Skip to main content Accessibility help
×
Home

Tetralogy of Fallot with unilateral absent pulmonary artery

  • Murat Ugurlucan (a1), Ahmet H. Arslan (a1), Yahya Yildiz (a1), Sibel Ay (a1), Resmiye T. Besikci (a1) and Sertac Cicek (a1)...

Abstract

Background

Tetralogy of Fallot is a common congenital cardiac malformation. A rare subgroup includes unilateral absence of the pulmonary artery, either the left or the right main branch. The literature lacks an established treatment for these cases, and surgical options carry certain mortality and morbidity.

Patients and methods

There were five patients who had single pulmonary artery and received surgical treatment among the 126 patients with the diagnosis of Tetralogy of Fallot, who were admitted to our institution between July, 2010 and November, 2011. All the patients were male. Ages ranged between 12 months and 8 years. The mean body mass index was 17.1 plus or minus 3.4 kilograms per square metre. Pulmonary artery Nakata index, Nakata index Z-score, and the McGoon index were used for the quantitative assessment of the pulmonary artery and to determine the surgical strategy.

Results

Urgent modified Blalock–Taussig shunt operations were performed in two patients with very low oxygen saturation and haemodynamic instability. These patients are scheduled for corrective procedures on an elective basis. There was one patient who received an elective shunt procedure; however, the post-operative course was complicated with the overflow phenomenon and the patient underwent total correction with a check-valved patch used to close the ventricular septal defect. The patient required extracorporeal membrane oxygenator support in the post-operative period. There were two patients who underwent total correction of the pathology uneventfully. Mortality did not occur. Mean durations of hospital stay and follow-up were 14 plus or minus 13.4 days and 184.5 plus or minus 89.3 days, respectively.

Conclusion

Our modest series with Tetralogy of Fallot with unilateral absent pulmonary artery indicates the feasibility of surgical correction in patients with appropriate unilateral pulmonary artery size and palliative procedures when the pulmonary artery size is smaller than that predicted for the age. Multi-centre long-term data of larger series are warranted in order to establish a treatment protocol.

Copyright

Corresponding author

Correspondence to: Dr S. Cicek, MD, Prof., FACC, FCCP, Anadolu Medical Center Hospital, Cardiovascular Surgery Clinic, Cumhuriyet Mahallesi, 2255 Sokak, No. 3, Gebze 41400, Kocaeli, Istanbul, Turkey. Tel: +90 262 678 50 00; Fax: +90 654 05 38; E-mail: sertac.cicek@anadolusaglik.org

References

Hide All
1. Kucera, V, Fiser, S, Tuma, S, Hucin, B. Unilateral absence of pulmonary artery: a report on 19 selected clinical cases. Thorac Cardiovasc Surg 1982; 30: 152158.
2. Ten Harkel, AD, Blom, NA, Ottenkamp, J. Isolated unilateral absence of a pulmonary artery: a case report and review of the literature. Chest 2002; 122: 14711477.
3. Halit, V, Olguntürk, R, Erer, D, Kula, S. Tetralogy of Fallot and absence of left pulmonary artery. Thorac Cardiovasc Surg 2008; 56: 430432.
4. Zhang, GC, Wang, ZW, Zhang, RF, Zhu, HY, Yi, DH. Surgical repair of patients with tetralogy of Fallot and unilateral absence of pulmonary artery. Ann Thorac Surg 1997; 64: 11501153.
5. Bockeria, LA, Podzolkov, VP, Makhachev, OA, et al. Surgical correction of tetralogy of Fallot with unilateral absence of pulmonary artery. Ann Thorac Surg 2007; 83: 613618.
6. Büchler, JR, Jatene, AD, de Andrade, J. Congenital unilateral absence of the right pulmonary artery and complex of Fallot. A rare association. Jpn Heart J 1986; 27: 885892.
7. Emanuel, RW, Pattinson, JN. Absence of the left pulmonary artery in Fallot's tetralogy. Br Heart J 1956; 18: 289295.
8. Cucci, CE, Doyle, EF, Lewis, EW. Absence of a primary division of the pulmonary trunk: an ontogenetic theory. Circulation 1964; 29: 124131.
9. Presbitero, P, Bull, C, Haworth, SG, et al. Absence of occult pulmonary artery. Br Heart J 1984; 52: 178185.
10. Goldsmith, M, Farina, MA, Shaher, RM. Tetralogy of Fallot with atresia of the left pulmonary artery: surgical repair using a homograft aortic valve. J Thorac Cardiovasc Surg 1975; 69: 458466.

Keywords

Type Description Title
VIDEO
Movies

Ugurlucan et al. supplementary movie
Movie

 Video (1.2 MB)
1.2 MB
VIDEO
Movies

Ugurlucan et al. supplementary movie
Movie

 Video (958 KB)
958 KB
VIDEO
Movies

Ugurlucan et al. supplementary movie
Movie

 Video (675 KB)
675 KB

Tetralogy of Fallot with unilateral absent pulmonary artery

  • Murat Ugurlucan (a1), Ahmet H. Arslan (a1), Yahya Yildiz (a1), Sibel Ay (a1), Resmiye T. Besikci (a1) and Sertac Cicek (a1)...

Metrics

Full text views

Total number of HTML views: 0
Total number of PDF views: 0 *
Loading metrics...

Abstract views

Total abstract views: 0 *
Loading metrics...

* Views captured on Cambridge Core between <date>. This data will be updated every 24 hours.

Usage data cannot currently be displayed