Hypertrophic cardiomyopathy with left ventricular dilatation

There is increasing interest in the notion that some patients with hypertrophic cardiomyopathy (HCM) progress to morphological and functional manifestations similar to those of dilated cardiomyopathy (DCM). From 165 consecutive patients with HCM, 20 patients with left ventricular dilatation (left ventricular end-diastolic diameter greater than or equal to 50 mm) were selected and designated as dilated HCM. The diagnosis of HCM was established in these patients either by detection of the classical form of HCM in family members, with 2-dimensional echocardiographic evidence of asymmetric septal hypertrophy (ASH; septal thickness greater than or equal to 15 mm and a ratio of septal to posterior wall thickness greater than or equal to 1.3); or by demonstrating myocardial fiber disarray in autopsy or biopsy samples. The clinical manifestations of these patients with dilated HCM were then compared with those of other forms of HCM without left ventricular dilatation; 1) 40 patients with hypertrophic obstructive cardiomyopathy (HOCM) who had resting intraventricular pressure gradients of 20 mmHg or more, 2) 80 patients with non-obstructive HCM, each of whom had ASH of the entire ventricular septum (typical ASH), and 3) 25 non-obstructive patients whose hypertrophy was localized to the apical region of the ventricular septum (apical ASH). Patients having apical hypertrophy with a spade-like configuration on the left ventriculogram were excluded from the study. Compared with HOCM and typical ASH groups, the patients with dilated HCM had family histories of significantly more frequent HCM and less frequent hypertension. The patients with dilated HCM also had significantly less fractional shortening (FS), decreased interventricular septal thickness, greater left ventricular end-diastolic pressure (LVEDP), and left ventricular dilatation. During the follow-up period (average: 3.5 years), seven patients (35%) with dilated HCM died; five from congestive heart failure (CHF), one suddenly, and one three days following mitral valve replacement. The other five patients had CHF at the time of their follow-up examination. The patients with apical ASH had clinical features similar to those of dilated HCM; a higher familial frequency, less marked septal hypertrophy, and higher LVEDP. They tended to develop left ventricular dilatation, associated with reduced fractional shortening, although left ventricular diameter at end-diastole did not exceed 50 mm. These findings suggested that dilated HCM is not a rare condition. It is observed in 12% of consecutive patients with HCM.(ABSTRACT TRUNCATED AT 400 WORDS)     

Predictors of survival and sudden death in patients with stable severe congestive heart failure due to ischemic and nonischemic causes: a prospective long term study of 200 patients

This prospective study of 200 stable outpatients with New York Hospital Association (NYHA) class III congestive heart failure on maximal medical therapy was done to determine which factors affect survival, to record the incidence of sudden death, and to identify prognostic features which characterize patients at high risk of sudden death. Congestive heart failure was due to coronary artery disease in 151 patients (76%). After an average follow-up of 40 months, 96 patients (48%) had died: 30 (15%) suddenly, 41 (22%) of low output, and 25 (13%) of other causes. Of the 30 patients dying suddenly 12 had autopsies, and acute myocardial infarction was found in nine. Of the 41 patients dying of low output 15 had autopsies, and recent myocardial infarction was found in five. Nine of the 25 patients dying of other causes died of acute myocardial infarction. Multivariate stepwise analysis revealed that severity of ventricular arrhythmias (modified Lown classification), exercise tolerance and left ventricular ejection fraction were the most important determinants of survival. In patients with coronary artery disease, complex ventricular arrhythmias detected by ambulatory Holter monitoring were frequent in all groups and were not clinically useful in predicting which of these patients were at a higher risk of dying suddenly. In contrast, patients without coronary artery disease who died suddenly had a higher incidence of nonsustained ventricular tachycardia and a tendency towards more frequent ventricular arrhythmias in general. The authors conclude that in ambulatory patients with stable NYHA class III heart failure, the severity of ventricular arrhythmias is a predictor of survival.(ABSTRACT TRUNCATED AT 250 WORDS)     

Anterior QRS loop in hypertrophic cardiomyopathy.

Frank vectorcardiograms (VCGs) were reviewed in 45 patients with hypertrophic cardiomyopathy (HCM), 26 with obstruction and 19 without obstruction. Twelve of the 19 patients without obstruction and five of the 26 patients with obstruction were found to have predominantly anterior QRS loops. Fourteen patients had a large left anterior QRS loop with increased anterior and leftward force; the posterior and terminal rightward force were within the normal range, and the T loops were displaced posteriorly and to the right opposite to the QRS loop. Asymmetric septal and apical hypertrophy were noted echocardiographically and/or angiographically. Increased electrical force from the asymmetric hypertrophy of the septal and apical area is proposed to explain this large left anterior loop. Three patients had a QRS loop located anteriorly and to the right with electrocardiograms (ECGs) resembling those of posterolateral myocardial infarction or right ventricular hypertrophy. These finding suggest that (1) hypertrophic cardiomyopathy may be another cause of an anterior QRS loop; (2) the recognition of the large left anterior loop in the VCG in patients with a left ventricular hypertrophy pattern in the ECG is helpful in the diagnosis of HCM, especially the nonobstructive form; and (3) hypertrophic cardiomyopathy should be considered in the differential diagnosis of myocardial infarction or right ventricular hypertrophy.     

Acute myocardial infarction with diminutive right coronary artery and obstructive hypertrophic cardiomyopathy without significant coronary stenoses

Focal tissue abnormalities consistent with regional ischemia have been reported in patients with hypertrophic cardiomyopathy (HCM). Coronary microvascular dysfunction has been also reported to be present in patients with HCM despite normal epicardial coronary arteries. Moreover, it has been demonstrated that in the case of HCM and idiopathic left ventricular hypertrophy, hypoplastic coronary arteries as diminutive vessels are present and that obstructive hypertrophic cardiomyopathy is associated with enhanced thrombin generation and platelet activation. Previously, it has been described an acute myocardial infarction in a young athlete with non-obstructive hypertrophic cardiomyopathy and normal coronary arteries. We present a case of an acute myocardial infarction with diminutive right coronary artery and obstructive hypertrophic cardiomyopathy without significant coronary stenoses. To our knowledge, this is the first report of an acute myocardial infarction with diminutive right coronary artery and obstructive hypertrophic cardiomyopathy without significant coronary stenoses.     

Sudden death of a case of hypertrophic obstructive cardiomyopathy 19 months after successful percutaneous transluminal septal myocardial ablation.

A 56-year-old male with hypertrophic obstructive cardiomyopathy complicated with medically refractory paroxysmal atrial fibrillation and congestive heart failure was treated with percutaneous transluminal septal myocardial ablation. The resting left ventricular outflow tract gradient decreased from 70 mmHg to 0 mmHg after the procedure, and clinical symptoms improved dramatically. However, the patient died suddenly 19 months later and autopsy revealed nontransmural myocardial fibrosis with an irregular border in the interventricular septum.     

Prognostic significance of programmed ventricular stimulation in survivors of acute myocardial infarction

The prognostic significance of the response to programmed ventricular stimulation was studied in 75 stable survivors of acute myocardial infarction. Programmed ventricular stimulation induced sustained ventricular arrhythmias in 33 (44%) patients and did not induce these arrhythmias in 42 (56%) patients. During a mean follow up of 18 months, four patients died suddenly and three developed spontaneous sustained ventricular tachycardia. The occurrence of arrhythmic events was not significantly different in patients with inducible sustained arrhythmias and those without, but such events were predicted by the presence of mild congestive heart failure. Although the inducibility of sustained ventricular tachycardia (but not ventricular fibrillation) seemed to identify a high risk subset with an arrhythmic event rate of 21% compared with 5.5% in others, it had a low sensitivity (57%) and a low positive predictive accuracy (21%) for arrhythmic events. Programmed ventricular stimulation is not helpful in identifying a subset of patients at high risk after an uncomplicated acute myocardial infarction.     

Prognostic significance of programmed ventricular stimulation in survivors of acute myocardial infarction.

The prognostic significance of the response to programmed ventricular stimulation was studied in 75 stable survivors of acute myocardial infarction. Programmed ventricular stimulation induced sustained ventricular arrhythmias in 33 (44%) patients and did not induce these arrhythmias in 42 (56%) patients. During a mean follow up of 18 months, four patients died suddenly and three developed spontaneous sustained ventricular tachycardia. The occurrence of arrhythmic events was not significantly different in patients with inducible sustained arrhythmias and those without, but such events were predicted by the presence of mild congestive heart failure. Although the inducibility of sustained ventricular tachycardia (but not ventricular fibrillation) seemed to identify a high risk subset with an arrhythmic event rate of 21% compared with 5.5% in others, it had a low sensitivity (57%) and a low positive predictive accuracy (21%) for arrhythmic events. Programmed ventricular stimulation is not helpful in identifying a subset of patients at high risk after an uncomplicated acute myocardial infarction.     

Whole-genome DNA sequencing: The key to detecting a sarcomeric mutation in a ‘false genotype-negative’ family with hypertrophic cardiomyopathy

The authors report the clinical and genetic investigation of a family with hypertrophic cardiomyopathy (HCM). The individuals described are three affected first-degree relatives (father, daughter and son), one affected niece and unaffected nephew and niece. Those affected all share a very similar phenotype consisting of asymmetric HCM, with hypertrophy particularly affecting the septum and the anterior wall, and similar electrocardiographic features, including a short PR interval. Case 1 (proband) presented with obstructive HCM and had undergone myectomy and mitral valve replacement. Case 2 (oldest offspring of Case 1) had non-obstructive HCM with exertional angina and NYHA II heart failure (HF) symptoms; she developed non-sustained ventricular tachycardia during follow-up and received a single-chamber ICD for primary prevention of sudden cardiac death. Case 3 (son of case 1) presented with asymptomatic non-obstructive HCM and developed NYHA II HF symptoms during follow-up. Case 4 had non-obstructive HCM, mainly with NYHA II HF symptoms. Testing of the proband for sarcomeric mutations and phenocopies was initially negative. After eight years of clinical follow-up, the suspicion of an undiscovered pathogenic gene mutation shared among the members of this family led us to enroll the proband in a whole-genome sequencing research project, which revealed a heterozygous pathogenic intronic MYBPC3 variant (c.1227-13G>A [rs397515893]), cosegregating with the phenotype.     

Prognostic value of echocardiographic evaluation of septal function in acute anteroseptal myocardial infarction.

To determine the clinical usefulness of echocardiography in patients with anteroseptal myocardial infarction, echocardiograms were performed within 24 hours of admission on 40 patients with acute transmural anteroseptal myocardial infarction. Twenty-one patients had normal septal motion and septal systolic thickening, and 19 patients had abnormalities of one or both of these measurements. Of the 21 patients who had normal septal motion and thickening, only five developed congestive heart failure, none developed bundle branch block, and none died. Of the 19 patients with abnormal septal motion and/or thickening, 17 developed congestive heart failure (p less than .001), seven developed bundle branch block (p less than .001), and six died (p less than .001). Therefore, (1) electrocardiographic evidence of septal infarction does not correlate with abnormalities of the portion of septum seen on echocardiogram, and (2) patients with anteroseptal myocardial infarction and abnormalities of the septum on echocardiogram have more complications and a higher in-hospital mortality rate. These patients may have more extensive myocardial infarction predisposing to pump failure and possibly involving the conduction system.     

Mid‐Ventricular Obstructive Hypertrophic Cardiomyopathy and Apical Aneurysm Mimicking Acute ST‐Elevation Myocardial Infarction

Nonischemic ST‐segment elevation may be confused as acute ST‐elevation myocardial infarction (STEMI), especially in patients with atypical presenting symptoms. Among the possible differential diagnosis, hypertrophic cardiomyopathy (HCM) should be considered. Mid‐ventricular obstructive hypertrophic cardiomyopathy (MVOHCM) is a rare type of cardiomyopathy, accounting for approximately 5% of all HCM cases. ST‐segment elevation on electrocardiogram (ECG) in patients with MVOHCM is a rare clinical presentation. We present a case of MVOHCM and apical aneurysm mimicking acute STEMI     

Candida myocarditis without valvulitis

Thirty-one patients with systemic candidiasis at postmortem examination were found to have Candida involvement of the myocardium without valvulitis. Retrospective examination of their clinical course demonstrated that a new conduction disturbance was seen in 10, supraventricular arrhythmias in 5, QRS changes mimicking myocardial infarction in 3, and pronounced T wave changes in 13. Hypotension or shock was seen in 13 patients and could not be explained by coexistent bacteremia or blood loss in 8. One patient died suddenly. Of 19 patients with systemic candidiasis without myocardial invasion, 4 had minor T wave changes and one had a supraventricular arrhythmia. Candida invasion of the heart significantly complicates the clinical course in systemic candidiasis and should be suspected when a young person without preexistent heart disease has cultures positive for a Candida organism, a significant arrhythmia, conduction disturbance or other dramatic QRS change. The effect of therapy on Candida invasion of the heart is unknown.     

Hypertrophic cardiomyopathy and transmural myocardial infarction without significant atherosclerosis of the extramural coronary arteries

Clinical and morphologic features of transmural myocardial infarction (associated with insignificant or absent atherosclerosis of the extramural coronary arteries) are described in seven patients with hypertrophic cardiomyopathy. Marked chronic congestive heart failure associated with supraventricular arrhythmias occurred in six of the seven patients, each of whom had no or mild left ventricular outflow tract obstruction under basal conditions. No patient had typical angina pectoris, and only one patient had clinically evident acute myocardial infarction. Infarction may have caused cardiac arrest in one other patient, but was “silent” in the remaining five patients.

At necropsy, six of the seven patients had extensive myocardial scarring involving the ventricular septum, left ventricular free wall and one or both left ventricular papillary muscles; in four patients portions of the right ventricular wall were also scarred. Six patients had dilated ventricular cavities, including two who were known to have nondilated ventricular cavities earlier in their clinical course.

It is concluded that transmural myocardial infarction in the absence of significant coronary atherosclerosis is a not uncommon finding (prevalence rate 15 percent) in a population of patients who had died from hypertrophic cardiomyopathy. Although transmural infarction is possibly a secondary event, it more likely contributes causally to the clinical deterioration of some patients with hypertrophic cardiomyopathy, leading to ventricular dilatation and progressive fatal cardiac failure.     

Incidence and significance of pericardial effusion in acute myocardial infarction as determined by two-dimensional echocardiography

To determine the incidence and clinical significance of pericardial effusion after acute myocardial infarction, two-dimensional echocardiography was serially performed in 66 consecutive patients. Pericardial effusion was observed in 17 (26%); the effusion was small in 13 patients, moderate in 3 and large with signs of cardiac tamponade in 1. In this patient, two-dimensional echocardiography strongly suggested myocardial rupture. The observation of pericardial effusion was not associated with age, sex, previous myocardial infarction, atrial fibrillation or treatment with heparin. It was more often a complication of anterior than of inferior acute infarction. Patients with pericardial effusion had higher peak levels of creatine kinase and lactic dehydrogenase and a higher wall motion score index. More patients with pericardial effusion had congestive heart failure or ventricular arrhythmias, developed a ventricular aneurysm or died within 1 year after their infarction. In conclusion, pericardial effusion is frequently visualized by two-dimensional echocardiography after acute myocardial infarction and its presence is associated with an increased occurrence of complications and cardiac death.     

Simple criteria for differentiation of Fabry disease from amyloid heart disease and other causes of left ventricular hypertrophy

AIMS: Fabry disease may be difficult to differentiate from other causes of left ventricular hypertrophy such as other myocardial storage diseases (including amyloidosis), hypertrophic cardiomyopathy (HCM), or hypertensive heart disease (HHD). We sought to determine simple criteria to best differentiate the above mentioned cardiac diseases. METHODS AND RESULTS: All patients in a six-year time period with left ventricular hypertrophy due to Fabry disease (13 patients), biopsy proven cardiac amyloidosis (16 patients), non-obstructive HCM (17 patients), and 22 randomly selected patients with advanced HHD were compared. Retrospective analysis of clinical characteristics, findings of electrocardiogram (ECG) and echocardiography by blind review was performed. RESULTS: No single clinical characteristic or findings of ECG or echocardiography could reliably differentiate between the various diseases. Increased echogenicity/granular sparkling, valvular abnormalities, abnormal renal function, and diastolic function were not helpful discriminators. In a univariate analysis, four criteria (acroparesthesia, anhydrosis, absence of hypertension and presence of Sokolow criteria for left ventricular hypertrophy in the ECG) were significant for Fabry disease. By logistic regression analysis, the following most suitable discriminative parameters were identified: hypertension in HHD (specificity 82%), orthostasis and/or pericardial effusion for amyloidosis (specificity 93%), papillary muscle anomaly in non-obstructive HCM (specificity 92%), and Fabry disease if neither hypertension orthostatis, pericardial effusion nor a papillary muscle anomaly was present (specificity 87%). CONCLUSION: A combination of symptoms, echocardiographic findings and ECG in unexplained left ventricular hypertrophy may help to differentiate amyloidosis, non-obstructive HCM and hypertensive heart disease from Fabry disease. The results of this preliminary study will have to be confirmed in a prospective study.     

Myocardial bridges and ischemic heart disease

The clinical, electrocardiographs and angiocardiographic dataare reported of 37 patients with myocardial bridges, in theabsence of coronary atherosclerosis. An intramyocardial courseof the left anterior descending coronary artery was presentin all cases: myocardial bridges on the posterior descendingand first diagonal branch were associated in one patient. The clinical presentation was that of ischemic heart diseasein all cases, with stable angina in nine, unstable angina in19, the intermediate syndrome in five, and a history or evidenceof previous myocardial infarction in four. In 20 cases, left ventricular wall motion appeared to be abnormalon the antero-lateral or apical profile. One patient died suddenly the day following hospital discharge;of 27 patients followed over a 3–36 month interval, onlyfour (15%) were free of symptoms on medical treatment. The frequent association of myocardial bridges with a clinicalpicture of ischemic heart disease which is sometimes severe,is emphasized together with the disappointing results of medicaltreatment.     

Complete heart block in acute myocardial infarction: drug therapy

A prospective investigation of medication in the treatment of complete heart block in acute myocardial infarction (AMI) was undertaken in the Coronary Care Unit of the Municipal Hospital, Copenhagen, during the period Nov. 24, 1967, to August 31, 1970.The material comprises 32 consecutive cases, corresponding to 8 per cent of all patients with verified acute myocardial infarction treated in the Unit during the same period. Treatment consisted in atropine, isoprenaline, and occasionally corticosteroids.Twelve patients died. In five the medication was without sufficient effect, and transvenous intracardial demand pacemakers were therefore employed. Three of these patients died. In the remaining nine fatal cases, sinus rhythm had been achieved in seven before death. The causes of death in these cases were: cardiac collapse, pulmonary embolism, or myocardial rupture. Two patients died after ventricular fibrillation.The mortality rate among the patients admitted in a state of cardiogenic shock was high, but with these exceptions it was not possible to predict the prognosis from the condition on admission.Eight out of 15 patients with infarctions of the anterior wall and 3 out of 16 patients with infarctions of the posterior wall died. The highest mortality rate was encountered in the combination of anterior wall infarction and a widened QRS complex.It cannot be ruled out that the chronotropic treatment may have resulted in an increased tendency to ectopic ventricular dysrhythmias, but this did not influence the mortality rate.The results obtained correspond to those observed in comparable materials using prophylactic pacemaker technique.It is concluded that by medication it is as a rule possible to achieve results similar to those of pacemaker therapy in complete heart block in acute myocardial infarction. However, pacemaker implantation may be life-saving in some cases and is indicated under all circumstances in Stokes-Adams attacks, persistent bradycardia with reduced cardiac output, and in repeated ventricular tachycardia during medication. In addition, prophylactic pacemaker implantation should be considered in anterior wall infarction with widened QRS complex, as this type of patient may frequently and suddenly develop complete heart block directly from sinus rhythm.     

Right and left ventricular volume parameters in cardiomyopathy (author's transl)

In 41 patients with different types of cardiomyopathy (19 congestive = COCM, 6 hypertrophic obstructive = HOCM, and 16 hypertrophic non-obstructive = HCM) ventricular enddiastolic volume (EDV), endsystolic volume (ESV), and ejection fraction (EF) were determined angiographically and values of both ventricles were compared. In the mean, volume parameters increased significantly and EF of both ventricles decreased as compared to control values of patients with coronary heart disease without myocardial infarction. In COCM volume parameters reached pathologic values as in the total patient group whereas in HOCM and in HCM values did not differ significantly from control. Left ventricular function was reduced more often and, in the mean, more severely than right ventricular function. This became evident e.g. from the incidence of enddiastolic volume increase and the course of the regression line. A similar finding can be derived from the reversal of the normal relation of volume parameters of both ventricles. Only in rare cases right ventricular function was more severely impaired than left ventricular function. These findings indicate a simultaneous impairment of both ventricles in cardiomyopathy. The more pronounced left ventricular function disturbance may be attributed to the higher left ventricular work load. Thus, left ventricular biopsies might be of greater diagnostic significance than right ventricular biopsies.     

Vectorcardiographic study of initial QRS forces in left bundle branch block associated with myocardial infarction, primary myocardial disease and valvular heart disease

Distribution of the initial 10–30 msec QRS forces was determined on the Frank vectorcardiograms (VCGs) of 31 patients with complete left bundle branch block (LBBB). Of nine patients with coronary artery disease, three had all 10–30 msec vectors directed posteriorly in the horizontal plane (HP) and associated inferior or apical left ventricular asynergy. Frontal plane initial 30 msec QRS forces were oriented superiorly in two of these three subjects. In five patients with isolated anterior wall myocardial infarction, the HP 10 msec QRS vectors were anterior, followed by posteriorly directed 20–30 msec QRS vectors. Twelve patients with primary cardiomyopathy had initial QRS vectors distributed as follows: 10–30 msec posterior and left in two; 10–20 msec anterior and left in five; 10 msec anterior and left with the 20–30 msec posterior and left in four. In one patient the 10 msec was posterior and right, and 20–30 msec posterior and left. Of 10 patients with valvular heart disease three manifested posterior 10–30 msec QRS vectors. In four the 10 msec vector was anterior and left and 20–30 msec posterior and left. The 10–20 msec were anterior and left and 30 msec posterior and left in two subjects. There was no correlation between posteriorly directed initial QRS forces and left ventricular hypertrophy in the latter group. We conclude that: 1) posterior orientation of the initial 10 msec QRS vectors in the presence of LBBB is not specific for myocardial infarction; 2) when present in patients with obstructive coronary artery disease such abnormal posterior forces correlate with anterior and probable co-existing infero-apical infarction and, 3) factors aside from left ventricular hypertrophy can produce abnormally directed initial 10–30 msec QRS vectors in subjects with valvular heart disease.     

肥厚型心肌病患者小动脉弹性指数水平与左心室流出道梗阻的关系

目的：探讨肥厚型心肌病（HCM）患者小动脉弹性指数（C2）水平与左心室流出道梗阻的关系. 方法：纳入2010年1月至2013年7月间因肥厚型心肌病于我院心内科住院的患者69例,收集相关临床资料,比较C2水平与相关指标的关联性.再根据左心室流出道梗阻情况的不同,将HCM患者分为梗阻性HCM与非梗阻性HCM,比较两组患者的C2水平. 结果：N末端B型利钠肽原（NT-proBNP）、左室流出道最大压差（LVOTPG）与C2呈显著负相关,左室短轴缩短率（LVFS）与C2呈显著正相关,左室后壁宽度（LVPWD）与C2无显著相关性,超敏肌钙蛋白I（c-TNI）、左室射血分数（LVEF）、左室舒张末期内径（LVEDD）与C2呈非线性相关.梗阻性HCM患者的C2水平显著低于非梗阻性HCM患者. 结论：C2与LVOTPG密切相关,该指标可作为HCM患者左心室流出道梗阻病情进展的动态评价指标.     

Paralytic ileus following myocardial infarction

Clinically severe adynamic ileus has been found to be a rare complication of acute myocardial infarction. Two such patients have been presented; each died suddenly after a prolonged hospital course. Complete autopsy study was made, and each was found to have died from a very recent myocardial infarction. In Case 1, clinical diagnosis was directed to mesenteric arterial occlusion with bowel infarction, and Case 2 clinically suggested intraor retroperitoneal hemorrhage. The post-mortem studies indicated that both patients had congestive failure secondary to arteriosclerotic heart disease and that the ileus was probably a manifestation of mesenteric vascular insufficiency. The prognosis of intestinal obstruction following myocardial infarction must rest upon the cause of the ileus and need not be ominous. In these cases, conservative management of the adynamic ileus was effective; however, they emphasize that the surgical implications of ileus must always be considered and directed to the pericardium as well as the abdomen.