Incidence, clinical profile and short term outcome of cerebral abscess in cyanotic congenital heart diseases.

Background: Brain abscess is a serious infection of brain parenchyma in patients with cyanotic congenital heart disease (CCHD) and around 25-46 % of unrepaired CCHD patients develop brain abscess. Aim of this study was to determine the incidence, clinical features, microbiology and factors associated with early and short term outcome of cerebral abscess in CCHD. Method: ology: This is a retrospective study, conducted at Pediatric cardiology department, National Institute of Cardiovascular Diseases (NICVD) Karachi. The data was collected from January 2019 to December 2021. All CCHD patients between 1 and 25 years of age were included. Data of patients with cerebral abscess was reviewed. Results: Among the 544 pediatric patients hospitalized in the last two years, brain abscesses were identified in 51 (9.3 %). Polycythemia (31.4 %) was the most significant contributing factor, especially in patients aged above 10 years. The most frequently seen CCHD was tetralogy of fallot (TOF) 60.8 %. Majority of the patients (84.3 %) had a single abscess while 15.7 % had multiple abscesses. E coli (9.7 %) was the most common isolated pathogen. Immediate complication identified was cerebral edema in 22 (43.1 %). Four patients (7.8 %) died, 47 (92.2 %) patients completed treatment course. 45.1 % had complete recovery however, 17 (33.3 %) had neurological deficits, 8 (15.7 %) had seizures and 2 (3.9 %) patients had residual abscess. Prolonged hospitalization was observed in patients of age group <10 years. Conclusion: In patients with underlying CCHD,early referrals and intervention are key to mitigating the severe consequences of cerebral abscesses and can drastically improve patient outcomes.

Immediate and Short-Term Outcome of Percutaneous Atrial Septal Defects Closure in Adult Patients.

Background Atrial septal defect secundum (ASD II) is the commonest of the congenital heart diseases in the adult population and the closure of ASD II causes a significant improvement in hemodynamics and the clinical status of the patient. However, it carries certain risks, especially the development of post-closure pulmonary edema and congestive cardiac failure, which may lead to death. Therefore, this study was designed to share our experience and to evaluate the immediate and short-term outcomes of percutaneous closure of ASD II in adult patients presenting at a tertiary care cardiac center in Karachi, Pakistan. Methodology In this study, we included 30 adult (≥ 40 years) patients who underwent percutaneous ASD II closure at the pediatric cardiology department of the National Institute of Cardiovascular Diseases (NICVD), Karachi, Pakistan, between June 1, 2017, and July 31, 2019. Data for this study were extracted from a prospectively collected departmental database. Extracted data for this study consisted of demographic profile, comorbid conditions, echocardiographic findings, cardiac catheterization data, pre and post six-month electrocardiographic findings, and New York Heart Association (NYHA) Functional Classification (FC). Results Out of 30 adult patients, 18 (60%) were female. The mean age of the patients at the time of ASD closure was 51.43 ± 7.09 years, ranging between 40 and 67 years. None of the patients had any post-intervention complications. Mean systolic pulmonary artery pressure (SPAP) on cardiac catheterization pre-implantation was 49.8 ± 16.3 mmHg range was 20-90 mmHg while SPAP immediately after device implantation was 37 ± 11.4 range 20 to 65 mmHg with p<0.001. Mean SPAP on pre-catheterization echocardiography was 58.8 ± 14.3, range 30-95 mmHg, while after six months, the mean SPAP was 34.5 ± 7.2, range 20-45 mmHg, with p<0.001 in 28 (93.3%) patients (in two patients, there was no TR). There was no mortality up till six months after the intervention. The functional class (FC) after six months of the procedure improved in most, 90% (27), of the patients. Conclusion There were no complications immediately after the procedure. After six months, post-procedure SPAP decreased to < 50 mmHg in the majority of patients (96.6%). Functional class (FC) improved in most (90%) of the adult (≥ 40 years) patients undergone percutaneous atrial septal defects closure. Therefore, percutaneous closure of ASD II is a safe and effective procedure for adult patients.

Comparison of Size of Pulmonary Artery and Its Branches on Transthoracic Echocardiography Versus Computed Tomographic Angiography in Patients with Tetralogy of Fallot.

Background Transthoracic echocardiography (TTE) plays a vital role in the assessment of the surgical management of patients with tetralogy of Fallot (TOF). Accurate assessment of the main pulmonary valve annulus, main pulmonary artery (MPA), and branch pulmonary arteries are crucial for decision-making regarding the surgical approach in the form of total correction. It is also important for performing a systemic-to-pulmonary artery shunt operation and affects the outcome. In some patients with poor echogenic windows, it is sometimes difficult to obtain accurate measurements. Cardiac computed tomographic angiography (CTA) can be a superior diagnostic modality. Therefore, the aim of this study was to evaluate the degree of agreement between TTE and CTA in assessing the main pulmonary valve annulus and the size of the MPA and its branches among patients with TOF patients. Methodology Patients above one year of age, with TOF, presented during the study period of six months - from January 1, 2019, to June 30, 2019, were included in the study. All the patients had TTE and cardiac CTA to assess the annulus and the size of the MPA and its branches (right pulmonary artery (RPA) and left pulmonary artery (LPA)). CTA measurement of all parameters was compared with TTE measurement of the same on three different views each by computing the Bland-Altman plot and Pearson correlation coefficients. Results A total of 73 TOF patients were included in this study. The correlation coefficients between CTA and TTE for the measurement of the annulus were 0.767 and 0.833 for the parasternal short-axis view and the subcostal view, respectively. The correlation coefficients between CTA and TTE for the measurement of MPA were 0.820 and 0.866 for the parasternal short-axis view and the suprasternal view, respectively. The correlation coefficients between CTA and TTE for the measurement of RPA were 0.883 and 0.897 for the parasternal short-axis view and the suprasternal view, respectively. Similarly, the correlation coefficients between CTA and TTE for the measurement of LPA were 0.848 and 0.877 for the parasternal short-axis view and the suprasternal view, respectively. Conclusion In conclusion, there is a strong correlation and agreement between cardiac CTA and TTE for the assessment of the annulus and the size of the pulmonary artery (PA) and its branches in patients with TOF.

Early and Intermediate-Term Outcome of Balloon Aortic Valvuloplasty in Children With Aortic Stenosis and Left Ventricular Dysfunction at Tertiary Care Hospital.

Background Left ventricular (LV) dysfunction in patients with aortic valve stenosis (AVS) is seen in two scenarios: in neonates and in elderly patients. Neonatal AVS may present as a congestive cardiac failure (CCF), while older children rarely present with CCF if they have not been diagnosed early. Only a few reports of LV dysfunction with AVS have been described in the literature. However, there is a paucity of data regarding the safety and effectiveness of balloon aortic valvuloplasty (BAV) in children with AVS with LV dysfunction. Therefore, the aim of this study was to evaluate outcomes to establish the safety and effectiveness of BAV in children with AVS and LV dysfunction in improving LV function and survival. Methods A total of 160 BAVs were performed from 2004 to 2017; of these, 41 (25.6%) patients had LV dysfunction. We reviewed these cases, and data were obtained on clinical features, echocardiographic parameters including LV ejection fraction (LVEF) and LV dimensions, LV posterior wall, interventricular septal thickness, pressure gradient across the valve, aortic valve morphology and annulus and aortic insufficiency (AI), and angiographic parameters such as aortic and LV pressures, AI and annulus size, and balloon size. Echocardiography was done before the procedure, one day after intervention, at three months, at six months, and on regular follow-up. Mortality during and after the procedure and at follow-up was reported. Results Children who had undergone BAV for AVS and LV dysfunction within the age range of six to 192 months showed a significant reduction in peak-to-peak pressure gradient (PPG) from 73.5 ± 30 mmHg to 26.7 ± 6.7 mmHg and improvement in LVEF from 32.8 ± 11% to 54.3 ± 12.7% after 24 hours. Instantaneous gradient on echocardiography after three months showed PPG was 29.8 ± 7.7 mmHg and mean LVEF was 63 ± 8.6%. Mean LV end-diastolic pressure was 20.8 ± 4.7 mmHg and decreased to 13 ± 2.4 mmHg. Four patients died, all of whom had severe LV dysfunction - one died during the procedure and three died within six to 20 hours after successful BAV. On average follow-up of 6.4 ± 3.8 years, with a range of three months to 13 years, there was no mortality, pressure gradient increased to 40 ± 16.3 mmHg (range, 20 to 90 mmHg), and three had BAV after one, four, and six years, respectively. There was an increase in AI from mild to moderate in five patients, but they did not require any intervention. Four patients had aortic valve replacement (AVR) with two patients having an increase in pressure gradient and AI after eight and 13 years, respectively. One patient had AI (+3) after BAV had AVR after three years, and one patient who had a very thick and dysplastic aortic valve with LVEF of 20% and pulmonary hypertension (PH) had AVR after six months. Conclusion Patients with AVS who develop LV dysfunction deteriorate and die soon without treatment. Our data suggest that BAV in children with aortic stenosis and LV dysfunction is safe and effective in the normalization of LV function.

Correlation between Echocardiography and Cardiac Catheterization for the Assessment of Pulmonary Hypertension in Pediatric Patients.

Introduction Cardiac catheterization is widely considered the "gold standard" for the diagnosis of pulmonary hypertension. However, its routine use is limited due to its invasive nature. Therefore, the aim of this study was to evaluate the correlation between pulmonary artery pressures obtained by various parameters of transthoracic echocardiography and cardiac catheterization. Methods This study includes 50 consecutive patients with intracardiac shunt lesions diagnosed with severe pulmonary hypertension on echocardiography and admitted for cardiac catheterization at the National Institute of Cardiovascular Diseases (NICVD) in Karachi, Pakistan. Cardiac catheterization and transthoracic echocardiography were performed in all patients simultaneously and systolic (sPAP) and mean pulmonary artery pressure (mPAP) were assessed with both modalities. Correlations and agreement, in terms of Bland-Altman plot, were computed between both modalities for sPAP and mPAP. Results Out of 50 patients, 46% (23) were male and mean age was 7.49 ± 4.45 years. On cardiac catheterization, sPAP was 93.92 ± 17.91 mmHg and mPAP was 67.0 ± 14.28 mmHg. Correlation between cardiac catheterization and echocardiography for the assessment of sPAP was 0.917 (p<0.001), and mPAP was 0.832 (p<0.001) for mean gradient of tricuspid regurgitation (PGTRmean), 0.749 (p<0.001) for peak gradient of pulmonary regurgitation (PGPRpeak), 0.691 (p<0.001) for Acceleration time across right ventricular outflow tract (RVOT), and 0.752 (p<0.001) for end gradient of pulmonary regurgitation (PGPRend). Bland-Altman plot showed moderate agreement between two modalities. Conclusion A positive but modest correlation was observed between hemodynamic parameters of transthoracic echocardiography and cardiac catheterization for assessment of pulmonary artery pressures. Transthoracic echocardiography can reliably be used as an initial non-invasive modality for the assessment of pulmonary artery hypertension and can obviate the need of right heart catheterization in some patient especially with mild pulmonary hypertension.

To determine frequency of etiological factors in short statured patients presenting at an endocrine clinic of a tertiary care hospital.

OBJECTIVE: To determine the frequency of etiological factors in short statured patients presenting at endocrine clinic of National Institute of Child Health, Karachi. METHODS: This descriptive cross sectional study was conducted at Endocrine clinic of National Institute of Child Health, Karachi. One hundred children (48 boys and 52 girls) aged 3-15 years (mean 9.9±3.4) with short stature from January 2007 to July 2007 were evaluated during that period. RESULTS: Constitutional growth delay (CGD) and familial short stature (FSS) were identified as the most common, 55% of all short stature cases. Non-endocrinal causes as a single entity was detected in 17 children. Most common etiological factors in order of frequency were normal variant of growth (CGD, FSS), Hypothyroidism, Growth Hormone deficiency (GHD), and Celiac disease. GHD was found in 13% of total cases and it comprises 44% among endocrinal causes. Boys outnumbered girls with ratio of 2.7:1 (p<0.05). CONCLUSION: Most common cause of short stature was normal variants of growth as a group. Children with height falling below 0.4(th) percentile are more likely to have pathological cause.