Isolated congenital left ventricular diverticulum.

Two cases of isolated left ventricular diverticulum are presented. A 12-year-old boy and a 7-year-old girl, both asymptomatic, were admitted to our Department for evaluation of an abnormal electrocardiogram and an abnormal cardiac silhouette, respectively. Both patients had the diagnosis confirmed by cardiac catheterization and angiography and underwent successful surgical correction of the abnormality. We discuss the diagnosis, prognosis, and surgical treatment of this entity and we advocate early surgical treatment for all diagnosed ventricular diverticula, even if asymptomatic.

Late reoperations after repair of tetralogy of Fallot.

Twenty-two patients underwent 23 late reoperations after total correction of tetralogy of Fallot from 1965 to 1990. Indications for reoperation included: isolated ventricular septal defect (VSD) in 9 patients (41%), isolated right ventricular outflow tract (RVOT) obstruction in 3 patients (13.7%), VSD associated with a RVOT obstruction in 7 patients (31.8%), aneurysm of the pericardial RVOT patch in 1 patient (4.5%), aortic insufficiency with a residual VSD in 1 patient (4.5%), and tricuspid regurgitation in 1 patient (4.5%). The reoperation consisted of closure of a residual VSD in 17 patients, relief of a RVOT gradient in 11, insertion of a RVOT valve in 4, tricuspid valve replacement in 1 (reoperated twice), aortic valve replacement in 1, and excision of a RVOT aneurysm in 1. Two patients died in hospital (9%) but there were no early deaths in the 11 patients reoperated upon after 1978. Mean follow-up period was 135 months. There were 2 late deaths. The actuarial 20-year survival was 87%. Of the surviving patients, 16 (89%) were in New York Heart Association class I, 1 (5.5%) was in class II, and one (5.5%) was in class III. One patient required a second reoperation for tricuspid bioprosthesis degeneration and 1 patient had moderate recurrent RVOT gradient due to calcified pulmonary bioprosthesis. This study tends to support the policy of recommending reoperation in the presence of surgically significant residual defects. Reoperation is associated with a low early mortality and good long-term results.

[Immediate and late arrhythmia in patients operated on for tetralogy of Fallot]. / Aritmie immediate e a lungo termine nei pazienti operati per tetralogia di Fallot.

UNLABELLED: Arrhythmias are a frequent complication after repair of tetralogy of Fallot (TOF). We present our experience with 97 patients with special consideration for early and late hyperkynetic arrhythmias. The most frequent, 4% of the patients, was in junctional tachycardia. Late arrhythmias can be atrial or ventricular. The incidence of ventricular arrhythmias in the literature range from 42 to 82%. In the Authors experience multiforme ventricular ectopy was present in 80% of the patients, 20 years after surgery. We suggest an exercise test and averaging ECG in all the patients. Thirty six percent of patients with supraventricular tachycardia were symptomatic. CONCLUSION: as the incidence of arrhythmias after correction of TOF is high, it is very important to periodically reassess these patients. Antiarrhythmic treatment is indicated in all symptomatic patients, especially in those with major arrhythmias (SVT, AF and VT).

[Influence of left anterior hemiblock on electrocardiographic indices of left ventricular hypertrophy (author's transl)]. / Influenza dell'emiblocco anteriore sinistro sui criteri elettrocardiografici di diagnosi di ipertrofia ventricolare sinistra

The influence of left anterior hemiblock (LAH) on some electrocardiographic criteria of left ventricular hypertrophy (LVH) was investigated. 131 subjects were studied: 34 of them were normal (control group); 30 were carriers of isolated LAH; 38 were carriers of isolated LVH; 29 showed a pattern of combined LVH and LAH. The maximal voltages of QRS waves in leads usually analyzed for LVH, the QRS duration and the time of onset of the intrinsicoid deflection were compared among the groups. The specificity, the sensitivity and the performance of some LVH criteria were examined as well. LAH has been shown to conceal LVH on precordial leads, while it tends to make LVH more manifest on limb leads. The Romhilt and Estes' point score system proved to be the least affected by LAH. On the grounds of theoretical considerations and of empirical trials, we suggest modifying the Romhilt and Estes' point score system; furthermore we suggest modifying Sokolow's and Grant's criteria only when LAH is present. Such modified criteria have improved the accuracy of LVH prevision.

The use of propafenone in the treatment of tachyarrhythmias in children.

Propafenone was given to 60 children (mean age 4.5 years) with paroxysmal re-entrant supraventricular tachycardia (rSVT: 41 cases), postoperative automatic junctional tachycardia (JET: eight cases), automatic atrial tachycardia (AT:four cases), ventricular tachycardia (VT:four cases) and atrial flutter (AF: three cases). In acute cases (29) propafenone was administered intravenously (mean dose 1.3 +/- 0.5 mg.kg-1) chronic oral treatment (mean dose of either 11 +/- 3.3 mg.kg.day-1 or 265 +/- 78 mg.m-2 x day-1) was given to 48 children, in 2-3 divided doses. Overall efficacy was 76% for acute and 69% for chronic treatment, with best results in paroxysmal rSVT. It was effective in 89% acutely of those treated acutely and in 69% of those administered chronically. The efficacy of propafenone in the prevention of rSVT was strictly related to the incidence of attacks before treatment:propafenone was indeed inefficacious in 6/11, 6/14 and 0/12 of patients with daily, weekly and monthly attacks respectively. Side effects were observed in 25% of patients: 6% required suspension of therapy due to pro-arrhythmic effects in one patient on intravenous administration, peripheral neuropathy in one case and hypotension in two postoperative JET patients. In conclusion, its efficacy and limited side effects make propafenone a relatively safe and very useful drug in the treatment of various tachyarrhythmias. However, risk of unpredictable toxic levels requires careful use of the drug especially in the first days of treatment, when a daily monitoring of ECG is strongly recommended.

[Comparative evaluation of the isometric test and of dynamic exercise in the diagnosis of coronary insufficiency (author's transl)]. / Valutazione comparativa dello sforzo isometrico e del lavoro dinamico nella diagnosi dell'insufficienza coronarica

Fifty patients with exertional coronary insufficiency performed an exercise test on a bicycle-ergometer and an isometric exercise with an instrument set up by the authors with the collaboration of the technical laboratory of the hospital. All cases had EKG positivity during dynamic exercise and only 2 patients had a positive EKG answer during isometric exercise. Angina occured in 70% of cases during dynamic exercise and in 12% of cases during isometric exercise. The difference in the increasing heart rate of the two types of exercise was statistically significant, as was the difference between absolute values of heart rate reached at the end of the exercise. The difference in the values of arterial pressure reached in the two types of exercise was also statistically significant. Moreover the comparison of product heart rate, time arterial pressure, time 10(-2) bordered on statistical significance. 31 of the 50 patients who underwent coronary angiography, demonstrated severe impairment. During isometric exercise a higher percentage of disturbances was observed. The authors came to the conclusion that isometric exercise is not advisable for the diagnosis of coronary insufficiency, either for the low specificity or the potential risk.

Clinical and electrophysiologic evolution of the Wolff-Parkinson-White syndrome in children: impact on approaches to management.

There is less certainty about the recommendations for radio-frequency ablation as a therapeutic option for Wolff-Parkinson-White syndrome in children as opposed to adults because of the different natural history and the age-related risks of ablation. To help decision-making, we evaluated the long term clinical evolution and electrophysiologic characteristics of pre-excitation in our series of children and young adults. We reviewed the clinical course of 109 patients below the age of 18 years who had been followed up over a period of 9+4 years, with a range from one to 27 years. This corresponded to 986 patient-years. We examined also the electrophysiologic data from 98 of those patients who underwent a transesophageal study. At the discovery of pre-excitation, 59% of patients were asymptomatic, while 29% developed supraventricular tachycardia during follow-up. The peak incidence of the onset of supraventricular tachycardia occurred during infancy. These patients had the highest incidence of subsequent spontaneous disappearance of the tachycardia (53%), such a favourable evolution being encountered in only 12% of patients in whom the abnormal rhythm first appeared after 12 years of age. Chronic medical treatment was required in 47% of patients, and proved completely effective in 45% of cases. The potential to induce atrial fibrillation, and the incidence pre-excitation considered to be high risk, with the shortest pre-excited RR interval equal to or less than 220 msec, was lowest in the group of patients aged less than 6 years of age, and highest in those older than 12 years of age (p <0.001). Pathways producing arrhythmia with high risk were more common in symptomatic (29%) than in asymptomatic patients (7%) (p<0.001). No mortality occurred. On the basis of our findings, we suggest that ablation should be avoided before the age of 5 or 6 years. Thereafter, the procedure should become the first line of treatment for symptomatic patients older than 12 years of age.

[Defects of the interatrial septum: surgical correction without the hemodynamic test. Analysis of 145 cases operated on]. / Difetti del setto interatriale: correzione chirurgica senza esame emodinamico. Analisi su 145 casi operati.

Surgical correction of ostium secundum atrial septal defects may be performed, in most cases, without cardiac catheterization, on the ground of clinical and two-dimensional echocardiographic (2D echo) findings. Consequently it's useful to identify the reliability of 2D echo and the indications for angiography in these patients (pts). One hundred forty-five patients operated in "A. De Gasperis" Division of Cardiac Surgery in Milan from January 1982 to December 1986 are reviewed. Patients with ostium primum atrial septal defects or subjected to cardiac catheterization and angiography in other institutions are excluded. Altogether two-dimensional echocardiography was performed in 131 pts, cardiac catheterization in 78. Sixty-four pts were studied with both 2D echo and angiography. In 35 pts (with only 2D echo examination) radionuclide angiocardiography was performed. The results of the different techniques were compared with intra-operative findings, defining sensitivity and specificity in relation to detection of the site of the defect and of the pulmonary venous return. There were no statistically significative differences between echo 2D and cardiac catheterization (Figs. 1 and 2). Two-dimensional echocardiography confirms its cost effective and psychological advantages, especially in younger patients. Hemodynamic and angiographic assessment should be performed as follows: 1) when associated anomalies are suspected; 2) in cases of non-satisfying quality of the 2D echo examination; 3) when diagnostic incoherence between clinical and instrumental data is present; 4) in patients with pulmonary hypertension.

[Complications and sequelae of cardiac electrostimulation in children. Our experience with 47 children]. / Complicanze e sequele dell'elettrostimolazione cardiaca in età pediatrica. Nostra esperienza in 47 bambini.

Permanent cardiac pacing is now easily feasible in children and even in small infants, but the long-term results of this procedure are not well known. We analyzed our experience to determine the morbidity of pacing in children. Over the past 10 years, 47 pediatric patients (pts) required pacemaker implantation in our institution. The mean age was 8.3 +/- 4 years (1 day-17 years) and mean body weight was 23 +/- 14 Kg (2.2-60 Kg). 25 pts had heart disease. 40 children had an A-V block (congenital in 22 cases, post-operative in 17 pts, and secondary to a systemic disease in 1 case); 7 pts had a sick sinus syndrome, primitive in 4 and postoperative in 3 cases. The first pacemaker implantation was epicardial in 17 and transvenous in 30 pts. The pacing was single-chamber in 45 pts (VVI 32, VVIr 7, AAI 5, AAIr 1) and dual-chamber in 2 pts (DDD 1, VDD 1). Two newborns, both with a congenital A-V block and severe heart failure, died in the first hours after epicardial pacing. Two other children, both with congenital heart disease, died during follow-up, but the death was not pacemaker-related. Finally, two children were lost to follow-up. The mean follow-up of the 41 remaining pts was 5.2 +/- 3.5 years (4 months-10 years). Twelve children (29%) required 19 implant revisions and the causes were: lead fracture (26%), rising stimulation threshold (26%), growth problems (21%), erosion and/or pocket infection (21%). Revisions were more common in epicardial (52%) than in endocardial (22%) implantation.(ABSTRACT TRUNCATED AT 250 WORDS)

Transoesophageal electrophysiological evaluation of paediatric patients with Wolff-Parkinson-White syndrome.

Forty-one consecutive children with the Wolff-Parkinson-White (1-15 years: mean 8 years), 22 symptomatic [including 20 with supraventricular tachycardia (SVT) and one with ventricular fibrillation (VF) episode--Group A) and 19 asymptomatic (Group B)], underwent transoesophageal electrophysiological examination. The mean anterograde effective refractory period of the anomalous connection (AERPAC) was 249 +/- 34 ms with no significant difference between the two groups. However, values of less than 220 ms were more frequently observed in Group A than in Group B (23% vs 11% Pns). The effective atrial refractory period was significantly shorter in Group A (183 +/- 34 ms) than in Group B (220 +/- 24 ms) (P less than 0.02). The refractory period of the AV node was 205 +/- 41 ms in Group A and 244 +/- 63 ms in Group B. Orthodromic SVT was induced exclusively in 75% of Group A patients. Atrial fibrillation (AF) was induced in a total of 46% of patients (45% of Group A, 47% of Group B) and was considered easily induced in 37%. The minimum RR interval between pre-excited beats was significantly shorter in Group A (221 +/- 23 ms) than in Group B (321 +/- 143 ms) (P less than 0.04). Four of the patients in whom AF was easily inducible had an RR interval less than 250 ms; three were symptomatic (one with VF). While the AERPAC is generally short in children, and atrial vulnerability generally slight, 9% of children with a short AERPAC had high atrial vulnerability, thus placing them at risk for life-threatening arrhythmias.

[Idiopathic recurrent ventricular tachycardia in children: characteristics and long-term prognosis]. / La tachicardia ventricolare idiopatica incessante nel bambino: sue caratteristiche e prognosi a distanza.

BACKGROUND: The aim of this study was to assess the prognosis and the clinical course of incessant idiopathic ventricular tachycardia (VT) with left bundle branch morphology, in a group of 14 children consecutively evaluated in our institutions between 1983 and 1994. METHODS: Mean age of these patients was 8 +/- 2 years, and they were followed for 56 +/- 32 months. In all the children, VT was present for more than 80% of the day, in non sustained runs. The mean rate of VT was of 142 +/- 30 beats/min. Four children were symptomatic. The evaluation of these children was obtained by treadmill stress test (12/14), cardiac catheterization (13/14), electrophysiologic study (10/14), nuclear magnetic resonance (7/14), and serial echocardiographic and Holter examinations. RESULTS: Right ventricular dysplasia was suspected in 2 cases on the basis of nuclear magnetic resonance and angiographic data. In both these cases VT was not suppressed by exercise. Medical treatment was started in 9/14 patients (69%), it was completely effective in 4 (44%) and partially effective in 2 further cases (22%). Thirteen out of fourteen patients had a regular follow-up. No patient died. All patients remained asymptomatic and in 6 (46%) VT disappeared. In 4 of these last 6 patients therapy has been previously effective. Two children, one with right ventricle dysplasia, continue antiarrhythmic treatment. CONCLUSIONS: Incessant VT is well tolerated in pediatric age. The frequency of spontaneous remissions is relatively high (46% of cases). In few patients this arrhythmia is associated with evident cardiac abnormalities. In our opinion, medical treatment is necessary in symptomatic patients and in those with heart abnormalities.

[Amiodarone therapy in childhood: efficacy and side effects]. / La terapia con amiodarone in età pediatrica: efficacia ed effetti collaterali.

In the adult, Amiodarone is a very effective drug in the treatment of ventricular and supraventricular arrhythmias. The presence of severe side effects such as some alterations in the thyroid function and/or pulmonary fibrosis have restricted the use in children. Nevertheless, research has shown that there is a low incidence of collateral side effects and this therapy in infancy can be very effective. For this reason we evaluated a group of 27 children with supraventricular (19 patients) and ventricular (8 patients) arrhythmias. The mean age of patients treated was 6 +/- 5 years (2 days-13 years). The follow-up period was of 13 +/- 10 months. Amiodarone has been used in 9 patients intravenously, with the loading dose of 5 mg/Kg followed by an infusion of 10 mg/Kg/day. In 18 patients we administered the drug orally with a loading dose of 10 mg/Kg/day for a period of 10 days, thereafter the maintenance was of 5-7 mg/Kg/day for 5 days every week. The patients were all checked for thyroid function and Holter monitoring quarterly; they were given an ophthalmologic examination (every 6 months) and a chest-x-ray and echocardiography annually. The efficacy of intravenous treatment was judged successful in 56% of patients, partially successful in 22% and ineffective in the remaining 22%. The oral treatment was completely effective in 77% of children, partially in 5% and ineffective in 18%. In one case we had to suspend the therapy because we found high values of T3 and T4. During the treatment, in 86% of cases, we had blood level fluctuations of T3 and T4, however these did not exceed the normal ranges. The most important side effect observed has been the photosensitivity found in 22% of children. Moreover we observed a reduction of sinusal automatism, which was more marked in patients less than year old. In 4 cases an A/V block of first degree appeared. In all patients we found changes of ventricular repolarization, while corneal deposits appeared in only one child after a year of therapy and did not cause an impairment of visual acuity. In conclusion we can assert that Amiodarone is a very effective drug in children, specially in small babies, where it can safety be used as a first choice drug.

[Factors limiting adaptation to exercise after the Fontan operation: role of altered pulmonary function]. / Fattori limitanti l'adattamento allo sforzo dopo intervento di Fontan: ruolo di eventuali anomalie polmonari.

BACKGROUNDS: The abnormal adaptation to exercise after Fontan operation, is primarily due to a suboptimal increase in cardiac output. We postulated that abnormal pulmonary function could cooperate in reducing effort tolerance of these patients (pts). METHODS: We have evaluated 10 pts, mean age 15 +/- 8 yrs, with a mean postoperative follow-up of 6 +/- 2 yrs. Seven pts were asymptomatic while 3 had mildly decreased effort tolerance. These pts were evaluated with echocardiogram, cycle ergometer stress test, basal and effort spirometry and perfusion lungs scanning. RESULTS: Basal ejection fraction was normal in 9 pts and mildly depressed in 1 pt. Basal cardiac index was 1.8 + 2.2 l/min/m2 and increased to 4.8 + 5 l/min/m2 during exercise. Effort tolerance was 73% +/- 21% of predicted values, with maximal O2 consumption of 60% +/- 13% of predicted. Basal spirometry showed a mild restrictive pattern in 7 pts with a decrease in total pulmonary capacity and in forced vital capacity (mean values respectively 79% +/- 14% and 75% +/- 18% of predicted), otherwise respiratory reserve resulted normal in all pts. Carbon monoxide diffusion was impaired in all pts with a mean value of 49% +/- 10% of predicted. O2 saturation near normal in basal condition decrease during exercise (mean value 96% +/- 2% and 91% +/- 7%). The VE/VO2 and Vd/Vt ratio were both abnormally increased (respectively 111% +/- 28% and 186% +/- 39% of predicted). Lung scanning was abnormal in 6 pts with defects of perfusion localized prevalently in left lung (5/6) especially in upper lobe (4/6). CONCLUSIONS: After Fontan operation exercise tolerance is reduced as consequence of 1) a suboptimal increase in cardiac output and 2) abnormal gas diffusion probably due to a damage of alveolo-capillary membrane and/or to a ventilation/perfusion mismatch as showed by abnormalities in lungs perfusion and by increase Vd/Vt ratio.

[Fontan's operation: results, immediate and long-term postoperative complications]. / Intervento di Fontan. Risultati, complicanze postoperatorie e a distanza.

The Fontan operation and its modifications can be relatively safely performed in the treatment of many complex congenital heart diseases, such as univentricular heart and tricuspid atresia. The main postoperative complications following the Fontan procedure and the incremental risk factors involved in their development are investigated. Between January 1984 and January 1988 eleven patients (6 females and 5 males), ranging in age from 2 to 15 years (mean age: 7.3 +/- 3.7) and in weight from 10.8 to 50 Kg (mean weight 22.3 +/- 12.7) underwent the Fontan operation in our Department. No hospital death occurred. The mean postoperative stay in the Intensive Care Unit was 6.3 +/- 3.9 days. There were two surgical re-entries for the same patient: cardiac tamponade (the day of operation) and residual atrial septal defect (2 days following the operation). Eight patients had significant signs of venous stasis, with severe hepatomegaly and pleural effusion. The 11 patients discharged were followed-up for a period of between 3 and 48 months (mean follow-up: 26.4 +/- 18). There were 2 cases of recurrent pleural effusion, 10 to 15 days after discharge. One late death occurred 2 years after the Fontan procedure (massive pulmonary embolism after re-operation). Short and medium-term rate of complications is related to increased post-operative values of mean right atrial pressure (greater than 15 mmHg). Mean right atrial pressures greater than 15 mmHg appear to be correlated with Nakata index values less than 250 mm2/m2. The experience reviewed confirms the excellent results of the Fontan operation, as regards survival and functional recovery.(ABSTRACT TRUNCATED AT 250 WORDS)

[Mid-term clinical condition and prognosis in adrenergic ventricular tachycardia in children with apparently normal hearts]. / Clinica e prognosi a medio termine delle tachicardie ventricolari adrenergiche in bambini con cuore apparentemente sano.

INTRODUCTION: Ventricular tachycardia is the most common diagnosis among children with exercise or emotion-related syncope. The aim of the study was to assess the prognosis and the clinical course of children, without long QT syndrome, with exercise-related ventricular tachycardia and apparently normal heart. METHODS: Since January 1984, we studied 14 children (mean age 9.7 +/- 3.8 years) with exercise-related ventricular tachycardia, and, at the initial detection, without demonstrable heart disease, as assessed by clinical evaluation, electrocardiogram and mono-bidimensional echocardiogram. Six patients had exercise related syncope, 1 exercise intolerance and 7 were asymptomatic. All patients underwent exercise testing on treadmill and 24-hour ECG monitoring. All patients were treated with antiarrhythmic drugs. All patients were tested by exercise testing and 24 hour ECG monitoring, alternatively, every six months, during therapy or after complete wash-out of the drug used. RESULTS: Ventricular tachycardia, detected by exercise testing and Holter monitoring, was polymorphic in 4 symptomatic patients, monomorphic with left bundle branch block morphology and inferior axis in the others. The mean rate of monomorphic ventricular tachycardia was 224 +/- 35 bpm in the symptomatic children and 180 +/- 33 bpm in the others (p = 0.03). Successful chronic treatment was achieved with beta-blockers in 5 cases, propafenone in 6, sotalol in 2 and flecainide plus metoprolol in 1. During follow-up (6 +/- 2.7 years), 1 patient with polymorphic ventricular tachycardia, on therapy, suddenly died during exercise, the other symptomatic patients had no variation of their arrhythmia on routine tests, except 1 who, after propafenone was stopped, had ventricular fibrillation during exercise testing. Among the 7 asymptomatic patients, 5 had no evidence of ventricular tachycardia on routine test after 2 +/- 3 years; the others had no variation of their treatment. CONCLUSION: Exercise related ventricular tachycardia in children seems: 1) to have more severe prognosis if symptomatic or polymorphic; 2) mostly to arise from right ventricle; 3) to be successfully treated by antiarrhythmic drugs with beta-blocking activity.

[The incomplete atrio-ventricular canal. Operative and long term results in 100 cases (author's transl)]. / Il canale atrio-ventricolare incompleto. Risultati immediati e a distanza di 100 casi operati.

100 patients with endocardial cushion defect of the partial type underwent surgical correction between the years 1957-1975. The age of patients ranged from 3 to 57 years with a mean of 18 years. In 89 patients there was a mitral insufficiency. The repair of the mitral valve was performed in 78 patients; in 21 was repaired also the tricuspidal valve. The hospital mortality was of 8 patients. The mortality was correlated with the age of the patients, the pulmonary pressure and with the presence of malformations of the tricuspid valve. A major complication was represented by a complete atrioventricular block that occurred in 8 patients, in 5 of these the block disappeared before the dimission from the hospital. 86 patients have been followed for a period variable from 2 to 228 months and an average of 61 months. The late mortality was of 4 patients. Two patients have been reoperated of mitral valve replacement because of residual important mitral insufficiency. The phonocardiographic study, done in 50 patients, showed a presence of a holosystolic murmur in 20. The standard X-ray chest and electrocardiogram iid not show important changes between the pre and post operative examination.

[beta-Blocking therapy in obstructive hypertrophic cardiomyopathy. Long term results (author's transl)]. / Risultati a distanza della terapia betabloccante nella cardiomiopatia ipertrofica ostruttiva.

34 patients have been controlled after beta-blocking therapy, for a mean period of 5 years. Symptoms and evolution: syncope disappeared, angoy passed from 47% to 23%, dyspnea from 65% to 47%, dizziness from 70% to 54%, weakness from 30% to 37%. A systolic murmur was present in 75% of the cases. Two patients died by heart failure. Phonocardiogram: the systolic murmur was unchanged, like the carotid pulse. Paradoxical splitting of the 2 degrees sound was more frequent, atrial sound unimodified, isometric contraction shortened (60%) and the Q-1 degree sound interval prolonged (90%). Electrocardiogram: 1 degree A/V block appeared in 24% of the cases, complete A/V block in 9%, atrial fibrillation in 3%. Left atrial enlargement was more frequent; left ventricular hypertrophy unchanged. Heart catheterization (10 cases, after a mean period of 5.5 years): left ventricular pressure gradient passed from 80% to 90%; a low cardiac index from 20% to 30%; telediastolic pressure of left ventricle was unmmodified in 10% of cases, more elevated in 50%, less elevated in 40%. Chest X ray: cardiac size was unchanged in 65% of cases, enlarged in 32%; smaller in 3%. In conclusion, symptoms improved in most of the patients; no case of sudden death was observed. Some data however show that the evolution of the myocardiopathy goes on to congestive heart failure and arise doubts on the real usefullness of beta-blocking drugs in the disease.