Right ventricular myxoma – A case report Miksom desne komore

Introduction. Myxomas arising from the right ventricle are extremely rare. Case report. We presented a 71-year-old patient with worsening symptoms of the exertional dyspnea and atypical chest pains lasting 6 months. A transthoracic and transesophageal echocardiogram revealed a large, 2.6 2.2 cm, ovoid, well-circumscribed, echogenic mass in the right ventricle outflow tract attached by small pedicle, partly obstructing the right ventricular outflow tract and protruding through the pulmonic valve during systole. The tumor was completely removed with the stalk and 5 mm of the surrounding tissue. The histopathological findings confirmed the diagnosis of myxoma. Conclusion. This case illustrates the usefulness of echocardiography both in diagnosis of patients with atypical symptoms without family history and associated syndromes (like Carney’s complex), and in surgical approach planning. It also stresses the importance of surgical excision of tumor as soon as possible following the diagnosis to prevent the complications such are: valvular obstruction, pulmonary embolization and syncopes.


Introduction
Primary tumors of the heart are rare with the incidence of 0.02% to 0.05% 1 .The majority of them are benign with myxomas accounting for 50%, predominantly from the left and right atrium.Myxomas arising from the right ventricle are extremely rare [2][3][4] .
We reported a patient with myxoma located in the right ventricle outflow tract creating subsequently a partial obstruction and protruding into the pulmonic valve during the systole.

Case report
A 71-year-old retired textile worker with a long history of hypertension and smoking was hospitalized because of worsening symptoms of exertional dyspnea and chest pains (piercing duration of several seconds) atypical for angina pectoris that lasted 6 months.Clinical examination revealed decreased breath sound, regular heart rhythm with resting bradicardia of 48 beats per minute, diminished heart sounds, mild (1/6) systolic murmur at the left upper sternal border (which did not change with position or respiration), and hypertension with blood pressure (BP) 160/90 mmHg.Other clinical findings were normal.
The results of routine blood tests (as well as D-dimer) were within the normal range except erythrocyte sedimentation rate (24 mm/h), fibrinogen (4.6 g/L.) and C-reactive protein (11.6 mg/L).
The ECG showed sinus bradycardia and the signs of early depolarization.
The chest x-ray was normal.
The transthoracic echocardiogram revealed a large, 2.6 2.2 cm, ovoid, well circumscribed, echogenic mass in the right ventricle outflow tract which was attached by small pedicle, partly obstructing the right ventricular outflow tract and protruding through the pulmonic valve during each systole (Figure 1).The right ventricle was not enlarged; pulmonary artery and its branches were not dilated.Apart from the aortic valve sclerosis, other findings were normal.
Transesophageal echocardiogram clearly revealed mobile tumor with the stalk arising from the right ventricular outflow tract.
Ventilation/perfusion scanning confirmed pulmonary embolism (hypoperfusion in the apical part of the left lung).
Multidetector Computed Tomography of the pulmonary artery did not show intraluminal thrombotic masses of the main pulmonary artery and its branches.
Color duplex scan of lower extremities veins, the pelvis and abdomen showed normal findings.
Due to the patient's age and the existing chest pain coronary angiography was required, and duly performed.Nevertheless, coronary angiogram was normal.
The patient was operated on using the extracorporeal circulation.Cardiopulmonary bypass was instituted with the bicaval cannulation; returning blood to the ascending aorta.Both venae cavae were snared, and longitudinal right ventriculotomy was performed.The tumor was excised together with the stalk and 5 mm of the surrounding tissue.Tumor basis was thermocauterized, and thereupon the right ventricular wall was sutured.The patient was weaned from cardiopulmonary bypass and the chest was closed routinely.Postoperative course was uneventful.Pathologically, the macroscopic specimen demonstrated the ovoid mass weighing 12 g, 3.0 2.5 2.0 cm in diameter, with hemorrhagic areas.The histopathological findings confirmed the diagnosis of myxoma (Figure 2).

Discussion
Primary heart tumors are rare and the majority of them are atrial myxomas.Only sporadic cases of myxomas arising from the right ventricular outflow tract have been reported in the literature [5][6][7] .In a series of 81 myxomas operated in our institution during the period of 29 years, this is the first patient with right ventricular myxoma.
Clinical symptoms of myxomas depend on its position and size, are atypical and vary to a large extent in terms of general symptoms, heart symptoms and embolic events.The majority of patients have a variety of different and atypical symptoms.That is why some authors call myxomas "the great masquerader".Asymptomatic cases are rare.Screening for myxoma is important in cases of family history, in complex myxoma, or Carney disease 8 .Our patient had atypical symptoms and the diagnosis was made by echocardiography.We would like to stress the importance of this diagnostic tool, especially the importance of transesophageal echocardiography in determination of myxomas attachment, anatomical relationship and planning of surgical approach.
Our patient had signs of pulmonary microembolism and partial pulmonary valve obstruction in each systolic phase.Surgical excision of right ventricular myxomas must be accomplished as soon as possible after the diagnosis has been established to prevent serious complications such are valvular obstruction, pulmonary embolization and syncope.
Surgical intervention offers the cure for patients with sporadic intracardiac myxomas.Familial myxomas have greater tendency to recur, even many years after the operation.Although only several cases of recurrence of the right ventricular myxoma have been reported so far 9, 10 routine echocardiography control after the operation is advised.

Conslusion
The presented case illustrates the usefulness of echocardiography both in diagnosing and planning surgical approach in patients with the myxomas.It also stresses the importance of surgical excision of the tumor as soon as possible following the diagnosis establishing to prevent serious complications such are valvular obstruction, pulmonary embolization and syncopes.

Fig. 2 -
axis view -showing a large echogenic mass in the right ventricle; b) Short axis view -showing the large, ovoid tumor mass in the right ventricle attached to the outflow tract; c) Short axis view -showing the tumor protruding into the main pulmonary artery in systolic phase.Right ventricular myxoma a) Intraoperative photograph during resection; b) Histological findings (HE, 400).Strana 611 Obrenovi -Kir anski B, et al.Vojnosanit Pregl 2013; 70(6): 609-611.