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Biomedical subjects

J Ottenkamp

Publications and source records attributed to J Ottenkamp.

At least 55 records · Page 3Linked to original sources
PubMed1991

Follow-up of arterial switch operation.

This report describes the experience of the Leiden University Hospital with the arterial switch operation for transposition of the great arteries, with and without ventricular septal defect, and for the Taussig-Bing-anomaly. The longest follow-up was 13 years and 9 months. Follow-up shows good results with almost all surviving patients in functional class I and with a sinus rhythm. Supravalvular pulmonary artery stenosis is noted in some of the patients but is almost always mild and does not show any tendency to progression. Insufficiency of the aortic valve is seen less frequently. Until now it has not caused great concern but, of course, follow-up is still limited.

Double Outlet Right Ventricle↗
PubMed1989

Correction of atrioventricular septal defect. Results influenced by Down syndrome?

The role of Down syndrome (DS) in the outcome of the surgical correction of atrioventricular septal defect (AVSD) was investigated by the analysis of clinical events among 85 patients. Complete AVSD (cAVSD) was present in 49 patients, of whom 36 (73%) had DS. Thirty patients (83%) survived surgical treatment. Of the 13 patients without DS, 7 (54%) survived. There were four deaths and one late death. Thirty-six patients had partial AVSD (pAVSD), 5 (14%) of whom had DS; all 5 patients are still alive. Thirty (97%) of the 31 patients with pAVSD without DS survived the operation. There were two late deaths. Preoperative selection did not account for the favorable results in children with DS. All early postoperative deaths in cAVSD occurred in children younger than 2 years; low body weight for age seemed to be an important factor in adverse outcome. A large number of patients without DS had other serious congenital malformations. In view of the relatively favorable results in patients with DS, it does not seem warranted to maintain a conservative diagnostic and therapeutic approach of AVSD solely because of concomitant DS.

Chi-Square Distribution↗
PubMed1989

Anatomic correction for transposition of the great arteries: first follow-up (38 patients).

Between April 1983 and October 1985, 38 consecutive patients with transposition of the great arteries (TGA) underwent anatomic correction. Ages ranged from 1 day to 284 weeks (mean 26.2 weeks). Simple TGA was present in 17 patients, 17 had an associated ventricular septal defect, and the remaining four had a Taussig-Bing anomaly. Hospital mortality was 2 of 38, with 1 late death. Follow-up time varied from 6 to 35 months. Postoperative cardiac catheterization was performed in 34 of the 36 early survivors. Right ventricular outflow tract obstruction with a systolic gradient greater than 20 mmHg was found in four. A residual left-to-right shunt was found in nine; Qp/Qs exceeded 2.0 in only one. In four, the pulmonary-to-systemic resistance ratio calculated was greater than 0.3. All 35 survivors are in excellent condition. Only one patient has slight aortic insufficiency. Most pre- and postoperative electrocardiographic abnormalities disappeared in due course. Echocardiography revealed normal left ventricular dimensions and fractional shortening. The aortic root diameter showed in almost all a value above the 95th percentile of normal. Postoperatively, the aortic root has grown parallel to, but above, the 95th percentile and as yet has shown no tendency to normalization.

Angiocardiography↗
PubMed1987

The concurrence of fetal supraventricular tachycardia and obstruction of the foramen ovale.

Fetal supraventricular tachycardia (FSVT) is a cardiac arrhythmia that bears a high mortality rate. In the majority of cases no morphological anomalies are found and death is caused by congestive heart failure. This report presents two cases of FSVT in which cross sectional echocardiography revealed obstruction of the foramen ovale with dilatation of the right heart without structural cardiac anomalies. After birth sinus rhythm re-established under digitalis medication in both cases. The association between premature obstruction of the foramen ovale and unexplained intrauterine death in the third trimester of gestation is suggested.

Adult↗
PubMed1987

Tricuspid atresia. Microscopic findings in relation to "absence" of the atrioventricular connexion.

In 10 out of 31 cases of tricuspid atresia (absent atrioventricular connexion), gross examination of the morphologically right ventricle suggested the presence of a tiny inlet portion in addition to the trabecular portion. Serial sections of two of these hearts have proven this diagnosis: a fibrous remnant in continuity with the central fibrous body was present not only above the left ventricle but also above the right ventricle. We interpreted this as a remnant of the atrioventricular valve and we also observed that the ventricle had a hypoplastic inlet portion. This inlet portion was bounded by a microscopically small septomarginal trabeculation containing the right bundle branch. Therefore, the diagnosis of absent connexion, which after clinical or gross anatomical investigation remains an appropriate one, cannot always be maintained after microscopical study if it is considered that the fibrous remnant represents evidence of a connexion initially present between the right ventricle and the right atrium.

Heart Atria↗
PubMed1987

Long-term results after the Fontan operation for tricuspid atresia.

Between 1968 and 1977, 32 patients who underwent the Fontan operation for tricuspid atresia at Bordeaux, France, or Leiden, The Netherlands, survived at least 1 year after surgery. These patients were evaluated clinically 7 to 16 years (mean 8.9 years) after surgery by review of clinical records, questionnaire, or direct examination. There were five deaths: one during arrhythmia, the second sudden and unexplained, and three others after reoperation. Four of the five patients had evidence of obstruction of the atriopulmonary or atrioventricular conduit. There were eight reoperations. Two were for residual atrial septal defects, one for an atrial septal defect and an intrapulmonary arteriovenous fistula, and one for a residual ventricular septal defect. The other four were reoperated for severe conduit or homograft obstruction. One of these four also had a residual shunt, severe mitral regurgitation, and reduced left ventricular function. One patient was found to have left ventricular dysfunction 3 months after surgery, which persisted. Another patient had left ventricular dysfunction unmasked after successful mitral annuloplasty for severe mitral regurgitation. No other patients had clinical evidence of left ventricular dysfunction. Recurrent supraventricular tachycardia occurred in four patients and was eventually fatal in one patient with associated conduit obstruction. Of the 27 survivors, 13 are in NYHA class I, 13 are in class II, and one is in class III. We conclude that clinical results of the Fontan operation remain good in most patients 7 to 16 years after surgery.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1986

The arterial switch operation. An eight-year experience.

Sixty-six patients (23 neonates with transposition of the great arteries and intact ventricular septum, 33 infants and children with transposition and a large ventricular septal defect, and 10 with double-outlet right ventricle with a subpulmonary ventricular septal defect have received an arterial switch repair since 1977. Eight (one with transposition and intact ventricular septum, six with transposition and ventricular septal defect, and one with double-outlet right ventricle with subpulmonary ventricular septal defect) died in the hospital. All other patients have had follow-up as of June, 1985. Including the hospital deaths, the 11 month actuarial survival rate for the entire group was 81%, and no deaths have occurred among the 33 patients alive at that time and traced as long as 8 years. The hazard function for death has only a single early phase, and its 70% confidence limits overlap the hazard function of a matched general population by 12 months after the operation. Incremental risk factors for death included low birth weight (but not weight or age at operation), transposition of the great arteries with large ventricular septal defect, double-outlet right ventricle with subpulmonary ventricular septal defect, and presence of a patent ductus arteriosus. Coronary artery morphology and position of the great arteries were not risk factors. Long aortic cross-clamp time was possibly (p = 0.11) a risk factor. Early date of operation was a risk factor (p = 0.004); thus, predicted 1 year survival rate, including hospital deaths, after the arterial switch operation in 1985 is 99.9% (70% confidence limits 99.0%-100%) for neonates with transposition and intact ventricular septum and 99.7% (98.4%-99.9%) for those with transposition and a large ventricular septal defect or double-outlet right ventricle. The late functional status was excellent, and the rhythm was sinus in 96% of the 55 surviving patients. A formal comparison with the results of the atrial switch repair indicates that the arterial switch repair is superior.

Aorta↗
PubMed1985

Transposition of the great arteries and narrowing of the aortic arch. Emphasis on right ventricular characteristics.

Of 279 patients with transposition of the great arteries, 14(5%) had narrowing of the aorta, including local coarctation (6), isthmus hypoplasia (6), isthmus atresia (1), and kinking of the aorta (1). There were six deaths in 10 surgically treated patients; in addition four patients died before operation. Two of the four survivors had a subpulmonary malalignment ventricular septal defect with angiocardiographic narrowing of the right ventricular outflow tract, two had evidence of redundant muscle tissue obstructing the right ventricular outflow tract. Necropsy showed the presence of anatomical right ventricular outflow tract or inflow tract obstruction or both in all 10 cases. Outflow tract obstruction was represented by anterior displacement of the infundibular septum and ventriculoinfundibular fold (in hearts with a subpulmonary malalignment ventricular septal defect) or by redundant muscle tissue; inflow tract obstruction was represented by hypoplasia of the tricuspid valve. It is concluded that the combination of transposition of the great arteries and narrowing of the aorta is always accompanied by right ventricular outflow or inflow tract obstruction or both; the right ventricular abnormalities are probably responsible for the presence of the aortic arch anomalies by reducing aortic flow during morphogenesis. Successful surgical treatment of this complex anomaly is feasible in selected cases.

Angiocardiography↗
PubMed1985

Percutaneous balloon pulmonary valvuloplasty.

Percutaneous pulmonary valvuloplasty was performed in 27 patients with congenital pulmonary valve stenosis. A fall in the transvalve gradient of at least 15 mm Hg occurred in 22 patients. In five there was little change in the severity of the stenosis; in three of these the pulmonary valve was dysplastic. None of the successfully treated patients had a dysplastic valve. The two other failures, early in the series, were probably due to inadequate balloon size. In one patient the procedure was performed twice, with a successful result from the second dilatation with a larger balloon. Follow up studies in a further six patients showed no evidence of restenosis in those who had been successfully treated and no late improvement in the remainder. There were no important complications. Percutaneous pulmonary valvuloplasty should be the initial treatment for congenital pulmonary valve stenosis, although when the valve is dysplastic the result is less likely to be satisfactory.

Adolescent↗
PubMed1985

[The effect of lowering the hematocrit on polycythemia and thrombocytopenia in congenital cyanotic heart defect].

The clinical history of a 15 year old girl with Down's syndrome and a complete atrioventricular septal defect undergoing blood-letting and plasma administration as a palliative treatment of polycythaemia and thrombocytopenia. A clear clinical improvement and a rapid increase of the thrombocyte count has been observed. The palliative treatment has to be continued with intervals after the therapeutic trial.

Adolescent↗
PubMed1985

Tricuspid atresia. Morphology of the outlet chamber with special emphasis on surgical implications.

An anatomic study of 28 heart specimens with tricuspid atresia showed 15 hearts with ventriculoarterial concordance and a right-sided anterior outlet chamber. Of these 15 hearts, 12 showed evidence of restricted pulmonary blood flow. In eight of these 12 specimens, an anterior deviation of the outlet septum, relative to the primary septum, was present. This deviation caused a narrowing inside the outlet chamber and functioned as infundibular pulmonary stenosis. The outlet chambers of the 15 hearts with ventriculoarterial concordance were compared with those of 13 hearts with tricuspid atresia and ventriculoarterial discordance and also a right-sided anterior outlet chamber. In the latter group of 13 hearts, five showed evidence of restricted pulmonary blood flow, at least partially caused by posterior deviation of the outlet septum relative to the primary septum. A clear difference in outlet chamber morphology was found without exception between specimens with and without ventriculoarterial concordance. Clinical data in three long-term survivors of the Fontan procedure demonstrate that the special morphology of the outlet chamber can pose surgical problems. Especially in patients with ventriculoarterial concordance, when the outlet chamber is incorporated into the pulmonary circuit, the surgeon must give special attention to the specific morphology to prevent undesirable sequelae.

Adolescent↗
PubMed1984

Tricuspid atresia with overriding imperforate tricuspid membrane: an anatomic variant.

An anatomical study of 34 specimens presenting with tricuspid atresia, in which particular emphasis was devoted to the atrioventricular junctional area, revealed 5 cases with an imperforate valve. In 1 heart the valve was completely committed to the right ventricle. In the other 4, however, the membrane was related not only to the right ventricle but also to the left ventricle. A hypoplastic right ventricular inlet portion was found in these 4 specimens. In 2 cases the tricuspid membrane overrode an inconspicuous inlet septal defect. In the other 2 hearts, as studied macroscopically, no defect was present underneath the membrane, which was connected to both right and left ventricles. These 4 specimens also had other ventricular septal defects. In classical tricuspid atresia, characterized by absence of the inlet portion of the right ventricle, the inlet septum is absent. In contrast, these 4 hearts did have an hypoplastic inlet septum.

Atrioventricular Node↗