Aortic coarctation: a benign lesion?

Transcription

1 Aortic coarctation: a benign lesion? A surgeon s path out of stubborness From hypoplastic arches to molecular biology Y. d'udekem The Royal Children s Hospital, Melbourne, Australia

2 Coarctation of the aorta: 3-4 per 10,000 live births

3 Aortic coarctation repair a cure?? 1944 Circulation 1989;80:

4 The passage of time 1995: Cliniques Saint-Luc, Brussels, Belgium 2015: Royal Children s Hospital, Melbourne, Australia

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6 Hypoplastic arch

7 Hypoplastic arch 40-80% of coarctation patients

8 Hypoplastic arch: Definition Ratio Transverse Arch/Asc Ao: < 0.6 (prox) <0.5 (dist) Autopsy spec Moelart Circulation 1976 < 0.76 Comparison with controls/echo Morrow JACC 1986 < 0.95 (prox) < 0.87 (dist) Compar with controls/angiograms Clarkson Paediatr Cardiol 1985/Weber JACC 1993 Ratio transverse Arch/Desc Ao < 0.9 Angiograms Clarkson Paediatr Cardiol 1985 < 0.9 MRI Smith Maia JTCVS 2004 Absolute value diameter arch < weight (kgs) 1mm Karl JTCVS 1992 Z-scoring arch diameter <-2

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13 Hypoplastic arches and coarctation Postulate: After coarctation repair, the hypoplatic transverse arch grows RCH Melbourne, Australia

14 Growth of the hypoplastic arch Siewers y? Tr A/Aao Myers y PTA/DTA Brouwer mo? Z-score: -7-1 Jahangiri y DTA 10% hypoplasia Kiraly d DTA DASV-AAFV/LVSF RCH Melbourne, Australia

15 Hypoplastic arches and coarctation RCH Melbourne, Australia

16 Patients Characteristics : 20 pts Mean age at coarctation repair: 9±6 days Mean age at last follow-up: 10±6 years Proximal transverse arch Distal transverse arch RCH Melbourne, Australia

17 Growth of the proximal arch [2] Liu et al. Interactive cardiovascular and thoracic surgery. 2010;10(4): RCH Melbourne, Australia

18 305 pts with up to 20 years follow-up After coarctation repair, half of the patients with hypoplasia of the transverse arch will develop arch reobstruction and a third will become hypertensive. The technique of endto-side repair performed through a sternotomy seems to alleviate these issues, and could be offered to a larger proportion of patients with hypoplasia of the aortic arch. Many of these patients are lost to follow-up during adolescence, at a time when ongoing care seems mandatory. RCH Melbourne, Australia

19 Gold standard: 24-hour ambulatory blood pressure monitoring 1. Correlates with end-organ damage in children & adults 2. O Sullivan et al: Sensitivity of casual BP measurements in detecting HT only 66% [2] Palatini et al. Ambulatory blood pressure predicts end-organ damage... J Hypertens 1999 Apr:17(4): [3[ Urbina E. et al. Ambulatory blood pressure monitoring in children and adolescents... Hypertension Sep;52(3): [4] O Sullivan et al. Prevalence of hypertension in children after early repair of coarctation of the aorta... Heart 2002 Aug;88(2):163-6

20 Master T.B. 14 y.o. male: 24-hr BP 161/84 mmhg Supposedly normotensive at last f/up

21 Master T.B. 14 y.o. male: 24-hr BP 161/84 mmhg Supposedly normotensive at last f/up

22 Hypoplastic arches : small arches 62 pts had echo and 24 hour-bp monitoring Median age at coarctation repair : 11 days Median age at study : 17 years

23 27% resting hypertension 60% abnormal 24-hour BP monitoring 32% had arch reobstruction 90% of those with obstruction were hypertensive 50% of hypertensive pts had obstruction 1/3 rd of thoracotomy had obstruction vs 0% with end-to-side repair/sternotomy 32% of the pts had left ventricular hypertrophy No relationship with obstruction or hypertension

24 End-toside repair!! He is obsessed.

25 2013 Heartkids Grant-in-aid Measuring the burden of untreated hypertension amongst patients with repaired coarctation. In 87 pts from Victoria 24 hour Blood Pressure monitoring Transthoracic echocardiography Retinal scans

26 Unpublished study 142 pts post-coa repair Hypoplastic arch: 56% (79/142) Mean f/up: 21 ± 7 years Resting hypertension: 27% (39/142) 24-hour BP: Hypertensive: 43% (61/142) Prehypertensive: 32% (45/142) Antihypertensive meds: 2% (3/142) 50% of LV hypertrophy 26

27 Unpublished study (n=142)

28 Causes of late hypertension Older age at repair Residual arch reobstruction Arch geometry Neurohormonal and vascular causes 28

29 Causes of late hypertension Older age at repair Residual arch reobstruction Arch geometry Neurohormonal and vascular causes 29

30 Arch geometry and hypertension Yes 1. Ou P, Bonnet D, Auriacombe L, Pedroni E, Balleux F, Sidi D, Mousseaux E. Late systemic hypertension and aortic arch geometry after successful repair of coarctation of the aorta. European Heart Journal. 2004;25: Donazzan L, Crepaz R, Stuefer J, Stellin G. Abnormalities of aortic arch shape, central aortic flow dynamics, and distensibility predispose to hypertension after successful repair of aortic coarctation. World Journal for Pediatric and Congenital Heart Surgery. 2014;5: No 3. Lashley D, Curtin J, Malcolm P, Clark A, Freeman L. Aortic arch morphology and late systemic hypertension following correction of coarctation of aorta. Congenit Heart Dis. 2007;2: Liu JYJ, Jones B, Cheung MMH, Galati JC, Koleff J, Konstantinov IE, Grigg LE, Brizard CP, d'udekem Y. Favourable Anatomy After End-to-Side Repair of Interrupted Aortic Arch. Heart Lung Circ. 2013;23: De Caro E, Trocchio G, Smeraldi A, Calevo MG, Pongiglione G. Aortic arch geometry and exercise-induced hypertension in aortic coarctation. AJC. 2007;99: Ntsinjana HN, Biglino G, Capelli C, Tann O, Giardini A, Derrick G, Schievano S, Taylor AM. Aortic arch shape is not associated with hypertensive response to exercise in patients with repair

31 Causes of late hypertension Older age at repair Residual arch reobstruction Arch geometry Neurohormonal and vascular causes 31

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33 Biopsies prox & distal to CoA 19 pts <3 mths of age 14 with PDA 5 with ligamentum arteriosum Greater expression of hypertension-related genes in prox aorta c.f. distal aorta in those without PDA 33

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35 The effect of intracardiac shunting at time of coarctation repair (142 pts) Patients with PDA/intracardiac shunt less likely to develop late hypertension (39% vs. 58%; p=0.07). Patients with VSD/AVSD were less likely to develop late hypertension (34% vs. 52%; p=0.05).

36 Blood pressure Patients with early post-op hypertension were more likely to develop late hypertension (53% vs. 28%; p=0.006) Patients with a PDA/intracardiac shunt were less likely to develop early post-op hypertension (40% vs. 87%; p<0.0001).

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38 Should we treat prehypertension? Harrap S. et al. Brief angiotensin converting enzyme inhibitor treatment in young... Hypertension. 1990;16:

39 Neurohormonal study Aim #1: To elucidate pathways leading to hypertension after coarctation repair by examining the degree of sympathetic nervous activation, endothelial function, and arterial compliance in patients after coarctation repair, and their relationships to hypertension. Aim #2: To elucidate the genetic predisposition leading to the development of hypertension after coarctation repair.

40 Neurohormonal study

41 Neurohormonal study Unable to elucidate differences between normotensive & hypertensive groups - Only 3/11 patients with hypertension on repeat 24-hour BP monitoring

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46 Future directions My way: Treat them all!!! Determine the phenotype of future hypertensive patients early in life and randomize

47 2015 Heartkids Grant-in-aid Exercise-induced hypertension in childhood following neonatal aortic coarctation repair. 50 pts 24 hour Blood Pressure monitoring Transthoracic echocardiography Exercise induced hypertension

48 Conclusion Late hypertension late after coarctation repair is an issue Real impact? Incidence : 40 % For hypoplastic arches: residual obstruction For normal arches: neurohormonal activation 24 hour-bp monitoring not such gold standard We can solve this

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