Download Pediatric Cardiothoracic Surgery

Pediatric Cardiothoracic Surgery
Bjarni Torfason, dósent
Yfirlæknir hjarta- og lungnaskurðdeildar
Landspítalans
Háskóli Íslands Læknadeild
Cardiothoracic Surgery
Undirsérgreinar:
•
•
•
•
Adult Cardiac Surgery
General Thoracic Surgery
Pediatric Cardiothoracic Surgery
Transplantation
Pediatric Cardiothoracic Surgery
• Thorax
–
–
–
–
Veggur
Pleura
Lungu
Mediastinum
• Hjarta og æðar í thorax
– Meðfæddir galla
– Áunnir gallar/ sjúkdómar
Pediatric Cardiothoracic Surgery
• Thorax
–
–
–
–
Veggur
Pleura
Lungu
Mediastinum
• Hjarta og æðar í thorax
– Meðfæddir gallar
– Áunnir gallar/ sjúkdómar
Pectus carinatum
Pectus excavatum
Pectus excavatum - Nuss
Pectus excavatum - Nuss
•
Viðmið:
a/b > 3,2
Video
10:46
Spangartaka í ágúst 2007
3 árum frá Nuss reconstruction
Pediatric Cardiothoracic Surgery
• Thorax
–
–
–
–
Veggur
Pleura
Lungu
Mediastinum
• Hjarta og æðar í thorax
– Meðfæddir gallar
– Áunnir gallar/ sjúkdómar
Thorax Feb 2005 Vol 60 Suppl 1
www.brit-thoracic.org.uk
BTS guidelines
for the
Management of Pleural Infection
in Children
Dr Ian Balfour-Lynn
Royal Brompton Hospital
3 year old boy – 1w fever, malaise, cough, DIB
IVABs no improvement so transferred
Drain inserted, urokinase, IV cefuroxime
B/C – Pneumococcus. Pleural fluid - sterile
4 days later – well but febrile, drain out & home next day
Back to normal by 2 weeks
7 week follow up -
Paediatric Pleural Diseases Subcommittee
of the BTS Standards of Care Committee
•
•
•
•
•
•
•
•
•
Dr Ian Balfour-Lynn Paediatric Respiratory Medicine, Royal Brompton
Hospital
Dr Ed Abrahamson Paediatric A&E & General Paediatrics, Chelsea &
Westminster Hospital
Mr Gordon Cohen Pediatric Cardiothoracic Surgery, Seattle, USA
Dr John Hartley Microbiologist, Great Ormond Street Hospital
Dr Susan King Radiologist, Bristol
Mr Dakshesh Parikh Paediatric Surgeon, Birmingham
Dr David Spencer Paediatric Respiratory Medicine, Newcastle
Dr Anne Thomson Paediatric Respiratory Medicine, Oxford
Dr Donald Urquhart SpR North Thames Paediatric Respiratory
Medicine Training Scheme
New presentation
Clinical suspicion
parapneumonic effusion
Pneumonia diagnosis
Treatment failure at 48 hours
Chest x-ray
Pleural effusion?
YES
Confirm on chest ultrasound
Refer to respiratory paediatrician
Refer to respiratory paediatrician
Suggestion of
malignancy?
Refer to respiratory paediatrician
Suggestion of
malignancy?
Small volume
diagnostic tap
YES
Refer to respiratory paediatrician
Suggestion of
malignancy?
Small volume
diagnostic tap
YES
NO
Suggestion
of
infection?
YES
Intravenous antibiotics
Refer to respiratory paediatrician
Suggestion of
malignancy?
Small volume
diagnostic tap
YES
NO
Suggestion
of
infection?
YES
Intravenous antibiotics
Medical
option
Early surgical
option
Medical
option
Insert chest drain
Pleural fluid microbiology & cell
diff.
Echogenic or loculated on U/S?
Thick fluid draining?
YES
Intrapleural
fibrinolytics
Medical
option
Insert chest drain
Pleural fluid microbiology & cell
diff.
Echogenic or loculated on U/S?
Thick fluid draining?
YES
Intrapleural
fibrinolytics
Early surgical
option
Consider chest CT scan
VATS
or
Early mini-thoracotomy
Medical
option
Early surgical
option
Insert chest drain
Pleural fluid microbiology & cell
diff.
Echogenic or loculated on U/S?
Thick fluid draining?
Consider chest CT scan
VATS
or
Early mini-thoracotomy
YES
Intrapleural
fibrinolytics
Is the patient better?
(fluid drained and sepsis improved)
Is the patient better?
(fluid drained and sepsis improved)
YES
Remove tube
Stop IV antibiotics
Oral antibiotics 1-4 weeks
Discharge & follow-up
Is the patient better?
(fluid drained and sepsis improved)
NO
Consult with paediatric
thoracic surgeon
re. late surgery
Consider chest CT scan
YES
Remove tube
Stop IV antibiotics
Oral antibiotics 1-4 weeks
Discharge & follow-up
SIGN levels of evidence
• I – meta-analyses, RCTs (incl.
systematic reviews) I++, I+, I• II – case-control or cohort studies
(incl. systematic reviews) II++, II+, II• III – case reports, case studies
• IV – expert opinion
SIGN grades of recommendations
• A – evidence from meta-analysis,
systematic review, RCT (I++ or
applicable I+)
• B – evidence from applicable II++ or
extrapolated I++, I+
• C – evidence from applicable II+ or
extrapolated II++
• D – evidence from III or IV
SIGN ratings
107
46
120
50
100
40
80
30
60
40
13
22
23
I
II
III
IV
7
4
10
20
0
20
0
0
A
B
C
D
Levels of evidence
Grades of recommendations
n=165
n=57
Clinical picture
• All children with parapneumonic effusion or
empyema should be admitted to hospital. [D]
• If a child remains pyrexial or unwell 48 hours
after admission for pneumonia, parapneumonic
effusion / empyema must be excluded. [D]
Diagnostic imaging
• Postero-anterior or anteroposterior radiographs should be
taken, there is no role for a
routine lateral radiograph. [D]
• Ultrasound must be used to
confirm the presence of a
pleural fluid collection. [D]
• Chest CT scan should not be
performed routinely. [D]
Diagnostic analysis of pleural fluid
• Aspirated pleural fluid should be sent for
differential cell count. [D]
• Tuberculosis and malignancy must be
excluded in the presence of pleural
lymphocytosis. [C]
• Biochemical analysis of pleural fluid is
unnecessary… [D]
Referral to tertiary centre
• A respiratory paediatrician should
be involved early in the care of all
patients requiring chest tube
drainage for a pleural infection.
[D]
GOSH
• Patients with chest drains should
be managed on specialist wards
by staff trained in chest drain
management. [D]
Conservative management
(antibiotics ± simple drainage)
• Effusions which are enlarging and / or
compromising respiratory function should not
be managed by antibiotics alone. [D]
Repeated thoracocentesis
• If a child has significant pleural infection then a
drain should be inserted at the outset, and repeated
taps are not recommended. [D]
Antibiotics 1
• All cases should be treated with
intravenous antibiotics and must include
cover for S pneumoniae. [D]
• Broader spectrum cover is required for
hospital-acquired infections, as well as
those secondary to surgery, trauma and
aspiration. [D]
Antibiotics 2
•
•
•
•
•
•
Cefuroxime
Co-amoxiclav
Penicillin and flucloxacillin
Amoxicillin and flucloxacillin
Clindamycin
Discharge: oral co-amoxiclav 1- 4 wks
Chest drains 1
• If GA is not being used, IV sedation should only be
given by those trained in the use of conscious
sedation, airway management & resuscitation of
children, using full monitoring equipment. [D]
• Ultrasound should be used to guide thoracocentesis or
drain placement. [C]
Chest drains 2
• Since there is no evidence that large bore chest
drains confer any advantage, small drains
(including pigtail catheters) should be used
whenever possible to minimise patient discomfort.
[C]
• The drain should be clamped for 1 hour once 10
mls/kg are initially removed. [D]
B
Intrapleural fibrinolytics
• Intrapleural fibrinolytics shorten hospital stay and are
recommended for any complicated parapneumonic
effusion (thick fluid with loculations) or empyema
(overt pus). [B]
• Urokinase should be given twice daily for 3 days (6
doses in total) using 40,000 units in 40 mls 0.9% saline
for children aged 1 year or above, and 10,000 units in 10
mls 0.9% saline for children aged under 1 year. [B]
Thomson et al Thorax 2002;57 343-7
Surgery
• Failure of chest tube drainage, antibiotics and
fibrinolytics should prompt early discussion with a
thoracic surgeon. [D]
• Patients should be considered for surgical treatment if
they have persisting sepsis in association with a
persistent pleural collection, despite chest tube
drainage and antibiotics. [D]
• Organised empyema in a symptomatic child requires
formal thoracotomy and decortication. [D]
Other management
• Chest physiotherapy is not beneficial and
should not be performed in children with
empyema. [D]
• Secondary thrombocytosis (platelet count
>500 x109/L) is common but benign; antiplatelet therapy is not necessary. [D]
Follow-up
• Children should be followed up after discharge
until they have recovered completely and their
chest radiograph has returned to near normal. [D]
• Underlying diagnoses – for example,
immunodeficiency, cystic fibrosis – may need to
be considered. [D]
The messages
• The evidence on which to base recommendations
is poor / absent
• Adult data are not transferable
• This is a tertiary condition
• Children with empyema almost always have an
excellent outcome – whatever the management
• Trials are needed…
Pediatric Cardiothoracic Surgery
• Thorax
–
–
–
–
Veggur
Pleura
Lungu
Mediastinum
• Hjarta og æðar í thorax
– Meðfæddir gallar
– Áunnir gallar/ sjúkdómar
Andnauð við fæðingu
What is your differential diagnosis?
• Congenital diaphragmatic hernia
• Pneumothorax
• Congenital cystic adenomatoid malformation
(CCAM)
• Pulmonary sequestration
– Intrapulmonary
– Extrapulmonary
• Congenital Lobar Emphysema
• Bronchogenic Cyst
CDH
NGT
Treatment: CDH
• Delayed surgical approach; not a surgical
emergency
• Conventional vent, Oscillator, “gentle ventilation”,
possible ECMO (10-15%)
– Goal is prevention of barotrauma
• Primary repair; patch sometimes needed
• Overall survival 50-80 %
CDH
•
•
•
•
Bochdalek: posterolateral defect; usually on left
Morgagni: retrosternal (anterior); presents late
Lung hypoplasia affects both sides.
Pulmonary hypertension / persistent fetal
circulation are the greatest challenges.
• Most repairs do not necessitate a postoperative
chest tube.
Differential Diagnosis
Congenital Lobar Emphysema:
- isolated idiopathic hyperinflation of one lobe;
respiratory difficulties often at birth or in infancy;
worsens with time by air trapping
Differential Diagnosis
Pulmonary Sequestration:
- a segment of lung without anatomic
-
bronchial communication,
systemic arterial supply from
thoracic or abdominal aorta
“Extralobar”: often incidental
“Intralobar”: found within normal
lung parenchyma (lower lobes);
prone to infection
Differential Diagnosis
Congenital Cystic
Adenomatoid
Malformation (CCAM)
- solid/cystic lung
malformation
- Can present at prenatal U/S
or resp distress at birth or
with infection in first few
years of life. If large, can
cause fetal hydrops.
Differential Diagnosis
Bronchogenic Cyst
- Cyst found in hilum, mediastinum, or within lung
parenchyma.
- Can compress airway and cause atelectasis,
pneumonia, air trapping.
Pediatric Cardiothoracic Surgery
• Thorax
–
–
–
–
Trauma
Veggur
Pleura
Lungu
Mediastinum
• Hjarta og æðar í thorax
– Meðfæddir gallar
– Áunnir gallar/ sjúkdómar
Pediatric Thorax Trauma
+ see last years lectures Tx Trauma
Mechanism of Injury and Associated
Mortality
AGE
0-5
6-10
>10
Proportion
36%
27%
37%
Mechanism
MV=70.3% Peds=42.9% MV=61.9%
Peds =16.6% MV=41.4% GSW=27.6%
Struck=9.9% Bike=10.5 Bike=8.2%
Stab=2.3% Sport=3.7% Fall=1.5%
Fall=1.5%
Fall=0.9%
Sport=0.8%
Organ Weight During Violent
Impact at Various Velocities
Weight (kg)
40 km/h
72 km/h
105 km/h
HEART
(0.35)
BRAIN
(1.5)
3.5 kg
14 kg
31.5 kg
15 kg
60 kg
135 kg
Whole
Body
(70)
700 kg
2800 kg
6300 kg
Mechanism of Injury by Age
7,000
6,000
5,000
4,000
MVC
Violence
Fall
3,000
2,000
1,000
0
0 2 4 6 8 10 12 14 16 18 20 22 24 26 28 30 32 34 36 38 40 42 44 46 48 50 52 54 56 58 60 62 64 66 68 70 72 74 76 78 80 82 84 86 88
1 3 5 7 9 11 13 15 17 19 21 23 25 27 29 31 33 35 37 39 41 43 45 47 49 51 53 55 57 59 61 63 65 67 69 71 73 75 77 79 81 83 85 87 89
Age (years)
Figure 12A
Number of patients injured by the
most common mechanism of injury
categories at each age from 0 to
89. Violence was defined for the
following mechanisms of injury:
GSW(gunshot wounds), stab
wounds and assault/fight. Total N =
453,806. Mechanism of injury
definitions in Appendix B.
Prevalence of Pediatric Trauma
• Trauma is the leading cause of death in infants and
children
• Trauma is the cause of 50% of deaths in people
between 5 and 34 years of age
• Motor vehicle related accidents account for 50%
of pediatric trauma cases
• $16 billion is spent annually caring for injuries to
children less than 16 years of age
Anatomic Characteristics of the Pediatric Patient
and Significance to Trauma Care
Variable
Significance
•Large volume of blood in head
•Cerebral edema develops rapidly
•Poor muscular support in neck
•Flexion/extension injuries occur
•Decreased alveolar surface area
•Increased metabolic rate
•Injury leads to rapid compromise
•Decreased airway caliber
•Increased airway resistance
•Heart higher in chest,
•Small pericardial sack
•Prone to injury and cardiac
tamponade
•Thin walled, small abdomen
•Organs not well protected
•Bones soft and pliable
•Fractures less common
•Renal function not well developed
•Prone to develop acute renal
failure
•Large body surface area
•Prone to hypothermia
Anatomic and Physiologic
Differences Child/Adult
• Pliable chest wall
– Transmission of force within the thoracic
skeleton to the pulmonary parenchyma
• Mobility of the mediastinal structures
– Vulnerability to tension pneumothorax and
vascular disruptions
Chest Trauma
• 5 - 10 % of all injuries involve the chest
• 2/3 chest injured children have injury in another
organ system
• thoracic injuries account for ¼ of pediatric trauma
related deaths
• Rib fractures are a marker of significant force and
occur with < 50 % of chest trauma
Chest Trauma Cont’d
• PATTERN OF INJURY
• Pulmonary contusion is common +/- direct
intrapulmonary hemorrhage or
pneumothorax, usually without rib fractures
• RARE : diaphragmatic rupture, aortic
transection, major tracheobronchial tears,
flail chest, sternal fractures and cardiac
contusion
Chest Trauma Cont’d
• 2nd leading cause pediatric trauma death
• compliant chest wall ∴ rib fract uncommon
– significant injuries w/o external signs
– if rib fracture present, expect severe injury
• treat conservatively:
– 15% require more than chest tube
• pulmonary contusion most common, aortic
injury rare
Chest Trauma Cont’d
• Traumatic asphyxia
– Sudden compression elastic chest wall against closed
glottis→↑ intrathoracic pressure→obstruction of
SVC/IVC→ capillary extravasation: petechiae face,
neck ,chest, periorbital edema, retinal hemorrhages,
resp distress, hemoptysis, pulmonary/cardiac
contusions, liver injuries, pneumothorax
• Treat: chest tube prn, ventilate, PEEP,
elevate head
Pediatric Cardiothoracic Surgery
• Thorax
–
–
–
–
Veggur
Pleura
Lungu
Mediastinum
• Hjarta og æðar í thorax
– Meðfæddir gallar
– Áunnir gallar/ sjúkdómar
Cardiovascular Surgery
Knee-chest Position
Frumkvöðlar
• 1952 Robert Edward Gross
– Atrial trekt “Open heart surgery” ASD 0/3
• 1952 Floyd John Lewis
– Hypothermia “Open heart surgery” ASD 9/11
• 1953 Dr. John Gibbon
– CPB support ASD 1/5
• 1954 Dr. Walt Lillehei
– Cross circulation VSD
– Tetralogy of Fallot
• 1952 Robert Edward Gross
– Atrial well “Open heart surgery” ASD 0/3
• 1952 Floyd John Lewis
– Hypothermia “Open heart surgery” ASD 9/11
• 1953 Dr. John Gibbon
– CPB support ASD 1/5
• 1954 Dr. Walt Lillehei
– Cross circulation VSD
– Tetralogy of Fallot
Hiti og kuldi
• Hiti er merki um líf og bruna
• Metabolisk vörn í náttúrunni:
– Hibernation: dýr liggja í dvala yfir veturinn.
– Animals seek cool environment when ischemic
• ” kæling matvæla fyrirbyggir skemmdir.
• Reynslan hefur kennt okkur að:
– ”Ice on injuries reduces swelling and tissue damage”
• Physiological and biochemical rationale
– Tissue metabolic rates decrease as body
temperature decreases
• 1952 Robert Edward Gross
– Atrial well “Open heart surgery” ASD 0/3
• 1952 Floyd John Lewis
– Hypothermia “Open heart surgery” ASD 9/11
• 1953 Dr. John Gibbon
– CPB support ASD 1/5
• 1954 Dr. Walt Lillehei
Surface Cooling Limitations
– Cross circulation VSD
Surface cooling used in
COOL AID feasibility trial
– Tetralogy of Fallot
demonstrated limitations of
‹
*
‹
‹
‹
‹
surface cooling
Target temperature of 32°C
Actual temperatures reached
as low as 28°C
Wide range in actual
temperatures and slow
cooling times
Intensive cooling regime
including blankets, alcohol
wipes with paralyzed and
intubated patients
* Stroke 2001; 32: 1847-54
Target
Temperature
Frumkvöðlar
• 1952 Robert Edward Gross
– Atrial well “Open heart surgery” ASD 0/3
• 1952 Floyd John Lewis
– Hypothermia “Open heart surgery” ASD 9/11
• 1953 Dr. John Gibbon
– CPB support ASD 1/5
• 1954 Dr. Walt Lillehei
– Cross circulation VSD
– Tetralogy of Fallot
Frumkvöðlar
• 1952 Robert Edward Gross
– Atrial well “Open heart surgery” ASD 0/3
• 1952 Floyd John Lewis
– Hypothermia “Open heart surgery” ASD 9/11
• 1953 Dr. John Gibbon
– CPB support ASD 1/5
• 1954 Dr. Walt Lillehei
– Cross circulation VSD
– Cross circulation Tetralogy of Fallot
• CPB
–
–
–
–
–
–
–
–
–
–
svæfingalækningar
Blóðflokkar
EKG 1903
Positive pressure ventilation
Rtg.1895 Contrast 1923
Hjartaþræðing
Heparin 1915
Protamin
Oral anticoagulants 1940
Roller pump
• 1955 John W. Kirklin
– teamwork
• “Cardiac team”
–
–
–
–
Cardiac Surgery
Anesthesiology assoc. 1972
Perfusion technology
Cardiac intensive care medicin
• 1955 Dr. DeWall Bubble oxygenator
• Nszih Zuhdi Hemodilution techniques
• 1960 combined pump and hypothermia
– Ca. 1.000.000 / Y / US
•
•
•
•
•
CABG,VALVES, CONG.
NEUROSURG..
HEART/LUNG TRANSPL.,
THORAC.AORTA
ECMO
pH og CO2
Alpha stat
– sbr,. Dvali dýra
• Basiskt
pH stat
cerebral flæði
súrt
– Peter C. Laussen Paediatric Anaesthesia March 2002
1883
Theodore Bilroth
' The Surgeon who should attempt to suture a wound
of the heart would lose the respect of his colleagues.
Cardiac Surgery Reykjavik
2002
Cardiac Surgical output total:
243 cases
Coronary Surgery
182
Valve
38
Congenit
16
Other:ECMO3,MAZE1,Aneurysms7, AICD12 etc.:
26
TMR
CABG+Valve
OPCAB
CABG
Fjöldi aðgerða
2005
2006
2007
CABG
120
108
133
OpCAB
45
22
28
Lokuaðg.
66
54
64
Hjartaviðg.
9
3
7
Ósæðaaðg.
12
12
6
ECMO
0
3
8
Gervilunga (iLA)
2
1
0
Hjálparhjarta
0
1
4
Aðrar HLV
7
1
8
22
27
39
259
200
246
IABP
Fjöldi milli ára
140
120
133
120
108
Fjöldi
100
80
66
64
54
60
45
40
39
22
28
22
20
9
3 7
12 12
6
0 3
8
2 1 0
0 1 4
Gervilunga
(iLA)
Hjálparhjarta
7
1
27
8
0
CABG
OpCAB
Lokuaðg.
Hjartaviðg.
Ósæðaaðg.
ECMO
Meðferð
2005
2006
2007
Aðrar HLV
IABP
Neonatal Surgery
Reykjavík
Boston
Risk Categories
1= ASD, PDA (>30 d) , coarct (>30 d)
2=ASD/VSD, TOF, Glenn, sub AS
3=AVR, Ross procedure, MVR
4=arterial switch, Truncus arteriosus
5=truncus and interrupted arch
6= Norwood, Damus-Kaye-Stansel
Congenital Disease
•
•
•
•
•
Patent Ductus Arteriosus
Aortic Coarctation
Atrial Septal Defect
Ventricular Septal Defect
Valvular Heart Disease
– Ross Procedure
Open Heart Surgery
•
•
•
•
•
Management of chest /thoracotomy tubes
Risk for Infection
Risk for Hypo/Hyperthermia
Risk for Fluid Volume Overload
Complications
–
–
–
–
–
–
–
–
Hemmorhage
Shock
Heart Block
CHF
Post Cardiac Surgery Syndrome
Post Perfusion Syndrome
Infection
Atelectasis
ECC :
Venous (IVC and SVC) Cannulae
Aortic Cannula
Oxygenator
Pump
Heater/Cooler
CardioPulmonary Bypass
Hjartastarfsemin
Samhæfing
PM
Indian Pacing Electrophysiol. J. 2003;3(1):23
Video:
Pacemaker
1:20
Video
Pacemaker
• Usually placed sub-xyphoid or mid to lower
abd
• Teach parents how to take child’s pulse
daily
• Batteries can last up to15 years
Nýgengi hjartagalla
• 1 in every 120 births has CHD
• Mild – resolve by themselves
• Moderate –Non life threatening – but
require treatment
• Severe – multiple operations , lifetime
medication
Pediatric Cardiac Disease
•
•
•
•
Congenital
Cyanotic: 22%
Acyanotic: 68%
Acquired: 10%
–
–
–
–
–
• Algengustu
Congenital
Kawasaki disease
Rheumatic
Tubercular
Collagen
Endocarditis
–
–
–
–
–
–
VSD
ASD
PDA
TOF
PS
AS
25%
6%
6%
5%
5%
5%
Ceylon Med J 2001 Sep; 46 (3): 96-8; Indian J Pediatr. 2001 Aug;68 (8):757-7
Nelson’s Textbook of pediatrics; 17 ed.
Common acyanotic lesions
•
•
•
•
•
•
•
•
•
•
Ventricular septal defects
Atrial septal defects
Atrio-ventricular septal defects
Patent ductus arteriosus
Truncus arteriosus
Pulmonary stenosis
Aortic stenosis
Mitral stenosis/incompetence
Coarctation of aorta
Tricuspid regurgitation
Common Cyanotic Lesions
Decreased flow
1. Tetralogy of Fallot
2. Tricuspid Atresia
3. Severe Pulmonic Stenosis
4. Ebstein’s anamoly
Increased Flow
5. Transposition of the great vessles
6. VSD with pulmonary atresia
Common Lesions “producing”
cyanosis
7. Truncus Arteriosus
8. Hypoplastic left heart
9. Single ventricle
10.TAPVR with infradiaphragmatic
obstruction
Heart Transplant
• Pre and Post-Op same as any heart surgery
• Long-term Consequences
– Atherosclerosis
– Lifetime use of immunosuppressant drugs
– Number one complication is Rejection
• Hyperacute
• Acute
• Chronic
Classification of Congenital Heart
Disease
• Eldri
– Acyanotic
– Cyanotic
• Nýrri
– Increased Pulmonary
Blood Flow
– Obstructed Blood Flow
– Mixed Blood Flow
– Decreased Pulmonary
Blood Flow
Timing and Type of Surgery
• Cardiac catheterization procedures
– Balloon atrial septostomy
– Balloon valvuloplasty
– Balloon angioplasty
• Open versus Closed
• Palliative versus Corrective
– Trend towards early, corrective surgery, even in
preterm or low birth weight infants
Types of Closed Heart Surgery
• Patent Ductus Arteriosus
• Coarctation of the Aorta
• Blalock-Taussig Shunt
Types of Open Heart Surgery
Septal Defects:
• Atrial Spetal Defect
• Ventricular Septal Defect
• Atrioventricular Septal Defect
Defects Causing An Obstruction to Blood Flow:
• Pulmonary Stenosis
• Aortic Stenois
• Tetralogy of Fallot
Defects Involving The Great Arteries
• Transpostion of the Great Arteries (Arterial Switch)
Others:
• Hypoplastic Left Heart Syndrome(Norwood operations)
• Aortic Valve Replacement (Ross Procedure)
Cardiac disease with normal/decreased
vasculature
•
•
•
•
•
•
•
•
Viral myocarditis
Tetralogy of Fallot
Pulmonary atresia
Tricuspid atresia
Endocardial fibroelastosis
Aberrant left coronary artery
Cystic medial necrosis
Diabetic mother
(http://www.emedicine.com/emerg/topic28.htm)
Pediatric Cardiothoracic Surgery
• Thorax
–
–
–
–
Veggur
Pleura
Lungu
Mediastinum
• Hjarta og æðar í thorax
– Meðfæddir gallar
– Áunnir gallar/ sjúkdómar
Congenital Disease
•
•
•
•
•
Patent Ductus Arteriosus
Aortic Coarctation
Atrial Septal Defect
Ventricular Septal Defect
Valvular Heart Disease
– Ross Procedure
Patent Ductus Arteriosus
• Small defect no
symptoms.
• Large defect:
–
–
–
–
–
Wide pulse pressure
Enlarged heart
Thrill in L second IS
Continuous murmur
X-ray: prominent
pulmonary artery with
increased vascular
markings.
Patent Ductus Arteriosus
Acyanotic Heart Defect
– PDA
•
•
•
•
Incidence 5-10% CHD
Hemodynamic
Diagnosis
Management
– Med
– Surgical Mort <1%
Video:
Ductus
2:43
Aortic Coarctation
Acyanotic Heart Defect
Adult Týpa
Aorta
Ductus
Aorta
Neonatal Týpa
Coarctation of Aorta
• COA
• 7 % of defects
• Congenital narrowing of the descending
aorta
• 80% have aortic-valve anomalies (Bicusp)
• Difference in BP in arms and legs (severe
obstruction)
Diagnosis and Treatment
• In 50% the narrowing is not severe enough
to cause symptoms in the first days of life.
• When the PDA closes a higher resistance
develops and heart failure can develop.
• Pulses in the groin and leg will be
diminished
• Echocardiogram will show the defect in the
aorta
Treatment
• Prostaglandin may be given to keep the PDA open
to reduce the pressure changes
• The most common repair is resection of the
narrowed area with re-anastomosis of the two ends
• Surgical complications – kidney damage due to
clamping off of blood flow during surgery
• High blood pressure post surgery – may need to be
on antihypertensives
• Antibiotic prophylactic need due to possible aortic
valve abnormalities.
Coarctation of the Aorta
Acyanotic Heart Defect
•
•
•
•
Incidence 8% CHD
Hemodynamic
Diagnosis
Management
– Med
– Surgical Mort <5%
» Ath mun á nýbura- og fullorðinspresentation
Extended end to end eða
subclavian flap reconstruction
Aortic Coarctation
‹Video 2
4:51
Atrial Septal Defects
Acyanotic Heart Defects
• Types of ASD:
Atrial Septal Defects: secundum
• Most common form of
ASD (fossa ovalis)
• In large defects, a
considerable shunt of
oxygenated blood flows
from the left to the right
atrium.
• Mostly asymptomatic
• The 2nd heart sound is
characteristically widely
split and fixed.
Secundum
Atrial Septal Defects:primum
• Situated in the lower portion of
the atrial septum and overlies
the mitral and tricuspid valves.
In most instances, a cleft in the
anterior leaflet of the mitral
valve is also noted.
• Combination of a left-to-right
shunt across the atrial defect
and mitral insufficiency
• C/F similar to that of an ostium
secundum ASD
Atrial Septal Defect
•
•
•
•
•
ASD
6-10% of defects
Blood in left atrium flows into right atrium
Pulmonary hypertension
Reduced blood volume in systemic
circulation
• If left untreated may lead to pulmonary
hypertension, congestive heart failure or
stroke as an adult.
Diagnosis and Treatment
• Diagnosis: heart murmur may be heard in
the pulmonary valve area because the heart
is forcing an unusually large amount of
blood through a normal sized valve.
• Echocardiogram is the primary method used
to diagnose the defect – it can show the hole
and its size and any enlargement of the right
atrium and ventricle in response to the extra
work they are doing.
Treatment
• Surgical closure of the atrial septal defect
• After closure in childhood the heart size
will return to normal over a period of four
to six months.
• No restrictions to physical activity post
closure
Atrial Septal Defect
• Enlargement of the right
ventricle
• Enlargement of atrium
• Large pulmonary artery
• increased pulmonary
vascularity is.
Atrial Septal Defects
• Secundum ASDs are well tolerated during childhood.
• Antibiotic prophylaxis for isolated secundum ASDs is
not recommended.
• Surgery closure is advised for all symptomatic
patients and also for asymptomatic patients with a
Qp:Qs ratio of at least 2:1.
• Secundum defects are approached surgically but
some secundum defects can be closed by
transcatheter device
• Ostium primum defects are approached surgically
• Sinus venosum defects are approached surgically
Atrial Septal Defects
Acyanotic Heart Defects
– ASD
•
•
•
•
Incidence 5-10% CHD
Hemodynamic
Diagnosis
Management
– Med
– Surgical Mort <1%
Atrial Septal Defect
ASDHjartagallar video\ASD.AVI
Video:
6:44
Ventricular septal Defects
• Small VSD
– Asymptomatic
– A loud, harsh, or
blowing holosystolic
murmur.
• Large VSD
– dyspnea, feeding
difficulties, poor growth,
profuse perspiration,
recurrent pulmonary
infections, and cardiac
failure in early infancy.
80%
Ventricular
Septal
Defect
(VSD)
Small VSDs, the chest radiograph is usually normal
Large VSD: The presence of right ventricular hypertrophy, olegeimic lung fields
(pulmonary hypertension or an associated pulmonic stenosis), gross
cardiomegaly with prominence of both ventricles, the left atrium.
Ventricular Septal defects
• 30–50% of small defects close spontaneously, most
frequently during the 1st 2 yr of life.
• Small muscular VSDs are more likely to close (up to
80%) than membranous VSDs are (up to 35%).
• infants with large defects have repeated episodes of
respiratory infection and heart failure despite optimal
medical management.
• Surgical repair prior to development of an irreversible
increase in pulmonary vasculalr resistance (usually
prior to the patient's second birthday).
Ventricular Septal Defect
•
•
•
•
•
•
VSD
25-30% of defects
Opening in the ventricular septum
Left-to-right shunt
Right ventricular hypertrophy
Deficient systemic blood flow
VSD
• Small holes generally are asymptomatic
• Medium to moderate holes will cause
problems when the pressure in the right side
of the heart decreases and blood will start to
flow to the path of least resistance (from the
left ventricle through the VSD to the right
ventricle and into the lungs)
• This will generally lead to CHF
Diagnosis and Treatment
• Diagnosis – heart murmur – clinical pearl a
louder murmur may indicate a smaller hole
due to the force that is needed for the blood
to get through the hole.
• Electrocardiogram – to see if there is a
strain on the heart
• Chest x-ray – size of heart
• Echocardiogram – shows size of the hole
and size of heart chambers
Treatment VSD
• CHF: diuretics of help get rid of extra fluid
in the lungs
• Digoxin if additional force needed to
squeeze the heart
• FTT or failure to grow may need higher
calorie concentration
• Will need prophylactic antibiotics before
dental procedures if defect is not repaired
Surgical Repair
• Over a period of years the vessels in the
lungs will develop thicker walls – the
pressure in the lungs will increase and
pulmonary vascular disease
• If pressure in the lungs becomes too high
the un-oxygenated blood with cross over to
the left side of the heart and un-oxygenated
blood with enter the circulatory system.
• If the large VSD is repaired these changes
will not occur.
Acyanotic Heart Defects
– VSD
•
•
•
•
Incidence 20-25% CHD
Hemodynamic
Diagnosis
Management
– Med
– Surgical Mort <5%
Pulmonary artery banding
Ventricular Septal Defect
Video:
VSD
2:34
Aortic Valve disease
Acyanotic Heart Defects
– AS
•
•
•
•
Incidence 5% CHD
Hemodynamic
Diagnosis
Management
– Med
– Surgical Mort 1-2%%, infant 15-20%
http://www.hsforum.com/stories/storyReader$1469
• Ross
Video:
Ross
10:22
Tetralogy of Fallot (TOF)
• 5-6% of defects
• Most common cardiac malformation
responsible for cyanosis in a child over 1
year
TOF
• Four Components
– VSD
– Pulmonary stenosis – narrowing of pulmonary
valve
– Overriding of the aorta – aortic valve is
enlarged and appears to arise from both the left
and right ventricles instead of the left ventricle
– Hypertrophy of right ventricle – thickening of
the muscular walls because of the right
ventricle pumping at high pressure
Clinical Manifestations
• Depends on degree of right ventricular
outflow tract obstruction.
• Right-to-left shunt
• Clubbing of digits
• “tet” spells
– flexing knees forward and upward
• Irritability due to low oxygen levels
Diagnosis
• Cyanosis
• Oxygen will have little
effect on the cyanosis
• Rtg
• Loud heart murmur
• Echocardiogram – demonstrates the four
defects characteristic of tetralogy
Treatment
• If oxygen levels are extremely low prostaglandins
may be administered IV to keep the PDA open
• Complete repair is done when the infant is about 6
months of age
• Palliation with BT shunt may be needed
• Correction includes
– Closure of the VSD with dacron patch
– The narrowed pulmonary valve is enlarge
– Hypertrophy of right heart should remodel within a few
months when pressure in right side is reduced
TOF
– TOF
•
•
•
•
Incidence 5-6% CHD
Hemodynamic
Diagnosis
Management
– Med
– Surgical Mort varies + 5-10/2POY
Palliative Procedures for TOF
Current Indications
Neonates with severe pulmonary artery atresia
Aberrant course of the LAD from the RCA
Procedures Performed
•Blalock-Taussig
•Modified Blacock Taussig
•Central
•Potts
•Waterston
Disadvantages of Palliation
•Pulmonary artery distortion
•Ventricular volume loading
•Increased surgical risk from additional thoracotomy
Most centers recommend primary surgical correction as the preferred procedure since
mortality is very low. Recent series have reported down to 0% hospital mortality.
www.ctsnet.org/doc/4954
BT, Modified BT
Cyanotic Heart Defects
– PA
•
•
•
•
•
Incidence <1% CHD
Hemodynamic
Manifestation
Diagnosis
Management
FONTAN
– Med
– Surgical septostomy,Blalock-Taussig,valvotomy:
» Mort: 10-25%, OutflTractReconstr 25%
» Fontan <40%
Cardiac disease with increased vasculature
•
•
•
•
•
•
•
Atrioventricular septal defects
Congestive cardiac failure
Transposition of great arteries with VSD
Total anomalous pulmonary venous drainage
Truncus arteriosus
Single ventricle without pulmonary stenosis
Hypoplastic left heart syndrome
Cyanotic Heart Defects
– TA
•
•
•
•
•
Incidence <1% CHD
Hemodynamic
Manifestation
Diagnosis
Management
– Med
– Surgical,
» Mort 30%,
» Rastelli´s Mort20-60%
Cyanotic Heart Defects
– TGA-Complete Transposition of the GA
•
•
•
•
•
Incidence 5% CHD
Hemodynamic D-TGA
Manifestation
Diagnosis
Management - Cardiac emergency
– Med septostomycatlab/open Mort 10-25% 90%/1y
» Surgical
» Ventricular-level: Rastelli, or
» Arterial-level Jatene´s, or
» atrial Senning, Mustard´s
TGA: Arterial-level Jatene´s
Jatene operation for
transposition of the great
arteries
•
•
•
•
Median sternotomy
By-Pass is initiated
The Heart is arrested
Ligation of ductus arteriosus
• Aorta is transected distal
to the commissures
• The pulmonary artery is transected
• The coronary arteries are removed with a
cuff of aortic wall (button).
• Then the coronary arteries are sutured to the
pulmonary stump creating a new (neo-)
aortic stump
• The aortic artery stump is
then passed posterior to
the pulmonary
bifurcation (PA) and
sutured to the
reconstructed arterial
stump of the left ventricle
outflow tract (the
LeCompte maneuver).
Lastly, the pulmonary artery is
joined to the new pulmonary
artery stump creating normal
circulatory anatomy and
physiology
TGA: Ventricular level Rastelli
TGA: Atrial-level Senning/Mustard
Cyanotic Heart Defects
– Hypoplastic Left Heart Syndrome
•
•
•
•
•
Incidence 1-2% CHD
Hemodynamic
Manifestation
Diagnosis
Management
– Med
» Surgical NorwoodI:mixingII:FontanRA-PA
MorIt 75% II50%
» NWIII,IV
» Transplantation??
Cyanotic Heart Defects
– Total Anomal Pulm Venous Return
•
•
•
•
•
Incidence 1% CHD
Hemodynamic PV-RA
Manifestation
Diagnosis
Management
– Med Balloon septostomy
» Surgical
Mort 10-25%
Pediatric Cardiothoracic Surgery
• Thorax
–
–
–
–
Veggur
Pleura
Lungu
Mediastinum
• Hjarta og æðar í thorax
– Meðfæddir gallar
– Áunnir gallar/ sjúkdómar
Pericardial Effusion
• Presenting complaint
–
–
–
–
–
–
–
Precordial pain
Cough
Dyspnoea
Abdominal pain
Vomiting
Fever
Other organs involvement
• Signs:
–
–
–
–
–
–
–
–
Position: leaning forward.
Puffy face
Friction rub
Absent apical impulse
Muffled heart sounds
Pulsus paradoxus
Distended neck veins
Low QRS complex, T
inversion
Pericardial Effusion
• A relatively large
pericardial effusion
must be present to
cause an enlarged
cardiac shadow with
the usual “water
bottle” configuration
on a chest
roentgenogram
Video: Chilothorax
8:56
Infective Endocarditis
Presenting symptoms and clinical features include:
•
•
•
•
•
•
•
•
•
•
•
Fever
Malaise
Fatigue
Anorexia
Weight loss
Splenomegaly
Cardiac murmur
Petechiae
Roth spots
Janeway lesions
Osler nodes
Some of the more diagnostic symptoms (the latter half of the above list)
are occurring less frequently in patients with subacute IE, making
diagnosis a greater challenge.
Petechiae
© 2007 About, Inc
Roth Spots
Janeway Lesion
Osler Nodes
© 2005 The Regents of the University of California
The figure on the left is of a mitral valve vegetation shown by echocardiogram.
The figure to the right shows one portion (called a leaflet) of the mitral valve.
Cabell, C. H. et al. Circulation 2003;107:e185-e187
Copyright ©2003 American Heart Association
Class I Indications for Surgery
• Acute AR or MR with heart failure.
• Acute AR with tachycardia and early
closure of the MV.
• Fungal endocarditis.
• Annular or aortic abscess.
• Sinus or aortic aneurysm.
• Persistent bacteremia and valve dysfunction
– After 7-10 days of appropriate antibiotics.
Circulation. 98(18):1949-1984, 1998
Other Indications for Surgery
• Class IIa
• Class III
– Recurrent emboli after
appropriate abx.
– Agent with known
poor response to abx
(GNR) with valve
dysfunction.
• Class IIb
– Mobile vegetations
>10 mm.
Circulation. 98(18):1949-1984, 1998
– Early infections of MV
that can likely be
repaired.
– Persistent pyrexia and
leucocytosis with
negative blood
cultures.
Congestive Cardiac Failure
• Enlarged heart
• Plethoric lung fields
specially at bases
Heart Failure
• Biventricular pacing: Adults, but children?
Heart Failure
• ECMO and Counterpulsation IABP, Axial
flow pump Impella, HMII
Heart Failure
ECMO
1:53
Video:
ECMO
Heart Failure
• Bridge to improvement
• Bridge to transplantation
• Destination therapy
Impella
Animation
Impella inn
HMII nám
Heart Failure
• Heart transplantation
Organ Transplantation in the United States
1 Jan 1988 – 31 Jan 2005
Organ
Number
Kidney
Liver
Pancreas
Intestine
Heart
Lung
201,846
69,057
16,674
977
36,764
13,322
Total
338,640
www.unos.org
Types of Lung Recipients
• Pulmonary fibrosis
• Emphysema/Alpha-1
antitrypsin deficiency
• Pulmonary hypertension
– Primary
– Congenital heart disease
• Cystic fibrosis
ADULT LUNG TRANSPLANTATION
Indications By Year (Number)
1250
Cystic 70
Fibrosis
IPF
Emphysema
A1A
Number of Transplants
60
1000
50
40
750
30
500
20
10
250
0
Myopathy
0
PPH
1982
1983
1984
1985
1986
1987
1988
1989
1990
1991
1992
1993
1990 1991 1992 1993 1994 1995 1996 1997 1998 1999 2000 2001 2002 2003
ISHLT
Transplant Year
Many variables account for the dramatic
progress in outcomes of CHD
• Better understanding of anatomy, embryology,
genetics, pathophysiology, and natural history
• Improved diagnosis
• Support technology (e.g., cardiorespiratory
support and monitoring technology in the OR
and CICU, ECMO, mechanical assist devices)
• Pharmacotherapy (e.g., pressors, ACE
inhibitors, β-blockers, NO, Sildenofil, Bosentan)
• Surgical techniques
• Transcatheter therapy
Tal Geva 2/04
•
Organ transplants in Icelandic
patients
(1998
2007)
2008
Heart
– 7 transplants in 6 patients
– 5 are alive
• Heart-lung
– 4 transplants
– 2 are alive
• Lung
– 6 transplants
– 4 are alive
• Kidney
– 187 transplants in 167
patients
– 110 transplants were from
live donors and 77 from
deceased donors
– 111 are alive, 105 with a
functioning transplant
• Liver
– 29 transplants in 26 patients
– 5 children recieved a live
donor transplant
– 18 are alive
Organ transplants from deceased
donors in Icelandic patients, 1998-2007
1998 1999 2000 2001 2002 2003 2004 2005 2006 200
7
Heart
Kidney
Liver
Lung
*Same patient
2
1
1
2
3
2
1
1
1
1
2*
3
2
2
Total
2
3
6
2
21
2
3
2
15
1
1
4
Frekari lesning
Common types of congenital heart
defects
Congenital heart defects are
abnormalities that develop before
birth. They can occur in the heart's
chambers, valves or blood vessels.
A baby may be born with only one
defect or several that tend to occur
in combination. Of the dozens of
heart defects, some are mild and
may need minimal or no medical
treatment even through adulthood,
while others are life-threatening,
either immediately to the newborn
or over time. Here's a look at some
of the more common congenital
heart defects. Compare them to
the NORMAL HUMAN HEART,
SHOWN HERE
Congenital Heart Disease
Type of Defect
Mechanism
Ventricular Septal
Defect (VSD)
There is a hole within the membranous or muscular portions of the intraventricular septum that
produces a left-to-right shunt, more severe with larger defects
Atrial Septal Defect
(ASD)
A hole from a septum secundum or septum primum defect in the interatrial septum produces a modest
left-to-right shunt
Patent Ductus
Arteriosus (PDA)
The ductus arteriosus, which normally closes soon after birth, remains open, and a left-to-right shunt
develops
Tetralogy of Fallot
Pulmonic stenosis results in right ventricular hypertrophy and a right-to-left shunt across a VSD,
which also has an overriding aorta
Transposition of
Great Vessels
The aorta arises from the right ventricle and the pulmonic trunk from the left ventricle. A VSD, or
ASD with PDA, is needed for extrauterine survival. There is right-to-left shunting.
Truncus Arteriosus
There is incomplete separation of the aortic and pulmonary outflows, along with VSD, which allows
mixing of oxygenated and deoxygenated blood and right-to-left shunting
Hypoplastic Left
Heart Syndrome
There are varying degrees of hypoplasia or atresia of the aortic and mitral valves, along with a small
to absent left ventricular chamber
Coarctation of Aorta
Either just proximal (infantile form) or just distal (adult form) to the ductus is a narrowing of the
aortic lumen, leading to outflow obstruction
Total Anomalous
Pulmonary Venous
Return (TAPVR)
The pulmonary veins do not directly connect to the left atrium, but drain into left innominate vein,
coronary sinus, or some other site, leading to possible mixing of blood and right-sided overload
Ventricular septal defect
Sometimes called a hole in the heart, this
defect — the most common congenital
heart defect — occurs when the septum,
the muscular wall separating the right and
left ventricles, fails to fully form. The hole
allows oxygen-rich blood to leak from the
left ventricle into the right ventricle,
instead of moving into the aorta and on to
the body. Too much blood may flood the
lungs.
This defect can lead to heart failure,
excessive blood pressure in the lungs
(pulmonary hypertension), infections of
the heart (endocarditis), irregular heart
beats (arrhythmias) and delayed growth.
Small holes may heal on their own or
cause no symptoms. Larger holes may
require surgical repair by stiching
together or covering with a patch.
Atrial septal defect
Similar to a ventricular septal defect,
this is a hole that occurs when the
septum separating the right and left
atria doesn't close properly. This allows
blood from the left atrium to flow into
the right atrium, instead of into the left
ventricle and on to the aorta and the
rest of the body.
The defect can cause several
complications, including arrhythmias,
heart failure, stroke and, in rare cases,
pulmonary hypertension. Minor cases
may cause no symptoms and may not
require treatment. Larger defects may
require surgical closure or cardiac
catheterization.
Patent ductus arteriosus
Before birth, a temporary blood vessel
called the ductus arteriosus connects the
pulmonary artery and the aorta. This
allows blood to bypass the lungs
because oxygen is delivered to the fetus
through the placenta and umbilical cord.
The temporary vessel normally closes
within a few hours or days of birth since
the lungs take over. If it remains open
(patent), some blood that should
circulate through the body is misdirected
to the lungs.
This defect can cause heart failure or
endocarditisse heart failure or
endocarditis. In infants, it can be closed
with medications. In older children and
adults, plugs, coils or surgery can be
used to close the vessel.
Pulmonary stenosis
In this condition, the flow of blood from
the right ventricle to the pulmonary artery
is obstructed by narrowing at the
pulmonary valve. When there's an
obstruction (stenosis), the right ventricle
must pump harder to get blood into the
pulmonary artery. The defect may occur
along with other defects, such as
thickening of the muscle of the right
ventricle immediately below the valve.
In many cases, pulmonary stenosis is
mild and doesn't require treatment. But
because it can cause heart failure,
arrhythmias or enlargement of the right
heart chambers, surgery may be
necessary to repair the stenosis or
replace the valve. Special balloons to
widen the valve (balloon valvuloplasty)
may also be used.
Tetralogy of Fallot
This defect is a combination of four
(tetralogy) congenital abnormalities.
The four defects typically are ventricular
septal defect (VSD), pulmonary stenosis,
a misplaced aorta and a thickened right
ventricular wall (right ventricular
hypertrophy). They usually result in an
insufficient amount of oxygenated blood
reaching the body.
Complications of tetralogy of Fallot (fuhLOE) include cyanosis — sometimes
called "blue baby syndrome," since the
lips, fingers and toes may have a bluish
tinge from lack of oxygen — as well as
poor eating, inability to tolerate exercise,
arrhythmias, delayed growth and
development, and stroke. Surgical repair
of the defects is required early in life.
Transposition of the great vessels
(arteries)
With this defect, the positions of
the aorta and the pulmonary artery
(the great arteries) are reversed
(transposed). The aorta arises from
the right ventricle instead of the left
and the pulmonary artery arises from
the left ventricle instead of the right.
This creates a circulatory pattern that
prevents nourishing oxygenated
blood from reaching the body.
This condition would quickly be fatal
to a newborn except it's generally
accompanied by another defect —
commonly a septal defect or patent
ductus arteriosus — that does allow
oxygen-rich blood to get to the body.
Surgical repair is usually necessary
shortly after birth.
Truncus arteriosus
This is a defect in which the normally
distinct pulmonary artery and aorta
merge into one single great vessel
(truncus) arising from the right and left
ventricles. In addition, there's usually a
large ventricular septal defect, essentially
turning the right and left ventricles into a
single chamber. This allows oxygenated
and unoxygenated blood to mix. Too
much blood may flow to the lungs,
flooding them and making it difficult to
breathe. It can also result in lifethreatening pulmonary hypertension.
Surgery is needed to close the septal
defect with a patch and to separate the
pulmonary arteries from the trunk. A
conduit is placed to connect the right
ventricle to the pulmonary artery.
Because the conduit doesn't grow with
the child, repeat surgery may be
necessary over time
Hypoplastic left heart syndrome
In this condition, the left side of the heart is
underdeveloped (hypoplastic), including
the aorta, aortic valve, left ventricle and
mitral valve. As a result, the body doesn't
receive enough oxygenated blood. In the
first few days after a baby is born, the
ductus arteriosus remains open (patent),
allowing normal circulation, so the baby
may seem fine initially. But when the
ductus arteriosus naturally closes, signs
and symptoms begin, including a bluish
cast to the skin from lack of oxygen,
difficulty breathing and poor feeding. This
condition may be accompanied by an atrial
septal defect.
Treatment options for this life-threatening
condition are a heart transplant or a
multistage surgical procedure done during
the first few years of life.
Coarctation of the aorta
This is a narrowing
(coarctation), or constriction,
in a portion of the aorta.
Coarctation forces the heart to
pump harder to get blood
through the aorta and on to the
rest of the body.
This defect can cause several
life-threatening complications,
including severe hypertension,
aortic aneurysm, dissection or
rupture, endocarditis, brain
hemorrhage, stroke, heart
failure and premature coronary
artery disease. Repair is
typically recommended before
age 10, either by surgically
removing the affected portion or
widening it through balloon
angioplasty and placement of a
stent.
Aortic stenosis
This is a defect that narrows
or obstructs the aortic valve
opening, making it difficult for
the heart to pump blood into
the aorta. Mild cases may not
have symptoms initially, but they
can worsen over time.
The defect can cause heart
enlargement, left-sided heart
failure, arrhythmias, endocarditis
and fainting. Treatment includes
surgical repair or replacement of
the valve or, in young children,
widening through balloon
valvuloplasty.
If there's an extra conduction
pathway, the electrical signal
may arrive at the ventricles too
soon. This condition is called
Wolff-Parkinson-White syndrome
(WPW). It's in a category of
electrical abnormalities called
"pre-excitation syndromes."
Ebstein's anomaly
This is a defect of the tricuspid valve, which
controls blood flow between the heart's right
atrium and right ventricle. The valve is
positioned lower than normal into the
ventricle instead of remaining between the
atrium and the ventricle. Consequently, the
ventricle is too small and the atrium too
large, and neither functions properly. The
valve is also malformed, often allowing
blood to leak from the ventricle into the
atrium. This defect often occurs along with
other heart defects, including patent
foramen ovale, atrial septal defect or WolffParkinson-White syndrome.
Severe cases are life-threatening. Milder
cases may have no signs or symptoms until
adulthood. Treatment is with medications or
with surgery to repair or replace the
tricuspid valve, as well as treatment of
associated conditions.
Atrioventricular canal defect
This is a combination of defects,
including a large hole in the center of
the heart and a single common valve
instead of the separate tricuspid and
mitral valves. Also called atrioventricular
septal defect, this defect is classified by
whether it's only partial, involving only
the upper chambers of the heart,
or complete, in which blood can travel
freely among all four chambers of the
heart. Both forms allow extra blood
to circulate to the lungs, causing the
heart to enlarge.
The condition is often associated with
Down syndrome. Infants may also have
trouble breathing and not grow well.
Surgery is often done in infancy to close
the hole and reconstruct the valves.
References
• Text
– Rogers: Textbook of Pediatric Intensive Care
– Critical Cardiac Disease of Infants and Children
• On-line
– Picubook.net
– Pedi-heart web-site
– http://www.cincinnatichildrens.org/health/heart
-encyclopedia/default.htm
Complications
ComplicationsofofParenteral
ParenteralNutrition
Nutrition
--Technical
Technical
•
•
Placement complications
– Pneumothorax
– Arterial lacerations
– Hemothorax
– Mediastinal hematoma
– Nerve injury
Late complications
– Erosion of catheter
– Subclavian thrombosis
– Septic thrombosis
–
–
–
–
–
Sympathetic effusion
Thoracic duct injury
Air embolism
Hydrothorax
Catheter embolism
POTraC
POTraC 2000
2000
Complications
ComplicationsofofParenteral
ParenteralNutrition
Nutrition
--Metabolic
MetabolicComplications
Complications
•
•
•
•
Plasma electrolyte abnormalities
Trace mineral deficiency
– zinc, copper, chromium, selenium
Essential fatty acid deficiency
Disorders of glucose metabolism
– Hypoglycemia
– Hyperglycemia
– Diabetic patient; hyperosmolar nonketotic coma
– Liver function derangements
POTraC
POTraC 2000
2000
Complications
ComplicationsofofParenteral
ParenteralNutrition
Nutrition
––Septic
SepticComplications
Complications
•
•
Catheter Infection
1. Absence of proocol
2. Degree of colonization of the pericatheter skin; > 103
3. G(+) organism from remote site seeding the fibrin
sleeve along catheter; vs G(-) organism
4. Candida from the gut
Management of patient with suspected catheter sepsis
POTraC
POTraC 2000
2000
Prevention
PreventionofofCatheter
CatheterComplications
Complications
•
Catheter Placement