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Resection of ventricular rhabdomyomas in infants presenting with cardiac failure

Published online by Cambridge University Press:  01 December 2008

Narendra R. Dereddy ,
Suvro S. Sett  and
Usha Krishnan
Narendra R. Dereddy
Affiliation:
Department of Pediatrics, Maria Fareri Children’s Hospital, Westchester Medical Center, Valhalla, New York, United States of America
Suvro S. Sett
Affiliation:
Department of Cardiovascular Surgery, Maria Fareri Children’s Hospital, Westchester Medical Center, Valhalla, New York, United States of America
Usha Krishnan*
Affiliation:
Department of Pediatric Cardiology, Maria Fareri Children’s Hospital, Westchester Medical Center, Valhalla, New York, United States of America
*
Correspondence to: Usha Krishnan, MD, Division of Pediatric Cardiology, 618, Munger Pavilion, New York Medical College, Valhalla, NY 10595, USA. Tel: +1 (914) 493 8372; Fax: +1 (914) 594 4513; E-mail: usha_krishnan@nymc.edu
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Abstract

Rhabdomyomas are the most common cardiac tumours in children. They sometimes cause significant obstruction of the ventricular out flow tracts. We report a series of 3 neonates diagnosed antenatally with multiple rhabdomyomas, who developed significant obstruction of the ventricular outflow tracts following birth. They underwent surgical resection in the neonatal period with good outcome. Antenatal diagnosis of obstructive cardiac tumours allows for planning for appropriate early intervention.

Keywords

Cardiac tumourstuberous sclerosisventricular outflow tract obstruction
Type
Brief Report
Information
Cardiology in the Young , Volume 18 , Issue 6 , December 2008 , pp. 635 - 637
Copyright
Copyright © Cambridge University Press 2008

Rhabdomyomas are the most common cardiac tumours in children, and are usually associated with tuberous sclerosis. They are benign tumours that regress spontaneously, and usually do not require any treatment unless they cause obstruction to the ventricular outflow or inflow tracts, or disturbances of conduction. We report a series of 3 neonates diagnosed antenatally with multiple cardiac rhabdomyomas causing obstruction to the ventricular outflow tracts, which were successfully resected in the neonatal period with excellent results.

Case reports

Baby R was a 4.1 kg, full-term female baby born to a 20 year old mother. Fetal echocardiography during the second trimester had shown multiple right ventricular tumours. Echocardiography after birth showed multiple masses in the right ventricular outflow tract, with obstruction and moderate tricuspid regurgitation (Fig. 1). The baby gradually developed poor feeding with an enlarging liver. Repeated echocardiography at 1 week showed severe obstruction across the right ventricular outflow tract, with a gradient of 80 mm. The tumour was resected through a pulmonary arteriotomy under cardiopulmonary bypass, and the patient made a rapid recovery. Cerebral magnetic resonance imaging showed bilateral frontal cortical tumours, confirming the diagnoses of tuberous sclerosis. Over the subsequent 6 months, her cardiac tumours have regressed, with no residual gradient across the outflow tract.

Figure 1 Subcostal echocardiogram view (a) showing the tumour in the right ventricular outflow tract, with colour flow interrogation (b) showing acceleration at the site of obstruction.

Baby V was born at term, to a primigravid 25 year old, and had been shown by prenatal echocardiography to have multiple intracardiac masses producing severe obstruction of the left ventricular outflow tract. She also had episodes of fetal supraventricular tachycardia. Due to severe respiratory distress, she was placed on a ventilator soon after birth. The atrial tachycardia was refractory to multiple medications. Echocardiography revealed multiple tumours in the left ventricle, the largest one in the ventricular septum obstructing the outflow tract (Fig. 2), producing a gradient of over 60 mm. In view of possible life-threatening aortic obstruction, she was referred for surgical resection. Under cardiopulmonary bypass, a portion of the septal and valvar tumours obstructing the left ventricular outflow tract was removed. The postoperative period was complicated by recurrent supraventricular tachycardia, which required the addition of flecainide for treatment. Cerebral magnetic resonance imaging showed bilateral subependymal hamartomas along the lateral ventricles and bilateral frontal cortical tumours consistent with the diagnoses of tuberous sclerosis. After 1 year of follow-up, most of her tumours have regressed, and she is being weaned off anti arrhythmic medication.

Figure 2 Parasternal long-axis echocardiogram view (a) showing tumours in the left ventricular outflow tract, with color flow interrogation (b) showing acceleration at the site of obstruction.

Baby S was a full-term baby boy, weighing 4 kg, born with an antenatal diagnosis of multiple cardiac tumours. Postnatal echocardiography showed multiple cardiac tumours, with one of them causing severe obstruction of the left ventricular outflow tract. Due to increasing gradient, the tumour was resected through an aortotomy at 10 days of age. She had no aortic regurgitation post operatively and her tumours were not echocardiographically evident at six months of follow-up. Cerebral magnetic resonance imaging showed subependymal as well as cortical tumours in the left middle frontal gyrus, confirming the diagnoses of tuberous sclerosis.

Discussion

The prevalence rate of cardiac tumours was 0.14% in pregnancies referred for fetal echocardiography, with rhabdomyomas accounting for nine-tenths.Reference McAllister1, Reference Holley, Martin and Brenner2 They are usually multiple, in nine-tenths of cases, and extend into the cardiac cavities in half. They always involve the left ventricle, with right ventricular involvement in four-fifths. They are associated with tuberous sclerosis in up to four-fifths of cases.Reference Beghttti, Gow, Haney, Mawson, Williams and Freedom3–Reference Smith, Watson, Patel and Super6 Children may be totally asymptomatic, or may present variously with congestive cardiac failure, low cardiac output due to intracardiac obstruction to flow, arrhythmias of various types, or with sudden death.Reference Muhler, Kienast, Turniski-Harder and Von Bernuth7, Reference Ibrahim, Thakker, Miller and Barron8 Surgical intervention is indicated in the event of haemodynamic compromise, as was seen in all our 3 cases. Even if multiple tumours are present, only the haemodynamically significant ones need to be resected, as the other tumours regress spontaneously.Reference Black, Kadletz, Smallhorn and Freedom9 In all 3 of our patients, surgery was effective in providing complete relief of obstruction, with regression of tumours on follow-up. All patients, however, remain on anti-seizure therapy and early intervention for their neurological problems.

Acknowledgement

We thank Michael Scotton, our echocardiographer, for his help in retrieving and processing the images.

References

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Figure 0

Figure 1 Subcostal echocardiogram view (a) showing the tumour in the right ventricular outflow tract, with colour flow interrogation (b) showing acceleration at the site of obstruction.

Figure 1

Figure 2 Parasternal long-axis echocardiogram view (a) showing tumours in the left ventricular outflow tract, with color flow interrogation (b) showing acceleration at the site of obstruction.