Laflamme- Chapter 7 (Congenital Heart + Pregnancy) Flashcards
Describe the Right atrial appendage?
-Large base, Triangular shape, Numerous pectinate muscles
What is left atrial appendage morphology?
-Narrow base, finger-shaped
What is the ductus venosus?
-Allows oxygenated umbilical blood to short-circuit the liver and reach the IVC
What is the foramen ovale?
-Allows blood derived from the IVC to go directly to the left heart perfusing the coronary arteries
What is the PDA?
Allows blood to go from the right heart to the aorta (to the placenta)
Order the 4 types of ASD by prevalence
-Secundum ASD (80%): In the fossa ovale
-Primum ASD (15%): Close to the AV valves associated with the AV canal defect
-Sinus Venosus (5%): Close to the origin of the SVC, associated with pulmonary venous connection
-Coronary sinus ASD (1%): Communication between the coronary sinus and the LA, associated with left sided SVC
What are two things that make an ASD large?
- > 10mm size
- Qo/Qs > 1.5:1
What is Lutembacher’s syndrome?
ASD + Rheumatic MS
What is Holt Oram syndrome?
-Autosomal dominant, TBX5 mutation, abnormalities of hands, secundum ASD
What are 4 physical exam findings of ASD?
-Fixed split S2
-Left parasternal heave
-TR murmur in the presence of right heart failure
-Palpable dilated PA
What are four complications of ASD?
-Right heart failure
-Atrial arrhythmias
-Paradoxical embolism
-PHT
Name 5 indications for closure of ASD?
-Dilation of right chambers
-Paradoxical embolism
-Platypnea-orthodexia
-PHT with marked left to right shunt > 1.5 :1 or with significant reactivity to vasodilator
-Do not close if irreversible PHT with sPAP > 2/3 SBP or PVR > 2/3 SVR
Name 3 anatomic criteria for percutaneous closure of ASD?
- Secundum ASD < 38mm with adequate margins
- > 5mm distance from AV valves, Coronary sinus, origin of SVC/IVC
- Normal pulmonary venous return
Also: ASVD ASD, Unroofed coronary sinus, Venosus ASD
What % of patients have PFO?
25%
How many beats needed within to see shunt/PFO on bubble study?
3 beats
What are the 4 types of VSD and describe them
Subarterial: Below the Ao and P valves , associated with AR
Membranous: Membranous septum, adjacent to Ao or T valves
AV canal: Below AV valve
Muscular: Completely surrounded by muscle
Name 7 complications of VSD?
-Left heart failure
-Pulmonary hypertension
-Progressive AR
-Double chamber RV
-Progressive subaortic stenosis
-Arrhythmias: AF, PVCs, NSVT, Post op block
-Endocarditis
What are 5 indications for VSD closure?
-Significant VSD: Symptoms or LV dysfunction, Qp:Qs > 2:1 or sPAP > 50mmhg
-Subarterial or membranous VSD with AR > moderate
-In the presence of severe PHTN (sPAP > 2/3 sbp or PVR > 2/3 SVR) closure should only be considered i nthe case of left to right shunt >1.5:1 or significant reactivity to vasodilators
-Double chamber RV with significant obstruction (MG > 40mmhg)
-Endocarditis
Name 5 complications of AV septal defect?
-Left and right AV regurgitation
-Left heart failure
-Right heart failure
-PHTn
-Arrhythmias
Name 6 physical exam findings of PDA?
-Continuous machinery murmur in left 2nd left IC space
-Increased pulse pressure
-Bounding pulse
-LV dilatation
-S3
-Associated thrill
What is echo finding of PDA?
-Flow reversal from aorta to PA visible on PSAX
-Diastolic flow reversal in the descending aorta
What are 4 complications of PDA?
-Left heart failure
-Progressive PHTn
-Arrhythmias
-Endarteritis/Ductus aneurysm
What are the consequences of supravalvular aortic stenosis?
-Coronary artery dilatation, stenosis, early CAD (due to high pressures on the coronaries)
What do you see on Ventriculography on Supravalvular stenosis?
-Ballerina foot pattern of LV
What genetic complex is associated with subvalvular aortic stenosis?
-Shone complex
What is the pathology for Aortic Coarctation?
-Stenosis in the region of the ligamentum arteriosum
Name 6 associated abnormalities with Aortic Coarctation?
-BAV
-Turner syndrome
-Shone complex
-VSD
-Aneurysm of the circle of willis
-Aortopathy
What are 4 congenital abnormalities associated with Turner Syndrome?
-Coarct
-BAV
-ASD
-VSD
What is criteria for significant coarct?
-HTN in the arms with trans-CoA gradient on catheterization > 20mmhg or with SBP in arms > 20mmhg higher than SBP i nlegs
What are physical exam findings in the absence of collaterals with coarct? absence of collaterals?
-Absence: HTN in arms, SBP in arms higher than in legs, radiofemoral delay on palpation of pulse, interscapular ejection murmur
-Presence: continuous crescendo-decrescendo murmur in left parasternal region of left scapula
What CXR sign do you see with coarctation and what is it caused by?
-3 sign: indentation of the aorta at the site of CoA with distal and proximal dilatation
What is the characteristic echo finding of Aortic Coarctation on echo?
-Increased velocity in descending aorta with diastolic extension of anterograde flow
-Decreased velocity of systolic flow and diastolic extension of antegrade flow
What are the 4 features of TOF?
-VSD
-Overriding Aorta
-RVH
-Subpulmonary stenosis
Describe the three steps of corrective surgery for TOF?
VSD closure -> RVOT disobstruction -> infundibulectomy or subannular patch or transannular patch
What are 4 associated abnormalities with TOF?
-DiGeorge syndrome (22q11 deletion)
-Right aortic arch
-Secundum ASD
-Congenital coronary artery anomalies
What are 7 post correction complications for TOF?
-PR
-RV dysfunction
-Residual RVOT obstruction
-Residual VSD
-LV dysfunction
-Aortic root dilation
-Arrhythmia (atrial or VT)
What are 6 indications for post-corrective surgery for TOF?
-Severe PR with mod-severe RV dilatation (EDV > 170)
-Residual VSD shunt >1.5:1
-Moderate to severe RVOT Obstruction
-Severe AR
-Aortic root > 55mm
-RVOT aneurysm or false aneurysm
What are 5 risk factors for VT for TOF?
-Inducible VT on EPS
-QRS > 180 msec
-Significant PR or RVOT
-NSVT on holter
-Syncope
Describe DTGA
-AV concordance, VA discordance
-RA connected to the RV which is connected to the Ao
-Position of the aorta is anterior to the right of the PA
What are associated abnormalities with D-TGA (5)?
-VSD, LVOT obstruction, ASD, Coart, Congenital coronary artery anomalies
Describe the two procedure that fix D-TGA ?
-Atrial Switch/Mustard: Blood is redirected to the atria by means of a conduit made from pericardium allowing physiological correction towards the subpulmonary LV Pulmonary venous blood is redirected towards the subaortic RV
-Arterial Switch: Switching of the Ao and PA
What are complications post DTGA repair? (7)
Systemic RV failure
Systemic RV ischemia
Systemic AV regurgitation
Obstruction of the baffle
Baffle leak
Arrhyhtmias
Subpulmonary obstruction
What is the criteria for Ebsteins anomaly?
-Apical displacement of the TV > 8mm
What are three associated abnormalities with Ebsteins?
-Accessory pathways
-Secundum ASD
-RVOT obstruction
What are four ECG findings of Ebsteins?
-RA dilatation/Himalayan P waves
-Pre-excitation
-RBBB
-Q waves in V1
What is the genetics of Marfan syndrome?
-Autosomal dominant FBN1 gene mutation
What are 5 cardiac issues with Marfan syndrome?
-Aortic dilatation
-MVP
-TVP
-AR
-Left heart failure
What are indications for Aortic repair for TAA in marfan?
- Ascending Ao > 50mm
-Ao > 45mm with progression > 5mm/year or progressive AR or family history dissection
-Ao > 40mm before pregnancy
What cut off is pregnancy contraindicated in TAA?
45 mm
Name 6 extra cardiac signs with Marfan?
-Pectus Carinatum
-Pectus excavatum
-Long limbs (Arm span to height ratio > 1.05)
-Long fingers
-Flat foot
-Lens dislocation
Describe ALCAPA syndrome
Anomolous left coronary artery arising from the PA
What are 6 clinical features of anomalous coronary arteries?
-Sudden death
-Retrosternal chest pain
-Arrhythmia
-LV dysfunction
-Syncope
What are 4 indications for surgery for anomalous coronary arteries?
-Coronary artery coursing between the great vessels with documented ischemia
-Ectopic origin of the LMCA coursing between the Ao and Pa
-Ectopic origin of the right coronary artery coursing between the Ao and PA with documented ischemia
-Ectopic origin of the LAD coursing between the Ao and Pa.
Name 5 hemodynamic changes with pregnancy?
-Increase 40-50% of plasma volume
-Increase 10-20% of HR
-Increase 30-50% of CO
-Decrease PVR
-Increase venous pressure in legs
Name 7 changes to the physical exam of a normal pregnant woman?
-Tachycardic
-Increased CVP
-Hyperdynamic state (pulse, apex)
-Laterally displaced apex
-Accentuated S2 split
-S3
-Left parasternal ejection murmur
What % of predicted functional capacity indicates a high risk pregnancy?
< 70%
What are the 4 components of the CARPREG score?
-History of HF, TIA, Stoke, Arrhythmia
-NYHA III or cyanosis
-MVA < 2 or AVA < 1.5 or LVOT > 30mmHg
-LVEF < 40%
Name 7 conditions where pregnancy is relatively contraindicated?
-Severe PAH
-Severe LV dysfunction
-Previous PPCM with residual impairment of LV function
-Severe MS or AS
-Marfan Ao > 4.5
-BAV Ao > 5
-Unrepaired Coa
How to monitor LMWH during pregnancy?
-Dose according to anti-Xa level 4h post injection (target 0.8-1.2) review once a week, replace by UFH > 36h
How to anticoagulate during pregnancy when Warfarin dose 5mg or less a day?
-Continue warfarin, replace by UFH at the end of pregnancy prior to planned delivery
How to anticoagulate in pregnancy if Warfarin > 5mg daily
-LMWH or IV heparin during the first trimester, replace by Warfarin for 2nd and 3rd trimesters, then replace by UFH prior to planned delivery
What are 5 risk factors for PPCM?
-Twin pregnancy
-AA
-Advanced age
-Pre ecmlampsia
-Smoking
How to manage PPCM?
-Hydralazine/Nitrates, Beta blocker, Digoxin, Diuretics
What is this?
Ballerina Foot Pattern -> Supravalvular Aortic Stenosis
What is this?
3 Sign -> Aortic Coarctation
What are 2 features of Severe PR?
-Continuous doppler regurgitation envelope is dense and reaches zero before the end of diastole
What are 4 prerequisites for a Fontan?
mPAP < 15mmhg
Normal sized PA
Normal RV function
Absence of significant AV regurgitation
What is this?
Scimitar Syndrome (Anomolous Pulmonary Vein into the IVC)
4 complications of Coronary AV fistula?
Dilation of the nutrient artery
Ischemia
Rupture
Endarteritis
6 contraindications for oral contraceptives?
-Cyanotic Heart Disease
-Atrial Fibrillation
-Mechanical Valve
-Thromboembolic history
-Fontan
-LVEF < 40%
