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Gülhis Batmaz*, Resmiye Besikçi**, Gülseren Arslan***,Ihsan Kafadar****, Gülay Ahunbay*****. Spontaneous Regression of Huge Cardiac Rhabdomyoma in An Infant. Images Paediatr Cardiol 2000;5:4-10 |
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Associate Professor in Pediatric Cardiology Unit, University of Istanbul Cerrahpasa Medical Faculty, Cerrahpasa Istanbul |
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Medical Doctor in Pediatric Cardiology Unit, Baksrköy SSK Hospital, Istanbul |
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Medical Doctor in Pediatric Neurology Unit, Baksrköy SSK Hospital, Istanbul |
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Medical Doctor in Pediatric Neurology Unit, University of Istanbul Cerrahpasa Medical Faculty Cerrahpasa Istanbul |
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Professor in Pediatric Cardiology Unit, University of Istanbul, Cerrahpasa Medical Faculty, Cerrahpasa Istanbul |
| Neonate | Rhabdomyoma | Tuberose sclerosis |
Article
Introduction
Rhabdomyomas are the most common primary cardiac tumors in childhood.1-3
This neoplasm may be associated with tuberous sclerosis tuberose sclerosis
which is an autosomal-dominant disaese affecting the brain, skin, kidney,
heart and characterised by infantile epilepsy, mental retardation, facial
adenoma sebaceum. Although the indication of surgical resection of symptomatic
tumors is well established,4 medical follow-up is prefered unless
critical obstruction or dysrhythmias are present.5 We describe
a newborn with a huge cardiac rhabdomyoma in the left ventricular outflow
tract that initially produced moderate obstruction, but resolved spontaneously.
Case Report
A male neonate aged four hours was admitted
to Cerrahpa?a Medical Faculty because of a heart murmur detected two
hours earlier. Antenatal history and delivery were uneventful and birth
weight was 4370g. Physical examination was normal except for a grade 3/6
systolic ejection murmur which was heard maximally at the left sternal
border in the third intercostal space. Heart rate was regular at 126/min
and blood pressure was 65 mmHg in the right arm.
Echocardiography showed normal chamber dimensions and anatomy, along
with a spherical mass originating from the mitral valve, just below the
aortic valve, interposed between the ventricular septum and mitral valve
anterior leaflet. Multiple additional masses were present within the left
and right ventricular cavities (figures 1 and 2). The location, number
and size of each tumor were:
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Neurologic and radiologic features of tuberose sclerosis appeared, with
convulsions and subependymal hamartomas at 6 month of age. But cardiologically
the patient has remained asymptomatic. Follow-up investigations showed
spontaneous regression of the tumors within eight months and the outflow
gradient disappeared completely (figure 5). The convulsions were controlled
medically and physical and neurologic development appear appropriate for
age.
Discussion
Rhabdomyoma is the most common heart tumor in infancy. A clinicopathologic
study showed that such tumors are usually multiple (92%), often intracavitary
(50%), and occur more frequently in the left ventricle than in the right
ventricle (100 % vs 81 %).2 Cardiac rhabdomyomas are frequently
associated with tuberose slerosis,6 with a prevalence of 30-
80%.1- 3, 7-12
The other primary heart tumours at this age (hamartomas, myxomas and fibromas) differ both clinically and ultrasonographically from rhabdomyomas. As most rhabdomyomas are multiple, the diagnosis is generally beyond doubt, and may be made even in the absence of histologic confirmation.13 Multiple intracavitary tumours are considered as an important marker of tuberose sclerosis, even in antenatal period.14 Multiple tumors, or a single tumor plus involvement of other organs (e.g. central nervous system, kidney, skin) that are compatible with the diagnosis of tuberose sclerosis, or a single tumor with positive family history of tuberose sclerosis, is highly suggestive of rhabdomyomas.1
Cardiac rhabdomyomas may be asymptomatic, or may cause a variety of clinical symptoms depending on their size and location.3,15 In the majority of cases, symptoms occure at an early age or even before birth.6,14 The spectrum of clinical manifestation ranges from cardiac murmur to sudden death.5 The presenting symptom may be arrhythmia, cardiac murmur, complete or variable atrioventricular block, pericardial effusion, cardiomegaly, cardiac failure or sudden death.3, 6, 10, 17 The variety of symptoms can be explained on the basis of obstruction of blood flow, myocardial involvement and disturbance of the cardiac rhythm.
The value of surgical resection of symptomatic tumours is well established.4 While most rhabdomyomas appear to regress spontaneously, some infants may benefit from surgery for obstructive lesions at an early stage.13 With surgery, it is possible to remove obstruction or an arrhythmogenic substrate. Rarely, even heart transplantation may be indicated in patient with severe myocardial involvement.18 But surgical intervention is neither possible nor indicated in every child.19 Consequently, a conservative approach is preferrable and useful in most cases. Unless critical obstruction or dysrhythmias is present, medical follow-up should be preferred 5 since these tumours demonstrate benign pathological characteristics and tend to regress over time.1,12, 13,15,19,20 The chance of spontaneous regression does not depend on the initial size, number or location of tumours.15 Partial or complete spontaneous regression of rhabdomyomas has been reported in 54% of cases.1 This regression may take place in a period as short as three weeks.1
Echocardiography is useful in determining tumour size, number and location. It is also a useful diagnostic method for evaluation of hemodynamic consequence of tumours. Serial echocardiographic studies are both useful and safe in monitoring tumour size, and they provide acceptable follow-up information.21
References:
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