Jaap Ottenkamp

Pulmonary regurgitation in the late postoperative follow-up of tetralogy of Fallot. Volumetric quantitation by nuclear magnetic resonance velocity mapping.

BackgroundPulmonary 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 ResultsIn 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 neariy 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 ventricle but not with right ventricular ejection fraction (r= −.41, P=NS). ConclusionsNMR velocity mapping is an accurate method for the noninvasive, volumetric quantification of pulmonary regurgitation after surgical correction of tetralogy of Fallot.

ECG predictors of ventricular arrhythmias and biventricular size and wall mass in tetralogy of Fallot with pulmonary regurgitation

Background: In patients with the tetralogy of Fallot, QRS prolongation predicts malignant ventricular arrhythmias. QRS prolongation may result from right ventricular dilatation. The relation of ECG markers to biventricular wall mass and volumes has not been assessed. Objective: To investigate the relations of surface ECG markers of depolarisation and repolarisation to right and left ventricular volume and biventricular wall mass. Methods: 37 Fallot patients (mean (SD) age 17 (9) years) were studied 14 (8) years after surgical repair; 34 had important pulmonary regurgitation. Left and right ventricular size was assessed from tomographic magnetic resonance imaging (MRI), and the amount of pulmonary regurgitation by velocity mapping MRI. QT, QRS, and JT duration and interlead dispersion markers were derived from a standard 12 lead ECG. Results: Mean QRS duration was significantly prolonged (133 (31) v 91 (11) ms in controls), as were dispersion of QRS (36 (17) v 20 (6) ms), QT interval (87 (48) v 42 (20) ms), and JT interval (93 (48) v 42 (19) ms). Biventricular volumes were increased (right ventricular end diastolic volume, 129 (41) v 70 (9) ml/m2; left ventricular end diastolic volume, 83 (16) v 69 (10) ml/m2), as was right ventricular wall mass (24 (7) v 17 (2) g/m2). QRS duration correlated best with right ventricular mass (r = 0.55, p < 0.01). Conclusions: In patients operated on for tetralogy of Fallot and with pulmonary regurgitation, ECG predictors of ventricular arrhythmias are influenced by several mechanical factors that may occur simultaneously. These include increased right ventricular volume, but also increases in left ventricular volume and in right and left ventricular wall mass.

A new diagnostic approach to vascular rings and pulmonary slings: the role of MRI.

UNLABELLEDnThe conventional diagnostic work-up of a patient suspected of having a vascular cause for stridor, or dysphagia, includes esophagography and bronchoscopy to delineate the abnormal structure without imaging the structure itself. Cine-angiography is regarded as the golden standard, but is not routinely performed. Magnetic resonance imaging (MRI) is non-invasive and has the important advantage over cine-angiography of depicting all structures in the field of view. Color Doppler echocardiography depicts the great vessels, but not the esophagus and trachea. In 14 patients with obstructive symptoms and in four patients without obstructive symptoms MRI successfully imaged the abnormal structure, as was the case in two symptomatic patients using computer tomography. In this series, the findings were confirmed at surgery or by cine-angiography.nnnCONCLUSIONnwe suggest that in patients suspected of having a vascular cause for stridor or dysphagia, MRI should be performed. If there is need for a screening procedure, color Doppler echocardiography should be used and if that is equivocal or non-conclusive, esophagography and bronchoscopy should be used. If MRI is difficult to interpret, it should be augmented by magnetic resonance angiography before considering cine-angiography.

Aortic elasticity and size are associated with aortic regurgitation and left ventricular dysfunction in tetralogy of Fallot after pulmonary valve replacement

Background: Aortic wall pathology and concomitant aortic dilatation have been described in tetralogy of Fallot (TOF) patients, which may negatively affect aortic valve and left ventricular systolic function. Objective: To assess aortic dimensions, aortic elasticity, aortic valve competence and biventricular function in repaired TOF patients after pulmonary valve replacement (PVR) using magnetic resonance imaging (MRI). Methods: MRI was performed in 16 patients with TOF after PVR (10 male; mean age 31 years (SD 15)) and 16 age and gender-matched healthy subjects. Results: TOF patients showed aortic root dilatation (mean difference 7.8–8.8 mm, p<0.01 at all four predefined levels) and reduced aortic elasticity (pulse wave velocity in aortic arch 5.5 m/s (1.2) vs 4.6 m/s (0.9), pu200a=u200a0.04; aortic root distensibility 1.4/10−3 mm Hg (1.7) vs 5.7/10−3 mm Hg (3.6), p<0.01). Minor degrees of aortic regurgitation (AR) (AR fraction 6% (8) vs 1% (1), p<0.01) and reduced left ventricular ejection fraction (LVEF) were present (51% (8) vs 58% (6), pu200a=u200a0.01), whereas right ventricular ejection fraction (RVEF) was within normal limits (47% (8) vs 52% (7), pu200a=u200a0.06). The degree of AR fraction was associated with dilatation of the aortic root (r u200a=u200a 0.39–0.49, p<0.05) and reduced aortic root distensibility (r u200a=u200a 0.44, pu200a=u200a0.02), whereas reduced LVEF was correlated with degree of AR and RVEF (r u200a=u200a 0.41, pu200a=u200a0.02 and r u200a=u200a 0.49, p<0.01, respectively). Conclusions: Aortic root dilatation and reduced aortic elasticity are frequently present in patients with TOF, in addition to minor degrees of AR and reduced left ventricular systolic function. Aortic wall pathology in repaired TOF patients may therefore represent a separate mechanism leading to left ventricular dysfunction, as part of a multifactorial process of left ventricular dysfunction.

Right ventricular hypertrophy and diastolic dysfunction in arterial switch patients without pulmonary artery stenosis

Objective: To assess pulmonary flow dynamics and right ventricular (RV) function in patients without significant anatomical narrowing of the pulmonary arteries late after the arterial switch operation (ASO) by using magnetic resonance imaging (MRI). Methods: 17 patients (mean (SD), 16.5 (3.6) years after ASO) and 17 matched healthy subjects were included. MRI was used to assess flow across the pulmonary trunk, RV systolic and diastolic function, and RV mass. Results: Increased peak flow velocity (>1.5 m/s) was found across the pulmonary trunk in 14 of 17 patients. Increased RV mass was found in ASO patients: 14.9 (3.4) vs 10.0 (2.6) g/m2 in normal subjects (p<0.01). Delayed RV relaxation was found after ASO: mean tricuspid valve E/A peak flow velocity ratiou200a=u200a1.60 (0.96) vs 1.92 (0.61) in normal subjects (pu200a=u200a0.03), and E-deceleration gradientsu200a=u200a−1.69 (0.73) vs −2.66 (0.96) (p<0.01). After ASO, RV mass correlated with pulmonary trunk peak flow velocity (ru200a=u200a0.49, p<0.01) and tricuspid valve E-deceleration gradients (ru200a=u200a0.35, pu200a=u200a0.04). RV systolic function was well preserved in patients (ejection fractionu200a=u200a53 (7)% vs 52 (8)% in normal subjects, pu200a=u200a0.72). Conclusions: Increased peak flow velocity in the pulmonary trunk was often observed late after ASO, even in the absence of significant pulmonary artery stenosis. Haemodynamic consequences were RV hypertrophy and RV relaxation abnormalities as early markers of disease, while systolic RV function was well preserved.

Major coronary anomalies in childhood

Abstract. Major coronary artery anomalies are extremely rare in childhood. We wanted to assess the historical and diagnostic features and the therapeutic options of three distinct types of coronary artery anomalies: abnormal origin of the left coronary artery from the pulmonary artery (ALCAPA), coronary fistula and coronary stenosis. In a retrospective study, 33 children with these types of coronary artery disease were identified, 15 with ALCAPA, 12 with fistula and six with coronary stenosis. History, physical examination, ECG, X-ray, echocardiography, angiography, therapy and outcome were reviewed. ALCAPA showed distinct typical echocardiographic characteristics. Coronary artery fistula could be identified by a typical murmur and echocardiographic evidence of coronary dilatations. Coronary stenosis should be suspected by the clinical picture in a specific context. Conclusion: rare coronary artery anomalies can be accurately diagnosed in childhood. Timely therapy yields good prognosis.

Stenting of the vertical vein in obstructed total anomalous pulmonary venous return as rescue procedure in a neonate

A newborn girl with progressive respiratory distress and cyanosis was found to have severely obstructed supracardiac total pulmonary venous return (TAPVR). Stenting of the stenosis provided immediate and effective relief of the obstruction until corrective surgery was performed. Catheter intervention should be considered in high‐risk infants with obstructed supracardiac TAPVR as part of the strategy for preoperative cardiovascular stabilization.

The Use of Transesophageal Echocardiography Monitoring of Transcatheter Closure of a Persistent Ductus Arteriosus

We performed transesophageal echocardiography (TEE) with pediatric biplane probes throughout the procedure of transcatheter closure of the ductus arteriosus in 42 out of 46 patients who were scheduled for this intervention. Patient ages ranged from 15 months to 14 years and body weight from 9–53 kg. Both Rashkind double umbrella devices and coils were used. The described approach resulted in fewer contrast injections and was helpful in monitoring the procedure and in deciding when to release the device. To the best of our knowledge this is the first report describing a large series where continuous monitoring of transcatheter ductus closure was combined with x‐ray fluoroscopy and TEE.

On-Line Automated Border Detection for Echocardiographic Quantification of Right Ventricular Size and Function in Children

Abstract. Rapid, accurate assessment of right ventricular (RV) size is important for the management of children with congenital heart disease. The usefulness of the Acoustic Quantification system of automated border detection (ABD) and on-line quantification (AQ) for assessment of RV size was tested in 36 children. AQ data were compared to ``corrected AQ measurements (after correction for cavity areas erroneously included in the region of interest) required for AQ. Furthermore, the influence of necessary changes to gain settings was tested in ``lateral gain control (LGC) images obtained by removal of ABD overlays. All results were compared to conventional echocardiography (echo), and agreement with magnetic resonance imaging (MRI) RV areas was assessed. Systematic differences (±) limits of agreement with MRI (transverse plane) for conventional echo and AQ (apical four-chamber view) were as follows: end-diastolic −0.8 ± 3.8 (conventional echo) versus −1.7 ± 4.6 (AQ) cm2/m2 (p < 0.001); end-systolic −1.3 ± 3.2 versus −4.9 ± 5.8 (AQ) cm2/m2 (p < 0.001); fractional area change 7.8 ± 17.0% versus 26.9 ± 31.4% (AQ) (p < 0.001). Differences between conventional echo, LGC, and corrected AQ areas were not statistically significant. The best agreement between MRI and echocardiography was with conventional echo. We conclude that automated border detection of the RV can be performed successfully with the AQ system at a fixed point in the cardiac cycle. For adequate assessment of RV function manual corrections of online AQ results are still required, which results in an important reduction of the time gain of on-line quantification.n

Low agreement between cardiologists diagnosing left ventricular hypertrophy in children with end-stage renal disease

BackgroundMonitoring of the appearance of left ventricular hypertrophy (LVH) by echocardiography is currently recommended for in the management of children with End-stage renal disease (ESRD). In order to investigate the validity of this method in ESRD children, we assessed the intra- and inter-observer reproducibility of the diagnosis LVH.MethodsEchocardiographic measurements in 92 children (0–18 years) with ESRD, made by original analysists, were reassessed offline, twice, by 3 independent observers. Smallest detectable changes (SDC) were calculated for continuous measurements of diastolic interventricular septum (IVSd), Left ventricle posterior wall thickness (LVPWd), Left ventricle end-diastolic diameter (LVEDd), and Left ventricle mass index (LVMI). Cohen’s kappa was calculated to assess the reproducibility of LVH defined in two different ways. LVHWT was defined as Z-value of IVSd and/or LVPWd>2 and LVHMI was defined as LVMI> 103 g/m2 for boys and >84 g/m2 for girls.ResultsThe intra-observer SDCs ranged from 1.6 to 1.7 mm, 2.0 to 2.6 mm and 17.7 to 30.5 g/m2 for IVSd, LVPWd and LVMI, respectively. The inter-observer SDCs were 2.6 mm, 2.9 mm and 24.6 g/m2 for IVSd, LVPWd and LVMI, respectively. Depending on the observer, the prevalence of LVHWT and LVHMI ranged from 2 to 30% and from 8 to 25%, respectively. Kappas ranged from 0.4 to 1.0 and from 0.1 to 0.5, for intra-and inter- observer reproducibility, respectively.ConclusionsChanges in diastolic wall thickness of less than 1.6 mm or LVMI less than 17.7 g/m2 cannot be distinguished from measurement error in individual children, even when measured by the same observer. This limits the use of echocardiography to detect changes in wall thickness in children with ESRD in routine practice.