A rare congenital anomaly

1. Presented by-
Jheelam Biswas
Ayesha Rahman
Shohely Pervin
Salma Afroz
Curtsey - Dr. Nanda Lal Das

2.  Hypoplastic right heart syndrome (HRHS)
is a congenital rare heart disease refers to
underdevelopment of the right side of the
heart means the structures like heart
chambers, heart valves and blood vessels.
 The syndrome is characterized by -
 cyanosis of the skin, nails, lips and other parts of the
body immediately after birth
 failure to thrive with the advancement of age.

3.  The aim of this report is to present a rare
disorder along with its clinical and
electrophysiological findings.
 And, finally emphasizing in the rehabilitative
treatments for increasing the survival and its
progress.

4.  Mimi, a six years old girl second issue of a non-
consanguineous marriage presented with the
complaints of bluish discoloration of lips and
tongue and gradually increasing respiratory
distress since birth and easy fatigability for last
15 days.
 According to her mother, the child has bluish
discoloration of lips and tongue since birth and
respiratory distress following feeding and crying
which was progressively increasing with age.
The respiratory distress was relieved by
squatting position.

5.  For last 15 days, she was unable to walk at a
small distance and became easily fatigue even
after taking a small amount of food. She has no
history of prolonged fever, cough, weight loss,
leg pain, convulsion or unconsciousness.
 There was no other child in her family with the
similar sort of illness.

6. On examination findings showed-
 dysponea, cyanosis, conjunctival congestion,
Tachycardia, clubbing in the fingers of all four limbs.
 underweight with moderately stunted growth.
 a visible pulsation on neck, bulging precordium, tapping
apex beat, loud S2 with single component and murmur.

8.  The hematological investigation showed-
 Hemoglobin level :24 gm/dl
 PCV : 70%
 all other indices were normal.
 The x- ray chest revealed-
 cardiomegaly with suggestive of pulmonary hypertension
 prominent right paratracheal shadow.
 The ECG findings revealed-
 right atrial enlargement (tall P wave, 7mm),
 left ventricular hypertrophy (deep S wave in V1).

11.  According to echocardiogram and color Doppler
study –
 PFO shunting right to left,
 tricuspid valve annulus measuring 15mm,
 no tricuspid regurgitation, straddling (type C),
 overriding of tricuspid valve,
 large inlet and trabecular VSD measuring 23mm
shunting bidirectional mostly right to left and
 pulmonary valve annulus measuring 6 mm, severe
valvular stenosis.

13. • During the cyanotic spell the immediate
management was done by-
 placing the child in knee chest position,
 oxygen inhalation,
 Tab. PROPRNOLOL (10 mg, 1 tablet stat and ½ tablet 8
hourly) ,
 Inj, Sodium Bi Carbonate (75ml IV slowly over 10
minutes) and
 5% Baby Saline (700ml IV over 4 hours).
 The subsequent follow up was done by counting
respiratory rate, heart rate with the measurement SpO2.

14. • The definitive management was surgical.
 Bidirectional Glenn shunt was done
 Followed by completion of Fontan operation.

15.  Hypoplastic right heart syndrome is a rare
condition. It is consists of-
 pulmonary valve atresia,
 a hypoplastic right ventricle and
 a small tricuspid valve.
 There are two types of this syndrome.
 A small right ventricle with a thick wall but working
tricuspid valve
 A normal right ventricle with complete but malfunctioning
tricuspid valve.

17.  Due to underdevelopment of the right ventricle, it
can’t contain the proper amount of blood from the
right atrium.
 As the ventricle has failed to grow and development
of the ventricles muscle structure is poor, so
additional problems are encountered as the heart
attempts to pump blood to the pulmonary valve for
transfer to the lungs.
 The proper amount of blood pumped from the right
atrium is not sufficient and this causes the blood to
be not pumped efficiently to the lungs.
 An inadequate blood supply to the lungs means a
poor return of oxygenated blood to the body so the
babies are tend to be cyanotic shortly after birth.

18.  The condition must be diagnosed clinically by
discernible murmur and an echo cardiogram can
confirm the diagnosis.
 The hypoplastic right heart syndrome must be
treated immediately after birth by-
 keeping PDA open by PEG1
 followed by Blalock Taussing shunt.
 When the child is around 3 months he/she will undergo
the Glenn shunt where the superior vena cava is
attached to the pulmonary arteries.

19.  In the next three to five years, the surgeons will
complete the Fontan, which connects the inferior
vena cava to the superior vena cava through an
intra-cardiac baffle or tunnel, or through an extra-
cardiac shunt. The result is complete bypass of
the right ventricle, so blood flows back from the
body directly into the lung. Many surgeons now
prefer the extra-cardiac shunt because it does
not require heart lung bypass and has a lower
risk of inducing arrhythmias.
 The predicted survival is 15-30 years.

20.  http://www.thic.com/hyporight.htm
yourtotalhealth.ivillage.co.hypoplastic-left-
heart-syndrome
http://www.wisegeek.com/what-is-
hypoplastic-right-heart-syndrome.htm
http://allhearts.blogspot.com/2007/03/hypo
plastic-right-heart-syndrome.html

21. Thank You