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Canadian Journal of Cardiology

Determinants and Prognostic Implications of Left-Heart Filling Pressures in Tetralogy of Fallot

Published:November 11, 2019DOI:https://doi.org/10.1016/j.cjca.2019.10.040

      Abstract

      Background

      Elevated pulmonary artery wedge pressure (PAWP) is the hallmark of left-heart failure and is responsible for heart failure symptoms and mortality. Although PAWP typically correlates with right atrial pressure (RAP), it is primarily dependent on left-heart myocardial properties and volume status. As right-heart disease can occur in the absence of left-heart disease in patients with tetralogy of Fallot (TOF), we hypothesized that RAP was the primary determinant of PAWP in this population.

      Methods

      A cohort study of adults with TOF that underwent right-heart catheterization at Mayo Clinic Rochester (1990 to 2017) to determine the relationship among RAP, PAWP, and mortality.

      Results

      Among 213 patients (male 105; age 37 ± 14 years), the mean PAWP was 14 ± 5 mm Hg, and RAP was 11 ± 5 mm Hg. RAP was the strongest predictor of PAWP (β = 0.68, standard error = 0.06, P < 0.001), independent of left-heart disease and atherosclerotic cardiovascular risk factors. The patients with high PAWP also had normal tissue Doppler velocities, suggesting normal left-ventricular myocardial properties. PAWP was an independent predictor of death/transplant (hazard ration [HR] 1.11, 95% confidence interval [CI], 1.03-1.20, P = 0.004). However, when RAP was incorporated into the regression model, RAP (and not PAWP) became the independent predictor of outcomes (HR 1.14, 95% CI, 1.06-1.22, P = 0.001).

      Conclusions

      The current study showed that RAP was the primary determinant of PAWP and accounts, to some extent, for the mortality in patients with TOF and high PAWP. The data provide new insight in the pathophysiology of disease progression for symptomatic patients with TOF.

      Résumé

      Introduction

      La pression artérielle pulmonaire d’occlusion (PAPO) élevée qui est la caractéristique de l’insuffisance cardiaque gauche est responsable des symptômes de l’insuffisance cardiaque et de la mortalité. Bien qu’il existe généralement une corrélation entre la PAPO et la pression auriculaire droite (PAD), la PAPO dépend principalement des propriétés myocardiques du cœur gauche et du statut volémique. Puisque l’insuffisance cardiaque droite peut survenir en l’absence d’une insuffisance cardiaque gauche chez les patients atteints de la tétralogie de Fallot (TF), nous avons posé l’hypothèse que la PAD était le déterminant principal de la PAPO dans cette population.

      Méthodes

      L’étude de cohorte portait sur des adultes atteints de TF qui avaient subi un cathétérisme du côté droit du cœur à la clinique Mayo de Rochester (de 1990 à 2017) pour déterminer la relation entre la PAD, la PAPO et la mortalité.

      Résultats

      Les 213 patients (105 hommes de 37 ± 14 ans) avaient une PAPO moyenne de 14 ± 5 mmHg et une PAD de 11 ± 5 mmHg. La PAD était le prédicteur le plus fort de la PAPO (β = 0,68, erreur type = 0,06, P < 0,001), indépendamment des facteurs de risque de maladie cardiovasculaire athérosclérotique et d’insuffisance cardiaque gauche. Les patients qui avaient une PAPO élevée avaient aussi des valeurs normales de vélocités au Doppler tissulaire, qui suggèrent des propriétés myocardiques normales du ventricule gauche. La PAPO était un prédicteur indépendant de mortalité/transplantation (rapport de risque [RR] 1,11, intervalle de confiance [IC] à 95 %, 1,03-1,20, P = 0,004). Toutefois, lorsque la PAD était incorporée au modèle de régression, la PAD (et non la PAPO) était le prédicteur indépendant des issues cliniques (RR 1,14, IC à 95 %, 1,06-1,22, P = 0,001).

      Conclusions

      L’étude a montré que la PAD était le déterminant principal de la PAPO et explique, dans une certaine mesure, la mortalité des patients qui avaient la TF et une PAPO élevée. Les données apportent un nouvel éclairage sur la physiopathologie de la progression de la maladie chez les patients symptomatiques atteints de TF.
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      References

        • Budts W.
        • Roos-Hesselink J.
        • Radle-Hurst T.
        • et al.
        Treatment of heart failure in adult congenital heart disease: a position paper of the Working Group of Grown-Up Congenital Heart Disease and the Heart Failure Association of the European Society of Cardiology.
        Eur Heart J. 2016; 37: 1419-1427
        • Gilboa S.M.
        • Devine O.J.
        • Kucik J.E.
        • et al.
        Congenital heart defects in the united states: estimating the magnitude of the affected population in 2010.
        Circulation. 2016; 134: 101-109
        • Zomer A.C.
        • Vaartjes I.
        • van der Velde E.T.
        • et al.
        Heart failure admissions in adults with congenital heart disease; risk factors and prognosis.
        Int J Cardiol. 2013; 168: 2487-2493
        • Holland D.J.
        • Prasad S.B.
        • Marwick T.H.
        Prognostic implications of left ventricular filling pressure with exercise.
        Circ Cardiovasc Imaging. 2010; 3: 149-156
        • Bemis C.E.
        • Serur J.R.
        • Borkenhagen D.
        • Sonnenblick E.H.
        • Urschel C.W.
        Influence of right ventricular filling pressure on left ventricular pressure and dimension.
        Circ Res. 1974; 34: 498-504
        • Ishida Y.
        • Meisner J.S.
        • Tsujioka K.
        • et al.
        Left ventricular filling dynamics: influence of left ventricular relaxation and left atrial pressure.
        Circulation. 1986; 74: 187-196
        • Gilbert J.C.
        • Glantz S.A.
        Determinants of left ventricular filling and of the diastolic pressure-volume relation.
        Circ Res. 1989; 64: 827-852
        • Reddy Y.N.V.
        • Obokata M.
        • Egbe A.
        • et al.
        Left atrial strain and compliance in the diagnostic evaluation of heart failure with preserved ejection fraction.
        Eur J Heart Fail. 2019; 21: 891-900
        • Obokata M.
        • Borlaug B.A.
        Left ventricular filling pressures in heart failure with preserved ejection fraction: is the tail now wagging the dog?.
        JACC Heart Fail. 2017; 5: 802-804
        • Tyberg J.V.
        • Taichman G.C.
        • Smith E.R.
        • Douglas N.W.
        • Smiseth O.A.
        • Keon W.J.
        The relationship between pericardial pressure and right atrial pressure: an intraoperative study.
        Circulation. 1986; 73: 428-432
        • Cullen S.
        • Shore D.
        • Redington A.
        Characterization of right ventricular diastolic performance after complete repair of tetralogy of Fallot: restrictive physiology predicts slow postoperative recovery.
        Circulation. 1995; 91: 1782-1789
        • Gatzoulis M.A.
        • Clark A.L.
        • Cullen S.
        • Newman C.G.
        • Redington A.N.
        Right ventricular diastolic function 15 to 35 years after repair of tetralogy of Fallot: restrictive physiology predicts superior exercise performance.
        Circulation. 1995; 91: 1775-1781
        • Bokma J.P.
        • Winter M.M.
        • Kuijpers J.M.
        • et al.
        Role of acquired cardiovascular disease in tetralogy of fallot patients >50 years of age.
        J Am Coll Cardiol. 2017; 69: 2465-2466
        • Mascherbauer J.
        • Zotter-Tufaro C.
        • Duca F.
        • et al.
        wedge pressure rather than left ventricular end-diastolic pressure predicts outcome in heart failure with preserved ejection fraction.
        JACC Heart Fail. 2017; 5: 795-801
        • Miranda W.R.
        • Egbe A.C.
        • Hagler D.J.
        • et al.
        Filling pressures in Fontan revisited: comparison between pulmonary artery wedge, ventricular end-diastolic, and left atrial pressures in adults.
        Int J Cardiol. 2018; 255: 32-36
        • Miranda W.R.
        • Borlaug B.A.
        • Hagler D.J.
        • Connolly H.M.
        • Egbe A.C.
        Haemodynamic profiles in adult Fontan patients: associated haemodynamics and prognosis.
        Eur J Heart Fail. 2019; 21: 803-809
        • LaFarge C.G.
        • Miettinen O.S.
        The estimation of oxygen consumption.
        Cardiovasc Res. 1970; 4: 23-30
        • Gorter T.M.
        • Obokata M.
        • Reddy Y.N.V.
        • Melenovsky V.
        • Borlaug B.A.
        Exercise unmasks distinct pathophysiologic features in heart failure with preserved ejection fraction and pulmonary vascular disease.
        Eur Heart J. 2018; 39: 2825-2835
        • Obokata M.
        • Reddy Y.N.V.
        • Pislaru S.V.
        • Melenovsky V.
        • Borlaug B.A.
        Evidence supporting the existence of a distinct obese phenotype of heart failure with preserved ejection fraction.
        Circulation. 2017; 136: 6-19
        • Drazner M.H.
        • Hamilton M.A.
        • Fonarow G.
        • Creaser J.
        • Flavell C.
        • Stevenson L.W.
        Relationship between right and left-sided filling pressures in 1000 patients with advanced heart failure.
        J Heart Lung Transplant. 1999; 18: 1126-1132
        • Drazner M.H.
        • Prasad A.
        • Ayers C.
        • et al.
        The relationship of right- and left-sided filling pressures in patients with heart failure and a preserved ejection fraction.
        Circ Heart Fail. 2010; 3: 202-206
        • Drazner M.H.
        • Brown R.N.
        • Kaiser P.A.
        • et al.
        Relationship of right- and left-sided filling pressures in patients with advanced heart failure: a 14-year multi-institutional analysis.
        J Heart Lung Transplant. 2012; 31: 67-72
        • Sabate Rotes A.
        • Eidem B.W.
        • Connolly H.M.
        • et al.
        Long-term follow-up after pulmonary valve replacement in repaired tetralogy of Fallot.
        Am J Cardiol. 2014; 114: 901-908
        • Schwartz M.C.
        • Rome J.J.
        • Gillespie M.J.
        • et al.
        Relation of left ventricular end diastolic pressure to right ventricular end diastolic volume after operative treatment of tetralogy of Fallot.
        Am J Cardiol. 2012; 109: 417-422
        • Khairy P.
        • Harris L.
        • Landzberg M.J.
        • et al.
        Implantable cardioverter-defibrillators in tetralogy of Fallot.
        Circulation. 2008; 117: 363-370
        • Smiseth O.A.
        • Refsum H.
        • Junemann M.
        • et al.
        Ventricular diastolic pressure-volume shifts during acute ischemic left ventricular failure in dogs.
        J Am Coll Cardiol. 1984; 3: 966-977
        • Smiseth O.A.
        • Frais M.A.
        • Kingma I.
        • et al.
        Assessment of pericardial constraint: the relation between right ventricular filling pressure and pericardial pressure measured after pericardiocentesis.
        J Am Coll Cardiol. 1986; 7: 307-314
        • Janicki J.S.
        • Weber K.T.
        The pericardium and ventricular interaction, distensibility, and function.
        Am J Physiol. 1980; 238: H494-H503
        • Yancy C.W.
        • Jessup M.
        • Bozkurt B.
        • et al.
        2013 ACCF/AHA guideline for the management of heart failure: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines.
        J Am Coll Cardiol. 2013; 62: e147-e239
        • Ponikowski P.
        • Voors A.A.
        • Anker S.D.
        • et al.
        2016 ESC Guidelines for the diagnosis and treatment of acute and chronic heart failure: the Task Force for the diagnosis and treatment of acute and chronic heart failure of the European Society of Cardiology (ESC) developed with the special contribution of the Heart Failure Association (HFA) of the ESC.
        Eur Heart J. 2016; 37: 2129-2200
        • El-Harasis M.A.
        • Connolly H.M.
        • Miranda W.R.
        • et al.
        Progressive right ventricular enlargement due to pulmonary regurgitation: clinical characteristics of a "low-risk" group.
        Am Heart J. 2018; 201: 136-140