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1-006 Reporting a rare of case of hypertrophic cardiomyopathy, bicuspid aortic valve with a new diagnosis of coarctation of aorta in a 42-year-old male

heartjnl · 2025-08-13 · canonical JSON source

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A 42-year-old male with a history of asthma, hypertension, and oesophagitis presented with recurrent chest pain, palpitations, dizziness, and syncopal episodes. Despite antihypertensive therapy, his blood pressure remained persistently elevated, prompting an extensive evaluation for treatment-resistant hypertension.In 2023, echocardiography and cardiac MRI revealed hypertrophic cardiomyopathy (HCM) alongside a bicuspid aortic valve (BAV) and mild aortic regurgitation. Since then, he experienced multiple emergency visits and hospital admissions due to chest pain and uncontrolled hypertension despite multiple antihypertensive medications. During his most recent admission, a retrospective review of his history and investigations, including MRI and chest X-ray, along with a significant pressure gradient between the upper and lower limbs (152/80 mmHg vs. 110/70 mmHg), raised suspicion of a vascular abnormality. A CT aortogram (figure 1) confirmed post-ductal coarctation of the aorta (CoA) as the primary cause of his hypertension.1 Reassessment of prior imaging revealed the characteristic ‘3 sign’ and intercostal rib notching on chest X-ray (figure 2).. Additionally, CoA was present on the initial cardiac MRI but had not been reported, as the primary focus was BAV and HCM. The diagnosis led to a multidisciplinary decision recommending surgical correction.Abstract 1-006 Figure 13D reconstruction of CT chest showing CoAAbstract 1-006 Figure 2CXR with ‘3 sign’ and intercostal rib notchingThis case highlights the rare coexistence of CoA and HCM in adults, an association previously reported but not fully understood.2 While Noonan syndrome links these conditions in children, no known syndrome explains their concurrence in adults. Such cases suggest screening for CoA in adults with HCM and hypertension. It also emphasizes the importance of evaluating secondary causes of hypertension in younger patients and ensuring comprehensive imaging referrals to improve diagnostic accuracy.Ultimately, this case reinforces the potential association between HCM and CoA while highlighting the diagnostic challenges involved and the necessity of considering secondary causes in cases of resistant hypertension.1 References Prisant LM, Mawulawde K, Kapoor D, Joe C. Coarctation of the aorta: a secondary cause of hypertension. The Journal of Clinical Hypertension 2004;6(6):347–52.Yang H, Wang H, Li Z, Yan J, Song YE, Zeng H, et al. Aortic coarctation associated with hypertrophic cardiomyopathy in a woman with hypertension and syncope: a case report with 8 year follow-up. Front Cardiovasc Med. 2021;8:818884.