Right Ventricular Volumes, Ejection Fraction, and Systolic Function Indices in Normal Neonates by Three-Dimensional Speckle-Tracking Echocardiography.

The aim of this study was to test the feasibility of measuring right ventricular (RV) volumes, ejection fraction (EF), and systolic function indices in neonates using three-dimensional speckle-tracking echocardiography (3D-STE). Thirty-eight neonates underwent complete echocardiographic evaluation, including the acquisition of three full-volume 3D datasets or more from each of the apical, parasternal, and subcostal windows while naturally sleeping. Datasets were analyzed using a commercially available software (Tomtec). Global RV 3D volumes and EF were measured. In addition, 2D free wall longitudinal strain (LS), tricuspid valve annulus (TVA), tricuspid annular plane systolic excursion (TAPSE) and its index to RV length (TAPSEi), and fractional area change (FAC) were obtained from a non-shortened apical 4-chamber view of the RV, derived from the 3D dataset. Three or more datasets obtained from the apical window were available for analysis for each subject. At least one dataset was adequate for analysis in all subjects. Mean indexed 3D diastolic, systolic, stroke volumes, and EF were measured at 28.5 ± 3.4 ml/m2, 13 ± 2.0 ml/m2, 15.6 ± 1.9 ml/m2, and 54.6 ± 3.2%, respectively. Free wall 2D LS was calculated at (- 27.9 ± 2.5%). In addition, mean TVA measured 11.1 ± 0.8 mm, TAPSE measured 6.8 ± 0.9 mm, and TAPSEi and FAC were calculated at 24.2 ± 2.1 and 46 ± 3.4%, respectively. 3D-STE is feasible in normal neonates without the need for sedation. Reference values of RV 3D volumes and 2D indices of systolic function were obtained. These data could be helpful in patients where the size or systolic function of the RV is in question. Larger studies are required to establish nomograms for the above indices in this age group.

Atrial function in the Fontan circulation: comparison with invasively assessed systemic ventricular filling pressure.

Abnormal atrial mechanics in biventricular circulations have been associated with elevated left heart filling pressures. Similar associations in the Fontan circulation are unknown. The aim of this study was to examine the relationship between atrial mechanics and invasively assessed hemodynamic parameters late after the Fontan operation. Thirty-nine Fontan patients with echocardiographic and invasive hemodynamic studies done within 48 h were included and were compared to 40 age-matched healthy controls. Atrial and ventricular strain measurements were measured offline using 2-dimensional speckle-tracking. Mean age was 10.2 ± 6.7 years and 24 (62%) were male. Atrial strain measures were lower in Fontan patients compared to healthy controls. There was no significant association between atrial strain measurements and Fontan systemic ventricular filling pressures (SVFP) as indicated by pulmonary artery occlusion pressures, direct left atrial pressure or systemic ventricular end-diastolic pressure. Global atrial strain was not correlated with segmental atrial strain in the pulmonary venous atrium. Global atrial reservoir strain was positively correlated with pulmonary vascular resistance (r = 0.508, p = 0.045). Global atrial conduit strain was positively correlated with E/A ratio of the AV valve inflow (r = 0.555, p = 0.002). Atrial and ventricular strain measurements were not significantly correlated. In patients with a Fontan, global atrial function is significantly depressed, and is uncoupled from segmental left lateral atrial function. Global as well as segmental atrial mechanics are not significantly associated with SVFPs in Fontan patients. Instead, global atrial reservoir function appears to parallel pulmonary vascular resistance.

Measurement of Left Ventricular Dimensions and Ejection Fraction in Neonates by Three-Dimensional Echocardiography: A Comparative Study Between Philips QLAB and TOMTEC Software-Are the Values Interchangeable?

Three-dimensional echocardiography is being used to evaluate left ventricular dimensions and ejection fraction in clinical practice. The validity and normal values have been established in a large group of normal adults, children and neonates. The aim of this study was to compare left ventricular dimensions and ejection fraction obtained from the same 3-dimensional echocardiography datasets using 2 commercially available applications: Philips QLAB and Tomtec 4D LEFT VENTRICLE-ANALYSIS. Fifty consecutive newborns or neonates coming to their first newborn visit within the first 3 weeks of live, were recruited. 38 babies underwent full Echocardiographic evaluation, including the acquisition of several 3-dimensional datasets, while naturally sleeping. Left ventricular dimensions, volumes and ejection fraction were measured using Philips QLAB version 9.0 and results were compared to results of the same datasets, analysed using TomTec 4D LV software. Mean left ventricular diastolic, and systolic volumes indexed to body surface area and ejection fraction were: 24.7 ± 3.6 ml/m2, 9.2 ± 1.3 ml/m2 and 62 ± 3.8% using TomTec and 26.6 ± 3.8 ml/m2, 10.4 ± 2 ml/m2 and 63 ± 3.1% using QLAB, respectively. Mean indexed left ventricular diastolic, and systolic volumes measured with QLAB were significantly higher as compared to TomTec with insignificant difference in the ejection fraction. Normal left ventricular indices obtained from 3-dimensional echocardiography datasets were established, using Philips QLAB and TomTec 4D LV ANALYSIS software. Measurements obtained were significantly different between those venders, and hence, may not be used interchangeably.

Percutaneous Management of a Mechanical Complication Post Mitral Valve Surgery in a Child.

A 4-year-old girl with congenital mitral regurgitation status post mechanical mitral valve replacement (MVR) using a 16 mm Carbomedics valve (LivaNova) at the age of 5 months underwent a redo MVR using a 21 mm aortic prosthesis implanted in an inverted position for progressed increased mitral valve gradient due to pannus formation. Post operatively, echocardiogram showed good mitral valve prosthesis function with severe depressed left ventricular function (ejection fraction, 19%) and markedly segmental dyskinesia in the lateral wall. Her diagnosis of compression to the proximal dominant left circumflex artery and subsequent percutaneous coronary intervention are outlined here.

Gothic aortic arch and cardiac mechanics in young patients after arterial switch operation for d-transposition of the great arteries.

BACKGROUND: In patients who have undergone arterial switch operation (ASO) for d-transposition of the great arteries a gothic aortic arch (GA) morphology has been found and it has been associated with abnormal aortic bio-elastic properties. HYPOTHESIS: GA is frequent in ASO patients and may have an impact on cardiac mechanics. Our study aims were to assess 1- the occurrence of GA in a large sample of patients after ASO; 2- the association between GA and aortic bio-elastic properties; and 3- the impact of GA on left ventricular (LV) function using speckle tracking echocardiography (STE). METHODS: We studied one hundred and five asymptomatic patients, who have undergone first stage ASO for d-TGA, with normal left ventricular ejection fraction (LVEF ≥53%). RESULTS: Forty-six (44%) patients showed a GA (mean age 11.5±7.2years, 26 males) while fifty-nine (56%) patients (mean age 9.6±6.7years, 37 males) did not present GA. The two groups were comparable for age, sex, BSA, and office blood pressure values. In group GA aortic root was significantly dilated (27.4±7.5mm vs. 21.2±6.9mm, p<0.0001), aortic stiffness index (Group GA=1.8±1.2 vs. 1.4±0.7, p=0.025) was significantly increased, left atrial volume was larger (p=0.0145), global longitudinal strain (Group GA=-18.4±2.5% vs. -20.1±3.3%, p=0.012) and basal LV longitudinal strains (Group GA=-16.9±4.8% vs. -20.4±7.0%, p=0.013) were significantly reduced. CONCLUSIONS: After ASO the presence of a GA is associated with a significantly dilated aortic root, stiffer aortic wall, larger left atrial volume, and worse LV longitudinal systolic deformations, well known predictors of cardiovascular morbidity and mortality.

Left Ventricular Mechanics in Patients with Abnormal Origin of the Left Main Coronary Artery from the Pulmonary Trunk Late after Successful Repair.

OBJECTIVE: Our aim was to evaluate left ventricular (LV) mechanics by using speckle tracking echocardiography (STE) in asymptomatic patients with abnormal origin of the left main coronary artery from the pulmonary trunk (ALCAPA), late after successful repair, in the presence of LV ejection fraction (EF) >50%. METHODS: We studied 30 ALCAPA patients (median age 4 years, range 1-25 years, NYHA class I, LVEF >50%) and 16 healthy age- and sex-matched controls (median age 5 years, range 1-25 years). All underwent standard echocardiographic evaluation and STE. RESULTS: LV dimensions and LVEF (63.6 ± 8.2% vs. 64.1 ± 5.1%, p = 0.826) were not different between patients and controls. Diastolic parameters were significantly abnormal in our patients versus controls (E/e' average: 11.9 ± 5.8 vs. 6.6 ± 3.0, p = 0.0014). Global LV longitudinal strain was significantly lower in ALCAPA patients versus controls (-17.6 ± 3.5% vs. -23.4 ± 3.1%, p < 0.0001). LV torsion (9.1 ± 4.9° vs. 11.9 ± 3.3°, p = 0.046) was significantly impaired in ALCAPA patients. CONCLUSIONS: After successful repair in asymptomatic ALCAPA patients, despite an LVEF >50%, diastolic function, LV longitudinal deformation and LV torsion remain impaired. We suggest including a detailed study of the diastolic function and cardiac mechanics in the clinical follow-up of these patients to identify the subgroup of patients at higher risk.

Normal Range of Left Ventricular Strain, Dimensions and Ejection Fraction Using Three-dimensional Speckle-Tracking Echocardiography in Neonates.

AIMS: Three-dimensional speckle-tracking echocardiography (3D-STE) is a promising new technique to evaluate left ventricular (LV) mechanics. The feasibility and normal values of LV strain using 3D-STE have recently been established in adults and children. Unfortunately, no data are available in neonates. The aims of this study were to evaluate the feasibility and establish normal values of 3D LV volumes, ejection fraction (EF), and the 4 normal strains in healthy neonates. MATERIALS AND METHODS: Of 50 consecutive newborns who were delivered at our hospital or returned to their first newborn follow-up within the first 3 weeks of life, 38 babies underwent full echocardiographic evaluation, including the acquisition of at least 3 full volume data sets from the apical window, while naturally sleeping. Data sets were analyzed offline. Global LV longitudinal, circumferential, and radial strain, as well as 3D LV volumes and EF, were measured using 3D-STE. RESULTS: Of the 50 newborns, 2 patients were excluded because of significant intra-cardiac shunts, and in another 10 subjects, parents did not give consent. At least one data set was adequate for analysis in all the remaining subjects. Mean indexed LV diastolic, systolic volumes, and EF were 24.7 ± 3.6 ml/m2, 9.2 ± 1.3 ml/m2, and 63% ± 3.7%, respectively. Normal global longitudinal, circumferential, radial, and tangential 4D strain were -20.9% ± 2.8%, -32.4% ± 3.1%, 44.3% ± 3.4%, and -39.7% ± 3.4%, respectively. CONCLUSIONS: 3D-STE is feasible in newborns without the needed for sedation. Reference values of normal, regional, and global LV 4D strain and volumes were obtained.

Tetralogy of Fallot, Down's Syndrome, Left Ventricular Noncompaction, and Multiples Thrombi.

We report a unique case of left ventricular noncompaction (LVNC), tetralogy of Fallot, and multiples biventricular thrombi in a Down's syndrome. Of interest, speckle tracking analysis detected an abnormal LV myocardial longitudinal deformation, despite the normal ejection fraction and absence of NC at the baseline evaluation.

Cardiac reoperations following the Ross procedure in children: spectrum of surgery and reoperation results.

OBJECTIVES: The Ross procedure is the preferred aortic valve replacement (AVR) choice in small children. Nonetheless, it is a complicated surgery and there are concerns that subsequent cardiac reoperations are exceptionally complex and associated with high morbidity and mortality. We examine the surgical spectrum and report outcomes of cardiac reoperations in patients who had undergone the Ross procedure during childhood. METHODS: Records of 227 consecutive children (<18 years old) who had undergone the Ross procedure at our institution from 1991 to 2004 were reviewed. Our patient cohort was 50 patients who underwent 58 cardiac reoperations following the Ross procedure during the follow-up. Time-related outcomes were analyzed. RESULTS: From 1992 to 2009, 50 patients, 37 males (74%), underwent cardiac reoperation at a mean age of 15.6±5.2 years and a mean interval of 3.9±3.0 years following the Ross procedure. Risk factors for cardiac reoperation following the Ross procedure on multivariable analysis were rheumatic fever, aortic regurgitation, concomitant cardiac surgery, use of fresh homografts and earlier era of surgery. Overall, 32 (55%) reoperations were isolated procedures whereas 26 (45%) were more complex involving 2-4 simultaneous cardiac procedures. In total, 92 procedures were performed including AVR (n=31), homograft replacement (n=23), mitral valve replacement (n=18), mitral valve repair (n=11), tricuspid valve repair (n=5) and other (n=4). There was no operative mortality and one late death. Survival was 98% at 10 years. During the follow-up, 8 of 50 patients required further cardiac surgery following initial reoperation with freedom from additional cardiac surgery of 82% at 10 years. Subsequent cardiac surgery risk was higher in patients with pre-operative aortic regurgitation and those who had concomitant surgery at time of Ross on log-rank analysis. Among survivors, 96% are in New York Heart Association class I/II. CONCLUSIONS: A wide range of cardiac reoperations may be required in children following the Ross procedure, especially those with underlying rheumatic aetiology, aortic regurgitation and multivalvular involvement. Despite complexity, reoperation following the Ross procedure can be performed with low mortality and good mid-term results. This information should be taken into consideration during the selection of aortic valve substitute in children.

Outcomes and associated risk factors for mitral valve replacement in children.

OBJECTIVE: We aim to report time-related outcomes following mitral valve replacement (MVR) in children and to identify factors affecting outcomes. METHODS: Clinical records from 307 children who underwent MVR between 1985 and 2004 were reviewed. Competing-risks methodology determined time-related prevalence of three mutually exclusive end-states: death, mitral reoperation and survival without subsequent MVR, and their associated risk factors. RESULTS: Mean age was 11.4 ± 5.6 years including 36 (12%) patients < 2 years old. There were 154 (50%) males. Underlying pathology was rheumatic fever (n = 195, 64%), congenital (n = 83, 27%) and other (n = 29, 9%) with congenital pathology predominant in younger children while rheumatic fever predominant in older children. Hemodynamic manifestation was regurgitation (83%), stenosis (5%), or mixed disease (12%). One hundred and twenty-six patients (41%) had undergone a prior cardiac surgery including mitral surgery (n = 96, 31%). Initial mitral prosthesis was mechanical (n = 229, 75%), tissue (n = 71, 23%), or homograft (n = 7, 2%). Concomitant cardiac surgery was required in 141 patients (46%). Competing-risks analysis predicted that 20 years following MVR, approximately 17% of patients have died, 51% have undergone mitral reoperation and only 33% were alive and free from mitral reoperation. Risk factors for death without mitral reoperation included younger age < 3 years [PE (parameter estimates): +1.66 ± 0.31, p < 0.001], longer cross-clamp time (PE: +0.11 ± 0.04/10 min, p = 0.005), postoperative complications (PE: +1.5 8 ± 0.31, p < 0.001), and higher prosthesis size/body surface area (BSA)-predicted mitral annulus ratio (PE: + 0.48 ± 0.10, p < 0.001). Risk factors for mitral reoperation included implantation of homograft or tissue prosthesis (PE: +1.12 ± 0.23, p < 0.001) and smaller prosthesis size (PE: +0.06 ± 0.03/1 mm, p = 0.05). Fifteen-year freedom from pacemaker implantation, endocarditis, bleeding, and thromboembolism was 92%, 96%, 82%, and 92%, respectively. CONCLUSIONS: Mortality and mitral reoperation are common after MVR in children and outcomes can be predicted based on patient's age, prosthesis size, and other associated factors. Some modifiable factors such as avoiding oversized prostheses may improve outcomes especially in the smallest children.

Determinants of short- and long-term outcomes following triple valve surgery.

BACKGROUND AND AIM OF THE STUDY: Triple valve surgery (3VS) is required in some cardiac disorders that simultaneously involve the aortic valve (AV), mitral valve (MV), and tricuspid valve (TCV). Herein are reported details of the authors' experience with 3VS, and their attempts to identify those factors associated with a poor clinical outcome. METHODS: The medical records of patients who underwent 3VS between 1985 and 2005 were reviewed. A competing-risks methodology was used to determine the time-related prevalence and associated factors for three mutually exclusive end states after 3VS, namely death, valve reoperation, and survival without subsequent valve reoperation. RESULTS: A total of 206 consecutive patients (124 females, 82 males; median age 34 years; range: 12-82 years) underwent 3VS. The most common underlying pathology for all valves was rheumatic, followed by repair failure and prosthesis dysfunction for AV and MV, and functional regurgitation for TCV. In total, 72 patients (35.0%) had undergone prior valve surgery. At 3VS, the MV and AV were usually replaced (> 70%), whereas the TCV was usually repaired (91%). The operative mortality (prior to hospital discharge) was 11%, and 15-year survival 68%. At 15 years after 3VS, 21% of the patients had died, without subsequent valve reoperation, 50% had undergone valve reoperation, and 30% remained alive without valve reoperation. Baseline factors associated with early mortality after 3VS were older age at operation, TCV replacement, and the use of a mechanical MV during 3VS. A late risk of mortality was associated with poor left ventricular function and renal impairment at the time of the initial 3VS, and MV prosthesis prior to the initial 3VS. Younger age at 3VS and MV repair during the index 3VS were associated with a higher constant risk of valve reoperation, while female gender, MV prosthesis prior to index 3VS, MV replacement with a bioprosthesis during 3VS, and rheumatic TCV disease were associated with an increased late risk of valve reoperation. CONCLUSION: Both, primary and reoperative 3VS can be performed with acceptable operative mortality and long-term survival. However, patients continue to be at risk of valve reoperation, which steadily increases over time.

Simultaneous aortic and mitral valve replacement in children: time-related outcomes and risk factors.

BACKGROUND AND AIM OF THE STUDY: Valve replacement in children is problematic, and associated with high anticoagulation-related complications and increased reoperation requirements. Multiple valve replacement may further increase morbidity and worsen outcome. The results are reported of combined aortic valve replacement (AVR) and mitral valve replacement (MVR) in children. METHODS: The medical records of children who underwent simultaneous AVR and MVR between 1984 and 2004 were reviewed, and the short-term and long-term results and variables affecting outcomes explored. The mean duration of follow up was 9.7 +/- 6.6 years. RESULTS: A total of 84 patients (62 males, 22 females; mean age 15.0 +/- 2.2 years) was identified. The underlying pathology was mainly rheumatic (94%) and endocarditis (4%). Among the patients, 21 (25%) had undergone a prior cardiac surgery. The implanted valves were either mechanical (n = 71) or bioprosthetic (n = 13). The average aortic and mitral valve sizes were 22 mm and 29 mm, respectively. In total, 21 patients had concomitant cardiac surgery, most commonly tricuspid valve repair (n = 18). The mean cardiopulmonary bypass time and ischemic time were 142 +/- 47 min and 107 +/- 33 min, respectively. Survival at 30 days and at one year was 96% and 94%, respectively. The overall 15-year survival was 78% (bioprosthesis 92% versus mechanical 76%; p = 0.4). The 15-year freedom from cardiac reoperation was 59%, and 68% and 75% for mitral and aortic reoperation, respectively. Significant risk factors for reoperation were the use of a bioprosthetic valve (p = 0.003) and female gender (p = 0.03). Freedom rates from endocarditis, thromboembolic and bleeding complications at 15 years were 90%, 92%, and 96%, respectively. Among survivors, 95% were in NYHA class I/II. CONCLUSION: Children with rheumatic fever and endocarditis may require simultaneous AVR and MVR. Although the operative mortality is acceptable, patients continue to have constant attrition with time, especially those who have received mechanical prostheses. The risk of cardiac reoperation requirement is high in all patients. Despite the greater need for reoperation, bioprosthetic valves could be offered to selected patients, such as females and those who are non-compliant with anticoagulation regimens.

Results of left atrioventricular valve reoperations following previous repair of atrioventricular septal defects.

OBJECTIVES: We report results of left atrioventricular valve reoperations (LAVVR) following atrioventricular septal defect (AVSD) repair and examine variables predictive of outcome. METHODS: Multiple demographics and operative variables were analyzed to determine factors affecting survival and reoperation. RESULTS: Forty patients following partial (n = 9) or complete (n = 31) AVSD repair underwent 47 LAVVR (1992-2005). Median age was 0.87 years (24 days-7.7 years) at initial AVSD repair and 3.15 years (84 days-13.6 years) at subsequent LAVVR with median interval between AVSD repair and LAVVR of 1.76 years (1 day-12.9 years). First LAVVR included repair (n = 20) or replacement (n = 20). Operative mortality was 10% and five-year survival was 76 +/- 6%. Significant risk factors were complete AVSD (p < 0.001), valve replacement (p < 0.001) for early death, and young age at time of LAVVR (p = 0.03) for late death. Five-year freedom from LAVV re-intervention was 100% for replacement versus 55 +/- 13% for repair (p = 0.006). Overall, ejection fraction increased to 61 +/- 3% versus 42 +/- 2% preoperatively (p < 0.01), and left-ventricle end-diastolic dimension Z-score decreased to 0.05 +/- 0.36 versus 3.1 +/- 0.3 preoperatively (p < 0.01). Eighty-seven percent of children were in New York Heart Association class I/II at latest follow-up. CONCLUSIONS: LAVVR results in significant clinical improvement and lasting recovery in ventricular chamber function and size. Valve repair offers survival advantage and should be aggressively attempted; however, it is only achievable in 50% of cases. Valve replacement is necessary in cases associated with complex LAVV morphology or following repair failure. At intermediate follow-up, patients continue to be at risk of major valve-related morbidity, requirement for re-intervention, and cardiac death.

Coronary arterial compression treated by stenting after replacement of the mitral valve in a child.

We describe the clinical history of a nine years old girl with Shone's syndrome. She underwent balloon angioplasty of the aortic coarctation in infancy, and later developed severe sub-aortic stenosis and moderate mitral valvar stenosis. The mitral valve was therefore replaced with a mechanical prosthesis, and the sub-aortic shelf was resected. Immediately after the operation, she developed signs of myocardial ischemia. Coronary angiography showed compression of the middle part of the circumflex artery by the mechanical prosthesis, the obstructed segment being successfully dilated using a coronary arterial stent.

Hoarseness after pulmonary arterial stenting and occlusion of the arterial duct.

We report a 12-year-old girl who had multiple congenital cardiac lesions, specifically an arterial duct, left pulmonary arterial stenosis, an atrial septal defect in the oval fossa, and mild Ebstein's malformation of the tricuspid valve. Therapeutic transcatheter intervention was performed to stent the left pulmonary artery, occlude the arterial duct with a coil, and place a device to close the atrial septal defect. Subsequent to the catheterization, she complained of hoarseness, which was shown to be due to entrapment of the left recurrent laryngeal nerve between the coil used to close the arterial duct and the stent placed in the left pulmonary artery. Laryngoscopy confirmed paralysis of the recurrent laryngeal nerve.