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Noncompaction cardiomyopathy is a recently described rare congenital cardiomyopathy; patients can be asymptomatic or develop diastolic and/or systolic left ventricular dysfunction with heart failure, systemic emboli or ventricular arrhythmias. Long-term prognosis is poor. Currently, diagnosis is based on findings on 2D echocardiography; in the current case report we demonstrate the use of MRI to diagnose noncompaction cardiomyopathy.

To study biventricular systolic function and mass of the heart in young patients with residual pulmonary regurgitation who had undergone surgical correction of tetralogy of Fallot.Transverse gradient-echo magnetic resonance (MR) images covering both ventricles were obtained in 19 patients who had undergone corrective surgery for tetralogy of Fallot at the age of 1.5 years +/- 1 and in 12 age-matched control subjects. In addition, MR velocity maps of the pulmonary artery were obtained. Biventricular volumes, ejection fraction and myocardial mass, and pulmonary flow volumes were measured. Exercise tests were performed in 17 patients.The right ventricular ejection fraction was lower (P.001) and the right ventricular mass was higher (P.0005) in patients than in control subjects; the left ventricular ejection fraction was lower (P.0005) in patients and correlated statistically significantly with pulmonary regurgitation (r = -.68; P.005). Exercise performance inversely correlated with pulmonary regurgitation (tau = -0.5; P = .01).In children who undergo early surgical repair of tetralogy of Fallot, residual pulmonary regurgitation correlates with biventricular systolic dysfunction and diminished exercise capacity. Despite successful surgical correction, right ventricular hypertrophy may persist.

BACKGROUND Pulmonary regurgitation frequently occurs after surgical correction of tetralogy of Fallot. To date, reliable quantitation of pulmonary regurgitation has not been possible, and therefore the clinical significance of pulmonary regurgitation is controversial. Nuclear magnetic resonance (NMR) velocity mapping allows accurate measurement of volumetric flow. The feasibility and accuracy of NMR velocity mapping to quantify pulmonary regurgitation volumes are studied in patients after Fallot repair. METHODS AND RESULTS In 18 patients (mean age, 16.5 +/- 6.5 years), late (12.6 +/- 5.2 years) after Fallot surgery, forward and regurgitant volume flow was measured in the main pulmonary artery with NMR velocity mapping. To validate the measurements of pulmonary forward flow, right ventricular stroke volume was used as an internal reference standard. Pulmonary regurgitation volumes were compared with the differences between the corresponding right and left ventricular stroke volumes. Ventricular volumes were measured with a multisection gradient echo NMR method. In addition, the relation between pulmonary regurgitation and right ventricular volumes was studied. Measurements of pulmonary regurgitation volume with NMR velocity mapping closely corresponded with the tomographically determined volumes (r = .93). Forward pulmonary volume flow was nearly identical to right ventricular stroke volume (r = .98). Pulmonary regurgitation volume was significantly correlated with end-diastolic volume (r = .82, P < .0005), end-systolic volume (r = .63, P < .01), and stroke volume (r = .89, P < .0005) of the right ventricular but not with right ventricular ejection fraction (r = .41, P = NS). CONCLUSIONS NMR velocity mapping is an accurate method for the noninvasive, volumetric quantification of pulmonary regurgitation after surgical correction of tetralogy of Fallot.

With the increasing number of patients surviving after therapeutic intervention for congenital heart disease (CHD), accurate and frequent follow-up of their morphologic and functional cardiovascular status is required, preferably with a noninvasive imaging technique. Echocardiography, either transthoracic or transesophageal, has been the first choice for this purpose, and will probably keep that status, at least in a large segment of the CHD spectrum. Magnetic resonance imaging (MRI) is an established method for high-resolution visualization of cardiovascular morphology. In the past decade, newer MRI techniques have been developed that allow functional evaluation of CHD patients. Particularly the introduction of breath-hold imaging, contrast-enhanced MRA and user-friendly computer software for image analysis may move functional MRI of CHD from the science laboratory to clinical use. It is already evident that MRI is superior to echocardiography in certain areas of limited echocardiographic access, such as the pulmonary artery branches and the aortic arch in adult patients. But MRI has also a unique potential for accurate volumetric analysis of ventricular function and cardiovascular blood flow, without any geometric assumptions. If supported by increased cooperation between cardiologists and radiologists, MRI will grow into a useful noninvasive imaging tool that, together with echocardiography, will obviate the need for invasive catheter studies for diagnostic purposes.

A complex congenital cardiac malformation in a female patient was evaluated several times by angiocardiography and echocardiography in childhood but a definite diagnosis was not established. Segmental analysis of the heart and the great vessels by magnetic resonance imaging when the patient was 34, however, showed a double outlet left ventricle in which the aorta was situated anterior to and to the left of the pulmonary trunk and an associated subaortic ventricular septal defect with pulmonary valve stenosis. This is the first time that this extremely rare cardiac malformation has been diagnosed by magnetic resonance imaging.

To determine the feasibility and accuracy of measuring tricuspid volume flow with magnetic resonance (MR) velocity mapping in healthy children and in patients after a Mustard or Senning repair.MR studies were performed in 14 healthy children (mean age, 12 years +/- 3) and in 12 patients (mean age, 17 years +/- 5) late after a Mustard or Senning repair. MR measurements of tricuspid volume flow were validated against right ventricular stroke volumes measured tomographically. Diastolic filling parameters were derived from the flow measurements.Tricuspid volume flow and right ventricular stroke volume showed close agreement in the healthy children (r = .98) and in the patients (r = .94). Children after Senning repair, compared with healthy children, showed a delayed and higher peak tricuspid flow rate during early filling and a lower peak flow rate during atrial contraction (P.05).MR measurement of tricuspid flow is feasible and accurate in healthy children and in patients after a Mustard or Senning operation, who often demonstrate abnormal tricuspid flow patterns.