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Biomedical subjects

J Rohmer

Publications and source records attributed to J Rohmer.

At least 19 recordsLinked to original sources
PubMed1991

One stage repair of aortic arch anomalies and intracardiac defects.

Between August 1985 and May 1990, 27 neonates and infants underwent combined correction of intracardiac and aortic arch anomalies through a median sternotomy. Coarctation (CoA) was combined with VSD (6), AVSD (2), Taussig-Bing (TB) heart (5), transposition of the great arteries (TGA) (1), TGA + VSD (2), congenitally corrected TGA + VSD (1) and VSD + myxoid stenotic outlet valves (1). Interrupted aortic arch (IAA) was combined with VSD (10) and TB heart (1). Two patients had IAA type B as well as CoA. Age at operation varied from 2 to 243 days with a mean age of 51 days. Twenty patients (70%) were younger than 30 days. One TGA + VSD and all TB hearts had maligned outlet septum and right ventricular outflow tract obstruction (RVOTO). Posterior outlet septum deviation and left ventricular outflow tract obstruction (LVOTO) occurred in 8 patients with malalignment VSD and IAA (7) or CoA (1). Aortic arch reconstruction was performed using extended end-to-end anastomoses. In 3 patients, arch hypoplasia necessitated patch implantation. VSDs were closed through the right atrium unless the anatomy dictated otherwise. One TB heart was reconstructed with a Kawashima procedure. All other TB hearts and all TGAs were corrected with arterial switch operation. Obstructing outlet septum was resected whenever necessary. Follow-up was complete and included echo-Doppler control. Eleven patients had postoperative heart catheterisation. Early mortality was 18.5% (5 patients). Persisting LVOTO or RVOTO was responsible. There was no late mortality. Five patients were reoperated upon: 3 for stenotic anastomoses and 2 for a subaortic membranous stenosis. Successful balloon dilatation of recoarctation was performed four times.(ABSTRACT TRUNCATED AT 250 WORDS)

Anastomosis, Surgical↗
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↗
PubMed1991

The neural crest as a possible pathogenetic factor in coarctation of the aorta and bicuspid aortic valve.

Patients (n = 109) operated on for coarctation of the aorta were analyzed for occurrence of associated cardiac and noncardiac anomalies. Attention was also paid to the prevalence of cardiac anomalies in the relatives of these patients. Of the patients with coarctation of the aorta, 57 (52%) had a bicuspid aortic valve. Forty-three (39%) of the 109 patients had one or more noncardiac anomalies. In 29 (27%) patients the noncardiac anomaly involved the head/neck structures. Noncardiac anomalies were much more prevalent in patients with coarctation and bicuspid aortic valve, especially anomalies involving the head/neck structures: 44% compared to 8% of patients with a normal aortic valve. Congenital cardiac malformations were present in relatives in the first or second degree of 18% of the patients. Bicuspid aortic valve was more prevalent in patients with an affected relative (75%) than in patients with unaffected relatives (47%). Recent studies showed that the neural crest plays an important role in the development of cardiac and a variety of noncardiac structures. The cardiac structures derived from the neural crest involve the outflow tract of the heart and the aortic arch system. Maldevelopment of neural crest cells could therefore be responsible for the combined occurrence of outflow tract (e.g., bicuspid aortic valve), aortic arch (e.g., coarctation), and noncardiac anomalies. This study supports the concept that some anomalies of the aortic arch system, including aortic coarctation, are cardiovascular manifestations of a spectrum of anomalies involving the head and neck region that may be due to a genetic-environmental disorder of the neural crest.

Aortic Coarctation↗
PubMed1990

[Closure of the persistent ductus arteriosus (Botalli) using a catheter procedure; the initial 50 patients treated in The Netherlands].

Experience gained with non-surgical catheter occlusion of patent ductus arteriosus in 50 patients (1-65 years) is described. Embolisation of the Rashkind device into the right pulmonary artery occurred in 1 patient. In another patient the device was removed because the prongs of the proximal umbrella could not be visualized. In 4 patients a considerable residual shunt was successfully treated with implantation of a second device. In 1 of these 4 patients this resulted in disappearance of considerable haemolysis as well. Five patients, however, still have a--haemodynamically insignificant--residual shunt. In 43 patients catheter occlusion of the ductus arteriosus was 100% successful. The advantages and disadvantages of this method are briefly compared with those of surgical treatment.

Adolescent↗
PubMed1990

Coronary artery fistula as source of pulmonary circulation in pulmonary atresia with ventricular septal defect.

Four patients are described with pulmonary atresia and ventricular septal defect, in whom the pulmonary circulation was dependent on a fistula from the left coronary artery to the pulmonary artery. The issue in this complex anomaly is complete preoperative diagnosis, including anatomic information on the coronary artery fistula and the pulmonary vasculature. This was achieved in the last 2 patients. In the last patient echocardiography turned out to be an important diagnostic tool in this rare anomaly and facilitated selective angiocardiography. All 4 patients were successfully operated by closing the fistula, closing other aortopulmonary connections and inserting a valved conduit between right ventricle and pulmonary artery. The ventricular septal defect was closed in 3 patients with a patch. In the setting of an already existing pulmonary hypertension and a possibly inadequate pulmonary arterial system at surgery, a perforated patch was inserted in the ventricular septal defect of the remaining patient.

Adolescent↗
PubMed1990

[Points of departure in sports counseling of children with congenital heart defects].

When counseling children with congenital heart disease who want to participate in sports, it is, generally speaking, preferable to choose a dynamic sport rather than a static one. Also, the degree of intensity is of major importance. In this connection it is useful to differentiate between recreation-oriented and achievement-oriented participation in sports. The extra risks for a child with congenital heart disease are progression of the severely of the anomaly, rhythm-disturbances, sudden death, and complications due to medication. For the majority of these children the recommendation will be a strictly individual one, based on the (exercise)electrocardiogram, the 24-hours-ECG monitoring, and the echo-Doppler-cardiogram. Invasive techniques are usually unnecessary.

Arrhythmias, Cardiac↗
PubMed1989

[Consensus heart and vascular diseases and sports].

A consensus meeting on persons with a cardiovascular disease engaging in sports was held on 25 November 1988. The purpose was to arrive at uniformity in counselling and attendance. For these purposes, the exercise tolerance of the cardiovascular patients has to be weighed against the expected work load. The recommendations should be based on examinations suitable to the nature of the disorder. Guidelines for performance- and recreation-directed athletic activities are presented for a number of cardiovascular diseases. In counselling and attendance the medication used should also be taken into account. Expertise in counselling and attendance is of great importance for prevention of injuries. This is mostly achieved by team work with the sports physician coordinating.

Cardiovascular Agents↗
PubMed1989

[The effect of the anthracycline derivative doxorubicin on myocardial function in children].

Echocardiograms of 47 children treated with doxorubicin in combination with other cytostatic agents in the period 1979-1984 were reviewed. The authors compared the first and the last echocardiographic registration of every individual patient, analysing the left ventricular dimensions and the shortening fraction which is the indicator of myocardial contractility. Statistical analysis revealed a significant dilation of the left ventricle with a decrease of contractility in the whole group of patients during the therapy. Seven out of 9 patients who had an abnormal shortening fraction at the last measurement died of malignancy. Three children, all in remission of their malignancy, developed a dilated cardiomyopathy that was fatal in two. The possibilities for prevention of this serious complication by means of serial echocardiography during treatment with anthracycline derivatives are discussed.

Adolescent↗
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↗
PubMed1989

[A loud second heart sound].

When a loud second heart sound is heard in a child, the possibility of pulmonary hypertension must be borne in mind. The second heart sound is also loud in patients with congenitally corrected transposition (CGT) because of the anterior position of the aortic valve. Therefore, it is difficult to distinguish the relatively rare isolated CGT from cardiac disease with high pressure in the pulmonary artery. We discuss this diagnostic problem on the basis of a two year old boy with a loud second heart sound.

Angiocardiography↗
PubMed1988

Assessment of hypertrophy in myocardial biopsies taken during correction of congenital heart disease.

Myocardial biopsies were obtained from 27 patients undergoing corrective cardiac surgery for congenital heart disease. Normal hearts of 18 autopsied patients were used as reference. The biopsy material was assessed for desoxyribonucleic acid (DNA) concentration and ploidy profile of cell nuclei in order to quantitate myocardial hypertrophy at the time of operation. DNA-concentration decreased significantly with age (r = -0.76; p less than 0.001). Ploidy profile of myocardial nuclei correlated with age: the relative number of diploid nuclei decreased (r = -0.67; p less than 0.001), the relative numbers of tetraploid and octoploid nuclei increased with age (r = 0.58; p less than 0.01 and r = 0.77; p less than 0.001 respectively). At 8 years of age the patients with congenital heart disease reached myocardial DNA-concentrations comparable with those in normal adult hearts. At higher age the patients with congenital heart disease exceeded normal adult values for myocardial DNA-concentration. These findings are interpreted to represent rapid development of hypertrophy with an early onset, reaching at 8 years of age values observed in normal adult hearts. Quantitation of myocardial hypertrophy by DNA-concentration and ploidy profile of nuclei may offer a means to explain some of the factors of influence on the outcome of corrective cardiac surgery for congenital heart disease in relation to its timing. Our data stress the need for preventing irreversible myocardial damage by timely (surgical) therapy.

Adult↗
PubMed1986

Levotransposition of the aorta: identification of segmental cardiac anatomy using MR imaging.

Several reports have shown that high-quality magnetic resonance (MR) images can be obtained in patients with congenital heart disease. However, little attention has been paid to the segmental analysis of cardiac MR images. The authors believe that this approach is essential in the evaluation of complex congenital cardiac abnormalities. The segmental approach is based on the morphologic identification of the different cardiac compartments and the type of connection among these compartments. The authors applied the basic principles of the segmental approach to the interpretation of MR images obtained in a group of patients with levotransposition of the aorta. Results indicate that this logical step-by-step method can be used to advantage in the accurate analysis of MR images obtained in patients with complex congenital cardiac disease. In this report, the authors discuss the results obtained when the segmental approach is used to interpret cardiac MR images.

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↗