Advertisement

Assessment and Implications of Right Ventricular Afterload in Tetralogy of Fallot

Published:September 09, 2019DOI:https://doi.org/10.1016/j.amjcard.2019.08.035
      Patients with tetralogy of Fallot (TOF) have abnormal right ventricular (RV) afterload because of residual or recurrent outflow tract obstruction, often with abnormal pulmonary artery (PA) vascular function. The purpose of this study was to determine if RV afterload was independently associated with death and/or heart transplant in patients with TOF. This is a retrospective study of TOF patients that underwent cardiac catheterization for clinical indications at Mayo clinic between 1990 and 2015. Invasively measured RV systolic pressure (RVSP) was used to define RV afterload. To explore clinical utility for echocardiographic estimates of invasive data, correlations between invasive and Doppler-derived indices of RV afterload were examined. Among 266 patients with TOF (age 35 ± 14 years, TOF-pulmonary atresia 117 [44%]), RVSP was 72 ± 28 mm Hg, PA systolic pressure 45 ± 19 mm Hg, mean PA pressure 27 ± 10 mm Hg, pulmonary vascular resistance 4.2 ± 3.1 WU, and PA wedge pressure 14 ± 5 mm Hg. Over a mean follow up of 12.9 years, there were 35 deaths and 4 heart transplants. Invasively measured RVSP (hazard ratio 1.25, 95% confidence interval 1.12 to 1.37; p <0.001) and TOF-pulmonary atresia (hazard ratio 1.18, 95% confidence interval 1.08 to 1.41; p = 0.023) were independent risk factors for death and/or transplant. Doppler-derived RVSP was well-correlated with invasive RVSP (r = 0.92, p <0.001), and was also independently associated with the combined end point. RVSP, a composite measure of RV afterload, is independently prognostic in patients with TOF, and can be reliably assessed using Doppler echocardiography. Further study is required to test whether interventions to reduce RVSP can improve outcomes in patients with TOF.
      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'

      Subscribe:

      Subscribe to American Journal of Cardiology
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect

      References

        • Wald RM
        • Valente AM
        • Gauvreau K
        • Babu-Narayan SV
        • Assenza GE
        • Schreier J
        • Gatzoulis MA
        • Kilner PJ
        • Koyak Z
        • Mulder B
        • Powell AJ
        • Geva T
        Cardiac magnetic resonance markers of progressive RV dilation and dysfunction after tetralogy of Fallot repair.
        Heart. 2015; 101: 1724-1730
        • Oosterhof T
        • van Straten A
        • Vliegen HW
        • Meijboom FJ
        • van Dijk AP
        • Spijkerboer AM
        • Bouma BJ
        • Zwinderman AH
        • Hazekamp MG
        • de Roos A
        • Mulder BJ
        Preoperative thresholds for pulmonary valve replacement in patients with corrected tetralogy of Fallot using cardiovascular magnetic resonance.
        Circulation. 2007; 116: 545-551
        • Nollert G
        • Fischlein T
        • Bouterwek S
        • Bohmer C
        • Klinner W
        • Reichart B
        Long-term survival in patients with repair of tetralogy of Fallot: 36-year follow-up of 490 survivors of the first year after surgical repair.
        J Am Coll Cardiol. 1997; 30: 1374-1383
        • Geva T.
        Is MRI the preferred method for evaluating right ventricular size and function in patients with congenital heart disease?: MRI is the preferred method for evaluating right ventricular size and function in patients with congenital heart disease.
        Circ Cardiovasc Imaging. 2014; 7: 190-197
        • Geva T.
        Repaired tetralogy of Fallot: the roles of cardiovascular magnetic resonance in evaluating pathophysiology and for pulmonary valve replacement decision support.
        J Cardiovasc Magn Reson. 2011; 13: 9-10
        • Tang D
        • Yang C
        • Geva T
        • del Nido PJ
        Right ventricular local longitudinal curvature as a marker and predictor for pulmonary valve replacement surgery outcome: an initial study based on preoperative and postoperative cardiac magnetic resonance data from patients with repaired tetralogy of Fallot.
        J Thoracic Cardiovasc Surg. 2014; 147: 537-538
        • Bokma JP
        • Winter MM
        • Oosterhof T
        • Vliegen HW
        • van Dijk AP
        • Hazekamp MG
        • Koolbergen DR
        • Groenink M
        • Mulder BJ
        • Bouma BJ
        Preoperative thresholds for mid-to-late haemodynamic and clinical outcomes after pulmonary valve replacement in tetralogy of Fallot.
        Eur Heart J. 2016; 37: 829-835
        • Latus H
        • Binder W
        • Kerst G
        • Hofbeck M
        • Sieverding L
        • Apitz C
        Right ventricular-pulmonary arterial coupling in patients after repair of tetralogy of Fallot.
        The J Thoracic Cardiovasc Surg. 2013; 146: 1366-1372
        • Kilner PJ
        • Balossino R
        • Dubini G
        • Babu-Narayan SV
        • Taylor AM
        • Pennati G
        • Migliavacca F
        Pulmonary regurgitation: the effects of varying pulmonary artery compliance, and of increased resistance proximal or distal to the compliance.
        Int J Cardiol. 2009; 133: 157-166
        • Bedard E
        • McCarthy KP
        • Dimopoulos K
        • Giannakoulas G
        • Gatzoulis MA
        • Ho SY
        Structural abnormalities of the pulmonary trunk in tetralogy of Fallot and potential clinical implications: a morphological study.
        J Am Coll of Cardiol. 2009; 54: 1883-1890
        • LaFarge CG
        • Miettinen OS
        The estimation of oxygen consumption.
        Cardiovasc Res. 1970; 4: 23-30
        • Lang RM
        • Badano LP
        • Mor-Avi V
        • Afilalo J
        • Armstrong A
        • Ernande L
        • Flachskampf FA
        • Foster E
        • Goldstein SA
        • Kuznetsova T
        • Lancellotti P
        • Muraru D
        • Picard MH
        • Rietzschel ER
        • Rudski L
        • Spencer KT
        • Tsang W
        • Voigt JU
        Recommendations for cardiac chamber quantification by echocardiography in adults: an update from the American Society of Echocardiography and the European Association of Cardiovascular Imaging.
        J Am Soc Echocardiogr. 2015; 28 (e14): 1-39
        • Abbas AE
        • Franey LM
        • Marwick T
        • Maeder MT
        • Kaye DM
        • Vlahos AP
        • Serra W
        • Al-Azizi K
        • Schiller NB
        • Lester SJ
        Noninvasive assessment of pulmonary vascular resistance by Doppler echocardiography.
        J Am Soc Echocardiogr. 2013; 26: 1170-1177
        • Nagueh SF
        • Smiseth OA
        • Appleton CP
        • Byrd 3rd, BF
        • Dokainish H
        • Edvardsen T
        • Flachskampf FA
        • Gillebert TC
        • Klein AL
        • Lancellotti P
        • Marino P
        • Oh JK
        • Popescu BA
        • Waggoner AD
        Recommendations for the evaluation of left ventricular diastolic function by echocardiography: an update from the American Society of Echocardiography and the European Association of Cardiovascular Imaging.
        J Am Soc Echocardiogr. 2016; 29: 277-314
        • Reddy YNV
        • Andersen MJ
        • Obokata M
        • Koepp KE
        • Kane GC
        • Melenovsky V
        • Olson TP
        • Borlaug BA
        Arterial stiffening with exercise in patients with heart failure and preserved ejection fraction.
        J Am Coll Cardiol. 2017; 70: 136-148
        • Melenovsky V
        • Hwang SJ
        • Lin G
        • Redfield MM
        • Borlaug BA
        Right heart dysfunction in heart failure with preserved ejection fraction.
        Eur Heart J. 2014; 35: 3452-3462
        • Khairy P
        • Aboulhosn J
        • Gurvitz MZ
        • Opotowsky AR
        • Mongeon FP
        • Kay J
        • Valente AM
        • Earing MG
        • Lui G
        • Gersony DR
        • Cook S
        • Ting JG
        • Nickolaus MJ
        • Webb G
        • Landzberg MJ
        • Broberg CS
        • Alliance for Adult Research in Congenital C
        Arrhythmia burden in adults with surgically repaired tetralogy of Fallot: a multi-institutional study.
        Circulation. 2010; 122: 868-875
        • Valente AM
        • Gauvreau K
        • Assenza GE
        • Babu-Narayan SV
        • Schreier J
        • Gatzoulis MA
        • Groenink M
        • Inuzuka R
        • Kilner PJ
        • Koyak Z
        • Landzberg MJ
        • Mulder B
        • Powell AJ
        • Wald R
        • Geva T
        Contemporary predictors of death and sustained ventricular tachycardia in patients with repaired tetralogy of Fallot enrolled in the INDICATOR cohort.
        Heart. 2014; 100: 247-253
        • Bokma JP
        • Geva T
        • Sleeper LA
        • Babu Narayan SV
        • Wald R
        • Hickey K
        • Jansen K
        • Wassall R
        • Lu M
        • Gatzoulis MA
        • Mulder BJ
        • Valente AM
        A propensity score-adjusted analysis of clinical outcomes after pulmonary valve replacement in tetralogy of Fallot.
        Heart. 2018; 104: 738-744
        • Geva T
        • Mulder B
        • Gauvreau K
        • Babu-Narayan SV
        • Wald R
        • Hickey K
        • Powell AJ
        • Gatzoulis MA
        • Valente AM
        Preoperative predictors of death and sustained ventricular tachycardia after pulmonary valve replacement in patients with repaired tetralogy of Fallot enrolled in the INDICATOR cohort.
        Circulation. 2018; 138: 2106-2115
        • Borlaug BA
        • Koepp KE
        • Melenovsky V
        Sodium nitrite improves exercise hemodynamics and ventricular performance in heart failure with preserved ejection fraction.
        J Am Coll Cardiol. 2015; 66: 1672-1682
        • Borlaug BA
        • Melenovsky V
        • Koepp KE
        Inhaled sodium nitrite improves rest and exercise hemodynamics in heart failure with preserved ejection fraction.
        Circ Research. 2016; 119: 880-886
        • Tapson VF
        • Torres F
        • Kermeen F
        • Keogh AM
        • Allen RP
        • Frantz RP
        • Badesch DB
        • Frost AE
        • Shapiro SM
        • Laliberte K
        • Sigman J
        • Arneson C
        • Galie N
        Oral treprostinil for the treatment of pulmonary arterial hypertension in patients on background endothelin receptor antagonist and/or phosphodiesterase type 5 inhibitor therapy (the FREEDOM-C study): a randomized controlled trial.
        Chest. 2012; 142: 1383-1390