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J Thorac Cardiovasc Surg 1994;107:1378-1379
© 1994 Mosby, Inc.

LETTERS TO THE EDITOR

Pulmonary arteriovenous fistulas and polysplenia syndrome

Department of Pediatric Cardiology and
Cardiac Surgery
Ospedale Pediatrico Bambino Gesu
Rome, Italy

To the Editor:

In our experience since 1982 there have been only two patients with a combination of congenital heart disease and pulmonary arteriovenous (AV) fistulas. Both cases also involved left atrial isomerism with azygos continuation (polysplenia syndrome).

In the first patient—a female infant with polysplenia syndrome, total anomalous pulmonary venous connection to the coronary sinus, and partial AV septal defect with moderate mitral valve incompetence—diffuse bilateral pulmonary AV fistulas were angiographically detected when the patient was 2 months of age. The patient was not considered a candidate for surgical correction because it was feared that separation of the two circulations could critically reduce the pulmonary/systemic blood flow ratio. The patient was discharged with medical therapy and died at home 3 months later.

The second patient had polysplenia syndrome, ventricular L-loop, ventricular septal defect, mirror-image alignment of the great arteries, subpulmonary stenosis, and partial anomalous pulmonary venous connection of the right lung to the right atrium. At 3 months of age, the patient underwent a Blalock-Taussig shunt and subsequently a cavopulmonary connection (Kawashima operation) combined with an atrial septectomy. During the next 14 months, the patient had recurrence of progressive cyanosis, caused by acquired diffuse pulmonary AV fistulas of the left lung. The patient eventually died after various surgical procedures, including a left pneumonectomy. Histologic evaluation of the left lung showed diffuse peribronchial pulmonary AV fistulas at the level of the small muscular arteries.

Our review of the literature showed that the association of pulmonary AV fistula with polysplenia syndrome was first reported by Feigl and associates ¹ in 1989. Pulmonary AV fistulas have also been described as a late complication of a classic Glenn shunt, with a rate of between 19% and 25% of the cases. Furthermore, the only two patients reported with early occurrence of pulmonary AV fistulas after classic Glenn shunt (at 2 and 4 years) had azygos continuation of the inferior vena cava and therefore presumably had polysplenia syndrome. ² Interestingly, both patients had normal upper/lower lobe perfusion ratio differing from the usual late pattern of reduced upper/lower lobe perfusion ratio seen after a Glenn shunt. Finally, the only two cases of post-Fontan pulmonary AV fistula that we found in the literature also occurred in patients with polysplenia syndrome who apparently underwent a Kawashima modification of the Fontan operation. ³ We believe that the association between polysplenia syndrome and pulmonary AV fistulas is more than occasional. This idea is shared by Kawashima (Kawashima Y, personal communication), who also observed the early occurrence of pulmonary AV fistulas in his series of patients with azygos continuation who underwent cavopulmonary anastomosis.

We speculate that polysplenia syndrome with azygos continuation is a predisposing factor for the development of pulmonary AV fistula. Cavopulmonary anastomoses or Fontan operation could accelerate this pulmonic complication.

References

1. Feigl A, Feigl D, Tamir A, Berant M, Blieden L. Multiple pulmonary arterio-venous fistulae, atrial septal defect, interrupted inferior vena cava in the polysplenia syndrome [Abstract]. In: Abstracts of the Third World Congress of Pediatric Cardiology. Bangkok: 1989:33.
   
2. Cloutier A, Ash JM, Smallhorn JF, et al. Abnormal distribution of pulmonary blood flow after the Glenn shunt or Fontan procedure: risk of development of pulmonary arteriovenous fistulae. Circulation 1985;72:471-9.[Abstract/Free Full Text]
   
3. Moore JW, Kirby WC, Madden WA, Gaiter NS. Development of pulmonary arteriovenous malformation after modified Fontan operation. J THORAC CARDIOVASC SURG 1989;98:1045-50.[Abstract]
   

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This Article Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Roberto Di Donato Adriano Carotti Carlo Marcelletti Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Amodeo, A. Articles by Marcelletti, C. Search for Related Content PubMed PubMed Citation Articles by Amodeo, A. Articles by Marcelletti, C.