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@#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.
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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%.
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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.
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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.
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Adult , Humans , Aging , Aorta , Aortic Coarctation , Arrhythmias, Cardiac , Atherosclerosis , Cardiovascular Diseases , Coronary Artery Disease , Coronary Vessels , Diabetes Mellitus , Heart Defects, Congenital , Heart Failure , Heart Ventricles , Hypertension , Hypertension, Pulmonary , Incidence , Obesity , Prevalence , Risk Factors , ThrombosisABSTRACT
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.
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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.
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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.
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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.
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Objective To investigate the incidence and risk factors of acute kidney injury (AKI) after cardiopulmonary bypass(CRB) in children with non cyanotic congenital heart disease. Methods A total of 150 children who underwent cardiopulmonary bypass operation from 2012 May to 2014 May were analyzed retrospectively. The patients were divided into two groups:AKI group (n=29) and non AKI group (n=121). Clinical data were collected and compared between two groups, including gender, age and baseline serum creatinine level. Multivariate Logistic regression analysis was used to evaluate the risk factors for AKI after operation. Results There was no death in the 150 cases. Comparison between groups, there were significant differences in age, history of heart failure and (or)pneumonia, baseline serum creatinine level, baseline uric acid, body mass, CPB) time, aortic clamping time, mechanical ventilation time, postoperative transfusion and hypotension. Multivariate Logistic regression analysis showed that age (≤1 years old), the history of pneumonia and (or) heart failure, CPB time (＞40 min), aortic clamping time (＞30 min) were the independent risk factors for AKI after operation. Conclusion It is important to choose the best age for surgery in children, actively prevent preoperative complications and shorten the CPB and aorta blocking time for preventing the occurrence of AKI.
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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.
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Se describe el caso clínico de una paciente de 62 años de edad con cardiopatía congénita cianótica, quien fue ingresada en el Servicio de Cardiología del Hospital Provincial Docente Clinicoquirúrgico "Saturnino Lora Torres" de Santiago de Cuba por presentar disnea. Al examen físico se constató cianosis, arritmia completa y soplo sistólico en mesocardio, con signos de insuficiencia cardíaca refractaria del lado derecho. Los exámenes complementarios efectuados confirmaron el diagnóstico de síndrome de Eisenmenger atribuible a comunicación interventricular. A pesar de la medicación que recibía evolucionó desfavorablemente hasta que falleció. Es poco frecuente que pacientes con esta complicación sobrevivan hasta la tercera edad.
A case report of a 62 year-old patient with cyanotic congenital heart disease, who was admitted to the Cardiology Department of "Saturnino Lora" Provincial Teaching Clinical Surgical Hospital of Santiago de Cuba to present with shortness of breath was described. On physical examination cyanosis, complete arrhythmia and systolic murmur in mesocardium with signs of refractory heart failure of the right side were found. Complementary tests confirmed the diagnosis of Eisenmenger syndrome attributable to ventricular septal defect. Despite receiving medication she made bad progress and died. It is rare for patients with this complication survive into old age.
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BACKGROUND: The prostaglandin E1(PGE1) is a well known protent dilator of arteriosus. Maintaining of the patency of ductus arteriosus is crucial for the survival of patients suffering from ductus-dependent cyanotic congenital heart disease. We aimed to analyse the efficacy and the influencing factors upon PGE1 in patients suffering from this disease. METHODS: Between May 1991 and April 1993, 26 neonates and infants with ductus- dependent cyanotic congenital heart disease received on intravenous infusion of PGE1 in the Division of Pediatric Cardiology. Yonsei Cardiovascular Center. The result was a dramatic improvement in systemic arterial oxygen tension and oxygen saturation during infusion of PGE1with a dependency on the infusion of PGE1. We evaluated the arterial blood gas analysis both at the immediate pre-infusion stage and 2 hours after infusion. We aimed to analyse the factors which may influence the intravenous of PGE1to infant suffers of ducts-dependent cyanotic congenital heart disease, such as pulmonary atresia(n=14), severe pulmonary stenosis(n=7) or complete transposition of the great arteries(n=5). RESULTS: 1) There was a significant increase in PaO2 and Oxygen saturation 2 hours after the infusion of PGE1. This appeared to be unrelated to the different forms of the disease when compared with the pre-infusion values. 2) The infants' responsiveness of the ductus arteriosus appeared to be age related with significant differences emerging between the 2 group(p<.05). In infants younger than 9 hours old, the differences in PaO2 changes between pre-infusion and post-infusion of PGE1 were 16.3+/-3.7mmHg compared to just 10.4+/-0.4mmHg in infants older than 96 hours. 3) No significant difference emerged between an increase in PaO2or oxygen saturation relating to the shape of ductus arteriosus ; or the level of PaO2prior to the infusion. 4) The side effects of PGE1were as follows ; fever(84.6%),loose stool(61.5%), apnea(30.8%) and hypotension(15.4%), etc.. CONCLUSION: PGE1provides excellent medical palliation for infants suffering from ductus-dependent cyanotic congenital heart disease until the pulmonary arteries are large enough for a modified Blalock-Taussig shunt ; or until corrective surgery is possible.
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Humans , Infant , Infant, Newborn , Alprostadil , Blalock-Taussig Procedure , Blood Gas Analysis , Cardiology , Ductus Arteriosus , Heart Defects, Congenital , Infusions, Intravenous , Oxygen , Pulmonary ArteryABSTRACT
Objective To improve the recognition of renal impairment with long- standing cyanotic congenital heart disease, to avoid occurrence of renal impairment before and after cardiac surgery. Methods The clinical, laboratory data and treatment course of renal impairment in one child with long - standing cyanotic congenital heart disease were investigated in this study, and the related literature were reviewed Results After 10 years of cyanotic congenital heart disease course, this child presented with nephritic syndrome, renal dysfunction, hyperuricaemia, secondary polycythaemia. After loosen the limitation on fluid intake,decrease the blood viscosity and urine protein,the child was transferred to other hospital for cardiac surgery. Conclusions Children with long- standing cyanotic congenital heart disease can lead to renal impairment, we must pay attention to renal impairment to decrease incidence of acute renal failure after cardiac surgery.
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Fourteen cases of brain abscess with cyanotic congenital heart disease operated during 10 years from 1974 to 1983 were reviewed. The abscess associated with cyanotic congenital heart disease constituted 21.8%(14/64) of total brain abscesses during this period. Brain abscess occurred in 1.8%(14/784) of the patients with cyanotic congenital heart disease. Of 14 cases, 12 had a tetralogy of Fallot and others were transposition of the grear vessels and pulmonary atresia. A peak in the age distribution was seen at 5 to 11 years of age and the preponderance of males was present(2:1=M:F). The major symptoms and signs were due to the increased ICP and 21.4%(3/14) of the abscesses was nonfebrile. All of the abscesses were supratentorial and located in temporal, parietal, occipital, frontal area in order of frequency. In one case multiple abscess was present. Sterile cultures were obtained in 57.1%(8/14) of the abscesses. Anaerobic streptococcus, bacteroides, streptococcus, staphylococcus, G(-) rods were cultured. All of the abscesses were treated by surgical management with the overall mortality of 4.3%(2/14). Total excision of the abscess generally resulted in better outcome. Patients were operated upon in the stabilized stage of the disease clinically with the aid of serial CT scan evidence. The introduction of CT scan has facilitated early diagnosis, location, and investigation of the evolution of a abscess to obtain a better postoperative result. However, recent progress in neurosurgery and cardiac surgery has made it possible to extend the normal life of these patients. Therefore we should have to give our attention to the surgical management of the brain abscess associated with cyanotic congenital heart disease seriously.