This study was performed in 24 patients with tetralogy of Fallot in whom shunt operation was performed instead of total correction because of small left ventricular end-diastolic dimension. Left ventricular end-diastolic dimension was measured using M-mode and two-dimensional echocardiography pre- and at least one year postoperatively. There was no change in the postoperative left ventricular size in two patients. However, in the other 22 patients, the left ventricular dimension was increased to 70 to 103 percent of normal left ventricular size. According to the findings of this study, we can conclude that the patients in whom shunt operation was performed would most likely have an increased left ventricular size over time.

Anastomosis, Surgical↗

PubMedDate not supplied

Absent pulmonary valve syndrome with agenesis of the left pulmonary artery.

Absent pulmonary valve syndrome is represented by rudimentary nodules without identifiable leaflets, and it is commonly associated with a ventricular septal defect and tetralogy of Fallot. Unilateral absence of a pulmonary artery is also a rare congenital anomaly. The following is a case report of a one-day-old newborn with cyanosis. The physical examination revealed a to-and-fro murmur and no expansion of the left hemithorax. Echocardiography revealed tetralogy of Fallot, absent pulmonary valve, and enlarged main and right pulmonary arteries. The catheterization and angiography confirmed the diagnosis and absence of the left pulmonary artery.

Cyanosis↗

PubMedDate not supplied

Leprechaunism (Donohue's syndrome): a case report.

Features of leprechaunism, including low-birth weight, a grotesque elfin face with clubbed nose, hirsutism, large low-set ears, enlarge external genitalia, distended abdomen with emaciated extremities, and growth and motor retardation were present in a 3-month-old girl. Because of parental consanguinity in this patient, a recessive mode of inheritance seems likely.

Abnormalities, Multiple↗

PubMedDate not supplied

Familial sick sinus syndrome in two siblings.

Two siblings with sinus node disease are presented. The patients were severely affected and required permanent pacing. A cardiac electrophysiologic study was conducted in Case 2, which revealed an atrioventricular conduction disturbance in addition to sinus node dysfunction. The parents and other siblings showed no evidence of sinus node disorder. The occurrence of this disease in these siblings suggests an autosomal dominant mode of inheritance with variable penetrance.

Bundle of His↗

PubMedDate not supplied

Long-term results of patients with congenital complete atrioventricular block.

Congenital complete atrioventricular block is an uncommon disorder with a prognosis which is usually favorable. The disorder is sometimes associated with syncope, sudden death or cardiac pacing. It is difficult to determine the patients at risk of sudden death. In this retrospective study, hospital records of children who had been admitted to the Hacettepe University Children's Hospital between 1970-1990 for evaluation of complete heart block were examined. The study population, consisting of 39 children, 27 males and 12 females, had electrocardiograms consistent with complete heart block. These patients, diagnosed as having congenital complete A-V block, had an otherwise normally structured heart, and were followed up for a period of from one month to 15 years. Age at diagnosis ranged from 27 days to 17 years (mean: 86.9 +/- 48 months). Of the 14 patients with symptoms (five with syncope, eight with exercise intolerance and one with presyncope), nine were paced electively and have done well. Clinical and laboratory features of the asymptomatic and symptomatic groups were compared to evaluate potential risk factors.

Adolescent↗

PubMedDate not supplied

"Acquired" subvalvular aortic stenosis after repair of several congenital cardiac defects.

Discrete subvalvular aortic stenosis is a progressive lesion. In this report we presented nine patients who had no significant left ventricular-aortic obstruction at initial cardiac catheterization or echocardiographic examination, but later developed significant subvalvular aortic stenosis. Associated lesions included ventricular septal defect in three, patent ductus arteriosus in two, aorticopulmonary window in one, tetralogy of Fallot in one, supramitral membrane in one, and ventricular septal defect and patent ductus arteriosus in one case. Nine patients were diagnosed with subvalvular aortic stenosis 18 months to eight years after surgical correction. Eight of the patients required surgery for subvalvular obstruction. In conclusion, discrete subaortic stenosis is a rare, late complication of the surgical repair of several congenital heart defects. It is a progressive lesion after surgery; therefore these patients require careful follow-up.

Aortic Stenosis, Subvalvular↗

PubMedDate not supplied

Cardiorespiratory function in Duchenne and Becker muscular dystrophy.

The purpose of this study was to investigate the cardiorespiratory function in Duchenne (DMD) and Becker muscular dystrophy (BMO) patients and to determine whether there is a correlation between these functions and muscular strength. The study involved 32 patients with progressive muscular dystrophy (28 DMD and four BMD). The mean age of the patients was 9.6 +/- 3.5 years. Cardiac investigations were performed in all of the patients, and pulmonary function tests were obtained in 16 cases. In five cases (31%), vital capacity (VC) was less than 80 percent of the predicted value. There was a good correlation between VC and muscular strength. There were various cardiologic findings in 50 percent of the cases with DMD. Electrocardiographic changes were present in 43 percent of the patients. Left ventricular systolic function in the patients who could not walk was significantly lower than that of the patients who could walk. There may be some unknown mechanisms that preserve left ventricular function relatively in the normal range in spite of cardiac involvement.

Adolescent↗

PubMedDate not supplied

Short- and intermediate-term efficacy of amiodarone in infants and children with cardiac arrhythmia.

The antiarrhythmic effect of amiodarone was examined in this retrospective study in a group of 20 patients with a mean age of 8.5 +/- 6.7 years (range 42 days to 20 years, median 9 years). Five patients with atrial flutter, one patient with atrial fibrillation, two patients with an intermediate rhythm between atrial flutter and atrial fibrillation, four patients with chaotic atrial tachycardia, three patients with atrioventricular reentry tachycardia, two patients with junctional ectopic tachycardia, and three patients with ventricular arrhythmias were treated with amiodarone. The mean duration of therapy was 9.1 +/- 12.3 months (range 1 month to 4 years). Before amiodarone treatment, 18 patients had been unresponsive to various antiarrhythmic drugs (range 1-8, median 2). Two patients received amiodarone as an initial therapy. It was administered orally at a dose of 10 mg/kg once per day for 10 days and then decreased to 5 mg/kg once per day. Amiodarone was effective in 16 patients (80%). Side effects occurred in three patients, including thyroid dysfunction, elevation of liver enzymes, and keratopathy. All side effects disappeared upon cessation of the therapy. We recommend amiodarone for the treatment of childhood arrhythmias, especially for the refractory types.

Administration, Oral↗

PubMedDate not supplied

Vasovagal syncope: asystole provoked by head-up tilt testing under sertraline therapy.

Syncope is defined as a sudden transient loss of consciousness. Vasovagally mediated hypotension and bradycardia are believed common, yet difficult to diagnose, causes of syncope in healthy children and adolescents. These episodes are often both sudden and sporadic in nature and, if recurrent and severe (malignant vasovagal syncope), can be a source of morbidity and possibly mortality. Head-up tilt testing has emerged as a useful investigation in patients who are thought to have recurrent vasovagal syncope with systemic hypotension, bradycardia, or both, and it has been suggested as a potential method to test for vasovagal episodes. Sertraline hydrochloride, a serotonin reuptake inhibitor, has been reported to be effective in preventing the vasovagal syncopal episodes in children and adults. Here, two cases of recurrent, unexplained syncope are presented. Both were under sertraline therapy and underwent provocative head-up tilt testing that resulted in asystole.

1-Naphthylamine↗