CAR/01(R).VASCULOPATHY IN A CASE OF SPINA BIFIDA OCCULTA
K R Lahiri; S V Kondekar, R K Vasvani, Rahul Kasliwal
Department of pediatrics, Seth G S Medical College and KEM Hospital Mumbai 400012
Ten year old girl presented with small ulcers and blackish discoloration of toe tips of left foot,
slowly resulting in decreasing length of left toes over last 2 years. She also complained of
decreased sensation over left foot and pain in left foot while walking; on and off since last two
years. There was no history of swelling, trauma or fever. On examination, child was
hemodynamically stable, with all peripheral pulses well felt except left dorsalis pedis artery while
left posterior tibial artery pulsations were felt normally. Right dorsalis pedis and posterior tibial
artery pulsations were felt normally. On examination of toes, four healing ulcers smaller than 1 cm
diameter were present one on each (except little toe) toe with resorption (shortening) of second toe
and deformed first and fourth toes, with absent left dorsalis pedis artery. There was no sensory loss
or obvious muscular atrophy or joint movement restriction. A tuft of hairs was also noted over
lumbosacral area, but there was no sinus or bony defect felt. Rest of the systemic examination was
normal. Clinical differentials were peripheral vascular disease/ vasculitis, collagen vascular disease
or thrombotic disorders. Investigations showed normal hemogram and lipid profile, normal
prothrombotic profile including C3, ACLA, ANA and antidsDNA Left lower limb arterial Doppler
study was suggestive of probably congenital hypoplasia of dorsalis pedis with distal part of left
anterior tibial artery and dorsalis pedis artery showing small sized lumen with decreased flow.
There was no thrombus. Simultaneusly MRI spine was done; that confirmed diastematomyelia type
II with teethering of spinal cord, more on the left side. During course in ward, child was started on
oral nicotinic acid, (which acts as a peripheral vasodilator). Gradually over 2 weeks therapy,
pulsations of left dorsalis pedis artery appeared. Child was further investigated for digital
subtraction angiography that documented post therapy normal lower limb angiogram. To conclude,
the peripheral vascular disease or congenital dorsalis pedis artery hypoplasia on Doppler study cant
be taken as gold standard diagnosis; it can at times have a hidden cause of autonomic neuropathy as
a part of teethering of spinal cord due to diastematomyelia, as was likely in this case.
CAR/02(P).CORRELATION OF ELECTROENCEPHALOGRAM (EEG) AND THE
ACTIVE AND INACTIVE FORMS OF NEUROCYSTICERCOSIS.
Nilofer Mujawar
Department of Pediatrics,NKP Salve Institute of Medical Sciences and Research Centre,
Digdoh Hills, Opposite CRPF gate, Hingna Road, Nagpur - 440019
Introduction-The most common parasitic infection of the central nervous system (CNS) is
cysticercosis. It is caused by the larval form of Taenia solium. Aim and Objectivese : The aim of the
present study was to correlate the EEG and active and inactive forms of NCC as seen on the CT
Scan of the brain. Material and Methods- 157 patients in the age group of 3 years to 21 years
presenting with unprovoked seizures were included in the study. The CT Scan of brain revealed
NCC in various stages.The interictal EEG of these patients was studied. Results-88 patients
(56.05%) were males and 69(43.95%) were females.51 patients(32.48%) had a normal EEG ,52
patients(33.12%) had generalized seizures activity, while 54(34.39%) had focal slowing. 94
patients(59.88%) had active forms while 63(40.13%) had inactive forms of NCC. Of those whose
EEG was normal 47.06% had active forms and 52.94% had inactive forms. Of those with
generalized seizure activity 84.62% had active forms while 15.38% had inactive forms. Of those
with focal seizure activity 48.15% had active forms while 51.85% had inactive forms. ConclusionsEEG abnormality does not depend so much on number of lesions as on viability of cysts. The
incidence of generalized Seizure activity is higher in patients with active forms. Focal seizures have
slightly higher incidence with inactive forms. EEG can be normal in patients with active forms also.
CAR/03(O).THERAPEUTIC DRUG MONITORING OF DIGOXIN: USE OF SALIVA AS A
NON-INVASIVE MATRIX
U P Kulkarni, M N Muranjan, N J Gogtay, N A Kshirsagar
Department of Pediatrics and Department of Clinical Pharmacology, Seth GS Medical College and
KEM Hospital, Parel, Mumbai-400012
INTRODUCTION: Digoxin has a low therapeutic index. Hence estimation of serum digoxin
concentration for therapeutic drug monitoring (TDM) is recommended to ensure adequate
digitalization and avoid toxicity Saliva has been shown to be an effective alternative to serum for
TDM of many drugs. Saliva monitoring is non-invasive and offers a convenient biologic matrix in
children. OBJECTIVE: To assess the clinical utility of saliva for TDM of digoxin and to co-relate
saliva levels with serum levels. DESIGN: Prospective cohort study. METHODS: Consecutive
pediatric outpatients and indoor admissions who were on oral digoxin therapy for at least 6 days and
compliant with medication were enrolled. Blood sample and citric acid stimulated saliva sample
were obtained at least 6 hours after the last dose of digoxin and analyzed using a solid-phase 125I
radioimmunoassay kit for digoxin. A co-relation analysis was done between saliva and serum
concentration, at 5% significance and 80% power. RESULTS: Of the 33 patients screened, 18
patients (10 males and 8 females) formed the per protocol population. The mean serum and salivary
levels of digoxin were 0.94 ± 1.42 ng/ml and 0.26 ± 0.28 ng/ml respectively. No correlation
between serum and salivary digoxin level was observed when all patients were analyzed (r = 0.282,
p = 0.2569); however on exclusion of patients with toxic digoxin levels in serum, a statistically
significant correlation was observed (r = 0.5947, p = 0.0187). CONCLUSION: Saliva may be
unreliable as an alternative matrix to serum for TDM of digoxin. Presence of correlation on
exclusion of patients having serum digoxin level in toxic range is suggestive of presence of a
digoxin excreting mechanism which excretes a fixed fraction of serum digoxin in saliva at
therapeutic and subtherapeutic levels of serum digoxin and gets saturated when serum digoxin is in
the toxic range.
CAR/04(P).TOTAL ANOMALOUS PULMONARY VENOUS CONNECTIONS ( TAPVC ) :
OUR EXPERIENCE OF A TERTIARY CARE PEDIATRIC CENTRE
P Kumar, A Dhall, R Girish , R Ghuliani
Department Of Pediatrics, Army Hospital Research and Referral, Delhi Cantt - 110010
Aims and objectives : Total anomalous pulmonary venous connection ( TAPVC ) consists of
abnormality of blood flow where all four pulmonary veins can have supracardiac, cardiac,
infracardiac, or mixed type of drainage instead of their normal place i.e. left atrium. Present study
was undertaken to study their various clinical profiles, management issues and their follow up over
next 2 years. Method: We came across twenty two patients with TAPVC at our centre in 3 years.
Their clinical profile, echocardiographic and operative findings were analyzed. Patients were
followed up for 2 years. Results : There were 14 males and 8 females. They were infracardiac in 6,
supracardiac in 10, cardiac in 4 and were mixed type in 2. In 2 patients TAPVC was associated with
complex congenital cardiac defects and were inoperable. All patients with infra-diaphragmatic type
had an acute presentation at mean age of 7 days ( 3 days to 22 days ) and required emergency
surgery. In cardiac type 2 patients had drainage of all pulmonary veins to coronary sinus. In mixed
type various combinations were seen. Diagnosis was confirmed by echocardiography in all cases
except 3 cases where cardiac catheterization was required . In total 18 patients underwent surgery
at various centers. Two patients died after 1 and 3 months of surgery while 16 did well in immediate
post operative period. 10 Patients were followed up for 2 years while 6 were lost to follow up.
Conclusion : TAPVC though uncommon, poses a diagnostic challenge most of the time. Early and
accurate diagnosis, careful pre operative care and prompt surgery are the keys for favourable
outcome.
CAR/05(P).WHICH VENTRICULAR SEPTAL DEFECTS REQUIRE CLOSURE ?
EXPERIENCE AT A PEDIATRIC CARDIAC CENTRE
P Kumar, A Dhall, R G Holla, R Ghuliani
Department Of Pediatrics, Army Hospital Research and Referral, Delhi Cantt - 110010
Which Ventricular Septal Defects require closure ? Experience at a Pediatric Cardiac Centre
Objectives – To study natural history and identification of indications for closing Ventricular septal
defects ( VSD ) Methods – Children between 0 to 12 years with isolated VSDs were followed at
three monthly intervals for a period of two years with repeated clinical and echocardiographic
examinations. Results –VSD was perimembranous in 75 % , 12 % were muscular , 2.5% were
Subpulmonic and multiple in 3 % of cases. VSDs were small in 67%, moderate sized in 22.5 %
and large in 10.5 % cases. Spontaneous closure occurred in 14% patients mainly in first year of all
types of VSDs. PA pressures were normal in 71 %, whereas 8%, had mild, 5 % moderate and 16
% severe PAH. Infants with severe PAH (7 %) underwent urgent surgery at mean age 5.8 months
without prior cardiac catheterization. All patients did well except 2 cases who died due to causes
unrelated to PAH. Majority of patients with mild PAH showed decrease in pressures over 2 years
follow up. Half of patients with moderate PAH had decrease in PA pressures and remained on
medical follow up while remaining underwent surgery. Conclusions: PA pressures (derived from
echocardiographic studies) should be the main guiding factor for interventional or surgical closure
of VSDs. Spontaneous closure occurs in majority of small defects in infancy irrespective of its
location. Large defects invariably have severe PAH hence should be closed after confirmation of
their operability by cardiac catheterization. Patients with moderate PAH require meticulous follow
up as they have tendency to develop severe PAH. Assessment of Qp / Qs was not consistent and
was found unreliable.
CAR/06(P).RECURRENT CEREBRAL THROMBOEMBOLISM
MYXOMA
Surekha Joshi, Sushma Malik, Manoj Patil, Mona Gajre, Uday Jadhav
Govt Qtrs, Bld no 1, Flat no 25, Haji Ali,Mahalaxmi, Mumbai-400034
FROM
ATRIAL
Introduction: Cardiac Myxoma is a rare cause of cerebrovascular disease in children. Primary
cardiac tumors in children are seldom found and myxoma (14%) is the third common type after
rhabdomyomas and fibromas. Case Report: 11 year old male child presented with sudden fall and
right hemiplegia. Examination revealed stable vitals and right hemiplegia with UMN facial palsy.
Cardiac examination revealed soft systolic murmur and rest of systems including fundus were
normal. CT and MRI brain showed ischemic area in left basal ganglia and capsular region. On
further work up the 2D-ECHO revealed pedunculated left atrial myxoma (4.3 x 2.1cm) with
moderate MR. In the ward, hemiplegia recovered to nearly 80% and patient’s surgery for myxoma
was contemplated. However in the meanwhile, patient had another embolic episode with seizures
and transient right hemiplegia which recovered in 48 hrs. Subsequently patient was operated, the
mass was completely excised and mitral valve replaced. He has been asymptomatic post surgery for
last 2 months. Discussion: The heart is the most common source of cerebral emboli in children.
Most of them have an underlying heart disease, but in some instances a rare cause like a tumor may
be discovered. Clinical presentation of myxoma is dependant on its site & size. 90% are solitary and
pedunculated and 70- 80% are in left atrium, which may lead to cerebral embolization and right side
cause pulmonary emboli. Myxomas are friable and recurrent emboli are well known. The
neurological manifestation consists of acute deficits as the only presenting feature. ECHO, 3D CT
& cardiac MRI are the diagnostic modalities. Surgical excision is often curative and a damaged
valve may require replacement. It is recommended that screening be done in family members as
10% are known to be familial and few have syndromic association ( NAME & LAMB).
Postsurgical follow up is necessary as it can recur ( 1-5% in sporadic and 12% in familial variety).
Conclusion-A high index of suspicion in cases presenting as acute CVA may alert us to suspect this
potentially curable cardiac tumor in children, even in absence of cardiac symptoms.
CAR/07(P).VENTRICULAR SEPTAL DEFECT WITH RHEUMATIC HEART DISEASE:
AN ASSOCIATION?
Milind S. Tullu, Radha G. Ghildiyal, Shrinivas Tambe, Sunil Jumnake, Asmita Advirkar
Department of Pediatrics, T.N. Medical College & BYL Nair Hospital, Mumbai 400008
Introduction: We report the rare co-existence of congenital heart disease, CHD (incidence 8 per
1000 live births) with rheumatic heart disease, RHD (prevalence of 2-11 per 1000 in school-aged
children) in a patient who also had infective endocarditis. Case Report: A five-years-old girl was
diagnosed as a case of CHD (large perimembranous ventricular septal defect-VSD) at 6 months of
age (color doppler examination done owing to murmur detected by physician). At four years of age,
she developed breathlessness & increased precordial activity. Color doppler revealed VSD (with
tricuspid valve aneurysm) with RHD (moderately severe mitral regurgitation with mild aortic
regurgitation and thickened valves). There was absence of arthritis or involuntary movements. She
developed fever (in June 2006) and color doppler revealed VSD with RHD and vegetations on
tricuspid valve. She had tachypnea, tachycardia, cardiomegaly and right bundle branch block.
Hemoglobin level was 13 gm%, total leukocyte count was 17600/cumm, ESR was 40 mm at end of
1 hour with abnormal ASLO titre. The recurrence of rheumatic carditis was treated with intravenous
antibiotics, prednisolone (with aspirin) and anti- cardiac failure management. Corrective surgery is
planned in recent future. Discussion: Thakur et al had reported that prevalence of RF/RHD was
significantly higher in children with CHD-8.8% as compared to those without CHD-0.3%).
Bokhandi & Tullu et al have also reported association of VSD and RHD in 2 cases (amongst 5 cases
of CHD with RHD). It is not possible to determine whether the presence of CHD with RHD is a
mere coincidence or whether the presence of CHD actually predisposes to RHD.
CAR/08(P).PERICARDIAL EFFUSION IN A PATIENT WITH KOCHER-DEBRESEMELAIGNE SYNDROME
Praveen Dharaskar, Milind S. Tullu, Santosh Kondekar, Rajwanti K. Vaswani, Keya R. Lahiri
Department of Pediatrics, Seth G.S. Medical College & KEM Hospital, Mumbai- 400012
Introduction: Kocher-Debre-Semelaigne (KDS) syndrome is a rare disorder causing
pseudohypertrophy of muscles due to long standing hypothyroidism. Case report: A ten-year-old
female presented with complaints of swelling all over body & breathlessness since two years; and
difficulty in walking & getting up from sitting position since five months. She had a pulse rate of
76/min, respiratory rate of 24/min, and blood pressure of 90/60 mm Hg. Her height was 116 cm
(expected 136 cm), and weight was 20 kg (expected 32 kg). She had dull facies, hypertrophy of
both calf muscles, and non-pitting edema of legs. Cardiovascular system examination revealed apex
beat in left 5th intercostal space in midclavicular line, left heart border in midaxillary line (not
coinciding with the apex), & muffled heart sounds. Nervous system examination revealed normal
tone, proximal muscle weakness (muscle power 4/5) & normal distal muscle power, diminished
deep tendon reflexes, flexor plantar reflexes, and normal sensory system. Features of
hypothyroidism with pseudohypertrophy of muscles suggested the diagnosis of KDS syndrome.
Investigations revealed normal blood counts, anemia (Hb 9.5g%), pericardial effusion
(echocardiography & CT chest), normal liver and renal function tests and bone age of 8 years.
Thyroid function tests showed low T3 < 40 ng/dl (normal 70-200 ng/dl), low T4 < 1 microg/dl
(normal 5.5-13.5 microg/dl), high TSH > 75 microIU/ml (normal 0.2-5.0 microIU/ml). USG thyroid
was normal, technetium scan showed normal position of thyroid but poor uptake and antimicrosomal antibodies were absent. The patient was started on thyroxine replacement therapy and
responded well. Discussion: The association of hypothyroidism and muscle pseudo-hypertrophy is
called as Kocher-Debre-Semelaigne syndrome. The association of this syndrome with pericardial
effusion has not yet been reported.
CAR/09(R).MULTIPLE INTRACARDIAC RHABDOMYOMAS IN A NEONATE: A CASE
REPORT.
Vaishali Joshi; Lorraine Fernandes;Philomena P..D’Souza.
Department of Pediatrics, Goa Medical College, Bambolim, Goa..403202
Primary congenital cardiac tumors are rare. Incidence of cardiac tumors is 1-2 cases per 10,000 live
births 90% of which are benign. Among the benign tumors rhabdomyoma is the common tumor.
We would like to report a case of neonate with multiple intracardiac (intracavitary tumors)
antenatally detected as fetal dysrhythmia.There is strong association between cardiac
rhabdomyomas and Tuberous Sclerosis(50-86%). Though many of the manifestations of Tuberous
Sclerosis develop over time, in our case the neonate had CNS and skin manifestations along with
cardiac rhabdomyoma at birth itself. Though the presence of cardiac dysrhythmia in cardiac
rhabdomyomas indicates poor prognosis in our case neonate had one brief episode of
cardiorespiratory arrest and subsequently had no complications .Baby was managed conservatively.
Rhabdomyomas showed considerable regression on follow up echo at 1 year and no progression of
manifestations of tuberous scelerosis was noticed .Child is presently doing well.
CAR/10(P).CHILDHOOD HYPERTENSION
Palak T Hapani, K M Mehariya, Maulik Shah
O / 95, Civil Hospital, Asarva, Ahmedabad – 380016.
Introduction: Incidence of hypertension (HT) in chilodren is 0.6 – 11 %, common causes being
renal and endocrinal. Design, setting & method: Retrospective study from July 99 to June 01. Age
group – 1 month to 5 yrs. BP taken for 3 sittings for Ht, wt, age, sex & defined as per AAP II Task
Force. Investigations stepwise. Total 60 pts studied were divided in 2 groups- I acute HT, II
chronic HT. Treatment protocol as per II task force, and outcome recorded. Observations: 32 had
acute HT, while 28 had chronic HT. Clinical features included fever – 12 acute, 9 chronic; oliguria
– 16 acute, 14 chronic; puffiness of face – 18 acute, 23 chronic; oedema feet – 12 acute, 19 chronic;
abd. Pain – 5 acute, 7 chronic, headache – 2 acute, 6 chronic; convulsion – 2 acute, 4 chronic.
Investigations revealed albuminuria in 26 acute cases & 11 chronic, microscopic hematuria in 17
cases of acute HT & 4 chronic, pyuria in 8 acute and 9 chronic cases, altered RFT in 5 acute and 10
chronic, and ASO positive in 8 acute and 5 chronic cases of HT. Abnormal findings on USG were
seen in 20 cases of acute and 22 of chronic HT, those on IVP were 8 & 6 respectively. Etiologies of
acute HT were, renal – 23, CNS – 3, GBS – 2, Cushingoid synd – 3 & steroid toxicity – 1. Chronic
HT was due to renal parenchymal disease in 16, coarctation of aorta in 1, aorto-arteritis – 1, HUS –
1 and 3 other cases. All pts of acute HT were controlled with drugs, control achieved in 7 days in
26, while 6 pts. took 7-10 days. Of pts. with chronic HT, 5 were cured while 2 had refractory HT.
Conclusions: HT in childhood is fairly common, & certain diseases predisose to HT, most cases can
be successfully controlled medically.
CAR/11(P).SUPRAVENTRICULAR TACHYCARDIA AND WPW SYNDROME WITH
EVENTRATION OF DIAPHRAGM - A RARE ASSOCIATION
Yogesh P Khachane, Dr. Leslie Lewis, Dr. Ganesh P.
Kasturaba Medical College, Manipal, Karnataka, 576104.
Supraventricular tachycardia is the most common symptomatic arrhythmia in young patients,
frequently associated with Wolff-Parkinson-White (WPW) syndrome due to AV bypass tract
providing re-entry circuit. In very few cases association of eventration of diaphragm with WPW
syndrome have been reported. Case Report : Term, AGA born to non-consanguineous parents,
presented on Day19 of life with inconsolable cry and breathlessness since 2 days. At admission,
child was in shock with severe respiratory distress. Air entry was reduced in left infra-axillary,
infra-scapular region. Apex beat was not localized, S1 S2 better heard on Right side. Per-Abdomen:
liver 4cm, firm, spleen 2cm. Chest X-ray showed homogenous opacity in left lower zone. Diagnosis
of Pneumonia with septic shock was made; child was put on IMV support, started on Inotropes after
2 boluses. Child improved hemodynamically within 24 hrs, was extubated after 36 hrs. Within 48
hrs the liver size came down to 2cm. USG thorax was suggestive of partial eventration of
diaphragm. On day 30, child was irritable, pale, having tachycardia, poor peripheral pulsations.
ECG was suggestive of SVT with heart rate 300/min. Managed with IV Adenosine. Repeat ECG
showed characteristic delta waves suggestive of WPW syndrome. 2-D Echocardiography and
thyroid function tests were essentially normal. Child was started on prophylactic therapy with
Amiodarone. Discussion : Eventration of diaphragm itself may present with cardiorespiratory
failure, simulating pneumonia. However suspicion of SVT should be kept in mind in such cases.
CAR/12(O).PREVALENCE OF HYPERTENSION AMONG URBAN SCHOOL GOING
CHILDREN IN SHIMLA
N Grover, Avinash Sharma, S L Kaushik, Rajiv Bhardwaj
Deptt. Of Pediatrics, IGMC, Shimla, HP-171006
Hypertension is a major public health problem worldwide and is one of the risk factors for coronary
artery disease and cerebrovascular disease. The development of adult hypertension may start very
early in life, and children with high blood pressures tend to have high blood pressure in later life.
Design: cross-sectional school-based survey. Settings and methods: 566 apparently healthy urban
school going children of both sexes from 11-17 years were examined. Height and weight were
recorded and BMI calculated. Blood pressure was measured thrice in each child and the mean
calculated. BP was measured according to the fourth task force recommendations. BP was
compared for each child according to age, height and sex. The children who were found to have
hypertension, high normal BP or were prehypertensives were examined at least four weeks later and
again the blood pressure was compared for age, height and sex. Results: 311(54.9%) were boys and
255(45.1%) were girls. Mean age was 13.56 years with a standard deviation of 1.73 years. 35(6.2%)
children were overweight and 5(0.9%)were obese. On initial examination 151(26.7%) children were
hypertensive, 110(19.4%) had high normal BP and 12(2.1%) had prehypertension. On subsequent
examination 39(6.9%) children were found to be hypertensive, 39(6.9%) had high normal BP and
22(3.9% had prehypertension). Out of these 100 children who had their BP above the normal range
46(46%) had a family history of hypertension and out of these 100 children 14 were overweight and
3 were obese. In all there were 40 children whose BMI was high for their age and sex (35
overweight and 5 obese), 17(42.5%) out of these 40 children had high BP for their age, sex and
height. In contrast there were 526 children who had normal BMI out of which 83(15.78%) had high
blood pressure. Conclusion: prevalence of sustained hypertension among urban school going
children in the age group of 11-17 years was 6.9% and importantly 3.9% children had
prehypertension. Prevalence of hypertension was high among overweight and obese children.
CAR/13(P).DOUBLE AORTIC ARCH
A.R.Dhongade,R.R. Palliana, R.K.Dhongade, M. Deogaonkar
Sant Dyaneshwar Medical Education and Research center, Pune-411052
Double aortic arch is a common form of complete vascular ring encircling the esophagus and the
trachea.Although various forms of double aortic arch are present the common defining feature is
presence of left and right aortic arches.This a rare form of cardiac malformation but can be managed
surgically. We present a case of 2 month old male baby full term normal delivery with birth weight
of 2.85 kg,who was brought with complaints of ''noisy breathing, cough, fever and repeated
episodes of vomiting since birth. Child had multiple hospital admissions for right sided aspiration
pneumonias.On examination baby was febrile, tachypneic with''noisy breathing''and biphasic stridor
was evident. Respiratory system revealed crepitations and decreased breath sounds on the right side
of chest .Cardiovascular system no murmur audible .Chest xray revealed right sided apical
aspiration pneumonia.His condition failed to improve inspite of iv antibiotics. barium swallow done
which revealed an impression on the posterior esophageal wall at the level of aortic arch on the
lateral view.CT angiography revealed right sided aortic aorta splitting into larger right arch and
smaller left arch.Both the arches were passing around the esophagus and trachea producing a
vascular ring. All images were viewed at lung window and no abnormality. 2Decho study
sugesstive of aortic anomaly no other structural cardiac defect.Patient underwent surgery for
resection of the vascular ring and is now doing well.Conclusion: Any infant with biphasic stridor
sould undergo chest xray and barium swallow to detect presence of a rare vascular anomaly.
Outcomes are excellent after repair of double aortic arch.
CAR/14(R).ECTOPIA CORDIS: A CASE REPORT
Vaishali Joshi; Mimi Silveira; Celin Andrade
Department of Pediatrics, Goa medical college, Goa.
Ectopia cordis is a rare congenital abnormality occurring in 5.5 to 7.9 per million live births. This
defect is characterized by partial or complete displacement of the heart out of the thoracic cavity.
Five types have been described in literature i.e. cervical, cervicothoracic, thoracic,
thoracoabdominal and abdominal. In this report we would like to present a neonate with ectopia
cordis cervicothoracic type but with minimal defect in the sternum. Majority of ectopia cordis have
associated cardiac defects and it has also been reported with other congenital anomalies as
abdominal wall defects, cranial facial malformations, cleft lip and palate, neural tube defects,
pulmonary hypoplasia,chromosomal defects etc. In our case no associated defects were found on
thorough examination and investigations. Baby was born by lower section cesarean section to a
gravida two female. Baby was hemodynamically stable at birth.. This was the first case of ectopia
cordis found in our institute which has 500-600 newborns seen every month. Literature reveals that
ectopia cordis is one of the rarest but impressive congenital anomaly.
CAR/15(P).CLINICAL PROFILE OF CONGENITAL HEART DISEASE IN CHILDREN
WITH SPECIAL REFERENCE TO ECHO CORRELATION
Abbas Hyderi, Farhan ul Haque, B. Vijaykumar
House No. 1702, 31st Cross, Second Block, Rajajinagar Bangalore – 560010
Background and objectives: Congenital heart disease (CHD) is a common congenital malformation
and is the leading cause of mortality in children with congenital malformation. With an early
diagnosis majority are amenable to treatment. The diagnosis of CHD with echocardiography is near
accurate. In India with limited resources, clinical acumen still forms the backbone of diagnosis and
management. Hence, it needs to be ascertained as to what would be the diagnostic reliability of
clinical diagnosis. Objective of the study were as follows : (1) To study the clinical profile including
modes of presentations of congenital heart disease in children. (2) To study the correlates of
different congenital heart diseases through demographic and socioeconomic variables. (3) To study
the correlation between physical diagnosis (clinical examinations / chest X-ray / ECG) and
echocardiography diagnosis. Methods: Sixty cases of children aged 1 month to 12 years with
features suggestive of CHD were studied for clinical profile and sociodemography. Clinical
diagnosis included history, physical examination, chest X-ray and electrocardiogram (ECG). Finally
a correlation between clinical diagnosis and echocardiography was deduced. Results: CHD
comprised of 2.17% of all hospital admissions, with acyanotic in 83.3% and cyanotic in 16.7%. The
sex ratio was 1.72:1. Hindus and Muslims represented 80% and 18% respectively with rural urban
ratio being 60:40. Majority of parents were illiterate’s from lower social classes. Further, maternal
age in majority of cases was between 21-30 years and paternal age between 30-40 years with
consanguinity in 23.3%. Extra cardiac malformations were observed in 13.3% of cases. Most
common mode of presentation in CHD was chest retraction and in CCHD was cyanosis. About 31%
presented with CCF. History of RRTI was noted in 31.67%. In CHD the predominant clinical sign
was a thrill, whereas in CCHD cyanosis and clubbing was predominant. An abnormal second heart
sound was noted in 47% cases and the most common murmur was an ejection systolic murmur. The
clinico echo correlation was found to be 75%, with partial correlation in 15% and non-correlated in
10%. Interpretation and Conclusion: Infants were the most common and majority males. ACHD
was more common, VSD was most common. TOF was the most common CCHD. The most
common presenting symptom in CHD was chest retraction, breathlessness. Among CCHD cyanosis,
chest retractions and breathlessness were common. A third of CHDs presented in CCF. History of
RRTI was seen in 31%. Auscultation of second heart sound was a key in a diagnosis of CHD and
was abnormal in 46.7%. Most common murmur on auscultation were ESM and LSM / PSM.
Majority were poor, illiterates from rural areas and Muslims had higher CHDs with more incidence
of consanguinity. In clinical diagnosis of CHD, chest X-ray and ECG were invaluable tools with the
use of which an accuracy of 75% was achieved. The accuracy varies with experience, age group
studied (neonates included or excluded) and type of lesion. With a small sample size of 60 random
cases from a hospital, the conclusions and interpretation have its own limitations and need to be
confirmed by larger community based studies.
CAR/16(P).EMERGENCY USE OF STENTS IN CRITICALLY ILL INFANTS <3 MONTHS
OLD: TECHNIQUES AND OUTCOMES
Kohli V, Sachdev MS, Joshi RK, Joshi R.
Pediatric Cardiology & Congenital Cardiac Surgery unit, ACAP, Indraprastha Apollo, New Delhi.
Background: Complex cardiac disease may be associated with obstruction to blood flow.
Occasionally this can be overcome with prostaglandin infusion. Surgery is a natural consequence to
use of prostaglandin. In contemporary interventional practice use of stents in newborns has
increased due to appropriate hardware becoming available. Methods & Results: Over last 18 months
5 newborns underwent stent implantation. Mean age was 42 days (range 3 days to 3 mo). Mean
weight was 4 kg (range 1.3 to 5 kg). 2/5 were premature; 2/5 were post-neonatal and 1/5 was
neonatal term baby. Stent was implanted in: PDA (duct dependent circulation) 3/5, Aorta
(coarctation) stenting 1/5, MAPCA stenting 1/5. In 5/5 stent implantation was undertaken to treat
life threatening emergency. Implantation was done via arterial sheath in 4/5 and via venous in 1/5.
Arterial sheath used included 4F Aventis in 3/4 and 3F in 1/4. Diagnoses included complex duct
dependent cyanotic heart disease 4/5 (implantation in PDA 3/4, MAPCA 1/4); VSD with
interrupted aortic arch 1/5 (implantation in aorta). 1 patient was post operative and implantation in
this patient was done on post-op day 0.Outcome: 3/5 patients had surgery done post procedure; 1/5
is awaiting surgery and 1 died post-procedure (premature, Single Ventricle of RV morphology, birth
weight 1.3 Kg). Conclusion: Stent implantation in newborn and early infancy for life threatening
emergencies is technically feasible and is a bridge to definitive surgical repair. They can be
successfully used in premature infants also though extreme premie’s do carry a higher risk.
CAR/17(P).PERICARDIOCENTESIS OF LOCALIZED COLLECTION CAUSING
TAMPONADE IN NEWBORNS
Kohli V, Joshi R.
Pediatric Cardiology & Congenital Cardiac Surgery Unit, ACAP, Indraprastha Apollo, New Delhi.
Background: Pericardiocentesis is an emergency procedure in patients with pericardial tamponade.
This procedure is usually performed under Echocardiographic guidance after due evaluation of the
fluid collection. Occasionally the collection is localized and not generalized. In these situations
pericardiocentesis may not be possible. Methods:We report 2 cases where the fluid collection was
localized to the right of right atrium in newborns and was causing tamponade. Both the effusions
were drained percutaneously by echocardiographic guidance. The evaluation of the site of drainage,
plane of drainage, determination of anticipated needle path, minimum and maximum depth of
needle insertion and range of these 2 numbers was done echocardiographically prior to drainage.
Both the procedures were uneventful. 30 ml fluid was drained in patient 1 and 50 ml in patient 2.
Etiology was postoperative collection in pt 1 and congenital leukemia in patient 2. Surgical chest
tube drainage was kept on standby as also for potential pneumothorax.Conclusion: Critical
evaluation of localized collections maybe possible percutaneously in collections to the right of right
atrium.
CAR/18(P).CORONARY ANGIOPLASTY OF LEFT ANTERIOR DESCENDING TOTAL
OCCLUSION IN A CHILD FOLLOWING KAWASAKI DISEASE
Sachdev MS, Roy V, Kohli V.
Pediatric Cardiology & Congenital Cardiac Surgery Unit, ACAP, Indraprastha Apollo Hospital,
New Delhi
Background: Percutaneous transluminal coronary angioplasty (PTCA) has an emerging role in
management of stenosis developing following Kawasaki disease.Aim: To report a successful PTCA
for chronic total occlusion of left anterior descending artery in a 4 year old with Kawasaki
Disease.Methods and Result: A 4 year old child with history suggestive of Kawasaki Disease before
4 months, not treated with IVIG presented with signs and symptoms suggestive of acute heart
failure. Electrocardiogram showed evidence of old anterolateral myocardial infarction with presence
of Q waves. Echocardiogram was suggestive of Kawasaki disease with dilated Left ventricle (LV)
with EF of 35 % with intraventricular thrombi. Coronary angiogram showed complete occlusion of
LAD. Percutaneous transluminal coronary angioplasty was performed using 1.5 mm coronary
balloon over an extrastiff coronary wire with other coronary wires placed in diagonals for support
during inflation. Following the PTCA good antegrade flow was established in the LAD with
evidence of improvement in clinical and echocardiographic parameters. 3 month follow showed
significant improvement in the LV function.Conclusion: PTCA is gradually becoming the
procedure of choice in children with acute or chronic occlusion of the coronary artery following
Kawasaki disease. It is technically challenging but a safe alternative to pedicle grafts being
performed in these patient as they allow for growth of coronary arteries.
CAR/19(P).SURGICAL IMPLICATIONS OF MAL-ALIGNMENT OF AORTA &
PULMONARY COMMISSURES IN D-TRANSPOSITION OF GREAT ARTERIES.
Sachdev MS, Joshi R, Kohli V.
Pediatric Cardiology & Congenital Cardiac Surgery Unit, ACAP, Indraprastha Apollo Hospital,
New Delhi.
BACKGROUND: Translocation of the coronary artery to the neoaorta is essential to the arterial
switch operation (ASO). Coronary origin, location of ostia and the sinus from which they arise have
a significant impact on the surgery. We prospectively evaluated the impact of commissural
malalignment (CM) on the ASO. METHODS: During pre-operative echo assessment, detailed
evaluation of the commissures of aorta and the pulmonary artery was performed and their surgical
implication anticipated. Intra-operative findings were noted and operative steps recorded.
RESULTS: CM was diagnosed preoperatively in 4 patients by echocardiography. Patterns of CM
seen were (1) bicuspid pulmonary valve (2) Minor degree of CM (<30º) (3) Major degree of CM
(>30º). CM resulted in more extensive proximal dissection of the coronary artery. Effect of CM on
implanted coronary anatomy i.e. both coronary ostia could be reimplanted in same sinus or in
separate sinuses is also described. There was no mortality associated with ASO at our centre.
CONCLUSIONS: CM can be detected preoperatively by echo accurately. The surgical planning of
the arterial switch operation is influenced by the presence of CM. Final coronary implantation
anatomy is also influenced by CM. Further studies are needed to evaluate the long-term
implications of CM.
CAR/20(O).SPECTRUM OF DIAGNOSIS, MANAGEMENT AND OUTCOME OF
TACHYARRHYTHMIA’S IN INFANCY.
Sachdev MS, Kohli V.
Pediatric Cardiology & Congenital Cardiac Surgery Unit, ACAP, Indraprastha Apollo, New Delhi.
Background: We describe the spectrum of arrhythmia in infancy and its contemporary management
in a Pediatric Cardiac Unit over an 18 month period. Methods: Data related to infants admitted with
tachyarrhythmia was collected prospectively from April 2005 to August 2006. Type of arrhythmia,
initial treatment for control, long term medications and recurrence on follow up were studied.
Results: 10 infants, aged 2 days-5 months (7/10 newborns) were admitted with tachyarrhythmia.
The surface electrocardiogram (sECG) was suggestive of ventricular tachycardia (VT) in1/10 and
SVT 9/10. The type of SVT was atrioventricular reentry tachycardia (AVRT) in 6/9; combined
AVRT and Atrioventricular node reenty tachycardia (AVNRT) in 1/9 and ectopic atrial tachycardia
(EAT) in 2/9. Structural heart disease was present in 3/10 (Ebstein’s disease, perimembranous VSD
and muscular VSD in one patient each). Tachycardia associated cardiomyopathy was present in
2/10 (1 each with SVT and VT). VT was treated with amiodarone (1/10). SVT acute control was
achieved with adenosine in 5/9. Chronic control of SVT: propanolol (5/9); propranolol with digoxin
(1/9); flecainide (1/9); flecainide with propranolol (2/9). On follow up, no recurrence was noted in
8/10. 7 of these were newborns and on single medication. 2/10 with recurrence were diagnosed
after neonatal period, were on multiple medications and one with structural heart disease.
Recurrences were controlled with increase in dose of flecainide up to 110 mg/m2. Complication of
therapy was seen in 1/10 with VT: complete heart block requiring temporary pacemaker for 2 days.
Conclusion: Newborns with tachyarrhythmia have excellent control on single medication and
usually do not have recurrence. Infants with structural heart disease; difficult to control arrhythmias
at diagnosis and diagnosis beyond neonatal period are high risk factors for recurrences.