MRI evaluation of myocardial viability.

Cardiac magnetic resonance imaging (MRI) has become an accurate noninvasive imaging procedure for the study of postischaemic residual cardiac function, thanks to the evolution of MRI machines, postprocessing software and, above all, sequences. After infarction, and in chronic myocardial ischaemia, the degree of contractile dysfunction is one of the main determinants of longterm survival. The identification and quantification of viable dysfunctional myocardium and the possibility of improving its contractility after revascularisation improves patient prognosis and quality of life. In current clinical practice, myocardial viability is evaluated with stress echocardiography and nuclear methods. Thanks to its intrinsic characteristics and to the delayed-enhancement technique (DE-MRI), MRI has recently emerged as the only noninvasive modality able to provide a three-dimensional (3D) evaluation of cardiac viability with a multiparametric approach.

Cryptogenic stroke in children: possible role of patent foramen ovale.

Stroke is a rare disorder in childhood. The majority of these cases is of an ischemic nature. In spite of the long list of known causes, many strokes remain undetermined, so-called cryptogenic strokes. Increasing evidence indicates that, in the young adults, many cases of cryptogenic stroke are presumably due to paradoxical embolism. In childhood, this is usually not considered, unless in the context of a complex cyanotic heart disease. We present two cases, a 6-year-old boy and an 11-year-old girl who had an episode of cerebral ischemia documented by MRI, and in whom the only anomaly found was a patent foramen ovale (PFO) with intermittent left to right shunt at rest, but with a large right to left shunt during the Valsalva maneuvre. We assumed that, in our cases, in the absence of identifiable causes, the cerebral ischemia was most likely due to paradoxical embolism through the PFO. Of course, as in the adult, the paradoxical embolism could not be proved, but in our opinion it remains the most concrete possibility. Therefore, in presence of a cryptogenic stroke, a PFO should be investigated also in children.

Long-term outcome after senning operation for transposition of the great arteries.

BACKGROUND: The Senning operation for transposition of the great arteries (TGA) was first introduced by Senning in 1959 and was revived by Quaegebeur et al. in the late 1970s, thus becoming the intervention of choice for the correction of TGA in many centers. HYPOTHESIS: The purpose of this study was to evaluate the long-term follow-up of a group of patients undergoing surgery with the Senning procedure for TGA. METHODS: From November 1978 to November 1987, 73 consecutive patients underwent the Senning operation. The 70 survivors had an average follow-up of 19 years (16-25 years). RESULTS: (1) Cardiac rhythm: with time there was a progressive decrease in stable sinus rhythm (60% after 20 years) and a progressive increase of supraventricular tachyarrhythmias requiring therapy (10% after 20 years). (2) Right ventricular function: 20% of the patients had reduced ejection fraction. (3) Late mortality: in the last 12 years of follow-up years there were two sudden deaths (2.8%). (4) Functional status: 80% of patients were in NYHA class I, 17% in class II, and 3% in class III. CONCLUSIONS: Our results confirm that the patients who undergo the Senning procedure have a progressive loss of sinus rhythm, an increase in active arrhythmias, and other important adverse outcomes such as late sudden death and a decrease in right ventricular function; however, most patients (93% in our series) are alive and in good functional status.

Device closure of fenestrated atrial septal defects: use of a single Amplatz atrial septal occluder after balloon atrial septostomy to create a single defect.

Successful transcatheter device closure of secundum atrial septal defect has achieved worldwide acceptance with the clinical use of the Amplatz atrial septal occluder. However, device closure of multiple fenestrated secundum atrial septal defects remains a challenge for the interventionist and in some cases may be only effectively closed surgically. This case report of multiple fenestrated atrial septal defects represents a further expansion of technologic efforts to improve the results and simplify the mechanism of device closure of fenestrated defects. When device closure with more than one device seems impractical or disadvantageous, consolidation of multiple defects in a fenestrated fossa ovalis by simple balloon atrial septostomy as demonstrated by this case report provides an easy mechanism to allow implantation of a single large device which will effectively close all of the defects. This approach is similar to the surgical approach for resection of the fenestrated membrane with secondary patch closure of the enlarged defect. Excellent and complete closure of a fenestrated secundum atrial septal defect is illustrated in this case report.

[New methods of percutaneous closure of patent ductus arteriosus]. / Nuova metodica di chiusura percutanea del dotto arterioso pervio.

Various devices have been developed for the percutaneous closure of persistent patent ductus arteriosus (PDA). In recent years Gianturco coils have become increasingly widespread for this purpose. Poor control during positioning is a major disadvantage of Gianturco coils, with resultant embolization of coils and residual shunts. Very recently a new screwing detachable coil is available (Cook Cardiology, Europe). Between January 1996 and January 2000, 34 consecutive patients with PDA underwent cardiac catheterization. Thirty cases were judged suitable for this procedure, on the base of diameter and morphology of the ductus. Three cases were excluded because of the ductal diameter was too large (> 4.2 mm) and one because of its morphology (type B). In 28 patients a single coil was positioned, while in one case 2 coils were simultaneously used. In 29 cases (97%) complete closure was obtained (in 18 cases immediately, in 6 after 24 hours, in 5 after 1 month). One case showed a residual shunt and a second coil was applied eighteen months later with complete closure. So, considering also this last case, the closure was achieved in 100%. There were neither embolization or other complications. In conclusion, screwing detachable coil for PDA closure is a safe, very effective and at low cost technique. Therefore nowadays this procedure represents the method of choice for occlusion of small PDA (minimal diameter < or = 4 mm).

[Transcatheter closing of patent ductus arteriosus using detachable coils]. / Chiusura transcatetere del dotto arterioso pervio con spirali a distacco controllato.

Various devices have been developed for the percutaneous closure of persistent patent ductus arteriosus (PDA). In recent years Gianturco coils have become increasingly widespread for this purpose. Poor control during positioning is a major disadvantage of Gianturco coils, with resultant embolization of coils and residual shunts. Modifications to improve the positioning of coils have been developed. Very recently a new screwing detachable coil is available (Cook Cardiology, Europe). Between January 1996 and March 1999, 24 consecutive patients with PDA underwent cardiac catheterization. Twenty cases were judged suitable for this procedure, on the base of diameter and morphology of the ductus. Three cases were excluded because of the ductal diameter was too large (> 4.2 mm) and one because of its morphology (type B). In 19 patients a single coil was positioned, while in 1 case 2 coils were simultaneously used. In 19 cases (95%) complete closure was obtained (in 15 cases immediately, in 2 after 24 hours, in 2 after 1 month). One case showed a residual shunt and a second coil was applied eighteen months later with complete closure. So, considering also this last case, the closure was achieved in 100%. There were neither embolization or other complications. In conclusion, screwing detachable coil for PDA closure is a safe, very effective and at low cost technique. Therefore nowadays this procedure represents the method of choice for occlusion of small PDA (minimal diameter < or = 4 mm).

Exercise test in detecting anomalous behaviour of blood pressure in patients successfully operated on for coarctation of the aorta.

Twenty-eight patients operated with success for isolated coarctation of the aorta (i.e. with normal blood pressure and upper/lower limb gradient < or = 20 mmHg at rest) underwent exercise testing to evaluate blood pressure and upper/lower limb pressure gradient during exercise. At maximum effort: 57% (16/28) of the patients were hypertensive and 43% (12/28) of patients increased upper/lower limb gradient over 35 mmHg. No significant correlation was found between the age at surgery (before or after 3 years of age) and maximal systolic blood pressure on exercise and maximal pressure gradient on exercise. The 12 patients with an exercise pressure gradient > 35 mmHg underwent digital angiography or magnetic resonance of the aorta. In 7 cases a mild residual narrowing was found (5 with mild transverse aortic arch hypoplasia, 2 with a mild residual coarctation). In 5 cases no residual narrowing was present. Many factors are thought to be involved in the anomalous behaviour of blood pressure during effort: in some cases anatomic factors, as residual narrowings of the aorta, in other cases functional factors, as increased forearm vascular reactivity, altered baroceptor function, different reactivity and structure of the pre- and post-stenotic aorta, etc., in other cases finally, both factors, anatomic and functional. We conclude that the exercise testing provides the best information on blood pressure modifications during activity and it allows to us to identify those patients which, because of exercise-induced hypertension, remain at risk of premature cardiovascular disease, also after a successful operation. However, when hypertension is found during effort, a strenous physical activity should be avoided and antihypertensive treatment may be required. So the cardiovascular risk due to hypertension can be reduced in the long term follow-up. Corrective surgery for coarctation of the aorta, introduced in 1944, has completely modified the natural history of the disease. Nowadays the operative risk is very low for isolated coarctation and the great majority of the patients is asymptomatic after surgical repair. Nevertheless, their life expectancy is substantially shortened, if compared with the survival curve of a normal population. The vascular and cardiovascular accidents, usually related to residual systemic hypertension, are the most common cause of this. Some studies in the literature have shown that many patients with normal blood pressure and no or little residual upper/lower limb pressure gradient at rest, may develop an anomalous blood pressure response e and/or a high upper/lower limb pressure gradient during exercise. We have studied by exercise test a group of patients successfully operated on for isolated coarctation of the aorta to evaluate the behaviour of the systolic blood pressure and the upper/lower limb pressure gradient during exercise. The aim was to recognize the patients who, inspite of very good operative result, remain at cardiovascular risk in the long-term follow-up.

Early and late postoperative complete heart block in pediatric patients submitted to open-heart surgery for congenital heart disease.

The incidence of complete heart block (CHB) following open-heart surgery for congenital heart disease is about 1%. Most of postoperative CHBs are the consequence of procedures involving the closure of ventricular septal defect; they usually occur immediately after surgery or early in the postoperative period; in few cases they also may occur many months or years after surgery. Early postoperative CHB can be transient or permanent. Permanent pacing is generally not recommended in the former. On the contrary, if CHB persists after at least two weeks of temporary pacing, permanent pacing is needed because the block is usually due to His bundle damage or to trifascicular damage and this is associated with excessive bradycardia and risk of asystole. Late postoperative CHB can be due to the recurrence of previous transient early postoperative CHB or to the progression of postoperative His-Purkinje conduction troubles suggesting trifascicular damage. Permanent pacing is obviously needed in case of documented late postoperative CHB. The prophylactic use of permanent pacing in patients at risk of late postoperative CHB is still a controversial point. Electrophysiologic studies should be performed in such patients. The occurrence of second degree AV block within or below the bundle of His during atrial pacing at rate lower than 200/min can be considered a good marker of impending CHB. In this case prophylactic permanent pacing should be recommended, especially in patients with coexisting problems of troublesome or malignant tachyarrhythmias who have to be treated with antiarrhythmic drug therapy that may favour the progression to CHB.

Congenital pulmonary steal phenomenon associated with tetralogy of Fallot, right aortic arch, and isolation of the left subclavian artery.

Isolation of the left subclavian artery has been reported in cases of tetralogy of Fallot with a right aortic arch. As the isolated left subclavian artery is supplied by the left vertebral artery (in which the blood flow direction is inverted), this anomaly is usually responsible for a congenital subclavian steal phenomenon. In our case the isolated left subclavian artery was connected by a patent ductus arteriosus to the main pulmonary artery. Therefore the left vertebral artery did not supply the distal left subclavian artery but supplied the main pulmonary artery (congenital pulmonary artery steal).

[Defense and promotion of life of children with heart diseases. Nobody's monopoly, everybody's goal and responsibility]. / Difesa e promozione della vita del bambino cardiopatico. Monopolio di nessuno, compito e responsabilità per tutti.

The suffering of children with congenital heart disease, or with other clinical conditions, inquires people and urges us to face and solve many problems. It makes us to reflect on the life's secrets and bring us to consider that every condition can be or became negative if accepted with indifference, inertia, fear or convenience. The clinicians must always fight the disease, not passing by with indifference to those who live and suffer. The physicians, the researchers should not surrender to what is unknown. They must always be looking to the not-yet of the human possibilities, to the not-yet of research. Human problems become difficult to solve it we do not enter in one echosystem of solidarity. The ABC (Association of families with children suffering of congenital heart disease) represents an answer to many problems related to the presence of a heart condition in infants and children.

[Heart transplant: an emotional storm for the child and his family]. / Il trapianto cardiaco: una tempesta emotiva sul bambino e la sua famiglia.

The purpose of the present study was a psychological analysis of the emotional state of a family awaiting heart transplantation. Current practice suggests that such emotional reaction is characterized by mixed and contradictory feelings which require further investigation. Subjects were 5 children waiting for transplant and 5 with previous transplant. Methods were: 1) open interview with children and parents; 2) projective drawing test for children (Corman family test, H.T.P., L.A.D.S., M.U.C.T., K.F.D.); 3) "palinomas" questionnaire which investigates: awareness of the illness, child relation with the food, feelings of competence and control, self-esteem and perception of the self. "Palinomas" was created in Italy by a hospital psychological team in order to give a chance to the child to talk about himself and to maintain control over the situation. The information we obtained by "palinomas" revealed to be very useful to understand the personality of the child through the main areas reported above. Within the limits of an introductory study, results indicate that family needs an active psychological support in order to develop a more positive attitude.

[Cardiac arrhythmias in childhood]. / Le aritmie cardiache nell'infanzia.

The knowledge of various dysrhythmias and their prognostic value permits the selection of appropriate diagnostic and management strategies. The most frequent pediatric dysrhythmias are described: premature atrial and ventricular contractions, supraventricular tachycardias, preexcitation syndrome, long Q-T syndrome, sick sinus syndrome, A-V blocks congenital and acquired. These arrhythmias may be related to structural abnormalities of the conduction system or may reflect temporary cardiac immaturity, as in the newborn, or may occur after surgical repair of a congenital cardiac lesion. It is important decide if the arrhythmias is benign and likely to resolve spontaneously or if it is potentially dangerous. Sometimes an arrhythmias indicates underlying extracardiac disease such as central nervous system disease, sepsis, hypoglycemia, drug toxicity, severe tissue hypoxia, electrolyte abnormalities. In these instances the treatment of the underlying cause will correct the rhythm abnormalities.

[Percutaneous dilatation of pulmonary valve stenosis]. / Dilatazione percutanea delle stenosi valvolari polmonari.

We present 11 cases of pulmonary valve stenosis who underwent percutaneous dilatation angioplasty. Patients were between 2 and 15 years old (mean age = 6.5 yrs) and all of them except 3 had moderate or severe degree of valve stenosis. Pulmonary anulus diameter ranged between 11 and 23 mm. at ECHO and between 10 and 21 mm. at angio. The catheter was chosen to be 20-40% larger than pulmonary anulus. The mean pressure gradient between RV and PA was 65 mmHg. before dilatation and 21 mmHg. after the procedure. The procedures were without complications, except one in which we observed an intermittent idioventricular rhythm for few days after the procedure. Percutaneous dilatation angioplasty has to be considered the treatment of choice for pulmonary valve stenosis.

[Persistent supraventricular tachycardia in childhood]. / Le tachicardie sopraventricolari persistenti in età pediatrica.

Persistent or chronic supraventricular tachycardia is defined as the one occurring in more than 10% of cardiac rhythm during 24 hours. It may begin at any age, persisting months or years, and frequently it is discovered occasionally because it can be asymptomatic. The persistent supraventricular tachycardia (PSVT) represents 20-30% of all supraventricular tachycardias in pediatric age. The two main forms of PSVT are: atrial ectopic tachycardia, and persistent junctional reciprocating tachycardia (PJRT). The authors present 9 cases of children affected by PJRT seen from 1972 to 1985. They discuss the clinical course (mean follow up = 9.5 years), the pharmacologic treatment, the results obtained. They underline the particular resistance to conventional antiarrhythmic therapy of this form. On the other hand an antiarrhythmic treatment is recommended to avoid the risk of congestive heart failure. Nevertheless the prognosis is considered benign because in the majority of cases this type of arrhythmia disappears later on.

[Cardiomyopathy in Friedreich's ataxia]. / La cardiomiopatia nella atassia di Friedreich.

Friedreich's ataxia (FA) is an hereditary degenerative disease involving the spino-cerebellar via which in 10-15% of the cases is associated with symptomatic cardiac disease. Abnormal ECG or ECHO finding are present even in absence of cardiac symptoms in 100% of the patients. A 7 y.o. girl with the clinical picture of FA with cardiac involvement is presented. The features of the cardiomyopathy present in FA studied with ECHO, myocardial perfusion with Thallium 201 and with histologic examination and its relationship with the hypertrophic cardiomyopathy are discussed. Different theories aiming to discover a unique biochemical factor responsible of both the neurological and cardiac disorders are presented.