Transesophageal echocardiography has gained an established role in the evaluation and management of the adult patient with acquired cardiac disease. Unlike the transthoracic approach, the transesophageal approach provides excellent imaging of posterior cardiac structures in virtually all patients.

We studied 28 fetal rats with atrioventricular septal defect with a common orifice produced from 300 fetuses treated with bis-diamine (200mg) on the ninth and tenth day of pregnancy. After undergoing rapid whole- body freezing on the 21st day, they were studied by means of serial cross-sectional photographs of the frozen thorax. Forty-one fetuses with normal hearts subsequent to treatment with bis-diamine served as controls. Atrioventricular septal defect was isolated in three, or was associated with either tetralogy of Fallot (n=14) or common arterial trunk (n=11). Fetal hydrops was associated with evidence of regurgitation of the atrioventricular valves. In a subgroup with larger right atrial volumes (increased by more than two standard deviation over the mean in the controls), the following parameters were significantly increased: subcutaneous edema, pericardial effusion, thickening of the left atrioventricular valve, cardiac chamber volumes and masses. The superior and inferior caval veins, and the descending aorta were significantly smaller in fetuses with atrioventricular septal defect than in controls. Fetal atrioventricular septal defect, therefore, was associated frequently with hydrops and increased volumes of the right atrium and both ventricles. Hydropic changes included cutaneous edema and increased pericardial, pleural, and peritoneal fluid.

We have studied 140 patients undergoing corrective surgery for aortic coarctation between 1952 and 1972, following them for a mean period of 23 years. Of the patients, 19 underwent surgery in infancy (age range 15 days to 190 days, mean 84 days), 52 during childhood, with a mean age of 11.8 years (range 1 years), and 69 as adults (age range 18−50 years, mean 30.5 years). All infants were symptomatic at presentation and fared poorly because of associated cardiac lesions. Their operative mortality was 11%, and mortality prior to discharge from hospital was 21%. Of the survivors, 38% required surgery for recurrent coarctation. There were three late deaths. Of the survivors, 90% were asymptomatic at long-term follow-up. Only six children (11%) were symptomatic at presentation. There was no operative or early postoperative mortality, and only three late deaths (6%). Of the 49 survivors, 96% were asymptomatic, and only six required antihypertensive medication. The recoarctation rate was 9% (four patients). In the group undergoing surgery as adults, 42% were symptomatic at presentation with 90% being hypertensive. There were two postoperative deaths and a late mortality of 22%, which was mainly related to the cardiovascular complications. None of the adults had suffered recoarctation, but 48% remained hypertensive. Our study confirms that the prognosis of patients with aortic coarctation presenting in infancy is related to presence of associated cardiac anomalies and operative complications. The prognosis in older patients, and particularly adults, is related to residual hypertension and resultant cardiovascular disease. Surgical correction reduces symptoms and improves life expectancy, regardless of the age at operation, with the best overall prognosis being obtained for those undergoing surgery as children.

To identify the echocardiographic features and natural history of dilated cardiomyopathy in fetal life, we reviewed records of all fetal echocardiograms over the 10 year period 1980–1989 to identify cases of dilated cardiomyopathy without left ventricular outflow obstruction and conducted a retrospective review of the fetal echocardiograms and clinical course of those fetuses with dilated cardiomyopathy. In addition, all fetal losses, perinatal deaths and live-born infants presenting with dilated cardiomyopathy in the first seven days of life were identified from the British Columbia Pediatric Cardiovascular Pathology Registry and from inpatient records. The pathological findings and outcome for this group were also reviewed. Marked left ventricular dilatation and dysfunction without left ventricular outflow obstruction was diagnosed in five of 1,158 fetuses undergoing feta lechocardiography. These five fetuses were correctly distinguished from two others with critical aortic stenosis and severe left ventricular dysfunction. Two of the five fetuses had additional right ventricular dysfunction and dilatation and developed hydrops, while the remaining three with isolated severe left ventricular dysfunction did not and showed normal somatic growth. All fetuses were live-born with spontaneous labour occurring in four of five cases at a mean gestational age of 36.5 weeks. Four of the five infants died, with three deaths occurring in the first week of life. During the same 10 year period, two of 2450 autopsied stillbirths (both hydropic) were diagnosed with dilated cardiomyopathy. An additional 15 neonates (including the five identified by fetal echocardiography) were diagnosed in the first week of life with dilated cardiomyopathy, congenital myocarditis or endocardial fibroelastosis. Four were hydropic (all with associated right ventricular dysfunction). Only three of the 15 survive. Fetal echocardiography can accurately distinguish dilated cardiomyopathy from left ventricular outflow obstruction. In the absence of right-sided disease or premature closure of the oval fossa, severe left ventricular dysfunction is well tolerated in utero. The prognosis for fetal dilated cardiomyopathy is very poor.

Because left ventricular mass may be important in judging feasibility of Fontan type of palliation in tricuspid atresia, this study was undertaken to generate data on left ventricular mass, volume and mass to volume ratio in newborns and infants with tricuspid atresia, native pulmonary stenosis and concordant ventriculoarterial connections prior to any surgical palliation to obtain values for “normal” left ventricular dimensions in tricuspid atresia. The left ventricle was evaluated in the apical two and four chamber view. From these two perpendicular imaging planes, we calculated mass as difference between epicardial and endocardial volume x 1.05 (specific gravity of heart muscle). Mass divided by volume at end-diastole yields the index: mass to volume. Data from 23 newborns and infants with tricuspid atresia were compared to data from 30 age-matched controls with normal hearts. In both patient and control groups, growth of the left ventricle was not linear but related to the 1.4 (volume) or 1.25 (mass) power of body surface area. The equation best describing relation between left ventricular volume and body surface area in normals is volume = 60.7(body surface area)1.4−0.2 ml; in patients with tricuspid atresia volume calculates as 96.7(body surface area)1.4+0.9 ml. In relation to normal infants, infants with tricuspid atresia had a left ventricular volume of 167 (135–206)% of normal and a left ventricular mass of 163 (132–201)% of normal. Left ventricular mass assessed in normals calculates as mass = 59.9(body surface area)1.25+1.3 grams; in patients with tricuspid atresia it calculates as mass= 117.5 (body surface area)1.25−0.9 grams. The index of left ventricular mass to volume in patients with tricuspid atresia measured 1.32, not significantly different from controls, in whom this index calculates as 1.34.

Selective coronary angiography was performed in 110 cases (aged 1–45 years) of tetralogy of Fallot. Anomalies of surgical importance were seen in 26 cases (23.6%). In 17 (15.4%) these anomalies existed alone, and in nine (8.2%) they coexisted with relatively minor anomalies. Isolated minor anomalies involving the terminal branches existed in 15 cases (13.6%). The coronary arterial anatomy was normal in the other 69 (62.7%) cases. Our study shows that coronary angiography can safely be performed in patients with tetralogy of Fallot, providing the surgeon with vital information for subsequent planning of the time and type of surgery required.

We report a case of an infant with isomerism of the right atrial appendages in whom a spleen-like nodule was found at post-mortem. The nodule, on histology, was found to be a fragment of liver. The cardiac lesions were typical of those associated with right isomerism, a complex generally described as the “asplenia syndrome.” The presence of either a spleen, or a nodule masquerading as a spleen, does not preclude an isomeric arrangement of the atrial appendages nor its commonly associated constellation of cardiovascular anomalies.

Tetralogy of Fallot with absence of the leaflets of the pulmonary valve is often complicated by marked respiratory involvement. Newer imaging modalities such as magnetic resonance imaging and color Doppler echocardiography underscore the inter-relationship of the cardiorespiratory systems in this disorder.

Division of the left atrium is a rare congenital lesion, but one which is amenable to complete surgical correction. We describe twin brothers, demonstrated to be monozygotic, who presented in infancy with cardiac failure, pulmonary congestion and mild cyanosis. Both were found to have systemic and pulmonary venous return to a common atrial chamber, separated from the left atrial appendage and the vestibule supporting the mitral valve by a typical muscular diaphragm with a single small orifice. In one twin, there was also persistence of the left superior caval vein, while, in the other twin, a remnant of the primary atrial septum was present. Excision of the diaphragm and neoseptation of the atrial chambers was performed in both infants. At 10 years after surgery, there was normal growth and development in both twins, with no evidence of recurrent obstruction.

A five day old infant underwent repair of coarctation by resection and end-to-end anastomosis of the proximal and distal segments of the arch. Four weeks later, cross-sectional Doppler echocardiography showed a tight recoarctation proximal to the origin of the left subclavian artery. A dilated proximal left subclavian artery was demonstrated, with continuous flow from it into the descending aorta. These findings were confirmed at catheterization. This “vertebral arterial steal” disappeared following transcatheter balloon angioplasty.

We describe the unusual association of a divided left atrium (cor triatriatum) with an atrioventricular septal defect with separate atrioventricular orifices in an eight-year-old asymptomatic girl. The diagnosis had been made by cross-sectional echocardiography combined with Doppler technology and color-flow mapping. These methods together demonstrate both the anatomic and hemodynamic findings.

Formation of aortic aneurysms is an important and not infrequent sequel of surgical repair of aortic coarctation. Detection is often haphazard and fatal ruptures are reported. We report a series of cases where resonance imaging identified aneurysmal dilatation of the thoracic aorta after either patch aortoplasry or subclavian flap repair of aortic coarctation. In one, rupture of the aneurysm occurred five months following the initial resonance study. Repeat imaging confirmed the clinical suspicion of rupture and successful surgical resection was subsequently performed. Magnetic resonance is a practical and reproducible imaging technique which is particularly valuable in the identification of abnormalities within the thoracic aorta following surgical aortoplasty or interventional procedures for aortic coarctation. As a method of routine surveillance in these groups of patients, it is superior to any alternative technique.

Isomerism of the left atrial appendages is often associated with cyanosis due to intracardiac structural abnormalities such as common atrium and/or anomalous pulmonary or systemic venous connections. We report two cases of children with cyanosis and left isomerism but no other lesions within the heart. In these cases, multiple small pulmonary arteriovenous malformations were found bilaterally, resulting in intrapulmonary right-to-left shunting and systemic desaturation.

A seven-month-old boy with severe respiratory distress because of absence of the leaflets of the pulmonary valve underwent banding of the pulmonary trunk. Postoperative recovery was uneventful with dramatic improvement of his general and hemodynamic status. Repair was performed successfully almost two years later when the child had grown satisfactorily and was in stable condition.

Balloon dilation of native aortic coarctation was performed in a three-month-old boy. Angiography immediately after dilation revealed a localized bulge on the anterior as pect of the descending aorta interpreted initially as an aneurysm. Angiography four months later, however, failed to detect this bulge. We speculate that the early aneurysmal appearance of the anterior wall of the aorta may have been caused by factors other than a tear in the aortic adventitia.

We report a patient with tetralogy of Fallot and pulmonary atresia who displayed an atypical aortopulmonary collateral artery extending from the ascending aorta to the pulmonary trunk. The internal thoracic artery, instead of the right subclavian artery (which was aberrant), was used as the source ofblood during unifocalization of the right pulmonary arteries to avoid the potential formation of a vascular ring. Unifocalization in the left lung was carried out by enlargement with a patch of the previously stenotic Blalock-Taussig shunt. The palliation set the scene for final intracardiac repair achieved one month after unifocalization of the right sides. The postoperative course was good and uneventful.

Repair of severe hypoplasia of the aortic arch with coarctation must be based on the individual anatomy of the lesion and, where necessary, one should take into consideration associated cardiac abnormalities. We report a surgical technique which was employed when standard procedures for reconstruction of the arch had failed to relieve the obstruction adequately. A conduit from the carotid artery to the descending aorta was used successfully in two patients to abolish residual stenosis.

We describe an eleven-year-old boy with severe retardation in physical growth and severe rheumatic valvar disease. At surgery, a sample of the atrial appendage showed Aschoff nodules, although no antecedent of rheumatic fever was recorded. The patient was HLA-DR5 positive. The case emphasizes the strong association of malnutrition with severe rheumatic heart disease in northeast Brazil.

Obstruction to the left ventricular outflow tract is not uncommon in patients with congenitally corrected transposition. A case of congenitally corrected transposition is described with significant subpulmonary obstruction which was due to a subpulmonary tag. The patient required an aortopulmonary shunt. Her clinical course was subsequently significantly influenced by arrhythmias associated with an accessory pathway. She died suddenly from an arrhythmia. Post-mortem examination confirmed a large subpulmonary tag which originated from both sides of the ventricular septum—a previously unreported finding. Careful evaluation of the nature of left ventricular outflow obstruction is important in this condition as it may alter the surgical management.