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Atypical Presentation of Right Ventricular Cardiac Hamartoma in a Young Man Cover

Figures & Tables

Figure 1

Preoperative transthoracic echocardiogram. (A) Transthoracic echocardiogram without contrast and (B, C) with intravenous contrast-enhancing agent. (B) Parasternal long axis view demonstrating asymmetric septal thickening/mass with contrast enhancement and (C) protrusion of the mass into the right ventricular outflow tract on parasternal short axis.

Figure 2

Preoperative cardiac imaging. Magnetic resonance imaging using steady state free precession cine sequence: (A) 3-chamber view, (B) short axis at the mid-level, (C), first pass perfusion, and (D) delayed enhancement after gadolinium showing a large right ventricular (RV) mass measuring 4.9 × 3.7 cm invading the RV free wall, with (E) intense first pass perfusion and significant late gadolinium enhancement cardiac computed tomography demonstrating short axis basal anteroseptal mass protruding into the RV outflow tract with central contrast enhancement and multiple septal perforator feeding vessels.

Figure 3

H&E stain at increasing magnification 100×. (A) The resected lesion was composed of disorganized, hypertrophic cardiac myocytes with interstitial fibrosis. (B) There were also scattered thickened intramural arteries, dilated venules, and small collections of adipocytes 200×. (C) At intermediate magnification, the myocytes were haphazardly arranged and enlarged with sarcoplasmic vacuolation and nuclear enlargement 400×. There were scattered enlarged nuclei, some with mild hyperchromasia, irregular nuclear contours, and inconspicuous nucleoli.

Table 1

Cardiac hamartoma literature search. A literature search revealed 30 patients with a diagnosis of hamartoma of mature cardiac myocytes from 1998 to 2020. The hamartoma was localized to the left ventricle in 13 patients, the right atrium in 7 patients, and the right ventricle in 3 patients; 2 patients showcased multiple locations. Ten of these patients (33%) were asymptomatic at time of presentation.1-21 CT: computed tomography; RVR: rapid ventricular response; WPW: Wolff-Parkinson-White.

YEAR PUBLISHEDAUTHORAGEGENDERCLINICAL SYMPTOMSDIAGNOSTIC EVALUATIONTUMOR LOCATIONTUMOR SIZE
1998Sturtz et al.24MaleHypertension, palpitations, and premature contractionsLeft ventricle
1998Burke et al.22MaleAsymptomaticEchocardiogramRight ventricle5 cm
1998Burke et al.28MaleWPW syndrome and an episode of syncopeEchocardiogramRight atrium
1998Burke et al.9MaleSudden deathAutopsyRight atrium1 × 2 cm
2001Dinh et al.33MaleGeneralized tachycardiaEchocardiogramLeft ventricle4.5 × 3.1 × 4.4 cm
2004Chu et al.76MaleHistory of hypertensionIn surgeryCrista terminalis0.5 × 1 × 0.5 cm
2005Martínez QM et al.33MalePalpitations and dyspneaEchocardiogramLeft ventricle4.5 × 5.5 cm
2008Movahedi et al.58MaleProgressive dyspneaIn surgeryRight atrium1.5 × 1 × 0.5 cm
2008Fealey et al.0.5MaleAsymptomatic
2008Fealey et al.0.5FemaleAsymptomatic
2008Fealey et al.1.2MaleAsymptomatic
2008Fealey et al.10MaleAsymptomaticEchocardiogramLeft ventricle5 × 3 cm
2008Fealey et al.16FemaleAsymptomaticEchocardiogramRight ventricle8 × 9 cm
2008Fealey et al.57MaleSudden death
2008Fealey et al.74MaleExertional dyspnea
2008Menon et al.16FemaleWeight lossEchocardiogramRight ventricle8 × 7 × 3 cm
2008Menon et al.10MaleAsymptomaticEchocardiogramLeft ventricle0.3 × 0.2 cm
2009Hsu et al.19FemaleIntermittent palpitations and dizzinessEchocardiogramLeft ventricle4 × 7 cm
2009Galeone et al.56FemaleAsymptomaticChest CTPulmonary infundibulum9 × 9 × 4 cm
2011Dell’Amore et al.35FemalePalpitations and dyspneaEchocardiogramLeft ventricle4.2 × 3.3 × 2.7 cm
2013Raffa et al.41FemaleChest painEchocardiogramRight atrium2.5 × 1.3 cm
2017Ayoub et al.14MaleAsymptomaticEchocardiogramLeft ventricle9 × 5 × 6 cm
2017Hadravská et al.39FemaleRuptured aneurysm and severe pneumoniaAutopsyLeft ventricle4.5 × 3 × 3 cm
2017Abuzaid et al.21FemaleChest pain and dyspneaEchocardiogramLeft ventricle1.6 × 1.3 × 1.9 cm
2017Liu et al.64MaleDyspneaEchocardiogramLeft ventricle2 × 3 cm
2018Mantilla-Hernandez et al.23FemaleParoxysmal nocturnal dyspnea, RVR and edemaAutopsyRight atrium
2018Negri et al.44FemaleAsymptomaticEchocardiogramLeft ventricle4 × 3 cm
2018Kumari et al.43MaleChest pain, orthopnea, and palpitationsEchocardiogramLeft ventricle8 × 6 cm
2019Fu et al.41MaleCough, edema, dyspnea on exertionEchocardiogramRight atrium2 × 1 × 0.5 cm
2019Zhou et al.41MaleDyspnea and edemaEchocardiogramRight atrium1.8 × 1.4 cm
DOI: https://doi.org/10.14797/mdcvj.1158 | Journal eISSN: 1947-6108
Language: English
Page range: 102 - 107
Submitted on: Aug 3, 2022
Accepted on: Sep 22, 2022
Published on: Oct 4, 2022
Published by: Houston Methodist DeBakey Heart & Vascular Center
In partnership with: Paradigm Publishing Services

© 2022 Ramez Barsoom, Lamees I. El Nihum, Qasim Al Abri, Areeba Ali, Susan L. Haley, Mohammed A. Chamsi-Pasha, Moritz C. Wyler von Ballmoos, Thomas E. MacGillivray, Michael J. Reardon, published by Houston Methodist DeBakey Heart & Vascular Center
This work is licensed under the Creative Commons Attribution-NonCommercial 4.0 License.