Kuberan Pushparajah1, James K Wong2, Hannah R Bellsham-Revell3, Tarique Hussain1, Israel Valverde2, Aaron Bell3, Aphrodite Tzifa2, Gerald Greil1, John M Simpson1, Shelby Kutty4, Reza Razavi5. 1. Division of Imaging Sciences, King's College London BHF Centre, NIHR Biomedical Research Centre at Guy's and St Thomas' NHS Foundation Trust, London, UK Department of Congenital Heart Disease, Evelina London Children's Hospital, Guy's and St Thomas' NHS Foundation Trust, London, UK. 2. Division of Imaging Sciences, King's College London BHF Centre, NIHR Biomedical Research Centre at Guy's and St Thomas' NHS Foundation Trust, London, UK. 3. Department of Congenital Heart Disease, Evelina London Children's Hospital, Guy's and St Thomas' NHS Foundation Trust, London, UK. 4. University of Nebraska/Creighton University Joint Division of Pediatric Cardiology, Omaha, NE, USA. 5. Division of Imaging Sciences, King's College London BHF Centre, NIHR Biomedical Research Centre at Guy's and St Thomas' NHS Foundation Trust, London, UK Department of Congenital Heart Disease, Evelina London Children's Hospital, Guy's and St Thomas' NHS Foundation Trust, London, UK Paediatric Cardiovascular Sciences, Rayne Institute, King's College London and Evelina London Children's Hospital, Westminster Bridge Road, London SE1 7EH, UK reza.razavi@kcl.ac.uk.
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 µg/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 ± 0.59 WU m(2) 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. Published on behalf of the European Society of Cardiology. All rights reserved.
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 µg/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 ± 0.59 WU m(2) 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. Published on behalf of the European Society of Cardiology. All rights reserved.
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