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.

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).

[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.

[Abnormal left pulmonary artery: a cause of severe newborn cardiorespiratory insufficiency]. / Arteria polmonare sinistra aberrante: una causa di insufficienza cardiorespiratoria neonatale grave.

Aberrant left pulmonary artery (vascular sling) indicates the vascular anomaly in where the left pulmonary artery arises from the right pulmonary artery, passes over the right mainstem bronchus, loops to the left and courses between the trachea and esophagus to the left hilus. Almost invariably respiratory symptoms result from compression of the trachea and right mainstem bronchus by the anomalous vessel as well as by associated tracheobronchial defects. A case is reported. The distinctive radiologic features include anterior indentation of the barium esophagogram and a characteristic pulmonary angiogram. Surgery is the only resolutive treatment for symptomatic patients, but results are often poor owing to associated intracardiac defects and intrinsic tracheobronchial anomalies.

[Hepatic hemangiomatosis with congestive cardiac failure and development into a cholostatic hepatopathy]. / Emangiomatosi epatica con scompenso cardiaco congestizio ed evoluzione in epatopatia colostatica.

The authors describe a case of hepatic hemangiomathosis in a three months old infant with a clinical picture of multiple cutaneous hemangioma, epatomegaly and severe cardiac failure. The cardiac catheterization with selective angiography showed extensive communication between the mammary artery and the hepatic circulation with the presence of large bloody areas into the markedly enlarged liver. The medical treatment with digitalis, diuretics, and corticosteroids obtained rapid resolution of the cardiac failure and of the A-V fistula signs. A clinical picture of severe liver cholostatic disease developed few months later which was confirmed by histological examination. The subsequent course has been favorable with complete clinical resolution of the liver disease.

[Epidemiological study of congenital heart diseases in the province of Parma during the years 1972-80 (author's transl)]. / Studio epidemiologico delle cardiopatie congenite nei nati in Parma e provincia negli anni 1972-80.

We have carried out an epidemiological-statistical study about congenital heart disease on 33.245 live-born infants in Parma from 1972 to 1980. 327 newborns with congenital heart disease were found; the incidence was 9,8/1000 live-born. The lowest value was 5,8/1000 live-born in 1972, the highest was 14,5/1000 live-born in 1977. In the majority of cases diagnosis was only clinical VSD, PDA, ecc.), whereas in the more complex cardiac malformations and in patients with severe congestive heart failure diagnosis was confirmed at cardiac catheterization and at surgery. In 60 cases diagnosis was confirmed also at autopsy. The congenital heart disease most frequently observed was VSD (33%), followed by PDA (13,4%) and TGA (8,8%), ecc. Clinical status at 1 year of age in all patients was evaluated. The Authors think that infant cardiac program is necessary to improve the results on congenital heart disease control.

[Primary pulmonary hypertension in childhood: familial aspects]. / Ipertensione polmonare primitiva (I.P.P.) nell'infanzia: familiarità.

Primary pulmonary hypertension (PPH) is a rare, progressive and usually fatal disease. Its pathogenesis remains obscure. Probably multiple genetic and environmental factors play a role in the occurrence of PPH. PPH sometimes runs in families. We have studied two families in which some members were affected: in family A two sibs, their father and probably their paternal grandmother, in family B two sibs. When PPH is present in more than one generation (family A), an autosomal dominant mode of inheritance would be likely, whereas, when the disease is present in only one generation (family B), an autosomal recessive mode of inheritance is more probable. Treatment of PPH is based on pulmonary vasodilator drugs, because one of the most important mechanisms is thought to be an inappropriate vasoconstriction. Some drugs like oxygen, tolazoline, acetylcholine, isoproterenol seem to get poor results, while phentolamine, nifedipine, hydralazine and diazoxide show promise. Diazoxide appears to have some advantages because it reduces not only pulmonary arteriolar resistance, but also pulmonary artery pressure, without producing tachycardia.

[Hemodynamic study of pulmonary vascular resistances in subjects with Down's syndrome with atrioventricular canal]. / Studio emodinamico delle resistenze vascolari polmonari in soggetti Down con canale atrioventricolare.

Down patients frequently present congenital heart disease (CHD). Among these the most common is complete atrioventricular canal (CAV). The Authors studied by cardiac catheterization 2 groups of subjects with CAV:21 Down-patients, aged 5-48 months (mean = 24.7) and 17 non-Down patients aged 6-50 months (mean = 23.1). Hemodynamic data were compared and in particular pulmonary vascular resistances (PVR) were analysed. Mean pulmonary resistance was 11.1 UM2 for Down children and 4.6 UM2 for non-Down children. Considering the subjects under 2 years of age, PVR greater than or equal to 10 UM2 was present in 5 of 14 (35.7%). Down children, while none in the non-Down group. The cause of this behaviour is unknown; some Authors, in pathological studies, found in Down-patients primitive structural anomalies of the vascular bed with decrease of total vascular surface area. This fact might explain higher values of PVR in Down-patients than in non-Down patients.