1. Abstract Case Summary Conclusion/Discussion References
A case of multiple intracardiac rhabdomyomas in the Left ventricle causing left ventricle outflow tract obstruction.
Resham Kaur, PGY 3, Driscoll Children’s Hospital
Dilli Bhurtel, PGY 3, Driscoll Children’s Hospital
John Brownlee, MD, Pediatric Cardiology, Driscoll Children’s Hospital
Introduction
Patient was found to be positive for Tuberous Sclerosis gene with no family history of tuberous sclerosis. Neurology and ophthalmology evaluation revealed a normal head ultrasound and normal eye exam. Nephrology evaluation revealed echogenic kidneys with no other renal abnormality. Patient was discharged with appropriate follow up with these specialties.
Intracardiac tumors in the newborn period are mostly rhabdomyomas. These are common in patients with tuberous sclerosis. These are the common primary cardiac tumor. These can be diagnosed in the prenatal period by the fetal echocardiography. Sudden death, including still births, has been attributed to cardiac rhabdomyomas. Natural history is marked by spontaneous regression of the tumors.
Abstract
Multiple intra-cardiac rhabdo-myomas were identified on the prenatal sonogram of a preterm infant. Patient was found to have left ventricle outflow tract obstruction and hemodynamic compromise at birth. Here we describe the management of the case where favorable outcome was achieved with the medical management despite the indication for surgery initially.
Case Summary
A 33 week preterm male, 1600 grams at birth was born to a 35 y/o mother with unremarkable prenatal serology. Pregnancy was complicated by hypertension and fetal intra-cardiac masses on prenatal sonogram. Apgar scores at birth were 8 and 9 at 1 minute and 5 minutes respectively. Patient was symptomatic at birth with significant metabolic acidosis and inadequate systemic blood flow. Patient was started on the prostaglandin drip at birth to support the ductal dependent systemic flow and was intubated.
Echocardiogram at birth showed multiple pedunculated tumors in both ventricles. There were two masses in the left ventricle that were creating intermittent left ventricular outflow tract obstruction in a ball valve mechanism. No right ventricular outflow tract obstruction, no mitral stenosis or tricuspid stenosis was found on the echocardiogram. There was a small patent foramen ovale and tiny mid muscular ventricular septal defect with left to right shunting. Large patent ductus arteriosus (PDA) was present with bidirectional shunting. There was normal biventricular systolic function.
Due to the high risk of cardiopulmonary bypass in this age/weight range, much higher than the risk of one or more of the lesions breaking off and embolizing, it was decided to observe and closely follow the patient on prostaglandin till the patient achieved a weight 2 kg or the tumor was regressing in size.
Patient was extubated at four days of age to four liters per minute of oxygen and was subsequently weaned off to room air with saturations above 90%. Prostaglandin was discontinued on day of life seventeen after the serial repeat echocardiograms showed regression in the tumor size and decreased obstruction to the left ventricle outflow tract and patient was seen to have good perfusion off the prostaglandin, after the PDA closed. .
Parasternal long axis view LVOT mass (A) has regressed in size (5x6mm) Pressure gradient across aortic valve was 36/22 mm HG.
Conclusion/Discussion
Rhabdomyomas constitute 45% to 80% of all primary cardiac tumors in the pediatric age group. These are the most common primary cardiac tumor. These can be diagnosed in the prenatal period by the fetal echocardiography. Sudden death, including still births, has been attributed to cardiac rhabdomyomas. Natural history is marked by spontaneous regression of the tumors.
Usually rhabdomyomas cause right ventricular outflow tract obstruction but in our patient, rhabdomyomas caused left ventricular outflow tract obstruction causing significant symptoms. The indications for surgery include spontaneous regression and intractable arrhythmias. Although our patient had significant hemodynamic compromise at birth and surgery was considered but due to prematurity and increased risk of surgery, watchful waiting till patient achieved a significant weight of 2 kg was done and in the interval, significant regression of the tumor was seen and hence surgery was not done. The main learning point from this case report is that even if the patient has hemodynamic compromise with rhabdomyomas, watchful waiting with ensuring adequate systemic blood flow is still an option.
References
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Long axis parasternal view showing multiple intra-cardiac masses and a large mass (9 X 8 mm) size- obstructing LVOT.
Apical anterior view showing LVOT obstruction pressure gradient across was 51/37 mm Hg
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