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Differences in right ventricular-pulmonary vascular coupling and clinical indices between repaired standard tetralogy of Fallot and repaired tetralogy of Fallot with pulmonary atresia
Authors:S Buddhe  V Jani  S Sarikouch  L Gaur  A Schuster  P Beerbaum  M Lewin  S Kutty
Institution:1. Division of Pediatric Cardiology, Department of Pediatrics, Seattle Children''s Hospital, 91805 Seattle, WA, USA;2. Blalock Taussig Thomas Heart Center, The Johns Hopkins Hospital and School of Medicine, 1800 Orleans St, 21287 Baltimore, MD, USA;3. Department of Heart- Thoracic- Transplantation- and Vascular Surgery, Hannover Medical School, 30625 Hannover, Germany;4. Department of Cardiology and Pneumology, University of Goettingen School of Medicine, 37075 Göttingen, Germany;5. Department of Pediatric Cardiology and Pediatric Intensive Care, Hannover Medical School, Hannover Medical School, Hannover, Germany
Abstract:PurposeThe purpose of this study was to compare ventricular vascular coupling ratio (VVCR) between patients with repaired standard tetralogy of Fallot (TOF) and those with repaired TOF-pulmonary atresia (TOF-PA) using cardiovascular magnetic resonance (CMR).Materials and methodsPatients with repaired TOF aged > 6 years were prospectively enrolled for same day CMR, echocardiography, and exercise stress test following a standardized protocol. Sanz's method was used to calculate VVCR as right ventricle (RV) end-systolic volume/pulmonary artery stroke volume. Regression analysis was used to examine associations with exercise test parameters, New York Heart Association (NYHA) class, RV size and biventricular systolic function.ResultsA total of 248 subjects were included; of these 222 had repaired TOF (group I, 129 males; mean age, 15.9 ± 4.7 SD] years range: 8–29 years]) and 26 had repaired TOF-PA (group II, 14 males; mean age, 17.0 ± 6.3 SD] years range: 8–29 years]). Mean VVCR for all subjects was 1.54 ± 0.64 SD] (range: 0.43–3.80). Mean VVCR was significantly greater in the TOF-PA group (1.81 ± 0.75 SD]; range: 0.78–3.20) than in the standard TOF group (1.51 ± 0.72 SD]; range: 0.43–3.80) (P = 0.03). VVCR was greater in the 68 NYHA class II subjects (1.79 ± 0.66 SD]; range: 0.75–3.26) compared to the 179 NYHA class I subjects (1.46 ± 0.61 SD]; range: 0.43–3.80) (P < 0.001).ConclusionNon-invasive determination of VVCR using CMR is feasible in children and adolescents. VVCR showed association with NYHA class, and was worse in subjects with repaired TOF-PA compared to those with repaired standard TOF. VVCR shows promise as an indicator of pulmonary artery compliance and cardiovascular performance in this cohort.
Keywords:Tetralogy of Fallot  Magnetic resonance imaging  Pulmonary atresia  Pulmonary artery  Right ventricular dysfunction  CMR"}  {"#name":"keyword"  "$":{"id":"kw0035"}  "$$":[{"#name":"text"  "_":"Cardiac magnetic resonance  CPET"}  {"#name":"keyword"  "$":{"id":"kw0045"}  "$$":[{"#name":"text"  "_":"Cardiopulmonary exercise test  Ea"}  {"#name":"keyword"  "$":{"id":"kw0055"}  "$$":[{"#name":"text"  "_":"Arterial elastance  EDVi"}  {"#name":"keyword"  "$":{"id":"kw0065"}  "$$":[{"#name":"text"  "_":"Indexed end-diastolic volume  Ees"}  {"#name":"keyword"  "$":{"id":"kw0075"}  "$$":[{"#name":"text"  "_":"Ventricular elastance  EF"}  {"#name":"keyword"  "$":{"id":"kw0085"}  "$$":[{"#name":"text"  "_":"Ejection fraction  ESVi"}  {"#name":"keyword"  "$":{"id":"kw0095"}  "$$":[{"#name":"text"  "_":"Indexed end-systolic volume  IQR"}  {"#name":"keyword"  "$":{"id":"kw0105"}  "$$":[{"#name":"text"  "_":"Interquartile range  LV"}  {"#name":"keyword"  "$":{"id":"kw0115"}  "$$":[{"#name":"text"  "_":"Left Ventricle  MAPCA"}  {"#name":"keyword"  "$":{"id":"kw0125"}  "$$":[{"#name":"text"  "_":"Major aortopulmonary collateral arteries  NYHA"}  {"#name":"keyword"  "$":{"id":"kw0135"}  "$$":[{"#name":"text"  "_":"New York Heart Association  PA"}  {"#name":"keyword"  "$":{"id":"kw0145"}  "$$":[{"#name":"text"  "_":"Pulmonary atesia  PI"}  {"#name":"keyword"  "$":{"id":"kw0155"}  "$$":[{"#name":"text"  "_":"Pulmonary insufficiency  QRS"}  {"#name":"keyword"  "$":{"id":"kw0165"}  "$$":[{"#name":"text"  "_":"QRS duration on 12 lead electrocardiogram  rTOF"}  {"#name":"keyword"  "$":{"id":"kw0175"}  "$$":[{"#name":"text"  "_":"repaired tetralogy of Fallot  RV"}  {"#name":"keyword"  "$":{"id":"kw0185"}  "$$":[{"#name":"text"  "_":"Right ventricle  SV"}  {"#name":"keyword"  "$":{"id":"kw0195"}  "$$":[{"#name":"text"  "_":"Stroke volume  TOF"}  {"#name":"keyword"  "$":{"id":"kw0205"}  "$$":[{"#name":"text"  "_":"Tetralogy of Fallot  VVCR"}  {"#name":"keyword"  "$":{"id":"kw0215"}  "$$":[{"#name":"text"  "_":"Ventricular vascular coupling ratio
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