Mohamed Y. Abd El Rahman

Preserved regional atrial contractile function following extra-atrial rather than intra-atrial type Fontan operation : A tissue Doppler imaging study

SummaryObjectiveTo determine the univentricular and atrial function in patients following Fontan operation using tissue Doppler echocardiography (TDE).SettingHospital-based outpatient clinic and inpatient unit.PatientsThirty-six patients (mean age 13 ys [2–34 ys]) after Fontan procedure and 30 healthy individuals matched for age and heart rate.Main outcome measuresPulsed wave Doppler derived Tei index was determined for global univentricular function. Tissue-Doppler derived strain rate and strain were measured for regional ventricular function. Different planimetric approaches were used to evaluate the global atrial function. Strain rate was measured for regional atrial contractile function.ResultsThe Tei index, reflecting global univentricular function, was significantly higher in Fontan patients than in the control group (p<0.001). The regional ventricular contractile function, which was determined by TDE derived strain and strain rate, was significantly lower in Fontan patients than in healthy controls (p<0.001). The global atrial function evaluated planimetrically, and the regional atrial function assessed using the TDE strain rate, were significantly lower in Fontan patients than in normal controls (p<0.001, p<0.001, respectively). A comparison of different types of Fontan operations with extra-atrial or intra-atrial baffle showed a better regional left atrial wall strain rate in patients with extra-atrial baffle than with intra-atrial baffle.ConclusionPatients who have undergone the Fontan procedure may present with altered ventricular and atrial performance independent of whether the extra-atrial or intraatrial type Fontan operation was performed. However, the regional atrial contractile function seems to be better preserved following the extra-atrial type Fontan operation.

Left ventricular systolic dysfunction in asymptomatic Marfan syndrome patients is related to the severity of gene mutation: insights from the novel three dimensional speckle tracking echocardiography.

Background In asymptomatic Marfan syndrome (MFS) patients we evaluated the relationship between the types of fibrillin-1 (FBN1) gene mutation and possible altered left ventricular (LV) function as assessed by three-dimensional speckle tracking echocardiography (3D-STE). Methods and Results Forty-five MFS patients (mean age 24±15 years) and 40 age-matched healthy controls were studied. Genetic evaluation for the FBN1 gene was carried on 32 MFS patients. Gene mutation (n = 15, 47%) was classified as mild when the mutation resulted in nearly normally functioning protein, while mutations resulting in abnormally function protein were considered to be severe (n = 17, 53%). All patients and controls underwent 3D-STE for evaluation of LV function by an echocardiographer blinded to the results of the genetic testing. Compared to controls, MFS patients had significantly lower 3D-STE derived LV ejection fraction (EF, 57.43±7.51 vs. 62.69±4.76%, p = 0.0001), global LV longitudinal strain (LS, 14.85±2.89 vs. 17.90±2.01%, p = 0.0001), global LV circumferential strain (CS, 13.93±2.81 vs. 16.82±2.17%, p = 0.0001) and global LV area strain (AS, 25.76±4.43 vs. 30.51±2.61%, p = 0.0001). Apart from the global LV LS all these parameters were significantly lower in patients with severe gene mutation than in those with mild mutation (p<0.05). In the multivariate linear regression analysis only the type of mutation had a significant influence on the 3D-STE derived LVEF (p = 0.017), global CS (p = 0.005) and global AS (p = 0.03). Conclusions In asymptomatic MFS patients latent LV dysfunction can be detected using 3D STE. The LV dysfunction is mainly related to the severity of gene mutation, suggesting possible primary cardiomyopathy in MFS patients.

Prediction of Pulmonary Regurge and Right Ventricular Function in Asymptomatic Repaired Tetralogy of Fallot Patients in Developing Countries: A Comparison to Cardiac Magnetic Resonance Imaging

Objective: To assess the value of conventional echocardiographic measurements for quantification of pulmonary regurge and right ventricular function in comparison to Cardiac Magnetic Resonance Imaging (CMR) after surgical repair of Tetralogy Of Fallot (TOF). Methods: Twenty five asymptomatic children after TOF repair (9.2 ± 4 years) were compared to twenty five age matched healthy children. Echocardiographic quantification of pulmonary regurge was assessed by (1) pulmonary regurge jet width/pulmonary artery diameter, (2) pulmonary pressure half time, (3) pulmonary regurge index; pulmonary regurge duration to diastole duration, (4) no flow time; diastole duration - pulmonary regurge duration and (5) diastole/systole velocity time integral ratio. Measurements derived from conventional echocardiography were compared to pulmonary regurgitant fraction, right ventricular volumes and ejection fraction by CMR. Results: On CMR, the pulmonary regurgitant fraction was 28.64 ± 10.2%. By conventional echocardiography, pulmonary regurge index and no flow time were found to offer the best prediction for severity of pulmonary regurge. Pulmonary regurge index of 64 msec has sensitivity of 81% and specificity of 100% (AUC=0.894) in identifying significant pulmonary regurge. Compared to controls, patients after TOF repair showed significantly lower right ventricle myocardial velocities, higher E/ E‘ ratio and prolonged MPI. Among the TOF patients, right ventricular MPI showed significant negative correlation (r=-0.402; P=0.008) with tricuspid valve annulus peak systolic velocity (S‘) and significant positive correlation (r= 0. 413; P=0.04) with right ventricle stroke volume by CMR. Conclusion: Conventional echocardiography can offer a simple, readily available and accurate tool for quantification of pulmonary regurge and right ventricular function during mid- term follow up after surgical repair of tetralogy of Fallot.

Left circumflex coronary artery to coronary sinus fistula diagnosed in infancy

Coronary artery fistulas are rare anomalies, their incidence range from 0.1 to 0.2% of congenital heart defects. The left circumflex coronary artery (LCX) fistula draining into the coronary sinus (CS) is a less common form, and most cases described in the literature were adult cases. We are describing this type of fistula in an 8-month-old asymptomatic female presenting with a continuous murmur over the pericordium. Electrocardiogram was normal. Echocardiography revealed a dilated LCX and CS with turbulent flow in CS; 2-D speckle tracking echocardiography revealed normal left ventricular strain with no regional wall abnormalities. Multidetector computed tomography demonstrated the fistula. As the patient was asymptomatic, with no ventricular dilatation or dysfunction, we decided conservatively. <Learning objective: Coronary artery fistulas are rare anomalies that require complementary diagnostic modalities. 2D echocardiography, color flow Doppler, 2D speckle tracking, and multidetector computed tomography are needed to delineate the anomaly and to conclude the prognosis and long-term follow-up plans.>.

Letter by Abdul-Khaliq et al regarding article, "Right ventricular mechanics and QRS duration in patients with repaired tetralogy of Fallot: implications of infundibular disease".

To the Editor: We read with great interest the article by Uebing et al,1 and we congratulate the authors on this important work. The authors have cited our work1,2 and compared their results on right ventricular delay to our published data on intraventricular left ventricular (LV) dyssynchrony. In our article, we concluded that LV asynchrony may exist in patients with right bundle-branch block after tetralogy of Fallot repair and that this electromechanical delay was associated with lower global and regional LV function as well as physical performance.2 We would like to comment on the authors’ critical remark …

Letter Regarding Article by Thambo et al, “Detrimental Ventricular Remodeling in Patients With Congenital Complete Heart Block and Chronic Right Ventricular Apical Pacing”

To the Editor: We read with great interest the article by Thambo et al,1 and we congratulate the authors on this important work. We have conducted a similar study in children and adults with congenital atrioventricular block as well as other congenital heart diseases. Our initial results indicate significant left ventricular asynchrony and altered global left ventricular function after long-term right ventricular pacing, which is in agreement with Thambo et al. However, we would like to comment on the technical methods used by Thambo et al to evaluate asynchrony. Preimplantation asynchrony was assessed retrospectively by pulsed Doppler and M-mode echocardiography. This method may yield no precise data on ventricular asynchrony and regional mechanoelectrical interaction. Postimplantation ventricular asynchrony was assessed by tissue Doppler–derived velocity. In contrast, we use tissue Doppler–derived strain to determine the left ventricular asynchrony. …