Editorial Comment

The Flow From Below

Neil Wilson,* MD

John Radcliffe Hospital, Paediatric CardiologyOxford, United Kingdom

The paper of Butera, Marini, and MacDonald servesto remind us of the continuing morbidity pediatric car-diologists of all persuasions encounter in the unnaturalhistory of patients with univentricular heart circulation.The surgical strategy for univentricular heart haslargely evolved via a diverse sequence of palliativeoperations to total cavopulmonary connection. Thisconsists of a superior vena cava anastomosed usuallyto the right pulmonary artery aspect of the pulmonaryartery bifurcation (so-called bidirectional Glenn anasto-mosis) and subsequent direction of inferior vena cavablood flow to the main pulmonary artery. This isachieved either by way of an extracardiac conduit ofprosthetic material or a ‘‘lateral tunnel" constructedincorporating the native right atrial wall and a hemicy-linder of prosthetic material. Whilst the concept of this‘‘passive" (it is not) force providing pulmonary bloodflow may seem extraordinary, it has seen many thou-sands of patients afforded good quality survival toadult life. These longer term and better quality of lifesurvivors by and large have been those whose ‘‘single"ventricular mass is predominantly of left ventricle mor-phology. This physiological concept and a surgicalremedy to facilitate survival is ascribed to truly inno-vative surgeons, Francis Fontan and Guillermo Kreut-zer, the latter recently documenting the record 34-yearongoing survival of one of his early patients [1].

Butera and colleagues describe a panoply of thera-pies, medical and interventional, which have been usedto treat one of the most harrowing long term sequelaeof this circulation. The distressing feature they describein their patient is the occurrence of protein losingenteropathy in the context of apparently low pulmo-nary artery pressure and resistance. Their reportingserves to remind us that current thoughts of cause/effect from high systemic venous pressures secondary

to high ventricular end diastolic pressure relaying backthrough high pulmonary vascular resistance is far fromthe whole story. Flow pattern physiology must haveplayed a part in Butera’s patient and I applaud theirlogic of closing the albeit small persisting source ofpulsatile antegrade pulmonary blood flow in the con-text of low pulmonary resistance. A small pulsatilecontribution to pulmonary blood flow is thought bymany to be beneficial, demonstrated almost 30 yearsago by Bull and De Leval connoisseurs of flow dy-namics and energy forces in this circulation [2]. Sonow we know, flow streaming from such sources maybe deleterious.So paediatric cardiologists remain in the dark. We

take our guess at fenestration yes or no, antegrade pul-satile flow, good or bad. Eventually sadly, we are leftwith a failing circulation. We would probably all agreethat in a ‘‘failing Fontan" clinical situation, assumingall measures are taken surgically, interventionally andmedically to ensure pulmonary vascular resistance islow, maintaining systemic venous flow energy canonly be good for the circulation [3].So here’s a suggestion: Improve hepatic venous

return to the inferior vena cava and kick some energyinto pulmonary blood flow. Transjugular IntrahepaticPortosystemic shunt: TIPS therapy for protein losingenteropathy in patients with a Fontan Kreutzer circula-tion. ‘‘Go with the flow from below.’’

REFERENCES

1. Kreutzer GO, Schlichter AJ, Kreutzer C. The Fontan Kreutzer

procedure at 40: An operation for the correction of pulmonary

atresia. Semin Thorac Cardiovasc Pediatr Card Surg Ann

2010;13:84–90.

2. Bull C, De Leval MR, Stark J. J Thorac Cardiovasc Surg

1983;85:21–31.

3. Goldberg DJ, Shaddy RE, Ravishankar C, Rychik J. The failing

Fontan: Etiology, diagnosis and management. Expert Rev Cardio-

vasc Ther 2011;9:785–93.

Conflict of interest: Nothing to report.

*Correspondence to: Neil Wilson, Headley Way, Oxford, Oxfordshire,

United Kingdom OX3 9DU. E-mail: Neilwil1@aol.com

Received 11 August 2011; Revision accepted 12 August 2011

DOI 10.1002/ccd.23347

Published online 23 September 2011 in Wiley Online Library

(wileyonlinelibrary.com).

' 2011 Wiley-Liss, Inc.

Catheterization and Cardiovascular Interventions 78:589 (2011)