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

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 ResultsWe 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

Pulmonary hypertension in the changing landscape of congenital heart disease: Global differences and a possible driver of end-stage heart failure

“…and Couperus et al. focus on some of the questions encountered in contemporary clinical congenital cardiology practice [ 1 , 2 ].…”

“…This change in phenotype of PAH-CHD is nicely highlighted in the article by Couperus et al. which provides a fascinating insight into the tailored treatment of some of the patients presenting with pulmonary hypertension after having had surgical repair or palliation in the past [ 1 ]. Although the current guidelines definitively provide a handhold [ 14 ], it is undeniable that some cases fall between the cracks of these guidelines.…”

“…In their article, Couperus et al. describe two patients who had progressive right ventricular failure due to pulmonary hypertension (one in the presence of a recanalised central shunt with left-to-right shunting), one patient with worsening cyanosis in the setting of a hypoplastic pulmonary vasculature resulting in pulmonary hypertension with right-to-left shunting and one with pulmonary hypertension associated with increased left atrial pressure due to atrioventricular valve stenosis in the setting of a univentricular heart [ 1 ]. It is clear that all four patients had heart failure with pulmonary (arterial) hypertension being the main driver of the heart failure symptoms, but their specific cases may not be covered in detail by recently published recommendations for CHD heart failure [ 15 ].…”

“…It is clear that all four patients had heart failure with pulmonary (arterial) hypertension being the main driver of the heart failure symptoms, but their specific cases may not be covered in detail by recently published recommendations for CHD heart failure [ 15 ]. It is also immediately clear that heart failure in adults with CHD is vastly different from ‘classic’ heart failure in the general population, developing at an earlier age (patients were 23 to 49 years of age at the time of intervention [ 1 ]), and often with an underlying ‘heart failure-inducing’ haemodynamic lesion. In such circumstances surgery or percutaneous interventions may improve heart failure, even in the setting of pulmonary hypertension.…”

Pulmonary hypertension: the importance of a multidisciplinary approach

“…Couperus et al. describe the tailored interventional treatment of complex CHD patients suffering from PH with an impaired pulmonary flow, and emphasised the importance of a multidisciplinary approach [ 16 ]. Next to optimal medical PAH-specific therapy, an invasive circulatory adjustment might be necessary to decrease right ventricular overload.…”