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Biomedical subjects

M Gewillig

Publications and source records attributed to M Gewillig.

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

Management of late complications after classic Fontan procedure by conversion to total cavopulmonary connection.

Classic Fontan procedures, such as atriopulmonary connection or atrioventricular connection, are associated with right atrial dilatation and several late complications. Seven patients with univentricular heart who presented with exercise intolerance (n = 7), severely dilated right atrium (n = 7) with pulmonary vein compression (n = 3), atrial arrhythmias resistant to medical treatment (n = 4), cyanosis (n = 4), diffuse effusions and oedema (n = 1), and protein-losing enteropathy (n = 1), underwent conversion to total cavopulmonary connection 5.8-14.4 years after a previous atriopulmonary connection (n = 6) or atrioventricular connection (n = 1). A 14-year-old boy who, preoperatively, was in ventricular failure and a very poor state died early after conversion because of low cardiac output. All survivors had either marked or partial clinical improvement with regression of cardiomegaly, absence of pulmonary vein compression or cyanosis, and recovery of sinus rhythm. Conversion to total cavopulmonary connection appears to be effective in the treatment of late complications after classic Fontan procedures. It should be considered early in symptomatic patients, before significant ventricular dysfunction and clinical deterioration ensue.

Adolescent↗
PubMed1999

Homograft valve insertion for pulmonary regurgitation late after valveless repair of right ventricular outflow tract obstruction.

OBJECTIVE: Pulmonary regurgitation after valveless repair of right ventricular outflow tract obstruction (RVOTO) results in progressive right ventricular (RV) dilatation and dysfunction in an increasing number of patients. Since 1989, we have exclusively used cryopreserved homografts to restore pulmonary valve competence in these patients. Our 9-year-experience with pulmonary valve insertion (PVI) in such cases has been reviewed to evaluate the indications for this procedure and its benefits. METHODS: From 1989 to 1998, 49 patients (original diagnosis: tetralogy of Fallot in 42 patients and pulmonary stenosis in seven) aged from 3 to 42 years (mean 18 +/- 9 years) underwent PVI with homografts late (mean 13 +/- 7 years) after valveless repair of RVOTO (transannular patch, n = 38; pulmonary valvulotomy therefore tau chi infundibular patch, n = 11). Preoperatively, all patients had severe pulmonary regurgitation, cardiomegaly, significant to severe RV dilatation and dysfunction, fatigue, reduced exercise tolerance, and were in NYHA class II (n = 43) or III (n = 6). Ten patients had ventricular arrhythmia. RESULTS: There was one early death, due to air embolism, and one late death, due to ventricular arrhythmia. All survivors but one, who subsequently underwent heart transplant, had symptomatic improvement after homograft insertion. The mean RV end-diastolic diameter decreased from 38 +/- 9 to 26 +/- 8 mm (P < 0.01), and cardiothoracic ratio decreased from 0.62 +/- 0.07 to 0.54 +/- 0.04 (P < 0.01). Good late homograft function was the rule, with all the survivors being free of reoperation for valve failure. At a mean follow-up of 42 +/- 28 months, 41 patients (87% of the survivors) were in New York Heart Association (NYHA) class I and six in class II. Within this group three patients are still in treatment for RV failure and five for ventricular arrhythmias. In these patients, the average interval between RVOTO repair and PVI was significantly longer than in the others (18 +/- 7 vs. 12 +/- 6 years, P < 0.01). CONCLUSION: Homograft PVI is safe and provides clinical improvement with a significant reduction in RV volume overload and excellent mid-term results in most patients with severe PR late after RVOTO repair. This procedure should be undertaken early in symptomatic patients, before severe RV failure and ventricular arrhythmias ensue.

Adolescent↗
PubMed1999

Delineation of the critical deletion region for congenital heart defects, on chromosome 8p23.1.

Deletions in the distal region of chromosome 8p (del8p) are associated with congenital heart malformations. Other major manifestations include microcephaly, intrauterine growth retardation, mental retardation, and a characteristic hyperactive, impulsive behavior. We studied genotype-phenotype correlations in nine unrelated patients with a de novo del8p, by using the combination of classic cytogenetics, FISH, and the analysis of polymorphic DNA markers. With the exception of one large terminal deletion, all deletions were interstitial. In five patients, a commonly deleted region of approximately 6 Mb was present, with breakpoints clustering in the same regions. One patient without a heart defect or microcephaly but with mild mental retardation and characteristic behavior had a smaller deletion within this commonly deleted region. Two patients without a heart defect had a more proximal interstitial deletion that did not overlap with the commonly deleted region. Taken together, these data allowed us to define the critical deletion regions for the major features of a del8p.

Abnormalities, Multiple↗
PubMed1999

Presenting symptoms and clinical features in 130 patients with the velo-cardio-facial syndrome. The Leuven experience.

During the last 5 years, we diagnosed in Leuven 130 patients with a 22q11 deletion. The deletion was familial in 14 out of 110 index patients (12%), which is significantly less compared to previous studies. In 10 patients, the deletion was maternal, in 4 patients paternal. A cardiac defect was the main presenting symptom in 49% of patients. The other patients were ascertained through developmental delay (16%), behavioural disturbances (7%), otorhinolaryngological manifestations (6%), psychiatric manifestations (3%) and mental retardation (2%). In one patient hypocalcemia was the presenting symptom. In another patient the severe immune deficiency led to diagnosis. Most patients presented a wide variety of the classical features of the Velo-Cardio-Facial syndrome. Velopharyngeal incompetence, learning difficulties or mostly mild mental retardation were almost always present, whereas clinical significant hypocalcemia or immune disturbances were rare. Previously un(der)recognised features include polyhydramnios, renal malformations and laryngotracheamalacia or laryngeal stenosis.

Abnormalities, Multiple↗
PubMed1998

Prenatal diagnosis of a terminal short arm deletion of chromosome 8 in a fetus with an atrioventricular septal defect.

We report the prenatal diagnosis at 30 weeks of gestation of a del(8)(p21.3-->pter) in a growth-retarded fetus with an unbalanced atrioventricular septal defect (AVSD) and a hypoplastic right ventricle. This observation further confirms the association of AVSD with terminal deletions of chromosome 8p. Terminal deletions of chromosome 8p are more frequent than previously thought, but small terminal deletions can easily be overlooked. This observation illustrates that when an AVSD is diagnosed prenatally, special attention should be paid to distal chromosome 8p.

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

Mid-term results of the Ross procedure.

METHODS: Fifty patients underwent a Ross operation between June 1991 and October 1996. Preoperative diagnosis was: congenital aortic valve disease (31 patients), complex left ventricular outflow tract (LVOT) obstruction (11 patients), outgrowth of a small aortic valve prosthesis (five patients) and valve endocarditis (three patients). Half of the interventions were reoperations. All operations were root replacements. A pulmonary homograft was used in 45 patients. An aorticoventriculoplasty was combined with the root replacement in the 16 patients with LVOT obstruction and a too small aortic valve prosthesis. An enlargement or reduction plasty of the ascending aorta was necessary in seven patients. RESULTS: The mean age was 20.9 years (range: 2.5-54 years). The mean follow up was 34.2 +/- 21 months and was 94% complete. Two patients died after 8 days (low cardiac output due to myocardial fibrosis) and 17.4 months (sudden death), respectively, resulting in a survival of 95 +/- 4% at 4 years. Those two deaths occurred in the group of patients undergoing Ross procedure and aorticoventriculoplasty. Two autografts were replaced after respectively 2 days (technical failure) and 44 months (progressive root dilatation) resulting in a reoperation-free incidence of 93 +/- 6% at 4 years. Other postoperative major complications occurred in six patients. All survivors had regular echo-Doppler examination. All autografts except one had a systolic gradient below 10 mmHg at the last examination. Thirty-four autografts had no leak, ten showed grade 1-2 regurgitation. Two patients showed a higher than grade 3 regurgitation: one leak remains stable with normal left ventricular dimensions and function, one autograft was replaced by a mechanical valve. CONCLUSION: This experience demonstrates that the medium-term results of the Ross procedure are excellent even in complex LVOT obstructions.

Adolescent↗
PubMed1998

Ventilatory inefficiency for carbon dioxide during exercise in patients with pulmonary hypertension.

The purpose of this study was to analyse the ventilatory efficiency for carbon dioxide (CO2) in the assessment of exercise tolerance in patients with pulmonary hypertension. Two groups of patients were studied. One group consisted of 37 patients with normal pulmonary artery pressure who underwent surgical closure of a left-to-right shunt. Another group was composed of 10 patients with pulmonary hypertension. Both patient groups were compared with the mean value of an age-matched pool of normal control subjects. Aerobic exercise performance was assessed by determination of the ventilatory threshold. The ventilatory efficiency for CO2 was assessed by calculating the slope of ventilation over carbon dioxide below the ventilatory threshold. The slope of ventilation vs. carbon dioxide output in patients with pulmonary hypertension (48 +/- 12) was steeper than the slope (31.3 +/- 7.0) in patients with normal pulmonary artery pressure (P < 0.05). Significant correlations (P < 0.05) were found between the pulmonary artery pressure and the slope of pulmonary ventilation over carbon dioxide (r = 0.92) and also with the physiological dead space-tidal volume ratio at maximal exercise (r = 0.55). This was associated with a severe reduction in exercise performance in patients with pulmonary hypertension, whereas in patients with normal pulmonary artery pressure it was only slightly below the lower limit of normal. These results demonstrate that patients with pulmonary hypertension have an inefficient ventilatory response to metabolically produced CO2 during exercise, which severely limits exercise tolerance.

Adolescent↗
PubMed1998

Tilt training: a new treatment for recurrent neurocardiogenic syncope and severe orthostatic intolerance.

Medical treatment of neurocardiogenic syncope is insufficient in many cases. We have observed a therapeutic effect of repeated head-up tilt testing. Therefore, we have started a program of tilt training for heavily symptomatic patients. After hospital admission, they were tilted daily (60 degrees inclination) until syncope, or until a duration of 45-90 minutes (90 sessions in 13 patients). The mean tilt tolerance, at the first diagnostic head-up tilt table test, was 22.3 minutes (st. dev. 10.9). Before hospital discharge, 12/13 patients could sustain the full duration of tilt table testing without any symptom. In one patient syncope persisted. The patients were instructed to continue a program of daily tilt training at home, by standing against a wall for 30 minutes, one or two times per day. This resulted in a complete disappearance of syncope in all 13 patients. Orthostatic intolerance and the excessive autonomic reflex activity of neurocardiogenic syncope can be remedied by a program of continued tilt training, without the administration of drugs.

Adult↗
PubMed1998

Percutaneous treatment of stenosed major aortopulmonary collaterals with balloon dilatation and stenting: what can be achieved?

BACKGROUND: The natural history of major aortopulmonary collateral arteries (MAPCAs) in patients with pulmonary atresia and ventricular septal defect (PA-VSD) is frequently complicated by progressive stenosis, leading to pulmonary hypoperfusion and debilitating hypoxaemia. OBJECTIVE: To evaluate balloon dilatation and stenting for relief of stenoses and improvement of pulmonary flow in patients with PA-VSD. DESIGN: Retrospective analysis of all patients where dilatation of MAPCA stenoses was attempted. PATIENTS: Twelve patients with stenotic MAPCAs. INTERVENTIONS: Dilatation was attempted in 25 stenoses. Vessels were stented if elastic recoil was noticed (n = 3), in the presence of long segment stenosis (n = 4) or marked tortuosity (n = 1). MAIN OUTCOME MEASURES: Diameter of stenoses before and after dilatation as well as arterial oxygen saturation data. Patients proceeding to surgical therapy. RESULTS: Two stenosed MAPCAs could not be crossed by a catheter. Four lesions were non-dilatable despite the use of high inflation pressures (18 atm). Six stenoses could be completely dilatated using angioplasty only; in five, only partial dilatation was obtained; eight stenoses needed stenting. In the group with partial expansion the mean (SD) diameter increased from 1.7 (0.8) to 3.5 (1.7) mm (p < 0.05); where full dilatation was achieved it increased from 2.1 (0.8) to 4.8 (1.9) mm (p < 0.05); and in the stented group in increased from 2.3 (0.9) to 5.0 (2.5) mm (p < 0.01). Percutaneous arterial oxygen saturation increased from 75(8%) to 82(8)% (p < 0.001). No complications were experienced during the procedures. Repeat dilatation was attempted in six stenoses, but only two procedures were successful. There were two episodes of vasospasm and in one an aneurysm had developed after redilatation. Two patients proceeded to outflow plasty and two subsequently had a unifocalisation procedure. CONCLUSIONS: Pulmonary blood flow can be improved using balloon angioplasty or stents in patients with stenotic MAPCA; however, 17% of the lesions were not dilatable. Procedures are generally safe, but carry a small risk of vasospasm, dissection, occlusion or aneurysm formation.

Adolescent↗
PubMed1998

Self expandable stents for relief of venous baffle obstruction after the Mustard operation.

OBJECTIVE: Obstruction of the venous pathways after Mustard repair for transposition of the great arteries is associated with an increased risk of arrhythmia and sudden death. The purpose of this study was to assess the effectiveness of the largest (tracheal 22 x 40 mm) Wallstents in treating baffle obstructions. DESIGN: Retrospective analysis of patients with stented venous pathways. SUBJECTS: Eleven patients with baffle obstruction after Mustard repair for transposition of the great arteries. INTERVENTIONS: Stenoses were dilated with an 18 or 20 mm balloon. However, recoil was noticed in 11 patients: immediately (n = 7) or on repeat angiography (n = 4). Eighteen stents were implanted (mean (SD)) 18 (3.3) years postoperatively. After dilatation a tracheal Wallstent (11.5 F) was deployed. MAIN OUTCOME MEASURES: Relief of obstruction, haemodynamic improvement. RESULTS: In the inferior vena cava, 10 stents were deployed in seven baffle obstructions with an increase in diameter from 9.8 (2.4) mm to 16.5 (1.4) mm (p < 0.01) and a mean (SD) pressure gradient decrease from 5.1 (3.6) mm Hg to 1.4 (2.0) mm Hg; in the superior vena cava, eight stents were implanted increasing the diameter from 9.1 (3.7) mm to 15.6 (3.8) mm (p < 0.001) with a decrease in mean pressure gradient from 5.1 (2.7) mm Hg to 1.9 (1.5) mm Hg. No complications were experienced during implantation. No anticoagulation was prescribed. During follow up (1.7 (0.6) years; range, 0.9-2.6) no problems were noted; five patients were re-catheterised without change in measurements. There was no evidence of peal formation in any of the stents. CONCLUSION: It is concluded that Wallstents are safe, easy to use, and effective in relieving baffle obstruction. Anticoagulation does not seem necessary.

Adolescent↗
PubMed1998

Polyhydramnios as a prenatal symptom of the digeorge/velo-cardio-facial syndrome.

Prenatal diagnosis of the DiGeorge/velo-cardio-facial syndrome has become possible since it was recognized that this syndrome is caused by a submicroscopic deletion in chromosome 22q11. In a sporadic patient presenting a conotruncal heart defect and polyhydramnios, the del 22q11 was made prenatally by fluorescence in situ hybridization (FISH) after amniocentesis. Seven additional patients with a del 22q11 were identified, who presented during pregnancy with polyhydramnios. In one of them, unilateral hydronephrosis was present. These findings further add to a growing list of clinical presentations of a del 22q11 and suggest that in patients with polyhydramnios and a conotruncal heart defect or uropathy, fetal karyotyping should be complemented by FISH for a del 22q11.

Amniocentesis↗
PubMed1998

Primary balloon dilatation in coarctation of the aorta complicated by severe aortic insufficiency.

The combination of coarctation of the aorta in the presence of severe aortic insufficiency poses a serious clinical problem. Although successful single- and two-stage repair for combined coarctation in the presence of severe aortic regurgitation has been described, the surgical management of this lesion remains particularly difficult. The analysis of larger series of patients operated upon for coarctation reveals significant early mortality rate in patients with associated severe aortic insufficiency. Although the exact cause of the acute left ventricular failure remains unclear and is a matter of debate, one can assume that changes in the haemodynamics, resulting in global myocardial ischaemia from impaired coronary blood supply or a massive volume overload of the left ventricle after the correction of the coarctation, could have led to myocardial irritability and left ventricular failure. We present a three-stage repair with subtotal relief of the coarctation by balloon angioplasty and stenting first; elective aortic valve replacement in a second stage and finally total balloon dilatation of the residual stenosis at the previously subtotal dilated coarcted segment.

Adolescent↗
PubMed1998

Terminal deletion of chromosome 10q26: delineation of two clinical phenotypes.

We present genotype-phenotype correlations in two patients with distal 10q deletion. A patient with a small terminal deletion presented mild mental retardation and behavioral difficulties with hyperactivity, whereas the patient with a larger deletion, had multiple congenital anomalies and moderate mental retardation. Our observation confirms the previous suggestion that larger deletions of distal chromosome 10q are associated with a more severe clinical presentation, whereas hyperactive behavior may be a specific feature of small terminal deletions of chromosome 10q26.

Abnormalities, Multiple↗
PubMed1997

A recognisable behavioural phenotype associated with terminal deletions of the short arm of chromosome 8.

We report the clinical findings in 5 patients with a terminal deletion of the short arm of chromosome 8. Mild developmental delay was constantly present, in association with microcephaly in 4 of 5 patients. Facial anomalies were mild or absent. A congenital heart defect was present in 3 patients: an atrioventricular septal defect (AVSD) in 2 and an atrial septal defect type II (ASDII) with pulmonary stenosis in one. A highly similar pattern of behavioural difficulties was present in the 3 older children (8-11 years), with outbursts of aggressiveness and destructive behaviour. Follow-up in one patient showed that at the age of 16 years, these behavioural problems had largely disappeared. This observation suggests that in addition to mental retardation, microcephaly, congenital heart defect (typically AVSD), a terminal deletion of chromosome 8p may be associated with a characteristic behavioural phenotype during childhood.

Behavioral Symptoms↗