Magnetic resonance imaging catheter stress haemodynamics post-Fontan in hypoplastic left heart syndrome

Kuberan Pushparajah, James K. Wong, Hannah R. Bellsham-Revell, Tarique Hussain, Israel Valverde, Aaron Bell, Aphrodite Tzifa, Gerald Greil, John M. Simpson, Shelby Kutty, Reza Razavi

Research output: Contribution to journalArticle

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Abstract

Aims Exercise limitation is common post-Fontan. Hybrid X-ray and magnetic resonance imaging (XMR) catheterization allows haemodynamic assessment by means of measurement of ventricular volumes and flow in major vessels with simultaneous invasive pressures.We aim to assess haemodynamic response to stress in patients with hypoplastic left heart syndrome (HLHS) post-Fontan. Methods and results Prospective study of 13 symptomatic children (NHYA 2) with HLHS post-Fontan using XMR catheterization. Three conditions were applied: baseline (Stage 1), dobutamine at 10 mg/kg/min (Stage 2), and dobutamine at 20 μg/kg/min (Stage 3). Seven consecutive patients received inhaled nitric oxide (iNO) at peak stress. Control MRI data were from normal healthy adults. In the HLHS patients, baseline mean pulmonary vascular resistance (PVR) was 1.51±0.59WU m2 and aortopulmonary collateral flow was 17.7±13.6% of systemic cardiac output. Mean right ventricular end-diastolic pressure was 6.7±2.5 mmHg which did not rise with stress. Cardiac index (CI) increased at Stage 2 in HLHS (40%) and controls (61%) but continued to increase at Stage 3 only in controls (19%) but not in HLHS. The blunted rise in CI in HLHS was due to a continuing fall in end-diastolic volume throughout stress, with no significant change in PVR or CI at peak stress in response to iNO. Conclusion Cardiac output post-Fontan in HLHS at peak stress is blunted due to a limitation in preload which is not responsive to inhaled pulmonary vasodilators in the setting of normal PVR.

Original languageEnglish (US)
Pages (from-to)644-651
Number of pages8
JournalEuropean heart journal cardiovascular Imaging
Volume17
Issue number6
DOIs
StatePublished - Jun 1 2016

Fingerprint

Hypoplastic Left Heart Syndrome
Catheters
Hemodynamics
Magnetic Resonance Imaging
Vascular Resistance
Dobutamine
Catheterization
Cardiac Output
Nitric Oxide
X-Rays
Vasodilator Agents
Prospective Studies
Exercise
Blood Pressure
Pressure
Lung

Keywords

  • Fontan
  • MRI
  • dobutamine
  • hypoplastic left heart syndrome
  • pulmonary vascular resistance

ASJC Scopus subject areas

  • Radiology Nuclear Medicine and imaging
  • Cardiology and Cardiovascular Medicine

Cite this

Pushparajah, K., Wong, J. K., Bellsham-Revell, H. R., Hussain, T., Valverde, I., Bell, A., ... Razavi, R. (2016). Magnetic resonance imaging catheter stress haemodynamics post-Fontan in hypoplastic left heart syndrome. European heart journal cardiovascular Imaging, 17(6), 644-651. https://doi.org/10.1093/ehjci/jev178

Magnetic resonance imaging catheter stress haemodynamics post-Fontan in hypoplastic left heart syndrome. / Pushparajah, Kuberan; Wong, James K.; Bellsham-Revell, Hannah R.; Hussain, Tarique; Valverde, Israel; Bell, Aaron; Tzifa, Aphrodite; Greil, Gerald; Simpson, John M.; Kutty, Shelby; Razavi, Reza.

In: European heart journal cardiovascular Imaging, Vol. 17, No. 6, 01.06.2016, p. 644-651.

Research output: Contribution to journalArticle

Pushparajah, K, Wong, JK, Bellsham-Revell, HR, Hussain, T, Valverde, I, Bell, A, Tzifa, A, Greil, G, Simpson, JM, Kutty, S & Razavi, R 2016, 'Magnetic resonance imaging catheter stress haemodynamics post-Fontan in hypoplastic left heart syndrome', European heart journal cardiovascular Imaging, vol. 17, no. 6, pp. 644-651. https://doi.org/10.1093/ehjci/jev178
Pushparajah, Kuberan ; Wong, James K. ; Bellsham-Revell, Hannah R. ; Hussain, Tarique ; Valverde, Israel ; Bell, Aaron ; Tzifa, Aphrodite ; Greil, Gerald ; Simpson, John M. ; Kutty, Shelby ; Razavi, Reza. / Magnetic resonance imaging catheter stress haemodynamics post-Fontan in hypoplastic left heart syndrome. In: European heart journal cardiovascular Imaging. 2016 ; Vol. 17, No. 6. pp. 644-651.
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abstract = "Aims Exercise limitation is common post-Fontan. Hybrid X-ray and magnetic resonance imaging (XMR) catheterization allows haemodynamic assessment by means of measurement of ventricular volumes and flow in major vessels with simultaneous invasive pressures.We aim to assess haemodynamic response to stress in patients with hypoplastic left heart syndrome (HLHS) post-Fontan. Methods and results Prospective study of 13 symptomatic children (NHYA 2) with HLHS post-Fontan using XMR catheterization. Three conditions were applied: baseline (Stage 1), dobutamine at 10 mg/kg/min (Stage 2), and dobutamine at 20 μg/kg/min (Stage 3). Seven consecutive patients received inhaled nitric oxide (iNO) at peak stress. Control MRI data were from normal healthy adults. In the HLHS patients, baseline mean pulmonary vascular resistance (PVR) was 1.51±0.59WU m2 and aortopulmonary collateral flow was 17.7±13.6{\%} of systemic cardiac output. Mean right ventricular end-diastolic pressure was 6.7±2.5 mmHg which did not rise with stress. Cardiac index (CI) increased at Stage 2 in HLHS (40{\%}) and controls (61{\%}) but continued to increase at Stage 3 only in controls (19{\%}) but not in HLHS. The blunted rise in CI in HLHS was due to a continuing fall in end-diastolic volume throughout stress, with no significant change in PVR or CI at peak stress in response to iNO. Conclusion Cardiac output post-Fontan in HLHS at peak stress is blunted due to a limitation in preload which is not responsive to inhaled pulmonary vasodilators in the setting of normal PVR.",
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AU - Hussain, Tarique

AU - Valverde, Israel

AU - Bell, Aaron

AU - Tzifa, Aphrodite

AU - Greil, Gerald

AU - Simpson, John M.

AU - Kutty, Shelby

AU - Razavi, Reza

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N2 - Aims Exercise limitation is common post-Fontan. Hybrid X-ray and magnetic resonance imaging (XMR) catheterization allows haemodynamic assessment by means of measurement of ventricular volumes and flow in major vessels with simultaneous invasive pressures.We aim to assess haemodynamic response to stress in patients with hypoplastic left heart syndrome (HLHS) post-Fontan. Methods and results Prospective study of 13 symptomatic children (NHYA 2) with HLHS post-Fontan using XMR catheterization. Three conditions were applied: baseline (Stage 1), dobutamine at 10 mg/kg/min (Stage 2), and dobutamine at 20 μg/kg/min (Stage 3). Seven consecutive patients received inhaled nitric oxide (iNO) at peak stress. Control MRI data were from normal healthy adults. In the HLHS patients, baseline mean pulmonary vascular resistance (PVR) was 1.51±0.59WU m2 and aortopulmonary collateral flow was 17.7±13.6% of systemic cardiac output. Mean right ventricular end-diastolic pressure was 6.7±2.5 mmHg which did not rise with stress. Cardiac index (CI) increased at Stage 2 in HLHS (40%) and controls (61%) but continued to increase at Stage 3 only in controls (19%) but not in HLHS. The blunted rise in CI in HLHS was due to a continuing fall in end-diastolic volume throughout stress, with no significant change in PVR or CI at peak stress in response to iNO. Conclusion Cardiac output post-Fontan in HLHS at peak stress is blunted due to a limitation in preload which is not responsive to inhaled pulmonary vasodilators in the setting of normal PVR.

AB - Aims Exercise limitation is common post-Fontan. Hybrid X-ray and magnetic resonance imaging (XMR) catheterization allows haemodynamic assessment by means of measurement of ventricular volumes and flow in major vessels with simultaneous invasive pressures.We aim to assess haemodynamic response to stress in patients with hypoplastic left heart syndrome (HLHS) post-Fontan. Methods and results Prospective study of 13 symptomatic children (NHYA 2) with HLHS post-Fontan using XMR catheterization. Three conditions were applied: baseline (Stage 1), dobutamine at 10 mg/kg/min (Stage 2), and dobutamine at 20 μg/kg/min (Stage 3). Seven consecutive patients received inhaled nitric oxide (iNO) at peak stress. Control MRI data were from normal healthy adults. In the HLHS patients, baseline mean pulmonary vascular resistance (PVR) was 1.51±0.59WU m2 and aortopulmonary collateral flow was 17.7±13.6% of systemic cardiac output. Mean right ventricular end-diastolic pressure was 6.7±2.5 mmHg which did not rise with stress. Cardiac index (CI) increased at Stage 2 in HLHS (40%) and controls (61%) but continued to increase at Stage 3 only in controls (19%) but not in HLHS. The blunted rise in CI in HLHS was due to a continuing fall in end-diastolic volume throughout stress, with no significant change in PVR or CI at peak stress in response to iNO. Conclusion Cardiac output post-Fontan in HLHS at peak stress is blunted due to a limitation in preload which is not responsive to inhaled pulmonary vasodilators in the setting of normal PVR.

KW - Fontan

KW - MRI

KW - dobutamine

KW - hypoplastic left heart syndrome

KW - pulmonary vascular resistance

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