Results of total correction of tetralogy of Fallot performed in adults.

Today, total correction of tetralogy of Fallot is rarely performed in adults. In a 10-year period, 40 patients aged 20 to 67 years underwent intracardiac repair in our institution. Twenty-eight of them had had a palliative procedure 11 to 30 years earlier. Preoperatively, 23 patients were in New York Heart Association (NYHA) Functional Class II, 14 were in Class III, and 3 were in Class IV. Operative mortality was 2.5% (1/40). Follow-up ranged from 1 year to 11 years (average, 3 years). One patient died of a noncardiac cause 4 years after operation. Residual cardiac defects were observed in 4 patients. Postoperatively, 30 patients were in NYHA Functional Class I, 8 were in Class II, and 1 was in Class III. Major ventricular arrhythmias were recorded in 7 (35%) of 20 patients. Radionuclide angiography demonstrated impaired right ventricular function in 8 patients. Left ventricular impairment was present in 2. Total correction of tetralogy of Fallot can be performed safely in adults with low mortality and good functional improvement. The incidence of residual cardiac defects is low. The long-term importance of impaired ventricular function and arrhythmias remains to be ascertained.

Pulmonary stenosis with intact ventricular septum: documentation of development of the lesion echocardiographically during fetal life.

We describe a case of severe pulmonary stenosis with intact interventricular septum diagnosed during fetal life at 34 weeks gestational age. An echocardiographic examination had been performed on the same fetus at 20 weeks, when no cardiac anomalies were observed. It is concluded that this form of pulmonary stenosis might be a lesion either acquired during fetal life or becoming progressively more severe in the prenatal period.

Conservative management of fetal bigeminy arrhythmia leading to persistent bradycardia.

A case of persistent fetal bradycardia first detected at 32 weeks of gestational age is described. The diagnosis of atrial ectopic beats was made by means of M-mode echocardiography. Since the fetus was normally grown and showed no signs of distress, the vaginal route of delivery was allowed, also because continuous M-mode echocardiographic monitoring was possible during labour.

[Neural regulation of heart rate. Spectrum analysis of the RR interval in healthy subjects]. / Modulazione neurale della frequenza cardiaca. Analisi spettrale dell'intervallo RR in soggetti sani.

Dynamic 24-hour recordings were obtained in 10 healthy non hospitalized subjects. Spectral analysis of RR interval variabilities provided quantitative markers of sympatho-vagal balance throughout the day and night. The low frequency (0.1 Hz) component was considered a marker of sympathetic activity whereas the high frequency component (0.25 Hz) a marker of vagal tone. We observed an early morning rise in the sympathetic activity markers in all our patients, with a second smaller rise in the early afternoon. We believe this rise may be the trigger of the increased rate of major cardiovascular events reported to happen in the first hours of the morning.

Myopericarditis versus viral or idiopathic acute pericarditis.

OBJECTIVE: To investigate the relative incidence, clinical presentation and prognosis of myopericarditis among patients with idiopathic or viral acute pericarditis. DESIGN: Prospective observational clinical cohort study. SETTING: Two general hospitals from an urban area of 220 000 inhabitants. PATIENTS: 274 consecutive cases of idiopathic or viral acute pericarditis between January 2001 and June 2005. MAIN OUTCOME MEASURES: Relative prevalence of myopericarditis. Clinical features at presentation including echocardiographic data (ejection fraction (EF), wall motion score index (WMSI)) and follow-up data at 12 months including complications, results of echocardiography, electrocardiography and treadmill testing. RESULTS: Myopericarditis was recorded in 40/274 (14.6%) consecutive patients. At presentation, the following clinical features were independently associated with myopericarditis: arrhythmias (odds ratio (OR) = 17.6, 95% confidence interval (CI) 5.7 to 54.1; p<0.001), male gender (OR = 6.4, 95% CI 2.3 to 18.4; p = 0.01), age <40 years (OR = 6.1, 95% CI 2.2 to 16.9; p = 0.01), ST elevation (OR = 5.4, 95% CI 1.4 to 20.5; p = 0.013) and a recent febrile syndrome (OR = 2.8, 95% CI 1.1 to 7.7; p = 0.044). After 12 months' follow-up an increase of EF (basal EF 49.6 (5.1)% vs 12-month EF 59.1 (4.6)%; p<0.001) and decrease of WMSI (basal WMSI 1.19 (0.27) vs 12-month WMSI 1.02 (0.09); p<0.001) were recorded in patients with myopericarditis, with a normalisation of echocardiography, electrocardiography and treadmill testing in 98% of cases. Use of heparin or other anticoagulants (OR = 1.1, 95% CI 0.3 to 3.5; p = 0.918) and myopericarditis (OR = 2.3, 95% CI 0.7 to 7.6; p = 0.187) was not associated with an increased risk of cardiac tamponade or recurrences. CONCLUSIONS: Myopericarditis is relatively common and shows a benign evolution also in spontaneous cases not related to vaccination.

Fixed subaortic stenosis. Natural history of patients with mild obstruction and follow-up of operated patients.

Fifty-five patients, 37 men and 18 women, aged 8 to 71 years (median 23) with a diagnosis of fixed subaortic stenosis were seen in our institution between January 1966 and December 1983. Their subvalvular gradient varied between 0 and 135 mmHg. Aortic regurgitation was present in 44 patients. Thirty-eight patients were operated on. Sixteen patients had some additional cardiac disease, either congenital, or acquired. There were 2 early and 2 late deaths. Fifteen patients were studied again after surgery: the average peak-to-peak gradient has decreased from 88 +/- 28 to 19 +/- 16 mmHg and is zero in 5. No patient has needed a reoperation, so far. Of the 17 non-operated patients, 13 had a gradient of less than 50 mmHg; they are all in Class I-II after 1-5 years. The gradient has increased to 70 mmHg in 1 of the 4 recatheterised cases. Four patients have died, 2 suddenly, 2 of congestive heart failure. They all had a gradient of 55 mmHg or more. In conclusion, in asymptomatic patients of a somewhat "older" age, with a high prevalence of a discrete form of subaortic stenosis, an operation can be deferred if the pressure gradient is less than 50 mmHg, and there are no associated lesions. Identification of patients running the risk of sudden death remains elusive.

Long term results (15-30 years) of surgical repair of aortic coarctation.

The late outcome in 226 patients who survived surgical repair of aortic coarctation was assessed 15-30 years after operation. Twenty six patients died during the follow up mainly from causes related to surgical repair or to associated cardiovascular anomalies. The survival rates of patients operated on between the ages of four and 20 years are 97%, 97%, 92% at 10, 20, and 30 years after operation. For patients operated on after the age of 20 the corresponding rates are 93%, 85%, and 68%. This difference is statistically significant from the fifteenth year of follow up onwards. The survival of patients operated on before the age of 20 is not significantly different from that of a comparable general Italian population. Recoarctation occurred in only 8% of patients who had end to end anastomosis, whereas it occurred in 35% of those who had other types of operation. Two thirds of the patients were hypertensive at the last visit. The actuarial curve shows that blood pressure was normal in most patients 5-10 years after operation, but 30 years after coarctation repair only 32% of patients are expected to be normotensive. Thus early repair of aortic coarctation appears to improve long term survival. Intervention in older patients and when blood pressure is high seem to be the most important predictors of late hypertension.

[Preclinical left ventricular diastolic dysfunction in insulin-dependent diabetes]. / Disfunzione diastolica ventricolare sinistra preclinica nel diabete insulino-dipendente.

BACKGROUND: Isolated alterations of the left ventricular diastolic function have been described in diabetic insulin-dependent patients (IDDM), even in the absence of old age, hypertension, ischemic heart disease, left ventricular hypertrophy. Such alterations have been associated with microangiopathy but it is not known whether it is reversible or if there is a relation with the way the therapy is given. METHODS: Fifty-five subjects have been studied, of which 15 were healthy, 30 recently diagnosed IDDM without microangiopathy and 10 IDDM with microangiopathy. All the patients were under 35 years old and did not present risk factors for coronary artery disease, hypertension or autonomic neuropathy. The maximal exercise stress test proved negative. The diastolic function was studied using the results of Doppler echocardiography of the mitral flow and of isovolumetric relaxation time, with continuous and discrete parameters. RESULTS: The velocity of wave A and E, the relationship between them and their integrals are significantly greater in diabetics with microangiopathy than in those without it and in healthy subjects. There are no significant differences between healthy and diabetic subjects without microangiopathy using continuous parameters. Using discrete parameters diastolic damage is absent in the healthy subjects and is present in 48% of diabetics without microangiopathy and in 90% of those with it. CONCLUSIONS: Slight preclinical diastolic dysfunction is present in young recently diagnosed IDDM without microangiopathy. More severe dysfunction is present when there is also microangiopathy.