Christian Lilje - Academia.edu (original) (raw)

Papers by Christian Lilje

Research paper thumbnail of Global longitudinal strain analysis of the singe right ventricle: leveling the playing field

Journal of the American Society of Echocardiography, 2022

Research paper thumbnail of Abnormal Origin of one Pulmonary Artery from the Ascending Aorta–Embryologic Considerations

Fetal and Pediatric Pathology, 2016

Anomalous origin of one of the pulmonary arteries from the aorta is unusual. The reported morbidi... more Anomalous origin of one of the pulmonary arteries from the aorta is unusual. The reported morbidity and mortality is mostly due to early onset vascular disease. Early surgical intervention has significantly improved outcomes. The diagnosis of this abnormality is challenging. The nomenclature used is inconsistent. Familiarity with this abnormality and consistent use of definitions and classifications is mandatory. An attempt is made to clarify misleading inconsistencies. An older ontogenetic theory is revisited.

Research paper thumbnail of Utility of N-Terminal Brain Natriuretic Peptide Plasma Concentrations in Comparison to Lactate and Troponin in Children with Congenital Heart Disease Following Open-Heart Surgery

Pediatric Cardiology, 2005

We conducted a prospective study in a pediatric cardiac intensive care unit in order to determine... more We conducted a prospective study in a pediatric cardiac intensive care unit in order to determine the diagnostic value of N-terminal brain natriuretic peptide (N-BNP) plasma concentration in the perioperative care of children with congenital heart disease (CHD). N-BNP plasma concentrations were determined by using a validated enzyme immunoassay. We measured N-BNP the day before surgery and up to 15 days postoperatively in 23 children (age range, 0.25-11 years) undergoing cardiac surgery due to various CHDs. Supply and duration of catecholamines, vasodilators, and respiratory therapy were determined and correlated to N-BNP. In addition, troponin T (TnT) and arterial Lactat (aL) concentrations were measured simultaneously. We found a significant correlation between preoperative and maximal N-BNP levels and dosage of vasodilators (r = 0.41, p < 0.02 and r = 0.83, p < 0.01, respectively). Maximal TnT and aL levels were not correlated to dosage of vasodilators. The dosage and duration of catecholamines, the duration of respiratory therapy, and the plasma concentration of TnT and aL were not correlated to pre-or perioperative N-BNP. Maximal TnT and aL levels were correlated to duration (r = 0.53, p < 0.01 and r = 0.48, p < 0.02) and dosage (r = 0.52, p < 0.02 and r = 0.60, p < 0.01) of catecholamines and duration of respiratory therapy (r = 0.57, p < 0.01 and r = 0.50, p < 0.02). As recent studies show, N-BNP appears to be a powerful neurohumoral indicator of ventricular function and prognosis for guiding therapy in the outpatient department or for discriminating cardiac from noncardiac symptoms. In contrast, the value of N-BNP for guiding perioperative therapy in pediatric cardiac intensive care units is limited.

Research paper thumbnail of Detection of Partial Anomalous Pulmonary Venous Connection by Magnetic Resonance Imaging

Pediatric Cardiology, 2005

Research paper thumbnail of Real-Time 3-Dimensional Echocardiography for Follow-up of Waterston Shunt

Journal of the American Society of Echocardiography, 2008

Research paper thumbnail of Magnetic resonance imaging follow up of total cavopulmonary connection

Research paper thumbnail of Ethics Consultation: The Least Dangerous Profession?

Cambridge Quarterly of Healthcare Ethics, 1993

Whether ethics is too important to be left to the experts or so important that it must be is an a... more Whether ethics is too important to be left to the experts or so important that it must be is an age-old question. The emergence of clinical ethicists raises it again, as a question about professionalism. What role clinical ethicists should play in healthcare decision making – teacher, mediator, or consultant – is a question that has generated considerable debate but no consensus.

Research paper thumbnail of Isolated Congenital Right Atrial Aneurysm

World journal for pediatric & congenital heart surgery, 2016

Isolated congenital right atrial aneurysm is rare. Indications for surgery in asymptomatic patien... more Isolated congenital right atrial aneurysm is rare. Indications for surgery in asymptomatic patients with moderate-size right atria remain controversial. Evidence in support of medical management and timing of prophylactic surgery is reviewed. We propose the use of three echocardiographic indices to help identify inappropriate atrial growth and facilitate surgical decision-making.

Research paper thumbnail of A modified noninvasive screening protocol for pulmonary hypertension in children with sickle cell disease-Who should be sent for invasive evaluation?

Pediatric blood & cancer, Jan 5, 2017

Invasive studies have shown that prevalence and severity of pulmonary hypertension (PH) in patien... more Invasive studies have shown that prevalence and severity of pulmonary hypertension (PH) in patients with sickle cell disease (SCD) tend to be overestimated if based exclusively on Doppler-derived tricuspid regurgitant velocity (TRV) as surrogate noninvasive marker with a cutoff ≥2.5 m/s. We aimed to better define a subgroup of pediatric SCD patients who should be sent for invasive evaluation of pulmonary artery pressure (PAP) based on a modified echocardiographic PH screening protocol that implements evidence from Doppler-catheter comparative studies. Charts of 121 pediatric patients with stable SCD were reviewed regarding echocardiographically assessed risk for elevated PAP/PH and associated clinical characteristics. TRV cutoff was refined at ≥2.9 m/s to avoid overestimating the risk for PH. TRV was combined with additional echocardiographic parameters to avoid underestimating the PH risk. Ninety-one patients qualified for analysis. Based on our modified echocardiographic protocol,...

Research paper thumbnail of Further evidence that variants in PPP1CB cause a rasopathy similar to Noonan syndrome with loose anagen hair

American journal of medical genetics. Part A, Feb 1, 2017

Further Evidence That Variants in PPP1CB Cause a Rasopathy Similar to Noonan Syndrome with Loose ... more Further Evidence That Variants in PPP1CB Cause a Rasopathy Similar to Noonan Syndrome with Loose Anagen Hair Regina M. Zambrano,* Michael Marble, Stuart A. Chalew, Christian Lilje, Alfonso Vargas, and Yves Lacassie* Division of Genetics, Department of Pediatrics, Louisiana State University Health Sciences Center School of Medicine, and Children’s Hospital, New Orleans, Louisiana Division of Endocrinology, Department of Pediatrics, Louisiana State University Health Sciences Center School of Medicine, and Children’s Hospital, New Orleans, Louisiana Division of Cardiology, Department of Pediatrics, Louisiana State University Health Sciences Center School of Medicine, and Children’s Hospital, New Orleans, Louisiana

Research paper thumbnail of Pediatric Pulmonary Embolism: Diagnostic and Management Challenges

World journal for pediatric & congenital heart surgery, Jan 12, 2016

A rare case of massive pulmonary embolism is presented in an oligosymptomatic teenager with predi... more A rare case of massive pulmonary embolism is presented in an oligosymptomatic teenager with predisposing factors. Computed tomography pulmonary angiography supported by three-dimensional reconstruction was diagnostic. The embolus qualified as massive by conventional anatomical guidelines, but as low risk by more recent functional criteria. Functional assessment has complemented morphologic assessment for risk stratification in adult patients. Such evidence is scarce in pediatrics. The patient underwent surgical embolectomy, followed by prophylactic anticoagulation, without further events. Diagnostic and management challenges are discussed.

Research paper thumbnail of Tetralogy of fallot and anomalous origin of the left pulmonary artery from the ascending aorta associated with absent right internal carotid artery

World journal for pediatric & congenital heart surgery, 2015

Anomalous origin of the left pulmonary artery from the ascending aorta is a rare cardiac malforma... more Anomalous origin of the left pulmonary artery from the ascending aorta is a rare cardiac malformation more commonly described with tetralogy of Fallot (TOF). The association of absent right carotid artery with TOF and anomalous left pulmonary artery from the ascending aorta has been rarely reported in the past. This case highlights the need for careful evaluation of the aortic arch and head vessels anatomy in patients with TOF because of the potential neurologic complications after surgery.

Research paper thumbnail of How I felt about it: Thoughts on a consultation

Monatsschrift Kinderheilkunde, 2007

Zusammenfassung??Wir berichten von der Auseinandersetzung eines jugendlichen Patienten mit einem ... more Zusammenfassung??Wir berichten von der Auseinandersetzung eines jugendlichen Patienten mit einem bevorstehenden chirurgischen Eingriff. Aufgrund bilateraler Hydronephrose bei Urethralklappen war eine Nierentransplantation erfolgt. Rezidivierende Harnwegsinfektionen, zwei Transplantatabsto?ungen und ein vesico-ureteraler Reflux bei Low-Compliance-Harnblase hatten zu einer kompensierten Insuffizienz der Transplantatniere gef?hrt. Es war eine Blasen-Augmentationsplastik vorgesehen.Bereits im Vorfeld hatte der Junge offenbar Angst, nicht zu verstehen bzw. nicht verstanden zu werden.

Research paper thumbnail of Ross-Konno Procedure in Children – Mid-term Results

The Thoracic and Cardiovascular Surgeon, 2010

The aim of this study was to analyze the midterm results following the Ross-Konno procedure in ch... more The aim of this study was to analyze the midterm results following the Ross-Konno procedure in children. Between 1999 and 2008, 29 patients with complex left ventricular outflow tract obstruction underwent the Ross-Konno procedure. There were 12 (41%) infants (group A) and 17 (59%) older patients (group B). The median age at operation was 3.3 years (range, 6 days to 16 years). At 7 years of follow-up, survival was 96% (1 late death), with no differences between groups A and B. Freedom from aortic regurgitation ≥ mild was 81%, with no differences between groups A and B. No residual gradient was noted in the left ventricular outflow tract in either group. Freedom from mitral regurgitation ≥ mild was 100% in group B and 41% in group A (P = .0029). The mitral regurgitation was associated with morphological abnormalities of the mitral valve and with development of endocardial fibroelastosis after failed intervention during the newborn period. Freedom from reoperation was 73% in group B and 24% in group A (P = .0093). All patients are now in sinus rhythm, and 43% are without medication. With the technical aspects of this procedure well accomplished, mortality is low, and the functional outcome is encouraging. At midterm follow-up, there was no residual or recurrent outflow tract obstruction and an excellent function of the neoaortic valve. The higher incidence of mitral regurgitation in infants, which was associated with morphological abnormalities of mitral valve and development of endocardial fibroelastosis, is worrisome.

Research paper thumbnail of MRT in der postoperativen Diagnostik bei funktionell univentrikulärem Herz: Korrelation zu Echokardiographie und Kardangiographie

RöFo - Fortschritte auf dem Gebiet der Röntgenstrahlen und der bildgebenden Verfahren, 2002

To determine the value of MRI in the postoperative evaluation of a singular ventricle compared to... more To determine the value of MRI in the postoperative evaluation of a singular ventricle compared to echocardiography and cardiac catheterization. Thirty-one patients (range: 6 months to 30 years) with a functional single ventricle following palliative corrective operations. Five patients had a Blalock-Taussig-Shunt, 8 patients a Glenn-Anastomosis and 18 a cavopulmonary shunt (6 with classic Fontan-Circulation, 12 with modified cavopulmonary anastomosis). The results in terms of postoperative morphologic changes were compared to percutaneous echocardiography (31/31) and cardiac catheterization (6/31). Echocardiography, which was performed on all patients, could not visualize the entire length of the tunnel, the Glenn-Anastomosis or the central pulmonary arteries in 70 % of the patients due to an inadequate acoustic window. MRI was able to show the entire tunnel in 11/12 patients and the central pulmonary arteries in 30/31 patients. The exact anatomy was seen in all 6 patients undergoing cardiac catheterization. MRI is useful in the postoperative evaluation of a functionally single ventricle. It is superior to echocardiography. Cardiac catheterization should be reserved for patients with inconclusive MRI findings.

Research paper thumbnail of MRT der Pulmonalarterien bei Kindern nach arterieller Switch-Operation (ASO) bei Transposition der großen Gefäße (d-TGA)

RöFo - Fortschritte auf dem Gebiet der Röntgenstrahlen und der bildgebenden Verfahren, 2005

To determine the value of MRI in postoperative evaluation of patients with arterial-switch-operat... more To determine the value of MRI in postoperative evaluation of patients with arterial-switch-operation (ASO) for d-TGA. 14 consecutive patients with d-TGA and ASO were examined with MRI in addition to ultrasound because the acoustic window in echocardiography was insufficient. Nine patients had a pulmonary-artery-bifurcation anterior to the aortic root (group 1), and five (group 2) laterally (two of them left, three of them right). MRI was performed in six patients as contrast-enhanced MR-angiography, in 8 patients as native examination. Diameters of pulmonary arteries were measured in two perpendicular views at the origin and in two further locations each with a distance of one cm. One patient showed a supravalvular pulmonary artery stenosis with subsequent pulmonary artery aneurysm, which had not been detected in echocardiography. This patient showed only indirect signs of pulmonary artery stenosis in echocardiography. Compared to group 1, pulmonary arteries in group 2 generally had greater diameters. Those pulmonary arteries in Group 2 which cross the mediastinum underneath the aortic arch had lower diameters compared to the pulmonary artery positioned on the other side. In group 1, the left-sided pulmonary artery had lower diameters than the right-sided pulmonary arteries. It seems remarkable that the narrowing of pulmonary arteries is associated with a flattening of the transversal vessel diameter. Narrowing of pulmonary arteries after ASO is associated with oval vessel diameters. Therefore, multiplanar diagnostic methods are necessary. MRI meets this prerequisite and is therefore advantageous to echocardiography in follow-up examinations at patients with ASO for d-TGA. Special attention should be focused on the main pulmonary artery, on the left pulmonary artery in patients with preaortic pulmonary artery bifurcation as well as on the pulmonary artery which crosses the mediastinum in patients with laterally placed pulmonary artery bifurcation. Angiography should only be performed when intervention is planned.

Research paper thumbnail of Benign course of central pontine myelinolysis in a patient with anorexia nervosa

Pediatric Neurology, 2002

All recent descriptions of the structural changes in the kidney which follow a dietary depletion ... more All recent descriptions of the structural changes in the kidney which follow a dietary depletion of magnesium are in agreement that "calcification" involving

Research paper thumbnail of Double Discordance in the Hypoplastic Left Heart

Pediatric Cardiology, 2008

A neonate was transferred for an intracardiac mass. Initially, the mass was mistaken for a hypopl... more A neonate was transferred for an intracardiac mass. Initially, the mass was mistaken for a hypoplastic right ventricle, a thrombus, or a tumor. Only a thorough segmental and sequential analysis showed double discordance with a hypoplastic left ventricle. No such entity has yet been described.

Research paper thumbnail of Schwere Neuropathie nach Chemo- und Radiotherapie eines Ewing-Sarkoms

Klinische Pädiatrie, 1995

Research paper thumbnail of Noninvasive Follow-up of Complications After the Takeuchi Operation

Journal of the American Society of Echocardiography, 2007

Anomalous origin of the left coronary artery from the pulmonary artery is a rare lesion. Untreate... more Anomalous origin of the left coronary artery from the pulmonary artery is a rare lesion. Untreated, up to 90% of patients die before 1 year of age. The Takeuchi operation and direct coronary artery reimplantation have resulted in low mortality rates and excellent functional outcome. We present the case of a 6-yearold girl with impaired exercise tolerance in whom anomalous origin of the left coronary artery from the pulmonary artery was detected. A Takeuchi operation was performed. Routine follow-up was successfully done by echocardiography. The postoperative anatomy was visualized in detail, including a baffle-topulmonary artery fistula and a coronary artery stenosis. Potentially, given current echocardiographic technology in the hands of experienced operators, routine follow-up may be done noninvasively, leaving angiography for nonroutine indications.

Research paper thumbnail of Global longitudinal strain analysis of the singe right ventricle: leveling the playing field

Journal of the American Society of Echocardiography, 2022

Research paper thumbnail of Abnormal Origin of one Pulmonary Artery from the Ascending Aorta–Embryologic Considerations

Fetal and Pediatric Pathology, 2016

Anomalous origin of one of the pulmonary arteries from the aorta is unusual. The reported morbidi... more Anomalous origin of one of the pulmonary arteries from the aorta is unusual. The reported morbidity and mortality is mostly due to early onset vascular disease. Early surgical intervention has significantly improved outcomes. The diagnosis of this abnormality is challenging. The nomenclature used is inconsistent. Familiarity with this abnormality and consistent use of definitions and classifications is mandatory. An attempt is made to clarify misleading inconsistencies. An older ontogenetic theory is revisited.

Research paper thumbnail of Utility of N-Terminal Brain Natriuretic Peptide Plasma Concentrations in Comparison to Lactate and Troponin in Children with Congenital Heart Disease Following Open-Heart Surgery

Pediatric Cardiology, 2005

We conducted a prospective study in a pediatric cardiac intensive care unit in order to determine... more We conducted a prospective study in a pediatric cardiac intensive care unit in order to determine the diagnostic value of N-terminal brain natriuretic peptide (N-BNP) plasma concentration in the perioperative care of children with congenital heart disease (CHD). N-BNP plasma concentrations were determined by using a validated enzyme immunoassay. We measured N-BNP the day before surgery and up to 15 days postoperatively in 23 children (age range, 0.25-11 years) undergoing cardiac surgery due to various CHDs. Supply and duration of catecholamines, vasodilators, and respiratory therapy were determined and correlated to N-BNP. In addition, troponin T (TnT) and arterial Lactat (aL) concentrations were measured simultaneously. We found a significant correlation between preoperative and maximal N-BNP levels and dosage of vasodilators (r = 0.41, p < 0.02 and r = 0.83, p < 0.01, respectively). Maximal TnT and aL levels were not correlated to dosage of vasodilators. The dosage and duration of catecholamines, the duration of respiratory therapy, and the plasma concentration of TnT and aL were not correlated to pre-or perioperative N-BNP. Maximal TnT and aL levels were correlated to duration (r = 0.53, p < 0.01 and r = 0.48, p < 0.02) and dosage (r = 0.52, p < 0.02 and r = 0.60, p < 0.01) of catecholamines and duration of respiratory therapy (r = 0.57, p < 0.01 and r = 0.50, p < 0.02). As recent studies show, N-BNP appears to be a powerful neurohumoral indicator of ventricular function and prognosis for guiding therapy in the outpatient department or for discriminating cardiac from noncardiac symptoms. In contrast, the value of N-BNP for guiding perioperative therapy in pediatric cardiac intensive care units is limited.

Research paper thumbnail of Detection of Partial Anomalous Pulmonary Venous Connection by Magnetic Resonance Imaging

Pediatric Cardiology, 2005

Research paper thumbnail of Real-Time 3-Dimensional Echocardiography for Follow-up of Waterston Shunt

Journal of the American Society of Echocardiography, 2008

Research paper thumbnail of Magnetic resonance imaging follow up of total cavopulmonary connection

Research paper thumbnail of Ethics Consultation: The Least Dangerous Profession?

Cambridge Quarterly of Healthcare Ethics, 1993

Whether ethics is too important to be left to the experts or so important that it must be is an a... more Whether ethics is too important to be left to the experts or so important that it must be is an age-old question. The emergence of clinical ethicists raises it again, as a question about professionalism. What role clinical ethicists should play in healthcare decision making – teacher, mediator, or consultant – is a question that has generated considerable debate but no consensus.

Research paper thumbnail of Isolated Congenital Right Atrial Aneurysm

World journal for pediatric & congenital heart surgery, 2016

Isolated congenital right atrial aneurysm is rare. Indications for surgery in asymptomatic patien... more Isolated congenital right atrial aneurysm is rare. Indications for surgery in asymptomatic patients with moderate-size right atria remain controversial. Evidence in support of medical management and timing of prophylactic surgery is reviewed. We propose the use of three echocardiographic indices to help identify inappropriate atrial growth and facilitate surgical decision-making.

Research paper thumbnail of A modified noninvasive screening protocol for pulmonary hypertension in children with sickle cell disease-Who should be sent for invasive evaluation?

Pediatric blood & cancer, Jan 5, 2017

Invasive studies have shown that prevalence and severity of pulmonary hypertension (PH) in patien... more Invasive studies have shown that prevalence and severity of pulmonary hypertension (PH) in patients with sickle cell disease (SCD) tend to be overestimated if based exclusively on Doppler-derived tricuspid regurgitant velocity (TRV) as surrogate noninvasive marker with a cutoff ≥2.5 m/s. We aimed to better define a subgroup of pediatric SCD patients who should be sent for invasive evaluation of pulmonary artery pressure (PAP) based on a modified echocardiographic PH screening protocol that implements evidence from Doppler-catheter comparative studies. Charts of 121 pediatric patients with stable SCD were reviewed regarding echocardiographically assessed risk for elevated PAP/PH and associated clinical characteristics. TRV cutoff was refined at ≥2.9 m/s to avoid overestimating the risk for PH. TRV was combined with additional echocardiographic parameters to avoid underestimating the PH risk. Ninety-one patients qualified for analysis. Based on our modified echocardiographic protocol,...

Research paper thumbnail of Further evidence that variants in PPP1CB cause a rasopathy similar to Noonan syndrome with loose anagen hair

American journal of medical genetics. Part A, Feb 1, 2017

Further Evidence That Variants in PPP1CB Cause a Rasopathy Similar to Noonan Syndrome with Loose ... more Further Evidence That Variants in PPP1CB Cause a Rasopathy Similar to Noonan Syndrome with Loose Anagen Hair Regina M. Zambrano,* Michael Marble, Stuart A. Chalew, Christian Lilje, Alfonso Vargas, and Yves Lacassie* Division of Genetics, Department of Pediatrics, Louisiana State University Health Sciences Center School of Medicine, and Children’s Hospital, New Orleans, Louisiana Division of Endocrinology, Department of Pediatrics, Louisiana State University Health Sciences Center School of Medicine, and Children’s Hospital, New Orleans, Louisiana Division of Cardiology, Department of Pediatrics, Louisiana State University Health Sciences Center School of Medicine, and Children’s Hospital, New Orleans, Louisiana

Research paper thumbnail of Pediatric Pulmonary Embolism: Diagnostic and Management Challenges

World journal for pediatric & congenital heart surgery, Jan 12, 2016

A rare case of massive pulmonary embolism is presented in an oligosymptomatic teenager with predi... more A rare case of massive pulmonary embolism is presented in an oligosymptomatic teenager with predisposing factors. Computed tomography pulmonary angiography supported by three-dimensional reconstruction was diagnostic. The embolus qualified as massive by conventional anatomical guidelines, but as low risk by more recent functional criteria. Functional assessment has complemented morphologic assessment for risk stratification in adult patients. Such evidence is scarce in pediatrics. The patient underwent surgical embolectomy, followed by prophylactic anticoagulation, without further events. Diagnostic and management challenges are discussed.

Research paper thumbnail of Tetralogy of fallot and anomalous origin of the left pulmonary artery from the ascending aorta associated with absent right internal carotid artery

World journal for pediatric & congenital heart surgery, 2015

Anomalous origin of the left pulmonary artery from the ascending aorta is a rare cardiac malforma... more Anomalous origin of the left pulmonary artery from the ascending aorta is a rare cardiac malformation more commonly described with tetralogy of Fallot (TOF). The association of absent right carotid artery with TOF and anomalous left pulmonary artery from the ascending aorta has been rarely reported in the past. This case highlights the need for careful evaluation of the aortic arch and head vessels anatomy in patients with TOF because of the potential neurologic complications after surgery.

Research paper thumbnail of How I felt about it: Thoughts on a consultation

Monatsschrift Kinderheilkunde, 2007

Zusammenfassung??Wir berichten von der Auseinandersetzung eines jugendlichen Patienten mit einem ... more Zusammenfassung??Wir berichten von der Auseinandersetzung eines jugendlichen Patienten mit einem bevorstehenden chirurgischen Eingriff. Aufgrund bilateraler Hydronephrose bei Urethralklappen war eine Nierentransplantation erfolgt. Rezidivierende Harnwegsinfektionen, zwei Transplantatabsto?ungen und ein vesico-ureteraler Reflux bei Low-Compliance-Harnblase hatten zu einer kompensierten Insuffizienz der Transplantatniere gef?hrt. Es war eine Blasen-Augmentationsplastik vorgesehen.Bereits im Vorfeld hatte der Junge offenbar Angst, nicht zu verstehen bzw. nicht verstanden zu werden.

Research paper thumbnail of Ross-Konno Procedure in Children – Mid-term Results

The Thoracic and Cardiovascular Surgeon, 2010

The aim of this study was to analyze the midterm results following the Ross-Konno procedure in ch... more The aim of this study was to analyze the midterm results following the Ross-Konno procedure in children. Between 1999 and 2008, 29 patients with complex left ventricular outflow tract obstruction underwent the Ross-Konno procedure. There were 12 (41%) infants (group A) and 17 (59%) older patients (group B). The median age at operation was 3.3 years (range, 6 days to 16 years). At 7 years of follow-up, survival was 96% (1 late death), with no differences between groups A and B. Freedom from aortic regurgitation ≥ mild was 81%, with no differences between groups A and B. No residual gradient was noted in the left ventricular outflow tract in either group. Freedom from mitral regurgitation ≥ mild was 100% in group B and 41% in group A (P = .0029). The mitral regurgitation was associated with morphological abnormalities of the mitral valve and with development of endocardial fibroelastosis after failed intervention during the newborn period. Freedom from reoperation was 73% in group B and 24% in group A (P = .0093). All patients are now in sinus rhythm, and 43% are without medication. With the technical aspects of this procedure well accomplished, mortality is low, and the functional outcome is encouraging. At midterm follow-up, there was no residual or recurrent outflow tract obstruction and an excellent function of the neoaortic valve. The higher incidence of mitral regurgitation in infants, which was associated with morphological abnormalities of mitral valve and development of endocardial fibroelastosis, is worrisome.

Research paper thumbnail of MRT in der postoperativen Diagnostik bei funktionell univentrikulärem Herz: Korrelation zu Echokardiographie und Kardangiographie

RöFo - Fortschritte auf dem Gebiet der Röntgenstrahlen und der bildgebenden Verfahren, 2002

To determine the value of MRI in the postoperative evaluation of a singular ventricle compared to... more To determine the value of MRI in the postoperative evaluation of a singular ventricle compared to echocardiography and cardiac catheterization. Thirty-one patients (range: 6 months to 30 years) with a functional single ventricle following palliative corrective operations. Five patients had a Blalock-Taussig-Shunt, 8 patients a Glenn-Anastomosis and 18 a cavopulmonary shunt (6 with classic Fontan-Circulation, 12 with modified cavopulmonary anastomosis). The results in terms of postoperative morphologic changes were compared to percutaneous echocardiography (31/31) and cardiac catheterization (6/31). Echocardiography, which was performed on all patients, could not visualize the entire length of the tunnel, the Glenn-Anastomosis or the central pulmonary arteries in 70 % of the patients due to an inadequate acoustic window. MRI was able to show the entire tunnel in 11/12 patients and the central pulmonary arteries in 30/31 patients. The exact anatomy was seen in all 6 patients undergoing cardiac catheterization. MRI is useful in the postoperative evaluation of a functionally single ventricle. It is superior to echocardiography. Cardiac catheterization should be reserved for patients with inconclusive MRI findings.

Research paper thumbnail of MRT der Pulmonalarterien bei Kindern nach arterieller Switch-Operation (ASO) bei Transposition der großen Gefäße (d-TGA)

RöFo - Fortschritte auf dem Gebiet der Röntgenstrahlen und der bildgebenden Verfahren, 2005

To determine the value of MRI in postoperative evaluation of patients with arterial-switch-operat... more To determine the value of MRI in postoperative evaluation of patients with arterial-switch-operation (ASO) for d-TGA. 14 consecutive patients with d-TGA and ASO were examined with MRI in addition to ultrasound because the acoustic window in echocardiography was insufficient. Nine patients had a pulmonary-artery-bifurcation anterior to the aortic root (group 1), and five (group 2) laterally (two of them left, three of them right). MRI was performed in six patients as contrast-enhanced MR-angiography, in 8 patients as native examination. Diameters of pulmonary arteries were measured in two perpendicular views at the origin and in two further locations each with a distance of one cm. One patient showed a supravalvular pulmonary artery stenosis with subsequent pulmonary artery aneurysm, which had not been detected in echocardiography. This patient showed only indirect signs of pulmonary artery stenosis in echocardiography. Compared to group 1, pulmonary arteries in group 2 generally had greater diameters. Those pulmonary arteries in Group 2 which cross the mediastinum underneath the aortic arch had lower diameters compared to the pulmonary artery positioned on the other side. In group 1, the left-sided pulmonary artery had lower diameters than the right-sided pulmonary arteries. It seems remarkable that the narrowing of pulmonary arteries is associated with a flattening of the transversal vessel diameter. Narrowing of pulmonary arteries after ASO is associated with oval vessel diameters. Therefore, multiplanar diagnostic methods are necessary. MRI meets this prerequisite and is therefore advantageous to echocardiography in follow-up examinations at patients with ASO for d-TGA. Special attention should be focused on the main pulmonary artery, on the left pulmonary artery in patients with preaortic pulmonary artery bifurcation as well as on the pulmonary artery which crosses the mediastinum in patients with laterally placed pulmonary artery bifurcation. Angiography should only be performed when intervention is planned.

Research paper thumbnail of Benign course of central pontine myelinolysis in a patient with anorexia nervosa

Pediatric Neurology, 2002

All recent descriptions of the structural changes in the kidney which follow a dietary depletion ... more All recent descriptions of the structural changes in the kidney which follow a dietary depletion of magnesium are in agreement that "calcification" involving

Research paper thumbnail of Double Discordance in the Hypoplastic Left Heart

Pediatric Cardiology, 2008

A neonate was transferred for an intracardiac mass. Initially, the mass was mistaken for a hypopl... more A neonate was transferred for an intracardiac mass. Initially, the mass was mistaken for a hypoplastic right ventricle, a thrombus, or a tumor. Only a thorough segmental and sequential analysis showed double discordance with a hypoplastic left ventricle. No such entity has yet been described.

Research paper thumbnail of Schwere Neuropathie nach Chemo- und Radiotherapie eines Ewing-Sarkoms

Klinische Pädiatrie, 1995

Research paper thumbnail of Noninvasive Follow-up of Complications After the Takeuchi Operation

Journal of the American Society of Echocardiography, 2007

Anomalous origin of the left coronary artery from the pulmonary artery is a rare lesion. Untreate... more Anomalous origin of the left coronary artery from the pulmonary artery is a rare lesion. Untreated, up to 90% of patients die before 1 year of age. The Takeuchi operation and direct coronary artery reimplantation have resulted in low mortality rates and excellent functional outcome. We present the case of a 6-yearold girl with impaired exercise tolerance in whom anomalous origin of the left coronary artery from the pulmonary artery was detected. A Takeuchi operation was performed. Routine follow-up was successfully done by echocardiography. The postoperative anatomy was visualized in detail, including a baffle-topulmonary artery fistula and a coronary artery stenosis. Potentially, given current echocardiographic technology in the hands of experienced operators, routine follow-up may be done noninvasively, leaving angiography for nonroutine indications.