1. OPTIMIZING CARDIAC HEALTH: ROLE OF
PHYSIOTHERAPIST IN CONGENITAL
HEART DISEASE
PRESENTED BY SUPARNA BANDYOPADHYAY MODERATOR: DR. SHILPASREE SAHA (PT)
2ND YEAR, MPT (NEUROLOGY) ASSISTANT PROFESSOR, NIHS, KOLKATA
NIHS, KOLKATA

2. Content ..
Introduction to congenital heart disease
Epidemiology
Risk factors of congenital heart disease
Classification of congenital heart disease
Signs and symptoms of congenital heart
disease
Assessment of congenital heart disease
Plan of care
Pt management & recent advancement

3. CONGENITAL HEART DISEASE
• CHD refers to the structural heart defects that are present at birth.
A substantial proportion of patients with CHD have significant problems involving
other organ systems or specific chromosomal and single gene disorder
• Trisomy of 21
Down
syndrome
• 45 XO
Turner
Syndrome
• TBX5, AD
Holt Oram
VSD & TOF
ASD & VSD
Coarctation of
Aorta

4. PREVALENCE AND INCIDENCE OF CHD
CHD accounts for nearly one-third of all major congenital
anomalies. responsible for 28% of all congenital birth defects.1
Globally the prevalence of CHD in infancy is estimated at 8-12 per
1000 live birth.1
25% cases are life threatening and require early interventions
Over 200,000 children are estimated to be born with congenital
heart disease in India every year
About one-fifth of these suffer from critical heart disease requiring
early intervention
1. Reference: Saxena, A. Congenital Heart Disease in India: A Status Report. Indian Pediatr 55, 1075–1082 (2018). https://doi.org/10.1007/s13312-
018-1445-7

5. PREVALENCE AND INCIDENCE OF CHD
Regional distribution of infants with critical
heart disease accessing surgery as compared to
the total number born with critical heart
disease.1
Incidence of different types of congenital heart
defects in Asia2
2. Chung, I.-M.; Rajakumar, G. Genetics of Congenital Heart Defects: The NKX2-5 Gene, a Key Player. Genes 2016, 7, 6.
3

6. Gestational
Diabetes
Febrile illness
during 1st
trimester
Family history of
malformation, history of
having a child with birth
defects, twin pregnancy
Viral infection
e.g. - Rubella
Systemic autoimmune
condition (Lupus)- complete
heart block
Vitamin deficiency
Advanced
maternal age >35
Drugs- Anticonvulsants, NSAIDs, sodium
valproate
Exposure to organic solvents
like pesticides & ionizing
radiation
RISK FACTORS OF CHD4,5,6
4.Abqari S, Gupta A, Shahab T, Rabbani MU, Ali SM, Firdaus U. Profile and risk factors for congenital heart defects: A study in a tertiary care hospital.
Ann Pediatr Cardiol. 2016 Sep-Dec;9(3)
5. in, X., Ni, W., Wang, G. et al. Incidence and risk factors of congenital heart disease in Qingdao: a prospective cohort study. BMC Public Health 21,
1044 (2021).
6. Naghavi-Behzad M, Alizadeh M, Azami S, Foroughifar S, Ghasempour-Dabbaghi K, Karzad N, Ahadi HR, Naghavi-Behzad A. Risk Factors of Congenital
Heart Diseases: A Case-Control Study inNorthwest Iran. J Cardiovasc Thorac Res. 2013;5(1):5

7. CHD
Acyanotic
Left- to-
right shunts
Outflow
obstruction
Cyanotic
TOF TGA
Classification of CHD
Pulmonary
stenosis
Aortic
stenosis
Coarctation
of aorta
VSD
ASD
PDA
Right to left shunt
Truncus
arteriosus

8. Cyanotic and Acyanotic heart disease
Acyanotic heart
disease
Volume related
Acyanotic Congenital
Heart Defects
Pressure related
Acyanotic Congenital
Heart Defects
the blood is shunted from the left to right
side of the heart most often due to the
structural defects
Cyanotic heart disease
It is a class of congenital heart disease where the deoxygenated blood bypassing the lungs enters into the systemic
circulation due to any structural abnormality or malpositioning of the great arteries or any condition that increases
PVR that results in development of collateral circulation
A cyanotic heart defect causes a decrease in oxygen saturation, which causes the lips, toes, nail beds to appear
blue (cyanosis is Greek for blue).

9. ATRIAL SEPTAL DEFECT (ASD)
ASD is a condition characterized by the presence of
abnormal communication between two Atrium.
Types of ASD
ASD
Ostium primum
Ostium
secundum The GAP lies at the fossa ovalis
The gap lies inferior to the fossa ovalis.
This type of ASD is accompanied by a
congenital cleft in the mitral and/or
tricuspid valve which may lead to MR &
TR respectively

10. Ventricular septal defect(VSD)
VSD is a condition characterized by the presence of abnormal communication between two
ventricular septum.
• The defect may occur in an isolated manner or may be associated with other congenital
defects.
• A simple VSD usually causes left-to-right shunting and congestive heart failure. This defect usually
does not appear immediately after birth. It appears at 6 to 8 weeks of age, when the PVR has
decreased enough that the shunt becomes large
• Up to 50% of small VSDs close spontaneously and never become symptomatic.
With a large defect, excess blood goes
to the lung and causes pulmonary
congestion,
which leads to shortness of breath. A
large volume of blood returns from
the lungs to the left heart
which, over time, becomes
overburdened and enlarged. Heart
failure may even occur causing a
backup of fluid in the lungs and other
body tissues
Types of VSD
Peri membranous Inlet type Outlet type Trabecular
type

11. Patent ductus arteriosus
PDA when the fetal communication between the aorta and the pulmonary
artery remains open after birth and allows blood flow between the two vessels
A large
opening can
cause
pulmonary
congestion
Congestive
heart failure
Edema
Risk factor
Preterm birth
Congenital
rubella
syndrome
Chromosomal
abnormalities
fetal alcohol
spectrum
disorder
Prognosis
If left untreated, the disease may progress from left-to-right shunt to right-to-left shunt cyanotic heart,
called Eisenmenger syndrome

12. Aortic and pulmonary stenosis
PVS is a heart valve disorder where there is a narrowing in the pulmonary valve
Aortic valve stenosis in children is a congenital heart defect that causes fixed form of
hemodynamically significant left ventricular outflow tract obstruction with progressive course

13. Coarctation of aorta
The coarctation of the aorta is located at the junction of the arch with the descending aorta. It is a sharp
indentation involving the anterior, posterior, and lateral walls of the aorta
• This defect increases pressure in the arteries closest to the heart, the head, and the arms, causing upper-
body hypertension, with reduced circulation and diminished pulses in the lower extremities
• Blood pressure differs from upper extremities (high) to lower extremities (low).
• Most children require surgery to remove the constriction and reconnect the aorta. In 10% to 15% of
children, re-coarctation occurs, requiring further intervention, often with balloon dilation via angioplasty

14. Tetralogy of Fallot
A clinical syndrome characterized by
VSD causes
blood mixing
freely between
the ventricles
pulmonary
stenosis, causes
a right
ventricular
outflow tract
obstruction
The third
component is
an aorta
positioned
above the
Ventricular
septum
hypertrophy of
the right
ventricle is
caused by
increased
pressure due to
the right
ventricular
outflow
obstruction
• The prevalence of tetralogy of Fallot is estimated to be 0.02–0.04%, which corresponds to approximately 200 to
400 cases per million live births
• Although males and females were initially believed to be affected equally, more recent studies have shown that
TOF affects males more than females
• Genetically, it is most commonly associated with Down syndrome and DiGeorge syndrome
• If left untreated, TOF carries a 35% mortality rate in the first year of life, and a 50% mortality rate in the first
three years of life

15. Transposition of the great arteries.
TGA occurs when the aortic artery arises from the right ventricle and
the pulmonary artery from the left ventricle.
These errors cause deoxygenated blood to circulate around the body
and the already oxygenated blood returns to the lungs

16. • Common presenting symptoms:
Dyspnoea on exertion
Dyspnoea at rest
Recurrent episodes of cough fever
Common presenting sign & symptoms(Acyanotic CHDs
Mechanism
 Heart failure
 Persistent pulmonary congestion
Large sized VSD
Large sized PDA
Lung infection
Moderate to large size
VSDs
Large PDA
Uncommonly ASD
CHDs remain asymptomatic for long time:
ASD (osteum secondum type)
Small sized VSDs
Small sized PDA

17. Common presenting signs
Growth retardation(Symptomatic
individual)
• Signs of Respiratory distress
Chest signs of Lung infection
Tachycardia
• Raised JVP
Crepitation present over both lungs
• Tender enlarged liver
Edema (feet)
Features of heart failure

18. Common presenting symptoms(Cyanotic CHDs)
Cyanosis – Central
• Hyper cyanotic blue spell
Dyspnoea on exertion
• Growth failure
Recurrent respiratory infections
Management of cyanotic spell
Central cyanosis
• Clubbing of nails
Features of CHF(raised JVP, enlarged tender liver,
oedema)

19. Assessment of CHDs
History taking
 Demographic data, chief complaint, perinatal history, socioeconomic history,
developmental history of child, feeding
 Medical and surgical history
Examination
Age-appropriate Arterial Blood Gas Values
Vital Signs
• Heart rate,
• Blood pressure
• Respiratory rate.
Consider these values for patients at rest, and observe how they change with position changes
and activity to give an indication of the child’s cardiovascular response to activity. The trends
of the vital signs are very important.

20. General Appearance
• Body type ( built, Cachectic)
• Child’s state of consciousness
• Activity level of the child
• Use of any assistive devices
• Respiratory pattern
• edema or ascites
• Note general coloring:
 Pale
 Cyanosis
 Plethora
Pain
Age-appropriate pain scales
Visual analog scale (VAS) or the Wong-Baker FACES scale.

21. • observational/behavioral pain scale may be used. These include the face, legs, activity, cry, consolability
(FLACC)
• COMFORT scale, which has been validated for infants and young children
Integument
• glossy, turgid, loose, bruised, or broken down
• Capillary refill time
• Edema
• Nail clubbing
• Central or peripheral edema
Thoracic deformities should be examined, including the
• pectus excavatum
• pectus carinatum
• Barrel chest
• Rib flaring,
• Mid-trunk folds.
• Examination of the rib angles and intercostal spaces
Thorax and Respiratory Examination

22. Musculoskeletal Examination
• Range of motion,
• Postural alignment,( Scoliosis, kyphosis, )
Strength
• Manual muscle testing may not offer an accurate measure of the child’s strength, whereas dynamometry can
provide an alternative, objective means to specifically assess strength.
• Be sure to teach breathing techniques while assessing strength to avoid Valsalva maneuver during exertion.
• Consider using an eight-repetition maximum to fatigue, rather than a one-repetition maximum so as to
determine initial level of resistance
Functional Mobility
Functional mobility examination includes bed mobility, transfers, balance, gait, and stairs, as well as
developmentally appropriate activities
Aerobic Capacity and Endurance
• 6-minute walk test for functional capacity
• DYSPNOEA INDEX (DI)

23. Goal of the PT management
Functional
capacity
Mobility
Chest
clearance
Chest
expansion
Correction of
posture
Strength
and ROM

24. Goal specific Physiotherapy management
Positioning
Positioning may include
• Turning schedules
• Special devices or equipment, or recommendations for postures and positions(Positioning devices such as Multi-
Podus boots help control plantar flexion contractures, enhance hip rotation, and protect the heel)
• Positioning can also enhance oxygen transport and pulmonary function.
• Use Vital Sign Trends charts to place the child in different positions and see where the best ventilation-perfusion
matching may be so as to raise SpO2 and decrease heart rate, blood pressure, and respiratory rate
Multi-Podus boot
Postural Education and Awareness
• Postural correction and training
• Exercise for strength training and postural control
Breathing Exercises:
• Deep breathing exercise
• Breathing games- Blowing bubbles, air hockey, blowing a windmill, and sniffing stickers are an
excellent start to improve the child’s respiratory status
• Diaphragmatic breathing training, inspiratory muscle training- Incentive spirometers, and deep-
breathing techniques

25. Airway Clearance Techniques
Autogenic drainage
Phase I
Phase II
Phase III
Unstick the secretion
Collection of the secretions
Evacuation
ACBT
Postural Drainage
Use different positions for 10-15 minutes to clear the secretions
from the lung with the help of the force of gravity
Percussion
Vibration
Shaking
Breath control

26. Aerobic and Endurance Training
• Bicycle, treadmill, elliptical, upper body ergometer (UBE), or maybe over-ground exercise such as walking.
• The frequency should be at least three times a week and up to seven times a week. When a child is very ill, the
duration may be as little as short bouts of 2 to 5 minutes, with rest breaks between bouts.
• Lower-intensity stretching may be tolerated during rest breaks. Duration should progress to 30 to 45 minutes
as the child improves or transitions to the outpatient or early intervention setting
• Generally, intensity should begin at 60% to 65% of the maximal level of work. Intensity can also be prescribed
on the basis of RPE with the Borg scale, which should fall between 11 and 15 on the 20-point scale
• It is essential to remember that following a heart transplant, a patient does not have normal exercise response
due to loss of the vegus nerve, and therefore requires warm-up to increase the heart rate in order to have an
effect from the circulating catecholamines in the blood, followed by a cooldown.
Strength Training
Strength training is an important component of physical therapy for children of an appropriate age. Following
cardiac surgery, children generally have sternal precautions in place for 6 to 8 weeks, which may include lifting
precautions for greater than 10 pounds. With this caveat, strength training is a valuable tool in the treatment of
children with CHD both pre- and postoperatively. Patients should always be taught proper breathing techniques
with lifting to prevent a Valsalva maneuver and an unnecessary rise in blood pressure

27. Functional Mobility
Transfer training, gait training, balance training, and stair climbing are functional tasks that should be included
as necessary in physical therapy intervention.
Developmental Activity
Age-appropriate gross and fine motor play is very important for this population. Although there may be
many tubes and wires to manage in the acute care setting, an infant should be exposed to all positions,
including prone.
Neurodevelopmental outcomes of CHD
Children with CHD are at high risk for a myriad of neurodevelopmental challenges, the causes of which are
multifactorial and not yet fully understood. Early developmental milestones, including cognition, language, and
motor skills, are often delayed. One report found that 54% of infants with any type of single ventricle defect
were receiving early intervention for any developmental domain by 6 months, 62% by 12 months, and 67% by 2
years, while 45% of infants with two ventricle defects were receiving developmental intervention by 6 months,
43% at 12 months, and 52% at 2 years.
Rehabilitation
Cognitive training
Motor training
Sensory training

28. Recent advancement
Meyer M et. al conducted a RCT in 2021To improve health-related physical fitness (HRPF) (primary outcome) and
health-related quality of life (HRQoL) with a web-based motor intervention program in pediatric patients
with congenital heart disease.
Overall, 70 patients (13.0 ± 2.6 years; 34% girls) aged 10-18 years with moderate or complex CHD severity were
randomly allocated 1:1 to an intervention or control group. The intervention group trained 3 times per week for
20 minutes in a web-based exercise program over a period of 24 weeks. The control group followed the lifestyle per
usual. At baseline and follow-up, HRPF was assessed via 5 tasks of the FITNESSGRAM and converted to a HRPF z
score. HRQoL was assessed with KINDL self-report questionnaire
Study found that 24 weeks of web-based exercise intervention with an aimed volume of 60 minutes of exercise per
week was safe but did not improve HRPF and HRQoL in children with moderate or complex CHD
.
Meyer M, Brudy L, Fuertes-Moure A, Hager A, Oberhoffer-Fritz R, Ewert P, Mueller J. E-health exercise
intervention for pediatric patients with congenital heart disease: a randomized controlled trial. The Journal
of Pediatrics. 2021 Jun 1;233:163-8

29. Francisco José Ferrer-Sargues et. all in 2021 conducted a cohort study to check the Effects of Cardiopulmonary
Rehabilitation on the Muscle Function of Children with Congenital Heart Disease. Fifteen CHD subjects,
between 12 and 16 years of age, with reduced aerobic capacity on a cardiopulmonary exercise test, were
included in a three-month, 24-session cardiopulmonary rehabilitation program (CPRP) . Sessions lasted 70 min,
including endurance and strength-resistance training.
The sessions were structured as follows: (a) Warm-up phase (5 min): This included diaphragmatic breathing,
articular mobility exercises, and a light walk. (b) Endurance training phase (20 min): exercise was carried out in a
continuous modality using a treadmill (Magna Pro RC, BH Fitness, Madrid, Spain), and a static bicycle (BH Rhyno
Max H491) and included two min of warm-up and another two min of cooldown. The first eight sessions were
performed in a uniform continuous modality, adjusting the intensity to the subject’s VT1 HR. In sessions 09–16,
the load was increased progressively up to the VT2. The last eight sessions included rhythm modulations,
switching to varying continuous training, which oscillated between the VT1 and the VT2 HR [25]. (c) Resistance
training phase (20 min): during the first eight sessions, the subjects completed three series with four analytical
exercises, working out especially eight muscle groups (the deltoids, biceps brachii, triceps brachii, abdominals,
trunk extensors, quadriceps, hamstrings, and calves) [10,26]. The subjects made 10–15 repetitions of each
exercise, with a 20 s rest. The training was carried out with light and medium resistance bands. In the following
eight sessions, we emphasized exercises that included neuromuscular control using gymnastics equipment
such as dumbbells, bosu, medicine balls, steps, and Pilates balls, as well as doing plyometric workouts. These
functional routines included three series with four exercises in each one. The subjects completed 10–15
repetitions or 40 s work for each exercise, with a 20 s rest. During the last eight sessions, multi-circuits and
adaptive noncompetitive sports were trained, in addition to exercises related to daily living activities.

30. A substantial and statistically significant improvement was observed in the subjects’ handgrip strength (kg) (p <
0.001), biceps brachii and quadriceps femoris strength (N) (p < 0.001), as well as triceps surae fatigue process
(repetitions) (p = 0.018), with a maintenance of the results six months after the intervention. These results suggest
that a CPRP could potentially improve the peripheral muscle function of children with CHD.
Ferrer-Sargues FJ, Peiró-Molina E, Cebrià i Iranzo MÀ, Carrasco Moreno JI, Cano-Sánchez A, Vázquez-Arce MI,
Insa Albert B, Salvador-Coloma P. Effects of cardiopulmonary rehabilitation on the muscle function of children
with congenital heart disease: a prospective cohort study. International Journal of Environmental Research And
Public Health. 2021 May 30;18(11):5870.

31. Thank you