Totally anomalous pulmonary venous connection to the azygos vein is a rare congenital heart malformation in which all the pulmonary venous blood returns anomalously to the azygos vein. Among 111 consecutive patients with totally anomalous pulmonary venous connection undergoing surgical correction at our institution between June 1982 and September 1997, this malformation was present in seven cases. By echocardiography, using a subxyphoid short-axis view at the atrial level and a modified suprasternal sagittal view, the malformation was diagnosed when the pulmonary venous confluence was traced posteriorly and superiorly relative to the right pulmonary artery and right bronchus, finally reaching reach the superior caval vein. Totally anomalous pulmonary venous connection to the azygos vein was misdiagnosed in the first two patients, both by echocardiography and angiocardiography. In the subsequent five patients, a precise diagnosis was obtained by echocardiography. Echocardiography, therefore, can be considered an accurate diagnostic tool permitting recognition of totally anomalous pulmonary venous connection to the azygos vein, and permitting corrective surgery without recourse to catheterization and angiography.

A BIFOLIATE CONDITION OF THE PULMONARY: valve may occur as an isolated phenomenon in otherwise normal hearts or in association with other anomalies in congenitally malformed hearts. The former condition is very rare, whereas the latter condition is quite common.

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.

We describe three infants < 3 months of age seen consecutively with dilated cardiomyopathy who presented initially with left bundle branch block on the surface 12-lead electrocardiogram. Each infant subsequently had a poor outcome: two died and one required heart transplantation. These results suggest that the presence of left bundle branch block on the 12-lead electrocardiogram conveys a poor prognosis in infants with dilated cardiomyopathy.

A rare case of double-outlet right ventricle with intact ventricular septum diagnosed by foetal echocardiography at 21 weeks of gestation is described. Amniocentesis revealed trisomy–18. The cardiac diagnosis was confirmed at autopsy.

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.

One of the important complications of transcatheter occlusion of the patent duct by insertion of either the Rashkind double umbrella or coil devices is intravascular haemolysis, particularly the severe form which occurs in 0.5–0.6% of cases. The incidence of subclinical or mild intravascular haemolysis including morphologic changes in the red cells, however, is not known, especially in the group of patients with residual shunt. We studied laboratory and haematologic changes in 37 patients subsequent to transcatheter occlusion. Their ages ranged from 2 years 2 months to 11 years 8 months (6.2 + 2.7 years), and the duration of occlusion had ranged from 1 month to 2 years 11 months (1.24 + 0.89 years). In 30 cases we had used the Rashkind double umbrella device, and nine of these patients (30%) had a residual shunt. The other seven ducts were closed with detachable coils, and five of these cases (71%) had residual shunting. There was evidence of mild or subclinical intravascular haemolysis in almost half the cases, although no patient developed acute intravascular haemolysis. Fragmented red cells, blur cells and spherocytes from blood smears were found in up to one-third of the overall series, with no differences found according to persistence of shunting. Haemosiderin was found in the urine of the group with residual shunting and in 13–23% of those with complete occlusion. Serum hypohaptoglobin was found in 9–13% of the patients with a residual shunt, and in 9–23% of those with complete occlusion. Four of the patients had haptoglo-bin levels <50 mg/dl. There were no patients with reticulocytes counts >2% in the blood smears. We conclude that subclinical or mild intravascular haemolysis, along with morphologic changes in the red blood cells, are significant findings subsequent to occlusion of the patent arterial duct with either the Rashkind double umbrella or detachable coils.

Double outlet left ventricle is an extremely rare anomaly. Until recently, the diagnosis was usually established by angiography or at postmortem. There are only a few reports describing the echocar-diographic findings in this lesion, and as far as we know, no report showing the anatomy as well as the velocity and pattern of flow by color Doppler echocardiography. The patient reported here underwent surgery at the age of four years, when an aortic homograft was placed between the right ventricle and the pulmonary trunk. This biventricular repair had to be changed into a Fontan-type procedure, 15 years later since the hypoplastic right ventricle did not grow adequately.

The natural history of a ductal aneurysm detected prenatally is presented. Neurologic concerns in the neonate and absence of cardio-respiratory compromise permitted serial echo demonstration of constriction, thickening and subsequent closure with formation of thrombus in the aneurysm.

Isolated anomalous drainage of the right superior caval vein to the left atrium is a very rare cause of cyanosis in the newborn. Herein, the cross-sectional echocardiography and colour Doppler findings of this malformation are described.

Reported is a case with a rare association of divided left atrium, supramitral stenosing ring of the left atrium, connection of the left superior caval vein to the roof of the left atrium, unroofed coronary sinus with an interatrial communication at the mouth of the unroofed sinus and ventricular septal defect. The need for a complete echocardiographic examination in the presence of pulmonary venous obstruction is emphasized. Surgery was successful in spite of significant preoperative pulmonary hypertension.

Isomerism of the atriums has often been described with various complex cardiac malformations. As far as is known, a case of ‘ventricular isomerism’ has never been recorded. Described is a specimen where, on the basis of morphological criterions, there are two right ventricles.

We discuss the clinical course of aneonate with idiopathic hypertrophic cardiomyopathy who showed rapid progression of stenosis of the right ventricular outflow tract and reduction in size of the right ventricular cavity due to thicken ing of the ventricular septum. Medical treatment proved unsuccessful. We suggest that balloon atrial septostomy combined with construction of a Blalock-Taussig shunt may be effective in view of the hemodynamics, which mimic pulmonary atresia with intact interventricular septum.

Cyanosis as a result of right-to-left shunting across a ventricular septal defect is commonly encountered in patients with congenital heart disease when systolic pressure in the right ventricle exceeds that in the left ventricle. Reported is the case of a child who remained cyanosed after surgical correction of pulmonary atresia despite right ventricular systolic pressure being lower than left ventricular pressure. Colour-flow Doppler showed a residual ventricular septal defect, with right-to-left shunting in diastole alone.

A 28-year-old female patient with complete transposition, ventricular septal defect and persistence of the arterial duct underwent a palliative arterial switch procedure in 1976 at 7 years of age. Therefore, she has survived for 22 years and lives a near normal life. She is married, has been counselled against pregnancy and has increasing cyanosis with the typical features of the Eisenmenger syndrome.