A 12-year-old girl with a history of an undiagnosed ventricular septal defect (VSD) presents with shortness of breath, headaches, dizziness, leg swelling, and blue lips. Examination finds cyanosis, clubbing, leg edema, tachycardia, and an enlarged liver. Chest x-ray shows enlarged right ventricle and pruned pulmonary arteries. She is diagnosed with Eisenmenger syndrome due to longstanding left-to-right shunt through her VSD reversing to right-to-left, and she requires management of complications like arrhythmias, bleeding, and right heart failure.

1. Preop ICU care of VSD
Dr.Ritajyoti Sengupta
Consultant Pediatric Intensivist,

2. Echo-Large PM VSD with trabecular extension,severe PAH
Extremities cool with
prolonged capillary refill time.
CVS -- gallop and holosystolic murmur
Liver edge palpable at
4 cm below RCM
Poor feeding for the last 12 h.
Tachypnea for
the last 12 h.
Grunting, retractions,
and wheezing bilaterally.
Fussy and showing signs
of respiratory distress
Capillary blood
gas –
pH 7.28,
PCO2 20 mmHg,
NaHCO3
11 mEq/L,
lactate
5.8 mmol/L.
Vital signs :
Temp 37.5 C,
HR 175 /min,
RR 75 /min,
BP 62/34 mmHg,
SPO2 - 94% room
air.
5mo full-term male 4.2 kg

3. cardiomegaly
left lung atelectasis
After the first 4–6 weeks of life, PVR DECREASES...
A PORTION OF LV STROKE VOLUME SHUNTED
THROUGH VSD INTO PULMONARY CIRCULATION
---pulmonary blood flow increase s AND
heart failure develop caused by
increased volume load

4. Qp and Qs relationship in presence of VSD

5. Risks of salbutamol neb,
100% oxygen,
fluid boluses
to remember
Avoiding β-agonists bronchodilators
---TO AVOID INCREASE IN AFTERLOAD
Maintaining optimal preload
Decreasing oxygen consumption
by sedation and invasive
or non invasive respiratory support
Improving contractility-----
inotropic support but avoid
tachycardia
Admit in PICU
Maintain SpO2 in the high 80s or low 90s
Apply positive end-expiratory pressure (PEE
Increased pulmonary blood
flow causes volume overload
and signs and symptoms of
CCF---poor feeding, tachypnea,
grunting, retractions, cardiac
wheezing, tachycardia,
hepatomegaly, gallop,
metabolic acidosis with
respiratory alkalosis, and
cardiomegaly on CXR
Target---
Optimizing hemodynamic status
Management ACUTE DECOMPENSATED HF/CCF

6. ideal oxygen
saturation in
these patients
ranges between
88 and 94%.
ventilation reduces the
afterload on the left
ventricle, ---improving
left ventricular systemic
output.
Positive end-expiratory
pressure (PEEP), ideally with a
FiO2 of 0.21 increase afterload
on the right ventricle
limit left-to-right shunting---
decrease pulmonary circulation
improve systemic circulation.
improve lung compliance,
decrease work of breathing and
total body oxygen consumption
Invasive or Noninvasive,
Ventilation

7. TREATMENT AT DISCHARGE FROM PICU
MEDICATION
ORAL DIURETICS
ORAL DIGOXIN--
-out of fashion
ORAL ACE
INHIBITORS
FREQUENT CALORIE
DENSED SMALL FEEDS
CHECK WEIGHT GAIN
MULTI VITAMINS
PNEUMOCOCCAL AND
HiB VACCINES
STOP FUROSEMIDE
TEMPORARILY IF
DIARRHEA/DEHYDRATI
ON

8. Syndromic VSD with suspected Airway issues
7mo male 5KG
weakness, poor feeding,
failure to thrive, tachypnea,
nonproductive cough.
HR of 142/min
BP 75/45 mmHg,
RR 60/min, T 36.8 °C,
SpO2 is 94%.
Awake and alert and tachypneic with
mild wheezing and retractions
occasional inspiratory stridor
Marked hypotonia, almond-shaped
eyes with epicanthal folds, and a
large protruding tongue, pot
bellied
ON EXAM: a
Active precordium and
a pulmonary ejection
murmur as well as a
holosystolic murmur
at the apex radiating
to the axilla. His liver
is enlarged. Pulses are
equal on all four
extremities. His chest
x-ray shows
cardiomegaly and
increased pulmonary
vascular markings.
• ECHO: LARGE PM VSD ,MODERATE MR
• ,SEVERE PAH

9. KARYOTYPING---->Trisomy 21
• Anticipated airway issue---higher incidences of subglottic
stenosis and tracheomalacia------->Virtual Bronchogram/FOB
• Chance of hypothyroidism--FT4,TSH ASSAY
• Prone to infection----autoimmune disorders
• Other organ system abnormality--delayed
development,hypotonia,
• Severe PAH and early Eisenmenger syndrome
• Obstructive Sleep Apnea
• GERD
• Atlantoaxial/Atlanto occipital Instability

10. 6-mo female 4.5KG unrepaired large VSD
Low grade fever, runny nose,
congestion, and cough for the
last 3 days.
Taking longer to feed and is
breathing “hard.” off late. baby
chokes and gags with coughing
but denies any color change or
apnea.
Vitals temperature of
38.3 °C, heart rate of
180/min, respirations of
70/min, blood pressure of
82/50 mmHg, and oxygen
saturation of 91% in room air.
Tachypneic with moderate
intercostal and subcostal
retractions with bilateral
wheezing.
A grade 3/6 holosystolic
murmur along the left sternal
border. Peripheral pulses are
well felt and capillary refill is
2 s. Abdominal exam reveals a
liver edge palpated 3 cm
below right costal margin

11. Brochiolitis
Bronchiolitis diagnosed clinically
Supportive care with oxygen and hydration.
Fluids judiciously in children with CCF
No role for of bronchodilators
Nebulized hypertonic saline (3%)
reduction in airway edema
mucus plugging
improved mucociliary clearance
Heated, humidified, high-flow nasal cannula
(HFNC) therapy
Need for MV two times higher
in children with RSV infection

12. RSV and VSD
most common viral pathogen
causing ALRIs in children
Severe RSV-ALRI among kid
aged <5 years especially
hemodynamically significant
CHD like VSD
Immunoprophylaxis with
PALIVIZUMAB -- for prevention
of RSV infection in CHD
Palivizumab - reduce RSV
hospitalizations among children
with hemodynamically
significant underlying CHD by
45% and the length of hospital
stay (LOS) by up to 76% up to
two years of age with CHD
NO standard universal protocol
for palivizumab use
Risk of RSV--- higher
hospitalization /higher ICU
admission /requiring oxygen
supplementation /more
mechanical ventilation /high
case-fatality ratio
The Journal of Infectious Diseases® 2019;XX(XX):1–8

13. Echo shows a large unrestrictive VSD
with severe pulmonary hypertension.
intubated and ventilated
for severe breathing
difficulty
desaturation with oxygen
86%
4-day history of fever, cough,
breathing difficulty.
history of recurrent RTI
poor weight gain.
EXAM--- Cachectic without any dysmorphic
features.
Significant respiratory distress,
bounding pulses, bilateral crepitations,
laterally displaced apical impulse,
grade 3/6 systolic murmur in the left
LSB, and hepatomegaly.
stays in a remote village
1year male weighs 5 kg (birth weight 2.4 kg)
VSD with FTT with LRTI

14. CXR
Empiric
intravenous
antibiotics
Laboratory
Investigations
CBC ESR CRP
LFT/RFT,
bacterial cultures of
blood and tracheal
secretions,
electrolytes, ABG
ECG,CXR, and ECHO
Respiratory secretions
were tested for viral
pathogens
cardiomegaly and right and left lung lobar
consolidation.

15. WHY VENTILATION
• For one or more of the following reasons:
• 1. Increased work of breathing with impending respiratory
failure;
• 2. heart failure not responding to maximal pharmacologic
support;
• 3. acute respiratory failure with significant hypercarbia,
hypoxemia, or both despite supplemental oxygen.

16. Decision to Operate
Earlier:--- attempted to wean
the patient from MV
by using standard guidelines
if the patient showed clinical
improvement in the first
48 hours.
Now:--perform corrective
surgical intervention at the
earliest safe opportunity,
determined by improving
clinical and ventilatory
parameters.
minimum requirements to be necessary
to proceed with surgical intervention:
1. Absence of fever for 48 hours or longer;
2. Decreasing leukocyte counts, if initially
increased, and increasing platelet counts,
if initially reduced;
3. At least partial radiologic clearance of
lung infiltrates;
4. Improved arterial blood gases (PaO2 60
mm Hg and PaCO2 less than 50 mm Hg
on FiO2 0.4 using PCV with maximum
peak inspiratory pressures of 30-35 mm
Hg and tidal volumes of 10mL/kg);
5.documentation of predominant left-to-
right systolic shunting on color Doppler
echocardiography.
Documentation of negative bacterial
cultures and normalization of gas
exchange, although desirable, were not
mandatory.
J Thorac Cardiovasc Surg 2004;127:
1466-73

17. Treatment Options
For a sick ventilated infant with a large VSD and severe pneumonia, one of the following 3 management strategies
medical management and corrective
surgical intervention during
the same hospitalization
medical management,
hospital discharge, and,
assuming survival,
subsequent elective
surgical repair
medical management and
palliative surgical
intervention with
pulmonary artery banding
during the same
hospitalization
Many physicians in the
developing world would
favor the second or third
management strategy
J Thorac Cardiovasc Surg 2004;127:
1466-73

18. Nutritional management for severe FTT with VSD
Infants with acyanotic lesions
--- more pronounced decrease in
weight gain velocity as
compared to length
reduced ability to fight infection
or poor surgical wounds healing
,prolonged ICU and
total hospital length of stay
Enteral nutrition --
preferred mode of nutritional
support in paediatric ICUs
Increase the caloric density
of infant formulas and breast milk
Require caloric densities
of >20 kcal/oz for CHD INFANTS
For patients not enterally fed
parenteral nutrition (PN)
good option
Early Complementary FEEDING

19. Nutritional Plan
Counselling about proper feeding
Calculation of energy requirements
according to age with a range
between 90-110 Kcal/kg/day)
multiplied by stress factor of 1.2
to calculate extra calories for
the cardiac condition
PROTEIN 1.1-1.6 g/kg/day
Micronutrients/Iron
For complicated cases and cases
with severe FTT
hospital admission for treatment
of the complication, starting
Ryle'S TUBE feeding as early as
from first days of admission
if the patient can tolerate feeding
and increase the amount
gradually till reaching the full
caloric requirements.
Through regular follow up visits
every 2 weeks or 1 month at
each visit: weight and length
measurement
DOI: 10.24953/turkjped.2017.04.011

21. EVIDENCES
Low body weight
Kogon et al.--
did not affect the
duration of the operation,
ACC, CPB, and MV
Schipper et al no
complications despite
prolonged hospital stay, MV
and ICU stay.
Schipper et al
Down syndrome
longer duration of MV
and ICU stay
PediatrCardiol(2017)
38:264–270
DOI10.1007/s00246-016-1508-2
Caution
infants,approximately <4.5 kg
https://doi.org/10.1016/j.
jtcvs.2018.11.111
.

22. 12-year-old girl admitted in PICU
Shortness of breath NYHA CLASS 2,
worse with physical activity.
Frequent headaches and sometimes
feels dizzy.
Progressive swelling of her feet and
lower legs,
At times bluish discoloration of her lips.
Was diagnosed with a hole in her heart
as an infant but was told it will close
on its own.NO follow-up for this
condition
Examination well-nourished child,
central cyanosis, digital
clubbing, and pitting edema of
both lower extremities.
Tachypneic, with a HR of 110/min,
RR 32/min, BP 110/65 mmHg,T
37.0 °C.
No murmur but a loud single
second heart sound. The liver is
enlarged.
Her hemoglobin is 20.6 g/dL and
hematocrit 65%.
CXR -- an enlarged right ventricle,
prominent central pulmonary
arteries, and pruning of the
peripheral pulmonary arteries.

23. Eisenmenger VSD
COMPLICATIONS---
Cardiac arrhythmias,
hemoptysis, infections, and
right heart failure,Pulmonary artery
(PA)dilatation and
in situ thrombosis
associated morbidities e.g
.iron deficiency, thrombosis,
gout, renal dysfunction,
cholelithiasis, and cerebral
thrombosis
Disease-specific targeted PAH
therapy Sildenafil Bosentan
Aldosterone antagonists
(eg, Spironolactone) and loop diuretics
Use of diuretics JUDICIOUSLY
Secondary erythrocytosis,
iron deficiency, and venesection
Prophylactic or routine venesection
to maintain a hematocrit level <65%---
NOT RECOMMENDED
Anticoagulation ----not routine
coexisting conditions such as
atrial fibrillation, pulmonary
thromboembolic,
congestive heart failure, or embolic
events
Adequate contraception,
endocarditis prophylaxis,
immunization against influenza, and pneumococcal infections.

24. Noncardiac Surgery with VSD
high-risk category ---after
operation--observed
in ICU by experienced staffs
Laparoscopic and video-assisted
surgery preferred
Good pain relief and control of nausea-vomiting
along with the specific events to look for such as
dysrhythmias, bleeding and thromboembolic
events, PHT crises dehydration, pain,
ventilator issues and other
complications
Factors that are likely to
require transfer to ICU
1.Signicant intracardiac
shunting
2.More than mildly elevated
3pulmonary vascular resistance
4.More than moderate left
ventricular dysfunction or
failure
5.More than mild right
ventricular dysfunction or
failure
6.A systemic right ventricle
7.Pregnancy in a patient with
signicant congenital heart
disease
8.New onset of symptomatic
tachy- or brady-arrhythmias