Cyanotic Congenital Cardiac Disease Research Articles

Analysis of Congenital Heart Defect in Pakistan

Background and objectives: While the defect has been studied extensively in Pakistan, there have been just a few of research focusing on KPK populations. Because of this, the goal of this study was to identify neonatal congenital heart disease in Pakistani children. Methodology: This cross sectional study was conducted in Sheikh Zayed Medical College during June 2021 to November 2021. Echocardiography was used to diagnose congenital heart disease in 1683 neonates who reported to the outpatient department (OPD) with a variety of ailments. Results: Out of 1683 babies, a total of 303 (or 18%) had a congenital heart condition. It was found that 62.5 per cent of people infected with the virus were men, while 37.5% of them were women. 78 per cent of the cases included infants between the ages of one and three months. Patients described a variety of symptoms they were experiencing. Asthma, pneumonia, and cyanosis were the most prevalent causes of death (4 per cent). The great majority of patients had the cyanotic congenital cardiac disease (98 per cent). (77.9 per cent), followed by PDA (11.5 per cent), ASD (7.9 per cent), TOF (0.9 percent), and aortic stenosis (0.9 per cent) were the most common congenital heart defects observed in the study. (Aortic stenosis was the least common disease) (2.2 per cent). Conclusion: To detect cardiac murmurs in our newborn population, the doctors in our paediatric outpatient clinics must increase their performance. This may result in a significant decrease in sales.

Our experience of epidural anesthesia for cesarean section in pregnant women with corrected tetralogy of fallot: A case report

Tetralogy of Fallot (TOF) is the most commonly encountered cyanotic congenital cardiac disease in pregnancy. While most pregnancy cases after surgical repair of TOF have focused on cardiovascular and obstetric concerns, relatively few authors have focused on specific intraoperative and postoperative anesthetic management strategies. Determine the type of anesthesia in these patients is very difficult and current recommendations are based only on reported clinical experiences and pathophysiological concepts. In this case report, we aimed to share our epidural anesthesia experience for caesarean surgery in 37 weeks pregnant who underwent corrected TOF surgery.

Cyanotic Congenital Cardiac Disease and Scoliosis: Pre-Operative Assessment, Surgical Treatment, and Outcomes

Objective: Congenital heart disease (CHD) is associated with the development of scoliosis. Improvements in cardiac care have extended survival of children with cyanotic CHD which possess a need for correction of scoliosis. There is limited information on spinal care for these patients. We present 3 patients with CHD who underwent surgical correction of scoliosis. Materials and Methods: We reviewed demographic and clinical data on patients with cyanotic CHD. Results: Patient 1 underwent posterior spinal fusion T3–L3 at the age of 16 years. He had a double inlet left ventricle and was treated with completion of a Fontan circulation. Hypotensive anaesthesia was used but he lost 3,000 mL of blood. The operative time was 370 min and most of the blood loss occurred in the second half of the procedure. Patient 2 underwent posterior spinal fusion T5–T12 when aged 14 years. She had transposition of the great vessels corrected over multiple surgeries. Hypotensive anaesthesia was used, she had blood loss of 300 mL, and the surgical time was 282 min. Patient 3 underwent posterior spinal fusion extending from T5–T12 when he was 17 years old. He had a double inlet left ventricle and was treated with completion of a Fontan circulation. Hypotensive anaesthesia was used, he had blood loss of 1,021 mL, and a surgical time of 342 min. Conclusion: Scoliosis surgery in patients with complex cardiac disease may be indicated to treat progressive deformities which produce severe symptoms. A multidisciplinary approach including a spinal surgeon as well as a cardiologist, haematologist, respiratory paediatrician, and spinal anaesthetist can evaluate the general medical condition and weigh the benefits and risks of surgery. Deformity correction can be performed using a meticulous technique and has produced a series of satisfactory outcomes.

Anaesthesia for Complex Cyanotic Congenital Cardiac Disease in a Child for Emergency Laparotomy

Anaesthesia for Complex Cyanotic Congenital Cardiac Disease in a Child for Emergency Laparotomy

0079 - Cyanotic Congenital Cardiac Disease and Scoliosis: Preoperative Assessment, Surgical Treatment and Outcome

ABSTRACT: Congenital heart disease (CHD) affects 1% of live births (10% cyanotic CHD). There is well-recognised association between CHD and scoliosis. Improvements in cardiac care have extended survivorship with increase in the need for deformity correction. There is limited information on the spinal care of these patients. In patients with univentricular heart it has been recommended that surgery is performed earlier in life to prevent bleeding complications. We present 3 patients with CHD who underwent scoliosis correction and review the literature on this rare condition.

Cardiac protective effects of remote ischaemic preconditioning in children undergoing tetralogy of fallot repair surgery: a randomized controlled trial.

AimsRemote ischaemic preconditioning (RIPC) by inducing brief ischaemia in distant tissues protects the heart against myocardial ischaemia-reperfusion injury (IRI) in children undergoing open-heart surgery, although its effectiveness in adults with comorbidities is controversial. The effectiveness and mechanism of RIPC with respect to myocardial IRI in children with tetralogy of Fallot (ToF), a severe cyanotic congenital cardiac disease, undergoing open heart surgery are unclear. We hypothesized that RIPC can confer cardioprotection in children undergoing ToF repair surgery.Methods and resultsOverall, 112 ToF children undergoing radical open cardiac surgery using cardiopulmonary bypass (CPB) were randomized to either a RIPC group (n = 55) or a control group (n = 57). The RIPC protocol consisted of three cycles of 5-min lower limb occlusion and 5-min reperfusion using a cuff-inflator. Serum inflammatory cytokines and cardiac injury markers were measured before surgery and after CPB. Right ventricle outflow tract (RVOT) tissues were collected during the surgery to assess hypoxia-inducible factor (Hif)-1α and other signalling proteins. Cardiac mitochondrial injury was assessed by electron microscopy. The primary results showed that the length of stay in the intensive care unit (ICU) was longer in the control group than in the RIPC group (52.30 ± 13.43 h vs. 47.55 ± 10.34 h, respectively, P = 0.039). Patients in the control group needed longer post-operative ventilation time compared to the RIPC group (35.02 ± 6.56 h vs. 31.96 ± 6.60 h, respectively, P = 0.016). The levels of post-operative serum troponin-T at 12 and 18 h, CK-MB at 24 h, as well as the serum h-FABP levels at 6 h, after CPB were significantly lower, which was coincident with significantly higher protein expression of cardiac Hif-1α, p-Akt, p-STAT3, p-STAT5, and p-eNOS and less vacuolization of mitochondria in the RIPC group compared to the control group.ConclusionIn ToF children undergoing open heart surgery, RIPC attenuates myocardial IRI and improves the short-term prognosis.

A rare case of dengue fever in patient with dual inlet left ventricle

Dengue viral infection is one of the differential diagnoses in patients having fever with thrombocytopenia. Early diagnosis and timely supportive treatment form the basis for the successful management of these patients. Dengue infection associated with cyanotic congenital cardiac disease makes management more difficult and is associated with a fatal outcome. We report a case in which the patient presented with fever and thrombocytopenia diagnosed to be a case of dengue infection (NS-1 antigen-positive) and associated cyanotic congenital heart disease (double inlet left ventricle/univentricular heart). The patient was successfully managed with fluids and platelet transfusion. This case report mainly emphasizes the difficulties faced in hemodynamic monitoring and supportive management during the acute course of the disease.

857: Incidence of perinatal and infant death in a large cohort with congenital cardiac disease

To examine the incidence of intrauterine fetal death (IUFD) and neonatal and infant mortality by gestational age at birth in cyanotic and non-cyanotic congenital cardiac disease in a large statewide database.

Etienne‐Arthur Louis Fallot and his tetralogy

Étienne-Arthur Louis Fallot (1850-1911) is one of the most significant medical figures of the 19th century with an eponymous congenital cardiac malformation. His initial account of the four anatomical features making up his tetralogy proved remarkably significant in the progression of clinical descriptions of the lesions producing cyanotic congenital cardiac disease. Although subsequently the cause of appreciable controversy, the accuracy of his initial descriptions underscore the current diagnostic and therapeutic approach to the malformation now known uniformly as tetralogy of Fallot.

An adult patient with tetralogy of Fallot and anomalous left anterior descending artery after conduit type of repair (RCD code: IV‑2A.1)

Tetralogy of Fallot is one of the most common cyanotic congenital cardiac disease concerning 10% newborn with congenital heart failures. About 3% of those patients have anomalous left anterior descending coronary artery. We report a case of 21 – year – old patient with tetralogy of Fallot and with coronary artery abnormality after staged cardiac surgeries. Due to an anomalous coronary artery, crossing the right ventricule outflow tract, the patient needed to conduit type of repair. JRCD 2014; 1 (8): 19–26

Management of adults with Tetralogy of Fallot

Tetralogy of Fallot is the most common form of cyanotic congenital cardiac disease. Patients with previously repaired tetralogy of Fallot are the most common patients seen in the Program for Adults with Congenital Heart Disease at The Johns Hopkins All Children's Heart Institute. Guidelines for the management of these patients are available from multiple sources including The American College of Cardiology (ACC) and The American Heart Association (AHA), The Canadian Cardiovascular Society, and The European Society of Cardiology (ESC). These guidelines describe multiple components related to the care for these patients including strategies for medical follow-up, the management of arrhythmias and electrophysiological diseases, and the treatment of chronic pulmonary insufficiency and stenosis. Several new strategies are available for replacement of the pulmonary valve including transcatheter replacement of the pulmonary valve and replacement of the pulmonary valve with a self-manufactured bicuspid polytetrafluoroethylene pulmonary valve.

Vascular endothelial growth factor and its soluble receptor in infants with congenital cardiac disease

Patients with cyanotic congenital cardiac disease often develop major aortopulmonary collaterals. Vascular endothelial growth factor is a key promoter of angiogenesis. Its soluble receptor-1 acts as a potent antagonist. We studied 30 infants with cyanotic congenital cardiac disease and 27 infants with acyanotic congenital cardiac disease. Central venous plasma vascular endothelial growth factor and soluble vascular endothelial growth factor receptor-1 levels were measured before, and 24 and 96 hours after surgery. There was no difference between plasma vascular endothelial growth factor levels in infants with cyanotic and those with acyanotic congenital cardiac disease. In cyanotic infants, the soluble vascular endothelial growth factor receptor-1 levels tended to be higher than in the acyanotic infants. In conclusion, there is no significant difference in the plasma levels of vascular endothelial growth factor and its soluble receptor-1 between infants with cyanotic and those with acyanotic congenital cardiac disease.

Late signs of cyanotic congenital cardiac disease

Late signs of cyanotic congenital cardiac disease

Erythrocyte indexes, iron metabolism, and hyperhomocysteinemia in adults with cyanotic congenital cardiac disease

A high percentage of cyanotic adults (37%) with cyanotic congenital cardiac disease (CCD) presented with depleted iron stores (13 of 52) or latent iron deficiency (6 of 52), even in a CCD center in which cyanotic patient phlebotomy is mostly avoided. In many of these patients, hypochromia and microcytosis was frequent, whereas hyperchromia and macrocytosis were relatively common.Furthermore, 50% of patients presented with hyperhomocysteinemia, possibly related to folate or B vitamin deficiencies, which may increase red blood cell size and color, explaining the lack of microcytosis and hypochromia in many cyanotic patients with iron deficiency.

99 EFFECTS OF CHRONIC HYPOXIA ON CARDIAC ELECTROPHYSICLOGICAL FUNCTION

Because of arrhythmias seen in children with cyanotic congenital cardiac disease, we compared electrophysiological (EP) function in normoxically raised rabbits (N) with EP function in rabbits raised in an hypoxic environment (H, ambient pO2=60 torr). Twelve week-old rabbits (N, H) were anesthetized with pentobarbital sodium and alpha-chloralose. Insulated stainless steel wires were sutured directly to the right atrium for pacing. Surface ECG and arterial blood pressure (EP) were recorded. An intra-cardiac electrogram was recorded from a bipolar catheter inserted via the left superior vena cava into the coronary sinus. Data are presented in milliseconds and are compared as irean ± standard error of the mean. Significance was set at p<0.02. The control cycle length in H (330±9.9) was significantly longer than in N (231±16.2). Rapid atrial pacing resulted in a corrected pacemaker recovery time (CPRT) that was not significantly different between the two groups. Single programed extra stimuli ware used to evaluate atrial and AV nodal effective (AERP, AVNERP) and functional refractory periods (AFRP, AVNFRP). Statistically significant differences were found in AFRP, AVNFRP, and in AERP when results of N were compared to H. AVNERP was not significantly different. BP did not differ between H and N prior to or during the pacing protocol. We conclude that, in this model, chronic hypoxia during maturation alters control cycle length and influences cardiac EP function. This information may be important in evaluating arrhythmias in children with cyanotic congenital cardiac disease.

Complex congenital heart disease: A multiplicity of therapeutic options

DR. ROBERTS: In this report, a boy with cyanotic congenital cardiac disease will be presented and problems manifested in him will be discussed. Dr. Scott will describe his patient. DR. SCOTT: T. H. (CHNMC No. 299 3972), a 7year-old black boy who died in December 1976, was noted to have cyanosis and a precordial murmur at 2 weeks of age. Shortly thereafter signs of congestive heart failure developed. At 3 months of age, cardiac catheterization (Table I) provided a diagnosis of transposition of the great arteries with single ventricle. The pulmonary trunk was banded at this time with reduction of the systolic pressure in this artery from 85 to 45 mm. Hg (Fig. 1). Despite this procedure, however, the child failed to thrive and cyanosis increased. Inadequate intracardiac mixing of blood was considered the reason and accordingly a Rash-

Acquired pulmonic atresia: Total obstruction to right ventricular outflow after systemic to pulmonary arterial anastomoses for cyanotic congenital cardiac disease

Attention is called to the development of total obstruction to right ventricular outflow in patients with cyanotic congenital cardiac disease after systemic to pulmonary arterial anastomoses. The clinical and necropsy features are described in 4 patients in whom right ventricular outflow obstruction developed (in 3 at valve level-“acquired pulmonic atresia”-and in 1 at infundibulum) from 13 to 19 years after pulmonary to systemic shunt operations. The mechanism of development of right ventricular outflow obstruction in these patients is discussed.

Aneurysmal dilatation of the coronary arteries in cyanotic congenital cardiac disease: Report of a forty year old patient with the Taussig-Bing complex

Clinical, hemodynamic, angiocardiographic and autopsy observations are described in a forty year old woman with right ventricular origin of the aorta and biventricular origin of the pulmonary trunk. She lived longer than any previously described patient with this congenital malformation. At angiography and at autopsy the extramural coronary arteries were found to be aneurysmally dilated and tortuous. Coronary arterial ectasia has been described once before in patients with cyanotic congenital cardiac disease, but the remarkable degree of dilatation and tortuosity found in our patient has not been recorded.

Complete transposition of the great vessels in total situs inversus: Anatomic, electrocardiographic and radiologic observations

A rare instance of complete transposition of the great vessels occurring in a 31 month old child with total situs inversus is reported as a basis for demonstrating the pathologic, electrocardiographic, vectorcardiographic and radiologic aspects of this rare condition. In the usual instance of total situs inversus with cyanotic congenital cardiac disease, transposition of the “corrected” type is commonly present and associated with inversion of the ventricles and atrioventricular valves.

Changes in bone associated with cyanotic congenital cardiac disease

Changes in bone associated with cyanotic congenital cardiac disease