Atrial fibrillation in children.

Atrial fibrillation is rare in children. Previous reports associated it with severe rheumatic heart disease and a poor prognosis. This review is of the unique experience of 35 cases of atrial fibrillation in children in the past 22 years; 23 patients were boys. The age of onset ranged from 1 day to 19 years (average, 8 years). Associated cardiac conditions were severe rheumatic mitral regurgitation (3 cases), cardiomyopathy (5), atrial tumors (2), infective endocarditis (1), paroxysmal atrial tachycardia of infants (4), idiopathic paroxysmal atrial fibrillation (1), Marfan's syndrome with mitral regurgitation (1), endocardial fibroelastosis (1), and structural congenital heart malformations (17). Surgical correction of congenital heart lesions was directly related to the development of atrial fibrillation in 14. Varying arrhythmias of the sick-sinus syndrome were observed in five children. The atrial fibrillation was paroxysmal or transient in 21 patients and persistent in 14. Treatment depended on the underlying condition. Digoxin was used in all cases and cardioversion attempted in ten; no patient was given anticoagulants. Three children had cerebral emboli, with residual defects. Eighteen patients are known to be alive, 13 are dead, and 4 are lost to follow-up. Atrial fibrillation in childhood is an indication for complete investigation of the patient and for the institution of treatment appropriate to the underlying disease.

Facial and immunological anomalies associated with tetralogy of Fallot.

Dysmorphic facial features were present in 9 of 31 children with tetralogy of Fallot anatomy (29%). These anomalies included hypertelorism, low-set ears, small mouth, short philtrum, and micrognathia. Ten children had pulmonary atresia, 13 (42%) had a right aortic arch, and 13 had extracardiac congenital anomalies. There were 16 children in the series (52%) who had hospital admissions for important or recurrent infections, and 18 who had immune deficiency: low levels of T lymphocytes were found in 9, low levels of complement in 8, and low immunoglobulins in 3. Embryologically, the cardiac outflow tracts, the aortic arch, the face, and the thymus develop at the same time, and all receive migrating cells from the neural crest. Teratogenic factors possibly produce this constellation of anomalies, which is in the spectrum of the Di George syndrome (third and fourth pharyngeal pouch syndrome). It is of importance for the management of such children, that their immune deficiency be recognized and treated appropriately.

Oral health of children with congenital cardiac diseases: a controlled study.

Congenital cardiac disease (CCD) is one of the most common developmental anomalies in children. Affected children require special care in dentistry because of their susceptibility to infective endocarditis from oral infections, yet little information is available on the oral health of children with CCD. The present study, which investigated 39 children with CCD and 33 healthy control siblings, showed that CCD children generally suffered poorer oral health. In patients with primary dentitions, 52% of CCD children had enamel hypoplasia, compared with only 23% in the control group. In addition, CCD children had significantly more teeth with untreated dental decay (mean dmft 4.2 vs. 2.3), and more endodontically treated teeth. Children with CCD also had less than optimal professional and home dental care. Only 31% had professional advice regarding increased preventive dental health behavior, and only 15% used fluoride supplements, although the children resided in a nonfluoridated area. Furthermore, significantly fewer CCD children had parental help with tooth brushing compared to control children. This study shows that children with CCD should be targeted for vigorous preventive dental care.

Immunological detection of cardiac glycosides in plants.

Australian native and introduced plants were examined, using digoxin immunoassays, to detect the presence of cross-reacting cardiac glycosides. These compounds were found in 27 species from 20 genera. The assay technique can also be used on serum samples to confirm cardiac glycoside ingestion.

Persistent fetal circulation. Two cases with myocardial dysfunction and severe acidosis.

Two neonates with the most severe form of persistent fetal circulation are described. Their presentation mimicked severe congenital heart disease. As well as persistent pulmonary hypertension and right-to-left shunting through the ductus arteriosus, they had poor myocardial contractility and atrioventricular valve regurgitation. Associated acidaemia was extreme. They survived with medical management, and the functional abnormalities resolved.

Truncus arteriosus and facial dysmorphism.

Facial features of infants with truncus arteriosus were photographed and reviewed. Anomalies included hypertelorism, low set ears, micrognathia, down-slanting palpebral fissures, short philtrum and small mouths. Associated cardiovascular defects were interrupted aortic arch, double aortic arch, right aortic arch and aberrant brachiocephalic vessels. Potential teratogenic factors included maternal diabetes, syphilis, alcohol ingestion, carbimazole therapy and infant chromosomal anomalies. The facial features, together with the rare cardiac abnormalities of truncus arteriosus and aortic arch defects are similar to those described in Di George syndrome (defect of fourth branchial arch and derivatives of third and fourth pharyngeal pouches). However none of these patients had symptomatic hypocalcaemia or absence of the thymus. It is concluded that this association of truncus arteriosus, aortic arch abnormalities and facial anomalies involves first and fourth branchial arch maldevelopment, and indicates embryological insult between the fourth and seventh weeks of gestation.

Sick sinus syndrome. Symptomatic cases in children.

In 20 children needing treatment for symptomatic sick sinus syndrome, the average age at presentation was 7.1 years and ranged from 9 months to 18 years. Symptoms were never precise but, in retrospect, 5 children had syncope, 7 had a rapid heart action, 6 had dyspnoea or tachypnoea, 2 had nonspecific chest pains, 2 had pale spells, and 1 had a sudden hemiplegia. Symptoms followed cardiac surgery in 15 cases and were related to unoperated congenital heart disease in 2 and to myocarditis in 2. The aetiology was unknown in 1 case. The type of cardiac surgery resulting in the development of the sick sinus syndrome was predominantly related to atrial suturing. Both tachy- and bradydysrhythmias were found, including wandering atrial pacemaker (9 cases), junctional rhythm (19 cases), supraventricular tachycardia (9 cases), atrial flutter (11 cases), and atrial fibrillation (2 cases). Both atrial (8 cases) and ventricular (7 cases) premature beats were seen. All patients were given trials of drug therapy but difficulties were encountered. Cardioversion was used for tachyarrhythmias in 11 cases without serious problems. Six children had permanent cardiac pacemakers inserted with good results. Recognition of the sick sinus syndrome in childhood is important and treatment must be regulated by the severity of symptoms.

The association between immunodeficiency and congenital heart disease.

The predilection of children with congenital heart disease (CHD) to infection may be explained in part by an underlying immunodeficiency disorder. Some 13 syndromes in which immunodeficiency and CHD may coexist have been reported in the medical literature. In addition, immunoglobulin and T-cell deficiencies have been found in nonsyndromal patients with CHD. The diagnosis of immunodeficiency should be entertained in such children, as early recognition of an immunodeficiency disorder can result in improved antimicrobial and immunological management.

Fetal and neonatal congenital complete heart block.

Fifteen cases of congenital complete heart block were diagnosed in utero or at birth, over a nine-year period. Six cases were confirmed by fetal echocardiography between 22 and 36 weeks of gestation. Two cases were treated in utero for cardiac failure, and serial ultrasonography was used to monitor the progress. Structural heart disease was present in seven (47%) infants; in four of these infants the defect was a corrected transposition of the great arteries. Ventricular pacing was required in seven patients--four infants with anatomically-normal hearts and three infants with associated structural heart disease. Eight children did not require permanent pacing, and remained well after a mean follow-up period of 23 months (range, one to 72 months). The mortality was highest in those patients with structural heart disease, three of whom died as neonates. Only one baby died with an anatomically-normal heart. Antinuclear antibodies were present in the mothers of seven of the eight infants who were born with a structurally-normal heart. Four mothers either had pre-existing connective-tissue disease or had developed manifestations of such disease subsequent to pregnancy. Of this group, all the women were antibody-seropositive to the nuclear antigen SS-A during pregnancy. Mothers of three infants had antinuclear antibodies in low titres, were seronegative for antibody to SS-A and remained free of the manifestations of connective-tissue disease. Six of the seven most-recent cases have been diagnosed in utero, which confirms a trend towards the more-frequent prenatal diagnosis of congenital heart disease as a result of the increased use of fetal monitoring and ultrasonography.

Oral contraceptives and myocardial infarction.

Between January 1970 and December 1972 22 women aged between 31 and 45 years were admitted to the coronary care unit with acute myocardial infarction and six of these (27%) had been taking oral contraceptives. There were nine women aged 40 or less and five of them (55%) had been on oral contraceptives while three of the other four had been sterilized by tubal interruption.Both these figures of prevalence of oral contraceptive use are significantly greater than estimates for the general population of women of similar age. For those aged 30-44 years, current estimates suggest that it is between 8 and 11%.All the women in this study had risk factors recognized as being associated with the premature development of ischaemic heart disease, and the prevalence of these risk factors was similar in those taking oral contraceptives as in those not doing so. Oral contraceptives probably enhance the chance of developing myocardial infarction in women whose risk is increased for other reasons.

Sick sinus syndrome: experience of a cardiac pacemaker clinic.

Out of a pacemaker clinic population of 182 patients 21 (11.5%) were found to have the sick sinus syndrome. Their ages ranged from 30 to 80 years and averaged 62. Ischaemic heart disease was more commonly an aetiological factor than in patients with chronic atrioventricular heart block. Other aetiologies were familial cardiomyopathy, postcardiac surgery, and dystrophia myotonica.Cardioversion before pacemaker insertion was a hazardous procedure. After insertion the occurrence of tachycardias and the need for drug therapy were reduced. One patient no longer required a pacemaker once atrial fibrillation became established.A high incidence of cerebral embolization was observed and the use of anticoagulant drugs therefore merits serious consideration.Failure of inhibition of demand-type pacemakers occurred in two patients. Two patients who exhibited competition with fixed-rate pacemakers died. Two patients were treated with electrodes surgically implanted on the right atrium. It is suggested that fixed-rate pacemakers may be contra-indicated and that sequential atrioventricular demand pacing is theoretically ideal.

The middle aortic syndrome: an important feature of Williams' syndrome.

The middle aortic syndrome, with diffuse narrowing of the thoracic and abdominal aorta, was present in 10 of 18 patients with Williams' syndrome (55%). There were 3 thoracic coarctations, and 2 abdominal coarctations, with gradients greater than 20 mmHg across the zone of narrowing. Seven patients had mild renal arterial stenosis, and 6 had visceral arterial stenoses. Ten were hypertensive. Measured dimensions of the aortic lumen failed to increase with age in 3 males who had serial angiographic studies. One developed mesenteric arterial stenosis, with mild bilateral renal arterial stenoses, between the ages of 9 and 19 years. Aortic intravascular ultrasound performed in 2 patients confirmed abnormally thick vessel walls with small lumens. Diffusely narrowed and thick-walled stiff arteries, lacking elastin, are a feature of Williams' syndrome. The arteriopathy tends to progress with age, and systemic hypertension is common in teenagers and beyond. The middle aortic syndrome was present in more than half our patients, and does not necessarily reflect a bias because of cardiologic referral. Aortography with measurement of aortic diameters and delineation of the visceral branches is an important requirement for complete evaluation of patients with Williams' syndrome.

Primary cardiac tumours in a paediatric population.

OBJECTIVE: To review the presentation, diagnosis, histology and outcome of primary cardiac tumours presenting to a paediatric cardiac unit over a 20-year period. METHODS: Hospital records and data bases were searched for the years 1980-2000. RESULTS: There were 12 patients with four histological tumour types including a predominance of rhabdomyoma, as well as myxoma, fibroma and myocardial hamartoma. Diagnosis was made in utero, by ultrasound in five cases and in the neonatal period in a further three cases. Six cases (50%) required surgical intervention and there were three tumour-related deaths. Two infants with large left ventricular tumours diagnosed in utero developed univentricular physiology, acting like hypoplastic left heart syndrome at birth. CONCLUSIONS: Despite an absence of malignant histology there was significant mortality and morbidity among the patients reviewed. The development of univentricular physiology in infants with large left ventricular tumours is rare and is a difficult management problem.

Coronary artery spasm causing myocardial infarction and ventricular fibrillation.

A case of acute transmural anteroseptal infarction and ventricular fibrillation, in a woman with normal coronary arteries is presented. Severe coronary artery spasm was demonstrated angiographically, and is postulated as the cause of infarction and arrhythmia.

Congenital paroxysmal atrial tachycardia.

Ten infants who had paroxysmal atrial tachycardia in utero or at birth are reported. Because of apparent fetal distress, caesarean section was performed in 4 cases and labour was induced in 1. Birthweight was generally large for gestational age. Severe ascites and hydrops at birth were manifestations of cardiac failure. Atrial flutter was recorded in 4 infants and supraventricular tachycardia in 5. The WoLff-Parkinson-White syndrome became evident later in 2. Digoxin was given to all 10 infants, and cardioversion was required and was effective in 4. Known recurrences in childhood have occurred in only 1 patient. Congenital atrial tachyarrhythmias may be commoner than generally believed, and fetal electrocardiography may help to avoid unnecessary termination of pregnancy. Blood sugar determinations are important, since neonatal hypoglycaemia was found. Cardioversion should be performed promptly in severely ill infants or if there is no response to digoxin. Care is required to avoid digoxin toxicity.

Vegetations in infective endocarditis. Clinical relevance and diagnosis by cross sectional echocardiography.

Cross sectional echocardiography identified vegetations in 45 of 62 (73%) patients who had clinical evidence of active infective endocarditis. The sensitivity of this technique in diagnosing vegetations in infective endocarditis was 93% and the specificity 89%. The predictive value of a positive test was 96% and that of a negative test 80%. Vegetations were detected with a similar frequency on the aortic and mitral valves. The incidence of valvar incompetence, congestive heart failure, and the need for surgical intervention was similar in the patients with and without vegetations. Embolism occurred in 47% of those patients with vegetations and in 12% of those without. The mortality rate was 27% in those with vegetations, and no patient without vegetations died. Thus cross sectional echocardiography is accurate in diagnosing vegetations in patients with infective endocarditis, and this finding identifies patients at high risk of embolic complications and death.

Diagnosis and natural history of Ebstein's anomaly.

Thirty five cases of Ebstein's anomaly were diagnosed by cardiac catheter study or echocardiography between 1962 and 1984. The anomaly was an isolated abnormality in 27 patients; the remainder had additional heart lesions. Six patients have died and four of these had other cardiac lesions. Thirteen are currently symptom free. Twelve cases presented with cyanosis and heart murmur on the first day of life. Eight of these had associated thrill which is a rare finding in neonates. As pulmonary vascular resistance fell there was clinical improvement. This was assisted by oxygen treatment. Another two cases presented in the first week of life and 10 in the first decade with a murmur or supraventricular tachycardia. Eleven cases presented as adolescents or adults with a murmur, cardiomegaly, or cardiac symptoms. Three patients were seen initially in the sixth decade. Clinical diagnosis was correct in only one adult. Twenty five patients had cardiac catheterisation. Important arrhythmias occurred in 10 cases, and two required cardioversion. Difficulty was experienced in entering the pulmonary artery in 11 infants and children. Diagnosis at initial catheter study was incorrect in four patients. Echocardiograms were recorded in 29 cases. Earlier M mode measurements of time delay of tricuspid closure compared with mitral closure did not always lead to the correct diagnosis. Cross sectional studies gave good images of tricuspid leaflet displacement, its tethering, and the atrialised portion of the right ventricle and have facilitated the diagnosis of more cases in recent years. Echocardiography is the procedure of choice for diagnosis of Ebstein's anomaly.

The immunocompetence of children with congenital heart disease.

We studied the immunocompetence of 18 children with conotruncal malformations (13 with tetralogy of Fallot, 5 with truncus arteriosus) and 22 children with cardiac shunt lesions. There were reduced total T cell percentages and T helper cells in the conotruncal group but no T cell abnormality in the shunt group. Also, 7 of the 18 cases in the conotruncal group had facial dysmorphism reminiscent of the Di George syndrome. These results suggest that patients with conotruncal malformations fall into the wide spectrum of the Di George syndrome. There was some humoral deficiency in both groups with reduced levels of immunoglobulins IgG and IgA and low levels of complement C3 and C4. The clinical records showed a high frequency of infections. Hospital admissions for these episodes had occurred in 61% of the conotruncal group and 32% of the shunt group. Thus, there is an increased susceptibility to infection in children with congenital heart disease, and the predilection to infection has an immunological basis.

Endocardial fibroelastosis and infantile polycystic disease.

A child with infantile polycystic disease who developed cardiac failure from endocardial fibroelastosis is described. Endocardial fibroelastosis has been reported in adult type polycystic disease in childhood. Endocardial fibroelastosis should be suspected in a child with polycystic disease who develops unexplained cardiac failure.

Fetal echocardiography in the diagnosis of congenital heart disease.

Fetal echocardiography provides an opportunity to diagnose congenital heart disease as early as the midtrimester of pregnancy, allowing for proper planning of perinatal care and counselling of the parents. This paper reviews the accuracy and outcomes of fetal cardiac ultrasound studies at The Prince Charles Hospital over the past 4 years. A total of 43 fetuses, of gestational ages 16-39 weeks, were studied. The indications for these were: previous sibling with cardiac anomaly (16 cases); abnormal heart on obstetric ultrasound scan (USS) (13 cases); abnormalities found on obstetric USS with normal appearing heart (one case); fetal bradycardia (seven cases); fetal tachycardia (four cases); irregular fetal heart beat (two cases). At birth all fetuses assessed because of a previously affected sibling were normal. Of the 13 referred because of structural heart lesions suspected on obstetric USS, eight were abnormal, four were normal and one was terminated without autopsy. Important rhythm disturbances occurred in nine of the 11 referred because of abnormal heart rates. One false positive diagnosis of a possible coarctation was made, and in four cases an abnormality was noted on USS but the diagnosis was not completely correct. Nine of the 19 infants with abnormalities detected have died. Fetal echocardiography is an accurate and useful method of diagnosing congenital heart disease in utero. Although the mortality of affected fetuses is high, antenatal diagnosis allows planning of medical care and offers the greatest chance of a successful outcome.