[The isolated agenesis of a branch of the pulmonary artery. A case report: its diagnosis and therapy]. / Agenesia isolata di un ramo dell'arteria polmonare. Presentazione di un caso: diagnosi e terapia.

We illustrate a case of so-called right pulmonary artery agenesis, discovered in a 3-month-old baby. This kind of malformation is rarely reported in neonatal age being most often discovered in the adulthood. The patient presented with cardiac failure, cyanosis and radiological features of cardiomegaly and left side pulmonary venous congestion. The diagnosis was strongly suspected by two-dimensional echocardiography and confirmed at cardiac catheterization. The patient was successfully operated upon with connection of main pulmonary artery to hilar right pulmonary artery. To the best of our knowledge, this is the first reported case of diagnosis and effective surgical therapy of right pulmonary artery agenesis in neonatal age.

[Perfusion catheter and cardio-pulmonary support standby: organizational strategy for high risk PTCA. Description of a case treated successfully]. / Catetere perfusore e supporto cardio-polmonare standby: strategia organizzativa per PTCA ad alto rischio. Descrizione di un caso tentato con successo.

The organizational strategy used in a high risk Coronary Angioplasty procedure is discussed. The attempted vessel was a severely stenosed Right Coronary Artery in a patient who had undergone triple graft, with residual obstruction of Left Main Artery and only one patent venous graft on a marginal branch of the Circumflex artery. The procedure was performed using a Stack type autoperfusion balloon angioplasty catheter, with a Cardiopulmonary Support System held in standby.

[Persistence of the right venous sinus valve simulating a right atrial myxoma in a case of total anomalous pulmonary venous return. Echocardiographic, anatomo-clinical and surgical aspects]. / Persistenza della valvola del seno venoso destro simulante un mixoma atriale destro in un caso di ritorno venoso polmonare anomalo totale. Aspetti ecocardiografici, anatomoclinici e chirurgici.

A case of persistence of the right venous sinus valve that on echocardiographic examination simulated right atrial myxoma is reported in a patient with total abnormal pulmonary venous return in the coronary sinus. Echocardiography showed a mobile, pedunculated mass present in systole in the right atrium that shifted to diastole in the right ventricle, highly suggestive of right atrial myxoma. The right ventricle also showed a volume overload and a space without echoes behind the left atrium. A membrane was encountered in the right atrium at surgery. This was removed and the venous return corrected.

[Aortic insufficiency caused by valvular dysplasia in childhood. Apropos of 3 cases]. / Insufficienza aortica da displasia valvolare nella fanciullezza. A proposito di tre casi osservati.

Three patients whose aortic incompetence (A.I.) was recognized in childhood, have had an angiographic study 10, 9 and 4 years, respectively, following the first clinical diagnosis of their valvular disease. A.I. was related, in all three cases, to a dysplastic valve ("floppy valve syndrome"); in none of them was there an abnormal dilatation of ascending aorta. In our opinion, the absence of an abnormal aortic root dilatation separates our cases from those in whom aortic regurgitation is part of Marfan's syndrome, either in its full form or in the "forme fruste". From the clinical viewpoint, the presence of a normal ascending aorta could explain the mild course of these cases, as compared to the rapid progression observed in Marfan's syndrome. In the attempt to differentiate the two types of dysplastic valvular disease from an etiologic viewpoint, we suggest that the basic problem in our patients could be an alteration of the valvular connective tissue development, rather than a genetic disorder, like the one possibly responsible for Marfan's syndrome.

Double outlet right ventricle with atrioventricular discordance. Report on cases having situs solitus, dextrocardia, pulmonary stenosis and levomalposition of the aorta.

We describe six patients with situs solitus of viscera and atria, dextrocardia, atrioventricular discordance, ventricular septal defect, double outlet from the morphological right ventricle, pulmonic stenosis and levomalposition of the aorta. Four patients were male and two female; their age ranged from 3.5 to 31 years (mean 13.8 years). All had various degrees of disability, and presented with cyanosis, clubbing and high hematocrit levels. One patient had an atrio-ventricular block that varied from first to third degree; another patient showed intermittent junctional rhythm. At angiography the ventricular septum appeared to be almost perpendicular to the frontal plane in most cases, so that the anteroposterior projection resulted in a true axial view. One overriding left atrioventricular valve and one straddling right atrio-ventricular valve were demonstrated; no significant incompetence of either valve was observed. The ventricular septal defect was always single and related to the subpulmonary outflow. Pulmonic stenosis was valvular in every patient; an additional infundibular obstruction was present in one case. In two cases an additional stenosis was discovered at the supravalvular level. The left pulmonary branch was stenotic in one case; it was hypoplastic, with controlateral dilatation, in two cases; both pulmonary arteries were dilated in one case. The aorta was always to the left of the pulmonary artery, either anterior or side by side. Three patients were operated on in different Institutions: one had a pulmonic valvotomy at the age of six years; one, aged twenty, had a right Blalock-Taussig shunt; the third, with overriding left atrioventricular valve, underwent a modified Fontan operation at the age of thirty years with success.(ABSTRACT TRUNCATED AT 250 WORDS)

[AQRS left axial deviation of more than -30 degrees and incomplete right branch block in isolated ventricular septal defects]. / Deviazione assiale sinistra di AQRS oltre -30 gradi e blocco di branca destra incompleto nei difetti isolati del setto ventricolare.

Electrocardiographic findings in five out of forty-three cases of isolated Ventricular Septal Defects (VSD) demonstrated incomplete Right Bundle Branch Block (RBBB) with left AQRS deviation of more than -30 degrees. These features are said to be characteristic of VSD of the Persistent Common Atrioventricular Canal type. However, in two of our five cases a defect of the Inlet Septum was documented angiographically; in the remaining three cases a defect of the peri-Membranous septum was clearly demonstrated. Moreover hemodynamic data in our patients indicate that right ventricular diastolic overload could not account for RBBB in all of the cases. The clinical importance derived from this experience is that ECG findings of RBBB and left AQRS deviation of more than -30 degrees are not necessarily associated with, and therefore do not represent certain proof of the presence of VSD of the A-V Canal type. The association of this ECG pattern with VSD's other than the A-V Canal type, as previously observed, appears to have an anatomic explanation, which however needs further substantiation through electrophysiologic studies.

Aortico-left ventricular tunnel in infancy. Two surgical cases.

Two infants with aortico-left ventricular tunnel and associated aortic valvular disease were operated upon. The first patient, age 28 months, had successful surgical closure of the anomalous communication. Four years postoperatively he is asymptomatic, but mild aortic regurgitation persists. The second infant, aged 3 months (the youngest reported surgical case), also had severe aortic stenosis (dysplastic valve) and mitral regurgitation. She died, twelve hours after the operation, from intractable left ventricular failure. On the basis of the previously reported cases (22), the clinical profile of the malformation is outlined with special reference to associated aortic valve lesions. Early operation is recommended because of the poor natural history of the condition.

[Situs inversus and dextrocardia. Analysis of the associated congenital heart diseases (author's transl)]. / Situs inversus con destrocardia. Analisi delle cardiopatie congenite associate.

13 cases of congenital heart diseases in patients having atrial situs inversus and dextrocardia, all studied by means of angiography, are described following an up to date segmental approach. The type of atrioventricular connection was concordant in the 11 biventricular hearts; double inlet in the 2 univentricular hearts. The mode was always via two atrioventricular valves. In two cases the atrioventricular flows realized a criss-cross condition, being horizontal the interventricular septum, superior the morphologically right ventricle and inferior the morphologically left ventricle. In none of the univentricular hearts was an accessory chamber identified; they were both considered to be of indeterminate type. Ventriculo-arterial connections were: concordant in 3 cases (23%), including one case of Tetralogy with pulmonary atresia; discordant in 3 cases (23%); double outlet in 7 cases *54%). The aortic valve was in the assumed position (left and posterior to the pulmonary valve) only in the 3 cases with concordant as a rule. A ventricular septal defect was actually found in all cases; in one it was associated with pulmonary vascular disease. Obstruction to the pulmonary flow (subvalvular, valvular or vascular) was ascertained in 85% of cases, whereas aortic obstruction was never found. Overall, important congenital heart malformations appear to be frequently associated with situs inversus and dextrocardia. The ventriculo-arterial junction is identified as the most affected point.

[Tricuspid atresia: hemodynamic and surgical considerations (author's transl)]. / Atresia della tricuspide: considerazioni emodinamiche e chirurgiche

Recently some patients with tricuspid atresia underwent physiological correction, as indicated by Fontan, as an alternative to classical procedures. The aims of the hemodynamic and contrastographic study are illustrated and exemplified, in view of the type of surgery. 49 patients underwent palliative operations: 47 shunts and 2 banding. The most frequent procedure was the Waterston shunt. The total mortality was 10.2%, but no deaths occurred after the third month of life. Finally, the future and the limits of "total" correction are discussed.

[Left ventricle-right atrial communication. Surgical, hemodynamic and clinical aspects (author's transl)]. / Comunicazione ventricolo sinistro - atrio destro. Aspetti clinici, emodinamici e chirurgici

Anatomical, clinical radiographic and electrocardiographic aspects in 12 cases of left ventricle-right atrial communication are described. The usual severity and precocity of symptoms are outlined. At cardiac catheterization the level of the shunt was not found to be exactly correlated with the anatomic type of the defect; the mean QP/QS was 2,7 (1,4-5,8); only one case of pulmonary hypertension was detected and the mean RP/RS was 0,19 (0,06-0,83). Contrastographic aspects are emphasized and the importance of having two orthogonal projections is outlined. 9 patients underwent surgical repair with good results, except for the patient with pulmonary hypertension, who died soon after the operation: this patient shouldn't have been operated on. In all cases only right atriotomy was made, and the defect was closed by a dacron patch; no patient had a tricuspid plastic; one perforation and one cleft of the septal leaflet of the tricuspid valve were sutured directly. In view of the simplicity of surgical correction, wide indications are possible, considering, too, that there have been no reports on the spontaneous closure of the defect.

[Aneurysm of the left ventricle in infants. Report of two cases successfully treated (author's transl)]. / Aneurisma del ventricolo sinistro in eta' pediatrica. Osservazioni su due casi operati con successo

Aneurysm of the left ventricle in infants is quite a rare cardiac malformation. In this paper two cases are described, six and seven years old respectively, the first one with a subvalvular annular type of aneurysm, the second with an apical type, both surgically treated with success. Ths most known and most frequent aetiological factors are discussed as well as their possible complications. Surgical treatment (aneurysmectomy) is emphasized as the only effective therapy to date.