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Biomedical subjects

J V De Giovanni

Publications and source records attributed to J V De Giovanni.

At least 19 recordsLinked to original sources
PubMed2001

Early results of right ventricular-pulmonary artery conduits in patients under 1 year of age.

OBJECTIVES: Management strategies for the repair of many complex heart defects require the implantation of a valved conduit between the right ventricle (RV) and the pulmonary artery (PA), often using aortic or pulmonary homograft valves. Their limited availability, however, has led to the development and use of new conduits. We retrospectively compared our experience with small homografts in patients of less than 1 year of age with the TissueMed bioprosthetic valved conduit. METHODS: From March 1994 to November 1997 29 patients in their first year of life underwent conduit implantation for complex heart defects. These were retrospectively reviewed in order to determine the incidence of death or conduit stenosis. Seventeen patients received homografts and 12 TissueMed conduits. RESULTS: Diagnoses and operative details including conduit size were similar in the two groups and in all cases complete repair of the underlying defect was carried out. Early post-operative mortality was 4/17 (23.5%) in the homograft group and 3/12 (25%) in the TissueMed group. Echo Doppler evaluation within 1 month of operation showed no right ventricular outflow tract (RVOT) obstruction in any of the survivors. In the TissueMed group 8/9 (77%) survivors have gone on to develop significant RVOT obstruction within 12 months of operation. There have been three late deaths in this group all related to severe RVOT obstruction. Two patients died during an attempt at balloon dilatation and one patient died of progressive right heart failure. Five patients had successful replacement of the TissueMed conduit. One child remains well with no evidence of RVOT obstruction. At operation to replace conduit, or at autopsy, the stenoses were related to the deposition of fibrous tissue at the anastomotic suture lines. In the homograft group none of the survivors developed RVOT obstruction during the first 12 months post-operatively. There was one late death (non-cardiac in origin) and one child is awaiting conduit replacement 40 months after initial implantation for obstruction. CONCLUSIONS: The homograft is a satisfactory conduit for re-establishment of RV-PA continuity in infancy. Further work needs to be undertaken in order to elucidate the mechanisms of early graft failure in bioprosthetic conduits if these are to be a suitable alternative for RV outflow reconstruction in infants.

Blood Vessel Prosthesis↗
PubMed1999

Divided left atrium in association with tricuspid atresia and discordant ventriculo-arterial connections.

Division of the morphologically left atrium is a rare abnormality, constituting around 0.1% of congenital heart disease. It may coexist with other cardiac abnormalities, and this association may obscure its recognition. We report an uncommon association with tricuspid atresia and discordant ventriculo-arterial connections, the latter dominating the clinical picture. Accurate diagnosis was made by transthoracic cross-sectional echocardiography, and the patient was referred to surgery without need for cardiac catheterisation.

Abnormalities, Multiple↗
PubMed1999

Left ventricular diastolic function in congenital myotonic dystrophy.

OBJECTIVE: Examination of left ventricular function and conduction abnormalities in myotonic dystrophy. DESIGN: Twelve patients (median age, 13.7 years) with myotonic dystrophy had detailed electrocardiography and echocardiography performed. Echocardiographic parameters were compared with body surface area (BSA) matched median normal values. RESULTS: Fractional shortening was slightly reduced (by 28-29%) in three patients and three patients had mild mitral valve prolapse. Diastolic function was abnormal; isovolumic relaxation time (IVRT) and duration of early filling were prolonged compared with control values (median IVRT, 74 v 61 ms). Peak E velocity was increased (median, 0.82 v 0.78 m/s) but atrial phase filling was normal. Heart rate was reduced (median, 68 v 81 beats/min). Conduction abnormalities were common but showed no clear relations with diastolic abnormalities. CONCLUSIONS: Young patients with myotonic dystrophy have myocardial diastolic dysfunction as well as abnormal electrophysiology. The prognostic implications of such abnormalities require further study.

Adolescent↗
PubMed1998

Recovery pattern of left ventricular dysfunction following radiofrequency ablation of incessant supraventricular tachycardia in infants and children.

OBJECTIVE: To assess recovery pattern of left ventricular function secondary to incessant tachycardia after radiofrequency ablation in a group of infants and children. DESIGN AND SETTING: A combined prospective and retrospective echocardiographic study carried out in a tertiary paediatric cardiac centre. PATIENTS: Echocardiographic evaluation of left ventricular size and function in nine children with incessant tachycardia, before and after successful radiofrequency ablation. Age at ablation ranged from 2 months to 12.5 years (mean 4.1 years). Recovery of left ventricular function was analysed in relation to age at ablation (group I < 18 months, group II > 18 months). MAIN OUTCOME MEASURE: Ventricular recovery pattern. RESULTS: Seven of the nine children had left ventricular dysfunction; six of these also had left ventricular dilatation. All children with left ventricular dysfunction had normalisation of ejection fraction and fractional shortening; left ventricular dilatation also improved, but the improvement occurred after recovery of function. There was a shorter recovery time for left ventricular function in younger (group I) than in older children (group II) (mean (SD) 5.7 (7.2) months v 31.3 (5.2) (p < 0.002). CONCLUSIONS: Tachycardia induced cardiomyopathy is reversible following curative treatment with radiofrequency. Recovery of left ventricular systolic function precedes recovery of left ventricular dilatation. Time course to recovery is shorter in younger children.

Age Factors↗
PubMed1998

Adenosine induced transient cardiac standstill in catheter interventional procedures for congenital heart disease.

OBJECTIVE: To describe the use of intravenous adenosine to create transient cardiac standstill during balloon dilatation procedures for congenital heart defects. SETTING: A tertiary paediatric cardiac centre. DESIGN AND PATIENTS: This was a prospective pilot study. Thirteen patients born with congenital heart disease and who had stenotic lesions requiring relief were considered for the technique. All were suitable for balloon dilatation. Their ages ranged from 2 months to 30 years, mean (SD) 9.9 (9.8) years. The dose of adenosine varied from 0.125 mg/kg to 0.555 mg/kg, mean 0.33 (0.127). RESULTS: Two patients only developed sinus bradycardia in response to adenosine, which may have been related to the technique of administration. The other 11 experienced a period of asystole, which ranged from 2.4 to 10.8 seconds, mean 4.99 (2.27), and a total atrioventricular block period of 5.0 to 21.2 seconds, mean 9.47 (4.64). The interval between adenosine injection and the onset of asystole varied from 2.4 to 15.8 seconds, mean 8.05 (3.6), depending on cannula size, site of administration, and cardiac output. The peak gradient across the stenotic lesions fell from 52.3 (23.7) to 17.8 (11.9) mm Hg (p < 0.001). Apart from one short episode of atrial fibrillation there were no complications. CONCLUSIONS: Intravenous adenosine is a safe and effective agent for creating transient cardiac standstill during balloon dilatation procedures for congenital heart disease. This achieves stability which is likely to improve results and reduce complications. It may have applications in other fields of cardiac intervention where an immobile heart is desirable during the critical phase of a procedure.

Adenosine↗
PubMed1997

Occlusion of persistent left superior vena cava to unroofed coronary sinus using vena cava filter and coils.

A 48 year old female with complex cyanotic heart disease and pulmonary hypertension was partly cyanosed because of a persistent left superior vena cava draining into an unroofed coronary sinus. The left superior vena cava, which measured 22 mm in diameter, was successfully occluded with a Günther Tulip Vena Cava Mreye Filter which acted as a barrier for embolisation coils.

Cyanosis↗
PubMed1996

Complete atrioventricular septal defects: the influence of associated cardiac anomalies on surgical management and outcome.

OBJECTIVE: Major associated cardiac anomalies are known to increase the risk of repair of complete atrioventricular septal defects (CAVSDs). The purpose of this study was to examine the effects of such anomalies on the current surgical management of CAVSDs and their influence on outcome following repair. METHODS: We performed a retrospective review of a 100 consecutive non-isomeric patients undergoing repair of CAVSD at our institution, between January 1989 and December 1994; patients with partial or intermediate defects were excluded. Complete atrioventricular septal defect patients with other major cardiac abnormalities (complex) were then compared to those with isolated CAVSDs. RESULTS: There were 15 patients (15%) with associated anomalies; 3 had tetralogy of Fallot, 1 patient had pulmonary atresia, 6 had hypoplastic left or right ventricle, 1 had tetralogy of Fallot and hyperplastic right ventricle, 2 patients had double outlet right ventricle, 1 had hypoplastic aortic arch and 1 patient had aortic coarctation. The median age at operation was similar for both groups (4.2 months), while the median weight was not significantly different for isolated CAVSDs compared to complex (4.2 months vs 3.4 months, P = 0.89), but there was a higher incidence of trisomy 21 (70/85, 82% vs 8/15, 53.3%, P = 0.01). Two of the 85 isolated CAVSD patients (2.3%) had undergone palliative pulmonary artery banding, while 5 of the 15 complex patients (33.3%) had either banding or Blalock-Taussig shunts performed. The technique of CAVSD repair was identical in each group. All complex patients had standard repair of their associated anomalies. Hospital mortality was higher in the complex group (3/15, 20% vs 2/85, 2.3%, P = 0.004); all early deaths in the complex group occurred in patients with a hypoplastic ventricle. Reoperation for left atrioventricular valve regurgitation was required in six isolated CAVSD patients (7.1%) and in one complex (6.6%). CONCLUSIONS: In the absence of significant ventricular hypoplasia, the early results of surgical repair in patients with CAVSDs and associated cardiovascular anomalies are similar to those achieved in patients with isolated CAVSD.

Aorta, Thoracic↗
PubMed1996

Management and outcome of infants and children with right atrial isomerism.

OBJECTIVES: To assess the current results and outcome of surgery in infants and children with right atrial isomerism and complex congenital heart disease. SETTING: Tertiary referral centre. METHODS: 20 consecutive children with right atrial isomerism and complex congenital heart disease underwent surgery over a 6 year period between August 1987 and July 1993. The results and outcome were analysed according to age, presentation, and surgical procedures. RESULTS: Patients were divided into two groups depending on age at presentation and initial surgery: group A comprised 11 patients who required surgical intervention in the first month of life (mean age 5 days); and group B comprised nine patients who required initial surgical intervention after the first month of life (mean age 6.8 months). Seven (64%) of the 11 patients in group A had obstructed pulmonary venous drainage and ten (91%) had pulmonary atresia. There were seven early deaths (64%), including the five patients who required systemic to pulmonary artery shunt and simultaneous repair of obstructed pulmonary veins. The long-term survival rate in this group was 18% (two of 11). Pulmonary venous obstruction was present in two (22%) of the nine patients in group B and four (44%) had pulmonary atresia. There were no early deaths. One patient died after a second palliative procedure. There was one late sudden death. Four patients had a Fontan operation with no deaths. Two of the remaining three patients meet the Fontan criteria. The long-term survival rate in this group was 78% (seven of nine). CONCLUSIONS: Surgical management of patients with right atrial isomerism who have complex congenital heart disease carries a high mortality and remains palliative. The overall survival rate was 45% (nine of 20); 18% in patients requiring surgery in the first month of life (group A) and 78% in patients requiring surgery after the first month of life (group B) (P < 0.001). Of the total of 20 patients, nine were potential candidates for a Fontan operation. Seven of these have undergone a Fontan procedure with five survivors.

Child, Preschool↗
PubMed1995

Univentricular heart with systemic outflow obstruction: palliation by primary Damus procedure.

In 24 consecutive infants (19 male and 5 female) with complex forms of single-ventricle physiology and systemic outflow obstruction, a modified Damus operation without the use of exogenous material was undertaken in conjunction with creation of an aortopulmonary shunt 3.5 mm in diameter. The median age at operation was 6 days (range, 1 to 170 days) and the median weight, 3.4 kg (range, 2.6 to 4.6 kg). There were nine early deaths. All 15 survivors (median follow-up, 6.5 months) were clinically well without major systemic ventricular dysfunction or atrioventricular or arterial valve regurgitation. Ten of them have undergone a superior vena cava-pulmonary shunt (one death), and 1 has required patch angioplasty of the aortic arch and innominate artery with revision of the aortopulmonary shunt. The 4 other survivors are awaiting a cavopulmonary shunt. Univariate analysis yielded the chronologic rank for an individual procedure (higher risk of death early in the series), presence of aortic arch atresia, and presence or absence of transposition of the great arteries as predictors of death. This aggressive surgical approach provides excellent early palliation, and because the operation prevents abnormal ventricular hypertrophy from pressure or volume overload, systemic ventricular function is optimally conserved for a future Fontan-type procedure.

Cardiac Surgical Procedures↗
PubMed1995

Influence of ethnic origin on the pattern of congenital heart defects in the first year of life.

OBJECTIVE: To assess the prevalence and patterns of congenital heart defects in infants requiring hospital admission in a defined population and to determine the differences in ethnic groups. DESIGN: A three year retrospective analysis of all hospital admissions for paediatric congenital heart defects in a single centre. SETTING: Tertiary referral centre for infant cardiac services in the West Midlands region, United Kingdom. PATIENTS AND METHODS: Indian, Pakistani, Bangladeshi and other individuals from the Indian subcontinent constitute 5.8% of the total population of the West Midlands region. Some 9% of infants, however, are Asian because of a high birth rate. All infants with confirmed congenital heart defects resident in this region who required hospital admission between April 1990 and March 1993 were classified as Asians and non-Asian, mainly white, infants. RESULTS: Of 1111 infants with congenital heart defects born in the West Midlands and admitted to the hospital, 17.0% were Asian, significantly more than the percentage of Asian infants in the population (P < 0.0001). Asian infants had a higher proportion of complex congenital heart disease (7% v 2.1%, P < 0.001), whereas coarctation of the aorta was more common in non-Asian (3% v 9.1%, P = 0.003). Persistent arterial duct seemed to be more common in Asian children (16% v 10%, NS), but this group included preterm infants admitted for duct ligation. There was no significant difference between the two groups in the other nine categories of congenital heart defects. CONCLUSIONS: The estimated prevalence of congenital heart defects requiring hospital admission was higher in Asian infants than in non-Asian (9.45 per 1000 v 4.56 per 1000, P < 0.0001). Complex congenital heart defects were more common in Asian infants whereas coarctation of the aorta was more common in non-Asian.

Asia↗
PubMed1995

Surgery for infants with a hypoplastic systemic ventricle and severe outflow obstruction: early results with a modified Norwood procedure.

OBJECTIVE: Prospective audit of the first year of implementation of a modified approach to palliation for infants with hypoplastic systemic ventricle and severe systemic outflow obstruction. SETTING: Tertiary referral centre for neonatal and infant cardiac surgery. PATIENTS AND METHODS: 17 of 19 infants (aged < 35 days) presenting to Birmingham Children's Hospital in 1993 with hypoplastic systemic ventricle and severe outflow obstruction underwent surgery. This was performed using a new modification of the Norwood-type arch repair, without the use of exogenous material, and a 3.5 mm Gore-tex shunt between the innominate and right pulmonary arteries. The Gore-tex shunt was replaced by a cavopulmonary shunt between 3 and 5 months later. Clinical, morphological, and functional determinants of outcome were examined. RESULTS: 10 (59%) infants survived initial surgery. All proceeded to cavopulmonary shunt without further loss. Significant atrioventricular valve regurgitation seemed to be the main risk factor for poor outcome. If this was excluded, the morphology of the dominant ventricle seemed to have little effect on the outcome of initial surgery. CONCLUSIONS: Early survival was achieved in 59% of patients in the first year of implementation of a protocol for surgery in infants with hypoplastic systemic ventricle and severe outflow obstruction. The construction of a neoaorta without the use of exogenous material may allow improved later growth of the neoaorta. Early cavopulmonary shunt can be performed safely and should reduce mid-term complications from cyanosis and systemic ventricular volume loading.

Female↗
PubMed1995

Combined atrial and arterial switch procedure for congenital corrected transposition with ventricular septal defect.

OBJECTIVES: A combined atrial and arterial switch procedure was performed in selected patients with congenitally corrected transposition to establish the morphological left ventricle as the systemic ventricle. Immediate and early follow up results are presented. BACKGROUND: Progressive right ventricular dysfunction and tricuspid regurgitation are common in patients with congenitally corrected transposition who undergo repair of associated lesions. A surgical procedure which re-establishes the left ventricle as the systemic ventricle should improve functional results. METHODS: Four symptomatic children aged from 9 months to 3 years 1 month (mean 2 years 3 months) with congenitally corrected transposition and ventricular septal defect underwent both an atrial and arterial switch procedure and were followed up for a mean of 12 months (range 6-21 months). RESULTS: There were no early or late deaths. Conduction abnormalities worsened in two patients. Hospital stay ranged from 8 to 17 days (mean 13 days). The cardiothoracic ratio decreased from a mean (range) of 0.65 (0.6 to 0.71) to 0.58 (0.52 to 0.6). Currently, three patients are in functional class I and one child is in functional class II. CONCLUSIONS: The combination of an atrial and an arterial switch procedure in symptomatic children with congenitally corrected transposition establishes the left ventricle as the systemic ventricle. The initial experience is encouraging with excellent immediate and early follow up results.

Child, Preschool↗