Title : Cardiac rhabdomyoma in an adult - A case report
Abstract:
Cardiac rhabdomyoma is a hamartoma comprised of cardiac myocytes. It is most commonly diagnosed in infancy, 70 to 90% of whom have TSC (Tuberous sclerosis complex). It usually shows spontaneous regression within the first two years of life and hence is extremely rare in adults. It is usually asymptomatic. However, depending on size and location, it can cause outflow or inflow tract obstruction, tachyarrhythmia & heart failure where surgery is necessary. We present a 32year old woman presented with palpitation for which she was evaluated with TTE & Cardiac MRI showed heterogenous enhancement (5X3X2 cm) in anterolateral wall of LV which showed more in favour of Rhabdomyoma on intraoperative Frozen biopsy.
Introduction: Cardiac rhabdomyoma is a benign tumour comprised of cardiac myocytes. They are most commonly found in the ventricles, but rarely can be located in the atria. Echocardiography is the imaging modality of choice. It is usually asymptomatic. However, they can interfere with valvular function, cause outflow or inflow tract obstruction and aberrant electrical conduction leading to heart failure and lethal arrhythmias.
Case Presentation: A 52/F presented to our OP with chief complaint of on & off palpitation in the last 6 months with history of occasional chest discomfort. She denied chest pain, dyspnoea, peripheral oedema, or syncope. Vital signs were normal. Physical examination revealed a normal S1 and S2 with regular rhythm and a rate of 84/min. There was no murmur. Lungs were clear to auscultation bilaterally with no adventitious sounds. Electrocardiogram (ECG) revealed normal sinus rhythm. Echocardiography revealed a well-circumscribed (55mm x 25mm) hyperechogenic mass attached to the lateral wall of left ventricle. Left ventricular systolic function was normal. Doppler echocardiography did not show any left ventricular inflow or outflow tract obstruction. Both the atria were normal in size.The right ventricle was normal in size and function. There was no stenosis or significant regurgitation across any of the valves. MRI chest & abdomen showed Normal study.
Surgical Steps: Midlinesternotomy done - Systemic Heparinization - Aorta bicaval cannulation - Corecooled - Aorticcrossclamping - Antegraderootcardioplegia - LV opened parallel to LAD 3cm ventriculotomy incison between 1st diagonal and OM. Mass excised sent for frozen biopsy which came as Rhabdomyoma - Caretaken not to injure mitral & aortic valve appratus. Left ventriculotomy closed with felt strips using 1 o prolene - suture line reinforecd with 2nd layer of 4 o prolene - left heart deaired through root - cross clamp removed - CPB weaned off - Normal rhythm restored spontaneosuly - Post procedure shifted to ICU with stable hemodynamics with minimal inotropic support - Discharged on POD6 and on followup.
Discussion: Cardiac rhabdomyoma is usually diagnosed in infancy, with 70% to 90% of these children having TSC. (1) However, TSC-associated cardiac rhabdomyomas commonly regress spontaneously within the first two years of life, thus being extremely rare in adults. The literature review does not reveal any evidence for a potential malignant transformation, (2-5) Most commonly, cardiac rhabdomyomas are present in multiple sites in the myocardium and individual rhabdomyomas may vary in size from a few millimetres to several centimetres. They are generally asymptomatic. However, depending on size and location, these tumours can interfere with valvular function, cause inflow or outflow tract obstruction or aberrant electrical conduction leading to heart failure or lethal arrhythmias. Echocardiography with complete (5-7) Doppler study is the imaging modality of choice followed by cardiac MRI which helps with better tissue delineation. mTor inhibitors (everolimus /sirolimus) seems to be effective and partial resection is preferred if there is a high risk of damage to vital structures. Orthotopic heart transplant is considered extremely rare when tumour is so large (8). Prognosis is usually good after excision and recurrence rate is limited and routine postoperative care is necessary (9,10). Interpersonal communication between clinician and surgeon plays a vital role. Each step of management needs to be communicated to the family and requires a great team effort and performance.
Conclusion: The present case is an extremely rare presentation in adults. It is important that an early correct identification is made to avoid an unnecessarily aggressive resection, yet providing potentially curative therapy. Even though not much literature exists, we only recommend surgery if symptomatic and complications.
