A case of hypertrophic cardiomyopathy complaining of chest pain during treatment for nephrotic syndrome

A woman complaining of dyspnea and chest pain since childhood, was referred to our hospital with an initial diagnosis of biventricular hypertrophic cardiomyopathy. Multimodality imaging evaluation revealed massive right ventricular (RV) hypertrophy and severe RV outflow tract obstruction, with a final diagnosis of double chambered RV associated with small ventricular septal defect with right-to-left shunt and right partial anomalous pulmonary vein return. This represents an uncommon combination of congenital abnormalities, extremely rarely diagnosed in adulthood.

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Apical hypertrophic cardiomyopathy: an uncommon cause of exertional chest pain in children

Cardiology in the Young ◽

10.1017/s1047951119002403 ◽

2019 ◽

Vol 29 (12) ◽

pp. 1533-1535 ◽

Cited By ~ 1

Author(s):

Qu-ming Zhao ◽

Lan He ◽

Fang Liu

Keyword(s):

Chest Pain ◽

Hypertrophic Cardiomyopathy ◽

Apical Hypertrophic Cardiomyopathy ◽

Cardiac Angiography ◽

First Case ◽

Asymptomatic Children

AbstractApical hypertrophic cardiomyopathy is an uncommon morphologic variant of hypertrophic cardiomyopathy, which is rarely diagnosed in childhood. To date, very few cases of asymptomatic children younger than 18 years have been reported in the literature. To the best of our knowledge, this is the first case of paediatric apical hypertrophic cardiomyopathy presenting with exertional chest pain, with characteristic electrocardiographic, echocardiographic, MRI, and cardiac angiography findings.

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P881 Young patient with left ventricular hypertrophy and accidentally discovered aortic dissection: hypertensive heart or hypertrophic cardiomyopathy?

European Heart Journal - Cardiovascular Imaging ◽

10.1093/ehjci/jez319.522 ◽

2020 ◽

Vol 21 (Supplement_1) ◽

Author(s):

M Gawor ◽

M Franaszczyk ◽

E Kowalik ◽

M Spiewak ◽

I Michalowska ◽

...

Keyword(s):

Chest Pain ◽

Hypertrophic Cardiomyopathy ◽

Aortic Dissection ◽

Troponin T ◽

Global Longitudinal Strain ◽

Interventricular Septum ◽

Positive Family History ◽

Left Ventricular ◽

Jugular Veins ◽

Rare Differential Diagnosis

Abstract A 36-year-old male with positive family history of sudden cardiac death (his uncle"s son died suddenly at the age of 25), hospitalized a month ago in a local hospital due to acute hypertensive cardiogenic pulmonary edema, was referred to our institution for further evaluation with suspicion of hypertrophic cardiomyopathy. On admission patient was asymptomatic, without fatigue, exertional dyspnoea, chest pain or syncope. On physical examination his BP was significantly elevated (180/100 mmHg). The lungs were clear on auscultation, liver was not enlarged, jugular veins were normal, there was no oedema of lower extremities. Abdominal auscultation revealed vascular murmur in umbilical region. The baseline level of NT-proBNP was 811.4 (range 0–125) pg/mL, and high-sensitivity cardiac troponin T was 20.2 (range 0–14) ng/L. The standard 12-lead electrocardiogram demonstrated sinus rhythm, left atrial enlargement and left ventricular (LV) hypertrophy with nonspecific ST segment and T-wave changes (Fig. 1A). No significant pathology was present on chest X-ray (Fig. 1B). Transthoracic echocardiography revealed significant concentric LV hypertrophy with preserved LV ejection fraction (EF 70%) and moderately decreased global longitudinal strain (GLS-13.7%). There was mild dilatation of left atrium. Ascending aorta diameter was in normal range (Fig. 1C-D). Cardiac magnetic resonance (CMR) scan confirmed concentric LV hypertrophy with the maximal wall thickness of 18 mm at interventricular septum, and increased myocardial mass (LV mass index 124 ml/m2, range 59–92). Moreover, small areas of late gadolinium enhancement were found in LV segments (Fig. 1E-F). Due to presence of vascular murmur in abdomen, ultrasound imaging was performed. The exam revealed abdominal aortic dissection (Fig. 1G-H). Patient was transferred to the computed tomography (CT) unit to confirm the diagnosis. Aortic dissection originated below renal arteries and involving common illiac arteries was detected (Stanford B). The presence of thrombi within the lumen created by the aortic dissection suggested chronic presentation. Patient was managed conservatively with strict blood pressure control and close follow up arranged. We decided to perform genetic analysis. Currently we are awaiting the results in hope that it will help us to establish the diagnosis and differentiate hypertensive heart from hypertrophic cardiomyopathy. In conclusion, aortic dissection typically presents with tearing chest pain and severe hemodynamic compromise. Painless dissection, like in this case, is relatively rare. Differential diagnosis between hypertensive heart and hypertrophic cardiomyopathy is crucial as it has direct therapeutic impact. Abstract P881 Figure 1

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Apical hypertrophic cardiomyopathy: chest pain and myocardial perfusion defects result from regional diastolic persistence of hyperdynamic cardiac contractility

European Heart Journal ◽

10.1093/eurheartj/eht309.p2972 ◽

2013 ◽

Vol 34 (suppl 1) ◽

pp. P2972-P2972

Author(s):

E. Stephenson ◽

P. Monney ◽

R. Boubertakh ◽

J. Nackvi ◽

N. Sekhri ◽

...

Keyword(s):

Chest Pain ◽

Hypertrophic Cardiomyopathy ◽

Myocardial Perfusion ◽

Cardiac Contractility ◽

Apical Hypertrophic Cardiomyopathy ◽

Perfusion Defects ◽

Myocardial Perfusion Defects

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Upright exercise stress echocardiography may unmask dynamic left ventricular obstruction also beyond hypertrophic cardiomyopathy

European Heart Journal ◽

10.1093/eurheartj/ehab724.057 ◽

2021 ◽

Vol 42 (Supplement_1) ◽

Author(s):

C Cotrim ◽

H Cafe ◽

I Goncalves ◽

J Guardado ◽

N Cotrim ◽

...

Keyword(s):

Chest Pain ◽

Hypertrophic Cardiomyopathy ◽

Sudden Death ◽

Stress Echocardiography ◽

Continuous Wave ◽

Left Ventricular ◽

Exercise Stress ◽

Exercise Stress Echocardiography ◽

Left Ventricular Outflow ◽

Orthostatic Position

Abstract Background Dynamic left ventricular outflow obstruction (LVOTO) during exercise stress echocardiography (ESE) is recommended in hypertrophic cardiomyopathy (HCM) to identify the obstructive phenotype. Aim To assess left ventricular outflow gradient (LVOTG) during ESE in different conditions. Methods In a single-group, prospective, observational study, we performed peak and/or post-treadmill ESE with systematic assessment of LVOTG in the orthostatic position by continuous-wave Doppler in 1333 subjects (837 males, mean age 38,2±20 ranging from 6 to 87 years) recruited over a period of twenty years, from 2001 to 2021. Peak LVOTG ≥30 mm Hg was considered abnormal for LVOTO during ESE. We enrolled 7 different populations: asymptomatic healthy controls (n=35); HCM (n=81); genotype-positive, phenotype negative asymptomatic HCM (n=6); patients with chest pain symptoms, suspected myocardial ischemia and either normal coronary arteries (INOCA, n=131,or with very low pre-test probability of coronary artery disease (probable INOCA, n=416) and; fatigue and suspected heart failure with preserved ejection fraction (HFpEF, n=206); amateur athletes with ischemia-like ECG changes during exercise-test or symptoms such as near syncope or chest pain or dizziness (n=457); aborted sudden death and with negative screening (n=1). Results Technical success rate of LVOTG assessment was 1333/1333 at rest and at peak stress (feasibility 100%). Imaging and analysis time were <1 minute. LVOTG at rest was present in 25 pts (2.8%) of the overall population: 23 HCM, 1 INOCA, and 1 HFpEF. Overall prevalence during ESE was 432/1333 (32%). During ESE, LVOTO (see Figure 1 and 2) was 0% (0/35) in normals, 58% (47/81) in HCM (23 with obstruction at rest), 33% (2/6) in genotype-positive, phenotype negative HCM, 37% (33/131) in INOCA, 40% (135/416) in athletes and 1/1 in the patient with aborted sudden death on strenuous exercise. Conclusion LVOTO in orthostatic position is detectable during treadmill ESE in several cardiovascular conditions associated with symptoms such as dyspnea, chest pain or near syncope, and even in asymptomatic patients with genotype-positive, phenotype-negative HCM. The identification of the obstructive phenotype is easy to capture during ESE without any significant additional imaging and analysis burden and can be important also outside HCM. FUNDunding Acknowledgement Type of funding sources: None. Figure 1 Figure 2

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