The Journal of Tehran University Heart Center, Oct 11, 2022
Keutel syndrome (KS) as a scarce autosomal recessive disorder is characterized by hearing loss, m... more 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.
Thrombotic events are very unusual in neonatal period. This congenital anomaly may be caused by c... more Thrombotic events are very unusual in neonatal period. This congenital anomaly may be caused by certain risk factors such as polycythemia, perinatal asphyxia, septicemia, maternal diabetes, dehydration, and low cardiac output, or it may occur upon catheterization of central lines. A 20-day-old neonate was referred to our ward with primary signs of hypertrophic cardiomyopathy (HCMP). Echocardiography and CT-angiography were performed to find stenosis or no stenosis of aortic valve, which showed a great mass into the ascending aorta. This mass was resected and removed successfully and thereafter the aortic arch was repaired. There was no prothrombic disorder in his family history.
noninvasive parameter for comparison and follow-up. Here, we report the result of an echocardiogr... more noninvasive parameter for comparison and follow-up. Here, we report the result of an echocardiographic index of pulsatility in ECTCPC type of Fontan circulation in comparison with biventricular circulation and few cases of pulsatile Fontan. Methods Twenty one patients with fenestrated ECTCPC, 7 patients with pulsatile Fontan and 18 normal individuals were enrolled the study. Pulsatile Fontan group is composed of a limited group of patients with ECTCPC in our center that a restrictive antegrade flow through the main pulmonary artery has been preserved or adjusted. Patients were excluded if they were not in •
Journal of Shahid Sadoughi University of Medical Sciences, Oct 26, 2020
Introduction: Congenital heart diseases (CHD) are the most common congenital anomalies in childre... more Introduction: Congenital heart diseases (CHD) are the most common congenital anomalies in children that can adversely influence children and families mental health, physical performance, and family economic. The aim of present study was to compare the quality of life (QOL) in mothers with children with CHD before and after cardiac surgery in South Khorasan Province (East of Iran). Methods: In this cross sectional study the QOL of 60 mothers with CHD children admitted in Vali-e Asr Hospital were evaluated before and after cardiac surgery in 2017. They were incorporated upon provision of informed consent. The Mac new Heart Disease Health -related quality of life Questionnaire scale was completed by the mothers 10 days before and three months after the surgery. The data were analyzed in SPSS software (version 22) using relative tests. p<0.05 was considered as significant difference. Results: The results of the present study showed that the mean of QOL score were significantly increased from 40.8±2.7 in initial time to 66.50± 2.2 in 3 months after surgery (p<0.001). Conclusion: Cardiac surgery in children with CHD could improve the overall QOL score and all of its subscales (physical health, emotional health and social health) of their parents.
Background: Neonatal thrombotic diseases can cause mortality or serious morbidity and disability.... more Background: Neonatal thrombotic diseases can cause mortality or serious morbidity and disability. Case report: In this report, we present a case of neonatal inferior vena cava thromboembolism with several underlying factors. Hereditary thrombophilia and genetic mutation in plasminogen activator inhibitor-1 and MTHFR A1298C genes in conjunction with cleft palate resulted in poor lactation and hypernatremic dehydration. A peripherally inserted central catheter in the inferior vena cava was an additional underlying factor. Thrombosis mass was dislodged to the right atrium while asymptomatic and accidentally detected during routine echocardiography. Conclusion: Surgical thrombectomy was done successfully, and the mass was removed from the right atrium.
Iranian Journal of Allergy Asthma and Immunology, Sep 2, 2020
Myocarditis is an inflammatory disease of the myocardium with lymphocyte infiltration and myocyte... more 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.
Keutel syndrome (KS) as a scarce autosomal recessive disorder is characterized by hearing loss, m... more 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. Mos...
Background: Treadmill exercise testing is a crucial diagnostic tool for evaluating congenital and... more 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.
Background: Neonatal thrombotic diseases can cause mortality or serious morbidity and disability.... more Background: Neonatal thrombotic diseases can cause mortality or serious morbidity and disability.Case report: In this report, we present a case of neonatal inferior vena cava thromboembolism with several underlyingfactors. Hereditary thrombophilia and genetic mutation in plasminogen activator inhibitor-1 and MTHFR A1298C genesin conjunction with cleft palate resulted in poor lactation and hypernatremic dehydration. A peripherally insertedcentral catheter in the inferior vena cava was an additional underlying factor. Thrombosis mass was dislodged to theright atrium while asymptomatic and accidentally detected during routine echocardiography.Conclusion: Surgical thrombectomy was done successfully, and the mass was removed from the right atrium.
Thrombotic events are very unusual in neonatal period. This congenital anomaly may be caused by c... more Thrombotic events are very unusual in neonatal period. This congenital anomaly may be caused by certain risk factors such as polycythemia, perinatal asphyxia, septicemia, maternal diabetes, dehydration, and low cardiac output, or it may occur upon catheterization of central lines. A 20-day-old neonate was referred to our ward with primary signs of hypertrophic cardiomyopathy (HCMP). Echocardiography and CT-angiography were performed to find stenosis or no stenosis of aortic valve, which showed a great mass into the ascending aorta. This mass was resected and removed successfully and thereafter the aortic arch was repaired. There was no prothrombic disorder in his family history.
Journal of Shahid Sadoughi University of Medical Sciences, 2020
Introduction: Congenital heart diseases (CHD) are the most common congenital anomalies in childre... more Introduction: Congenital heart diseases (CHD) are the most common congenital anomalies in children that can adversely influence children and families mental health, physical performance, and family economic. The aim of present study was to compare the quality of life (QOL) in mothers with children with CHD before and after cardiac surgery in South Khorasan Province (East of Iran). Methods: In this cross sectional study the QOL of 60 mothers with CHD children admitted in Vali-e Asr Hospital were evaluated before and after cardiac surgery in 2017. They were incorporated upon provision of informed consent. The Mac new Heart Disease Health -related quality of life Questionnaire scale was completed by the mothers 10 days before and three months after the surgery. The data were analyzed in SPSS software (version 22) using relative tests. p<0.05 was considered as significant difference. Results: The results of the present study showed that the mean of QOL score were significantly increa...
COVID-19 as a viral infection is usually asymptomatic in children, but complication of this disea... more COVID-19 as a viral infection is usually asymptomatic in children, but complication of this disease in children is not rare and may be fatal. Hyper inflammation of COVID-19 is a potential fatal in undiagnosed children. It is very similar to Kawasaki disease but with higher mortality and morbidity. Multisystem inflammatory syndrome in children (MIS-C) and multi-organ involvement was reported in hyper inflammation syndrome following COVID19. Herein we report our algorithmic approach to Kawasaki-like syndromes due to COVID-19 in our center. Based-on this approach we had no mortality during the last 5 months.
Iranian Journal of Allergy, Asthma and Immunology, 2020
Myocarditis is an inflammatory disease of the myocardium with lymphocyte infiltration and myocyte... more 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...
Catecholaminergic polymorphic ventricular tachycardia (CPVT) is an uncommon heritable disease pre... more Catecholaminergic polymorphic ventricular tachycardia (CPVT) is an uncommon heritable disease presenting with syncope or sudden cardiac death. Two genes involved in calcium homeostasis, the ryanodine receptor gene and the calsequestrin 2 (CASQ2) gene, have been implicated in this disease. We describe a young man presenting with exercise‐induced syncope, clinically diagnosed as CPVT. Genetic analysis revealed two mutations, p.Y55C (c.164A>G) and p.P308L (c.923C>T), in the CASQ2 gene. Subsequent familial analysis indicates a compound heterozygous form of inheritance.
The time interval between tricuspid valve closure and pulmonary valve opening, termed the isovolu... more The time interval between tricuspid valve closure and pulmonary valve opening, termed the isovolumic contraction time of the right ventricle, was evaluated echographically in 38 normal children and within 24 hours of cardiac catheterization in 53 children with congenital heart disease and normal conduction as assessed with the electrocardiogram. In the 53 patients with congenital heart disease, isovolumic contraction time was strongly influenced by right ventricular afterload, as defined by pulmonary arterial end-diastolic pressure (r = 0.87). It was possible to utilize isovolumic contraction time to separate patients with normal or elevated values for pulmonary arterial end-diastolic pressure. Similar correlations were demonstrated between isovolumic contraction time and mean pulmonary arterial pressure and calculated pulmonary vascular resistance. Evaluation of 15 children with complete right bundle branch block revealed values for isovolumic contraction time that did not significantly differ from those of patients with similar pulmonary arterial end-diastolic pressure but no conduction abnormalities. These findings indicate that serial echographic evaluation of the interval from tricuspid valve closure to pulmonary valve opening can give an accurate reproducible assessment of right ventricular afterload in many children with congenital heart disease and complete right bundle branch block
We describe two newborn infants with aortic arch obstructive malformations who became anuric afte... more We describe two newborn infants with aortic arch obstructive malformations who became anuric after initiation of captopril. Since angiotensin converting enzyme inhibitors can alter renal blood flow by reduction in angiotensin II and blocking autoregulation phenomenon, it is important to use them with great caution in neonates with aortic arch obstructive malformations, while monitoring their renal function closely.
Background: Despite the great progress in the surgery of complex congenital heart diseases, there... more Background: Despite the great progress in the surgery of complex congenital heart diseases, there is still concern regarding adverse neurological events. We aimed to determine the pre- and on-pump modifiable factors that could predict the neurocomplications after pediatric cardiac surgery.Methods: In a prospective study, modifiable factors such as arterial blood gas, serum lactate, serum glucose, mean arterial pressure, and nasopharyngeal temperature were measured before and during cardiopulmonary bypass (CPB). Moreover, the CPB time, the aortic cross-clamp time, and the deep hypothermic circulatory arrest time were recorded. If there were adverse neurological complications, brain computed tomography scan was done.Results: From 435 patients with congenital heart diseases that underwent cardiac surgery, 364 patients at a mean age of 22 months were enrolled in the study. Thirty-three (9%) patients had adverse early-onset neurological events. Seizure and hemorrhage were the most common...
BACKGROUND: Adding pulsation to the Fontan circulation might change the fate of patients palliate... more 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.
Context: Tetralogy of Fallot (TOF) is one of the most important congenital heart diseases that it... more Context: Tetralogy of Fallot (TOF) is one of the most important congenital heart diseases that its prognosis can be improved by surgery. However, Right Ventricular (RV) function may be disrupted because of pulmonary regurgitation and other preoperative and intraoperative factors. Access to a simple and reproducible diagnostic index of RV function is important. Echocardiographic index of Right Ventricular Myocardial Performance (RVMPI) has been used as a simple and noninvasive method for the assessment of RV function. Evidence Acquisition: This article review was conducted utilizing PubMed, Web of Science, Scopus and Google Scholar databases with the keywords of "Right Ventricle (RV)", "Myocardial Performance Index (MPI)", and "Tetralogy of Fallot (TOF) repair or correction". Results: RVMPI as a non-geometric echocardiographic index was compared with other echocardiographic indices, QRS duration in ECG, Cardiac Magnetic Resonance (CMR) findings, and exercise capacity in the pediatric and adult studies. Conclusions: This narrative review suggested that RVMPI, especially the tissue Doppler-derived has been used as a useful index of RV function in the follow-up of the repaired TOF patients. However, more research, including systematic reviews are necessary to determine the potential implication of RVMPI in the assessment of RV dysfunction.
The Journal of Tehran University Heart Center, Oct 11, 2022
Keutel syndrome (KS) as a scarce autosomal recessive disorder is characterized by hearing loss, m... more 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.
Thrombotic events are very unusual in neonatal period. This congenital anomaly may be caused by c... more Thrombotic events are very unusual in neonatal period. This congenital anomaly may be caused by certain risk factors such as polycythemia, perinatal asphyxia, septicemia, maternal diabetes, dehydration, and low cardiac output, or it may occur upon catheterization of central lines. A 20-day-old neonate was referred to our ward with primary signs of hypertrophic cardiomyopathy (HCMP). Echocardiography and CT-angiography were performed to find stenosis or no stenosis of aortic valve, which showed a great mass into the ascending aorta. This mass was resected and removed successfully and thereafter the aortic arch was repaired. There was no prothrombic disorder in his family history.
noninvasive parameter for comparison and follow-up. Here, we report the result of an echocardiogr... more noninvasive parameter for comparison and follow-up. Here, we report the result of an echocardiographic index of pulsatility in ECTCPC type of Fontan circulation in comparison with biventricular circulation and few cases of pulsatile Fontan. Methods Twenty one patients with fenestrated ECTCPC, 7 patients with pulsatile Fontan and 18 normal individuals were enrolled the study. Pulsatile Fontan group is composed of a limited group of patients with ECTCPC in our center that a restrictive antegrade flow through the main pulmonary artery has been preserved or adjusted. Patients were excluded if they were not in •
Journal of Shahid Sadoughi University of Medical Sciences, Oct 26, 2020
Introduction: Congenital heart diseases (CHD) are the most common congenital anomalies in childre... more Introduction: Congenital heart diseases (CHD) are the most common congenital anomalies in children that can adversely influence children and families mental health, physical performance, and family economic. The aim of present study was to compare the quality of life (QOL) in mothers with children with CHD before and after cardiac surgery in South Khorasan Province (East of Iran). Methods: In this cross sectional study the QOL of 60 mothers with CHD children admitted in Vali-e Asr Hospital were evaluated before and after cardiac surgery in 2017. They were incorporated upon provision of informed consent. The Mac new Heart Disease Health -related quality of life Questionnaire scale was completed by the mothers 10 days before and three months after the surgery. The data were analyzed in SPSS software (version 22) using relative tests. p&amp;amp;amp;lt;0.05 was considered as significant difference. Results: The results of the present study showed that the mean of QOL score were significantly increased from 40.8±2.7 in initial time to 66.50± 2.2 in 3 months after surgery (p&amp;amp;amp;lt;0.001). Conclusion: Cardiac surgery in children with CHD could improve the overall QOL score and all of its subscales (physical health, emotional health and social health) of their parents.
Background: Neonatal thrombotic diseases can cause mortality or serious morbidity and disability.... more Background: Neonatal thrombotic diseases can cause mortality or serious morbidity and disability. Case report: In this report, we present a case of neonatal inferior vena cava thromboembolism with several underlying factors. Hereditary thrombophilia and genetic mutation in plasminogen activator inhibitor-1 and MTHFR A1298C genes in conjunction with cleft palate resulted in poor lactation and hypernatremic dehydration. A peripherally inserted central catheter in the inferior vena cava was an additional underlying factor. Thrombosis mass was dislodged to the right atrium while asymptomatic and accidentally detected during routine echocardiography. Conclusion: Surgical thrombectomy was done successfully, and the mass was removed from the right atrium.
Iranian Journal of Allergy Asthma and Immunology, Sep 2, 2020
Myocarditis is an inflammatory disease of the myocardium with lymphocyte infiltration and myocyte... more 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.
Keutel syndrome (KS) as a scarce autosomal recessive disorder is characterized by hearing loss, m... more 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. Mos...
Background: Treadmill exercise testing is a crucial diagnostic tool for evaluating congenital and... more 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.
Background: Neonatal thrombotic diseases can cause mortality or serious morbidity and disability.... more Background: Neonatal thrombotic diseases can cause mortality or serious morbidity and disability.Case report: In this report, we present a case of neonatal inferior vena cava thromboembolism with several underlyingfactors. Hereditary thrombophilia and genetic mutation in plasminogen activator inhibitor-1 and MTHFR A1298C genesin conjunction with cleft palate resulted in poor lactation and hypernatremic dehydration. A peripherally insertedcentral catheter in the inferior vena cava was an additional underlying factor. Thrombosis mass was dislodged to theright atrium while asymptomatic and accidentally detected during routine echocardiography.Conclusion: Surgical thrombectomy was done successfully, and the mass was removed from the right atrium.
Thrombotic events are very unusual in neonatal period. This congenital anomaly may be caused by c... more Thrombotic events are very unusual in neonatal period. This congenital anomaly may be caused by certain risk factors such as polycythemia, perinatal asphyxia, septicemia, maternal diabetes, dehydration, and low cardiac output, or it may occur upon catheterization of central lines. A 20-day-old neonate was referred to our ward with primary signs of hypertrophic cardiomyopathy (HCMP). Echocardiography and CT-angiography were performed to find stenosis or no stenosis of aortic valve, which showed a great mass into the ascending aorta. This mass was resected and removed successfully and thereafter the aortic arch was repaired. There was no prothrombic disorder in his family history.
Journal of Shahid Sadoughi University of Medical Sciences, 2020
Introduction: Congenital heart diseases (CHD) are the most common congenital anomalies in childre... more Introduction: Congenital heart diseases (CHD) are the most common congenital anomalies in children that can adversely influence children and families mental health, physical performance, and family economic. The aim of present study was to compare the quality of life (QOL) in mothers with children with CHD before and after cardiac surgery in South Khorasan Province (East of Iran). Methods: In this cross sectional study the QOL of 60 mothers with CHD children admitted in Vali-e Asr Hospital were evaluated before and after cardiac surgery in 2017. They were incorporated upon provision of informed consent. The Mac new Heart Disease Health -related quality of life Questionnaire scale was completed by the mothers 10 days before and three months after the surgery. The data were analyzed in SPSS software (version 22) using relative tests. p<0.05 was considered as significant difference. Results: The results of the present study showed that the mean of QOL score were significantly increa...
COVID-19 as a viral infection is usually asymptomatic in children, but complication of this disea... more COVID-19 as a viral infection is usually asymptomatic in children, but complication of this disease in children is not rare and may be fatal. Hyper inflammation of COVID-19 is a potential fatal in undiagnosed children. It is very similar to Kawasaki disease but with higher mortality and morbidity. Multisystem inflammatory syndrome in children (MIS-C) and multi-organ involvement was reported in hyper inflammation syndrome following COVID19. Herein we report our algorithmic approach to Kawasaki-like syndromes due to COVID-19 in our center. Based-on this approach we had no mortality during the last 5 months.
Iranian Journal of Allergy, Asthma and Immunology, 2020
Myocarditis is an inflammatory disease of the myocardium with lymphocyte infiltration and myocyte... more 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...
Catecholaminergic polymorphic ventricular tachycardia (CPVT) is an uncommon heritable disease pre... more Catecholaminergic polymorphic ventricular tachycardia (CPVT) is an uncommon heritable disease presenting with syncope or sudden cardiac death. Two genes involved in calcium homeostasis, the ryanodine receptor gene and the calsequestrin 2 (CASQ2) gene, have been implicated in this disease. We describe a young man presenting with exercise‐induced syncope, clinically diagnosed as CPVT. Genetic analysis revealed two mutations, p.Y55C (c.164A>G) and p.P308L (c.923C>T), in the CASQ2 gene. Subsequent familial analysis indicates a compound heterozygous form of inheritance.
The time interval between tricuspid valve closure and pulmonary valve opening, termed the isovolu... more The time interval between tricuspid valve closure and pulmonary valve opening, termed the isovolumic contraction time of the right ventricle, was evaluated echographically in 38 normal children and within 24 hours of cardiac catheterization in 53 children with congenital heart disease and normal conduction as assessed with the electrocardiogram. In the 53 patients with congenital heart disease, isovolumic contraction time was strongly influenced by right ventricular afterload, as defined by pulmonary arterial end-diastolic pressure (r = 0.87). It was possible to utilize isovolumic contraction time to separate patients with normal or elevated values for pulmonary arterial end-diastolic pressure. Similar correlations were demonstrated between isovolumic contraction time and mean pulmonary arterial pressure and calculated pulmonary vascular resistance. Evaluation of 15 children with complete right bundle branch block revealed values for isovolumic contraction time that did not significantly differ from those of patients with similar pulmonary arterial end-diastolic pressure but no conduction abnormalities. These findings indicate that serial echographic evaluation of the interval from tricuspid valve closure to pulmonary valve opening can give an accurate reproducible assessment of right ventricular afterload in many children with congenital heart disease and complete right bundle branch block
We describe two newborn infants with aortic arch obstructive malformations who became anuric afte... more We describe two newborn infants with aortic arch obstructive malformations who became anuric after initiation of captopril. Since angiotensin converting enzyme inhibitors can alter renal blood flow by reduction in angiotensin II and blocking autoregulation phenomenon, it is important to use them with great caution in neonates with aortic arch obstructive malformations, while monitoring their renal function closely.
Background: Despite the great progress in the surgery of complex congenital heart diseases, there... more Background: Despite the great progress in the surgery of complex congenital heart diseases, there is still concern regarding adverse neurological events. We aimed to determine the pre- and on-pump modifiable factors that could predict the neurocomplications after pediatric cardiac surgery.Methods: In a prospective study, modifiable factors such as arterial blood gas, serum lactate, serum glucose, mean arterial pressure, and nasopharyngeal temperature were measured before and during cardiopulmonary bypass (CPB). Moreover, the CPB time, the aortic cross-clamp time, and the deep hypothermic circulatory arrest time were recorded. If there were adverse neurological complications, brain computed tomography scan was done.Results: From 435 patients with congenital heart diseases that underwent cardiac surgery, 364 patients at a mean age of 22 months were enrolled in the study. Thirty-three (9%) patients had adverse early-onset neurological events. Seizure and hemorrhage were the most common...
BACKGROUND: Adding pulsation to the Fontan circulation might change the fate of patients palliate... more 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.
Context: Tetralogy of Fallot (TOF) is one of the most important congenital heart diseases that it... more Context: Tetralogy of Fallot (TOF) is one of the most important congenital heart diseases that its prognosis can be improved by surgery. However, Right Ventricular (RV) function may be disrupted because of pulmonary regurgitation and other preoperative and intraoperative factors. Access to a simple and reproducible diagnostic index of RV function is important. Echocardiographic index of Right Ventricular Myocardial Performance (RVMPI) has been used as a simple and noninvasive method for the assessment of RV function. Evidence Acquisition: This article review was conducted utilizing PubMed, Web of Science, Scopus and Google Scholar databases with the keywords of "Right Ventricle (RV)", "Myocardial Performance Index (MPI)", and "Tetralogy of Fallot (TOF) repair or correction". Results: RVMPI as a non-geometric echocardiographic index was compared with other echocardiographic indices, QRS duration in ECG, Cardiac Magnetic Resonance (CMR) findings, and exercise capacity in the pediatric and adult studies. Conclusions: This narrative review suggested that RVMPI, especially the tissue Doppler-derived has been used as a useful index of RV function in the follow-up of the repaired TOF patients. However, more research, including systematic reviews are necessary to determine the potential implication of RVMPI in the assessment of RV dysfunction.
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Papers by Reza Shabanian