Ann Thorac Surg 2001;71:1686-1688
© 2001 The Society of Thoracic Surgeons
Case report
Transposition with absent pulmonary valve syndrome: early repair of a rare case
Guido Oppido, MDb,
Adriano Carotti, MDb,
Sonia B. Albanese, MDb,
Antonio Amodeo, MDb,
Luciano Pasquini, MDa,
Roberto M. Di Donato, MDb
a Department of Pediatric Cardiology, Bambino Gesù Hospital, Rome, Italy
b Department of Cardiac Surgery, Bambino Gesù Hospital, Rome, Italy
Accepted for publication July 10, 2000.
Address reprint requests to Dr Di Donato, Department of Pediatric Cardiology, Ospedale Bambino Gesù, Piazza S. Onofrio 4, 00165 Rome, Italy
e-mail: didonato{at}opbg.net
 |
Abstract
|
|---|
The congenital absence of the pulmonary valve cusps can occur either isolated or in association with other heart lesions. We report a very rare case of a 40-day-old infant with transposition of the great arteries, ventricular septal defect, pulmonary annular stenosis, absent pulmonary valve and aneurysmal dilation of the central pulmonary arteries, who received surgical treatment at our institution.
|
Introduction
|
|---|
Since Chevers [1] first described the congenital absence of the pulmonary valve cusps, many cases have been reported either isolated or associated with other heart lesions. Absent pulmonary valve is relatively common in tetralogy of Fallot, but it has been described also in association with patent ductus arteriosus, atrial septal defect, atrioventricular canal defect, double-outlet right ventricle or interrupted aortic arch. Its association with transposition of the great arteries (TGA) is very rare and was described only in two anatomical cases [2, 3].
Herein, we report the case of a 40-day-old infant with S,D,A, transposition of the great arteries (TGA), ventricular septal defect (VSD), pulmonary annular stenosis, absent pulmonary valve and aneurysmal dilation of the main pulmonary artery and its branches, who received successful surgical treatment at our institution.
The patient, a 3.4 kg male born after a full-term uncomplicated pregnancy, presented at birth with mild cyanosis and a 2/6 systolic heart murmur. Diagnosis was made by 2D-Echo showing TGA with anterior aorta, unrestrictive subpulmonary VSD, patent foramen ovale with left to right shunt, absence of pulmonary valve cusps causing severe pulmonary regurgitation, pulmonary annular stenosis and a huge dilation of the central pulmonary arteries with a 25 to 30 mm main pulmonary artery diameter. At cardiac catheterization, the diagnosis was confirmed (Fig 1A) and a 50 mm Hg gradient across the pulmonary annulus was measured. Within the first month of life, the child developed heart failure and progressive respiratory insufficiency with radiologic evidence of bilateral pulmonary emphysema.
View larger version (86K):
[in this window]
[in a new window]
|
Fig 1. (A) Preoperative. Right ventricular angiogram showing the aneurysmal pulmonary arterial dilation. (B) Postoperative. Right ventricular angiogram showing homogeneously normal-sized pulmonary arteries.
|
|
Through a midline sternotomy, moderately hypothermic cardiopulmonary bypass (at the initial temperature of 30°C) was instituted using aortic and bicaval cannulations (Fig 2A). Thorough bilateral dissection of the pulmonary arteries was carried out and their peripheral branches were surrounded and snared. The main pulmonary artery was transected and its proximal stump was oversewn. The free edge of the redundant pulmonary confluence was generously trimmed. Working through the pulmonary confluence, a large portion of the posterior wall was excised by a lozenge-shaped incision (Fig 2B) and its continuity was reestablished with a running absorbable suture. The anterior wall was also reduced by bilateral wedge resections extended into the largest distal pulmonary artery branches (Fig 2C). The following suture-reconstruction downsized the branch pulmonary arteries to a diameter of 7 to 8 mm and the pulmonary confluence to a diameter of 10 to 12 mm (Fig 2D). During further cooling to 24°C, a 10 mm porcine pulmonary valved conduit (Sheligh Inc, Millburn, NJ) was distally anastomosed to the pulmonary confluence. The aorta was then cross-clamped and blood cardioplegic solution was administered. A longitudinal right ventricular infundibulotomy was performed and the left ventricle was baffled to the aorta through the VSD using an autologous glutaraldehyde-treated pericardial patch. After the aortic cross-clamp was released during rewarming, the xenograft was proximally anastomosed to the infundibulotomy with an autologous pericardial hood augmentation (Fig 2E). Cardiopulmonary bypass was discontinued without difficulty.
View larger version (44K):
[in this window]
[in a new window]
|
Fig 2. (A) Cardiopulmonary bypass instituted by aortic and bicaval cannulation. (B) Lozenge-shaped excision of a large portion of the posterior wall of redundant pulmonary arteries. (C) Posterior pulmonary arterial wall continuity reestablished with a running absorbable suture; anterior wall reduction by bilateral wedge excision. (D) Anterior wall suture-reconstruction of pulmonary arteries. (E) Final result.
|
|
Postoperative outcome was substantially uneventful. Two-dimensional echocardiography demonstrated an estimated right ventricular pressure of 38 mm Hg, absent residual shunt at ventricular level and unobstructed right and left ventricular pathways. The patient was extubated after a 14-day course of assisted ventilation due to some residual pulmonary air-trapping and transferred to the ward on the 30th postoperative day, because of a slowly healing chest wound.
During his hospital stay the signs of airway obstruction progressively disappeared and the child was discharged completely free of symptoms 40 days postoperatively. Thereafter, the child has been asymptomatic and thriving well. Nineteen months postoperatively, he underwent cardiac catheterization because of increased right ventricular pressure due to conduit overgrowth. Pulmonary angiography showed homogeneously normal-sized pulmonary arteries (Fig 1B). One month later, the child underwent uneventful xenograft replacement by a 17 mm pulmonary homograft valved conduit.
|
Comment
|
|---|
To our knowledge, this one-month-old infant represents a unique case of TGA/VSD with absent pulmonary valve undergoing successful complete repair. Like the more common variant of absent pulmonary valve in association with tetralogy of Fallot, this unusual combination also presents the characteristic features of pulmonary annular hypoplasia, and aneurysmal dilation of central pulmonary arteries. We can speculate that the greater degree of pulmonary overflow in TGA/VSD might promote an even greater dilation of the aneurysmal pulmonary arteries compared to tetralogy of Fallot. The compression of the tracheobronchial tree secondary to pulmonary arterial dilation characterizes the early clinical presentation of the syndrome. Surgical relief of airway compression has been reported to play a fundamental role in the outcome of infants with tetralogy of Fallot and absent pulmonary valve [4, 5]. We had a similar experience with this rare case of TGA/VSD associated with absent pulmonary valve. In fact, extensive resection of redundant pulmonary arterial wall both in its posterior and in its anterior aspect proved an optimal expedient to achieve the necessary airway relief with secondary long-lasting clinical beneficial effects.
|
Acknowledgments
|
|---|
We thank Dr Emilio Ferretti for his skillful artwork.
|
References
|
|---|
-
Chevers N. Recherches sur les maladies de l’arteire pulmonaire. Arch Gen Med 1847;15:488-508.
-
Ovseyevitz J., Attie F., Buendia A. Congenital absence of the pulmonary valve leaflets associated with complete transposition of the great arteries. Int J Cardiol 1982;1:263-271.[Medline]
-
Rabinovitch M., Grady S., David I., Van Praagh R., et al. Compression of intrapulmonary bronchi by abnormally branching pulmonary arteries associated with absent pulmonary valves. Am J Cardiol 1982;50:804-813.[Medline]
-
Stellin G., Jonas R.A., Goh T.H., Brawn W.J., et al. Surgical treatment of absent pulmonary valve syndrome in infants: relief of bronchial obstruction. Ann Thorac Surg 1983;36:468-475.[Abstract]
-
Godart F., Houyel L., Lacour-Gayet F., Serraf A., et al. Absent pulmonary valve syndrome: surgical treatment and considerations. Ann Thorac Surg 1996;62:136-142.[Abstract/Free Full Text]
This article has been cited by other articles:
|
|
|
|
 
M. A. Norgaard, N. Alphonso, A. E. Newcomb, C. P. Brizard, and A. D. Cochrane
Absent pulmonary valve syndrome. Surgical and clinical outcome with long-term follow-up.
Eur. J. Cardiothorac. Surg.,
May 1, 2006;
29(5):
682 - 687.
[Abstract]
[Full Text]
[PDF]
|
|
|
Top
Abstract
Introduction
