Duct dependent Pulmonary Circulation

Cardiovascular malformations are the commonest type of congenital malformation (6-

8/1000) and responsible for more deaths in the first year of life than any other birth defects. A
sixth of the affected children have a duct dependent circulation, with a persistent ductus arteriosus
being necessary for survival. Over the past few years, great efforts have been made to screen these
duct-dependent congenital heart diseases (CHD) in the newborn.
History
1-day old girl term baby, Appropriate for gestational age.
birthweight 3.1 kg
with birth order 3rd (1st child 18 yrs. and 2nd 15 years both apparently healthy).
Mother 41 years and father 42 years.
Mother had Gestational diabetes and gestational hypertension, on Insulin, labetalol, nifedipine and
ecospirin.
Antenatal USG told to be normal.
Baby got delivered by emergency LSCS, in view of elevated blood pressure of mother.
Baby cried immediately after birth and first few hrs were uneventful. At about 10 hrs after birth,
cyanosis was noticed and pre and post ductal desaturation 65-80%. Hence evaluated.
Echo showed Single ventricle of LV morphology and Pulmonary stenosis.
History of feeding well prior to desaturation.
Baby shifted to SCTIMST for further evaluation and management.
On Physical examination
Baby active, cyanosed peripheries
HR: 140 bpm
BP: 87/58 mm Hg
Saturation: 72%
RR: 40 bpm
CVS: S1 – Normal
S2 Single.
3/6 ESM in RVSB.
 Systolic murmurs are graded on their intensity using the
following method: Grade 1/6 - Barely audible. Grade 2/6 -
Audible, but faint. Grade 3/6 - Easily heard. Grade 4/6 -
Very easily heard
Resp: B/L NVBS with no added sounds.
Blood investigations:
Hb: 16.1 mg/dl
Platelet: 2.25L/Cumm
TC: 31,600
Polymorphs:38
Eosinophils:0
Lymphocytes: 62
ESR: 4
BUN: 5 mg/dl
S. Creatinine: 1.0 mg/dl
Sodium: 131 mg/dl
Potassium: 3.6 mg/dl
Chloride: 96 mg/dl
Alkaline phosphatase:136 mg/dl
SGOT: 45 mg/dl
SGPT: 17 mg/dl
Total proteins: 5.9 mg/dl
Albumin: 3.3 mg/dl
Globulin: 2.6 mg/dl
Bilirubin Direct: 4.6 mg/dl
Conjugate: 0.2 mg/dl
CXR: CTR 60%. (The ratio between the maximal horizontal cardiac diameter and the maximal
horizontal inner thoracic cage diameter. A CTR > 0.5 (or > 50%) is considered abnormal).
Decreased pulmonary blood flow / Pulmonary oligemia.
Echo: Situs solitus, Levocardia
Large inflow VSD BDS
Hypoplastic Right ventricle
Aorta from Right ventricle
Long segment Pulmonary atresia
Closing PDA
5 mm Os ASD BDS
Echo: Common atrium
Atretic right AVV(Atrioventricular valve), Cleft AML (anterior mitral leaflet )
Large subaortic VSD
Single ventricle of LV type
Long segment pulmonary atresia
RPA 4mm LPA 2.8 mm
? Non confluent Pulmonary arteries (each pulmonary artery arises independently)
RPA and LPA supplied collaterals
Moderate MR, Good ventricular function
Diagnosis:
Cyanotic Congenital Heart Disease, Situs solitus, Levocardia, Single ventricle of LV morphology
Long segment Pulmonary Atresia with Large subaortic VSD
Bilateral PDA dependent Pulmonary Circulation
Atretic Right AV valve
Cleft AML
Moderate MR
Good ventricular function
Not in HF
 Discussion - Duct Dependent Pulmonary Circulation
In fetal circulation, the right side of the heart has higher pressures than the left side of the
heart. This pressure difference allows the shunts to remain open. The three vascular structures
most important in the transitional circulation are the ductus venosus, foramen ovale, and ductus
arteriosus. In postnatal circulation, when the baby takes its first breath, pulmonary resistance
decreases, and blood flow through the placenta ceases.
In the developing fetus, the ductus arteriosus connects the pulmonary artery to the
descending aorta, allowing most of the blood ejected from the right ventricle to bypass the
nonfunctioning lungs and transfer to the aorta and then to the placenta for oxygenation.
Endogenous prostaglandins, primarily prostaglandin E2 (PGE2) and prostaglandin I2 (PGI2), are
produced within the lumen of the ductus to maintain patency. Functional closure of DA occurs in
15 hrs. after birth with the contraction of medial smooth muscle due to increased PO2 and
decreased PGE1. Anatomical closure in 3 weeks by replacement of smooth muscle fibers with
fibrosis creating ligamentum arteriosus and the duct loses the ability to reopen.
However, in certain heart conditions where there is a block to the blood flow to lungs or
the body, or a condition where the blood vessels supplying the lungs and body are switched
(transposition of great arteries), an open ductus is necessary for survival.
A neonate is said to have a ductal‐dependent lesion when the pulmonary or systemic blood
flow is dependent on the ductus arteriosus remaining patent.
If duct closure cause significant decrease in systemic circulation, the condition is called
Duct Dependent systemic Circulation.
If duct closure cause significant decrease in pulmonary circulation, the condition is called
Duct Dependent Pulmonary Circulation.
In Duct-dependent pulmonary Circulation Lungs are under perfused in these babies. PDA
diverts partially saturated systemic blood towards the pulmonary circulation to improve the
overall saturation. (During systole?)
The conditions which demand ductus patency for pulmonary circulation are

 Pulmonary atresia with or without VSD

 Critical pulmonary stenosis
 Tricuspid atresia
 Tetralogy of Fallot
 Severe Ebstein’s anomaly
 Severe TR

In these conditions Severe restriction of pulmonary blood flow, and postnatal

constriction of the ductus causes severe hypoxemia, cyanosis and death.
Clinical presentation
Usually coincides with the time of ductal constriction and closure. Often present in the
first few days of life with incremental cyanosis.
Clinical presentation table
Neonatal cardiac evaluation
Airway, breathing, circulation
History and physical examination.
Inspection and palpation
Activity, cyanosis
Peripheral perfusion
Dysmorphism and systemic review.
Auscultation – single S2, murmurs +/-
Saturations and blood gases 100% oxygen (Bag and mask or by intubation)
10 minutes
Liver size
Right arm PO2
Hyperoxia test
>250 – unlikely Cyanotic CHD
Septic workup.
100- 250 – possible cyanotic CHD

<100 – Cyanotic CHD

CXR – Normal
Pulmonary hyperemia – increased vascular markings, increased pulmonary blood flow
Pulmonary oligemia – decreased pulmonary blood flow.
Diagnosis of Duct dependance – Echo is the gold standard

 Delineate anatomy
 Assess size of PDA, degree of restriction or constriction
 Assess PVR / SVR ratio on the flow pattern across the duct.

Management
Ensure Airways and optimize oxygen.
Breathing – if needed, ventilate to off load work of breathing.
Circulation
Drugs – cyanosis or if the baby is in shock start Prostaglandin.
Management aim:
Ensure pulmonary blood flow by keeping the duct patent until the baby is ready for a
definitive corrective surgery.

 Drugs
 Stenting the Ductus Arteriosus
 Shunts

Drugs

PGE1 is a potent dilator of the ductus arteriosus in human neonates. Alprostadil (PGE1) is
a naturally occurring prostaglandin that was approved by the Food and Drug Administration
(FDA) in 1981 for use in infants with CHD that required maintenance of ductal patency until
palliative or corrective surgery could be performed. Ductus usually reopens within 30 minutes to
two hours of initiating PGE1, with the clinical response usually being instant if the duct is vital for
the infant's hemodynamic status.

PGE1 infusion protocol

 Starts at 0.05 – 0.1 mcg/kg/min.

 Depending on the clinical response (saturation, femoral pulse, urine output) infusion rates
can be increased up to 0.4mcg/kg/min. The maintenance dose of the Alprostadil
(Prostaglandin E1) infusion is determined by titration according to the infant's response -
oxygenation versus adverse effects.
 Once the desired response is obtained, that is a stable saturation of 70-80%, maintain
patency by continuing infusions at 0.01 – 0.05 mcg/ kg/min.
Prostaglandin PGE1 – Nursing Considerations
Preparation

 Available as PROSTIN VR as 1 ml ampoules containing 500 mcg/ml.

 Dilute 0.1 ml (50 mcg) in 50 cc 5% Dextrose under laminar air flow to avoid sepsis.
 The remaining PGE1 is stored in a fridge at 2-8o C.
 Dilute 0.1 ml (50 mcg) in 50 ml 5%D; in this dilution, each ml equals 1 mcg of PGE1

Administration

 Continuous IV infusion via a syringe pump because of the rapid metabolism of this drug.
Since 60% to 80% of PGE1 is metabolized on first pass through the lungs, it must be
administered by continuous infusion.
 Y-site compatible with caffeine citrate, dopamine, heparin, and morphine.
  Do NOT mix with blood or blood product.
 Use a separate line for Prostaglandin E1 administration. Administer into a large vein. Do
NOT flush line. Maintain a patent IV line at all times. Ensure reliable I.V. access, observe
closely for IV infiltration and resite immediately if this occurs.
 Compatible with Normal Saline, 5% Glucose, 10% Glucose.
 Change solution and tubing every 24 hours.

Observation and documentation

 Monitor for adverse reactions, especially decreased respiratory effort, periodic breathing or
apnoea.
 Monitor arterial pressure closely. If arterial pressure falls, a bolus of fluid (10- 20 ml/kg) is
required. It may be necessary to decrease the rate of infusion.
 Closely monitor heart rate, respirations, and temperature. Document hourly.
 Assess the duct for patency
 Monitor oxygenation with blood gases and pulse oximetry. Pulse oximetry is mandatory
due to risk of apnoea and to monitor therapeutic effect
 Assess for signs of improvement.

Storage

 Store in refrigerator at 2°- 8°C.

 Discard ampule after use. In case of glass vials we can keep this for 48 hrs, as the shelf life
is more.
 Diluted solution stable for 24 hours at room temperature.

Prostaglandin PGE1- Adverse effects

Short‐term effects (within the first 120 hours of PGE1 therapy)

 Hyperthermia (body temperature greater than 37.2 °C) - often in first 24 hours, needs tepid
sponging. Never an indication to stop PGE1. Ensure that insensible fluid losses of a febrile
neonate are met adequately
 Jitteriness or seizures - Rare, may need cessation of PGE1 infusion. Rule out other
metabolic and infective causes.
 Apnea (cessation of breathing for 20 seconds or greater) - Often noted in first hours
common in < 2 kg, preterm
if severe stop infusion
 Bradycardia: if severe stop infusion
 Flushing: often noted in first few hours
 Diarrhea (more than eight stools per day or loose stools containing blood in the absence of
radiologic evidence of necrotizing enterocolitis).
  Arrhythmias.
 Cutaneous vasodilation and flushing.
 Hypotension (mean blood pressure less than 10th percentile for age) –
 Manifests as cold extremities,
 delayed capillary refill > 3 min tachycardia,
 low urine Rx: IV fluid bolus 5-10 ml/kg

Long‐term adverse effects (120 hours or greater of PGE1 therapy) evaluated any time during
hospital stay or follow‐up in the first six months of life.

 Cortical hyperostosis of the long bones (measured by persistently elevated alkaline

phosphatase and x‐ray changes of extensive symmetrical periosteal reactions in long bones
sometimes associated with clinical findings of limb edema).
 Gastric outlet obstruction (measured by ultrasound findings of elongated and thickened
pyloric musculature or marked antral mucosal hypertrophy).
 Development of medial edema/hemorrhage, abnormal interruption of the internal elastic
lamina and intimal tears (seen histologically after surgery or autopsy).
 Radiographic visible calcifications corresponding to the anatomic distribution of brown
adipose tissue especially along the great vessels of the neck, within the infraclavicular
areas and axilla (suggestive of brown fat necrosis).

PDA stenting
Ductal stenting is an effective alternative procedure to palliate the patients with duct-dependent
pulmonary circulation. The aims of DS are to provide adequate blood flow with equal distribution
through the pulmonary circulation in an acceptable time-frame for a particular surgical definitive
treatment of each patient.
The diameter, the length, and the type of the stent are major factors to be considered for
this aim. Stent diameter should be selected on the basis of the patient size, ductal morphology,
expected duration of palliation, and the type of the next surgical treatment.
Krichenko classification of PDA
Krichenko classification of PDA is based on angiography and includes Type A (“conical”)
ductus, with well-defined aortic ampulla and constriction near the pulmonary artery end. B, very
large type B (“window”) ductus, with very short length. C, Type C (“tubular”) ductus, which is
without constrictions. D, Type D (“complex”) ductus, which has multiple constrictions, and type
E (elongated) PDA.
Types
Bare metal coronary stents (BMS) have been mostly used. However, the reintervention rate
due to in-stent restenosis of BMS is 17% to 25% at 6 months.
 Drug-eluting coronary stents (DES) are more commonly used with less luminal loss and
less unplanned reintervention. With the latest generation of the DES, these can be over-expanded
from 4 mm up to 5.5 mm. This characteristic provides a room for further dilation in growing
infants before they reach ultimate surgical repair.
Bioabsorbable stents are new generation, currently being developed.
Pre-Procedural Assessments and Preparation

 Transthoracic Echocardiogram (TTE)

The following details should be obtained from TTE: (1) Overall cardiac morphology, (2)
Origin, shape, and length of the DA, and (3) Pulmonary artery connection and presence of
associated pulmonary coarctation.
 Cardiac Computed Tomography (CCT) and 3-Dimensional (3D) Planning
This modality provides precise shape, length, constriction of the DA as well as information
about the pulmonary artery and the adjacent vessels. Additionally, it provides a guidance for
the appropriate angiographic projections during cardiac catheterization.

Three-dimensional cardiac computed tomography in different origins of the ductus arteriosus

(DA). (A) Originated from the proximal descending aorta. (B) Originated from the aortic arch. (C)
Originated from the aortic branches.
 Management of the Prostaglandin E1 (PGE1)
Neonates who are on PGE1 infusion, ductal constriction should be reevaluated 4−6 h before
the procedure. If the ductal waist is larger than 3 mm, PGE1 should be reduced to a minimum
dose or discontinued with the aim to maintain the oxygen saturation around 75–80% and the
ductal waist of 2.5–3 mm. It is mandatory to confirm that the DA is properly constricted before
starting the procedure. Otherwise, there would be a high risk of stent migration.
 Antiplatelet Therapy
There is no universally accepted antiplatelet treatment protocol for the DS. it is reasonable to
start antiplatelet drug(s), such as aspirin and/or clopidogrel, a day before the procedure, to
minimize the possibility of acute stent thrombosis.
Here we Give a loading dose of ecospirin as per order for PDA stenting.
Ductal Stenting Procedure
Ductal stenting is a procedure with one of the highest risks for morbidity and mortality in
neonates. It should be performed in the neutral thermal environment under general anesthesia.
Vascular access should be obtained under ultrasound guidance. Heparin 50–100 U/kg should be
administered in order to maintain ACT > 250 s. Subsequently, selective angiograms are performed
in the appropriate positions, and are crucial to profile the ductal anatomy.
Approaches
(A) From right axillary artery.
(B) From left axillary artery.
(C) From femoral vein to ascending aorta.
(D) From femoral artery.
Instruments
Diagnostic catheters
Catheters for stent delivery
0.014″ guidewires
Coronary stents 3.5 mm, 4.0 mm, and 4.5 mm
Lengths 8-18 mm
Coronary balloons 2.5-4.5 mm
The Straight Ductus Arteriosus

(A) angiography demonstrates tightly constricted pulmonary artery end of the DA. (B) 0.014”
coronary guidewire crossed the DA. (C) Stent covered entire length of the DA.

The Tortuous Ductus Arteriosus

 Stenting of a tortuous vertical ductus arteriosus (DA). (A) Selective arch aortogram near
origin of the DA. (B) Repeated selective angiogram after placing the guidewire in the right PA
demonstrated stretching of the DA. (C) Length interrogation was performed with markers of
the coronary balloon catheter (without inflation). (D) Introducing the stent with the same
length of the previous balloon catheter. (E) Inflation of the stent at its nominal pressure. (F)
Final angiogram showed satisfactory stent position with unobstructed flow to both Pas.
 Technical challenges of ductal stenting in the tortuous DA include difficulty in ductal
crossing, inaccurate measurement of ductal length, and non-uniform change of ductal
configuration after stretching with the guidewire and the stent
The Bilateral Ductus Arteriosus
These types of bilateral DAs usually supply disconnected central branch pulmonary arteries.
Stenting of the DA can be achieved with the same principles and methods, as mentioned earlier,
but each Ductus separately, with two access site and two stents.

Complications
I. Stent Thrombosis- can occur acutely (within 24 h), sub acutely (within 30 days), or late (≥30
days) after stent implantation.
Acute stent thrombosis
• To start oral antiplatelet dose of aspirin 24 h before the procedure
 • maintain activated clotting time >250 s throughout the procedure
• Infuse heparin of 15–20 U/kg/hr for 24–48 h.
Subacute and late stent thrombosis
Starting dual-antiplatelet therapy (DAPT) after the procedure and continuing them for at
least 6 months before switching to 3–5 mg/kg/day of oral aspirin until the next surgical
intervention.
If stent thrombosis occurs during the procedure, the DA should be rewired for balloon
dilation with 0.5–1 mm larger than the stent diameter. Local infusion of streptokinase or alteplase
should be given simultaneously.
II. Spasm of the ductal arteriosus- This is an uncommon complication (<1%), it tends to occur
with manipulation of guidewire and placement across the ductus.

 Re-start the PGE1 infusion

 If the problem recurs, it is best to abandon the procedure

III. Stent Migration

 Surgical removal of the migrated stent

IV. Pulmonary Over-Circulation- Suitable oxygen saturation after ductal stenting should be
approximately 85–90%. Higher ductal shunting after the procedure may cause pulmonary over-
circulation and/or significant diastolic run-off that may lead to coronary and systemic
hypoperfusion.

 Intravenous inotropic support and diuretics

V. Pulmonary Under-Circulation – mostly occur when the ductal tissue is not entirely covered,
leading to ductal constriction, or when the pulmonary artery is additionally jailed by stent
migration.
Provide adequate oxygenation/ventilation, intravascular volume, hemoglobin, and cardiac
output.
If desaturation persists, Echo to assess discrete ductal constriction, stent occlusion, or
jailing of the pulmonary artery. Consider re-intervention if needed.
Post-Procedural Care
After the procedure, the patient should be transferred to the neonatal intensive care unit for
close monitoring with arterial and central venous lines. The patient can be extubated once
hemodynamic and neurologic conditions are stable. Heparin and anti-platelets should be given as
previously mentioned. Close follow-up, especially at 3–6 months after the procedure, is
recommended since the risk of re-intervention is high during this period.
 Ductal stenting is an effective alternative procedure to palliate the patients with duct-
dependent pulmonary circulation. It requires comprehensive planning, proper catheter laboratory
environment/instruments, skillful and careful operators, and seamless teamwork to make this
procedure safe and successful.
Nursing considerations
Hemodynamic management
• Continuous cardiac monitoring
• Arterial BP monitoring
• Blood gas analyses, saturation
• Proper administration of inotropes and timely tapering and weaning off.
Ventilation and extubation
• Ventilator care management
• Plan early extubation once infant wakes up with no respiratory distress or neurological
impairment
• PRVC  SIMV(PRVC) + PS  NIV  alternate NIV and O2 cycles 
O2 via prongs  room air
Pain and sedation
• Dexmedetomidine and morphing infusion
• Sedation interval and observation
Fluid management
• Volume replacement with blood products
• Increased to normal requirement for the age of the child gradually
• Strict intake and output chart
• Diuretic therapy, as required.
Nutrition
• Assess tolerance to feed, bowel sounds, bowel openings, abdominal distention.
• Gradually change from RT feeds to oral feeds.
• Ensure weight gain.
Watch for Post procedure complications
Follow meticulous infection control practices
Discharge instructions
• Identification of warning signs
Cyanosis, Apnoea, Urine output, infection (IE prophylaxis)
• Feeding
• Regular follow up and drug compliance
• Immunization
• Emergency helpline numbers – 0471

The Blalock and Taussig Shunt

 The main aim of the palliative surgery is to increase pulmonary blood flow in a controlled
manner to alleviate cyanosis and improve exercise tolerance in the patients.
Indications
A. To increase blood flow to the lungs
B. When Complete repair is not possible
C. To encourage growth of pulmonary arteries
Types
Classic Blalock-Taussig shunt - between the subclavian and pulmonary arteries
Modified BT shunt- PTFE graft between the subclavian artery and the pulmonary artery
Central shunt - between the ascending aorta and the main pulmonary artery
Potts shunt - between the descending aorta and left pulmonary artery
Waterston - Cooley shunt- between the ascending aorta and right pulmonary artery
Sano shunt - between the right ventricle to pulmonary artery