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New insights into the aspects of pulmonary diffusing capacity in Fontan patients

Published online by Cambridge University Press:  03 April 2013

Lars Idorn ,
Birgitte Hanel ,
Annette S. Jensen ,
Klaus Juul ,
Jesper I. Reimers ,
Kim G. Nielsen  and
Lars Søndergaard
Lars Idorn*
Affiliation:
Department of Cardiology, Rigshospitalet, Copenhagen, Denmark
Birgitte Hanel
Affiliation:
Department of Paediatrics and Adolescent Medicine, Section of Paediatric Pulmonary Service, Rigshospitalet, Copenhagen, Denmark
Annette S. Jensen
Affiliation:
Department of Cardiology, Rigshospitalet, Copenhagen, Denmark
Klaus Juul
Affiliation:
Department of Paediatrics and Adolescent Medicine, Section of Pediatric Cardiology, Rigshospitalet, Copenhagen, Denmark
Jesper I. Reimers
Affiliation:
Department of Paediatrics and Adolescent Medicine, Section of Pediatric Cardiology, Rigshospitalet, Copenhagen, Denmark
Kim G. Nielsen
Affiliation:
Department of Paediatrics and Adolescent Medicine, Section of Paediatric Pulmonary Service, Rigshospitalet, Copenhagen, Denmark
Lars Søndergaard
Affiliation:
Department of Cardiology, Rigshospitalet, Copenhagen, Denmark
*
Correspondence to: Dr L. Idorn, MD, Department of Cardiology, Blegdamsvej 9, section 2014, DK 2100, Rigshospitalet, Copenhagen East, Denmark. Tel: +0045 35450807; Fax: +0045 35452230; E-mail: [email protected]
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Abstract

Background: Patients with a functionally univentricular heart, palliated a.m. Fontan, consequently have non-pulsatile pulmonary blood flow and are known to have a reduced pulmonary diffusing capacity. However, the cause of this reduction remains unclear. We aimed to assess the possible determinants in the aetiology of a reduced diffusing capacity and also to assess whether it could be increased. Furthermore, we aimed to search for predictors of a reduced diffusing capacity. Material and methods: A total of 87 Fontan patients (mean age 16.3 ± 7.6 years) performed advanced pulmonary function tests and maximal cycle ergometer tests. A total of 10 Fontan patients and nine matched controls performed a supine pulmonary function test after a supine rest. Results: In the sitting pulmonary function test, the mean z-scores were: diffusing capacity, 2.38 ± 1.20; pulmonary capillary blood volume, 2.04 ± 0.80; and alveolar capillary membrane diffusing capacity, 0.14 ± 0.84. In the supine compared with the sitting pulmonary function test, the diffusing capacity increased by 51.7 ± 11.9% in the Fontan group and by 23.3 ± 17.7% in the control group (p < 0.001); moreover, the pulmonary capillary blood volume increased by 48.3 ± 17.4% in the Fontan group and by 20.2 ± 13.9% in the control group (p = 0.001). In a multiple linear regression analysis including the explanatory variables of surgical data and exercise data at rest and peak exercise, the resting cardiac index was an independent predictor of the diffusing capacity (regression coefficient: 0.18, p < 0.001). Conclusions: The pulmonary diffusing capacity was reduced in Fontan patients because of a reduced pulmonary capillary blood volume, whereas the alveolar capillary membrane diffusing capacity was preserved. The diffusing capacity was highly increasable in Fontan patients compared with controls, and the resting cardiac index was an independent predictor of the diffusing capacity.

Keywords

Fontan procedurecongenital heart diseasepulmonary diffusing capacityexercise test
Type
Original Articles
Information
Cardiology in the Young , Volume 24 , Issue 2 , April 2014 , pp. 311 - 320
Copyright
Copyright © Cambridge University Press 2013 

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