[Impact of preoperative 64-row multislice computed tomography for congenital aortic stenosis; report of a case].

A 63-year-old woman was diagnosed as having severe aortic stenosis (AS) with 98 mmHg peak pressure gradient detected by echocardiography. Since, preoperative enhanced 64-row multislice computed tomography (MSCT) showed bicuspid aortic valve with only 2 sinuses of Valsalva, congenital aortic stenosis was suspected. The left and right coronary arteries originated from respective sinus of Valsalva, and severely thickened cusps of aortic valve were detected clearly by preoperative 64-row MSCT. Aortic valve replacement with a 21 mm ATS mechanical bileaflet prosthesis was performed without aortic annulus enlargement. The postoperative course was uneventful and postoperative 64-row MSCT indicated good performance of the ATS valve. Preoperative 64-row MSCT could be useful to detect complex aortic valve disease in detail. Moreover. 64-row MSCT might be a reliable tool to evaluate valvular heart disease.

Electrocardiographic features differentiating dilated cardiomyopathy from hypertrophic cardiomyopathy.

To determine the usefulness of electrocardiographic (ECG) features in differentiating between hypertrophic cardiomyopathy with features mimicking dilated cardiomyopathy (D-HCM) and true dilated cardiomyopathy (DCM), we compared ECGs of 52 consecutive patients (11 with D-HCM, 41 with DCM). Left atrial dimension, left ventricular internal dimension, and septal and posterior wall thickness were employed as echocardiographic indexes, while QRS duration, amplitude of RV5 or V6 + SV1, number of abnormal Q waves, P-terminal force in V1, and frontal plane QRS axis were used as ECG parameters. The patients with D-HCM demonstrated a larger number of abnormal Q waves (P < .0001), greater prolongation of QRS duration (P < .0001), and lower amplitude of RV5 or V6 + SV1 (P < .0001). In all cases of D-HCM, atrial overload was observed and abnormal QRS axis in 9 (82%) of the 11 patients. These features were noted in 21 (51%) and 17 (41%), respectively, of the 41 DCM patients (P < .005 and P < .05, respectively). Despite significant differences in the echocardiographic parameters between D-HCM and DCM, excluding left ventricular end-diastolic dimension, ECG abnormalities were more significant between the two groups. The results indicate that ECG features are extremely useful in differentiation between DCM and D-HCM.

Clinical significance of abnormal postexercise systolic blood pressure response in patients with hypertrophic cardiomyopathy.

The clinical significance of abnormal postexercise systolic blood pressure (SBP) response in patients with hypertrophic cardiomyopathy (HCM) was assessed by treadmill exercise testing in 30 normal subjects and 96 patients with HCM. SBP recovery ratios were derived by dividing the SBP at 1 and 3 min after exercise by the peak exercise SBP. The upper normal limit of the SBP ratio was defined by two standard deviations (SD) from the mean in 30 normal subjects. A postexercise SBP ratio that exceeded the upper normal limits of the SBP ratio was considered to be an abnormal SBP response in patients with HCM. Twenty-seven (28%) of 96 patients with HCM showed an abnormal SBP response. Compared with the normal SBP response group (69 cases) among patients with HCM, the abnormal SBP response group had lower SBP response during exercise (22 +/- 25 vs 62 +/- 26 mmHg : mean +/- 1 SD, p < 0.05), more prolonged QRS width (110 +/- 21 vs 92 +/- 20 msec, p < 0.05), higher incidences of ventricular tachycardia (12 vs 7 cases, p < 0.001), and sudden death (7 vs 0 cases, p < 0.0001). The defect area of Tl-201 myocardial scintigraphy was more extensive in the abnormal SBP response group (2.9 +/- 1.7 segments) than in the normal SBP response group (1.1 +/- 1.3 segments, p < 0.05). During atrial pacing (120/min), pulmonary artery wedge pressure was slightly elevated from 10 +/- 2 (at rest) to 14 +/- 3 mmHg (during pacing) (p<0.001), cardiac index showed no significant changes, and time constant T was shortened from 58 +/- 13 to 48 +/- 10 msec (p < 0.001) in the normal SBP response group, but in the abnormal SBP response group pulmonary artery wedge pressure was highly elevated from 12 +/- 5 to 20 +/- 3 mmHg (p < 0.0001), cardiac index was decreased from 2.5 +/- 0.7 to 2.1 +/- 0.6 l/min/m2 (p < 0.05), and the time constant T had no significant changes. These observations suggest that patients with HCM and abnormal postexercise SBP response have an abnormal cardiac response during exercise and extensive myocardial damage.

[Pulmonary hypertension due to glycogen storage disease type II (Pompe's disease): a case report].

A rare case of pulmonary hypertension due to glycogen storage disease type II (Pompe's disease) was reported. An 18-year-old girl was admitted to Kawasaki Medical School Hospital because of cyanosis, dyspnea on exertion and amenorrhea. She was 149 cm in height and 29 kg in body weight. Clinical examination revealed that pulmonary artery pulse and right ventricular heave were palpable over the precordium. On auscultation, an accentuated pulmonic second heart sound, pulmonic ejection sound and diastolic decrescendo murmur (Levine III/VI) were heard in the second intercostal space at the right sternal border. Her skeletal muscles, especially her intercostal muscles were generally weak and atrophic. Her electrocardiogram showed a pulmonary P-wave and right ventricular hypertrophy. The chest X-ray revealed right ventricular enlargement and a dilated pulmonary trunk. On echocardiography, the right ventricle and the main pulmonary artery were dilated, and a systolic notch of the pulmonary valve was found. Swan-Ganz catheterization disclosed that pulmonary artery pressure, right ventricular pressure and mean pulmonary capillary wedge pressure were 76/35 (50) mmHg, 76/12 mmHg and 10 mmHg, respectively. Respiratory function tests showed severe restrictive ventilatory impairment with hypercapnea and hypoxemia. On biopsy of the left quadriceps femoris muscle, the most striking finding was numerous intracytoplasmic vacuoles. The small vacuoles were stained with PAS and acid phosphatase. Electron microscopy showed massive glycogen accumulation in the sarcoplasm and membrane bound vacuoles (glycogenosome). Alpha-1, 4-glucosidase activity in the peripheral lymphocytes was definitely decreased. Her pulmonary hypertension resulted from respiratory muscular atrophy and alveolar hypoventilation caused by Pompe's disease.

[Clinical significance of electrocardiographic P-terminal force in V1 in patients with hypertrophic cardiomyopathy].

The clinical usefulness of P-terminal force in lead V1 (P-TF) was evaluated in 49 patients with hypertrophic cardiomyopathy. These 49 patients were categorized as 17 with an abnormal P-wave (P-TF greater than or equal to 3.0 msec.mV: group A) and 32 with a normal P-wave (P-TF less than 3.0 msec.mV: group B). Systolic blood pressure response (delta SBP) during the treadmill exercise test was significantly less in the group A than in the group B (delta SBP: 36 +/- 22 mmHg, 58 +/- 26 mmHg, respectively). Left atrial dimension measured from the M-mode echocardiogram was 37.8 +/- 5.0 mm in the group A and 31.0 +/- 5.2 mm in the group B. These differences were statistically significant (p less than 0.05). Left ventricular end-diastolic pressure averaged 19.5 +/- 7.9 mmHg in the group A and 13.5 +/- 4.0 mmHg in the group B (p less than 0.05). When comparing the group A and B, negative dp/dt / positive dp/dt of the group A was significantly less (0.69 +/- 0.14, 0.90 +/- 0.18, respectively) and the time constant T of the group A was significantly prolonged (86 +/- 30 msec, 56 +/- 10 msec, respectively). According to classifications of hypertrophic cardiomyopathy, all patients with the obstructive type had an abnormal P-wave. On the contrary, the majority of patients with apical hypertrophy had a normal P-wave.(ABSTRACT TRUNCATED AT 250 WORDS)

Right ventricular outflow obstruction secondary to post-infarction aneurysm: a possible new syndrome.

Two patients with extensive anterior myocardial infarction developed a hitherto unreported type of right ventricular outflow tract obstruction. A 71-year-old woman, who had had an acute infarction 10 years before, was admitted for sustained ventricular tachycardia. A loud ejection murmur was heard in the mid-precordium. The echocardiogram and left ventriculogram showed a septal aneurysm, with a systolic gradient of 21 mmHg between the right ventricular outflow tract and apex. The ejection murmur was detected in the outflow tract by intracardiac phonocardiography. The second patient was a 60-year-old man who had had an acute infarction at age 47. He was also referred because of ventricular tachycardia, and his clinical situation was almost the same as that of the first case. Our search of the literature failed to disclose any similar case with a loud ejection murmur confirmed by intracardiac phonocardiography to be due to an obstructive septal aneurysm.

[Usefulness of measuring right ventricular anterior wall thickness in patients with right ventricular overload using M-mode echocardiography].

Right ventricular anterior wall thickness measured by M-mode echocardiography and right ventricular systolic pressure obtained by right heart catheterization were correlated in 62 patients with chronic right ventricular overload including congenital heart disease and primary pulmonary hypertension. The patients were divided into two groups; one, with right ventricular systolic pressures of 39 mmHg or less; the other, 40 mmHg or more. The following results were obtained. 1. The correlation coefficient for right ventricular anterior wall thickness and right ventricular systolic pressure was r = 0.90 (p less than 0.001), and the regression equation was y = 13.2x-1.3. 2. Right ventricular end-diastolic dimension increased significantly in both groups, but no statistically significant differences were detected between the two. Right ventricular anterior wall thickness increased significantly in the group with higher right ventricular pressures (7.1 +/- 0.5 mm vs 3.1 +/- 0.5 mm). 3. When right ventricular anterior wall thickness was more than 4.0 mm, pulmonary hypertension was detected, with a sensitivity of 97.5% and a specificity of 90.9%. In conclusion, measurements of right ventricular anterior wall thickness by M-mode echocardiography via the anterior chest wall proved to be potentially useful in predicting right ventricular systolic pressures in patients with chronic right ventricular overloads.

[Clinical features and significance of hypertrophic cardiomyopathy with atrial fibrillation].

To elucidate the clinical features of hypertrophic cardiomyopathy (HCM) with atrial fibrillation (Af), the complications and prognosis of 18 patients with HCM complicated by Af (Af group) were compared with those of 20 patients without Af (non-Af group). The Af group was categorized depending on duration as 1. Af group I (four patients) with persistent Af at diagnosis, 2. Af group II (10 patients) with transient Af of more than five hours' duration, and 3. Af group III (four patients) with Af less than five hours' duration. The results were as follows: 1. During the follow-up period, among the 18 patients in the Af group, five including four patients in the Af group I, developed arterial embolism, 10 developed congestive heart failure, and three died suddenly. The patients in the non-Af group had excellent courses without serious complications. 2. Electrical cardioversion was performed in six patients in the Af group II, and sinus rhythm was restored in all of them, resulting in improved clinical courses. Thus, no complications developed in four patients whose sinus rhythm was maintained and whose clinical courses were favorable. 3. The P-terminal force in V1 prior to onset of Af in the Af groups II and III, left atrial dimension, and the cardiothoracic ratio in the Af group, were significantly greater than that of the non-Af group (p less than 0.01, p less than 0.05, p less than 0.05, respectively). In conclusion, HCM associated with Af has a poor prognosis, with severe complications such as heart failure, arterial embolism and sudden death. Cardioversion may be an effective method of choice of intervention in patients with recently-developed Af. P-terminal force in V1, left atrial dimension, and cardiothoracic ratio are regarded important factors in the development of Af.

[Mid-systolic ejection murmur with thrill caused by right ventricular outflow tract obstruction secondary to septal aneurysm following myocardial infarction: a case report].

A 71-year-old woman with a history of previous myocardial infarction was transferred to our hospital for evaluation of chest pain and ventricular tachycardia. On admission, a loud mid-systolic ejection murmur accompanied by a thrill was found at the left sternal border in the third intercostal space, and it was significantly accentuated in the post-extrasystolic beat. Abnormal Q waves and ST elevations were noted in leads I, aVL and V5,6 on electrocardiograms. Echocardiograms, confirmed a septal-to-apical aneurysm, and a thin interventricular septum (IVS) with paradoxical motion. Right ventricular (RV) catheterization showed a pressure gradient of 21 mmHg between the outflow tract (RVOT) and the apex, and a mid-systolic ejection murmur was recorded in the RVOT on an intracardiac phonocardiogram. Coronary arteriograms revealed total occlusion of the left anterior descending artery in its proximal portion, and a 90% stenosis of the circumflex artery. A left ventriculogram demonstrated a septal-to-apical aneurysm with a markedly reduced ejection fraction of 0.16. A right ventriculogram showed obstruction to RVOT caused by systolic ballooning of the IVS. In this patient, the mid-systolic ejection murmur was probably caused by the obstruction of the outflow tract secondary to septal aneurysm following old myocardial infarction.

[Reversed Hegglin syndrome in Takayasu arteritis].

To study the "reversed Hegglin syndrome" in Takayasu arteritis, 57 patients were selected, and divided into four groups; Group I: 12 with Takayasu arteritis with aortic regurgitation (AR), Group II: 27 with Takayasu arteritis without AR, Group III: six with annuloaortic ectasia and severe AR, and Group IV: 12 with severe AR of other miscellaneous etiologies. QII-QT intervals were compared among the four groups. The reversed Hegglin syndrome (QII-QT greater than or equal to 40 msec) was observed in 58 percent of Group I patients and 37 percent of Group II patients. This phenomenon was not seen in any patients in Group III or IV. Sixteen of the 17 patients who had the reversed Hegglin syndrome belonged to the thoracic aortic type or diffuse type of Takayasu arteritis. Most patients who did not have this syndrome either had a localized lesion or a prolonged QT interval. The reversed Hegglin syndrome, therefore, is related to widespread lesions involving the thoracic aorta due to Takayasu arteritis, and may be attributed to a delay in closure of the aortic second heart sound due to a decrease in aortic impedance of the diseased proximal aorta.