The DA and the case of the plug.
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Biomedical subjects
Publications and source records attributed to J Rohmer.
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Few data exists on the differences in long-term outcome between Mustard and Senning operations. We reviewed available data of all hospital survivors of these operations and assessed risk factors for late death and sinus node dysfunction. Of those patients undergoing the Mustard operation, 60 were hospital survivors (46 simple transposition, 14 complex); of those patients undergoing the Senning operation, 62 were hospital survivors (43 simple, 19 complex). Median duration of follow-up was 16 years (maximum 25 years) for Mustard operation, 11 years (maximum 20 years) for Senning operation. No reoperations were done, except for pacemaker implantation. No differences were found between the two groups with regard to baffle-associated problems, right ventricular failure, sudden death (6% in both groups), and functional status at final follow-up (New York Heart Association class I or II, except for four patients). For patients undergoing the Mustard operation, survival at 16-year follow-up was 91% with simple transposition and 60% with complex transposition (p = 0.027); for both groups of patients undergoing the Senning operation, survival at 16-year follow-up was 78%. Survival in the absence of rhythm disturbance at 16-year follow-up was 18% for Mustard operation and 53% for Senning operation (p < 0.001). In multivariate analysis, significant independent risk factors for late death turned out to be complex transposition (versus simple) and active arrhythmias. The only significant risk factor for the occurrence of sinus node dysfunction was the Mustard operation. We conclude that apart from the difference in the loss of sinus rhythm, no differences were found in the long-term clinical results of the two types of operations.
Between 1953 and 1985, 109 consecutive patients (17% with isolated coarctation) younger than 3 years of age underwent resection of aortic coarctation. These patients had nonelective operations because of congestive heart failure or severe systemic hypertension not responding to medical therapy. Special surgical techniques were used to lower the prevalence of restenosis. Hospital mortality was 32% (35 patients). Logistic regression analysis proved that age at operation, pulmonary artery banding, and type of repair were independent predictors of hospital death. Late mortality occurred in 9 patients. Associated cardiac anomalies were an independent prognostic factor for late mortality. The operation rate for recoarctation was low (5.8%). However, the follow-up study revealed that 30 patients (41%) had recoarctation. The Kaplan-Meier estimate of recoarctation is 86% after 30 years' follow-up in patients undergoing classic end-to-end anastomosis with silk sutures (n = 48). None in the group with an "extended" anastomosis and polypropylene sutures (n = 26) had recoarctation. The Cox analysis revealed age at operation under 6 months to be prognostic for recoarctation. Because of the shorter period of follow-up, the extended anastomosis with polypropylene sutures proved not to be a significant prognostic factor for recoarctation. In the late postoperative period (mean follow-up 16.7 years), blood pressure was elevated in 49% of the patients. At last follow-up 27 (36%) of the 74 survivors had aortic valve disease. Patients operated on for coarctation of the aorta under the age of 3 years need lifelong follow-up for detection of restenosis, hypertension, and valvular disease. Reoperation-free rate is not a good criterion to judge the outcome of operation for coarctation. Longer follow-up will be needed to investigate whether or not the use of the extended anastomosis technique with polypropylene sutures lowers the prevalence of recoarctation.
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Between 1984 and December 1990 balloon valvuloplasty for valvular pulmonary stenosis was planned and performed in 92 children over 6 months of age (range 0.8-17.9 years). Valvuloplasty decreased the invasively determined peak systolic gradient from 61 +/- 34 (mean +/- SD) to 27 +/- 20 mmHg (P < 0.0001). At follow-up cardiac catheterization, performed in 22 patients after 13 +/- 5 months, the gradient was 22 +/- 23 mmHg (ns). Initially the continuous wave Doppler gradient decreased from 61 +/- 23 to 26 +/- 12 mmHg (P < 0.0001). It then remained unchanged both at early follow-up after 0.6 +/- 0.3 years, being 23 +/- 12 mmHg, and at long-term follow-up after 3 +/- 1.7 years (21 +/- 10 mmHg, ns). In the 41 patients treated before the end of 1986 the residual continuous wave Doppler gradient at long-term follow-up after 5.1 +/- 0.8 years was 21 +/- 10 mmHg. Mild pulmonary regurgitation was present in 77% of the patients at early follow-up and in 83% at the last follow-up. Right ventricular hypertrophy on the electrocardiogram was present in 83% of the children before valvuloplasty. At early follow-up it had decreased to 44% with a further decrease to 24% at the last follow-up. Comparison of patients with initial invasive systolic gradients greater and smaller than 50 mmHg revealed no differences between either group with regard to the continuous wave Doppler gradient at last follow-up, the incidence of pulmonary regurgitation and the occurrence of significant complications.(ABSTRACT TRUNCATED AT 250 WORDS)
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)
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.
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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.
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.
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.
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.
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.
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.
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.
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.
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.
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