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Tetralogy of Fallot with unilateral absent pulmonary artery

Published online by Cambridge University Press:  18 July 2012

Murat Ugurlucan ,
Ahmet H. Arslan ,
Yahya Yildiz ,
Sibel Ay ,
Resmiye T. Besikci  and
Sertac Cicek
Murat Ugurlucan
Affiliation:
Anadolu Medical Center Hospital, Cardiovascular Surgery Clinic, Istanbul, Turkey
Ahmet H. Arslan
Affiliation:
Anadolu Medical Center Hospital, Cardiovascular Surgery Clinic, Istanbul, Turkey
Yahya Yildiz
Affiliation:
Anadolu Medical Center Hospital, Cardiovascular Surgery Clinic, Istanbul, Turkey
Sibel Ay
Affiliation:
Anadolu Medical Center Hospital, Cardiovascular Surgery Clinic, Istanbul, Turkey
Resmiye T. Besikci
Affiliation:
Anadolu Medical Center Hospital, Cardiovascular Surgery Clinic, Istanbul, Turkey
Sertac Cicek*
Affiliation:
Anadolu Medical Center Hospital, Cardiovascular Surgery Clinic, Istanbul, Turkey
*
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
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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.

Keywords

Tetralogy of Fallotpulmonary arteryunilateralabsent
Type
Original Articles
Information
Cardiology in the Young , Volume 23 , Issue 3 , June 2013 , pp. 423 - 430
Copyright
Copyright © Cambridge University Press 2012 

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References

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