Acute coronary syndrome with non-obstructive coronary arteries (ACS-NOCA) in patients with hypertrophic cardiomyopathy

Puwanant, Sarinya; Trongtorsak, Angkawipa; Wanlapakorn, Chaisiri; Songsirisuk, Nattakorn; Ariyachaipanich, Aekarach; Boonyaratavej, Smonporn

doi:10.1186/s12872-021-02373-z

Cited by 9 publications

(6 citation statements)

References 32 publications

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“…Mechanisms that are unique to HCM include (i) increased left ventricular wall thickness and (ii) increased left ventricular intracavitary pressures due to outflow or mid‐cavitary obstruction 44 . Indeed, acute coronary syndrome without obstructive coronary artery is not an uncommon initial presentation (prevalence 9–14%) in patients with HCM 45 . Macrovascular disease is also common in HCM with a prevalence of up to 53% 46,47 .…”

Section: Discussionmentioning

confidence: 99%

“…44 Indeed, acute coronary syndrome without obstructive coronary artery is not an uncommon initial presentation (prevalence 9-14%) in patients with HCM. 45 Macrovascular disease is also common in HCM with a prevalence of up to 53%. 46,47 Bariatric surgery can theoretically improve control of chronic stable angina and prevent acute coronary syndrome in HCM via addressing risk factors of both microvascular and macrovascular disease (e.g.…”

Section: Potential Mechanismsmentioning

confidence: 99%

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Effects of bariatric surgery on cardiovascular‐related acute care use in patients with hypertrophic cardiomyopathy

et al. 2023

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Aims Prior studies have suggested causal relationships between obesity and acute cardiovascular events. It has been also known that the risk of acute cardiovascular events is reduced by bariatric surgery. However, little is known about whether bariatric surgery lowers the risk of acute cardiovascular events in patients with obesity and hypertrophic cardiomyopathy (HCM). In this context, we aimed to investigate whether bariatric surgery is associated with a reduced risk of cardiovascular‐related acute care use in patients with HCM. Methods and results In this population‐based study, the bariatric surgery group consisted of patients with HCM who underwent bariatric surgery from January 2004 to December 2014. The control group included those who have obesity and HCM and received non‐bariatric elective intra‐abdominal surgery during the same period. The outcome was cardiovascular‐related acute care use (i.e. emergency department (ED) visits or unplanned hospitalizations for cardiovascular disease) during a 1‐year post‐surgery period. We used the SPARCS database, a population‐based ED and inpatient database in New York State. We constructed logistic regression models with generalized estimating equations to compare the risk of the outcome events during sequential 6‐month post‐surgery periods. We adjusted for age, sex, number of ED visits and hospitalizations for cardiovascular disease within 2 years before the index surgery, and the Elixhauser co‐morbidity measures. We also performed propensity score (PS)‐matching and inverse probability treatment weighting analyses using these variables. The analytic cohort consisted of 207 adults with obesity and HCM, including 147 patients who underwent bariatric surgery and 60 in the control group. The risk was not significantly different in the 1–6 months post‐surgery period. By contrast, in the 7–12 months post‐surgery period, the risk of cardiovascular‐related acute care use was significantly lower in the bariatric surgery group (adjusted odds ratio 0.23; 95% CI 0.068–0.71; P = 0.01) compared with the control group. In the PS‐matched cohort, there were no significant differences in the baseline characteristics. The PS‐matched analysis demonstrated lower risk of the outcome event in the bariatric surgery group in the 7–12 months post‐surgery period. The inverse probability treatment weighting analysis replicated the findings. Conclusions Bariatric surgery was associated with a lower risk of cardiovascular‐related acute care use in the 7–12 months post‐surgery period in this population‐based study.

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Section: Discussionmentioning

confidence: 99%

Section: Potential Mechanismsmentioning

confidence: 99%

Effects of bariatric surgery on cardiovascular‐related acute care use in patients with hypertrophic cardiomyopathy

et al. 2023

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“…Davlat et al ( 23 ) demonstrated the association of left ventricular non-compaction with ACS. Moreover, Puwanant et al ( 24 ) presented a cohort of 200 patients with hypertrophic cardiomyopathy (HCM) suffering a higher prevalence of ACS. Therefore, it is essential to distinguish the onset of ACS as a significant symptom before the dominant phenotype of cardiomyopathies appears.…”

Section: Discussionmentioning

confidence: 99%

Misdiagnosed myocarditis in arrhythmogenic cardiomyopathy induced by a homozygous variant of DSG2: a case report

Liu

Zhang

et al. 2023

Front. Cardiovasc. Med.

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BackgroundArrhythmogenic cardiomyopathy (ACM) is an inherited cardiomyopathy that is rarely diagnosed in infants or young children. However, some significant homozygous or compound heterozygous variants contribute to more severe clinical manifestations. In addition, inflammation of the myocardium and ventricular arrhythmia might lead to misdiagnosis with myocarditis. Here, we describe an 8-year-old patient who had been misdiagnosed with myocarditis. Timely genetic sequencing helped to identify this case as ACM induced by a homozygous variant of DSG2.Case presentationThe proband of this case was an 8-year-old boy who initially presented with chest pain with an increased level of cardiac Troponin I. In addition, the electrocardiogram revealed multiple premature ventricular beats. Cardiac magnetic resonance revealed myocardial edema in the lateral ventricular wall and apex, indicating localized injuries of the myocardium. The patient was primarily suspected to have acute coronary syndrome or viral myocarditis. Whole-exome sequencing confirmed that the proband had a homozygous variation, c.1592T > G, of the DSG2 gene. This mutation site was regulated by DNA modification, which induced amino acid sequence changes, protein structure effects, and splice site changes. According to MutationTaster and PolyPhen-2 analyses, the variant was considered a disease-causing mutation. Next, we used SWISS-MODEL to illustrate the mutation site of p.F531C. The ensemble variance of p.F531C indicated the free energy changes after the amino acid change.ConclusionIn summary, we reported a rare pediatric case initially presenting as myocarditis that transitioned into ACM during follow-up. A homozygous genetic variant of DSG2 was inherited in the proband. This study expanded the clinical feature spectrum of DSG2-associated ACM at an early age. Additionally, the presentation of this case emphasized the difference between homozygous and heterozygous variants of desmosomal genes in disease progression. Genetic sequencing screening could be helpful in distinguishing unexplained myocarditis in children.

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“…Частота больных с инфарктом миокарда в анамнезе в нашем исследовании была намного выше, чем в Тайваньском исследовании (15 % и 3 %) [12], а частота больных с инфарктом миокарда без значимых стенозов коронарных артерий была сопоставима с результатами исследования Puwanant S. et al (7,5 % и 9 % соответственно) [13]. По-видимому, помимо атеросклероза коронарных артерий, при ГКМП микрососудистая дисфункция, стеноз интрамуральных коронарных артерий и дисфункция эндотелия могут способствовать развитию ишемии и/или инфаркта миокарда.…”

Section: Discussionunclassified

Features of Diagnostics and Course of Hypertrophic Cardiomyopathy in Real Clinical Practice

Reznik,

Nguyen,

Dikaeva

et al. 2023

Arhivʺ vnutrennej mediciny

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Introduction and purpose. Hypertrophic cardiomyopathy (HCM) is characterized by left ventricular (LV) wall thickening not associated with increased afterload (hypertension and aortic stenosis), is usually caused by mutations in sarcomeric protein genes, and is inherited in an autosomal dominant manner. Unlike HCM, myocardial hypertrophy in its phenocopies is associated with the accumulation of substances such as amyloid, glycogen, etc. in the myocardium. The aim of our work was to analyze the features of the diagnosis and course of HCM in real clinical practice. Material and methods. A retrospective analysis of medical records of 80 patients (56.3 % of men) discharged with a diagnosis of HCM from a multidisciplinary hospital in Moscow in the period from 2007 to 2021 was carried out. The diagnosis of HCM in all patients was established on the basis of echocardiography data. The median age (25th and 75th percentiles are indicated in brackets) was 57 (48.5; 63) years. The duration of hospitalization was 8 (6; 12.5) days. Results. The reason for hospitalization was angina syndrome in 35 %, suspicion of acute coronary syndrome in 16.3 %, paroxysmal atrial fibrillation (AF) in 11.3 %, decompensation of chronic heart failure in 11.3 %, syncope in 7.5 % %, hypertensive crisis in 3.8 %, coronary angiography in 3.8 %, pacemaker implantation in 2.5 %, consultation with an arrhythmologist in 2.5 %, implantation of a cardioverter-defibrillator in 1.2 %, medical examination to resolve the issue of fitness for military service in 1.2 %, acute cerebrovascular accident in 1.2 %, hypotension in 1.2 %, drug bradycardia in 1.2 % of patients. Before hospitalization, a history of myocardial infarction was diagnosed in 15 %, arterial hypertension — in 53.8 %, chronic heart failure — in 77.6 %, chronic kidney disease — in 21.3 % of patients. Prior to the analyzed hospitalization, a history of myocardial infarction was diagnosed in 15 %, arterial hypertension in 53.8 %, chronic heart failure in 77.6 %, chronic kidney disease in 21.3 % of patients. LV wall thickness ≥1.5 cm was detected in 91.2 %, symmetrical form of hypertrophy — 22.1 %, apical — 5.2 %, papillary muscle hypertrophy — 1.3 %, interventricular septum — 71.4 % of patients. Permanent obstruction of the LV outflow tract (LVOTO) was detected in 62.8 % (9.0 % of patients had a history of septal reduction), transient LVOTO — in 1.3 %, non-obstructive HCM — in 35.9 %. The ejection fraction (EF) of the LV (according to Simpson) was 63 (55-70) %, CHF with reduced LV EF <40 % was detected in 3.8 %, with a moderately reduced LV EF (40-49 %) — in 5 %, with preserved LV EF — in 68.8 % of patients. Anterior systolic movement of the anterior leaflet of the mitral valve occurred in 47.5 %, prolapse of the anterior leaflet of the mitral valve was described in 7.14 %. Mitral regurgitation was registered in 75 % of patients. 45 % of patients with HCM suffered from AF: permanent 15 %, paroxysmal 23.8 %, persistent 6.2 % of patients. During hospitalization, ventricular tachycardia was registered in 7.5 %, supraventricular tachycardia — 3.8 %, conduction disturbances were noted in 36.3 % of patients, of which atrioventricular block in 6.3 %, blockade of the right bundle branch block in 21.3 %, left bundle branch block in 15 %, and Wolff-Parkinson-White syndrome in 1.3 % . Implantation of a pacemaker in history was in 5 %, including in connection with MorgagniAdams-Stokes attacks — in 3.8 % of patients. During a median follow-up of 87 (interquartile range 45–131.5) months, 13.8 % of patients with HCM died. In deceased patients, LVOTO was significantly more common (in the dead 100 %, in the living 58.2 %, p = 0.006) and AF (in the dead 72.7 %, in the living 40.6 %, p = 0.047). Genetic testing and exclusion of HCM phenocopies was not performed during hospitalization and was not recommended for any patient. Conclusion. In real clinical practice, in most cases, only phenotypic diagnosis of HCM is carried out according to echocardiography, and screening for genetic mutations and HCM phenocopies is not performed. It is necessary to widely introduce genetic testing and screening for HCM phenocopies for the timely diagnosis of pathology that requires the appointment of specific pathogenetic therapy to improve the prognosis of patients

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Acute coronary syndrome with non-obstructive coronary arteries (ACS-NOCA) in patients with hypertrophic cardiomyopathy

Cited by 9 publications

References 32 publications

Effects of bariatric surgery on cardiovascular‐related acute care use in patients with hypertrophic cardiomyopathy

Effects of bariatric surgery on cardiovascular‐related acute care use in patients with hypertrophic cardiomyopathy

Misdiagnosed myocarditis in arrhythmogenic cardiomyopathy induced by a homozygous variant of DSG2: a case report

Features of Diagnostics and Course of Hypertrophic Cardiomyopathy in Real Clinical Practice

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