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. 2024 Mar 9;13(6):1567.
doi: 10.3390/jcm13061567.

Pulmonary Artery Dilatation Due to Pressure or Volume Overload in Congenital Heart Disease

Affiliations

Pulmonary Artery Dilatation Due to Pressure or Volume Overload in Congenital Heart Disease

Monika Kaldararova et al. J Clin Med. .

Abstract

Background: Pulmonary artery dilatation is described mostly in association with pulmonary hypertension. Patients/Methods: Study analysis: 60 patients with pulmonary arterial hypertension in congenital heart disease (PAH-CHD); 64 with repaired tetralogy of Fallot/pulmonary regurgitation (rTOF/PR); and 80 healthy (NORMAL). Measured were: main pulmonary artery (MPA) diameter and MPA/ascending aorta (Ao asc) ratio, by echocardiography (ECHO) and computer tomography or magnetic resonance imaging (CT/MRI). Results: In MPA diameter, significant differences between PAH-CHD, rTOF/PR, and NORMAL were found (median): 37 vs. 27 vs. 21 mm (p < 0.0001). In MPA/Ao asc ratio, there was a difference between PAH-CHD and NORMAL (median): 1.3 vs. 0.8 (p < 0.0001), but not between rTOF/PR and NORMAL: 0.74 vs. 0.8 (p = 0.3). Significant MPA dilatation (>40 mm) was present: in PAH-CHD, 35% (ECHO) and 76.9% (CT/MRI) of patients, while in rTOF/PR, 3.1% (ECHO) and 7.8% (CT/MRI). Severe MPA dilatation (>50 mm) occurred only in PAH-CHD: 16.7% (ECHO) and 31.4% (CT/MRI), while not in rTOF/PR. There was a significant correlation between ECHO and CT/MRI measurements, but ECHO was underestimated in all parameters. Conclusions: MPA dilatation due to pressure overload is more frequent and more severe; volume overload also leads to MPA dilatation but is less severe. The MPA/Ao asc ratio is not reliable for MPA dilatation estimation in rTOF/PR.

Keywords: pulmonary arterial hypertension; pulmonary artery diameter; pulmonary artery dilatation; pulmonary artery to ascending aorta ratio; pulmonary regurgitation; repaired Tetralogy of Fallot.

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Conflict of interest statement

M.K. has received fees from Actelion Pharmaceuticals Ltd., AOP Orphan, outside the submitted work. K.B. has received fees from AOP Orphan, Berlin Chemie—Menarini Group, and Egis outside the submitted work. A.K. declares no conflict of interest. E.D. declares no conflict of interest. J.PM. declares no conflict of interest. F.K. declares no conflict of interest. T.H. has received fees from Sanofi and Novartis outside of the submitted work. A.R. has received fees from Actelion Pharmaceuticals Ltd., AOP Orphan, outside the submitted work. T.V. has received fees from Actelion Pharmaceuticals Ltd., AOP Orphan, outside the submitted work. I.S. has received fees from Actelion Pharmaceuticals Ltd., AOP Orphan, and MSD outside the submitted work.

Figures

Figure 1
Figure 1
Pulmonary artery measurement: (A) scheme; (B) by echocardiography; and (C) by computer tomography (MPA—main pulmonary artery; Ao—aorta).
Figure 2
Figure 2
Parameters measured by ECHO, compared in groups (NORMAL, PAH-CHD, and rTOF/PR): (A). MPA diameter; (B). Ao asc diameter; and (C). MPA/Ao asc ratio. NORMAL—control healthy subjects; PAH-CHD—pulmonary artery hypertension associated with congenital heart defects; rTOF/PR—repaired Tetralogy of Fallot with severe pulmonary regurgitation; ECHO—echocardiography; MPA—main pulmonary artery; and Ao asc—ascending aorta.
Figure 3
Figure 3
Correlation of parameters by age, measured by ECHO, compared in groups (NORMAL, PAH-CHD, and rTOF/PR): (A). MPA diameter; (B). Ao asc diameter; and (C). MPA/Ao asc ratio. NORMAL—control healthy subjects; PAH-CHD—pulmonary artery hypertension associated with congenital heart defects; rTOF/PR—repaired Tetralogy of Fallot with severe pulmonary regurgitation; ECHO—echocardiography; MPA—main pulmonary artery; and Ao asc—ascending aorta.
Figure 4
Figure 4
ECHO and CT/MRI correlation: (A) in MPA diameter; (B) in Ao asc diameter; and (C) in MPA/Ao asc ratio. PAH-CHD—pulmonary artery hypertension associated with congenital heart defects; rTOF/PR—repaired Tetralogy of Fallot with severe pulmonary regurgitation; ECHO—echocardiography; CT/MRI—computer tomography/magnetic resonance imaging; MPA—main pulmonary artery; and Ao asc—ascending aorta.
Figure 5
Figure 5
(AC) MPA dilatation in PAH-CHD (CT); (DF) MPA plus RVOT dilatation in rTOF/PR (CT). PAH-CHD—pulmonary arterial hypertension associated with congenital heart defects; rTOF/PR—repaired Tetralogy of Fallot with isolated severe pulmonary regurgitation; PA—pulmonary artery; MPA—main pulmonary artery; RVOT—right ventricular outflow tract; LPA—left pulmonary artery; RPA—right pulmonary artery; Ao—aorta; and CT—computer tomography.

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