![]() On cross section, a central cavitary area was noted, with fibrous material adherent to the inner lining and an average wall thickness of 0.8 cm ( Figure 1). Grossly, appearance was of a tan pedunculated mass measuring 3.8 × 2.4 × 1.5 cm, with a tan-gray, smooth, firm surface and infarction/degenerative changes. Postoperative electrocardiogram was unchanged and did not show any abnormal cardiac rhythms. ![]() Postoperatively, 2-dimensional echocardiogram was repeated, showing absence of the previously present mass, a competent, nonregurgitant tricuspid valve with some thickening of the anterior leaflet, and normalization of right ventricular pressure. Maximal resection was attempted without creating a defect in the tricuspid valve, leaving some areas of fibrotic, grossly abnormal tissue in an attempt to recreate physiologic anatomy. Intraoperatively, the tumor base was located at the leading edge of the anterior leaflet of the tricuspid valve, with some involvement of the chordae tendineae and the papillary muscles. Extravasated red blood cells are seen adjacent to capillaries (arrows). (c) H&E stain, 200x: stellate fibroblasts displaying small, elongated, dense nuclei and eosinophilic cytoplasm with indistinct cell borders enmeshed within a myxoid stroma. Gross pathology (b) shows a resected tumor, measuring 3.8 × 2.4 × 1.5 cm with infarction and degenerative changes. Left ventricular morphology, size, and function were normal.Įchocardiogram (a) shows an evident pedunculated mass, attached to the base of the anterior leaflet of the tricuspid valve and right ventricular septum extending into the right ventricular outflow tract. Right ventricular pressure was significantly elevated between 100 and 110 mmHg based on tricuspid regurgitation jet positional evaluation was not performed. The mass extended into the right ventricular outflow tract and caused marked obstruction of the pulmonary artery during systole ( Figure 1). Sedated, supine 2-dimensional echocardiogram with Doppler showed a pedunculated mass with an estimated size of 4.3 × 2.0 × 1.4 cm occupying most of the right ventricle, attached to the base of the anterior leaflet of the tricuspid valve and right ventricular septum. Electrocardiogram showed sinus tachycardia, right axis deviation, and right ventricular hypertrophy but was otherwise unremarkable. Vital signs were appropriate, and exam was normal other than a grade III/VI systolic ejection murmur best appreciated at the upper left sternal border with an S4 gallop. ![]() Medical and family/social history was noncontributory, with native-Hawaiian background and normal growth. These episodes worsened in the 2 weeks prior to presentation, occurring up to 3 times nightly. A 14-month-old male presented with 3 months of worsening paroxysmal nocturnal dyspnea, with multiple sleep interruptions consisting of episodic respiratory distress, cyanosis, and coughing.
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