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
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.
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.
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).
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.
KeywordsPulmonary atresia Pulmonary stenosis Tetralogy of Fallot Cardiovascular magnetic resonance Echocardiography
Jon Detterich was supported by a grant from the National Institutes of Health (5 K23 HL119627-03) during the conduct of this study.
Compliance with Ethical Standards
Conflict of interest
All authors declare no conflicts of interest.
All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.
For this type of study formal consent is not required.
- 1.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 CrossRefGoogle Scholar
- 6.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 CrossRefGoogle Scholar
- 7.Lewis AB, Wells W, Lindesmith GG (1986) Right ventricular growth potential in neonates with pulmonary atresia and intact ventricular septum. J Thorac Cardiovasc Surg 91:835–840Google Scholar
- 8.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 CrossRefGoogle Scholar
- 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 CrossRefGoogle Scholar
- 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 CrossRefGoogle Scholar
- 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 CrossRefGoogle Scholar
- 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 CrossRefGoogle Scholar
- 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 CrossRefGoogle Scholar