Congenital solitary kidney with renovascular hypertension diagnosed by means of captopril-enhanced renography and magnetic resonance angiography.

A 24 year-old woman had a congenital solitary kidney with renovascular hypertension due to fibromuscular dysplasia. She had been treated as having essential hypertension until she developed preeclampsia and HELLP (hemolysis, elevated liver enzymes, and low platelet count) syndrome at 28 weeks of gestation. Plasma renin activity and captopril test results did not indicate any abnormalities. However, renography revealed captopril-induced deterioration. Magnetic resonance angiography was also useful to detect renal artery stenosis. These findings were confirmed by renal angiography. After successful percutaneous transluminal renal angioplasty, her blood pressure and the pattern of captopril renography normalized.

A critical increase in right-to-left shunt after acute myocardial infarction in a 68-year-old male with tetralogy of Fallot.

A male patient with tetralogy of Fallot accompanied by aortic regurgitation had maintained sufficient exercise capacity for a number of decades with the status of acyanotic tetralogy of Fallot. When he was 67 years old, he suffered a posterior wall acute myocardial infarction and direct percutaneous coronary angioplasty successfully revascularised the target lesion in the left circumflex artery. However, a few months after the onset of the myocardial infarction, his shortness of breath became clinically significant and was associated with increased right-to-left shunt and increased right ventricular end-diastolic pressure, as well as hypoxia. At 68 years old, therefore, total corrective repair of the tetralogy with replacement of the aortic and pulmonary valves was performed. The patient was asymptomatic after the successful operation. This report suggests that coronary artery disease can be one of the potential factors in inducing critical hemodynamic changes in aging patients with congenital heart disease, especially those who have a shunt between the right and left chambers. The unique clinical course is described with some discussion of the repair of tetralogy in adults.

Impact of mitral regurgitation on long-term survival in patients with ischemic cardiomyopathy: efficacy of combined mitral valve repair and revascularization.

Ischemic cardiomyopathy complicated by severe mitral regurgitation (MR) has a poor prognosis. In such cases, whether mitral valve repair for MR improves the prognosis of survival remains unclear. In this study, 50 patients diagnosed with ischemic cardiomyopathy at our hospital between August 1991 and August 1996 were studied to examine the long-term prognosis and factors determining the prognosis. Among 17 patients with the complication of severe MR, 11 underwent mitral valve repair (repair group) and 6 did not (nonrepair group). Among the 33 patients without MR, 15 underwent revascularization (revascularization group) and 18 received medical treatment alone (medical group). Patients with MR showed significantly poorer baseline activities of daily living (ADL) [New York Heart Association (NYHA) class III or above: MR(+) vs MR(-) = 14 vs 8; P = 0.0001] and survival rate [MR(+) vs MR(-); log rank = 3.8, P = 0.05]. In contrast, patients in whom mitral valve repair was actively performed to resolve MR had favorable outcomes for both ADL (NYHA class improved from 3.9 +/- 0.3 to 2.7 +/- 1.0; P = 0.0004) and survival rate (MV repair vs nonrepair: long rank = 10.1, P = 0.0015). In addition, among patients without MR, the revascularization group showed more favorable results in terms of ADL (NYHA class improved from 3.5 +/- 0.7 to 2.5 +/- 0.8; P = 0.0059) and survival rate (revascularization vs medical: log rank = 3.7, P = 0.05), irrespective of improvement of left ventricular function. When the factors determining the prognosis for ischemic cardiomyopathy were examined by multivariate analysis, whether or not revascularization was conducted, the presence or absence of mitral regurgitation, and if present, whether or not mitral valve repair was performed were identified as independent factors determining the prognosis (revascularization: hazard ratio = 0.121, P = 0.012; absence of MR: hazard ratio = 0.104, P = 0.050; mitral valve repair: hazard ratio = 0.018, P = 0.005). These results showed that revascularization should be conducted as actively as possible in patients with ischemic cardiomyopathy; in addition, for those patients with mitral regurgitation, mitral valve repair should be conducted actively to relieve it.

Trends over the last 20 years in the clinical background of young Japanese patients with coronary artery disease.

BACKGROUND: The prevalence, pathogenesis, and clinical background of coronary artery disease (CAD) in patients aged 40 years or less in Japan are not well understood. METHODS AND RESULTS: Temporal trends in the clinical background, including growth from childhood, of young patients with CAD over the last 20 years were examined. The study group comprised 38 patients who were 40 years of age or less (7 patients in 1980-84, phase I; 10 patients in 1985-89, phase II; 10 patients in 1990-94, phase III; 11 patients in 1995-99, phase IV). Among the classic coronary risk factors, obesity significantly increased in prevalence. An increase in patients with multiple risk factors was seen (0, 10%, 20%, and 36% in phases I, II, III, and IV, respectively). There was no significant change in the prevalence of familial hypercholesterolemia, sequelae of Kawasaki disease or vasospastic angina. All phase III and IV patients with multiple risk factors had moderate to severe obesity, and 83% had been overweight since childhood. CONCLUSIONS: These results suggest that the number of young patients with CAD because of multiple risk factors has been increasing, and most of them have been overweight since childhood. Thus, for primary prevention it is essential to control cardiovascular risk factors in overweight children.

Percutaneous cardiopulmonary support aids resuscitation from sustained ventricular tachycardia.

A 67-year-old man was transferred to hospital because of acute circulatory failure resulting from sustained left ventricular tachycardia (LVT) and dysfunction. Transthoracic echocardiography revealed severely impaired left ventricular contraction and dyskinesis of the apical wall. Neither anti-arrhythmic agents nor direct current cardioversion was effective; the patient was resuscitated by immediate use of percutaneous cardiopulmonary support and intraaortic balloon counterpulsation. Ventricular contraction returned to normal following restoration of normal sinus rhythm with amiodarone and cibenzoline. The pathogenesis of LVT accompanied by transient ventricular dyskinesis is discussed with regard to the efficient use of a mechanical circulatory support system in resuscitation.

Nocturnal oxygen desaturation correlates with the severity of coronary atherosclerosis in coronary artery disease.

STUDY OBJECTIVES: It has been suggested that sleep-disordered breathing (SDB) is a risk factor for ischemic heart disease, and may be associated with increased morbidity and mortality due to cardiovascular disease. The aim of this study was to examine the relation between nocturnal oxygen desaturation (NOD) due to SDB and the Gensini score, which is given to define the severity of coronary atherosclerosis, based on coronary angiograms findings, in patients with coronary artery disease. DESIGN: We examined the NOD index (ODI) (desaturation of > 3%/events per hour) using pulse oximetry in 59 consecutive patients with coronary artery disease (ejection fraction, > 40%) that was diagnosed by coronary angiography, 30 patients with angina pectoris and 29 patients with old myocardial infarction. The Gensini score was calculated for each patient from the coronary arteriogram. The patients were classified into the following three groups according to the severity of oxygen desaturation: ODI of < 5 events per hour (group N; 16 patients); ODI of > or = 5 but < 15 events per hour (group A; 27 patients); and ODI of > or = 15 events per hour (group B; 16 patients). The groups then were examined for the relation between the ODI and the Gensini score. RESULTS: Of the total number of patients, 72.9% had a nocturnal ODI of more than five events per hour. The Gensini score was significantly higher in groups A and B than in group N, and showed a significant positive correlation with the ODI (R = 0.45; p = 0.01) in all patients. Multiple regression analysis showed that the ODI was the most significant, independent determinant of the Gensini score among the coronary risk factors tested, and that it explained 13.4% of the variance. CONCLUSION: These findings suggest that NOD due to SDB may be an important contributor to coronary atherosclerosis in the patients with cardiovascular disease.

A case of acute rheumatic fever accompanied by transient aortic regurgitation.

Though acute rheumatic fever (RF) is now rare in Japan, it continues to be an important disease condition that physicians should be prepared to diagnose and treat. We describe a patient with acute RF accompanied by transient aortic regurgitation (AR). The AR was detected only by echocardiography. There were no other indications, and it disappeared after treatment with prednisolone. The changes in cardiac valves in the early phase of RF have been the subject of only a few case studies. Echocardiography is quite valuable in the workup of patients with acute RF and should be performed even if there are no signs of cardiac involvement.