Fifty one years have passed since gross and Crafoord independently reported successful repair of aortic coarctation. One could be forgiven for assuming that all the surgical controversies have now been settled. This is far from the case, as the numerous publications (frequently with opposing view points) related to timing, technique and complications bear testament. Perhaps the passage of the golden anniversary of this operation should stimulate a degree of reflection among surgeons and cardiologists.

To assess the effect of a modified Fontan operation on systemic venous blood flow and the hepatic circulation, we compared 11 patients having an atriopulmonary connection and 35 with total cavopulmonary anastomosis. The Doppler echocardiographic study of the caval venous, hepatic venous and portal venous flow was performed so as to calculate the pulsatility ratio and the variation of flow with respiration. All patients had undergone cardiac catheterization. In addition, we included specific laboratory investigations to assess function of various organs.

Significantly lower maximum velocities of flow at inspiration (0.31±0.12 rn/sec vs 0.45±0.14 m/sec) and expiration (0.23±0.09 rn/sec vs 0.32±0.11 m/sec), less pulsatility (0.43 vs 0.16) and a lower ratio of systolic to diastolic velocity (1.22 vs 1.85) were found in the patients having a cavopulmonary as compared to an atriopulmonary anastomosis. Peak velocities of hepatic venous flow during inspiration and expiration were significantly lower in those with a cavopulmonary anastornosis (p=O.OOl and p<O.OOl, respectively). In these patients, forward flow was extremely dependent on respiration, with decrease or cessation of antegrade flow during expiration in 22 patients. The velocity of portal venous flow was also significantly lower in these patients, although the pulsatility ratio did not differ significantly between the groups (0.5 ± 0.21 and 0.57 ± 0.23, respectively). The ratio of inspiratory and expiratory velocities showed no significant difference between the groups, nor was there any correlation between the pulsatility ratio of the venous vessels or the ratio of peak flow velocities during expiration and the mean systemic venous/right atrial pressure on postoperative cardiac catheterization. Hypoproteinemia was found in 8 patients after total cavopulmonary anastomosis; 9 of 10 patients with protein C deficiency belonged to this group.

The dependence of hepatic venous flow on respiration in the presence of a chronically elevated systemic venous pressure in patients after the total cavopulmonary anastornosis may influence hepatic function in the postoperative period.

Severe mechanical hemolysis ocurred in a 22 month old girl after placement of a 38-5-10 coil in the arterial duct. Shé had previously undergone percutaneous closure using the Rashkind technique 14 months before insertion of the coil, but remained with a moderate residual shunt. Surgical removal of the devices and division of the duct were required to abolish the hemolysis.

It has already been suggested that redundancy of the primary atrial septum could be associated with primary fetal arrhythmias. There are few reports, however, concerning the mobility of the primary septum and its behavior during fetal life. In this study, fifteen fetuses without anatomic heart disease were serially evaluated by fetal echocardiography. The time interval between three separate examinations was at least two weeks. A redundancy index was calculated as the ratio between the maximum excursion of the flap valve of the septum and the maximal left atrium as seen in a four chamber view. The indexes observed in the three examinations were: 0.55 ± 0.15, 0.48 ± 0.13 and 0.41 ± 0.13, respectively. Analysis of variance for normal distribution and Tukey's test were performed. A significant decrease (p < 0.05) in the redundancy index was observed with increasing gestational age. We postulate that this finding may be related to the decrease in frequency of premature atrial beats throughout fetal life.

Management of pulmonary vascular resistance in neonates with congenital heart disease is important for stabilization before and after surgical interventions. Thus, we determined which combina tion of positive end-expiratory pressure ventilation and fraction of oxygen in the inspired air increases pulmonary vascular resistance without compromising delivery of oxygen to the tissue. Eight piglets were anesthetized, intubated and ventilated. Pulmonary flow and pulmonary arterial and left atrial pressures were monitored continuously. At all levels of inspired oxygen (1.00, 0.21 and 0.15), ventilation at a pressure of 15 cm of water increased pulmonary vascular resistance. At all levels of positive pressure ventilation, a fraction of 0.15 of inspired oxygen increased pulmonary vascular resistance. The combination of a ventilatory pressure of 15 cm of water and inspired oxygen of 1.00, or ventilatory pressure at 5 cm of water and oxygen delivery of 0.15, produced similar changes in pulmonary vascular resistance (19.1 ± 2.8 vs. 20.0 ± 3.8 mmHg/(L/min)) and cardiac output (0.78 ± 0.07 vs. 0.93 ± 0.10 L/min) but, the higher level of positive pressure plus 1.00 inspired oxygen gave a significantly higher arterial oxygen saturation (0.99 ± 0.03 vs. 0.72 ± 0.19%) and delivery of oxygen to the tissues (13.7 ± 2.9 vs. 7.4 ± 1.5 ml 02/min, p<0.05). Thus, both high positive pressure ventilation and hypoxia increase pulmonary vascular resistance. Only high pressure ventilation plus high concentrations of inspired oxygen, however, increased pulmonary vascular resistance without compromising delivery of oxygen, suggesting that this combination is a superior means of increasing pulmonary vascular resistance.

The morphology and mechanism of obstruction to the pulmonary venous pathway in patients following either Mustard or Senning repair of complete transposition was assessed using transoesophageal echocardiography. Seven patients underwent catheterization and complete transoesophageal study in both transverse and longitudinal planes, followed by balloon dilation of the obstructed venous pathway in five of seven under transoesophageal echocardiography guidance. A complete scan of both systemic and venous pathway was obtained in all patients. Four patients with a Mustard repair were found to have a ‘tubular’ baffle, with stenosis resulting from a discrete wedge of tissue arising from the atrial free wall in association with fibrous adhesions to the baffle. In the three patients with a Senning repair the intra-atrial baffle showed a characteristic ‘peaked’ appearance, with stenosis of the venous pathway stenosis related directly to con tracture of the patch used to augment the atrial free wall. The mechanism of obstruction appears to be inherent to the different surgical techniques. Indwelling transoesophageal echocardiography provided immediate haemodynamic and morphologic assessment of the efficacy of dilation of the obstructed venous pathway.

We describe 4 patients in whom the pulmonary veins were compressed between the descend ing aorta and the atrial part of the heart in consequence of an abnormal distribution of the pulmonary blood flow after the Fontan procedure.

Between January 1985 and April 1994, 42 children aged between 7 and 14 years (mean 11.3 years) underwent repair of severely incompetent rheumatic mitral valves with no accompanying mitral stenosis. In 19 patients the tricuspid valve was severely incompetent, while 8 patients had severe aortic incompetence. Overall, the repair incorporated shortening of elongated tendinous cords and insertion of a Duran or Carpentier prosthetic ring. The repair was the sole procedure in 15 patients, whilst 19 patients also had a De Vega tricuspid valvar annuloplasty and 8 had repair or replacement of the aortic valve. There were no hospital deaths. Postoperative transthoracic echocardiographic studies revealed trivial residual mitral valvar regurgitation in 6 patients, mild regurgitation in 18, moderate in 15, and regurgitation severe enough to warrant replacement of the mitral valve in 3 patients. The mean follow-up period was 37 months (maximum 120 months, minimum 1 month). 0139 patients followed-up, 28 attended for more than 5 years. Of these, 7 underwent replacement of the mitral valve for severe regurgitation within 4 years of the repair. Severe mitral regurgitation in 3 patients was controlled by medical therapy. One was eventu ally lost to follow-up, and one patient died of causes unrelated to surgery. The remaining 16 patients had absent to moderate mitral valvar regurgitation. These results contrast with 10 repairs of congenital mitral incompetence, where no patients required re-operation in the immediate 5 years period of follow-up. Every effort should be made in children with rheumatic mitral incompetence to preserve the natural valve by con servative repair, despite the fact that repair of the incompetent rheumatic mitral valve is not so durable as repair of congenitally incompetent valves.

To evaluate left ventricular diastolic filling in infants with ventricular septal defect, which has yet to be documented, we measured various Doppler echocardiographic indexes from transmitral flow in the following groups: 10 infants with ventricular septal defect without pulmonary hypertension; 10 infants with ventricular septal defect with pulmonary hypertension; and 9 normal infants to serve as controls. The peak A, total velocity time integral, E area, and A area in patients without pulmonary hypertension were all significantly larger than those in controls. The peak ratio E/A, and 1/3 filling fraction, in patients without pulmonary hypertension were significantly lower than in controls. The peak A, A area, and deceleration time in patients with pulmonary hypertension were significantly larger than in patients without pulmonary hypertension and controls. The peak E/A, area E/A, and 1/3 filling fraction in patients with pulmonary hypertension were significantly lower than in those without pulmonary hypertension and controls. The index of left ventricular mass, as well as the index of end-diastolic left ventricular wall thickness, correlated strongly with peak A, A area, and deceleration time. The ratio between the systolic pulmonary and systemic pressures correlated strongly with peak A, A area, peak E/A, area EtA, and 1/3 filling fraction.

These results demonstrated that the patterns of left ventricular filling in infants with ventricular septal defect were different from those in normal infants, and suggested that the abnormal patterns may indicate the insufficiency of adaptation of left ventricle (increase of left ventricular compliance) for volume overload in the presence of a ventricular septal defect..

We describe three cases of sternal defects of varying severity associated with other congenital anomalies. In the most severe case, both anterior and posterior defects were seen, with near-absence of the sternum and pericardium continuous with a large omphalocele. This resulted in external location of organs usually confined within the thoracic and abdominal cavities. A ventricular septal defect was present, and the arterial duct was absent. The course of the ascending aorta was anomalous. The baby had anencephaly and rachischisis. In the intermediate case, a proximal sternal cleft was associated with shortening of the ster num, and absence of the manubrium. Anterior pericardial and diaphragmatic defects were seen, while a scalp defect and an encephalocele were present on the posterior aspect of the head. This baby had tricuspid atresia. The qremaining case had only an anterior defect with a shortened sternum. A supra-umbilical omphalocele contained a left ventricular diverticulum without interposing pericardium or diaphragm. Ventricular and atrial septal defects were present. The first two cases can be considered as representing fail ure of development of both an anterior and a posterior midline field. The third case, much milder than the other two, represents failure of development of an anterior field.

Tetralogy of Fallot, when associated with atrioventricular septal defect permitting shunting at ventricular level, represents a complex cyanotic congenital malformation. Experience with surgical repair is limited, and results vary considerably. Between 1984 and 1996, we repaired 14 consecutive patients with this combination seen in our center. Their ages ranged from 8 months to 21 years (median 7.4 years). Six (42.9%) had Down's syndrome. In eight patients the correct diagnosis was made using echocardiogra phy alone. In the remaining six patients, who had previously-constructed arterial shunts and/or suspected pulmonary arterial stenosis, catheterization and angiocardiography were also performed. The repair consisted of double patch closure of the septal defect, reconstruction of two atrioventricular orifices, and relief of pulmonary stenosis at all levels. In five patients with a hypoplastic pulmonary trunk, a monocusp transannular patch (four patients) or an allograft (one patient) was used for restroration of continuity from the right ventricle to the pulmonary arteries. Patch enlargement of one or both pulmonary arteries was necessary in five patients. One patient (7.1%) died early, and another late. The twelve surviving (85.8%) patients have been followed for 1.2–12.5 years after surgery (median 4.9 years, mean 5.9 ± 3.9 years). During the follow-up, reoperation was necessary for repair of residual ventricular septal defect and pulmonary regurgitation in two patients, and closure of an atrial septal defect and alteration to left atrioventricular valvar regurgitation in one patient. Seven patients are in class I of the New York Heart Association, four in class II, and one in class III. Tetralogy of Fallot associated with atrioventricular septal defect can be corrected with low mortality and good long-term results. Residual lesions, however, have a tendency to progress, especially when seen in combination. After surgery, all patients need long-term close follow-up.

We describe a recipient of an orthotopic cardiac transplant who developed severe ventricular dysfunction following an episode of sepsis. He had normal cardiac biopsies and responded to treatment with thiamine. Causes other than rejection should be considered in patients who have received a cardiac transplant and who present in cardiac failure.

Formation of thrombus in the heart is a rare event in children. Genetic risk factors determined for thrombophilia with additional exogenous risk factors may, none the less, promote cardiac thrombosis. We report on a boy without predisposing cardiac disease, heterozygous for resistance to activated protein C, who developed multiple thrombuses attached to the mitral valve which led to recurrent arterial throm boembolism. In addition this patient showed enhanced levels of lipoprotein (a).

We report a case of a fetus presenting intermittent ventricular tachycardia during midgestation, whose mother had long QT syndrome. The arrhythmia disappeared spontaneously, and the electrocardiogram of the baby after birth showed prolonged QTc. As far as we are aware, this is the first case in which ventricular tachycardia was documented during fetal life in a baby with long QT syndrome.

A 10-year-old girl required replacement of the aortic valve because of massive regurgitation caused by Kawasaki disease at the age of 3 months. Postoperatively, left ventricular function and mass improved remarkably, with reduction of both hypertrophy and interstitial fibrosis. During a follow-up period of 10 years, she has been free from any cardiac event.