Concentric Hypertrophic Cardiomyopathy Research Articles

THE DIAGNOSTIC VALUE OF CARDIAC MRI IN DETERMINING HYPERTROPHIC CARDIOMYOPATHY PHENOTYPES Using T1 and T2 mapping and late gadolinium enhancement to accurately diagnose hypertrophic cardiomyopathy

Objective: Hypertrophic cardiomyopathy (HCM) is a genetic disorder characterized by thickening of the heart muscle and may have serious consequences. Diversity in the distribution and degree of hypertrophy has led to the classification of HCM types. Cardiac MR (CMR) imaging is a non-invasive tool that maintains accurate and detailed data about cardiac functions and structure. The aim of the study was to observe and compare the CMR characteristics of the three HCM morphologic types (asymmetric, concentric, and apical). Materials and Methods: 56 patients (female/male: 29/58, mean age 46.31±15.12, body surface area (BSA) 1.88±0.19 m2) who were referred for HCM diagnosis or follow-up between January 2020- January 2023 were included in this single-center retrospective study. The imaging procedure was conducted using a 1.5 T device (Aera, Siemens Medical Systems, Erlangen, Germany). Cardiac volume and functions were determined quantitatively. The location and amount of late gadolinium enhancement (LGE) was evaluated qualitatively. The statistical analyses were performed using SPSS version 25.0. A P value under 0.05 was considered statistically significant. Results: LGE revealed myocardial fibrosis in apical HCM (ApHCM), a higher prevalence than in asymmetric and concentric HCM. ApHCM and asymmetric HCM patients widely presented midmyocardial fibrosis, where most concentric HCM patients had patchy involvement. The mean native myocardial T1 values for individuals with concentric HCM was found to be higher compared to those with asymmetric and ApHCM and the healthy participants. Conclusion: CMR imaging enables the multiparametric evaluation of HCM through the utilization of native T1 and T2 mapping techniques, as well as LGE. Both T1 and T2 values have the potential to be used in conjunction with LGE to identify regions of myocardial fibrosis.

Hypertrophic cardiomyopathy in a girl with Danon disease

A prospective observation for 4 years (from 14 to 17 years) is presented for a girl with Danone’s disease. The early onset of the disease was noted, the clinical picture was dominated by syncopal and syncopal conditions. According to the clinical picture, presyncopal conditions were of a vasovagal nature, which was verified by the results of the Tilt test when the vasodepressor variant was detected. The disease was characterized by isolated cardiac disorders in the form of concentric hypertrophic cardiomyopathy, widespread fibrous changes in the myocardium. According to the standard ECG and daily ECG monitoring, ventricular preexitation syndrome was detected. Electrophysiological examination established the nodo-ventricular tract. Myopathy, cognitive impairment and visual changes were absent. A mutation in the heterozygous state was detected in exon 5 of 9 exons of the LMP2 gene, leading to the acquisition of a premature stop codon, was identified. X:g.119581719G>A ENST00000434600.2: c.718C>T. The mutation was verified by Sanger. The case in the pedigree is sporadic. As a prevention of sudden cardiac death, implantation of a cardioverter-defibrillator was performed. Effective relief of induced stable ventricular fibrillation (Burst stimulation protocol) was achieved only with the use of a ventricular electrode with two shock coils.

Increasing Trends in Obesity-Related Cardiovascular Risk Factors in Romanian Children and Adolescents-Retrospective Study.

Childhood obesity has become a global public health issue and its assessment is essential, as an obese child is a future overweight or obese adult. Obesity is no longer a matter of exercising more and eating less, with several factors coming into play and dictating the pattern of fat accumulation and the ease/difficulty of reducing it. In the current paper, we aimed to analyze the cardiovascular impact of obesity in a large number of patients alongside the paraclinical changes that occur due to weight gain, and to perform an analysis on the increase in prevalence throughout our research. The main cardiovascular conditions identified were hypertension (15.36%), septal or concentric hypertrophic cardiomyopathy (11.15%), atherosclerosis risk (13.04%), and hypercholesterolemia (20.94%). We have used echocardiography to measure the thickness of epicardial adipose tissue (useful for assessing the patient's cardiovascular risk), and we observed that it was greater in children with moderate and severe obesity alongside diastolic dysfunction of the left ventricle in the whole group, without any connection with hypertension or coronary impairment. Obese children will be affected by increased cardiovascular mortality and morbidity in adulthood and they may experience early cardiovascular dysfunction. We want to strongly underline the importance and necessity of programs for the early detection and prevention of obesity and its complications, especially since interesting phenomena such as the "obesity paradox" exist and prove that obesity is far less understood than it is at a first glance.

Can Diastolic Dysfunction Grade Distinguish Cardiac Amyloid From Other Causes of Pathologic Concentrically Increased Left Ventricular Wall Thickness?

Determining the underlying cause of increased left ventricular (LV) wall thickness on echocardiography can be difficult. Cardiac amyloid (CA) is one such cause and can be associated with severe diastolic dysfunction (DD). To compare the grade of DD between four groups of patients with concentrically increased LV wall thickness. Eighty-seven unselected, consecutive patients with diagnosed cardiomyopathy were included: 19 concentric hypertrophic cardiomyopathy (cHCM), 23 Fabry disease, 25 hypertension (HTN) and 20 cardiac amyloid (CA). DD was graded according to the 2016 ASE/EACVI guidelines. Grade 1 DD was seen in 11 (58%), 15 (65%), 21 (84%) and 5 (25%) patients from the HCM, Fabry, HTN and CA groups respectively (p=0.0039). No significant difference occurred in the distribution of Grade 2 DD. Grade 3 DD was seen in 3 (16%), 1 (4%), 0 and 11 (55%) patients from the HCM, Fabry, HTN and CA groups respectively (p<0.0001). Only 6% of non-CA patients had Grade 3 DD (p<0.0001, compared to CA). The sensitivity and specificity of Grade 3 DD for CA was 55% and 94%, respectively. Low tissue velocities (e’, a’, s’ all < 5cm/s) were present in 50% of CA patients, and the combination of this marker with Grade 3 DD was 100% specific for CA. Severe DD occurred disproportionately more frequently in CA and the combination of this and low tissue velocities <5 cm/s had high specificity for CA. The clinical corollary is that patients with this combination should be considered to have CA until proven otherwise.

Dimensional Assessment of the Heart in Hypertrophic Cardiomyopathy Patients in Tertiary Care Hospitals

Hypertrophic cardiomyopathy (HCM) is one of the most prevalent disorders responsible for sudden cardiac death. Presentation of the symptoms varies due to the degree of thickening, and functional ability of the cardiomyocytes. The aims of the current study were to assess the clinical features, and cardiac morphology. This was a descriptive study with some analytical components. Thirty-four adult HCM patients were included within a duration of four months by patient selection checklist. After informed written consent, relevant information was noted and analyzed. Frequency distribution of phenotypes were, 56% asymmetric septal hypertrophy, 29% concentric hypertrophy, and 15% apical hypertrophy. Breathlessness and chest discomfort were present in 56% and 62% patients respectively, and higher in asymmetric septal HCM. Palpitation was very frequent in concentric HCM (90%). ECG revealed left ventricular hypertrophy in 85% of patients, and 79% of them had ST change. The interventricular septal thickness was narrower in apical type (14.80mm). The posterior wall thickness was higher in concentric HCM (19.20mm). The left atrial size was smaller in Concentric type(34.60mm).

Wolff Parkinson white pattern in Danon disease: When preexcitation is not what it seems

Danondisease is a rare genetic disorder with an X-linked dominant inheritance affecting both skeletal and cardiac muscle. Its characteristic cardiac phenotype consists on a severe, non-obstructive and concentric hypertrophic cardiomyopathy (HCM) usually associated with a Wolff-Parkinson-White (WPW) type preexcitation pattern. Whether this corresponds to the presence of an AV or another type of accessory pathways, such as fasciculoventricular pathways (FVP) remains controversial in the literature. However, we describe the case of a teenager with Danon disease and preexcitation who develops a first degree AV block without any change in his QRS morphology, fact that favors the hypothesis of the presence of a FVP. This finding has important clinical implications in the management and prognosis of these complex patients. The absence of an AV accessory pathway decreases their risk of potential SCD in the context of a fast atrial arrhythmia and their chances of having a reentrant AV tachycardia.

6 Prevalence and CMR characteristics of apical HCM: Abstract 6 Table 1

Hypertrophic cardiomyopathy (HCM) is the most common cause of sudden cardiac death in young adults. The 3 main phenotypes are asymmetric (most common), concentric and apical. Literature suggests apical HCM is rare and more benign, but data is scarce. We sought to describe prevalence and characteristics of apical HCM in a large CMR service. Methods We reviewed 3,100 scans (Jan 2014–Mar 2015). Protocol included cines, early and late gadolinium enhancement imaging. 114 consecutive HCM patients were identified. Asymmetric HCM defined as septal/ free wall thickness ratio > 1.3; apical HCM as apical wall thickness > 15mm or apical/basal wall thicknesses ≥ 1.3–1.5. Concentric HCM defined as symmetrical hypertrophy of ventricular wall without regional preferences. Non-apical HCM (asymmetric and concentric phenotypes) were compared with apical HCM. Fisher’s exact t-test and unpaired t-test were performed for statistical significance (P-value Results 10 patients were excluded, leaving 104 patients, median age 60years; 70% male. 70% had non-apical HCM (5 patients concentric HCM, the rest asymmetric HCM) and 30% apical HCM. Mean maximum LV wall thickness, indexed LV mass, stroke volume, prevalence of LVOTO and SAM were greater in non-apical group. Presence of LGE was high in both groups (>85%) and wasn’t statistically different. Univariate predictors of apical HCM included maximum LV wall thickness, indexed stroke volume, LVOT obstruction (Table 1). In the multivariate model, maximum wall thickness remained the only significant predictor. Conclusions Our study suggests that prevalence of apical HCM is almost 1/3rd of all observed cases. It also demonstrates that prevalence of LGE was high in apical HCM, suggesting that better prognosis of apical HCM based on the absence of myocardial fibrosis, should be reconsidered. Further large trials are needed to better understand the pathophysiology.

Survival and clinical behavior of hypertrophic cardiomyopathy in a latin american cohort in contrast to cohorts from the developed world.

BackgroundHypertrophic cardiomyopathy (HCM) is the most common hereditary heart disease with diverse phenotipyc, genetic expession and clinical presentations. The evolution of patients with HCM in Latin America has not been properly described being the frequency, the long-term prognosis as well as the predominant phenotypic expression still unknown. The aim of this study was to determine the survival rate of HCM patients having different phenotypes in a Mexican cohort of patients.MethodsClinical and echocardiographic data obtained from 77 Mexican patients with recently diagnosed HCM were analyzed. The follow-up was of 12.5 years.Results96.1% of patients were in functional class I/II according to the New York Heart Association, 2.6% in class III and 1.3% in class IV. Only 3.9% of them went to surgery for myectomy. During the follow-up, 17 patients (22%) died: 4/9 (44%) had apical HCM, 5/20 (25%) had obstructive septal asymmetric HCM, 6/35 (17%) had nonobstructive septal asymmetric HCM and 2/3 (15%) had concentric HCM. The survival rate was worse for patients with apical HCM, followed by those with obstructive and nonobstructive septal asymmetric HCM and patients showing concentric HCM had the best survival rates. There is significant difference in survival rates which declined in 65% in a 9 years-period. Log rank test showed significant differences (p < 0.002).ConclusionThe survival rate of patients with HCM was worse in those with apical variety. The majority of patients received medical treatment. The indication for myectomy was below that observed in other international centers.

Successful electrical cardioversion in a massive concentric hypertrophic cardiomyopathy with atrial fibrillation

A 59-year-old man with a known history of nonobstructive hypertrophic cardiomyopathy and chronic atrial fibrillation was admitted to our clinic with weakness, palpitation, and exertional dyspnea. Electrocardiogram showed atrial fibrillation with high ventricular rate (120 beats per minute), intraventricular conduction delay, and left ventricular (LV) hypertrophy with ST-segment depression and inverted T waves. A transthoracic echocardiogram showed massive LV concentric hypertrophy. Although there was no gradient increase in the LV outflow tract, marked turbulent flow was seen in midventricular region by colored Doppler echocardiography. On the fourth day of admission, transesophageal echocardiography was done and showed no thrombus in the left atrium. Electrical cardioversion with 100 J was applied to the patient, and atrial fibrillation was returned to sinus rhythm. His control Doppler echocardiogram revealed peak systolic resting gradient of 54 mm Hg, with an increase to 84 mm Hg at Valsalva maneuver at the LV outflow. Cardiac magnetic resonance showed concentric LV hypertrophy with a 35-mm thickness in diastole, mild scar tissue in LV anterior wall midapical segments, and right ventricle wall thickness with a 10 mm in diastole. There was no bradycardia or tachycardia in 24-hour Holter and exercise electrocardiographic testing.

Mechanisms of exercise intolerance in patients with hypertrophic cardiomyopathy

To determine the relation between echocardiogram findings and exercise capacity in hypertrophic cardiomyopathy (HCM). Sixty-three patients (48 +/- 15 years) were referred for cardiopulmonary testing and exercise echocardiography. They were classified by morphology: proximal (n = 11), reverse curvature (n = 32), apical (n = 7), and concentric HCM (n = 13). There were more women in proximal and reverse curvature groups. Proximal HCM patients were older. Maximal left ventricular thickness was highest in reverse curvature group. At peak exercise, concentric HCM achieved the lowest percent predicted maximal Vo2. Excluding apical group, no significant differences in gradient were noted between groups. Overall, no statistically significant correlation was found between peak Vo2, wall thickness, and gradient. Significant correlations were noted between peak Vo2 and indexed left atrial (LA) volume (r = -0.52), lateral E' (r = 0.50), and lateral E/E' ratio (r = -0.46). A multivariate model including age, lateral E', indexed LA volume, and mitral A wave explained 46% of the variance in peak Vo2 (P = .01). Lateral E' and indexed LA volume are negatively correlated with functional capacity. Although patients with concentric morphology achieved the lowest peak Vo2, wall thickness and gradient did not predict exercise capacity.

Myocardial deformation abnormalities in paediatric hypertrophic cardiomyopathy: are all aetiologies identical?

Hypertrophic cardiomyopathy (HCM) is a disease with a heterogeneous clinical and morphological presentation. It can be secondary to mutations in genes encoding for sarcomeric and non-sarcomeric proteins. The pattern of ventricular hypertrophy can vary from isolated basal septal to concentric hypertrophy. We investigated if there are differences in regional myocardial function in different forms of HCM. We performed echocardiograms on children with (i) isolated asymmetric septal HCM, (ii) isolated concentric HCM, (iii) Friedreich's ataxia associated with concentric HCM, and (iv) healthy controls. Wall thickness, left ventricular dimensions, ejection fraction, and mitral inflow were measured. Peak early diastolic myocardial velocities, peak systolic myocardial velocities, peak systolic strain rate (SR), peak systolic strain (epsilon), post-systolic shortening and time to maximal epsilon were measured in the basal and mid-septum and basal lateral wall to evaluate longitudinal myocardial function. Similar data were acquired and analysed in the anterior septum and infero-lateral wall to evaluate the radial myocardial function. All three groups with HCM had had increased wall thickness, reduced left ventricular dimensions, and evidence of impaired diastolic filling compared to controls. All forms of HCM had reduced early diastolic and systolic myocardial velocities and peak systolic SR and peak systolic epsilon compared with controls in all myocardial segments investigated. Children with asymmetric septal HCM had reduced systolic deformation, increased post-systolic shortening, and prolonged time to maximal epsilon in the basal septum compared with the other two groups with HCM. There were no differences in any echocardiographic variable between patients with isolated concentric HCM and Friedreich's ataxia and resulting HCM. Myocardial deformation is abnormal in all forms of paediatric HCM. Myocardial deformation is more reduced and associated with post-systolic shortening in the more hypertrophied basal septum in patients with asymmetric septal HCM. In contrast, this reduction is uniformly distributed in all myocardial segments in patients with concentric HCM irrespective of whether HCM results from isolated or secondary HCM. Our findings suggest the pattern of hypertrophy influences myocardial deformation more than the underlying cause of HCM.

High-Dose Beta-Blocker Hypertrophic Cardiomyopathy Therapy in a Patient with Friedreich Ataxia

Heart involvement in patients with Friedreich ataxia (FRDA) is a condition marked by inevitable progressive deterioration, with premature death. There is currently no specific treatment for FRDA. Recently, a therapy with idebenone, a short-chain coenzyme Q10 analogue, was reported to reduce left ventricular mass by 20% in about half of FRDA patients, but a double-blind placebo-controlled study did not fully confirm these results. In this case report, we present a 5-year follow-up of symmetrical concentric hypertrophic cardiomyopathy (HCM) in an FRDA patient treated with high-dose propranolol. The therapy resulted in a reduction in the thickness of the septal and posterior left ventricular walls and complete normalization of diffuse electrocardiographic repolarization abnormalities. To the best of our knowledge, this is the first such case to be reported in the literature, demonstrating the positive effects of high-dose beta-blocker treatment on heart involvement in patients with FRDA.

A neonate with macrosomia, cardiomyopathy and hepatomegaly born to an HIV-infected mother

Perinatal treatment with highly active antiretroviral drugs (HAART) is effective to prevent mother-to-childtransmission of HIV-1; however, adverse effects on the fetus may occur. We present a neonate with macrosomia, hypertrophic cardiomyopathy, and hepatomegaly, whose mother used HAART during pregnancy. The mother has been HIV infected since 1992. During her first pregnancy (1997) she used zidovudine and lamivudine; a healthy HIV-negative daughter was born. Good viral suppression and immune reconstitution was achieved with stavudine, didanosine and nelfinavir (Fig. 1). Severe lipodystrophy developed and from 1999 the viral load became detectable. In 2000 she was pregnant again; HAART was continued with a temporary switch in the 7th month, unsuccessful because of vomiting. Resistance testing showed mutations in the reverse transcriptase and protease gene leading to resistance to zidovudine and nelfinavir, respectively. At 40 weeks of gestation, decreased fetal movements and a non-reactive CTG necessitated an emergency caesarean section. An asphyctic girl was born. Macrosomia (birth weight 5290 g, >P97; length 57 cm, >P97; head circumference 37 cm, >P97) was present without dysmorphic signs. Physical examination revealed hepatomegaly and red skin lesions on the arms, back and armpits. A chest X-ray film demonstrated cardiomegaly, but no lung abnormalities. Echocardiography showed concentric hypertrophic cardiomyopathy. In the mother, normal values of glucose, HbA1c, insulin, and C-peptide excluded diabetes mellitus. Liver and kidney functions were normal and serology for syphilis, hepatitis A, B and C was negative. In the neonate, abnormal haematology, liver tests, clotting, and extreme hypoglycaemia were present (Table 1). Metabolic disorders were meticulously investigated in blood, urine, and CSF (Table 1). The plasma amino acid profile showed increased glutamine (1103 lmol/l) and decreased arginine levels (13 lmol/l) with a normal carnitine concentration (18.7 lmol/l) and high urinary lactate excretion. These biochemical abnormalities were ascribed to the coexistent asphyxia. Muscle biopsy did not show any biochemical or enzymatic abnormality compatible with a mitochondrial respiratory chain defect. Chromosomal karyotype was 46XX. All microbiological cultures of blood and secretions were negative. Serology and PCR tests excluded recent infection with Toxoplasma, enteroviruses, adenovirus, rubella, CMV and other herpes viruses. HIV-RNA was undetectable at day 5. Severe complications, including hypoglycaemia, intra-cerebral haemorrhages and seizures developed. The clinical condition worsened and the neonate died 7 days postnatally. At autopsy, the brain showed postnatally acquired ischaemic lesions and bilateral intraventricular haemorrhage. No microorganisms were found. The heart was enlarged with long-standing subendocardial ischaemia (weeks). No evidence was found for mitochondrial damage (intact cytochrome-c-oxidase components on histochemistry and electron microscopy). Hepatomegaly with extreme cholestasis compatible with long-standing drug-induced toxicity was found. There was subcutaneous fat necrosis in both armpits. A term infant with macrosomia, skin lesions, hepatomegaly and concentric hypertrophic cardiomegaly is described. These clinical findings could not be explained by asphyxia, chromosomal abnormality or any known M. H. Godfried Department of Internal Medicine, AMC, Amsterdam, The Netherlands

Coexistent Atrioventricular and Nodoventricular Pathways in a Patient with Hypertrophic Cardiomyopathy

A 17-year-old girl with concentric hypertrophic cardiomyopathy presented with a wide complex tachycardia and underwent electrophysiological study. She was found to have an antidromic tachycardia utilizing a decremental atrioventricular fiber as the anterograde limb with retrograde conduction occurring through the septum. Ablation of a right free-wall pathway rendered tachycardia noninducible, yet ventricular preexcitation remained. After ablation there was evidence of a second nodoventricular connection. We believe this to be the first report of coexistent "Mahaim" fibers; one a decremental atrioventricular connection and the second nodoventricular.

Endomyocardial biopsies for early detection of mitochondrial disorders in hypertrophic cardiomyopathies

Considering the high proportion of unexplained hypertrophic cardiomyopathies on the one hand and the occurrence of cardiomyopathies in several mitochondrial disorders on the other, we hypothesized that isolated hypertrophic cardiomyopathies in infancy could occasionally be the result of defects of oxidative phosphorylation. By means of a scaled-down technique, we were able to investigate oxidative phosphorylation on minute amounts of endomyocardial tissue (1 mg) in three patients with concentric hypertrophic cardiomyopathy (shortening fraction in diameter, 18% to 27%; normal mean ± 1 SD, 33 ± 3%) and in control subjects. Although the absolute respiratory chain enzyme activities in the endomyocardial biopsy specimens of the patients were within the low normal range, the determination of the activity ratios allowed us to ascribe hypertrophic cardiomyopathies to respiratory chain enzyme abnormalities in all three cases (complex I, two cases; multiple enzyme deficiency, one case). The respiratory chain enzyme activity ratios, which are normally constant irrespective of the tissue tested, were markedly abnormal in all three patients (cytochrome c oxidase/reduced nicotinamide-adenine dinucleotide cytochrome c reductase, 4.6 to 10.4; normal mean ± 1 SD, 2.9 ± 0.5). We conclude that mitochondrial disorders should be regarded as potential causes of hypertrophic cardiomyopathy in early infancy. Because cardiac catheterization is routinely performed for hemodynamic investigation of cardiomyopathies, we suggest that endomyocardial biopsies be considered as a tool for early detection of mitochondrial cardiomyopathies, especially in hypertrophic forms of the disease. (J P EDIATR 1994; 124:224-8)

Left ventricular emptying dynamics in patients with asymmetric septal hypertrophy and concentric hypertrophic cardiomyopathy

We studied the dynamics of left ventricular (LV) emptying in 8 patients with asymmetric septal hypertrophy (ASH), 6 patients with concentric hypertrophic cardiomyopathy (CHC), and 6 normal controls. Four patients with ASH had resting systolic gradients greater than 20 mmHg, all had significant post premature ventricular contraction (PVC) systolic pressure gradients. LV volume (V) was obtained by frame-by-frame analysis of cineangiograms. End-diastolic volume was similar for all groups; end-systolic volume was significantly less in ASH and CHC patients than in normals. Maximum dV/dt was similar in ASH and CHC, and significantly greater than normals. Total systolic contraction time (SCT), i.e., time from peak volume to last cine frame at minimum volume, was similar for all groups, but the time required to eject 90% of stroke volume (90%T), as a fraction of SCT, was shorter for ASH (0.52 +/- 0.07) and CHC patients (0.51 +/- 0.05) than normals (0.67 +/- 0.07) (p less than 0.05 vs myopathy groups). In the sinus beat following a PVC, however, this ratio decreased significantly in normals and CHC patients, but did not change in ASH patients. We conclude that ASH and CHC have similar exaggerated systolic LV ejection dynamics in the basal state; the failure of ASH patients with post-PVC systolic outflow gradient to reduce 90% T/SCT post PVC may reflect obstruction to LV emptying.

The echocardiographic assessment of cardiomyopathy: diagnosis, classification and problems.

Two-hundred-and-sixty-three patients with cardiomyopathy were studied by M-mode echocardiography. Measurements of left ventricular cavity size, wall thickness and myocardial contraction were used to classify cardiomyopathy into "congestive" (212 patients) and "hypertrophic" (50 patients) groups; the "hypertrophic" group was further divided into asymmetric septal hypertrophy (37) and symmetric (concentric) mural thickening (13). Using clinical and electrocardiographic information as well as echocardiographic data, the latter group could then be further classified into "concentric infiltrative cardiomyopathy" (9) and "concentric hypertrophic cardiomyopathy" (4). The former either presented with signs of restriction or were known to have systemic amyloidosis; the electrocardiograph showed low voltage and myocardial contraction was impaired in advanced cases. The latter had evidence of severe left ventricular hypertrophy and resembled asymmetric septal hypertrophy clinically. Problems encountered with the echocardiographic diagnosis of congestive cardiography were mainly concerned with proper clinical interpretation of the echocardiographic data whilst technical difficulties in recording the echocardiogram and in interpretation of tracings were a common problem in hypertrophic cardiomyopathy.

Hypertrophic cardiomyopathy in Friedreich's ataxia: symmetric or asymmetric?

We evaluated 15 patients with Friedreich's ataxia (FA) to define the incidence of myocardial involvement and the type of cardiomyopathy observed. All patients with FA had either ECG, vectocardiographic or echocardiographic abnormalities, suggesting some degree of myocardial involvement. In contrast to reports indicating that asymmetric septal hypertrophy (ASH), often obstructive, is associated with FA, symmetric, concentric hypertrophic cardiomyopathy (SCH) was the predominant abnormality (sixty-seven percent of patients). Echocardiograms should be performed periodically in all FA patients since this technique allows the detection of cardiac hypertrophy.