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.

Primary cardiac tumors are rare in infancy and childhood. We describe the features of large intramural ventricular cardiac fibromas discovered in two children. In the first, a left ventricular apical tumor was discovered by means of echocardiography and left ventriculography in a symptomless 8-year-old boy. The intramural fibroma was successfully resected. In the other case, an 8-month-old female infant with severe congestive heart failure, sudden ventricular tachycardia and fibrillation occurred during left ventriculography. At necropsy a tumor was seen bulging in the anterior wall of the left ventricle and in the ventricular septum. Histology showed the tumor to be a fibroma.

We discuss a patient with recoarctation of the aorta who, 4 weeks after reoperation, presented with fresh haemoptysis and was subsequently found at delayed emergency surgery to have a leaking false aortic aneurysm. The case emphasizes the significance of haemoptysis following repair of recoarctation, the difficulty of reoperation due to abnormalities of the aortic wall, and the value of serial magnetic resonance imaging in demonstrating regions of different signal intensity from haemorrhages of varying age around a leaking false aortic aneurysm.

A large mass arising from the aortic valve caused supravalvar aortic stenosis in a 4-year-old girl. The differential diagnosis included an organized thrombus or myxoma. Following surgical excision, there was resolution of choreioform movements and tongue thrusting, which had been present for 1 week preoperatively.

We describe a patient with tricuspid atresia who, after a Fontan operation three years earlier, presented with severe protein-losing enteropathy. After conversion to a cavopulmonary connection, the symptoms regressed and substitution with albumin and immunoglobulin was no longer required.

A 24-year-old female without previous symptoms or known heart disease consulted a general practitioner with dyspnoea which had slowly worsened over a period of two years. She was treated for asthma, without investigations, until she presented with rapid atrial fibrillation, chest pain and pulmonary oedema necessitating admission to hospital, where she was found to have “mitral” regur-gitation (of the left-sided atrioventricular valve) and congenitally corrected transposition. At operation the grossly regurgitant left-sided morphologically tricuspid valve was replaced by a “top-hat” inverted aortic valve homograft. The procedure resulted in intermittent heart block, needing a pacemaker, and continued myocardial failure which progressed with new right-sided valvar regurgitation. She died a few months later. Post mortem examination confirmed the double discordance, and showed a fistula around the tricuspid valvar prosthesis, left atrial thrombus and changes in the mitral valve. There was also an unsuspected area of old myocardial ischaemia, associated with severe hypoplasia of the circumflex branch of the right-sided coronary artery. This was considered probably to have been of embolic origin.

We describe a case of Candida endomyocarditis in a neonate not diagnosed until autopsy. Echocardiography had demonstrated a left atrial mass. Since cardiac candidiasis seems to be occuring with greater frequency in neonatal intensive care units, echocardiography and blood cultures should be done to exclude this previously rare event.

Many of the controversies surrounding the description of ventricular septal defects arise from differences in the perspectives from which they are viewed. In this review, we analyse these defects as seen in cross-sectional echocardiographic images, correlating them with morphologic specimens. The classification we advocate, which now has a suitable pedigree, is a simple descriptive method distinguishing between perimembranous, muscular, and doubly committed types of defects. The approach is to categorise the defects as seen from the right ventricle, the usual port of access for surgeons. The term ‘perimembranous’ highlights the proximity of the atrioventricular conduction axis to the margin of the defects in which the remnant of the membranous septum forms a direct border. This system is applicable to all interventricular communications, no matter how malformed the heart may be in which they are enclosed.

Despite the close association of certain constellations of cardiac malformations with splenic status (right isomerism / asplenia, left isomerism / polysplenia), the association is not perfect. Given the risk of infection in the patient lacking splenic tissue, it seems prudent to assess the state of the spleen separately from the cardiovascular system, in order to decide on antimicrobial prophylaxis. A wide variety of tests is available to assess splenic function and/or anatomy. Counting pitted erythrocytes or Howell-Jolly bodies in the peripheral blood smear can be a good indicator of the lack of splenic function, although the former appears more sensitive and quantitative than the latter. Radionuclide spleen scans can provide both anatomic and functional information about the spleen, and may be a useful adjunctive test. Relatively poor spatial resolution, and interfering hepatic activity (even with spleen-specific scans), mitigate its usefulness in the young infant. The reliability of computed tomography and magnetic resonance imaging in detecting splenic tissue in the heterotaxy syndromes is unproven and perhaps only fair at best. They are probably most helpful as adjunctive tests. Ultrasonography offers excellent spatial resolution, portability, and lack of ionizing radiation, but is operator- and patient-dependent. Nonetheless, ultrasonography, coupled with an examination of the peripheral blood for Howell-Jolly bodies or pitted erythrocytes, is probably the set of tests with highest sensitivity and greatest convenience. Management strategies for the asplenic patient are also discussed.

A mildly symptomatic 28-year-old women with Eisenmenger syndrome became pregnant against advice, and presented at 17 weeks insisting on continuing the pregnancy. She underwent an emergency Caesarean section for pre-eclampsia at 26 weeks, delivering a live 620 g boy, who survived to leave the hospital. In the post-partum period, she presented increasing hypoxemia, despite treatment with noradrenaline, nitric oxide and prostacyclin. She died 2 weeks after delivery from pulmonary haemorrhage. Necropsy showed histological signs of severe pulmonary vascular disease, with evidence of diffuse fibrinoid necrosis, vasculitis and haemorrhage.