Massive Calcified Intramural Hematoma of the Right Ventricular Free Wall with 31-year Follow-up

Madhu Shukla; Jagdish Chander Mohan

doi:10.4103/jiae.jiae_62_22

INTERESTING CASE REPORT

Year : 2023 | Volume : 7 | Issue : 1 | Page : 47-50

Massive Calcified Intramural Hematoma of the Right Ventricular Free Wall with 31-year Follow-up

Madhu Shukla, Jagdish Chander Mohan
Department of Cardiology, Institute of Heart and Vascular Diseases, Jaipur Golden Hospital, New Delhi, India

Date of Submission	05-Dec-2022
Date of Decision	24-Dec-2022
Date of Acceptance	25-Dec-2022
Date of Web Publication	10-Feb-2023

Correspondence Address:
Prof. Jagdish Chander Mohan
A51, Hauz Khas, New Delhi - 110 016
India

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jiae.jiae_62_22

Abstract

A 21-year-old girl presented with an episode of ventricular tachycardia in September 1991. She was found to have a large (8 cm × 5 cm) mass in the right ventricle and was operated. The mass was full of thrombi and fresh blood that was evacuated. She remained asymptomatic till May 2022, when she presented with another episode of ventricular tachycardia. She also had a history of effort angina of 2 months' duration. Multimodality imaging evaluation revealed a large calcific cystic mass in the right ventricular free wall with an unruptured aneurysm of the right sinus of Valsalva, a markedly distorted right ventricular cavity, and the Brugada pattern of electrocardiogram. The case is reported for its rarity, long follow-up, and strikingly high-quality images.

Keywords: Calcified intramyocardial hematoma, computed tomography imaging, unruptured aneurysm of valsalva sinus

How to cite this article:
Shukla M, Mohan JC. Massive Calcified Intramural Hematoma of the Right Ventricular Free Wall with 31-year Follow-up. J Indian Acad Echocardiogr Cardiovasc Imaging 2023;7:47-50

How to cite this URL:
Shukla M, Mohan JC. Massive Calcified Intramural Hematoma of the Right Ventricular Free Wall with 31-year Follow-up. J Indian Acad Echocardiogr Cardiovasc Imaging [serial online] 2023 [cited 2023 May 29];7:47-50. Available from: https://jiaecho.org/text.asp?2023/7/1/47/369552

Introduction

Intramyocardial hematoma occurs following the rupture of intramural blood vessels into the media and subadventitial region. Spontaneous intramyocardial hematoma of the right ventricular free wall presenting as pseudotumor is rare,^[1],[2] although it has been reported more often following myocardial infarction, chest trauma, coronary artery bypass operation, and as a complication of percutaneous coronary intervention.^[3],[4],[5] Nonresorption of intramyocardial hematoma despite surgical evacuation and its persistence as a large calcified cystic mass has not been reported. We herein report such a case with a 31-year follow-up who subsequently also developed an unruptured aneurysm of the right sinus of Valsalva.

Case Report

This 53-year-old female was first seen when she was an unmarried girl of age 21 years. She presented with ventricular tachycardia at 230 beats/min (bpm) and left bundle branch block morphology. After cardioversion, she underwent a complete evaluation and was found to have a massive tumor-like mass with variegated echogenicity in the right ventricle. Surgical exploration showed it to be a large (8 cm × 5 cm) intramyocardial hematoma which was evacuated and plicated as reported earlier.^[1] The patient remained asymptomatic till May 22, 2022, when she presented with another episode of ventricular tachycardia at 160 bpm. Surprisingly, the tachycardia responded to diltiazem. She also complained of effort angina for the past 2 months. On examination, she was comfortable with a pulse rate of 78 bpm and sitting blood pressure of 130/78 mmHg at rest. There were no other physical findings and no evidence of heart failure. Detailed blood biochemistry including calcium-phosphorus metabolism and renal function was normal. Detailed immunologic investigations carried out post facto were normal. The 12-lead electrocardiogram showed sinus rhythm, type 2 Brugada pattern, and generalized T-wave inversion with QRS duration of 110 ms [Figure 1]. Cinefluorography just before selective invasive coronary angiography showed an oblong calcified mass (7 cm × 5.5 cm × 4 cm) situated anteriorly behind the sternum [Figure 2]. Selective invasive coronary angiography revealed a normal coronary angiogram, although the right coronary artery could not be hooked selectively due to the distorted right sinus of Valsalva [Figure 3]. Transthoracic echocardiography in short-axis view showed dilated right sinus of Valsalva with an aneurysm projecting into the right ventricular outflow tract with no continuous flow [Figure 4], [Video 1] and [Video 2]. The left-sided structures were normal but it was difficult to visualize the right atrium and the right ventricle on transthoracic echocardiography. Definity contrast injection (0.3 mL followed by a saline bolus of 3 mL) showed a distorted right ventricular cavity by a contrast-free mass all along the free wall [Figure 5]. Computed tomography (CT) angiogram revealed this oblong mass lying in the free wall of the right ventricle (7 cm × 5.5 cm × 4 cm) with thin high-attenuation interrupted peripheral walls and low-attenuation signals from the cavity. The pouch-like aneurysm of the right sinus of Valsalva was also visualized [Figure 6].

Figure 1: Type 2 Brugada pattern on 12-lead electrocardiogram

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Figure 2: Cinefluorography in multiple views showing an oblong calcific mass situated behind the sternum. (a) Anteroposterior (AP) view, (b) left anterior oblique view, and (c) right lateral view. Sternal sutures are seen

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Figure 3: Invasive coronary angiogram in the right anterior oblique view showing normal left coronary artery (a) and right coronary artery (b). The calcific mass is seen beside the right coronary artery

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Figure 4: (a) A transthoracic echocardiographic short-axis view showing an aneurysm of the right sinus of Valsalva with a protrusion. (b) The aneurysm of the right sinus on the aortogram in lateral view and (c) an aneurysm of the right sinus on CT angiogram (arrows). AV: Aortic valve, CT: Computed tomography, LA: Left atriume, LV: Left ventricle, RVOT: Right ventricular outflow tract, RA: Right atrium, RV: Right ventricle

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Figure 5: Transthoracic parasternal long-axis view (a) and modified four-chamber view with Definity contrast injection (b). Distorted right ventricular cavity can be seen in b. AV: Aortic valve, RV: Right ventricle

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Figure 6: Volume-rendered CT angiogram (a) and sagittal view (b) showing normal coronary arteries, aneurysm of the right sinus of Valsalva, and an oblong calcific mass (7 × 4) in relation to the anterior wall of the right ventricle. CT: Computed tomography, LV: Left ventricle

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[Additional file 1]

Video 1: Modified transthoracic echocardiographic 4-chamber view with cavity opacification by Definity ultrasound contrast. Distortion of the right ventricular cavity is seen by a mural mass with no intervening interface.

[Additional file 2]

Video 2: Computed tomographic angiogram in sagittal view showing the abnormal structures.

Several management options including internal defibrillator implantation were discussed but the patient chose to be on conservative management with antianginal drugs for microvascular angina.

Discussion

Intramural hematoma of the right ventricle is a rare form of cardiac rupture with massive intramyocardial hemorrhage without any apparent tear of the wall, occurring either spontaneously or after acute infarction, or related to primary coronary interventions.^{[1],[2],[3],[4],[5]}

This patient, who was operated upon 31 years back to evacuate a massive intramyocardial hematoma of the right ventricular-free wall, presented with an episode of ventricular tachycardia with preceding history of effort angina and was found to have a large cystic calcific mass in relation to the right ventricle. We also detected an incidental unruptured aneurysm of the right aortic sinus and normal coronary angiogram on both invasive and noninvasive angiography. This cystic calcific mass probably represents residual and recurrent hematoma whose peripheral part got calcified over time. Dystrophic calcification occurs in myocardial hemorrhage, necrosis, or fibrosis.^[6] Pressure necrosis and/or inflammatory change at the periphery promotes calcification. Damaged myocardial tissue creates a calciumphilic surface for calcium salt deposition.^[6] The common etiologies of dystrophic calcification include myocardial infarction and ventricular aneurysms and rare ones include hypertrophic cardiomyopathy, sarcoidosis, and tumors. We are not aware of a calcified hematoma of the right ventricle reported in the literature. CT is the gold standard for the detection of calcification, with an increased sensitivity in detecting the location and character of the calcification, as was in this case.

A sinus of Valsalva aneurysm is an enlargement of the aortic root area between the aortic valve annulus and the sinotubular junction. The sinus of Valsalva aneurysms have an incidence of approximately 0.1% in the general population.^[7] Although most are congenital, acquired aneurysms can be caused by conditions affecting the aortic wall, such as infections, degenerative diseases, thoracic trauma, Marfan syndrome, and Behçet disease.^[8] Unruptured aneurysms that do not require surgical intervention (no pressure effects or annular dysfunction) are managed conservatively using serial follow-up surveillance imaging.^[8] It is possible that our patient has an indeterminate connective tissue disease that can link intramural hematoma with the subsequent development of the aneurysm of the sinus of Valsalva. However, we could not establish this connection with any known syndrome.

Our case represents a diagnostic, pathogenetic, and management enigma. Atypical presentation with two episodes of ventricular tachycardia 31 years apart with a relatively asymptomatic period in between amazes us and we did not feel a compelling need for an implantable defibrillator in her.

Conclusion

This is a rare case of pseudotumor of the right ventricle secondary to dystrophic calcification in the periphery of contained spontaneous intramyocardial hemorrhage of the right ventricular free wall. A multimodal imaging approach was useful in accurate evaluation.

Consent

The authors confirm that written consent for submission and publication of this case report including image(s) and associated text has been obtained from the patient in line with COPE guidance.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that her name and initials will not be published and due efforts will be made to conceal her identity, but anonymity cannot be guaranteed.

Ethical approval

No ethical approval is required. All identifiable patient information has been removed from this manuscript.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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