جستجوی مقالات مرتبط با کلیدواژه « congenital heart defect » در نشریات گروه « پزشکی »

Diagnosis and treatment of ventricular septal defect (VSD) during infancy is of great importance in improving the infant’s health. Patent ductus arteriosus (PDA) and coarctation of the aorta are the most common disorders associated with VSD. Patent ductus arteriosus needs to be closed using surgical or non-surgical procedures. This article aims to present a case of repairing coarctation of the aorta by stenting inaninfant withVSDdueto the placement of a large Amplatzer during ductus arteriosus closure.

The patient was a one-and-a-half-year-old girl with Down syndrome and congenital heart disease. In the initial examination, a second loud heart sound and a systolic ejection murmur could be heard at the left sternal border. The results of the previous echocardiography showed the presence of VSD, PDA, and coarctationof the aorta. She underwent angioplasty a fewmonths ago, and since the coarctation had not been repaired by balloon aortoplasty due to a large amplatzer. Therefore, the surgeon could not repair the VSD because of uncorrected pulmonary hypertension. Hence, we decided to repair the coarctation by stenting. After two weeks of medication treatment, the patient underwent angiography, and a formula stent placement was done for the patient.

Pulmonary artery blood flow correction is an effective factor in treating patients with VSD that can be achieved by using the correct amplatzer placement. We corrected the aortic coarctation caused by improper amplatzer placement using stenting.

Despite the recent experiences and technical advances in the repair of congenital ventricular septal defects (VSDs), there is still the possibility of postoperative complications and even death among patients undergoing treatment. This study aimed to determine and compare the short-term complications of congenital anomaly (VSDs) repair using the pericardial patch and GORE-TEX patch.
 

This cohort study evaluated 100 patients undergoing VSD repair surgery in 2 repair groups of the pericardial patch (50 patients) and the GORE-TEX patch (50 patients) in Rajaie Cardiovascular Medical and Research Center between 2019 and 2020. All the patients’ information was recorded in a checklist and analyzed using the SPSS software via the Mann–Whitney and χ2 tests.
 

There was a significant difference in the duration of aortic clamping between the pericardial patch and GORE-TEX patch groups (P=0.03). The rate of complications in the GORE-TEX patch group was significantly higher than that in the pericardial patch group (34% vs 14%; P=0.01). The cost of treatment was significantly higher in the GORE-TEX patch group than in the pericardial patch group (P=0.0001).
 

The lengths of surgery, cardiopulmonary bypass, and aortic clamping, as well as short-term complications and treatment costs, were lower in the pericardial patch group than in the GORE-TEX patch group. (Iranian Heart Journal 2022; 23(4): 13-19)

The aim of this study was to investigate the relationship between congenital heart disease in fetal echocardiography with high NT size in fetal ultrasound in mothers referring to Hazrat Masoumeh Hospital in 2019.

In this analytical study, information of all pregnant women who underwent ultrasound screening in the first trimester of pregnancy and their NT size was determined, as well as information from fetal echocardiographic results in the next stage. Fetal echocardiography was extracted from the files and then the relationship between cardiovascular abnormalities with NT size and variables such as birth, weight, gestational age and sex in these mothers were checked and examined. Finally, the relationship between congenital heart defects and NT size was investigated using t-test.

The mean size of NT in 152 neonates without cardiovascular disease was 1.67. 0.3 and this value was 0.5 ± 1.86 mm in 40 neonates with cardiovascular disease. Also, a statistically significant relationship was found between NT size and cardiovascular disease in neonates (P = 0.00). There was also a statistically significant relationship between neonatal gender (P = 0.71), maternal age (P = 0.88), between number of pregnancies (P = 0.26), NT size (P = 0.76), type of pregnancy (P = 0.63), gestational age (P = 0.4), and NT size was not found on ultrasound.

Fetal echocardiography is a non-invasive method for early detection of congenital heart disease that is suitable not only in high-risk pregnancies but also in low-risk pregnancies. Increased NT size is also associated with cardiovascular disease in infants.

Ebstein’s anomaly (EA) is a congenital heart defect that causes cyanosis and arrhythmia. The treating physician has difficulty when it comes to pregnant women since patients frequently reach reproductive age. How to manage these patients during pregnancy or approach patients who are planning to become pregnant is an important issue. The aim of this study was to evaluate previous and current literature reviews, as well as case studies, to better understand how to treat Ebstein’s abnormality in pregnancy.

This study is a literature review with case report. Here, we review the literature on this subject to discuss how to manage Ebstein’s anomaly in pregnancy. We analyzed the literature from different perspectives. We also focused on three of Ebstein’s anomaly-affected women’s pregnancies.

Three women had four pregnancies, all of which were delivered vaginally or through cesarean section. There were no preterm births. 2.540.88 kg was the average birth weight. There were no cardiac abnormalities in any of the three infants. One patient had Ebstein’s abnormality and congenitally corrected transposition of the great arteries (ccTGA).

In Ebstein’s abnormality, pregnancy is often well tolerated. However, the maternal risks of pregnancy correlated with the severity of anatomical malformations and the presence of cyanosis or simultaneous other cardiac anomalies.

Echocardiography currently represents a significant proportion of cardiac medical expenditure, while many researchers believe that it would be wise to limit the use of this technique, through the enhancement of diagnosis techniques contributing to clinical auscultation. Hence, this study was conducted to evaluate the efficiency of detection of the newly invented Doppler Phonolyser Machine (DPM) compared to the findings by the conventional stethoscope in detecting heart murmurs. In a cross-sectional study, a total of 112 patients referred to the pediatric cardiology clinic were enrolled between Jan. 2017 and Jan. 2018. In step one, the patients were initially examined by a pediatric cardiologist, and then in step two, they were randomly divided into two groups (A and B) and were blindly re-examined by a 2nd-year pediatric resident. In Group A (Statoscope group), heart auscultation was performed using only a conventional Statoscope whereas, in Group B (Statoscope + DPM), heart sounds were detected and analyzed using a stethoscope and DPM. In the third step, the diagnostic results made by the pediatric cardiologist and the pediatric resident in groups A and B were compared with the final diagnostic echocardiography made by a pediatric cardiologist. Heart murmurs as one of the most common reasons for cardiologist referrals comprise about 65% of referrals to pediatric cardiologists. There was only a moderate correlation between a stethoscope and echocardiography to identify cardiac abnormality based on heart murmurs (56%), while there was a significant correlation between the diagnoses made by Doppler Phonolyser accompanied with the conventional statoscope when compared with the echocardiography results (٪85). Doppler Phonolyser is an innovative smart machine that could efficiently detect and analyze abnormal heart sounds and might play a key role in more accurate and earlier diagnosis of congenital and structural heart defects by general physicians and non-cardiologist specialists, and may potentially reduce the cost of treatment as well as anxiety of the patient's family.

Congenital heart diseases, as the most common congenital anomaly, are a major cause of serious morbidity and mortality.

This study aimed to evaluate the level of alpha-fetoprotein in the second trimester of pregnancy and its association with congenital heart defects.

Following a cross-sectional design, singleton pregnant women in the second-trimester were enrolled for the α-fetoprotein (AFP) screening test. Those with normal screening results at the first and second tests were assigned to the control group. Mothers with nuchal translucency (NT) screening equal or greater than 3% of the 99th percentile, without chromosomal anomalies, confirmed with amniocentesis, were referred for a fetal echocardiogram and in presence of congenital heart defects symptoms, assigned to the intervention group. The study groups were compared concerning the serum AFP level and maternal and neonatal outcomes in the second trimester of pregnancy. Statistical significance was considered when P-value < 0.05.

A total of 270 women were enrolled in the study, with a mean AFP level of 1.868 ± 0.87 and 1.374 ± 0.39 in the case and control groups, respectively. The mean level of AFP was significantly different between the two groups (P < 0.001). In the second trimester, an AFP level lower than 1 was associated with a significant increase in the likelihood of developing congenital heart anomalies in the fetus.

This study demonstrated that the value of maternal AFP level in the second trimester can be considered as an appropriate screening test to predict the incidence of congenital heart defects in neonates.

Pulmonary agenesis is a very rare congenital disorder with a chromosome recessive inheritance. Half of the cases with pulmonary agenesis suffer from other congenital disorders such as cardiovascular, skeletal, and gastrointestinal defects. Clinical manifestations vary from respiratory problems to recurrent chest infections, which occur in different stages of life including infancy, childhood, and adolescence. The mortality rate of pulmonary agenesis is about 50% among neonates, especially if it is associated with other disorders like cardiac anomalies. In this paper, we report a case of left-sided lung agenesis in a 32-year-old woman. (Iranian Heart Journal 2021; 22(4): 135-139)

Congenital heart diseases (CHDs) constitute the most common congenital malformations in pediatrics and comprise up to 25% of all congenital anomalies. They contribute significantly to morbidity and mortality in children.
 
This retrospective hospital-based study was conducted on children undergoing echocardiography for suspected CHDs between January 2019 and December 2019 in a tertiary teaching hospital.
 
Out of 460 patients examined, 160 (35%) patients were diagnosed with CHDs, and 33 (7%) children had acquired heart diseases. Male gender was predominant (82/160; 51%), while female patients comprised 49% (78/160) of the study population. The majority of the patients (130/160; 81%) had acyanotic CHDs, whereas 30 (19%) patients had cyanotic CHDs. The most frequent type of acyanotic CHD was ventricular septal defect (59/160; 37%), followed by pulmonary stenosis (22/160; 14%) and patent ductus arteriosus (21/160; 13%). Tetralogy of Fallot was the most common cyanotic CHD in that it was diagnosed in 8 (5%) patients.
 
Ventricular septal defect was the most common acyanotic CHD in this study, while tetralogy of Fallot was the most frequent cyanotic CHD.  To our knowledge, there are no previous data on CHD in our country. We hope that the results of this study will provide a database for future investigations on the incidence and prevalence of CHDs in tertiary hospitals in Somalia and raise awareness about the significance of their early detection and surgical interventions. (Iranian Heart Journal 2021; 22(1): 10-15)

Context: Congenital heart disease (CHD) is a leading cause of mortality by birth defects with significant social and economic burden. Pulse oximetry as a safe and non-invasive screening method, and with its potential for early detection of CHD has improved neonatal health outcomes.

 The aim of this study was to systematically review economic evaluation studies that compared pulse oximetry with current programs to diagnose early detection of CHD in full-term newborns.
Data Sources: A systematic review was conducted according to preferred reporting items for systematic reviews and meta-analyses (PRISMA) guidelines, and related articles published from 1995 up to March 2020 were searched in different databases (MEDLINE, EMBASE, PubMed, Science Direct, Google Scholar, Scopus, NHS EED, Science Citation Index, MagIran, Cochrane Library, EconLit and SID). The articles were selected based on inclusion and exclusion criteria. Consolidated health economic evaluation reporting standards (CHEERS) statement checklist was used to qualitatively evaluate the papers. Overall, 7 articles were included in the study.

 Timely diagnosis was considered as main effectiveness health outcome in most studies. The highest and lowest values of incremental cost-effectiveness ratio (in two-phase studies) were €139,000 and $100 per infant in the Netherlands and Colombia respectively; and (in one-phase studies) were £24,000 and £1,489 per infant both belonging to the UK. Implementing pulse oximetry method concurrent with the clinical examination is more cost-effective. The reviewed studies had been conducted in high-income and upper middle-income countries; therefore, when the results are generalizing by policy makers in different health systems, a substantial precaution approach is needed.
 

Pulmonary arterial hypertension (PAH) specific drug therapy using bosentan has significantly improved quality of life and survival, although PAH is still an incurable disease. Recent studies suggest metformin may have additional treatment benefits in PAH. We therefore investigated in vitro pulmonary artery reactivity after combination therapy of bosentan and metformin in PAH patients as compared with bosentan monotherapy in a prospective, randomized study.

Adult patients with PAH associated with congenital heart defects (PAH-CHD) were randomised to receive bosentan (initially at 62.5 mg twice daily for 4 weeks and then 125 mg twice daily) for 3 months with or without the combination treatment of metformin (500 mg twice daily). Vessel reactivity of isolated pulmonary arteries was examined using a wire myograph.

Phenylephrine (PE)-induced contractions of arteries in patients received combination therapy were significantly attenuated at concentrations of 3 × 10-7 M, 10-6 M and 3 × 10-6 M, compared to those received bosentan monotherapy. After denudation, PE-induced contractions at concentrations of 3 × 10-6 M and 10-5 M were significantly decreased in the combination therapy group. AMP-activated protein kinase (AMPK) inhibitor compound C abrogated the inhibitory effects of metformin on PE-induced contractility. AMPK and eNOS phosphorylation in the pulmonary arteries of patients treated with combination therapy was increased compared to monotherapy (P < 0.05).

Adding metformin to bosentan therapy in patients with PAH-CHD decreased in vitro pulmonary artery contraction induced by PE, which is possibly related to increased AMPK phosphorylation.

Pulmonary morbidity is a common complication of cardiac surgery, and the most common type of morbidity is atelectasis. The risk factors related to pulmonary morbidities and atelectasis have been previously explored in coronary artery bypass graft and valvular surgery. In this study, we sought to determine the risk factors related to atelectasis after adult congenital heart disease surgery (ACHDS). This cross-sectional study was conducted on 43 patients (mean=36.3 and SD=16.37) who underwent ACHDS. The risk factors related to atelectasis were identified using a questionnaire which contained demographic factors, types of CHD, underlying diseases, and the length of postoperative stay in bed. The collected data were subsequently analyzed with SPSS. Of the 43 patients, only 3 (7%) had atelectasis after ACHDS. The tetralogy of Fallot surgery and delayed postoperative ambulation (>24 h) had a P value of 0.027 and 0.000, respectively. The other factors had P values higher than 0.05 and were, thus, not related to atelectasis. The tetralogy of Fallot surgery and delayed postoperative ambulation (>24 h) were associated with the rate of atelectasis after ACHDS.

The prevalence of celiac disease (CD) is remarkably varied in Down syndrome(DS)patientscompared with other diseases. This study aimed to assess celiac disease prevalence in Down syndrome children with and without congenital heart defects (CHD) and its comparison with controls.
This case-control study was performed at a single center on 132 participants in three groups. Clinical and genetic tests were performed on all patients suspected with Down syndrome to confirm their diseases. After that in patients with confirmed Down syndrome echocardiography was carried out to diagnosis of CHD. Healthy children selected randomly among those who referred to the center for annual check-up. Statistical evaluation was done using SPSS-16.
For the factors of age, weight, height and Body Mass Index (BMI) not observed significant differences between three groups of participants, but it would be observed statistically differences for the variable of tTG- IgA. For variables of weight, tTG- IgA and BMI was observed statistically different in the case and controls. The status of tTG- IgA (normal or 20) had significant correlation with three groups of controls, Down syndrome with and without CHD. The status of tTG- IgA also had significant correlation with groups of case and controls. In comparison of tTG- IgA in DS patients with and without CHD, no significant differences were observed.
The prevalence of CD in DS patients was higher compared the controls population; and in DS patients with CHD was higher compared the DS patients without CHD.

Congenital lesions of the mitral valve are relatively rare and are associated with a wide spectrum of cardiac malformations. The surgical management of congenital mitral valve malformations has been a great challenge.. The aim of this study was to evaluate the early and intermediate-term outcome of congenital mitral valve (MV) surgery in children and to identify the predictors for poor postoperative outcomes and death..Patients and In this retrospective study, 100 consecutive patients with congenital MV disease undergoing mitral valve surgery were reviewed in 60-month follow-up (mean, 42.4 ± 16.4 months) during 2008 - 2013. Twenty-six patients (26%) were under one-year old. The mean age and weight of the patients were 41.63 ± 38.18 months and 11.92 ± 6.12 kg, respectively. The predominant lesion of the mitral valve was MV stenosis (MS group) seen in 21% and MR (MR group) seen in 79% of the patients. All patients underwent preoperative two-dimensional echocardiography and then every six months after surgery. Significant improvement in degree of MR was noted in all patients with MR during postoperative and follow-up period in both patients with or without atrioventricular septal defect (AVSD) (P = 0.045 in patients with AVSD and P = 0.008 in patients without AVSD). Decreasing trend of mean gradient (MG) in MS group was statistically significant (P = 0.005). In patients with MR, the mean pulmonary artery pressure (PAP) had improved postoperatively (P < 0.001). Although PAP in patients with MV stenosis was reduced, this reduction was not statistically significant (P = 0.17). In-hospital mortality was 7%. Multivariate analysis demonstrated that age (P < 0.001), weight (P < 0.001), and pulmonary stenosis (P = 0.03) are strong predictors for mortality. Based on the echocardiography report at the day of discharge from hospital, surgical results were optimal (up to moderate degree for MR group and up to mild degree for MS group) in 85.7% of patients with MS and in 76.6% of patients with MR. Age (P = 0.002) and weight (P = 0.003) of patients are strong predictors for surgical success in multivariate analysis.. Surgical repair of the congenital MV disease yields acceptable early and intermediate-term satisfactory valve function and good survival at intermediate-term follow-up. Strong predictors for poor surgical outcome and death were age smaller than 1 year, weight smaller or equal than 6 kg, and associated cardiac anomalies such as pulmonary stenosis..

Cayler cardio-facial syndrome is a rare syndrome associated with asymmetric crying faces with congenital heart disease. We report a newborn that was diagnosed as case of Cayler Cardio-facial syndrome based on clinical features and was confirmed with FISH analysis. A term male baby, born to non-consanguineous couple through normal vaginal delivery was diagnosed to have asymmetric crying faces with deviation of angle of mouth to left side at the time of birth. The baby had normal faces while sleeping or silent. Mother was known case of hypothyroidism and was on treatment. Baby was diagnosed as case of Cayler Cardio-facial Syndrome and was investigated with echocardiogram, brain ultrasound, total body X-ray examination, X-ray of cervico-thoracic vertebral column and fundus examination. Echocardiogram showed muscular VSD, brain ultrasound was normal and fundus examination showed tortuous retinal vessels. Whole body X-ray and lateral X-ray of cervico-thoracic vertebral column were not suggestive of any skeletal abnormalities. The other associated malformation was right ear microtia. Baby FISH karyotype analysis showed deletion of 22q11.2 deletion. Baby was discharged and now on follow-up. Cayler syndrome is a rare syndrome which must be suspected if a baby has asymmetrical cry pattern and normal facies when baby sleeps. Patient must be evaluated with echocardiography to find out associated cardiac malformations. These infants should undergo FISH analysis for 22q11.2 deletion syndrome.

Ectopia cordis (EC) is the presence of a live, beating heart outside the thorax and is one of the most unique congenital anomalies. EC has been a weak association noted with trisomy 18 and a few cases have been associated with other chromosomal abnormalities. Pentalogy of cantrell (PC) is a rare congenital syndrome of abdominal wall defect, lower sternal defect, diaphragmatic pericardial defect, anterior diaphragmatic defect, and intracardiac abnormalities. It has a rare frequency of about 1/100,000 births. The hallmark of this syndrome is an omphalocele associated with EC. Only a few patients with the full spectrum of the pentalogy have been described. Only very few patients survive attempts at surgical repair, the main causes of death being tachyarrhythmias, bradycardia, low blood pressure, rupture of the diverticulum, and heart failure. Early diagnosis is important about discuss the option of elective termination with the parents if intracardiac anomalies of incompatible with life are noted. We report two cases of PC diagnosed in early second trimester.