Combined congenital aortic stenosis and coarctation of aorta with severe left ventricle dysfunction and pulmonary hypertension in an infant-one case, multiple challenges
DOI:
https://doi.org/10.18203/2320-6012.ijrms20240231Keywords:
AS, COA, Left ventricle dysfunction, Pulmonary hypertensionAbstract
Aortic stenosis (AS) is commonly associated with coarctation of aorta (COA). Due to chronic increase in afterload, it can lead to left ventricle (LV) dysfunction and ultimately LV failure. We present a case of 10-month-old infant with combined AS and COA, complicated by severe LV dysfunction and severe Pulmonary hypertension (PH). There are no guidelines as to which condition should be treated first although both staged and combined procedure have been reported. It poses multiple challenges in the perioperative management. Although performing a balloon aortic valvuloplasty first would reduce the surgical risk for COA repair significantly, but due to lack of facilities for same in this age group, we proceeded with a high-risk COA repair via thoracotomy, avoiding the need for cardiopulmonary bypass (CPB). Application of Aortic cross clamp was associated with a further increase in afterload and hypotension owing to severe LV dysfunction in contrast to the expected increase in proximal pressures, and required high inotrope support to manage hemodynamics. Postoperative course was challenging as well owing to severe pulmonary hypertension. Very few similar cases have been reported in literature.
References
McLennan D, Caputo M and Taliotis D. Severe Aortic Stenosis and Severe Coarctation of the Aorta: A Hybrid Approach to Treatment. Front Surg. 2017;4:16.
Hoffman JI, Kaplan S. The incidence of congenital heart disease. J Am Coll Cardiol. 2002;39(12):1890.
Tokel K, Yildirim SV, Varan B, Ekici E. Sequential balloon dilatation for combined aortic valvular stenosis and coarctation of the aorta in a single catheterization procedure: a prognostic evaluation based on long-term follow up. J Invasive Cardiol. 2006;18(2):65-9.
Tokunaga S, Kado H, Imoto Y, Shiokawa Y, Yasui H. Successful staged-Fontan operation in a patient with ectopia cordis. Ann Thorac Surg. 2001;71(2):715-7.
Mulay AV, Ashraf S, Watterson KG. Two-stage repair of adult coarctation of the aorta with congenital valvular lesions. Ann Thorac Surg. 1997;64(5):1309-11.
Luciani GB, Lucchese G, Carotti A, Gianluca B, Piero A, Giuseppe C et al. Two decades of experience with the Ross operation in neonates, infants and children from the Italian Paediatric Ross Registry. Heart. 2014;100:1954-9.
Dorobantu DM, Taliotis D, Tulloh RM, Sharabiani MTA, Mohamed Ahmed E, Angelini GD, Stoica SC. Surgical versus balloon valvotomy in neonates and infants: results from the UK National Audit. Open Heart. 2019;6(1):e000938.
Ross DN. Replacement of aortic and mitral valves with a pulmonary autograft. Lancet. 1967;2(7523):956-8.
Wright GE, Nowak CA, Goldberg CS, Ohye RG, Bove EL, Rocchini AP. Extended resection and end-to-end anastomosis for aortic coarctation in infants: results of a tailored surgical approach. Ann Thorac Surg. 2005;80(4):1453-9.
Kim TH, Shin YR, Kim YS, Kim DJ, Kim H, Shin HJ, Htut AT, Park HK. Central-Approach Surgical Repair of Coarctation of the Aorta with a Back-up Left Ventricular Assist Device for an Infant Presenting with Severe Left Ventricular Dysfunction. Korean J Thorac Cardiovasc Surg. 2015;48(6):407-10.
Panchanatheeswaran K, Lakshmanan M, Koyilil V. Coarctation of Aorta Repair in an Infant Using Arch to Descending Thoracic Aorta Bypass. 2020.
