Indications for cardiovascular magnetic resonance in children with congenital and acquired heart disease: an expert consensus paper of the Imaging Working Group of the AEPC and the Cardiovascular Magnetic Resonance Section of the EACVI.

This article provides expert opinion on the use of cardiovascular magnetic resonance (CMR) in young patients with congenital heart disease (CHD) and in specific clinical situations. As peculiar challenges apply to imaging children, paediatric aspects are repeatedly discussed. The first section of the paper addresses settings and techniques, including the basic sequences used in paediatric CMR, safety, and sedation. In the second section, the indication, application, and clinical relevance of CMR in the most frequent CHD are discussed in detail. In the current era of multimodality imaging, the strengths of CMR are compared with other imaging modalities. At the end of each chapter, a brief summary with expert consensus key points is provided. The recommendations provided are strongly clinically oriented. The paper addresses not only imagers performing CMR, but also clinical cardiologists who want to know which information can be obtained by CMR and how to integrate it in clinical decision-making.

Giant infantile fibroma of the right ventricle - surgical debulking and tumor plication.

Cardiac fibromas are rare lesions which occur predominantly in infants and children. In a 2-week-old premature infant with progressive exertional dyspnea, a huge cardiac tumor (5.0 x 4.5 x 5.0 cm) obstructing the right ventricle was diagnosed. Due to tumor progression with resulting obstruction of the right ventricular outflow tract (RVOT), surgery became necessary at 6 months. The tumor was partially resected, creating a crater-like defect, and the resection margins were subsequently plicated. Histological examination confirmed infantile fibroma. The combination of early diagnosis, the time and opportunity for cardiac development and immediate excision once symptoms occur is supposed to improve survival.

Indications for cardiovascular magnetic resonance in children with congenital and acquired heart disease: an expert consensus paper of the Imaging Working Group of the AEPC and the Cardiovascular Magnetic Resonance Section of the EACVI.

This article provides expert opinion on the use of cardiovascular magnetic resonance (CMR) in young patients with congenital heart disease (CHD) and in specific clinical situations. As peculiar challenges apply to imaging children, paediatric aspects are repeatedly discussed. The first section of the paper addresses settings and techniques, including the basic sequences used in paediatric CMR, safety, and sedation. In the second section, the indication, application, and clinical relevance of CMR in the most frequent CHD are discussed in detail. In the current era of multimodality imaging, the strengths of CMR are compared with other imaging modalities. At the end of each chapter, a brief summary with expert consensus key points is provided. The recommendations provided are strongly clinically oriented. The paper addresses not only imagers performing CMR, but also clinical cardiologists who want to know which information can be obtained by CMR and how to integrate it in clinical decision-making.

Proton-decoupled myocardial 31P NMR spectroscopy reveals decreased PCr/Pi in patients with severe hypertrophic cardiomyopathy.

Disturbed myocardial energy metabolism may occur in patients with primary hypertrophic cardiomyopathy (HCM). A noninvasive way to gain insight into cardiac energy metabolism is provided by in vivo 31P nuclear magnetic resonance (NMR) spectroscopy. 31P NMR spectroscopy with proton decoupling was performed in 13 patients aged 13-36 years with HCM on a 1.5 T Magnetom with a double resonant surface coil. A 2D chemical shift imaging (CSI) sequence in combination with slice selective excitation was used to acquire spectra of the anteroseptal region of the left ventricle (volume element: 38 mL). The chemical shifts of the phosphorus metabolites, intracellular pHi, and coupling constants J(alphabeta) and J(gammabeta) were calculated. Peak areas of 2,3-diphosphoglycerate (DPG), Pi, and adenosine triphosphate (ATP) were determined and corrected for blood contamination, saturation, and differences in nuclear Overhauser enhancements (NOE). The maximum thickness of the interventricular septum (IVSmax) was determined from tomographic long-axis images and expressed as number of standard deviations above the mean of the normal population (Z score). The patients were then divided into 2 groups: 6 patients with moderate HCM (HCMm, Z score < or = 5) and 7 patients with severe HCM (HCMs, Z score > 5). No differences between both groups and a control group of healthy volunteers (n = 16) were found with respect to phosphocreatine (PCr)/gamma-ATP ratio, pHi, or the coupling constants. Only the PCr/Pi ratio differed significantly from the control group (HCM(all), alpha < 0.05, HCMs, alpha < 0.02, 2-sided U test). The decrease of the PCr/Pi ratio in patients with HCM is probably caused by ischemically decreased oxygen supply in the severely hypertrophied myocardium.

S-100 after correction of congenital heart defects in neonates: is it a reliable marker for cerebral damage?

BACKGROUND: Newborns undergoing cardiac operation may acquire some extent of neuronal damage. An early diagnosis is especially hard regarding neonates. In the past years, S-100 has been widely discussed as a marker revealing perioperative damage to the brain. METHODS: Sequential blood samples from 33 neonates undergoing repair of congenital heart disease were taken perioperatively. Samples of 12 healthy neonates were taken at birth as a control group. The newborns were divided into four groups: cyanotic and acyanotic disease operated on in deep hypothermic circulatory arrest, operation without deep hypothermic cardiac arrest, and operation without extracorporeal circulation. RESULTS: Even in healthy neonates, serum S-100 levels were at 10-fold values compared with adults. On admission, S-100 values in the operative groups were similar. During extracorporeal circulation, levels rose to a certain degree. Cyanotic newborns operated on in deep hypothermic cardiac arrest had significantly higher S-100 levels compared with acyanotic newborns also operated on in deep hypothermic cardiac arrest (p < 0.001). Two newborns who experienced seizures postoperatively had the highest absolute S-100 levels. One child with a poor neurologic outcome but no seizures did not have different values when compared with her group. CONCLUSIONS: In this study, S-100 seemed to be a possible marker for a certain degree of neurologic deficit after cardiac operation in neonates, especially regarding postoperative seizures. The missing peaks of this protein in one newborn with poor neurologic outcome show that it is not possible to exclude damage to the brain with normal postoperative values. These results suggest that the mechanism of cerebral damage and S-100 release into the blood in neonates with a developing central nervous system and blood-brain barrier is not fully understood.

Detection of phosphomonoester signals in proton-decoupled 31P NMR spectra of the myocardium of patients with myocardial hypertrophy.

Proton-decoupled 31P NMR spectroscopy at 1.5 T of the anterior left ventricular myocardium was used to monitor myocardial phosphate metabolism in asymptomatic patients with hypertrophic cardiomyopathy (HCM, n = 14) and aortic stenosis (AS, n = 12). In addition to the well-known phosphorus signals a phosphomonoester (PME) signal was detected at about 6.9 ppm in 7 HCM and 2 AS patients. This signal was not observed in the spectra of normal controls (n = 11). We suggest that in spectra of patients with myocardial hypertrophy the presence of a PME signal reflects alterations in myocardial glucose metabolism.

Fast acceleration-encoded magnetic resonance imaging.

Direct acceleration imaging with high spatial resolution was implemented and tested. The well-known principle of phase encoding motion components was applied. Suitable gradient switching provides a signal phase shift proportional to the acceleration perpendicular to the slice in the first scan of the sequences. An additional scan serving as a reference was recorded for compensation of phase effects due to magnetic field inhomogeneities. The first scan compensated for phase shifts from undesired first- and second-order motions; the second scan was completely insensitive to velocity and acceleration in all directions. Advantages of the proposed two-step technique compared to former approaches with Fourier acceleration encoding (with several phase encoding steps) are relatively short echo times and short total measuring times. On the other hand, the new approach does not allow us to assess the velocity or acceleration spectrum simultaneously. The capabilities of the sequences were tested on a modern 1.5 T whole body MR unit providing relatively high gradient amplitudes (25 mT/m) and short rise times (600 micros to maximum amplitude). The results from a mechanical acceleration phantom showed a standard deviation of 0.3 m/s2 in sequences with an acceleration range between -12 and 12 m/s2. This range covers the expected maximum acceleration in the human aorta of 10 m/s2. Further tests were performed on a stenosis phantom with a variable volume flow rate to assess the flow characteristics and possible displacement artifacts of the sequences. Preliminary examinations of volunteers demonstrate the potential applicability of the technique in vivo.

High-resolution cardiac imaging using an interleaved 3D double slab technique.

A three-dimensional (3D) gradient-echo sequence with interleaved double-slab excitation was developed and optimized for the requirements in pediatric cardiac imaging. For this purpose high contrast between blood and myocardium signal should be obtained without the use of contrast agents. An acceptable measuring time for a large region examined with high spatial resolution should be achieved as well, especially with regard to the small structures of the heart and vessels of infants. The presented approach works with gradient moment nulling and a short echo time of 5.5 ms resulting in generally high signal intensity and only minor signal losses due to turbulent flow. The sequence allows simultaneous ECG-gated recording of two separately excited slabs with small thickness (10 mm) and with a distance of several centimeters between them. Thus, common effects of presaturation in 3D imaging can be avoided, although a relatively short measuring time is achievable. In order to get a 3D data set with good signal homogeneity of blood and of the other structures across a large volume of interest several double-slab measurements with suitable positions must be performed. The latter aspect is especially important for postprocessing techniques as multiple planar reconstruction and maximum intensity projection. Examples of applications of the new technique and appropriately postprocessed images are presented allowing demonstration even of subtle cardiac malformations.

Imaging of the human cardiovascular system using the rapid echo flow-rephased spin-echo technique.

Using a flow-rephased spin-echo technique with a short echo time of TE = 9.7 msec, "white blood" multislice and single slice first echo images of the human heart were acquired using standard 1.5 T whole-body imagers. The technique almost completely eliminates phase shifts for flow with constant velocity and constant acceleration, so the investigations with healthy volunteers show images which are almost free of the artifacts often found in ECG-gated standard spin-echo imaging of the heart. T1-weighted images with good contrast between tissue and blood are achieved at any time during the whole heart cycle. The results obtained indicate that the technique might be helpful for imaging small vessels, vessels which contain slowly flowing blood, and vessels located in regions with static field inhomogeneities, for example, lung vessels.

[Clinical implication of parameter-optimized 3D-FISP MR angiography (MRA) in children with aortic coarctation: comparison with catheter angiography]. / Stellenwert einer parameteroptimierten 3D-FISP-MR-Angiographie bei Kindern mit Aortenisthmusstenose im Vergleich zur Katheterangiographie.

PURPOSE: To implement parameter-optimized 3D-FISP MR angiography (MRA) with interleaved double-slab excitation and to compare the result with catheter angiography in children with aortic coarctation. MATERIALS AND METHODS: Eighteen children aged 2 - 15 years (mean 9.1 years) underwent MR imaging on a 1.5T body scanner (Magnetom Vision, Siemens, Germany). All patients had undergone correlative catheter angiography. T1-weighted turbo spin echo (TSE) images (TR 600 ms, TE 17 ms, flip 160 degrees, slice thickness 2 - 4 mm) were obtained in axial and parasagittal orientation, followed by an optimized 3D-FISP MR angiography in a sagittal plane (TR 12.5 ms, TE 5.5 ms, flip 22 degrees, matrix 256 x 256, slice thickness 1.25 mm). All children were sedated but on spontaneous breathing. Image quality was graded by two experienced reviewers using a 4-point scoring system. Source images and reformatted maximum intensity projections (MIP) were analyzed for blood-tissue contrast as well as size and focal stenoses of the aortic arch. RESULTS: Aortic coarctation was found in 13 of 18 patients, using the 3D-FISP MRA. A high correlation value (r = 0.96) was found compared to catheter angiography. Image quality was high in 94 % with well defined blood-tissue contrast in all cases. The sensitivity to flow and breathing motion was low. Examination time was about 15 minutes depending on volume of interest and heart rate. Diagnostic accuracy has shown improvement using a combined analysis of source and MIP images. The mentioned technique has provided an excellent display of thoracic vasculature. CONCLUSION: MR imaging represents an excellent tool for non-invasive examination of the cardiovascular system of children. The 3D-MRA allows the recording of a large 3D data set without the use of contrast agent and within an adequate measurement period, particularly in small infants unable to hold their breath. In addition, hemodynamic significance of aortic coarctation, the existence of collateral vessels and other congenital heart diseases can be described reliably by using this technique.

[Differential diagnosis and prognosis of primary and post-myocarditis cardiomyopathy in childhood]. / Zur Differentialdiagnose und Prognose der primären und postmyokarditischen Kardiomyopathie im Kindesalter.

It is still difficult to differentiate between the various stages of myocarditis and primary dilated cardiomyopathy (DCM). Hence, we analyzed the history, as well as the laboratory and virological data of 22 children with a dilated, poorly functioning left ventricle, aged 2 months to 16.7 years (m = 4.2 y), on whom we had performed endomyocardial biopsies about 4 months after the beginning of the illness. Specimens were investigated by light and electron microscopy and, in addition immunoserological (n = 15) and immunohistological investigations (n = 7) were performed. On the basis of cellular infiltration in the histological examination we diagnosed resolving/resolved myocarditis in 6 patients and DCM in 15 patients. Previous respiratory infection or sudden onset were found in 40-50% of patients in both groups. Antimyolemmal and antisarcolemmal antibodies showed no preference. Follow-up (3.1 +/- 2.8 years) of the patients showed equal mortality (33% vs. 38%) in the post-myocarditis and DCM group. Complete normalization of all findings in 3 patients of the DCM group makes the classification among the post-myocarditis group probable, which would, then give a lower mortality rate (22% vs. 46%) and a chance of restitution in 55% of cases. In any one specific case all the diagnostic methods do not allow undoubted distinction between both entities. The prognosis of myocarditis seems to be better than that of DCM in childhood.

[Secondary diseases of the heart muscle and their differential diagnosis in childhood]. / Sekundäre Herzmuskelerkrankungen und ihre Differentialdiagnose im Kindesalter.

Possible causes of specific cardiac muscle disease, diagnosis, follow-up and the therapeutic management are discussed on the basis of a series of cases. 8 out of 30 patients who showed the clinical picture of dilative cardiomyopathy (DCM) were found to have a specific cardiac muscle disease. 4 patients had DCM following adriamycin therapy. Fibromuscular dysplasia with renal hypertension, thalassaemia major with secondary haemosiderosis, long-overlooked and untreated athyroidism each caused one case of dilative specific cardiac muscle disease. Once DCM was preceded by the Kawasaki syndrome for over 2 years. Amongst 47 patients with hypertrophic cardiomyopathy there were two children who had undergone ACTH treatment, 6 children born of diabetic mothers, 4 cases of Pompe's disease, and one patient with hypothyroidism resulting in reversible hypertrophy of the cardiac muscle. Different neurodegenerative diseases were associated with cardiac muscle disease in 4 cases, partly dictating the clinical course. Extremely rare was the development over 6 years of cardiac hypertrophy following a burns injury.

An unusual location of deep venous thrombosis associated with ischemic stroke and persistent foramen ovale.

Up to 40% of ischemic strokes have no known cause (cryptogenic). The prevalence of persistent foramen ovale (PFO) amongst patients with cryptogenic stroke (CS) is twice as high as that of the normal population, therefore suggesting a causal relationship between the two entities. However, PFO by itself is not sufficient to cause stroke, as an embolic source is needed. This source is often unknown, making the causal relationship between CS and PFO hard to demonstrate. The most frequent, although still seldom, identifiable cause of embolism in an otherwise cryptogenic stroke associated with PFO is a deep venous thrombosis (DVT) of the lower extremities. Here, we present a unique case of brachiocephalic venous DVT associated with PFO and ischemic stroke in a young patient. As the search for DVT in patients with PFO and stroke is often limited to the lower extremities, this case may suggest that an unspecified number of DVTs are overlooked. Our report lends support to paradoxical embolism as a mechanism of stroke in patients with PFO and does, at least in selected cases, suggest a more detailed search for DVT beyond the lower extremities.

Transarterial and transvenous approach for transcatheter closure of a large coronary artery fistula with the Amplatzer vascular plug.

Interventional occlusion of coronary artery fistulas has become a well-accepted alternative to surgical therapy. However, occlusion of high-flow lesions in children, necessitating implantation of large occluding devices, may be limited by the requirement of large delivery catheters. This report describes the interventional occlusion of a large coronary artery fistula in an 8-year-old girl. Complete occlusion was achieved by subsequent transvenous and transarterial implantation of two Amplatzer vascular plugs (AVP). The AVP is an interesting alternative for interventional occlusion of large coronary artery fistulas in children that can be deployed safely by rather small guiding catheters.

[Dimenhydrinate overdosage in a 3(1/2) year-old-girl with dilative cardiomyopathy]. / Dimenhydrinat-Intoxikation bei einem 3(1/2) Jahre alten Mädchen mit dilatativer Kardiomyopathie.

Dimenhydrinate overdosage in a 3(1/2) year-old-girl with dilative cardiomyopathy. Dimenhydrinate (Vomex(R)) is frequently used in the treatment of sickness and vomiting. The symptoms of overdosage present like an anticholinergic syndrome. We report on the clinical findings of an intoxication with dimenhydrinate in a 3(1/2) year-old-girl with functional dilative cardiomyopathy following a congenital left ventricular diverticle. Especially in small children, with the application of 40 mg suppositories once or twice per day the maximum dose of 3.75 mg/kgBW/d is achieved.

[Systematic evaluation of the proximal cardiac veins for echocardiographic diagnosis of total anomalous pulmonary venous connection]. / Nichtinvasive Diagnostik der totalen Lungenvenenfehleinmündung durch systematische echokardiografische Darstellung der herznahen Venen.

PURPOSE: Total anomalous pulmonary venous connection (TAPVC) is a rare congenital cardiovascular malformation that is characterized by an absent connection of the pulmonary veins to the left atrium. Echocardiographic differentiation of TAPVC and persistent pulmonary hypertension of the newborn (PPHN) can be a great diagnostic challenge. The aim of our study was the assessment of a systematic echocardiographic approach to evaluate the feasibility and reliability of noninvasive diagnosis of TAPVC. METHODS: Between January 1995 and January 2005, 15 consecutive patients with isolated TAPVC were diagnosed in our institution. 5 patients had supracardiac type, 5 cardiac type, 3 infracardiac and 2 had mixed type of TAPVC. Since 2001 preoperative diagnosis was performed exclusively by echocardiography (8/15 patients). RESULTS: TAPVC could be diagnosed correctly in all cases using a systematic echocardiographic approach with careful investigation of the proximal cardiac veins. Infracardiac type ofTAPVC was diagnosed using systematic evaluation of the liver with visualization of a pathological vessel with venous flow directed away from the heart. In all cases the echocardiographic findings were confirmed during surgery. CONCLUSION: Echocardiography with Doppler and color Doppler sonography is an effective method for noninvasive diagnosis of TAPVC. The presented systematic echocardiographic approach permits reliable noninvasive differentiation of TAPVC and PPHN.

[Echocardiographic diagnosis of the aorto-pulmonary window]. / Echokardiografische Diagnose des aortopulmonalen Fensters.

UNLABELLED: PURPOSE/MATERIALS AND METHODS: The aorto-pulmonary window is a rare congenital cardiac malformation consisting of a connection between the ascending aorta and the main pulmonary artery. Based on 5 consecutive patients who were treated in our tertiary referral centre between 7/2000 and 11/2005, we report the echocardiographic features as well the limitations of this diagnostic method in the assessment of this rare malformation. RESULTS: Dilation of the left atrium and ventricle due to the large left-to-right-shunt was the characteristic echocardiographic feature in all patients with aorto-pulmonary window. In all patients we were able to visualise the window by 2D-echocardiography. Pulsed- and continuous-wave Doppler showed diastolic negative flow in the descending aorta and in systemic arteries due to left-to-right shunting in diastole via the aorto-pulmonary window. All 5 patients had significant associated cardiovascular malformations. They were accurately diagnosed by echocardiography. Coronary anomalies could be excluded noninvasively in all patients. CONCLUSION: Echocardiography is the method of choice for diagnosis of aortopulmonary window. Detection of aortopulmonary window, however, requires a systematic and careful investigation of all cardiovascular anatomic details, since this anomaly is frequently associated with substantial additional cardiac anomalies, which would by themselves be sufficient to explain the haemodynamic abnormalities of the patient. Cardiac catheterisation is required only in cases with inadequate information about coronary artery anatomy or in older patients with pulmonary hypertension, to determine the patient's suitability for surgical correction.

Stereolithographic reproduction of complex cardiac morphology based on high spatial resolution imaging.

BACKGROUND: Precise knowledge of cardiac anatomy is mandatory for diagnosis and treatment of congenital heart disease. Modern imaging techniques allow high resolution three-dimensional (3D) imaging of the heart and great vessels. In this study stereolithography was evaluated for 3D reconstructions of multidetector computed tomography (MDCT) and magnetic resonance imaging (MRI) data. METHODS: A plastinated heart specimen was scanned with MDCT and after segmentation a stereolithographic (STL) model was produced with laser sinter technique. After scanning the STL model with MDCT these data were compared with those of the original specimen after rigid registration using the iterative closest points algorithm (ICP). The two surfaces of the original specimen and STL model were matched and the symmetric mean distance was calculated. Additionally, the heart and great vessels of patients (age range 41 days-21 years) with congenital heart anomalies were imaged with MDCT (n=2) or free breathing steady, state free-precession MRI (n=3). STL models were produced from these datasets and the cardiac segments were analyzed by two independent observers. RESULTS: All cardiac structures of the heart specimen were reconstructed as a STL model within sub-millimeter resolution (mean surface distance 0.27+/-0.76 mm). Cardiac segments of the STL patient models were correctly analyzed by two independent observers compared to the original 3D datasets, echocardiography (n=5), x-ray angiography (n=5), and surgery (n=4). CONCLUSIONS: High resolution MDCT or MRI 3D datasets can be accurately reconstructed using laser sinter technique. Teaching, research and preoperative planning may be facilitated in the future using this technique.