Case Of Hypertrophic Cardiomyopathy Research Articles

Screening of MYH7 gene mutation sites in hypertrophic cardiomyopathy and its significance.

Background:There have been few reports of mutations in the beta-myosin heavy chain (MYH7) gene in hypertrophic cardiomyopathy (HCM), which is associated with sudden cardiac death caused by HCM. This study aimed to screen the mutation sites in the sarcomeric gene MYH7 in Chinese patients with HCM. We also planned to analyze the pathogenicity of the mutation site as well as its significance in clinical and forensic medicine.Methods:From January 2006 to June 2017, autopsy cases were collected from the Department of Pathology, the Affiliated Hospital of Qingdao University. The experiment was to detect MYH7 gene status in formalin-fixed paraffin-embedded tissues from 18 independent autopsy cases who suffered HCM related sudden death (fatal HCM) and 20 cases without cardiomyopathy. Common mutation exon fragments of MYH7 gene were amplified by polymerase chain reaction. The end-of-deoxygenation method and gene cloning method were further performed to analyze the mutation sites. Homologous comparison among mutant sites was conducted using BLAST online database.Results:The 1336th nucleotide of MYH7 gene at exon 14 was converted from T to G in one HCM case, resulting in the conversion of threonine (Thr) at position 446 to proline (Pro). In another case, the 1402th nucleotide at exon 14 was converted from T to C, resulting in the conversion of phenylalanine (Phe) at position 468 to leucine (Leu). Homologous comparison results showed that the two amino acid residues of Thr446 and Phe468 are highly conserved among different species.Conclusions:Our results showed fatal HCM harbored mutations of Thr446Pro and Phe468Leu in the MYH7 gene. It is significant for clinical and forensic medicine to further explore the functions and detailed mechanisms of these mutations.

Whole MYBPC3 NGS sequencing as a molecular strategy to improve the efficiency of molecular diagnosis of patients with hypertrophic cardiomyopathy.

Hypertrophic cardiomyopathy (HCM) is the most common heritable cardiomyopathy, historically believed to affect 1 of 500 people. MYBPC3 pathogenic variations are the most frequent cause of familial HCM and more than 90% of them introduce a premature termination codon. The current study aims to determine the prevalence of deep intronic MYBPC3 pathogenic variations that could lead to splice mutations. To improve molecular diagnosis, a next-generation sequencing (NGS) workflow based on whole MYBPC3 sequencing of a cohort of 93 HCM patients, for whom no putatively causative point mutations were identified after NGS sequencing of a panel of 48 cardiomyopathy-causing genes, was performed.Our approach led us to reconsider the molecular diagnosis of six patients of the cohort (6.5%). These HCM probands were carriers of either a new large MYBPC3 rearrangement or splice intronic variations (five cases). Four pathogenic intronic variations, including three novel ones, were detected. Among them, the prevalence of one of them (NM_000256.3:c.1927+ 600 C>T) was estimated at about 0.35% by the screening of 1,040 unrelated HCM individuals.This study suggests that deep MYBPC3 splice mutations account for a significant proportion of HCM cases (6.5% of this cohort). Consequently, NGS sequencing of MYBPC3 intronic sequences have to be performed systematically.

A clinical case of hypertrophic cardiomyopathy and family hyperlipidemia

Introduction of the next generation sequencing to the clinical practice made it possible to accurately diagnose a number of cardiac diseases, and to study accumulation of pathological variants of genes in families thus identifying individuals at risk of the disease much earlier. The authors present a patient with two genetically determined cardiological diseases familial hyperlipidemia and hypertrophic cardiomyopathy.

Abstract 776: MYBPC3 Mutations in Indian Patients With Hypertrophic Cardiomyopathy

Introduction: Hypertrophic cardiomyopathy (HCM), the most common inherited heart disease, is characterized by thickening of the ventricular wall, myocyte disarray, and fibrosis, resulting in impaired cardiac function and leading to adverse ventricular remodeling, heart failure and increased risk for sudden cardiac death. Importantly, mutations in the gene encoding cardiac myosin binding protein-C ( MYBPC3 ), a muscle contractile protein,account for approximately 50% of the reported HCM cases. However, the prevalence of penetrance of MYBPC3 mutations in Indian patients with HCM is yet to be systematically studied. Methods: The Institutional Human Ethics Committee at the University of Puneapproved the present study. HCM patients undergoing treatment in theCardiology Unit of Bharti Hospital, Pune, were identified. With the approval of written informed consent, blood samples were obtained from all patients and their relatives and screened by single-strand conformation polymorphism (SSCP) and direct DNA sequencing, followed by correlation with clinical outcome. Results: Of the 23 probands screened in this study, one, #16, showed a mutation in exon 07 (A/G) at chromosomal position 47370041, causing a protein change at position NP_000247.2:p.S236G, and reported as SNP status ID rs386584787. The mutation was localizedto the immunoglobulin domain of cardiac myosin binding protein-C, causing an amino acid change from serine to glycine with a score of 0.00, as shown by thePolyPhen-2 (Polymorphism Phenotyping v2) tool.G>C transversion of intron variant in exon 21 of MYBPC3 results in a new exon splicing enhancer (ESE) site which may alter splicing. G>A transition at g. 20167 is an intron variant of a previously reported status (rs373904644). This polymorphism results in the creation of an intronic splicingenhancer (ISE) site. The proband had left ventricular wall thickness of 24 mm, ejection fraction of 60 (%), and left ventricular internal diameter of 30 mm, suggesting early onset of HCM. Conclusion: The MYBPC3 mutations might play a key role in thedevelopment of HCM in the Indian population, indicating the soundness of establishing a systematic screening of all HCM patients.

Case Study: Dilated Cardiomyopathy Evolves into Apical-Variant Hypertrophic Cardiomyopathy

Introduction Apical-variant hypertrophic cardiomyopathy (HCM) is a form of HCM in which hypertrophy is limited to the left-ventricular apex. Apical-variant HCM is rarely obstructive, though there is still an increased risk of atrial fibrillation, myocardial infarction, and sudden cardiac death (SCD). Apical-variant HCM has not previously been described in a patient with a prior diagnosis of dilated cardiomyopathy (DCM). Case A 53-year-old female with a history of hypertension and type-II diabetes mellitus presented with progressive dyspnea on exertion, fatigue and lower extremity swelling. Family history was significant for SCD of her mother at age 45. An initial transthoracic echocardiogram (TTE) showed DCM with an ejection fraction (EF) 15% and left ventricular end diastolic dimension (LVEDD) of 6.8 cm. The patient was placed on guideline-directed medical therapy (GDMT) and ultimately received cardiac resynchronization therapy with a defibrillator (CRT-D). One year later, EF was 25% with LVEDD 7.1 cm (Figure 1). A subsequent TTE two years later showed resolution of DCM with EF 55% and LVEDD of 5.1 cm. However, a new finding on this TTE was apparent: the apex demonstrated a spade-like shape on contraction consistent with apical-variant HCM (Figure 2). The patient underwent genetic testing but no genetic cause of either DCM or HCM was identified. Discussion HCM is caused by autosomal-dominant inheritance of a defective gene in sarcomere proteins. DCM and HCM are linked in that some etiologies of DCM are driven by sarcomeric protein abnormalities. DCM is also a rare phenotypical variant of HCM generally occurring very late in the disease and evolving from an initially hypertrophic myocardium. The opposite, in which DCM evolves into apical-variant HCM, to the best of our knowledge, has not been described in the literature. This relationship, as well as a family history of early SCD, suggest an inherited sarcomeric disorder manifesting as two discrete phenotypes within the same individual. This evolution may be due to treatment with GDMT, or it may be a rare expression of the disease's natural history. Only a fraction of cases of idiopathic DCM and HCM have an identifiable genetic cause and, thus, lacking an identified mutation does not preclude an inherited cardiomyopathy.

Analysis of 51 proposed hypertrophic cardiomyopathy genes from genome sequencing data in sarcomere negative cases has negligible diagnostic yield

PurposeIncreasing numbers of genes are being implicated in Mendelian disorders and incorporated into clinical test panels. However, lack of evidence supporting the gene-disease relationship can hinder interpretation. We explored the utility of testing 51 additional genes for hypertrophic cardiomyopathy (HCM), one of the most commonly tested Mendelian disorders. MethodsUsing genome sequencing data from 240 sarcomere gene negative HCM cases and 6229 controls, we undertook case-control and individual variant analyses to assess 51 genes that have been proposed for HCM testing. ResultsWe found no evidence to suggest that rare variants in these genes are prevalent causes of HCM. One variant, in a single case, was categorized as likely to be pathogenic. Over 99% of variants were classified as a variant of uncertain significance (VUS) and 54% of cases had one or more VUS. ConclusionFor almost all genes, the gene-disease relationship could not be validated and lack of evidence precluded variant interpretation. Thus, the incremental diagnostic yield of extending testing was negligible, and would, we propose, be outweighed by problems that arise with a high rate of uninterpretable findings. These findings highlight the need for rigorous, evidence-based selection of genes for clinical test panels.

TNNT2 5 bp deletion, sedentary lifestyle & abdominal adiposity accentuate the phenotypic severity in hypertrophic cardiomyopathy patients

Hypertrophic cardiomyopathy (HCM) is a common cardiac genetic disorder having phenotypic heterogeneity, which can be attributed to interaction of certain environmental risk factors and lifestyle modifications. HCM cases and clinically evaluated controls were evaluated for lifestyle, anthropometric, physiological and biochemical variables, followed by screening of HCM associated genetic variations (5bpDel in TNNT2, 25bpDel in MYBPC3, 287bpDel in ACE and T-786C in eNOS). Sedentary lifestyle, waist-hip ratio, total serum cholesterol and triglycerides along with 5bpDel in TNNT2 gene were found to be significantly associated with the disease condition (p < 0.05; adjusted for age and sex). Further analysis of these significant risk factors in cases shows that sedentary lifestyle, waist-hip ratio along with 5 bp Del in TNNT2 gene had an added effect on disease severity. Among HCM patients, we found that LV and IVS wall thickness are more regulated by physical activity as compared to genetic defects. This study infers that these modifiable risk factors may not express independently but plays an important role in disease phenotype as a contributor, with major/minor risk stratification. Management of risk factors such as physical activity and eating habit may benefit HCM patients having TNNT2 deletion polymorphism in living a quality life with good prognosis.

Genetic basis and outcome in a nationwide study of Finnish patients with hypertrophic cardiomyopathy.

AimsNationwide large‐scale genetic and outcome studies in cohorts with hypertrophic cardiomyopathy (HCM) have not been previously published.Methods and resultsWe sequenced 59 cardiomyopathy‐associated genes in 382 unrelated Finnish patients with HCM and found 24 pathogenic or likely pathogenic mutations in six genes in 38.2% of patients. Most mutations were located in sarcomere genes (MYBPC3, MYH7, TPM1, and MYL2). Previously reported mutations by our study group (MYBPC3‐Gln1061Ter, MYH7‐Arg1053Gln, and TPM1‐Asp175Asn) and a fourth major mutation MYH7‐Val606Met accounted for 28.0% of cases. Mutations in GLA and PRKAG2 were found in three patients. Furthermore, we found 49 variants of unknown significance in 31 genes in 20.4% of cases. During a 6.7 ± 4.2 year follow‐up, annual all‐cause mortality in 482 index patients and their relatives with HCM was higher than that in the matched Finnish population (1.70 vs. 0.87%; P < 0.001). Sudden cardiac deaths were rare (n = 8). Systolic heart failure (hazard ratio 17.256, 95% confidence interval 3.266–91.170, P = 0.001) and maximal left ventricular wall thickness (hazard ratio 1.223, 95% confidence interval 1.098–1.363, P < 0.001) were independent predictors of HCM‐related mortality and life‐threatening cardiac events. The patients with a pathogenic or likely pathogenic mutation underwent an implantable cardioverter defibrillator implantation more often than patients without a pathogenic or likely pathogenic mutation (12.9 vs. 3.5%, P < 0.001), but there was no difference in all‐cause or HCM‐related mortality between the two groups. Mortality due to HCM during 10 year follow‐up among the 5.2 million population of Finland was studied from death certificates of the National Registry, showing 269 HCM‐related deaths, of which 32% were sudden.ConclusionsWe identified pathogenic and likely pathogenic mutations in 38% of Finnish patients with HCM. Four major sarcomere mutations accounted for 28% of HCM cases, whereas HCM‐related mutations in non‐sarcomeric genes were rare. Mortality in patients with HCM exceeded that of the general population. Finally, among 5.2 million Finns, there were at least 27 HCM‐related deaths annually.

Clinical and mutation profile of pediatric patients with RASopathy-associated hypertrophic cardiomyopathy: results from a Chinese cohort

BackgroundThe RASopathies are a class of developmental disorders caused by germline mutations in the RAS-mitogen-activated protein kinase (MAPK) pathway. Hypertrophic cardiomyopathy (HCM) has been frequently described in children with RASopathy, but only a minority of patients have received formal genotyping. The purpose of this study was to evaluate the genetic basis and clinical outcome of pediatric patients with RASopathy-associated HCM.MethodsWe retrospectively reviewed the mutation spectrum and clinical outcome of all the patients with RASopathy derived from 168 pediatric HCM cases referred to our institution between January 2012 and July 2018.ResultsA heterozygous missense mutation in one of known RASopathy genes was identified in 46 unrelated children with HCM. Mutations in the PTPN11 gene were the most prevalent (19/46); this was followed by mutations in RAF1 (11/46), KRAS (5/46), RIT1 (4/46), BRAF (3/46), SOS1 (2/46), HRAS (1/46), and SHOC2 (1/46). Moreover, two compound heterozygous missense mutations in the LZTR1 gene were identified in one patient with the Noonan syndrome phenotype and HCM. The median age at the diagnosis of HCM was 3.0 months (range 0 months to 8.1 years). Twenty-one of the patients had significant left ventricular outflow tract obstruction and 32 had concomitant congenital heart disease. Three patients with a mutation in exon 13 of the PTPN11 gene died of cardiac failure at the ages of 3.0, 3.5, and 6.0 months. The remaining 44 patients were alive after an average follow-up time of 3.9 years (0.5 to 17.1 years, median 2.9 years) from the initial diagnosis of HCM, including 5 patients with spontaneous regression of their cardiac hypertrophy.ConclusionsRASopathy-associated HCM is a heterogeneous genetic condition characterized by early-onset cardiac hypertrophy and a high prevalence of co-existing congenital heart disease, which is most frequently related to specific mutations in the PTPN11 gene. Rapidly progressive HCM, resulting in an early death, is uncommon in RASopathy patients except those with specific mutations in exon 13 of the PTPN11 gene.

Biventricular Hypertrophic Cardiomyopathy with Severe Right Ventricular Outflow Track Obstruction

Hypertrophic cardiomyopathy (HCM) has diverse pathophysiological and clinical features, according to the extent and severity of the hypertrophy development. Hypertrophy mostly involves the left ventricle and sometimes causes a left ventricular outflow tract obstruction. Right ventricular involvement is less frequent, and even the severe form of a right ventricular outflow tract (RVOT) obstruction by concurrent right ventricular hypertrophy in a patient with HCM is rare. We report a case of biventricular HCM with a clinically, morphologically, and hemodynamically significant RVOT obstruction, which had been treated successfully with surgical myectomy. Keywords: Hypertrophic cardiomyopathy; Biventricular hypertrophy; Right ventricular outflow tract obstruction 중심 단어: 비후성 심근증; 양 심실 비후; 우심실 ìœ ì¶œë¡œ 폐쇄

Quantitative approaches to variant classification increase the yield and precision of genetic testing in Mendelian diseases: the case of hypertrophic cardiomyopathy

BackgroundInternational guidelines for variant interpretation in Mendelian disease set stringent criteria to report a variant as (likely) pathogenic, prioritising control of false-positive rate over test sensitivity and diagnostic yield. Genetic testing is also more likely informative in individuals with well-characterised variants from extensively studied European-ancestry populations. Inherited cardiomyopathies are relatively common Mendelian diseases that allow empirical calibration and assessment of this framework.MethodsWe compared rare variants in large hypertrophic cardiomyopathy (HCM) cohorts (up to 6179 cases) to reference populations to identify variant classes with high prior likelihoods of pathogenicity, as defined by etiological fraction (EF). We analysed the distribution of variants using a bespoke unsupervised clustering algorithm to identify gene regions in which variants are significantly clustered in cases.ResultsAnalysis of variant distribution identified regions in which variants are significantly enriched in cases and variant location was a better discriminator of pathogenicity than generic computational functional prediction algorithms. Non-truncating variant classes with an EF ≥ 0.95 were identified in five established HCM genes. Applying this approach leads to an estimated 14–20% increase in cases with actionable HCM variants, i.e. variants classified as pathogenic/likely pathogenic that might be used for predictive testing in probands’ relatives.ConclusionsWhen found in a patient confirmed to have disease, novel variants in some genes and regions are empirically shown to have a sufficiently high probability of pathogenicity to support a “likely pathogenic” classification, even without additional segregation or functional data. This could increase the yield of high confidence actionable variants, consistent with the framework and recommendations of current guidelines. The techniques outlined offer a consistent and unbiased approach to variant interpretation for Mendelian disease genetic testing. We propose adaptations to ACMG/AMP guidelines to incorporate such evidence in a quantitative and transparent manner.

Electrocardiographic and echocardiographic evaluation of a large cohort of peri-pubertal soccer players during pre-participation screening.

The early diagnosis of cardiac abnormalities in young athletes may be helpful not only to identify subjects potentially at risk of sudden cardiac death but also to prevent stress-related cardiac dysfunction and cardiovascular events during the life of these subjects. The aim of our study was to investigate the prevalence of cardiac abnormalities in a population of young male soccer players undergoing pre-participation screening through electrocardiogram and trans-thoracic echocardiography. All consecutive male football players undergoing pre-participation screening comprehensive of medical history, physical examination, 12-lead electrocardiogram and trans-thoracic echocardiography at the FMSI Sport Medicine Institute in Rome between January 2008-March 2009 were enrolled in the study. Overall, 2261 consecutive young athletes aged 12.4 ± 2.6 years were evaluated. Training-unrelated electrocardiogram abnormalities were observed in 65 (2.9%) athletes. Abnormal trans-thoracic echocardiography was observed in 102 athletes (4.5%), including two cases of hypertrophic cardiomyopathy, eight of mild left ventricular hypertrophy, six of mild left ventricular dilation and 17 of bicuspid aortic valve. An abnormal electrocardiogram was associated with anomalous trans-thoracic echocardiography in 11/65 (16.9%) cases. All athletes requiring sport disqualification were identified by electrocardiogram. Notably, among 2216 athletes with a normal electrocardiogram, 91 had abnormal trans-thoracic echocardiography, including six cases of left ventricular dilation and six of ventricular hypertrophy. In a wide population of peri-pubertal male athletes, evaluation of the electrocardiogram identified all cardiac diseases requiring sport disqualification. Trans-thoracic echocardiography alone allowed the identification of cardiac abnormalities potentially leading to cardiomyopathies or major cardiovascular events over time.

A case of hypertrophic cardiomyopathy combined with muscular ventricular septal defect and abnormal origin of right coronary artery

BackgroundHypertrophic cardiomyopathy (HCM) is a myocardial disease. However, the coexistence of HCM with muscular ventricular septal defect (VSD), especially those with both incomplete spontaneous closure and coronary abnormal origin, is relatively rare.Case presentationWe report herein a unique case of HCM accompanied with incomplete spontaneous closure of muscular VSD and abnormal origin of right coronary artery (RCA) in a 26-year-old man, which was diagnosed by combination of transthoracic 2-dimensional (2D), color Doppler, Contrast-enhanced echocardiography and computed tomography angiography (CTA).ConclusionsTo our knowledge, this is the first report that HCM along with the incomplete spontaneous closure of muscular VSD and anomalous RCA arising from left coronary sinus was revealed through combination of transthoracic 2D, color Doppler, Contrast-enhanced echocardiography and CTA. These observations indicated that other associated anomalies in patients with HCM could be easily missed if examined by the single echocardiography. Therefore, HCM-associated congenital abnormalities should be screened by combination of transthoracic 2D, color Doppler, contrast-enhanced echocardiography, and CTA.

A chance encounter with a case of hypertrophic cardiomyopathy.

A chance encounter with a case of hypertrophic cardiomyopathy.

Mid cavity hypertrophic obstructive cardiomyopathy with apical aneurysm

Hypertrophic cardiomyopathy (HCM) with mid-ventricular cavity obstruction and apical aneurysm formation is a rare phenomenon. It is associated with worse outcomes due to increased incidence of ventricular tachycardia, thrombus formation and stroke apart from other traditional HCM complications such as outflow tract obstruction and heart failure. It can occasionally be missed on traditional echocardiography and better delineated by cardiac catheterization on left ventriculogram. We present a case of HCM with mid-ventricular obstruction and apical aneurysm formation who presented with stroke with hospital course complicated by ventricular tachycardia requiring an implantable cardioverter defibrillator.

A Rare Case of Hypertrophic Cardiomyopathy Associated with Congenital Mitral Stenosis

A Rare Case of Hypertrophic Cardiomyopathy Associated with Congenital Mitral Stenosis

Texture analysis of cardiovascular magnetic resonance cine images differentiates aetiologies of left ventricular hypertrophy

To investigate whether unenhanced cardiovascular magnetic resonance (CMR) balanced steady state free precession (bSSFP) cine images could be analysed using textural analysis (TA) software to differentiate different aetiologies of disease causing increased myocardial wall thickness (left ventricular hypertrophy [LVH]) and indicate the severity of myocardial tissue abnormality. A mid short axis unenhanced cine frame of 216 patients comprising 50 cases of hypertrophic cardiomyopathy (HCM; predominantly Left ventricular outflow tract obstruction [LVOTO] subtype), 52 cases of cardiac amyloid (CA; predominantly AL: light chain subtype), 68 cases of aortic stenosis (AS), 15 hypertensive patients with LVH (HTN+LVH), and 31 healthy volunteers (HV) underwent TA of the CMR cine images (CMRTA) using TexRAD (TexRAD Ltd, Cambridge, UK). Among the HV, 16/31 were scanned twice to form a test-retest reproducibility cohort. CMRTA comprised a filtration-histogram technique to extract and quantify features using six parameters. Test-retest analysis in the HV showed a medium filter (3 mm) was the most reproducible (intra-class correlation of 0.9 for kurtosis and skewness and 0.8 for mean and SD). Disease cohorts were statistically different (p<0.001) to HV for all parameters. Pairwise comparisons of CMRTA parameters showed kurtosis and skewness was consistently significant in ranking the degree of difference from HV (greatest to least): CA, HCM, LVH+HTN, AS (p<0.001). Similarly, mean, standard deviation, entropy, and mean positive pixel (MPP) were consistent in ranking degree of difference from HV: HCM, CA, AS and HTN+LVH. Radiomic features of bSSFP CMR data sets derived using TA show promise in discriminating between the aetiologies of LVH.

Genetic basis of hypertrophic cardiomyopathy in children.

Previous investigations assessing the genetic cause of pediatric hypertrophic cardiomyopathy (HCM) found underlying genetic mutations in 50-60% of cases. The purpose of our study was to analyze whether this number can be augmented by applying next-generation sequencing and directing further diagnostics by discussing unsolved cases in a multidisciplinary board. 42 patients with the diagnoses of HCM made before age 18years were treated in our center from 2000 to 2016. Genetic analysis was performed in 36 subjects, a genetic defect was detected in 29 (78%) patients. 15 individuals (42%) had pathogenic variants in genes encoding sarcomere proteins, and 5 (14%) in genes coding for components of the RAS/MAPK signaling pathway. 4 subjects (11%) had mutations in the GAA gene (Pompe disease), and 3 (8%) had Frataxin repeat expansions (Friedreich's ataxia). One patient each showed a mutation in BAG3 and LMNA. Discussion of unsolved HCM cases after performing next-generation sequencing (28 genes) in an interdisciplinary board unraveled the genetic cause in 9 subjects (25%). A definite genetic diagnosis can be reached in nearly 80% with HCM of childhood onset. Next-generation sequencing in conjunction with a multidisciplinary cooperation can enhance the diagnostic yield substantially. This may be important for risk stratification, treatment planning and genetic counseling.

Hypertrophic Obstructive Cardiomyopathy and Pregnancy: A Clinical Case Series

AbstractHypertrophic obstructive cardiomyopathy (HOCM) is a primary myocardial disorder caused by mutations in several different genes coding for contractile proteins. Although HOCM is being increasingly diagnosed in women of reproductive age due to widespread use of echocardiography and screening programs, there are fewer studies of HOCM in pregnancy owing to the rarity of the disease and poor recognition. Our clinical case series includes five cases of hypertrophic cardiomyopathy and its management. The authors describe five women presenting with HOCM with varied symptoms. All patients except one had favorable outcome. Neonatal outcome was good except for a second trimester intrauterine fetal demise. Intensive care unit (ICU) requirement ranged from 5 to 10 days. Two cases were established cases of HOCM. Pain relief was adequate with narcotics, although regional anesthesia was given to one with HOCM with corrected obstruction. No hypotension or deterioration was noted. Although it is believed that HOCM is well tolerated in pregnancy, it is important to have the diagnosis since management differs. Inadvertent regional anesthesia or hypotension due to blood loss or heightened sympathetic response to pain may prove detrimental to these patients. Maternal and fetal outcome are good. Prenatal routine echocardiography screening may prove efficient in identifying high-risk women, for early referral and better management.

Three Separate Coronary Arteries with Hypertrophic Cardiomyopathy

Coronary arteries anomalies are an uncommon congenital heart disease. Anomalous origin of three separate ostia is the rarest condition. Hypertrophic cardiomyopathy is the most common inherited disease. There have been just few cases of hypertrophic cardiomyopathy in relation with congenital coronary anomaly. In this report, we describe a rare case of anomalous origin of all three coronary arteries from separate ostia with apical hypertrophic cardiomyopathy.J MEDICINE JUL 2018; 19 (2) : 136-137