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Infantile hemangiomas (IH) complicated by ulceration, disfigurement, functional impairment or life-threatening conditions need early, safe and effective treatment. This study explores the impact of propranolol on complicated IH. We report our experience of 62 patients treated with oral propranolol for complicated IH. The effect of propranolol was assessed using a score on a visual analogue scale integrated with echo, magnetic resonance or endoscopic findings. The average age at the beginning of the treatment was seven months [standard deviation (SD)±8.9], with a median of four months (range 1-53 months). The average age at the end of the treatment was 15 months (SD±8.4), with a median of 13 months (range 7-59 months). The mean treatment length was eight months (SD±3.2). Oral propranolol was successful in 95.2% of the patients in reducing the volume, the intensity of color and the elevation of IH. Statistically significant improvement of IH volume was observed in the first two months of therapy (P≤0.001), and between the second month and the end of the treatment (P

OBJECTIVE To describe a multicenter experience with patent foramen ovale (PFO) and atrial septal defect (ASD) device closure for presumed paradoxical emboli in children and young adults ( PATIENTS AND METHODS Medical records were reviewed of patients who had device closure of an ASD or PFO, who were younger than 35 years, and who had a history of presumed paradoxical embolus between January 1999 and August 2005 at Mayo Clinic, Rochester, Minn, University of Parma, Parma, Italy, and Loyola University Medical Center, Maywood, Ill. RESULTS Forty-five patients fulfilled the inclusion criteria. Median patient age was 29.0 years (range, 5.0-34.9 years), and 23 patients (51%) were male. Clinical diagnoses included the following: stroke, 30 (67%); transient ischemic attack, 13 (29%); myocardial infarction, 1 (2%); and renal infarct, 1 (2%). Overall, 42 patients (93%) had a PFO, and 3 (7%) had an ASD. Seventeen patients had known cardiovascular disease risk factors: tobacco use (10 patients), hypercoagulable states (7 patients), systemic hypertension (3 patients), and hyperlipidemia (2 patients). No major procedural complications occurred. Median follow-up evaluation was performed at 5.3 months (range, 2.5-40.0 months). Forty-four patients (98%) had no recurrent neurologic events and no residual atrial shunt by contrast transthoracic echocardiography. CONCLUSIONS Cryptogenic ischemic events occur in young patients and have serious sequelae. The potential for paradoxical embolization through a PFO or an ASD should be assessed in all such patients. In our short-term follow-up, device closure was a safe alternative therapeutic option for children and young adults with presumed paradoxical emboli.

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.

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.

We report an 8-year-old girl who presented with clinical features of an acute myocardial infarction. The angiographic appearance of the coronary arteries was normal. A thrombophilic state caused by a homozygote genotype for the prothrombin G20210A mutation was detected, and a patent foramen ovale (PFO) with right-to-left shunting after Valsalva maneuver was demonstrated by transesophageal contrast echocardiography. No other embolic source was identified. We suggest that paradoxical embolization through a PFO resulted in a myocardial infarction in this young patient with hereditary thrombophilia. We closed the patient’s PFO with a 25-mm PFO occluder. She was anticoagulated with warfarin for 6 months. After 6 months, a contrast echocardiogram showed no evidence of residual atrial shunt. There has been no evident recurrent paradoxical embolization.

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.

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).

Severe pallid breath-holding spells (BHSs) are based on parasympathetic hyperactivity, leading to cardiac asystole, pallor, brain ischemia, loss of consciousness, and reflex anoxic seizures. In recent years, an increasing number of patients with severe pallid BHSs have been successfully treated with pacemaker implantation. We present the case of a 13-month-old girl suffering from repeated severe pallid BHSs, causing asystole, loss of consciousness, and generalized anoxic seizures. She underwent treatment with oral glycopyrrolate, an anticholinergic drug, and an oral retard preparation of theophylline. The aim of the treatment was to decrease cardiac inhibition with glycopyrrolate and to bring about a positive chronotropic effect with theophylline. In our case, the combined therapy was effective in suppressing syncope and reflex anoxic seizures associated with BHSs This avoided the need for ventricular pacemaker implantation.

Background Acquired complete heart block, in pediatric age is mainly the results of direct injury to conduction tissue during cardiac surgery or cardiac catheterisation. It can also be observed in different clinical settings as infectious diseases, neoplasia, and inflammatory diseases. It has a wide range of presentation and in some settings it can appear a dramatic event. Although a rare finding during acute rheumatic fever, with a transient course, it may need a specific and intensive treatment. Case presentation We report the case of an Adams-Stokes attack in an adolescent with acute rheumatic carditis and complete atrio-ventricular block. The attack was the first symptom of carditis. We reviewed the literature and could find 25 cases of complete atrio-ventricular block due to rheumatic fever. Ten of the 25 patients experienced an Adams-Stokes attack. Nineteen of the 25 patients were certainly in the pediatric age group. Seven of the 19 pediatric cases experienced an Adams-Stokes attack. In 16/25 cases, the duration of the atrio-ventricular block was reported: it lasted from a few minutes to ten days. Pacemaker implantation was necessary in 7 cases. Conclusion Rheumatic fever must be kept in mind in the diagnostic work-up of patients with acquired complete atrio-ventricular block, particularly when it occurs in pediatric patients. The insertion of a temporary pacemaker should be considered when complete atrio-ventricular block determines Adams-Stokes attacks. Complete heart block during acute rheumatic fever is rare and is usually transient. Along with endocarditis, myocarditis and pericarditis, complete atrio-ventricular block has been recognized, rarely, during the course of acute rheumatic carditis.

A 2-month-old girl with poor appetite and failure to thrive was admitted because of pallor, dyspnea, and tachycardia with periodic gallop rhythm. Chest x-ray (Figure 1) showed cardiomegaly, and echocardiography (Figure 2) revealed left ventricular dilation (diastolic diameter, 41.5 mm [normal

Subacute thyroiditis is a rare disease in childhood and is considered postviral in origin. Epstein-Barr virus (EBV) involvement has been suspected, but just once demonstrated in an adult female. We report a case of subacute thyroiditis during infectious mononucleosis in a 3-year-old girl. The diagnosis of infectious mononucleosis was supported, in addition to the typical clinical symptoms, by presence of EBV DNA both in plasma and leukocytes. Subacute thyroiditis was diagnosed based on elevated thyroid hormone levels with thyrotropin (TSH) suppression, high inflammation markers and almost absent (99)TC uptake by thyroid. Moreover after 3 months from diagnosis thyroid function went back to normal as well as the (99)TC uptake.

This is a case report of a Fontan patient with previous Potts anastomosis who underwent stent implantation for left pulmonary artery stenosis. At follow-up the patient developed a fistula between the descending thoracic aorta and the left pulmonary artery at the site of the stent. This represents a late complication of stent placement.

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.

Twenty-eight patients operated with success for isolated coarctation of the aorta (i.e. with normal blood pressure and upper/lower limb gradientor = 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 gradient35 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.

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 diameteror = 4 mm).

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.

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.

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.

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.

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.

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.