Echocardiographic estimation of pulmonary arterial and right atrial pressures in children with congenital heart disease: a comprehensive prospective study and introduction of novel equations.

BACKGROUND: Pediatric pulmonary hypertension (PH) is characterized by a mean pulmonary arterial pressure exceeding 20 mmHg. There is limited research on the suitability of adult-based methods for estimating PH in pediatric populations. Using established formulas for adults, this study aimed to evaluate the correlation between echocardiographic estimates of systolic, diastolic, and mean pulmonary arterial pressures, and mean right atrial pressures in children with congenital heart disease (CHD). METHODS: A prospective study was conducted involving children with CHD undergoing cardiac catheterization without prior cardiac surgery. We used echocardiography to estimate pulmonary and right atrial pressures and compared these with invasively measured values. Four reliable regression equations were developed to estimate systolic, diastolic, and mean pulmonary arterial pressures, and mean right atrial pressures. Cutoff values were determined to predict the occurrence of PH. Linear regression, Bland-Altman analysis, and receiver operating characteristic curve analysis were performed to assess the accuracy of echocardiography and establish diagnostic thresholds for PH. RESULTS: The study involved 55 children (23 with normal pulmonary arterial pressure and 32 with PH) with acyanotic CHD aged 1 to 192 months. Four equations were developed to detect high pulmonary arterial pressures, with cutoff values of 32.9 for systolic pulmonary arterial pressure, 14.95 for diastolic pulmonary arterial pressure, and 20.7 for mean pulmonary arterial pressure. The results showed high sensitivity and moderate specificity but a tendency to underestimate systolic and mean pulmonary arterial pressures at higher pressures. CONCLUSIONS: The study provides valuable insights into the use of adult-based echocardiographic formulas for estimating PH in pediatric patients with acyanotic CHD.

COVID-19-Associated Acute Necrotizing Encephalopathy: A Case Report.

COVID-19 is a pandemic disease in which most patients have pulmonary symptoms. However, several cases of CNS involvement associated with COVID-19 have been reported. Acute necrotizing encephalopathy of childhood (ANEC) is a rare CNS complication of viral infections such as influenza, herpes virus, and COVID-19, leading to high mortality and morbidity rates. Several cases of COVID-19-associated acute necrotizing encephalopathy (ANE) have been reported since March 2020 in adults, with just a few cases in pediatrics. This article reports a 5-month-old child who presented with seizures, with the final diagnosis of ANE as a complication of COVID-19. MRI findings of ANEC, as reported in most COVID-19-associated ANEC case reports, involve bilateral, symmetric, multifocal lesions in the central thalami. Moreover, the brainstem, cerebral white matter, and cerebellum could be affected. The prognosis of COVID-19-associated ANE is poor, leading to neurologic dysfunction or mortality. COVID-19-associated ANE cases must be reported, especially in pediatrics, with detailed clinical history, laboratory data, and radiologic findings to introduce diagnostic criteria, prognosis, and a management protocol.

Coronary artery involvement in a patient with IgG4-related disease.

Immunoglobulin G4-related disease (IgG4-RD) is a chronic fibro-inflammatory disorder of obscure etiology characterized by significant infiltration of IgG4-positive plasma cells toward several organs. Coronary artery involvement is rarely seen in IgG4-RD patients; thereby, we aim to outline the noninvasive imaging findings of this rare case. Cardiac magnetic resonance (CMR) and coronary computed tomography angiography (CCTA) from a 15-year-old female diagnosed with IgG4-RD via histopathological assessment of orbital biopsy, were analyzed. CMR showed a severely reduced left ventricular ejection fraction and akinesia of the basal to mid-lateral, anterior, and septal walls. Inflammation of the basal to apical lateral wall and subendocardial infarction of the basal to apical lateral and mid inferoseptal walls were also evident. CCTA findings showed stenosis in branches of the left main artery (LM), left anterior descending artery (LAD), and right coronary artery (RCA), aortitis, and aortic wall thickening. After courses of proper treatment with prednisolone, Cellcept, and adalimumab, follow-up CMR showed significant improvement in LV systolic function and resolution of inflammation. Although IgG4-RD is an uncommon cause of coronary artery disease, it can cause lethal complications such as myocardial infarction. Hence, clinicians should be aware of cardiac complications in these patients.

Pulsatility Index in Different Modifications of Fontan Palliation: An Echocardiographic Assessment.

BACKGROUND: Adding pulsation to the Fontan circulation might change the fate of patients palliated by this procedure. Our aim was to compare the pulsatility index (PI) of the pulmonary artery (PA) between the various modifications of Fontan palliation. METHODS: Doppler-derived PI was measured in PA branches of a cohort of 28 patients palliated by 6 modifications of Fontan procedure. A group of normal individuals was included for comparison. RESULTS: Atriopulmonary connection (APC) group had the highest PA branches PI and statistically was close to the PI of the normal individuals (right pulmonary artery [RPA] PI of 1.58 vs. 1.63; p = 0.99 and left pulmonary artery [LPA] PI of 1.54 vs. 1.68; p = 0.46, respectively). The lowest PA branches PI was seen in the group of extracardiac total cavopulmonary connection (RPA PI of 0.62 and LPA PI of 0.65). Other 4 modifications including the extracardiac conduit with oversewn pulmonary valve, extracardiac conduit with preserved adjusted antegrade flow, extracardiac conduit from inferior vena cava onto the rudimentary right ventricle and lateral tunnel had a mean "RPA and LPA" PI of "1.19 and 1.17", "1.16 and 1.11", "1.13 and 1.11", "0.82 and 0.84", respectively. The modified Dunnett's post hoc test has shown a significant statistical decline in PI of all modifications compared to the normal individuals except for the APC group. CONCLUSIONS: Fontan palliated patients in different groups of surgical modification showed a spectrum of Doppler-derived PI with the highest amounts belong to the groups of pulsatile Fontan.

Prenatal diagnosis of accessory mitral valve tissue in a fetus with persistent dysrhythmia.

BACKGROUND: Accessory mitral valve tissue (AMVT) is a rare congenital cardiac anomaly that mainly diagnosed in the first decade of life. However, asymptomatic cases may not be diagnosed even up to adulthood. We report a fetus with AMVT to show the diagnostic ability of the fetal echocardiography for detection of this pathology in the prenatal period. CASE PRESENTATION: AMVT was diagnosed in a 26-week-old male fetus with persistent dysrhythmia. Dysrhythmia could not be aborted and controlled by sotalol till the third trimester evaluation. Apical left ventricular (LV) diverticulum was the additional finding in his fetal echocardiogram. After birth, he was in sinus rhythm and echocardiography confirmed the presence of AMVT, however, without any evidence of LV apical diverticulum. CONCLUSIONS: The diagnosis of AMVT in the prenatal period is possible by fetal echocardiography.

Color tissue doppler imaging of tricuspid annular plane systolic and diastolic excursion in children: A comparison of normal, volume-overloaded and pressure overloaded right ventricles.

BACKGROUND: Tricuspid annular plane (TAP) systolic excursion (TAPSE) is a reproducible M-mode parameter for the measurement of longitudinal shortening of the right ventricle (RV). To date, all attention has been focused on the systolic excursion of TAP and the diastolic excursion of the annular plane back to the base has been ignored. This study aims to compare the quantitative (excursion, slope, and duration) and qualitative (velocity, acceleration, and indentation) characteristics of TAP systolic and diastolic excursion, using color tissue Doppler imaging, in three groups of children with normal RV (NORV), volume overloaded RV (VORV), and pressure overloaded RV (PORV) and normal pulmonary arterial pressure. SUBJECTS AND METHODS: A prospective case-control study was performed in three groups of children with normal heart, VORV and PORV. TAPSE and tricuspid annular plane diastolic excursion (TAPDE) were quantitatively and qualitatively analyzed and compared between the three groups. Statistical analysis was performed using IBM SPSS Statistics for Windows. RESULTS: TAPSE, TAPDE, TAPSE slope, TAPSE slope/TAPDE slope, TAPDE duration and TAPDE duration/RR interval were lower in PORV (TAPSE: PORV: 14.45 ± 4.30, NORV: 20.45 ± 5.46, P = .003, TAPDE:PORV: 14.39 ± 4.61, NORV: 20.28 ± 5.65, P = .004, TAPSE slope:PORV: 4.79 ± 1.40, NORV: 7.15 ± 1.98, P = .001, .001, TAPDE duration:PORV: 201.1 ± 87.9 ms, NORV: 292.1 ± 97.9, P = .006, TAPDE duration/RR interval: PORV: .37 ± .09, NORV: .48 ± .08, P = .0002). CONCLUSION: Pressure-overload on RV produced more impairment of TAPSE and TAPDE patterns than volume overload. Values of TAPSE and TAPDE in patients with VORV and PORV stay in two ends of the normal spectrum. The harmful impact of pre-tricuspid volume overload seems to be less than the post-tricuspid volume overload.

Tracheobronchial Tree Ossification in a 5-Year-Old Boy with Keutel Syndrome: A Case Report.

Keutel syndrome (KS) as a scarce autosomal recessive disorder is characterized by hearing loss, multiple peripheral pulmonary stenoses, abnormal cartilage calcification, and morphological defects including midface hypoplasia and brachytelephalangism. We herein describe a 5-year-old boy who was referred for the evaluation of incidentally auscultated heart murmurs. He had no obvious abnormalities at birth but suffered from recurrent episodes of infectious otitis media during infancy. Physical examination revealed facial abnormalities, such as a broad nasal bridge, a sloping forehead, maxillary hypoplasia, and brachytelephalangism. Chest radiography showed tracheobronchial tree calcification. Transthoracic echocardiography illustrated peripheral pulmonary artery stenosis, moderate tricuspid regurgitation, and pulmonary hypertension. Computed tomography angiography confirmed calcification and segmental stenosis in the peripheral pulmonary arteries. The patient was diagnosed with KS. Most of these patients have a good prognosis. During the follow-up of these patients and examinations, we should pay attention to their symptoms related to upper respiratory tract infections, the extent of hearing, and the possibility of tracheal and pulmonary artery stenosis development. KS is a disease with a good prognosis, and a careful initial examination of babies, including facial appearance and heart auscultation, may lead to the early diagnosis of this disease.

Exercise-induced electrocardiographic changes after treadmill exercise testing in healthy children: A comprehensive study.

Background: Treadmill exercise testing is a crucial diagnostic tool for evaluating congenital and acquired heart disease in the pediatric population. This study aimed to perform a comprehensive evaluation of exercise-induced electrocardiographic (ECG) changes in children. Although there are numerous studies on exercise testing in various cardiac pathologies, studies on exercise-induced ECG changes in normal children with coverage of all ECG parameters of atrial and ventricular depolarization and repolarization are very scant, if any. Aims and Objectives: This study aimed to investigate the exercise-induced ECG changes in healthy children and evaluate the effects of gender and four different formulas of heart rate correction of Bazett, Fridericia, Framingham and Hodges on ventricular repolarization parameters pre-and post-exercise. Materials and Methods: Between April 2019 and April 2020, all children with normal electrocardiogram, echocardiogram and exercise test, high-quality ECG tracings and consent for participation were enrolled in this prospective study. Twenty electrocardiographic parameters were measured and 25 indices were calculated. P-value < 0.05 was considered significant. Results: Seventy-four healthy children were studied. Amplitudes of P, S, and T waves increased significantly after the exercise. All durations, except P wave time to peak and T peak -T end /QT (Tp-e/QT) interval decreased significantly with exercise. Generally, the parameters of ventricular repolarization were not statistically significant between males and females. There were significant differences among the heart-rate corrected values of intervals of QTc, QoTc, JTc, J point to peak T and Tp-e/QTc by various formulas. There was no U wave either at pre-exercise or post-exercise. QT interval was shortened by 24.6 % ± 12.1 % with exercise. The ECG-derived estimated duration of mechanical systole and diastole decreased with exercise. The percentage of decrease in diastole was more than systole (43.79 %± 13.31% versus 33.74% ±15.79 %, respectively, P-value < 0.001). Conclusion: Diastolic time decreased more than systolic time with exercise and systolic time to diastolic time increased with exercise. Hodges' and Fridericia's formulas resulted in the longest and shortest QT and QoT, JT, and JTP, respectively. Thus, using a single value as the cut-off for long QT syndrome can lead to under or over-diagnosis. Nomograms incorporating data on age, heart rate, and heart rate correction formula are indispensable for accurate long QT diagnosis. Furthermore, gender differences in ventricular repolarization parameters are not generally present in 5 to 14-year-old healthy children. The lack of U wave in this study may implicate the need for more careful investigation in the presence of U wave in the treadmill exercise testing of healthy children.

Acute aortic dissection in a patient with Williams syndrome infected by COVID-19.

Cardiovascular involvement in COVID-19 has different features. Here we report the ominous fate of a neglected adolescent with Williams syndrome that was infected by SARS-CoV-2 and ended by acute aortic dissection.

A case of catecholaminergic polymorphic ventricular tachycardia masquerading as an intractable seizure.

A 5-year-old boy with the history of intractable seizure for the past 2 years was transferred to the emergency room for cardiopulmonary resuscitation because of the prolonged seizure and profound cyanosis. He was intubated and resuscitated by cardioversion for a bizarre shape ventricular tachycardia (VT). After noxious stimulation, he showed multiple polymorphic ventricular premature beats that were followed by a bidirectional VT in favor of catecholaminergic polymorphic VT. The genetic assessment was positive for CASQ2 mutation. In the follow-up, the arrhythmia was controlled by nadolol, however with a prominent neurological sequela.

Fresh frozen plasma prime and the level of gammaglobulin after pediatric cardiopulmonary bypass.

Different organ perturbation and multiple complications might occur after cardiopulmonary bypass (CPB). A variety of solutions might be used for pump priming with different advantages and disadvantages. The advantage of fresh frozen plasma (FFP) inclusion in pump prime has been shown in post-CPB coagulation management. Acquired hypogammaglobulinemia is the disadvantage of albumin (ALB) pump prime. Our aim was to assess the impact of FFP prime on the post-pump serum level of immunoglobulin G (IgG) and its subclasses. Fifty-six patients under the age of 5 years old who were scheduled for cardiac surgery on CPB were randomly primed with FFP or ALB. Any innate or acquired immune deficiency was considered as exclusion criteria. The pre-CPB and 24-hour post-CPB collected blood samples were analyzed by the nephelometric method for the plasma level of IgG and its four subclasses. Twenty-two patients (mean age and weight of 13 months and 6.8 kilograms) in the ALB prime group and 26 patients (mean age and weight of 15 months and 8.1 kilograms) in the FFP prime group completed the study. Using paired t-test and repeated measures ANOVA test, patients in the ALB prime group had a significant drop in the post-CPB serum level of total IgG (597±138 mg/dL to 379±179 mg/dL, P value <0.001) and its two subclasses of IgG1 and IgG3. In contrast, there was a slight elevation in the serum level of total IgG (549±207 mg/dL to 630±180 mg/dL, P value =0.008) and its two subclasses of IgG2 and IgG4 in patients who had FFP prime solution. In conclusion, compared to the ALB prime solution, FFP inclusion in prime could hamper the pediatric post-CPB induced hypogammaglobulinemia.

Intravenous Immunoglobulin Therapy in Myocarditis.

Myocarditis is an inflammatory disease of the myocardium with lymphocyte infiltration and myocyte necrosis leading to a wide range of clinical presentations including heart failure, arrhythmia, and cardiogenic shock. Infectious and noninfectious agents may trigger the disease. The fact that immunosuppressive drugs are useful in several kinds of autoimmune myocarditis is proof of the autoimmune mechanisms involved in the development of myocarditis. Pathogenic mechanisms in myocardial inflammation are including inflammasome activation followed by myocyte destruction, myocarditis, and pericarditis. Intravenous immunoglobulin (IVIG) is a serum product made up of immunoglobulins, widely used in a variety of diseases. This product is effective in several immune-mediated pathologies. As well as the determined usage of IVIG in Kawasaki disease, IVIG may be useful in several kinds of heart failure including fulminant myocarditis, acute inflammatory cardiomyopathy, Giant Cell Myocarditis, and peripartum cardiomyopathy. Generally, IVIG is used in two different doses of low dose (200 to 400 mg/kg) and high dose (2 g/kg) regimen. The exact therapeutic effects of IVIG are not clear, however over the last decades, our knowledge about its mechanism of function has greatly enhanced. IVIG administration should be based on the accepted protocols of its transfusion. In this review article, we try to provide an overview of the different kinds of myocarditis, pathologic mechanisms and their common treatments and evaluation of the administration of IVIG in these diseases. Furthermore, we will review current protocols using IVIG in each disease individually.

Echocardiographic Assessment of Pulmonary Arteries Pulsatility Index in Fontan Circulation.

BACKGROUND: Late complications after Fontan procedure may be due to the absence of pump and pulsatile pulmonary blood flow in this type of palliation. Our aim was to quantify the degree of pulsation by echocardiographic method in patients with extracardiac total cavopulmonary connection (ECTCPC) in comparison with biventricular circulation and few cases of pulsatile Fontan. METHODS: In a case series study, pulsatility index (PI) derived by echocardiographic method were compared between 20 patients with ECTCPC, 6 patients with pulsatile Fontan and 18 normal individual aged 4 to 20 years old. All patients were in New York Heart Association class of I and there was no report of complication. RESULTS: In patients with ECTCPC pulmonary artery branches Doppler flow study showed lower peak and mean velocities compared to the pulsatile Fontan and normal groups. ECTCPC patients had PI of 0.59 ± 0.14 and 0.59 ± 0.09 for right and left pulmonary arteries (RPA and LPA) respectively. PI was higher in patients with preserved antegrade flow (RPA PI = 0.94 ± 0.26, LPA PI = 0.98 ± 0.27) and in normal individuals (RPA PI = 1.59 ± 0.12, LPA PI = 1.64 ± 0.17) for both branches (p = 0.000). CONCLUSION: Using a Doppler derived index for pulsatility, patients with ECTCPC had the least pulsation. The pulmonary artery flow pattern in patients with preserved antegrade flow showed higher pulsatility indices in both branches. Normal individuals had the greatest pulsatility index.

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.

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.

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.

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.