120 international Journal of Cardiology, 21(1990) 120-121 Elsevier

CARD10 10422

Double orifice tricuspid valve with left atrioventricular valve atresia and univentricular connexion to a dominant left ventricle

Albert0 Cabrera, Javier Pilar and Esteban Pastor Division of Pediatric Cardiology, Children’s Hospital Cruces, Vizcaya. Spain

(Received 26 April 1989; revision accepted 1 November 1989)

We present a case of univentricular connexion to a dominant left ventricle with double orifice tricuspid valve, left atrioventricular valvar atresia, discordant ventriculo-arterial connexion and aortic arch hypoplasia. Diagnosis was made by cross-sectional echocardiography and angiocardiography. This case is exceedingly rare.

Key words: Congenital heart disease; Univentricular heart; Atrioventricular valvar atresia

Introduction

A double orifice of the tricuspid valve is an unusual cardiac anomaly, but can occur when the right atrium is

connected with either one or both ventricles [l]. In the latter variety, each orifice connects with a different ventricle, giving rise to the situation often termed “dou- ble outlet atrium” [l). The left atrioventricular valve

may be patent or atretic [2] and there can be univentri-

cular connexion to a dominant left ventricle [3]. We

have now encountered this anomaly in a patient in whom diagnosis was suspected by cross-sectional echo-

cardiography and angiocardiography and confirmed by

necropsy.

Case Report

The patient was a full-term newborn infant. Physical examination revealed mild perioral cyanosis and a hy-

Fig. 1. a. The dominant left ventricle. A band (B) divides the tricuspid valve into two separate orifices. The anterior right orifice is larger (white arrow). AR0 = anterior right orifice; PRO = posterior right orifice; * = ventricular septal defect. b. The posterior right

orifice (big white arrow). It presents multiple papillary muscles fused in the form of a parachute valve.

Correspondence to: A. Cabrera, Alameda Recalde 35B-2O Dcha, 48011 Bilbao, Spain.

0167-5273/90/$03.50 0 1990 Elsevier Science Publishers B.V. (Biomedical Division)

Fig. 2. Left atrium with atresia of the atrioventricular valve due to absent atrioventricular connexion ( * ). The atrium com- municates through an atria1 septal defect (ASD) with the right

atrium. LA = left atria1 appendage.

perdynamic heart. A systolic ejection murmur was heard

along the left sternal border. Femoral pulses were

stronger than axillary ones. The electrocardiogram dem- onstrated right atrial and left ventricular hypertrophy.

A chest X-ray showed moderate cardiomegaly and pulmonary congestion. Cross-sectional echocardiogra- phy from the subcostal four-chamber view showed that

the right atrium connected through a double orifice to a dominant left ventricle, the left atrioventricular valve being atretic. The aorta arose from an anterior rudimen- tary right ventricle while the pulmonary trunk came

from the dominant ventricle. Cardiac catheterization

revealed oxygen saturations of 50% in the superior caval vein, 69% in the right atrium, 99% in the left atrium,

85% in the ventricle and 86% in the pulmonary artery. The right atria1 pressure had a mean value of 8 mm Hg,

while the mean left atria1 pressure was 40 mm Hg. After a Rashkind procedure, the mean right atria1 pressure was 3 mm Hg and the mean left atria1 pressure was 6

mm Hg. Left ventricular angiocardiography confirmed the echocardiographic diagnosis. Surgery was performed when the infant was 3 days old. The hypoplactic aortic arch was enlarged with a bovine pericardial patch. the arterial duct was closed and the pulmonary trunk was

banded. The patient died at the age of 25 days following staphylococcal sepsis. Necropsy confirmed the presence of a dominant left ventricle (Fig. la) with an anterior rudimentary right ventricle and a discordant

ventriculo-arterial connexion. The right atrioventricular valve had two orifices. The posterior right orifice was smaller, and was formed by the inferior and septal

121

leaflets of the tricuspid valve (Fig. lb). The left atrio-

ventricular connexion was absent (Fig. 2).

Discussion

A double orifice tricuspid valve with atresia of the left atrioventricular valve is a very rare cardiac anomaly.

The first case was reported by Pisenti in 1890 I-121. It may occur as an isolated defect or in association with other cardiac malformations such as tetralogy of Fallot, Eb- stein’s malformation, coarctation of the aorta, complete

transposition or with double outlet right ventricle. De- pending on the location of the defect, three types of

double orifices are possible. It may be commissural when the accessory orifice is at the end of a valvar

commissure and its subvalvar apparatus is the normal one for that commissure. It may be central (when a fibrous band divides the tricuspid valve into either equal or unequal parts). It may take the form of a fenestration when the accessory orifice is a hole in one of the leaflets. It is essential for the identification of

double orifice within the tricuspid valve that both orifices should be provided with a subvalvar tension

apparatus.

The present case is an example of double orifice tricuspid valve in the setting of univentricular connex-

ion to a dominant left ventricle. The atrial septum has a normal structure and orientation, in contrast to classic cases of “double outlet right atrium and straddling of the tricuspid valve” [5]. The left atrium shows absence of the left atrioventricular connexion.

References

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