The purpose of this study was to assess cardiorespiratory responses to submaximal exercise in patients with univentricular atrioventricular connection after the Fontan operation, and to evaluate whether changes occur during medium-term follow-up. Eighteen patients (age 12.1±5.5 years) underwent graded exercise test on a treadmill 2.3±1.4 year after the Fontan repair. Ventilatory gases were measured using breath-by-breath analysis. Results were compared to gender/age-matched controls. Twelve patients (age 14.2±5.4 years) were reevaluated 2.4±2.1 years after the first test. Aerobic exercise performance was subnormal in all patients during the first test. At the lowest level of exercise, the ventilatory threshold was already surpassed in 6/18 patients, while it was reduced in all other patients (p<0.001). All patients were in stable sinus rhythm throughout the test. Heart rate at all exercise levels was ±10% below normal (p<0.05). The respiratory frequency was increased at all exercise levels (p<0.001). The ventilatory equivalent for oxygen was increased (p<0.001), and the end-tidal tension of carbon dioxide was decreased (p<0.001). The ratio of physiological dead space/tidal volume was increased in all patients (p<0.001), while the normal decrease of this ratio during exercise was not observed. Upon reevaluation heart rate, respiratory rate, oxygen uptake, venti latory equivalent for oxygen, end-tidal carbon dioxide tension and physiological dead space did not change signifi cantly. Only a slight further decrease in ventilatory threshold was observed. Aerobic performance after the Fontan procedure ranges widely from just above resting metabolic rate to the lower limit of normal. Dyspnea during exercise is exacerbated by a decreased ventilatory threshold, increased physiological dead space, and decreased respir-atory efficiency. Cardiorespiratory response to exercise, nonetheless, remains relatively stable during medium-term follow-up.

Since 1985 a physical and instrumental cardiac study was carried out in 41 children with perinatal HIV-1 infection in order to assess the prevalence and natural history of cardiac involvement. Children were followed for 66 (range 8-109) months; eight of 41 died of HIV-1 related diseases. The cumulative proportion of children with cardiac manifestations at age of seven years was 14.9% (95% CI: 4.9-31.5%). Cardiac abnormalities were detected in eight of 41 patients (19.5%) and were more significantly (p=0.003) recorded in children who died of HIV-1 related diseases (five of eight, 62.5%). Left ventricular systolic function was normal in most patients during the follow-up and did not depend on zidovudine treatment. Two patients had a severe dilated cardiomyopathy and underwent subsequently a stable regression as far as the clinical and instrumental frame was concerned. Only one patient died because of cardiac disease. We conclude that in our group ofperinatally HIV- 1 infected children, cardiac involvement is relatively rare, represents a late complication compared to other related HIV-1 diseases and occurs in advanced stages of illness. Cardiac disease is therefore a hallmark of poor prognosis, but is rarely the ultimate cause of death.

For the visualization of the coronary arterial patterns in complete transposition (concordant atrioventricular and discordant ventriculoarterial connections), balloon occlusion aortography has been performed in right and left anterior oblique or in frontal and lateral views. These views, however, are often unsatisfactory because of super-imposition of the aortic sinuses. The so-called “laid-back” view is a newly developed angiographic projection in which the aortic sinuses and the intervening commissures are imaged as if they were seen from below and the front. We performed such laid-back aortography in 36 consecutive patients with an echocardiographic diagnosis of complete transposition. In 23 of 36 patients, we obtained additional aortograms in right and left anterior oblique views. For the evaluation of the diagnostic value of the laid-back view, as compared with that of the combined right and left anterior oblique views, both sets of aortograms were reviewed independently by the five reviewers who were asked to make a diagnosis and to specify their levels of confidence (“definite,” “probable,” and “possible”) for their diagnosis. Error rates were significantly lower for the laid-back aortograms than for the combined right and left aortograms (4 and 11%, respectively). An increased level of confidence was significantly associated with the laid-back aortograms. We illustrate the anatomy of various coronary arterial patterns seen by laid-back aortography.

The long-term natural progression of cardiac rhythm and the incidence of serious arrhythmias in relation to previous procedures and associated heart defects were studied in a group of 76 patients after an arterial switch operation for complete transposition and compared to a group of 24 patients who had undergone intraatrial corrections (Mustard or Senning operation). Standard and 24-hour ambulatory electrocardiograms were reviewed. Mean follow-up was 7.9 years (range 2-16) after an arterial switch and 15.2 years (range 6-26) following the Mustard or Senning procedures. One patient died after an arterial switch from pulmonary hypertension (age 9.9 years), and three patients died suddenly, presumably from arrhythmias, following atrial redirection procedures (ages 13, 18 and 20 years). Symptomatic arrhythmias were not seen after the arterial switch operation. Three patients required pacemaker implantation after atrial redirection, and a further two required medication to control tachyarrhythmias. Survival analysis of sequential electrocardiograms showed a mean maintenance of sinus rhythm during 12.9 years (95% confidence interval 11.4-14.5) after the arterial switch and 9.0 years (7.3-10.7) after atrial procedures (p=0.003). Development of heart rate was significantly different (p=0.001), with higher rates in adolescents after an arterial switch. Twenty-four-hour recordings were abnormal in five of 72 patients following arterial correction, disclosing excessive ventricular extrasystoles in four (three monomorphic, one polymorphic) and a wandering pacemaker in one. After atrial procedures, 11 of 19 were abnormal (p<0.001), with sinus or atrial bradycardia in eight, atrial flutter in two, and monomorphic ventricular tachycardias in one. Abnormal findings on either 12-lead or 24-hour electrocardiograms were seen in 22% of patients following an arterial switch procedure and 83% of patients undergoing atrial redirection (p<0.001). Sinus rhythm, therefore, is preserved longer and arrhythmias are less frequent and less severe after the arterial switch operation than after the Mustard or Senning operations.

Pericardial effusion requires attention because of its underlying pathology and its potential for producing hemodynamic compromise. It was our hypothesis that the etiology of and danger from large pericardial effusions are different at different ages. We reviewed the clinical data on 44 consecutive patients seen between 1986 and 1993. All (age range 0-19 years) had a large pericardial effusion identified by echocardiography, and 38 had fluid available for analysis, pericardiocentesis in these cases being undertaken on the basis of the clinical decision of the attending physician. History, physical examination, chest x-ray, electrocardiogram, and echocardiographic Doppler findings were evaluated retrospectively. Of the patients, 17 presented with tamponade. Of the 14 patients aged less than one year, 12 had tamponade (85%), whereas in the 30 patients older than one year, only five (16%) had tamponade. Surgical drainage was needed in seven patients. Typical physical and electrocardiographic findings (distant heart sounds, pericardial friction rub, decreased QRS voltage, and ST-T wave abnormalities) were present in less than half the patients. Etiologies of the effusion included infection in 10 (four less than one year old, and six greater than one year of age). Among these, two unusual pathogens were detected: Mycobacterium avium-intracellulaire and Candida tropicalis. In eight patients, the effusion was due to trauma (six less than one year old, and two older than one year), and in 11 it was due to post-pericardiotomy syndrome (two less than one year of age and nine older than one year). Malignancy was the cause in six (all older than one year of age), radiation in two, and collagen vascular disease, drug induced, and uremia in one each, all older than one year. Only three patients had idiopathic effusions (6%). These data suggest that, unlike adults, an etiologycan be found in most children with large pericardial effusions. Those less than one year of age are much more likely to present in cardiac tamponade.

A patient with complete transposition and a ventricular septal defect was clinically improved by an atrial septectomy at nine years but continued sepsis in the thoracotomy scar resulted in blood-stream infection with Staphylococcus aureus at the age of 24 years. A deep nylon suture was found and removed at surgical exploration. Five months later she again became febrile and had a vasculitic skin rash which responded to antibiotic therapy. At that time she was cyanosed and had a restricted exercise tolerance and was found to have aneurysmal dilatation of the pulmonary trunk. A year later she had acute chest pain with increased breathlessness, fever, hepatomegaly and pulmonary regurgitation. The fever did not respond to antibiotic therapy and she died suddenly following removal of a Hickman line. At postmortem examination, there was found to be a false aneurysm in the mediastinum due to rupture of the aneurysmal pulmonary trunk. No evidence of infection was found and it was considered that the low grade pyrexia had been due to the large hematoma within the false aneurysm.

A case of left ventricular obstruction is reported in a fetus of 21 weeks gestation. Obstruction to the left ventricular inflow and outflow tracts was documented by prenatal echocardiography and, at autopsy, the cause was found to be a large blood cyst. It is likely that the associated hypoplasia of the left heart was a result of reduced blood flow beyond this obstructive lesion.

We report an unusual case with discordant atrioventricular and ventriculoarterial connections. In addition to the commonly associated intracardiac anomalies ofventricular septal defect and subpulmonary tissue tags, a supravalvar ring was present above the left-sided, morphologically tricuspid valve. This case highlights the value of color flow Doppler echocardiography in making the diagnosis, particularly as the supravalvar ring was seen poorly angiographically.

A case is reported where unguarding of the tricuspid orifice was associated with Uhl's malformation. Echocardiographic features of the condition permitted the diagnosis during life. A therapeutic plan is proposed which involves surgical closure of the unguarded tricuspid orifice.

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.

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.

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.