S14 indian heart journal 67 (2015) s12–s16

Neonates (up to 1 month)

Acyanotic (n = 233, 89.96%) Cyanotic (n = 26, 10.04%)

Acyanotic (n = 684, 84.65%) Cyanotic (n = 124, 15.35%) Barkatpura, Hyderabad 500027, India

VSD 318 (46.49%) TOF 72 (58.06%) Introduction: Tetralogy of Fallot (TOF) is the most common cause
ASD 146 (21.35%) TGA 12 (9.68%) of cyanotic congenital heart disease in ventricular septal defect
Above 1year and pulmonary stenosis physiology. TOF is associated with many
syndromes, the most common being Down syndrome, Velocardio-
Acyanotic (n = 825, 83.69%) Cyanotic (n = 160, 16.31%) facial syndrome, Holt Oram syndrome, and Poland's syndrome.
Incidence of VACTERL syndrome is rare compared to the above
VSD 306 (37.27%) TOF 120 (75.0%) mentioned syndromes.
ASD 160 (21.35%) TGA 8 (5.0%) Case report: A 12-year-old boy was brought with complaints of
PDA 92 (11.21%) DORV 12 (7.5%) shortness of breath and palpitations on exertion. He had history of
squatting episodes. Prenatal and perinatal history in mother was
normal. He attained developmental milestones on par with his
peers. His weight was <2SD for his age, mid arm circumference –
13 cm. On examination he had dysmorphic features – left absent
VSD device closure with PDA device
thumb, kyphoscoliosis, low set ears, low hair line, hypoplastic mand-
ible, deciduous teeth, radial hypoplasia with pandigital clubbing, and
Manoj Chopda JVP was normal in height and waveform. Cardiovascular examination
Nashik, Maharashtra 422005, India – LV apex, parasternal heave 3/3, systolic thrill left 2,3 ICS.S1 normal,
S2 normal split, Grade 5/6 MSM at left 2nd and 3rd ICS selective
conduction to left side. He was evaluated. Hb 13.5 gm/dl, WBC counts
Background: Transcatheter closure of ventricular septal defect
were within normal limits. The following are noted, ECG – normal
using PDA devices is gaining acceptance in selected cases as
sinus rhythm; extreme right axis deviation 1508; RVH with strain;
compared to surgical closure which is associated with morbidity
Katz Wachtel phenomenon; Chest X ray – oligemic lung fields; and no
and mortality. Potential advantages of the transcatheter closure
cardiomegaly. ECHO showed situs solitus, large subaortic ventricular
over conventional surgery include a smaller incision, shorter stay,
septal defect with overriding of aorta <50%, with severe valvular
and fewer complications.
pulmonic stenosis and subvalvular infundibular dynamic obstruc-
Methodology: A total 78 cases underwent echo and cardiac cath.
tion. The gradients across the pulmonic valve were 92/63 mmHg and
Out of 78 patients, 10 patients sent to surgery because of improper
at the infundibulum was 53/14 mmHg. Pulmonic valve was doming.
size or location of the VSD. A total of 68 cases were having defect
RVH was present with free wall thickness of 0.7 cm. Based on the
size ranging from 3 mm to 10 mm. The location of VSD was
dysmorphic features, he was categorized as syndromic TOF.
perimembranous in 60 cases, muscular in 08 cases, and apical
Discussion: VACTERL is an association of nonrandom occurrence
in 01 case. The procedure was performed in 68 cases with PDA
of Vertebral, Anorectal anomalies, Cardiac anomalies, Tracheoe-
device.
sophageal anomalies, Radial hypoplasia/renal anomalies, and
Procedure: LV angiography showed VSD. LIMA catheter was passed
limb defects. All features need not present at a single time for
in LV with rotation and pull-back technique, then crossed VSD and
diagnosis. According to Genetic Home Reference and National
was placed in RV. Double length Terumo 0.03500
260 cm wire was
Library of Medicine (ghr.nlm), three anomalies ought to be present
taken and passed through RV, RA, IVC, SVC, or PA. Snared through
for labelling a patient to have VACTERL association. The present
femoral venous and arterio venous loop was created with the help
patient had Vertebral anomalies (Scoliosis), Cardiac anomalies
of Terumo wire. PDA sheath was passed through RA, RV and then
(TOF), Renal anomalies (left kidney shrunken), Radial hypoplasia,
into aorta with Kissing technique sheath was positioned in LV. PDA
absent thumb. The close differential diagnosis for this patient was
device was loaded in sheath. Device was positioned on LV side first
Holt Oram Syndrome, it can be excluded in individuals with con-
and then on RV side. Position was checked on 2D-Echo which was
genital malformations involving – ulnar ray only, kidney, vertebra,
found to be accurate. Wire and catheter was removed. Post pro-
craniofacies, auditory system (hearing loss or ear malformations),
cedure treatment with steroids was advised as per standard treat-
lower limb, anus, or eye.
ment. Anti-platelet aspirin was recommended for one month.
Clinical implications: TOF association with VACTERL is rare and
Results: Procedure was done successfully in 66 without any com-
hence reported.
plications. In 15 cases, transient BBB was developed on during
procedure. In 1 case, complication of CHB occurred during proce-
dure in which temporary pacemaker (TPM) was implanted for 3
days. In another case, complication of CHB occurred in ICU after 3 h
A study of incidence and pattern of
in which TPM was done for 2 days. In 2 cases, device was embo-
lized, 1 device successfully retrieved with help of the basket snare coronary artery anomalies in western
and continued procedure with bigger size device, another 1 case Rajasthan
went to surgery. A total of 1 case of death was observed on 5th day,
which was due to stroke. At 1-year follow-up, no shunt was noted L. Satish *, Sanjeev Sanghvi
in ECHO and ECG remained same with no additional conditional
defect. MDM Hospital Campus, Shastri Nagar, Jodhpur, Rajasthan 342003, India
Conclusion: Transcatheter closure is safe and efficacious in
selected cases of perimembranous and muscular VSD using PDA Background and objectives: Coronary artery anomalies are a
device. diverse group of congenital disorders whose manifestations and