Double discordance in the hypoplastic left heart.

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.

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.

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.

[MRI of pulmonary arteries in follow-up after arterial-switch-operation (ASO) for transposition of great arteries (d-TGA)]. / MRT der Pulmonalarterien bei Kindern nach arterieller Switch-Operation (ASO) bei Transposition der grossen Gefässe (d-TGA).

PURPOSE: To determine the value of MRI in postoperative evaluation of patients with arterial-switch-operation (ASO) for d-TGA. MATERIAL AND METHODS: 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. RESULTS: 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. CONCLUSION: 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.

[MRI of congenital heart disease in childhood]. / MR-Tomographie kongenitaler Herzvitien im Kindesalter.

MRI provides a non-invasive diagnostic tool complementing echocardiography on one hand, and showing advantages over echocardiography, on the other hand, especially after corrective procedures. The multiple different MRI sequences need to be adapted to examinations of children and patients with congenital heart disease (CHD), and can be used to detect morphologic changes, blood-flow in the heart and thoracic vessels and diastolic or systolic function of myocardium. Several factors determine the success of the examination of a complex congenital heart disease or a postoperative situs. Pediatric radiologists and radiologists experienced in congenital heart diseases have to work in close cooperation. Echocardiography should be performed before MRI. The results of prior examinations and the clinical history of the patients, including possible palliative or reconstructive operations, must be available before MRI to guide the planning of the examination. With a systematic segmental approach to the situs, to the atrial and ventricular morphology, and to the visceroatrial, atrioventricular and ventriculoarterial connections, most CHDs can be diagnosed correctly. With appropriate knowledge, MRI can also be performed quite accurately after complex operations and may avoid or delay invasive diagnostic procedures. The aim of this article is to impart knowledge, to mention problems and to provide guidance in the performance of cardiac MRI in patients with CHD.

[MRI in the diagnosis of non-compacted ventricular myocardium (NCVM) compared to echocardiography]. / MR-Tomographie in der morphologischen Darstellung der isolierten non-compaction des linksventrikulären Myokards (NCVM).

PURPOSE: To report the detection of non-compacted ventricular myocardium (NCVM) with MRI compared to echocardiography in 8 patients. MATERIAL AND METHODS: Non-compaction of the ventricular myocardium is a congenital disorder characterized by an altered structure of the myocardial wall resulting from an intrauterine arrest in endomyocardial embryogenesis. The morphological findings consist of a prominent meshwork of multiple myocardial trabeculations and deep intertrabecular recesses, communicating with the left ventricular cavity. 8 consecutive patients (mean age 7.3 years) with clinical and echocardiographic signs of NCVM were examined by MRI (1.5 T, Vision, Siemens) in short axis and 2- and 4-chamber views, using T (1)-weighted TSE and Cine-GRE in 6 patients and true FISP sequences in 2 patients. MRI and echocardiography were evaluated for visibility, signs of NCVM and involvement of myocardial wall segments. Thickness was measured for non-compacted and compacted myocardium and the non-compacted to compacted (N/C) ratio calculated. RESULTS: MRI diagnosed 6 of 8 patients of having NCVM. Myocardial thickness as measured by echocardiography and MRI showed a good correlation in compacted myocardium (r = 8.82) and no correlation in non-compacted myocardium (r = 0.4). In 2 cases, non-compacted myocardium was detected but echocardiography did not reach the N/C ratio > 2 as required to diagnose NCVM in accordance with the criteria found in the literature. Both patients were also misdiagnosed by MRI performed with Cine-GRE. MRI reached a N/C ratio > 2 in only three patients. Newer TruFisp sequences showed no definite advantages. Extent of non-compaction could be visualized correctly with MRI. CONCLUSION: Echocardiography is the method of choice to detect NCVM. MRI can be an alternative in some cases. The diagnosis of NCVM should not be considered until N/C ratio is over 2.

Acute heart failure during spinal surgery in a boy with Duchenne muscular dystrophy.

Patients with Duchenne muscular dystrophy (DMD) are at high risk of perioperative complications. DMD may be accompanied by heart failure resulting from dystrophic involvement of the myocardium, which can be subclinical in the early stages of the disease. This case demonstrates that a normal preoperative ECG and echocardiograph cannot exclude the development of heart failure during anaesthesia in DMD patients undergoing major surgery.

[MRI in postoperative assessment of univentricular heart disease: correlation with echocardiography and angiography]. / MRT in der postoperativen Diagnostik bei funktionell univentrikulärem Herz: Korrelation zu Echokardiographie und Kardangiographie.

PURPOSE: To determine the value of MRI in the postoperative evaluation of a singular ventricle compared to echocardiography and cardiac catheterization. MATERIALS AND METHODS: 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). RESULTS: 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. CONCLUSION: 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.

[Interventional occlusion of persistent ductus arteriosus Botalli with Gianturco spirals]. / Interventioneller Verschluss des persistierenden Ductus arteriosus Botalli mit Gianturco-Spiralen.

Interventional duct occlusion is increasingly performed with detachable coils. In a series of 33 patients (34 procedures), we consistently used Gianturco-coils which were the initially employed coils. They are not securable but can be implanted in a simple manner. The rate of embolization was high (28%) in the first 14 procedures; however, it declined to 5% in the last 20 patients. We modified the technique by partially blocking the duct, when necessary, with a catheter which was advanced from the pulmonary artery and by implanting up to three coils. There was only one embolization and one small residual shunt in the last 20 patients. The mean fluoroscopy time in these patients was 11.0 +/- 5.8 minutes and decreased to 7.0 +/- 3.2 minutes in the last 10 patients with successful closure. Considering our experience, we continue to recommend the use of Gianturco-coils especially in the small duct which can be closed quickly at low cost.

Congenital aneurysm of the descending aorta in a neonate.

A large aneurysm of the descending aorta of unknown etiology was diagnosed in utero. Medical treatment with propranolol was continued after surgery which was performed at the age of 2 months.

[Severe neuropathy after chemo- and radiotherapy of Ewing sarcoma]. / Schwere Neuropathie nach Chemo- und Radiotherapie eines Ewing-Sarkoms.

A 15 year old patient presenting with a lumbar Ewing's Sarcoma was treated according to the EICESS-92 pilot study (EVAIA branch). Local therapy consisted of definitive irradiation given simultaneously to chemotherapy courses 5 to 7. Close to the end of treatment the patient developed progressive peripheral neurologic deficits. Such combined toxic treatment side effects are unique and unforeseen in the study protocol.