We sought to assess left ventricular regional function in patients with and without left ventricular wall scar tissue in the long term after repair of an anomalous origin of the left coronary artery from the pulmonary artery.

A total of 20 patients aged 12.8±7.4 years were assessed 10 (0.5–17) years after the repair of an anomalous origin of the left coronary artery from the pulmonary artery; of them, 10 (50%) patients showed left ventricular wall scar tissue on current cardiac MRI. Left ventricular regional function was assessed by two-dimensional speckle-tracking echocardiography in 10 patients with scar tissue and 10 patients without scar tissue and in 10 age-matched controls.

In patients with scar tissue, MRI-derived left ventricular ejection fraction was significantly reduced compared with that in patients without scar tissue (51 versus 61%, p<0.05), and echocardiography-derived longitudinal strain was significantly reduced in five of six left ventricular areas compared with that in healthy controls (average relative reduction, 46%; p<0.05). In patients without scar tissue, longitudinal strain was significantly reduced in two of six left ventricular areas (average relative reduction, 23%; p<0.05) and circumferential strain was reduced in one of six left ventricular areas (relative reduction, 56%; p<0.05) compared with that in healthy controls.

Regional left ventricular function is reduced even in patients without left ventricular wall scar tissue late after successful repair of an anomalous origin of the left coronary artery from the pulmonary artery. This highlights the need for meticulous lifelong follow-up in all patients with a repaired anomalous origin of the left coronary artery from the pulmonary artery.

In patients with CHD, cardiac MRI is often indicated for functional and anatomical assessment. With the recent introduction of MRI-conditional pacemaker systems, cardiac MRI has become accessible for patients with pacemakers. The present clinical study aims to evaluate safety, susceptibility artefacts, and image reading of cardiac MRI in patients with CHD and MRI-conditional pacemaker systems.

CHD patients with MRI-conditional pacemaker systems and a clinical need for cardiac MRI were examined with a 1.5-T MRI system. Lead function was tested before and after MRI. Artefacts and image readings were evaluated using a four-point grading scale.

A total of nine patients with CHD (mean age 34.0 years, range 19.5–53.6 years) received a total of 11 cardiac MRI examinations. Owing to clinical indications, seven patients had previously been converted from conventional to MRI-conditional pacemaker systems. All MRI examinations were completed without adverse effects. Device testing immediately after MRI and at follow-up showed no alteration of pacemaker device and lead function. Clinical questions could be addressed and answered in all patients.

Cardiac MRI can be performed safely with high certainty of diagnosis in CHD patients with MRI-conditional pacemaker systems. In case of clinically indicated lead and box changing, CHD patients with non-MRI-conditional pacemaker systems should be considered for complete conversion to MRI-conditional systems.

Flow profiles are important determinants of fluid–vessel wall interactions. The aim of this study was to assess blood flow profiles in the aorta and pulmonary trunk in patients with transposition and different ventriculoarterial connection, and hence different mechanics of the coherent pump.

In all, 29 patients with operated transposition – concordant atrioventricular and discordant ventriculoarterial connection, and no other cardiac malformation – and eight healthy volunteers were assessed with cardiac magnetic resonance imaging: n = 17 patients after atrial redirection, with a morphologic right ventricle acting as systemic pump and a morphologic left ventricle connected to the pulmonary trunk, and n = 12 patients after the arterial switch procedure, with physiologic ventriculoarterial connections. Flow-sensitive four-dimensional velocity-encoded magnetic resonance imaging was used to analyse systolic flow patterns in the aorta and pulmonary trunk, relating to helical flow and vortex formation.

In the aorta, overall helicity was present in healthy volunteers, but it was absent in all patients independent on the operation technique. Partial helices were observed in the ascending aorta of 58% of patients after arterial switch. In the pulmonary trunk, mostly parallel flow was seen in healthy volunteers and in patients after arterial switch, whereas vortex formation was present in 88% of patients after atrial redirection.

Blood flow patterns differ substantially between the groups. In addition to varying mechanics of the coherent pumping ventricles, the absent overall helicity in all patients might be explained by the missing looping of the aorta in transposition.

To assess if flow-sensitive four-dimensional velocity-encoded cine magnetic resonance imaging adds value in diagnosing patients with suspected partial anomalous pulmonary venous drainage.

In six patients with echocardiographically suspected partial anomalous pulmonary venous drainage, anatomy was evaluated using standard magnetic resonance imaging including angiography. Functional analysis included shunt calculations from flow measurements. We used four-dimensional velocity-encoded cine magnetic resonance imaging for visualisation of maldraining pulmonary veins and quantification of flow via the maldraining veins and interatrial communications, if present.

In all patients, the diagnosis of partial anomalous pulmonary venous drainage was confirmed by standard magnetic resonance imaging. Shunt volumes ranged from 1.4:1 to 4.7:1. Drainage sites were the superior caval vein (n = 5) or the vertical vein (n = 1). Multiple maldraining pulmonary veins were found in three patients. Pulmonary arteries and veins could be clearly distinguished by selective visualisation using four-dimensional velocity-encoded cine magnetic resonance imaging. Flow measured individually in maldraining pulmonary veins in six patients and across the interatrial communication in three patients revealed a percentage of the overall shunt volume of 30–100% and 58–70%, respectively.

Selective visualisation of individual vessels and their flow characteristics by four-dimensional velocity-encoded cine magnetic resonance imaging facilitates in distinguishing adjacent pulmonary arteries and veins and thus improves the accurate diagnosis of maldraining pulmonary veins. By detailed quantification of shunt volumes, additional information for planning of treatment strategies is provided. This method adds clinical value and might replace contrast-enhanced magnetic resonance angiography in these patients in the future.

In this study we sought to determine, first, whether maximal exercise capacity reflects ventricular function, and second, whether the age of the patient, and the age of completion of the Fontan circulation, influence ventricular function and exercise performance.

Cardiac magnetic resonance imaging and cardiopulmonary exercise testing were performed in 29 patients at a median time of 6.9 years after completion of the Fontan circulation. We divided the patients into 2 groups, the first 19 having their operation below the age of 18 years, and the second group, of 10 patients, having completion of the Fontan circulation when they were older than 18 years. Parameters for ventricular function and exercise were compared for both groups with controls.

Compared to controls, the younger patients had normal end-diastolic ventricular volumes, but significantly impaired ventricular function, lower maximal work load and consumption of oxygen. The older patients had greater end-diastolic ventricular volumes, and significantly poorer ventricular function than both the younger patients and the controls. Maximal work load and consumption of oxygen were significantly lower in the older patients than in the younger ones and the controls.

Patients with the Fontan circulation have an impaired systolic ventricular function, which correlates with maximal exercise capacity and uptake of oxygen. Those having completion of the Fontan circulation when younger than 18 years had significantly better ventricular function and exercise performance than those who had completion of the Fontan circulation at an older age. An early creation of the Fontan circulation may preserve cardiac function and exercise capacity.

Our purpose was to evaluate the effect of a treatment over six months with bisoprolol on the surrogate parameters of N-Terminal-pro brain natriuretic peptide, subsequently to be described as brain natriuretic peptide, peak uptake of oxygen, and ventricular function assessed by magnetic resonance imaging in grown ups and adults who had undergone surgical correction of tetralogy of Fallot.

We designed a prospective, randomized, double-blind, placebo controlled trial. We enrolled 33 patients, aged 30.9 plus or minus 9.5 years in either class 1 or 2 of the grading of the New York Heart Association class with both levels of brain natriuretic peptide greater than 100 pg/ml and a reduced peak uptake of oxygen less than 25 ml/kg/min. During treatment with Bisoprolol, the levels of brain natriuretic peptide increased significantly from 206 plus or minus 95 to 341 plus or minus 250 pg/ml (p< 0.05), and those of atrial natriuretic peptide from 4117 plus or minus 1837 to 5340 plus or minus 2102 fmol/ml (p = 0.0005). These measures remained unchanged in the group of patients receiving the placebo. Peak uptake of oxygen did not differ significantly in either group, nor did treatment have any significant effect on right and left ventricular volumes and ejection fractions as determined by magnetic resonance imaging. The clinical state as judged within the grading system of the New York Heart Association was also unchanged by beta-blockade.

Beta blockade with Bisoprolol seems to have no beneficial effect on asymptomatic or mildly symptomatic patients with right ventricular dysfunction secondary to repaired tetralogy of Fallot with residual pulmonary regurgitation and/or stenosis.