|Year : 2020 | Volume
| Issue : 3 | Page : 75-76
Focal right ventricular apical hypertrophy or apical muscular ventricular septal defect
Zahra Alizadeh Sani1, Taimoor Etemad2, Mohaddeseh Behjati3, Zahra Khajali3, Roohallah Alizadehsani4, Abbas Khosravi4, Saeid Nahavandi4, Sheikh Mohammed Shariful Islam5
1 Rajaie Cardiovascular Medical and Research Center; Omid Hospital, Iran University of Medical Sciences, Tehran, Iran
2 Omid Hospital, Iran University of Medical Sciences, Tehran, Iran
3 Rajaie Cardiovascular Medical and Research Center, Iran University of Medical Sciences, Tehran, Iran
4 Institute for Intelligent Systems Research and Innovation, Deakin University, Geelong, VIC, Australia
5 Institute for Physical Activity and Nutrition, Deakin University, Melbourne; Cardiovascular Division, The George Institute for Global Health; Sydney Medical School, University of Sydney, Sydney, Australia
|Date of Submission||28-Aug-2020|
|Date of Decision||10-Sep-2020|
|Date of Acceptance||23-Sep-2020|
|Date of Web Publication||26-Oct-2020|
Dr. Roohallah Alizadehsani
Institute for Intelligent Systems Research and Innovation, Deakin University, Geelong, VIC 3216
Source of Support: None, Conflict of Interest: None
A 28-year-old male patient was referred to our imaging center with the differential diagnosis of a right ventricular (RV) apical mass. He was a known case of the ventricular septal defect (VSD) with a history of spontaneous closure at the age of 2 years. His chief complaint was chest pain and palpitation. He was referred with a transthoracic echocardiography report of RV apical hypertrophy with almost apical obliteration or RV apical mass. Cardiovascular magnetic resonance imaging demonstrated few intramyocardial crypts of basal anteroseptal left ventricular (LV) segment. There was no evidence of concomitant LV apical hypertrophy. Indeed, there was a small conical- and tunnel-shaped serpiginous apical-infundibular muscular VSD (3.6 mm RV side's diameter and 7.6 mm LV side's diameter) resulted in a localized and severely hypertrophied RV apical segment and no significant shunt (QP/QS: 1.16). There was no evidence of other cardiac mass.
Keywords: Apical-infundibular ventricular septal defect, apical right ventricular hypertrophy, right ventricular (RV) apical mass
|How to cite this article:|
Sani ZA, Etemad T, Behjati M, Khajali Z, Alizadehsani R, Khosravi A, Nahavandi S, Shariful Islam SM. Focal right ventricular apical hypertrophy or apical muscular ventricular septal defect. Res Cardiovasc Med 2020;9:75-6
|How to cite this URL:|
Sani ZA, Etemad T, Behjati M, Khajali Z, Alizadehsani R, Khosravi A, Nahavandi S, Shariful Islam SM. Focal right ventricular apical hypertrophy or apical muscular ventricular septal defect. Res Cardiovasc Med [serial online] 2020 [cited 2020 Dec 3];9:75-6. Available from: https://www.rcvmonline.com/text.asp?2020/9/3/75/298991
| Background|| |
Apical ventricular septal defects (VSDs) are the defects that exist between the apexes of both the left ventricle (LV) and the right ventricle (RV). RV apex is composed of two distinct apexes (sinus or inflow part and outlet or infundibular part) which are separated by multiple dense trabeculae passing from the interventricular septum toward the anterior free wall. In this case report, we described the cardiovascular magnetic resonance (CMR) finding of a case of apical infundibular VSD, who referred to us with focal RV apical hypertrophy or RV apical mass.
| Case Report|| |
Our case was a 28-year-old man who referred to our imaging center with the differential diagnosis of RV apical mass. He was a known case of the VSD with the patient's history of its spontaneous closure at the age of 2 years. His chief complaint was chest pain and palpitation. Given the history of arrhythmia documented via electrocardiogram, he was under treatment with propranolol daily [Figure 1]. He was referred with the documented transthoracic echocardiography report of the presence of RV apical hypertrophy, with almost apical obliteration and probable RV apical mass [Figure 2]. He underwent CMR imaging on heart rate of 81 bpm and blood pressure of 135/80 mmHg. CMR imaging demonstrated few intramyocardial crypts of the basal anteroseptal LV segment. There was no evidence of concomitant LV apical hypertrophy. Indeed, there was a small conical- and tunnel-shaped serpiginous apical muscular VSD (3.6 mm RV side's diameter and 7.6 mm LV side's diameter) that opened to the infundibular apical part of the RV with severely hypertrophied hypertrabeculated RV apical segment and no significant shunt (QP/QS: 1.16). There was no evidence of other cardiac mass [Figure 3]. Finally, he was scheduled for medical follow-up.
|Figure 1: Electrocardiogram demonstrates incomplete right bundle branch block with deep T inversion in precordial leads|
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|Figure 2: Apical four-chamber views that show small apical ventricular septal defects in the apex that opens into the right ventricle|
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|Figure 3: There is a small defect in the interventricular septum with bulging toward RV apex accompanied with apical right ventricular hypertrophy. (a) Mid to apex short-axis image in true SSFP sequence. (b) True SSFP cine sequences images in the axial view|
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| Discussion|| |
Mid-muscular VSD is the most common type of VSDs with significant proportions located in the apical part (1). In Kumar et al.'s study, the prevalence of apical muscular VSD is about 7%. In patients with apical muscular VSD, the apical trabeculae are hypertrophied, and it resembles a separate septum within the RV apex. Hence, this appearance may be caused misdiagnosis in echocardiography by focal apical hypertrophy and even RV apical mass.
Focal RV apical hypertrophy is a rare case and could occur in isolation or in association with LV hypertrophy that could be secondary to hypertrophic cardiomyopathy or other infiltrative conditions. Isolated RV hypertrophy could be seen in the presence of idiopathic pulmonary artery hypertension, that is, rarely focal. A report by Chatterjee and Narayan demonstrated a case of apical infundibular VSD that was misdiagnosed by double-chamber RV. CMR is a useful imaging tool in congenital heart disease, and it can show the small defects in the apical part of the septum besides helping to consider the hemodynamic effect of VSD by calculating QP/QS. The interventional approach in these types of VSDs is also challenging, and catheter device closure of the defects in the apical part needs precise evaluation by imaging techniques.
| Conclusions|| |
Apical muscular VSD could be misdiagnosed by RV apical hypertrophy or double-chamber RV because of severe trabeculation in the RV apex. CMR is a useful imaging tool for differentiating these abnormalities.
Informed consent was obtained from all individual participants included in the study.
Consent for publication
Consent for publication was obtained for every individual person's data included in the study.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that his name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Kumar K, Lock JE, Geva T. Apical muscular ventricular septal defects between the left ventricle and the right ventricular infundibulum. Diagnostic and interventional considerations. Circulation 1997;95:1207-13.
Chatterjee D, Narayan P. Idiopathic isolated right ventricular apical hypertrophy. Acta Cardiol Sin 2018;34:288-90.
[Figure 1], [Figure 2], [Figure 3]