nach oben

Pediatric Cardiology

Erschienen in:

23.10.2018 | Original Article

Differences in Right Ventricular Physiologic Response to Chronic Volume Load in Patients with Repaired Pulmonary Atresia Intact Ventricular Septum/Critical Pulmonary Stenosis Versus Tetralogy of Fallot

verfasst von: Andrew L. Cheng, Abraham M. Kaslow, Jay D. Pruetz, Jimmy C. Lu, John C. Wood, Jon A. Detterich

Erschienen in: Pediatric Cardiology | Ausgabe 3/2019

Einloggen, um Zugang zu erhalten

Abstract

Background

Patients with pulmonary atresia with intact ventricular septum and critical pulmonary stenosis (PAIVS/CPS) have wide variation in right ventricle (RV) size, systolic function, and diastolic function at birth. Establishment of antegrade pulmonary blood flow creates the potential for RV dilation from chronic pulmonary insufficiency. Future surgical decisions are based on RV size and function, largely supported by longitudinal studies of patients with Tetralogy of Fallot (TOF). Given potential differences in RV physiology and lack of similar data in PAIVS/CPS, the objective of this study was to determine differences in RV size, systolic function, and diastolic function between patients with PAIVS/CPS versus TOF.

Methods

We retrospectively collected cardiovascular magnetic resonance (CMR) data in 27 patients with PAIVS/CPS (ages 13.3 ± 8.8 years) and 78 with TOF (11.4 ± 5.4 years). RV volumes, ejection fraction (EF), regurgitant fraction, end-diastolic forward flow across the pulmonary valve, and right atrial cross-sectional area were calculated.

Results

There was no difference between the groups in RV end-diastolic volume (RVEDVi), RVEF, or pulmonary regurgitation. RVEF tended to decrease in TOF when RVEDVi exceeded 164 ml/m². In PAIVS/CPS, RVEDVi less frequently reached 164 ml/m² and was not associated with RVEF. There was worse RV diastolic dysfunction in PAIVS/CPS, with 1.5 times larger right atrial area and two times higher pulmonary end-diastolic forward flow (p < 0.0001).

Conclusions

Patients with PAIVS/CPS have similar RV size, systolic function, and pulmonary regurgitation as TOF. However, impaired RV diastolic function may limit extremes of RV dilatation and impact long-term management of PAIVS/CPS.

Gersony WM, Bernhard WF, Nadas AS, Gross RE (1967) Diagnosis and surgical treatment of infants with critical pulmonary outflow obstruction. Study of thirty-four infants with pulmonary stenosis or atresia, and intact ventricular septum. Circulation 35:765–776. https://doi.org/10.1161/01.CIR.35.4.765 CrossRefPubMed

Ashburn DA, Blackstone EH, Wells WJ et al (2004) Determinants of mortality and type of repair in neonates with pulmonary atresia and intact ventricular septum. J Thorac Cardiovasc Surg 127:1000–1008. https://doi.org/10.1016/j.jtcvs.2003.11.057 CrossRefPubMed

Jahangiri M, Zurakowski D, Bichell D et al (1999) Improved results with selective management in pulmonary atresia with intact ventricular septum. J Thorac Cardiovasc Surg 118:1046–1055CrossRefPubMed

Sano S, Ishino K, Kawada M et al (2000) Staged biventricular repair of pulmonary atresia or stenosis with intact ventricular septum. Ann Thorac Surg 70:1501–1506CrossRefPubMed

Yoshimura N, Yamaguchi M (2009) Surgical strategy for pulmonary atresia with intact ventricular septum: initial management and definitive surgery. Gen Thorac Cardiovasc Surg 57:338–346CrossRefPubMed

Alwi M, Geetha K, Bilkis AA et al (2000) Pulmonary atresia with intact ventricular septum percutaneous radiofrequency-assisted valvotomy and balloon dilation versus surgical valvotomy and Blalock Taussig shunt. J Am Coll Cardiol 35:468–476. https://doi.org/10.1016/S0735-1097(99)00549-5 CrossRefPubMed

Lewis AB, Wells W, Lindesmith GG (1986) Right ventricular growth potential in neonates with pulmonary atresia and intact ventricular septum. J Thorac Cardiovasc Surg 91:835–840PubMed

Ovaert C, Qureshi SA, Rosenthal E et al (1998) Growth of the right ventricle after successful transcatheter pulmonary valvotomy in neonates and infants with pulmonary atresia and intact ventricular septum. J Thorac Cardiovasc Surg 115:1055–1062. https://doi.org/10.1016/S0022-5223(98)70405-2 CrossRefPubMed

Rao PS, Liebman J, Borkat G (1976) Right ventricular growth in a case of pulmonic stenosis with intact ventricular septum and hypoplastic right ventricle. Circulation 53:389–394. https://doi.org/10.1161/01.CIR.53.2.389 CrossRefPubMed

10.

Karamlou T, Poynter JA, Walters HL et al (2013) Long-term functional health status and exercise test variables for patients with pulmonary atresia with intact ventricular septum: a Congenital Heart Surgeons Society study. J Thorac Cardiovasc Surg 145:1018–1027.e3. https://doi.org/10.1016/j.jtcvs.2012.11.092 CrossRefPubMed

11.

Geva T (2013) Indications for pulmonary valve replacement in repaired tetralogy of Fallot: the quest continues. Circulation 128:1855–1857CrossRefPubMedPubMedCentral

12.

Geva T (2011) Repaired tetralogy of Fallot: the roles of cardiovascular magnetic resonance in evaluating pathophysiology and for pulmonary valve replacement decision support. J Cardiovasc Magn Reson 13:9CrossRefPubMedPubMedCentral

13.

Wald RM, Valente AM, Gauvreau K et al (2015) Cardiac magnetic resonance markers of progressive RV dilation and dysfunction after tetralogy of Fallot repair. Heart 101:1724–1730. https://doi.org/10.1136/heartjnl-2015-308014 CrossRefPubMed

14.

Sallach JA, Tang WHW, Borowski AG et al (2009) Right atrial volume index in chronic systolic heart failure and prognosis. JACC Cardiovasc Imaging 2:527–534. https://doi.org/10.1016/j.jcmg.2009.01.012 CrossRefPubMed

15.

Friedberg MK, Fernandes FP, Roche SL et al (2012) Impaired right and left ventricular diastolic myocardial mechanics and filling in asymptomatic children and adolescents after repair of tetralogy of Fallot. Eur Heart J Cardiovasc Imaging 13:905–913. https://doi.org/10.1093/ehjci/jes067 CrossRefPubMed

16.

Helbing WA, Niezen RA, Cessie SLE et al (1997) Right ventricular diastolic function in children with pulmonary regurgitation after repair of tetralogy of Fallot: volumetric evaluation by magnetic resonance velocity mapping. J Am Coll Cardiol 28:1827–1835. https://doi.org/10.1016/S0735-1097(96)00387-7 CrossRef

17.

Lai WW, Geva T, Shirali GS et al (2006) Guidelines and standards for performance of a pediatric echocardiogram: a report from the task force of the pediatric council of the American Society of Echocardiography. J Am Soc Echocardiogr 19:1413–1430. https://doi.org/10.1016/j.echo.2006.09.001 CrossRefPubMed

18.

Oosthoek PW, Moorman AFM, Sauer U, Gittenberger-de Groot AC (1995) Capillary distribution in the ventricles of hearts with pulmonary atresia and intact ventricular septum. Circulation 91:1790–1798. https://doi.org/10.1161/01.CIR.91.6.1790 CrossRefPubMed

19.

Bulkley BH, D’Amico B, Taylor AL (1983) Extensive myocardial fiber disarray in aortic and pulmonary atresia. Relevance to hypertrophic cardiomyopathy. Circulation 67:191–198. https://doi.org/10.1161/01.CIR.67.1.191 CrossRefPubMed

20.

Patel RG, Freedom RM, Moes CA et al (1980) Right ventricular volume determinations in 18 patients with pulmonary atresia and intact ventricular septum. Analysis of factors influencing right ventricular growth. Circulation 61:428–440CrossRefPubMed

21.

Knauth AL, Gauvreau K, Powell AJ et al (2008) Ventricular size and function assessed by cardiac MRI predict major adverse clinical outcomes late after tetralogy of Fallot repair. Heart 94:211–216. https://doi.org/10.1136/hrt.2006.104745 CrossRefPubMed

22.

Geva T, Sandweiss BM, Gauvreau K et al (2004) Factors associated with impaired clinical status in long-term survivors of tetralogy of Fallot repair evaluated by magnetic resonance imaging. J Am Coll Cardiol 43:1068–1074. https://doi.org/10.1016/j.jacc.2003.10.045 CrossRefPubMed

23.

Valente AM, Cook S, Festa P et al (2014) Multimodality imaging guidelines for patients with repaired tetralogy of Fallot: a report from the American society of echocardiography: developed in collaboration with the society for cardiovascular magnetic resonance and the society for pediatric radiol. J Am Soc Echocardiogr 27:111–141. https://doi.org/10.1016/j.echo.2013.11.009 CrossRefPubMed

24.

Hoashi T, Kagisaki K, Kitano M et al (2012) Late clinical features of patients with pulmonary atresia or critical pulmonary stenosis with intact ventricular septum after biventricular repair. Ann Thorac Surg 94:833–841. https://doi.org/10.1016/j.athoracsur.2012.04.071 CrossRefPubMed

25.

Puranik R, Tsang V, Lurz P et al (2012) Long-term importance of right ventricular outflow tract patch function in patients with pulmonary regurgitation. J Thorac Cardiovasc Surg 143:1103–1107. https://doi.org/10.1016/j.jtcvs.2011.09.039 CrossRefPubMed

26.

Zdradzinski MJ, Qureshi AM, Stewart R et al (2014) Comparison of long-term postoperative sequelae in patients with tetralogy of Fallot versus isolated pulmonic stenosis. Am J Cardiol 114:300–304CrossRefPubMedPubMedCentral

27.

Joynt MR, Yu S, Dorfman AL et al (2016) Differential impact of pulmonary regurgitation on patients with surgically repaired pulmonary stenosis versus tetralogy of Fallot. Am J Cardiol 117:289–294. https://doi.org/10.1016/j.amjcard.2015.10.025 CrossRefPubMed

28.

Lam YY, Kaya MG, Goktekin O et al (2007) Restrictive right ventricular physiology. Its presence and symptomatic contribution in patients with pulmonary valvular stenosis. J Am Coll Cardiol 50:1491–1497. https://doi.org/10.1016/j.jacc.2007.06.042 CrossRefPubMed

29.

Luijnenburg SE, Peters RE, Van Der Geest RJ et al (2013) Abnormal right atrial and right ventricular diastolic function relate to impaired clinical condition in patients operated for tetralogy of Fallot. Int J Cardiol 167:833–839. https://doi.org/10.1016/j.ijcard.2012.02.011 CrossRefPubMed

30.

Geva T (2014) MRI is the preferred method for evaluating right ventricular size and function in patients with congenital heart disease. Circ Cardiovasc Imaging 7:190–197. https://doi.org/10.1161/CIRCIMAGING.113.000553 CrossRefPubMedPubMedCentral

Titel: Differences in Right Ventricular Physiologic Response to Chronic Volume Load in Patients with Repaired Pulmonary Atresia Intact Ventricular Septum/Critical Pulmonary Stenosis Versus Tetralogy of Fallot
verfasst von: Andrew L. Cheng
Abraham M. Kaslow
Jay D. Pruetz
Jimmy C. Lu
John C. Wood
Jon A. Detterich
Publikationsdatum: 23.10.2018
Verlag: Springer US
Erschienen in: Pediatric Cardiology / Ausgabe 3/2019
Print ISSN: 0172-0643
Elektronische ISSN: 1432-1971
DOI: https://doi.org/10.1007/s00246-018-2009-2

Neu im Fachgebiet Kardiologie

19.04.2024 | Vorhofflimmern | Nachrichten

Update Kardiologie

Bestellen Sie unseren Fach-Newsletter und bleiben Sie gut informiert.

Newsletter bestellen

Live-Webinar: Aktuelle Leitlinien bei Herz-Kreislauf-Erkrankungen

Springer Medizin

Differences in Right Ventricular Physiologic Response to Chronic Volume Load in Patients with Repaired Pulmonary Atresia Intact Ventricular Septum/Critical Pulmonary Stenosis Versus Tetralogy of Fallot

Abstract

Background

Methods

Results

Conclusions

Neu im Fachgebiet Kardiologie

Parodontalbehandlung verbessert Prognose bei Katheterablation

Antihypertensiva schützen auch alte Menschen noch vor Demenz

Sind Betablocker auch für ältere herzschwache Personen nützlich?

Stillende Frauen haben geringeres kardiovaskuläres Risiko

Update Kardiologie

Live-Webinar: Aktuelle Leitlinien bei Herz-Kreislauf-Erkrankungen

Springer Medizin

Abstract

Background

Methods

Results

Conclusions

Bitte loggen Sie sich ein, um Zugang zu diesem Inhalt zu erhalten

Weitere Artikel der Ausgabe 3/2019

Extubation in the Operating Room After Fontan Procedure: Does It Make a Difference?

The Transjugular Approach: An Alternative Route to Improve Ablation Success in Right Anteriorly and Anterolaterally-Located Supraventricular Tachycardia Substrates in Children

Artificial Intelligence-Assisted Auscultation of Heart Murmurs: Validation by Virtual Clinical Trial

Z-Score Reference Ranges for Fetal Heart Functional Measurements in a Large Brazilian Pregnant Women Sample

Association of miR-146a Gene Polymorphism at loci rs2910164 G/C, rs57095329 A/G, and rs6864584 T/C with Susceptibility to Kawasaki Disease in Chinese Children

Can Abbreviated Cardiac Magnetic Resonance Imaging Adequately Support Clinical Decision Making After Repair of Tetralogy of Fallot?

Neu im Fachgebiet Kardiologie

Parodontalbehandlung verbessert Prognose bei Katheterablation

Antihypertensiva schützen auch alte Menschen noch vor Demenz

Sind Betablocker auch für ältere herzschwache Personen nützlich?

Stillende Frauen haben geringeres kardiovaskuläres Risiko

Update Kardiologie