Abnormal Pulmonary Arterial Elastance Is Associated With Reduced Exercise Capacity in Tetralogy of Fallot

Alexander Egbe, Nandan S. Anavekar, Heidi M. Connolly

Research output: Contribution to journalArticle

Abstract

Background A previous study reported histologic abnormalities in the pulmonary artery ( PA ) of patients with tetralogy of Fallot ( TOF ). However, the potential effect of these anatomical findings on PA vascular function has not been studied. We hypothesized that TOF patients had abnormal PA vascular function, and that PA vascular function was associated with exercise capacity. Methods and Results This a study of adult TOF patients who had cardiac magnetic resonance imaging and echocardiogram (on the same day) at Mayo Clinic, 2002-2015. In order to test the study hypothesis, we compared PA elastance index ( PAE i) between 207 TOF patients and a referent group of 8 subjects without structural heart disease. PAE i was calculated as a quotient of PA systolic pressure and cardiac magnetic resonance imaging-derived right ventricular stroke volume. Mean age was 33±13 and 36±4 years in the TOF and referent groups respectively. TOF patients had higher PAE i compared with the referent group (0.62±0.12 versus 0.48±0.08 mm Hg/ mL /m2; P=0.001). There was a good correlation between PAE i and peak oxygen consumption (adjusted R2=0.73; r=0.85; P<0.001). After multivariate adjustment for potential confounders, PAE i was independently associated with peak oxygen consumption (adjusted R2=0.69; r=0.83; P<0.001). Conclusions The high PA elastance in the TOF group may be attributed to abnormal PA vascular function. The association between PAE i and exercise intolerance suggests that PA vascular dysfunction may contribute to exercise intolerance, which is an important clinical problem in this population. Further studies are required to validate our findings and explore potential therapies to improve PA vascular function in this population.

Original languageEnglish (US)
Pages (from-to)e011731
JournalJournal of the American Heart Association
Volume8
Issue number12
DOIs
StatePublished - Jun 18 2019

Fingerprint

Tetralogy of Fallot
Pulmonary Artery
Exercise
Lung
Blood Vessels
Oxygen Consumption
Magnetic Resonance Imaging
Stroke Volume
Population
Heart Diseases
Blood Pressure

Keywords

  • pulmonary artery
  • pulmonary vascular disease
  • tetralogy of Fallot

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

Cite this

Abnormal Pulmonary Arterial Elastance Is Associated With Reduced Exercise Capacity in Tetralogy of Fallot. / Egbe, Alexander; Anavekar, Nandan S.; Connolly, Heidi M.

In: Journal of the American Heart Association, Vol. 8, No. 12, 18.06.2019, p. e011731.

Research output: Contribution to journalArticle

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abstract = "Background A previous study reported histologic abnormalities in the pulmonary artery ( PA ) of patients with tetralogy of Fallot ( TOF ). However, the potential effect of these anatomical findings on PA vascular function has not been studied. We hypothesized that TOF patients had abnormal PA vascular function, and that PA vascular function was associated with exercise capacity. Methods and Results This a study of adult TOF patients who had cardiac magnetic resonance imaging and echocardiogram (on the same day) at Mayo Clinic, 2002-2015. In order to test the study hypothesis, we compared PA elastance index ( PAE i) between 207 TOF patients and a referent group of 8 subjects without structural heart disease. PAE i was calculated as a quotient of PA systolic pressure and cardiac magnetic resonance imaging-derived right ventricular stroke volume. Mean age was 33±13 and 36±4 years in the TOF and referent groups respectively. TOF patients had higher PAE i compared with the referent group (0.62±0.12 versus 0.48±0.08 mm Hg/ mL /m2; P=0.001). There was a good correlation between PAE i and peak oxygen consumption (adjusted R2=0.73; r=0.85; P<0.001). After multivariate adjustment for potential confounders, PAE i was independently associated with peak oxygen consumption (adjusted R2=0.69; r=0.83; P<0.001). Conclusions The high PA elastance in the TOF group may be attributed to abnormal PA vascular function. The association between PAE i and exercise intolerance suggests that PA vascular dysfunction may contribute to exercise intolerance, which is an important clinical problem in this population. Further studies are required to validate our findings and explore potential therapies to improve PA vascular function in this population.",
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N2 - Background A previous study reported histologic abnormalities in the pulmonary artery ( PA ) of patients with tetralogy of Fallot ( TOF ). However, the potential effect of these anatomical findings on PA vascular function has not been studied. We hypothesized that TOF patients had abnormal PA vascular function, and that PA vascular function was associated with exercise capacity. Methods and Results This a study of adult TOF patients who had cardiac magnetic resonance imaging and echocardiogram (on the same day) at Mayo Clinic, 2002-2015. In order to test the study hypothesis, we compared PA elastance index ( PAE i) between 207 TOF patients and a referent group of 8 subjects without structural heart disease. PAE i was calculated as a quotient of PA systolic pressure and cardiac magnetic resonance imaging-derived right ventricular stroke volume. Mean age was 33±13 and 36±4 years in the TOF and referent groups respectively. TOF patients had higher PAE i compared with the referent group (0.62±0.12 versus 0.48±0.08 mm Hg/ mL /m2; P=0.001). There was a good correlation between PAE i and peak oxygen consumption (adjusted R2=0.73; r=0.85; P<0.001). After multivariate adjustment for potential confounders, PAE i was independently associated with peak oxygen consumption (adjusted R2=0.69; r=0.83; P<0.001). Conclusions The high PA elastance in the TOF group may be attributed to abnormal PA vascular function. The association between PAE i and exercise intolerance suggests that PA vascular dysfunction may contribute to exercise intolerance, which is an important clinical problem in this population. Further studies are required to validate our findings and explore potential therapies to improve PA vascular function in this population.

AB - Background A previous study reported histologic abnormalities in the pulmonary artery ( PA ) of patients with tetralogy of Fallot ( TOF ). However, the potential effect of these anatomical findings on PA vascular function has not been studied. We hypothesized that TOF patients had abnormal PA vascular function, and that PA vascular function was associated with exercise capacity. Methods and Results This a study of adult TOF patients who had cardiac magnetic resonance imaging and echocardiogram (on the same day) at Mayo Clinic, 2002-2015. In order to test the study hypothesis, we compared PA elastance index ( PAE i) between 207 TOF patients and a referent group of 8 subjects without structural heart disease. PAE i was calculated as a quotient of PA systolic pressure and cardiac magnetic resonance imaging-derived right ventricular stroke volume. Mean age was 33±13 and 36±4 years in the TOF and referent groups respectively. TOF patients had higher PAE i compared with the referent group (0.62±0.12 versus 0.48±0.08 mm Hg/ mL /m2; P=0.001). There was a good correlation between PAE i and peak oxygen consumption (adjusted R2=0.73; r=0.85; P<0.001). After multivariate adjustment for potential confounders, PAE i was independently associated with peak oxygen consumption (adjusted R2=0.69; r=0.83; P<0.001). Conclusions The high PA elastance in the TOF group may be attributed to abnormal PA vascular function. The association between PAE i and exercise intolerance suggests that PA vascular dysfunction may contribute to exercise intolerance, which is an important clinical problem in this population. Further studies are required to validate our findings and explore potential therapies to improve PA vascular function in this population.

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