We describe post azygos obstruction of the superior caval vein following repair of a mixed variety of totally anomalous pulmonary venous connection. Balloon dilation was immediately successful, and has provided persistent relief at a follow-up of 20 months.

The structural complexity of the right ventricle has made quantitative evaluation difficult. Conventional cross-sectional echocardiographic methods are limited by geometric assumptions and the position of the planes used for imaging. Previous reports have demonstrated accurate three-dimensional echocardiographic quantitation of the right ventricle in-vitro and in experimental animals. We adapted a previously described method for three-dimensional reconstruction of the left ventricle to compute right ventricular volume and ejection fraction in a clinical setting.

We examined 29 patients aged from 2 to 42 years with pulmonary hypertension, by three-dimensional echocardiography and resonance imaging. Correlation and agreement were calculated for volumes and ejection fractions. Three-dimensional echocardiographic reconstruction, when compared to resonance imaging, yielded r values of 0.95 and 0.93, and mean differences (bias) of 31% ± 19% and 33% ± 18%, for systolic and diastolic volumes respectively. Interobserver variability was low (12.9% and 8.0%). Ejection fraction as calculated by three-dimensional echocardiography showed close agreement with resonance images (bias=l% ±7%). Three dimensional echocardiography is now a method of measuring right ventricular ejection fraction in the clinical setting which produces results comparable to those of resonance imaging. Volume measurements correlated well for systole and diastole, but consistently underestimated values produced from resonance images.

Anomalous origin of the coronary arteries is a well-known phenomenon, but is less well reported in world literature. We present our findings in a one year old boy in whom there was bifid, or “y”, origin of the right coronary artery, co-existing with a fistula from the right coronary artery which opened into the right ventricle.

A patient is reported in whom cardiac strangulation occurred through a lateral pericardial defect surgically created for repair of congenital heart disease. To avoid this potentially fatal complication, the inferior rim of an iatrogenic lateral pericardial defect should be tacked down to preclude cardiac herniation.

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.

The Fontan operation was initially designed to provide a “physiological correction” of tricuspid atresia, but its indications have been extended to more complex forms of congenitally malformed hearts. In selected patients with two adequately sized ventricles, but highly complex intra cardiac anatomy which makes a biventricular repair more hazardous and which would require multiple reoperations, a univentricular repair using Fontan's principle has been proposed. Between 1978 and 1992, a modified Fontan operation was carried out in 34 such patients. Of the patients. 13 had double outlet right ventricle and 21 had transposed great arteries. Anomalies of ventricular loop were present in 14 patients. A non-committed ventricular septal defect was found in 26 whilst multiple defects were present in 10. All patients had anomalies of the atrioventricular valves, with straddling of the tricuspid valve,11 and/or of the mitral valve11, and abnormal tricuspid5, or mitral4 cordal attachments. Two patients had mild tricuspid hypoplasia, 1 had mitral hypoplasia and 3 had a cleft mitral valve. Fourteen patients had anomalies of both atrioventricular valve but only 6 had mild atrioventricular valvar incompetence. Pulmonary stenosis or atresia was seem in 26. All the patients had at least one or more (mean 2.35) intracardiac anomalies that preculded a biventricular repair. The Fontan operation was performed at a median age of 4.75 years. There were 4 early (11.4%, 70% CL: 5.1–19.8) and 2 late deaths, with a 4 year actuarial survival rate of 79.6±8.1%. Univariate analysis did not reveal any risk factor for early of late death. Follow-up at a mean interval of 3.6 ±2.6 years was available in all but 1 survivor. There were 6 early and 2 late reoperations, and 3 patients required implantation of a pacemaker. In conclusion, the modified Fontan operation can provide satisfactory early and mid term results in patients with two ventricles but with complex intracardiac anatomy which precludes a biventricular repair.

We describe a neonate with tricuspid atresia, absent pulmonary valve, right ventricular hypoplasia, small venrricular septal defect, left ventricular ourflow tract obstruction and a patent arterial duct. These finding were diagnosed by echocardiography and confirmed by cardiac catherterization and postmortem examination. This is a unique report of a functionally single ventricle variant of absent pulmonary valve syndrome and biventricular outlet obstuction.

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.

In 3 patients with isomeric morphologically left atrial appendages, univentricular atrioventricular connection, concordant ventriculoarterial connections, bilateral superior caval veins, with the left one draining via the coronary sinus, together with absence of any communicating vein, interruption of inferior caval vein with drainage via a right-sided (n=2) or left-sided (n=l) azygos vein, the hepatic venous blood was rerouted via the large coronary sinus into the pulmonary arterial circulation. In a fourth patient with similar pathology, having interruption of the left-sided inferior caval vein with drainage to the left-sided superior caval vein via a left-sided azygos vein and a large communicating vein, the pathway from the left superior caval vein to the coronary sinus was correspondingly small. An extracardiac conduit was therefore constructed between the hepatic veins and the left pulmonary artery so as to reroute the hepatic venous blood into the pulmonary arterial circulation. At a mean follow-up of 8.5 months, all patients are clinically well and none of them have developed pulmonary arteriovenous malformations. To avoid the latter complication in Fontan physiology, especially in the setting of an interrupted inferior caval vein with drainage via the azygos vein, we believe that it is preferable to reroute the hepatic venous blood into the pulmonary circulation.

We describe a case of anomalous origin of the right subclavian artery from the right pulmonary artery detected by cross-sectional echocardiography in an infant with type B interruption of the aortic arch. Preoperative recognition and surgical reimplantation of the right subclavian artery are important to prevent subsequent subclavian steal syndrome.

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.

We report experimental use of a new device for transcatheter closure of atrial septal defects. The device was implanted in surgically created atrial septal defects in two pigs. The devices were easy to implant, and the procedures were short. Both devices were implanted correctly in the desired position, and closed the defect completely. No procedural complications nor problems were encountered. The technique is certainly worth trying in the clinical setting. The device appears to be particularly favourable for use in children because it can be implanted through a 7 French sheath.

The concept of sequential analysis of congenitally malformed hearts is now widely accepted, although some aspects remain controversial. In this review, we analyse the evolution of the scgmental approach, showing how our own concepts of analysis have chaanged little since our initial description, heart dicrates the presence of the atrial, ventriclar, and arterial segments, Analysis depends upon recognizing the morphologic differences of the components within each segment, and then how these parts are joined together across the atrioventicular and ventriculo-arterial Junctions. The distinction of chambers and arterial trunks follows the principle of the morphologic method, namely that scructures be identified on the basis of their own intrinsic morphology, and not according to other features which themselves might be variable. Differentiation of junctional arrangements as opposed to segmental stucture resolves ongoing controversics such as the nature of “univentricular hearts”.Analysis in the fashion proposed shows that there is nothing intrinsically complicated with malformed hearts.

A patient with aortitis presented in infancy with dermatitis gangrenosa. Coarctation of the aorta was subsequently produced by granulomatous tissue. We report 30 years of medical observation.

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.

A 5-week-old male, born with an aneurysm of the arterial duct forming part of a vascular ring, underwent successful surgery without cardiopulmonary bypass. From our search of the literature, it seems, that this combination of rare congenital malformations has not previously been reported.

Previous studies have demonstrated the individual association of subpulmonary tissue tags, dissection of the pulmonary trunk and primitive neuroectodermal tumors with congenital heart disease. We are reporting a case of double inlet left ventricle with all these associations as it is exceptionally rare for all features to be manifest in one individual.

A 3-year-old boy developed acute pulmonary edema following 6 months of recurrent fevers and thrombocytosis. Echocardiography demonstrated moderate-to-severe insufficiency of both mitral and aortic valves. Angiography and intravascular ultrasound were used to diagnose this unusual presentation of Takayasu's arteritis. To the best of our knowledge, this is the first histological demonstration of acute and chronic inflammatory changes in the mitral valve in these circumstances.

We describe an unusual case of congenitally corrected transposition in which left-sided obstructions included a supravalvar tricuspid ring, a parachute deformity of the valve itself, and coarctation of the aorta. Surgical correction was performed in 3 stages. Initially we repaired the coarctation and banded the pulmonary trunk. Then we removed the band and, finally, attempted unsuccessfully to replace the tricuspid valve. When coarctation of the aorta occurs in congenitally corrected transposition, it is always wise to search carefully for abnormalities of the tricuspid valve.