Patterns and significance of distribution of left ventricular hypertrophy in hypertrophic cardiomyopathy: A wide angle,two dimensional echocardiographic study of 125 patients

Four patterns of distribution of left ventricular hypertrophy were identified by a wide angle, two dimensional echocardiographic study of 125 patients with hypertrophie Cardiomyopathy. Most commonly (52 percent of patients) hypertrophy involved substantial portions of both the ventricular septum and anterolateral left ventricular free wall (type III). In other patients, hypertrophy was confined to the anterior portion of the ventricular septum (type I), involved the entire ventricular septum but not the left ventricular free wall (type II) or was identified in regions of the left ventricle other than the basal anterior ventricular septum (type IV). In patients with the latter type, conventional M mode echocardiography failed to identify the presence of hypertrophy and therefore the diagnosis of hypertrophic Cardiomyopathy could be established only with two dimensional echocardiography.Patients with the most widespread hypertrophy involving most of the ventricular septum as well as portions of the free wall (type III) differed from patients having the other patterns of hypertrophy. (1) They more commonly experienced moderate to severe functional limitation (38 of 65 [58 percent]versus 16 of 60 [27 percent]; probability [p] <0.001), and (2) they more often demonstrated obstruction to left ventricular outflow under basal conditions (36 of 65 [55 percent]versus 11 of 60 [18 percent]; p <0.001). Hence, in patients with hypertrophic Cardiomyopathy, wide angle two dimensional echocardiography is capable of detecting myocardial hypertrophy that involves a variety of patterns and is more extensive than may be appreciated with M mode echocardiography. Although left ventricular hypertrophy is “asymmetric” in most patients with hypertrophic Cardiomyopathy, it is usually not confined to the ventricular septum and often involves the anterolateral left ventricular free wall, but it rarely involves the posterior portion of the left ventricular free wall (through which the M mode beam passes).     

Relation between marked changes in left ventricular outflow tract gradient and disease progression in hypertrophic cardiomyopathy

Spontaneous and persistent changes in left ventricular (LV) outflow gradient have been observed occasionally in patients with hypertrophic cardiomyopathy (HC). However, the significance and frequency of such hemodynamic alterations have not been established. In this study, the serial preoperative hemodynamic status of 409 patients with HC was analyzed. Basal LV outflow tract obstruction either spontaneously appeared (or increased) or disappeared (or decreased) in 19 nonoperated patients (about 5%). Changes in hemodynamic state were shown by serial cardiac catheterization in 17 patients and by catheterization and M-mode echocardiography in 2 patients. In most patients (12 of 19), subaortic obstruction under basal conditions appeared or increased; 8 became more symptomatic and in 4 the condition remained stable. Reduction or loss of LV outflow gradient occurred in 7 patients; in 5 of these the condition deteriorated clinically and in 2 it did not change. Hence, in 13 of the 19 patients (70%), spontaneous changes in the magnitude of the basal LV outflow gradient were associated with symptomatic progression. The mechanism of the decrease or disappearance of subaortic obstruction in those patients who deteriorated clinically appeared to be related in 4 patients to impaired global and/or segmental LV function. Chronic atrial fibrillation probably contributed to the worsening clinical condition in 2 of these patients as well as in 2 others. In conclusion, substantial changes in the magnitude of basal subaortic obstruction may occur in a small proportion of patients with HC as part of the natural history of their disease, and such hemodynamic alterations are usually associated with clinical deterioration. It is exceedingly rare for the hemodynamic state of a patient with HC to change from totally nonobstructive to obstructive or vice versa, because such patients usually retain the capacity to generate gradients with provocative maneuvers.     

Echocardiographic analysis of ventricular septal dynamics in hypertrophic cardiomyopathy and other diseases

It has been suggested that the adynamic or hypokinetic appearance of the ventricular septum is a unique echocardiographic feature of hypertrophic cardiomyopathy (HC). To determine how characteristic of HC the adynamic septum is, 70 patients with this disease, and 31 with other cardiac diseases that produce left ventricular (LV) hypertrophy and pressure overload (aortic valvular stenosis or systemic hypertension), and 25 subjects with normal hearts were studied by echocardiography. On M-mode echocardiography, 53 of 70 patients (75%) with HC had an abnormally low value for percent systolic thickening of the septum associated with either reduced or normal septal excursion; however, 17 patients (25%) showed normal septal dynamics. Twenty of 31 patients (64%) with other cardiac diseases that produce pressure overload showed normal septal thickening and excursion, while 11 (36%) had reduced systolic thickening associated with either diminished or normal excursion. Greatly reduced values for percent systolic thickening of the septum were present both in patients with HC (13 +/- 1%) and in patients with other cardiac diseases (21 +/- 2%). However, differences in systolic septal thickening between the 2 groups were largely a manifestation of the greater absolute diastolic septal thickness in patients with HC. When values for percent systolic thickening were normalized for diastolic septal thickness, or when systolic thickening was compared in only patients with similar diastolic septal thicknesses, differences in septal thickening between patients with HC and those patients with other cardiac diseases were not significant.(ABSTRACT TRUNCATED AT 250 WORDS)     

Hypertrophic cardiomyopathy with ventricular septal hypertrophy localized to the apical region of the left ventricle (apical hypertrophic cardiomyopathy)

Clinical and morphologic features are described in a unique subgroup of seven patients with hypertrophic cardiomyopathy. Five patients either died suddenly or are alive but severely symptomatic. In each patient ventricular septal hypertrophy was demonstrated on two dimensional echocardiography or at necropsy to be virtually confined to its apical one-half. However, conventional M mode echocardiography was unreliable in identifying this site of hypertrophy because It was often inaccessible to the path of the M mode beam. Apical distribution of septal hypertrophy does not constitute a separate disease entity, but rather appears to be part of the morphologic spectrum of hypertrophic cardiomyopathy, as judged from two findings: (1) genetic transmission of hypertrophic cardiomyopathy in relatives of each study patient; and (2) marked disorganization of cardiac muscle cells in the left ventricular wall of the two patients studied at necropsy.

Apical distribution of septal hypertrophy in these patients was associated with relatively mild T wave inversion in the electrocardiogram and characteristic angiographic appearance of the left ventricle with mid ventricular constriction and a small, often poorly contractile apical segment. These electrocardiographic and angiographic features differ from those previously described in Japanese patients with “apical hypertrophic cardiomyopathy” in whom “giant” T wave inversion and a “spade-like” appearance of the left ventricle were characteristic.     

“Malignant” hypertrophic cardiomyopathy: Identification of a subgroup of families with unusually frequent premature death

Eight families were identified in which premature cardiac death due to hypertrophic cardiomyopathy occurred with unusual frequency. A total of 69 first degree relatives in the eight families were studied; 41 relatives had evidence of hypertrophic cardiomyopathy and 31 (75 percent) died of their heart disease. Eighteen of these 31 patients were less than 25 years of age at the time of death. Death was sudden and unexpected in 23 of the 31 patients; in 15 of these 23 patients sudden death was the initial manifestation of cardiac disease. The remaining eight patients (seven were from two families) died after a chronic cardiac illness characterized by congestive heart failure, atrial fibrillation or thromboembolic events.

Hence, premature cardiac death occurs frequently in certain families with hypertrophic cardiomyopathy. Such deaths are usually sudden, often occur in previously asymptomatic subjects and are common in children and young adults. These findings suggest that some families may manifest an unusually virulent expression of hypertrophic cardiomyopathy. Although this study cannot establish the precise prevalence with which “malignant” hypertrophic cardiomyopathy occurs, such families appear to be uncommon.     

Sudden death in patients with hypertrophic cardiomyopathy: Characterization of 26 patients without functional limitation

Sudden death is a recognized complication in symptomatic patients with hypertrophic cardiomyopathy. However, its occurrence in patients with no or transient previous cardiac symptoms presents a particularly challenging diagnostic and therapeutic dilemma. Therefore, 26 patients with hypertrophic cardiomyopathy whose death was the first definitive manifestation of cardiac disease were evaluated. Their ages ranged from 8 to 49 years (mean 18) and 23 were less than 25 years of age; 19 were male and 7 female. Of the 26 patients, 13 died during or immediately after moderate or severe physical exertion. Of 12 patients with previous cardiac catheterization, 6 had no or a small left ventricular outflow tract gradient under basal conditions and 6 had an outflow gradient of 50 mm Hg or greater. Left ventricular end-diastolic pressure was elevated in nine patients, and the ventricular septum was moderately to severely thickened (17 mm or more) in all patients. The electrocardiogram was abnormal in all 19 patients studied before death. Thus, sudden death may be the first definitive manifestation of cardiac disease in some patients with hypertrophic cardiomyopathy. Although the effects of patient selection in this study group cannot be excluded, sudden death was common in children and young adults and was often related to physical exertion; each patient showed a distinctly abnormal electrocardiogram and moderate to severe ventricular septal thickening.     

The heart in massive (more than 300 pounds or 136 kilograms) obesity: analysis of 12 patients studied at necropsy

Observations are described in 12 massively obese patients (5 women, 7 men), aged 25 to 59 years (mean 37), who weighed 312 to more than 500 pounds (mean 381). Seven patients had had systemic hypertension, 4 hypersomnia or sleep apnea, 2 diabetes mellitus, and 1 patient symptomatic coronary artery disease. Five patients died suddenly from undetermined causes, 2 from right-sided congestive heart failure, 1 patient from acute myocardial infarction; 1 from aortic dissection; 1 from intracerebral hemorrhage; 1 from a drug overdose, and 1 soon after an ileal bypass. The heart weight was increased in all 12 patients. The heart weight to body weight ratio expressed as a percent ranged from 0.22 to 0.61 (mean 0.37) (normal for men 0.42 to 0.46 [mean 0.43], normal for women 0.38 to 0.46 [mean 0.40]). The left ventricular cavity was dilated in 11 patients and the right ventricular cavity in all 12. Only 2 patients (aged 42 and 59 years) had 1 or more major epicardial coronary arteries narrowed greater than 75% in cross-sectional area by atherosclerotic plaque, 1 of whom had no symptoms of myocardial ischemia. Of 664 five-millimeter segments from the 4 major epicardial coronary arteries from 11 patients (mean 60 per patient), 431 (65%) were narrowed 0 to 25% in XSA, 143 (21%) were narrowed 26 to 50%, 73 (11%) were narrowed 51 to 75%, and 17 (3%) were narrowed 76 to 100%.(ABSTRACT TRUNCATED AT 250 WORDS)     

Patterns of systolic anterior motion of the mitral valve in hypertrophic cardiomyopathy: assessment by two-dimensional echocardiography

A variety of patterns of systolic anterior motion (SAM) of the mitral valve were identified by realtime, 2-dimensional echocardiography in 62 patients with hypertrophic cardiomyopathy. In 36 patients (58%), both the anterior and posterior mitral leaflets appeared to participate importantly in SAM, although the anterior leaflet actually contacted or most closely approached the ventricular septum during systole because of its anterior anatomic position. In 19 patients (31%), SAM was produced selectively by the posterior mitral leaflet. In only 6 patients (10%) was the anterior leaflet alone responsible for SAM. In just 1 patient did the chordae tendineae appear to be primarily responsible for the SAM. In 51 patients (82%), only the distal portion of the anterior or posterior mitral leaflet (and possibly the attached proximal chordae tendineae) approached or contacted the septum in systole; in 10 patients both the body and tip regions of the anterior leaflet produced mitral-septal apposition. Hence, in obstructive hypertrophic cardiomyopathy, (1) the morphologic structures responsible for moderate to severe SAM are not identical in all patients, and a variety of patterns of SAM occur; (2) the posterior mitral leaflet plays an important role in SAM in almost 90% of patients, either by producing SAM alone (31%) or by moving anteriorly in concert with the anterior leaflet (58%); (3) SAM produced selectively by the anterior mitral leaflet is relatively uncommon; and (4) SAM is usually produced primarily by the distal portions of the mitral leaflets (with or without the attached chordae tendineae).     

Exercise performance after septal myotomy and myectomy in patients with obstructive hypertrophic cardiomyopathy

The effect of left ventriculomyotomy and myectomy on exercise capacity and cardiac function in patients with obstructive hypertrophic cardiomyopathy has not previously been determined. In this study, 29 patients were evaluated during graded treadmill exercise before and after operation. Postoperatively, 27 of 29 patients reported symptomatic improvement and had greatly reduced left ventricular outflow gradient. Twenty-five of 28 patients (89 percent) attained higher exercise levels after operation, and this was accompanied by an increase in total body oxygen consumption from 16 to 21 ml/min per kg (P less than 0.005). A significant increase in cardiac index during maximal exercise also accompanied this improved exercise performance (5.0 to 5.7 liters/min per m2, P less than 0.05). The increase in maximal cardiac index was associated with greater desaturation of mixed venous blood (34 to 24 percent, P less than 0.02) in patients with preoperative angina. At a given level of mixed venous oxygen saturation (30 percent), overall mean cardiac index was higher postoperatively (4.6 to 5.2 liters/min per m2, P less than 0.05). These results suggest that, although several mechanisms probably contribute to symptomatic improvement after myotomy and myectomy, enhanced cardiac performance plays an important role in the majority of patients.     

Hypertrophic cardiomyopathy: Recent observations regarding the specificity of three hallmarks of the disease: Asymmetric septal hypertrophy,septal disorganization and systolic anterior motion of the anterior mitral leaflet

Neither asymmetric septal hypertrophy (ASH), marked cell disorganization in the ventricular septum nor systolic anterior motion of the anterior mitral leaflet (SAM) is pathognomonic of hypertrophic Cardiomyopathy. However, each is uncommonly found in patients with other cardiac disorders and is therefore a highly specific hallmark of hypertrophic cardiomyopathy. Disproportionate septal thickening does occur in about 10 percent of older children and adults with various acquired or congenital heart diseases. In these patients it usually appears to be secondary to the underlying hemodynamic state. However, disproportionate septal thickening is the usual finding in the developing embryonic and fetal heart and it is relatively common (prevalence rate about 25 percent) in normal neonates and infants with congenital heart disease.Likewise, although cell disorganization in the ventricular septum may occur with other cardiac malformations, extensive disorganization is present in about 90 percent of patients with hypertrophic cardiomyopathy and in only about 5 percent of patients with other cardiac diseases. Finally, systolic anterior motion of the anterior mitral leaflet is characteristic of those patients with hypertrophic cardiomyopathy who have obstruction to left ventricular outflow, and it rarely appears (prevalence rate about 3 percent) under basal conditions in other hemodynamic states or cardiac diseases. Hence, in analyses comprising over 1,600 patients the specificity of asymmetric septal hypertrophy, marked septal disorganization and systolic anterior motion of the anterior mitral leaflet was at least 90 percent (90, 93 and 97 percent, respectively). Furthermore, the sensitivity of extensive septal disorganization for hypertrophic cardiomyopathy was 90 percent.The data currently available therefore suggest that the vast majority of patients fulfilling the basic anatomic criteria for hypertrophic cardiomyopathy (that is, a hypertrophied nondilated left ventricle in the absence of a cardiac or systemic disease that itself could produce left ventricular hypertrophy) have a distinct disease entity with diverse clinical manifestations. The majority of such patients appear to have a genetically transmitted disease, but it is not known precisely what proportion of these patients have phenotypically similar but etiologically separate disease entities.     

Atrial systole and left ventricular filling in hypertrophic cardiomyopathy: effect of verapamil

Many patients with hypertrophic cardiomyopathy (HC) have impaired left ventricular (LV) rapid diastolic filling. To quantitate the contribution of atrial systole to LV filling, we used radionuclide angiography to study 30 normal volunteers and 42 patients with HC before and after oral administration of verapamil (320 to 560 mg/day). LV time-activity curves were constructed by combined forward and reverse gating from the R wave, and the onset of atrial systole was determined by the P-R interval. The percent of LV stroke volume filled during rapid diastolic filling and atrial systole was then computed. Peak LV filling rate during rapid diastolic filling was expressed in end-diastolic volume (EDV)/second. Peak rate of rapid diastolic filling was not different in normal patients and those with HC (3.3 +/- 0.6 versus 3.3 +/- 1.1 EDV/s) and was within the normal range in 34 patients with HC (81%). However, the contribution to LV filling volume by rapid diastolic filling was diminished in patients with HC (83 +/- 7% normal, 67 +/- 17% HC, p less than 0.001) and the contribution of atrial systole was increased (16 +/- 8% normal, 31 +/- 18% HC, p less than 0.001). LV filling volume during atrial systole was above the upper normal limit of 31% in 17 patients (40%), including 13 patients with a normal peak filling rate. After verapamil, peak filling rate increased (to 4.2 +/- 1.2 EDV/s, p less than 0.001), percent LV filling during rapid diastolic filling increased (to 83 +/- 7%, p less than 0.001), and percent LV filling during atrial systole decreased (to 16 +/- 9%, p less than 0.001). Percent LV filling volume during atrial systole was abnormal after verapamil in only 3 patients (7%). Hence, although the peak rate of rapid diastolic filling may be normal in patients with HC, the contribution to LV filling by rapid diastolic filling is reduced and that of atrial systole is thereby increased. Increased rate and magnitude of rapid diastolic filling during verapamil is associated with decrease and normalization of the contribution of atrial systole to LV filling. These data suggest that many patients with HC are at risk of hemodynamic decompensation with the onset of atrial fibrillation or other tachyarrhythmias and loss of the atrial contribution to LV filling. This risk may be reduced during verapamil therapy.     

Verapamil therapy: A new approach to the pharmacologic treatment of hypertrophic cardiomyopathy: III. Effects of long-term administration

To determine the efficacy of long-term therapy with verapamil in patients with hypertrophic cardiomyopathy, 78 patients began treatment with the drug in the hospital. Sixty-two patients (79 percent) were in New York Heart Association functional class III or IV despite treatment with beta receptor blocking drugs. Fifty-four percent of all patients evaluated (42 of 78) and 63 percent of those discharged from the hospital (42 of 68) experienced sustained symptomatic improvement 6 to 30 months (median 14 months) after initiation of verapamil therapy. Of these 42 patients in improved condition, 25 had improvement by at least one New York Heart Association functional class, 14 improved by less than one functional class, two felt better taking verapamil than propranolol, and in one patient verapamil controlled asymptomatic ventricular tachycardia. Of the 53 patients who had the obstructive form of the disorder and were considered operative candidates, 25 (47 percent) experienced sufficient improvement so as to forgo operation. In patients remaining on verapamil therapy, the duration of treadmill exercise performed 5 days after the start of verapamil therapy increased by 3.1 ± 0.6 minutes (53 ± 10 percent, p < 0.001) from the value obtained with no medication before verapamil. A further increase of 2.3 ± 0.6 minutes (25 ± 7 percent, p < 0.0025) over the initial value with verapamil was recorded on the patients' last vistt (median 12 months after the start of therapy). Echocardiographic measurements of wall thicknesses and left atrial dimension demonstrated no significant changes during 1 year of verapamil treatment in 31 patients. Administration of verapamil was associated with adverse hemodynamic effects in 9 patients (12 percent) and adverse electrophysiologic effects In 10 (13 percent): Three patients died (with pulmonary edema) and 6 had to have treatment terminated. These results indicate an important role for long-term verapamil therapy in the treatment of hypertrophic cardiomyopathy, but patients must be carefully selected and followed up closely for the development of important adverse hemodynamic or electrophysiologic effects.     

Exercise, electrocardiographic and functional responses after percutaneous transluminal coronary angioplasty

Exercise testing after successful PTCA showed improved cardiac functional status on examination of electrocardiographic and symptomatic responses, myocardial perfusion and global and regional left ventricular function. Sixty-six patients were studied before and after persistently successful PTCA. Follow-up studies an average of 8 months after the successful procedure showed an incidence of abnormal testing of only 7% using both electrocardiographic and subjective symptomatic criteria during treadmill studies and no abnormal studies with thallium scintigraphy. Radionuclide cineangiography demonstrated similar left ventricular ejection fractions at rest before and after PTCA, but an improvement of 9 ± 10% (p < 0.001) in the exercise ejection fraction at follow-up. However, 52% of patients with paired data still had an abnormal radionuclide cineangiographic study after successful PTCA, raising the question of the presence of subclinical ischemia or a false-positive result.     

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.     

Quantitative measurement of electrical instability as a function of myocardial infarct size in the dog

To investigate the relation between electrical instability and myocardial infarct size, 20 foxhounds were studied in the awake state 3 to 5 days after closed chest coronary occlusion. Programmed right ventricular stimulation was performed with use of an epicardial electrode. After six paced beats at 10 percent greater than control rate, single and then double extrastimuli were introduced, scanning from late diastole to ventricular refractoriness in steps of 10 to 20 ms. Abnormal responses observed after this provocation were repetitive ventricular response, unsustained ventricular tachycardia, sustained ventricular tachycardia and ventricular fibrillation. Scores for electrical instability were determined for each dog, with higher scores assigned for more hazardous tachyarrhythmias (ventricular fibrillation greater than sustained ventricular tachycardia greater than unsustained ventricular tachycardia greater than repetitive ventricular response) and for those provokable later in diastole. An electrical instability index derived from these scores correlated well with infarct size measured with tetrazolium staining (r = 0.94). When scores were given only for the type of abnormal response elicited, excluding the effect of diastolic timing and the number of extrastimuli or vice versa, there was no significant difference in correlation with infarct size (r = 0.85 versus 0.92). Thus the results demonstrate that inducible electrical instability early after infarction is directly related to infarct size. Further, these data demonstrate the usefulness of an electrical instability index derived from the results of programmed right ventricular stimulation in assessing the severity of ischemic damage to the heart.     

Can noninvasive exercise test criteria identify patients with left main or 3-vessel coronary disease after a first myocardial infarction?

This study attempts to determine whether exercise treadmill testing with clinical, electrocardiographic, and thallium-201 myocardial perfusion imaging data can identify which patients have left main or 3-vessel (anatomically high-risk) coronary artery disease (CAD) after their first transmural myocardial infarct (MI). Twelve exercise test criteria for high-risk disease were compared in 40 patients referred for cardiac catheterization; 34 had a history of chest pain and 17 had angiographically defined high-risk CAD. A thallium image defect outside the vascular distribution of the MI was the most reliable criterion to distinguish patients with high-risk CAD (p = 0.00052 for Fisher's exact test of discrimination). Thallium imaging was somewhat more sensitive (92 versus 65%, p = 0.108) when patients with negative thallium imaging criteria who failed to achieve 85% of the age-predicted maximal heart rate were excluded. Failure to achieve 85% of predicted heart rate was by itself a useful criterion for detecting high-risk CAD (p = 0.017), especially in patients not taking propranolol (p = 0.004). Development of positive S-T segment depression at less than 70% predicted heart rate also discriminated left main or 3-vessel disease from less extensive CAD (p = 0.016). Other criteria failed to discriminate significantly between high-risk and less extensive CAD in patients after their first MI (p greater than 0.05). S-T segment depression (p = 0.199) or chest pain (p = 0.577) during exercise testing were particularly unreliable. Further, none of the criteria for high-risk CAD were influenced by irreversible left ventricular dysfunction. It is concluded that patients with thallium imaging defects outside the region of the infarct, decreasing blood pressure during exercise, failure to achieve 85% of predicted heart rate, or S-T depression at less than 70% of predicted heart rate have a high probability of having left main or 3-vessel disease. Patients without these criteria have a very low probability of having high-risk CAD and probably do not need coronary angiography for the purpose of excluding these high-risk coronary lesions after a first MI.     

Formation of cartilage in bioprosthetic cardiac valves implanted in sheep: A morphologic study

Foci of cartilage were found in 12 of 120 bioprostheses implanted in young sheep for 13 to 24 weeks, but in none of 47 bioprostheses implanted for < 13 weeks. Cartilage was found more frequently (p < 0.01) in bioprotheses implanted in the tricuspid position than in those implanted in the mitral position. In porcine aortic valvular bioprostheses, the cartilage was preferentially localized in the region of the muscle shelf; in pericardial bioprostheses, it occurred in the fibrous sheaths covering the cusps. In both instances, the cartilage was found to undergo calcification and was considered to be formed by metaplasia of connective tissue cells of host origin.     

Myocardial ultrastructure in patients with chronic aortic valve disease

Light and electron microscopic observations were made on left ventricular myocardium removed at operation from 16 patients with chronic aortic valve disease. In all 16 patients most cardiac muscle cells were hypertrophid, and surrounded by small amounts of fibrous tissue. In two of the six patients with pure aortic regurgitation and in four of the five patients with combined aortic stenosis and regurgitation, cardiac muscle cells with evidence of degeneration were present in addition to hypertrophied, nondegenerated cells. Degenerated cardiac muscle cells were not observed in the six patients with predominant aortic stenosis. Cardiac muscle cells with mild degeneration showed focal myofibrillar lysis, with preferential loss of thick myofilaments, and focal proliferation of tubules of sarcoplasmic reticulum. More severely degenerated muscle cells showed a marked decrease in the numbers of myofibrils and T tubules and proliferation of sarcoplasmic reticulum or mitochondria, or both. Severely degenerated cells usually were present in areas of marked fibrosis, often were atrophic, had thickened basement membranes and had lost their intercellular connections. These findings suggest that degenerated cardiac muscle cells have poor contractile function and may be responsible for impaired cardiac performance in some patients with chronic aortic valve disease.