Abstract
Dr. O.P. Yadava, CEO & Chief Cardiac Surgeon, National Heart Institute, New Delhi, India, and Editor-in-Chief, Indian Journal of Thoracic and Cardiovascular Surgery, in conversation with Prof. Richard Jonas, Paediatric Cardiac Surgeon from Washington DC, USA, on Failing Fontan.
Keywords: Failing Fontan, Single ventricle, Protein losing enteropathy, Heart failure, Fenestration, Mechanical circulatory support
Prof. Jonas discusses various manifestations of failing Fontan. They are essentially similar to right heart failure, viz. pleural effusion, hepatomegaly and oedema. At times, there may be certain special features like protein losing enteropathy causing chronic osmotic diarrhoea. There may also be bronchial casts, which the patient may cough up, or may even have to be extracted bronchoscopically and they may develop intractable supraventricular tachyarrhythmias. Actually, a failing Fontan is quite a diverse condition ranging from just one feature to a combination of them. Prof. Jonas stresses that under a loosely used term—‘Single ventricle’—there are more than a dozen different forms of single ventricle. The Fontan’s operation was essentially designed for tricuspid atresia type of single ventricle. Now it has been applied even to hypoplastic left heart syndrome and to heterotaxy syndromes [1].
Causes of failing Fontan are very complex and multifarious [2]. Most of these patients have pulmonary venous anomalies, systemic venous anomalies, and even ciliary malfunction, besides the ventricle-related issues, and they all contribute to the failing Fontan. In heterotaxy syndromes, the failure is usually related to the atrioventricular (AV) valve regurgitation, ventricular dysfunction, and increase in pulmonary vascular resistance, while in the hypoplastic left heart, it is mainly due to progressive right ventricular failure. Prof. Jonas also stresses on the fact that though both diastolic and systolic failures may take place in a failing Fontan, the main problem is inability of the ventricle to fill at low pressures and therefore it is essentially a diastolic failure [3].
Another important factor for the late failure of Fontan is an increase in the pulmonary vascular resistance, which is not seen early. The registry data of Australia and New Zealand, which goes back to 40–45 years, has shown that beyond 20–25 years, patients develop increase in pulmonary vascular resistance and this could be related to the non-pulsatile flow that is inherent to the Fontan circulation [4]. Prof. Jonas feels that atriopulmonary connection leads to dilatation of the atrium with thrombosis and arrhythmias and therefore cavopulmonary connection, previously applying the lateral tunnel type but more recently the extracardiac conduit or intra/extracardiac conduit, is the way to go [5]. For management of these patients, fenestration may be a stop-gap arrangement, but not a long-term fix. Prof. Jonas is very optimistic on development of long-term mechanical circulatory support for these patients [6], which may take over from heart transplant as a realistic option in the future.
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Funding
Provided by the Indian Association of Cardiovascular and Thoracic Surgery.
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The authors declare that they have no conflict of interest.
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Supplementary information
The online version of this article (10.1007/s12055-020-01112-x) contains supplementary material, which is available to authorized users.
References
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