Dynamic Right Ventricular Outflow Tract Obstruction from a Pedunculated Angiofibroma

Dear Editor, We present a rare case of a pedunculated angiofibroma originating in the right ventricular outflow tract (RVOT), resulting in dynamic obstruction.[1,2] Although anesthetic and echocardiographic management followed standard protocols, this case emphasizes the importance of early diagnosis, accurate imaging, and multidisciplinary care. A 49-year-old man without prior comorbidities was referred after auscultation revealed a grade III/VI systolic murmur at the pulmonic area. Transthoracic echocardiography (TTE) showed a mobile, homogeneously dense mass prolapsing into the RVOT during systole, measuring approximately 7.2 cm², with preserved pulmonary valve function [Figure 1a]. Significant obstruction was noted, evidenced by elevated tricuspid transvalvular gradient and right-sided chamber pressures [Figure 1b]. Color Doppler imaging confirmed the limitation of transpulmonary flow [Figure 1c].Figure 1: Presurgical in-hospital transthoracic echocardiogram and angiocardiography. (a) Short axis image: large, rounded mass of 7.2 cm², unique, quite mobile with heterogeneous echogenicity, located on the pulmonary valvular plane and occupying a large part of the right ventricular outflow tract. (b) R. tricuspid: The mass generates significant obstruction to pulmonary transvalvular flow, resulting in increased right chamber pressures, reflected in the severe elevation of the tricuspid transvalvular gradient. (c) Color Doppler short-axis view of the great vessels: the significant degree of obstruction in the right ventricular outflow tract and the limitation of transpulmonary flow. (d) Angiocardiography shows a proximal oval vascular network of the anterior descending arteryCT pulmonary angiography demonstrated a hypodense, intraluminal lesion (40 × 27 mm) located in the RVOT and extending into the pulmonary artery. Coronary angiography revealed a vascular network at the pulmonary trunk from the left anterior descending artery [Figure 1d]. Given the risk of sudden obstruction, the patient underwent urgent surgical intervention. Via median sternotomy and standard extracorporeal circulation (without aortic clamping), arteriotomy of the pulmonary trunk exposed a 2 × 2 cm encapsulated, pedunculated mass adherent to the pulmonary valve [Figure 2a and b]. The lesion was excised completely with preservation of valvular anatomy. The patient had an uneventful postoperative course with stable hemodynamics and no need for vasopressors. TTE confirmed complete mass removal and preserved function.Figure 2: Intraoperative findings and surgical resection of angiofibroma. (a and b) Primary cardiac tumor, adherent to pulmonary valve. (c) Angiofibroma with homogeneous surface, encapsulated. (d) Section with smooth surface, degenerative myxoid appearance. (e) Angiofibroma after fixation in 10% formalinMacroscopically, the tumor exhibited a homogeneous, encapsulated surface with areas of myxoid degeneration [Figure 2c and d]. Fixation in formalin preserved structural integrity [Figure 2e]. Histopathological evaluation revealed a proliferation of medium-caliber and cleft-like vessels within a hypocellular myxoid stroma. Some vascular structures contained thrombi. CD34 immunostaining highlighted endothelial architecture, while smooth muscle actin was expressed in stromal and perivascular cells. The Ki-67 proliferation index was low (5%), supporting a benign phenotype [Figure 3a-c].Figure 3: Histological analysis of angiofibroma. (a) Hematoxylin-eosin staining shows a lesion composed of medium-caliber vessels with thrombus (asterisk). (b) Numerous elongated vessels on hypocellular lax stroma. (c) CD34 immunostaining highlights the cleft-branched vascular pattern with a stromal population lacking marker expression (inset)Primary cardiac tumors are uncommon, with benign variants comprising the majority. Vascular tumors, such as angiofibromas, are exceedingly rare and not included in the 2015 WHO classification.[3] They differ histologically from hemangiomas due to their heterogeneous vascular patterns and stromal characteristics.[1] The clinical presentation of cardiac tumors is influenced more by location than histology. RVOT obstruction may lead to dyspnea, fatigue, and right heart failure, although our patient was asymptomatic. This case underscores the importance of physical examination and echocardiography in identifying occult intracardiac lesions. Although intraoperative transesophageal echocardiography (TEE) was not used, it would ideally assist in monitoring tumor mobility, risk of embolization, and immediate post-resection cardiac function. In this case, TEE was unavailable, but the procedure was uneventful, with TTE guiding diagnosis and follow-up. Anesthetic and surgical management were conventional, and the patient remained stable without inotropic support, likely due to early diagnosis and effective resection. To our knowledge, this represents one of the very few documented cases of RVOT angiofibroma.[4] It reinforces the value of imaging in preoperative planning and the diagnostic role of histopathology. Awareness of this rare entity may aid in timely recognition and intervention, preventing potential hemodynamic compromise. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.