Single-Stage Complete Repair versus Multistage Repair of Tetralogy of Fallot with Borderline Pulmonary Arteries
Background: Tetralogy of Fallot is the most common cyanotic congenital heart defect. Borderline pulmonary anatomy has been associated with a higher risk of mortality and morbidity. Strategies to manage this condition—namely, single- or multistage repair—have long been debated.
Objective: The overall outcomes of patients with tetralogy of Fallot with borderline pulmonary arteries (McGoon ratio 1.3 to 1.7) with regard to the need for a single-stage or multistage repair and the outcome of each surgical management were evaluated.
Patients and methods: A retrospective, nonrandomized comparative study designed to evaluate patient outcomes comprised 60 patients with tetralogy of Fallot with borderline pulmonary arteries who underwent surgery at the Cardiothoracic Surgery Academy, Ain Shams University, Cairo, Egypt, between January 2016 and December 2017. After gaining approval from the affiliated ethical and research committee, and informed consent of the guardians, the patients were assigned into one of two groups. Shunt group included 30 patients managed surgically by a modified Blalock-Taussig (MBT) shunt as a part of a multistage repair, and repair group included 30 patients managed surgically by single-stage complete repair. The medical records of the patients were reviewed, and data relating to age, sex, weight, and preoperative oxygen saturation were collected. All patients underwent preoperative echocardiography and multislice computed tomography (CT) with angiography. The follow-up was performed by echocardiography at discharge and at one month and six months after surgery. Multislice CT with angiography was performed in patients who received a shunt once the echocardiography showed acceptable pulmonary arteries.
Results: The patients’ age ranged from 5 to 50 months with a mean age of 18.63 ± 9.15 (19.84 ± 12.34 for the shunt group and 17.43 ± 8.54 for the repair group). The weight ranged from 5 kg to 18 kg with a mean of 9.6 ± 2.53 (8.82 ± 2.79 for the shunt group and 10.41 ± 2.63 for the repair group). The mean preoperative O2 saturation was 68.95% ± 7.8% for the shunt group and 87.93% ± 6.18% for the repair group. The median McGoon ratio was 1.4 for the shunt group and 1.6 for the repair group, the difference of which was highly significant (P < .0001). The mortality rate in our study was 10% (10% for the shunt group and 10% for the repair group). The morbidity incidence rate was 26.6% for the shunt and repair groups. The ICU stay ranged from 2 to 31 days, with a median of three days for the shunt group (mean 3.61 ± 1.91) and four days for the repair group (mean 6.07 ± 6.63 days). The calculated P value showed a significant difference between the two groups concerning ICU stay. The postoperative SO2 significantly increased to a mean of 85.58 ± 7.05 in the shunt group and 98.14 ± 3.36 in the repair group (P < .0001).
Conclusion: There was no statistically significant difference between multistage repair and single-stage complete repair regarding morbidity and mortality. Regarding ICU stay, patients in the single-stage had a better outcome. A McGoon ratio of 1.5 can be used as a guideline in the decision-making process.
Allam A, Hashem A. 2014. Fate of right ventricle outflow gradient after fallot repair. J Egyptian Soc Cardiorthorac Surg 22:53-8.
Anagnostopoulos P, Azakie A, Natarajan S, Alphonso N, Brook MM, Karl TR. 2007. Pulmonary valve cusp augmentation with autologous pericardium may improve early outcome for tetralogy of Fallot. J Thorac Cardiovasc Surg 133:640-7.
Anderson RH, Path MRC, Allwork SP, Ho SY, Lenox CC, Zuberbuhler JR. 1981. Surgical anatomy of tetralogy of fallot. J Thorac Cardiovasc Surg 81:887-96.
Chen B-B, Chen S-J, Wu M-H, Li YW, Lue HC. 2007. EBCT - McGoon ratio. A reliable and useful method to predict pulmonary blood flow non-invasively. 32:1-8.
Dyamenahalli U, McCrindle BW, Barker GA, Williams WG, Freedom RM, Bohn DJ. 2000. Influence of perioperative factors on outcomes in children younger than 18 months after repair of tetralogy of Fallot. Ann Thorac Surg 69:1236-42.
Ferencz C, Rubin JD, McCarter RJ, et al. 1985. Congenital heart-disease: prevalence at livebirth. The Baltimore Washington Infant Study. Am J Epidemiol 121:31-6.
Frigiola A, Redington AN, Cullen S, Vogel M. 2004. Pulmonary regurgitation is an important determinant of right ventricular contractile dysfunction in patients with surgically repaired tetralogy of Fallot. Circulation 110: II153-7.
Gladman G, McCrindle BW, Williams WG, Freedom RM, Benson LN. 1997. The modified Blalock-Taussig shunt: Clinical impact and morbidity in Fallot’s tetralogy in the current era. J Thorac Cardiovasc Surg 114:25-30.
Hirsch JC, Mosca RS, Bove EL. 2000. Complete repair of tetralogy of Fallot in the neonate. Results in the modern era. Ann Surg 232:508-14.
Horneffer PJ, Zahka KG, Rowe SA, et al. 1990. Long-term results of total repair of tetralogy of fallot in childhood. Ann Thorac Surg 50:179-85.
Ishikawa S, Takahashi T, Sato Y, et al. 2001. Growth of the pulmonary arteries after systemic-pulmonary shunt. Ann Thorac Cardiovasc Surg 7:337-40.
Jahangiri M, Lincoln C, Shinebourne EA. 1999. Does the modified Blalock-Taussig shunt cause growth of the contralateral pulmonary artery? Ann Thorac Surg 67:1397-9.
Kanter KR, Kogon BE, Kirshbom PM, Carlock PR. 2010. Symptomatic neonatal tetralogy of Fallot: repair or shunt? Ann Thorac Surg 89:858-63.
Karl TR, Sano S, Pornviliwan S, Mee RBB. 1992. Tetralogy of fallot: favorable outcome of nonneonatal transatrial, transpulmonary repair. Ann Thorac Surg 54:903-7.
Laas J, Engeser U, Meisner H, et al. 1984. Tetralogy of fallot: development of hypoplastic pulmonary-arteries after palliation. Thorac Cardiovasc Surg 32:133-8.
Lillehei CW, Cohen M, Warden HE, et al. 1955. Direct vision intracardiac surgical correction of the tetralogy of fallot, pentalogy of fallot, and pulmonary atresia defects: report of 1st 10 cases. Ann Surg 142:418-45.
Maghur HA, Ben-Musa AA, Salim ME, Abuzakhar SS. 2002.The modified Blalock-Taussig shunt: A 6-year experience from a developing country. Pediatr Cardiol 23:49-52.
Mitchell SC, Korones SB, Berendes HW. 1971. Congenital heart disease in 56,109 births: incidence and natural history. Circulation 43:323-32.
Morales DL, Zafar F, Fraser Jr CD. 2009. Tetralogy of Fallot repair: the right ventricle infundibulum sparing (RVIS) strategy. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 54-8.
Neill CA, Clark EB. 1994. Tetralogy of fallot: the 1st 300 years. Texas Hear Inst J 21:272-9.
Stewart RD, Mavroudis C, Backer CL. 2013. Pediatric Cardiac Surgery, Fourth Edition. Eds: Mavroudis C and Backer CL. Blackwell Publishing Ltd. p. 415.
Ross ET, Costello JM, Backer CL, Brown LM, Robinson JD. 2015. Right ventricular outflow tract growth in infants with palliated tetralogy of fallot. Ann Thorac Surg 99:1367-72.
Sabri MR, Sholler G, Hawker R, Nunn G. 1999. Branch pulmonary artery growth after Blalock-Taussig shunts in tetralogy of Fallot and pulmonary atresia with ventricular septal defect: A retrospective, echocardiographic study. Pediatr Cardiol 20:358-63.
Sasikumar D, Sasidharan B, Tharakan JA, Dharan BS, Mathew T, Karunakaran J. 2014. Early and 1-year outcome and predictors of adverse outcome following monocusp pulmonary valve reconstruction for patients with tetralogy of Fallot: A prospective observational study. Ann Pediatr Cardiol 7:5-12.
Saygi M, Ergul Y, Tola HT, et al. 2015. Factors affecting perioperative mortality in tetralogy of Fallot. Pediatr Int 57:832-9.
Singh SP, Chauhan S, Choudhury M, et al. 2014. Modified Blalock Taussig shunt: Comparison between neonates, infants, and older children. Ann Card Anaesth 17:191-7.
Stewart RD, Backer CL, Young L, Mavroudis C. 2005. Tetralogy of Fallot: Results of a pulmonary valve-sparing strategy. Ann Thorac Surg 80:1431-9.
Taussig HB. 1979. Tetralogy of fallot: early history and late results. Am J Roentgenol 133:423-31.
Wilder TJ, Van Arsdell GS, Pham-Hung E, et al. 2016. Aggressive patch augmentation may reduce growth potential of hypoplastic branch pulmonary arteries after tetralogy of Fallot repair. Ann Thorac Surg 101:996-1004.
Author Disclosure & Copyright Transfer Agreement
In order to publish the original work of another person(s), The Heart Surgery Forum® must receive an acknowledgment of the Author Agreement and Copyright Transfer Statement transferring to Forum Multimedia Publishing, L.L.C., a subsidiary of Carden Jennings Publishing Co., Ltd. the exclusive rights to print and distribute the author(s) work in all media forms. Failure to check Copyright Transfer agreement box below will delay publication of the manuscript.
A current form follows:
The author(s) hereby transfer(s), assign(s), or otherwise convey(s) all copyright ownership of the manuscript submitted to Forum Multimedia Publishing, LLC (Publisher). The copyright transfer covers the exclusive rights to reproduce and distribute the article and the material contained therein throughout the world in all languages and in all media of expression now known or later developed, including but not limited to reprints, photographic reproduction, microfilm, electronic data processing (including programming, storage, and transmission to other electronic data record(s), or any other reproductions of similar nature), and translations.
However, Publisher grants back to the author(s) the following:
- The right to make and distribute copies of all or part of this work for use of the author(s) in teaching;
- The right to use, after publication in The Heart Surgery Forum, all or part of the material from this work in a book by the author(s), or in a collection of work by the author(s);
- The royalty-free right to make copies of this work for internal distribution within the institution/company that employs the author(s) subject to the provisions below for a work-made-for-hire;
- The right to use figures and tables from this work, and up to 250 words of text, for any purpose;
- The right to make oral presentations of material from this work.
Publisher reserves the right to grant or refuse permission to third parties to republish all or part of the article or translations thereof. To republish, such third parties must obtain written permission from the Publisher. (This is in accordance with the Copyright Statute, United States Code, Title 17. Exception: If all authors were bona fide officers or employees of the U.S. Government at the time the paper was prepared, the work is a “work of the US Government” (prepared by an officer or employee of the US Government as part of official duties), and therefore is not subject to US copyright; such exception should be indicated on signature lines. If this work was prepared under US Government contract or grant, the US Government may reproduce, royalty-free, all or portions of this work and may authorize others to do so, for official US Government purposes only, if the US Government contract or grant so requires.
I have participated in the conception and design of this work and in the writing of the manuscript and take public responsibility for it. Neither this manuscript nor one with substantially similar content under my authorship has been published, has been submitted for publication elsewhere, or will be submitted for publication elsewhere while under consideration by The Heart Surgery Forum, except as described in an attachment. I have reviewed this manuscript (original version) and approve its submission. If I am listed above as corresponding author, I will provide all authors with information regarding this manuscript and will obtain their approval before submitting any revision. I attest to the validity, accuracy, and legitimacy of the content of the manuscript and understand that Publisher assumes no responsibility for the validity, accuracy, and legitimacy of its content. I warrant that this manuscript is original with me and that I have full power to make this Agreement. I warrant that it contains no matter that is libelous or otherwise unlawful or that invades individual privacy or infringes any copyright or other proprietary right. I agree to indemnify and hold Publisher harmless of and from any claim made against Publisher that relates to or arises out of the publication of the manuscript and agree that this indemnification shall include payment of all costs and expenses relating to the defense of any such claim, including all reasonable attorney’s fees.
I warrant that I have no financial interest in the drugs, devices, or procedures described in the manuscript (except as disclosed in the attached statement).
I state that the institutional Human Subjects Committee and/or the Ethics Committee approved the clinical protocol reported in this manuscript for the use of experimental techniques, drugs, or devices in human subjects and appropriate informed consent documents were utilized.
Furthermore, I state that any and all animals used for experimental purposes received humane care in USDA registered facilities in compliance with the “Principles of Laboratory Animal Care” formulated by the National Society for Medical Research and the “Guide for the Care and Use of Laboratory Animals” prepared by the Institute of Laboratory Animal Resources and published by the National Institutes of Health (NIH Publication No. 85-23, revised 1985).