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Asian Cardiovasc Thorac Ann 2002;10:270-272
© 2002 Asia Publishing EXchange Pte Ltd

CASE STUDY

Correction of Absent Pulmonary Valve Syndrome Using a Pericardial Valved Conduit

Sukasom Attanawanich, MD, Pongsak Khowsathit, MD1, Wichaya Withurawanit, MD

Cardiovascular Thoracic Unit Department of Surgery
1 Pediatric Cardiology Unit Department of Pediatrics Faculty of Medicine Ramathibodi Hospital, Mahidol University Bangkok, Thailand
For reprint information contact: Sukasom Attanawanich, MD Tel: 66 2 201 1315 Fax: 66 2 201 1316 Cardiovascular Thoracic Unit, Department of Surgery, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Rama VI Road, Bangkok 10400, Thailand.

   ABSTRACT
TOP
 ABSTRACT
INTRODUCTION
 CASE REPORT
 DISCUSSION
 REFERENCES
 
Absent pulmonary valve syndrome in a 4-month-old infant was successfully corrected using a fresh autologous pericardial trileaflet valved conduit. He recovered from operation with only mild pulmonary regurgitation at 4 months postoperatively. This technique is an effective alternative for infants with congenital heart disease who need tissue valved conduits. It may be more suitable than the aortic homograft by reason of the shortage of small homografts and its lower costs.


   INTRODUCTION
TOP
 ABSTRACT
 INTRODUCTION
CASE REPORT
 DISCUSSION
 REFERENCES
 
Absent pulmonary valve (PV) syndrome is a relatively rare condition, accounting for about 3% of cases of tetralogy of Fallot in autopsy series and clinical reviews.1 The central pulmonary arteries (PAs) are usually hugely dilated or aneurysmal, and 93% of patients show the intracardiac characteristics of tetralogy of Fallot.2 The intracardiac repair of the defect carries a high risk in infants.3,4 The clinical manifestations may be pulmonary, cardiac, or often both, such as tachypnea, bronchospasm, air trapping, cyanosis, or respiratory failure.5 Surgical treatment is needed to alleviate and prevent bronchial compression and right-sided heart failure. The optimal surgical treatment is the insertion of a homograft with repair of the intracardiac anatomy.6–8 The lack of small homografts limits transplantation in infants. The high cost of commercial homografts is another major reason for not using homografts. Our group has developed an alternative method using a fresh autologous pericardial valved conduit. We describe the successful use of such a valved conduit to reconstruct the defective PV in a 4-month-old infant.


   CASE REPORT
TOP
 ABSTRACT
 INTRODUCTION
 CASE REPORT
DISCUSSION
 REFERENCES
 
A 4-month-old boy presented with clinical manifestations of severe pulmonary regurgitation (severe respiratory distress and cyanosis). The chest radiograph showed increased pulmonary vascularity and a boot-shaped heart. The electrocardiogram showed right-axis deviation and right ventricular hypertrophy. The echocardiogram revealed a large subpulmonary ventricular septal defect (VSD), a hypoplastic PV ring with complete insufficiency of the PV, and markedly enlarged main PA and branches (Figure 1Go). He was diagnosed with tetralogy of Fallot with absent PV syndrome.



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  		Figure 1. Preoperative echocardiogram of a 4-month-old infant with tetralogy of Fallot and absent pulmonary valve.

 
The patient underwent complete repair of the defects and a pericardial valve replacement. During operation, the PV showed hypoplasia with atresia of all leaflets. The PV annulus and the PA and its right and left branches measured 3, 12, 20, and 15 mm, respectively. The subpulmonary VSD was closed. The main PA was transected at 2 mm above the pulmonary annulus. The anterior walls of the right and left PAs were partially removed to reduce their size. The fresh autologous pericardial valve was constructed based on a metallic trileaflet valved conduit template of 12-mm diameter (Figure 2Go). The pericardium was prepared from the right phrenic to the left phrenic nerve. It was then wrapped around the template and fastened with 6/0 polypropylene sutures. The main PA, the PV, and the right ventricular outflow tract were reconstructed with the pericardial valved conduit and a small patch of pericardium (Figure 3Go). The patient was weaned off cardiopulmonary bypass with 5 µgákg-1ámin-1 of dopamine.



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  		Figure 2. The fresh autologous pericardial valved conduit was constructed based on a metallic trileaflet valved conduit template of 12-mm diameter.

 


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  		Figure 3. Insertion of the autologous pericardial valved conduit and reconstruction of the right ventricular outflow tract using a small patch of pericardium.

 
Postoperatively, the patient’s hemodynamics were satisfactory, but he showed signs of respiratory insufficiency. Evaluation of the airway by fiberoptic bronchoscopy revealed tracheomalacia, left broncho-malacia, and a tracheogenic bronchus of the right upper lobe. He was put on nasal continuous positive airway pressure for 2 weeks and extubated on postoperative day 21. He was discharged on postoperative day 30.

Postoperative echocardiograms at 4-month follow-up showed the VSD patch at the outlet septum without residual VSD. The right ventricular outflow tract was not narrowed. The PV ring was 8 mm in diameter. There was mild pulmonary regurgitation with a peak regurgitation pressure gradient of 4 mm Hg. There was also mild pulmonary stenosis. Both the right and left PAs were 6 mm in diameter. The left ventricular systolic function was good with a left ventricular ejection fraction of 63%.


   DISCUSSION
TOP
 ABSTRACT
 INTRODUCTION
 CASE REPORT
 DISCUSSION
REFERENCES
 
Replacement with the fresh autologous pericardial valved conduit is feasible, inexpensive, and more practical than using other options such as the aortic valve homograft, Dacron porcine valved conduit, and the valveless autologous tissue conduit. By using templates of varying sizes, a conduit of the appropriate size can be chosen. There was only mild pulmonary regurgitation post-operatively and at short-term follow-up. We believe our technique is an effective alternative for correcting absent PV syndrome in infants.


   Acknowledgments
 
The authors thank Dr. Paitoon Gojaseni for reviewing the manuscript.


   REFERENCES
TOP
 ABSTRACT
 INTRODUCTION
 CASE REPORT
 DISCUSSION
 REFERENCES
 

  1. Lev M, Eckner FAD. The pathologic anatomy of tetralogy of Fallot. Dis Chest 1964;45:251–61.

  2. Calder AL, Brandt PW, Barratt-Boyes BG, Neutze JM. Variant of tetralogy of Fallot with absent pulmonary valve leaflets and origin of one pulmonary artery from the ascending aorta. Am J Cardiol 1980;46:106–16.[Medline]

  3. Ilbawi MN, Fedorchik J, Muster AJ, Idriss FS, DeLeon SY, Gidding SS, et al. Surgical approach to severely symptomatic newborn infants with tetralogy of Fallot and absent pulmonary valve. J Thorac Cardiovasc Surg 1986; 91:584–9.[Abstract]

  4. Stellin G, Jonas RA, Goh TH, Brawn WJ, Venables AW, Mee RB. Surgical treatment of absent pulmonary valve syndrome in infants: relief of bronchial obstruction. Ann Thorac Surg 1983;36:468–75.[Abstract]

  5. Pinsky WW, Nihill MR, Mullins CE, Harrison G, McNamara DG. The absent pulmonary valve syndrome. Considerations of management. Circulation 1978;57: 159–62.[Abstract/Free Full Text]

  6. Snir E, de Leval MR, Elliott MJ, Stark J. Current surgical technique to repair Fallot’s tetralogy with absent pulmonary valve syndrome. Ann Thorac Surg 1991;51:979–82.[Abstract]

  7. Laks H, Hellenbrand WE, Kleinman CS, Stansel HC Jr, Talner NS. Patch reconstruction of the right ventricular outflow tract with pulmonary valve insertion. Circulation 1981;64(Pt 2):154–61.

  8. Mavroudis C, Turley K, Stanger P, Ebert PA. Surgical management of tetralogy of Fallot with absent pulmonary valve. J Cardiovasc Surg (Torino) 1983;24:603–9.[Medline]





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