Arno A.W. Roest

Pulmonary valve insertion late after repair of Fallot's tetralogy

Objectives: To analyze the results of pulmonary valve insertion late after initial repair of Fallot’s tetralogy. Pulmonary insufficiency (PI) after correction of Fallot’s tetralogy is usually well tolerated in the short term, but is associated with symptomatic right ventricular dilatation and an increased risk of ventricular arrhythmias over longer periods of time. Methods: From 1993 to July 2000, 51 patients were reoperated for PI at a mean age of 25.7 ^ 11.9 years. The mean age at initial repair was 6.4 ^ 7.2 years. Patients with a conduit inserted at initial operation, with absent pulmonary valve syndrome or with a more than moderate ventricular septal defect at reoperation were excluded from the study. A cryopreserved pulmonary (96%) or aortic (4%) homograft was implanted in the orthotopic position with the use of cardiopulmonary bypass 19.3 ^ 9.1 years (2.7‐40.3 years) after initial correction. Preoperative symptoms (New York Heart Association, NYHA class), degree of PI (echo-Doppler, MRI), right ventricular dimensions (MRI) and QRS duration were compared to findings at last follow-up. Results: Follow-up is complete and had a mean duration of 1.7 ^ 1.4 years. Hospital mortality was 2%. No serious morbidity occurred. Severe PI was present preoperatively in all patients. At last follow-up echo-Doppler studies showed PI to be absent or trivial in 96% and mild in 4% of patients. In 13 patients MRI studies were performed both pre- and postoperatively: in this group PI was reduced from a mean of 48 to 4%. After 6 months NYHA capacity class had improved significantly from 2.3 ^ 0.6 to 1.4 ^ 0.5. After 1 year end-diastolic and end-systolic right ventricular volumes were reduced significantly. Right ventricular ejection fraction and QRS duration remained unchanged. Conclusions: PI late after correction of Fallot’s tetralogy may lead to serious symptomatic right ventricle dilatation. After pulmonary homograft insertion right ventricular dimensions decrease rapidly and functional improvement is observed in almost all patients. q 2001 Elsevier Science B.V. All rights reserved.

Biventricular response to supine physical exercise in young adults assessed with ultrafast magnetic resonance imaging

Simultaneous assessment of left ventricular (LV) and right ventricular (RV) response to exercise is limited with the current imaging modalities. Magnetic resonance imaging (MRI) techniques are now under development that allow near real-time evaluation of biventricular function under physical stress. This approach may open new avenues to study heart function in response to exercise in health and disease. The aim of this study was to evaluate biventricular response to supine physical exercise using ultrafast MRI. Biventricular volumes and function were examined in 16 healthy volunteers (mean age 18 +/- 2 years) using an ultrafast MRI sequence at rest and during an exercise protocol on a MRI compatible bicycle ergometer. Exercise level was individualized at the workload corresponding to 60% of the maximal oxygen uptake. All subjects completed the exercise MRI examination, allowing functional evaluation. Stroke volume of both ventricles increased from rest to exercise (left ventricle, 89 +/- 14 ml vs 102 +/- 19 ml, p < 0.05; right ventricle, 88 +/- 14 ml vs 101 +/- 16 ml, p < 0.05). Ejection fraction also increased in both ventricles from rest to exercise (left ventricle, 63 +/- 6% vs 74 +/- 6%, p < 0.05; right ventricle, 61 +/- 6% vs 70 +/- 6%, p < 0.05). End-systolic volume of the left and right ventricles decreased from rest to exercise (left ventricle, -33 +/- 12%, p < 0.05; right ventricle, -25 +/- 12%, p < 0.05), whereas LV and RV end-diastolic volumes remained unchanged. The results fit well with current concepts of cardiac physiology, and therefore we conclude that ergometer-induced exercise MRI is a valid approach to assess physiologic changes in LV and RV function simultaneously.

Evaluation of congenital heart disease by magnetic resonance imaging

Abstract. Magnetic resonance imaging has proven to be useful in the assessment of patients with complex congenital heart disease and in the post-surgical follow-up of patients with corrected congenital heart disease. A thorough understanding of the congenital cardiac malformations that can be encountered is needed and the use of the sequential segmental analysis helps to standardize the evaluation and diagnosis of (complex) congenital heart disease. After surgical correction of congenital heart defects, patients must be followed over extended periods of time, because morphological and functional abnormalities may still be present or may develop. The use of echocardiography may be hampered in these patients as scar tissue and thorax deformities limit the acoustic window. Magnetic resonance imaging has proven to be advantageous in the follow-up of these post-surgical patients and with the use of several different techniques the morphological as well as functional abnormalities can be evaluated and followed over time.

Relation of Left Ventricular Twist and Global Strain with Right Ventricular Dysfunction in Patients After Operative “Correction” of Tetralogy of Fallot

In patients with corrected tetralogy of Fallot (cToF), left ventricular (LV) dysfunction is closely related to right ventricular (RV) dysfunction, indicating adverse ventricular-ventricular interactions. However, the mechanism that links RV dysfunction to LV dysfunction remains unclear. In this prospective study, 32 patients with cToF and 19 controls were enrolled. With cardiac magnetic resonance imaging, biventricular ejection fractions were assessed. Using 2-dimensional speckle tracking, global and regional RV and LV strains and LV twist were assessed. To detect and characterize ventricular-ventricular interaction, the relation between global and regional RV mechanics and global and regional LV mechanics was assessed. Global RV strain, global LV strain, and LV twist were decreased in patients with cToF. Global RV strain correlated with global LV strain (r = 0.66, p <0.001) and LV twist (r = -0.72, p <0.001), indicating the presence of adverse ventricular-ventricular interaction. Furthermore, close relations were observed between apical RV strain and apical LV strain (r = 0.62, p <0.001) and apical LV rotation (r = -0.67, p <0.001). In conclusion, RV strain was significantly related to LV strain and LV twist in patients with cToF and controls. Furthermore, apical RV strain correlated with apical LV strain and apical LV rotation, indicating adverse apical ventricular-ventricular interactions.

Vortex flow during early and late left ventricular filling in normal subjects: Quantitative characterization using retrospectively-gated 4D flow cardiovascular magnetic resonance and three-dimensional vortex core analysis

BackgroundLV diastolic vortex formation has been suggested to critically contribute to efficient blood pumping function, while altered vortex formation has been associated with LV pathologies. Therefore, quantitative characterization of vortex flow might provide a novel objective tool for evaluating LV function. The objectives of this study were 1) assess feasibility of vortex flow analysis during both early and late diastolic filling in vivo in normal subjects using 4D Flow cardiovascular magnetic resonance (CMR) with retrospective cardiac gating and 3D vortex core analysis 2) establish normal quantitative parameters characterizing 3D LV vortex flow during both early and late ventricular filling in normal subjects.MethodsWith full ethical approval, twenty-four healthy volunteers (mean age: 20±10 years) underwent whole-heart 4D Flow CMR. The Lambda2-method was used to extract 3D LV vortex ring cores from the blood flow velocity field during early (E) and late (A) diastolic filling. The 3D location of the center of vortex ring core was characterized using cylindrical cardiac coordinates (Circumferential, Longitudinal (L), Radial (R)). Comparison between E and A filling was done with a paired T-test. The orientation of the vortex ring core was measured and the ring shape was quantified by the circularity index (CI). Finally, the Spearman’s correlation between the shapes of mitral inflow pattern and formed vortex ring cores was tested.ResultsDistinct E- and A-vortex ring cores were observed with centers of A-vortex rings significantly closer to the mitral valve annulus (E-vortex L=0.19±0.04 versus A-vortex L=0.15±0.05; p=0.0001), closer to the ventricle’s long-axis (E-vortex: R=0.27±0.07, A-vortex: R=0.20±0.09, p=0.048) and more elliptical in shape (E-vortex: CI=0.79±0.09, A-vortex: CI=0.57±0.06; <0.001) compared to E-vortex. The circumferential location and orientation relative to LV long-axis for both E- and A-vortex ring cores were similar. Good to strong correlation was found between vortex shape and mitral inflow shape through both the annulus (r=0.66) and leaflet tips (r=0.83).ConclusionsQuantitative characterization and comparison of 3D vortex rings in LV inflow during both early and late diastolic phases is feasible in normal subjects using retrospectively-gated 4D Flow CMR, with distinct differences between early and late diastolic vortex rings.

Pulse Oximetry Measures a Lower Heart Rate at Birth Compared with Electrocardiography

OBJECTIVE To examine the effect of time after birth on heart rate (HR) measured by pulse oximetry (PO) (HRPO) and electrocardiography (ECG) (HRECG). STUDY DESIGN HRECG and HRPO (collected at maximum sensitivity) were assessed in 53 term and preterm infants at birth. ECG electrodes and a PO sensor were attached as soon as possible and HRECG and HRPO were compared every 30 seconds from 1-10 minutes after birth. Data were compared using a Wilkinson signed-rank test. Clinical relevance (eg, HR <100 beats per minute [bpm] was tested using a McNemar test). RESULTS Seven hundred fifty-five data pairs were analyzed. Median (IQR) gestational age was 37 (31-39) weeks. Mean (SD) starting time of PO and ECG data collection was 99 (33) vs 82 (26) seconds after birth (P = .001). In the first 2 minutes after birth, HRPO was significantly lower compared with HRECG (94 (67-144) vs 150 (91-153) bpm at 60 seconds (P < .05), 81 (60-109) vs 148 (83-170) bpm at 90 seconds (P < .001) and 83 (67-145) vs 158 (119-176) at 120 seconds (P < .001). A HR <100 bpm was more frequently observed with a PO than ECG in the first 2 minutes (64% vs 27% at 60 seconds (P = .05), 56% vs 26% at 90 seconds (P < .05) and 53% vs 21% at 120 seconds (P < .05). HR by ECG was verified by ultrasound for outflow from a subset of infants. CONCLUSIONS In infants at birth, HRPO is significantly lower compared with ECG with clinically important differences in the first minutes.

Cardiac resynchronization therapy in paediatric and congenital heart disease patients

The number of patients with congenital heart disease (CHD) has significantly increased over the last decades. The CHD population has a high prevalence of heart failure during late follow-up and this is a major cause of mortality. Cardiac resynchronization therapy (CRT) may be a promising therapy to improve the clinical outcome of CHD and paediatric patients with heart failure. However, the CHD and paediatric population is a highly heterogeneous group with different anatomical substrates that may influence the effects of CRT. Echocardiography is the mainstay imaging modality to evaluate CHD and paediatric patients with heart failure and novel echocardiographic tools permit a comprehensive assessment of cardiac dyssynchrony that may help selecting candidates for CRT. This article reviews the role of CRT in the CHD and paediatric population with heart failure. The current inclusion criteria for CRT as well as the outcomes of different anatomical subgroups are evaluated. Finally, echocardiographic assessment of mechanical dyssynchrony in the CHD and paediatric population and its role in predicting response to CRT is comprehensively discussed.

Tetralogy of Fallot: 3D Velocity-encoded MR Imaging for Evaluation of Right Ventricular Valve Flow and Diastolic Function in Patients after Correction

PURPOSE To evaluate three-dimensional (3D) velocity-encoded (VE) magnetic resonance (MR) imaging, as compared with two-dimensional (2D) VE MR imaging, for assessment of pulmonary valve (PV) and tricuspid valve (TV) flow, with planimetry as the reference standard, and to evaluate diastolic function in patients with a corrected tetralogy of Fallot (TOF). MATERIALS AND METHODS Local institutional review board approval was obtained, and patients or their parents gave informed consent. Twenty-five patients with a corrected TOF (12 male, 13 female; mean age, 13.1 years +/- 2.7 [standard deviation]; age range, 8-18 years) and 19 control subjects (12 male, seven female; mean age, 14.1 years +/- 2.4; age range, 8-18 years) underwent planimetric MR imaging, 2D VE MR imaging, and 3D VE MR imaging for TV and PV flow evaluation. For evaluation of diastolic function, PV and TV flow were summated. Data were analyzed by using linear regression analysis, paired and unpaired t testing, and Bland-Altman plots. RESULTS Strong correlations between the 2D VE MR and 3D VE MR measurements of PV flow (for forward flow: r = 0.87, P < .01; for backward flow: r = 0.97, P < .01) were observed. With PV effective flow as a reference, 3D TV effective flow measurements were more accurate than 2D TV effective flow measurements: In patients, the mean 2D TV effective flow versus 2D PV effective flow difference was 17.6 mL +/- 11 (P < .001), and the mean 3D TV effective flow versus 3D PV effective flow difference was -1.2 mL +/- 4.7 (P = .22). Diastolic functional impairment in patients could be detected at 3D VE MR imaging diastolic assessment. CONCLUSION Three-dimensional VE MR imaging is accurate for PV flow assessment and is more accurate than 2D VE MR imaging for TV flow evaluation. Assessment of diastolic function with 3D VE MR imaging can facilitate ongoing research of diastolic dysfunction in patients with a corrected TOF.

Noninvasive measurements of hemodynamic transition directly after birth

Background:Cardiac output depends on stroke volume and heart rate (HR). Only HR is used to monitor hemodynamic transition.Methods:In 24 term newborns born via cesarean section, HR and preductal blood pressure (BP) were measured. Also, using echocardiography, left ventricular dimensions and (Doppler derived) left ventricular output (LVO) were examined at 2, 5, and 10 min after birth.Results:Mean (SD) HR and BP did not change with time (mean HR: 157 (21) bpm at 2 min, 154 (17) bpm at 5 min, and 155 (14) bpm at 10 min; mean BP: 51.2 (15.4) mm Hg at 2 min, 50.5 (11.7) mm Hg at 5 min, and 49.6 (9.5) mm Hg at 10 min). Left ventricular end-diastolic diameter increased from 2 to 5 min (14.3 (1.3) vs. 16.3 (1.7) mm; P < 0.001) and stabilized at 10 min (16.7 (1.4) mm). LVO increased between 2 and 5 min (151 (47) vs. 203 (55) ml/kg/min; P < 0.001) and stabilized at 10 min (201 (45) ml/kg/min). LVO increase was associated with rise in left ventricular stroke volume (r = 0.94; P < 0.001), not with rise in HR (r = 0.37; P value not significant).Conclusion:Left ventricular dimensions and LVO significantly increased the first 5 min after birth and stabilized at 10 min, whereas BP remained stable. LVO and left ventricular dimension increase are presumably due to increasing left ventricular preload resulting from pulmonary blood flow and ductal shunting increase.

Umbilical blood flow patterns directly after birth before delayed cord clamping

Background Delayed umbilical cord clamping (DCC) affects the cardiopulmonary transition and blood volume in neonates immediately after birth. However, little is known of blood flow in the umbilical vessels immediately after birth during DCC. The objective is to describe the duration and patterns of blood flow through the umbilical vessels during DCC. Methods Arterial and venous umbilical blood flow was measured during DCC using Doppler ultrasound in uncomplicated term vaginal deliveries. Immediately after birth, the probe was placed in the middle of the umbilical cord, pattern and duration of flow in vein and arteries were evaluated until cord clamping. Results Thirty infants were studied. Venous flow: In 10% no flow was present, in 57% flow stopped at 4:34 (3:03–7:31) (median (IQR) min:sec) after birth, before the cord was clamped. In 33%, flow continued until cord clamping at 5:13 (2:56–9:15) min:sec. Initially, venous flow was intermittent, increasing markedly during large breaths or stopping and reversing during crying, but then became continuous. Arterial flow: In 17% no flow was present, in 40% flow stopped at 4:22 (2:29–7:17) min:sec, while cord pulsations were still palpable. In 43% flow continued until the cord was clamped at 5:16 (3:32–10:10) min:sec. Arterial flow was pulsatile, unidirectional towards placenta or bidirectional to/from placenta. In 40% flow became continuous towards placenta later on. Conclusions During delayed umbilical cord clamping, venous and arterial umbilical flow occurs for longer than previously described. Net placental transfusion is probably the result of several factors of which breathing could play a major role. Umbilical flow is unrelated to cessation of pulsations.