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.     

Anomalous chordae tendineae of the left ventricle. Echocardiographic study

Left ventricular false tendons (or anomalous bands) have been described in several anatomic studies. Recently the echocardiographic features of such false tendons have been reported also. We have found a prevalence of 36 cases in 1,600 consecutive patients examined (2.2%). False tendons represent a rather common and benign phenomenon. Echocardiography is the most useful tool in the detection of false tendons.     

Morphologic course of the left ventricle after renal transplantation. Echocardiographic study

OBJECTIVE: To characterize and to quantitate the morphologic changes in left ventricle, in renal transplant patients (Pts) treated with cyclosporine, through sequential echocardiographic examinations. DESIGN: A prospective study of renal transplant patients, between October/88 and May/89, who maintained good function of the renal graft during the follow-up. SETTING: Cardiology and Nephrology departments of Santo António Hospital. MATERIAL AND METHODS: 20 patients, 13 men and 7 women, mean age of 33 +/- 10, ranging from 20 to 56, constitute the final group of the study. These patients have been receiving dialysis during 3.8 +/- 2.3 years (0.4-8). Seven patients were excluded, five by echocardiographic criteria and another two because of chronic renal graft disfunction (creatinine greater than 2.0 mg%). The echocardiographic examinations were performed during the first week, and 1, 2, 3, 6 and 12 months after renal transplantation. The following measurements were performed: left ventricular end-diastole diameter (LVED), interventricular septal thickness (IVST), posterior wall thickness (PWT) and left ventricular mass index (LVMI). The measurements were obtained in M-mode following the conventional recommendations. Average values of at least 3 cardiac cycles were used. Heart rate, blood pressure, creatinine, hematocrit, body surface area and fistula patency, were determined at the time of each echocardiogram. MAIN RESULTS: The LVED decreased progressively until the third month, from 51.9 +/- 7 mm to 47.8 +/- 6 mm (p less than 0.001), remaining stable thereafter. The baseline value of IVST, 13.6 +/- 5 mm, was similar at the twelfth month, 13.8 +/- 2 mm (ns). The baseline value of PWT, 13.7 +/- 4 mm, decreased gradually since the second month, having reached 12.7 +/- 2 mm at the twelfth month (ns). The LVMI (g/m2) reduced progressively, from 243 +/- 82 to 190 +/- 38 at the end of the study (p less than 0.05. A high incidence of arterial hypertension was detected during the follow-up period; at the twelfth month, 18 patients (90%) were on antihypertensive drug therapy, 11 of which had blood pressure greater than or equal to 160/95 mmHg. CONCLUSIONS: We verified, one year after the renal transplantation, a significant decrease of LVMI, that was mainly determined by the LVED reduction. Left ventricular walls thickness had no significant variation; we think that the high incidence of hypertension during the follow-up period, in part due to the pressure effect of cyclosporine, may have been responsible for this fact.     

Echocardiographic diagnosis of pseudoaneurysm of the left ventricle.

The echocardiographic features of postinfarction pseudoaneurysm of the left ventricle are described for the first time. Because ultrasound allows the detection of soft-tissue structures in a manner not possible with other diagnostic techniques, the left ventricular wall can be visualized separating the left ventricular cavity from the saccular aneurysm which is delineated by pericardium and/or extracardiac tissue. In addition to these anatomic findings, relevant qualitative hemodynamic data can also be obtained. Echocardiography seems to be a safe and specific method for the diagnosis of left ventricular pseudoaneurysm. It is suggested that echocardiography should be used in the incipeint phase of pseudoaneurysm formation to detect subacute cardiac rupture.     

Echocardiographic study of U wave inversion in the electrocardiograms of hypertensive patients

The prevalence of U wave inversion was evaluated in 58 adult patients with hypertension, and a possible mechanism for it was examined using M-mode echocardiographic indices. U wave inversion was the most common electrocardiographic abnormality, occurring in 34% of patients; voltage criteria for left ventricular hypertrophy were present in only 14% of patients, and ventricular strain pattern was not detected in any patient. Nonetheless, on echocardiography left ventricular posterior wall thickness was increased in 58% of patients. However, neither U wave inversion nor conventional voltage criteria for left ventricular hypertrophy was strongly predictive for this finding. The authors conclude that U wave inversion is a frequent finding in patients with hypertension, often occurring alone. Although it does not appear to be closely linked to the presence of left ventricular hypertrophy, it may relate to other, perhaps subtle, abnormalities of diastolic ventricular relaxation.     

A case of multiple coronary microfistulas to the left ventricle and apical myocardial hypertrophy coexisting with stable angina

A coronary artery fistula consists of a communication between a coronary artery and a cardiac chamber, a great artery or the vena cava. It is the most common congenital anomaly that can affect coronary perfusion. However, coronary fistulas to one of the cardiac chambers and coexisting apical myocardial hypertophy are infrequent anomalies, and usually are found unexpectedly. Herein, we report a case in which all three major coronary arteries emptied into the left ventricle with apical hypertrophy, through multiple microfistulas.     

Giant post-infarction pseudoaneurysm of the left ventricle manifesting as severe heart failure

A 57 year-old female was admitted for chronic heart failure (HF) with NYHA class IV symptoms. Transthoracic echocardiography revealed ruptured left ventricular (LV) lateral and posterior wall between their basal and middle segments resulting in giant, round pseudoaneurysm formation with a diameter of 12 cm. Bidirectional flow through a 2.9 cm orifice between the LV and the pseudoaneurysm cavity was shown. A 12-cm diameter pseudoaneurysm was resected and the orifice was closed with a Dacron patch. Twelve months after the diagnosis, the patient is in a stable condition with NYHA class II HF symptoms.     

Echocardiographic diastolic abnormalities of the left ventricle in inflammatory joint disease.

Echocardiographic early diastolic abnormalities have been shown recently in 50% of men with ankylosing spondylitis. Similar techniques were used to investigate subjects with rheumatoid arthritis and psoriatic arthritis with or without spondylitis. These subjects had no clinical, radiographic, or electrocardiographic evidence of cardiac or respiratory disease. Echocardiographic abnormalities seen resembled those of ankylosing spondylitis in that the interval between minimum left ventricular dimension and mitral valve opening was prolonged in 12 of 22 subjects with rheumatoid arthritis and in seven of 11 subjects with psoriatic arthritis. Isovolumic relaxation time was significantly prolonged in four subjects with rheumatoid arthritis and one with psoriatic arthritis. Unlike ankylosing spondylitis, however, there was consistent reduction in peak rate of left ventricular dimension increase in subjects with rheumatoid arthritis and psoriatic arthritis. In addition, the dimension increase during atrial systole was greater than normal in nine subjects with rheumatoid arthritis and two with psoriatic arthritis. The most likely cause of these abnormalities is increased connective tissue deposition in the myocardium.     

Echocardiographic study of hypertrophic cardiomyopathy localized in the free wall of the left ventricle

Previous studies have shown that the Hypertrophic Cardiomyopathy may involve the left ventricle free wall, without involving the intraventricular septum. We describe two young sisters who were suspected of having Hypertrophic Cardiomyopathy because of their family history and because of abnormal electrocardiographic findings. M-mode echocardiography showed normal ventricular septal thickness, increased thickness of the left ventricular posterior wall, decreased diastolic posterior wall velocity, normal left ventricular outflow tract. Two-dimensional echocardiography showed that hypertrophy was limited to left ventricular free wall, especially to the lateral and anterior segments. Echocardiography can thus identify unusual forms of hypertrophic cardiomyopathy.     

Echocardiographic diagnosis of left ventricular myxoma. Description of a case and review of the literature. Myxomas of the left ventricle

A case of left ventricular myxoma diagnosed by echocardiography and successfully removed by left ventriculotomy is reported. This is a 21 year old male, with the few symptoms which simulating an hypertrophic cardiomyopathy in contrast to the large size of the tumour. It is possible that myxomas are responsible for sudden death. Therefore, in presence of new cardiac signs kind and relevance, the possibility of a myxoma should be considered. The diagnosis can be easily ruled out (or confirmed) by echocardiography, which represents a valuable tool in the diagnosis of myxomas.     

Prevalence, characteristics and clinical correlates of left ventricular hypertrophy in stable hypertension. Echocardiographic study

251 in stable hypertensives were studied with M-Mode and two-dimensional echocardiography, in order to evaluate the prevalence of left ventricular hypertrophy (LVH), its patterns and clinical correlates. 54 subjects (21%) had been treated previously with antihypertensive drugs while the remaining 197 (79%) had not. A normal left ventricle was found in 69 subjects (27.1%; group A); a concentric LVH (h/r greater than or equal to 0.45) was found in 99 (39.4%; group B), while an eccentric LVH (left ventricular myocardial mass index greater than 140 g/m2, h/r less than 0.45) in 83 (43.1%; group C). An asymmetric LVH (septum to posterior wall thickness ratio greater than or equal to 1.3) was found in 33 subjects (3 did not fulfill the criteria for LVH, 21 had a concentric, and 10 an eccentric LVH). Mean age was significantly higher in group B as compared to groups A and C. The body mass index was comparable in all groups. Mean systolic blood pressure was significantly higher in groups B and C as compared to A. The duration and the severity of hypertension did not differ among the three groups. W.H.O. stage III was absent in group A, but no differences were found between group B and C as for W.H.O. stage distribution. We conclude that such factors as BMI, duration and severity of hypertension poorly correlate with the occurrence of LVH and its patterns. The fact that group B hypertensives were older than the others on average is against the hypothesis that eccentric hypertrophy follows the concentric pattern. Whether these two patterns represent separate entities in relation to different hemodynamic profiles deserves further investigation.     

Follow-up study of diastolic paradoxical flow in the left ventricle with apical hypertrophic cardiomyopathy

OBJECTIVES: Left ventricular apical asynergy and cardiovascular complications were evaluated in patients with apical hypertrophic cardiomyopathy, in whom apical diastolic paradoxical flow toward the base could be detected by Doppler color flow echocardiography. METHODS: Twenty-nine patients with apical hypertrophic cardiomyopathy were followed up with echocardiographic examinations for at least 5 years. They were divided into three groups: those who persistently exhibited paradoxical flow (Group A, n = 13), those in whom paradoxical flow developed during the follow-up period (Group B, n = 8), and those in whom paradoxical flow was not detected during the follow-up period (Group C, n = 8). Peak flow velocity and duration of paradoxical flow, the presence or absence of apical asynergy, and cardiovascular complications were evaluated. RESULTS: The mean follow-up period was 7.3 +/- 1.4 years. In Groups A and B, the apical wall motion deteriorated over time, whereas there was no asynergy change in Group C. Further, peak flow velocity and duration of paradoxical flow increased throughout the follow-up period in Groups A and B, and were correlated with the severity of apical asynergy. Ventricular tachycardia and cerebrovascular complications also occurred more often in patients with paradoxical flow. CONCLUSIONS: The presence of paradoxical flow was related to the severity of apical asynergy and cardiovascular complications, and may be an important marker for evaluating the clinical course of patients with apical hypertrophic cardiomyopathy.     

Echocardiographic findings and function analysis of the left ventricle in infants with endocardial fibroelastosis (author's transl)

7 infants with the clinical picture and the typical hemodynamic and angiocardiographic findings of endocardial fibroelastosis were studied echocardiographically. The echocardiograms were digitized and analyzed by the method of Gibson and Brown and compared with those of 8 normal infants. The echoes of EFE-patients showed a nearly twice as large LV-diameter. The mitral valve was displaced posteriorly, the septal motion in 4 of them abnormal. The shortening fraction was significantly reduced, the LPEP/LVET quotient increased. The comparison of LPEP with the Q to mitral valve closing interval revealed a considerable prolongation of isovolumetric contraction time. The result of computer analysis was a decrease of peak Vcf and maximum lengthening rate. The echocardiographic pattern is typical but not pathognomonic, it gives no prognostic information.     

Idiopathic calcified apical aneurysm of the left ventricle in an asymptomatic child.

To our knowledge, this is the eighth reported case of isolated, idiopathic, noncontractile apical left-ventricular aneurysm in the child. Referral to the physician is likely to be made because of a systolic murmur, unusual cardiac contour, cardiomegaly, or apical calcification on chest x-ray or, rarely, a systemic embolus. Heart failure is uncommon. The electrocardiogram shows abnormal Q-waves and/or inferior and lateral ST-T changes. Careful image intensification fluoroscopy will demonstrate apical calcification in one-half of the cases.