Case report:
A 40 years old woman presented at the emergency department because of progressive dyspnea, orthopnea, edema, weakness. She has involved with COVID-19 about 2 months ago according to polymerase chain reaction (PCR) and chest computed tomography (CT), But she didn’t recover from respiratory symptoms in spite of adequate treatment. In admission time, she has respiratory distress, tachycardia, tachypnea, edema, cyanosis and coldness of extremely and systemic hypotension (Blood Pressure: 90/65 mmHg). O2 saturation in air room was 86% and with noninvasive O2 therapy increased to 89%.
Electrocardiogram (ECG) showed sinus tachycardia, right axes deviation and right ventricle (RV) strain pattern in precordial leads (Figure 1). Chest X ray showed significant cardiomegaly, RV enlargement and prominent main of PA and left PA without evidence of pulmonary venous congestion (Figure 2). Spiral chest CT demonstrated evidence of previous COVID-19 involvement with ground glass appearance and air trapping in both lungs and also cardiomegaly and large size pericardial effusion (Figure 3).
Transthoracic echocardiography (TTE) showed severe RV enlargement with severe RV systolic dysfunction, right atrial (RA) enlargement and abnormal interventricular septal motion. D shaped appearance of Interventricular septum in parasternal short axis (PSAX) view with mid systolic flattening, representing severe RV pressure overload and signs of severely increased RV afterload pattern. In PSAX view, very large size nonhomogeneous solid mobile mass is seen in distal right ventricular outflow tract (RVOT) extended to main PA and has attachment to pulmonary valve (PV). The PV obstruction was severe and blood flow to main PA was reduced. Left and right PA and pulmonary bifurcation were evaluated, that were spared without any mass. Color doppler study of tricuspid valve (TV) inflow showed severe tricuspid regurgitation (TR) and hemodynamic study with continuous doppler wave in apical 4 chamber view demonstrated TR peak velocity of 4.4 m/s (TR peak gradient: 77 mmHg). Evaluation of inferior vena cava (IVC) in subcostal view was done. IVC was severely enlarged (2/75 cm diameter) without respiratory collapse. Therefore, hemodynamic study estimated RA pressure about 20 mmHg, more compatible with chronic course of disease and indicating of more gradual increasing of RV pressure rather than acute event. Massive pericardial effusion was seen more localized in posterolateral of left ventricle (LV) (maximum diameter: more than 3 cm). There was significant respiratory variation in Doppler study of mitral valve (MV) and TV diastolic inflow velocity more than 30% (Figure 4).
Laboratory data containing hematologic, inflammatory and biomarkers were measured. D dimer was increased about 4000 μg/l and pro-brain natriuretic peptide (pro-BNP) 12500 ng/d.
According to previous history of the COVID-19, ECG manifestation, echocardiography, chest X ray, spiral chest CT, laboratory data and abnormal hemodynamic condition, massive sub-acute PTE was highly suggested. Due to our patient hemodynamic disturbance, massive pericardial effusion and giant mass, emergent cardiac consultation with cardiovascular surgical team for surgical resection was done and the patient was taken to operating room.
Thoracotomy and median sternotomy was done and then cardiopulmonary bypass was established. Main PA was incised. Unexpectedly, out of previous suspicious, our surgeon encountered with large size tumoral solid mass with firm texture that was attached to right leaflet of PV and protruded to main PA and RVOT, which appearance was highly suggestive for sarcoma rather thrombosis. Therefore, mass completely was resected and because of tumoral involvement, right leaflet of PV removed (Figure 5). Reevaluation for residual masses in other parts was done with intraoperative echocardiography. TEE confirmed there was not any other masses in PA. Severe pulmonary insufficiency (PI) was seen after removal of right leaflet of PV. Staged operation for reconstruction of RVOT with conduit PV was postponed after preparing the result of tumor pathology and probably need for adjuvant chemotherapy. Patient had stable hemodynamic in ICU, intubated easily and discharged after 7 days without any cardiac event.
Pre-discharge TTE was done. RV and RA were severely enlarged. RV systolic function was severely impaired and severe TR and severe PI was seen. No residual mass was seen in RVOT, PV, PA and main of PA. Continuous doppler wave study showed peak TR velocity 4 m/s and short pressure half time (PHT:24 ms) and in 2D study evidence of increased RV after load pattern was permanent. IVC plethora and engorgement without respiratory collapse was seen (Figure 6).
Pre-discharge pulmonary CT angiography was done that any mass or thrombus in pulmonary vasculature wasn’t seen. In addition, abdominopelvic and brain CT for further evaluation were performed which were not abnormal.