Author + information
- Received January 9, 2017
- Revision received January 31, 2017
- Accepted February 9, 2017
- Published online April 17, 2017.
- George Joseph, DMa,∗ (, )
- Sujith Thomas Chacko, DMa,
- Elizabeth Joseph, DNBb and
- Vishnu Chandra Kumar, MDc
- aDepartment of Cardiology, Christian Medical College, Vellore, India
- bDepartment of Radiology, Christian Medical College, Vellore, India
- cDepartment of Pathology, Christian Medical College, Vellore, India
- ↵∗Address for correspondence:
Dr. George Joseph, Department of Cardiology, Christian Medical College, Vellore-632004, Tamil Nadu, India.
A 52-year-old man was evaluated for dry cough, progressive breathlessness, and exertional syncope. He had distended neck veins, bilateral leg edema, and a grade 3/6 ejection systolic murmur in the left parasternal region. Transthoracic echocardiography (Figure 1A) and cardiac magnetic resonance imaging (Figure 1B) revealed an obstructive intracavitary mass in the right ventricular outflow tract (RVOT) and pulmonary artery producing a peak systolic pressure gradient of 75 mm Hg. The mass did not infiltrate adjacent structures, but multiple subcentimeter nodules were present in both lungs consistent with metastases. Positron emission tomography (Figures 1C to 1E) showed the mass and metastatic nodules to be metabolically active, but an active primary focus could not be detected. Fluoroscopy-guided transjugular endomyocardial biopsy of the lesion was performed. Histopathologic findings obtained from the biopsy specimens, including spindle cell morphology and positive staining for pancytokeratin and vimentin (Figures 1F to 1H), and the immunohistochemical profile, were suggestive of a metastatic sarcomatoid carcinoma.
Because the patient’s clinical condition was deteriorating rapidly, a palliative stenting procedure (Figures 1I and 1J) was performed. A 22 × 45 mm self-expanding stent (Wallstent, Boston Scientific, Natick, Massachusetts) was deployed across the RVOT extending into the right pulmonary artery. This improved flow through the RVOT significantly and produced dramatic resolution of the patient’s symptoms. He was discharged from hospital after receiving 3 cycles of palliative chemotherapy. However, the symptoms recurred after 3 months, at which time he expired. The primary focus remained undetected because an autopsy was not performed.
Most cardiac tumors are metastatic and the right heart is more often involved than the left because it receives the systemic venous and lymphatic drainage (1). Metastatic RVOT obstruction is rare, and may occur in the absence of widespread malignancy; new-onset right heart failure and left parasternal systolic murmur should arouse clinical suspicion (1). The prognosis is generally poor. Palliative RVOT stenting (2) is useful for symptomatic relief.
All authors have reported that they have no relationships relevant to the contents of this paper to disclose.
- Received January 9, 2017.
- Revision received January 31, 2017.
- Accepted February 9, 2017.
- 2017 American College of Cardiology Foundation