A human embryo of 11 mm crown-rump length (six weeks, stage 15) is described, in which a muscular defect is present in the ventricular septum, in addition to the interventricular communication normal for this stage of development. The defect consists of large confluent spaces between very loose trabeculations. Its morphology is identical to that of the central muscular ventricular septal defect well recognized in the mature heart. It is suggested that the defect was caused by widening of the sinusoidal spaces which are temporarily present in the normal developing septum.

We describe the symptoms, the techniques for imaging and the surgical therapy in a patient with divided right atrium (‘cor triatriatum dexter’) combined with atresia of the superior caval vein. In addition to peripheral venous congestion and cyanosis, macrocephaly was the most prominent sign, the high pressure in the superior caval vein disturbing the circulation of cerebrospinal fluid. Transesophageal echocardiography was the optimal technique for imaging the dividing shelf in the right atrium. Cardiac catheterization after surgical excision of the shelf and reconstruction of the junction between the superior caval vein and the right atrium excluded any residual gradient or shunt.

Implantation of the Rashkind double umbrella device for closure of the persistently patent arterial duct is now routine in many units. The use of this device in non-ductal positions, however, has been reported in only a limited number of patients, and its impact on the subsequent clinical course has not been established. A retrospective review of all such occlusions, therefore, was undertaken to address the early results and impact on clinical management. From 9/1988 through 2/1994, implantation of a double umbrella device was attempted in 21 patients (eight female, 13 male) with cardiovascular communications other than a persistent arterial duct excluding the intentional fenestrations made in the modified Fontan procedure. Median age at implantation was 2.6 years (three months-12.4 years) and weight was 11.6 kg (4.1–45) kg. Lesions included four ventricular septal defects, an aortopulmonary window, an aortopulmonary collateral artery, an atrial septal defect, four Gore-tex® shunts, and a residual persistent communication between the superior caval vein and the right atrium following either a bidirectional cavopulmonary anastomosis, or a classical Glenn operation. Eight patients underwent occlusion of unintentional residual right-to-left (six) or left-to-right (two) communications following the Fontan procedure. Implantation was accomplished in all without mortality, utilizing either 12 mm (13) or 17 mm (eight) devices. Surgical removal of the device was required in one patient four weeks subsequent to implantation. At latest follow-up (1–66 months, median four months), color-flow Doppler studies revealed complete occlusion in 17 (77%) patients. Implantation of the double umbrella in sites other than the duct is, therefore, feasible and simplified technically with a modification of the delivery system. Such novel applications of transcatheter intervention using double umbrella devices may avoid or complement subsequent surgery.

We describe a multicystic tumor of the atrial septum associated with a fatal cardiac malformation. The tumor contained bronchogenic cysts and two types of vascular hamartomas. A complex form of double outlet right ventricle was present. We speculate that this tumor formed at a critical time in cardiac embryogenesis and contributed to the abnormal formation of the heart.

In recent years, the percutaneous closure of small and medium-sized patent arterial ducts has been achieved using occluding spring coils. We describe our experience in 33 patients with this technique using a snare to facilitate the procedure. All patients had a clinically apparent patent arterial duct and underwent an attempt at transcatheter closure at a mean age of 5.6 years. In one patient, the duct was a residual lesion following surgical ligation, and in three was residual following attempted closure with a Rashkind double-umbrella. The mean narrowest diameter of the ducts was 1.9 mm. The coil embolized in two of the first four patients, and subsequent to that experience we used a snare to improve delivery. Of the 33 patients, implantation was successful in 32 (97%) using one [29 patients] or two [two patients] coils and in one by a combination of a double-umbrella device and an occluding spring coil. The mean fluoroscopic screening time for the whole group was 26.5 minutes, this time decreasing to 18.5 minutes in the last 26 patients [p <0.05]. Color-Doppler echocardiogram performed the morning after placement of the coils has shown residual leaks in nine of 32 patients [28%]. At a mean follow-up of 7.6 months, repeated imaging has shown residual leaks in four of these patients [12.5%]. No patient had a residual continuous murmur immediately following occlusion. Based on our results, we conclude that occlusion of small to medium-sized ducts using coils appears to be effective. The use of a snare to hold and manipulate the coil as it is delivered improves both control of the coil and the accuracy of its placement.

Syncope occurring simultaneously with documented normal blood pressures has been termed “psychogenic” in some patients. Two cases of basilar arterial migraine are presented, both with a diagnosis of syncope and documented normal blood pressures during the syncopal episodes. These findings highlight that syncope seen in the setting of basilar migraine is a variant from the typical form of neurally mediated syncope or cardiac arrhythmia. There is a need to consider the diagnosis of migraine when normal blood pressures and heart rates are recorded during such syncopal episodes.

We report here a patient with right cervical arch and anomalous origin of the left subclavian artery from the diverticulum of Kommerell. This uncommon abnormality can give rise to symptoms due to compression of the trachea and esophagus. Unlike right aortic arch with mirror image pattern of branching, however, this abnormality is seldom associated with intracardiac defects. Most patients are asymptomatic, and surgery is indicated only if severe symptoms are present.

Percutaneous transcatheter embolization of the arterial duct, using new detachable coils, was performed in 19 patients aged from two to 16 years. The minimal ductal diameter ranged from 0.5 to 2.7 mm. We used a 5 mm diameter coil, 8 cm in length and/or an 8 mm diameter coil, which was 10 cm in length. The coil was connected to a delivery wire with a screw system, and could be detached by rotation of the delivery wire. The coil was straightened by inserting a mandril, with loops then being formed by withdrawal of the mandril. In all patients, the coil was advanced retrogradely and either one or two coils were successfully implanted. Implantation was performed in four patients without the catheter entering the pulmonary trunk across the arterial duct. The ductal ampulla was either absent or very small in five patients, so we implanted a coil which had been cut short. Angiography revealed no residual shunting in 14 patients (74%) and trace residual shunting in five patients. Migration of the coils to the pulmonary arteries, or to a systemic artery, did not occur in any patient. Follow-up using Doppler color flow imaging showed complete closure of the arterial duct in all patients (100%), and no turbulence either in the left pulmonary artery or in the descending aorta in any patient. The use of the new detachable coil may improve the accuracy of placement of the device and reduce the incidence of its migration.

An intravascular stent was successfully implanted in a 15-year-old gid with stenosis of the left pulmonary artery subsequent to a modified Fontan correction. Complaints of tiredness, edema, ascites and enlargement of the left breast disappeared. Unfortunately, she died of a rhythm disturbance unrelated to the stent. Autopsy revealed excellent endothelialization of the stent without any thrombosis and with patency of the orifice of the branch to the upper lobe of the lung.

A left-sided hepatic vein connected to the coronary sinus is reported in a case of a 22-month-old boy with isomerism of the left atrial appendages, complete atrioventricular septal defect and azygous continuation of the inferior caval vein. The diagnosis of the anomalous hepatic vein was made intraoperatively and successful biventricular repair has been accomplished. To the best of our knowledge, this is the first communication on this peculiar entity diagnosed during life, notwithstanding Nabarro's description of a similar autopsy finding in 1903. Aspects of the development of this rare entity are discussed.

We report the case of an infant who developed severe cardiac compromise due to hypertrophic obstructive cardiomyopathy that developed while he was being treated with dexamethasone for bronchopulmonary dysplasia. This case is illustrative, as previous reports have described steroid-induced hypertrophic obstructive cardiomyopathy as being transient and benign. In this infant, the hypertrophic obstructive cardiomyopathy, waxed, waned, waxed, and waned again during two courses of dexamethasone, thus strengthening a causal link with steroid therapy. A high index of suspicion of hypertrophic obstructive cardiomyopathy as the etiology of hypoperfusion in steroid-treated infants with bronchopulmonary dysplasia is important. Serial echocardiographic monitoring of these infants is helpful in identifying cardiac compromise.

A combined procedure of a bilateral bidirectional cavopulmonary shunt and a central aortopulmonaiy shunt was performed on an 18-year-old boy with pulmonary atresia. Arterial oxygen saturation was elevated after the combined shunt, whereas the ventricular stroke work was reduced. Such a combined shunt is a useful alternative prior procedure which does not produce ventricular overload in older or higher-risk candidates for the Fontan procedure.

The case of a 27-year-old male with Ebstein's malformation combined with a ventricular septal defect is reported. On its right ventricular side the defect was covered by the dysplastic septal tricuspid leaflet, creating a small chamber in continuity with the left ventricle, but without shunt into the remaining components of the right ventricle. The ventricular septal defect and a patent oval foramen were closed operatively after a cerebral embolic event to eliminate probable sources of thromboemboli preventing the inherent risks of life-long anticoagulation for a young patient. Intraoperative morphology confirmed the preoperative findings.

A 37-year-old man with tricuspid atresia, in whom a chronic right apical aspergilloma had stimulated formation of extensive aortopulmonary collateral circulation, suffered reversal of flow within his long-standing fistulous Glenn anastomosis. By reversing the flow through the pulmonary arteriovenous fistula and raising oxygen saturation in the right atrium, the acquired aortopulmonary collateral circulation prevented the increase in cyanosis which usually occurs when fistulous changes develop late after the Glenn operation.

The increase in use of endovascular devices in the treatment and diagnosis of malformations of the cardiovascular system has created a need for another device suitable for retrieval of dislodged catheters or fragments in children. We describe the use of a snare made of nickel-titanium (nitinol) cable for retrieval of a broken pacemaker electrode lodged in the superior caval vein.

Absence of myocardial fibers in the right ventricle is the essence of so-called Uhl's anomaly, which should be distinguished from the fatty replacement producing arrhythmogenic right ventricular dysplasia of the adolescent and young adult. In this report, we describe a newborn with nearly complete absence of the myocardium of the left ventricle. The infant died on the seventh day because of myocardial incompetence of the left ventricle, which was unable to open the aortic valve.