Approach To A Patient With Congenital Heart Disease

Approach to a Patient with
Congenital Heart Disease
Prof Dr Md Kabiruzzaman.
MBBS,MD, FNIC,AFACC.
Professor, Cardiology, NHFH & RI
Definition
• Congenital cardiovascular disease is defined

as an abnormality in cardiocirculatory
structure or function that is present at birth,
even if it is discovered much later.
Ref: BRAUNWALD’S Heart Disease, 8th Ed, P-1561

CHD
• Congenital cardiovascular malformations

usually result from altered embryonic
development of a normal structure or failure
of such a structure to progress beyond an
early stage of embryonic or fetal
development

Congenital Heart Disease :
Classification
• (A) Acyanotic group.

(i) Diseases with Lt-to-Rt shunt.
(ii) Diseases with obstructive lesions
• (B) Cyanotic group
CLASSIFICATION
• A. (i) Acyanotic with Lt-Rt shunts
1. Atrial Septal Defect (ASD)
2. Ventricular Septal Defect (VSD)
3. Patent Ductus Arteriosus (PDA)
4. Atrio-Ventricular Canal Defect (AVCD)
5. Arterio-venous malformation
CLASSIFICATION (cont)
A. (ii) Acyanotic with Obstructive lesions
1.Co-arctation of aorta
2.Interrupted aortic arch
3. Aortic stenosis
4.Mitral valve and supra valvular stenosis
5.Hypoplastic left heart syndrome
6.Pulmonary stenosis
• B.(i) Cyanotic with R-L shunt .
1.TOF
2.Tricuspid atresia
3. Total anomalous Pulmonary venous
connection (TAPVC).
4.Ebstein’s
5.Pulmonary atresia with VSD
6.Pulmonary atresia with intact ventricular
septum
• B.(ii) Cyanotic with Admixure lesion :
1.TGA
2.Uni-ventricular heart
3.Truncus arteriosus
What is the Target goal to
approach CHD
• Diagnosis
• Anatomical characteristics of the disease.
• Physiological assessment.
• Hemodynamic burden of the disease.
• Medical Treatment & counseling to
Pt/Parent.
• Operability &/or Intervention.
Approach to CHD
• Despite the availability of advanced imaging

technologies, a through “history & physical
examination is the core of evaluating
children with suspected heart disease”.
Ref: Moss and Adams’ Heart Disease in Infants, Children, and Young Adult. 8 th ed, 2013
Approach to CHD
• History
• Physical examination
• Investigation
• Management.
Cardiovascular symptoms
• Breathlessness
• Tiredness & Lethergy
• Slow feeding, Poor weight gain
• Pallor or Cyanosis
• Palpitation.
Ref: Macleod’s clinical examination

General inspection
Question to consider include

• Does the child look ill or undersized ?
• Is the breathing rate increased ?
• Is there grunting or wheezing ?
• Is the child blue around the lips or the
extremities ?
• Is central cyanosis present?
• Is there flushing of the face, sweating or
clubbing of the fingers & toes.
Approach to CHD
Appearance / Look
• Presence of characteristic facial or somatic

features of an underlying syndrome may be a
strong clue to the type of heart disease at
any age, e.g.,
• Williums ,Noonan, Down, Charge, Ellis-van
Cleveld, Holt-Oram, Leopard, Turner, Alagille.
Ref: BRAUNWALD’S Heart Disease, 10th Ed (2015), P-1399

Approach to CHD
• Physical examination :
• ►General Examination.
• ►Cardiovascular Examination.
• ►Respiratory System Examination.
• ►Abdominal Examination.
Approach to CHD
►Respiratory System Examination.
♦ Count the rate of respiration.
♦ Auscultate both lung field : bilateral
crepitation in important sign of left heart
failure.
Note :Unlike adults where crepitation is
commonly basal, but in neonates & infants it is
diffusely heard & more often associated with
rhonchi.
Ref: Clinical Diagnosis of Congenital Heart Disease. Editors: M Satpathy,BR Mishra,2 nd ed,p21
Approach to CHD
►Abdominal Examination.
♦ Normally liver is palpable (2-3 cm below
costal margin) at midclavicular line upto
4-5 yrs of age, after that it remains
palpable 1 cm till late childhood.
►If liver is palpable further downward it is
enlarged.
Note : Infants with enlarged liver, if irritable,
dyspneic, it indicate presence of CHF.
Approach to CHD
♦ If Live is midline & symmetrically palpable,

it indicates cardiac malposition &
underlying complex heart disease.
Approach to CHD
♦ On percussion tympanic resonance due to

gastric air on left side & liver dullness on right
side indicate situs solitus.
Approach to CHD
♦ Spleen is not normally palpable.

If spleen is palpable in a pt having a cardiac
murmur with unexplained fever it indicates
possibility of infective endocarditis.
Approach to CHD
►Investigations :
♦ ECG, CXR & Echocardiography (with Dopple
study) are part of clinical examination.
♦ Echo is diagnostic test for almost all CHD
♦ Cardiac Catheterization, MRI, CT-cardiac
angiography are needed for selected cases.
Maternal Infections During Pregnancy:
Associated with CHD
• Rubella : PDA, Pulmonary artery branch

stenosis, Congenital Rubella Syndrome.
• Mumps : Endocardial fibroelastosis.
• Coxsackie virus (in late pregnancy) :
Myocarditis.
• German measles : PDA and PS.
Maternal Diseases
Predispose the Child for certain type of CHD
• Diabetes : TGA, VSD.

• SLE : Complete Heart Block (CHB).
• Phenylketonuria : VSD, ASD, PDA.
Maternal Drug Intake
(Before &/or during early Pregnancy)
Predispose the Child for particular defects with CHD
• Alcohol: (Fetal alcohol syndrome)- VSD, TGA,

PDA.
• Trimethadione : TGA, TOF.
• Amphetamine : VSD.
• Phenytoin : Pulmonary Stenosis (PS), AS,
Coarctation of Aorta (CoA), PDA.
• Lithium : Ebstein’s anomaly.
Must have idea about –
Position of Body organs.

Visceral situs or sidedness
This concept applies to structures/organ
systems that are not bilaterally symmetric.
• Usually has three possible arrangements:

1) Situs solitus or normal.
2) Situs Inversus or mirror image of normal.
3) Situs Ambiguous or something else.
Ref : Echocardiography in Pediatric and Adult Congenital Heart Disease. Ed: Eidem et al 2010.

• 1) Situs solitus or normal:
Liver & Cecum to the right,
Stomach & Spleen to the Left.

• 2) Situs Inversus, or Inverted :
Liver & Cecum to the Left,
Stomach & Spleen to the Right.

• 2) Situs Ambiguous :
Any other patterns- frequently the liver is
bilateral and there is intestinal malformation;
gastric position is variable (the spleen, when
present , is always posterior to the stomach).
Cardiac Orientation
This represents the orientation of the base to

apex axis of the heart: -
• 1) Levocardia : Base-to-apex axis is pointed
from upper right to lower left
• 2) Dextrocardia : Base-to-apex axis is pointed
from upper left to lower right.
• 3) Mesocardia : Base-to-apex axis is pointed
nearly directly from superior to inferior
& usually is in the midline.
Cardiac Position
This represents the gross position of “most” of

the heart relative to the midline: -
• 1) Levoposition : most of cardiac mass is to
the left of midline.
• 2) Dextroposition :. most of cardiac mass is
to the right of midline.
• 3) Mesoposition : the heart is evenly
distributed around the midline..
Implication of circulatory changes at Birth
• At birth the pressures in the Lt & Rt sided chambers
& arteries are more or less identical.
• The Rt & Lt ventricles have equal wall thickness.
• The Aorta & main pulmonary artery (MPA) are of
equal thickness.
• Hence, even if there is communication between the
two sides, there is no significant flow from the Lt to
Rt side or vice versa.
Ref: Bedside Approach in the Diagnosis of Congenital Heart Diseases. Editor- Rajendra Tandon. 1998.p-2
Pressures in the chambers & great vessels in
fetal life as compared to adults.
PVR= Pulmonary vascular resistance in units
Before Birth Adult
RA LA RA LA
10 mmHg 10 mmHg 0–6 5 – 12
mmHg mmHg
RV LV RV LV
60/10 60/10 25/ 0 – 6 120/ 0 –12
mmHg mmHg mmHg mmHg
PA PVR= AO PA AO
PVR
60/40 250u 60/40 = 1u 120/75
25/10
mmHg mmHg mmHg mmHg
Implication of Pressures & Resistances
• Normal vascular resistance =

• Systemic : 800 to 1600 dynes/sec/m2. or
10 to 20 Wood units (U).
• Pulmonary : 80 to 240 dynes/sec/m2. or
1 to 3 Wood units (U) .
• Pulmonary vascular resistance (PVR) drops to adult

levels within a period of 2 to 3 wks in normal
babies.
• In presence of a VSD or PDA, the fall in resistance is
slower & takes 6 to 10 wks in full term babies & 3 to
6 wks in preterm babies.
• The murmur of VSD or PDA is not heard at birth
since the pressure on the two sides are nearly
identical.
• With the fall in pulmonary vascular resistance, the
Lt-to-Rt shunt increases & the murmur becomes
audible in 3 to 4 days.
• Clinically, separation of VSD from PDA at this stage
is not possible, since both defects initially present
with an ejection systolic murmur.
• The murmurs gradually increase in intensity as the

resistance in the pulmonary circuit falls.
• By 6 to 10 wks or more when the resistance reaches
its lowest value, the maximum shunt becomes
apparent.
• The full term babies, therefore, develop congestive
failure from a volume overload due to Lt-to-Rt
shunt by 6 to 10 wks.
• Since PVR fall more rapidly in preterm babies,
congestive failure can appear earlier than six wks &
may occur as earlier 3 wks.
• The increased thickness of RV myocardium causes

poor distensibility of RV in diastole.
• The thickness of RV takes longer time to regress-
approximately 6 month or so.
• Hence the features of Lt-to-Rt shunt, in ASD,
becomes obvious by 6 months or more & the
clinical diagnosis of the defect is generally possible
beyond 6 months.
• The high PVR & increased thickness of RV in

neonates & infants causes decreased Lt-to-Rt shunt,
so recognition of ASD,VSD & PDA at birth may not
be possible clinically.
• But, identification of ASD, VSD, PDA is possible by
Echocardiography
• On the other hand, obstructive lesions like aortic

stenosis or pulmonic stenosis & regurgitant lesions
like mitral or tricuspid regurgitation are operative
at birth.
• So, presence of murmur at birth would suggest that
the newborn has an obstructive lesion or a
regurgitant lesion.
• So, the significance of knowing when the murmur in

a child become audible is important since it can
easily separate obstructive & regurgitant lesions
from those resulting in a Lt-to-Rt shunt.
Age Periods
• Infancy ----- ------------ First year of life.

Neonatal period--- First month of life.
• Childhood -------------- 1 to 18 yrs.
Pre-school child -- 1 to 4 yrs,
(Considered as Toddler)
School child ------- 5 to 18yrs.

Physical Examination
Growth Measurements
• Growth Measurement :
Weight.
Height .
Head size.
Note:- Record measurements on centile charts &

evaluate whether height & weight are within
normal range or growth failure for age & sex.
Weight (growth measurement)
• An Average infant should gain in wt :
• In the early weeks, approx 30gm/day after
the 10th day.
• At 6 weeks expected gain in wt would be
almost 1kg.
• By 5mon, the birth wt should have doubled.
• By a year it should have trebled.

Weight
• For the next few yrs the expected wt in kgs

can be estimated from the formula :
Age in (years + 4 ) × 2.

Approach to CHD
• Significant heart disease is usually associated

with failure to thrive.

Respiratory Rates as per age
Age Respiratory rate

(per minute)
Newborn 40 - 60
Infants Up to 30
1 – 4 years 24 - 26
Adolescents 18 - 20
• Note :- any recession when breathing.
General examination
Respiration
• Breathing rate- best counted by observing

movements of the abdomen.

Approach to CHD
:Breathing pattern :
►Unlabored (“happy”) tachypnnea often

accompanies cyanotic heart disease.
►Increased work of breathing & sometimes
grunting are associated with left-sided
obstructive lesions or respiratory illness.
Approach to CHD
:Breathing pattern :
►Grunting with closure of the glottis provides

positive end-expiratory pressure & is
seen in infants who have pulmonary edema.
►Parents may also observe intercostal or
subcostal retractions when the child is
undressed.
Approach to CHD
H/O Exertion
• For Infant physical exertion is eating.

• For Adolescent & Adult it is walking
Approach to CHD
• Infant’s primary physical exertion is eating.
• Details feeding history should be obtained.
Approach to CHD
Normal Feeding pattern of Infants
• Most newborns feed –

2 to 3 oz every 2 to 3 hours.
• Generally, normal infants should be able to
complete their feeding in <30 minutes.
Approach to CHD
Feeding pattern of infants in Heart Failure
• A longer time to finish each feeding,

• Low volume consumed,
• Excessive diaphoresis, &
• Increased work of breathing during feeding
are signs of heart failure or poor cardiac
output.
Approach to CHD
Feeding pattern of infants in Heart Failure
• Children with congestive heart failure take

breaks during a feeding because of rapid
breathing or to fall asleep during feeding
only to awaken after a few minutes & feed a
small amount again.
Approach to CHD
: Diaphoresis :
►This can occur during feeding or during sleep.

►Diaphoresis in this circumstance generally
indicates activation of the sympathetic nervous
system in pts who have low cardiac output.
Approach to CHD
:Palpitation :
► Common complaint in older children
► Evaluate the circumstances in which they
occur (e.g., rest vs. exercise),
►The frequency & duration of the complaint.
►Associated symptoms such as fatigue,
shortness of breath, or chest pain, pallor,
diaphoresis.
Approach to CHD
:Chest Pain :
► Common complaint in adolescents & is generally

noncardiac in nature.
►Exercise-induced chest pain may be found in
significant left ventricular hypertrophy,
congenital coronary artery abnormalities, or
coronary abnormalities associated with
Kawasaki syndrome or can be due to noncardiac
condition such as exercise-induced
bronchospasm.
Approach to CHD
Cyanosis
• Central Cyanosis can be difficult to diagnose

clinically when mild but should be actively
excluded by oximetry in any pt with
suspected CHD.

Approach to CHD
:Pulse Oximetry Screening :
►Spot check of pulse oximeter saturation be
performed in every newborn after 24
hours of life .
► With 95% accepted as lower limit of normal.
►If a new born fails to maintain oxygen
saturation >95%, further screening would
be planned.
Approach to CHD
:Pulse Oximetry Screening :
►True cyanosis requires desaturation of 5 g%

of hemoglobin & is difficult to detect unless
the arterial saturation is ≤ 85% in a child with
normal hemoglobin levels .
Approach to CHD
Cyanosis
• Central cyanosis , which reflects true arterial
desaturation, is characterized by blueness of
the tongue & oral mucosa.
• Central cyanosis is most likely related to
cardiac or respiratory disease.
Approach to CHD
Cyanosis
• Central cyanosis can be due to-
► Rt-to-Lt Shunt,
► Pulmonary venous unsaturation due to
lung disease
Approach to CHD
Cyanosis : Pulmonary / Cardiac
• Pulmonary venous unsaturation can be
separated from cyanosis due to Rt-to-Lt
shunt by giving oxygen to the patient.
► Cyanosis due to pulmonary venous
unsaturation tend to disappear on giving
oxygen.
► Cyanosis due to a Rt-to-Lt shunt is
unaffected
Approach to CHD
Cyanosis : Uniform / Differential.
• Cyanosis can be (i) Uniform or (ii) Differential.

• (i) Uniform cyanosis means that the Rt-to-Lt
shunt is taking place at the atrial,
ventricular or the ascending aorta level.
• (ii) Differential cyanosis indicates that there is
a Rt-to-Lt shunt through the Patent
ductus arteriosus (PDA).
Approach to CHD
Cyanosis
• In Differential cyanosis there is Rt-to-Lt

shunt through the PDA.
• Blood flow from PA to PDA to Descending
AO.
• It does not reach the right arm & usually the
Lt arm as well as they receive blood from the
Lt ventricle.
Approach to CHD
Cyanosis
• Differential cyanosis : Two types -

(a) Pink fingers with Blue toes .
(b) Blue fingers but Pink Toes (Reversed
differential cyanosis).
Approach to CHD
Differential Cyanosis
(a) Pink fingers with Blue toes :

► indicating the great arteries are
normally connected to the respective
ventricles.
► occurs only if there is severe pulmonary
arterial hypertension.
Approach to CHD
(b) Blue fingers but Pink Toes (Reversed

differential cyanosis) :
the diagnosis is Complete Transposition
of Great Arteries (TGA), since the
Pulmonary artery in TGA carries more
oxygenated blood than the aorta
connected to Right ventricle.
Approach to CHD
• Note : the presence or absence of coarctation

of the aorta does not play any role
in differential cyanosis
Approach to CHD
Cyanosis
• Important to distinguish between

constant cyanosis & episodic cyanosis.
Ѫ In most forms of cyanotic congenital heart
disease , cyanosis is constant.
Approach to CHD
Constant Cyanosis
• Should suggest the presence of -

► Congenital heart disease with hypoxemia
related to transposition physiology,
► Inadequate pulmonary blood flow, or
► Intracardiac mixing
Approach to CHD
Episodic Cyanosis
• Due to hypoxemia related to hypercyanotic
episodes from TOF physiology.
• Occur in TOF, in some pts with DORV, or in
pts who have subpulmonic stenosis
associated with a univentricular circulation.
Note :
• Clubbing may not be apparent before 6

months of age even when cyanosis has been
obvious since birth.

Approach to CHD
Palpation
♣ Palpate for –
►Pulses,
► Apex beat,
► Thrill.
Pulses
• The average resting pulse rate ranges from

about 120/min in infants to 80/min in
older children.
• Palpate for *Rate, *Rhythm, *Quality,
*Amplitude.
• Evaluate Radio-Femoral delay.

Definition of Tachycardia & Bradycardia in
Pediatric age group
Age Tachycardia Bradycardia
Newborn >160 <100

Infants >120 <90
Toddler & >100 <60
Children
Pulses
• Examination of upper & lower limb

peripheral pulses is important.
• Delay, absence, or reduction of a pulse is
important clue to the presence of arterial
obstruction & its site.

Pulses
• The Lt brachial pulse is often compromised

by surgery for coarctation, & blood pressure
should not be measured in only the Lt arm.
• Similarly, other palliative procedures
(Blalock-Taussig-Thomas shunt, Interposition
grafts) may affect either or both upper limb
pulses.

Pulses
• Just as in acquired disease, pulse volume &

character also provide important information
regarding the severity of obstructive or
regurgitant left-sided heart disease

Pulses : Characters
• A low volume pulse (usually with a narrow

pulse pressure) reflects low cardiac output.
• Pulsus alternans signifies severe systemic
ventricular dysfunction.
• Pulsus paradoxus points to cardiac
tamponade.

Arterial Blood Pressure
Methods :
• Flush Methods : useful in small infants.
• Palpatory method : for small children &
infants. The pulse can be palpated to obtain
the systolic blood pressure.
• Auscultatory methods : usually easy in
children over the age of 3.
• Doppler ultrasonic method.
Ref: Hutchison’s Clinical Methods
Arterial Blood Pressure
Average BP readings (mmHg) in
Infants & Children.
• Newborn --- 65/45.
• At 1 year ---- 75/50.
• At 4 years --- 85/60.
• At 8 years --- 95/65.
• At 10 years --- 100/70.
Ref: Hutchison’s Clinical Methods

Normal Blood Pressure (in mm Hg)
Age Systolic Diastolic

(mmHg) (mmHg)
Neonates 60-70 20-60
Infants 87-105 53-65
Toddler 95-105 53-65
Children 97-112 57-71
Adolescents 112-128 66-80
Palpation: Apex Beat.
• Evaluate Orientation of Cardiac Apex :

►Levocardia : - Apex beat on the Left side
of mid-Line.
► Dextrocardia : - Apex beat on the Right
side of the mid-Line.
► Mesocardia : - Apex beat on the mid-Line
Palpation: Apex Beat.
• Up to age of 3 the apex beat is usually
palpable in the 4th ICS just outside the
mammary line.
• Thereafter it is within the mammary line in
the 5th or 6th ICS.
• Apex may not be palpable in infants & young
children unless the heart is enlarged.

Palpation
• Palpable S2 in pulmonary area suggests

increased pulmonary arterial pressure.

Palpation : Thrills
• Systolic thrill (1)
► At lower Lt sternal edge suggests
Ventricular Septal Defect (VSD).
► At the apex mitral incompetence,
► At the upper Rt sternal edge
aortic stenosis.

Palpation: Thrills
• Systolic thrill (2)
► At the upper Lt sternal edge pulmonary
stenosis or PDA.
► In the suprasternal notch may
accompany aortic stenosis or less
often PS or coarctation aorta.

Auscultation
• The Rules of auscultation also follow those

developed for acquired heart disease.
• However, cardiac & vascular malposition
may significantly affect the appreciation of
heart sounds & murmurs.

Auscultation
• Heart sounds : S1, S2, S3.

• Presence of murmurs

Auscultation
Heart sounds :
• S1 : is single.
• S2 : is generally split, with widening of the
two components during inspiration &
narrowing during expiration. Splitting is
best heard at 2nd Lt ICS.
• S3 : at the apex is common in normal infants
& young children.
Auscultation
• The presence of S3 in pts with Lt-to-Rt shunt, is due

to rapid ventricular filling in early diastole &
indicates that the pulmonary blood flow > 1.5 :1 to
2:1.
Auscultation
Murmur :
• Find out presence of murmur.
• Note : Site, timing & quality, intensity,
propagation & variations with positional
change.

Auscultation
Murmur :
• Distinguish between
Innocent & Organic murmurs.
Note : most murmurs in children are innocent; they

are not accompanied by thrills & tend to disappear
more readily with changes of position.
Approach to CHD
:Murmurs :
► Commonly, murmurs detected early in
the neonatal period originate from
atrioventricular valve regurgitation
or semilunar valve stenosis.
Approach to CHD
:Murmurs :
► Children with significant Lt-to-Rt shunt
lesions typically are asymptomatic until 4
weeks of age or later, when pulmonary
vascular resistance decreases to near
adult levels & pulmonary overcirculation
ensures.
► This may also be a first time a murmur is
heard.
Innocent murmurs (1)
• Several varieties:
• 1) Still’s murmur : a musical vibratory low-
pitched systolic murmur, best heard at the
mid-or lower left sternal edge.
• 2) Basal ejection systolic murmur : that is
high pitched & blowing at the upper sternal
border (left or right). This usually
disappears when the patient is upright.
• 3) Carotid Bruit : a short high-pitched
blowing systolic murmur heard in the neck
& confined to early systole.
• 4) Peripheral pulmonary artery branch
stenosis murmur : a blowing high pitched
ejection systolic murmur audible in the
early months of life in the pulmonary area &
over the back.

• 5) Venous hum : in the sitting or erect

position it is a low-pitched humming
systolic/diastolic murmur in the
infraclavicular areas (right > left). It is usually
absent in the supine position & is
abolished by occluding the veins in the
neck.

Murmurs : associated with some CHD
Lesion Nature of murmur Maximum site Other features
Ventricular Septal Harsh. Lower left sternal Mid-diastolic mitral
Defect (VSD). Pansystolic. edge. valve flow murmur.
Pulmonary stenosis Harsh. 2nd Lt intercostal Ejection click may
(PS). Ejection systolic. space (ICS). be present.
Aortic stenosis Harsh. 2nd Rt ICS or apex Ejection click.
(AS). Ejection systolic. during early Radiating to neck.
childhood.
Atrial Septal Defect Blowing. 2nd Lt ICS Parasternal lift.
(ASD) Ejection systole. Widely split S2.
Mid-diastolic
tricuspid valve flow
murmur.
Patent Ductus Continuous. 2nd Lt ICS & under Collapsing

Arteriosus (PDA) Lt Clavicle. peripheral pulses.

Auscultation
VSD
• An ejection systolic murmur indicates either a small

VSD or a very large VSD.
• Note : Small VSD acts as a stenotic opening whereas
large VSD by causing right ventricular
hypertension results in a Lt-to-Rt shunt only
during mid-systole & as such only an ejection
systolic murmur.
Auscultation
VSD
• The presence of pansystolic murmur indicates

small to medium sized VSD
Auscultation
• Cardiac & vascular malposition may

significantly affect the appreciation of heart
sounds & murmurs.

Auscultation
• For example, in TGA treated by an atrial

switch procedure, the aorta remains anterior
to the pulmonary artery. Consequently the
aortic component of the second sound can
be exceptionally loud, & the pulmonary
component may be virtually inaudible.

Auscultation
• When a valved conduit is present between
the RV & pulmonary artery, the pulmonary
closure sound may be extremely loud even
though pulmonary artery diastolic pressure is
low. This is because the conduit is frequently
adherent to the chest wall & assists in
transmission of sound to a stethoscope
placed close to it.

Auscultation
• Auscultation over the entire anterior &
posterior aspects of the chest wall is
important.
• The continuous murmurs of aorto-aortic
collateral arteries in coarctation may be
audible only between the shoulder blades
posteriorly.

Auscultation
• The presence of a localized distal pulmonary

artery stenosis or the presence of an
aortopulmonary collateral artery may be
detected only in a localized area of the chest
wall.

Hemodynamic interpretation of
Continuous Murmur
• Continuous murmur indicates a difference in
pressure both during systole & diastole.
• Hence if the murmur is due to blood flow
from aorta or its branches to the pulmonary
circulation it indicates that the pulmonary
artery pressure is normal or that it is only
slightly elevated & excludes significant
pulmonary arterial hypertension.
D/D of continuous murmur in an
Acyanotic patient
• Patent ductus arteriosus.
• Sinus of valsalva fistula to RA or RV.
• Aortopulmonary septal defect (A-P window).
• Coronary arteriovenous fistula.
• Anomalous left coronary artery from pulmonary artery.
• Systemic arteriovenous fistula.
• Coarctation of the Aorta.
• Peripheral pulmonic stenosis.
• Mitral stenosis with a small atrial septal defect.
• Venous hum.
Causes of continuous murmur in pts with
cyanotic congenital heart disease
• Bronchial collaterals in anomalies of TOF
• Patent ductus arteriosus (PDA) in pts with
TOF.
• Total anomalous pulmonary venous
connection (TAPVC).
• Pulmonary arteriovenous fistula.
• Surgically created shunts
Auscultation
Limitation
• One must realize the limitations of auscultation.

• Auscultation is a personal observation & as such
“soft data”, that is , unreliable (? ‼) .
• The accuracy of auscultation is heavily
dependent on clinical practice.
Does the child have heart disease ?
Nadas’ Criteria
Major Minor
1) Systolic murmur grade –III or more 1) Systolic murmur less than grade –III in
in intensity. intensity.
2) Diastolic murmur. 2) Abnormal second sound.
3) Cyanosis. 3) Abnormal electrocardiogram.
4) Congestive heart failure. 4) Abnormal thoracic roentgenogram.
5) Abnormal blood pressure.
Presence of one major or two minor criteria suggests the presence of heart
diaease.
It is necessary to emphasize that the criteria are general guidelines &

physicians have a right to decide presence or absence of heart disease
even if the criteria are not satisfied on the basis of knowledge, expertise
& evidence.
Clinical Impression
• Acyanotic or Cyanotic .
• Congestive heart failure.
• Other features e.g., RTI, Growth failure
target
• Complete & accurate diagnosis.
• Pulmonary artery pressure.
• Surgery timing total correction or palliative
• Followup
Hyperkinetic PAH & Obstructive PAH
• When the Pulmonary Arterial Hypertension (PAH) is

due to an increase in pulmonary blood flow it is
called Hyperkinetic PAH.
• On the other hand, when PAH is due to an increase
in pulmonary vascular resistance (PVR), it is called
obstructive PAH.
Hyperkinetic PAH & Obstructive PAH
• Hyperkinetic PAH is characterized by features of a

large Lt-to-Rt shunt combined with features
of PAH.
• Whereas obstructive PAH is characterized by the
findings of PAH associated with a small Lt-to-Rt
shunt.
Anomalies with Cyanosis &
Increased Pulmonary Blood Flow
• 1) Complete Transposition of great arteries.
• 2) Double outlet right ventricle (DORV) without
pulmonic stenosis (PS).
• 3) Tricuspid Atresia with increased pulmonary
blood flow (large VSD & no PS).
• 4) Persistent Truncus Arteriosus (PTA).
• 5) Single Ventricle without PS.
• 6) Total anomalous Pulmonary venous connection
(TAPVC).
Situs Solitus with
Cardiac Dextroversion
• Is associated with CHD in more than 90% of
cases.
• Up to 80% have a congenitally corrected
transposition with a high incidence of
associated VSD, Pulmonary stenosis, &
Tricuspid atresia.

Situs Inversus with
Cardiac Dextrocardia
• Carries a low incidence of CHD

Situs Inversus with
Levocardia
• Is virtually always associated with severe

CHD

Investigations
• Echocardiography – 2D,M-mode, Doppler.

• X – ray chest.
• ECG .
• Others depending upon clinical condition,
e.g., CT angiogram of heart & great vessels,
Cardiovascular MRI, Cardiac catheterization.
ECG
• Incomplete RBBB often indicates :
►Right ventricular hypertrophy secondary
to pressure overload, e,g,. Pulmonary
hypertension or pulmonary stenosis.
► RV Volume overload e.g,. ASD .
Rt ventricular volume overload is likely when
the r’ in V1 is < 7mm.

ECG
• Very wide QRS complexes should be seen as

possible manifestations of dilated &
dysfunctional ventricles, most specifically in
pts with a combination of repaired TOF,
complete RBBB & severe pulmonary
regurgitation.

ECG
• The ECG may be uninterpretable in pts with

abnormal cardiac or visceral situs unless it is
clear where the leads were placed.

ECG
• First-degree block is frequently seen in pt

with AV septals, cc-TGA, & Ebstein anomaly.
• Complete heart block most often occurs in
pts with cc-TGA.

ECG
• Deep q waves in the left chest leads can be

caused by Lt ventricular volume overload in a
young person with aortic or mitral
regurgitation.
• Pathologic Q waves can be evidence of the
anomalous origin of the left coronary from
the pulmonary artery.

Chest Radiograph
• The major interest in evaluating the

thoracic roentgenogram is to assess :
1) The Heart size & shape.
2) The Pulmonary vasculature &
3) Determination of the Situs.
Chest Radiograph
Cardiomegaly
• Decide the shape of Cardiomegaly :

Is that of Right or Left ventricular
enlargement or hypertrophy
Chest Radiograph
Cardiomegaly
• Rt ventricular hypertrophy tends to give a

somewhat smaller arc of the cardiac apex
which appears above the Lt hemidiaphragm
as an upturned apex.
• The classical example is that of Fallot’s
tetralogy (TOF)
Chest Radiograph
Cardiomegaly
• Left ventricular hypertrophy gives the

appearance of a broader apex which tends to
dip below the left hemidiaphragm as in
Tricuspid atresia
Chest Radiograph
Cardiomegaly
• Cardiomegaly indicates :
► Large shunt,
► Valvar regurgitation,
► Failing ventricle(s),
Note : uncommonly large cardiac silhouette
can be due to a dilated right or left atrium.
Chest Radiograph
Great arteries : Pulmonary Artery segment.
• Prominence of Main Pulmonary artery

segment is seen in :
► Lt-to-Rt Shunts,
► Valvar pulmonic stenosis &
► Pulmonary arterial hypertension.
Chest Radiograph
Great arteries : Pulmonary Artery segment.
• Absence of of Main Pulmonary artery

segment indicates :
► Infundibular pulmonic stenosis as in TOF
► The great vessels are abnormally related as in
Transposition of the Great arteries where
the pulmonary artery is posteriorly placed.
► Dysplastic pulmonary valve e,g,. Noonan’s
syndrome.
Chest Radiograph
Great arteries : Aorta
• Large Ascending Aorta indicates:

► Valvar Aortic Stenosis (post-stenotic dilatation)
► Coarctation of the Aorta ( due to
hypertension).
► Larger than normal aortic blood flow
e.g., PDA, VSD.
Note: Fallot’s physiology, the aorta is relatively large
in size
Chest Radiograph
Evaluation of the Pulmonary Vasculature
• Useful in classifying pts into :

► Lt-to-Rt shunts.
► Obstructive lesions &
► Cyanotic congenital heart diseases with
increased or decreased pulmonary
blood flow.
Chest Radiograph
• Basically on the bedside, a general impression is used

by “eyeballing” the chest X-ray :
► Does the vasculature look normal ?
► Does it fit with a shunt ?
► Does it suggest diminished blood flow ?
► Does it fit with Eisenmenger physiology ?
► Does it suggest pulmonary venous
hypertension ?
Chest Radiograph
• Best way to approach evaluation of vasculature is

to divide the Right hemithorax by two imaginary
vertical lines into :
► the inner third ,
► the middle third , &
► the outer third .
Note : Normally, middle third has some vascular
markings & the outer third has a very few but
definitely visible vessels
Chest Radiograph
• With a Lt-to-Rt shunt, the middle third shows fairly

large vessels with some “end on vessels”.
• In ischemic lung fields, the middle third does not
have significantly recognizable vessels.
• In Eisenmenger physiology, hilar area suggests a
large shunt but if the inner one-third is covered the
rest of the lung does not show vascular markings .
Chest Radiograph
• The pulmonary venous hypertension is

characteristically recognized by prominent upper
lobe vessels, smaller basal vessels, Kerley’s lines & a
generalized haze.
Chest Radiograph
Criteria for Shunt Vascularity
• (1) uniformly distributed vascular markings
with absence of the normal lower lobe
vascular predominance,
• (2) right descending pulmonary artery
diameter that exceeds 17mm,
• (3) a pulmonary artery branch that is larger
than its accompanying bronchus (best
noted in the Rt para-hilar area.)
Chest Radiograph
Criteria for Shunt Vascularity
• Prominent Vascularity is apparent only if the
pulmonary-to-systemic flow ratio is >1.5: 1.
• As a rule, overt cardiac enlargement usually
implies a shunt >2.5 : 1 .
• Note : Anemia, pregnancy, thyrotoxicosis, &
a pulmonary AV fistula may mimic shunt
vascularity.

ASD
Cardiomegaly from RA & RV
enlargement, dilated central pul
arteries with pulmonary plethora.
Small aortic knucle (reflecting chr
low-cardiac output state.)
VSD
LV dilation with
pulmonary plethora.
PDA
Cardiomegaly with
increased pulmonary vascular
marking
Normal-sized, boot-shaped heart (coeur en sabot)
with prominence of RV & concavity in the region of
underdeveloped RVOT & MPA. The pulmonary
TOF
vascular markings are typically diminished, & the
ascending aorta often prominent.
Chest Radiograph
Ebstein’s anomaly
• Cardiomegaly, prominent right atrium, narrow base

& markedly oligemic lung fields.
Chest Radiograph
Complete Transposition of Great Arteries
• Cardiomegaly with “egg on side” appearance,

narrow base & pulmonary plethora with intact IVS.
Chest Radiograph
Total Anomalous pulmonary venous Connection (TAPVC)
Mild cardiomegaly with figure of ‘8’ configuration &

pulmonary plethora. The upper part of ‘8’ is formed
by the anomalous veins draining into left ascending
vein which joins the innominate vein. Pulmonary
plethora is essential.
Chest Radiograph
Corrected Transposition of great arteries.
The upper left border is formed by the ascending

aorta.
Chest Radiograph
TOF with absent pulmonary valve .
Large left pulmonary artery in addition to the classical

features of TOF. Distal lung fields are oligemic.
Chest Radiograph
Cyanotic congenital heart disease.
Thymus regresses very fast in cyanotic patients so that

even in the first few weeks of life it is not
identifiable on the roentgenogram.
Investigation
• “Echocardiography : Doppler Echo.”

• Comprehensive, details Echo.
• Conclusive diagnosis in almost all case
Knee- chest position
Squatting position
Eisenmenger syndrome
• Defined as non-reactive pulmonary arterial

hypertension resuling in a right to left (Rt-to-
Lt) shunt at the atrial, ventricular or the great
arterial level.
• The pulmonary arterial hypertension is due

to the development of pulmonary vascular
obstructive disease which is an acquired
complication in patients with congenital
heart diseases who have increased
pulmonary blood flow.
• All the pts of Lt-to-Rt shunt as well as

cyanotic pts with increased pulmonary blood
flow are potentially candidates for the
development of Eisenmenger physiology.
• The physical findings in the pre-tricuspid &

post-tricuspid Rt-to-Lt shunts differ.
• Pt with pre-tricuspid shunt ,e.g., ASD, will
have Cardiomegaly.
• Pt with post-tricuspid shunt ,e.g., VSD,PDA,
do not have cardiomegaly.
Good Clinicians
Sixth Sense ‼
• It is not the sixth sense which makes him a good
clinician.
• The interpretation should be dependent on the
observation.
• An inference not consistent with the observations
indicated lack of theoretical knowledge as
well as absence of logical thinking
Management
• As per diagnosis of the Disease.

Wisdom - Knowledge
• Wisdom implies the timely and rightful
application of Knowledge.
• Knowledge may even be a pitfall or an
encumbrance unless you learn to use it justly.
• To gain wisdom is of all task of life, the most
difficult, and it is certainly no less arduous in
nursing than in many of life’s other activities.
--- Monningham.
Thank you

Approach To A Patient With Congenital Heart Disease

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Approach To A Patient With Congenital Heart Disease

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Approach to a Patient with

Congenital Heart Disease

• Congenital cardiovascular disease is defined

Ref: BRAUNWALD’S Heart Disease, 8th Ed, P-1561

• Congenital cardiovascular malformations

Ref: BRAUNWALD’S Heart Disease, 8th Ed, P-1561

• (A) Acyanotic group.

• Despite the availability of advanced imaging

Ref: Macleod’s clinical examination

Question to consider include

• Presence of characteristic facial or somatic

Ref: BRAUNWALD’S Heart Disease, 10th Ed (2015), P-1399

♦ If Live is midline & symmetrically palpable,

♦ On percussion tympanic resonance due to

♦ Spleen is not normally palpable.

• Rubella : PDA, Pulmonary artery branch

• Diabetes : TGA, VSD.

• Alcohol: (Fetal alcohol syndrome)- VSD, TGA,

Position of Body organs.

• Usually has three possible arrangements:

Position of Body organs.

Position of Body organs.

Position of Body organs.

This represents the orientation of the base to

This represents the gross position of “most” of

• Normal vascular resistance =

• Pulmonary vascular resistance (PVR) drops to adult

• The murmurs gradually increase in intensity as the

• The increased thickness of RV myocardium causes

• The high PVR & increased thickness of RV in

• On the other hand, obstructive lesions like aortic

• So, the significance of knowing when the murmur in

• Infancy ----- ------------ First year of life.

Ref: Macleod’s clinical examination

Note:- Record measurements on centile charts &

Ref: Macleod’s clinical examination

• For the next few yrs the expected wt in kgs

Ref: Macleod’s clinical examination

• Significant heart disease is usually associated

Ref: Macleod’s clinical examination

Age Respiratory rate

• Note :- any recession when breathing.

• Breathing rate- best counted by observing

Ref: Macleod’s clinical examination

►Unlabored (“happy”) tachypnnea often

►Grunting with closure of the glottis provides

• For Infant physical exertion is eating.

• Infant’s primary physical exertion is eating.

• Details feeding history should be obtained.

• Most newborns feed –

• A longer time to finish each feeding,

• Children with congestive heart failure take

►This can occur during feeding or during sleep.

► Common complaint in adolescents & is generally

• Central Cyanosis can be difficult to diagnose

Ref: BRAUNWALD’S Heart Disease, 10th Ed (2015), P-1399

►True cyanosis requires desaturation of 5 g%

• Cyanosis can be (i) Uniform or (ii) Differential.

• In Differential cyanosis there is Rt-to-Lt

• Differential cyanosis : Two types -

(a) Pink fingers with Blue toes :

(b) Blue fingers but Pink Toes (Reversed

• Note : the presence or absence of coarctation