Tricuspid Valve Hemangioma Associated with Hypoplastic Left Heart Syndrome Presenting as Sudden Infant Death Syndrome
Brijnandan GUPTA1, Shouriyo GHOSH2, Maikal KUJUR3, Khushbu KHETAN2, Tarun KUMAR2
1Department of Pathology, Lady Hardinge Medical College, NEW DELHI, India
2All India Institute of Medical Sciences, NEW DELHI, India
3Chhattisgarh Institute of Medical Sciences, Bilaspur, CHHATTISGARH, India
Keywords: Tricuspid valve, Hemangioma, Hypoplastic left heart syndrome, Sudden infant death syndrome
Primary cardiac tumors are rare in children with a low incidence varying from 0.0017 to 0.28% in autopsy studies. Approximately 90% of
the reported primary cardiac tumors in the pediatric population are benign and the most common subtype is rhabdomyomas accounting for
approximately 60%, while hemangiomas are rare primary tumors with a 5% incidence. Hypoplastic left heart syndrome is abnormal development
of the left-sided cardiac structures, leading to obstruction of blood flow from the left ventricle out-flow tract. Here we report a case of tricuspid
hemangioma in association with hypoplastic left heart syndrome, a rare association not previously reported in the literature.
Primary cardiac tumors are rare in children with a low
incidence varying from 0.0017 to 0.28% in autopsy studies
(1). Approximately 90% of the reported primary cardiac
tumors in the pediatric population are benign. The most
common subtype is rhabdomyomas accounting for
approximately 60%, while hemangiomas are rare primary
tumors with a 5% incidence (2). Hypoplastic left heart
syndrome (HLHS) is abnormal development of the leftsided
cardiac structures, leading to obstruction of blood
flow from the left ventricle out-flow tract. The syndrome
also includes underdeveloped left ventricle, aorta, and aortic
arch, along with mitral atresia or stenosis. The severity of
the degree of obstruction varies among patients (3). Here
we report a case of tricuspid hemangioma in association
with hypoplastic left heart syndrome, a rare association not
previously reported in the literature.
A female neonate was born to a 24-year-old third gravida
female at 38+6 weeks gestation by induced vaginal delivery.
The birth weight of the baby was 2.39 kg, which was
appropriate for the age. APGAR score was 8 and 9 at 1 and
5 minutes respectively. The baby cried immediately after
birth, was alert and active with stable vitals, and did not
have icterus or cyanosis. On the second postnatal day of
life, the baby was strangely found completely cyanosed at night and resuscitation was initiated. Despite all efforts,
the baby died. A complete autopsy was performed with the
clinical suspicion of sudden infant death syndrome (SIDS).
At autopsy, no external congenital abnormality was noted.
All the organs were in their normal anatomical locations.
Milk aspiration was noted in the tracheo-bronchial tree. On
dissection, a hypoplastic left heart with small left atrium,
poorly developed mitral valve, rudimentary left ventricle,
poorly developed ascending aorta and arch of aorta and
patent ductus arteriosus were noted. In addition, a brownish
hemorrhagic nodule measuring 0.8x0.6 cm was noted over
the tricuspid valve (Figure 1A-C
). No other gross anomaly
was noted in the internal organs. On histology, this nodule
was a cavernous hemangioma (Figure 1D,E
) and the
myocardium was histologically unremarkable (Figure 1F
Click Here to Zoom
|Figure 1: A) Brown nodule over the region of tricuspid valve (arrow) and dilated right side of heart, B,C) Poorly developed and
hypoplastic left heart, D) Photomicrograph of the brown nodule shows cavernous hemangioma with multiple vascular channels lined
by endothelial cells (H&E; x40), E) Higher magnification of cavernous hemangioma (H&E; x100), F) Photomicrograph showing
histologically unremarkable myocardium (H&E; x200).
Cardiac hemangiomas are rare and usually detected
during imaging or as an incidental finding at autopsy. As
observed from Table I
, there is no sex predilection with
occurrence over a wide age range. The size of the tricuspid
valve hemangiomas varies from 0.1 to 3 cm. Usually a
single lesion is identified, but rarely it can be multiple (4,5).
The mode of presentation of tricuspid hemangiomas can
vary from being completely asymptomatic to dyspnoea,
regurgitation and even severe cardio-respiratory distress
(4-8). Hemangiomas can occur in any portion of the heart including the wall of the chambers or valves and grossly
appear as red to brown hemorrhagic nodules. However,
hemangiomas at the valve region are relatively rare. On
microscopy, cavernous hemangiomas show dilated vascular
channels lined by endothelial cells. Tricuspid hemangiomas
have been detected incidentally during echocardiography
or at autopsy and in most of the cases this was an isolated
finding in the heart without other significant cardiac
pathology. The present case had simultaneous occurrence
of two extremely rare lesions of the heart, i.e. tricuspid
hemangioma and HLHS, which has not been documented
earlier in the literature. The diagnosis of a cardiac tumor
in the neonatal age group is difficult due to nonspecific
presenting symptoms. Transthoracic echocardiography is
an important diagnostic investigation for detecting cardiac
masses and is helpful in assessing the need for surgery.
Treatment of cardiac hemangiomas is primarily aimed at
surgical removal and repair. There are no data available on
the recurrence risk of cardiac hemangiomas and serial echo
studies are recommended to detect recurrence.
HLHS is a condition in which the left-sided structures (mitral
valve, left ventricle, aortic valve, aorta) are rudimentary and
inadequate to support the systemic circulation. HLHS has
been reported to occur in approximately 0.016 to 0.036% of
all live births (9). It may be associated with an intact atrial
septum or atrial septal abnormalities, the latter being crucial
for better prognosis after corrective surgery. HLHS with an
intact atrial septum is associated with dismal survival even
after corrective surgery due to its complex hemodynamics
and altered pulmonary physiology (10). As the brain and
head-neck region is supplied by an inconsistent retrograde
flow of blood from the PDA into the arch of the aorta, these
fetuses usually have microcephaly and neurodevelopmental
anomalies due to a chronic hypoxemic state both in-utero
and after birth. Early in-utero diagnosis of this condition
may help to perform timely interventions that improve
survival rates and prevent neurologic sequelae in the
survivors. The cause of death in the index case can be
attributed to HLHS. The importance of diagnosing this
condition either in the antenatal period or at autopsy is to
prevent recurrences in subsequent pregnancies, the risk
of which is considerably increased (10). The hemangioma in this case was an associated finding, and may have
aggravated the abnormal hemodynamics by causing
tricuspid regurgitation in an already compromised heart.
SIDS was ruled out in the index case in view of significant
findings in the heart. It can be said with considerable
conviction that these hemangiomas are not silent, as all
except one previously reported case were symptomatic (4-
8). In conclusion, tricuspid valve hemangioma is extremely
rare with only six cases previously reported in the literature.
The association between tricuspid valve hemangioma and
hypoplastic left heart syndrome is unique. The onus is to
develop next generation interventional fetal medicine for
the management of such cases and to improve the outcome.
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