Congenital heart diseases Flashcards

(47 cards)

1
Q

Patent ductus arteriosus (PDA)

Pathophysiology

A
  • Hereditary hypoplasia of the ductal smooth muscle causes the duct to remain open instead of colapsing at birth
  • Pulmonary artery resistance is lower than systemic resistance, blood shunts through the PDA from the aorta to the pulmonary artery (L to R shunt) –> volume overload of the L heart, eccentric hypertrophy, CHF
  • In very large PDAs, over circulation of the lungs may lead to inc. pulmonary vascular resistance –> flow in PDA shifts from L-R to R-L (usually ~6-8wks)
  • R to L shunting:
    • Over-circulation of the R heart which might lead to hypertrophy and R sided CHF
    • Blood in descending aorta has low PaO2 –> kidneys produce EPO –> erythrocytosis, hyperviscosity
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2
Q

Patent ductus arteriosus

Signalment

A
  • Seen commonly in toy and mini poodles, GSDs, and collies
  • Detected in puppies coming in for their first vaccine
  • More common in females
  • Cats die w/in a few weeks after birth with PDA
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3
Q

PDA

Probable owner complaint/usual history

A

Usualy asymptomatic; incidental finding during puppy vaccines

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4
Q

PDA

Common abnormalities on PE

A
  • Continuous machinery murmur–heard more in the dorsal 1/3 of the chest
    • Remember where defect is–have to listen under triceps
    • Murmur can sometimes only be heard at the thoracic inlet
    • Percordial thrill, bounding, water-hammer femoral pulse
  • Animals with R-L shunts may not have an audible murmur
    • Caudal cyanosis (vulva, penis)
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5
Q

PDA

Abnormalities found on special exam

A
  • Blood gas
    • Normal for L-R
    • Decreased caudal PaO2 and erythrocytosis in R-L
  • Rads
    • May see signs of L/R heart enlargement based on direction of shunt
    • May be able to see ductus diverticulum–best seen on DV
    • Prominent lung vessels in L-R shunts
      • Appear under-perfused in R-L
  • Echo
    • Signs of L/R heart enlargement
    • May see signs of elongated coniacl shape of the duct
  • Doppler–turbulence in the pulmonary artery distal to the pulmonic valve
  • Angiography
    • Aortic root or L heart injection to demonstrate L-R shunt
    • Jugular or R heart inj to see R-L shunt
    • Medium will fill pulmonary artries and aorta at the same time and allow for visualization of chamber enlargement
  • ECG–signs of L or R heart enlargement, arrhythmias if present
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6
Q

PDA

DDx

A
  • Concurrent aortic stenosis and aortic insufficiency
  • Pulmonic stenosis with regurg
  • VSD with aortic regurg
  • Aorticopulmonary window
  • Truncus arteriosus
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7
Q

PDA

Treatments

A
  • L-R
    • Surgical closure–isolated and tied closed with umbilical tape or closed with embolization coils or Amplatz canine duct occluders (ACDO)
    • Close ASAP if detected at young age (stabilize heart failure patients first)
  • R-L
    • Cannot surgically close–acute right heart failure
    • Treat clinical signs with medication
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8
Q

PDA

Prognosis

Prevention

A
  • Prognosis
    • W/o treatment 65% die w/in a year–most in heart failure by 16 months
    • 95% survive after surgery–excellent prognosis post-op
    • R-L shunts can be medically maintanined for 3-5 years at very low levels of activity
  • Prevention
    • Do not breed affected animals
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9
Q

Aortic stenosis

Pathophysiology

A
  • Fibrocartilaginous CT that completely or partially encircles aortic outflow tract
  • May be subvalvular (95% of dogs), valvular, or supervalvular
  • Increases afterload –> left ventricular concentric hypertrophy
  • Decreases diastolic filling and CO
  • Predisposes to ventricular dysrhythmias
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10
Q

Aortic stenosis

Signalment

Probable owner complaint/usual history

A
  • Signalment
    • Genetically transmitted in Newfoundlands
    • Other predisposed breeds: boxers, rotts, retrievers, GSDs, GSHPs
  • Complaint/history
    • Incidental finding in asymptomatic dogs at puppy vaccines
    • Most common signs are syncope or sudden death
    • May not develop murmur until ~3 months of age
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11
Q

Aortic stenosis

Common abnormalities on PE

A
  • Systolic murmur loudest over L heart base, radiates up carotid
  • Hypokinetic femoral pulses
  • To-and-fro murmur and hyperkinetic pulses if aortic insufficiency and regurg present
  • Possible ventricular heave–apical impulse
  • VPDs
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12
Q

Aortic stenosis

Abnormalities found on special exam

A
  • Rads–appear normal
    • Concentric hypertrophy hard to detect
    • Dilation of ascending aorta (lateral view)
  • Angio–small left ventricular cavity, post-stenotic dilation of the aorta
  • Echo–concentric left ventricular hypertrophy, subvalvular echogenic ridge/band, narrowing of L ventricular outflow tract
  • M-mode–left ventricular hypertrophy
  • Dopler–inc. velocity across aortic valve w/ regurg
    • 1.5-3m/s = mild
    • 3-4.5m/s = moderate
    • 4.5-5m/s = severe
    • <2.4m/s and no abnormalities seen = uncertain–recheck
  • Catheterization–pressure gradient across the aortic valve
    • <40mmHg = mild
    • >80mmHg = severe and high left ventricular end-diastolic pressure
  • ECG–usually normal
    • L ventricular enlargement
    • ST depression
    • VPD
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13
Q

Aortic stenosis

DDx

A
  • Atrial setal defect
  • Other causes of continuous murmurs or syncope
  • Physiological murmurs
  • Bact. endocarditis of aortic valve
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14
Q

Aortic stenosis

Treatment

A
  • Beta-blockers
    • Recommended in cases of syncope, moderate-severe gradients (~4m/s), ventricular arrhythmias or ST changes
    • Do not change pressure gradient, but reduce clinical signs and decrease risk of sudden death (antiarrhythmic, dec. myocardial O2 demand)
    • Warn owners that these drugs cannot be stopped abruptly–must titrate
    • Maximum safe dose
    • May improve diasstolic function–improve distensibility and diastolic filling
    • Caution: (-) inotropic/chronotropic effects
  • Sx correction or balloon valvulolasty–lowers pressure gradient, but does not inc. survival rate
  • Positive inotropes contraindicated
  • Dogs w/ CHF: standard therapy w/ diuretics and vasodilators should be used
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15
Q

Aortic stenosis

Prognosis

Prevention

A
  • Prognosis
    • Progressive (rapid in young dogs)
    • ~20% die suddenly before 3yrs of age
    • Depends on severity of lesion:
      • Severe: ~70% die before age 3
    • >3yrs old–usually mild lesion
      • Dependent on severity of outflow velocity and pressure gradient
        • <4m/s = normal life
        • >5m/s = likely to succumb
    • Predisposed to endocarditis
  • Prevention–do not breed affected dogs (dogs >12mo can be certified free of congenital heart dz)
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16
Q

Pulmonic stenosis

Pathophysiology

A
  • Stenosis may be subvalvular, valvular (88%), or supravalvular
  • Valve cusps may be fused
  • Commonly dysplastic–thickened and asymmetrical
  • Sometimes hypoplastic–valve annulus
  • Causes concentric hypertrophy of the R ventricle
  • High velocity through stenotic valve causes post-stenotic dilation of the pulmonary artery trunk
  • R atrium enlarged due to R ventricular filling ressures
  • May predispose to arrhythmias
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17
Q

Pulmonic stenosis

Signlament

Probable owner complaint/usual history

A
  • Signalment
    • Most common in English bulldogs, Scottish terriers, mini schnauzers, and wirehaired fox terriers
    • Polygenic in beagles
  • Complaint/history
    • Incidental finding during pupy vaccines
    • If clinical signs are present the lesion is more severe and forward heart signs are seen
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18
Q

Pulmonic stenosis

Common abnormalities on PE

A
  • Systolic heart murmur of L heart base
    • Radiates up the neck
  • Possible R CHF signs
  • Pulses and mm mostly normal
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19
Q

Pulmonic stenosis

Abnormalities found on special exams

A
  • Rads
    • R ventricular enlargement (inc. sternal contacton lat view) w/ post-stenotic dilation of the pulmonary trunk (1:00) on VD/DV views
    • Possible ascites and hepatomegaly
  • Echo
    • R ventricular hypertorphy, flattened sternum, large R atrium, restricted motion of the pulmonic valve
    • Post-stenotic dilation
  • M-mode–thickened septum and R ventricular free wall
  • Doppler
    • Velocity across pulmonic valve >1.2m/s suggests pulmonic stenosis (esp. if >2.0m/s)
    • Inc. pressure gradient
      • >80mmHg = severe
      • 10-50mmHg = mild
      • Pulmonic regurg
  • Angio
    • Narrowed passage in valve area
    • Thickened valves, post-stnotic dilation
  • Catheterization–gradient across pulmonary valve region
  • ECG–R ventricular enlargement; occasionally arrhythmias
20
Q

Pulmonic stenosis

DDx

A
  • Subaortic stenosis
  • Aortic septal defects
21
Q

Pulmonic stenosis

Treatment

A
  • Balloon valvuloplasty
    • Recommended for dogs with severe to moderate stenosis, those with clinical signs, or with moderat to severe right ventricular concentric hypertrophy/fibrosis/ischemia
    • Balloon catheter inserted via jugular vein, inflated at stenotic valve–tears stenotic tissue and reduces narrowing
    • Post-dilation pressures should be <50mmHg
    • Control heart failure and dysrhythmias as necessary pre-op
  • Annular ring hypoplasia or infundibular hypertrophy–patchgraft valvuloplasty
  • Single right coronary type R2A (bulldogs, boxers)–right ventricular to pulmonary artery conduit
  • Atenolol can be used to try and imrove diastolic function and arrhythmias if sx not possible
22
Q

Ventricular setal defect (VSD)

Pathophysiology

A
  • Failure of development of the setum between the ventricles
  • Severity depens on size of defect
  • Most occur in the membranous part of the septum allowing blood to flow from the L ventricle directly to the pulmonary arteries–> minimal effects of the R ventricle
  • Volume overload of the L ventricle results from increased venous return–L heart enlargement and poss. failure
  • Larger openings and those lower down the septum can increase the pressure in the R ventricle and lead to R heart enlargement as well
23
Q

VSD

Signalment

Probable owner complaint/history

A
  • Signlament
    • More common in cats
    • English bulldogs and keeshonds predisposed, but can occur w/o familial history
  • Complaint/history
    • Smaller defects usually incidental findings during pediatric vaccinations
    • Animals w/ larger defects may be stundted and exhibiting signs of L sided heart failure (syncope, organ dysfunction, exercise intolerance)
24
Q

VSD

Common abnormalities on PE

A
  • Systolic murmur–loudest over R sternal border
  • Smaller defects will have louder murmurs
  • Possible thrill over R hemithorax
25
**VSD** Abnormalities found on special exams
* Rads * Prominent pulmonary vessels * Signs of L heart enlargement--'tracheal bump' * Trachea in line w/ spinal column * If R heart enlarged may see inc. sternal contact * Echo * Can be seen and measured--need to distinguish from 'septal drop out' (common artifact) * Contrast can be used * Enlargement * Doppler * **Definitive diagnosis by documenting L-4 flow** * Can calculate size of the defect * Angio * Dye injected into L heart will enter R ventricle during systole * Die inj. into R heart will appear diluted after dye-free blood enters during systole * ECG * May show L/R heart enlargement * oss. right bundle branch block
26
**VSD** DDx Prognosis Prevention
* DDx * **Loud R-sided murmur possibly diagnostic** * Prognosis * Mild-moderate = good to excellent * Occasionally defect will spontaneously close * Development of pulmonary hypertension w/ R-L shunting results in a poorer prognosis (6 mo) * Prevention * Do not breed animals w/ defect
27
**VSD** Treatment
* Small VSDs--no treatment required, some may spontaneously close * Severe VSDs * Surgical repair is an otion but will require bypass--open heart surgery * Pulmonary banding--decreases the diameter of the pulmonary arteries --\> increase resistance and decrease shunting across the defect --\> less volume overload * Arterial vasodilators--asymptomatic animals with L heart enlargement * Reduce degree of L-R shunting * Hydralazine used--beware of GI upset, monitor BP closely
28
**Tetralogy of Fallot** Pathophysiology
* Lesion includes: pulmonic stenosis, overriding aorta (dextropositioning), VSD, hypertrophy of the R ventricle * Hemodynamic consequences depend on size of VSD and extent of pulmonic stenosis * Less severe stenosis will result in a presentation similar to VSD * L-R shunting--**rare** * **Severe stenosis more common**, and R-L shunting occurs * Deoxegenateed blood is nnot pumped into the lungs and instead goes out into circulation --\> cyanosis and kidney EPO prod. * CHF **rarely** occurs b/c the R-L shunting allows both ventricles to share the increase pressure * Right ventricular hypertrophy predisposes to arrhythmias
29
**Tetralogy of Fallot** Signalment Probable owner complaint/history
* Signalment * Autosomal recessive in Keeshond * Wirehaired terriers and English bulldogs predisposed * Complaint/history * Stunted growth * Exercise intolerance * Shortness of breath * Syncope
30
**Tetralogy of Fallot** Common abnormalities on PE
* Murmur heard during 1st vaccine visit (both sides of chest) and most patients are cyanotic * Might be a systolic murmur of pulmonic stenosis at the L heart base and a VSD murmur on the right * Murmurs may be very soft if there is erythrocytosis, severe stenosis, or minimal flow through the VSD
31
**Tetralogy of Fallot** Abnormalities found on special exams
* Lab--erythrocytosis, inc. PCV 60-70% * Rads--R heart enlargement, small pulmonary vessels * Echo * Overriding aorta and VSD * Hypertrophy of R ventricle * Doppler--inc. peak flow across pulmonary valve, shunting through VSD * Angio--ID's the VSD, R ventricular hypertrophy, pulmonic stenosis, and direction of the shunt * Catheterization--measurement of the pressure gradient across the VSD * ECG--possible R ventricular enlargement
32
**Tetralogy of Fallot** DDx Prevention
* DDx = other causes of cyanosis * Prevention = do not breed animals w/ this defect
33
**Tetralogy of Fallot** Treatment Prognosis
* Surgical procedures to re-route blood--variable success * Subclavian attached to pulmonary artery to increase oxygenation or connect aorta to pulmonary artery--like PDA * Beta blockers--possibly lessen MVO2 of R ventricle or imrove distensibility * Hydroxurea--anti-cancer drug, blocks RBC production * Phlebotomies and replace blood with sterile saline; low dose aspirin to prevent thromboembolism * **Vasodilators are contraindicated**--will make R-L shunting worse and increase hypoxia * Prognosis--depends on the severity; some dogs live a long (though inactive) life
34
**Atrial septal defect** Pathophysiology
* "Common atrium" * Blood flows to right atrium because of its thinner, distensible walls * R ventricular enlargement due to volume overload * Enlarged pulmonary vessels * L-R shunt
35
**Atrial septal defect** Signalment Probable owner complaint/history
* Signalment--found in younger animals during pediatric vaccinations * Complaint/history * Small defects present asymptomatically * Larger defects may present with heart failure signs
36
**Atrial septal defect** Common abnormalities seen on PE Abnormalities found on special exam DDx
* PE * Possible murmur heard over L heart base * May hear splitting of S2 * Rads--R heart enlargement, prominent pulmonary vessels * DDx--pulmonic or aortic stenosis
37
**Atrial septal defect** Treatment Prognosis Prevention
* Treatment * Dependent on size of the defect--most with small defects will remain asymtomatic * Surgical correction of large defects will require bypass * Prognosis--depends on size; worse if tricuspid stenosis is also involved * Prevention--don't breed
38
**Atrioventricular valve malformations** Pathophysiology
* Thickened or fused valves * Papillary muscles that are malpositioned, partially developed, or absent * Chordae tendinae that are too long, too short, or absent * Valvular insufficiency most commonly seen with dyslastic valves failing to meet during systole--\> regurg and volume overload * Valvular stenosis (rare)--stenotic valves decrease ventricular filling during diastole --\> inc. atrial pressure and CHF
39
**Atrioventricular valve malformations** Signalment Probable owner complaint/history
* Signalment * Most common congenital malformation in cats * Large breed dogs may be predisposed * History * Signs depend on the valve involved--L or R CHF signs
40
**Atrioventricular valve malformations** Common abnormalities seen on PE Abnormalities seen on special exam
Valve insufficiency--depend on valve affected
41
**Atrioventricular valve malformations** DDx Prevention
* DDx * Other causes of diastolic murmurs--PDA, possible aortic stenosis * Prevention--don't breed
42
**Atrioventricular valve malformations** Treatment Prognosis
* Treatment * Furosemide and an ACE inhibitor * Arterial vasodilators may be helpful by reducing regurg and promoting forward flow * Low sodium diet may be helpful * Prognosis * Depends on severity of defects
43
**Endocardial fibroelastosis** All the things
* Seen more in Burmese and Siamese kittens * Fibrosis of ventricular endocardium--\> stiffening of walls--\> decrease ability to dilate and fill during diastole * Left CHF by the time the animal is 2-4mo * **No treatment**
44
**Persistent right aortic arch (PRAA)** Pathophysiology Signlament
* Vascular ring formed by the ligamentum arteriosus dorsally, aorta to the right, pulmonary artery to the left, and cardiac base ventrally--constricts esophagus and trachea * Signalment: common occurrence in GSDs
45
**PRAA** Signs Abnormalities on special exam
* Signs * Megaesophagus cranial to the constriction = regurg of solid food * Asymptomatic at young age (liquid diet) * Rads: esophageal dilation cranial to the heart base
46
**PRAA** Treatment Prognosis DDx
* Treatment--surgical ligation and transection of LA * Guarded prognosis--esophageal fx may remain abnormal * Animals at risk for aspiration pneumonia * DDx: congenital megaesophagus
47
**Inherited ventricular arrhythmias in GSDs** All the things
* Possible genetic link in some lines of GSDs, otherwise idiopathic * Starting at 3 months, animals may be affected w/ mild VPDs or eisodes of VT and poss. sudden death * Severity will inc. until 7mo and then decreases * By 18mo risk of sudden death is markedly dec. * Severely affected animals can be given sotalol and mexiletine to dec. episodes of VT (**does not prevent sudden death)** * Sotalol alone is roarrhythmogenic and mexiletine alone has no affect * Drug trtmt until animals are 18 mo to 2 yrs