Dr. M.

Shahjehan Mirza
PGR Cardiology
Gulab Devi Hospital
 Case Presentation
 Bio Data
 XYZ/ 18y/ Female/ Unmarried/ Resident of Depal Pur

 Presenting Complaints
 Palpitations
 Cough with SOB 3 Months
 Body Aches
 Brief History of Presenting Complaints

 Palpitations

 Associated with exertion or picking heavy objects/ Relieved by taking

rest

 Cough with SOB

 More on exertion/ 1 flight of stair/Relieved by Rest/ Associated with

dry cough, more at night

 Body Aches

 Felt tiredness most of the time with generalized body aches

 Review of Systems
 Respiratory & CVS
 No history of Hemoptysis or chest pain

 Musculoskeletal
 No history of joint pains, oral ulcers, skin lesion and
Rash.
 Hematological
 No history of lumps and bumps, bruises or bleeding
 GIT
 No complaint of Itching, Skin discoloration or previous
history of hepatitis
 Past History
 She was admitted with same complaints in GDH 1 year back
 Dx of Pulmonary Stenosis was made
 Balloon valvotomy was done at that time in 2016
(Record not available)
 She was Discharged on oral Medicines but patient was non
compliant
 Family History
 No Significant Family Hx
 Personal and Social History
 Lower class
 Under matric Education
 works as Maid
 Non Alcoholic/ Non smoker
 Examination
 A Young, thin built female of normal height, cooperative, oriented
in person, place and time
 Pallor(+), Edema(-), jaundice(-), Cyanosis(-), Skin Rash(-)
 JVP
 Raised with Prominent A wave
 Vitals
 BP: 100/70 mmHg
 Pulse: 96bpm, Regular
 Temp: 98F
 R.R: 16/min
 SPO2: 98% at Room Air
 CVS
 Apex beat in 5th intercostal space 1cm medial to mid clavicular line
 S1 loud with Ejection systolic murmur of Grade 3-4, best heard in left
lower sternal border with a pulmonary ejection sound
 Soft S2 with Splitting
 Chest
 Normal Vesicular Breathing B/L with no added sound
 Abdomen
 Soft, Non tender
 CNS
 Intact
 Labs Findings
 ASOT -ive

 CRP -ive

 LFTs Normal

 RFTs Normal

 CBC Normal

 S/Electrolytes Normal
 ECG
 Right Axis Deviation with RV hypertrophy
 Echocardiography
 Dilated RA & RV
 Gradient Across Pulmonary Valve = 75mmHg
 Annulus = 19mm
 RV systolic Pressure = 70mmHg
 Normal Other valves
 EF = 60%
 Diagnosis
Severe Pulmonary valve Restenosis

Balloon Valvotomy of Pulmonary Stenosis

Pre and Post Procedure Parameters
Echocardiogrphy Echocardiogrphy

 Gradient Across PV  Gradient Across PV

75mmHg 37mmHg
Pulmonary Stenosis
 Anatomy
 Pulmonary valve is also called semilunar valve
because its cusps are semilunar in shape
 It has 3 cusps which are attached directly to the
vessel wall, there being no fibrous ring
 Cusps form small pockets with their mouths
directed away from the ventricular cavity
 Free margin of each cusp contain a central fibrous
nodule
 From each side of nodule a thin margin the lunule
extends up to the base of the cusps
 Valve close during ventricular diastole
 Definition and Etiology
 Obstruction to outflow from the right ventricle, whether with in
the body of the right ventricle, at the pulmonary valve, or in
the pulmonary arteries, is described as pulmonary stenosis.
 Often these obstructions occur with other major cardiac
abnormalities

 Causes
 Most commonly it is Congenital
 Rarely associated with Rheumatic heart disease
 Carcinoid tumor
 Sinus of Valsalva aneurysm or Aortic graft aneurysm
 Epidemiology
 PS is common form of Congenital Heart Disease
 Isolated PS comprises approx. 10% of Congenital Heart Diseases
 Except for critical stenosis in neonates, survival is the rule in
Congenital PS
 60% of patients with severe PS require intervention with in 10
years of diagnosis
 A slight female predominance exists
 Classification
 It is divided in further subtypes
 Pulmonary valvular stenosis
 Dysplastic valve
 Obstructive muscle
 Infundibular stenosis
 Peripheral pulmonary stenosis
 Pulmonary valvular stenosis
 Characterized by fused or absent commissure
 In most patients it is mobile, dome shaped structure with an
orifice that may be tiny and sometime eccentric
 Jet of blood through the valve usually causes post stenotic
dilatation, most often involving left and main pulmonary
arteries

 Dysplastic valve
 Consist of thickened, irregular, immobile tissue and variably
small pulmonary valve annulus
 Obstructive Muscle
 Obstructive muscle in the outflow tract, called
MODERATOR BANDS
 Is seen in patients with TETRALOGY OF FALLOT and in
some patients with MEMBRANOUS VENTRICULAR SEPTAL
DEFECT
 The muscular obstructions occur in the region of the
moderator band at the proximal infundibulum and in some
patients they tend to be progressive

 Infundibular Stenosis
 Associated with TETRALOGY OF FALLOT
 Rarely seen as isolated lesion
 Peripheral Pulmonary Stenosis
 May take several forms
 May be
 Single obstructive lesion
 Multiple similar lesion
 Symmetrical or Asymmetrical pulmonary arterial hypoplasia
 Some lesion are accessible to the surgeon but some located
more distally in the lungs and are surgically inaccessible
 There may be some proximal dilatation because of
hypertension and distal post stenotic dilatation because of jet
through the obstructive orifice
 Particular pattern of pulmonary artery hypoplasia is seen in
patient suffering from Maternal Rubella syndrome
 HISTORY
 Typical Mobile dome-shaped pulmonary valve stenosis is
relatively common
 Murmur of pulmonary stenosis is usually discovered at birth
 Because anatomic and physiological conditions necessary for its
production are present at birth
 Murmur at non precordial thoracic sites accompany pulmonary
artery stenosis are overlooked easily in infants and neonates
with rapid breath sounds.
 History of first trimester Maternal Rubella arouses suspicion of
pulmonary stenosis
 Familial recurrence of isolated pulmonary stenosis is uncommon
 Familial Noonan’s syndrome frequently occurs with dysplastic
pulmonary valve stenosis
 Familial pulmonary artery stenosis occurs as an isolated
anomaly
 But in Noonan’s syndrome with dysplastic pulmonary valve
stenosis, growth and development are poor
 Pulmonary artery stenosis associated with low birth weight and
physical or mental retardation
 Neonates with pulmonary valve stenosis experience rapidly
progressive cardiac failure and early death
 Mobile dome shaped pulmonary valve stenosis characterized by
normal birth weight and growth
 In a review of 69 cases
 The average age of death was 26 years
 7 patients survived to age of 50 years
 3 survived to age of 70-75 years
 In 21 adults, average follow up period was 50 years
 Longevity depends on 3 variables
 The initial severity of stenosis
 Whether a given degree of stenosis remains constant or
progressive
 Whether RV function is preserved
 Normal pulmonary valve orifice increases linearly with age and
body surface area
 Moderate to severe pulmonary stenosis tends to progress
 Stenosis of pulmonary artery is not progressive
 Occasional calcification is responsible for increasing degree of
stenosis in older adults
 Dyspnea and fatigue are mild as long as RV maintains a normal
SV at rest and augments its SV with exercise
 RV failure is the commonest cause of death
 Patient can experience light headedness, syncope or chest pain
 Physical Appearance
 Following 6 types of physical appearances are relevant in
patients with congenital Pulmonary Stenosis
 Pulmonary valve stenosis
 Noonan’s Syndrome
 Rubella Syndrome
 Williams Syndrome
 Alagille Syndrome
 Cornelia de Lange’s Syndrome
The chubby round bloated face Facial appearance of
of an infant with typical mobile arteriohepatic dysplasia (Alagille
dome shaped pulmonary valve Syndrome) characterized by
stenosis deeply set eyes, overhanging
forehead and small pointed chin
Noonan’s Syndrome in an 18 year Low posterior hairline of a
old man phenotype with webbing neonate with Noonan’s Syndrome
of the neck, low set ears,
abnormal auricles, hypertelorism
and small chin
4th century AD male with short
stature and broad webbed neck
suggesting Noonan’s Syndrome
Cornelia de lange Syndrome
include excessive body hair,
hearing loss, short stature,
skeletal abnormalities and GIT
problems
Congenital Rubella syndrome Williams Syndrome patient has
with severe B/L deafness and dental problems, distorted facial
B/L visual defects with cataract features, low birth weight,
and corneal opacity respiratory problems, mild
retardation
 Arterial Pulse
 When severe pulmonary stenosis is
accompanied by RV failure, especially
with coexisting LV dysfunction, the
arterial pulse is reduced
 Pulse pressure is small, rate of rise is
slow, peak is sustained and decline is
gentle.
 Asymmetry of Rt and Lt brachial and
carotid arterial pulses (Rt greater than
Lt) is a feature of supra valvular Stenosis
 Jugular Venous Pulse
 Jugular Venous A wave is distinctive and
increases progressively as the stenosis
increases
 Powerful Rt atrial contraction generates a
giant A wave via the superior vena cava and a
presystolic liver pulse via the inferior vena
cava
 With the advent of RV failure and TR, the
large A wave is accompanied by and increase
in the V wave
 Liver then manifest presystolic and systolic
pulsation
 Precordial Movement and Palpation
 Thrill associated with Pulmonary valve stenosis is maximal in the
left intercostal space With radiation upward and to the left
 Because intrapulmonary jet is directed upward and toward the left
pulmonary artery
 When secondary hypertrophic sub pulmonary stenosis coexists,
the thrill is maximal in the third or fourth left intercostal space
 The RV impulse is felt in between the ribs along left sternal
border
 In severe pulmonary stenosis, RV impulse is forceful and sustained
 Presystolic impulse along the lower left sternal border indicates
presystolic distension of the right ventricle in response to the
increased force of tight atrial contraction
 Auscultation
 High pitched ejection sound, clicking quality with
maximal intensity in 2nd left intercostal space, with
radiation upward and to the left
 The interval between the first heart sound and the
ejection sound varies inversely with the degree of stenosis
 The murmur duration increases with increasing degrees of
stenosis
 As severity increases, velocity of RV contraction increases,
pulmonary valve opens earlier and ejection sound occurs
earlier and merges with the first heart sound.
 When stenosis is severe, the murmur extends beyond the
aortic component of second heart sound, which is
partially or completely obscured
 Diagnostic Evaluation
 ACC/AHA guideline summary: Evaluation and monitoring of
pulmonic stenosis (PS) in adolescents and young adults

Class I
• Electrocardiogram for initial evaluation and repeat every 5-10 years
• Transthoracic Echocardiography for initial evaluation and repeat every 5-10
years
• Cardiac catheterization for evaluation of the transvalvular gradient if the
Doppler peak jet velocity is greater than 3 m/sec (estimated peak gradient
greater than 36 mmHg). Balloon dilation can be performed if indicated

Class III
• Cardiac catheterization for initial diagnostic evaluation
 Electrocardiogram
 The baseline electrocardiogram is often
normal, particularly in cases of mild or
moderate pulmonic stenosis
 In more severe cases, the mean frontal axis
may deviate to the right and varying degrees
of right ventricular hypertrophy may be
detected
 Right bundle branch block is also seen,
except in patients with Noonan syndrome
who invariably have a left bundle branch
block
 Echocardiography
 Two- and three-dimensional echocardiography are excellent
modalities for assessment of pulmonary valvular anatomy,
localization of the stenosis, and evaluation of right ventricular size
and function
 The 2006 ACC/AHA guidelines concluded that echocardiography is
the preferred test for evaluating the severity of pulmonic stenosis
 Typical valvular stenosis is characterized by mildly thickened leaflets
with restricted systolic excursion, leading to a domed appearance
 Presystolic doming of the valve may be seen during atrial contraction in
patients with more severe stenosis who have a noncompliant right
ventricle
 Truly dysplastic valves are characterized by markedly thickened and
seemingly immobile leaflets, hypoplasia of the valve annulus, and often
supra-annular narrowing
 Echocardiographic detection of pulmonic valve calcification is rare
 Continuous wave Doppler measurement of peak systolic velocities
provides estimates of the trans pulmonic gradient that are
correlate closely with simultaneous values obtained at cardiac
catheterization
 Color Doppler is particularly useful for the detection and
quantification of pulmonic regurgitation, using measurement of
the jet length and planimetered jet area

 Transesophageal echocardiography is NOT indicated in the

routine evaluation of pulmonic stenosis.

 However, it is often essential if the diagnosis of pulmonic valve

endocarditis is suspected
 Cardiac Catheterization
 Diagnostic cardiac catheterization is rarely required in patients with
pulmonic stenosis due to
 Extensive characterization of valvular structure, severity of stenosis,
and right ventricular size and function derived from echocardiography
 Invasive hemodynamic measurements and ventriculography may be
useful when the severity of stenosis is unclear or a significant
secondary infundibular stenosis is suspected in addition to the
valvular stenosis
 The 2006 ACC/AHA guidelines recommended cardiac catheterization
for evaluation of the severity of pulmonic stenosis in only one
condition:
 If the peak jet velocity on Doppler echocardiography is greater than 3
m/sec (estimated peak systolic gradient greater than 36 mmHg).
Balloon valvotomy is usually performed during the same procedure, if
clinically indicated
 Grading Severity
 Therapy of valvular pulmonic stenosis are determined by the
hemodynamic severity of the obstruction.
 Mild pulmonic stenosis is identified by
 A peak Doppler gradient across the valve < 36 mm Hg (equal to peak
Doppler jet velocity <3 m/sec)
 Moderate stenosis is identified by a
 Peak Doppler gradient of 36 to 64 mm Hg (peak jet velocity of 3 to 4
m/sec)
 Severe pulmonic stenosis is identified by
 Peak Doppler gradient >64 mm Hg (peak jet velocity of >4 m/sec)
 The 2009 EAE and the ASE recommendations, the 2006 ACC/AHA
valve guidelines, and the ESC guidelines for the management of
grown-up congenital heart disease classify the severity of
pulmonic stenosis according to the peak Doppler gradient
 The 2008 ACC/AHA guidelines for management of adults with
congenital heart disease may reflect the catheter-derived peak-
to-peak gradients more accurately than do peak Doppler
gradients
 A study of 90 patients with isolated pulmonic stenosis found
that
 The mean Doppler gradient more accurately reflected the
catheter-derived peak-to-peak gradient
 The peak Doppler gradient averaged 24 mmHg higher than the
catheter-derived peak-to-peak gradient.
 Monitoring
 2008 ACC/AHA adult congenital heart disease guidelines and
recommendations for monitoring asymptomatic patients with
pulmonic stenosis are
 For asymptomatic patients with peak Doppler gradient <30 mmHg,
follow-up physical examination, Doppler echocardiography, and
electrocardiogram are recommended at five-year intervals
 For asymptomatic patients with a peak instantaneous Doppler
gradient >30 mmHg, follow-up Doppler echocardiography is
recommended every two to three years
 Indications for Intervention
 For asymptomatic patients, balloon valvotomy is recommended
 With a domed pulmonic valve and
 Peak instantaneous Doppler gradient >60 mm Hg
 Mean Doppler gradient >40 mmHg (in association with moderate
pulmonic valve regurgitation)
 For symptomatic patients, balloon valvotomy is recommended
 With a domed pulmonic valve and
 peak instantaneous Doppler gradient >50 mmHg
 Mean Doppler gradient >30 mmHg (in association with moderate
pulmonic valve regurgitation)
 Surgical therapy is recommended for patients with
 Severe pulmonic stenosis
 An associated hypoplastic pulmonary annulus
 Severe pulmonary regurgitation
 Subvalvular pulmonic stenosis
 Supravalvular pulmonic stenosis
 Surgery is also preferred for most dysplastic valves and when
there is associated severe tricuspid regurgitation
 Balloon valvotomy is less effective in patients with
 Dysplastic valve (with or without Noonan syndrome), so surgery is
generally preferred
 However, the 2008 ACC/AHA guidelines note that balloon
valvotomy may be reasonable in patients with a dysplastic
pulmonary valve
 Who are either asymptomatic with a peak Doppler gradient >60
mmHg or mean Doppler gradient >40 mmHg
 Symptomatic with a peak Doppler gradient >50 mmHg or a mean
Doppler gradient >30 mmHg
 There are no definite indications for intervening on an
asymptomatic dilated pulmonary artery
 Long term Surgical Follow-up
 Long-term follow-up of surgical intervention before the current use of
percutaneous balloon valvotomy, comes from a report of 53 patients who
were initially treated between 1951 and 1982 at a mean age of 10 years.
Their status was determined 33 years later
 40 of the patients were treated with open valvotomy
 13 with infundibular resection
 The following findings were noted
 35 reinterventions were performed in 28 patients (53%), including
pulmonary valve replacement for regurgitation in 21
 Open valvotomy in 5
 and balloon valvotomy in 3
 Most of the reinterventions were performed more than 25 years after the
initial procedure
 20 patients developed an atrial arrhythmia
 6 of whom had radiofrequency ablation
 3 patients developed a ventricular arrhythmia
 Similar outcomes were noted at 22 to 33 year follow-up in a later
cohort of 90 consecutive patients who underwent surgery
between 1968 and 1980
 Survival was 93% at 25 years
 At last follow-up, 67% of patients were in NYHA class I and maximal
exercise capacity was 90% of normal
 Reintervention was required in 15%, primarily for pulmonary
regurgitation
 Reoperation for pulmonary regurgitation was required in 9%,
particularly after use of the trans annular patch technique.
 At last follow-up, moderate to severe pulmonary regurgitation was
present in 37%
 Supraventricular arrhythmias occurred only in patients with severe
pulmonary regurgitation and disappeared after reoperation
 There were no major ventricular arrhythmias
 Balloon Valvotomy
 When intervention is required and pulmonary valve anatomy is
suitable (e.g, domed), percutaneous balloon valvotomy is the
procedure of choice, with excellent short- and long-term
results
 Children with severe disease are usually treated early in life

 Procedural Considerations
 Use of an oversized balloon (approximately 1.2 to 1.25 times the
measured pulmonary annulus) aids in achieving a successful result
(defined as a final peak valvular gradient of <20 mmHg)
 Acute Response
 The procedure is generally well tolerated
 A mortality rate of 0.2% and major complication of 0.6% were reported by the
Valvuloplasty and Angioplasty of Congenital Anomalies registry
 Acute complications are generally minor and include

Vagal response Catheter induced ventricular ectopy

Right bundle branch block Transient or permanent high grade AV nodal
block

Pulmonary valve regurgitation Tricuspid regurgitation

Stroke Syncope
Pulmonary artery rupture Pulmonary edema
Cardiac Perforation Tamponade
 Transient severe right ventricular outflow tract obstruction
(“suicidal right ventricle”) has been reported after the pulmonic
valvular obstruction has been relieved.
 This may be treated by volume expansion and beta blocker therapy
and tends to regress with time
 A pooled analysis of studies involving 784 patients of all ages
showed that clinical success was achieved with balloon valvotomy
in 98%, with a fall in the systolic outflow gradient from 71 to 28
mmHg.
 Procedural mortality was less than 0.5%
 Among the 196 patients in whom such data could be obtained, trans
valvar and infundibular obstruction were each responsible for about
one-half of the total residual gradient.
 After balloon valvuloplasty, a residual infundibular gradient tended to
decline over several months as right ventricular hypertrophy
diminished.
 Long Term Efficacy
 The long-term efficacy of percutaneous balloon valvotomy was
evaluated in a series of 53 adolescent and adult patients 13 to 55 years
of age treated between 1985 and 1995
 The systolic pressure gradient across the pulmonary valve fell from 91 to 38
mmHg after the procedure and had fallen further at late follow-up an
average of 7 years later.
 In 7 of 53 patients who developed pulmonary insufficiency immediately after
balloon valvotomy, none had this complication at late follow-up.
 In another report, 62 patients 9 months to 44 years of age were followed
for 1 to 10 years (mean 6.4 years) after percutaneous balloon valvotomy
 The mean transvalvar gradient fell from 93 to 19 mmHg immediately after
the procedure, and was 18 mmHg at follow-up.
 Progressive spontaneous regression of infundibular hypertrophy was noted.
Restenosis occurred in less than 5% and was more likely in patients with
dysplastic valves
 Similar findings were noted in a series of 85 patients followed for up to
10 years.
 Repeat balloon dilation was required in 11%
 surgical intervention for subvalvular or supravalvular stenosis in 5%
 Residual pulmonary regurgitation was noted in the majority of patients, it
was mild and right ventricular volume overload did not ensue.
 Longer-term follow-up is available after surgical correction than after
balloon valvotomy. As noted above, patients who underwent surgical
valvotomy had, at 33 year follow-up, a 53 percent likelihood of
reintervention
 most reinterventions were performed more than 25 years after the
procedure
 The applicability of these findings to balloon valvotomy is uncertain
 Surgery tended to produce lower long-term gradients but a higher rate of
pulmonary insufficiency (45 versus 11 percent in one series)
 This may be important since pulmonary insufficiency requiring pulmonary
valve replacement was the indication for reintervention in three-quarters
of the patients in the surgical series