QJM Advance Access published July 24, 2016 QJM: An International Journal of Medicine, 2016, 1–2 doi: 10.1093/qjmed/hcw106 Advance Access Publication Date: 10 July 2016 Clinical picture
CLINICAL PICTURE
Left atrial myxoma any of the heart cavities but 75% occur in the left atrium.1,2 She was having heart failure symptoms as the tumor was obstructing the mitral valve, simulating mitral stenosis physiology. Myxomas could also cause acute paroxysmal dyspnea or syncope from ‘ball-valve blockade’ of mitral orifice or mitral insufficiency from interfering with the mitral valve closure or damaging mitral leaflets.2 Although other non-invasive modalities such as computed tomography and magnetic resonance imaging could be used to confirm the diagnosis of myxoma, transthoracic echocardiogram is often enough to diagnose myxoma when the stalk (Figure 1, white arrows) is visualized arising from interatrial septum. Transthoracic echocardiogram also helps in differentiating from other pathologies mimicking myxoma such as thrombus, other primary tumors and metastasis.1 She improved with supplemental oxygen, intravenous nitroglycerin and diuretic therapy. Family agreed for surgical removal as she was having frequent heart failure admissions. She underwent resection of left atrial myxoma with reconstruction of atrial septum with pericardial patch. Routine coronary angiogram prior to surgery showed normal coronaries with feeding
Figure 1. Echocardiogram showing large left atrial myxoma prolapsing into left ventricle during diastole (white arrows pointing to the stalk) LV: left ventricle; LA: left atrium.
Figure 2. Left coronary angiography showing normal coronaries with feeding vessels (white arrows pointing) to myxoma from left circumflex.
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An 81-year-old independently functional woman with a history of congestive heart failure presented with acute shortness of breath. The day prior to the presentation she complained of mild shortness of breath while having dinner but went to sleep and woke up early morning with difficulty in breathing and chest discomfort. Prior to this, she was hospitalized twice for heart failure in the last 3 months and was also diagnosed with left atrial myxoma. However, her family members refused surgery. She has no constitutional symptoms such as fever, fatigue, weight loss, etc. She was in respiratory distress (26 breaths per minute), hypoxic, saturating 88% on room air and tachycardic (120 beats per minute). She had elevated jugular venous pressure to 15 cm H2O, diffuse bilateral rales on lung auscultation, early diastolic murmur (tumor plop) at apex on precordium auscultation and one plus bilateral pitting edema. No signs of systemic embolization were found. Bedside echocardiogram showed large atrial myxoma measuring 6.68 4.16 cm filling entire left atrial cavity during systole and prolapsing into left ventricle during diastole (Figure 1) with significantly elevated pulmonary artery hypertension (45 mm Hg). Left ventricular systolic function was mildly reduced. Myxomas are the most common benign tumors of the heart and account for nearly fifty percent of all cardiac primary tumors.1,2 They can be found in
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vessels (Figure 2, white arrows) to myxoma from left circumflex. Histopathology confirmed the diagnosis. She did well during postoperative stay and was discharge to rehabilitation facility on eighth postoperative day. Surgery is the treatment of choice and usually indicated due to the potential for embolism or obstruction of the mitral valve orifice.1 In most cases, myxomas are solitary, but in their familial form, they can be multiple and recurrent.3 Carney’s syndrome is an autosomal dominant disorder characterized by myxomas in heart, skin and breast, spotty skin pigmentation (lentigines and blue nevi), endocrine tumors (adrenal, testicular, thyroid and pituitary) and peripheral nerve tumors (schwannomas).3
Photographs and text from: V. Namana, Department of Cardiology, Maimonides Medical Center, 4802 10TH AVE, Brooklyn, NY 11219, USA; R. Sarasam, Department of Medicine, Baystate Medical Center, 759 Chestnut St, Springfield, MA 01199, USA; R. Balasubramanian and J. Shani, Department of
Cardiology, Maimonides Medical Center, 4802 10TH AVE, Brooklyn, NY 11219, USA email:
[email protected] Conflict of interest. None declared.
References 1. Reynen K. Cardiac myxomas. N Engl J Med 1995; 333:1610–7. 2. Paulsen W, Nolewajka JA, Boughner RD, Kostuk WJ, Heimbecker RO, Mckenzie FN. Left atrial myxoma: report of six cases and review of the literature. CMA Journal 1980; 123:519–22. 3. Stratakis CA, Kirschner LS, Carney JA. Clinical and molecular features of the Carney complex: diagnostic criteria and recommendations for patient evaluation. J Clin Endocrinol Metab 2001; 86:4041–6.
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