Assessment of reversibility in pulmonary arterial hypertension and congenital heart disease

Abstract

Pulmonary arterial hypertension (PAH) in congenital heart disease (CHD) can be reversed by early shunt closure, but this potential is lost beyond a certain point of no return. Therefore, it is crucial to accurately assess the reversibility of this progressive pulmonary arteriopathy in an early stage. Reversibility assessment is currently based on a combination of clinical symptoms and haemodynamic variables such as pulmonary vascular resistance. These measures, however, are of limited predictive value and leave many patients in the grey zone. This review provides a concise overview of the mechanisms involved in flow-dependent progression of PAH in CHD and evaluates existing and future alternatives to more directly investigate the stage of the pulmonary arteriopathy. Structural quantification of the pulmonary arterial tree using fractal branching algorithms, functional imaging with intravascular ultrasound, nuclear imaging, putative new blood biomarkers, genetic testing and the potential for transcriptomic analysis of circulating endothelial cells and educated platelets are being reviewed.

Serum copeptin: a potential guide to clinical decision-making for congenital shunt closure

“…In their recently published article, van der Feen et al have discussed a variety of modalities in assessing the reversibility of PAH in the setting of congenital heart disease (CHD) 1. We agree on the potential utility of most of these modalities along with the limited, value of well-known biomarkers including natriuretic peptides in this setting 1.…”

“…To the Editor In clinical practice, pulmonary arterial hypertension (PAH) associated with congenital cardiac shunts may possibly progress to end-stage pulmonary arterial vasculopathy even after successful shunt closure potentially leading to substantially worse prognosis in certain settings 1. In their recently published article, van der Feen et al have discussed a variety of modalities in assessing the reversibility of PAH in the setting of congenital heart disease (CHD) 1.…”

“…In their recently published article, van der Feen et al have discussed a variety of modalities in assessing the reversibility of PAH in the setting of congenital heart disease (CHD) 1. We agree on the potential utility of most of these modalities along with the limited, value of well-known biomarkers including natriuretic peptides in this setting 1. On the other hand, we hold the opinion that copeptin (C-terminal provasopressin), a unique biomarker with multifaceted pathophysiological implications,2 as opposed to natriuretic peptides, might serve as an important adjunctive guide to decision-making for shunt closure in the setting of CHD.…”

Image diagnosis: Eisenmenger’s syndrome in patients with simple congenital heart disease

“…According to current guidelines, assessment of reversibility is limited to hemodynamic variables: those in favor of reversible PAH-CHD are a left-to-right shunt and a PVR index < 4 Woods units. Shunt closure is contraindicated when the net shunt is directed right-to-left, and is discouraged when the PVR index is > 8 Woods units [1]. When the PVR index is between 4 and 8 Woods units, 'individual patient evaluation in tertiary centers' is advised [12].…”

“…Pulmonary arterial hypertension (PAH) is a lethal syndrome characterized by increased PAP, PVR and normal pulmonary capillary wedge pressure [1]. Clinical presentation predominantly comprises symptoms of resulting right heart failure [2].…”