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Published in: Netherlands Heart Journal 6/2016

Open Access 01-06-2016 | Original Article

Tailored circulatory intervention in adults with pulmonary hypertension due to congenital heart disease

Authors: L. E. Couperus, I. R. Henkens, M. R. M. Jongbloed, M. G. Hazekamp, M. J. Schalij, H. W. Vliegen

Published in: Netherlands Heart Journal | Issue 6/2016

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Abstract

Background

Adults with pulmonary hypertension associated with congenital heart disease (PH-CHD) often have residual shunts. Invasive interventions aim to optimise pulmonary flow and prevent right ventricular failure. However, eligibility for procedures strongly depends on the adaptation potential of the pulmonary vasculature and right ventricle to resultant circulatory changes. Current guidelines are not sufficiently applicable to individual patients, who exhibit great diversity and complexity in cardiac anomalies.

Methods and Results

We present four complex adult PH-CHD patients with impaired pulmonary flow, including detailed graphics of the cardiopulmonary circulation. All these patients had an ambiguous indication for shunt intervention. Our local multidisciplinary Grown-Ups with Congenital Heart Disease team reached consensus regarding a patient-tailored invasive treatment strategy, adjacent to relevant guidelines. Interventions improved pulmonary haemodynamics and short-term clinical functioning in all cases.

Conclusions

Individual evaluation of disease characteristics is mandatory for tailored interventional treatment in PH-CHD patients, adjacent to relevant guidelines. Both strict registration of cases and multidisciplinary and multicentre collaboration are essential in the quest for optimal therapy in this patient population.
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Metadata
Title
Tailored circulatory intervention in adults with pulmonary hypertension due to congenital heart disease
Authors
L. E. Couperus
I. R. Henkens
M. R. M. Jongbloed
M. G. Hazekamp
M. J. Schalij
H. W. Vliegen
Publication date
01-06-2016
Publisher
Bohn Stafleu van Loghum
Published in
Netherlands Heart Journal / Issue 6/2016
Print ISSN: 1568-5888
Electronic ISSN: 1876-6250
DOI
https://doi.org/10.1007/s12471-016-0833-7

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