Two neonates with severe pulmonary stenosis deteriorated immediately after successful balloon valvoplasty as a result of increased infundibular obstruction. They were treated with β-adrenoceptor blockers and intravascular expansion with limited success. Phentolamine was then given, resulting in dramatic improvement. The children could be weaned from the ventilator within hours of starting this new therapeutic modality.

Doppler echocardiography allows accurate serial assessment of pulmonary valvar stenosis by measuring the velocity of the jet stream through the pulmonary valve. Between 1979 and 1997, we saw 174 patients with isolated pulmonary valvar stenosis. At admission their ages ranged from 9 days to 22.5 years. We measured the velocity over the pulmonary valve, and the thickness of the anterior wall of the right ventricle, and made a study of their electrocardiograms. We found that rapid increases and decreases occurred in almost every age-group. For patients with a trivial, mild or moderate level of stenosis, severe stenosis developed in 3, 10 and 9%, respectively. In most of the patients, 122 (90%), in whom there was more than one examination, a change in pressure gradient between −12mmHg/year and +3mmHg/year was found. Only 7 patients had an increase of more than 10mmhg per year. In contrast with our patients having aortic stenosis, these with stenosis of the pulmonary valve showed no rapid increase in early childhood. Indeed, in 58% the severity of the stenosis decreased. No correlation was found when comparing the echocardiographic measurements of the thickness of the anterior wall of the right ventricle with the voltages on the electrocardiogram. A significant relation was found however, between an increasing pressure gradient and thickened valvar leaflets (p = 0.017).

We postulated previously that variables related to pulmonary flow are independent predictors of levels of atrial natriuretic peptide in children with congenital heart disease. The aim of this study was to test this hypothesis in relation to other hemodynamic and clinical variables.

During catheterization we measured the levels of plasma N-terminal atrial natriuretic peptide prohormone in the plasma of 68 children with congenital heart disease. All had undergone complete clinical, echocardiographic and invasive hemodynamic investigations. The influence on the prohormone was analyzed for 10 different variables in a multiple linear regression model. The variability could be explained in large parts (adjusted R2 = 77.2%) by variations in atrial pressures or sizes, together with the degree of excessive pulmonary blood flow and signs of heart failure.

A value for atrial natriuretic peptide prohormone above 800 pmol/1 predicted hemodynamic imbalance (defined as elevated pressures in left or right atrium or the pulmonary arteries, and/or Qp/Qs > 1.5) with a specificity of 94%, a sensitivity of 73%, a positive likelihood ratio of 12.2, and a negative likelihood ratio of 0.29.

In conclusion, variables related to pulmonary blood flow are influential determinants of the levels of atrial natriureic peptide in children with congenital heart disease. Atrial pressures, and symptoms of heart failure are also of major importance.

We reviewed 22 cases of primary pediatric cardiac tumors followed at our institution from January 1981 through November 1997, analyzing them by subtype, age and manner of presentation, location, associated findings, interventions, and clinical course. Rhabdomyomas were the most common (11), followed by intrapericardial teratomas (2), myxomas (1), fibromas (1), hemangiomas (1), mesotheliomas (1), and rhabdomyosarcomas (1), with 4 undetermined tumors. The majority (77%) of tumors were diagnosed before the age of one year, including six prenatally. The most common presentations were murmurs (5), arrhythmias (5), and abnormal screening fetal ultrasound examinations (4). Tumors were located most frequently in the right ventricle (13) and left ventricle (7), with multiple tumors being present in 10 cases. Eight patients (36%) had associated arrhythmias or conduction abnormalities, and of the 11 patients with rhabdomyomas, tuberous sclerosis was diagnosed in 8. Eight patients underwent cardiac catheterization, including two for electrophysiologic study with radiofrequency ablation, and seven patients had complete or partial tumor resection. The follow-up period ranged from 2 months to 15 years, and there were 3 tumor-related deaths. Therefore, despite the benign histology of most primary pediatric cardiac tumors, there may be significant associated morbidity and occasional mortality. As echocardiographic techniques such as fetal ultrasonography have continued to improve, however, these cardiac tumors have increasingly been detected early before significant symptoms develop.

Congenital heart diseases have been studied much more extensively in children than in neonates. In this study, we report on the findings from 57 neonates seen from June of 1995 through June 1996 in the nursery of a large public hospital in Belém, Para, Brazil. All were routinely examined by a paediatrician just after birth, and, when indicated, these babies were referred to the cardiology unit of our Hospital for assessment by a paediatric cardiologist. Most of the diagnoses were made by means of Doppler and cross-sectional echocardiography with color flow mapping. Several abnormalities of the cardiovascular system were diagnosed. The most frequent was patency of the arterial duct. But, since many ducts closed spontaneously, ventricular septal defect was the most frequent lesion seen even in the nursery. Four defects (patent arterial duct, ventricular septal defect, atrial septal defect and pulmonary stenosis) together accounted for two thirds of all cardiac abnormalities. Associated non-cardiac anomalies were more frequent in those with simple lesions within the heart. All the babies with complex heart disease, and the majority of those designated as having significant lesions, died before they could be discharged. Several risk factors were investigated. Among maternal drugs, misoprostol emerged as having a possible teratogenic effect.

In a population-based study of 35,218 infants born alive during the 15 years from 1982 to 1996, 353(1%) were diagnosed as having a congenital heart defect, of whom 84 (24%) were diagnosed subsequent to discharge from hospital after birth (2.4/1000). Of these, 40 (48%) had a ventricular septal defect, 14 (17%) an atrial septal defect, 9 (11%) a patent arterial duct, 8 (10%) an aortic stenosis and 13 (15%) other defects. Compared with those in whom diagnosis was made before discharge, the group of patients with defects detected late had an increased prevalence of atrial septal defects, patent arterial duct and aortic stenosis, but less decreased prevalence of ventricular septal defects (p < 0.05). Median age at detection of the defects subsequent to discharge was 6 months (range 2 weeks–11 years). Seven patients (8%) presented with clinical symptoms of cardiac decompensation. The mortality rate was significantly lower in those in whom defects were detected late (1/84; 1%) as compared with those detected immediately after birth (37/269; 14%) (p < 0.05). The total rate for early detection was the same after using one clinical examination (8.2/1000) of newborns as our basic routine instead of two (7.1/1000) (p > 0.05). A substantial proportion of congenital cardiac malformations are detected after discharge from hospital after birth. Some patients with these lesions present with cardiac decompensation and are in need of medication and surgery. One clinical examination of newborns detects congenital malformations of the heart as efficient as two.

We report three cases of left ventricular aneurysm diagnosed prenatally and followed by fetal Doppler echocardiography. A review of the literature reveals a paucity of information about this rare cardiac malformation. Most of the described cases (6 out of 9) have remained asymptomatic during pregnancy and after birth. Our cases, in contrast, and three others in the literature, had an ominous prognosis presenting cardiac failure initially or during follow up and showing a dynamic evolution of the aneurysm. Cross-sectional echocardiography provides the diagnosis, revealing the thin-walled aneurysm, usually apical, to be connected by a broad neck to the left ventricle. Color and pulsed Doppler shows low velocity and to-and-fro flow in the aneurysm. Sequential fetal Doppler echocardiography detects the potential growth of the aneurysm relative to ventricular size, revealing any compromise of cardiac performance by a decreased mitral opening, reversed atrial shunting, a hypokinetic infero-posterior left ventricular wall, and a poor systolic thickening of the wall of the aneurysm. Compromise of cardiac function, and deleterious impact on development of the lungs during fetal life, may depend on the early onset, growth and location of the aneurysm, which may occupy most of the fetal chest. We discuss issues of prenatal diagnosis, sequential surveillance of the natural history, and factors of prognosis as well as myocardial histological data from one of our cases.

A 7-month-old presented with failure to thrive and a murmur. Echocardiography demonstrated a large mass in the right ventricular outflow tract, extending through the pulmonary valve. During anaesthetic induction this caused critical obstruction of the outflow tract and cardiac arrest. Pathological diagnosis showed the lesion to be a primary hemangioendothelioma. Despite surgical excision and steroid therapy, the mass continued to grow for a period of 8 weeks, but then began to regress spontaneously.

Thrombotic coronary arterial occlusion, and myocardial infarction, are rare in the newborn. We report such a happening presenting shortly after birth with cardiogenic shock, no left ventricular output and a systemic circulation dependent on flow from a patent arterial duct.

A child of a mother with maternal anti-Ro and anti-La antibodies presented antenatally with abnormal myocardial function, and was found to have a first degree heart block at birth. The extent of the abnormality in the conduction system progressed, with appearance of left bundle branch block in addition to further prolongation of PR interval. A pacemaker was implanted prophylactically, but patient has remained well, with no further deterioration in her atrioventricular conduction.

We report amiodarone-induced pulmonary toxicity in an 18-year-old who had undergone corrective surgery for tetralogy of Fallot 4 years earlier, and was treated with amiodarone because of recurrent malignant postoperative ventricular tachyarrhythmias. Toxicity was recognized on the basis of clinical features, chest X-ray, high-resolution contrast enhanced computerized tomography, and resolution of the findings subsequent to withdrawal of amiodarone and treatment with steroids. pulmonary toxicity due to amiodarone, as far as we know, has not been reported in children and young adults, and its occurence even in young adults requires wider appreciation.

The reentrant circuit of common atrial flutter is known to located in the right atrium between two anatomical barriers. Recent electrophysiologic studies have defined the tricuspid annulus as the anterior barrier, and the terminal crest and its continuation as the eustachian ridge as the posterior barrier. Construction of a bidirectional block to conduction between these two barriers by means of lesions created with radiofrequency current have been shown to be effective in ablating the flutter. We now find that surgical creation of such a block to conduction between the barriers by a simple modification of the atrial incision line is equally effective. In a 6-year-old boy, who was admitted to our hospital for closure of an atrial septal defect and treatment of sustained atrial flutter, the atriotomy was performed perpendicular to the terminal groove and extended towards the tricuspid annulus, placing some crythermal lesions between the end of the incision and the annuals. The special defect was closed using a Dacron patch. The child was free of arrhythmia both during the postoperative stay and over the initial three months of follow-up. We conclude that this simple modification of the atrial incision line provides cure of atrial flutter in children who require atriotomy for repair of congential cardiac anomalies. It may also be beneficial in preventing ‘incisional’ reentrant tachycardia.

Rhabdomyoma is the most frequent primary cardiac tumor seen during infancy. We report multiple rhabdomyomas diagnosed in twins in the neo natal period. To the best of our knowledge, this is the first instance of this happening.

One of conjoined thoracopagus twins, with separate hearts and a common pericardial sack, presented with respiratory distress because of a persistent arterial duct causing congestive heart failure in the neonatal period. Surgical ligation of the duct was performed prior to subsequent separation, with an excellent outcome.

We describe an infant with congenitally corrected transposition, ventricular septal defect and severe pulmonary stenosis. The heart occupied a midline position. Extension of ductal tissue had resulted in occlusion of the left pulmonary artery. As far as we are aware, this is the first report of an association of coarctation of the left pulmonary artery with corrected transposition.