Anatomy of the Aortic Valve and Root - A (Pediatric) Cardiac Surgeon s View

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Anatomy of the Aortic Valve and Root - A (Pediatric) Cardiac Surgeon s View Markus K. Heinemann Klinik für Herz-, Thorax- und Gefäßchirurgie Universitätsmedizin Mainz

X X X X X X X X 2?

Conflicts of Interest (?) I am a surgeon for congenital heart disease by training. Pediatric cardiac surgeons tend to be peculiar. Their views may be somewhat idiosyncratic. Fortunately, I have also done research and published on aortic dissection. So it might be worth staying in the room after all. Don t say you haven t been warned!

What exactly is the aortic root? Wikipedia (what else?): The aortic root is the portion of the aorta beginning at the aortic annulus and extending to the sinotubular junction. It is sometimes regarded as a separate entity from the rest of the ascending aorta. Between each commissure of the aortic valve and opposite the cusps of the aortic valve, three small dilatations are called the aortic sinuses. The sinotubular junction is the point in the ascending aorta where the aortic sinuses end and the aorta becomes a tubular structure.

What shall we focus on, then? Anulus (the enigma) Cusps (aka Leaflets ) Sinus (yes, that s the plural) Coronaries (lest we forget) Ascending Aorta (where the root ends)

Anulus Latin: anulus, pl.: anuli = small ring Why we should have an annulus, but simply an anus nobody knows. A: complex, 3-dimensional, crown-shaped structure (surgical) B: diameter at base of aortic root ( unanatomical, reference measure) from: Pershad A, Stone D, Morris MF, Fang K, Gellert G Aortic annulus measurement and relevance to successful TAVR J Interv Cardiol. 2013 Jun;26(3):302-9.

Anulus some harsh words Descriptions of the aortic root over the years have been BEDEVILED by accounts of a valve anulus. There are at least 2 RINGS within the root we describe the current problems which have arisen due to INDISCRIMINATE descriptions of a NONEXISTENT anulus. Loukas M, Bilinsky E, Bilinsky S, Blaak C, Tubbs RS, Anderson RH. The anatomy of the aortic root. Clinical Anatomy 2014;27:748-56

Anular Enlargement Classic Surgery (AVR) To prevent patient-prosthesis mismatch Simple and efficient The incision is directed toward the mid-mitral line (an imaginary line that divides the anterior leaflet into two equal halves), down to the level where the roof of the left atrium is attached to the fibrous curtain. A teardrop-shaped pericardial patch is sutured to this extended aortotomy with a continuous 4-0 polypropylene suture. Sommers KE, David TE. AVR with patch enlargement of the aortic annulus. Ann Thor Surg 1997;63:1608-12

Anuloplasty If you tighten your belt you lift up your trousers Anuloplasty increases coaptation height Lansac E et al. An external open ring for isolated aortic valve repair. Multimed Man Cardiothorac Surg 2011

Anuloplasty Decreasing anulus diameter increases coaptation height and area Measuring effective coaptation height helps to judge valve performance after reconstruction Marom G, Haj-Ali R, Rosenfeld M, Schäfers HJ, Raanani E. Aortic root numeric model: Annulus diameter prediction of effective height and coaptation in post aortic valve repair. J Thorac Cardiovasc Surg 2013; 145: 406-11

Cusps EMBRYOLOGY Fusion of outflow tract cushions Appearance of intercalated cushions Excavation of their distal margins Ingrowth of non-myocardial tissue for sinus Spicer DE, Bridgeman JM, Brown NA, Mohun TJ, Anderson RH. The anatomy and development of the cardiac valves. Cardiol Young 2014; 24: 1008 22

Cusps (anatomical) Usually 3 Sometimes 2 ( bicuspid ) stenosis, ascending aortic aneurysm Rarely 4 (quadricuspid) regurgitation, pediatric population or Syrian hamsters López-García A, Fernández MC, Durán AC, Sans-Coma V, Fernández B. Quadricuspid aortic valves in Syrian hamsters and their formation according to current knowledge on valvulogenesis. Japanese Journal of Veterinary Research 2015; 63: 37-43

Cusps (mathematical) Cusp geometric height (gh) higher than presumed?! Non-fused cusps in bicuspids larger than in tricuspids In case of retraction forget repair Schäfers HJ, Schmied W, Marom G, Aicher D. Cusp height in aortic valves. J Thorac Cardiovasc Surg 2013;146:269-74 Labrosse MR, Beller CJ, Robicsek F, Thubrikar MJ. Geometric modeling of functional trileaflet aortic valves: Development and clinical applications J Biomechanics 2006; 39: 2665 72

Sinus = bay, bulge Plural: Sinus (U-declination) the area behind the cusps providing room for the cusps sort of Hidden Valleys Antonio Maria Valsalva (1666-1723) anatomist and surgeon

Sinus Green sinotubular junction Blue mid-sinus level (widest) Red basal cusp attachment Red dot echo annulus Loukas M, Bilinsky E, Bilinsky S, Blaak C, Tubbs RS, Anderson RH. The anatomy of the aortic root. Clinical Anatomy 2014;27:748-56

Sinus Largest diameter of root anyway Pear / onion in connective tissue disease (Marfan etc) Dilation in bicuspids Enlarged in cyanotic CHD with limited pulmonary blood flow (ToF!) redirection towards one outlet already in fetal life root aneurysm Kim TS, Na CY, Baek JH, Yang JS. Aortic root and ascending aortic aneursym in an adult with a repaired tetralogy of Fallot. Korean J Thorac Cardiovasc Surg 2011;44:292-3

Sinus Kirklin, Barrat-Boyes, Cardiac Surgery Loukas M, Bilinsky E, Bilinsky S, Blaak C, Tubbs RS, Anderson RH. The anatomy of the aortic root. Clinical Anatomy 2014;27:748-56

Sinus Valsalva Aneurysm

Coronary Arteries Part of the root Remember the Alamo (here: early TAVI experience) Always in the way Make the David / Yacoub procedures tedious / unpopular May have abnormal pattern

Coronary Arteries EMBRYOLOGY (Oh no not you again!) Observations by Raymond Vieussens (1635 1715) Ingrowth rather than Outgrowth Debated by RH Anderson (of course) ALCAPA - how can it happen? Bogers AJJC, Gittenberger-de Groot AC, Dubbeldam JA, Huysmans HA.The inadequacy of existing theories on development of the proximal coronary arteries and their connexions with the arterial trunks. Int J Cardiol 1988;20:117-23 Spicer DE, Henderson DJ, Chaudhry B, Mohun DJ, Anderson RH. The anatomy and development of normal and abnormal coronary arteries. Cardiol Young 2015;25:1493-1505

Ascending Aorta Starts at Sino-tubular junction Is the first really tubular part of the aorta Contains more elastic fibers than the rest and is therefore happy to dilate Langer F, Aicher D, Kissinger A, Wendler O, Lausberg H, Fries R, Schäfers HJ. Aortic valve repair using a differentiated strategy. Circulation 2004;110:II67 II73 May be replaced supracoronarily (if justified) but is often inseparable from its root

Ascending Aorta Aortic Dissection Type A Ascending aortic aneurysm Dilation in bicuspids Extension of root pathology Children: supravalvular aortic stenosis (media)! (Williams-Beuren syndrome, aortoplasty Doty, Brom) Stamm C, Friehs I, Ho SY, Moran AM, Jonas RA, del Nido PJ. Congenital supravalvar aortic stenosis. A simple lesion? EurJCTS 2001;19:195-202

Ascending Aorta More MATHS : Tirone s Rule for graft diameter in ascending aortic replacement 1. Take circumference of sino-tubular junction 2. subtract diameter at brachiocephalic trunk take-off 3. multiply by zero 4. and add 28. (CFstj Dbrc) X 0 + 28 = Dgraft

Luckily, Life s not just mathematics Enjoy the course!