Sildenafil and ventriculo-arterial coupling in Fontan-palliated patients: a noninvasive echocardiographic assessment.

The fundamental role of pulmonary vascular resistance in the Fontan circulation is obvious. Medications decreasing this resistance may have an impact on the fate of this population. Hence, we assessed noninvasively the effect of oral sildenafil on the ventriculo-arterial coupling in patients with Fontan circulation. In a single-center, prospective case series study, 23 patients with fenestrated extracardiac total cavopulmonary connection age 12-31 years were enrolled in this study. Clinical characteristics and echocardiographic examination were performed before and after a 1 week course of sildenafil at 0.5 mg/kg every 8 h. Sildenafil had no effect on heart rate and blood pressure. However, oxygen saturation was significantly increased with sildenafil (87.6 ± 4.3 vs. 90.1 ± 3.6; P < 0.0001). The calculated noninvasive ventricular end-systolic elastance (Ees) was greater after sildenafil compared with the pre-sildenafil values (1.59 ± 0.17 vs. 1.72 ± 0.27 mm Hg/ml; P = 0.001). Moreover, significant decreases in arterial elastance (Ea) (1.62 ± 0.53 vs. 1.36 ± 0.43 mm Hg/ml; P < 0.0001), ventricular end-diastolic elastance (Eed) (0.05 ± 0.021 vs. 0.04 ± 0.013; P = 0.002), and, finally, ventriculo-arterial coupling index (0.99 ± 0.26 vs. 0.76 ± 0.15; P < 0.0001) were found after sildenafil administration. The intolerable side effects that led to stopping the sildenafil occurred only in one (4 %) patient. Sildenafil has increased ventricular systolic elastance and improved ventriculo-arterial coupling in patients palliated with Fontan circulation. Short-term sildenafil was well tolerated in most of the patients with only minor side effects.

A novel method for quantification of left ventricular noncompaction using two-dimensional echocardiography in children.

OBJECTIVE: Although there are several echocardiographic criteria, there is not yet a general consensus about the diagnosis of left ventricular noncompaction. The current criteria are mostly based on the areas with maximal noncompaction in the heart. The echocardiographer may miss this maximal point leading to a misdiagnosis. Accordingly, we suggested a new method to measure the percentage of myocardial noncompaction using two-dimensional echocardiography. METHODS: In this study, the new method was examined on 4 noncompaction and 26 dilated cardiomyopathies, and 25 normal subjects. The percentage of noncompaction was measured at 3 levels (apical, papillary muscle and mitral valve) and averaged. FINDINGS: The mean percentages of myocardial noncompaction were 3.59±2.27, 8.86±5.52 and 34.7±26.1 in the control, dilated cardiomyopathy and noncompaction groups, respectively. A value of 17% or greater could distinguish left ventricular noncompaction from dilated cardiomyopathy with 92% specificity and 100% sensitivity and from normal subjects with 100% specificity and sensitivity. This percentage had a statistically significant association with noncompacted to compacted myocardial thickness ratio (P<0.001). CONCLUSION: This method showed good correlations with the existing echocardiographic and magnetic resonance criteria. However, it is not dependent on finding the area of maximal involvement. Being comparable to magnetic resonance imaging in accuracy, it is easier to perform and more available.

The impact of preload alteration on the myocardial performance index through implementing positive end expiratory pressure.

Load independent methods should be used for the assessment of ventricular function. Debate still exists regarding whether tissue Doppler imaging (TDI) indices are influenced by preload. Here, we evaluated the effect of positive end expiratory pressure (PEEP) related preload reduction on both conventional pulsed Doppler (PD) and TDI myocardial performance index (MPI). Thirty-eight mechanically ventilated patients of 3 months to 12 years old (mean ± SD age of 30 ± 11 months) without overt heart disease were enrolled. Doppler mitral inflow velocities, isovolumetric contraction and relaxation times and aortic ejection time in addition to TDI peak systolic, early and late diastolic velocities from the basal segment of left ventricular lateral wall were determined for each patient before and after applying high PEEP (10 cmH(2) O).PD-MPI was load dependent (0.61 ± 0.22 vs. 0.78 ± 0.25, P = 0.002). However, TDI-MPI did not significantly change after the use of high PEEP declining the left ventricular volume loading (0.78 ± 0.21 vs. 0.84 ± 0.22, P = 0.23). Hence, regarding various interfering pathophysiologic factors particularly preload reduction, it seems that TDI-MPI would be a more reliable index for the assessment of ventricular function.

Assessment of left atrial ejection force in mildly asphyxiated newborns.

OBJECTIVE: Asphyxia-induced cardiac insult is one of the major causes of mortality and morbidity in the course of perinatal asphyxia. Nowadays, a remarkable trend of interest is sensed introducing a plausible modality for early detection of cardiac insults at the beginning stages of asphyxia. In this study we aimed to evaluate diagnostic utility of transmitral Doppler-derived parameters as well as left atrial ejection force index as a marker of left atrial contractile function in these patients. METHODS: In a prospective study selected cases of 26 asphyxiated newborns with preserved systolic function underwent conventional transmitral Doppler flow echocardiographic assessment. Left atrial ejection force index was further calculated for all patients. Data was compared with normal ranges of healthy newborns in order to clarify the diagnostic utility of these parameters for determining minor cardiac insults in this age group. FINDINGS: We found that mildly asphyxiated newborns showed an increase in the values of left atrial ejection force index (5.44±2.12 kilodyne vs. 6.66±2.17 kilodyne, P= 0.02) and left atrial filling fraction (39%±10% vs. 45%±8%, P= 0.01). Furthermore, the acceleration and deceleration rate of early filling flow peak velocity were decreased in this group of asphyxiated newborns. CONCLUSION: Assessment of left atrial ejection force in mildly asphyxiated newborns reveals that newborns with even mild asphyxia, although could not be categorized in conventional grading system, suffer to some extent from a ventricular filling abnormality. This type of latent ventricular filling abnormality could simply be unmasked by calculation of atrial ejection force index.

Right ventricular myocardial tissue velocities, myocardial performance index, and tricuspid annular plane systolic excursion in totally corrected tetralogy of fallot patients.

BACKGROUND: Longer survival after the total repair of the Tetralogy of Fallot increases the importance of late complications such as right ventricular dysfunction. This is a prospective study of the right ventricular function in totally corrected Tetralogy of Fallot patients versus healthy children. METHODS: Thirty-two healthy children were prospectively compared with 30 totally corrected Tetralogy of Fallot patients. Right ventricular myocardial tissue velocities, right ventricular myocardial performance index, and tricuspid annular plane systolic excursion were investigated as well as the presence and severity of pulmonary regurgitation. RESULTS: The two groups were age-and sex-matched. Mean systolic peak velocity (Sa) and tricuspid annular plane systolic excursion were significantly decreased, while myocardial performance index and early to late diastolic velocity (Ea/Aa) were significantly increased in the Tetralogy of Fallot patients. Early diastolic velocity (Ea) showed no significant difference between the two groups. Sa correlated significantly with tricuspid annular plane systolic excursion in both the normal children and totally corrected Tetralogy of Fallot patients. Myocardial performance index was significantly higher in the patients with moderate to severe pulmonary regurgitation than in those with mild regurgitation. However, there was no significant correlation between this index and right ventricular myocardial tissue velocities. CONCLUSION: In this study, systolic right ventricular function indices (Sa and tricuspid annular plane systolic excursion) were impaired in the totally corrected Tetralogy of Fallot patients. Myocardial performance index was affected by the severity of pulmonary regurgitation.

Myocardial performance index and atrial ejection force in patients with Duchenne's muscular dystrophy.

BACKGROUND: Progressive movement limitation in patients with Duchenne's muscular dystrophy (DMD) may mask the onset of cardiac involvement. We aimed to determine whether myocardial performance index (MPI), either by conventional Doppler (CD) or tissue Doppler imaging (TDI), could detect subclinical myocardial dysfunction in DMD patients. Furthermore, we assessed the atrial ejection force (AEF) to evaluate possible existence of latent diastolic dysfunction. METHOD: Twenty DMD patients without signs and symptoms of cardiac impairment and 20 age matched control group enrolled into the study. MPI for right ventricle and left ventricle (LV) was assessed with CD and TDI for comparison. RESULTS: No significant difference was detected in ejection fraction, fractional shortening, and AEF between two groups. CD derived MPI was higher for both ventricles in the patients (P < 0.0001). Moreover, the TDI derived MPI were significantly higher in the patients in mitral, tricuspid, and septal views (P < 0.0001). A significant decrease of mitral myocardial systolic wave velocity was detected in the patients, in favor of early involvement of the posterobasal LV myocardium. All acceleration and deceleration times' values for the late mitral inflow were higher whereas the rates were significantly lower in the patients. There was no significant difference between AEF of both groups. CONCLUSION: MPI might be a useful parameter for early detection of occult cardiac dysfunction in DMD patients when other simple and standard echocardiographic parameters are within the normal limits. Lack of atrial contribution in LV filling due to atrial contractile dysfunction may play a role in DMD cardiac dysfunction.

The Relationship between Coronary Artery Aneurysm and QT Interval Dispersion in Acute Phase of Kawasaki Disease.

OBJECTIVE: QT dispersion (QTd) has been proposed as a marker of ventricular repolarization inhomogeneity and several investigations have proved the relationship between it and cardiac ischemia, ventricular arrhythmia and sudden cardiac death. The aim of this study was to assess the relation between coronary artery involvement and QTd, and QTc dispersion (QTcd) in the acute phase of Kawasaki disease (KD). METHODS: We studied 65 patients with acute KD. Patients were divided into 3 groups. Group one consisted of 48 patients without coronary artery involvement. Group two comprised 13 patients with small to medium size aneurysm. In Group three there were 4 patients with giant aneurysm or multiple small to medium size aneurysms or thrombosis in coronary arteries. For each patient 12 lead electrocardiography was obtained, and QT, QTc, QTd, QTcd, and RR interval were calculated. FINDINGS: There were 40 males and 25 females with a mean age of 41.4±31.1 months. There was no significant difference in QT, QTc, RR measurements between 3 groups. QTd was greater in group 3 versus group 1 and 2, but the difference was not statistically significant (P=0.06). QTcd was significantly greater in group 3 than in groups 1 and 2 (75.02±11.53 ms versus 46.82±15.39 ms and 48.88±10. 55 ms respectively (P = 0.04). The sensitivity of QTcd ≥60 ms to detect the patients with severe coronary arteries involvement was 100%, the specificity was 93.4%, positive predictive value was 50%, negative predictive value was 100%, and accuracy was 93.8%. CONCLUSION: QTcd can be used as a predictive factor for diagnosis of severe coronary arteries involvement in the acute phase of KD.

Transcatheter Atrial Septal Defect Closure under Transthorasic Echocardiography in Children.

OBJECTIVE: Atrial septal defect (ASD) device closure is routinely done under the guide of transesophageal or intracardiac echocardiography which are expensive techniques and not easily affordable in developing countries. METHODS: Using metallic devices, we attempted 32 ASD device closures under transthoracic echocardiography. FINDINGS: Of those, 30 procedures were successful (94 %). In two patients with relatively large ASD we encountered difficulty in positioning the device. These patients were referred for surgical closure. CONCLUSION: ASD device closure can be carried out successfully in most patients under transthoracic echocardiography in situations where transesophageal or intravenous echocardiographies are not available or affordable.

Trans-catheter closure of patent ductus arteriosus-What is the best device?

BACKGROUND: Over the past three decades, transcatheter occlusion of patent ductus arteriosus (PDA) has evolved to be the procedure of choice. Gianturco and Flipper coils are the most commonly used coils in the United States for closure of small and moderate size PDAs. For larger PDAs, interventionalists in the United States commonly use the Amplatzer Duct Occluder (ADO) and those in Europe use the ADO or the Nit-Occlud Coils (NOC). A comparison between Gianturco coils, Flipper coils, ADO, and NOC has never been made. OBJECTIVE: To compare the success and complication rate associated with the four different devices used for transcatheter closure of PDA. Success was defined as complete closure of PDA with absence of a residual shunt (R.S.) at six months follow-up. METHODS: Two institutions collaborated in combining their data to evaluate the results of transcatheter closure of PDA. RESULTS: Totally, 546 patients underwent successful PDA occlusion at both institutions. Gianturco and Flipper coils were used in 120 (22%) and 119 (22%) patients respectively. A total of 152 (28%) patients received ADO and 155 (28%) patients received NOC. Immediate R.S. were noted in 226 (41.4%) patients in the entire study group with the NOC group having the highest percentage of R.S. (80/155, 51.6%, P = 0.004). Of the 484 patients with follow-up echocardiograms at 6 months, 35 (7.2%) patients had persistent R.S. The NOC (3/143, 2.1%) and ADO (5/150, 3.3%) groups had the least R.S. at six months follow-up. CONCLUSION: Per our definition of success, the Nit-Occlud coils and the Amplatzer duct-occluder devices had significantly higher success rate for PDA occlusion versus the coils. © 2010 Wiley-Liss, Inc.

Augmentation of left atrial contractile function: a herald of iron overload in patients with beta thalassemia major.

Early detection of myocardial iron overload is crucial for optimal management of patients with beta thalassemia major, which could lead to intensification of iron chelating therapy. In this study, we evaluate the conventional echocardiography and tissue Doppler imaging measurements in patients with beta thalassemia major and further introduce the assessment of atrial ejection force as a feasible price-saving approach for early detection of myocardial iron overload. During a 1-year period, 42 cases of beta thalassemia major aged <21 years and with preserved systolic function were evaluated with magnetic resonance T2* imaging (MRI T2*), conventional echocardiography, and tissue Doppler imaging techniques. Patients were classified into two groups according to their myocardial MRI T2* values, with and without critical iron loading. All patients with echocardiographic evidence of moderate and severe stages of diastolic dysfunction showed critical iron loading in their MRI T2*. After excluding those patients with severe and moderate ventricular diastolic filling abnormality, the atrial ejection force index (P = 0.002) and a number of volume indexes of the left atrium showed a significant difference between the two groups. None of the tissue Doppler imaging measurements showed a statistically significant difference between the two groups. The atrial ejection force index of 7.41, with a sensitivity of 93% and a specificity of 74%, was suggested to detect critical cardiac iron loading. These results imply that combining the atrial ejection force index with the transmitral-derived echocardiographic assessment is a feasible way to detect early stages of myocardial iron overload in patients with beta thalassemia major.

Lown-Ganong-Levine syndrome in a 3-month-old infant with isolated left ventricular noncompaction.

This report describes a 3-month-old boy with isolated left ventricular noncompaction admitted to a medical facility due to heart failure and dysrhythmia. His electrocardiogram showed a short PR interval and a normal QRS complex after abortion of supraventricular tachycardia in favor of Lown-Ganong-Levine syndrome or enhanced atrioventricular nodal conduction.

Avoiding cardiopulmonary bypass in extracardiac cavopulmonary connection: does it really matter?

OBJECTIVES: We examined the effect of avoiding cardiopulmonary bypass on the early outcome variables after fenestrated extracardiac total cavopulmonary connection. METHODS: Between May 2001 and January 2009, 102 patients with univentricular heart physiology underwent fenestrated extracardiac total cavopulmonary connection. Patients were divided into one of 2 groups: the cardiopulmonary bypass (n = 48) group and the no cardiopulmonary bypass (n = 54) group. In both groups there were patients with primary and staged fenestrated extracardiac total cavopulmonary connection. Duration of mechanical ventilation, pleural effusion, hemodynamic status, incidence of arrhythmia, and mortality were compared between the 2 groups. RESULTS: Both groups were matched, except for more cases of tricuspid atresia in the no cardiopulmonary bypass group (P = .014) compared with other diagnostic morphologies and higher preoperative hemoglobin levels in the no cardiopulmonary bypass group (P = .01). Avoiding cardiopulmonary bypass did not reveal any significant effect on postoperative outcomes. A cardiopulmonary bypass time of more than 120 minutes caused not only a meaningful increase in the mean of mechanical ventilation duration (35 +/- 9.6 vs 13 +/- 2.1 hours, P = .026) but also increased the incidence of mechanical ventilation for more than 12 hours (P = .04). Bypass time of more than 120 minutes did not have influence on any other postoperative variables. CONCLUSION: Avoiding cardiopulmonary bypass in fenestrated extracardiac total cavopulmonary connection had no direct effect on the early outcome variables.

Echocardiographic abnormalities and their correlation with bronchiectasis score in primary antibody deficiencies.

BACKGROUND: Primary antibody deficiencies are characterized by defective antibody production and recurrent infections. Patients usually present with recurrent respiratory tract infections with consequent chronic pulmonary damage and bronchiectasis, which could potentially influence cardiac function. Our aim was to assess noninvasively the cardiac complications due to pulmonary disease in patients with primary antibody deficiency. METHODS: A cross-sectional series of patients with primary antibody deficiency syndromes from our referral immunology center were recruited. Individuals undergoing high-resolution computed tomography (HRCT) and transthoracic echocardiography were reviewed. RESULTS: Thirty primary immunodeficient patients aged 5-55 years of age (21 males and 9 females) were enrolled in this study. Half of the patients (50%) were found to have bronchiectasis in HRCT imaging. In echocardiographic examination, 20 patients (67%) had at least one abnormality; among which pulmonary hypertension was the most common (33%). Patients with bronchiectasis had higher pulmonary artery pressures and HRCT bronchiectasis score was strongly correlated with pulmonary artery pressure (regression R = 0.59, P value = 0.001). CONCLUSION: Echocardiographic evaluation of right ventricular function and noninvasive estimation of pulmonary artery pressure could have an important diagnostic role in the follow-up and therapeutic management of patients with primary immune deficiency.

N-terminal pro-B-type natriuretic peptide and ventricular dysfunction in children and adolescents.

Our aim was further to clarify the diagnostic usefulness of N-terminal pro-B-type natriuretic peptide for detecting ventricular dysfunction in children, and its correlation with myocardial performance index and New York University Pediatric Heart Failure Index score. We also hypothesized that the level of this natriuretic peptide in the serum could predict the severity of diastolic abnormalities in children with cardiac failure. We enrolled 99 patients, aged from 3 months to 16 years, who had been referred for echocardiography to evaluate ventricular function. Echocardiographic evidence of left ventricular systolic and diastolic dysfunction was found in 20 and 42 patients, respectively. We classified these patients as having impaired relaxation, seen in 12 patients, pseudonormal patterns seen in 19 patients, and restrictive-like patterns of filling seen in 11 patients. The mean of the log-transformed values for N-terminal pro-B-type natriuretic peptide increased significantly according to the severity of diastolic dysfunction (p = 0.003, p = 0.022, p < 0.0001). A value of 178 pg/ml had a sensitivity of 88% and specificity of 81% for detecting abnormal diastolic function (p < 0.0001). Furthermore, the log-transformed values correlated with myocardial performance index (p < 0.0001) in a positive manner, and the levels increased significantly according to New York University Pediatric Heart Failure Index score, showing a linear correlation with a robust r value for regression (r = 0.89, p < 0.0001). Our findings suggest that higher levels of the peptide, having a good correlation with New York University Pediatric Heart Failure Index score and myocardial performance index, might be a suitable marker to rule out ventricular diastolic dysfunction in children.

Coenzyme Q10 improves diastolic function in children with idiopathic dilated cardiomyopathy.

We aimed to determine the effect of supplementation with coenzyme Q10 on conventional therapy of children with cardiac failure due to idiopathic dilated cardiomyopathy. In a prospective, randomized, double-blinded, placebo-controlled trial, we randomized 38 patients younger than 18 years with idiopathic dilated cardiomyopathy to receive either coenzyme Q10, chosen for 17 patients, or placebo, administered in the remaining 21. Echocardiographic systolic and diastolic function parameters were determined for every patient at baseline, and after 6 months of supplementation. The index score for cardiac failure in children as established in New York was used for assessing the functional class of the patients. After 6 months supplementation, 10 patients randomized to receive coenzyme Q10 showed improvements in the grading of diastolic function, this being significantly more than that achieved by those randomized to the placebo group (p value = 0.011). The mean score for the index of cardiac failure index for those receiving coenzyme Q10 was also lower than the control group (p value = 0.024).Our results, therefore, indicate that administration of coenzyme Q10 is useful in ameliorating cardiac failure in patients with idiopathic dilated cardiomyopathy through its significant effect on improving diastolic function.

The effect of ventricular septal defect enlargement on the outcome of Rastelli or Rastelli-type repair.

OBJECTIVE: Our purpose was to evaluate the effect of ventricular septal defect enlargement on the early and late morbidity and mortality of patients undergoing Rastelli or Rastelli-type operations. METHODS: A total of 49 patients who underwent Rastelli or Rastelli-type operations between 1991 and 2007 were included in a retrospective follow-up study. Patients were divided into 2 groups: group A had ventricular septal defect enlargement, and group B did not have ventricular septal defect enlargement for comparison. Risk factor analysis for early or late death included patient-related and procedure-related variables, with failure, arrhythmia, and atrioventricular block as outcome parameters. RESULTS: Median age and weight at the time of the operation were 6 years (range, 3 months-22 years) and 17 kg (range, 7-48 kg), respectively. The ventricular septal defect was enlarged in 28 (57%) patients. Ventricular septal defect enlargement showed a significant statistical relation with late ventricular dysfunction, arrhythmia, and residual ventricular septal defect (P = .023, P = .047, and P = .01, respectively, log-rank test). No relation was found between ventricular septal defect enlargement and permanent pacemaker implantation (P = .73, log-rank test). Furthermore, enlargement of the ventricular septal defect did not show any significant effect on the rate of early mortality (P = .69, Cox regression). Kaplan-Meier estimated survival for patients with ventricular septal defect enlargement was 74% at 5 years and 65% at 10 years. Freedom from late death in the group without ventricular septal defect enlargement was 100% at 5 and 10 years and 83% at 15 years. At a median follow-up of 4 years (range, 6 months-16 years), there were 12 late-onset deaths: 11 in group A (n = 28) and 1 in group B (n = 21). Ventricular septal defect enlargement greatly increased the risk of late death (P = .009, Cox regression). CONCLUSIONS: Septal resection in patients undergoing Rastelli or Rastelli-type operations has a substantial effect on late morbidity and is a predictive factor for long-term mortality.

Combination of aortopulmonary window and complete atrioventricular septal defect in a patient with heterotaxy syndrome.

A rare combination of aortopulmonary window and complete atrioventricular septal defect diagnosed in a 2-month-old infant with heterotaxy syndrome is presented. Being aware of this combination of cardiac anomalies before surgical intervention is crucial for perioperative anesthetic technique and preservation of the myocardium.

Left atrial ejection force in healthy newborn infants.

BACKGROUND: Atrial ejection force (AEF) expresses the force exerted by the left atrium to the mass of blood passing through the mitral valve during atrial systole. It provides a diagnostic and predictive parameter for evaluating left ventricular diastolic abnormalities and a physiologic assessment of atrial systolic function. METHODS: We obtained normal values of AEF in a group of 47 newborn infants with normal heart function and structure, using Doppler echocardiographic parameters of transmitral filling flow. AEF is defined as the product of the density of blood, the mitral valve area, and the square of peak A velocity [AEF = 0.5 x rho x mitral valve area x (peak A velocity)(2)]. RESULTS: Mean and SD of AEF was 1.12 +/- 0.42 kilodynes. Atrial filling fraction (r = 0.74, P value = .000), A acceleration rate (r = 0.67, P value = .000), A deceleration rate (r = 0.64, P value = .000), and heart rate (r = 0.70, P value = .000) showed a positive correlation with AEF. Rapid filling fraction (r = -0.71, P value = .000) and E/A ratio (r = -0.6, P value = .000) had a negative correlation with AEF. CONCLUSION: AEF index in neonatal period is augmented and comparable with the values in adult population that could be explained by the specific pattern of slow ventricular relaxation in newborn infants. Complex aspects of diastolic function in newborn infants could be assessed beyond a simple E to A ratio by providing an estimate of normal values for AEF in this age group.

Congenital right atrial aneurysm in an infant.

A 4-month-old infant with right atrial aneurysm, presenting with recurrent episodes of lower respiratory tract infection is described. There was no history of supraventricular tachyarrhythmias or embolic events. Transthorasic echocardiography definitely showed an aneurysmal sac (3.7 A-4.2 cm) originating from the right atrium with compressing effect on left side cardiac structures. Surgical excision of aneurysm allowed removal of mass effect and clinical improvement.

Pulmonary artery aneurysm in a child secondary to infective endarteritis.

A 5-year-old boy with main pulmonary artery aneurysm is described with two predisposing factors of patent ductus arteriosus and associated infective endarteritis. Transthorasic echocardiography showed definitely a saccular aneurysm originating from the main pulmonary artery at the site of a previous vegetation. Antibiotic therapy and surgical resection of the aneurysm allowed for clinical improvement.