Published Online
on June 23, 2003

A more recent version of this article appeared on July 1, 2003

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Submitted on October 1, 2002
Revised on April 1, 2003
Accepted on April 4, 2003

Basal Pulmonary Vascular Resistance and Nitric Oxide Responsiveness Late After Fontan-Type Operation

S. Khambadkone MD, MRCP^*, J. Li MBBS, M. R. de Leval MD, FRCS, S. Cullen MB, BCh, BaO, FRCPI, J. E. Deanfield FRCP, and A. N. Redington FRCP

From Cardiothoracic Unit, Great Ormond Street Hospital for Children NHS Trust and Grown Up Congenital Heart Unit (S.C.), Heart Hospital, London, UK.

^* To whom correspondence should be addressed. E-mail: s.khambadkone{at}ich.ucl.ac.uk.

Background--The pulsatile nature of pulmonary blood flow is important for shear stress-mediated release of endothelium-derived nitric oxide (NO) and lowering pulmonary vascular resistance (PVR) by passive recruitment of capillaries. Normal pulsatile flow is lost or markedly attenuated after Fontan-type operations, but to date, there are no data on basal pulmonary vascular resistance and its responsiveness to exogenous NO at late follow-up in these patients.

Methods and Results--We measured indexed PVR (PVRI) using Fick principle to calculate pulmonary blood flow, with respiratory mass spectrometry to measure oxygen consumption, in 15 patients (median age, 12 years; range, 7 to 17 years; 12 male, 3 female) at a median of 9 years after a Fontan-type operation (6 atriopulmonary connections, 7 lateral tunnels, 2 extracardiac conduits). The basal PVRI was 2.11±0.79 Wood unit (WU) times m² (mean±SD) and showed a significant reduction to 1.61±0.48 (P=0.016) after 20 ppm of NO for 10 minutes. The patients with nonpulsatile group in the pulmonary circulation dropped the PVRI from 2.18±0.34 to 1.82±0.55 (P<0.05) after NO inhalation.

Conclusions--PVR falls with exogenous NO late after Fontan-type operation. These data suggest pulmonary endothelial dysfunction, related in some part to lack of pulsatility in the pulmonary circulation because of altered flow characteristics. Therapeutic strategies to enhance pulmonary endothelial NO release may have a role in these patients.

Key words: Fontan procedure • pulmonary vascular resistance • endothelial dysfunction

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