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Clinical cases Internal 2018 vol. XV No.4 - www.srmi.ro 35 10.2478/inmed-2018-0028

GIANT LEFT ATRIAL MYXOMA CAUSING OBSTRUCTION AND . A CASE REPORT

Pașc Priscilla1,2 , Ioana Alexandra Coe 1 , Mircea Ioachim Popescu 1,2 1Clinical County Emergency Hospital of Oradea 2Faculty of Medicine and Farmacy of Oradea, University of Oradea Correspondent author: [email protected]

Abstract Cardiac myxoma is the most common adult cardiac tumor, with an incidence of 1: 1,000,000 in the general population. Usually occurring between 4-6 decades of life, especially in women, most of the myxomas appear sporadically and are of unknown etiology. Rare cases are genetically determined. Sudden death can occur in 15% of cases. While papillary tumors often complicate coronary or systemic thromboembolism, solid tumors, ovoids, cause failure by obstructing the mitral orifice. From a clinical point of view, the atrial myxoma may mimic a valvulopathy, , dilatation , bacterial , and may cause heart rhythm disorders, syncope, and systemic or pulmonary thromboembolism. I will present the case of a giant atrial myxoma, intermittent obstructive of the mitral orifice, evolving towards global heart failure. The lack of severe complications during the most likely long-lasting evolution represents an undiscovered curiosity. Keywords: cardiac myxoma, mitral valve obstruction, pulmonary hipertension, heart failure.

Rezumat Mixomul cardiac reprezintă cea mai frecventă tumoră cardiacă a adultului, cu o incidenă de 1:1.000.000 în populaia generală. De obicei apărute între decadele 4-6 de viaă, mai ales la femei, majoritatea mixoamelor apar sporadic și sunt de etiologie necunoscută. Rare cazuri sunt determinate genetic. Moartea subită poate apărea în 15% dintre cazuri. În timp ce tumorile papilare se complică adesea cu trombembolismul coronarian sau sistemic, tumorile solide, ovoide, determină insuficienă cardiacă prin obstruarea orificiului mitral. Din punct de vedere clinic, mixomul atrial poate mima o valvulopatie, insuficienă cardiacă, cardiomiopatie dilatativă, endocardită bacteriană și poate determina tulburări de ritm cardiac, sincope, infarct miocardic și trombembolism sistemic sau pumonar. În continuare, voi prezenta cazul unui mixom atrial gigant, obstructiv intermitent al orificiului mitral, cu evoluie spre insuficienă cardiacă globală. Lipsa complicaiilor acute severe pe parcursul evoluiei cel mai probabil îndelungate reprezintă o curiozitate încă nedeslușită. Cuvinte cheie: mixom cardiac, obstrucie a valvei mitrale, hipertensiune pulmonară, insuficienă cardiacă. 36

Clinical cases

Introduction conditions such as valvulopathy, heart failure, dilatation cardiomyopathy, bacterial According to literature data, primary cardiac endocarditis, atrial . For this tumors constitute a rarely encountered reason, the left atrium and myxoma , with an incidence ranging from should be differentiated especially by mitral 0.0017% to 0.19% in the general population. stenosis, mitral failure, cardiomyopathy, Approximately 75% of these tumors are subvalvular and valvular . benign, most of them being myxomas. Sudden death can occur in 15% of cases. Usually occurring between 4-6 decades of While papillary tumors often complicate with life, especially in women, most of the atrial coronary or systemic thromboembolism, mixes appear sporadically and are of solid tumors, ovoids, cause heart failure by unknown etiology. Rarely, they are obstructing the mitral orifice. At the time of genetically determined and autosomal diagnosis, over 50% of the patients found dominantly transmitted, for example, in have symptoms of congestive heart failure. Carney's Syndrome. Family myxoma is Other possible complications may be heart usually found in young men, with an average rhythm disorders, syncope, myocardial age of 21 years. Cardiac myxomas were also infarction. described in the child, where it occurs at a Two-dimensional echocardiography is the frequency of 6% of the child's benign heart diagnostic investigation. The treatment is tumors. surgical. Although atrial myxomas are In terms of location, they often develop in the benign, local recurrences due to inadequate left atrium, only in rare cases they are resection or malignancy are possible. detected in the right atrial cavity. Most are Occasionally, myxomas relapse away from solitary and pediculated and have frequently the initial site, the explanation for this as the attachment site the edges of the phenomenon being found in tumor foramen ovale, although they can adhere to intravascular microembolization. The risk of the wall of the atrium or posterior atrial. Mixes recurrence is greater in cases of familial vary in size, from 1 cm to over 10 cm, with a myxomas. rate of growth as variable. Cases with a growth rate of 1 cm / month were identified. Presentation of the case From a clinical point of view, the atrial myxoma symptomatology is polymorphic I will present the case of a 74-year-old man and may mimic a number of other cardiac with no cardiovascular history with bilateral Clinical cases 2018 vol. XV No.4 - www.srmi.ro 37

Figure 1. The electrocardiogram performed performed at hospital admission

Figure 2. Pulmonary radiography

Figure 3. Echocardiography performed at the admission-parasternal long axis section 38

Clinical cases

congenital blight who is presenting in the posterior mitral valve, of large size, reaching emergency room for phenomena of global 4/5 cm in diameter, which prolates heart failure manifested by dyspnea with intermittently through the mitral valve with orthopnea, decreased tolerance to effort, obstruction of the intracardiac blood flow fatigue, edema of lower limbs, oliguria. during diastole(figure 4). Also, the left and The general objective examination right atrium dilated, the left ventricle is reveals an altered general condition, undilated but with a moderately depressed important edema of the lower limbs, ejection fraction (33%). Continuous wave increased abdomen in volume, diffuse diffuse Doppler reveals a third degree tricuspid rallies in the respiratory system exam. The regurgitation with 2 jets and a moderate targeted examination of the cardiovascular secondary pulmonary hypertension. system detects tahiaritmic heartbeats, The investigations are completed with sistolic murmur at the cardiac apex and a abdominal ultrasound showing the presence blood pressure of130/81 mmHg. of small amount of (perihepatic, Laboratory tests reveal an increased perisplenic fluid , also in Morrison space). NTproBNP that advocates cardiac As for the treatment followed, the patient is decompensation, accompanied by a mild treated with at increased doses, hepatocytosis syndrome (due to liver blood resulting in increased diuresis and decreased stasis), renal impairment and mild body weight, clinically, with improvement in electrolyte disturbances. dyspnea and the remission of edema. In Emergencyelectrocardiogram records addition, digital drugs are associated for atrial , intermediate ORS axis and cardiac frequency control, and without significant ST-T changes (figure 1). (low molecular weight heparin), as the patient Pulmonary radiography, recommended in has a moderate-high risk of stroke (ChadsVasc patients with suspected heart failure to 3, Hasbleed 2). exclude pathology, highlights a slightly After stabilizing the patient, he is transferred increased bilateral peribronhovascular to the cardiovascular clinic for surgical drawing, minimal basal pleural effusion treatment. The myxoma is approached by a (figure 2). transeptal technique and is removed (figure 7, Echocardiography on admission reveals 8, 9). Durrring the same procedure, the the presence of a polilobate left atrial repair is done using “De Vega” myxoma (figure 3, 6) attached to the lateral technique (figure 10). The histopathological wall of the left atrium(figure 5), below the examination confirms the diagnosis of the Clinical cases Internal Medicine 2018 vol. XV No.4 - www.srmi.ro 39

Figure 4. Echocardiography- parasternal long axis section, showing the myxoma prolabating through the mitral orifice

Figure 5. Echocardiography- apical section- indicates the attachment site to the lateral wall of the left atrium through a pedicle that measures 13,7 mm

Figure 6. Echocardiography - apical section - highlights the polilobate form of the myxoma 40

Clinical cases

myxoma. At subsequent medical checkups, apparatus and generates stenosis or mitral the patient is clinically ameliorated, with insufficiency, creating the conditions of atrial increasing effort capacity and increasing and pulmonary pressure-overloading. Thus, quality of life. Echocardiography reveals a dyspnea is the dominant symptom that slightly improved ejected fraction. appears the earliest, initially at the effort, then becoming constant. Discussions The case presented is a special one from the point of view of its clinical similarity with a Atrial myxomas are the most common closely related mitral stenosis with natural primary cardiac tumors. Because of evolution to heart failure. Clinical- polymorphic and non-specific symptoms, pathological correlations have shown that the diagnosis is a challenge for the cardiologist. effects of mitral stenosis occur when the The left atrial mixem can be completely diameter of the tumor exceeds 5 cm. The asymptomatic in the early stages. In giant dimensions of the myxoma, which advanced stages, however, the clinical almost completely occupies the left atrium, findings of cardiac mixes are characterized by almost completely obstructs the mitral orifice the triad formed by obstruction of intracardiac during diastole, when it prolapses through blood flow, embolism and systemic the mitral valve. Its long pedal allows for manifestations. extensive movement, favoring obstruction of Congestive heart failure is the most common intracardiac blood flow. Secondary, form of clinical presentation. Depending on increases backward the pressure of the size, location, and characteristics, represented intracardiac cavities, causing progression to by the shape and type of implantation, the dilatation of the left atrium, then to the right cardiac myxoma may cause changes in cavities, eventually with the occurrence of cardiac activity. The main mechanism congestive heart failure phenomena. underlying these disorders is considered to be Factors favoring embolism in cardiac the obstruction of the intracardiac blood flow myxomas depend on both the tumor itself produced by the tumor. and the particularities of cardiac function. It The mobility and size of the cardiac myxoma has been found that some myxomas are more are characteristics that condition the emboligenic than others, depending on the intracardiac obstructive phenomena. The left characteristics of the tumor. The soft, friable, atrial location, which is the most common, with small implantation base (pediculous affects the functioning of the mitral valve tumors) and with perivalvular low septal Clinical cases Internal Medicine 2018 vol. XV No.4 - www.srmi.ro 41

Figure 7. Transeptal approach of the myxoma

Figure 8. Surgical removement of the cardiac myxoma

Figure 9. Surgical resection piece showing the polilobate form of the myxoma 42

Clinical cases

Figure 10. Tricuspid annuloplasty using De Vega technique

localization allowing large amplitude most likely long evolution. No other episodes movements is most frequently complicated of sudden health alterations that require with cerebral or systemic embolization. hospitalization have been reported ,as the Embolic events more frequently affect patient presented for the first time in his life in cerebral arteries, including retinal arteries. the emergency room.However, admission Embolisms in the pulmonary arteries, revealed an , most likely causing pulmonary infarction or acute secondary to the dilatation of the excessively pulmonary heart disease, in the visceral, overloaded left atrium. Studies in this regard renal or coronary arteries, manifested by have shown an incidence of approximately or infarction, are also possible. The 20% of atrial fibrillation associated with large multiple embolism or the detachment of the atrial myxomas. entire tumor mass and the passage into the Extremely diverse general manifestations, circulatory torrent are serious gravity such as fatigue, fever, weight loss, night situations with fatal prognosis in the short sweats, arthralgia, are supposed to be term. In our case, a curiosity of the case is the secondary to pulmonary hypertension, lack of severe acute complications during the haemolytic anemia or systemic Clinical cases Internal Medicine 2018 vol. XV No.4 - www.srmi.ro 43

microembolies. Early echocardiography plays intervention. However, the rarity of these an essential role in the diagnosis and clinical examples is not an argument for a superficial management of these patients. It allows postoperative follow-up, on the contrary, it differential diagnosis with valvular diseases advocates the requirement of regular and can estimate the transvalvular gradient. echocardiographic observation of these Only in the case of left atrial myxomas, the patients, throughout their lives. transmitral blood flow is reduced, and only the large myxomas may resemble severe mitral References 1.Najat N Mouine, Ilyass I Asfalou,Giant left atrial stenosis. In this case, echocardiography is myxoma mimicking severe mitral valve stenosis and essential, and can highlight the presence of severe pulmonary hypertension, Int Arch Med. 2013; 6: intracavitary masses. It can also make 13. differential diagnosis with other heart tumors 2.Praneet Iyer, Myo Myo Aung, A case of large atrial myxoma presenting as an acute stroke, J Community or intracardiac thrombi. In addition, it allows to Hosp Intern Med Perspect. 2016; highlight indirect signs of pulmonary 3.Maryam Sotoudeh Anvari, Mohammad Ali Boroumand , hypertension. Histopathologic and Clinical Characterization of Atrial Myxoma: A Review of 19 Cases, 01 October 2009, Surgical treatment is usually curative, as in Laboratory Medicine, Volume 40, Issue 10, 1 October our case, the patient underwent cardiac 2009, Pages 596599 surgery with a good result. Recurrent 4.Deva Japa, Mahpaekar Mashhadi, Giant left Atrial Myxoma Induces Mitral Valve Obstruction and Pulmonary myxoma is rare, between 1% and 5% of Hypertension, J Clin Diagn Res. 2016 Jan; 10(1): patients undergoing surgery develop tumor ED08ED09. recurrence. 5.Reynen K: Cardiac myxomas. N Engl J Med 1995, There are very rare cases published in the 333:16101617. 6. Swartz MF, Lutz CJ. Atrial Myxomas: Pathologic Types, literature with two recurrences, which Tumor Location and Presenting Symptoms. J Card Surg. generally occur 15-20 years after the first 2006;21(4):43540.