Alfonso Casta

Atrial septal aneurysms in infants and children

Interatrial septal aneurysm (IASA) has been detected with increasing frequency since the advent of two-dimensional echocardiography (2DE). In this study, we investigated the prevalence, characteristics, and predisposing factors in IASA formation in 724 infants, children, and adolescents undergoing 2DE. Among this group, 12 patients had an IASA (prevalence = 1.7%). Eleven of the 12 patients had abnormal atrial hemodynamics as a result of either structural heart disease or significant supraventricular dysrhythmias. Follow-up 2DE performed in 10 of 12 patients, 1 to 48 months (mean 16.5 months) after the initial diagnosis, demonstrated diminution or resolution of the IASAs in those patients who had normal hemodynamics following surgical or spontaneous closure of their intracardiac defect, or medical control of their dysrhythmias. In contrast, IASAs became more prominent in those patients with persistent hemodynamic abnormalities. The high prevalence rate and subsequent involution of many IASAs in infants suggest that in the presence of abnormal atrial hemodynamics, the neonatal atrial septum may be prone to aneurysm formation because of inherent structural weaknesses of the neonatal septal tissue. In the absence of continued hemodynamic stress., the majority of these IASAs in infants resolve with maturational changes in the septum and normal cardiac growth.

Subclavian-artery-to-innominate-vein fistula presenting with congestive failure in a newborn infant

SummaryCongestive heart failure associated with intrathoracic arterio-venous fistula has not, to our knowledge, been reported previously in a newborn infant. A 2×4 mm arterio-venous fistula between the subclavian artery and innominate vein presented on the first day of life with heart failure and required surgical intervention. The uncommon location of the fistula produced potentially misleading radiologic findings.When the infant was 2 days old the fistula and a 12 mm diameter persistent ductus arteriosus were ligated; following this, heart failure regressed.

Left ventricular bands (false tendons): Echocardiographic and angiocardiographic delineation in children

Left ventricular bands were prospectively identified in 31 of 218 (14.2%) children undergoing an initial two-dimensional echocardiogram for suspected or known cardiac disease. Left ventricular bands usually coursed from the midportion of the interventricular septum to the free wall of the left ventricle. Most of the bands were detected in the apical four-chamber view. In 7 of 31, an hourglass configuration of the left ventricular cavity was noted. Diagnostic left ventricular angiography was performed in 9 of 31 patients, and in seven of nine the band was delineated. The echocardiographic and angiographic findings in children with left ventricular bands are discussed and the literature is reviewed.

True congenital aneurysm of the septum primum not associated with obstructive right- or left-sided lesions: Identified by two-dimensional echocardiography and angiography in a newborn

SummaryAneurysms of the interatrial septum are unusual anomalies that have been demonstrated by (M-mode and 2-dimensional) echocardiography in patients with complex congenital heart defects. They have also been found at autopsy in adults without heart defects. Complications have resulted from these aneurysms in adults. A true aneurysm of the septum primum was an incidental finding identified by 2-dimensional echocardiography and angiography in a newborn with an atrial septal defect.

Decorticate posture following ‘cardiac cocktail’

SummaryTwo children undergoing cardiac catheterization became irritable 1–3 hours after IM premedication that included phenothiazines. IV diazepam was administered and their irritability subsided. Within another 30 minutes they developed a decorticate posture from which they recovered shortly after IV diphenhydramine hydrochloride. The decorticate posture is believed to be an idiosyncratic reaction to phenothiazines. IV diphenhydramine may be preferable to diazepam when irritability develops following premedications that include phenothiazines.

Is there a thyrotoxic cardiomyopathy in children

We measured left ventricular ejection fraction by radionuclide angiocardiography at rest and during graded exercise in eight thyrotoxic children. Five patients were reassessed after return to a clinically euthyroid state. In the thyrotoxic state the left ventricular ejection fraction was 69% +/- 8% at rest, and increased normally by 7% to 10% during exercise in four patients but did not change significantly or decreased by as much as 8% in the other four patients. The change in left ventricular ejection fraction during exercise correlated inversely with serum concentrations of thyroxine (r = -0.82, P less than 0.02) and triiodothyronine (r = -0.88, P less than 0.01). A comparison between patients who had normal left ventricular response to exercise with those who had abnormal response while thyrotoxic failed to uncover any significant differences between their resting ejection fractions or resting and exercise heart rates and systolic blood pressures. Three abnormal and two normal responders were reassessed after return to a clinically euthyroid state. The left ventricular ejection was 64% +/- 6% at rest, and increased during exercise in all patients. Our study suggests that thyrotoxicosis causes diminished left ventricular reserve (compensated functional cardiomyopathy) in some patients, but appears to be reversible. Severity of thyrotoxicosis, as measured by thyroid hormone blood levels, may be a determining factor in the development of functional cardiomyopathy.

Echo Doppler detection of external compression of the vertical vein causing obstruction in total anomalous pulmonary venous connection

External obstruction of the pulmonary venous return was detected by two-dimensional (2-D) and pulsed Doppler echocardiography and was confirmed by angiography and at surgery in two infants with isolated supracardiac total anomalous pulmonary venous connection (TAPVC) to the left vertical vein. In both infants, the left vertical vein ascended behind the left pulmonary artery and anterior to the left main stem bronchus. This arrangement produces mechanical obstruction to both vertical vein flow and pulmonary venous drainage. Early detection and surgical correction of obstructed pulmonary venous return are necessary to prevent severe right-sided heart failure and death. Doppler echocardiography is recommended when evaluating infants with TAPVC for possible anatomic or mechanical obstruction to pulmonary venous return.

Absent right atrioventricular connexion with the left atrium connected to the morphologically right ventricle, a right-sided rudimentary left ventricle, and right juxtaposition of the atrial appendages: documentation by angiography and cross-sectional echocardiography

In this report, a 12-hr-old male infant was demonstrated to have the absent connexion variant of right atrioventricular valve atresia with the left atrium connected to the morphologically right ventricle with a right-sided rudimentary left ventricle. The aorta arose from the right ventricle and there was pulmonary atresia, the pulmonary circulation being duct-dependent. In addition there was right juxtaposition of the atrial appendages. The definitive cross-sectional echocardiographic and angiographic findings are presented.

Acquired pulmonary atresia following placement of modified Blalock-Taussig shunt in tetralogy of Fallot

A 6-month-old infant developed pulmonary atresia following placement of a modified Blalock-Taussig shunt for tetralogy of Fallot with absent left pulmonary artery. This condition was diagnosed by failure of opacification of the pulmonary trunk and the right pulmonary artery following an injection of contrast in the right ventricle. At surgery the pulmonary valve was shown to be patent and free of calcifications. Acquired pulmonary atresia may complicate the clinical course of young children following the construction of a modified Blalock-Taussig shunt.

Capsular stroke in congenital complete heart block.

A normally developed 6 year old boy with congenital complete heart block presented with right hemiparesis of one month duration. The ventricular pacemaker which was implanted at two months of age had not been functioning for four years and was not replaced because there was no history of syncope, dizziness, or decreasing exercise tolerance even though the ventricular rate was 54 beats per minute. Computerized tomography revealed an infarct in the left internal capsule. Stokes-Adams attacks have been reported as a complication in children with congenital heart block but cerebrovascular accidents as in this patient are unusual (1, 5, 6, 9).