Anomalous origin of the left coronary artery from the pulmonary trunk is rare, occurring at an incidence of 1 in 300 000. If not diagnosed and treated early, it is life-threatening. Children with the anomaly usually present in infancy with congestive cardiac failure, and are occasionally referred for cardiac transplant. We investigated the medium term outcome for patients following creation of a two-coronary arterial circulation.

Between 1992 and 2007, we diagnosed 15 patients seen at our Institution as having anomalous origin of the left coronary artery from the pulmonary trunk. Over a period of 13 years, aortic reimplantation was undertaken in 12 of these patients, who form the studied cohort.

Direct reimplantation was performed in 5 patients. In 3 cases, a tension-free anastomosis was created using a caudally based flap. In another 3 cases, an extended flap was used, while a patch arterioplasty was fashioned in the final patient. There were no deaths. Left ventricular function recovered in all but one of the patients, and all patients had a reduction in the degree of mitral regurgitation.

Among the variety of surgical techniques, transfer of the anomalous left coronary artery to the aorta is the ideal method for long-term patency and adequate blood supply. This can be achieved by creating flaps based on the walls of the pulmonary trunk and aorta, producing a dual coronary arterial supply with no mortality and low morbidity.

The mitral valvar complex is difficult to visualise accurately in only two dimensions. Three-dimensional echocardiography gives new insight into the dynamic changes of intra-cardiac structures during the cardiac cycle. The aim of this study was to study the mitral annulus in systole and diastole in normal children using three-dimensional echocardiography, and to analyse the effect of regurgitation on annular function.

Three-dimensional echocardiographic datasets, acquired in 11 consecutive subjects with mitral regurgitation, and 20 normal subjects, were analysed offline using simultaneous multiplanar review.

The mitral valvar annular area decreased in diastole, and increased in systole, in both groups. The annulus in patients with mitral regurgitation is dilated compared to normal subjects, the systolic value for those with regurgitation having a mean of 6.79 plus or minus 2.55 centimetres2/metres2, and the diastolic value a mean of 5.01 plus or minus 1.78 centimetres2/metres2, as opposed to a systolic mean value of 5.28 centimetres2/metres2 plus or minus 1.68, p = 0.091, and diastolic mean value of 3.05 centimetres2/metres2 plus or minus 0.90, in normal subjects (p less than 0.0001). The proportional change in mitral valvar annular area from systole to diastole showed a trend towards being smaller in those with mitral regurgitation, although this did not reach significance (24.8% versus 41.13%, p equal to 0.249). Analysis of subgroups of patients with moderate or severe mitral regurgitation showed mitral excursion, expressed as percentage of left ventricular length, to be significantly less than in normal subjects, at 12.78 plus or minus 5.10% versus 15.84 plus or minus 4.23% (p equal to 0.012).

Mitral valvar annular area in children decreases in diastole, and increases in systole. In those with mitral regurgitation, the annulus is dilated and the dynamic annular function is depressed.

Establishing stable and adequate flow of blood to the lungs using a systemic-to-pulmonary arterial shunt in infants with low birth weight may involve significant morbidity and mortality. We reviewed our experience with this procedure in patients weighing less than 3 kilograms.

Between June, 2002, and June, 2007, we placed systemic-to-pulmonary arterial shunts in 32 infants weighing less than 3 kilograms, the range being 1.8 to 2.86 kg, with a median of 2.5 kg. The median age at placement of the shunt was 8 days, with a range from 2 to 70 days. In 17 patients (53%), the anatomic defects had produced a functionally univentricular heart, while 15 (47%) had defects which permitted staging to biventricular repair. Patients staged to univentricular palliation were much more likely to have a circulation dependent on the arterial duct as compared with those staged to biventricular palliation (p < 0.001). The latter patients tended to have smaller pulmonary arteries, significantly the left pulmonary artery, which has a median diameter of 3.6 versus 2.0 mm, p = 0.01. In all patients a saphenous venous homograft was used as the conduit, its size ranging in diameter from 2.5 to 4 mm, with a median of 3.0 mm.

The overall hospital mortality rate for the entire cohort was 6.25%, with 2 patients dying. There was no significant difference between the two groups with regard to length of stay in intensive care or hospital. Follow-up has ranged from 3 months to 4.7 years, with a mean of 2.1 years). Of those with functionally univentricular hearts, 3 have subsequently died, along with 1 patient having a biventricular circulation (p = 0.3). All deaths occurred before takedown of the shunt. A trend toward longer survival was noted in those with biventricular as compared to functionally univentricular circulations (p = 0.06).

Systemic-to-pulmonary arterial shunts can be constructed safely in infants with biventricular physiology born with low weight. Those having functionally univentricular circulations carry an increased rate of mortality for the period of shunting. Using the saphenous venous homograft permits use of smaller grafts, which does not significantly increase the risk for thrombosis or survival when compared to previous studies using polytetrafluoroethylene grafts.

Certain congenital cardiac defects may go undetected for several years due to lack of symptoms and signs. Our purpose was to determine the prevalence of such defects among children greater than one year of age.

The study was performed on subjects diagnosed with systemic hypertension, aged from 1 to 19 years, with a mean of 12.4 years, in whom we performed echocardiography, using a standard protocol, to establish any end-organ damage or to reveal any congenital cardiac defects.

We found a congenital cardiac defect in 5 (3.5%) of the 143 children evaluated. Of these, 4 had not previously been detected, specifically Ebsteins malformation of the tricuspid valve, with moderate regurgitation, a coronary arterial anomaly, a bicuspid aortic valve, and prolapse of the mitral valve permitting regurgitation. In the other patient, we found a non-significant tiny muscular ventricular septal defect.

Our transthoracic echocardiographic investigation revealed previously unsuspected congenital cardiac defects in 4 of 143 older children, with 3 of these requiring further management by a paediatric cardiologist. A similar prevalence has also been reported in older children evaluated echocardiographically for other diseases such as insulin-resistance and leukemia. Hence, it is possible that the prevalence of congenitally malformed hearts is higher than previously reported. When clinically indicated, clinicians should more readily consider obtaining an echocardiogram to help in the identification of such malformations.

Altered levels of coagulation factors are reported in patients with functionally univentricular physiology before and following the second and third stages of reconstructive surgery. The aims of our study were to determine if such abnormalities are also present in newborns with this physiology prior to the first stage of surgical treatment.

We studied 20 neonates with functionally univentricular physiology admitted to the Children’s Cardiac Centre in Slovakia, using 20 healthy neonates as age-matched controls. Demographic characteristics, and concentration of liver enzymes, serum albumin, and complete blood count, did not differ between the two groups. Concentrations of Factor II, V, VII, VIII, Protein C, Protein S and Antithrombin were compared between the groups, and assessed as variable factors for coagulation.

In those with functionally univentricular physiology, procoagulation Factor II (p < 0.001), VII (p < 0.001), VIII (p < 0.01), anticoagulation Protein C (p < 0.001), Protein S (p < 0.001) and Antitrombin III (p < 0.001) all were present in significantly lower values compared with findings in the control group. D-dimer (p < 0.0001) and Fibrin Degradation Products (p < 0.0001) were present at significantly higher levels, but the concentration of plasminogen was significantly lower (p < 0.0001). The activated partial thromboplastin time (p < 0.012), and the prothrombin time (p < 0.0001), was significantly prolonged in those with functionally univentricular physiology compared with their controls.

The presence of abnormal coagulation factors, markers of thrombolysis in the plasma, and increased risk of bleeding, suggests activation of haemostasis, and consumption of factors responsible for coagulation, in those with functionally univentricular physiology. The question arises whether the reported abnormalities are predictive of the known abnormalities of coagulation occurring during the second and third stages of surgical repair for patients with functionally univentricular hearts.

The prevalence of mitral valvar prolapse has been reported to be between 0.6 and 21%. The goal of our study was to evaluate its prevalence in young athletes who underwent hand-held echocardiography as a screening mostly in southern California.

We retrospectively analyzed 1742 echocardiograms that were performed as a part of a cardiac screening of teenage athletes. The total prevalence of mitral valvar prolapse was calculated and stratified based on gender.

We screened a total of 1172 male and 570 female high school athletes. The echocardiographic prevalence of mitral valvar prolapse was 0.9%. The prevalence was similar in both genders, at 1.2% in male and 0.7% in female athletes.

The prevalence of mitral valvar prolapse in young athletes mostly in southern California was found to be less than 1%, and was similar in both genders.

One of the most common uses of stents in patients with congenitally malformed hearts is treatment of pulmonary arterial stenosis. Although there are reports of fractured pulmonary arterial stents, little is known about the risk factors for, and implications of, such fractures.

We reviewed angiograms to identify fractures in stents previously inserted to relieve stenoses in pulmonary arteries from 1990 through 2001 in patients who also underwent follow-up catheterization at least 3 years after placement of the stent. We undertook matched cohort analysis, matching a ratio of 2 fractured to 1 unfractured stent.

Overall, 166 stents meeting the criterions of our study had been placed in 120 patients. We identified fractures in 35 stents (21%) in 29 patients. All fractured stents were in the central pulmonary arteries, 24 (69%) in the central part of the right pulmonary artery, and all were complete axial fractures, or complex fractures along at least 2 planes. Stent-related factors associated with increased risk of fracture identified by multivariable logistic regression included placement in close apposition to the ascending aorta (p = 0.001), and a larger expanded diameter (p = 0.002). There was obstruction across 28 of 35 fractured stents, which was severe in 11. We re-stented 21 of the fractured stents, and recurrent fracture was later diagnosed in 3 of these. A fragment of the fractured stent embolized distally in 2 patients, without clinically important effects.

In situ fracture of pulmonary arterial stents is relatively common, and in most cases is related to compression by the aorta. There is usually recurrent obstruction across the fractured stent, but fractured stents rarely embolize, and are not associated with other significant complications.

The modification of placing the shunt from the right ventricle to the pulmonary arteries, also known as Sano procedure, has allegedly improved results over the short term in surgical palliation of hypoplastic left heart syndrome with the Norwood procedure. With this in mind, we reviewed autopsied specimens from neonates and children who did not survive after either a classic arterio-pulmonary shunt, or the modified procedure with the shunt placed from the right ventricle to the pulmonary arteries, so as to evaluate the pathological substrates of the remodelling of the systemic right ventricle, assessing any differences induced by the 2 techniques.

We obtained the hearts from 11 patients with neonatal diagnosis of hypoplastic left heart syndrome who died after the first or second stages of the Norwood sequence of operations, comparing them with 6 normal hearts matched for age and weight. Macroscopic, microscopic and morphometric analysis were performed on each specimen, evaluating the diameter of the myocytes, extracellular matrix remodelling in terms of fibrosis and type of collagen, and vascularization in terms of capillary density.

Hypertrophy of the myocytes was significantly increased in the hearts from patients having either a classic arterio-pulmonary or the ventriculo-pulmonary modification of the shunt compared to controls (p < 0.05). Myocardial fibrosis was increased in those having a shunt placed from the right ventricle to the pulmonary arteries when compared to the other 2 groups. The ratio of collagen I to collagen III was similar in those undergoing a classic arterio-pulmonary shunt compared to controls (0.94), but was lower in those having a shunt placed from the right ventricle to the pulmonary arteries (0.61), with an increase in collagen type III. The density of capillaries was lower in those who had undergone a classic arterial shunt when compared to the others.

We have shown greater remodelling of the ventricular myocardial extracellular matrix in patients having a shunt from the right ventricle to the pulmonary arteries when compared to those having a classic arterio-pulmonary shunt, with this remodelling progressing even after the neonatal period. This may influence a later suboptimal ventricular performance.

Surgical results after repair of tetralogy of Fallot have remained excellent for the last decades, with current long-term rates of survival over 95%. Since functional capacity, quality of life, and social interactions are basically normal in this large group of patients, pregnancy obviously becomes a relevant issue for the female subgroup. In consequence, adequate obstetrical and cardiological management of pregnancy is particularly important.

To describe the outcomes of pregnancy, and fertility, in a series of women who underwent surgery for tetralogy of Fallot in a single centre.

We obtained data from hospital records, national registries, and questionnaires on 78 women who underwent surgical correction of tetralogy of Fallot between 1972 and 1992. Of 58 women who reached an age of at least 18 years, with 45 of this cohort currently surviving, 13 having died as adults, there were 54 pregnancies in 25 women. The recorded rate of spontaneous abortion was 15%, and infertility rate was 3.4%. There have been 41 life births, with a median weight at birth of 3.2 kg. Only 1 newborn was small for gestational age, and no one was born before the 36th week. The recurrence rate of congenital heart disease was high, at 9.8%. Cardiac complications during or after pregnancy were not observed, and only one woman had pre-eclampsia.

Pregnancy is well tolerated in women with tetralogy of Fallot, and an excellent neonatal outcome is expected. The recurrence risk of congenital cardiac disease, most often tetralogy of Fallot, is high.

To determine the long-term significance of right bundle branch block on left ventricular systolic and diastolic function in children subsequent to surgical closure of ventricular septal defect.

We studied 26 children who underwent surgical closure of a ventricular septal defect 11 ± 2 years postoperatively by use of conventional and tissue Doppler echocardiography, comparing the findings to those obtained from a control group. Of those having surgical correction 14 had postoperative right bundle branch block.

Irrespective of the presence of right bundle branch block, the peak systolic velocity of the mitral ring was lower in those undergoing surgical correction, with values of 5.2 ± 1.4 cm/s in those with right bundle branch block, 5.4 ± 1.2 cm/s in those without right bundle branch block after surgical correction, and 6.6 ± 1.0 cm/s in the control subjects (p < 0.01). In terms of diastolic function, the early septal velocity of transmitral inflow divided by the early diastolic mitral annular velocity was significantly higher in children with right bundle branch block, at 12 ± 3.0 cm/s compared to 8.4 ± 1.5 cm/s in the control subjects (p < 0.01), but not significantly higher in the children without right bundle branch block after correction compared to the control group. The fractional shortening percentage was similar in both patients and control subjects. The changes noted in left ventricular function were not significantly related to age at surgery, the period of follow-up, or the surgical method.

Systolic long axis function is significantly reduced in children after surgical closure of ventricular septal defects, irrespective of the presence of right bundle branch block. Diastolic dysfunction, in contrast, was observed primarily in children with post-operative right bundle branch block.