How to cite this article: Tempe DK, Dutta D, Minhas H, Garg M, Virmani S. A rare case of myxoma in the right ventricular outflow tract extending to the pulmonary artery. Ann Card Anaesth 2010;13:167-8
How to cite this URL: Tempe DK, Dutta D, Minhas H, Garg M, Virmani S. A rare case of myxoma in the right ventricular outflow tract extending to the pulmonary artery. Ann Card Anaesth [serial online] 2010 [cited 2022 May 19];13:167-8. Available from: https://www.annals.in/text.asp?2010/13/2/167/62944
A 22-year-old man was referred to our hospital with a one-month history of dyspnea on exertion and chest pain. On admission, his pulse rate was 80 / min and blood pressure was 116/70 mmHg. He was afebrile with no signs of congestive cardiac failure. On physical examination, ejection systolic murmur was present in the pulmonary area. The electrocardiogram (ECG) showed normal sinus rhythm at 80 beats/minute; ST segment depression in leads V1-V6 with right ventricular hypertrophy and right axis deviation. Transthoracic echocardiography (TTE) revealed a large mass extending from the right ventricular outflow tract (RVOT) to the pulmonary artery (PA) bifurcation with dilatation of the right atrium (RA) and right ventricle (RV). Mild tricuspid regurgitation (TR) and severe pulmonary arterial hypertension (PAH) with right ventricular systolic pressure (RVSP) of 90 mm Hg were also present. Abdominal ultrasound and venous Doppler of both lower limbs were normal.
The patient was scheduled for excision of the RVOT and PA mass via midline sternotomy using cardiopulmonary bypass. Intraoperative transesophageal echocardiography (TEE) confirmed the large mobile mass (2.10 Χ2.87 cm) in the RVOT [Figure 1]. The peduncle or its attachment was not clearly visible. The main PA was also occupied with the mass with a turbulent flow across the RVOT [Figure 2]. Pressure gradient at the level of main PA was 42 mm Hg. The RV dilation was present, but there was no significant TR. Surgical excision of the mass with partial preservation of the pulmonary valve was performed with reconstruction of the RVOT and PA with trans-annular pericardial patch. Operative findings revealed that the mass was adherent to the pulmonary valve and appeared to be attached to the underlying endocardium just below the pulmonary valve. There was no attachment of the mass with the PA or the endocardium of the RVOT. Histopathology of the tumor specimen revealed features consistent with myxoma. The postoperative period was uneventful and the patient was discharged on postoperative day 7.
Primary cardiac masses are rare and usually benign with myxoma being the most common. Nearly 75% of myxomas are located in the left atrium whereas right sided myxomas are rare (15-20%), with right ventricular (3-4%) or pulmonary artery myxomas being extremely rare., Symptoms may be variable and are determined by the tumor location and size. Myxomas originating from the right side of the heart can present with cardiac or extra-cardiac manifestations such as congestive heart failure, shortness of breath, syncope, fatigue, peripheral edema, jugular venous distention, and ascitis. The risk of life threatening complications indicate the importance of early diagnosis and prompt surgical resection. One of the main concerns during anesthesia is the risk of tumor embolization and pulmonary obstruction during anesthesia induction or at any stage thereafter. Hemodynamic instability, inconvenient manipulation of the tumor and heart must be avoided to prevent these complications. Femoral arterial-venous cannulation can be performed for cardiopulmonary bypass initiation in case complete RVOT obstruction with hemodynamic collapse occurs. TEE is an excellent diagnostic tool to investigate suspected intracardiac masses. It also plays a valuable role in the detection of central pulmonary artery embolism. The characteristic narrow stalk and tumor mobility are the distinguishing features of a myxoma. Further, they are homogenous or may have central areas of hyperlucency representing hemorrhage and necrosis.
The isolated RV or PA myxomas are described in the literature, but a myxoma within the RVOT extending across the pulmonary valve into the pulmonary artery is very rare with only a few cases reported so far.,,, In most of these cases myxomas were arising from the RV free wall and pulmonary valve seemed to be spared. This case seems to be unique in that the mass was adherent to the pulmonary valve. The tumor had a broad base and only the RVOT portion was mobile with a homogenous appearance (Video 1]- and [Video 2)- . The differential diagnosis for an intracavitary cardiac mass includes thrombus, myxoma, lipoma and nonmyxomatous neoplasm, most of which are malignant. The present case demonstrates the utility of TEE in the perioperative management of such a patient.