Congenital Heart Disease Flashcards

1
Q

Photo of fetal circulation (maybe a hot spot Q?)

A

The Placenta is a large low resistance vascular bed – PVR > SVR

CO is combined ventricular output, the RV is thicker and has 2/3rds of output, and LV is only 1/3rd

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

3 shunts of the fetal heart

A
  1. Ductus Arteriosus
  2. Foramen Ovale
  3. Ductus Venosus
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Ductus Arteriosus

A

largest vessel – protects the lungs against circulatory overload –> RV strengthens which increases pulmonary vascular resistance and decreases blood flow

  • should close within 24 hours of birth, yet can take 2 -3 weeks
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

How to close or keep ductus arteriosus open

A

Small opening: prostaglandin inhibitors (Indamethacin) to close … larger = surgery

if baby needs the shunt to get O2 rich blood –> PGE1 and bradykinin to KEEP DILATED

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

meconium aspiration

A

would continue PVR increase and fetal circulation to persist –> PULM HTN –> ECMO

worsened with hypoxia, acidosis, and hypothermia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Newborn Heart- Foramen Ovale

A

function: to shunt highly O2 rich blood from the RA to the LA
- Functional closure occurs in the first few hours after birth as the LAP > RAP
Probe patent foramen ovale = PFO
- 50% of 5 year olds
- 25% of 20 year olds

CONCERNED about: Paradoxical embolus
to prevent … SVR > PVR

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Ductus Venosus

A

Connects the umbillical vein to the inferior vena cava … flow is regulated though a eustachian valve

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Fetal Blood Flow

A

Blood enters from two areas: the IVC and SVC …

IVC from Moms blood (more O2) and SVC replies on patent foramen ovale and ductus arterosis d/t mixing of blood

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Fate of Shunts

A

First few days –> weeks is unstable circulation, PVR is still increased yet sensitive to changes

1st breath will DECREASE PVR
HPV is marked
PVR normalizes in 6wks ~ adult

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Newborn heart- Ventricular tissue

A

Fewer myocytes - 30%
Greater proportion of connective tissue
Relative RVH
- Decreased compliance
- More sensitive to preload
Ventricular interdependence
- Relatively noncompliant
- Relatively restricted in ability to change SV
- Cardiac output is more rate dependent
- By age 2-3 yrs CV system essentially that of a fit adult

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Autonomic Nervous System

A

PNS essentially complete at birth
SNS innervation of heart and vasculature incomplete - more bradycardia esp with stress (hypothermia, hypoxia, and acidosis)
Greater dependence on adrenal-circulating catecholamine system
Vagal tone predominates

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

normal neonate pressures

A
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

PEARLs about the preterm infant heart

A

More sensitive to depressant effects of inhaled agents - CO can decrease
Decreased responsive to catecholamines
Relatively high PVR persists
Pulmonary vasculature more sensitive to vasoconstriction by
* Hypoxia
* Acidosis
* Hypercarbia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Acyanotic Lesions

A

L – R shunt
Atrial septal defect (ASD)
Ventricular septal defect (VSD)
Atrioventricular Septal defect (AV Canal)
Patent ductus arteriosus (PDA)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Types of Atrial Septal Defects

A

1 Secundum ASD – at the Fossa Ovalis, most common.

There are 3 major types:

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

ASD Clinical Signs & Symptoms

A

Rarely presents with signs of CHF or cyanosis
Increased fatigability
Mild failure to thrive, can’t suck well
May have associated pulmonary hypertension –>cyanosis
Hyperactive precordium – RV heave – fixed split S2
II-III/VI systolic ejection murmur @LSB
Mid-diastolic murmur @LLSB
- afib if overloaded d/t stretch of atrial fiber muscles

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Treatment of ASD

A

Surgical or Cath closure for secundum ASD with Qp:Qs (flow) ratio > 2:1
Closure performed electively 2-5 years
Surgical correction earlier in children with CHF or pulmonary hypertension
Mortality <1%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Ventricular Septal Defect Types

A
  • Perimembranous (or membranous) – Most common.
  • Infundibular (subpulmonary or supracristal VSD) – involves the RV outflow tract.
  • Muscular VSD – can be single or multiple.
  • AVSD – inlet VSD, almost always involves AV valvular abnormalities.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

VSD Signs and Symptoms

A

Elevated RV & Pulmonary pressure
Hypertrophy
Small – moderate VSD usually asymptomatic – 50% will close by 2 years
Moderate – large VSD – symptomatic – surgical repair
II-III/VI harsh holosystolic murmur @ LSB
Prominent P2 – diastolic murmur
CHF – Respiratory failure

seen from birth - 6 mo.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

VSD Treatment

A

Small = no surgical intervention
Symptomatic = initial medical treatment with diuretics and afterload reducers
Surgical closure
Large VSD
Ages 6 – 12 mos. large VSD & pulmonary HTN
Ages > 24 mos. With Qp:Qs ratio > 2:1
Supracristal VSD of any size (aortic valve involvement)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Atrioventricular Septal Defect

A

Complete
Low primum ASD continuous with posterior VSD
Cleft in both septal leaflets of TV/MV
Large L – R shunt
TR/MR – pulmonary HTN
Incomplete
Primum ASD with cleft in MV & small VSD
Hemodynamics are dependent on lesion

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

AVSD – Signs & Symptoms

A

Incomplete may be indistinguishable from ASD
CHF
Recurrent pulmonary infection
Failure to thrive
Easy fatigability
Late cyanosis from pulmonary vascular disease w R to L shunt
Hyperactive precordium
Accentuate 1st heart sound
Wide splitting of S2
Pulmonary systolic ejection murmur w/thrill
Holosystolic murmur at apex w/radiation to axilla
Mid-diastolic rumbling murmur @ LSB
Marked cardiac enlargement on CX-ray

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

AVSD Treatment

A

Treat CHF symptoms
Surgery is always required
Pulmonary banding may be required in premature infants or infants < 5Kg
Mortality low w/incomplete 1 – 2%
Mortality w/complete 5%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

Patent ductus arteriosus (PDA) Signs and Symptoms

A

Small - asymptomatic
Large – may have symptoms of CHF – FTT – growth retardation
Bounding arterial pulses
Widened pulse pressure
Enlarged heart – prominent apical impulse
Continuous systolic murmur
Mid-diastolic murmur at apex
PAP = systolic pressures with larger PDA

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
PDA associated infections?
TORCH Toxoplasmosis Other: syphilis, varicella-zoster, parvovirus) Rubella CMV Herpes
26
PDA Treatment
Indomethacin (Prostaglanin inhibitor) Surgical or catheter closure - Ligation & division - Intravascular coil - Mortality is <1%
27
Cyanotic Lesions
R –> L shunt ... body-body Tetralogy of Fallot Total anomalous pulmonary venous return Truncus arteriosus Transposition of the great arteries Tricuspid atresia Hypoplastic left heart syndrome (also obstructive)
28
TOF
most common right obstructive lesion - RV hypertrophy - overriding aorta with mixed blood - R --> L shunt - PVR > SVR and diminished pulmonary blood flow - cyanotic, no murmur may have aortic ejection click
29
Tetralogy of Fallot: Preoperative Preparation
Heavy premedication Consider IM ketamine or inhalation induction but get rapid control of airway. (crying worsens hypoxia) Keep SVR up and PVR down, maintain heart rate Intraoperative TEE Oral premed/induction midazolam + ketamine
30
Tetralogy of Fallot: Perioperative concerns
Increase in PVR or decrease in SVR leading to Right to Left shunt Tet Spells pre induction (crying/anxiety) Polycythemia and bleeding - (polycythemia d/t kidneys sensing hypoxia, releasing more erythropoietin to make more RBCs) Air embolus RV failure (d/t pulmonary pressures being high and volume overload)
31
Intraop TOF goals
Maintain adequate tissue oxygenation and minimize R-->L shunt Avoid increasing O2 demand ***Maintain SVR, systemic BP - ketamine - vasopressin - phenylephrine Minimize PVR Avoid myocardial depression Avoid dehydration (especially if polycythemia is present) Minimize RVOT obstruction and PVR - O2 - Betablocker: propranolol - no air in lines - no N2O - infectious endocarditis prophylaxis
32
intraop drugs for TOF
Maybe: nitroglycerin phentolamine tolazoline prostaglandin E1 nitric oxide
33
Tet “spells”
Tachycardia - pallor - loss of consciousness - seizures - cardiac arrest Treatment Knee-chest position: to relax stenosis while maintain SVR O2 Morphine 0.1 to 0.2 mg/kg IM or IV: to decrease resp drive, and provide comfort Phenylephrine - to keep SVR UP and increases peripheral return by vasoconstriction of arterioles Propranolol 0.1 mg/kg: relieves spasm of infundibular muscle of the RVOT ABG - NaHCO3 if necessary Surgery
34
Total Anomalous Pulmonary Venous Return (TAPVR)
Pulmonary veins do NOT return back to LA TYPES: "Supra" cardiac : Pulm veins attached to Superior Vena cava "Infradiaphragmatic" : pulm veins -- inferior vena cava "Intra-cardiac": pulm veins - RA "Mixed" L veins to supra, and R veins to infra
35
TAPVR Repair
reroot the pulmonary veins to the LA and repair the ASD
36
Transposition of the Great Vessels Periop Concerns
Maintain Cardiac output with adequate HR Continue PGE1 Reduce PVR and maintain SVR Opioid/pancuronium technique Blood loss may be significant – As with all cardiac surgical procedures.
37
Truncus Arteriosus
Aortic and Pulmonary artery are ONE single vessel... VSD is essential for oxygenated blood --> brain and body
38
Truncus Arteriosus Treatment
- Digoxin: CA to strengthen the heart muscle and squeeze - Diuretics: for water balance - Ace inhibitors: to dilate vessels and decrease afterload, making it easier to pump
39
Transposition of Great Arteries
aorta and pulmonary artery SWAPPED - has PDA and PFO -- essential - RV is responsible for systemic pumping which its not ready for - LV hypotrophic -- not enough strength to pick up systemic pumping after correction
40
Transposition of Great Arteries surgeries
Mustard repair -- older surgery, patients now 30-60 yrs Arterial switch -- newer, swap with carotids switched
41
Transposition of great vessels Periop
Maintain Cardiac output with adequate HR Continue PGE1 Reduce PVR and maintain SVR Opioid/pancuronium technique Blood loss may be significant – As with all cardiac surgical procedures.
42
Tricuspid atresia
- More than 70% patients are severely cyanosed due to inadequate pulmonary flow through the PDA - Need a systemic to pulmonary shunt: Variety used, most common Fontan - Where pulmonary flow is high the PA is banded; Assessment of RV function is important here - Definitive repair leads to a cavopulmonary anastamosis (Fontan) and this is sometimes two staged, i.e. Hemi-Fontan or Bidirectional Glenn being the intermediary stage. - Patients present for BT shunt often on PGE1 - Meticulous airway management is key to maintain flow balances
43
Tricuspid Atresia - potential surgical issues
Hypoxemia: hypovolemia, low PBF CHF: volume shifts, anemia, and HTN Thrombosis: vena cava, RA, and pulmonary artery repair cant be done until 6-8weeks
44
Goals during surgery with tricuspid atresia
Monitor RA pressure via RA catheter, and maintain baseline pressure maintain systemic BP near baseline minimize myocardial depressants no air in line no N20 High FiO2 Normal HCT
45
Tricuspid atresia - treatment
Definitive repair leads to a cavopulmonary anastamosis (Fontan) and this is sometimes two staged, i.e. Hemi-Fontan or Bidirectional Glenn being the intermediary stage. Patients present for BT shunt often on PGE1 Meticulous airway management is key to maintain flow balances
46
Hypoplastic Left Heart Syndrome
cyanotic and obstructive disease -- blood flow via PDA (keep on PGE1s) - also have aortic atresia, LV and mitral valve hypoplastic pulmonary over circulation - inadequate systemic circulation - PULM HTN
47
hypoplastic left heart - treatment
Two options: 1. Cardiac transplantation 2. 2 or 3 stage procedure: New aorta created from the pulmonary artery Atrial defect is created to completely mix blood Pulmonary flow improved by shunt e.g. BT Later: Fontan (+/-preceded by a hemi Fontan)
48
Hypoplastic Left Heart Syndrome (HLHS) - Anesthetic Management
Maintain HR, preload and PGE1 Balance SVR and PVR Avoid too high PaO2 May need CO2 to avoid pulmonary over perfusion and hence systemic hypo perfusion Inotropic support may be necessary to support the ventricular (Milrinone, caution d/t decrease in PVR)
49
Pulmonary Stenosis
50
Pulmonary Stenosis Signs and Symptoms
Asymptomatic w/mild PS Mod – severe = RV failure Prominent jugular a-wave Split 2nd heart sound Ejection click Heart failure and cyanosis in severe cases
51
PS treatment
Mild – no intervention – close F/U Mod – Severe – relieve stenosis - Balloon valvuloplasty - Surgical valvotomy
52
Aortic Stenosis
obstruction to outflow from LV TYPES: 1. valvular (common) 2. subvalvular or subaortic - poor CO and forward flow 3. supervalvular (@ ascending aorta)
53
Coarctation of the Aorta
Narrowed Aorta ...
54
Coarctation Signs & Symptoms
Diminution or absence of femoral pulses Higher BP in the upper extremities as compared to the lower extremities 90% have systolic hypertension of upper extremities Pulse discrepancy between R and L arm Severe = Heart Failure - LE hypoperfusion – acidosis– shock II/VI systolic ejection murmur LSB Cardiomegaly
55
Coarctation Treatment
Severe coarctation – maintain ductus with = PGE1 - initial meds with inotropes and diuretics Surgical intervention to prevent LV dysfunction Angioplasty - balloon angioplasty @ 18months
56
Anesthesia for Non-Cardiac Surgery in Patients with Congenital Heart Disease
Congenital cardiac abnormalities are in 1% live births Rapid growth in corrective and palliative procedures Increasingly likely that anesthesia providers will encounter these cases coming for non-cardiac surgery
57
Anesthesia and CHD - must know...
the anesthetist understands the pathophysiology of the lesion and the pharmacology of the drugs used
58
Spectrum of disease concerns
Spectrum of disease: Congenital heart disease NOT YET treated Surgically corrected and symptom-free Surgically corrected but with residual problems Surgically palliated but stable Surgically palliated but still with severe symptoms or new problems
59
Questions to ask:
Questions to ask: Should the patient be referred for specialist cardiology service before surgery? Is entire procedure more suited to a specialist center? If we decide to proceed or are forced by an emergent event to proceed what do we need to know?
60
VSD considerations
VSD: L-R shunts and pulmonary overload pre-correction Endocarditis and arrhythmias post-correction - no air in lines - no N2O - endocarditis prophylaxis - maintain PVR - normal ventilation with PaCO2 = 40s - FiO2 <100% - Lower SVR via inhalational agents and propofol
61
ASD considerations
ASD: L-R shunts and pulmonary overload pre-correction Potential for paradoxical air embolus Endocarditis and arrhythmias post-correction
62
Pulmonary to System Flow Ratio
63
How to INCREASE PVR
Hypoxia Hypercarbia Metabolic acidosis Hypothermia IPPV Alpha adrenergic stimulation
64
how to decrease PVR
Oxygen Hypocarbia Metabolic alkalosis Volatile anesthetics Vasopressin PDE inhibitors
65
PDA considerations
Late problems rare once corrected
66
Atrioventricular septal defect considerations
Common in Down’s syndrome Can cause heart failure and pulmonary HTN Mitral regurgitation may persist after correction Endocarditis and arrhythmias post-correction
67
Hypoplastic Left Heart Syndrome considerations
Before surgery pulmonary circulation needs a patent duct Three stage palliative surgery lead to single ventricle and pulmonary flow through cavopulmonary connections Palliation may lead to heart failure and arrhythmias
68
Transposition of Great Vessels - considerations
Balloon septoplasty in first few hours of life Arterial/atrial switch performed depending age Residual risks of endocarditis, arrhythmias and right ventricular (systemic) failure.
69
Cyanosis present
Indicates persistent abnormality Associated with polycythemia altered hemostasis Ensure hydration, maintain SVR and reduce PVR Use high FiO2 Avoid sudden increase in oxygen requirement Meticulous removal of air
70
Pulmonary atresia - considerations
Treatment and outcome depend on a VSD Repeat palliations and residual cyanosis possible
71
Speed of induction
R --> L Shunt (body-body): slower inhalational, faster IV L --> R (lung-lung): inhalational maybe fater, IV slower
72
Pulmonary Disease present
Associated pulmonary lesions, vascular rings, compression form shunts, phrenic or RLN damage Rising PVR can eventual lead to a Rt to Lt shunt through an ASD or VSD, Eisenmenger’s syndrome End-tidal CO2 will frequently underestimate PaCO2 due to reduced pulmonary flow and increased dead space
73
if cardiac failure is present
Elective surgery should be postponed In emergent situation, invasive monitoring is mandatory and usually IPPV should be helpful except where there are cavopulmonary shunts that cause passive blood flow to the lungs
74
if arrythmias are present
Common problems post correction SVT, VT and complete heart block all seen
75
antibiotics and anticoagulation considerations
- Endocarditis risk has to be assumed in all but the most simple congenital cardiac lesions especially if uncorrected - Generally speaking anticoagulated patients should be switched from coumadin to heparin closer to the time of surgery and then d/c perioperatively - Emergent surgery, on balance, should call for reversal of anticoagulation
76
Pt's with single ventricle
- HLHS or pulmonary atresia with intact septum - Shunts inevitably cause some degree of cyanosis (eg BT shunt) - As child grows pulmonary flow inadequate and cavopulmonary shunts are needed (Fontan) - Spontaneous (negative pressure) ventilation - Morphology of “ventricle” determines outcome - Arrhythmias and ventricular failure are real risks