Pulmonary arterial hypertension in congenital cardiac disease – the need for refinement of the Evian-Venice classification

Abstract

Pulmonary hypertension associated with congenital systemic-to-pulmonary shunts has been classified, in the Evian-Venice classification, as Pulmonary Arterial Hypertension, which includes a heterogeneous group of conditions. Emerging options for treatment of patients with pulmonary arterial hypertension are related to those with the idiopathic form of the disease, but may also improve quality of life and survival in patients with pulmonary arterial hypertension associated with congenital cardiac disease. Despite the evident similarities in pulmonary vascular disease, it is important also to recognise the differences between patients in whom pulmonary arterial hypertension is the consequence of systemic-to-pulmonary shunts as opposed to those with other conditions. Patients with pulmonary hypertension associated with congenital cardiac disease themselves constitute a heterogeneous population, in which generalisation may be hazardous. Specific considerations need to be given to the type of cardiac diagnosis, the prognosis and evolution of pulmonary vascular disease, and the circulatory physiology before embarking on new strategies for medical treatment in the individual patient. In this review, we highlight the features that require specific attention in these patients. In addition, we discuss briefly the data currently available on the effectiveness of the new anti-proliferative drugs in patients with the Eisenmenger syndrome.

2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension

“…The criteria for shunt closure based on baseline PVR have been proposed (Table 24) based on available literature data [317][318][319]. Additional criteria include the type of the defect, age, the PVR:SVR ratio and the Qp:Qs ratio [320].…”

Pediatric Pulmonary Hypertension in the Netherlands

“…Unfortunately, the latest clinical classification of PH, updated by the inclusion of 4 clinical classes within PAH-CHD, does not yet suffice for categorizing the pediatric age-group specific presentations of PAH-CHD, illustrated by the group of accelerated PAH-CHD and CHD with abnormal development of pulmonary vasculature. 1,15 Adults with Eisenmenger syndrome are reported to have better survival than those with iPAH. [17][18][19] However, it is important to realize that such studies suffer from selection bias because most exclude patients who died during childhood.…”

“…21,22 The development of advanced PAH in CHD generally requires long-standing increased pulmonary blood flow with or without increased pulmonary pressures. 15 We describe a subgroup of infants with CHD with post-tricuspid shunt who showed accelerated development of PAH in the weeks to months after birth and whose survival was worse than that of infants with iPAH. This suggests additional factors in the pathogenesis of PAH, such as disturbed postnatal adaptation of the pulmonary vascular bed or increased susceptibility for development of PAH.…”

“…Patients with PAH-CHD with post-tricuspid shunt (or Eisenmenger syndrome) had unrestrictive shunt defects below the level of the tricuspid shunt (eg, ventricular septal defects [VSD]). 10,15 Accelerated PAH-CHD involved infants who developed advanced PAH in the first weeks to months of life in the presence of an unrestrictive post-tricuspid shunt defect. Because this is distinct from the usual time path for the development of PAH caused by systemic-to-pulmonary shunt, we previously named this group accelerated PAH-CHD.…”

Congenital Heart Disease and Pulmonary Hypertension

“…This chronic course allows patients to reset their normal level of activity, adjusting it to help compensate for the chronic hypoxia. [25][26][27][28][29] Therefore, they may not report the presence of significant symptoms with regular activity, underscoring the need for more objective assessment, as outlined later.…”