Abstract
Case Summary
A 38-year-old man with asymptomatic gene-positive hypertrophic cardiomyopathy was found to have extensive dystrophic calcification of the ventricular septum. We hypothesized that the extensive ventricular septal calcification would represent an area of severe myocardial fibrosis, resulting in calcification secondary to postsurgical (septal myectomy) changes.
Take-Home Message
Calcification of the ventricular septum is a rare finding, not previously described following septal myectomy, that should prompt a comprehensive evaluation and follow-up with multimodality imaging and testing.
Key Words: calcification, hypertrophic cardiomyopathy, septal myectomy
Graphical Abstract
Case Summary
A 38-year-old asymptomatic man with a history of gene-positive (MYBPC3) nonobstructive hypertrophic cardiomyopathy presented to our specialty clinic as a new consultation. He was diagnosed at age 16 and, at that time, underwent septal myectomy for severe (80 mm Hg) left ventricular outflow tract obstruction. In this age group, 20% to 30% of patients experience failure of medical therapy and require septal myectomy.1 Postoperatively, he developed complete heart block that necessitated an implantable cardioverter-defibrillator. During his initial visit at our center, he had normal vital signs and a body mass index of 40.8 kg/m2. The physical examination revealed a soft systolic murmur at the apex with an S4. A transthoracic echocardiogram demonstrated unusual hyperechogenic areas within the septum (acoustic shadowing), likely representing areas of calcified myocardium (Figures 1A and 1B). In addition, he had a left ventricular ejection fraction of 53%, normal right ventricular systolic function, grade 3 left ventricular diastolic dysfunction, and no left ventricular outflow tract obstruction. A coronary computed tomography angiogram revealed an unexpected pattern of severe calcification within the ventricular septum. The calcifications were prominent throughout the anterior septum, extending to the inferior septum without any involvement of the mitral annulus (Figures 1C to 1F). There was no evidence of calcification, plaque, or stenosis in the left anterior descending, left circumflex, or right coronary arteries (Figures 1G to 1I).
Take-Home Messages
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Myocardial calcification is an uncommon pathology that requires a comprehensive evaluation.
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The calcification of the ventricular septum after septal myectomy is a unique finding not previously described in the literature.
Figure 1.
Multimodality Imaging of the Ventricular Septal Calcification
Acoustic shadowing and calcification are seen on transthoracic echocardiography in the apical zoomed-in 4-chamber (A) and modified 4-chamber (B) views. Cardiac computed tomography angiography in the double oblique (C), 4-chamber (D and E), and short-axis (F) views demonstrates an unexpected pattern of severe calcification throughout the anterior septum, extending to the inferior septum, without any involvement of the mitral annulus. Coronary computed tomography angiography of the left anterior descending artery (G), left circumflex artery (H), and right coronary artery (I) shows no evidence of coronary calcification, plaque, or stenosis. CTA = computed tomography angiography; LA = left atrium; LAD = left anterior descending; LCX = left circumflex; LV = left ventricle; RCA = right coronary artery.
Discussion
Myocardial calcification of the ventricular septum is a rare phenomenon that can have various etiologies, from myocardial ischemia to systemic causes such as sepsis. It is categorized as metastatic (due to hypercalcemic states) or dystrophic (calcium deposits in areas of necrosis).2 We extensively reviewed our patient's medical history and charts for a possible explanation. There was no evidence of increased calcium homeostasis from pathologies such as hyperparathyroidism or renal disease. Interestingly, no calcification was seen in the coronary arteries or the mitral valve apparatus, the latter of which is more common in patients with late-stage hypertrophic cardiomyopathy. The patient had normal results on previous tests for coronary ischemia and no history of inflammation-related etiologies such as pericarditis or myocarditis. The patient's only cardiac surgical history was his septal myectomy. We carefully examined the surgical report and determined that there was no placement of a foreign material or patch during the procedure. There was no evidence of any myocardial calcification on either parent's echocardiogram.
The prognosis of myocardial calcification can vary depending on etiology and location. Calcified myocardium may be an incidental finding or could develop to cause multiple cardiac complications, including heart failure, focal wall motion abnormalities, and life-threatening arrhythmias.2 In our patient, we hypothesized that the extensive ventricular septal calcification would represent an area of severe myocardial fibrosis, resulting in calcification secondary to postsurgical changes. These changes in our patient likely led to diastolic dysfunction, and he will be monitored with regular cardiac imaging. Myocardial calcification has not previously been reported in the literature as a potential sequela of septal myectomy. To our knowledge, this is the first documented case to describe this association of dystrophic calcification of the ventricular septum in a postmyectomy patient.3
Funding Support and Author Disclosures
The authors have reported that they have no relationships relevant to the contents of this paper to disclose.
Footnotes
The authors attest they are in compliance with human studies committees and animal welfare regulations of the authors’ institutions and Food and Drug Administration guidelines, including patient consent where appropriate. For more information, visit the Author Center.
References
- 1.Bogle C., Colan S.D., Miyamoto S.D., et al. Treatment strategies for cardiomyopathy in children: a scientific statement from the American Heart Association. Circulation. 2023;148:174–195. doi: 10.1161/CIR.0000000000001151. [DOI] [PubMed] [Google Scholar]
- 2.Sozzi F.B., Gnan E., Faggiano A., et al. Extensive myocardial calcifications: a systematic literature review of a rare pathological phenomenon. Front Cardiovasc Med. 2024;11 doi: 10.3389/fcvm.2024.1367467. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 3.Maron B.J., Dearani J.A., Smedira N.G., et al. Ventricular septal myectomy for obstructive hypertrophic cardiomyopathy (analysis spanning 60 years of practice): AJC expert panel. Am J Cardiol. 2022;180:124–139. doi: 10.1016/j.amjcard.2022.06.007. [DOI] [PubMed] [Google Scholar]