Hostname: page-component-848d4c4894-nr4z6 Total loading time: 0 Render date: 2024-05-31T20:12:00.723Z Has data issue: false hasContentIssue false
  • English
  • Français

Long-term results after palliative intra-cardiac repair for tetralogy of Fallot and diminutive pulmonary arteries

Published online by Cambridge University Press:  20 June 2019

Yoichi Kawahira Open the ORCID record for Yoichi Kawahira [Opens in a new window] ,
Kyoichi Nishigaki ,
Koji Kagisaki ,
Takuji Watanabe  and
Kazuki Tanimoto
Yoichi Kawahira*
Affiliation:
Department of Pediatric Cardiovascular Surgery, Osaka City General Hospital, Osaka, Japan
Kyoichi Nishigaki
Affiliation:
Department of Pediatric Cardiovascular Surgery, Osaka City General Hospital, Osaka, Japan
Koji Kagisaki
Affiliation:
Department of Pediatric Cardiovascular Surgery, Osaka City General Hospital, Osaka, Japan
Takuji Watanabe
Affiliation:
Department of Pediatric Cardiovascular Surgery, Osaka City General Hospital, Osaka, Japan
Kazuki Tanimoto
Affiliation:
Department of Pediatric Cardiovascular Surgery, Osaka City General Hospital, Osaka, Japan
*
Author for correspondence: Yoichi Kawahira, MD, Department of Pediatric Cardiovascular Surgery, Osaka City General Hospital, 2-13-22 Miyakojimahondori, Miyakojimaku, Osaka 534-0021, Japan. Tel: +81-6-6929-1221; Fax: +81-6-6929-1091; E-mail: ykawahir@mac.com
Get access Rights & Permissions [Opens in a new window]

Abstract

Background:

In patients with tetralogy of Fallot with the diminutive pulmonary arteries, we sometimes have to give up the complete intra-cardiac repair due to insufficient growth of the pulmonary arteries. We have carried out palliative intra-cardiac repair using a fenestrated patch.

Methods:

Of all 202 patients with tetralogy of Fallot in our centre since 1996, five patients (2.5%) with the diminutive pulmonary arteries underwent palliative intra-cardiac repair using a fenestrated patch. Mean operative age was 1.8 years. Previous operation was Blalock–Taussig shunt in 4. At operation, the ventricular septal defect was closed using a fenestrated patch and the right ventricular outflow tract was enlarged. Follow-up period was 9.8 ± 2.6 years.

Results:

There were no operative and late deaths. Fenestration closed spontaneously on its own in four patients 2.7 ± 2.1 years after the intra-cardiac repair with a stable haemodynamics; however, the last patient with the smallest pulmonary artery index had supra-systemic pressure of the right ventricle post-operatively. The fenestration was emergently enlarged. Systemic arterial oxygen saturation was significantly and dramatically increased from 83.5 to 94% after the palliative intra-cardiac repair, and to 98% at the long term. A ratio of systolic pressure of the right ventricle to the left was significantly decreased to 0.76 ± 0.12 at the long term. Now all five patients were Ross classification class I.

Conclusion:

Although frequent catheter and surgical interventions were needed after the palliative intra-cardiac repair, this repair might be a choice improving quality of life with good results in patients with tetralogy of Fallot associated with the diminutive pulmonary arteries.

Keywords

Palliative intra-cardiac repairlong-term resultfenestrated patchtetralogy of Fallotdiminutive pulmonary artery
Type
Original Article
Information
Cardiology in the Young , Volume 29 , Issue 8 , August 2019 , pp. 1036 - 1039
Copyright
© Cambridge University Press 2019 

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

Soquet, J, Liava’a, M, Eastaugh, L, et al. Achievements and limitations of a strategy of rehabilitation of native pulmonary vessels in pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries. Ann Thorac Surg 2017; 103: 1519–1526.CrossRefGoogle ScholarPubMed
Marshall, AC, Love, BA, Lang, P, et al. Staged repair of tetralogy of Fallot and diminutive pulmonary arteries with a fenestrated ventricular septal defect patch. J Thorac Cardiovasc Surg 2003; 126: 1427–1433.CrossRefGoogle ScholarPubMed
Rabinowitz, EJ, Epstein, S, Kohn, N, Meyer, DB. Promoting pulmonary arterial growth via right ventricle-to-pulmonary artery connection in children with pulmonary atresia, ventricular septal defect, and hypoplastic pulmonary arteries. World J Pediatr Congenit Heart Surg 2017; 8: 564–549.CrossRefGoogle ScholarPubMed
Duncan, BW, Mee, RB, Prieto, LR, et al. Staged repair of tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries. J Thorac Cardiovasc Surg 2003; 126: 694–702.CrossRefGoogle ScholarPubMed
Bradley, SM, Erdem, CC, Hsia, TY, Atz, AM, Bandisode, V, Ringewald, JM. Right ventricle-to-pulmonary artery shunt: alternative palliation in infants with inadequate pulmonary blood flow prior to two-ventricle repair. Ann Thorac Surg 2008; 86: 183–188.CrossRefGoogle ScholarPubMed
Gerellia, S, van Steenberghea, M, Murtuzaa, B, et al. Neonatal right ventricle to pulmonary connection as a palliative procedure for pulmonary atresia with ventricular septal defect or severe tetralogy of Fallot. Eur J Cardio-Thoracic Surg 2014; 45: 278–288.CrossRefGoogle Scholar
Hopkins, WE, Hall, C. Paradoxical relationship between N-terminal proatrial natriuretic peptide and filling pressure in adults with cyanotic congenital heart disease. Circulation 1997; 96: 2215–2220.CrossRefGoogle ScholarPubMed
Saliba, Z, Butera, G, Bonnet, D, et al. Quality of life and perceived health status in surviving adults with univentricular heart. Heart 2001; 86: 69–73.CrossRefGoogle ScholarPubMed
Areias, ME, Pinto, CI, Vieira, PF, et al. Living with CHD: quality of life (QOL) in early adult life. Cardiol Young 2014; 24: 60–65.CrossRefGoogle Scholar
Müller, J, Hess, J, Hager, A. Exercise performance and quality of life is more impaired in Eisenmenger syndrome than in complex cyanotic congenital heart disease with pulmonary stenosis. Int J Cardiol 2011; 150: 177–181.CrossRefGoogle ScholarPubMed
Learn, C, Phillips, A, Chisolm, J, et al. Pulmonary atresia with ventricular septal defect and multifocal pulmonary blood supply: does an intensive interventional approach improve the outcome? Congenit Heart Dis 2012; 7: 111–121.CrossRefGoogle ScholarPubMed