Diagnostic and treatment dilemma in a case of chronic progressive dyspnea

Introduction: The myxoma is the most common adult heart tumor. The pathogenesis behind myxoma is not very clear. The majority of myxomas (80-90%) are lo-cated in the left atrium, with only a few in the right atrium. Most myxomas are solitary (90%). A congenital form which consist in the existence of several myxo-mas, located distantly from the fossa ovalis has been described. Although a possible distinct ethiopathoge-nesis between the two types of myxomas was stipula-ted, their histopathological appearance is identical. In clinical practice, solitary myxoma is more common in women, the average age of discovery is 50 years old.
Echocardiography usually establishes the diagnosis. Treatment consists of the complete surgical excision of the myxoma, which is indicated both in symptomatic and asymptomatic patients, given the risk of embo-lisms and valvular obstruction (the myxoma is a his-tologically benign tumor with possible malignant he-modynamic complications).
Case presentation: We present the case of a 53-year-old female patient who was accusing dyspnea with ortho-pnea, fatigue, asthenia, symptomatology that has pro-gressively aggravated over the past two years. The pati-ent was a known smoker. She had been diagnosed with stage II essential hypertension with moderate cardio-vascular risk and chronic obstructive lung disease. The patient had no family history of cardiovascular disease. Prior to admission, she was under beta blocker therapy (Betaloc Zok 50 mg/day). Upon admission the patient was conscious, cooperative, with an altered status. She was overweight (BMI=28.02 kg/m2), cardiac ausculta-tion revealed rhythmic heartbeats, a heart rate of 70 b/ min, a BP of 130/80 mmHg, lung auscultation revealed normal airflow and a respiratory frequency of 25 r/min. The resting electrocardiogram revealed sinus rhythm, HR=70 bpm, right bundle branch block, horizontal ST depression of about 1 mm and biphasic P wave in DIII suggestive of pulmonary P wave. Blood tests revealed a non-specific inflammatory syndrome (ESR=47 mm/h), hypercholesterolemia (total cholesterol=243 mg/dL, HDL cholesterol=37 mg/dL, LDL cholesterol=170 mg/ dL) and fasting blood glucose=125 mg/dL. During ho-spitalization, blood glucose is repeated, and a diabetes consultation is required. Type 2 diabetes is diagnosed and treatment with metformin (Siofor 1000 mg/day) is initiated.
Transthoracic echocardiography at admission reve-aled: A normal size left ventricle EF=55%. A well-defi-ned echogenic tumoral mass that occupied almost the entire right atrium. The tumor resembled a myxoma, with a surface area of about 20 cm2, which caused the interatrial septum to protrude into the left atrium. The right ventricle’s size was normal with a thicker, hypere-chogenic anterolateral wall (1-1.5 cm). We also noticed a functional tricuspid regurgitation, mild secondary pulmonary hypertension and a dilated right atrium. Transesophageal echocardiography is performed re-vealing a tumorous mass located in the RA.
Contrast chest and abdomen CT scan revealed: A well-defined hypodense tumor seen with and without contrast. The tumor is homogeneous, located within the right atrium and 5,3/5,7/5,9 cm in size. No other chest abnormalities were found. Abdomen without pathological features. Surgery was indicated for the immediate removal of the right atrial mass. The patient was transferred to the cardiovascular surgery depart-ment. Surgery is performed for removing the right atri-al myxoma. Post operational status is good. This type of intervention has low morbidity and excellent prognosis in the immediate and long term. The histopathological examination confirms the diagnosis of atrial myxoma. Grossly, the tumor presented as a solitary smooth, soft polypoid mass, attached to the posterior wall of the RA, 5,7 cm in diameter, with hemorrhagic, myxoid and solid areas. Microscopically, it consisted of cords and rings of syncytial cells with abundant eosinophi-lic cytoplasm, indistinct cell borders, oval nuclei, often surrounding blood vessels, embedded in a myxoid ma-trix. The patient is discharged, stable and afebrile.
The final diagnoses of the patient are: Surgically re-moved right atrial myxoma, stage II essential hyper-tension with high cardiovascular risk, Type 2 Diabetes, first degree tricuspid regurgitation, Mild secondary pulmonary hypertension, Mild hypercholesterolemia. The patient is discharged with the following recom-mendations: low sugar, fat and salt diet. Physical effort within the limits of physical tolerance. Drug treatment: betablocker (Betaloc Zok 50 mg/day), IECA (Ramipril 5 mg/day), Statins (Sortis 20 mg/day), metformin (Sio-for 1000 mg/day). The prophylaxy of bacterial endocar-ditis with antibiotics is mandatory for any surgical or with potential of bacteremia disemination procedu-re (dental extraction, catheterization of the urinary bla-dder, etc.). Suture suppression according to the schedu-le. First reevaluation after 6 months and then annually.
We have monitored the clinical and paraclinical pro-gress within 6 months of discharge. The patient’s clini-cal condition has improved, dyspnea and fatigue have disappeared. 6-month transthoracic echocardiography shows a normal size left ventricle with EF=55%, free RA, nor-mal interventricular and interatrial septum, no Pulmo-nary Hipertension.
Particularity of the case: The particularity of this case is the neglect of the patient’s progressive symptomato-logy and the erroneous diagnosis of chronic obstructive lung disease. The progressive dyspnea described by the patient was not due to respiratory pathology but to the atrial myxoma that caused tricuspid valve obstruction and heart failure. Also, very few cases of myxomas in the right atrium have been described, most myxomas being found in the left atrium (90%). The prognosis of the patient is very good taking into consideration the completely excised solitary myxoma, which generally has a low recurrence rate (3%).
Conclusions: Our patient’s tumor was situated at the posterior atrial wall, a rare site of origination. Familial history was absent in the patient. Myxoma can present in any age group, but, as in the patient, it occurs most often between the 3rd and 6th decades of life.
Adequate excision of the myxoma, along with resec-tion of normal tissue surrounding the base, prevents recurrence. In addition, careful handling of the myxo-ma itself may prevent intracardiac implantation or pe-ripheral embolization of the tumor fragments.

ISSN – online: 2734 – 6382
ISSN-L 1220-658X
ISSN – print: 1220-658X
The Romanian Journal of Cardiology is indexed by:
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CODE: 379
CME Credits: 10 (Romanian College of Physicians)