A 1 year female child, first in birth order, born term out of
nonconsanguineous marriage through normal vaginal delivery and had
uneventful postnatal period. She had history of recurrent episodes of
tachypnea. On examination patient was hemodynamically stable, afebrile
with heart rate of 84 beats/minutes in sinus rhythm, blood pressure of
112/65mm Hg with Spo2 of 99%. On cardiac examination there were normal
findings except soft systolic murmur. Echocardiography (echo) revealed a
single solid mass arising from papillary muscle extending into left
ventricular (LV) posterior wall upto crux of heart measuring 2.7x2.7cms
likely of Ventricular Fibroma. Cardiac MRI (CMR) revealed well defined
mass lesion of 3.9x3.8 cm arising from posterior wall of left ventricle
extending posteriorly with exophytic component showing homogenous
delayed enhancement confirming our diagnosis of ventricular fibroma. Her
Holter showed intermittent episodes of non-sustained broad complex
tachycardia suggestive of Ventricular Tachycardia. She was then
discharged on oral Amiodarone and Propanolol and kept on continuous
follow up. At 2 years of age she was readmitted with history of
palpitation and ECG done showed ventricular tachycardia. She was kept on
oral Amiodarone and Propanolol with continuous monitoring. Echo done
similar findings but the mass was gradually increasing in
size(37x35cms). Holter and ECG showed frequent episodes of wide QRS
tachycardia most likely VT,??SVT with aberrancy. Hemodynamics of the
child were maintained during the episodic cardiac arrhythmias. CMR was
repeated which showed increase in well defined mass measuring 43x42cms
arising from mid basal posterior inferior wall of LV extending
posteriorly with exophytic component . PET CT scan was done showed non
FDG avid nodular lesion in relation with left ventricle suggestive of
Fibroma. Her Thyroid profile is being monitored and is within normal
range. Sotalol has been introduced in the management of the child and
Amiodarone was withdrawn .It demonstrated transient episodes of
tachycardia which are non sustained. Child is being kept under regular
follow up
Primary cardiac tumors are rare with incidence of
0.03-0.32%.1Cardiac fibroma predominantly affects
children and is second most common benign tumor. The most common site is
left ventricle(57%) followed by right ventricle(27.5%) and
interventricular septum(17%).1Although cardiac
fibromas are asymptomatic some may present with serious complications
like arrhythmias and intracavitary obstruction. Ventricular tachycardia
have been reported in few cases of cardiac fibroma2,3which was also found in our case.
Our case was collectively discussed with pediatric cardiothoracic team,
radiology and electrophysiology meet. Surgery was deferred keeping in
view the extensive involvement of left ventricle which hindered the
cleavage plane. Few literatures recommend strategy of postponing surgery
and adequate management of ventricular tachycardia for tumors not
amenable to resection which would increase the chances of successful
surgical outcome later.2
REFERENCES:
1.Furqan A.Rajput,Faten Limaiem.Cardiac Fibroma.[Updated 2020 Apr
29].In:StatPearls[Internet].Treasure Island(FL):StatPearls
Publishing;2020 Jan-.
2.Alyssa L .Ritter,Eric J.Granquist,V.Ramesh Iyer,Kosuke Izumi.Cardiac
Fibroma with ventricular Tachycardia:An unusual Clinical Presentation of
Nevoid Basal Cell Carcinoma Syndrome.Mol Syndromol 2018;9:219-223
3.Alice Horovitz,Irene E.van Geldorp,Francis Roubertie,Jean-Benoit
Thambo.Large Right Ventricular Fibroma in a 6 month-old infant.Pediatr
Cardiol(2012) 33:1458-1460
LEGENDS
Figure 1: 12 lead ECG showing wide complex regular tachycardia
Figure 2:Two dimensional Echocardiography with apical four chamber
view(A) with posterior tilt(B) showing a single solid mass arising from
papillary muscle extending into left ventricular (LV) posterior wall
upto crux of heart.LA-Left atrium,LV-left ventricle
Figure 3: Two dimensional Echocardiography with subcostal long axis view
showing a single solid mass arising from papillary muscle extending into
left ventricular (LV) wall ,LA-Left Atrium,RA-Right Atrium
Figure 4:Contrast Cardiac MRI showing well defined mass lesion arising
from posterior wall of left ventricle extending posteriorly with
exophytic component showing homogenous delayed enhancement(A),Size of
the mass measuring 39x38mm(B)