Left axis deviation in healthy infants and children.

In a series of 1036 routine electrocardiograms, 14 children, without clinical and echocardiographic evidence of heart disease, showed a left axis deviation in the standard electrocardiogram. Six children also had an incomplete right bundle branch block. Left axis deviation is defined as a mean frontal QRS complex axis being -30 degrees or less. The age varied from three months to 10 years, with a clear predominance of males (13/14). The unusual finding of left QRS axis deviation in normal children is emphasized.

Anomalous origin of the right pulmonary artery from the ascending aorta: repair without cardiopulmonary bypass.

Three cases of anomalous aortic origin of the right pulmonary artery are reported. All patients presented with severe cardiac failure, two of them in the neonatal period. Clear visualization of the anomalous origin of the right pulmonary artery was obtained by cross-sectional echocardiography and the diagnosis was confirmed by cardiac catheterization. All patients underwent correction without the aid of cardiopulmonary bypass. The continuity between main and right pulmonary arteries was established by a polytetrafluoroethylene graft. One early death was due to bleeding and, probably, a pulmonary vasoconstriction crisis. In all patients the right ventricular pressure was systemic or suprasystemic before surgery and dropped to near normal in the operating room after correction. Two to five years after surgery the survivors are well. This rare, potentially lethal anomaly is amenable to correction; however, diagnosis should be followed by immediate surgical treatment.

Broncho-atrial discordance. A clinically diagnosed case.

Determination of the arrangement of the atrial chambers ("atrial situs") is the first step in sequential chamber analysis of complex congenital heart disease. Although bronchial pattern, as detected by chest X-ray, is an excellent guide for prediction of atrial arrangement, exceptions to this rule based on post-mortem examination have been described. We report a case in which discordance between the atrial arrangement (mirror-image) and bronchial morphology (usual) was diagnosed during life.

Retrograde pulmonary arteriography.

We performed retrograde pulmonary arteriography by means of a pulmonary venous wedge injection in 10 patients with no demonstrable intrapericardial pulmonary arteries by "conventional" angiographic techniques. In all cases but one, the procedure demonstrated the feasibility of a further operation. No complications were observed. Retrograde pulmonary arteriography is an important additional method for determining the existence of surgically accessible pulmonary arteries when other techniques have failed.

[Scimitar syndrome with or without the "scimitar sign"]. / Sindrome della scimitarra con o senza il "segno della scimitarra".

The so called scimitar syndrome is a congenital malformation characterized mainly by the anomalous venous return of the right lung in inferior vena cava. Many structure of the lung may be involved. Usually lobation and bronchial distribution are abnormal, the right pulmonary artery is hypoplastic or absent, a partial anomalous artery from aorta (pulmonary sequestration) may be present, the entire lung is small and various degree of displacement of the heart to the right is detectable. Associated congenital heart defects may also be present. The name of the syndrome comes from the presence of this vessel (scimitar's sign) on routine chest x-ray. This paper describes the clinical and angiographic reports of four cases, which may represent, according a unique embriological interpretation, a different instances of this syndrome. The anatomic characteristic, the sign of scimitar, could be also absent, as in cases 2 and 3. In the case 2 there was complete absence of right pulmonary artery and in the case 3 a severe hypoplastic right pulmonary artery with unique small venous return in left atrium. The authors opinion is that all the anomalies of lung vascular connections, that result from interference with the normal growth of the lung bud, its separation from systemic vascular channels, and the establishment of the lesser circulation should be included. Because the different components of thie malformation can be present in various combination, the sign of scimitar is not pathognomonic to define the syndrome.

Aorto-pulmonary window.

Aorto-pulmonary window (septal defect) is an uncommon congenital cardiac malformation accounting for only about 0.5% of the cases of congenital heart disease catheterised at our institution. Of 15 patients with this anomaly eight had associated cardiac malformations. Three patients presented in the neonatal period and in these patients the other cardiac anomalies (aortic interruption in two, pulmonary atresia in one) determined the clinical presentation and haemodynamic disturbance. A second group of five patients presented with heart failure during infancy and in these patients additional anomalies, present in three, were "incidental" findings. A third group of seven patients with similar physical signs but without heart failure did not present until after the first year of life and all were asymptomatic. Associated anomalies, present in two, were again "incidental" in that they did not influence the presentation. Of 12 patients without serious associated anomalies, five, operated on before 1970, had division and suture of the aorto-pulmonary window with one death. In two the defect was patched from the pulmonary artery but one required re-closure from the aorta. In six the defect was successfully patched from the aorta, which is now the preferred technique.

Changes of left ventricular contractility in alternating left bundle branch block.

A case of intermittent and at times alternating, nonrate-dependent left bundle branch block, with alternation of normal and reduced indexes of contractility (dP/dt, dP/dt/P) in a borderline normal heart is reported. The reduction of left ventricular contractility in the presence of left bundle branch block, as compared to what is found by other authors, is supposedly related to a different location of the block in the left branch (peripheral vs high).

[Persistence of fetal circulation. Clinical and angiographic evaluation of two cases. Review of the literature (author's transl)]. / Persistenza della circolazione fetale. Descrizione clinica ed angiocardiografica di due casi. Revisione della letteratura.

Persistent fetal circulation ("PFC") is a condition characterized by perduring after birth of fetal hemodynamic pattern: persistence of high pulmonary vascular resistance then occurs. "PFC" is a high mortality syndrome recently recognized as a cause of severe cyanosis and tachypnea in full-term newborns. Perinatal hypoxia, hyperviscosity, acidosis etc, may be also responsible of a persistence of high pulmonary vascular resistance. The increased right heart pressures cause a right to left shunt across the ductus arteriosus and foramen ovale not yet completely closed at birth. In this paper the clinical and angiographic evaluation of two full-term newborns, males, 5 days and 5 months old, are described, who presented with tachypnea, cyanosis and heart failure. Moreover we want to make a critical review of medical literature about this subject. The electrocardiogram of both patients showed right ventricular hypertrophy and myocardial ischemia. The chest roentgenograms showed cardiac enlargement and increased pulmonary vascular markings. Lung and central nervous system disorders were excluded by clinical and instrumental exams. Both patients underwent cardiac catheterization and angiocardiography in order to exclude a congenital heart disease. There was just evidence of pulmonary vascular resistance and right to left shunt at ductal and atrial level. O2 100% administration in one patient determined a decrease in pulmonary resistance and the shunting abolition. Clinical and hemodynamic patterns of this syndrome are very different. There are "simple forms" with normal myocardial function and "complex" higher mortality forms with evidence of cardiac involvement. It is often difficult to distinguish such forms from the other causes of central cyanosis and/or cardiac failure in the newborn as transposition of the great arteries, total anomalous pulmonary venous connection: the individualization of the syndrome then may be impossible without cardiac catheterization. A correct diagnosis for a precocious and reasonable treatment are extremely important for the survival of such critical newborns.

[Interventricular defect with discrete aortic stenosis below the defect]. / Difetto interventricolare con stenosi sottoaortica discreta sotto il difetto.

"Discrete" (fixed) subaortic stenosis associated with ventricular septal defect (VSD) is a rare but important anomaly. Two types of left ventricular outflow tract obstruction should be distinguished on the basis of its relation with the VSD, depending on whether the stenosis is above or below the defect. The four cases presented here are all in the latter category. Usually the patients belonging in the former category are associated with severe anomalies of the aortic arch. The diagnosis was made by clinical examination and by means of non invasive techniques, ecg, chest x-rays and ecocardiograms in all but one patient (the case "3"), in whom a subpulmonary stenosis (SPS) was associated. The final diagnosis was established by catheterization, which demonstrated the pressure gradient on the withdrawal curve from the apical part of the left ventricle to the aorta, and by contrastography. Three of four patients underwent total correction and are in good condition from 6 to 18 months after surgery, one of these cases had also SPS. In the remaining case, we thought the operation was not indicated on the basis of small size of VSD, of mild pressure gradient, and mild aortic valvular insufficiency (case "4"). The surgical approach to resect the "fixed" obstruction and to closure the VSD was carried out through a right atriotomy in two patients, and through a right ventriculotomy in the patient with SPS. The trans-aortic approach has to be discarded because it affords limited exposure of both defects and could increase the risk of damage of conducting tissue. A careful evaluation of aortic and left ventricular pressure, in association with angled angiography is highly recommended in the study of VSD.

Univentricular heart of right ventricular type: clinical, angiographic and electocardiographic features.

The sequential chamber analysis and angiographic, hemodynamic and clinical features, including those seen on electrocardiography, are presented in 18 patients with a univentricular heart of right ventricular type. In all cases a main chamber with a right ventricular trabecular pattern received both (or the sole) atrioventricular (A-V) connections, and a rudimentary chamber with a left ventricular trabecular pattern receiving no A-V connection was also present. The 18 patients were seen between 1971 and 1979; 6 died and the diagnosis was confirmed at autopsy. Twelve patients had double inlet, four absent right and two absent left A-V connections. Electrocardiography revealed right ventricular hypertrophy in all, and 11 had a superior mean frontal QRS axis. Chest roentgenography disclosed dextrocardia in 7 and levocardia in 11. Of 12 infants presenting with a marked decrease in pulmonary flow, 10 presented as ill neonates with hypoxia and acidosis. Four infants with increased pulmonary flow presented before 8 weeks with heart failure and mild cyanosis. Two with moderate pulmonary stenosis had mild cyanosis, but were asymptomatic. All had associated cardiac anomalies, pulmonary stenosis and persistent ductus arteriosus being the most common. For accurate diagnosis, angiographic demonstration of a posterior rudimentary chamber in addition to the main chamber of right ventricular trabecular pattern is mandatory.

Dextrocardia--value of segmental analysis in its categorisation.

Dextrocardia can be defined as a heart in the right chest with the major axis to the right. This definition, however, conveys no information regarding the chamber arrangements and internal anatomy of the heart. Of 40 patients satisfying this definition in the files of the Brompton Hospital, 33 had angiocardiographic data adequate for complete analysis in terms of connections, relations, and morphology of cardiac segments. They form the subject of this report. There were 16 (48%) patients with situs solitus, 11 (33%) with situs inversus, and six (18%) with situs ambiguus. Of the cases of situs ambiguus, four exhibited laevoisomerism and two dextroisomerism. Of the 16 patients with situs solitus, six had two ventricles and 10 had univentricular hearts; two patients had concordant and three discordant ventriculoarterial connections, seven had double outlet ventricle, and four a single outlet heart. Of the 11 patients with situs inversus, nine had two ventricles and two a univentricular heart of right ventricular type; the arterial connection was concordant in two, discordant in two, double outlet in six, and single outlet in one. Of the six patients with situs ambiguus and laevo or dextroisomerism, four had two ventricles, and two univentricular hearts; the arterial connection was concordant in one, double outlet in three, and single outlet in two. Segmental analysis and the use of basic descriptive terms are essential to define the complex anatomy of such hearts.

[Correlations between plasmatic concentrations and antiarrhythmic effect of bunaphtine (author's transl)]. / Correlazione tra concentrazioni plasmatiche ed effetto antiaritmico della bunaftine

Antiarrhythmic effect of Bunaphtine (1.5 mg/kg) (e.v. in 2 min) was studied in 35 patients with ectopic rhythms, both ventricular and supraventricular. Arrhythmia was checked by continuous electrocardiographic recording. Bunaphtinemia was determined by spectrophotometric method. Plasma concentration levels proved that Bunaphtine is cleared from the blood in two different stages: T1/2alpha = 0.5 min and T1/2beta = 84.7 min. Antiarrhythmic effect was observed in connection with the fast distribution phase and it was even more evident when plasma concentration fluctuated around 3-4 mg/l. Extrasystoles increased when Bunaphtine levels diminished. The minimum effective level showed fairly good individual differences. In three cases with congestive heart failure, hepatic and renal insufficiency the increase or the appearance of ventricular extrasystole was related to high Bunaphtinemia levels. Risk of toxicity seems to be related to the dose and the administration methods. Cardiac decompensation or hepatic and renal insufficiency determine more elevated and protracted Bunaphtinemia levels.