Brain abscess in a 12-year-old child with congenital heart disease: lessons learnt

Brain abscess is a rare and often life-threatening sequelae, observed in 5-20% of the patients with cyanotic congenital heart diseases (CHD). Cyanotic heart diseases involve a right-to-left shunt by-passing the pulmonary vascular bed, culminating in tissue hypoxia. We present a case of an uncorrected atrioventricular canal defect (AVCD) with a double outlet right ventricle (DORV) complicated by a brain abscess in a 12-year-old girl. This patient was a known case of CHD diagnosed at 2 years of age and presented to the neurology out-patient department with a holocranial headache, vomiting, and low-grade fever for 5 days. General examination was suggestive of cyanosis and low-set ears. Systemic examination revealed a pansystolic murmur with bilateral extensor plantar reflexes. MRI brain with contrast revealed an abscess in the right temporoparietal lobe with perilesional edema. The patient was managed conservatively. Nine days post-discharge, the patient presented with generalized tonic-clonic seizures and was readmitted and managed accordingly. Subsequently, she was referred to the cardiothoracic surgery department for correction of the CHD. However, the family gave negative consent for the same. The relatives of the case described here were counseled to get a surgical correction done for the cardiac defect at the time of diagnosis but with no success. The aim of publishing this case is to emphasize the need for timely diagnosis and correction of such anomalies to curb serious complications causing profound morbidity and mortality.

Frequency and pattern of congenital heart defects among admitted patient in a tertiary care hospital in Bangladesh

Background: Congenital heart disease (CHD) is the commonest of all congenital lesions accounting for nearly 28% of all congenital malformations that have significant impact on morbidity, mortality and heath care cost in children. The aim of study was to determine the pattern of distribution of CHD and the age at which initial diagnosis of CHD was made among children admitted under paediatric cardiology department, Bangladesh Shishu hospital and institute. Methods: This is a descriptive and prospective hospital-based study conducted in the pediatric cardiology department, Bangladesh Shishu hospital and institute. This study included all the patients admitted during January-June 2022 in pediatric cardiology department having confirmed diagnosis of CHD on basis of echocardiographic report. The collected data was entered and analyzed by using statistical package for social sciences v 24.0. Results: Out of 337 patient, 175 patient were male (51.9%) and 162 female (48.1%) with male female ratio of 1.08:1. Total 219 cases (64.9%) were acyanotic CHD and 118 (35.1%) were cyanotic congenital heart lesions. In acyanotic CHD ventricular septal defects (VSDs) constitute 38.8% followed by AV canal defect 6.7%, patent ductus arteriosus (PDA) 3.6%. In cyanotic CHD tetralogy of Fallot (TOF) was the commonest lesion accounted for 28 (8.3%) followed by d-transposition of great arteries 16 (4.7%), pulmonary atresia 15 (4.5%), total anomalous pulmonary venous circulation TAPVC 11 (3.3%), tricuspid atresia 9 (2.7%) and DORV, VSD, PS 10 (2.9%). The commonest combination was VSD with ASD in 15 cases (4.5%). Most of the patient was diagnosed in 1st year of age 280 (83.1%) Conclusions: CHD are very common in our setup and early detection of CHD is increasing. Overall burden of CHD is also increasing therefore a proper population-based study on a large scale is needed to estimate the prevalence accurately.

Are breath-holding spells always innocuous: A case report

The diverse clinical manifestations of Langerhans cell histiocytosis (LCH) present a dilemma to clinicians and pediatricians play an important role in its diagnosis and multidisciplinary approach. LCH, previously known as histiocytosis X, is an uncommon hematological disorder characterized by uncontrolled stimulation and proliferation of normal antigen-presenting cells, Langerhans cells. The purpose of this report is to describe the case of a 7-month-old female child with multisystem involvement who presented with breath-holding spells and to discuss the clinical, radiological, and histopathological features of LCH.

Brain abscess in older children with uncorrected tetralogy of Fallot

Tetralogy of Fallot is the most common cyanotic congenital heart disease. Corrective surgery is indicated during infancy for the best outcome, however, due to poor availability and affordability of surgery, many children of resource-poor settings live with uncorrected tetralogy of Fallot. They suffer from dreaded complications like brain abscess, in later years of life. We report 3 cases of tetralogy of Fallot who developed brain abscess during their childhood.

A STUDY ON PATTERN AND CLINICAL PROFILES OF CONGENITAL HEART DISEASES IN TERTIARY CARE CENTRE, KAKINADA.

BACKGROUND: Congenital heart disease is an abnormality of anatomical structure of the heart that is of paramount importance. The incidence is approximately 8 per 1000 live births. CHD not only contributes to signi?cant mortality and morbidity but also causes tremendous psychological stress and economical burden to whole family. OBJECTIVE: To study the pattern and clinical pro?le of congenital heart diseases in a tertiary care hospital and to aid for early stdetection and quick referral of children with congenital heart disease In this study, done between January 1 2020 to RESULTS: june 31st 2021, 54 children with con?rmed congenital heart disease were observed. ACHD was the most common type of CHD and of them, VSD was the most common type, followed by ASD. Based on sex distribution, 31 were male (55.5%) and 23 were female (44.5%), with a male: female ratio of 1.25:1. Most children belonged in the age group 6m – 1year (46.2%) followed by (42.5%) in 30days – 6months age. The common clinical presentation observed in this study was breathlessness. The common complication observed in the current study was heart failure followed by malnourishment and recurrent LRTI.

Inhaled nitric oxide improves postoperative hemodynamics in patients with cyanotic congenital heart disease combined with decreased pulmonary blood flow / 中国胸心血管外科临床杂志

@#Objective    To explore the hemodynamic effects of inhaled nitric oxide (iNO) on postoperative hemodynamic in patients with cyanotic congenital heart disease (CHD) combined with decreased pulmonary blood flow. Methods    From 2014 to 2018, there were 1 764 patients who received corrective repair of cyanotic CHD with decreased pulmonary blood flow in the Department of Pediatric Cardiac Surgery of Fuwai Hospital. We included 61 patients with the ratio of right ventricular systolic pressure to systolic blood pressure (SBP) ≥75% after weaning from cardiopulmonary bypass. There were 41 males and 20 females, with the age of 20.5 (9.0, 39.0) months and weight of 12.5±7.8 kg. The patients were divided into two groups: a conventional group (33 patients, conventional therapy only) and a combined therapy group (28 patients, iNO combined with conventional therapy). The hemodynamics during the first 24 hours after iNO therapy and the in-hospital outcomes of the two groups were investigated and compared. Results    There was no statistical difference between the two groups in demographic characteristics and surgical parameters (P>0.05). The hemodynamic effects of iNO within 24 hours included the decrease in the vasoactive inotropic score (VIS, 21.6±6.6 vs. 17.3±7.2, P=0.020) along with the increase in blood pressure (SBP: 73.7±9.7 mm Hg vs. 90.8±9.1 mm Hg, P<0.001) , the decrease in central venous pressure (10.0±3.1 mm Hg vs. 7.9±2.1 mm Hg, P=0.020), the decrease in lactate (2.2±1.7 mmol/L vs. 1.2± 0.5 mmol/L, P<0.001) and increase in urine output [2.8±1.7 mL/(kg·h) vs. 4.9±2.2 mL/(kg·h), P<0.001]. The decrease of VIS at 24 h after the surgery in the conventional therapy group was not statistically significant (22.1±7.9 vs. 20.0±8.5, P=0.232). Besides, we discovered that the need for renal replacement therapy (RRT) was less in the combined therapy group than that in the conventional therapy group, especially in the moderate complicated surgery [risk adjustment in congenital heart surgery (RACHS-1) ≤3] subgroup (9.5% vs. 40.7%, P=0.016). Conclusion    In pediatric patients after corrective repair of cyanotic and pulmonary blood follow decreased CHD with increased pulmonary vascular resistance, iNO combined with conventional therapy can improve the hemodynamics effectively. Compared with the conventional therapy, the combined therapy with iNO can decrease the VIS and the need for RRT, which is beneficial to the postoperative recovery of patients.

Study the universal critical congenital heart disease screening in a peripheral area of Uttarakhand, India

Background: Congenital heart disease (CHD) is the most frequently occurring congenital disorder, responsible for 28% of all congenital birth defects. The birth prevalence of CHD is reported to be 8-12/1000 live births. Considering a rate of 9/1000, about 1.35 million babies are born with CHD each year globally. Objective of research work to study the prevalence of CHD among newborn and its types.Methods: This cross-sectional study was carried among 34 cases of CHD/5126 newborn screened at 4 birthing places in Kashipur a small town in Uttrakhand at pediatrics department of Sahota Super-specialty hospital, Kashipur, Uttarakhand. Screening program between 22 August 2014, and March 30, 2019. All newborns, including preterm babies, delivered in these facilities were eligible for inclusion in this study.Results: Present study found the prevalence of CHD was 0.7 per 1000 children (34/5126). Around 26.5% participants have cyanotic CHD and 73.5% have acynotic CHD. Almost 44%, 28%, 20%, 4% and 4% participants of acynotic congenital heart diseases have VSD, ASD, PDA, AVSD and valvular PS respectively and 55.6%, 22.2%, 11.1% and 11.1% participants of cynotic congenital heart diseases have TOF, DORV/VSD, dTGA/VSD and tricuspid atresia respectively.Conclusions: Screening for congenital heart disease should be included as a part of newborn assessment as it is a common congenital problem. Early identification influences outcome. Barriers in implementation of the screening programmes in resource limited setting is a challenging feature. This study can provide observed data that can help in policy making in the health sector.

Pulse Oximetry a Non-Invasive Screening Test to Intensify Primary Diagnosis of Critical Cyanotic Congenital Heart Disease in Neonates- A Retrospective Study.

Pulse oximetry is an important screening technique to intensify timely diagnosis of critical cyanotic congenital heart disease in neonates which is a noninvasive, safe and an easy method. It is a highly specific test having low false positive rates. This screening test was conducted in Department of Pediatrics, Darbhanga Medical College & Hospital, Laheriasarai, Darbhanga, Bihar, between 24 hours and 36 hours after birth, with use of the right hand of infants and either foot to diminish false positive results.METHODSData for study has been collected from birth records from Darbhanga Medical College & Hospital, Bihar, and included- 1. saturation level from either feet and right hand for the primary and further recurrence of screening. 2. outcomes of the echocardiographic test findings. 3. important non-cardiac illness diagnosed at the hospitalization for infants which failed screening for cyanotic CHD. For the correction of errors related to data of the study, we satisfied autonomous investigations of pulse oximetry requiring AAP CCHD screening algorithm to identify infants to pass with a saturation ≥95% in hand or either foot and a ≤3% difference and those falling after finishing 3 transitional screening tests. After three consecutive measurements, there is difference of SpO2 of more than 3% between pre-ductal and post-ductal SpO2.RESULTSThe combination of pulse oximetry and physical examination improved the sensitivity to up to 57.14% with a specificity of 99.73%. The overall mortality rate of CCHD was 36.8% [7 out of 19]. The mortality rate of early detection were 37.5% [6 out of 16]. Late detection of CCHD has mortality rate of 33.3% [1 out of 3]. 123 [13.1%] and 70 [7.4%] newborns have either foot or right hand SpO2 level < 95% among 941 study newborns at the time of the initial screening. The mean SpO2 level of either feet and right arm of study newborns were 95.8% [SD 2.3] and 96.0% [SD 2.2] respectively. Either feet and right arm level of SpO2 <95% and either feet to right arm level of SpO2 difference of >3% among 142 [15.1%] study newborns.CONCLUSIONSPulse oximetry screening for primary diagnosis of critical cyanotic CHD is an easy, economical and non-invasive test which covers the essential criteria in addition to worldwide newborn screening panel. Extensive approval and implementation can considerably reduce the morbidity in newborns infants and is likely to be an additional important tool in low resource settings where most of newborn infants are born without prenatal diagnosis. Although, this study showed reduced sensitivity of pulse oximetry for critical cyanotic CHD nearly <50%.

Clinical profile and outcome of congenital cyanotic heart disease in neonatal period: a retrospective study

Background: Congenital Cyanotic Heart Disease (CCHD) is under reported during neonatal period and mortality rate is high in India. Aims and objectives of the study determine clinical presentation, maternal and neonatal risk factors and outcome of CCHD during neonatal period.Methods: A retrospective study conducted over 15-month period during March 2017 to June 2018 in a tertiary out born NICU. Neonates with echocardiographically confirmed case of structurally abnormal heart disease were analyzed.Results: Among 106 CCHD neonates, 60% neonates were asymptomatic, 22% had persistent cyanosis and 19% had tachypneic at birth. Cyanosis (35%) and tachypnea (30%) were common initial presentation in postnatal period. 22% neonates were readmitted with initial clinical symptom after discharge from birthing centre. CCHD were more common in male and term neonate, 34% were small for age, median age at admission and discharge were 5days (2-12) and 5 days (2.7-9.2) days respectively. Nineteen percent neonates had fetal distress and 6% neonates required aggressive resuscitation at birth. First trimester abortion (16%), maternal diabetes (10%) and hypothyroidism (7%) were common maternal risk factor. Nearly 20% neonates were diagnosed at referring hospital and 4.7% were transported with PGE1 during transport. TGA (17%) was most common lesion noted followed by pulmonary atresia (10%). Fourteen percent neonates died during the neonatal period. Disease specific death rate was highest for Single ventricle (42%) followed by TGA with IVS (37.5%) and aortic arch anomaly (28.5%) in this study.Conclusions: Early diagnosis and management may improve the survival in CCHD neonates.

A retrospective cross sectional study on neurological profile of children with congenital heart disease

Background: Children with Congenital Heart Disease have associated structural neurological    abnormalities and those surviving infancy are even subjected to various environmental factors   which might contribute to neurological abnormalities. Hence the objective is to study the neurological abnormalities of children with Congenital Heart Disease.Methods: A retrospective cross-sectional observation study was conducted over the period of 3 years (June 2016-May 2019). Data were collected from medical records department of 121 children of 2-12 years of age diagnosed with congenital heart disease. Neurological examination findings and neuro imaging and EEG findings where relevant    were noted. To assess the association of adverse neurological outcome and congenital heart disease, data analysis was        performed using Fisher's exact test.Results: 38% children of 2-12 years of age with congenital heart disease had adverse neurological profile. Neurological abnormalities were significantly associated with cyanotic heart disease (p value 0.0001). Statistically significant association were found between congenital cyanotic heart disease and seizure (p value=0.04), hemi paresis (p value=0.039), brain abscess (p value=0.012) and coma (p=0.01).Conclusions: Congenital cyanotic heart disease has significant associations with neurological abnormalities in children. These results demand attention for the   neurological health of the largely uncorrected cardiac disease in Indian children

Burden of congenital heart diseases in a tertiary cardiac care institute of high altitude area

Background: Congenital heart disease is one of the major causes of mortality and morbidity in the paediatric population of both the developing and developed countries. Variability in incidence and prevalence of CHD from various countries of Indian subcontinent and rest of the world could be because of genetic, cultural, and environmental factors.  The objective of the study was to find the prevalence and pattern of CHD in a tertiary care hospital in Kashmir (Jammu and Kashmir).Methods: All children admitted at territary care hospital with age 0-15 years were screened for congenital heart disease. The study was conducted for period of one year to ascertain the prevalence and spectrum of CHDs.Results: A total of 232 patients out of 23000, were found having CHDs measuring a prevalence of 10.5/1000. About 170 (73%) were the acyanotics, and 62 (27%) were cyanotic heart patients. Among the acyanotic heart diseases ventricular septal defect was the most frequent lesion seen in 54 (23%), followed by patent ductus arteriosus in 50 (22%) children. Among the cyanotic heart diseases tetralogy of Fallot was the most frequent cyanotic heart disease seen in 15 (6.4%) patients.Conclusions: Authors observed high prevalence of CHD in our population. The pattern and spectrum of CHD were comparable to national and international data.

Prevalence, profile of malnutrition and iron deficiency anemia in children with cyanotic congenital heart defects: a case control observational study in a tertiary care hospital in North India

Background: Children with congenital heart disease (CHD) are prone to malnutrition. Children with cyanotic CHD [CCHD] are specifically affected due to chronic hypoxia and iron deficiency anemia which is overlooked by pediatrician. This can have a significant effect on the outcome of surgery. Our objective was to determine the burden and determinant of malnutrition in children with several types of cyanotic congenital heart disease (CCHD).Methods: This case-control study included 80 children with symptomatic CCHD, and 40 healthy children matched for age and sex as a control group. Clinical evaluation and laboratory assessment of nutritional status were documented. Anthropometric measurements were recorded and Z scores for weight for age (WAZ), weight for height (WHZ), and height for age (HAZ) have been calculated. Haemoglobin, red cell indices and serum iron, total iron binding capacity and serum ferritin was done in cases and controls.Results: The overall prevalence of malnutrition was 72.5% in patients with CCHD and 22.5% in controls. Severe malnutrition was diagnosed in 68.9% of cases. All anthropometric measurements which markers of nutritional state are were significantly lower in the patients group compared to controls. The prevalence of iron deficiency anemia (IDA) was 47.5% in the study population. The study also showed that hemoglobin and hematocrit levels, RBC count were paradoxically higher in the cyanotic CHD as compared to the healthy controls though the iron studies revealed the iron de?ciency. The mean corpuscular volume (MCV), mean corpuscular hemoglobin (MCH) mean corpuscular hemoglobin concentration (MCHC), serum ferritin, serum iron, total iron binding capacity (TIBC), values were the parameters, which were found to be statistically signi?cant to differentiate the study groups.Conclusions: Malnutrition is a very common problem in children with symptomatic CCHD, the prevalence of IDA in children with CCHD was found to be high.

Spectrum of congenital heart disease in a tertiary care centre of Northern India

Background: Congenital heart disease (CHD) is very common disease and it is the major cause of childhood mortality and morbidity. Not much of Indian data are available particularly from the northern part of the country. It is important to mention that this part of India is unique with respect to its demographic and geographical location. There are needs to further explicate the spectrum and epidemiology of the CHD in this part of the country.Methods: A prospective hospital-based study carried out over a period of 24 months (January 2016 to January 2018) where all suspected children (<14 years) of CHD were subjected to echocardiographic study. The age, sex, clinical presentation and echo findings were well documented.Results: Out of total 3210 cases CHD was diagnosed in 2072 cases (64.54%). Most CHDs were diagnosed between 1 month and 6 years of age in both cyanotic and a cyanotic disease group. Incidence of a cyanotic CHD was n=1529 (47.6%) and cyanotic CHD was n=543 (17%) with the ratio of acyanotic to cyanotic 2.8:1. Ventricular septal defect was commonest CHD (35.85%) among acyanotic CHD and Tetralogy of Fallot was the commonest (12.2%) among cyanotic CHD.Conclusions: Burden of CHD is highly underestimated and unrecognised, especially in this part of the country. This study can provide observed data that can help in policy making in the health sector. The contrasting result with respect to complex CHD in present study indicates need for good and effective antenatal cardiac screening in high risk mothers.

Metabolic Syndrome in Adult Congenital Heart Disease

In adult congenital heart disease (ACHD), residua and sequellae after initial repair develop late complications such as cardiac failure, arrhythmias, thrombosis, aortopathy, pulmonary hypertension and others. Acquired lesions with aging such as hypertension, diabetes mellitus, obesity can be negative influence on original cardiovascular disease (CVD). Also, atherosclerosis may pose an additional health problem to ACHD when they grow older and reach the age at which atherosclerosis becomes clinically relevant. In spite of the theoretical risk of atherosclerosis in ACHD due to above mentioned factors, cyanotic ACHDs even after repair are noted to have minimal incidence of coronary artery disease (CAD). Acyanotic ACHD has similar prevalence of CAD as the general population. However, even in cyanotic ACHD, CAD can develop when they have several risk factors for CAD. The prevalence of risk factor is similar between ACHD and the general population. Risk of premature atherosclerotic CVD in ACHD is based, 3 principal mechanisms: lesions with coronary artery abnormalities, obstructive lesions of left ventricle and aorta such as coarctation of the aorta and aortopathy. Coronary artery abnormalities are directly affected or altered surgically, such as arterial switch in transposition patients, may confer greater risk for premature atherosclerotic CAD. Metabolic syndrome is more common among ACHD than in the general population, and possibly increases the incidence of atherosclerotic CAD even in ACHD in future. Thus, ACHD should be screened for metabolic syndrome and eliminating risk factors for atherosclerotic CAD.

Bilateral Spontaneous Urinoma in a Cyanotic Child

Background: Urinoma is an encapsulated collection of extravasated urine, secondary totrauma or obstructive uropathy. Spontaneous bilateral urinoma is rare. Casecharacteristics: 7-year-old boy with cyanotic heart disease and fever of unknown origin.Obeservation: The ultrasound abdomen and CT abdomen revealed bilateral spontaneousurinoma which was aspirated and was found to be infected. Following intravenous atibioticsthe child became afebrile, with subsequent renal scans showing no recurrence. Message:Hypoxia and consequent polycythemia may be responsible for perinephric leaks leading toNon-traumatic spontaneous urinoma.

Efficiency and safety of milk feeding rescue for failed chloral hydrate sedation in infants with cyanotic congenital heart disease / 上海交通大学学报（医学版）

Objective: To evaluate the efficiency and safety of milk feeding to rescue failed chloral hydrate sedation in infants with cyanotic congenital heart disease who underwent transthoracic echocardiography. Methods: Clinical data of the infants with known cyanotic congenital heart disease were retrospectively analyzed, who were not adequately sedated after initial dose of chloral hydrate in 2015 in Shanghai Children's Medical Center, ShanghaiJiao Tong University School of Medicine. Vital signs and oxygen saturations were recorded every 5 minutes, and adverse events were noted. For rescue, efficiency and safety of milk feeding were compared with phenobarbital intramuscular injection. Results: A total of 129 infants were enrolled in this study. Fifty-two infants were rescued by intramuscular phenobarbital and the other 77 ones were rescued by milk feeding. The proportion of infants successfully rescued by milk feeding was 55.8%, which was higher than that of intramuscular phenobarbital (30.8%, P=0.005). There was no statistical difference in incidence of adverse events between the two groups (P=0.565). Conclusion: Milk feeding is an effective and safe choice for rescue sedation for infants with cyanotic congenital heart disease undergoing transthoracic echocardiography.

Efficiency and safety of milk feeding rescue for failed chloral hydrate sedation in infants with cyanotic congenital heart disease / 上海交通大学学报（医学版）

Objective·To evaluate the efficiency and safety of milk feeding to rescue failed chloral hydrate sedation in infants with cyanotic congenital heart disease who underwent transthoracic echocardiography.Methods·Clinical data of the infants with known cyanotic congenital heart disease were retrospectively analyzed,who were not adequately sedated after initial dose of chloral hydrate in 2015 in Shanghai Children's Medical Center,Shanghai Jiao Tong University School of Medicine.Vital signs and oxygen saturations were recorded every 5 minutes,and adverse events were noted.For rescue,efficiency and safety of milk feeding were compared with phenobarbital intramuscular injection.Results·A total of 129 infants were enrolled in this study.Fifty-two infants were rescued by intramuscular phenobarbital and the other 77 ones were rescued by milk feeding.The proportion of infants successfully rescued by milk feeding was 55.8％,which was higher than that of intramuscular phenobarbital (30.8％,P=0.005).There was no statistical difference in incidence of adverse events between the two groups (P=0.565).Conclusion·Milk feeding is an effective and safe choice for rescue sedation for infants with cyanotic congenital heart disease undergoing transthoracic echocardiography.

Spectrum of Cyanotic Heart Defects and Their Modes of Presentation at Cardiology Clinic in a Tertiary Care Hospital.

Background: Cyanotic Congenital heart defect (CHD) is the high risk group which requires prompt medical attention. Immediate management can drastically alter the natural history otherwise most of the children will succumb to their defect very early in infancy. It is thus important to have reliable information of the profile of various cyanotic CHDs as well as their mode of presentation for the early detection. Methods: The study was carried out in Department of Pediatrics and Center of Cardiology, Jawaharlal Nehru Medical College, Aligarh. All patients referred with complaints or clinical examination suggestive of congenital heart defects were further evaluated with echocardiography. On echocardiography patients having congenital heart defects were included as cases which were further divided into cyanotic and acyanotic heart defects, preterms having PDA and PFO and those with acquired heart defects were excluded. The profile and mode of presentation of various cyanotic CHDs was further described in detail. Results: Acyanotic heart defects were 290(72.50%) of the total heart defects, while the contribution of cyanotic heart defects was 110 (27.50%). Out of all CHDs, VSD was the most common lesion with contribution of 152 (38.00%) cases, while among the cyanotic heart defect, Tetralogy of Fallot (TOF) was the most common lesion (18% of total cases). Spectrum of various cyanotic lesions were TOF 65.45%, Single Ventricle 12.72%, TAPVC (Total Anomalous Pulmonary Venous Connection) 8.18%, TGA(Transposition of Great Arteries) 7.27%, Tricuspid Atresia 3.63%, Ebsteins malformation 1.81% and Truncus Arteriosus 0.90%. Conclusion: Profile of various cyanotic CHDs was similar to the previous studies, however spectrum of various CHDs was quite wide, a large number of cases were missed in infancy and presented late.

Serum albumin perturbations in cyanotics after cardiac surgery: Patterns and predictions.

Introduction: Hypoalbuminemia is a well‑recognized predictor of general surgical risk and frequently occurs in patients with cyanotic congenital heart disease (CCHD). Moreover, cardiopulmonary bypass (CPB)‑induced an inflammatory response, and the overall surgical stress can effect albumin concentration greatly. The objective of his study was to track CPB‑induced changes in albumin concentration in patients with CCHD and to determine the effect of hypoalbuminemia on postoperative outcomes. Materials and Methods: Prospective observational study conducted in 150 patients, Group 1 ≤18 years (n = 75) and Group 2 >18 years (n = 75) of age. Albumin levels were measured preoperatively (T1), after termination of CPB (T2) and 48 h post‑CPB (T3). Primary parameters (mortality, duration of postoperative ventilation, duration of inotropes and duration of Intensive Care Unit [ICU] stay) and secondary parameters (urine output, oliguria, arrhythmias, and hemodynamic parameters) were recorded. Results: The albumin levels in Group 1 at T1, T2, and T3 were 3.8 ± 0.48, 3.2 ± 0.45 and 2.6 ± 0.71 mg/dL; and in Group 2 were 3.7 ± 0.50, 3.2 ± 0.49 and 2.7 ± 0.62 mg/dL respectively. All patients showed a significant decrease in albumin concentration 48 h after surgery (P < 0.01). Analysis between the groups, however, showed no statistical difference. Eleven patients expired during the study period, and nonsurvivors showed significantly lower serum albumin concentration 48 h after surgery 2.3 ± 0.62 mg/dL versus 3.7 ± 0.56 mg/dL in the survivors (P < 0.05). Receiver operating characteristic curve showed that a baseline albumin cut‑off value of 3.3 g/dL predicts mortality with a positive predictive value 47.6% and a negative predictive value of 99.2% (P < 0.05). A strong correlation was seen between albumin levels at 48 h with duration of CPB (r2 = 0.6321), ICU stay (r2 = 0.7447) and incidence of oliguria (r2 = 0.8803). Conclusions: The study demonstrated similar fall in albumin concentration in cyanotic patients (both adult and pediatric) in response to CPB. Low preoperative serum albumin concentrations (<3.3 g/dL) can be used to identify and prognosticate subset of cyanotics predisposed to additional surgical risk.

Effect of Sildenafil in the treatment of hypoxemia after operation of cyanotic congenital heart disease / 中华实用儿科临床杂志

Objective To study the effect of Sildenafil in the treatment of hypoxemia after operation of cyanotic congenital heart disease.Methods A total of 34 cases with hypoxemia after operation of cyanotic congenital heart disease were collected between January 2009 to January 2015.They took Sildenafil with the dosage of 0.3-0.5 mg/kg every 6 hour.The change of heart rate (HR),systolic blood pressure (SBP),central venous pressure (CVP),pulmonary artery pressure (PAP),arterial partial pressure of oxygen [pa (O2)],arterial partial pressure of carbon dioxide [Pa (CO2)],oxygenation index [pa (O2)/FiO2],peak airway pressure (Ppeak),positive inotropic drug score (PIDS) and the lower bound of liver were observed at 4 time points,including before taking the medicine,after taking the medicine for 1 hour,after taking the medicine 1 day and after the ventilator was removed.Based on the data,the therapeutic effects of Sildenafil were studied.Results All 34 cases were cured.At the time point of 1 hour after taking Sildenafil,PAP,CVP and Ppeak decreased significantly,but pa (O2) and pa (O2)/FiO2 increased significantly (P ＜ 0.05).At the time point of 1 day after taking Sildinafil,SBP increased steadily,but PAP and CVP continued to get lower,and PIDS decreased significantly,while the liver lower bound was significantly reduced (P ＜ 0.05).At the time point of removing the ventilator,pa (O2) reached to (144.12 ± 26.25) mmHg,and hypoxemia was corrected,but PAP was reduced to (37.47 ± 3.77) mmHg,PIDS decreased to (17.56-± 1.94) scores,and heart failure was corrected.Compared with the previous 3 time points,there were statistically significant differences (P ＜ 0.05).Conclusion Sildenafil can highly selectively decrease pulmonary vascular resistance and obviously promote the correction of hypoxemia after operation of cyanotic congenital heart disease.