Ralf Rauch

Are the children and adolescents with congenital heart disease living in Southwestern Ontario really overweight and obese

OBJECTIVE To assess the prevalence of overweight and obesity in children with congenital heart disease and compare them with age-matched healthy children in Southwestern Ontario, Canada. METHODS We compared the Center of Disease Control weight and body mass index z-scores of 1080 children, aged 2 to 18 years, who presented to our paediatric cardiology outpatient clinic from 2008 to 2010 for congenital heart disease with 1083 healthy controls. RESULTS In all, 18.2% of the children with congenital heart disease and 20.8% of healthy children were identified to be either overweight or obese. Overall, the weight category distribution had been similar between the congenital heart disease and healthy control groups, as well as between the congenital heart disease subgroups. There was no difference in normal weight and overweight/obese categories between children with congenital heart disease and healthy children. The underweight category, however, showed a significantly higher prevalence in congenital heart disease compared with healthy children (6.8 and 4.5%, respectively, p = 0.03). CONCLUSION The prevalence of overweight/obesity did not differ in children with congenital heart disease compared with age-matched healthy children; however, it is still high (18.2%). Obesity may represent an additional risk factor for the long-term cardiovascular health of congenital heart disease patients aside from the underlying heart defect.

Recanalisation of bilateral superior vena cava after total cavopulmonary connection

Systemvenöse Kollateralgefäße werden häufig nach Glenn- oder modifizierter Fontan-Operation beobachtet. Therapie der Wahl zum Verschluss kleinkalibriger Gefäße ist die Embolisation mit Hilfe von replatzierbaren Metallspiralen. Etwas problematischer ist die Situation bei Patienten mit großen Kollateralen wie z. B. einer rekanalisierten bilateralen oberen Hohlvene. Wir berichten über zwei Patienten, bei denen mehrere Jahre nach Fontan-Operation bei zunehmender Zyanose eine rekanalisierte linke bzw. rechte obere Hohlvene diagnostiziert wurde. Bei beiden Patienten gelang der interventionelle Verschluss dieser großlumigen Venen mittels 8 mm Amplatzer®-Muscular-VSD-Occluder, die arteriellen Sauerstoffsättigungen stiegen bei beiden Patienten postinterventionell an. Der Verschluss rekanalisierter oberer Hohlvenen nach Fontan’scher Kreislauftrennung ist mittels Amplatzer®-Muscular-VSD-Occluder technisch ohne Probleme möglich. Um die erneute Formation von venovenösen Kollateralen zu vermeiden muss darauf geachtet werden, dass der Okkluder in die V. cava knapp unterhalb der Mündung der V. azygos/hemiazygos platziert wird. Formation of systemic to pulmonary venous or systemic venous left atrial collaterals frequently occurs in patients after Glenn or Fontan-type operations. Embolization with detachable metal coils is the therapy of choice for the closure of small vessels. These devices however are not appropriate for the occlusion of large collaterals, e. g. recanalised bilateral caval veins. We report two patients who presented late after Fontan-type operations with a gradual decrease in oxygen saturation due to recanalisation of bilateral caval veins. Interventional closure of these large veins was carried out successfully with the use of 8 mm Amplatzer® muscular VSD Occluders, resulting in an increase of arterial oxygen saturations. The closure of recanalised bilateral superior caval veins after Fontan procedures is possible without technical problems by means of the Amplatzer® muscular VSD Occluder. In order to avoid future formation of venous collaterals via the azygos or hemiazgos system, the occluder should be placed in the vena cava below the orifice of the azygos/ hemiazygos vein.

Recanalisation of bilateral superior vena cava after total cavopulmonary connection. Interventional occlusion with the Amplatzer VSD Occluder.

Systemvenöse Kollateralgefäße werden häufig nach Glenn- oder modifizierter Fontan-Operation beobachtet. Therapie der Wahl zum Verschluss kleinkalibriger Gefäße ist die Embolisation mit Hilfe von replatzierbaren Metallspiralen. Etwas problematischer ist die Situation bei Patienten mit großen Kollateralen wie z. B. einer rekanalisierten bilateralen oberen Hohlvene. Wir berichten über zwei Patienten, bei denen mehrere Jahre nach Fontan-Operation bei zunehmender Zyanose eine rekanalisierte linke bzw. rechte obere Hohlvene diagnostiziert wurde. Bei beiden Patienten gelang der interventionelle Verschluss dieser großlumigen Venen mittels 8 mm Amplatzer®-Muscular-VSD-Occluder, die arteriellen Sauerstoffsättigungen stiegen bei beiden Patienten postinterventionell an. Der Verschluss rekanalisierter oberer Hohlvenen nach Fontan’scher Kreislauftrennung ist mittels Amplatzer®-Muscular-VSD-Occluder technisch ohne Probleme möglich. Um die erneute Formation von venovenösen Kollateralen zu vermeiden muss darauf geachtet werden, dass der Okkluder in die V. cava knapp unterhalb der Mündung der V. azygos/hemiazygos platziert wird. Formation of systemic to pulmonary venous or systemic venous left atrial collaterals frequently occurs in patients after Glenn or Fontan-type operations. Embolization with detachable metal coils is the therapy of choice for the closure of small vessels. These devices however are not appropriate for the occlusion of large collaterals, e. g. recanalised bilateral caval veins. We report two patients who presented late after Fontan-type operations with a gradual decrease in oxygen saturation due to recanalisation of bilateral caval veins. Interventional closure of these large veins was carried out successfully with the use of 8 mm Amplatzer® muscular VSD Occluders, resulting in an increase of arterial oxygen saturations. The closure of recanalised bilateral superior caval veins after Fontan procedures is possible without technical problems by means of the Amplatzer® muscular VSD Occluder. In order to avoid future formation of venous collaterals via the azygos or hemiazgos system, the occluder should be placed in the vena cava below the orifice of the azygos/ hemiazygos vein.

N-terminal pro-brain natriuretic peptide level as a screening tool for cardiac involvement in paediatric diseases of extracardiac origin

BackgroundSerum concentrations of N-terminal natriuretic pro-brain natriuretic peptide (NT-proBNP) correlate well with the severity of cardiac disease in children and adults. There are also paediatric illnesses of extracardiac origin that may cause deterioration of the cardiac function in some patients (pts). A high NP level can signify a concomitant cardiac involvement in respiratory distress, or secondary to a systemic disease.ObjectiveTo assess the potential of NT-proBNP as a screening tool for cardiac involvement in paediatric diseases of extracardiac origin.MethodsA literature search was performed in PubMed for the relevant studies with the subject brain natriuretic peptide (BNP) and NT-proBNP from 1995 to 2010 with stress on characteristics of the natriuretic peptides, paediatric reference values and systemic paediatric diseases with potential influence on the myocardial performance. The main focus has been NT-proBNP being the most stable NP and correlating well with BNP.ResultsMost studies agree that a high NT-proBNP level indicates cardiac dysfunction in different systemic diseases such as thalassemia and cystic fibrosis or in pts after chemotherapy. In special clinical scenarios as sepsis or respiratory distress, NT-proBNP helps to narrow down the differential diagnosis or can be used for risk stratification.ConclusionNT-proBNP can serve as a screening tool in various primarily non-cardiac paediatric diseases. Together with the clinical picture, a high level suggests further assessment by a paediatric cardiologist.

Thrombosis of an extracardiac fontan tunnel: Combined treatment of thrombolysis and stenting

Obstruction of the so‐called Fontan tunnel represents a life‐threatening complication and requires immediate intervention. We describe the successful use of prolonged thrombolysis with rt‐PA, allowing stent placement in an extracardiac tunnel in a 7‐year‐old boy.

Small coronary artery fistulae in childhood: a 6-year experience of 31 cases in a tertiary paediatric cardiac centre

BACKGROUND This study describes the incidence and course of children with small coronary artery fistulae over a period of 6 years who presented at a paediatric tertiary-care centre. Materials and methods Age at diagnosis, mode of presentation, location (origin and drainage), and association with a cardiac defect were documented and analysed. All patients obtained an electrocardiogram, and older patients were further evaluated with an exercise treadmill test. RESULTS A total of 31 patients were diagnosed with coronary artery fistula via transthoracic echocardiogram and comprised 0.43% of our entire patient group. Mean age was 6.14 years (standard deviation 5.4); 16 patients (52%) had associated cardiac defects. In the remaining 15 patients, the coronary artery fistula was discovered incidentally during diagnostic work-up for heart murmur or chest pain. Among all, 26 patients (84%) had left-sided and five patients (16%) had right-sided coronary artery fistulae. All right coronary artery fistula patients had associated cardiac defects; this was true for 42% of the patients with left coronary artery fistulae. None of the patients required any intervention due to the fistula, and spontaneous closure occurred in 12 patients (39%). CONCLUSION Small coronary artery fistulae in children are frequently an incidental finding, and many will close spontaneously. Our data are supportive of a conservative, observant approach in asymptomatic patients with small coronary artery fistula in the paediatric population.

Does obesity affect the non-invasive measurement of cardiac output performed by electrical cardiometry in children and adolescents?

Electrical cardiometry (EC) is a non-invasive and inexpensive method for hemodynamic assessment and monitoring. However, its feasibility for widespread clinical use, especially for the obese population, has yet to be determined. In this study, we evaluated the agreement and reliability of EC compared to transthoracic Doppler echocardiography (TTE) in normal, overweight, and obese children and adolescents. We measured stroke volume (SV) and cardiac output (CO) of 131 participants using EC and TTE simultaneously. We further divided these participants according to BMI percentiles for subanalyses: <85% normal weight (n = 41), between 85 and 95% overweight (n = 7), and >95% obese (n = 83). Due to small sample size of the overweight group, we combined overweight and obese groups (OW+OB) with no significant change in results (SV and CO) before and after combining groups. There were strong correlations between EC and TTE measurements of SV (r = 0.869 and r = 0.846; p < 0.0001) and CO (r = 0.831 and r = 0.815; p < 0.0001) in normal and OW+OB groups, respectively. Bias and percentage error for CO measurements were 0.240 and 29.7%, and 0.042 and 29.5% in the normal and OW+OB groups, respectively. Indexed values for SV were lower in the OW+OB group than in the normal weight group when measured by EC (p < 0.0001) but no differences were seen when measured by TTE (p = 0.096). In all weight groups, there were strong correlations and good agreement between EC and TTE. However, EC may underestimate hemodynamic measurements in obese participants due to fat tissue.

Fallot Tetralogie mit Abgang der rechten Pulmonalarterie aus der Aorta aszendens: Nichtinvasive Diagnostik und operative Korrektur bei einem 7 Wochen alten Säugling

Anomalous origin of one pulmonary artery from the ascending aorta is a rare anomaly which is almost always associated with hypertension in the contralateral pulmonary artery originating from the right ventricle. We report echocardiographic evaluation of an infant with tetralogy of Fallot and aortic origin of the right pulmonary artery. Since all relevant information regarding anatomy and hemodynamics could be obtained by echocardiography, cardiac catheterization and angiography were unnecessary. Surgical correction including transatrial patch closure of the ventricular septal defect, resection of the infundibular stenosis and direct reimplantation of the right pulmonary artery was performed successfully at the age of 7 weeks. The postoperative course was uneventful and persistent pulmonary hypertension was excluded by echocardiography. Our case shows that complete echocardiographic evaluation is possible in infants with this complex cardiac malformation. We recommend reserving cardiac catheterization for those patients with echocardiographic evidence of significant elevation of pulmonary vascular resistance. In order to avoid irreversible pulmonary vascular changes early surgical correction within the first 3 months of life should be performed in all children with aortic origin of a pulmonary artery including those with associated tetralogy of Fallot. Der Abgang einer Pulmonalarterie aus der Aorta aszendens ist eine seltene Anomalie, die meist auch mit einer pulmonalen Hypertension der regulär mit dem rechten Ventrikel konnektierten Pulmonalarterie einhergeht. Wir berichten über die echokardiographische Evaluation der Anatomie und Hämodynamik bei einem Kind mit Fallot Tetralogie und Abgang der rechten Pulmonalarterie aus der Aorta aszendens. Eine invasive Diagnostik durch Herzkatheteruntersuchung und Angiokardiographie wurde nicht notwendig. Die operative Korrektur mit transatrialem Patchverschluss des Ventrikelseptumdefektes, Resektion der subvalvulären Pulmonalstenose und direkter Reimplantation der rechten Pulmonalarterie erfolgte im Alter von 7 Wochen. Der postoperative Verlauf war komplikationslos, echokardiographisch konnte eine persistierende pulmonale Hypertension ausgeschlossen werden. Unser Fall zeigt, dass auch bei Kindern mit dieser komplexen kardialen Anomalie eine invasive Diagnostik nicht prinzipiell erforderlich ist. Eine Herzkatheteruntersuchung kann den Fällen vorbehalten bleiben, in denen sich echokardiographisch Hinweise für eine signifikante pulmonale Widerstandserhöhung ergeben. Um irreversible pulmonale Gefäßveränderungen zu vermeiden, sollte auch bei Kindern mit Fallot Tetralogie und Fehlabgang einer Pulmonalarterie aus der Aorta die operative Korrektur vor dem Alter von 3 Monaten erfolgen.

Cardiac footprints of a neonatal vein of Galen malformation

Vein of Galen arterio-venous malformation is a rare congenital anomaly, which presents in the neonatal period. High output heart failure in the absence of an underlying structural heart defect points towards the condition and is an important contributing factor for morbidity and mortality. We describe a case of a term newborn who presented with respiratory distress and cardiomegaly. The characteristic echocardiographic findings of high output cardiac failure were found and eventually led to the diagnosis of an underlying vein of Galen arterio-venous malformation. The relevant literature is discussed.

Images in CardiologyShone's Complex and Levoatriocardinal Vein: A Rare Association

![Figure][1] [![Graphic][3] ][3][![Graphic][4] ][4][![Graphic][5] ][5] A 10-week-old male infant was referred for a systolic heart murmur, mild congestive heart failure with tachypnea, tachycardia, mildly enlarged liver, and a chest x-ray showing an enlarged cardiac