Thoracic Aortic Disease Flashcards
How do you measure the diameter of an aorta?
Measure in all 3 axis, axial, sagittal and coronal
How do echo diameters compare to CT diameters?
Echo often underestimates the diameter by 1-2mm
Axial diameter often overestimates
To measure the aorta, do you use TTE or TEE?
TTE, there is no role for TEE in surveillance except in dissected aortas for intra-operative repairability
What is the risk of rupture of moderately dilated AAo 40-55mm
0.1% per year, so low risk
What is the risk of death or stroke from root replacement vs total arch replacement vs TAAAR
1-2% in root replacement vs 5% in total arch replacement vs 5-10% in TAAAR
What should be ordered in inflammatory aortic disease?
Medical treatment in most patients, consultation to rheumatology
Wait until inflammatory markers come down
If need to operate due to symptoms, give high dose steroids for several days before operation
Post-operatively needs steroids and DMARDs
List 3 connective tissue aortic diseases and their respective genes
Marfan syndrome FBN1 mutation
Loeys-Dietz syndrome TGFBR2 or TGFBR1 mutation
Ehlers-Danlos syndrome COL3A1 mutation
Turner syndrome 45,XO karyotype
Marfan’s syndrome, describe the gene, protein, cardiac manifestations and list 7 systemic manifestations
FBN1 mutation
Fibrillin-1 protein
Aortic aneurysm
Aortic insufficiency from root dilation
Mitral valve prolapse
Tricuspid valve prolapse
PA dilation
Aortic dissection
Dilated cardiomyopathy
Prolonged QT interval
Ectopia lentis
Dural ectasia
Arachnodactyly
Pectus excavatum or carinatum
Tall stature
Cataracts
Wrist-thumb sign
Protrusio aceetabulla
Arm span to height ratio >1.05
Pes planus
Scoliosis
Diminished elbow extension (<170 degrees)
What is the most common location of tear in TAAD?
Above the commissures between the NCC and LCC
How is valve resuspension done in TAAD?
Transmural pledgeted full thickness sutures at the top of each commissure.
Common mistake is to go too high above the commissure. Must go right at the commissure
Must also obliterate the false lumen at the root. Can use felt or bioglue.
How is AI caused in TAAD?
Caused by flap going into the NCC causing prolapse
Cannulation approach for TAAD Repair
Stable - Innominate not dissected - Sternotomy - Isolate Innominate (graft on before pericardium)
Stable - Innominate dissected - Sternotomy - Go R axillary artery - Central Venous cannulation
Stable - Innominate and axillary dissected - Sternotomy - Go direct aortic cannulation
Stable - All vessels dissected - Axillary dissection and approximate the true and false lumens to perfuse the true lumen with low threshold of alternative strategy like direct aortic cannulation - Make sure you have femoral and radial arterial lines
Unstable (tamponade) - Direct aortic cannulation (seldinger) with TEE guidance (not exam answer)
Unstable (tamponade) if CPR on the table - Femoral arterial cannulation - Femoral venous cannulation - Go on pump - Sternotomy and open
Unstable (tamponade) - Right axillary and femoral venous cannulation
Does pericardial effusion always mean ruptured Aortic dissection?
Often it isn’t, can be a sympathetic response or contained rupture, rarely a frank rupture
Describe the revised Ghent criteria?
In the absence of family history
Aortic root dilation (Z >=2) or dissection and ectopia lentis
Aortic root dilation (Z >=2) or dissection and FBN1 mutation
Aortic root dilation (Z >=2) or dissection and systemic score >= 7 points
Ectopia lentis and FBN! known aortic root dilation/dissection
In the presence of family history
Ectopia lentis and family history of Marfan
Systemic score >= 7 points and family history of Marfan
Aortic root dilation (Z>=2 in >20 years of age; Z >=3 in <20 years of age) and family history of Marfan
What are 7 systemic manifestations of Marfan’s syndrome?
Wrist and thumb sign - 3 points (1 point if only one sign present)
Severe Myopia
Reduced upper segment/lower segment (<0.85 Whites, <0.65) and Arm span to height ratio (>1.05)
Pectus excavatum or carinatum - 2 for carinatum
Dural ectasia - 2 points
Scoliosis
Reduced elbow extension
Kyphosis
Protrusio acetabuli - 2 points
Spontaneous Pneumothorax - 2 points
Skin striae
Pes planus - 2 points
Flat feet
Facial features 3/5 or more (dolichocephaly, enophthalmos, downslanting palpebral fissues, malar hypoplasia, retrognathia)
Describe Ehlers-Danlos Syndrome the gene, protein, transmission, and symptoms
Mutation in COL3A1
Type III Collagen
Autosomal dominant transmission
Specific facial features (thin nose, almond shaped eyes, thin lip, acrogeria)
Thin translucent skin
Propensity for bleeding
AV Fistula
Rupture of vessels or viscera (colonic perforation, uterine rupture during pregnancy etc)
Frequent bruising
Describe Loeys-Dietz Syndrome the gene, protein, 3 cardinal features, mean life expectancy if untreated?
Mutation in TGF2 beta receptor 1 and 2 or SMAD3
Autosomal dominant
Gene
3 cardinal features: Hypertelorism, Bivid uvula or cleft palate, arterial tortuosity with widespread vascular aneurysm and dissection, especially intracranial aneurysms
Life expectancy if untreated? 26 years
Describe Aneurysm-Osteoarthritis Syndrome, what is the gene, what is the transmission?
Mutation in SMAD3 gene that affects intracellular effector of TGF-B signalling
Similar to Loeys-Dietz, but with early OA
High incidence of dissection at moderate dilation (4-4.5cm)
Describe 2 causes of saccular aneurysms
Infections (Syphilis or Mycotic)
Atherosclerosis
Trauma
Takayasu’s or Behcet’s
Name 3 conditions that cause aortitis
Giant cell arteritis
Takayasu Arteritis
Behcet’s disease
Describe the classification of Takayasu’s Arteritis
Type 1 - Aortic arch involvement
Type 2 - Thoracoabdominal involvement, not the arch
Type 3 - Entire aorta is involved
Type 4- Pulmonary artery involvement
Autoimmune disorder
M:F ratio of 1:8
Describe 3 mechanisms of mycotic aneurysm formation
Contiguous spread from adjacent thoracic structures i.e. infective pericarditis, infected lymph nodes
Septic emboli from IE
Hematogenous dissemination from sepsis or IVDU
Describe optimal medical therapy in aortic diseases
Goal blood pressure <130/80 to reduce the risk of CV events (Class I AHA 2022 aortic guidelines)
Beta-blocker or ARB therapy for patients with Marfan’s syndrome to reduce the rate of aortic dilation.
Diet and smoking cessation
Lipid-lowering therapy
Avoid strenuous resistance and isometric exercise
Asymptomatic Marfan’s syndrome, list 5 reasons for early surgery
Aortic root >=5cm
Ascending aorta >=5cm
Aortic growth rate >0.5cm per year
Family history of aortic dissection <5cm and aorta >4.5cm
Bicuspid aortic valve and aorta >4.5cm
Concomitant valvular heart disease requiring surgical intervention
Young women anticipating pregnancy (4.1-4.5cm CCS, >4.0cm AHA)
Indexed aortic size >2.75cm/m2
CSA (cm2) / height (m) > 10 (AHA)
What are the indications for intervention on asymptomatic thoracic aortic aneurysms per the 2014 CCS Guidelines?
Aortic root - 5.5cm degenerative, 5.0-5.5 bicuspid, 5.0 Marfan’s, familial 4.5-5.0, other genetic thoracic aortopathies 4.0-5.0
Ascending - 5.5cm degenerative, 5.0-5.5 bicuspid, 5.0 Marfan’s, familial 4.5-5.0, other genetic thoracic aortopathies 4.2-5.0, concomitant cardiac surgery 4.5
Arch - 6.0cm degenerative, 5.5cm bicuspid, 5.5-6.0 all genetic aortopathies
Descending - 6.5cm degenerative, 6.5cm bicuspid, 5.5-6.0 all genetic aortopathies
What are the indications for intervention for aortic pseudoaneurysms?
Surgical intervention might be considered when the total diameter of the aorta (including the native aorta and the false aneurysm) meets the criteria for intervention or if the pseudoaneurysm is >2cm in maximum diameter
List 4 Class I indications for intervention on asymptomatic ascending aorta or root aneurysms
> 5.5cm without genetic disorder
<5.5cm with growth rate >0.3cm/yr for 2 years or >0.5cm/yr in 1 year
5.0cm with HTAAD, Marfan’s
4.5cm with Loeys-Dietz and TGFBR1 or 2
Male, 55 years old, came with murmur. Echo showed bicuspid valve. He is asymptomatic with respect to his aortic aneurysm. List 3 imaging modalities that would allow you to assess the aorta.
TTE/TEE
CTA
MRA
Angiography
Male, 55 years old, came with murmur. Echo showed bicuspid valve. He is asymptomatic with respect to his aortic aneurysm. His aneurysm is 4.7cm. When would you operate on his aneurysm?
> 5.5cm (Class I)
5cm with aortic dissection risk factor (family history of aortic dissection, aortic growth >=0.3cm/yr, aortic coarctation, “root phenotype” aortopathy) and experienced center (Class IIa)
5cm with cross-sectional aortic root or ascending aorta to height ratio >=10cm2/m (Class IIa)
4.5cm and undergoing valve surgery in experienced center (Class IIa)
5cm without aortic dissection risk factor and experienced center (Class IIb)
Male, 55 years old, came with murmur. Echo showed bicuspid valve. He is asymptomatic with respect to his aortic aneurysm. His aneurysm is 4.7cm. What 3 recommendations would you give this patient assuming his valve does not need intervention?
Repeat screening in 6 months
Screen 1st degree relatives for BAV/aortopathy
Optimize medical management (<130mmHg sBP, lipids <70mg/dl, smoking cessation)
Seek medical attention if any signs or symptoms of aortic dissection
Avoid strenuous exertion and resistance exercise
List 3 operations that can be done to replace the aortic root
- Valve-sparing aortic root repair (David or Yacoub)
- Bentall procedure (mechanical or bioprosthetic)
- Ross procedure
- Medtronic Freestyle xenograft
- Aortic root homograft
In a patient with trileaflet AV and mild or less AI, list 4 scenarios where you would consider a Bentall instead of VSRR according to the CCS 2016 Thoracic Aortic Surgery Guidelines
Emergency setting
Elderly
Multiple comorbidities
Poor LV function
Poor quality cusp tissue
What size of ascending aneurysm should a person be limited from driving, what about if they are a commercial driver?
> 6cm ascending for private driving, >5.5cm ascending for commercial driving (CCS 2014 Aortic)
6.5cm descending for private driving, >6cm descending for commercial driving (CCS 2014 Aortic)
When should patients return to private or commercial driving after open aortic repair?
6 weeks for private driving and 3 months for commercial driving (CCS 2014 Aortic)
What is the standard of care operation for Marfan’s syndrome with root aneurysm requiring intervention?
Valve sparing aortic root replacement
List 3 ways to reconnect the left main after redo-Bentall
Cabrol
Hemi-cabrol
Reimplantation
Ligate and bypass
You do a Bentall Procedure. After weaning from CPB, you note to have severe RV dysfunction. List 5 causes of RV dysfunction following root replacement.
Pulmonary hypertension
Poor myocardial protection
Right coronary embolism (air, atheroma, thombus)
Right button distortion or kinking
Right button dissection
Right button obstruction from valve prosthesis
Perioperative infarct (native CAD)
What are 3 techniques of the Ross procedure?
Subcoronary implantation
Aortic root replacement
Aortic root inclusion
List 4 advantages and 4 disadvantages of the Ross procedure
Advantages
No anticoagulation
Living tissue which can grow with the patient
Good hemodynamics
Lower risk of endocarditis
Disadvantages
Turns one valve disease into two valve disease
Technically complex
Pulmonary homograft failure associated with a 10% reoperation rate at 10 and 20% at 20 years
Autograft failure (root dilation and valvular regurgitation) associated with a 10% reoperation rate at 10 and 25% at 20 years
Redo-surgery is more challenging in previous Ross
Risk of coronary injury
List 3 absolute contraindications to Ross procedure
Unrepairable pulmonary valve leaflets
Size mismatch of pulmonary root and aortic root
Connective tissue disorder
List 3 relative contraindications to Ross procedure
Primary aortic insufficiency
Systemic inflammatory disorders (RA, SLE, rheumatic heart disease)
Coronary artery disease
List in order the sinus of valsalva most likely to rupture and which chamber is it most likely to rupture into?
RCC is most likely and is most likely to rupture into the RV
NCC is second most likely and most likely to rupture into the RA
LCC is least likely and most likely to rupture into the LA
Name 4 inflammatory diseases that affect the aorta
Takayasu’s arteritis
Giant cell arteritis
Behcet’s disease
Ankylosing spondylitis
Rheumatoid arthritis
SLE
Eosinophilic granulomatosis with polyangiitis
Granulomatosis with polyangiitis
Polyarteritis nodosa
Name the 6 diagnostic criteria for Takayasu’s arteritis
3 or more of:
Age of onset <=40 years
Limb claudication
Diminished brachial pulse
Difference of >10 mmHg systolic pressure between arms
Bruit over the subclavian artery or aorta
Abnormal angiogram
What is the Hata and Numano classification for Takayasu’s arteritis?
Type 1 - Branches of aortic arch
Type 2a - Ascending aorta, aortic arch and its branches
Type 2b - Ascending aorta, aortic arch and its branches, and descending aorta
Type 3 - Descending thoracic and abdominal aorta and/or renal artery
Type 4 - Affects only the abdominal aorta and/or renal artery
Type 5 combines the features of both Type 2b and Type 4.
In addition, involvement of the coronary or pulmonary artery should be defined as C(+) and P(+), respectively.
What is the Ueno classification for Takayasu’s arteritis?
Type 1 - Ascending aorta + arch
Type 2 - Descending thoracic aorta to iliac bifurcation
Type 3 - Extensive (combined Type 1 and 2)
Type 4 - Pulmonary artery involvement
A(+) - i.e. Type 1A - means includes aneurysmal changes rather than stenotic-occlusive disease
What are the indications for surgery in Takayasu’s arteritis? Describe the Ishikawa clinical classification
Surgical intervention is indicated for patients with symptomatic stenotic lesions or concomitant aneurysm formation.
Conservative treatment is generally suggested for Ishikawa Group I or II. Surgery in Group III may increase survival.
Ishikawa clinical classification
Group I - uncomplicated disease +/- PA involvement
Group IIA - mild-moderate single complication
Group IIB - severe single complication
Group III - two or more complications
Complications
1. Takayasu’s retinopathy, secondary hypertension, aortic regurgitation, aortic or arterial aneurysm.
According the the AHA 2011 Management of Carotid and Vertebral Artery Disease Guidelines, list 4 indications for subclavian or brachiocephalic occlusive disease
- Symptoms or signs of anterior cerebral ischemia (stenting, arterial reconstruction, and extra-anatomical bypass are 1st line)
- Symptoms or signs of posterior cerebral ischemia (carotid subclavian is 1st line)
- Symptoms or signs of upper limb claudication (stenting, arterial reconstruction, and extra-anatomical bypass are 1st line)
- When an IMA is planned to be used for CABG (carotid subclavian or stenting are 1st line)
List 4 management strategies for braciocephalic occlusive disease
- Bypass (anatomic or extra-anatomic)
- Transposition
- Stenting
- Endarterectomy
- Hybrid approaches
Coronary-subclavian steal syndrome, how do you relieve it after CABG?
Aortic-subclavian bypass
Carotid-subclavian bypass
Transposition of the ITA
Endovascular procedures
