Transcatheter closure of an unligated vertical vein with an Amplatzer Vascular Plug-II device.

: The usual surgical practice after repair of a Total Anomalous Pulmonary Venous Connection (TAPVC) is to ligate the vertical vein (VV). Many surgeons find it expedient to leave the VV unligated to reduce pulmonary arterial pressure, decrease perioperative pulmonary hypertensive crisis, provide better hemodynamics postoperatively (1), and enable the adaptation of cardiac chambers to a new workload. Afterwards, the unligated VV may cause significant left-to-right shunt, likewise an atrial septal defect, mandating later surgical ligation or device closure (2). This report details transcatheter occlusion of a patent VV using a device Amplatzer Vascular Plug II, after TAPVC repair in early infancy. The transcatheter occlusion of an unligated VV after repair of supracardiac TAPVC represents an effective alternative to surgical redo. The device Amplatzer Vascular Plug II achieves great results.

Cantrell's Syndrome with left ventricular diverticulum: a case report.

Congenital left ventricular diverticulum is a rare condition. When found, it is usually accompanied by other intracardiac malformations, so that again further examination is indicated. Furthermore, it is usually associated with thoracoabdominal wall defect, as seen in the spectrum of Cantrell's pentalogy, a congenital anomaly consisting of a lower sternal defect, diastasis recti, pericardial defect, anterior diaphragmatic hernia and cardiac malformation. This paper reports a case of Cantrell's Syndrome with left ventricular diverticulum. Successful total correction of ventricular diverticulum, ventricular defects and diastasi recti, was performed at two years of age. The clinical features, ambryology and surgical management of these defects are discussed.

Double-outlet right ventricle with intact ventricular septum.

In the present case report, we describe a malformation consisting of a double-outlet right ventricle with an intact ventricular septum diagnosed in a 3-day-old female newborn. To our knowledge 35 cases have been described in the literature. The diagnosis was made by two-dimensional echocardiography and confirmed by angiocardiography. An inadequate opening in the interatrial septum and hypoplasia of the mitral valve and left ventricle were present. In this condition the only outlet of the left ventricle is via the atrial septal defect. Balloon atrial septostomy was performed. At 1 month of age the patient required a right modified Blalock-Taussig shunt and surgical atrial septectomy. Five months after surgery, the patient was in good clinical conditions.

Early congestive heart failure due to origin of the right coronary artery from the pulmonary artery.

We describe a two-month-old infant with early congestive heart failure due to anomalous origin of the right coronary artery from the pulmonary artery. The diagnosis was made by two-dimensional and color flow Doppler echocardiography, confirmed by angiocardiography, and the case was successfully corrected at surgery. As opposed to the more frequent anomalous origin of the left coronary artery from the pulmonary trunk, this anomaly generally does not cause any typical clinical finding, often becoming an autoptic or surgical surprise after infancy or in adult age.

Pulmonary circulation in pulmonary atresia associated with the asplenia cardiac syndrome.

OBJECTIVE: The goal of this study was to determine the patterns of the pulmonary circulation in patients with pulmonary atresia and asplenia. BACKGROUND: The asplenic cardiac syndromes characteristically have complex cardiac anomalies including pulmonary stenosis or atresia. Definition of the pulmonary artery circulation and pulmonary venous connections is needed for consideration of surgical procedures. METHODS: In 35 patients, the sources of pulmonary blood flow, anatomic features of pulmonary arteries and pulmonary venous connections were determined from angiograms or autopsy specimens. RESULTS: The main pulmonary artery was absent or hypoplastic in 91% of patients; most had a ductus arteriosus. The right and left pulmonary arteries were confluent in 90% and usually of normal size (right 71%, left 63%). Total anomalous pulmonary venous connections were present in 38%. CONCLUSIONS: The anatomic features of the pulmonary arteries in pulmonary atresia associated with the asplenic cardiac syndrome are usually favorable for palliative surgical procedures. Total anomalous pulmonary venous connection may exist as a complicating factor.

[Anomalous insertion of the mitral valve. A rare form of subaortic obstruction in children]. / Anomala inserzione della mitrale. Una rara forma di ostruzione sottoaortica nell'infanzia.

Among different anatomical causes of subaortic obstruction, anomalous attachment of the mitral valve on ventricular septum is one of the rarest. We report our experience with 4 such cases of subaortic obstruction. Their age at first observation ranged between 1 and 6 months. All patients had viscero-atrial situs solitus, levocardia and normal atrio-ventricular and ventriculo-arterial connections. None had associated cardiac septal defects. Cases with atrio-ventricular canal were excluded. All patients showed a systolic ejection murmur and electrocardiographic features of left ventricular hypertrophy; 3 presented congestive heart failure in the first 3 months of life. Diagnosis was made by 2D-echocardiography and cardiac catheterization in 3 patients and intraoperatively in 1. Only in the last 2 patients, correct diagnosis was made at the initial 2D-echocardiographic examination. Due to left ventricular hypertrophy, in the first 2 patients hypertrophic obstructive cardiomyopathy was originally suspected. One patient died intraoperatively during attempted transaortic resection of subaortic obstruction. Other 2 patients underwent left ventricle-aortic conduit implantation. Of these 2 patients, 1 died 3 months later for sepsis and the other was reoperated upon 3 years later for mitral valve replacement and conduit take-down, with good clinical result. The fourth patient is waiting for surgical intervention. Subaortic obstruction due to anomalous attachment of the mitral valve on ventricular septum may present with early congestive heart failure; the best diagnostic tool is 2D-echocardiography. Left ventricle-aortic conduit may represent a surgical alternative to transaortic resection.

[Ventricular-infundibular morphology in visceral heterotaxia with left isomerism. An echocardiographic-angiocardiographic study]. / Morfologia ventricolo-infundibolare nell'eterotassia viscerale con isomerismo sinistro. Studio eco-angiocardiografico.

We report 2D-echocardiographic and angiocardiographic assessment of 28 cases of visceral heterotaxia with left atrial isomerism, with particular emphasis on infundibular ventricular morphology. Ventricular D-loop was present in 15 cases (52.6%), and ventricular L-loop in 13 (46.4%); 25/48 patients had concordant ventricular loop and cardiac position (89.3%). In 18 patients (64.3%) there were 2 balanced ventricles; of the other 10 patients, 9 (32.1%) presented right and 1 (3.6%) left ventricular dominance. Ventricular septal defect was present in 12 cases (42.8%). Ventriculo-arterial connections were concordant, with "normally related" great arteries in 9/15 cases with ventricular D-loop (60%). In these cases, ventricular morphology, connections and relations of the great arteries were typical of situs solitus. In the remaining 6 patients there was double-outlet right ventricle, also with normally related great arteries. In 12/13 cases (92.3%) with ventricular L-loop ventriculo-arterial connections were concordant with "mirror image normally related" great arteries. In these cases, ventricular morphology, connections and relations of the great arteries were typical of situs inversus. In one patient there was double-outlet right ventricle, also with mirror image normally related great arteries. Twelve patients (42.8%) had pulmonary stenosis and 5 had a systemic outflow obstruction (17.8%). From these observations we conclude that ventriculo-infundibular morphology, either of situs solitus-type or of situs inversus-type, is a typical anatomical feature of left atrial isomerism. These results may have important implications in the diagnosis and in the surgical management of patients with left atrial isomerism.

[Right atrial isomerism with pulmonary atresia: the angiocardiographic characteristics and pulmonary circulatory patterns]. / Isomerismo atriale destro con atresia polmonare: caratteristiche angiocardiografiche e modelli di circolazione polmonare.

The patterns of pulmonary circulation were assessed by angiography in a series of 24 patients with right atrial isomerism (asplenia syndrome) and pulmonary atresia. Mean age at catheterization was 21.5 days (range 1 day-11 months). The pulmonary arteries were confluent in 22 cases (91.7%). Eighteen patients (75%) had ductus-dependent pulmonary circulation in presence of confluent pulmonary arteries, similar to cases of pulmonary atresia combined with complex congenital heart disease. The ductus appeared in continuity with the aortic arch, shaping an acute angle with the descending aorta, as in cases of pulmonary atresia with ventricular septal defect. A bilateral ductus was present in 5 cases (20.8%), 3 with confluent and 2 with nonconfluent pulmonary arteries. One patient presented multiple systemic collaterals to the lungs. All the other patients had normal arborization of the pulmonary arteries. Seven patients presented an obstructed total anomalous pulmonary venous connection: 4 in inferior vena cava and 3 in superior vena cava. These anatomical observations provide useful information for the surgical palliation of these anomalies and, probably, warrant pre-operative angiographic evaluation in all the patients with right atrial isomerism.

Atrioventricular canal in Down syndrome. Prevalence of associated cardiac malformations compared with patients without Down syndrome.

The atrioventricular canal is the "classic" congenital heart anomaly in Down syndrome. We may learn more of the nature of this disorder by careful study of the anatomic characteristics of the cardiac lesions and by comparing these lesions in patients with and patients without Down syndrome. We reviewed the clinical characteristics (echocardiographic and angiocardiographic) of 220 patients with atrioventricular canal and compared the prevalence of anatomic types and associated cardiac malformations in children with (105) and without (115) Down syndrome. In patients with Down syndrome, the complete form of atrioventricular canal was prevalent, with a high frequency of associated Fallot's tetralogy. Partial atrioventricular canal and left-sided anomalies were more common in patients without Down syndrome. Down syndrome is associated with a simpler type of atrioventricular canal when compared with patients with a normal chromosome configuration.

[Non-surgical closure of patent ductus arteriosus in children with the Rashkind double disk occluder]. / Chiusura non chirurgica del dotto arterioso pervio in età pediatrica con dispositivo di Rashkind a doppio ombrello.

Since the first clinical application of transcatheter closure technique for patent ductus arteriosus in children and infants in 1977 by Rashkind and Coll., this technique is routinely performed only in a few major Pediatric Cardiology Centers. We report our successful series which is the first of its kind both in Italy and in Southern Europe. The series included 11 children aged 1.6 to 10 years (mean age 4.5 years) all affected with patent ductus arteriosus (PDA). Subaortic stenosis (1) and ventricular septal defect with pulmonary stenosis (1) were associated anomalies. In all of the cases, after the standard percutaneous cardiac catheterization, the Mullins method was attempted to advance and deliver the Rashkind PDA double-disk occluder. Successful closure was accomplished in 10 while almost complete closure was achieved in the 11th. Normal Doppler flow pattern after the procedure confirmed the successful results. No complications occurred. Although our experience is limited, non-surgical PDA closure provided an excellent alternative to surgical procedure.

[Interventricular muscular defect in the atrioventricular canal]. / Difetto interventricolare muscolare nel canale atrioventricolare.

The muscular ventricular septal defect associated with the atrioventricular canal is a malformation which has not yet been extensively studied. Between June 1982 and December 1989, 151 patients with atrioventricular canal underwent echocardiography and angiocardiography in our Department. Of these 95 (62.9%) had a complete form and 56 (37.1%) a partial. Among the 151 patients, 81 (53.6%) presented Down syndrome. We found 5 muscular ventricular septal defects in 4 patients; in 3 cases there was a single defect and in one case two defects. These defects were midmuscular in all patients and one patient also presented an apical defect. All 4 patients with muscular ventricular septal defect presented a complete atrioventricular canal and aortic coarctation; 3 out of 4 patients had a hypoplastic left ventricle with absence of Down syndrome. The muscular ventricular septal defect is a malformation which is rarely associated with atrioventricular canal (4/151 = 2.6%). In our experience, it was always associated with a complete form with aortic coarctation and was very rare in Down syndrome patients (1/81 = 1.2%). These findings may represent a peculiar association of anomalies which may be caused by fetal hemodynamic mechanisms.

[Isolated enlargement of the right atrium. Description of a successfully operated case]. / Ingrandimento isolato dell'atrio destro. Descrizione di un caso operato con successo.

We report a case of isolated right atrial enlargement. The patient was an 11 year old boy who had life-threatening supraventricular arrhythmias. Diagnosis was basically performed by Echo-2D Doppler. The surgical treatment performed on cardiopulmonary by-pass, was followed by immediate conversion to sinus rhythm. Histological examination showed chronic inflammatory aspects. Postoperative Echo-2D (2 years later) showed a moderate right atrial enlargement.

Enteric infections, cow's milk intolerance and parenteral infections in 118 consecutive cases of acute diarrhoea in children.

One hundred and eighteen consecutive cases of childhood acute diarrhoea (mean age: 10.5 months) were studied after admission to our Pediatric Unit in Naples over a 13-month period. A diagnosis was established in 92 patients (78%): 55% of patients were found to have an infectious enteritis (among them, 5 had ETEC infections and 1 had an ST-producing Klebsiella infection), 12% a parenteral infection, 11% cow's milk intolerance. The occurrence of gross blood (P less than 0.01), leukocytes, and reducing substances in the stools was more commonly associated with infectious enteritis than with diarrhoea due to all other causes. In 14 patients (8 of whom were malnourished), diarrhoea ran a prolonged course. In all, the eventual outcome was favourable. Our findings, while confirming that infectious enteritides account for most of acute diarrhoeas in children, stress the importance of parenteral infections and cow's milk intolerance in this condition. Also, the need for an accurate search for enterotoxigenicity of enterobacteria before ruling out their pathogenetic role is stressed.