The Kruskal-Wallis one-way analysis of variance on ranks showed that the imaging techniques had d... more The Kruskal-Wallis one-way analysis of variance on ranks showed that the imaging techniques had different precision in estimating the degree of bowel stenosis (p<0.001). The Tukey test showed that CTC was more precise than other techniques in estimating the degree of bowel stenosis (p<0.05); MRI-e was more precise than TVS (p<0.05). Conclusions: CTC is more precise than TVS, RWC-TVS and MRI-e in estimating the degree of stenosis of the bowel lumen in patients with colorectal endometriosis.
Backgroundā Incidence and prevalence rates for pediatric pulmonary hypertension (PH) and pulmonar... more Backgroundā Incidence and prevalence rates for pediatric pulmonary hypertension (PH) and pulmonary arterial hypertension (PAH) are unknown. This study describes the nationwide epidemiological features of pediatric PH in the Netherlands during a 15-year period and the clinical course of pediatric PAH. Methods and Resultsā Two registries were used to retrospectively identify children (0ā17 years) with PH. Overall, 3263 pediatric patients were identified with PH due to left heart disease (n=160; 5%), lung disease/hypoxemia (n=253; 8%), thromboembolic disease (n=5; <1%), and transient (n=2691; 82%) and progressive (n=154; 5%) PAH. Transient PAH included persistent PH of the newborn and children with congenital heart defects (CHD) and systemic-to-pulmonary shunt, in whom PAH resolved after successful shunt correction. Progressive PAH mainly included idiopathic PAH (n=36; iPAH) and PAH associated with CHD (n=111; PAH-CHD). Pulmonary arterial hypertension associated with CHD represented...
Incidence and prevalence rates for pediatric pulmonary hypertension (PH) and pulmonary arterial h... more Incidence and prevalence rates for pediatric pulmonary hypertension (PH) and pulmonary arterial hypertension (PAH) are unknown. This study describes the nationwide epidemiological features of pediatric PH in the Netherlands during a 15-year period and the clinical course of pediatric PAH. Two registries were used to retrospectively identify children (0-17 years) with PH. Overall, 3263 pediatric patients were identified with PH due to left heart disease (n=160; 5%), lung disease/hypoxemia (n=253; 8%), thromboembolic disease (n=5; &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt;1%), and transient (n=2691; 82%) and progressive (n=154; 5%) PAH. Transient PAH included persistent PH of the newborn and children with congenital heart defects (CHD) and systemic-to-pulmonary shunt, in whom PAH resolved after successful shunt correction. Progressive PAH mainly included idiopathic PAH (n=36; iPAH) and PAH associated with CHD (n=111; PAH-CHD). Pulmonary arterial hypertension associated with CHD represented highly heterogeneous subgroups. Syndromes were frequently present, especially in progressive PAH (n=60; 39%). Survival for PAH-CHD varied depending on the subgroups, some showing better and others showing worse survival than for iPAH. Survival of children with Eisenmenger syndrome appeared worse than reported in adults. For iPAH and PAH-CHD, annual incidence and point prevalence averaged, respectively, 0.7 and 4.4 (iPAH) and 2.2 and 15.6 (PAH-CHD) cases per million children. Compared to studies in adults, iPAH occurred less whereas PAH-CHD occurred more frequently. Pediatric PH is characterized by various age-specific diagnoses, the majority of which comprise transient forms of PAH. Incidence of pediatric iPAH is lower whereas incidence of pediatric PAH-CHD is higher than reported in adults. Pediatric PAH-CHD represents a heterogeneous group with highly variable clinical courses.
SamenvattingĀ Ā Doel. Onderzoeken hoe vaak myocarditis als doodsoorzaak wordt gevonden in de groep ... more SamenvattingĀ Ā Doel. Onderzoeken hoe vaak myocarditis als doodsoorzaak wordt gevonden in de groep van plotseling, onverwacht overleden kinderen (&amp;amp;amp;amp;amp;lt;2 jaar) die werden gemeld aan de Landelijke Werkgroep Wiegendood (1.9.1996-31.8.2006). Onderzoeken wat de betekenis is van deze bevinding, hoe vaak deze voorkomt en welk onderzoek dient te geschieden voor diagnose. Opzet. Retrospectief, beschrijvende studie. Methode. De gegevens van de plotseling overleden kinderen
Objectives: To investigate the cardiac defects (CHD) found in association with an increased nucha... more Objectives: To investigate the cardiac defects (CHD) found in association with an increased nuchal translucency (NT). Methods: Patients referred from January 1998 until February 2007 with an NT ā„ 95 th percentile at 11-14 weeks and where a cardiac defect was found were included. Chromosome analysis, fetal echocardiography and postnatal echocardiography were performed. After pregnancy termination a postmortem examination was performed when possible. Results: CHD were identified in 63 fetuses (major CHD in 50; 79%). The median NT was 4.8 (range, 2.6ā13) mm. Group 1 included 36 fetuses (57%) with a normal karyotype. Median NT was 5.2 (range, 2.8ā9) mm. Twenty-four (66.7%) had major CHD. CHD included VSD (10), HLHS (four), TGV (three), AVSD (three), PA + VSD/TOF (two), PS (two), uncertain cardiac diagnosis with hydrops (two), aortic arch abnormalities (two), PDA (two), DORV (one), ASD (one), tricuspid atresia (one), aortic incompetence (one), mitral valve abnormality (one) and persistent right umbilical vein (one). Group 2 included seven fetuses (11%) with a genetic disorder including: 18p deletion (two), 22q11 deletion (two), Coffin-Siris (one) and Holt-Oram (one). CHD found included HLHS, TGV, truncus arteriosus, VSD (two), ASD and DORV. Group 3 included 20 fetuses (32%) with abnormal numeric karyotype including 10 trisomy 21, six trisomy 18, two XO, one trisomy 13 and one trisomy 9. Median NT was 4.7 (range, 2.6ā13) mm. Nineteen had major CHD. CHD found with trisomy 21 included AVSD (six), inlet VSD (two) and RV non-compaction (one). In fetuses with trisomy 18, VSD (two), polyvalvular disease (two), HLHS (one) and aortic arch abnormality (one) were found. Both fetuses with XO had HLHS. PA + VSD was found with trisomy 13 and truncus arteriosus in trisomy 9. Conclusions: Various CHD are associated with an increased NT. Conotruncal defects, branchial arch derivative defects, left and right obstructive lesions and shunts were seen. Anomalous pulmonary venous drainage and cor triatriatum were not seen; thus abnormal targeted growth is not the mechanism linking CHD and an increased NT.
view that includes the distal aortic and ductal arches and proximal descending aorta using power ... more view that includes the distal aortic and ductal arches and proximal descending aorta using power Doppler 2D ultrasound. The AI-D angle was measured by one operator in 104 fetuses with a normal heart at 12 to 35 weeks of gestation (median: 23 weeks) and in 8 fetuses with confirmed CoAo postnatally (range = 18 to 31 weeks). Results: In normal fetuses, the AI-D angle ranged from 128.2 to 167 degrees (mean = 20.4, 95% CI = 149-151.8) and from 82.2 to 125 degrees (mean = 20.6, 95% CI = 89.4-117.9) in those with CoAo. The AI-D angle was significantly smaller in the CoAo group (difference between means = 46.7, 95% CI = 40.7-52.7). Conclusions: A new measurement to assess the aortic isthmal area in normals is described -the AI-D angle -and shown to be statistically different from the angle observed in cases of coarctation of the aorta. This may be helpful in the diagnosis of localised CoAo in the fetus and may improve accuracy of prenatal diagnosis.
Ultrasound in obstetrics & gynecology : the official journal of the International Society of Ultrasound in Obstetrics and Gynecology, 2015
To evaluate the prenatal detection of transposition of the great arteries (TGA), after the introd... more To evaluate the prenatal detection of transposition of the great arteries (TGA), after the introduction of a Dutch screening program in 2007, as well as the effect of prenatal detection on pre- and postsurgical mortality and morbidity. In a geographical cohort study, all infants with TGA who were born between 1 January 2002 and 1 January 2012 were included. The cases were divided into two groups: those with and those without a prenatal diagnosis. Pre- and postsurgical mortality was assessed, with a follow-up of 1 year. Presurgical morbidity was assessed in terms of cardiovascular compromise, metabolic acidosis, renal and/or hepatic dysfunction and closure of the duct before initiation of therapy. Of all cases (nā=ā144), 26.4% were diagnosed prenatally, with detection rates of 15.7% and 41.0% in the first and last 5 years of the study period, respectively. First-year mortality was significantly lower in cases with a prenatal diagnosis of TGA than in those without (0.0% vs 11.4%, resp...
Netherlands heart journal : monthly journal of the Netherlands Society of Cardiology and the Netherlands Heart Foundation, 2012
Communication between the aortic sinus and a cardiac chamber is a rare anomaly that can be diagno... more Communication between the aortic sinus and a cardiac chamber is a rare anomaly that can be diagnosed in children and young adults. We describe two cases with a tunnel between the aortic sinus and right atrium, discuss diagnostic modalities, and review the literature on this anomaly.
The prenatal diagnosis of cardiac defects can potentially reduce postnatal morbidity and mortalit... more The prenatal diagnosis of cardiac defects can potentially reduce postnatal morbidity and mortality. We wanted to evaluate prenatal cardiac diagnosis accuracy in a population referred for echocardiography. Single centre retrospective study of echocardiography referrals between April 1999 and December 2008. We compared the prenatal and postnatal cardiac diagnoses, the modified Aristotle and Wald scores. The final diagnosis Wald score was used to evaluate benefit. Six hundred fetuses were included. Diagnoses included: normal heart (312, 52%); congenital heart defect (CHD) (231, 38.5%); primary arrhythmia (39, 6.5%); or cardiomyopathy, myocarditis or cardiac tumor (18, 3%). The prenatal and postnatal Aristotle and Wald scores correlated in 81% and 86%, respectively, each with significant differences in 22 cases. Four significant CHDs were misdiagnosed, the surgical prediction was incorrect in 7 and 13 false positive diagnoses of aortic coarctation were made. In 76% (455/600) fetuses pre...
Nederlands tijdschrift voor geneeskunde, Jan 17, 2007
An 11-year-old girl, a 15-year-old boy and a 12-year-old girl all underwent percutaneous implanta... more An 11-year-old girl, a 15-year-old boy and a 12-year-old girl all underwent percutaneous implantation of a Melody pulmonary valve prosthesis to replace a stenotic and insufficient homograft in the pulmonary artery. Preoperatively, 2 of the children suffered from fatigue and dyspnoea on exertion The homografts had been implanted between the ages of 1-2, to establish surgical continuity between the right ventricle and the pulmonary artery. The anomalies were tetralogy of Fallot, pulmonary atresia with intact ventricular septum and pulmonary atresia with a ventricular septum defect. Percutaneous pulmonary valve replacement was successful in all 3 patients. After implantation, right ventricular pressure decreased to 30% of systemic pressure and regurgitation was not observed. All patients were discharged in a good condition on the day after the implantation. Percutaneous pulmonary valve replacement is a promising technique with good short-term results. In selected patients this percutan...
The Kruskal-Wallis one-way analysis of variance on ranks showed that the imaging techniques had d... more The Kruskal-Wallis one-way analysis of variance on ranks showed that the imaging techniques had different precision in estimating the degree of bowel stenosis (p<0.001). The Tukey test showed that CTC was more precise than other techniques in estimating the degree of bowel stenosis (p<0.05); MRI-e was more precise than TVS (p<0.05). Conclusions: CTC is more precise than TVS, RWC-TVS and MRI-e in estimating the degree of stenosis of the bowel lumen in patients with colorectal endometriosis.
Backgroundā Incidence and prevalence rates for pediatric pulmonary hypertension (PH) and pulmonar... more Backgroundā Incidence and prevalence rates for pediatric pulmonary hypertension (PH) and pulmonary arterial hypertension (PAH) are unknown. This study describes the nationwide epidemiological features of pediatric PH in the Netherlands during a 15-year period and the clinical course of pediatric PAH. Methods and Resultsā Two registries were used to retrospectively identify children (0ā17 years) with PH. Overall, 3263 pediatric patients were identified with PH due to left heart disease (n=160; 5%), lung disease/hypoxemia (n=253; 8%), thromboembolic disease (n=5; <1%), and transient (n=2691; 82%) and progressive (n=154; 5%) PAH. Transient PAH included persistent PH of the newborn and children with congenital heart defects (CHD) and systemic-to-pulmonary shunt, in whom PAH resolved after successful shunt correction. Progressive PAH mainly included idiopathic PAH (n=36; iPAH) and PAH associated with CHD (n=111; PAH-CHD). Pulmonary arterial hypertension associated with CHD represented...
Incidence and prevalence rates for pediatric pulmonary hypertension (PH) and pulmonary arterial h... more Incidence and prevalence rates for pediatric pulmonary hypertension (PH) and pulmonary arterial hypertension (PAH) are unknown. This study describes the nationwide epidemiological features of pediatric PH in the Netherlands during a 15-year period and the clinical course of pediatric PAH. Two registries were used to retrospectively identify children (0-17 years) with PH. Overall, 3263 pediatric patients were identified with PH due to left heart disease (n=160; 5%), lung disease/hypoxemia (n=253; 8%), thromboembolic disease (n=5; &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt;1%), and transient (n=2691; 82%) and progressive (n=154; 5%) PAH. Transient PAH included persistent PH of the newborn and children with congenital heart defects (CHD) and systemic-to-pulmonary shunt, in whom PAH resolved after successful shunt correction. Progressive PAH mainly included idiopathic PAH (n=36; iPAH) and PAH associated with CHD (n=111; PAH-CHD). Pulmonary arterial hypertension associated with CHD represented highly heterogeneous subgroups. Syndromes were frequently present, especially in progressive PAH (n=60; 39%). Survival for PAH-CHD varied depending on the subgroups, some showing better and others showing worse survival than for iPAH. Survival of children with Eisenmenger syndrome appeared worse than reported in adults. For iPAH and PAH-CHD, annual incidence and point prevalence averaged, respectively, 0.7 and 4.4 (iPAH) and 2.2 and 15.6 (PAH-CHD) cases per million children. Compared to studies in adults, iPAH occurred less whereas PAH-CHD occurred more frequently. Pediatric PH is characterized by various age-specific diagnoses, the majority of which comprise transient forms of PAH. Incidence of pediatric iPAH is lower whereas incidence of pediatric PAH-CHD is higher than reported in adults. Pediatric PAH-CHD represents a heterogeneous group with highly variable clinical courses.
SamenvattingĀ Ā Doel. Onderzoeken hoe vaak myocarditis als doodsoorzaak wordt gevonden in de groep ... more SamenvattingĀ Ā Doel. Onderzoeken hoe vaak myocarditis als doodsoorzaak wordt gevonden in de groep van plotseling, onverwacht overleden kinderen (&amp;amp;amp;amp;amp;lt;2 jaar) die werden gemeld aan de Landelijke Werkgroep Wiegendood (1.9.1996-31.8.2006). Onderzoeken wat de betekenis is van deze bevinding, hoe vaak deze voorkomt en welk onderzoek dient te geschieden voor diagnose. Opzet. Retrospectief, beschrijvende studie. Methode. De gegevens van de plotseling overleden kinderen
Objectives: To investigate the cardiac defects (CHD) found in association with an increased nucha... more Objectives: To investigate the cardiac defects (CHD) found in association with an increased nuchal translucency (NT). Methods: Patients referred from January 1998 until February 2007 with an NT ā„ 95 th percentile at 11-14 weeks and where a cardiac defect was found were included. Chromosome analysis, fetal echocardiography and postnatal echocardiography were performed. After pregnancy termination a postmortem examination was performed when possible. Results: CHD were identified in 63 fetuses (major CHD in 50; 79%). The median NT was 4.8 (range, 2.6ā13) mm. Group 1 included 36 fetuses (57%) with a normal karyotype. Median NT was 5.2 (range, 2.8ā9) mm. Twenty-four (66.7%) had major CHD. CHD included VSD (10), HLHS (four), TGV (three), AVSD (three), PA + VSD/TOF (two), PS (two), uncertain cardiac diagnosis with hydrops (two), aortic arch abnormalities (two), PDA (two), DORV (one), ASD (one), tricuspid atresia (one), aortic incompetence (one), mitral valve abnormality (one) and persistent right umbilical vein (one). Group 2 included seven fetuses (11%) with a genetic disorder including: 18p deletion (two), 22q11 deletion (two), Coffin-Siris (one) and Holt-Oram (one). CHD found included HLHS, TGV, truncus arteriosus, VSD (two), ASD and DORV. Group 3 included 20 fetuses (32%) with abnormal numeric karyotype including 10 trisomy 21, six trisomy 18, two XO, one trisomy 13 and one trisomy 9. Median NT was 4.7 (range, 2.6ā13) mm. Nineteen had major CHD. CHD found with trisomy 21 included AVSD (six), inlet VSD (two) and RV non-compaction (one). In fetuses with trisomy 18, VSD (two), polyvalvular disease (two), HLHS (one) and aortic arch abnormality (one) were found. Both fetuses with XO had HLHS. PA + VSD was found with trisomy 13 and truncus arteriosus in trisomy 9. Conclusions: Various CHD are associated with an increased NT. Conotruncal defects, branchial arch derivative defects, left and right obstructive lesions and shunts were seen. Anomalous pulmonary venous drainage and cor triatriatum were not seen; thus abnormal targeted growth is not the mechanism linking CHD and an increased NT.
view that includes the distal aortic and ductal arches and proximal descending aorta using power ... more view that includes the distal aortic and ductal arches and proximal descending aorta using power Doppler 2D ultrasound. The AI-D angle was measured by one operator in 104 fetuses with a normal heart at 12 to 35 weeks of gestation (median: 23 weeks) and in 8 fetuses with confirmed CoAo postnatally (range = 18 to 31 weeks). Results: In normal fetuses, the AI-D angle ranged from 128.2 to 167 degrees (mean = 20.4, 95% CI = 149-151.8) and from 82.2 to 125 degrees (mean = 20.6, 95% CI = 89.4-117.9) in those with CoAo. The AI-D angle was significantly smaller in the CoAo group (difference between means = 46.7, 95% CI = 40.7-52.7). Conclusions: A new measurement to assess the aortic isthmal area in normals is described -the AI-D angle -and shown to be statistically different from the angle observed in cases of coarctation of the aorta. This may be helpful in the diagnosis of localised CoAo in the fetus and may improve accuracy of prenatal diagnosis.
Ultrasound in obstetrics & gynecology : the official journal of the International Society of Ultrasound in Obstetrics and Gynecology, 2015
To evaluate the prenatal detection of transposition of the great arteries (TGA), after the introd... more To evaluate the prenatal detection of transposition of the great arteries (TGA), after the introduction of a Dutch screening program in 2007, as well as the effect of prenatal detection on pre- and postsurgical mortality and morbidity. In a geographical cohort study, all infants with TGA who were born between 1 January 2002 and 1 January 2012 were included. The cases were divided into two groups: those with and those without a prenatal diagnosis. Pre- and postsurgical mortality was assessed, with a follow-up of 1 year. Presurgical morbidity was assessed in terms of cardiovascular compromise, metabolic acidosis, renal and/or hepatic dysfunction and closure of the duct before initiation of therapy. Of all cases (nā=ā144), 26.4% were diagnosed prenatally, with detection rates of 15.7% and 41.0% in the first and last 5 years of the study period, respectively. First-year mortality was significantly lower in cases with a prenatal diagnosis of TGA than in those without (0.0% vs 11.4%, resp...
Netherlands heart journal : monthly journal of the Netherlands Society of Cardiology and the Netherlands Heart Foundation, 2012
Communication between the aortic sinus and a cardiac chamber is a rare anomaly that can be diagno... more Communication between the aortic sinus and a cardiac chamber is a rare anomaly that can be diagnosed in children and young adults. We describe two cases with a tunnel between the aortic sinus and right atrium, discuss diagnostic modalities, and review the literature on this anomaly.
The prenatal diagnosis of cardiac defects can potentially reduce postnatal morbidity and mortalit... more The prenatal diagnosis of cardiac defects can potentially reduce postnatal morbidity and mortality. We wanted to evaluate prenatal cardiac diagnosis accuracy in a population referred for echocardiography. Single centre retrospective study of echocardiography referrals between April 1999 and December 2008. We compared the prenatal and postnatal cardiac diagnoses, the modified Aristotle and Wald scores. The final diagnosis Wald score was used to evaluate benefit. Six hundred fetuses were included. Diagnoses included: normal heart (312, 52%); congenital heart defect (CHD) (231, 38.5%); primary arrhythmia (39, 6.5%); or cardiomyopathy, myocarditis or cardiac tumor (18, 3%). The prenatal and postnatal Aristotle and Wald scores correlated in 81% and 86%, respectively, each with significant differences in 22 cases. Four significant CHDs were misdiagnosed, the surgical prediction was incorrect in 7 and 13 false positive diagnoses of aortic coarctation were made. In 76% (455/600) fetuses pre...
Nederlands tijdschrift voor geneeskunde, Jan 17, 2007
An 11-year-old girl, a 15-year-old boy and a 12-year-old girl all underwent percutaneous implanta... more An 11-year-old girl, a 15-year-old boy and a 12-year-old girl all underwent percutaneous implantation of a Melody pulmonary valve prosthesis to replace a stenotic and insufficient homograft in the pulmonary artery. Preoperatively, 2 of the children suffered from fatigue and dyspnoea on exertion The homografts had been implanted between the ages of 1-2, to establish surgical continuity between the right ventricle and the pulmonary artery. The anomalies were tetralogy of Fallot, pulmonary atresia with intact ventricular septum and pulmonary atresia with a ventricular septum defect. Percutaneous pulmonary valve replacement was successful in all 3 patients. After implantation, right ventricular pressure decreased to 30% of systemic pressure and regurgitation was not observed. All patients were discharged in a good condition on the day after the implantation. Percutaneous pulmonary valve replacement is a promising technique with good short-term results. In selected patients this percutan...
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