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Biomedical subjects

C Wren

Publications and source records attributed to C Wren.

At least 55 records · Page 3Linked to original sources
PubMed1994

End-on aortogram: improved identification of important coronary artery anomalies in tetralogy of Fallot.

OBJECTIVE: To identify coronary artery anomalies in patients with tetralogy of Fallot with an aortogram taken with steep caudal and left oblique angulation ("end-on" aortogram). DESIGN: Prospective evaluation of end-on aortogram in the preoperative angiographic assessment of consecutive patients with tetralogy of Fallot. SETTING: Regional paediatric cardiology centre. PATIENTS: 34 patients, aged 3 months to 12 years (median age 9 months). METHODS: An aortogram was performed with steep caudal (38 degrees-45 degrees) and left oblique (0 degrees-30 degrees) angulation under general anaesthetic as part of routine preoperative angiographic assessment. RESULTS: The origins and courses of the coronary arteries were visualised in all patients and important coronary artery anomalies were identified in four patients: single left coronary artery; single right coronary artery (two patients); separate high origin of left anterior descending. These anomalous coronary vessels crossed the right ventricular outflow tract. CONCLUSIONS: It is important to identify preoperatively coronary arteries that may interfere with right ventricular outflow tract reconstruction. An aortogram with steep caudal and left oblique angulation is useful in identifying anomalous coronary arteries and more importantly it defines the relation of these vessels to the right ventricular outflow tract.

Aortography↗
PubMed1993

Ondine's curse and neurally mediated syncope--a new and important association.

A 6-year-old boy with congenital central hypoventilation syndrome (Ondine's curse) presented with presyncope. Investigation revealed a diagnosis of neurally mediated syncope. This previously unreported association provides strong evidence that autonomic dysfunction is implicated in both these conditions.

Autonomic Nervous System↗
PubMed1993

Endocardial pacemakers in children: lead length and allowance for growth.

Permanent endocardial pacing in small children is feasible but is limited by two problems: sufficient extra lead has to be left within the heart to allow for growth and the excess has to be coiled behind the pacemaker, limiting the benefit from smaller generators. The required intravascular lead length in 120 children and adults was measured on posteroanterior chest X ray and was correlated with standing height. Measurements were made from the mid-point of the left clavicle to the apex of the right ventricle in a curve simulating the usual endocardial lead position. In 60 children, aged 2.0-15.9 years, intravascular lead length (range 15.5-29.0 cm) correlated well with height (0.83-1.70 m), r = 0.91. In 60 adults, mean age 54.9 years, intravascular lead length (25.5-35.6 cm) also correlated well with height (1.45-1.85 m), r = 0.71. In 20 adults the excess extravascular lead length, measured during pacemaker implantation via the subclavian route, was 15.1-33.7 cm and was inversely correlated with height. A child's eventual adult height can be predicted and, using our data, the extra length of lead necessary to allow for growth can be computed. Available endocardial pacing leads are usually 58- to 64-cm long. The excess extravascular lead is a major practical difficulty in children. Shorter leads would avoid the problem of excess lead and facilitate long-term pacing in small children.

Adolescent↗
PubMed1993

Cardiac abnormalities in end stage renal failure and anaemia.

Thirteen anaemic children on dialysis were assessed to determine the incidence of cardiac changes in end stage renal failure. Nine children had an increased cardiothoracic ratio on radiography. The electrocardiogram was abnormal in every case but no child had left ventricular hypertrophy as assessed by voltage criteria. However, left ventricular hypertrophy, often gross, was found on echocardiography in 12 children and affected the interventricular septum disproportionately. Cardiac index was increased in 10 patients as a result of an increased left ventricular stroke volume rather than heart rate. Left ventricular hypertrophy was significantly greater in those on treatment for hypertension and in those with the highest cardiac index. Abnormal diastolic ventricular function was found in 6/11 children. Children with end stage renal failure have significant cardiac abnormalities that are likely to contribute to the high cardiovascular mortality in this group. Anaemia and hypertension, or its treatment, probably contribute to these changes. Voltage criteria on electrocardiogram are of no value in detecting left ventricular hypertrophy. Echocardiography must be performed, with the results corrected for age and surface area, in order to detect and follow these abnormalities.

Adolescent↗
PubMed1993

Ambulatory blood pressure monitoring.

Ambulatory blood pressure monitoring (ABPM) in adults is proving to be useful. The aim of this study was to determine if ABPM is accurate in the lower blood pressure range encountered in children and, equally important, whether it is acceptable to children. Thirty one children, between the ages of 6 and 18 years, were assessed using an ambulatory blood pressure monitor that uses an auscultatory method. Blood pressure was measured in the contralateral arm with a mercury sphygmomanometer and an oscillometric device at the beginning and end of the study for comparison. Over a blood pressure range of 90-130 mm Hg systolic and 40-80 mm Hg diastolic, a close agreement was found with the sphygmomanometer; the limits of agreement (+/- 2 SD) were 11.6 mm Hg for systolic blood pressure and 13.6 mm Hg for diastolic blood pressure. The bias was less than 1.0 mm Hg. The ambulatory device was worn by all patients for at least 16 hours with an average of 52 recordings per patient. The majority found the device comfortable to wear and were not woken from sleep.

Adolescent↗
PubMed1993

Stenting of the arterial duct combined with banding of the pulmonary arteries and atrial septectomy or septostomy: a new approach to palliation for the hypoplastic left heart syndrome.

OBJECTIVE: To assess the feasibility of pulmonary artery banding, atrial septectomy or septostomy, and percutaneous stenting of the arterial duct in babies with the hypoplastic left heart syndrome. PATIENTS: Four infants with hypoplastic left heart syndrome. SETTING: Two supraregional paediatric cardiac centres. METHODS: Ductal patency was maintained initially with prostaglandin E. Banding of the proximal branch pulmonary arteries was performed through a median sternotomy and open atrial septectomy was performed if balloon septostomy was not. Stainless steel stents (Johnson & Johnson) mounted in a balloon catheter were implanted into the arterial duct under radiographic control and expanded to a diameter of approximately 8 mm, prostaglandin treatment was then stopped. RESULTS: All the patients survived the immediate postoperative period and maintenance of wide ductal patency was achieved in three of the four patients by stent implantation. Two weeks after the procedure two babies died of right ventricular failure and respiratory infection: some distal ductal constriction had occurred in one where the stent was not quite sufficiently distally placed. One child was discharged home 15 days after treatment and was well at follow up at age 16 weeks and one was stable but required diuretic therapy five weeks after the procedure. CONCLUSIONS: This new approach is technically feasible. At least in the short term it seems to offer hope of effective palliation for the hypoplastic left heart syndrome and it warrants further study.

Cardiac Catheterization↗
PubMed1992

Endocarditis with acute mitral regurgitation caused by Fusobacterium necrophorum.

Infective endocarditis is uncommon in young children, especially in the absence of structural heart disease. We report the case of a 2-year-old boy who presented with acute rupture of the mitral valve chordae 6 weeks after an episode of Fusobacterium necrophorum septicemia. His heart had been structurally normal before. Mitral valve replacement was successfully performed. This is the first recorded case of endocarditis in a child caused by necrobacillosis.

Bacteremia↗
PubMed1992

Atrial flutter in the fetus and young infant: an association with accessory connections.

OBJECTIVE: To highlight the association between atrial flutter and accessory connections in the fetus and young infant. DESIGN: A retrospective review from January 1985 to January 1990. PATIENTS: Fetuses, neonates, and young infants with atrial flutter. RESULTS: Four fetuses and five infants presented with atrial flutter. In two fetuses and one infant sinus rhythm returned spontaneously. The other six required cardioversion. Three of them developed orthodromic atrioventricular re-entry tachycardia and each had evidence of an accessory connection. CONCLUSIONS: Because atrial flutter in the fetus and neonate is rare, the high incidence of accessory connections in this group points to a possible aetiology of "idiopathic" atrial flutter in this age group.

Atrial Flutter↗
PubMed1991

Hypoplastic left heart syndrome: more potential transplant recipients than suitable donors.

Neonatal cardiac transplantation offers the prospect of survival for babies with hypoplastic left heart syndrome--but only if suitable donors are available. In a retrospective survey in the Northern health region of England and Wales, the likely need for neonatal cardiac transplantation far outweighed the potential availability of donors. Over 8 years (1983-90) hypoplastic left heart syndrome was identified in 38 newborn babies and in 9 fetuses in utero. Of 41 live births (including 3 diagnosed prenatally) 31 would have been candidates for a cardiac transplant, but only 4 suitable donors could be identified (3 with anencephaly born alive during the same period and 1 who died between 1979 and 1986 after a head injury). Analysis of all infant deaths in 1987-89 revealed only 3 potential donors from 426 deaths in the 3 years. Although more widespread antenatal diagnosis may lead to fewer liveborn babies with hypoplastic left heart syndrome, these findings indicate that an alternative source of donors needs to be identified before neonatal cardiac transplantation can be widely used in the treatment of this disorder.

Abortion, Induced↗
PubMed1990

Natural history of supravalvular aortic stenosis and pulmonary artery stenosis.

Data from 35 patients with supravalvular aortic stenosis or pulmonary artery stenosis, or both, undergoing cardiac catheterization between 1973 and 1989 were analyzed retrospectively. Twenty-seven patients had supravalvular aortic stenosis: 11 required surgery after the first investigation and 8 (80%) of 10 others undergoing serial investigation showed an increase in the left ventricle to aorta pressure gradient. Angiographic measurements showed that the increase in the aortic pressure gradient was related to failure of normal growth of the ascending aorta lumen. Nineteen patients had pulmonary artery stenosis, with a right ventricular pressure greater than 33 mm Hg. At restudy, right ventricular pressure had decreased in 9 (82%) of 11 patients. This decrease in right ventricular pressure was associated with an increase in the systolic distensibility of the proximal pulmonary arteries, although there was no increase in the diastolic diameters. One patient had a rapid early increase in right ventricular pressure and no pulmonary artery growth. In two patients, multiple peripheral pulmonary artery stenoses became evident with time and produced persistent right ventricular hypertension. Supravalvular aortic stenosis is usually a progressive lesion, with an increase in left ventricular outflow tract pressure gradient related to poor growth of the ascending aorta. Pulmonary artery stenosis usually improves and only rarely limits prognosis.

Adolescent↗
PubMed1990

Supraventricular tachycardia in infants: response to initial treatment.

All patients with supraventricular tachycardia during the first 12 months of life who presented between 1977 and 1988 were identified by a retrospective survey of records in this hospital and by a questionnaire sent to paediatricians in the Northern region. Twenty two of 29 patients (76%) were in heart failure and seven (24%) had cardiogenic shock. Seven patients (24%) were free of symptoms. All had narrow QRS tachycardia at 215-315 beats/minute (mean (SD) 292 (21)). Initial treatment included digoxin (effective in seven of 14 patients, with overdose in three), verapamil (effective in three of three but fatal in one), cardioversion (effective in all 10 who were treated in this way), iced water applied to the face (effective in all 16 patients on 53 of 59 occasions, 90%). Initial treatment in local hospitals was less effective and associated with more complications than that given in the regional referral centre. Digoxin is often ineffective, return to sinus rhythm is delayed, and overdosing is common. Cardioversion is effective but tachycardia often recurs. Iced water is safe and effective, and should become the treatment of choice for termination of supraventricular tachycardia in neonates and young infants.

Adenosine↗
PubMed1990

Fetal and infantile hypertension caused by unilateral renal arterial disease.

Three children who presented with heart failure in infancy caused by severe hypertension as a result of unilateral renal arterial disease are described. One presented at 3 days of age with persistent fetal circulation and heart failure. He had abnormal great vessels that indicated that the hypertension was of long standing and therefore fetal; this has not been described previously. The other two children failed to thrive because of unrecognised hypertension and subsequently presented with heart failure. All three underwent unilateral nephrectomy which cured their hypertension, and all were thriving at the time of writing. The benefits of nephrectomy outweighed the operative risks and loss of renal function. Blood pressure should be measured in children who are failing to thrive as part of routine clinical practice.

Cardiac Output, Low↗
PubMed1990

Familial ventricular tachycardia: a report of four families.

Four cases of familial ventricular tachycardia are presented. In each family the proband was an adolescent girl. Twelve members in the four families were affected and all were female. The clinical and electrocardiographic features were relatively constant within each family but there were striking differences between families. These differences argue against a common electrophysiological or pathophysiological basis for the ventricular tachycardia.

Adolescent↗
PubMed1990

Idiopathic infantile arterial calcification in two siblings: failure of treatment with diphosphonate.

Two siblings with idiopathic infantile arterial calcification are reported. The fetal and postnatal echocardiographic features were a large pericardial effusion, thickened pulmonary and aortic valves, poor pulsation of the descending aorta, and calcification of the great vessels. In one patient calcification was first detected at 33 weeks' gestation. Despite treatment with disodium etidronate both children died.

Calcinosis↗