-
[show abstract]
[hide abstract]
ABSTRACT: We determined the relationship between aortic arch anatomy in tetralogy of Fallot with pulmonary stenosis and chromosomal or genetic abnormality, by performing analysis of 257 consecutive patients undergoing surgical repair from January, 2003 to March, 2011. Chromosomal or genetic abnormality was identified in 49 of the 257 (19%) patients. These included trisomy 21 (n = 14); chromosome 22q11.2 deletion (n = 16); other chromosomal abnormalities (n = 9); CHARGE (n = 2); Pierre Robin (n = 2); and Kabuki, Alagille, Holt-Oram, Kaufman McKusick, Goldenhar, and PHACE (n = 1 each). Aortic anatomy was classified as left arch with normal branching, right arch with mirror image branching, left arch with aberrant right subclavian artery, or right arch with aberrant left subclavian artery. Associated syndromes occurred in 33 of 203 (16%) patients with left arch and normal branching (odds ratio 1); three of 36 (8%) patients with right arch and mirror image branching (odds ratio 0.4, 95% confidence interval 0.1-1.6); seven of eight (88%) patients with left arch and aberrant right subclavian artery (odds ratio 36, 95% confidence interval 4-302); and six of 10 (60%) patients with right arch and aberrant left subclavian artery (odds ratio 8, 95% confidence interval 2-26). Syndromes were present in 13 of 18 (72%) patients with either right or left aberrant subclavian artery (odds ratio 15, 95% confidence interval 4-45). Syndromes in patients with an aberrant subclavian artery included trisomy 21 (n = 4); chromosome 22q11.2 deletion (n = 5); and Holt-Oram, PHACE, CHARGE, and chromosome 18p deletion (n = 1 each). Aberrant right or left subclavian artery in tetralogy of Fallot with pulmonary stenosis is associated with an increased incidence of chromosomal or genetic abnormality, whereas right aortic arch with mirror image branching is not. The assessment of aortic arch anatomy at prenatal diagnosis can assist counselling.
Cardiology in the Young 06/2013; · 0.76 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: In most newborns with left heart obstruction, the choice between a single-ventricle or biventricular management pathway is clear. However, in some neonates with a "borderline" left ventricle, this decision is difficult. Existing criteria do not reliably identify neonates who will have a good long-term outlook after biventricular repair (BVR). The objective of this study was prospective assessment of the outcome after BVR for newborns in whom the left ventricle (LV) was considered "borderline" by an expert group. This study was a prospective follow-up evaluation of neonates with obstructive left heart disease related to a "borderline" LV who underwent biventricular management between January 2005 and April 2011. Of 154 neonates who required intervention for left heart obstruction, 13 (7.8 %) met the echocardiographic (echo) inclusion criteria. At the first and last echo, the z-scores were respectively -1.76 ± 1.37 and -0.66 ± 1.47 (p = 0.013) for the mitral valve, -1.02 ± 1.57 and -0.23 ± 1.78 (p = 0.056) for the aortic valve, and 13.77 ± 5.8 and 20.85 ± 8.9 ml/m2 (p = 0.006) for the LV end-diastolic volume. At this writing, all 12 survivors are clinically well. However, LV diastolic dysfunction and pulmonary artery hypertension was present in 5 (36 %) of 12 patients. Endocardial fibroelastosis (EFE) was detected in five patients at the last follow-up echo, but only in two patients preoperatively. Cardiac magnetic resonance imaging did not confirm EFE in any of assessed patients. The study authors could not reliably predict the outcome after BVR for neonates with left heart obstruction and a "borderline" LV. The presence of EFE with consequent diastolic dysfunction is more important than LV volume in determining the outcome. Prospective identification of EFE remains challenging.
Pediatric Cardiology 03/2013; · 1.30 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: BACKGROUND: Patients who underwent surgery for aortic coarctation (COA) have an increased risk of arterial hypertension. We aimed at evaluating (1) differences between hypertensive and non-hypertensive patients and (2) the value of cardiopulmonary exercise testing (CPET) to predict the development or progression of hypertension. METHODS: Between 1999 and 2010, CPET was performed in 223 COA-patients of whom 122 had resting blood pressures of <140/90mmHg without medication, and 101 were considered hypertensive. Comparative statistics were performed. Cox regression analysis was used to assess the relation between demographic, clinical and exercise variables and the development/progression of hypertension. RESULTS: At baseline, hypertensive patients were older (p=0.007), were more often male (p=0.004) and had repair at later age (p=0.008) when compared to normotensive patients. After 3.6±1.2years, 29/120 (25%) normotensive patients developed hypertension. In normotensives, VE/VCO2-slope (p=0.0016) and peak systolic blood pressure (SBP; p=0.049) were significantly related to the development of hypertension during follow-up. Cut-off points related to higher risk for hypertension, based on best sensitivity and specificity, were defined as VE/VCO2-slope≥27 and peak SBP≥220mmHg. In the hypertensive group, antihypertensive medication was started/extended in 48/101 (48%) patients. Only age was associated with the need to start/extend antihypertensive therapy in this group (p=0.042). CONCLUSIONS: Higher VE/VCO2-slope and higher peak SBP are risk factors for the development of hypertension in adults with COA. Cardiopulmonary exercise testing may guide clinical decision making regarding close blood pressure control and preventive lifestyle recommendations.
International journal of cardiology 02/2013; · 7.08 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: OBJECTIVE: To evaluate the aortic arch elastic properties and ventriculoarterial coupling efficiency in patients with single ventricle physiology, with and without a surgically reconstructed arch. METHODS: We studied 21 children with single ventricle physiology after bidirectional superior cavopulmonary surgery: 10 with hypoplastic left heart syndrome, who underwent surgical arch reconstruction, and 11 with other types of single ventricle physiology but without arch reconstruction. All children underwent pre-Fontan magnetic resonance imaging. No patient exhibited aortic recoarctation. Data on aortic wave speed, aortic distensibility and wave intensity profiles were all extracted from the magnetic resonance imaging studies using an in-house-written plug-in for the Digital Imaging and Communications in Medicine viewer OsiriX. RESULTS: Children with hypoplastic left heart syndrome had significantly greater wave speed (P = .002), and both stiffer (P = .004) and larger (P < .0001) ascending aortas than the patients with a nonreconstructed arch. Aortic distensibility was not influenced by ventricular stroke volume but depended on a combination of increased aortic diameter and abnormal wall mechanical properties. Those with hypoplastic left heart syndrome had a lower peak wave intensity and reduced energy carried by the forward compression and the forward expansion waves, even after correction for stroke volume, suggesting an abnormal systolic and diastolic function. Lower wave energy was associated with an increased aortic diameter. CONCLUSIONS: Using a novel, noninvasive technique based on image analysis, we have demonstrated that aortic arch reconstruction in children with hypoplastic left heart syndrome is associated with reduced aortic distensibility and unfavorable ventricular-vascular coupling compared with those with single ventricle physiology without aortic arch reconstruction.
The Journal of thoracic and cardiovascular surgery 09/2012; · 3.41 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: The timing and indicators for surgical pulmonary valve replacement (PVR) in patients with pulmonary regurgitation (PR) after repair of tetralogy of Fallot (ToF) are controversial. In this study we tested the hypothesis that delaying PVR in patients with ToF and severe PR would lead to short-term progressive deterioration in right ventricular/left ventricular (RV/LV) dimensions or function. We compared PVR-treated patients with matched untreated patients who were eligible for PVR based on hemodynamic status.
A current cohort of 87 patients with ToF and free PR serial cardiovascular magnetic resonance (CMR) assessments at a median interval of 1.8 years (interquartile range [IQR], 1.4-2.1) were identified. During this interval, 51 patients had surgical PVR and 36 patients were managed conservatively. Twenty-five patients from each group were matched for comparison using propensity score matching (PSM). RV and LV measurements were assessed by CMR at rest at follow-up.
There was no significant deterioration in RV or LV measurements in the matched untreated patients over a median of 1.8 years. "Normalization" of right ventricular end-diastolic volume (RVEDV) and end systolic volume (ESV) after PVR occurred in the majority of patients during the study period, and no absolute ceiling beyond which the right ventricle did not normalize could be discerned. In a group of treated patients who were not matchable because of severe baseline characteristics, there was a significant improvement in resting cardiac output (CO) after PVR (from 2.9 to 3.3 L/min/m(2); p = 0.001).
Our data indicate that patients with intermediate RV dilatation and severe PR are at low risk for significant progression in the short term, which can guide the interval for CMR imaging and advise the timing for future PVR.
The Annals of thoracic surgery 09/2012; 94(5):1619-26. · 3.74 Impact Factor
-
Philipp Lurz,
Vivek Muthurangu,
Pia K Schuler,
Alessandro Giardini,
Silvia Schievano,
Johannes Nordmeyer, Sachin Khambadkone,
Claudio Cappeli,
Graham Derrick,
Philipp Bonhoeffer,
Andrew M Taylor
[show abstract]
[hide abstract]
ABSTRACT: Aims To assess the impact of relief of pulmonary stenosis (PS) and pulmonary regurgitation (PR) by percutaneous pulmonary valve implantation (PPVI) on biventricular function during exercise stress. Methods and results Seventeen patients, who underwent PPVI for PS or PR, were included. Magnetic resonance imaging was performed at rest and during supine exercise stress pre- and within 1-month post-PPVI, using a radial k - t SENSE real-time sequence. In patients with PS (n = 9), there was no reserve in right ventricular (RV) ejection fraction (EF) in response to exercise prior to PPVI (48.2 ± 12.1% at rest vs. 48.4 ± 14.8% during exercise, P = 0.87). Post-PPVI, reserve in RVEF in response to exercise was re-established (53.4 ± 15.0% at rest vs. 59.6 ± 17.3% during exercise, P = 0.003) with improvement in left ventricular stroke volume (LVSV) (45.4 ± 6.2 mL/m(2) at rest vs. 52.8 ± 8.8 mL/m(2) during exercise, P = 0.001). In patients with PR prior to PPVI (n = 8), LVSV during exercise increased (43.0 ± 8.5 vs. 54.3 ± 6.6 mL/m(2), P < 0.001) due to reduction in PR fraction during exercise (29.2 ± 5.2 vs. 13.6 ± 6.1%, P < 0.001). After PPVI, LVSV increased from rest to exercise (48.4 ± 8.8 vs. 57.2 ± 8.1 mL/m(2), P < 0.001) due to improved RVEF (45.5 ± 8.3 vs. 50.4 ± 6.9%, P = 0.001). There was a significantly higher increase in LVSV at exercise from pre- to post-PPVI in PS patients than in PR patients (ΔLVSV 8.2 ± 4.1 vs. Δ2.9 ± 4.1 mL/m(2), P = 0.01). The reduction in the RV outflow tract gradient correlated significantly with the improvement in LVSV during exercise (r = -0.73, P < 0.001). Conclusion Percutaneous pulmonary valve implantation in patients with PS leads to restoration of reserve in RVEF during exercise stress. In patients with PR, SV augmentation improves only mildly post-PPVI. Improvement in SV augmentation during exercise stress after PPVI is dependent mainly on afterload reduction.
European Heart Journal 07/2012; 33(19):2434-41. · 10.48 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: Systemic to pulmonary collaterals (SPCs) represent an additional and unpredictable source of pulmonary blood flow in patients with single ventricle physiology following bidirectional superior cavopulmonary connection (BCPC). Understanding their influence on patient outcomes has been hampered by uncertainty about the optimal method of quantifying SPC flow.
To quantify SPC flow by cardiac magnetic resonance (CMR) prior to total cavopulmonary connection (TCPC) in order to identify preoperative risk factors and determine influence on postoperative outcomes.
Single centre prospective cohort study.
Tertiary referral centre.
65 patients with single ventricle physiology undergoing CMR for preoperative assessment of TCPC completion underwent quantification of SPC flow. Clinical outcomes of 41 patients in whom TCPC was completed were obtained.
Early post-TCPC clinical outcomes associated with SPC flow were assessed, including postoperative chest drainage volume, postoperative chest drainage duration and length of intensive care and hospital stays. Additionally preoperative covariates associated with SPC flow were assessed including age at BCPC and CMR, SpO(2) at BCPC and CMR, ventricle type, pulmonary artery (PA) cross-sectional area and PA pulsatility. Different methods of CMR SPC flow quantification were compared.
Higher SPC flow was associated with increased postoperative chest drain volume (r=0.51, p=0.001), chest drain duration (r=0.43, p=0.005), and intensive care unit (r=0.32, p=0.04) and log-transformed hospital stays (r=0.31, p=0.048). The effect of SPC flow on outcome was independent of fenestration, ventricle type and function. Preoperative covariates associated with SPC flow included age at BCPC (β=-0.34, p=0.008), SpO(2) at time of CMR (β=0.34, p=0.004) and branch PA cross-sectional area (β=-0.26, p=0.036), model R(2)=0.34. Moreover, patients with pulsatile pulmonary blood flow had lower SPC flow than those without (0.8 vs 1.3 l/min/m(2) p=0.012). SPC flow calculated by the difference between pulmonary venous return and pulmonary artery flow (l/min/m(2)) showed greatest association with preoperative covariates and strongest correlation with postoperative outcomes compared with other methods of quantification.
CMR can provide an effective measurement of SPC flow prior to TCPC. Young age at BCPC, high preoperative oxygen saturation and smaller PAs are associated with increased SPC flow, which may promote increased postoperative pleural drainage and lengthen recovery.
Heart (British Cardiac Society) 06/2012; 98(12):934-40. · 4.22 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: We aimed to assess the impact of surgical pulmonary valve replacement (PVR) for severe pulmonary regurgitation (PR) on biventricular function and its effect on exercise capacity.
Seventy-three patients (mean age 23.6±11.5 years, 47 females) underwent surgical PVR for PR. Echocardiogram and magnetic resonance imaging to assess ventricular size and function, and a cardiopulmonary exercise test were performed before, and 1-year post-surgery. Median New York Heart Association class improved from 2 to 1 but peak oxygen uptake (VO2) did not change. Left ventricular (LV) cardiac output increased from 3.2±0.9 to 3.5±0.7 L/min (P=0.003). However, this was not associated with increased trans-mitral velocities (▵E=-0.13, P=0.004; ▵A=0.03, P=0.395), or increased heart rate (-0.002%, P=0.993). Trans-tricuspid rapid right ventricular (RV) filling increased significantly, whereas early diastolic myocardial velocity in RV wall decreased (E velocity: 0.57±0.14-0.65±0.21, P=0.034; and E/e' from 6.7±1.9 to 14.8±7.0, P<0.0001). RV and LV late diastolic velocities and their ratio to early velocities (A, a', E/A, and e'/a') correlated with pre- and/or post-PVR peak VO2. No correlations were found between indexes of systolic function and peak VO2, either before or after surgery. Doppler evidence of restrictive RV physiology resolved after elimination of PR.
Surgical PVR for PR improves RV filling and increases left ventricular stroke volume, however, this could not be demonstrated by conventional Doppler echocardiography. Diastolic ventricular function was associated with exercise capacity. Because of its load dependency, E/e' ratio failed in assessing diastolic function. Pre-systolic flow in pulmonary trunk in presence of severe PR does not determine intrinsic myocardial stiffness.
European heart journal cardiovascular Imaging. 02/2012; 13(8):697-702.
-
[show abstract]
[hide abstract]
ABSTRACT: To assess the cardiac catheterisation findings of all children in whom cardiac magnetic resonance imaging found great artery stenosis.
We conducted a retrospective analysis of all 45 consecutive children with congenital cardiac disease who were undergoing cardiac catheterisation for intervention on cardiac magnetic resonance-defined great vessel stenosis, between January, 2006 and August, 2008.
Following cardiac magnetic resonance, 60 significant great vessel stenoses were identified and referred to cardiac catheterisation for intervention. All patients were catheterised within a median and interquartile range of 84 and 4-149 days, respectively, of cardiac magnetic resonance. At cardiac catheterisation, the children were aged 11.5 years - with an interquartile range of 3.8-16.9 years - and weighed 34 kilograms - with an interquartile range of 15-56 kilograms. Comparing cardiac magnetic resonance and cardiac catheterisation findings, 53 (88%) findings were concordant and seven were discordant. In six of seven (86%) discordant observations, cardiac magnetic resonance defined moderate-severe great vessel stenosis - involving three branch pulmonary arteries and three aortas. This was not confirmed by cardiac catheterisation, which revealed mild stenoses and haemodynamic gradients insufficient for intervention. In one patient, a mild, proximal right pulmonary artery narrowing was found at cardiac catheterisation, which was not mentioned in the cardiac magnetic resonance report. There was no difference between discordant and concordant groups on the basis of patient age, weight, interval between cardiac magnetic resonance and cardiac catheterisation, or type of lesion.
Invasive assessment confirmed cardiac magnetic resonance-diagnosed great vessel stenosis in the majority of this cohort. The predominant discordant finding was lower catherisation gradient than predicted by morphologic and functional cardiac magnetic resonance assessment. Flow volume diversion - for example, unilateral pulmonary artery stenosis - and anaesthetic effects may account for some differences. Prospective refinement of cardiac magnetic resonance and interventional data may further improve the validity of non-invasive imaging thresholds for intervention.
Cardiology in the Young 08/2011; 22(2):178-83. · 0.76 Impact Factor
-
Journal of the American College of Cardiology 08/2011; 58(8):880. · 14.16 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: Cardiopulmonary exercise testing is increasingly used in children with congenital heart defects. Because of changes related to growth, the interpretation of exercise test results heavily relies on the presence of normative data. There is growing interest in the assessment of the ventilatory response to exercise in children with congenital heart disease, but normative data are lacking.
We studied 243 consecutive children (age, 13.2 ± 2.1 years; 128 boys) with maximal cardiopulmonary exercise testing. All children had normal clinical examination and echocardiograms. In all children, the slope of the relationship between minute ventilation and carbon dioxide production (VE/VCO(2) slope) was calculated using both only data until the respiratory compensation point (VE/VCO(2RC)) and using data until peak exercise (VE/VCO(2Peak)).
The exercise test was maximal in all children (peak respiratory exchange ratio, 1.2 ± 0.1). For all the cohorts, VE/VCO(2Peak) slope was 28.2 ± 3.7; and VE/VCO(2RC) slope was 24.5 ± 3.0, whereas peak oxygen uptake was 94.6% ± 14.0% of predicted value. Baseline spirometric function was normal in all children (vital capacity, 100% ± 14% and forced expired volume in the first second 97% ± 13% of predicted). From the age of 10 to 16 years, we observed a progressive decrease in both VE/VCO(2Peak) and VE/VCO(2RC) slopes (-0.833 and -0.705 per each year), with the highest reduction observed in boys. Gender-specific percentiles for both VE/VCO(2Peak) and VE/VCO(2RC) slopes were constructed.
Ventilatory response to exercise expressed as VE/VCO(2) slope seems to decrease progressively in the second decade of life. Because of age-related changes, interpretation of VE/VCO(2) slopes in this age range should be based on the reported percentiles rather than on the absolute values.
American heart journal 06/2011; 161(6):1214-9. · 4.65 Impact Factor
-
Archives of Disease in Childhood 03/2011; 96(5):409-10. · 2.88 Impact Factor
-
Philipp Lurz,
Johannes Nordmeyer,
Alessandro Giardini, Sachin Khambadkone,
Vivek Muthurangu,
Silvia Schievano,
Jean-Benoit Thambo,
Fiona Walker,
Seamus Cullen,
Graham Derrick,
Andrew M Taylor,
Philipp Bonhoeffer
[show abstract]
[hide abstract]
ABSTRACT: The purpose of this study was to assess the potential of late positive functional remodeling after percutaneous pulmonary valve implantation (PPVI) in right ventricular outflow tract dysfunction.
PPVI has been shown to impact acutely on biventricular function and exercise performance, but the potential for further late functional remodeling remains unknown.
Sixty-five patients with sustained hemodynamic effects of PPVI at 1 year were included. Patients were divided into 2 subgroups based on pre-procedural predominant pulmonary stenosis (PS) (n = 35) or predominant pulmonary regurgitation (PR) (n = 30). Data from magnetic resonance imaging and cardiopulmonary exercise testing were compared at 3 time points: before PPVI, within 1 month (early) and at 12 months (late) after PPVI.
There was a significant decrease in right ventricle end-diastolic volume early after PPVI in both subgroups of patients. Right ventricle ejection fraction improved early only in the PS group (51 ± 11% vs. 58 ± 11% and 51 ± 12% vs. 50 ± 11%, p < 0.001 for PS, p = 0.13 for PR). Late after intervention, there were no further changes in magnetic resonance parameters in either group (right ventricle ejection fraction, 58 ± 11% in the PS group and 52 ± 11% in the PR group, p = 1.00 and p = 0.13, respectively). In the PS group at cardiopulmonary exercise testing, there was a significant improvement in peak oxygen uptake early (24 ± 8 ml/kg/min vs. 27 ± 9 ml/kg/min, p = 0.008), with no further significant change late (27 ± 9 ml/kg/min, p = 1.00). In the PR group, no significant changes in peak oxygen uptake from early to late could be demonstrated (25 ± 8 ml/kg/min vs. 25 ± 8 ml/kg/min vs. 26 ± 9 ml/kg/min, p = 0.48).
In patients with a sustained hemodynamic result 1 year after PPVI, a prolonged phase of maintained cardiac function is observed. However, there is no evidence for further positive functional remodeling beyond the acute effects of PPVI.
Journal of the American College of Cardiology 02/2011; 57(6):724-31. · 14.16 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: A 12-year-old child with no previous medical history was referred with a 4-day history of cough, shortness of breath, and peripheral blood eosinophilia. Transthoracic echocardiography showed a soft tissue infiltrating the left ventricular free wall, the lateral mitral annulus, and the mitral valve leaflets. A soft tissue strand connecting the lateral left atrial wall and mitral leaflets across the mitral valve orifice was also identified, causing reduced opening and functional mitral stenosis. The diagnosis of Löeffler endocarditis was made, and after 10 weeks of treatment with oral prednisolone, there was complete resolution of symptoms and of the infiltrative tissue with normalization of mitral valve function. The present case highlights some atypical features of eosinophilic heart disease-like occurrence in paediatric age, the complete preservation of the right ventricle and left ventricular apex, and the presentation with mitral stenosis compared with mitral regurgitation typically observed in the late phase of the disease.
European Heart Journal – Cardiovascular Imaging 01/2011; 12(1):E3. · 2.32 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: To investigate the impact of balloon inflation pressure and balloon diameter on the expansion forces exerted in a stenosed vessel (congenital heart disease applications) using computational models.
A simplified three-dimensional model of a vessel with a cylindrical stenosis was created. Two low-compliance balloons with different inflation diameters (10 vs. 16 mm) were modelled. Finite element simulations of balloon expansions were performed. To dilate the stenosis from 4 to 10 mm lumen diameter, the large balloon needed less inflation pressure than the small balloon (0.55 vs. 1.00 MPa). Under these circumstances, the large balloon also achieved higher stresses at the stenotic vessel site (5.23 ± 0.10 vs. 3.97 ± 0.04 MPa, p<0.001). When using inflation pressures that led to equal surface stresses of both balloons, the large balloon could exert higher expansion forces onto the stenotic site, achieving higher stresses (5.18 ± 0.09 vs. 3.38 ± 0.01 MPa, p<0.001) and greater lumen diameter (9.73 vs. 8.68 mm).
In a computerised model of balloon dilatation, balloon diameter had a greater impact on the expansion force than inflation pressure. This finding emphasises the importance of choosing an appropriate balloon diameter to achieve optimal haemodynamic outcomes.
EuroIntervention: journal of EuroPCR in collaboration with the Working Group on Interventional Cardiology of the European Society of Cardiology 11/2010; 6(5):638-42. · 3.29 Impact Factor
-
Cardiology in the Young 10/2010; 20(5):555-6. · 0.76 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: Patients with anatomic repair of transposition of the great arteries (TGA) can present with branch pulmonary artery (PA) stenosis; however, its relation to an abnormal cardiopulmonary response to exercise is unknown. We investigated the relation between the PA anatomy and pulmonary blood flow (PBF) distribution and the cardiopulmonary response to exercise in patients with anatomic repair of TGA. We used cardiopulmonary exercise testing and magnetic resonance imaging to study 55 consecutive patients (62% male; age 14.4 ± 2.3 years) who had undergone neonatal anatomic repair of TGA. The peak oxygen uptake and slope of carbon dioxide elimination/minute ventilation was 79 ± 15% of predicted and 29.8 ± 3.8, respectively. Abnormal peak oxygen uptake (R = 0.363, p = 0.0082) and slope of carbon dioxide elimination/minute ventilation (R = 0.612, p <0.0001) values were associated with an abnormal right/left PBF distribution. However, although an increased ventilatory response to exercise appeared to be primarily related to an abnormal right/left PBF distribution, exercise capacity appeared to be related to the extent of the proximal PA branches and main PA stenosis (R = 0.476, p = 0.0004), suggesting that mechanical obstruction to PBF during exercise could be the main mechanism causing an abnormal exercise capacity. In conclusion, an abnormal PBF distribution related to branch PA stenosis or hypoplasia was associated with a reduced exercise capacity and increased ventilatory drive during exercise in patients with anatomic repair of TGA. Cardiopulmonary exercise test data can complement the anatomic and magnetic resonance imaging data in selecting those lesions that are functionally important.
The American journal of cardiology 10/2010; 106(7):1023-8. · 3.58 Impact Factor
-
Gerhard-Paul Diller,
Alessandro Giardini,
Konstantinos Dimopoulos,
Gaetano Gargiulo,
Jan Müller,
Graham Derrick,
Georgios Giannakoulas, Sachin Khambadkone,
Astrid E Lammers,
Fernando Maria Picchio,
Michael A Gatzoulis,
Alfred Hager
[show abstract]
[hide abstract]
ABSTRACT: previous studies have established an association between exercise intolerance and increased morbidity and mortality in congenital heart disease patients. We aimed to clarify if exercise intolerance is associated with poor outcome in Fontan patients and to identify risk factors for mortality, transplantation, and cardiac-related hospitalization.
a total of 321 Fontan patients (57% male, mean age 20.9 ± 8.6 years) who underwent cardiopulmonary exercise testing (CPET) at four major European centres between 1997 and 2008 were included. During a median follow-up of 21 months, 22 patients died and 6 patients underwent cardiac transplantation (8.7%), resulting in an estimated 5-year transplant-free survival of 86%. Parameters of CPET were strongly related to increased risk of hospitalization, but-with the exception of heart rate reserve-unrelated to risk of death or transplantation. In contrast, patients with clinically relevant arrhythmia had a 6.0-fold increased risk of death or transplantation (P < 0.001). Furthermore, patients with atriopulmonary/-ventricular Fontan had a 3.7-fold increased risk of death or transplantation compared with total cavopulmonary connection patients (P= 0.009). The combination of clinically relevant arrhythmia, atriopulmonary/-ventricular Fontan, and signs of symptomatic or decompensated heart failure was associated with a particularly poor outcome (3-year mortality 25%).
on short-term follow-up, most parameters of CPET are associated with increased risk of hospitalization but not death or transplantation in contemporary Fontan patients. Only decreased heart rate reserve and a history of clinically relevant arrhythmia, atriopulmonary/-ventricular Fontan, and/or heart failure requiring diuretic therapy are associated with poor prognosis, potentially identifying patients requiring medical and/or surgical attention.
European Heart Journal 10/2010; 31(24):3073-83. · 10.48 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: To determine the feasibility and safety of pre-stenting with a bare metal stent (BMS) before percutaneous pulmonary valve implantation (PPVI), and to analyse whether this approach improves haemodynamic outcomes and impacts on the incidence of PPVI stent fractures.
Retrospective analysis of prospectively collected data.
Tertiary paediatric and adult congenital heart cardiac centre.
108 consecutive patients with congenital heart disease underwent PPVI between September 2005 and June 2008 (54 with PPVI alone, 54 with BMS pre-stenting before PPVI).
There were no significant differences in procedural complication rates. Acutely, there was no difference in haemodynamic outcomes. Serial echocardiography revealed that in the subgroups of 'moderate' (26-40 mm Hg) and 'severe' (>40 mm Hg) right ventricular outflow tract (RVOT) obstruction, patients with pre-stenting showed a tendency towards lower peak RVOT velocities compared to patients after PPVI alone (p=0.01 and p=0.045, respectively). The incidence of PPVI stent fractures was not statistically different between treatment groups at 1 year (PPVI 31% vs BMS+PPVI 18%; p=0.16). However, pre-stenting with BMS was associated with a lower risk of developing PPVI stent fractures (HR 0.35, 95% CI 0.14 to 0.87, p=0.024). The probability of freedom from serious adverse follow-up events (death, device explantation, repeat PPVI) was not statistically different at 1 year (PPVI 92% vs BMS+PPVI 94%; p=0.44).
Pre-stenting with BMS before PPVI is a feasible and safe modification of the established implantation protocol. Pre-stenting is associated with a reduced risk of developing PPVI stent fractures.
Heart (British Cardiac Society) 10/2010; 97(2):118-23. · 4.22 Impact Factor
-
Philipp Lurz,
Alessandro Giardini,
Andrew M Taylor,
Johannes Nordmeyer,
Vivek Muthurangu,
Dolf Odendaal,
Bryan Mist, Sachin Khambadkone,
Silvia Schievano,
Philipp Bonhoeffer,
Graham Derrick
[show abstract]
[hide abstract]
ABSTRACT: The data describing the change in exercise capacity after surgical or interventional management of the patient with right ventricular (RV) outflow tract (OT) dysfunction are conflicting. The pathophysiologic consequences of RVOT interventions and the subsequent change in exercise performance are still poorly understood. We sought to assess the effect of percutaneous pulmonary valve implantation (PPVI) on exercise capacity in (1) patients with predominantly pulmonary stenosis (PS) and (2) in patients with predominantly pulmonary regurgitation (PR). A total of 63 patients with either predominantly PS (n = 37) or PR (n = 26) underwent PPVI. Cardiopulmonary exercise testing and magnetic resonance imaging were performed before and within 1 month after PPVI. On magnetic resonance imaging, the at rest effective biventricular stroke volumes improved in both groups after PPVI (p <0.001), but the ejection fraction improved only in the PS group. In the PS group, exercise capacity (peak oxygen uptake, p <0.001), ventilatory efficiency (p <0.001), and peak oxygen pulse (p <0.001) improved after PPVI. In the PR group, none of these parameters changed after PPVI (p = 0.6, p = 0.12, and p = 0.9, respectively). On multivariate analysis, the reduction in RVOT gradient was the only predictor of improved peak oxygen uptake when assessed in the whole patient group (r(part) = -0.59; p <0.001) or in the PS (r(part) = -0.45; p = 0.002) or PR groups alone (r(part) = -0.45; p = 0.02). In conclusion, acutely after PPVI, exercise capacity improves with the relief of stenosis but not regurgitation. A reduction in the RVOT gradient, even small gradients, was the only independent predictor of improved peak oxygen uptake in both patient groups, irrespective of improved pulmonary valve competence.
The American journal of cardiology 03/2010; 105(5):721-6. · 3.58 Impact Factor
