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Biomedical subjects

J Somerville

Publications and source records attributed to J Somerville.

At least 55 records · Page 3Linked to original sources
PubMed1999

Use of self expanding stents in stenotic aortopulmonary shunts in adults with complex cyanotic heart disease.

OBJECTIVE: To describe the use of self expanding stents in treating long segment stenosis of aortopulmonary shunts (APS) in adults. DESIGN: Clinical records, catheterisation data, cineangiograms, and operation notes of four consecutive patients undergoing stent implantation since December 1994 were studied retrospectively. SETTING: A tertiary referral centre for cardiac disease. SUBJECTS: Four patients underwent cardiac catheterisation because of clinical deterioration. Their age ranged between 23 and 32 years. The underlying diagnosis was complex cyanotic heart disease in all. Three had a stenotic interposition graft, and one had a classic Blalock shunt. RESULTS: There was one technical failure owing to migration of the stent distal to an ostial stenosis. The ability index, resting oxygen saturation, and exercise tolerance improved in the remainder. Their medium term results have been excellent. CONCLUSIONS: This technique may further palliate adult patients with complex congenital heart disease, though the long term patency of stents is unknown.

Adult↗
PubMed1998

Recurrence risks in offspring of adults with major heart defects: results from first cohort of British collaborative study.

BACKGROUND: Congenital heart defects are generally assumed to have a multifactorial aetiology. We have tested this hypothesis by studying adults with heart defects and their families. METHODS: We identified 1094 patients who survived surgery for major cardiac defects before 1970. We chose individuals with disturbance of situs or segmental connection, with atrioventricular septal defect or with tetralogy of Fallot. After exclusion and non-participation, 727 individuals were traced. Each was visited by an investigator and completed a detailed questionnaire. If possible, all "normal" offspring were examined by a paediatric cardiologist. FINDINGS: The 727 individuals had 393 live offspring. There were 71 miscarriages and five terminated pregnancies. Overall, we found recurrent heart defects in 16 liveborn offspring--a recurrence risk of 4.1%. This result differed significantly from sibling risk (2.1%; p=0.021). More congenital heart defects occurred in the offspring of affected women than in those of affected men (p=0.047); when all malformations (cardiac and non-cardiac) in the offspring were taken into account the excess was more significant (p=0.032). We found an excess of miscarriages in the offspring of affected women (p=0.001). In tetralogy of Fallot, heart defects occurred in seven (3.1%) of 223 offspring, 12 (2.2%) of 539 siblings, five (0.3%) of 1575 second-degree relatives, and eight (0.3%) of 2728 third-degree relatives. INTERPRETATION: Our findings do not support a polygenic basis for all heart defects. Atrioventricular septal defect seems to be a single-gene defect and tetralogy of Fallot a polygenic disorder with a small number of interacting genes. Our data suggest that isolated transposition of the great arteries is a sporadic defect.

Adult↗
PubMed1998

How to manage the Eisenmenger syndrome.

Without an understanding of the physiology of the Eisenmenger state (complex, syndrome or reaction) the management and outcomes of such cases will not be optimal. This is a problem for many patients scattered as occasional curiosities in practices and non-specialised clinics. The patient group with Eisenmenger reaction can lead adequate though symptomatic lives until late middle age or longer, but survival beyond age 50 years demands optimum care and attention from informed cardiologists. The cardiologist in charge should advise on lifestyle and all therapy, particularly for other systems, prevent disaster and educate patient and profession to keep the patient alive.

Adult↗
PubMed1998

Protein-losing enteropathy after the Fontan operation: an international multicenter study. PLE study group.

OBJECTIVE: This multicenter study retrospectively analyzes the data on 114 patients with protein-losing enteropathy after Fontan-type surgery. Special attention was given to the different treatment strategies used and their effect on outcome. METHODS AND RESULTS: In 35 participating centers 3029 Fontan operations were performed. The incidence of protein-losing enteropathy in the survivors was 3.7%. The median age at Fontan-type surgery was 8.2 years (range: 0.6 to 32.9 years). Median age at diagnosis of protein-losing enteropathy was 11.7 years with a median time interval between surgery and diagnosis of 2.7 years (range: 0.1 to 16.4 years). Most patients had edema (79%) and effusions (75%). Hemodynamic data revealed a mean right atrial pressure of 17 +/- SD 5.3 mm Hg with a cardiac index of 2.4 +/- 0.8 L/min/m2. Medical treatment only (n = 52) resulted in a complete resolution of symptoms in 25%, no improvement in 29%, and death in 46%. Surgical treatment (n = 52) was associated with relief of protein-losing enteropathy in 19%, no improvement in 19%, and death in 62%. In 13 patients 16 percutaneous interventions were performed. This resulted in symptomatic improvement after 12 interventions and no improvement after 4 interventions. CONCLUSIONS: We conclude that the current treatment of protein-losing enteropathy after Fontan operation is associated with a very high mortality and morbidity rate. Preventive strategies and new therapeutic approaches are necessary.

Adolescent↗
PubMed1998

The Brock procedure (closed infundibular resection) for Fallot's tetralogy: 43 years later.

Innovative cardiac surgery ('blind' or indirect infundibular resection) for tetralogy of Fallot on a child of 4 years was followed by survival for 43 years without further surgery. The patient remained well until about one year before death, when he developed clinical features of progressive biventricular failure associated with pulmonary hypertension and incompetence of the pulmonary and tricuspid valves. Postmortem examination showed severe damage to one of the leaflets of the pulmonary valve, interpreted as due to inadvertent avulsion during the original surgical procedure. A large ventricular septal defect was present, but there was no residual subpulmonary infundibular obstruction.

Autopsy↗
PubMed1998

Gastro-intestinal protein loss in late survivors of Fontan surgery and other congenital heart disease.

AIMS: Protein losing enteropathy is a serious complication of Fontan surgery. The aim of this study was to investigate gastro-intestinal protein loss in adults with congenital heart disease, both with and without Fontan surgery, and to correlate findings with systemic venous pressure. METHODS AND RESULTS: Forty eight patients were studied. The first group included adult survivors of Fontan surgery. The second and third groups were control patients with congenital heart disease who had not had Fontan surgery and had either normal or chronically elevated systemic venous pressure. Gastro-intestinal protein loss was assessed by measurement of faecal alpha-1-antitrypsin. Faecal alpha-1-antitrypsin levels were significantly higher in the Fontan group (0.55 +/- 0.15 mg. g-1 faeces, P = 0.002) and the control group with chronically elevated venous pressure (0.60 +/- 0.30 mg. g-1 faeces, P < 0.001) compared to the controls with normal venous pressure (0.29 = 0.12 mg. g-1 faeces). Of the 15 subjects who were found to have increased gastro-intestinal protein loss, only four had clinical protein-losing enteropathy. The degree of gastro-intestinal protein loss correlated significantly with venous pressure (P = 0.01) and with serum aspartate transaminase (P = 0.04). CONCLUSION: Increased gastro-intestinal protein loss is common in this select group of late survivors of Fontan surgery and in other subjects with congenital heart disease and chronic elevation of systemic venous pressure, and was present in patients who did not have protein-losing enteropathy. Increased faecal alpha-1-antitrypsin is an important finding in these patients as intervention at this stage, before the onset of florid protein-losing enteropathy, might prevent the development of further complications.

Adult↗
PubMed1998

Infective endocarditis in the grown-up congenital heart (GUCH) population.

AIMS: Infective endocarditis accounts for 4% of admissions to a specialized unit for grown-up congenital heart patients. This study defines lesions susceptible to infection, antecedent events, organisms, outcome and surgical treatment in a group of such patients. METHODS AND RESULTS: The grown-up congenital heart disease database was searched for all patients aged 13 years and above with adequate documentation of infective endocarditis retrospectively between 1983-1993 and thereafter between 1993-1996. There were 185 patients (214 episodes) divided into Group I: 128 patients unoperated or palliated and Group II: 57 patients after definitive repair and/or valve repair/replacement. In Group I, the commonest affected sites were ventricular septal defect in 31 (24%), left ventricular outflow tract in 22 (17%) and mitral valve in 17 (13%) and in Group II, left ventricular outflow tract in 20 (35%), repaired Fallot in 11 (19%), and atrioventricular defects in eight (14%). Infective endocarditis was not seen in secundum atrial septal defects before or after closure; in closed ventricular septal defects and ducts without left-sided valve abnormality; in isolated pulmonary stenosis; in unrepaired Ebstein: or after Fontan-type or Mustard operations. Surgery was performed in 39 patients: as an emergency in 17, and for failed medical therapy in 22. Only 87 (41%) of patients had a predisposing event: dental procedure or sepsis were the commonest events in Group I (33%) and cardiac surgery in Group II (50%). Streptococci species were found in 54% of Group I patients and in 45% of Group II. Staphylococci aureus was commoner in Group II (25%) compared to Group I (14%). Mean time from the onset of symptoms to diagnosis was 60 and 29 days in Groups I and II, respectively. Eight (4%) patients died as a result of septicaemia related to emergency or repeated surgery and Staphylococcus aureus infection. Recurrent attacks occurred in 21 (11%) patients. CONCLUSION: Reparative surgery does not prevent endocarditis except for closure of a ventricular septal defect and duct. Delay in diagnosis is serious since it contributes to mortality, although the overall mortality % is not high. Specific lesions are not affected so prophylaxis is probably unnecessary in those anomalies.

Adolescent↗
PubMed1998

Eisenmenger syndrome. Factors relating to deterioration and death.

AIMS: To assess the natural history, risk factors for death and deterioration of patients with Eisenmenger Syndrome. METHODS: The clinical course of 188 patients from three different cardiac centres specialized in adult congenital heart defects, followed for a median period of 31 years, was retrospectively analysed. According to the diagnosis, 64 males and 124 females, mean age at last follow-up 33.02+/-12.8 years, were divided into two groups: simple (128) and complex (60) congenital heart disease. Events analysed were: time variation of Ability Index, pulmonary and cerebral complications, non-cardiac surgery, urate metabolism and renal function, arrhythmias, pregnancy and contraception, medical therapy and transplantation, death from all causes and sudden death. RESULTS: Eighty-four percent of the overall population showed a satisfactory Ability Index (1 and 2) at the first attendance (median age 25 years). Patients with complex heart disease and Eisenmenger reaction had earlier clinical deterioration (18.6+/-11.3 vs 26.7+/-12.2) and shorter survival (25.8+/-7.9 vs 32.5+/-14.6 years). Thirty-eight (20.2%) patients had at least one episode of haemoptysis between 20 and 40 years of age but it did not modify overall survival; 25 (13.2%) had pulmonary thromboembolism at a mean age of 35.2+/-13.4 years. Fifteen (7.9%) had a stroke and 7 (3.7%) a cerebral abscess at a mean age of 31.4+/-15.7 and 24.1+/-4.9 years, respectively. Cerebral complications influenced the quality of life but did not modify survival. Patients who had venesection showed a 2.04 times greater hazard ratio for haemoptysis. Venesection did not reduce cerebral complications and in 20% caused anaemia and iron deficiency. Other non-cardiac surgery with general anaesthesia carried risks (23.5% of deaths). Significant maternal mortality (27%) in relation to pregnancy occurred with constant deterioration in physical status, high incidence of spontaneous abortions (35.8%) and cardiac abnormalities in offspring (20%). Sixty-one patients died during follow-up, mainly by sudden death (29.5%), heart failure (22.9%) and from haemoptysis (11.4%). Eight patients had heart and lung transplantation and five died 1 week to 4 years after transplant. Deterioration in Ability Index (worsening symptoms), age, complex defects, blood creatinine level, right ventricular dysfunction and non-cardiac surgery were variables which affected the prognosis adversely with uni- and multivariate analysis. CONCLUSIONS: patients with Eisenmenger syndrome can survive to the seventh decade with informed medical care and protection from special risks

Adult↗
PubMed1998

Small ventricular septal defects in adults.

AIMS: To establish the frequency of complications in adults with small ventricular septal defects, which have not undergone surgery. METHODS AND RESULTS: One hundred and eighty-eight adults aged 17-72 (mean, 29.2) years with a small ventricular septal defect were studied. They were referred to a national cardiac centre (National Heart Hospital) and specialized grown-up congenital heart unit. One hundred and thirty-eight were examined in 1994-95. Fifty patients (26.6%) had additional cardiovascular lesions, most commonly a bicuspid aortic valve and/or coarctation. Spontaneous closure occurred in 19 (10%) between the age of 17 and 45 (mean, 27) years. Twenty-one (11.2%) had infective endocarditis. Aortic regurgitation developed in 37 (19.7%) patients; it was severe in nine. Atrial arrhythmias (supraventricular tachycardia or atrial fibrillation) occurred in 12 patients. In four patients, atrial fibrillation produced severe right-sided congestion with a left ventricular to right atrial shunt and haemodynamic features suggesting 'restrictive cardiomyopathy'. Four patients had ventricular arrhythmia. Disproportionate left ventricular enlargement on echocardiography and/or chest radiography was present in 26 (13.8%) without lesions to account for it. CONCLUSIONS: Eighty nine patients (47%) aged 17-44 (mean, 26.8) years had no complications through many years, while spontaneous closure occurred in 19 (10%) during adulthood. Forty-six (25%) had serious complications: infective endocarditis (11%), progressive aortic regurgitation (5%), age-related symptomatic arrhythmias (8.5%) and atrial fibrillation the commonest. Accepting that there may be a referral bias for those with complications, the course of a small ventricular septal defect is not necessarily benign during adult life.

Adolescent↗
PubMed1998

Paul Wood.

Explore the source record for details and available documents.

Cardiology↗
PubMed1997

Pulmonary autograft procedure for aortic valve disease: long-term results of the pioneer series.

BACKGROUND: Pulmonary autograft replacement of the diseased aortic valve has not been widely practiced due to concerns regarding late autograft competence and the consequences of creating pulmonary valve disease. To investigate this, the fate of the pioneering series of patients has been determined. METHODS AND RESULTS: The 131 hospital survivors of the pulmonary autograft operation at the National Heart Hospital from 1967 to 1984 were identified and their outcomes determined to 1994. Age at operation was 11 to 52 years, and 109 patients were male. Autograft implantation was orthotopic subcoronary (107), free-standing root (20), or Dacron mounted (2). In 113 patients, homografts replaced the native pulmonary valve. Ten and 20 years after operation, survival was 85% and 61%, freedom from autograft replacement was 88% and 75%, and freedom from replacement of pulmonary position homografts was 89% and 80%, respectively. Causes of deaths (53) included chronic heart failure (13), complications of reoperation (12), and endocarditis (7). Autograft regurgitation, the most common indication for reoperation, appeared primarily technical in nature, usually due to cusp prolapse. Degeneration was found in only 3 of 30 explanted autografts, and the young patients showed no increase in late valve failure. Homografts outperformed other valve replacements in the pulmonary position, but patients with orthotopic subcoronary and root autografts survived similarly. CONCLUSIONS: The pulmonary autograft offers low rates of degeneration, endocarditis, and thromboembolism for a period lasting >20 years, particularly in the young, with reoperation mainly required for malpositioning of the autograft cusps. The capacity of the autograft to maintain viability with minimal degeneration is not matched by any other biological valve replacement.

Adolescent↗
PubMed1997

Cardiac surgery for grown-up congenital heart patients: survey of 307 consecutive operations from 1991 to 1994.

The cardiac surgery performed from 1991 to 1994 in a unit dedicated specifically for grown-up congenital heart (GUCH) patients was reviewed to determine the frequency of various procedures, incidence of first and reoperations, early mortality, and its determinants. The 295 patients, aged 16 to 77 years (31 +/- 13), had 307 operations. First operations (n = 128, 42%) were most commonly for closure of atrial septal defect (n = 40), aortic valve replacement (n = 31) or repair of aortic coarctation (n = 14). Reoperations were more frequent (n = 179, 58%) and divided among first corrective repair (n = 49), reoperation after corrective repair (n = 115), and further palliation (n = 15). First corrective surgery was mainly for aortic valve disease (n = 17), Fallot (n = 7), and lesions needing a Fontan procedure (n = 5). Reoperations after corrective repair were needed for aortic valve disease (n = 43), right-sided conduit (n = 30), or recoarctation (n = 11). Early mortality was influenced by presence of central cyanosis (9 of 49, 18% in cyanotic patients; 12 of 258, 5% in acyanotic; p <0.001), increased number of previous operations (0 = 4%, 1 = 7%, 2 = 11%, >2 = 13%; p = 0.003), and increasing age of patients. Cyanotic patients had more serious postoperative complications: pleural and pericardial effusions, severe bleeding, renal insufficiency, and sepsis, and their hospital stay was longer compared with acyanotic patients (20 +/- 17 vs 11 +/- 8 days; p <0.001). In GUCH patients, reoperations cause the largest demand on cardiac surgical services. Increased survival of patients with complex cardiovascular malformations brings difficult challenges not only to cardiologists but also to cardiovascular surgeons. There is a need to provide continued highly specialized care. Resources, patients, and funding should be concentrated in a few designated centers.

Adolescent↗