This document discusses the management of cyanotic patients. It covers several topics: 1. Causes of cyanosis including cardiac and non-cardiac causes such as lung diseases and neurological issues. 2. Evaluation of cyanotic newborns including detecting cyanosis and checking oxygen saturation levels. 3. Complications of cyanosis such as cyanotic spells, neurological complications, erythrocytosis, and anemia. Management of these complications is also discussed.

1. MANAGEMENT OF CYANOTIC
PATIENT
Dr. I.Tammi Raju

2. Management of Cyanotic Patients
• Causes of cyanosis-non cardiac
• Medical
– cyanotic spell
– Hematologic derangements
– Hyperviscosity symptoms
– Bleeding diatheses
– Neurological complications
– Renal complications
– Hyperuricemia
• Surgical
– Palliative
– definitive

3. Non-Cardiac Causes of Newborn Cyanosis
• Primary lung disease
• RDS, pneumonia, hypoplasia, CCAM, lymphangectasia, lobar
• Emphysema
• Mechanical interference with lung function
• Congenital diaphragmatic hernia, pneumothorax, pneumomediastinum,
chylothorax, choanal atresia, vascular ring, tracheal esophageal atresia,
anomalies of the airway/larynx, Pierre-Robin/ micrognathia
• Pulmonary hypertension
• Meconium aspiration, pneumothorax, RDS, sepsis, pneumonia

4. • CENTRAL NERVOUS SYSTEM
Seizure, meningitis, encephalitis, severe IVH, subdural or
subarachnoid hemorrhage,
• Severe hypermagnesemia
• Shock
• Sepsis, hypovolemia, adrenal crisis
• Severe hypoglycemia (IDM, SGA babies)
• Congenital neuromuscular condition (Werdnig-Hoffman)
• - METHEMOGLOBINEMIA

5. Evaluation of the Cyanotic Neonate
• Detecting cyanosis:
– ambient lighting
– skin color
– hemoglobin
if Hg is 20 gm/dl; 4 gm desaturated-visible cyanosis
if Hg is 10 gm/dl; 2 gm desaturated-not cyanotic
• Check Saturation
• HyperoxiaTest to Determine Intrapulmonary vs. Intracardiac Shunt

6. HyperoxiaTest
1. On room air (if tolerated)
-measure pO2 by blood gas
2. On 100% FIO2 - blow-by, mask, ETT
-repeat measurement of pO2
DANGERS: Pulmonary overcirculation
Closing the PDA

7. HyperoxiaTest - Interpretation
• pO2 < 100; cyanotic CHD likely
• pO2 100-150; cyanotic CHD possible
• pO2 > 150; cyanotic CHD unlikely

8. CYANOTIC SPELL
 Also called as Hyperpnoeic spell, Hypoxic spell,Anoxic or blue spell
orTet spell.
 Occurs in cyanotic congenital heart diseases with reduced Pulm.
Blood flow.
 A pediatric emergency- a typical episode can lead to death.
 Peak incidence between the age group of 2-6 months.
 Episodes beyond the age of 2 years are rare.
 About 40% of pts.With cyanotic congenital heart disease & decreased
blood flow, develop this spell.

9. CyanotiC spell (Contd.)
 A typical episode begins with a progressive
increase in rate & depth of respiration, resulting
in paroxysmal hyperpnoea,deepening cyanosis,
limpness & syncope, convulsions, CVA & even
death.
 The spells are usually self-limited and last for about <15-30 mins. duration.

10. CYANOTIC SPELLCYANOTIC SPELL (CONTD.)
 During the spell, the Ejection Systolic Murmur
(ESM) which is heard along the left sternal
border due to the Infundibular Stenosis, may
disappear.
 The ECG may show an increased amplitude of
P wave along with ST-T segment depression.

11. • CYANOTIC SPELLS are classically described in ;-
--Tetrology of Fallot
-- DORVWithVSD with PS
--TricuspidAtresia
-- SingleVentricle with Pulm. Stenosis
--Transposition of great arteries.
-- Eisenmenger’s Syndrome

13. Mechanisms of Cyanotic Spell
1. Increased vulnerability of respiratory Centre ;-
- When a child awakes from a deep & prolonged sleep a stress occurs due to
crying feeding or bowel movement.
- Any increase in activity orValsalva like maneuver leads to increased H/R,
increased cardiac o/p and increased venous return, while there is a fixed
obstruction to RVOT.
- Increased cardiac o/p results in a decrease in SVR by about 20% while BP
remains constant (BP=CO x SVR).
-The increased venous return passes from RV to LV in view of reduced SVR.

14. Vulnerability of Resp. centre (contd.)
- GUNTHER et al
As R to L shunt increases, the already low pulm. Blood flow further
declines and the cyanosis deepens.
- Desaturated blood entering the systemic circulation causes the
overreaction of resp. centre to chemical stimuli resulting in
hyperpnoea.
- Hyperpnoea further increases the CO and perpetuates the vicious
cycle.
- Also, theVena Cavae have a pumping effect on the blood flow with
respiration.
-This effect gets exaggerated in c/o reduced pulm. Blood flow and
RVOT obstruction.

15. 2. Infundibular Spasm - Activities which increases
the catecholamine secretions (feeding, crying, bowel
movement, effort, breath holding, infection, cardiac
catheterization), precipitate the Cyanotic Spell.
-The elevated NE levels may provoke RV Infundibular
narrowing, thus compromising the pulm. blood flow
and precipitating the spasm.

16. 3.SVT- paroxysmal SupraventricularTachy. or rapid atrial pacing
results in decreased RV volume and increase in R to L shunt a fall→
in systemic arterial O2saturation and an increase in RV outflow
obstruction precipitation of Cyanotic Spell.→
-This is not a widely accepted mechanism.
4. MET.ACIDOSIS – occurs in the setting of extremely limited pulm.
Blood flow.
- It is secondary to anaerobic metabolism.
- Since the arterial pO2 is below level, there will be a consistent
Lactate accumulation.
- However this mechanism can-not explain the paroxysmal nature of
the spell.

18. Mechanisms responsible for the rare occurrence of the spell after
the age of 2 years could be ;-
 The child learns to squat by habit as soon as he feels breathless.
 The respiratory centre matures by this age.
 Development of the collaterals.
 Fibrosis of the Infundibulum prevents it developing a spasm.

19. • Incresed SVR(kinking of major arterial circulation)- in
presence of fixed pulmonary outflow resistance
• Decreased Rt to Lt flow & more of RV blood into PA-
• increased oxygenated blood to LV-
• decreased CO2 –decreased stimulation of respiratory
center-
• decreased hyperventilation.
SQUATTING

20. • Decreased SV return
• decreased desaturated blood from lower limbs to heart-
• increased oxygenated RV blood will be shunted to LV
• This shunted blood have high PH & O2,low CO2.

21. ManaGeMent oF CyanotiC spell
A. Other possible mechanisms ;-
 Weight/volume of blood below the heart distends the arterial bed in
upright posture.
 Squatting removes this distending force and narrows the arterial bed.
 A small and immediate rise in arterial resistance and BP develops with
accompanying bradycardia.
 Bradycardia in turn breaks the vicious cycle initiated by tachycardia.

22. o Brotmacher(1957) proposed that squatting acutely angulated the lower limb
blood vessels.
o Blood flow to the lower limbs is decreased whereas the increased cardiac
output is confined to the upper part of the body.
o Result is the lessened Arteriovenous O2 difference and the hypoxia to vital
centers is reduced thereby providing relief to the child.

23. Some more postures providing the squatting equivalent relief,
as proposed byTaussig(1947);-
 Sitting down with legs drawn underneath.
 Sitting with legs drawn underneath
 Legs crossed while standing.
 Holding the child with legs flexed up to the abdomen.
 Lying down.

24. B. Morphine Sulphate(MS);-
- 0.2 mg/kg can be given I/M, S/C and I/V.
- Morphine suppresses the respiratory center and abolishes the hyperpnoea.
- Morphine has relaxing effect on Sub-Pulmonary Infundibulum– via CNS or
through peripheralVagotonic effect.
- Morphine sedates the patient thereby reducing the release of NE (Nor-
Epinephrine).

25. C. Oxygen has a little effect on arterial saturation as the shunt is a central
R to L shunt.
D. Propanolol ;- 0.01–0.25 mg/kg (average - 0.05mg /kg).
-- Half the dose is given as bolus ( slow push) and the remaining dose can
be given as infusion ( over 5 – 10 mins.)
-- Propanolol reduces the heart rate and thus may reverse the spell.
-- Isopreterenol in case of Beta-blocker overdose.

26. E. Soda – Bicarb (NaHCo3) – 1 mEq/kg I/V.
-The same dose can be repeated in 10 – 15 mins.
-- reduces the respiratory center stimulating effect of acidosis.
If the hypoxic spell does not respond well to the already mentioned measures of
treatment then;-
 Vasoconstrictors like Phenalepherine 0.02mg/kg I/V.

27.  Ketamine 1 – 3 mg/kg (average 2 mg/kg) I/V over 60 seconds.
-- It increases the SVR and sedates the child.
 GeneralAnesthesia (rarely required)- drugs like
Cyclopropane, Halothane produce rapid induction which
results in reduction of sympathetic activity and consequently NE secretion.
 Disopyramide (2 mg/kg ) has an advantage of not having any hypertensive
response.
-- It has no systemic vasodilatory response.
 Very rarely, when pt. is refractory to all these measures 0f treatment, then
urgent Systemic to PulmonaryArtery Shunt surgery.

28. PREVENTION OF SPELL ;-
o Propanolol (1–4mg/kg), orally in divided doses is highly effective.
-- It helps defer the surgery till a time when the child is older and the risk
of surgery is low.
Complications ;- repeated spells may lead to brain damage and mental
retardation.
-- Prolonged hypoxic spells may lead to CerebralVenous SinusThrombosis and
Smaller OccultThrombosis.
-- Repeated spells also hamper the growth

29. • 2.Neurological complications —
• Patients with cyanotic heart disease are at risk for paradoxical
cerebral emboli which may lead to stroke or brain abscess .
• The impact of microspherocytosis on the risk of cerebrovascular
events is uncertain, but a conservative approach toward phlebotomy is
generally recommended.
• Atrial arrhythmias and transvenous pacing leads may increase the risk
of embolic events.

30. • Nonischemic neurologic complications :
• INFECTIOUS DISORDERS
• Brain abscess — An association between brain abscess and congenital
heart disease is well established .
• the incidence has been estimated at 2 percent .
– Protracted headache
– Focal neurologic signs
– Persistent fever
– Seizures caused by an acute abscess may persist long after healing
or may recur years later because of focal scarring.
– The diagnosis of a fresh brain abscess can be made by
computerized tomography, which identifies the abscess itself and
the distinctive ring enhancement.

31. • The etiologies include
– septic cerebral embolus,
– contiguous extension of infection from otitis media, mastoiditis, or sinusitis, and
– facial or dental infection.
• Less clear is the pathogenesis of what has been called a "hematogenous"
brain abscess .
• Two preconditions, often associated with cyanotic congenital heart disease
appear to be necessary.
– Bacteremia involving the cerebral circulation
– A focal zone of cerebral vulnerability (ie, injury)
– Fischbein CA, RosenthalA, Fischer EG, et al. Risk factors of brain abscess in
patients with congenital heart disease.Am J Cardiol 1974; 34:97.

32. • A right-to-left shunt permits blood-borne bacteria, normally filtered by the
pulmonary circulation, to enter the systemic and therefore the cerebral circulations.
• An area vulnerable to bacteriologic infection can result from a silent sterile
paradoxical cerebral embolus or from a zone of encephalomalacia .
• Treatment of a brain abscess almost always requires aspiration using a CT-directed
stereotactic technique in conjunction with antibiotic therapy.
• Resection of the abscess is rarely required
– Shahler RM, Deuchar DC. Hematogenous brain abscess in cyanotic congenital heart
disease. Report of three cases, with complete transposition of the great vessels.Am J
Med 1972; 52:349.

33. • Septic cerebral aneurysm —
• A septic or bacterial cerebral aneurysm, often incorrectly referred to as a mycotic
aneurysm, usually results from a septic embolus originating at a site of infective
endocarditis .
• Thus, a patient with infective endocarditis who develops focal neurologic signs or
meningitis should undergo a cerebral CT scan followed by cerebral angiography .
• Urgent neurosurgical intervention is obligatory to prevent rupture which has a high
mortality .
– Yamada M, MiyasakaY,Takagi H,Yada K. Cerebral bacterial aneurysm and indications for
cerebral angiography in infective endocarditis. Neurol Med Chir (Tokyo) 1994; 34:697.

34. • 3.Erythrocytosis and anemia —
• Patients with cyanosis develop secondary erythrocytosis.
• This is a physiologic response, since the cyanotic blood simulates the
bone marrow to produce more red blood cells in an attempt to
improve tissue oxygenation.
• Stable erythrocytosis and a new equilibrium are usually established at
an appropriate level.

35. • In some patients, however, hematopoiesis increases dramatically and
the hemoglobin rises to over 20 g/dL with an hematocrit of more
than 65 percent.
• Symptoms of hyperviscosity may develop, such as headache, loss of
concentration, muscle weakness, and fatigue.
• Volume depletion can cause identical symptoms and must be
excluded.

36. • The treatment for symptomatic hyperviscosity is the lowering of red
blood cell mass via phlebotomy, which should be carried out slowly
with simultaneous infusion of isovolumic fluid.
• Target HCT-45-50%
• Amount of blood removed in phlebotomy-
Pt HCT-Desired HCT * body wt*100/Pt HCT

37. • Complications of phlebotomies
• Frequent phlebotomies and iron deficiency anemia should be avoided, since
rebound erythropoiesis can occur, with production of iron deficient red blood cells
(microspherocytes).
• Microspherocytes have reduced oxygen carrying capacity as well as increased
rigidity and fragility.
• As a result, there is an increase in whole blood viscosity (with the associated
symptoms noted above) as well as possible symptoms of iron deficiency anemia
(weakness, headache, irritability, and varying degrees of fatigue and exercise
intolerance).

38. • There is debate about a potential increase in risk of cerebrovascular events in
patients with rebound erythropoiesis and microspherocytosis.
• In one report, 162 patients with cyanotic congenital heart disease were
retrospectively evaluated for any well-documented cerebrovascular events that
occurred at ≥18 years of age.
• Twenty-two patients (13.6 percent) had 29 cerebrovascular events (a rate of about
1 percent per year).
• Patients who had a cerebrovascular event had a significantly increased tendency to
develop hypertension, atrial fibrillation, microcytosis (mean corpuscular volume
<82), and a history of phlebotomy.
• Even when patients with hypertension or atrial fibrillation were excluded, there was
an increased risk of cerebrovascular events associated with microcytosis.
 Ammash N,Warnes CA. Cerebrovascular events in adult patients with cyanotic
congenital heart disease. JAm Coll Cardiol 1996; 28:768.

39. • In another report, 112 patients with cyanotic congenital heart disease followed for
almost seven years were divided into two groups:
• those with "compensated" erythrocytosis (stable hematocrit of 46 to 73 percent,
iron replete, and absent or mild viscosity symptoms),
• those with "decompensated" erythrocytosis (unstable rising hematocrit of 62 to 75
percent, iron deficiency, and marked to severe hyperviscosity symptoms).
• No patient in either group suffered a stroke
 Perloff JK, MarelliAJ, Miner PD. Risk of stroke in adults with cyanotic congenital heart
disease. Circulation 1993; 87:1954.

40. • Guidelines and recommendations —
• Rather than solely using a target hematocrit, phlebotomy should be performed only
in patients with intrusive symptoms of hyperviscosity, and then only with caution in
the setting of iron deficiency. Some experts also recommend preoperative
phlebotomy to improve hemostasis.
• The ACC/AHA guidelines recommend therapeutic phlebotomy for hemoglobin
greater than 20 g/dL and hematocrit >65 percent, associated with headache,
increasing fatigue, or other symptoms of hyperviscosity in the absence of
dehydration or anemia.
• Repeated routine phlebotomies are not recommended .

42. • 4.Symptomatic anemia
• In cyanotic patients - due to iron deficiency resulting from rebound erythrocytosis
or bleeding.
• The ACC/AHA guidelines recommend avoidance of iron deficiency in patients
with cyanotic heart disease and/or Eisenmenger syndrome.
• target HB-15-17gm/dl
• The treatment of iron deficiency is iron therapy which should be administered with
care since iron supplementation may lead to rapid increases in red cell mass.

43. • Although there are no specific data regarding timing, recommended
iron supplementation be instituted when the mean corpuscular
volume is less than 82, since microspherocytes may increase the
cerebrovascular risk .
• Iron should be discontinued when the hematocrit begins to rise,
usually within 7 to 10 days.
Body wt * 2.3*(15-Pt Hb)+500

44. • 4.Hemostasis —
• Cyanotic patients have an increased risk of hemorrhage due to a
number of hemostatic abnormalities documented in up to 20 percent
of patients;
– elevations in the prothrombin and partial thromboplastin times,
– reduced coagulation factors,
– thrombocytopenia, and
– abnormal platelet function.
– However, pulmonary bleeding and menorrhagia are common.

45. • Pulmonary bleeding —(adult patients)
• Hemoptysis is external bleeding and does not reflect the extent of intrapulmonary
hemorrhage, which is a serious and life-threatening problem for Eisenmenger and
other cyanotic patients and can reflect rupture of the pulmonary trunk or a
bronchial artery .
• Bronchoscopy is associated with significant risk and rarely discloses the cause of
hemoptysis; as a result, this procedure should be avoided .
• CT-PA should be performed to determine the presence and extent of
intrapulmonary hemorrhage.
• ACC/AHA guidelines note that anticoagulation is contraindicated in patients with
active or chronic hemoptysis.

46. • Menorrhagia —
• Menorrhagia is a common problem in women with cyanotic heart
disease and, if severe, can lead to iron deficiency anemia.
• Suppression of menorrhagia with hormonal stimulation is often
helpful; however, hysterectomy is occasionally required.

47. • Anticoagulation — NOT recommended routinely.
Exceptions to this rule :
– Atrial fibrillation
– Documented pulmonary thrombosis or embolism
– Transient ischemic attack or stroke without evidence of
microcytosis or other identifiable and treatable cause
– Deep venous thrombosis
– Placement of an indwelling device (such as a permanent
pacemaker) in the right side of the heart.

48. • 5.Renal dysfunction —
• Renal dysfunction in cyanotic patients is primarily manifested as
decreased urate clearance, leading to hyperuricemia , and, in
occasional patients, proteinuria that is associated with a
glomerulopathy .
• The BUN and serum creatinine concentration are typically normal
but susceptibility to radiopaque contrast media and dehydration
remains a concern.

49. • The 2008ACC/AHA guidelines recommend that uric acid and
creatinine levels be assessed at least yearly in Eisenmenger syndrome
patients.
• Drugs that can impair renal function, such as ACE inhibitors,
diuretics, and nonsteroidal antiinflammatory drugs should be avoided
or used with caution.
• If administration of radiographic contrast agent is planned, the
glomerular filtration rate should be assessed, intravenous fluids
administered as appropriate and other precautions considered

50. • 6.Hyperuricemia —
• Among patients with cyanotic congenital heart diseases, the serum
uric acid rises in proportion to the degree of hypoxemia and
hemodynamic deterioration .
• Hyperuricemia can lead to gout but rarely to renal failure, since the
hyperuricemia is primarily due to reduced excretion rather than
increased production.
• Therapy is not necessary in asymptomatic patients.
• Among patients who develop gout, allopurinol or colchicine is
preferred; nonsteroidal antiinflammatory drugs should be avoided
because they interfere with platelet function and hemostasis and may
impair renal function.

51. • 7.Pigment gallstones —
• Increased red blood cell turnover and subsequent production of
unconjugated bilirubin can result in pigment (calcium bilirubinate)
gallstones.
• The major risk of cholelithiasis is the development of acute
cholecystitis with the need for emergency surgery.
• Surgery is not recommended until patients become symptomatic .

52. • 8.Orthopedic problems —
• Orthopedic problems, such as scoliosis, are present in approximately
25 to 30 percent of patients with congenital heart disease.
• The etiology of scoliosis is unknown but can be disabling, can
contribute to cyanosis and functional incapacity due to pulmonary
restriction, and may require consideration of high-risk surgical
intervention.

53. • 9.Other considerations —
• During long-distance flights, cyanotic patients should drink
nonalcoholic and noncaffeinated fluids to avoid dehydration and the
diuretic effects of alcohol and caffeine. Supplemental oxygenation
may be considered.
• Competitive sports should be avoided in cyanotic patients.

54. • 10.Endocarditis prophylaxis —
• AHA guidelines on the prevention of infective endocarditis
recommend antimicrobial prophylaxis for all patients with unrepaired
cyanotic congenital heart disease

55. • All dental procedures that involve manipulation of either gingival
tissue or the periapical region of teeth or perforation of the oral
mucosa.
• Procedures of the respiratory tract that involve incision or biopsy of
the respiratory mucosa.
• Procedures in patients with ongoing GI or GU tract infection.
• Procedures on infected skin, skin structure, or musculoskeletal tissue.
• Surgery to place prosthetic heart valves or prosthetic intravascular or
intracardiac materials.

56. • Prostaglandins PGE1(0.05 to 0.1mcg/kg/min.)/PGE2
• To maintain ductal patency
– HLHS
– PA intact IVS
– D-TGA

57. Prostaglandin E1
• Apnea
• Vasodilation/Hypotension
• Fever
• Seizures (rare)
• May “unmask” CHD with obstruction to PV return
– d-TGA-intact atrial septum
– TAPVR
– Mitral atresia with restrictive atrial septum

58. Side Effects of PGE-1 By BirthWeight
<2 >2
KG KG
_____________________________________
CV 37% 17%
CNS 16 16
Respiratory 42 10
Metabolic 5 2
Infectious 11 2
GI 11 3
Hematologic 5 2
Renal 0 2

59. • Apnea – caffeine, intubate
• Hypotension – volume, pressors
• Seizures – anti-seizure meds
Dealing with Side Effects of PGE-1

60. SURGERY
• Palliative-
• Curative-

61. CLASSIC BLALOCK-TAUSSIG SHUNT MODIFIED BLALOCK-TAUSSIG SHUNT
SYSTEMIC TO PULMONARY ARTERY SHUNTING
The classic Blalock-Taussig shunt consists of an end-to-side anastomosis of the subclavian
and pulmonary artery.
B, The modified Blalock-Taussig shunt consists of an interposition tube graft that
connects the subclavian artery to the ipsilateral pulmonary artery.

62. • Classic BT shunt 1945 –
artificial ductus arteriosus.
• involved ligation and division
of the left subclavian artery,
with anastomosis of the
proximal subclavian artery to
the PA. opposite to side of
arch-
• more mobility and less acute
angle so kinking is less likely.
• This resulted in a reliable
source of pulmonary blood
flow that usually increased over
time as the child grew .

63. • Disadvantages of classic BT
– Phrenic nerve damage
– Recurrent ;aryngeal nerve damage-vocal cord paralyisis
– Cervical sympathetic chain damage-horner
– Chylo thorax/haemothorax
– potential distortion and kinking of the PA in patients with a small
subclavian artery and
– possible abnormal growth and strength of the arm ipsilateral to
the shunt (steal phenomenon)

64. • Recent modified BT shunt
• Involves interposing a prosthetic graft
of polytetrafluoroethylene between
the subclavian artery and ipsilateral
right or left PA
• Benefits of the modified BT shunt
include technical ease of the
procedure (which can be performed
on either side of the aortic arch),
wide anastomoses proximally and
distally, and preserved blood flow to
the ipsilateral arm .

65. • Disadvantages
• Thrombosis of a modified BT shunt is not uncommon and may be life
threatening.
• Although usually a late complication (occurring months to years after
shunt creation), thrombosis can occur in the early postoperative
period as well.
• Diagnosis -echocardiography /CT angiography / MR imaging.
• While subtotal occlusion or stenosis of a BT shunt is typically treated
with shunt revision, treatment with balloon angioplasty and stent
placement has been described

66. • A seroma adjacent to the shunt.This is caused by seepage of fluid
across the polytetrafluoroethylene graft.
• If large enough- airway compression or pericardial tamponade.
• The diagnosis of a seroma-echocardiography/CT.
• US-guided percutaneous drainage of these seromas .

67. POTTS SHUNT
• Potts 1946
• between the descending aorta
and ipsilateral pulmonary
• When SCA small or poorly
developed
• Disadvantages
– Difficult to close during
definitive repair
– Pulmonary hypertension
due to enlargement of
anastamotic channel

68. Waterston
• 1962
• Waterston shunt consists of
a connection between the
ascending aorta and
pulmonary artery,
• more easely closed

69. • The original Glenn shunt 1958
• The shunt was intended to palliate congenital heart defects in which there was
hypoplasia or atresia of rightsided structures of the heart—conditions such as
tricuspid atresia, Epstein anomaly, and pulmonary atresia with intact ventricular
septum.
• consists of an end-to-end anastomosis of the right PA (divided from the main PA) to
the side of the SVC.
• The SVC is ligated at its entrance to the right atrium, directing all proximal SVC
blood flow to the right PA.
• The azygos vein is also ligated.

70. • BDG shunt
• The BDG shunt consists of an end-to-side
anastomosis between the SVC (again
divided from the right atrium) and the
right PA.
• Because the right PA is not divided from
the main PA in this procedure, the BDG
shunt directs flow from the SVC into the
right and left PAs.
• If there are two SVCs that are similar in
size, each is anastomosed to its respective
ipsilateral PA .
• Currently, the BDG shunt is used as a
staging procedure in children with single-
ventricle physiology who will ultimately
require a Fontan procedure .
• Because adequate flow through the shunt
depends on low pulmonary pulmonary
vascular resistance, this procedure is
typically performed between 3 and 9
months of life, after pulmonary vascular
resistance falls from elevated neonatal
levels.

71. • Even though most patients will have effective long-term palliation
with the BDG, one long-term complication is the development of
pulmonary arteriovenous fistulas.
• These malformations, which result in ineffective gas exchange, are
thought to be due to the lack of hepatic venous blood perfusing the
lungs.

72. • Humoral factors in the hepatic venous blood seem necessary to
prevent formation of these fistulous communications.
• These fistulas can be diagnosed by using contrast agent–enhanced
echocardiography, CT angiography, MR imaging, and conventional
angiography.
• The BDG shunt is usually followed at 2–4 years of age by the
complete Fontan procedure, which diverts all systemic venous return
to the pulmonary vascular bed directly.
• This directs hepatic blood flow to the lungs, prevents the
development of fistulas, and removes the volume overload on the
heart

73. DEFINITIVE REPAIR

75. THANK
YOU