately the atrial morphology. In none of their cases, however, was complete absence of spleen noted without left or right isomerism. Our case is unusual in two respects. The first is the absence of the spleen in the presence of normal atrial arrangement. It is possible, however, that small splenules, which are not resolved by ultrasound, have been missed. The second is that arrangement of the great vessels in the abdomen was strongly suggestive of right isomerism. To the best of our knowledge, this feature has not been described before when there was usual atrial arrangement.

International

Journal

9 1992 Elsevier

of Curdioloa.

Science Publishers

We conclude that, although rare, discordance between great vessel arrangement in the abdomen and the atria1 arrangement in complex congenital heart disease can exist. Discordance in bronchial and atrial arrangement. is known to exist [3]. In such cases, the ultimate arbiter would be direct demonstration of atrial morphology by angiography or by transesophageal echocardiography [4]. References Van Mierop LHS. Eisen S, Schiebler CL. appearance

of the tracheobronchial

visceral situs. Am J Cardiol Huhta

JC.

dimensional

Jeffrey

The radiographic

tree as an indicator

to

1970:26:432-435.

F. Smallhorn

echocardiographic

JF, Mcartney FJ. Two

diagnosis of situs. Br Heart

J 1982;48:98-108. Anderson

C.

Devine

Zuberbuhler JR. congenital

malformations

findings in children. Stumper Diagnosis

WA,

OF.

Andserson

Abnormalities

RH. Debich DE,

of the spleen in relation

to

of the heart: a survey of necropsy

Br. Heart

Sreeram

N,

J 19YO;h3:127-178.

Elzenga

NJ,

of atrial situs by transesophageal

phy. J Am Coil Cardiol

Sutherland

CR.

echocardiogra-

1990:16:442-446.

36 ( 1992) 363-366 B.V.

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CARD10 15112

The syndrome of absent or faint second heart sound, rapid systole and forward collapsing pulse M.A. Nassim Department

of Clinical Pharmacology, (Received

Fisons pk., Loughborough.

16 January 1992; revision accepted 23 March

Leicestwshire,

UK

1992)

A syndrome consisting of an absent or faint second heart sound, prominent cardiac impulse and non-regurgitant or forward collapse of the pulse is described. There was, moreover, no second sound or flow murmur audible in either the aortic or the pulmonary area even though the impulse and collapsing pulse suggested a hyperdynamic circulation; the upstroke was sharp but without the full waterhammer knock. It is argued that the dominant cause of the collapsing pulse will usually be vasodilatation, that absence or faintness of the second sound is due to a narrow

Correspondence

LEll ORH. UK.

to: Dr. M.A. Nassim, Dept.

of Clinical

Pharmacology.

Fisons plc., Bakewell

Rd.. Loughborough.

Leicestershire