To evaluate the potential efficacy of the combined administration of inhaled nitric oxide and oral beraprost sodium in producing pulmonary vasodilation, we studied 20 patients with pulmonary hypertension during cardiac catheterization. We also evaluated some of the mechanisms of vasodilation under these circumstances by investigating the levels of cyclic GMP and AMP in the pulmonary venous blood. A significant decrease in pulmonary vascular resistance was observed after the administration of nitric oxide (−37 ± 6%, p<0.01), and more intense decrease was observed after the combined administration of nitric oxide and beraprost sodium (−46 ± 6%, p<0.05). In addition, the ratio of pulmonary-to-systemic resistance also decreased to a greater extent with combined administration than with nitric oxide alone (−34 ± 6% vs −42 ± 7%, p<0.05). Conversely, systemic vascular resistance showed no change, neither after loading with nitric oxide nor after combined administration. Although equivalent increases in levels of cyclic GMP were observed after inhalation of nitric and the combined administration (mean 78% vs 65%), greater increases in levels of cyclic AMP were observed with the combined use (mean 15% vs 69%). Combined treatment with nitric oxide and beraprost sodium may prove markedly beneficial in patients with primary and postoperative pulmonary hypertension who do not respond adequately to inhalation of nitric oxide alone or conventional modes of treatment.

Pulmonary arteriovenous malformations are a frequent cause of progressive cyanosis after construction of a cavopulmonary anastomosis. Their formation complicates the management of children with single ventricle physiology after a bidirectional Glenn shunt or the Kawashima procedure. The key role of the liver in this phenomenon is suggested by the observation that providing modifications of the Fontan procedure which permit hepatic venous effluent to reach the pulmonary arterial circulation limit further development of the malformations. In addition, it is known that patients with end-stage hepatic failure develop pulmonary arteriovenous malformations that diminish after liver transplantation. We have begun purification of a factor derived from hepatocyte-conditioned media that is inhibitory for the proliferation of cultured endothelial cells. This substance is heat sensitive, and binds avidly to a copper-containing chromatography column. These clinical observations, and this preliminary experimental work, support the concept that hepatic-derived angiogenic factors may play a role in the development of pulmonary arteriovenous malformations after construction of cavopulmonary anastomoses.

Transvenous placement of pacemaker leads is the recommended method for permanent pacemaking in children. We evaluated 11 children with transvenous ventricular pacemaker leads by digital subtraction angiography in order to determine the incidence of venous obstruction. The ages at the implantation ranged from 4.5 to 11.2 years with a mean of 8.05±2.17 and a median of 7.9 years. The interval from implantation to the time of procedure ranged from 12 to 64 months, with a mean of 37.4±19.2 and a median of 45 months. Complete occlusion of the subclavian vein was demonstrated in two patients. We also showed significant stenosis of the venous system in five further patients. Of the 7 patients, 5 had an extensive venous collateral circulation. We did not observe obstruction of the superior caval vein, or superior caval venous syndrome. Although we did not demonstrate this syndrome, we propose future regular evaluation of all our children undergoing transvenous pacemaking. This point may be important when second or third leads are implanted.

Case records of 67 children who presented beyond infancy (57 male, 10 female) with congenital valvar aortic stenosis were reviewed to assess progression utilizing the Doppler derived peak gradient as the index of severity. Age at presentation ranged from 1–13 years, and mean follow-up was 67 ± 29 months (range 12–142 months). The patients were divided into three groups depending on the rate of progression. Those with the most rapid rate of progression were significantly older when aortic stenosis was detected and at the time of presentation compared to the other two groups, who progressed minimally or not at all (p < 0.002 and p < 0.01 respectively). There was no significant correlation between the progression of stenosis and other clinical or Doppler echocardiographic variables.

The cerebral abscess still occurs in children with cyanotic congenital heart disease. Mortality and morbidity have not yet been reduced in spite of new antibiotics and surgical techniques. We describe our experience with 185 patients having cyanotic congenital heart disease (117 with tetralogy of Fallot, 23 with complete transposition, 9 with tricuspid atresia, 4 with total abnormal venous return, 10 with double-inlet ventricle, 15 with double-outlet right ventricle and 7 with common arterial trunk) who have been followed regularly in our department. Their ages ranged between 3 years and 15 years. During the period of follow-up, cerebral abscess developed in 7 cases. Six of them had tetralogy of Fallot and one had complete transposition. One patient died before surgery could be performed. Operation was carried out in 6 patients and one died in the early postoperative stage. The others were as healthy as could be expected. Alpha-hemolytic streptococcus was isolated from the abscess in one patient, and Enterobacter in another. The abscesses were localized in the occipito-parietal region in 2 cases, the fronto-parietal region in 2 cases, and the parietal, temporal and occipital regions in one case each. Physical examination was normal apart from fever in 5 patients and, in these patients, the cause of fever could not be explained. Neurologic evaluations were all within normal range. If fever occurs unexpectedly in a patient with cyanotic congenital heart disease, therefore, it is important to rule out the possibility of a cerebral abscess.

Coexistence of atrioventricular block and Wolff-Parkinson-White syndrome is uncommon, but may occur in children, particularly with certain congenital heart defects. Our study describes 5 children who had pre-excitation and underlying disturbance of atrioventricular conduction. All of these patients had structural heart defects, consisting of discordant atrioventricular connections in three, double inlet left ventricle in one, and a ventricular septal defect associated with straddling of the tricuspid valve in the other. In 4 of the patients, the presence of atrioventricular block was initially masked by the pre-excitation. When the conduction through an accessory pathway is lost, the child may be left with atrioventricular block, which is poorly tolerated in the setting of complex cardiac anatomy. Awareness of this association of Wolff-Parkinson-White syndrome and atrioventricular block is important in understanding the natural history and the management of children with congenital heart defects that are associated with tenuous atrioventricular conduction.

Mortality from infectious endocarditis in the general paediatric population had decreased significantly over the last two decades. This is attributable to the increased awareness of the health care team in recognizing the early signs of this once fatal disease such that early antibiotic intervention is possible. Two groups, however, remain at high risk for infectious endocarditis. Advances in cardiac surgery have dramatically improved the prognosis for survival in many children with otherwise fatal congenital heart diseases. This group of children, along with the seriously ill who have central venous catheters in place, are currently at the highest risk for development of infectious endocarditis.1-5

Aortic atresia is rare in the setting of a normally developed left ventricle with a ventricular septal defect. In this combination, as far as we know, it has been described only with concordant ventriculo-arterial connections, for which seven one-stage biventricular repairs have now been described. We describe here the case of a 3½-month-old male infant with a similar combination but with discordant ventriculo-arterial connections and severe pulmonary hypertension. It was the right ventricle which achieved normal size in this arrangement, presumably because of the ventricular septal defect. One-stage biventricular correction was accomplished, employing a single pulmonary allograft, aided by massive doses of Prostaglandin El. As far as we know this is the first report of the combination of aortic atresia, discordant ventriculo-arterial connections, a ventricular septal defect and balanced ventricles. We complement our surgical account, nonetheless, with a description of a comparable specimen from our anatomic museum.

Only small groups of children, and especially infants, with hypertrophic cardiomyopathy have been analysed thus far. The clinical features, and the evolution, of this potentially heterogeneous disease, therefore, remain to be clarified. We describe the clinical profile of 35 patients with hypertrophic cardiomyopathy, 18 being younger than 1 year when first seen. Electrocardiography, chest radiography and echocardiography (m-mode, cross-sectional and color-Doppler) were performed in all patients. Ambulatory electrocardiographic monitoring was performed in 20 patients, and cardiac catheterization in 5 patients. The duration of follow-up was 6.6 years, and ranged from 1 month to 15 years. First-degree relatives of 9 infants and 13 children underwent complete echocardiographic evaluation.

Inheritance, along with presence of congestive heart failure, cyanosis, cardiac enlargement and right ventricular hypertrophy proved to be different in infants who presented with hypertrophic cardiomyiopa-thy as compared with those presenting in childhood. In contrast, echocardiography showed that the distribution of ventricular hypertrophy was similar in the two groups. Intractable congestive heart failure, which was the commonest cause of death, was an ominous prognostic sign in infants. Older children, however, tended to die suddenly. Spontaneous resolution of severe ventricular hypertrophy occurred in four infants of non-diabetic mothers within their first 6 months of age. We conclude that hypertrophic cardiomyopathy may represent a constellation of disease entities. The observed spontaneous resolution in 4 infants may represent a new form of the syndrome which carries a good prognosis.

Assessment of right ventricular volume and function is important in most congenital heart diseases before and after corrective or palliative surgery. Since transthoracic echocardiography is often substituted by transesophageal echocardiography in the perioperative setting, it is useful to compare transesophageal echocardiography with transthoracic echocardiography as performed preoperatively. We compared right ventricular volumes as calculated using these two methods from a four-chamber view in 21 children and adults with atrial septal defect. For right ventricular end-diastolic volumes of less than 70 ml, and end-systolic volumes of less than 40 ml, a close correlation was found between the techniques (r=0.99 and r=0.91, respectively), with a small degree of underestimation by transesophageal echocardio­graphy. For values larger than 70 nil and 40 ml, respectively, correlation decreased (r=0.41 for end-diastolic volumes and r=0.48 for end-systolic volumes) and underestimation of volume by transesophageal echocardiography increased. Underestimation of right ventricular end-diastolic volumes increased with increasing body surface area (r=0.74), and with progressive right ventricular enlargement (r=0.63). In patients with a body surface area of more than 1m2, the largest end-diastolic right ventricular length determined by transthoracic echocardiography was significantly longer than that derived by transesophageal echocardiography (p<0.001), whereas in smaller patients there was no significant difference between the two methods (p>0.1). If right ventricular volumes determined by transthoracic echocardiography using a four-chamber view are substituted by those obtained with transesophageal echocardiography in serial haemodynamic evaluation of patients with atrial septal defect, different correlation equations and, consequently, a different degree of underestimation by transesophageal echocardiography must be considered for large and small volumes. This increasing underestimation of larger right ventricular volumes seems to be based on foreshortening of the long cross-sectional axis of the right ventricle as seen in the transesophageal four-chamber view.

We report on a case of a newborn with echocardiographic diagnosis of anomalous origin of the right coronary artery from the main pulmonary artery. Intraoperative inspection demonstrated a proximal anomalous course of the right coronary artery but a normal origin from the right sinus of Vansalva of the Aorta. We suggest a complete investigation, including coronary angiography, when isolated and rare congenitaj malformations of the coronary arteries are suspected.

Anomalous origin of the right coronary artery from the pulmonary trunk is a rare congenital anomaly. Diagnosis is usually made at autopsy or incidentally in asymptomatic adults, but cardiopul-monary arrest and sudden death have been reported. The angiographic findings have been well described but the echocardiographic findings are less well documented.

This report describes the echo and colour Doppler findings in a 3-year-old child with an anomalous right coronary artery arising from the pulmonary trunk, as well as observations on myocardial perfusion demonstrated by nuclear angiography.

The combination of discordant atrioventricular and concordant ventriculoarterial connections is an extremely rare entity, with only 8 cases of successful surgical repair being previously reported. Although from the clinical standpoint this anomaly is similar to complete transposition, this unusual combination is clearly suited by its morpho-physiological features to an atrial rather than an arterial switch procedure. A 17-year-old boy with this malformation, associated also with severe pulmonary hypertension, underwent a palliative Senning procedure, leaving the ventricular septal defect open and banding the pulmonary trunk. Postoperatively, hypoxemia was markedly improved, and was associated with a reduction of the level of pulmonary hypertension, contributing to marked symptomatic improvement.

We describe a newborn who had echocardiogaphic features of severe hypertrophic cardiomyopathy and duct-dependent systemic blood flow. Blood flow to the upper body could be maintained only by prostaglandin El, as in the case of hypoplastic left heart syndrome. Forward flow was gradually established from the left ventricle, and the patient weaned from prostaglandin El. Although the patient's mother had poorly controlled diabetes, the patient at 24 months of age shows residual but lessened ventricular hypertrophy and increased cavity size.

Congenitally unguarded morphologically tricuspid valvar orifice is a rare abnormality, usually associated with pulmonary atresia. We describe such a case found in association with discordant atrioventricular and ventriculoarterial connections in which the aortic valve was atretic. Presentation was with respiratory distress due to severe cardiac failure after birth. Echocardiography demonstrated a dilated systemic ventricle with poor function, left atrial enlargement, and aortic atresia. The clinical findings were confirmed at autopsy.

Scimitar syndrome is a rare cardiopulmonary malformation. Presentation in infancy is usually associated with pulmonary hypertension and severe symptoms. We discuss treatment of such an infant. Two abnormal systemic vessels supplying the sequestered lower part of the right lung were embolized using catheter-inserted coils. The patent arterial duct was surgically ligated. These procedures resulted in a significant reduction of the shunt and the level of pulmonary hypertension, as well as in an impresssive improvement of symptoms.

We report the first infant case with coarctation of the aorta and mirror image right aortic arch, who presented with severe myocardial dysfunction and underwent successful balloon angioplasty for relief of coarctation, avoiding anesthetic and surgical risk. A follow-up cardiac cathetelization revealed no residual pressure gradient and continued angiographic improvement with excellent restoration in left ventricular function.