Main pulmonary artery dilation in association with congenital bicuspid aortic valve in the absence of pulmonary valve abnormality

Shelby Kutty, Sheetal Kaul, Christopher J. Danford, David Alan Danford

Research output: Contribution to journalArticle

28 Citations (Scopus)

Abstract

Background: Main pulmonary artery (MPA) dilation is reported to occur in association with pulmonary valve (PV) abnormalities and in genetic diseases of connective tissue. Aim: To determine whether MPA dilation occurs with bicuspid aortic valve (BAV) in the absence of PV abnormality. Methods: MPA and aortic dimensions were investigated in 194 subjects with BAV and 178 controls matched for age, sex and body surface area (BSA) using transthoracic two-dimensional echocardiography. Using control measurements, linear regression of each parameter versus age, gender, and BSA2/3 provided expected values. For each subject with BAV, the percentage divergence from expected value ((observed - expected value)/expected value) for each dimension was analysed. Results: MPA and aortic measurements showed normal distribution in all subjects. PV annulus and MPA were larger in BAV; 17.7% positive divergence of MPA dimension from expected was found in diastole, and 16.7% in systole. Aortic dimensions at all levels measured were larger in BAV; divergence was greater distally than proximally (10% divergence at annulus, 31% at ascending aorta). In BAV, divergence of ascending aortic dimensions from expected values increased with age (r2=0.142, p=0.02), but MPA divergence from expected values did not increase with age (r2=0.001, p=0.296). Conclusions: MPA dilation occurs in association with BAV in the absence of PV abnormality, suggesting primary vessel wall pathology predisposing to arterial dilation. A systemic abnormality of connective tissue common to both arteries may be responsible, or the dilation may result from a common developmental exposure as both originate from the embryologic conotruncus.

Original languageEnglish (US)
Pages (from-to)1756-1761
Number of pages6
JournalHeart
Volume96
Issue number21
DOIs
StatePublished - Jan 1 2010

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Pulmonary Valve
Pulmonary Artery
Dilatation
Connective Tissue
Inborn Genetic Diseases
Diastole
Bicuspid Aortic Valve
Systole
Body Surface Area
Normal Distribution
Echocardiography
Aorta
Linear Models
Arteries
Pathology

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

Cite this

Main pulmonary artery dilation in association with congenital bicuspid aortic valve in the absence of pulmonary valve abnormality. / Kutty, Shelby; Kaul, Sheetal; Danford, Christopher J.; Danford, David Alan.

In: Heart, Vol. 96, No. 21, 01.01.2010, p. 1756-1761.

Research output: Contribution to journalArticle

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abstract = "Background: Main pulmonary artery (MPA) dilation is reported to occur in association with pulmonary valve (PV) abnormalities and in genetic diseases of connective tissue. Aim: To determine whether MPA dilation occurs with bicuspid aortic valve (BAV) in the absence of PV abnormality. Methods: MPA and aortic dimensions were investigated in 194 subjects with BAV and 178 controls matched for age, sex and body surface area (BSA) using transthoracic two-dimensional echocardiography. Using control measurements, linear regression of each parameter versus age, gender, and BSA2/3 provided expected values. For each subject with BAV, the percentage divergence from expected value ((observed - expected value)/expected value) for each dimension was analysed. Results: MPA and aortic measurements showed normal distribution in all subjects. PV annulus and MPA were larger in BAV; 17.7{\%} positive divergence of MPA dimension from expected was found in diastole, and 16.7{\%} in systole. Aortic dimensions at all levels measured were larger in BAV; divergence was greater distally than proximally (10{\%} divergence at annulus, 31{\%} at ascending aorta). In BAV, divergence of ascending aortic dimensions from expected values increased with age (r2=0.142, p=0.02), but MPA divergence from expected values did not increase with age (r2=0.001, p=0.296). Conclusions: MPA dilation occurs in association with BAV in the absence of PV abnormality, suggesting primary vessel wall pathology predisposing to arterial dilation. A systemic abnormality of connective tissue common to both arteries may be responsible, or the dilation may result from a common developmental exposure as both originate from the embryologic conotruncus.",
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N2 - Background: Main pulmonary artery (MPA) dilation is reported to occur in association with pulmonary valve (PV) abnormalities and in genetic diseases of connective tissue. Aim: To determine whether MPA dilation occurs with bicuspid aortic valve (BAV) in the absence of PV abnormality. Methods: MPA and aortic dimensions were investigated in 194 subjects with BAV and 178 controls matched for age, sex and body surface area (BSA) using transthoracic two-dimensional echocardiography. Using control measurements, linear regression of each parameter versus age, gender, and BSA2/3 provided expected values. For each subject with BAV, the percentage divergence from expected value ((observed - expected value)/expected value) for each dimension was analysed. Results: MPA and aortic measurements showed normal distribution in all subjects. PV annulus and MPA were larger in BAV; 17.7% positive divergence of MPA dimension from expected was found in diastole, and 16.7% in systole. Aortic dimensions at all levels measured were larger in BAV; divergence was greater distally than proximally (10% divergence at annulus, 31% at ascending aorta). In BAV, divergence of ascending aortic dimensions from expected values increased with age (r2=0.142, p=0.02), but MPA divergence from expected values did not increase with age (r2=0.001, p=0.296). Conclusions: MPA dilation occurs in association with BAV in the absence of PV abnormality, suggesting primary vessel wall pathology predisposing to arterial dilation. A systemic abnormality of connective tissue common to both arteries may be responsible, or the dilation may result from a common developmental exposure as both originate from the embryologic conotruncus.

AB - Background: Main pulmonary artery (MPA) dilation is reported to occur in association with pulmonary valve (PV) abnormalities and in genetic diseases of connective tissue. Aim: To determine whether MPA dilation occurs with bicuspid aortic valve (BAV) in the absence of PV abnormality. Methods: MPA and aortic dimensions were investigated in 194 subjects with BAV and 178 controls matched for age, sex and body surface area (BSA) using transthoracic two-dimensional echocardiography. Using control measurements, linear regression of each parameter versus age, gender, and BSA2/3 provided expected values. For each subject with BAV, the percentage divergence from expected value ((observed - expected value)/expected value) for each dimension was analysed. Results: MPA and aortic measurements showed normal distribution in all subjects. PV annulus and MPA were larger in BAV; 17.7% positive divergence of MPA dimension from expected was found in diastole, and 16.7% in systole. Aortic dimensions at all levels measured were larger in BAV; divergence was greater distally than proximally (10% divergence at annulus, 31% at ascending aorta). In BAV, divergence of ascending aortic dimensions from expected values increased with age (r2=0.142, p=0.02), but MPA divergence from expected values did not increase with age (r2=0.001, p=0.296). Conclusions: MPA dilation occurs in association with BAV in the absence of PV abnormality, suggesting primary vessel wall pathology predisposing to arterial dilation. A systemic abnormality of connective tissue common to both arteries may be responsible, or the dilation may result from a common developmental exposure as both originate from the embryologic conotruncus.

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