An unusual case of coarctation of the aorta

1. An unusual case of coarctation of the aorta Peter Fletcher Nick Collins Brett Napier

2. AS, f19, Coarctation Presentation: • Female 19 with coarctation identified on routine transthoracic echo • Echo requested because of murmur “all over the chest radiating to neck and axilla”

3. AS, f 19, Coarctation ECHO: NHCS 8/6/11 • Height 168cm weight 59kg • Possible coarctation of the aorta, gradient ~30mmHg • Mild MR, TR, trivial PR • Normal LV • Mildly dilated LA • Upper normal pulmonary systolic pressure • Normal aortic valve

4. AS, f 19, coarctation Presenting illness • Minor SOB • Vague chest tightness, non-exertional • Frequent dizzy episodes, occasional faints • Always thin, was losing weight, now stable • Murmur known since childhood, never echo

5. AS, f 19, Coarctation Past Health • Some mentrual problems • Borderline HBP noted in Gynae clinic, no Rx • Nil else Family History • Hypertension Personal history • 10 cigs/d, minimal EtOH, no allergies • Meds: contraceptive implant

6. AS, f 19, Coarctation Examination • Well, thin • BP: sitting - RA 140/80, LA ?95/60 • R radial and both carotids normal • L radial, both femoral and all pedal pulses barely palpable • Systolic murmur LSE, base and neck ? click • Different systolic murmur apex and L side

7. AS, f 19, Coarctation Investigations • ECG: normal, PR 112ms • CXR: The heart is normal in size. There is slight prominence of soft tissue in the right para-tracheal region suggestive of a right sided aortic arch. There is some mild thinning of the posterior ribs, particularly in the left 6th and 7th ribs. No focal bony lesion however is identified. The lungs and pleural spaces are clear. • CT scan: • ECHO:

8. RIGHT COMMON CAROTID LEFT COMMON CAROTID RA/SVC LEFT VENTRICLE

9. LEFT COMMON CAROTID

10. LEFT SUBCLAVIAN ASCENDING AORTA SVC DESCENDING AORTA

11. RIGHT SUBCLAVIAN ARTERY

12. POSTERIOR VASCULAR RING - Aberrant right subclavian DESCENDING AORTA

13. Ascending Aorta Pulmonary Trunk Descending Aorta

14. Left Common Carotid Ascending Aorta Descending Aorta Posterior Ring

15. Right Common Carotid Ascending Aorta Left Common Carotid Posterior Ring Descending Aorta Left Subcalvian

16. Left Common Carotid Right Common Carotid Right subclavian – Diverticulum of Kommerell Left Subcalvian

17. Right Common Carotid Left Common Carotid Right Vertebral Left Vertebral

18. Complex Aortic Arch Anomalies….

19. Comments… • Unusual • Incorporates two aortic anomalies • Aortic coarctation • Vascular ring • Abnormal branching pattern • In this case well defined by cross sectional imaging • Echo • Functional information

20. Before I Get Carried Away.. • Embryology • Important • Title… • Reality • Vascular Rings • Focus aortic coarctation • Practical • Specific reference to late complications • Adult cardiology

21. However….

23. This Anomaly…. • Circumflex/retro-oesophageal aortic arch • Rare • Aortic arch and descending aortic arch opposite side of spine • Associated with hypoplasia retro-oesophageal aorta

25. Vascular Ring • Result of abnormal anatomy of great arteries and branches • Uncommon (less than 1% CHD) • Encircle oesophagus and/or trachea • Fibrous ligamentum from ductusarteriosus • Majority involve presence right aortic arch • Symptoms relate to tracheal (oesophageal) constriction • Stridor • Dyspnoea • Recurrent chest infections

26. Key Clinical Issue • Not constriction • Note pending investigations • Effect aortic coarctation • Functional data

35. Aortic Coarctation • Area narrowing junction aortic arch and descending aorta • Discrete vs hypoplasia • Presentation variable • Severity stenosis • Concomitant lesions • Ductal closure • Acute left ventricular failure • Shock

36. Co-Existing Lesions • Turners syndrome • Bicuspid aortic valve (85-95%) • VSD • Berry aneurysms (SAH) • Generalised aortopathy • Important follow up • BP/dissection risk • Pregnancy

37. “Mild” coarctation • Hypertension • Symptomatic • LVF • ICH • IE • Dissection • Asymptomatic

38. Indications for Intervention… • Surprisingly controversial • Young • Before age 6 • Late BP • Older • ACHD guidelines (Expert opinion - US/European/Canada) • Gradient >30mmHg (Symptomatic/Asymptomatic with hypertension at rest/exercise or LVH) • Aortic regurgitation • Aneurysmal dilatation (ascending aorta/prior intervention)

39. Why Controversial? • Which treatment • Surgical • Percutaneous • Limits balloon angioplasty alone • (Covered) stents • Vascular injury • Treatment not without morbidity.. • Spinal ischaemia • Recoarctation • Aneurysm formation • Stents – Rupture/Fracture

40. Why Controversial? • Effect treatment? • Some argue… • Hypertension may persist • Medical treatment for hypertension improved • Randomised trial?

41. In Repaired… • Hypertension • 24 hour BP monitor • EST • Valvular heart disease • Echocardiography • Aortic anatomy • Recoarctation/aneurysm • CT/MRI • Radiation during follow up

42. Pregnancy… • Tricky • Hypertension/PIH • Risk dissection

43. Summary • Vascular ring • Uncommon • Paediatric disease • Aortic coarctation • Surprising complex • Multifaceted in terms of treatment and follow up