Early Diagnosis of Congenital Heart Disease Improves Outcome

Congenital heart disease has an overall incidence of 8-10/1000 live births and is similar across the globe. The incidence may be higher in countries where consanguinity is high, suggesting an autosomal recessive gene as a risk factor. Advances in surgical expertise has improved outcome of simple and complex heart diseases. Early diagnosis and referral to centers caring for such babies is an important contributory factor to a better outcome. This review focuses on early diagnosis of congenital heart disease in neonates and children by Health Care Physicians (General and Family Physicians) and Pediatricians. Careful neonatal and pediatric cardiovascular examination, screening pulse oximetry on all newborns before hospital discharge and an early post natal follow up are important to diagnose CHD

Clinical profile and outcome of cyanotic congenital heart disease in neonates

OBJECTIVE: To determine the clinical profile and assess the outcome of all neonates diagnosed with cyanotic congenital heart disease. STUDY DESIGN: A case series. PLACE AND DURATION OF STUDY: The Aga Khan University Hospital from January 1998 to December 2000. PATIENTS AND METHODS: Neonates admitted with diagnosis of cyanotic congenital heart disease were evaluated for clinical diagnosis, survival and mortality. RESULTS: Forty four neonates met the inclusion criteria. Eleven babies (25%) had Tetralogy of Fallot or its variants. Other malformations were d-transposition of great arteries, tricuspid valve anomalies (tricuspid atresia and Ebstein\u27s anomaly), hypoplastic left heart syndrome, truncus arteriosus, total anomalous pulmonary venous return and complex congenital heart disease like single ventricle. Twenty eight (63.6%) neonates survived and 16 (36.4%) expired during hospital stay. Cause of death was surgical in 2 cases and medical problems in 14 babies. CONCLUSION: Tetralogy of Fallot or variants was the commonest cyanotic heart disease in neonates with frequency of 27.27%. Majority of neonates with congenital cyanotic heart disease showed survival with appropriate management

Surgical outcomes of pediatric patients with ventricular septal defects in a tertiary referral center in Pakistan: a retrospective cohort study

Background: The nature of complications and outcomes for Ventricular septal defect (VSD) repair differ between developed and developing countries. Understanding these differences can help optimize patient management according to geographical location.Objective: The purpose of this study was to investigate the risk factors associated with in-hospital peri- and post-surgical complications of VSD repair in a developing country.Design: A retrospective cohort study. Setting: A tertiary referral center. Participants: 117 patients under 18 years of age admitted for surgical closure of ventricular septal defect between July 1998 and June 2008.Main Outcomes: Only patients with isolated VSD or VSD associated with acyanotic congenital heart disease were included. Outcomes were defined as in-hospital minor and major complications and mortality.Results: Adverse complications occurred in 35.9% (42/117) and death in 3.4% (4/117) of cases. Age \u3e12 months (OR 0.17 13 months-5 years; 0.10 5-18 years), weight \u3e10 kg (OR 0.24 11-20 kg; 0.13\u3e21 kg), and absence of pulmonary artery hypertension (OR 0.43) were all significantly associated with a reduced rate of adverse events. A longer stay in intensive care unit/semi-intensive care unit (OR 11.6 5-7 days; OR 6.1 \u3e8 days) and larger size of ventricular septal defect (OR 5.4 medium size; 3.9 large size) were associated with an increased risk of adverse events. Infection (20%) and pneumonia (10%) were the commonest complications. Conclusions and relevance: Age under one year, weight less than ten kilograms, pulmonary artery hypertension, and moderate to large size ventricular septal defect are more likely to be associated with adverse outcomes after surgical repair of ventricular septal defect. Infection and pneumonia comprise almost one third of the total complications, compared to higher arrhythmic events in developed countries. Developing countries have a distinct spectrum of complications that may be avoidable and treatable

Congenitally Corrected Transposition of the Great Arteries in a Septuagenarian from the Developing Country of Pakistan

Congenitally corrected transposition of the great arteries (CCTGA) is a rare congenital cardiac defect with atrioventricular and ventriculoarterial discordance which leads to heart failure and limits patients\u27 lifespan. We report the case of a 70-year-old lady, from a poor province in Pakistan, who presented for the first time with palpitations and was diagnosed to have CCTGA. She had moderate pulmonic valve stenosis which was protective against heart failure. She had six children all born via spontaneous vertex delivery in her local village. This case exemplifies the fact that pulmonic stenosis is favourable for patients with CCTGA. In a country where the average life expectancy of females is only 68 years, the survival of our patient with CCTGA beyond the average lifespan is indeed interesting

Clinical screening for congenital heart disease in newborns at a tertiary care hospital of a developing country

Objective: To screen all newborns admitted to a tertiary care hospital to rule out congenital heart disease before discharge and to find out the utility of pulse oximetry to detect congenital heart disease. Methodology: This prospective study was done at Aga Khan University Hospital from January 2014 to December 2014 in 1,650 newborns over a period of 12 months. Pulse oximetry and clinical examination were done. Persistent oxygen saturation less than 95% was considered as positive pulse oximetry. Newborns who had positive pulse oximetry or abnormal clinical examinations findings were subjected to echocardiography. Result: Pulse oximetry was performed on 1,650 newborns, out of which 25 (1.5%) had congenital heart disease. Positive pulse oximetry cases were 16 (0.97%), out of which 10 had only positive pulse oximetry (negative clinical examination). Positive clinical examination cases were 45 (2.7%), out of which 39 cases had only positive clinical examinations (negative pulse oximetry). Six newborns had both positive pulse oximetry and positive clinical examination. Out of the 25 diagnosed cases of congenital heart disease, ventricular septal defect (VSD) was the most common congenital heart disease, followed by patent ductus arteriosus (PDA). The sensitivity, specificity, positive predictive value, and negative predictive value of pulse oximetry were 32%, 99.5%, 50%, and 98.9% respectively. Conclusion: The community setting of a developing country, a combination of pulse oximetry screening and clinical examination are better at detecting congenital heart defects than either test alon

Kawashima operation: functional modification of bidirectional Glen shunt with left superior vena cava in single ventricular morphology

Single ventricle physiology offers limited options with significant morbidity and mortality. The Glenn shunt is a mid-stage procedure for the Fontan circulation. With persistent left Superior Vena Cava (SVC) and Azygos continuation of the Inferior Vena Cava (IVC) to the SVC, needs the development of bilateral bidirectional Glenn shunts. Fifteen patients underwent Bidirectional Glen shunt procedure over a period of ten years. Of these two were found to have left SVC along with interrupted IVC and its Azygos continuation to the SVC. These two patients underwent the Kawashima operation. A seven years old girl was admitted via emergency with severe cyanosis. She had tricuspid atresia, azygos continuation of IVC with single ventricle physiology. She had a complicated postoperative course with prolonged hospital stay after bilateral bidirectional Glen shunt (Kawashima operation). She successfully has completed Fontan after 5 years. The second case ofa 7 year old girl had elective surgery for DORV (double outlet right ventricle), pulmonary atresia and azygos continuation of IVC and persistent left SVC. She had an uneventful postoperative course. The Kawashima operation is an established surgical procedure to deal with bilateral SVCs. The procedure is safe; outcome is favourable and prepares the patient for total cavo-pulmonary shunt

Reversible obstructive sleep apnea and right heart failure due to massive tonsillar hypertrophy

Obstructive sleep apnea is a relatively common but under diagnosed clinical entity in children. Adenotonsillar hypertrophy is the most common cause for upper airway obstruction in pediatric patients. If the obstruction to upper airways is not relieved, then the child can develop obstructive sleep apnea and its consequences. Treatment is simply to remove the obstruction thereby restoring patency of upper airway

Accuracy of echocardiography in diagnosing total anomalous pulmonary venous return

Objective: Total anomalous pulmonary venous return is an uncommon cyanotic congenital heart defect. Echocardiography is the initial diagnostic tool. Complimentary non-invasive modalities like cardiac computerized tomographic angiography and cardiac magnetic resonance imaging have replaced the need for cardiac catheterization in difficult cases. This study aimed to determine the accuracy of echocardiography in diagnosing total anomalous pulmonary venous return, and to determine the factors that may decrease its sensitivity.Methods: This was a cross-sectional study conducted at the Aga Khan University Hospital Karachi, Pakistan from January 2010 to August 2016. All patients who were diagnosed with Total anomalous pulmonary venous return on echocardiography and had subsequent confirmation either on cardiac CT angiography or surgery were included. The diagnostic accuracy of echocardiography was expressed as sensitivity. Previously described taxonomy was used to define diagnostic error. Univariate and multivariate analysis were done by logistic regression OR (95% CI) were reported to identify factors causing the diagnostic error.Results: High diagnostic sensitivity (81%) was found in isolated total anomalous pulmonary venous return and low (27%) in heterotaxy and mixed (20%) varieties. Poor acoustic windows and right isomerism were found to be significant factors responsible for the diagnostic error on multivariate analysis.Conclusion: Echocardiography can diagnose isolated total anomalous pulmonary venous return with high accuracy. Use of additional modalities may be required for a complete diagnosis in cases with mixed variety, heterotaxy and poor acoustic windows

Cardiac involvement in kawasaki disease in Pakistani children

Introduction: Coronary artery involvement is the most dreaded long-term complication of Kawasaki disease. Our aim was to look at the pattern of cardiovascular involvement in Pakistani children admitted with Kawasaki disease.Methods: This study included children admitted with Kawasaki disease at the Aga Khan University Hospital Karachi over a period of 14 years from January 1997 to December 2010. Information gathered included patient demographics, clinical features, investigations, echocardiographic findings, treatment and follow-up. Those with coronary artery involvement on initial echocardiogram remained on long-term follow-up with clinical examination and echocardiogram.Results: A total of 56 patients were admitted. (Mean age at diagnosis 33 ± 30 months, age range 2 months to 9 years). 18% of patients had incomplete features. Twenty-five percent (14/56) patients presented after 10 days of fever. Cardiac examination was normal except for tachycardia. Abnormal coronary arteries were seen in 23 patients (41%) - left main coronary artery in 23 (41%), left anterior descending and right main coronary artery in 20 (36%), circumflex branch in 17 (30%). Risk factors for cardiac involvement were male sex, fever \u3e10 days duration at the time of initial presentation and neutrophil percentage \u3e75% in the initial white blood cell counts. Fifty four of 56 cases received intravenous immunoglobulin (IVIG), Seventy-five percent of the patients received IVIG within 10 days of illness. Mean duration of follow-up was 2.5 years. Eight percent of the patients still continue to have abnormal coronaries. There was no mortality.Conclusions: A higher incidence of coronary artery involvement was found in our study. Presentation after 10 days of illness increases the risk of coronary artery involvement. High index of suspicion among the general pediatricians about the disease can possibly be helpful for early referral and treatment