This document discusses pulmonary arterial hypertension (PAH) in congenital heart disease (CHD). It begins with an overview of normal pulmonary circulation and its development from embryology through adulthood. It then covers the pathophysiology, definition, and classification of PAH. Specific topics discussed in more detail include PAH in single ventricle physiology, Eisenmenger syndrome, and PAH associated with decreased pulmonary blood flow. The natural history, clinical features, complications, signs and symptoms, physical exam findings, diagnostic workup, and ECG patterns of PAH in CHD are also summarized.

1. PULMONARY ARTERIAL
HYPERTENSION IN CHD
Dr. K.V.Siva Krishna

2. Emryology

4. Normal pulmonary circulation
• High flow, low pressure and low resistance circulation
• Unique double arterial blood supply
• Pulmonary arteries:
– Elastic: conducting vessel, ≥ 500 μm, highly distensible
– Muscular: 100-500 μm, no elastin, non distensible
– Arterioles: ≤ 100 μm, thin intima and single elastic lamina
• Bronchial arteries: nutrition to the airways

5. Development of Pulmonary
circulation
• It was observed from the postmortem
arteriograms that the vessels are
prominent in the newborn, whereas in the
adult they are obscured by a dense
background haze produced by the addition
of many small intra-acinar arteries not
present at birth.

6. • Foetus : With increasing age, muscle is
observed in arteries located more
peripherally within the acinus.
• At first nonmuscular arteries become
partially muscular, and later they become
fully muscularized.

7. • At birth: The muscularized arteries are
thick walled, but within a few days, the
smallest muscular arteries dilate, and their
walls thin to adult levels.

8. • @4 months of age: This process has
included the largest muscular pulmonary
arteries and is complete.
• Arteries grow both in number and size,
and they grow most rapidly in infancy.
• The latter process contributes >90% of the
smaller or intra-acinar pulmonary arterial
vessels present in the older child and adult

9. • Development of new arteries and
arterioles occur.
• Although alveoli also proliferate, the ratio
of alveoli to arteries actually decreases
from the newborn value of 20:1 to the
value of 8:1, which is achieved first in early
childhood and persists.

10. CHANGES AFTER BIRTH
• At birth PBF increases 8-10 times with a fall in
pulmonary pressure to a level less than 50% of
systemic pressure
• Pulmonary vascular resistance may be as high
as 8 to 10 Wood units immediately after birth, it
normally falls rapidly throughout the first week
of life.
• By 6 to 8 weeks, pulmonary vascular
resistance usually has reached a normal adult
level of 1 to 3 Wood units

12. • PH is defined as a mean pulmonary artery
pressure >25 mm Hg at rest or >30 mm
Hg during exercise.
• The term PAH describes a group of PH
patients characterized haemodynamically
by the presence of pre-capillary PH,
defined by a pulmonary artery wedge
pressure (PAWP) ≤15 mmHg and a PVRI
>3 Wood units*m2 (WU).
Definition

13. • In Single Ventricle physiology
• PAH is defined as
• PVRI > 3WU
• Trans pulmonary gradient (TPG) > 6mmhg

14. PATHOPHYSIOLOGY of PAH
• Panvasculopathy predominantly affecting small PA
• Exact mechanism is unknown, abnormalities in pulmonary
artery endothelial & smooth muscle cells (PASMCs) with
varying degrees of
I. Vasoconstriction, [PASMCs, K+ &Ca2+
channels]
II. Vascular proliferation, [PASMCs, ECM syn.]
SERT ON PASMCs: apoptosis, proliferation
III. Thrombosis, and
IV. Inflammation
contribute to the development of pulmonary
hypertension

15. Pathophysiology of PAH

16. PAH in children
• Causes
• Pulmonary vascular obstructive disease related
to congenital heart disease (i.e., Eisenmenger
syndrome) develops after a period of decreased
pulmonary vascular resistance and increased
pulmonary flow.

18. • In some children who have anatomically large systemic-
to-pulmonary shunts (e.g., ventricular septal defect or
patent ductus arteriosus), the pulmonary vascular
obstructive disease occurs without there having been a
large left-to-right shunt or signs of congestive heart
failure, suggesting that the PAH may be idiopathic
PAH rather than PAH related to the congenital
heart disease.

19. • In pulmonary hypertension of the newborn
(PPHN), there may be a failure of the
normal decline in PVR after birth, which
may be triggered by many factors related
to neonatal stress

20. Eisenmenger Syndrome
Definition:
Pulmonary hypertension at or near
systemic level with reversed or
bidirectional shunt between the pulmonary
and systemic circulation at aorto-
pulmonary, ventricular or atrial level and
pulmonary vascular resistance above 800
dyne/cm-5 (10 Wood Units)
Paul Wood, Br Med J, 1958

21. Hemodynamically,
• Eisenmenger syndrome (ES) is defined as
an elevation of the pulmonary vascular
resistance to 12 Wood Units or to a
pulmonary-to-systemic resistance ratio
equal to or greater than 1.0

22. Eisenmenger Syndrome – A
progressive disease

23. MECHANISM
• Multifactorial
– Vasoconstriction
– Proliferative and obstructive remodelling of the
pulmonary vascular bed
– Inflammation and thrombosis
– Failure of endothelial cell apoptosis, intimal
proliferation, and irreversible PAH

25. • Stage I - Medial hypertrophy (reversible)
• Stage II - Cellular Intimal hyperplasia in a abnormally
muscular artery (reversible)
• Stage III - Lumen occlusion from intimal hyperplasia
of fibroelastic tissue (partially reversible)
• Stage IV - Arteriolar dilation and medial thinning
(irreversible)
• Stage V - Plexiform lesion, which is an angiomatoid
formation (terminal and irreversible)
• Stage VI - Fibrinoid/necrotizing arteritis (terminal and
irreversible
Heath-Edwards Classification –
pathology of PAH
Rever
sible
Irrev
ersi
ble

26. Lung biospy
Grade Peripheral arteries Medial
thickness(muscular
arteries)
Arterial
concerntration
Grade A Muscle extened
into peripheral
arteries
<1.5 times N
NormalGrade B ( mild ) Increased
extension
1.5 to 2 times
Grade B (severe) > 2 times N
Grade C Arterial
concerntration
and size
reduced

27. • A biopsy showing severe grade C and
grade III or greater changes in 20% of
vessels indicative of severe vascular
disease that is unlikely to regress
postoperatively.
• Severe grade B, or grade II changes in
any vessel may preclude a favorable result
from a Fontan procedure.

28. PRESSURE = FLOW X RESISTANCE
HYPERKINETIC PAH OBSTRUCTIVE PAH

30. • Age of the patient and complexity of lesion influence
development of ES.
• Significant biologic variability exists in the clinical
presentation and prognosis of underlying CHD
• BMPR2 mutations in patients with PAH in CHD.
• These data raise the possibility that the presence of
a genetic predisposition in some patients with CHD
may contribute to the observed biologic variability

31. • Pathologically similar to primary PAH
• Endothelial proliferation is polyclonal in ES while
monoclonal in IPAH
• Certain vascular patterns are different between
the two-
– Persistent foetal pattern in large PA with elastic fibres
being long and densely packed as in the aorta
– persistence of the foetal type of muscular artery
• Better appreciated with VSD & PDA than ASD

32. LESS WITH ES THAN IPAH

34. Vascular abnormalities associated with pulmonary
hypertension

36. NATURAL HISTORY
• Survival, as measured by age reached
• 30 years of age: 75%
• 40 years of age: 70%
• 55 years of age: 55%
• IPAH –NIH registry -1,3,5 yr survival -
68%,48%,38%
Diller, et al..Model of chronic adaptation: right ventricular
function in Eisenmenger syndrome European Heart
Journal Supplements 9

37. • Transplant free survival --
• 97% at 1 year, 89% at 2 years, and 77% at
3 years for patients with ES
• 77%, 69%, and 35%, respectively, for
patients with PPH

38. • Have a long symptom free time period
• Patients have adjusted to a lower exercise capacity since
childhood.
• Patients may even do well throughout adolescence and
early adulthood.
• Many become symptomatic during their 30s and gradually
develop complaints and complications, particularly
cyanosis, exercise intolerance, dyspnea upon exertion,
etc..
• Rhythm disturbances, particularly atrial fibrillation,
corresponds to clinical deterioration and right heart failure.
• According to historical data, patients with ES usually die
between 30 and 35 years.
• However, survival to late adulthood has been reported

39. Second Congenital Heart Disease Natural
History Study (1993)
• Demontrated that patients with ES can survive for
several decades following diagnosis.
• In this study, 54% of 98 unoperated patients with
ventricular septal defects and ES were alive 20 years
after diagnosis
Kidd L, Driscoll DJ, Gersony WM, Hayes CJ, Keane JF,
O’Fallon WM, Pieroni DR, Wolfe RR, Weidman WH.
Second natural history study of congenital heart
defects. Circulation 1993;87(suppl I):38-51.

40. WHY GOOD PROGNOSIS?
1. RV appears to adapt to the rise in pressure
through hypertrophy and preservation of a fetal-
like phenotype
2. Regression of the physiologic right ventricular
hypertrophy does not occur and the right-to-left
shunt serves as an excess flow valve
3. Ability of Eisenmenger patients to maintain their
systemic CO at the expense of cyanosis

42. Eisenmenger Syndrome
Underlying Basic Lesions
Type of lesion Somerville’98(n=132) Daliento
’98(182)
Ventricular Septal Defect 45 71
Atrial Septal Defect 6 21
Patent ductus arteriosus 12 36
Atrio ventricular septal defect 16 23
Truncus arteriosus 15 11
Single ventricle 13 9
Transposition of great
arteries
5 8
Others 20 9

43. WHY EARLY ES IN POSTTRICUSPID SHUNT
THAN ASD?
• POST TRICUSPID SHUNT (VSD/PDA)
• PVR never comes down to normal due to high
pressure flow from infancy
• Regression of medial hypertrophy of SMC &
RVH does not occur
• DVP PAH & reversal of shunt at an early age
• PRETRICUSPID SHUNTS( ASD)
• Direction of shunt is determined by the Right
ventricular compliance so no shunt occurs till
3 months
• PVR reaches normal by 3 mths
• PAH & ES occurs late in life especially in a
large ASD
• PAH in ASD believed to be acquired or
unrelated to the defect

44. PAH associated with heart Defects with Decreased
Pulmonary Blood Flow
• Condition like
PA with intact IVS
TOF
• Associated because of
 Hypoplasia of pulmonary arteries.
 Intra-acinar pulmonary arteries are small and few
in number.
 Alveolar development is impaired (mostly
reduction in alveolar number)
 ↑ Hematocrit resulting in in-situ thrombus.

45. Signs and Symptoms
1. Exertional dyspnea,
2. Lethargy, and
3. Fatigue,
In ability to
increase cardiac
output
1. Exertional chest pain (ie, angina),
2. Exertional syncope, and
3. Peripheral edema
The PH
progresses and
right ventricular
failure develops.

46. Right ventricular wall stress
Myocardial oxygen demand
Subendocardial hypoperfusion
Angina
Dynamic
compression of the
left main coronary
artery by an
enlarged
pulmonary artery(if
PA > 40mm)

47. CLINICAL FEATURES
COMPLICATION FREQUENCY
1. HAEMOPTYSIS 20%
2. PULMONARY THROMBOEMBOLISM 13%
3. STROKE 8%
4. CEREBRAL ABSCESS 4%
5.I.E 3%
SYMPTOM FREQUENCY
D.O.E 84%
INCREASED CYANOSIS 59%
HYPERVISCOSITY 39%
ANGINA 13%
SYNCOPE 10%
CHF 8%

48. Signs and Symptoms
• Passive hepatic congestion may cause
anorexia and abdominal pain in the right upper
quadrant.
• Less common symptoms of PH include cough,
hemoptysis, and hoarseness (ie, Ortner's
syndrome) left recurrent laryngeal nerve
compressed by a dilated main pulmonary
artery.
• Dizziness or syncope
• Lower extremity edema and eventually ascites
• Cyanosis of the lips and skin

49. CYANOSIS & CLUBBING in ES
• Cyanosis
• Most florid when the shunt was at ventricular level and least with a
patent ductus.
• VSD -no case was truly acyanotic even at rest,42% had gross
cyanosis
• PDA -60% of were acyanotic in the head and upper extremities. only
4% had gross cyanosis,differential cyanosis-50%
• Clubbing
• PDA -absent in 76% of the cases ,considerable in only 5%;
• VSD-Absent 3% and gross 36%.
• ASD-Intermediate

50. • Squatting - uncommon
• Relatively more frequent with ventricular septal
defect (15%) than with atrial septal defect(5%) or
patent ductus (3%)(p.Wood)

51. PHYSICAL FEATURES
• Pulse-small about twice as often with atrial
septal defect (88%) as with ventricular
septal defect (37%) or patent ductus (50%).
• When full or water-hammer in quality, atrial
septal defect was never present
• Bidirectional aorto-pulmonary shunts -water-
hammer pulses (12%)(p.wood)

52. • Jugular Venous Pressure: small dominant a
wave measuring about 3 mm. Hg 20 to
25% of cases of each type.
• Large v waves from tricuspid incompetence
in 5% of all cases

53. • RV impulse palpable in ASD(57%),rare with
VSD/PDA
• An impulse over the pulmonary -66% of cases in
each group.
• Right atrial gallop -38% of cases with interatrial
shunt, but in only 2 to 3% of the others
• Pulmonary ejection click -in about two-thirds of all
cases
• Functional pulmonary ejection murmur, usually of
moderate intensity and relatively short duration, -
80% of all cases, -loud in 25%
• Thrill - one-half of the loud murmurs,10% overall

54. S2
• ASD –wide fixed,never single
• VSD –single (55%),wide varying(12%)
• PDA –narrow/wide
varying(50%),single(6%)

55. • Auscultation of the heart may also reveal a
systolic ejection murmur and, in more
severe disease, a diastolic pulmonic
regurgitation murmur.
• The right sided murmurs and gallops are
augmented with inspiration

56. • Right ventricular failure results in systemic venous
hypertension.
• This can lead to findings such as elevated jugular venous
pressure, a right ventricular third heart sound, and a high-
pitched tricuspid regurgitant murmur accompanied by a
prominent V wave in the jugular venous pulse if tricuspid
regurgitation is present.
• In addition, hepatomegaly, a pulsatile liver, peripheral edema,
and ascites may exist.

57. Work up
Imaging tests are useful in
1. detection of PH ,
2. assessment of severity of PH,
3. categorization – the specific group the
patient belongs to,
4. prognostication ,
5. serial followup of patients( those who
receive PH specific therapies)

58. ECG
• RVH – tall R in v1 , R/S >1 , monophasic R or qR
• Right atrial abnormality – tall peaked P in lead 2
• RIGHT axis deviation
• Secondary ST- T Changes in V1- V4.
• RBBB – uncommon manifestation
• RVH ± RAD is present nearly 90% of patients.
• A normal ECG is reported in <5% cases.

59. RBBB
Extreme right axis deviation (+180 degrees)
S1 Q3 T3
T-wave inversions in V1-4 and lead III
Clockwise rotation with persistent S wave in V6

60. Right axis deviation.
T-wave inversions in V1-4 (extending to V5).
Clockwise rotation with persistent S wave in V6.

61. • The rhythm is usually sinus , AF occurs
rarely in patients with advanced RV
disease and RV dysfunction.
• The annual risk of SVT is roughly 3%.
• Ventricular arrhythmias are unusual.

62. CXR
• It is abnormal in nearly 90% patients.
• Enlarged MPA
• Right pulmonary artery diameter > 14mm
in women , >16 mm in men.
• Prunning – rapid tapering of peripheral
pulmonary arteries and increased
translucency of peripheral lung fields due
to hypovascularity.

63. A right interlobar pulmonary diameter of greater than 16 mm or a hilar-
to-thoracic ratio of greater than 0.44 is specific but not sensitive for the
diagnosis of pulmonary hypertension.

65. • Cardiomegaly with right atrial and right
ventricular enlargement usually present.
• Right atrial appendage dilatation results in
obliteration of retrosternal space in its
upper part.
• Retrosternal space obliteration beyond its
lower 1/3rd indicates RV dilatation.

66. CXR – clue to etiology…
• Pulmonary venous hypertension – dilated
upper lobe veins , perivascular cuffing ,
ground glass appearance of lung fields , kerly
B lines, thickened minor fissure.
• Shunt lesions – shunt at atrial and
ventricular level may results in inconspicuous
aorta , shunt at arterial level leads to dilated
ascending aorta.
• Lund parenchymal diseases – ILD , COPD.
• Chronic thromboembolic PH- focal
hypovascularity
• Congenital absence of a pulmonary artery.

68. • Lateral chest radiograph
shows filling of the
retrosternal airspace, a result
of right ventricular dilatation.
• The right ventricle is in
contact with more than one-
third of the distance from the
sternodiaphragmatic angle to
the point where the trachea
meets the sternum.

69. Echocardiographic Assessment
• Echocardiography with Doppler studies is the most
useful first line investigation in a patient presenting
with clinical features suggestive of pulmonary
hypertension.
• It facilitates:
1) Estimation of pulmonary artery systolic pressure to
determine if PH is present.
2) Assessment of cardiac cause of PH
3) Assessment of severity of RV dysfunction
4) Assessment of prognostic variables

70. • Pulmonary valve M-Mode
• According to Wyeman the following M mode
signs are useful in diagnosing PAH.
1. Diminished or absent a
2. Presence of mid-systolic closure or notching
3. Fluttering of the posterior pulmonic leaflet

71. M-mode of the pulmonary valve showing rectification of diastolic curve and lack of an atrial
contraction dip in pulmonary hypertension patients (A). The mid-systolic dip (arrows), which is a
specific (although low-sensitivity) sign of significant pulmonary hypertension, can be observed in
the lining of the pulmonary valve during the systolic phase (B).

72. • These findings generally manifest in
moderate to severe PH.
• In pts with PAH ,colour reversal in MPA is
a common finding.
• Early systolic forward flow along the lateral
wall with subsequent late systolic flow

73. Pulsed wave
Doppler in the
pulmonary artery
showing rapid early
systolic
acceleration and
mid-systolic
slowing.

74. Estimating Pulmonary Artery
Systolic Pressure
• Echocardiographic evaluation of pulmonary artery systolic
pressure (PASP) relies on the fact that PASP approximates
right ventricular systolic pressure (RVSP) in the absence of
right ventricular outflow obstruction.
• The most accurate echocardiographic method for estimating
(PASP) uses the simplified Bernoulli equation to obtain a
systolic trans-valvular pressure gradient.
• DPRV-RA = 4(VTR)2
• Where VTR is the velocity of the tricuspid regurgitant jet. This
figure is added to an estimate of right atrial pressure (RAP) to
produce an estimate of RVSP.
• PASP » RVSP = 4(VTR)2 + RAP

77. ESTIMATION OF RAP
• Qualitative Features that suggest elevated
RAP in ECHO –
1. RA enlargement
2. Persistent bowing of atrial septum toward
left atrium
3. Dilated coronary sinus
4. Dilated IVC and hepatic veins.

79. Other methods to asses RAP
• Ratio between Tricuspid valve E velocity
and tricuspid annulus tissue doppler
velocity E’ .
• If the ratio is >6 , RAP of >10 mmhg is
predicted with a sensitivity of 79% and
specificity of 87.7%.

80. Hepatic vein (HV) flow velocities
• The HV flow velocity profile consists of 4
components, 2 in forward flow and 2 in flow
reversal, each with a systolic and diastolic
component (S, D and SR, SD).
• The velocities reflect changes in right atrial
pressure and compliance, analogous to the
pulmonary vein flow velocity changes for the left
atrium.
• Normally, there should be no prominent reversal of
velocities and S is higher than D.

82. • As RV filling pressure increases, flow
reversal in systole and diastole
becomes prominent and is augmented
by inspiration.
• HV diastolic flow reversal is seen in PH
and constrictive pericarditis. Respiratory
variation helps differentiate between them.
• It is augmented with expiration in
constriction, whilst in PH it remains
constant.

83. • Hepatic venous systolic filling fraction -
VTI of systolic flow/ systolic +diastolic flow
VTI
• A Value of<55% is suggestive of RAP
being heigher than 8mmhg with a
sensitivity of 86% and specificity of 90%.

84. • ESTIMATION of PAP in pts without PR
/TR
• Comprises nearly 15% of pts
• RV IVRT/HR >65 ms identifies those with
PASP >40 mmhg.
• But if RA pressure is high ,IVRT may be
shorter despite pumonary hypertension.

85. PUMONARY ARTERY ACCELERATION
TIME
• In normal individuals, AcT exceeds 140
milliseconds and progressively shortens
with increasing degrees of pulmonary
hypertension.
• The shorter the acceleration time, the
higher the pulmonary artery pressure.

87. MEAN PAP
• The mean pulmonary artery pressure can
also be estimated by Doppler.
• Mpap = 0.61 × spap + 2mmhg
• Mpap = 4 × early diastolic PR velocity
• MPAP= 4× mean velocity of TR +RAP
• MILD PAH mpap – 25 to 40 mmhg
• MODERATE - : 40 – 55 mmhg
• SEVERE - : >55 mmhg

88. • Mpap = 79-(0.45×acceleration time)
• If AT <120 ms ; Mpap = 90-(0.62×AT )
• This method is relatively easy to perform,
highly reproducible, and unlike pressure
estimates based on tricuspid regurgitation
velocity, Doppler recordings from the
RVOT are available in virtually all patients.

89. DIASTOLIC PAP
• Dpap =( 4×end diastolic velocity of PR
jet)+ RA pressure
• Dpap = 0.49 × SPAP
• In cases where PR jet is not available , RV
pressure at the time of pulmonary valve
opening from TR velocity spectrum can be
used.

90. PVR
• Pressures are flow dependent, so
assessment of disease severity cannot be
reliably done from systolic pressure alone.
• Ex – pts with advanced PH , decreased
RV function RVSP is lower than expected.
• In these situations calculation of PVR is
more dependable.

91. • PVR= ( TR velocity/RVOT VTI) 10₊0.16
• A TRV/RVO VTI cutoff value of 0.175 had
a sensitivity of 77% and specificity of 81%
to determine PVR >2 WU.
• If >0.275 ,PVR of >6wu is very likely.
• But this equation performed
poorly(underestimation) when PVR >8

92. • PVR = (RVSP – E/e’ )RVOT VTI
• This equation performed better than the
previous one when compared to invasively
measured PVR.

93. • LINDQVIST method-
PVR= mPAP – PAWP / CO
Mpap= PASP × 0.61+ 2 mmhg
PAWP is assumed to be 10 mmhg
CO = LVO VTI × CSA of LVOT × heart rate

94. PULMONARY VASCULAR
CAPACITANCE
• A measure of proximal PA distensibility
• PVCAP = stroke volume/ pulse pressure
SV / 4 × (TRV2- PRV 2)
A value of < 0.8ml/mmhg predicts higher
mortality in PAH.

95. Assessing Severity of RV
Dysfunction
• Assessment of right ventricular function is the
single most important aspect of the DE
examination in patients with known or
suspected PVD
• As the morbidity and mortality associated
with this condition is heavily dependent on
the degree of adaptation of the right ventricle
to its excessive pulmonary vascular load.

96. • The right ventricle is better suited for
volume work.
• When there is afterload mismatch RV
dilatation is seen.
• When RV dilates it assumes a bullet
shape in A4C view and circular in SAX
view.

97. • SYSTOLIC FLATTENING OF IVS
• Degree septal bowing is quantified by measuring LV
• ECCENTRICITY INDEX.
• Normally LV is circular in both systole and diatole ,with
both vertical and horizontal dimensions of the cavity is
equal.
• Normal index is 1.
• Mild septal flattening – 1.1 to 1.4
• Moderate – 1.5 to 1.8
• Severe - >1.8

99. TAPSE
• TAPSE can be derived from 2D echo or M-
Mode , is simple to perform and has been
shown to be highly reproducible, owed in
part to the lack of reliance on RV
endocardial definition or geometric
assumptions.

100. • Ghio et al. recently showed in a PAH
cohort that a TAPSE≤1.5 cm was
associated with a nearly three-fold higher
event rate (death or emergent lung
transplant) versus subjects with a
TAPSE>1.5 cm.

101. TDI
• Tissue Doppler imaging (TDI) can also be used to
measure the velocity of RV contraction in the
longitudinal axis (denoted S’ or Sa), correlates with
TAPSE (r=0.90), and is another simple and
reproducible method of RV function assessment.
• An S’ <10 cm/sec predicts a cardiac index <2.0
l/min/m2 with 89% sensitivity and 87% specificity.
• In addition, the RV TDI signal can be integrated to
measure the longitudinal tissue displacement.

102. TEI index
• The myocardial performance index (MPI or Tei-Doppler
index) uses a different approach to RV function
assessment, integrating systolic and diastolic function
parameters in a single measure.
• the formula IVRT+IVCT/RVET, where IVRT is the RV
isovolumic relaxation time, IVCT is the isovolumic
contraction time, and RVET is the RV ejection time.
• The time intervals are typically derived from tissue
Doppler signals.
• Increasing values represent worsening function, with an
increased RV MPI associated with decreased survival in
PAH.

103. Doppler Echocardiography

105. • Normal value is 0.28 ± 0.004
• It is prolonged in RV dysfunction.
• The upper reference limit is 0.40 by pulsed
doppler and 0.55 is by tissue doppler.
• Limitation – it is load dependent and may
get pseudonormalized if RA pressure are
high.

106. RV SIZE
• The normal RV measures approximately 2.5–3.5 cm at end-
diastole, with a planimetered area of 15–18 cm2.
• Typically, the RV dimension and area are two-thirds that of the
LV.
• The normal RV:LV ratio is approximately 0.6–0.8, with
increasing RV:LV ratios in patients with mild (0.8–1.0),
moderate (1.1–1.4), and severe (≥1.5) RV dilatation.
• A useful rule of thumb: RV:LV ratio should be <1.0,
• Value >1.0 : strongly suggestive of RV dilatation, often
coinciding with RV dysfunction.

107. RVFAC
• A more quantitative approach is to measure
the total systolic area change of the RV,
referred to as the RV fractional area of
change (RVFAC).
• This measure is derived from the
planimetered areas of the RV at end-diastole
and end-systole ([RVFAC=RV Area ED-RV
areaES/RV AreaED] × 100) from the apical
four-chamber view.

108. • Normal value is 56 ± 13.
• A value of <40% implies RV dysfunction.
• The RVFAC does not require geometric
assumptions and correlates with the RV ejection
fraction.
• However, incomplete visualization of the RV cavity
(more common in the setting of RV enlargement)
as well as suboptimal endocardial definition lead
to relatively high inter- and intra-observer
variablility.

109. STRAIN and STRAIN RATE
• 2D strain using speckle tracking method is
likely to be more sensitive to detect early
RV dysfunction.
• Normal strain rate at base : 1.5 -1.74
mid cavity :1.46 – 1.62
• Normal value for strain : 25% -33%

110. Echocardiographic Assessment of
Cardiac Causes of Pulmonary
Hypertension
• The most common cause of pulmonary
hypertension is left sided heart disease
resulting in venous pulmonary
hypertension.
• Echocardiography allows for assessment
of left ventricular systolic and diastolic
dysfunction as well as left sided valvular
disease and congenital heart disease.

111. PERICARDIAL EFFUSION
• In the setting of PAH, a mild to moderate circumferential
pericardial effusion is seen in up to half of patients.
• In general, a pericardial effusion typically indicates right heart
decompensation, and is likely conferred on the basis of
longstanding right atrial hypertension and impaired
myocardial lymphatic drainage.
• The presence of a pericardial effusion has been one of the
most consistent echocardiographic findings indicative of a
poor prognosis in PAH.
• Percutaneous or surgical pericardial drainage should be
avoided unless there is especially compelling evidence of
tamponade, as the effusion typically is the result (not the
cause) of RV failure and right atrial hypertension

112. PROGNOSTIC FACTORS
Best predictors of poor outlook
1.RA size- RA area index change of 5
cm2/m
2. Pericardial effusion
3. Degree of interventricuar septal shift

113. • MPAP >49 mmhg
• Dpap >29 mmhg
• Abnormal end diastolic septal curvature
• IVC >2cm with <50% inspiratory collapse
• TEI index >0.98
• TAPSE

114. CT CHEST
• Plays an important role in diagnosis of
certain causes of PH.
• CT findings in PH –
• 1. diameter of MPA > 29mm
• 2. MPA/ ascending thoracic aorta >1
• 3. A segmental artery to bronchus
diameter ratio > 1 in three or four lobes.
• 4. distensibility of PA <16.5%

115. the pulmonary artery measures 41 mm in
diameter

116. • Cardiac changes –
• 1. RVH – wall thickness >4 mm
• 2. RV dilatation – RV/LV diameter ratio >1
at midventricular level on axial images.
• 3.straightening or leftward bowing of IVS
• 4. reduced RV EF
• 5.dilated IVC / HEPATIC VEINS.

117. • MOSAIC pattern of lung attenuation – 77%
of pts with CTPH and 12% of IPAH
patients.
• Regions of hyperemic lung and adjacent
regions of oligemic lung.
• Other diseases – small airway diseases
and infiltrative lung diseases.

118. CT PULMONARY ANGIOGRAM
• Useful in imaging the vascular changes that
typically occur in CTPH.
• The central Pas are dilated , luminal irregularities
by organized thrombus, rat tail appearance of PA
branches ,bands ,pouches, webs or flaps,
stenosis, post stenotic dilatation and tortuosity.
• The sensitivity and specificity is 98 and 95% at
lobar level and 94% and 93% at segmental level
with reference to invasive angiography.

119. eccentrically located thrombus that forms obtuse
angles with the vessel wall

120. peripheral wedge-shaped area of hyperattenuation in the
lung (arrow), a finding that may represent an infarct, as well
as a linear band (arrowhead).

121. FUNCTIONAL STATUS
ASSESMENT
• Exercise testing O2 consumption-
<10.4ml/min/m2 associated with poor
prognosis
• 6 minute walk test-simple test-detect
exercise desaturation,functional
assessment

122. CARDIAC CATHETERISATION
Indications
• Not required for diagnosis
• It must be done in borderline cases to assess operability
• Response of pulmonary vasculature to pulmonary
vasodilators like 02, tolazoline and nitric oxide should be
assessed

123. PRECAUTIONS WHILE DOING
CATH• Polycythemia
– PCV of >65% =Phlebotomy (O-D/O x wt x70)
• Anaemia
– Appropriate Hb =38 –(0.25 xSaO2)
• Contrast use
– Less than 4ml/kg

124. HEMODYNAMICS IN ES VS PPH
• ES had greater pulmonary artery pressures (107
+/- 20 versus 97 +/- 21 mm Hg, p = 0.06)
• Patients with Eisenmenger syndrome had
greater systemic cardiac indexes (2.7 +/- 0.6
versus 2.2 +/- 0.8 L/min/m2, p < 0.05) and lower
mean right atrial pressures (5 +/- 2 versus 12 +/-
5 mm Hg, p < 0.0001) than patients with primary
pulmonary hypertension
Hopkins et al,Comparison of the
hemodynamics and survival of adults with
severe primary pulmonary hypertension or
Eisenmenger syndrome.J Heart Lung
Transplant. 1996 Jan;15(1 Pt 1):100-5

125. DETERMINING REVERSIBILTY
• Absolute PVR
>12 ASD,>8 VSD,>7 PDA
• PVRI/SVRI Ratio
<0.25 =operable
0.25-0.5 –operable,mild risk
0.5-0.75 – operable with high risk of post op PAH
>0.75 - Unoperable
• PASP >80% SBP
• PA mean >50% Sytemic mean
NO LONGER
APPLICABLE

126. VASODILATOR TESTING

129. FAVOURABLE PARAMETER FOR
ICR

130. PIT FALLS
• Reversibility seen in <10%
• Hemodynamic guidelines to ensure postoperative
success and long-term survival without pulmonary
hypertension are still not precise.
– Lock et al(1982) showed that in large VSD with PAH, fall in
PVR/SVR by >30% did not correlate with operative
survival or late PVR/SVR.
– Moller (1991) showed closure of VSD with high PVR of >7
WU had higher operative and long term mortality.
– Kannan et al (2003)showed that 21% of those who
showed vasoreacitivity had poor outcome with persistent
PAH

131. • Technical difficulties leading to calculation errors and
other medical conditions need to be considered
• Vishwanath S,Kumar.Assessment of operability of congenital
cardiac shunts with increased PVR;CCI 2008
• Controversy whether testing at maximal stimulation
(90% O2 and 80 parts per million of iNO) or a
conservative intermediate protocols (O2 21–30%,
iNO 40 PPM) or gradual increases in vasodilator
challenge would be better predictors.

132. PIT FALLS
• However, advanced therapies for PAH have a
combined vasodilative and antiproliferative effect.
• Thus, there is also evidence to suggest that the
pulmonary hemodynamics and the clinical status
may improve irrespective of the response to acute
vasodilator testing.
• Absent or a negative pulmonary vasoreactivity study
should not preclude initiating disease-targeting
therapy.

133. Pulmonary wedge angiography
• For the right-sided
angiogram, a 5F or
6F pulmonary
wedge catheter
was placed in the
lower lobe to a
level one rib space
below the takeoff of
the right pulmonary
artery.

134. • For the left-sided studies, the catheter was
placed two rib spaces below the takeoff of
the left pulmonary artery.
• After the catheter was positioned, the
balloon was inflated and contrast material
was injected at a dose of 0.3 ml/kg
(minimum 2 ml).
• Injections were by hand as this was
thought to be safer and simpler

135. Analysis of the Angiogram
• Tapering
• AP view
• Maximum expiration
• Arterial lumen diameter 2.5 &1.5mm
noted.
• The length of the artery between the two
diameters is measured for as many no. of
vessels as possible
• Average taken.

136. Rate of Tapering

137. • The length of the artery segment between
the two diameters reflected the rate of
tapering of the artery (the longer the
segment, the more gradual the tapering;
the shorter, the more abrupt).

138. • Background Haze
• The degree of filling of small peripheral
arteries that determines the background
haze.
• The degree of background haze was
assessed as being normal, mildly,
moderately or markedly reduced by
comparing the angiogram with standard
normal angiograms.

139. • Arterial concentration is reduced in
newborns and young infants.
• In patients younger than 6 months of age,
mild or moderate reduction was considered
normal, and in younger than 1 year of age
mild reduction was considered normal.
• Reduced background haze in patients with
PAH.

140. • Pulmonary Circulation Time:
• Pulmonary circulation time as the transit
time of the contrast material through the
capillaries and veins.
• Measured by counting the number of
frames between the time the balloon was
deflated and the time contrast material
was seen in the pulmonary veins at their
site of entry into the left atrium.
• This is divided by the frame rate of the
cine film

141. • Longer the circulation time severe the
pulmonary vascular disease.

142. • Quantitative assessment from a
pulmonary wedge angiogram of the rate of
tapering of the pulmonary arteries is useful
in patients with congenital heart disease
who have, or are at risk of developing,
severe pulmonary vascular changes and
fixed elevation in pulmonary vascular
resistance.
• More abrupt arterial tapering is more
suggestive of severe changes in the distal
pulmonary vascular bed

143. Management
• Pharmacological therapy
• Conventional therapy
• Anticoagulation
• CCB’s
• Prostacyclins
• Endothelin receptor antagonists
• PDE inhibitors
• Novel therapies

147. Novel therapies
Riocigaut
fasudil

148. THANK YOU