Multimodality imaging in congenital heart disease-related pulmonary arterial hypertension

Abstract

Pulmonary arterial hypertension (PAH) in adult patients with congenital heart disease (CHD) is associated with increased morbidity and mortality. The present review aims to discuss the clinical applications of invasive and non-invasive diagnostic modalities and to describe the strengths and weaknesses of each technique. Chest radiograph is an inexpensive investigation providing information on pulmonary arterial and hilar dilatation, pruning of peripheral pulmonary arteries and cardiomegaly. Transthoracic two-dimensional and Doppler echocardiography is the most widely used imaging tool. It provides information on cardiac anatomy and an estimate of haemodynamics and biventricular remodelling and function. In addition, echocardiography is valuable in assessing prognosis and monitoring the efficacy of therapy. Structural and functional changes associated with CHD-PAH, mainly affecting the right ventricle and pulmonary circulation, may represent an ideal target for evaluation with cardiac magnetic resonance. This non-invasive imaging modality has a low biological impact. CT plays an important role for patients with limited echocardiographic windows and those who are unable to undergo MRI (claustrophobia, poor compliance, presence of a pacemaker/implantable cardioverter defibrillator). It is the modality of choice for detailed assessment of pulmonary vessel obstruction or thrombosis. Finally, heart catheterisation remains the gold standard for diagnosing and confirming PAH in patients with CHD and for shunt evaluation. The diagnostic assessment of CHD-PAH requires great expertise and a deep knowledge of both CHD and PAH pathophysiology and should take place in a tertiary centre, where multiple data can be appropriately integrated and applied clinically.

Pulmonary arterial hypertension in congenital heart disease

“…Increased velocity of tricuspid regurgitation >3.4 m/sec, or, 2.9–3.4 m/sec along with presence of other echo signs make the diagnosis of pulmonary hypertension highly probable . As patients with CHD may have difficult echo windows which, combined with complex anatomy may not provide accurate diagnosis, cardiac MRI, or CT may be needed (in case of increased RV pressure can provide information on pulmonary arterial tree and distinguish, that is, peripheral PA stenosis from PAH).…”

“…Cardiac cath should always be performed in order to establish PAH diagnosis and to exclude PH due to left heart disease (group 2). It is also important to calculate pulmonary vascular resistance which is a determinant for future defect closure (Table ).…”

Pulmonary hypertension in adults with congenital heart disease: Updated recommendations from the Cologne Consensus Conference 2018

“…Besides the exact definition of the existing heart defect, the diagnostic approach in P(A)H-CHD includes the functional and hemodynamic grading of P(A)H [16]. Reference is made to current publications with regard to specific symptoms and the clinical assessment [5,10,[17][18][19][20][21][22][23][24][25][26][27][28][29][30][31][32]. Early diagnosis is important, because in patients with PAH, timely surgical or interventional correction can improve clinical course and prognosis.…”

“…Therefore, individual assessment of the parameters is important. Novel, standard echocardiography complementing parameters (tissue Doppler, speckle tracking, 3D features) allow a more exact quantification of global and regional ventricular function in P(A)H-CHD [18]. Because of the frequently limited ultrasound window, practicability and reproducibility, tricuspid annular plane systolic excursion (TAPSE) and fractional area change (FAC) continue to be recommended for analysis of RV function [18,34].…”

“…Although echocardiography is the established method for screening and assessment, a multimodal approach of imaging is recommended in CHD with and without PH/PAH. Besides transthoracic echocardiography (TTE), this includes 2D-and 3D-transesophageal echocardiography (3D-TEE), cardiac computerized tomography (CT) and MRI, as well as invasive diagnostics [18,34,35]. 3D-TEE plays an increasingly important role in the exact assessment of defects, in particular of shunts at the atrial and the atrioventricular (AV) valve level, and also in guiding interventional procedures [35,36].…”