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MYXOMA. PH SONNEKUS . LECTURE OUTCOMES. Case discussions 1-3 Definition Histology Macroscopic appearance Clinical presentation Diagnosis Management Prognosis. CASE 1. 61 yr male from Petrusburg Referred to internal medicine Pelonomi from orthopaedic ward
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MYXOMA PH SONNEKUS
LECTURE OUTCOMES • Case discussions 1-3 • Definition • Histology • Macroscopic appearance • Clinical presentation • Diagnosis • Management • Prognosis
CASE 1 • 61 yr male from Petrusburg • Referred to internal medicine Pelonomi from orthopaedic ward • Conservative Rx for a left pubic rami fracture • Workup for surgery: heartsonar revealed a mobile mass in the right atrium
CASE 1 • Medical Hx: • Hypertension • Current treatment • Ridaq and Renitec • Allergies: • none • Previous surgery: • none • Hospital admissions: • none
CASE 1 • Systemic history: • CVS: • No ortopnoea or PND • Dyspnoea NYHA gr 2 • No palipations • No oedema • Atypical chestpain-associated with heartburn • CNS: • vague history of syncope • RESP: • no complains • ABD: • no comlpains
CASE 1 • On Examination: • Vitals: • BP 170/100 mmHg • HR: 82 bpm, regular and strong • Sats: 98% • No fever • General: • no anaemia, oedema or cyanosis
CASE 1 • On examination: • CVS: • No Sx of fluid overload • Pulses felt • Apex normal 5th ICS MCL • S1 and S2 normal • No murmers • All other systems normal
CASE 1 • Spesial investigations: • Heartsonar: • mobile mass in the right atria • (?Thrombus/vegitation/myxoma) • ECG: • Left ventrucular hypertophy
CASE 2 • 62 yr Female from Winburg • Urology, December 2009 with c/o hematuria • Pre-op workup for TURBT • Presenting complaint: syncope and decreased effort tolerance
CASE 2 • Previous Medical History: • Hypertension, COPD, Ulserativecollitis, Papillary bladder tumour • Social: • 90 pack year smoking history • Current treatment: • Adalat XL, Enalapril, Inflammide and Asthavent inhalers • Allergy: • Penicillin, Sulphas, Aspirin, Elastoplast • Previous Surgery: • Appendisectomy, TURBT, Cholycystectomy, Hiatus hernia repair
CASE 2 • On Examination • Vitals • BP 125/62mmHg • HR: 78 bpm, regular and strong • Temp: 36.2 • No Sx of anaemia, cyanosis or oedema
CASE 2 • On examination: • CVS: • All peripheral pulses palpalble • No sx of fluid overload • Apex not diplaced • S1 and S2 normal • No murmers
CASE 2 • Special investigations • MRI: • LA mass 2 x 1.7 cm • CXR • TEE
SPECIAL INVESTIGATIONS • Heartsonar: • Mass in left atrium • Angiogram: • Significant stenosis of 80% on circumflex artery • Intermediate lesion on LAD
CASE 3 • 56 yr female from Phuthaditjana • Presenting Complaint: Dyspnoea NYHA gr 2 and atypical chest pain
CASE 3 • Previous Medical History: • Hypertension • Current treatment: • Pharmapress, Ridaq, Adalat XL, Slow K • Previous surgery: • none • Allergies: • None • Other: • Jehova’s witness
CASE 3 • On examination • Vitals: • BP 117/92mmHg • HR 90 bpm, irregular-irregular • Sats: 97% • General: • no oedema, anaemia, cyanosis
CASE 3 • On examination • CVS : • all peripheral pulses palpable • JVP raised • Apex 5th ICS AAL • S1 , S2 no S3 • Tricuspid incompetence 2/6 • RESP • no abnormalities • GIT • no abnormalities
SPECIAL INVESTIGATIONS • Heartsonar: • mass in right atrium Pulmonary hypertention • ECG: • Slow atrial fibrilation
INTRODUCTION • Adults • >75% of primary cardiac tumors are benign • Majority of benign lesions are myxomas
CARNEY COMPLEX • Inherited, outosomal dominant disorder • Consists of the following: • Cardiac and mucocutaneous myxomas • Lentiginosis • Endocrine dysfunction (Bilateral adrenal micronodular hyperplasia- may lead to Cushing’s syndrome)
DEFINITION • Myxo = new Latin from Greek word Muxa for mucos • A tumor of primitive connective tissue, filled with mucos
HISTOLOGY • Scattered cells with a mucopolysaccharide stroma. • Cells originate from a multipotent mesenchyme capable of neural and endothelial differentiation • Produce vascular endothelial growth factor • Angiogenisis and early stages of tumor growth
MACROSCOPIC APPEARANCE • Pendunculated • Gelatinous in consistancy • Surface: smooth, villous or friable • Vary wildly in size, ranging from 1-15cm in diameter • Weight anything from 15-189g
CLINICAL MANIFESTATIONS • Cardiovascular manifestations depend upon anatomic location. • +/- 80% of myxomas originate in left atrium, remainder are found in the right atrium.
LEFT ATRIUM • Grow into atrial lumen and cause symptoms of obstructing bloodflow or creating mitral regurgitation. • May simulate mitral valve disease • Produce heartfailure • Pulmonary hypertention
LEFT ATRIUM • Symptoms and signs • Dyspnoea • Ortopnoea • PND • Pulmonary oedema • Cough • Hemoptises • Fatigue
LEFT ATRIUM • Symptoms may worsen with change in position • Force of gravity pulls myxoma into the opening of mitral valve • Thrombo-embolic phenomena • Release tumour fragments or thrombi into systemic circulation. • Manifests as neurological or vascular symptoms
RIGHT ATRIUM • Pathology and haemodynamic implications: • Grow into atrial lumen • obstruct bloodflow • hemodynamic changes similar to tricuspid stenoses. • Thrombo-emobolic phenomena • Tumour fragments released into pulmonary circulation • Presents similar to pulmonary emboli.
RIGHT ATRIUM • Symptoms and signs • Fatigue • Peripheral oedema • Hepatomegaly • Ascitis • Prominent a waves • +/- diastolic murmer