Double-inlet left ventricle: successfully staged ventricular septation with 12.5 years follow-up.
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Blood Vessel Prosthesis Implantation↗
Biomedical subjects
Jaap Ottenkamp
Publications and source records attributed to Jaap Ottenkamp.
Disease-related difficulties and satisfaction with level of knowledge in adults with mild or complex congenital heart disease.
OBJECTIVES: To evaluate difficulties in daily life, and satisfaction with level of knowledge about their disease, in patients with congenital cardiac disease in order to improve counselling. METHODS: A self-administered questionnaire was completed by 80 patients with mild, and 76 with complex, congenital cardiac disease. They were aged from 17 to 32 years. RESULTS: Even patients with only mild malformations experienced difficulties related to their disease, but being found in only 11%, these were significantly less than those uncovered in 87% of those with complex disease (p < 0.001). Those patients with complex malformations frequently felt restricted in choices because of their disease in areas such as sport (59%), employment (51%), and education (34%). Other difficulties reported were: paying a higher premium for life insurance (29%), having to give up on a sport (28%), and being excluded from a job (18%). Depending on the item, between one-fifth and two-thirds of participants reported gaps in knowledge, most frequently for "causes of congenital cardiac disease", "future consequences", and "family planning". For 53% of those with mild anomalies, and 93% of severely affected patients, the cardiologist is the most important source of information. CONCLUSIONS: A minority of adults with mild, and a majority of those with complex congenital cardiac disease report difficulties in daily life. A substantial number of these patients feel that they have an inadequate level of knowledge about their disease. Our results suggest the need for a specific programme of counselling.
Activities of Daily Living↗
The need for cardiac follow-up in adults with mild congenital cardiac disease.
OBJECTIVE: To discuss the need for continuing cardiac surveillance in adults with hemodynamically insignificant congenital cardiac disease. METHODS: In 82 patients with mild congenital cardiac malformations, aged from 17 to 32 years, we investigated the subjective health status, the health-related quality of life, any difficulties encountered in daily life, the regularity of follow-up, current diagnosis, and antibiotic prophylaxis. RESULTS: The subjective health status, and the health-related quality of life, did not differ from those of the general population. Nevertheless, patients experienced unnecessary difficulties with choice of sport, obtaining insurance cover, and education. After clinical re-evaluation, diagnosis and antibiotic regimes had to be changed in 9 patients (11%). CONCLUSIONS: Patients with a mild congenital cardiac malformation consider themselves to be in good health. So as to fine tune the diagnosis, and update the information supplied to the patients, we suggest that at least a cardiological assessment be carried out at the age of 16 to 18 years. In this way, patients might be protected from unnecessary difficulties, such as restrictions for sport or the charging of unjustifiably high rates for insurance.
Activities of Daily Living↗
The risk of postthrombotic syndrome in children with congenital heart disease.
The lower extremities of 28 unselected children with congenital heart disease were investigated and classified according to the criteria for postthrombotic syndrome five to ten years after their first cardiac catheterization. For the clinical criteria, all patients completed a questionnaire and underwent a standardized physical examination of both legs. For the pathophysiologic criteria, the presence of venous outflow obstruction and reflux was evaluated by color duplex sonography in 24 of the 28 patients. Mild postthrombotic syndrome was present in half the patients. Partial or complete occlusion of the investigated vein was found in four patients (17%). In all patients studied, the venous valves of the deep system were competent. Postthrombotic syndrome frequently occurs in children with congenital heart disease. Prospective studies seem to be justified to investigate the precise incidence and potential risk factors.
Anticoagulants↗
The morphologic nature of noncommitted ventricular septal defects in specimens with double-outlet right ventricle.
BACKGROUND: Lev's contribution to the understanding of the morphology of hearts with double-outlet right ventricle and the surgical feasibility for correction is important and remains in current use. However, the term noncommitted ventricular septal defect remains enigmatic. The aim of this study was to elucidate the morphologic nature of the noncommitted ventricular septal defect in view of its surgical implications. METHODS: We examined 67 specimens with double-outlet right ventricle, focusing on the relationship of the ventricular septal defect to the semilunar orifices. RESULTS: The defect was subaortic, subpulmonary, or doubly committed in 55 specimens. In a further 8 specimens, the defect opened into the outlet portion of the right ventricle, but the distance between the ventricular septal defect and the semilunar orifice was extensive, either because of extreme dextroposition of the aorta or a broad ventriculoinfundibular fold, which, in some cases, was associated with a long-outlet septum. A truly noncommitted ventricular septal defect was found in the inlet in the remaining 4 specimens. An atrioventricular septal defect without extension to the outlet was present in 3 cases, and a ventricular septal defect limited to the inlet was found in another case. The ventriculoinfundibular fold, part of the outlet septum and septal band or septomarginal trabeculation, had fused to form a crestlike structure. The septomarginal trabeculation is a useful landmark in the right ventricle to differentiate the inlet from the outlet in different forms of double-outlet right ventricle. CONCLUSION: We do not suggest to discard the Lev terminology but rather to differentiate the noncommitted ventricular septal defect into 2 types: the truly noncommitted defect of the inlet type and the not-directly-committed defect, which does open into the outlet portion of the right ventricle. The implication for the surgeon is 2-fold. The tricuspid valve or right part of the atrioventricular valve is interposed between the noncommitted ventricular septal defect and the semilunar orifice. The not-directly-committed defect opens into the outlet portion of the right ventricle but is not directly subaortic or subpulmonary.
Abnormalities, Multiple↗
Isolated unilateral absence of a pulmonary artery: a case report and review of the literature.
OBJECTIVE: The purpose of the present study was to determine the symptomatology, diagnostic procedures, and therapeutic strategies of patients with an isolated unilateral absence of a pulmonary artery (UAPA). BACKGROUND: Isolated UAPA is a rare anomaly. Some case reports exist, but the best diagnostic and therapeutic approaches to these patients remain unclear. METHODS: A retrospective analysis was made of 108 cases reported between 1978 and 2000. The database of the National Library of Medicine (MEDLINE) was used to identify cases that were published in any language from 1978 onward. RESULTS: Of the 108 patients identified, 14 were asymptomatic. The median age was 14 years (range, 0.1 to 58 years). Most patients had symptoms such as frequent pulmonary infections (37%), dyspnea or limited exercise tolerance (40%), or hemoptysis (20%). Pulmonary hypertension was present in 44% of the patients. Surgical procedures were performed in 17% of patients, and the overall mortality rate was 7%. CONCLUSION: Only a few patients with isolated UAPA remain asymptomatic during follow-up. The diagnosis can be made by chest radiograph, echocardiography, CT scan, and MRI. Hilar arteries can be shown by cardiac catheterization and pulmonary venous wedge angiography. This is important since revascularization may improve pulmonary hypertension. The avoidance of high altitudes and pregnancy may further improve outcomes.
Adolescent↗