Resection of Left Ventricular Rhabdomyoma in a Neonate

This video features a one-day-old, 3kg neonate with a prenatal diagnosis of a cardiac mass. The postnatal transthoracic echocardiogram shows a large left ventricular mass occupying the majority of the left ventricular cavity and extending into the left ventricular outflow tract (LVOT). Other small masses also appear to be in the interventricular septum and on its right side. This suggests cardiac rhabdomyoma. A cardiac magnetic resonance imaging (MRI) confirms the findings. A brain MRI then shows features suggestive of tuberous sclerosis. Because of the large size of the mass and the presence of a gradient across the LVOT, the decision is made to proceed with resection. 

Through a median sternotomy, the thymus gland is resected and the ductus arteriosus is ligated. A cardiopulmonary bypass is initiated via aortic and bicaval cannulation. Then, after antegrade cardioplegic arrest, the apex of the left ventricle is delivered out of the pericardial cavity. Stay sutures with pledgeted 5-0 polypropylene sutures are placed at the apex of the left ventricle to assist with retraction. An apical left ventriculotomy is then created away from the location of the left anterior descending coronary artery. After this, the mass is clearly visualized.  

Using a combination of sharp dissection with Potts scissors and blunt peeling of the mass, the tumor is enucleated from the left ventricular cavity. Next, the integrity of the mitral valve, its subvalvular apparatus, and the aortic valve are checked. The left ventricular apical incision is then closed in two-layer fashion. The heart is then de-aired, and the aortic cross-clamp is removed. One thing of note, the atrial level shunt is closed subtotally.  

The patient is then weaned off cardiopulmonary bypass without difficulty after transesophageal echocardiogram confirms successful surgical result. The aortic cross-clamp and the cardiopulmonary bypass times are 99 and 131 minutes respectively. The patient is then extubated on the first postoperative day, and the remaining postoperative course was overall uneventful. 

A predischarge echocardiogram shows competent aortic and mitral valves as well as a mild decrease in the left ventricular function. The patient is therefore placed on load-reducing agents. She is discharged seventeen days later and does well during her follow-up. 

References

1. Ugurlucan M, Oztas DM, Aygun E, Aliyev B, Altay AY, Ozluk Y, Omeroglu RE, Alpagut U. Giant Rhabdomyoma Requiring Emergency Resection Early After Birth. Ann Thorac Surg. 2019 Jan;107(1):e65
2. Yuan SM. Fetal cardiac tumors: clinical features, management and prognosis. J Perinat Med. 2018 Feb 23;46(2):115-121
3. Jordan CP, Costello JP, Endicott KM, Reyes C, Hougen TJ, Cummings SD, Nath DS. Intracardiac tumor causing left-ventricular outflow-tract obstruction in a newborn. J Saudi Heart Assoc. 2016 Jul;28(3):170-2

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