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J Ottenkamp

Publications and source records attributed to J Ottenkamp.

At least 19 recordsLinked to original sources
PubMed2007

The involvement of Fc gamma receptor gene polymorphisms in Kawasaki disease.

Kawasaki disease is an acute febrile syndrome in infancy, characterized by vasculitis of medium-sized arteries. Without treatment the disease can lead to coronary artery lesions (CAL) in approximately 25% of the children. Therapy consists of intravenous immunoglobulins (IVIG), leading to a decrease of complications to 5-16%. Little is known about the working mechanisms of IVIG. In this study we evaluated the involvement of Fcgamma receptors (FcgammaRs) in Kawasaki disease by the determination of the frequency of known single nucleotide polymorphisms (SNPs) in the genes coding for the FcgammaRs and compared this with frequencies in a cohort of healthy controls. There was no difference in the distribution of the functionally relevant genotypes for FcgammaRIIa-131H/R, FcgammaRIIb-232I/T, FcgammaRIIIa-158 V/F and FcgammaRIIIb-NA1/NA2 between the patient group and the healthy controls. Furthermore, there were no polymorphisms linked to the disease severity as indicated by the absence or development of CAL during the disease. Altered transcription or expression of FcgammaR on specific cell types of the immune system may still play a role in susceptibility and treatment success, but at a level different from the functional SNPs in FcgammaR genes tested in this study.

Adolescent↗
PubMed2006

[Favourable results with surgical treatment in 43 children with hypoplastic left-heart syndrome or similar disorders, 1999-2005].

OBJECTIVE: To describe the results of surgical treatment of hypoplastic left-heart syndrome (HLHS) and HLHS-like disorders in the Amsterdam-Leiden Centre for Congenital Heart Disease, the Netherlands. DESIGN: Retrospective, descriptive. METHOD: Data were collected on 43 neonates with HLHS or similar disorders who underwent surgical treatment between December 1999 and December 2005. HLHS was present in 37 patients and 6 had disorders similar to HLHS (unbalanced atrioventricular septal defect, truncus arteriosus with hypoplastic left ventricle, double inlet left ventricle). Surgery was performed in 3 steps: Norwood operation shortly after birth (n = 43), bidirectional cavopulmonary anastomosis a few months later (n = 30) and total cavopulmonary connection at the age of 2-3 years (n = 10). During the Norwood operation, the first 21 patients received a modified Blalock shunt (between the right brachiocephalic artery and pulmonary artery), whereas the following 22 patients received a Sano shunt (between the right ventricle and pulmonary artery). RESULTS: Of the 43 patients, 11 died: 7 within 30 days of the first operation, 2 between the first and second operation, and 2 between the second and third operation. Actuarial survival for the entire group is 74% (32/43). The mortality rate was lower with the Sano shunt (9%; 2/22) than with the modified Blalock shunt (43%; 9/21). Catheter interventions were necessary in 10 patients: 6 had balloon dilatation of the distal aortic arch and 4 had balloon dilatation/stent placement for narrowed pulmonary arteries. With a median follow-up of 22 months (range: 1-75), 2 patients had marked neurological side effects. All 32 surviving patients were in good clinical condition.

Child, Preschool↗
PubMed2006

Surgical repair of aortopulmonary window: thirty-seven years of experience.

An aortopulmonary window (APW) is a communication between the ascending aorta and the pulmonary trunk in the presence of two separate semilunar valves. In order to increase our understanding about the surgical management of this rare lesion and its long-term results, we describe our experience over a 37-year period. Between 1968 and 2005, 18 patients were diagnosed with APW. Seventeen underwent surgical correction. Age at operation ranged from 22 days to 22 years (median, 0.20 years). Follow-up ranged from 2 weeks to 28.6 years (median, 11.0 years). Surgical closure was achieved using a single patch in 7 patients (41.2%) double patch in 4 (23.5%), primary closure in 3 (17.6%), clip in 2 (11.8%), and ligation in 1 (5.9%). Complex APW was present in 8 patients (44.4%). One patient was treated nonsurgically. There were no early or late deaths after surgery. Both primary closure and patch closure gave excellent long-term results. Sporadic postoperative complications were only associated with complex lesions. One patient who was treated conservatively died (of pulmonary hypertension) 21 years after diagnosis. Repair of APW is ideally performed in the first months of life, before irreversible PHT has developed. Various surgical repair techniques in this series of patients gave excellent short-term and long-term results, without significant hemodynamic sequelae.

Adolescent↗
PubMed2006

Vascular endothelial growth factor gene haplotypes in Kawasaki disease.

OBJECTIVE: To investigate whether common genetic variants in the vascular endothelial growth factor (VEGF) gene are associated with Kawasaki disease (KD) and the subsequent development of coronary artery lesions. METHODS: Common genetic variants in the VEGF gene were analyzed in an association study in a Dutch cohort of 170 KD patients and 300 healthy Dutch Caucasian controls. Genotyping was done with 5'-nuclease TaqMan assays and 3'-hybridization-triggered fluorescence minor groove binder Eclipse assays. RESULTS: An association with susceptibility to KD was observed with 2 of the 6 single-nucleotide polymorphisms analyzed in VEGF: -2594 A>C (rs699947) and the 236 bp 3' of STP C>T (rs3025039). Also for an 18-bp deletion in the promoter of VEGF a significant difference in the genotype and allele frequencies was observed between the KD patients and the controls. The haplotype CGCC (based on rs699947, rs2010963, rs25648, and rs3025039) was significantly associated with the development of KD (hap score 3.8; P = 0.0002). VEGF plasma levels were significantly higher in patients with the early phase of KD than in the healthy controls, and there was a trend toward higher VEGF plasma levels in KD patients with the -2594 CC and 236 bp 3' of STP CC genotypes. CONCLUSION: Our results suggest that polymorphisms of the VEGF gene may play a role in the pathogenesis of KD.

Adolescent↗
PubMed2006

Maternal hyperhomocysteinaemia is a risk factor for congenital heart disease.

OBJECTIVE: To investigate the inter-relation between mother and infant homocysteine, folate and vitamin B12 status and the risk of a child with congenital heart disease (CHD). DESIGN: Case-control study. SETTING: Erasmus MC, University Medical Centre, Rotterdam, the Netherlands. POPULATION: Participants were 149 case-mothers and their children with CHD (n = 151) and 183 control-mothers with their children (n = 175). METHODS: Approximately 17 months after the index-pregnancy maternal fasting, children's random venous blood samples were drawn to measure plasma total homocysteine, serum and red blood cell (RBC) folate, and serum vitamin B12 concentrations. Data were compared between cases and controls using the Mann-Whitney U test. The biochemical parameters were dichotomised according to the cutoff value of the 10th percentile of vitamin concentrations and the 90th percentile of homocysteine concentrations based on control data. Risk estimates for the association between CHD and the biochemical parameters were estimated in a logistic regression model. MAIN OUTCOME MEASURES: Medians (minimum-maximum) and odds ratios (OR) (95% confidence intervals [CI]). RESULTS: The OR (95% CI) of having a child with CHD was 2.9 (1.4-6.0) for maternal hyperhomocysteinaemia (>14.3 micromol/l). This finding is substantiated by a significant concentration-dependent risk (Ptrend = 0.004). Hyperhomocysteinaemic case-mothers showed significantly lower serum folate and vitamin B12 concentrations than normohomocysteinaemic case-mothers. Serum and RBC folate concentrations were significantly higher in case-children than that in control-children. CONCLUSIONS: Maternal hyperhomocysteinaemia is associated with an increased risk of CHD, partially due to low folate and vitamin B12 status. The folate status of children warrants further investigation.

Adult↗
PubMed2005

[Complete atrioventricular septal defect in children with Down's syndrome: good results of surgical correction at younger and younger ages].

OBJECTIVE: To evaluate the results of cardiosurgical treatment of children with Down's syndrome and a complete atrioventricular septal defect (cAVSD). DESIGN: Retrospective. METHOD: Data were collected from the records of all patients with Down's syndrome who had been subjected to primary corrective surgery for cAVSD in the period 1980-2003 in Leiden, The Netherlands. Exclusion criteria were: concomitant tetralogy of Fallot or interruption of the aortic arch and palliative banding of the pulmonary artery. Children with an abnormal shape of the ventricle making it impossible to correct biventricularly were not considered in the study. Data on mortality >30 days after the operation and on repeated surgery were included in the analysis if the duration of follow-up was at least 5 years. RESULTS: The group comprised 148 children: 75 girls and 73 boys. The median age at time of surgery was 20 weeks (range: 6 weeks to 3.7 years) and showed a statistically significant decrease during the period under investigation. Of 4 children lost to clinical follow-up actual data could be obtained. The median duration offollow-up was 6 years and 7 months (range: 38 days to 23 years and 11 months). 28 children (19%) died. The mortality within 30 days after the operation decreased from 0-38% in the period 1980-1989 via 0-30% in the period 1990-1999 to 0% in 2000-2003. The percentage ofreoperations related to the correction ofcAVSD (14%; 14/98) did not seem to increase. Of the correction-related reoperations, 14% (2/14) were followed by a second reoperation. Conclusion. In the period 1980-2003, children with Down's syndrome and a cAVSD were corrected surgically at a younger and younger age. The early mortality decreased to 0% in the years 2000-2003. Echocardiography in the first weeks of life for all children with Down's syndrome makes it possible to diagnose those with a cAVSD early enough for surgical repair.

Age Factors↗
PubMed2004

A cardiac-specific health-related quality of life module for young adults with congenital heart disease: development and validation.

This study represents the development and validation of a cardiac-specific module of the generic health-related quality of life (HRQoL) instrument, the TAAQOL (TNO/AZL Adult Quality Of Life), for young adults with congenital heart disease (CHD). Items were selected based on literature, an explorative previous study in CHD patients, interviews with patients, and the advice of experts. The newly developed Congenital Heart Disease-TNO/AZL Adult Quality of Life (CHD-TAAQOL) was tested in 156 patients with mild or complex CHD and consisted of three hypothesised subject scales: 'Symptoms' (9 items), 'Impact Cardiac Surveillance' (7 items), and 'Worries' (10 items). Cronbach's alpha for the three scales were 0.77, 0.78, and 0.82, respectively. Scale structure was confirmed by Principal Component Analysis, corrected item-scale and interscale correlations. Overall, 55% of reported health status problems were associated with negative emotions, which is an argument for assessing HRQoL as a concept distinct from health status. Convergent validity with validated generic instruments (TAAQOL and Short Form-36, SF-36) showed satisfactory coefficients. Discriminant validity was proven by significantly higher scores for mild CHD patients compared with those with complex CHD. In conclusion, the CHD-TAAQOL module together with the generic TAAQOL can be used to assess group differences for cardiac-specific HRQoL in young adults with CHD. Testing psychometric properties of the CHD-TAAQOL shows satisfactory results. However, to detect changes in HRQoL over time, further research is needed.

Adolescent↗
PubMed2002

[Good results after closure of a arterial septal defect during heart catheterisation instead of surgery].

OBJECTIVE: Evaluation of the first results in the Netherlands of percutaneous and transvenous closure of an ASD II in children with an Amplatzer Septal Occluder (ASO). DESIGN: Prospective. METHOD: Data were collected from children with an ASD II prior to, during and up to 24 months after the insertion of an ASO during heart catheterisation in Leiden University Hospital, the Netherlands. RESULTS: Between 1 January 1998 and 29 February 2000, 28 patients (12 girls, 16 boys; mean age: 74 months (range: 15-198 months)) underwent heart catheterisation to close an ASD II with an ASO. In 26 patients an ASO could be placed without significant complications. The size of the device varied from 9-34 mm (median 16 mm). In one patient ASD closure was not attempted because of multiple ASDs. In another patient the procedure was stopped after air embolism into the coronary arteries had occurred during preparation of ASO implantation. In 23/26 patients with an implanted ASO, no residual shunt was present after 24 hours. One child, in whom the defect was found to be closed after 24 hours and after three weeks, returned abroad and was lost to follow-up. After one year all defects (n = 22) were completely closed. CONCLUSION: Percutaneous transvenous closure of an ASD II with an ASO was possible, was not associated with any significant complications and had a high success rate, even in relatively young children with large defects.

Adolescent↗
PubMed2002

Health related quality of life and health status in adult survivors with previously operated complex congenital heart disease.

OBJECTIVE: To examine the impact of previously operated complex congenital heart disease on health related quality of life and subjective health status and to determine the relation between these parameters and physical status. DESIGN: Cross sectional; information on medical follow up was sought retrospectively. SETTING: Patients were randomly selected from the archives of the paediatric cardiology department, Leiden University Medical Centre, Leiden, The Netherlands, and approached irrespective of current cardiac care or hospital of follow up. PATIENTS: Seventy eight patients with previously operated complex congenital heart disease (now aged 18-32 years) were compared with the general population. MAIN OUTCOME MEASURES: Health related quality of life was determined with a specifically developed questionnaire (Netherlands Organisation for Applied Scientific Research Academic Medical Centre (TNO-AZL) adult quality of life (TAAQOL)) and subjective health status was assessed with the 36 item short form health survey (SF-36). Physical status was determined with the objective physical index, Somerville index, and New York Heart Association functional class. RESULTS: Health related quality of life of the patients was significantly worse than that of the general population in the dimensions gross motor functioning and vitality (p < 0.01). Correlations between health related quality of life and physical status were poor. Patients had significantly worse subjective health status than the general population in the dimensions physical functioning, role functioning physical, vitality, and general health perceptions (p < 0.01). Correlations between subjective health status and physical indices were weak. CONCLUSION: Adult survivors with previously operated complex congenital heart disease experienced limitations only in the physical dimensions of health related quality of life and subjective health status. Objectively measured medical variables were only weakly related to health related quality of life. These results indicate that, when evaluating health related quality of life, dedicated questionnaires such as the TAAQOL should be used.

Adolescent↗
PubMed2002

ECG predictors of ventricular arrhythmias and biventricular size and wall mass in tetralogy of Fallot with pulmonary regurgitation.

BACKGROUND: In patients with the tetralogy of Fallot, QRS prolongation predicts malignant ventricular arrhythmias. QRS prolongation may result from right ventricular dilatation. The relation of ECG markers to biventricular wall mass and volumes has not been assessed. OBJECTIVE: To investigate the relations of surface ECG markers of depolarisation and repolarisation to right and left ventricular volume and biventricular wall mass. METHODS: 37 Fallot patients (mean (SD) age 17 (9) years) were studied 14 (8) years after surgical repair; 34 had important pulmonary regurgitation. Left and right ventricular size was assessed from tomographic magnetic resonance imaging (MRI), and the amount of pulmonary regurgitation by velocity mapping MRI. QT, QRS, and JT duration and interlead dispersion markers were derived from a standard 12 lead ECG. RESULTS: Mean QRS duration was significantly prolonged (133 (31) v 91 (11) ms in controls), as were dispersion of QRS (36 (17) v 20 (6) ms), QT interval (87 (48) v 42 (20) ms), and JT interval (93 (48) v 42 (19) ms). Biventricular volumes were increased (right ventricular end diastolic volume, 129 (41) v 70 (9) ml/m(2); left ventricular end diastolic volume, 83 (16) v 69 (10) ml/m(2)), as was right ventricular wall mass (24 (7) v 17 (2) g/m(2)). QRS duration correlated best with right ventricular mass (r = 0.55, p < 0.01). CONCLUSIONS: In patients operated on for tetralogy of Fallot and with pulmonary regurgitation, ECG predictors of ventricular arrhythmias are influenced by several mechanical factors that may occur simultaneously. These include increased right ventricular volume, but also increases in left ventricular volume and in right and left ventricular wall mass.

Adolescent↗
PubMed2001

Normal development of the pulmonary veins in human embryos and formulation of a morphogenetic concept for sinus venosus defects.

A sinus venosus defect is a form of interatrial communication associated with abnormal drainage of the right pulmonary veins. Its morphogenesis still remains unclear. We therefore studied the normal development of pulmonary veins in human embryos in relation to the sinus venosus and the dorsal mesocardium using graphic reconstructions and HNK-1 immunohistochemistry. Twenty embryos, ranging from 4 to 7 weeks' gestation, were examined. At 4 weeks, the orifice of the nonlumenized common pulmonary vein is visible as an endothelial invagination within the sinus venosus segment. Development of the muscular septum primum and the ventral proliferation of extracardiac mesenchyme from the dorsal mesocardium positions the common pulmonary vein (CPV) eventually into the left atrium. The right wall of the CPV contributes to the posterior part of the atrial septum and is continuous with the dorsal sinuatrial fold (the future left venous valve). With the use of HNK-1 antigen expression as a marker for sinus venosus myocardium, this common wall between the right-sided sinus venosus and the CPV is demonstrated, and at 7 weeks the proximal part of the right upper pulmonary vein also becomes part of this common wall. This study demonstrates that the CPV develops within the sinus venosus segment and that later a common myocardial wall is present between the sinus venosus in the right atrium and the CPV. A deficiency of this wall explains the development of sinus venosus defects.

CD57 Antigens↗
PubMed2001

Anthracycline-induced clinical heart failure in a cohort of 607 children: long-term follow-up study.

PURPOSE: To determine the early and late cumulative incidence of anthracycline-induced clinical heart failure (A-CHF) after anthracycline therapy in childhood and to identify associated risk factors. PATIENTS AND METHODS: The cumulative incidence of A-CHF and the risk factors of A-CHF were assessed in a cohort of 607 children who had been treated with anthracyclines between 1976 and 1996. For 96% of the cohort, we obtained the clinical status up to at least January 1997. The mean follow-up time was 6.3 years. RESULTS: The cumulative incidence of A-CHF was 2.8%, after a mean follow-up time of 6.3 years and a mean cumulative dose of anthracyclines of 301 mg/m(2). A cumulative dose of anthracycline higher than 300 mg/m(2) was associated with an increased risk of A-CHF (relative risk, 11.8; 95% confidence interval, 1.6 to 59.5) compared with a cumulative dose lower than 300 mg/m(2). The estimated risk of A-CHF increased with time after the start of anthracycline chemotherapy to 2% after 2 years and 5% after 15 years. CONCLUSION: Up to 5% of patients will develop A-CHF 15 years after treatment, and patients treated with a cumulative dose of anthracyclines higher than 300 mg/m(2) are at highest risk for A-CHF. This is thus a considerable and serious problem among these young patients. The findings reinforce the need for strategies for early detection of patients at risk for A-CHF and for the evaluation of other chemotherapeutic possibilities or cardioprotective agents in relation to the survival.

Adolescent↗
PubMed2001

Pulmonary valve insertion late after repair of Fallot's tetralogy.

OBJECTIVES: To analyze the results of pulmonary valve insertion late after initial repair of Fallot's tetralogy. Pulmonary insufficiency (PI) after correction of Fallot's tetralogy is usually well tolerated in the short term, but is associated with symptomatic right ventricular dilatation and an increased risk of ventricular arrhythmias over longer periods of time. METHODS: From 1993 to July 2000, 51 patients were reoperated for PI at a mean age of 25.7+/-11.9 years. The mean age at initial repair was 6.4+/-7.2 years. Patients with a conduit inserted at initial operation, with absent pulmonary valve syndrome or with a more than moderate ventricular septal defect at reoperation were excluded from the study. A cryopreserved pulmonary (96%) or aortic (4%) homograft was implanted in the orthotopic position with the use of cardiopulmonary bypass 19.3+/-9.1 years (2.7-40.3 years) after initial correction. Preoperative symptoms (New York Heart Association, NYHA class), degree of PI (echo-Doppler, MRI), right ventricular dimensions (MRI) and QRS duration were compared to findings at last follow-up. RESULTS: Follow-up is complete and had a mean duration of 1.7+/-1.4 years. Hospital mortality was 2%. No serious morbidity occurred. Severe PI was present preoperatively in all patients. At last follow-up echo-Doppler studies showed PI to be absent or trivial in 96% and mild in 4% of patients. In 13 patients MRI studies were performed both pre- and postoperatively: in this group PI was reduced from a mean of 48 to 4%. After 6 months NYHA capacity class had improved significantly from 2.3+/-0.6 to 1.4+/-0.5. After 1 year end-diastolic and end-systolic right ventricular volumes were reduced significantly. Right ventricular ejection fraction and QRS duration remained unchanged. CONCLUSIONS: PI late after correction of Fallot's tetralogy may lead to serious symptomatic right ventricle dilatation. After pulmonary homograft insertion right ventricular dimensions decrease rapidly and functional improvement is observed in almost all patients.

Adult↗
PubMed2001

The use of transesophageal echocardiography monitoring of transcatheter closure of a persistent ductus arteriosus.

We performed transesophageal echocardiography (TEE) with pediatric biplane probes throughout the procedure of transcatheter closure of the ductus arteriosus in 42 out of 46 patients who were scheduled for this intervention. Patient ages ranged from 15 months to 14 years and body weight from 9-53 kg. Both Rashkind double umbrella devices and coils were used. The described approach resulted in fewer contrast injections and was helpful in monitoring the procedure and in deciding when to release the device. To the best of our knowledge this is the first report describing a large series where continuous monitoring of transcatheter ductus closure was combined with x-ray fluoroscopy and TEE.

Adolescent↗
PubMed2000

Spin echo MRI in the evaluation of hearts with a double outlet right ventricle: usefulness and limitations.

The surgical approach to a double outlet right ventricle (DORV) is dependent on the spatial relationship of the semilunar valves, outflow tracts and ventricular septal defect (VSD). The purpose of the study was to assess the value of MRI for the evaluation of cardiovascular anatomy in patients before and after surgery for DORV. Spin echo MRI was performed in 12 patients with DORV (eight preoperative and four postoperative patients). Thin-section MRI was performed in three orthogonal planes and selected angulated views were obtained. Conventional imaging by color Doppler echocardiography and cine-angiocardiography and surgical findings, when present, served as the reference standards. The results found that the spatial relationship between semilunar valves and VSD was accurately assessed by MRI in eight out of eight preoperative patients. In the four postoperative cases MRI depicted the morphology of both outflow tracts and provided adequate information on their patency. Of the eight preoperative patients, five have undergone corrective surgery and the MRI findings were confirmed. MRI provided additional information to conventional imaging preoperatively in three cases in which the VSD opened into the outlet portion of the DORV, without there being a direct relation to a semilunar valve. In two preoperative cases in which the VSD was directly committed to the aorta, conventional imaging was conclusive. MRI was unable to depict aberrant chordae tendineae in four out of four cases. We conclude that MRI provides accurate additional anatomic information in patients with DORV, which is helpful in presurgical planning as well as during follow-up. Spin echo MRI does not visualize aberrant chordae tendineae.

Adolescent↗
PubMed1999

Development of the cardiac conduction tissue in human embryos using HNK-1 antigen expression: possible relevance for understanding of abnormal atrial automaticity.

BACKGROUND: Abnormal atrial automaticity in young patients with structurally normal hearts is often located around the pulmonary veins and in sinus venosus-related parts of the right atrium. We hypothesize that these ectopic pacemaker sites correspond to areas of embryonic myocardium with an early phenotypic differentiation, as indicated by differences in antigen expression during normal cardiac development. METHODS AND RESULTS: In human embryos ranging in age from 42 to 54 days of gestation, the development of the cardiac conduction system was studied with the use of HNK-1 immunohistochemistry. HNK-1 stains the developing atrioventricular conduction system, ie, the bundle branches, His bundle, right atrioventricular ring, and retroaortic ring. In addition, the myocardium around the common pulmonary vein showed transient HNK-1 antigen expression. In the right atrium, 3 HNK-1-positive connections were demonstrated between the sinoatrial node and the right atrioventricular ring. An anterior tract through the septum spurium connects the sinoatrial node with the anterior right atrioventricular ring, and 2 posterior tracts connect the sinoatrial node with the posterior right atrioventricular ring through the right venous valve (future crista terminalis) and sinus septum, encircling the coronary sinus. The medioposterior part of the right atrioventricular ring connected to the His bundle and the medioanterior part form 2 node-like structures. CONCLUSIONS: In patients with abnormal atrial automaticity, the distribution of left and right atrial pacemaker foci correspond to areas of the embryonic myocardium that temporarily express the HNK-1 antigen.

Atrioventricular Node↗
PubMed1999

Postsurgical course of patients with congenitally corrected transposition of the great arteries.

Because congenitally corrected transposition is a rare congenital anomaly, little is known about the prognosis of patients with this syndrome. The present study evaluated the functional status and clinical course of 73 patients (42 male and 31 female) aged 10 days to 73 years. Follow-up was 1 week to 37 years (mean 12.7 years). Particular attention was paid to the systemic right ventricle, considering the current controversies about long-term right ventricular function. Survival of patients with this condition in general was significantly below normal. The total mortality rate was 11% (8 patients) after a mean follow-up of 12.7 years (range 10 days to 37 years). Mean age at death was 18.5 years (range 6 days to 63 years). The mean age of the survivors at latest follow-up was 21.4 years (range 4 months to 73 years). In most patients, right ventricular function deteriorated and tricuspid valve regurgitation increased, which began at a very young age. Patients without associated lesions developed complications at a higher age compared with the total group. Right ventricular and tricuspid valvular function deteriorated more frequently in patients following intracardiac operation (28% and 52%, respectively) compared with patients undergoing palliative intervention (16%) or no surgery at all (28%). We conclude that survival of patients with this condition is substantially reduced compared with the natural history of an age- and gender-matched general population. This could be explained by the associated cardiac defects, but also by the anatomic condition itself.

Adolescent↗