Surgical management of the complete atrioventricular septal defect: literature review and case report
DOI:
https://doi.org/10.18203/2349-2902.isj20263354Keywords:
Complete atrioventricular septal defect, Congenital heart defect, Down syndrome, Pulmonary hypertension, Left-to-right shuntAbstract
Complete atrioventricular septal defect is a complex congenital cardiac defect caused by failure of septal separation, frequently associated with Down syndrome. It is considered a significant challenge in terms of diagnostic and therapeutic management. Our work aims to provide an update on the clinical and therapeutic aspects of the complete AVSD managed by our team at University Hospital Ibn Sina of Rabat, highlighting the importance of developing pediatric cardiac surgery in our Moroccan healthcare system. A retrospective descriptive study involved 20 patients who underwent surgery for complete AVSD between January 2020 and December 2023. Medical records were used to collect clinical, echocardiographic, surgical, and follow-up data. The median age at surgery was 7.5 months, and 90% of the patients had Down syndrome. Among the 20 included patients, 19 survived the intervention, yielding a survival rate of 95%. Postoperative complications included transient severe pulmonary hypertension in 4 patients (20%), moderate residual mitral insufficiency in 6 patients (30%), and rare complications such as septic shock and chylothorax. The single-patch repair technique was employed in 95% of cases. Complete CAV requires a multidisciplinary approach. This study highlights the importance of early diagnosis and timely surgical intervention in improving clinical outcomes and reducing morbidity and mortality.
References
Chauvaud S. Canal atrioventriculaire. EMC Tech Chir Thorax. 2012;7(2):1-17.
Calabrò R, Limongelli G. Complete atrioventricular canal. Orphanet J Rare Dis. 2006;1:8.
Jacobs JP, Burke RP, Quintessenza JA, Mavroudis C. Congenital Heart Surgery Nomenclature and Database Project: atrioventricular canal defect. Ann Thorac Surg. 2000;69(4 Suppl):S36-43.
Pfister R, Myers PO, Hosseinpour AR, Kirsch M, Nowacka A, di Bernardo S, et al. Complete atrioventricular septal defect: modified 2-patch technique. Multimed Man Cardiothorac Surg. 2022;2022:103.
Singh RR, Warren PS, Reece TB, Ellman P, Peeler BB, Kron IL. Early repair of complete atrioventricular septal defect is safe and effective. Ann Thorac Surg. 2006;82(5):1598-602.
Craig B. Atrioventricular septal defect: from fetus to adult. Heart. 2006;92(12):1879-85.
Backer CL, Mavroudis C. Atrioventricular canal defects. In: Pediatric cardiac surgery. Wiley; 2013. p.342-60.
Agarwal Gupta N, Kabra M. Diagnosis and management of Down syndrome. Indian J Pediatr. 2014;81(6):560-7.
D'Alto M, Mahadevan VS. Pulmonary arterial hypertension associated with congenital heart disease. Eur Respir Rev. 2012;21(126):328-37.
Diogenes TCP, Mourato FA, de Lima Filho JL, Mattos SS. Gender differences in the prevalence of congenital heart disease in Down's syndrome: a brief meta-analysis. BMC Med Genet. 2017;18:111.
Majani NG, Koster JR, Kalezi ZE, Letara N, Nkya D, Mongela S, et al. Spectrum of heart diseases in children in a national cardiac referral center Tanzania, Eastern Africa: a six-year overview. Glob Heart. 2024;19(1):61.
Crawford FA. Atrioventricular canal: single-patch technique. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2007;10(1):11-20.
Abdelrehim AR, Alnajjar A, Syed A, Ashry M, Soliman RFB. Outcomes of complete atrioventricular septal defect surgical repair in Down patients. Egypt J Hosp Med. 2021;84(1):2184-91.
St Louis JD, Jodhka U, Jacobs JP, He X, Hill KD, Pasquali SK, et al. 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(6):2526-31.
Lindberg L, Olsson AK, Jögi P, Jonmarker C. How common is severe pulmonary hypertension after pediatric cardiac surgery? J Thorac Cardiovasc Surg. 2002;123(6):1155-63.
Prifti E. Repair of complete atrioventricular septal defect with tetralogy of Fallot. Transl Pediatr. 2017;6(1):1-7.
Iyer PU, Moreno GE, Caneo LF, Faiz T, Shekerdemian LS, Iyer KS. Management of late presentation congenital heart disease. Cardiol Young. 2017;27(S6):S31-9.
Nguyen N, Leon-Wyss J, Iyer KS, Pezzella AT. Paediatric cardiac surgery in low-income and middle-income countries: a continuing challenge. Arch Dis Child. 2015;100(12):1156-9.
Curran RD, Mavroudis C, Backer CL, Sautel M, Zales VR, Wessel DL. Inhaled nitric oxide for children with congenital heart disease and pulmonary hypertension. Ann Thorac Surg. 1995;60(6):1765-71.
Miller OI, Tang SF, Keech A, Pigott NB, Beller E, Celermajer DS. Inhaled nitric oxide and prevention of pulmonary hypertension after congenital heart surgery: a randomised double-blind study. Lancet. 2000;356(9240):1464-9.
Shi T, McAllister DA, O'Brien KL, Simoes EAF, Madhi SA, Gessner BD, et al. Global, regional, and national disease burden estimates of acute lower respiratory infections due to respiratory syncytial virus in young children in 2015: a systematic review and modelling study. Lancet. 2017;390(10098):946-58.
Lozano-Espinosa DA, Huertas-Quiñones VM, Rodríguez-Martínez CE. Impact of pulmonary hypertension and congenital heart disease with hemodynamic repercussion on the severity of acute respiratory infections in children under 5 years of age at a pediatric referral center in Colombia, South America. Cardiol Young. 2020;30(12):1866-73.
Daene M, De Pauw L, De Meester P, Troost E, Moons P, Gewillig M, et al. Outcome of Down patients with repaired versus unrepaired atrioventricular septal defect. Int J Cardiol Congenit Heart Dis. 2023;12:100452.
Atz AM, Hawkins JA, Lu M, Cohen MS, Colan SD, Jaggers J, et al. Surgical management of complete atrioventricular septal defect: associations with surgical technique, age, and trisomy 21. J Thorac Cardiovasc Surg. 2011;141(6):1371-9.
Malik M, Nuri MK. Surgical considerations in atrioventricular canal defects. Semin Cardiothorac Vasc Anesth. 2017;21(3):229-34.
Fong LS, Betts K, Bell D, Konstantinov IE, Nicholson IA, Winlaw DS, et al. Complete atrioventricular septal defect repair in Australia: results over 25 years. J Thorac Cardiovasc Surg. 2020;159(3):1014-25.e8.
Prifti E, Bonacchi M, Bernabei M, Crucean A, Murzi B, Bartolozzi F, et al. Repair of complete atrioventricular septal defects in patients weighing less than 5 kg. Ann Thorac Surg. 2004;77(5):1717-26.
Kolasiński W. Surgical site infections: review of current knowledge, methods of prevention. Pol Przegl Chir. 2018;91(4):41-7.
Garner BH, Anderson DJ. Surgical site infections: an update. Infect Dis Clin North Am. 2016;30(4):909-29.
Figuerola-Tejerina A, Rodríguez-Caravaca G, Bustamante-Munguira J, San Román-Montero JM, Durán-Poveda M. Epidemiological surveillance of surgical site infection and its risk factors in cardiac surgery: a prospective cohort study. Rev Esp Cardiol (Engl Ed). 2016;69(9):842-8.
Cogen AL, Nizet V, Gallo RL. Skin microbiota: a source of disease or defence? Br J Dermatol. 2008;158(3):442-55.
Zarb P, Coignard B, Griskeviciene J, Muller A, Vankerckhoven V, Weist K, et al. The European Centre for Disease Prevention and Control (ECDC) pilot point prevalence survey of healthcare-associated infections and antimicrobial use. Euro Surveill. 2012;17(46):20316.
Cahill TJ, Jewell PD, Denne L, Franklin RC, Frigiola A, Orchard E, et al. Contemporary epidemiology of infective endocarditis in patients with congenital heart disease: a UK prospective study. Am Heart J. 2019;215:70-7.
Russell IA, Zwass MS, Fineman JR, Balea M, Rouine-Rapp K, Brook M, et al. The effects of inhaled nitric oxide on postoperative pulmonary hypertension in infants and children undergoing surgical repair of congenital heart disease. Anesth Analg. 1998;87(1):46-51.
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