Controversy remains about the growth of the pulmonary arteries following a bidirectional superior cavopulmonary anastomosis in children with complex cyanotic congenital cardiac malformations. This is partially due to the morphological heterogeneity of the patients, and partially due to methodological differences in series published so far. It is further complicated by the variable use, in different centres, of additional sources of pulmonary blood flow. We believe that the fate of these arteries preoperatively is significantly influenced by the amount of pulmonary blood flow and the initial size of the arteries. Separate assessment of the pulmonary arterial development postoperatively is recommended for those who, initially, had relatively small as opposed to larger than normal pulmonary arteries. Measurement of the diameters of both pulmonary arteries just prior to their first point of branching, together with the use of Z-score evaluation rather than the Nakata index, is discussed. It remains to be established whether, over time, the bidirectional cavopulmonary anastomosis is effective in developing adequately the pulmonary arteries in preparation for an ultimate total cavopulmonary connection, or even as isolated long-term palliation. A prospective, multi-institutional study involving sequential non-invasive assessment of pulmonary arterial development (using, for example, magnetic resonance imaging) is required to solve the outstanding problems.

Fifteen patients requiring palliation for tetralogy of Fallot were treated by balloon dilation because of hypercyanotic spells. The mean age at dilation was 1.9 ± 0.7 years (range 0.5 – 3), and the mean weight 9.8 ± 2.1kg (range 6.0 –13.5). Dilation of the outflow tract was combined with dilation of the left and/or right pulmonary arteries in 5 patients. Successful dilation was achieved in 12 patients (80%), but failed in 3 patients with hypoplastic pulmonary arteries. In one patient, the stenosis of the right pulmonary artery could not be dilated because of a very sharp angle at the site of the stenosis. Two of the 3 patients in whom the procedure failed died of severe cyanotic spells within 24 hours of the unsuccessful procedure. No major complications occurred during or after the procedure in the cases undergoing successful dilation. The arterial oxygen saturation increased significantly, from 71 ± 5.7% to 89 ± 3.9%, immediately after the procedure (p < 0.005). During a period of follow up of 6 ± 3.7 months (range 1 – 13), the procedure was repeated on 3 occasions, and successfully accomplished in 2 of these. In conclusion, balloon dilation is a satisfactory palliative procedure for tetralogy of Fallot in those units in which total correction is not performed under 2 to 3 years of age.

Balloon valvoplasty was undertaken in 27 patients with tetralogy of Fallot for first-stage palli-ation. Indications were arterial saturation of oxygen <80%, hypoxic spells and duct-dependant pulmonary perfusion. The dilation was performed following diagnostic heart catheterization. Saturations improved from 75% ± 8.5 before valvoplasty to 85% ± 8.4 after the procedure, and worsened little to 83% ± 9.6 at follow-up after 3.4 months. The pulmonary valvar orifice was hypoplastic in most patients (Z = — 3.3 ± 1.2), and did not change after the procedure. The cross-sectional area of the pulmonary arteries was diminished initially, reflected by a Nakata index of 186 ± 95 mm2/rn2. After valvoplasty, we found widening of the vessels (Nakata index 225 ± 100 mm2/m2). At follow-up no further growth of the arteries was observed (Nakata index 209 ±109 mm2/m2). The procedure was complicated by hypoxic spells in three patients which were controlled by intravenous propranolol, and deep venous thrombosis in four patients. Our data demonstrate that balloon valvoplasty is feasible for initial palliation in patients with tetralogy of Fallot. It does not, however, produce growth of the pulmonary arteries or of the pulmonary valve.

Balloon dilation of the pulmonary valve was performed in 54 patients with tetralogy of Fallot with severe cyanosis, high haematocrit and severe valvar pulmonary stenosis. Clinical, echocardiographic, angiographic, and haemodynamic data were analyzed before and after the procedure. After balloon dilation, the systemic oxygen saturation increased from a mean value of 66% to 85<%. The mean value of the haematocrit before dilation was 55 + 13, and decreased to 47 after dilation (p < 0.002) in 2 months follow-up. Balloon dilation increased the size of the pulmonary valvar orifice from a mean value of 9 + 5mm to 11.5 + 2mm (p < 0.005). The mean Z score of the pulmonary valves, which was -3 + 1.3 before dilation, increased to -1.1 + 1.1 immediately after the procedure (p < 0.05).

The size of the right and left pulmonary arteries increased after dilation from 9mm to 10 mm, and from 8.7 + 2.4mm to 9.8 + 2.3mm, respectively (p <0.05). The comparable mean Z scores increased from -2.8 + 1.9 SD to -1.8 + 1.4 SD, and from -2.4 + 1.9 SD to -1.5 + 1.6 SD for the right and left branches, respectively (p < 0.05).

In patients with stenosis at the bifurcation of the pulmonary trunk and hypoplasia of the left artery, successful dilation of the pulmonary valve lead to an increase of flow and improvement in size of the hypoplas-tic segment.

In conclusion, initial balloon dilation of the pulmonary valve in tetralogy of Fallot resulted in increase of the Z score for the pulmonary valve and improved antegrade pulmonary blood flow, inducing growth of the pulmonary arteries and ameliorating the anatomic and physiologic preoperative condition.

We investigated the catheterization and angiographic findings of 26 patients with Williams‘ syndrome to evaluate the natural course of supravalvar aortic stenosis and peripheral pulmonary arterial stenosis. The severity of the stenosis was correlated with age and body surface area in terms of the pulmonary arterial index, right ventricular systolic pressure, sinutubular ratio(ratio of measured to mean normal diameter of sinutubular junction), and systolic pressure gradient across the sinutubular junction. In patients with pulmonary arterial stenosis (n=20), right ventricular systolic pressure tended to decrease, and pulmonary arterial index increased, with increase in age and body surface area. Between the groups with and without pulmonary arterial stenosis, there was significant difference in age (mean 4.70 vs. 9.87, p = 0.019), body surface area (0.62 vs. 1.16, p = 0.002), pulmonary arterial index (152 vs. 317, p=0.002) and right ventricular systolic pressure (73.9 vs. 33.0, p=0.006). As all patients showed similar diameters at the sinutubular junction regardless of age and body size, sinutubular ratio decreased with increases in age and body surface area. The group with abnormal coronary arteries (n = 7) had smaller sinutubular ratio (0.46 vs. 0.61, p=0.021) and higher pressure gradients between the left ventricle and the aorta (67.6 vs. 42.2, p=0.023) than did the group with normal coronary arteries. Stenosis of a coronary artery, or a branch of the aortic arch, was observed only in three patients with diffuse aortic stenosis.

Our results suggest that, with time, peripheral pulmonary arterial stenosis tends to improve, and supravalvar aortic stenosis to progress. Failure of growth of the sinutubular junction might be responsible for the progression of the aortic lesion. Progression of the aortic lesion may be associated with involvement of the coronary arteries.

Continuous monitoring of cardiac output in neonates would be of considerable benefit but, as yet, there is no practical method to achieve this aim. We have now evaluated the feasibility of using an intra-aortic Doppler probe. We introduced a pulsed Doppler probe of 0.46 mm diameter via the umbilical artery in two term and four preterm neonates. Indications in all patients for umbilical arterial catheter is always an unstable cardiopulmonary state. Body weights were between 770 and 3340 g. Velocities of blood flow in the thoracic aorta were continuously recorded to estimate cardiac output on-line for 12 h. No complications were encountered. It proved possible to derive high-quality Doppler curves. The received Doppler signal was stable but it proved sensitive to pathophysiologic changes in flow. Mean velocity of flow in the descending aorta was 16.4 cm/s (range 13.3_19.0 cm/s). We quantified flow by multiplying the mean velocity of the flow by the cross-sectional area of the descending aorta. Calculated mean flow was 135 ml/kg/min (range 111–179 ml/kg/min). These values are consistent with those measured by transcutaneous Doppler, and it should not be raised by left-to-right ductal shunts. This pilot study proved the feasibility of continuous monitoring of cardiac output. The technique should prove of great value in those infants with unstable circulatory conditions, and can be used even in infants with extremely low birth weights.

Banding of the pulmonary trunk is an important surgical procedure for patients who have con genital cardiac malformations with unrestricted pulmonary flow. We propose a new concept for determining in such circumstances the most appropriate length of the band used to constrict the pulmonary trunk in preparation for a Fontan operation. We studied 14 patients undergoing banding of the pulmonary trunk and measured the following parameters: diameter of aorta, diameter of pulmonary trunk, length of pulmonary arterial band and maximum flow velocity across the banded segment. We calculated an index from our orig inal parameter based on the formula; length of band/(diameter of aorta diameter of pulmonary trunk). The diameter of aorta was 9.5 ± 1.4 mm, and that of the pulmonary trunk was 9.6 ± 2.3 mm. The length of the band was 16.5 ± 3.4 mm, giving a calculated index of 0.188 ± 0.038. The maximum flow velocity was 4.02 ± 0.46 m/s. No correlation was found between the length of the band and body weight, and also no correlation was found between the length of the band and maximum flow velocity. The calculated index had a negative correlation with the maximum velocity of flow across the band (y = -8.13x + 5.56, R = 0.74, p < 0.01). We believe that the proposed index is a useful guide in determining the length of a pulmonary band when preparing patients for a Fontan operation.

We describe three infants < 3 months of age seen consecutively with dilated cardiomyopathy who presented initially with left bundle branch block on the surface 12-lead electrocardiogram. Each infant subsequently had a poor outcome: two died and one required heart transplantation. These results suggest that the presence of left bundle branch block on the 12-lead electrocardiogram conveys a poor prognosis in infants with dilated cardiomyopathy.

One of the important complications of transcatheter occlusion of the patent duct by insertion of either the Rashkind double umbrella or coil devices is intravascular haemolysis, particularly the severe form which occurs in 0.5–0.6% of cases. The incidence of subclinical or mild intravascular haemolysis including morphologic changes in the red cells, however, is not known, especially in the group of patients with residual shunt. We studied laboratory and haematologic changes in 37 patients subsequent to transcatheter occlusion. Their ages ranged from 2 years 2 months to 11 years 8 months (6.2 + 2.7 years), and the duration of occlusion had ranged from 1 month to 2 years 11 months (1.24 + 0.89 years). In 30 cases we had used the Rashkind double umbrella device, and nine of these patients (30%) had a residual shunt. The other seven ducts were closed with detachable coils, and five of these cases (71%) had residual shunting. There was evidence of mild or subclinical intravascular haemolysis in almost half the cases, although no patient developed acute intravascular haemolysis. Fragmented red cells, blur cells and spherocytes from blood smears were found in up to one-third of the overall series, with no differences found according to persistence of shunting. Haemosiderin was found in the urine of the group with residual shunting and in 13–23% of those with complete occlusion. Serum hypohaptoglobin was found in 9–13% of the patients with a residual shunt, and in 9–23% of those with complete occlusion. Four of the patients had haptoglo-bin levels <50 mg/dl. There were no patients with reticulocytes counts >2% in the blood smears. We conclude that subclinical or mild intravascular haemolysis, along with morphologic changes in the red blood cells, are significant findings subsequent to occlusion of the patent arterial duct with either the Rashkind double umbrella or detachable coils.

We discuss the clinical course of aneonate with idiopathic hypertrophic cardiomyopathy who showed rapid progression of stenosis of the right ventricular outflow tract and reduction in size of the right ventricular cavity due to thicken ing of the ventricular septum. Medical treatment proved unsuccessful. We suggest that balloon atrial septostomy combined with construction of a Blalock-Taussig shunt may be effective in view of the hemodynamics, which mimic pulmonary atresia with intact interventricular septum.

Closure of atrial septal defects be means of intravenous catheterisation has been undertaken using a variety of devices as an alternative to surgical closure.1–5 We describe the first case, to the best of our knowledge, of infective endocarditis complicating a successful transcatheter closure. This highlights the potential risk of this procedure, and emphasises the need for appropriate antibiotic prophylaxis until complete endothelialization of the device has occurred.

Division of the morphologically left atrium is a rare abnormality, constituting around 0.1% of congenital heart disease. It may coexist with other cardiac abnormalities, and this association may obscure its recognition. We report an uncommon association with tricuspid atresia and discordant ventriculo-arterial connections, the latter dominating the clinical picture. Accurate diagnosis was made by transthoracic cross-sectional echocardiography, and the patient was referred to surgery without need for cardiac catheterisation.

We present a 14-year-old female with a massive aneurysm of the pulmonary trunk occurring secondary to bacterial endocarditis of the patent arterial duct. Echo Doppler imaging, magnetic resonance and angiography confirmed the diagnosis. On intermediate follow-up after surgical correction, the echo studies and cardiac catheterization disclosed no residual lesions. The patient is now asymptomatic.

The teratogenic properties of phenytoin, including cardiac malformations, have been previously documented. We report one patient with foetal hydantoin syndrome and atrioventricular septal defect with common atrioventricular junction but separate right and left atrioventricular valves, an association that has not been described, to the best of our knowledge.

Aortopulmonary septal defect and tetralogy of Fallot is a rare combination. We report a case of tetralogy of Fallot, non-confluent pulmonary arteries with the left arising from the arterial duct, and a large aortopulmonary septal defect diagnosed by echocardiogram and confirmed by cardiac catheterization.

The majority of live births with severe congenital cardiac malformations that do not undergo intervention of some form die prematurely. The population of Malta provides an ideal closed cohort from which to monitor trends. We have observed a significant declining specific mortality from congenital heart disease, and circumstantial evidence relates this decline to the introduction of new techniques used in the diagnosis and treatment of these malformations.