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J Thorac Cardiovasc Surg 1996;111:1109
© 1996 Mosby, Inc.

LETTERS TO THE EDITOR

Systemic and venous connection in asplenia syndrome

Pediatric Cardiology
Bambino Gesú Hospital
Piazza Sant'Onofrio, 4
00165 Rome, Italy

To the Editor:

We read with interest the stimulating and useful paper of Rubino and associates ¹ on systemic and venous connection in asplenia syndrome.

Even in our angiocardiographic series we ² noted the correlation between atresia of the pulmonary valve and obstruction of the pulmonary venous connection. Moreover, we ³ reported the prevalence of bilateral ductus arteriosus in this subgroup of patients.

The study of Rubino and colleagues ¹ demonstrates the great variability in the site and mode of connection of the pulmonary and systemic veins in this syndrome. To underline this concept, we would like to report the unique features observed in a patient from our angiographic series, ² which were confirmed at autopsy.

This patient had visceral heterotaxy with asplenia, levocardia, single atrium, complete atrioventricular canal with right ventricular dominance, L-ventricular loop, L-transposition of the great arteries, and pulmonary atresia. Absence of the inferior vena cava with azygos continuation into the unilateral right superior vena cava was noted. In addition, total anomalous pulmonary venous connection into the azygos vein was present, so that the entire systemic and pulmonary venous drainages (except the suprahepatic and coronary veins) reached the heart by the unilateral superior vena cava.

An accurate preoperative assessment of systemic and pulmonary connections is essential for the surgical treatment of patients with asplenia syndrome. Despite the progresses of echocardiography, we believe that angiocardiographic study is still mandatory in these children.

References

1. Rubino M, Van Praagh S, Kadoba K, Pessotto R, Van Praagh R. Systemic and pulmonary venous connections in visceral heterotaxy with asplenia. J Thorac Cardiovasc Surg 1995;110:641-50.[Abstract/Free Full Text]
   
2. Vairo U, Marino B, Parretti di Iulio D, et al. Isomerismo atirale destro con atresia polmonare: caratteristiche angiocardiografiche e modelli di circolazione polmonare. G Ital Cardiol 1991;21:669-74.[Medline]
   
3. Formigari R, Vairo U, de Zorzi A, Santoro G, Marino B. Prevalence of bilateral patent ductus arteriosus in patients with pulmonic valve atresia and asplenia syndrome. Am J Cardiol 1992;70:1219-20.[Medline]
   

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