Hostname: page-component-8448b6f56d-xtgtn Total loading time: 0 Render date: 2024-04-24T23:19:31.142Z Has data issue: false hasContentIssue false
  • English
  • Français

Post-operative course of pulmonary artery pressure after complete atrioventricular canal defect repair

Part of: Atrioventricular Septal Defect (AVSD) Surgery

Published online by Cambridge University Press:  11 June 2021

Marie Emeline Pierre Louis ,
Adnan Bhutta ,
Adrian Holloway  and
Peter Gaskin Open the ORCID record for Peter Gaskin [Opens in a new window]
Marie Emeline Pierre Louis
Affiliation:
Houston Healthcare Clear Lake, Houston, TX, USA
Adnan Bhutta
Affiliation:
Division of Pediatric Critical Care, University of Maryland School of Medicine, Baltimore, MD, USA
Adrian Holloway
Affiliation:
Division of Pediatric Critical Care, University of Maryland School of Medicine, Baltimore, MD, USA
Peter Gaskin*
Affiliation:
Division of Pediatric Cardiology, University of Maryland School of Medicine, Baltimore, MD, USA
*
Author for correspondence: Peter Gaskin, Division of Pediatric Cardiology, University of Maryland School of Medicine, Baltimore, MD, USA. Tel: 410-328-4348; Fax: 410-328-8670. E-mail: pgaskin@som.umaryland.edu
Get access Rights & Permissions [Opens in a new window]

Abstract

Complete atrioventricular canal defect is a CHD associated with intra-cardiac shunting of blood, which can lead to irreversible changes in pulmonary vascular resistance and pulmonary artery hypertension if unrepaired. Patients with Trisomy 21 are at risk for early development of pulmonary artery hypertension if left uncorrected.1,2

Objectives:

The purpose of this study is to describe the evolution of pulmonary artery hypertension after repair of complete atrioventricular canal defect and to determine the time to normalisation of pulmonary artery pressure in both patients with and without Trisomy 21.

Methods:

This is a single centre, retrospective analysis of patients with complete atrioventricular canal defect admitted for surgical repair at the University of Maryland Medical Center from 2005 to 2015. T-test or Mann–Whitney test and Chi-Square or Fisher’s exact tests were used to compare the two groups (patients with Trisomy 21 and those without). Repeated measures of analysis of variance and serial measurement analysis were performed.

Results:

Twenty-nine patients meeting the inclusion criteria underwent repair of complete atrioventricular defects during the study period. The right ventricular pressure estimate remained elevated over time and did not show a significant difference between the two groups. Right ventricular to systolic blood pressure ratios for all patients remained > 0.5 over the time periods assessed.

Conclusions:

Our study suggests that in patients with complete atrioventricular canal defects, the right ventricular pressure remains elevated and does not normalise on echocardiograms performed up to one year after surgery, suggesting a sustained elevation in pulmonary vascular resistance.

Keywords

Pulmonary vascular resistanceatrioventricular canal defectpulmonary artery hypertension
Type
Original Article
Information
Cardiology in the Young , Volume 32 , Issue 3 , March 2022 , pp. 398 - 404
Check for updates
Copyright
© The Author(s), 2021. Published by Cambridge University Press

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

Sherman, SL, et al. Epidemiology of Down syndrome. Ment Retard Dev Disabil Res Rev 2007; 13: 221–227.10.1002/mrdd.20157CrossRefGoogle ScholarPubMed
Chi, TL, Krovetz, LJ. The pulmonary vascular bed in children with Down syndrome. J Pediatr. 1975; 86: 533–538.10.1016/S0022-3476(75)80142-9CrossRefGoogle Scholar
Freeman, SB, et al. Population- based study of congenital heart defects in Down syndrome. Am J Med Genet. 1998; 80: 213–217.10.1002/(SICI)1096-8628(19981116)80:3<213::AID-AJMG6>3.0.CO;2-83.0.CO;2-8>CrossRefGoogle ScholarPubMed
Cetta, F, Minich, LL. Atrioventricular septal defects. In: Allen, HD and Driscoll, DJ (eds). Moss & Adams’ Heart Disease in Infants, Children and Adolescents, Lippincott Williams and Wilkens, Philadelphia, 2008: 646–667.Google Scholar
Mitchell, SC, Korones, SB, Berendes, HW. Congenital heart disease in 56109 births incidence and natural history. Circulation. 1971; 43: 323–332.10.1161/01.CIR.43.3.323CrossRefGoogle ScholarPubMed
Setty, SP, Shen, I. Atrioventricular septal defects. In: Nichols, D (ed). Critical Heart Disease in Infants and Children, Mosby, Inc., Philadelphia, 2006: 593–608.10.1016/B978-032301281-2.50027-8CrossRefGoogle Scholar
Clapp, S, Perry, BL, Farooki, ZQ. Down’s syndrome, complete atrioventricular cana, l and pulmonary vascular obstruction disease. J Thorac Cardiovasc Surg. 1990; 100: 115–121.10.1016/S0022-5223(19)35606-5CrossRefGoogle Scholar
Freeman, SB, et al. Ethnicity, sex, and the incidence of congenital heart defects: a report from the National Down Syndrome Project. Genet Med. 2008; 10: 173–180.10.1097/GIM.0b013e3181634867CrossRefGoogle ScholarPubMed
Lopes, AA, et al. Repair of congenital heart disease with associated pulmonary hypertension in children. Consensus statement from the Congenital Heart Disease and Pediatric Task forces, Pulmonary Vascular Research Institute (PVRI). Pulm Circ. 2014; 4: 330–341.10.1086/675995CrossRefGoogle Scholar
Yamaki, S, et al. Pulmonary vascular disease and operative indications in complete atrioventricular canal defect in early infancy. J Thorac Cardiovasc Surg. 1993; 106: 398–405.10.1016/S0022-5223(19)34071-1CrossRefGoogle ScholarPubMed
Hals, J, Hagemo, P, Thaulow, E. Pulmonary vascular resistance in complete atrioventricular defect. A comparison between children with and without Down’s syndrome. Acta Pediatr. 1993; 82: 595–598.10.1111/j.1651-2227.1993.tb12763.xCrossRefGoogle ScholarPubMed
Rabinovitch, M, Keane, JF, Norwood, WI. Vascular structure in lung tissue obtained at biopsy correlated with pulmonary hemodynamic findings after repair of congenital heart defects. Circulation. 1984; 69: 655–667.10.1161/01.CIR.69.4.655CrossRefGoogle ScholarPubMed
Rao, PSM, Raju, V, Narayana, M. Flap valved closure of ventricular of ventricular septal defects with increased pulmonary vascular resistance. Interact Cardiovasc Thorac Surg. 2010; 11: 577–580.10.1510/icvts.2010.243865CrossRefGoogle ScholarPubMed
D’Alto, M, Mahadevan, VS. Pulmonary arterial hypertension associated with congenital heart disease. Eur Respir Rev. 2012; 21: 328–337.10.1183/09059180.00004712CrossRefGoogle ScholarPubMed
Kozlik-Feldmann, R, Hansmann, G, Bonnet, D. Pulmonary hypertension in children with congenital heart disease (PAH-CHD, PPHVD-CHD). Expert consensus statement on the diagnosis and treatment of pediatric pulmonary hypertension. The European Paediatric Pulmonary Vascular Disease Network, endorsed by ISHLT and DGPK. Heart. 2016; 102: ii42–ii48.10.1136/heartjnl-2015-308378CrossRefGoogle ScholarPubMed
Newfeld, EA, et al. Pulmonary vascular disease in complete atrioventricular canal defect. Am J Cardiol. 1977; 39: 721–726.10.1016/S0002-9149(77)80135-5CrossRefGoogle ScholarPubMed
D’Alto, M, et al. Hemodynamics of patients developing pulmonary hypertension after shunt closure. Int J Cardiol. 2013; 168: 3797–3801.10.1016/j.ijcard.2013.06.036CrossRefGoogle ScholarPubMed
Frontera-Izquierdo, P, Gabezuelo-Huerta, , G. Natural and modified history of complete atrioventricular septal defect-a 17 year study. Arch Dis Child. 1990; 65: 964–967.10.1136/adc.65.9.964CrossRefGoogle ScholarPubMed
Haworth, SG. Pulmonary hypertension in the young. Heart. 2002; 88: 658–664.10.1136/heart.88.6.658CrossRefGoogle Scholar
Boening, A, et al. Long-term results after surgical correction of atrioventricular septal defects. Eur J Cardio-Thorac Surg. 2002; 22: 167–173.10.1016/S1010-7940(02)00272-5CrossRefGoogle ScholarPubMed
Masuda, M, et al. Does Down syndrome affect the long-term results of complete atrioventricular septal defect when the defect is repaired during the first year of life? Eur J Cardio-Thorac Surg. 2005; 27: 405–409.10.1016/j.ejcts.2004.11.027CrossRefGoogle ScholarPubMed
Yock, PG, Popp, RL. Noninvasive estimation of right ventricular systolic pressure by Doppler ultrasound in patients with tricuspid regurgitation. Circulation. 1984; 70: 657–662.10.1161/01.CIR.70.4.657CrossRefGoogle ScholarPubMed
Currie, PJ, et al. Continuous-wave Doppler echocardiographic assessment of severity of calcific aortic stenosis: a simultaneous Doppler-catheter correlative study in 100 adult patients. Circulation. 1985; 71:1162–1169.10.1161/01.CIR.71.6.1162CrossRefGoogle ScholarPubMed
Parasuraman, S, Walker, S, Loudon, BL, et al. Assessment of pulmonary artery pressure by echocardiography-A comprehensive review. Int J Cardiol Heart Vasc. 2016; 12: 45–51.Google ScholarPubMed
Sharma, M, et al. A study to determine the prevalence of pulmonary arterial hypertension in children with Down syndrome and congenital heart disease. Med J Armed Forces India. 2013; 69: 241–245.10.1016/j.mjafi.2012.11.013CrossRefGoogle ScholarPubMed
Lee, JS, Cha, SG, Kim, GB, et al. Reversibility of pulmonary hypertension following surgical atrial septal defect closure in children with Down syndrome. J Cardiovasc Imaging. 2019; 27: 247–253.10.4250/jcvi.2019.27.e33CrossRefGoogle ScholarPubMed
Latus, H, Wagner, I, Ostermayer, S. Hemodynamic evaluation of children with persistent or recurrent pulmonary arterial hypertension following complete repair of congenital heart disease. Pediatric Cardiol. 2017; 38: 1342–1349.10.1007/s00246-017-1667-9CrossRefGoogle ScholarPubMed
Toth, R, et al. Down syndrome and postoperative complications after pediatric cardiac surgery: a propensity-matched analysis. Interact Cardiovasc Thorac Surg. 2013; 17: 691–697.10.1093/icvts/ivt267CrossRefGoogle Scholar
St Louis, JD, Jodhka, U, Jacobs, JP, Contemporary outcomes of complete atrioventricular septal defect repair: analysis of the Society of Thoracic Surgeons Congenital Heart Surgery Database. J Thorac Cardiovasc Surg. 2014; 148: 2526–2531.10.1016/j.jtcvs.2014.05.095CrossRefGoogle Scholar
Islam, S, Yasui, Y, Kaul, P. Hospital readmission of patients with congenital heart disease in Canada. Can J Cardiol. 2016; 32:987.e7–987.e14.10.1016/j.cjca.2015.12.018CrossRefGoogle ScholarPubMed
Awerbach, JD, Mallory, GB, Kim, S. Hospital readmissions in children with pulmonary hypertension: a multi-institutional analysis. J Pediatr. 2018; 195: 95–101.10.1016/j.jpeds.2017.11.027CrossRefGoogle ScholarPubMed
Pongiglione, G, Possidoni, A, Paparatti, UDL. Incidence of respiratory disease during the first two years of life in children with hemodynamically significant congenital heart disease in Italy: a retrospective study. Pediatr Cardiol. 2016; 37: 1581–1589.10.1007/s00246-016-1473-9CrossRefGoogle ScholarPubMed
Cabalka, AK. Physiologic risk factors for respiratory viral infections and immunoprophylaxis for respiratory syncytial virus for young children with congenital heart disease. Pediatr Infect Dis J. 2004; 23: S41–S45.10.1097/01.inf.0000108220.94201.1aCrossRefGoogle ScholarPubMed
Abman, SH, Hansmann, G, Archer, SL. Pediatric pulmonary hypertension guidelines from the American Heart Association and American Thoracic Society. Circulation. 2015; 132: 2037–2099.10.1161/CIR.0000000000000329CrossRefGoogle ScholarPubMed
Sohail, A, Korejo, HB, Shaikh, AS, et al. Correlation between echocardiography and cardiac catheterization for the assessment of pulmonary hypertension in pediatric patients. Cureus. 2019; 11: e5511.Google ScholarPubMed