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


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.
Department of Cardiology, Leiden University Medical Center, Leiden, Netherlands. l.e.couperus@lumc.nl.
L E
LE
Couperus
Department of Cardiology, Leiden University Medical Center, Leiden, Netherlands.
I R
IR
Henkens
Department of Cardiology, Leiden University Medical Center, Leiden, Netherlands.|Department of Anatomy, Leiden University Medical Center, Leiden, Netherlands.
M R M
MRM
Jongbloed
Department of Cardiothoracic Surgery, Leiden University Medical Center, Leiden, Netherlands.
M G
MG
Hazekamp
Department of Cardiology, Leiden University Medical Center, Leiden, Netherlands.
M J
MJ
Schalij
Department of Cardiology, Leiden University Medical Center, Leiden, Netherlands.
H W
HW
Vliegen
Congenital heart defects; Intervention; Pulmonary hypertension; Right ventricular dysfunction