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Mehnaz Atiq
10/2011; , ISBN: 978-953-307-660-7
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ABSTRACT: Pulsus alternans is a well-recognized clinical entity in which alternating strong and weak pulses are detected. It usually is secondary to underlying myocardial failure. Murmur alternans (alternation in murmur intensity) has been described in aortic stenosis and a few right-sided lesions such as pulmonary hypertension and embolism. This report describes a case of murmur alternans in critical pulmonary stenosis that also showed Doppler alternans on echocardiography. The underlying cause was right ventricular systolic dysfunction.
Pediatric Cardiology 07/2011; 32(7):1004-7. · 1.30 Impact Factor
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ABSTRACT: Congenital heart disease (CHD) is associated with multiple risk factors, consanguinity may be one such significant factor. The role of consanguinity in the etiology of CHD is supported by inbreeding studies, which demonstrate an autosomal recessive pattern of inheritance of some congenital heart defects. This study was done to find out the risk factors for CHD.
A case-control study was done on pediatric patients at a tertiary care hospital, Aga Khan University Hospital, located in Karachi, Pakistan. A total of 500 patients, 250 cases and 250 controls were included in the study.
Amongst the 250 cases (i.e. those diagnosed with CHD), 122 patients (48.8%) were born of consanguineous marriages while in the controls (i.e. non-CHD) only 72 patients (28.9%) showed a consanguinity amongst parents. On multivariate analysis, consanguinity emerged as an independent risk factor for CHD; adjusted odds ratio 2.59 (95% C. I. 1.73 - 3.87). Other risk factors included low birth weight, maternal co-morbidities, family history of CHD and first born child. On the other hand, medications used by the mother during the index pregnancy, maternal age and gender of the child did not significantly increase the risk of developing CHD.
Analyses of our results show that parental consanguinity, family history of CHD, maternal co-morbidities, first born child and low birth weight are independent risk factors for CHD.
Annals of Pediatric Cardiology 07/2011; 4(2):117-21.
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ABSTRACT: Implantation of cardiac pacemakers has been practiced for at least five decades with continuous developments of the hardware. The invention of dual chamber pacemakers has initiated a debate concerning its superiority over single chamber ventricular pacemakers. Throughout the world, surgeons have been using dual chambered permanent pacemakers with successful follow ups. However, Pakistan has not yet taken the advantage of such pacemaker devices till now. We report three cases that underwent a dual chamber permanent pacemaker implantation for the first time in children less than 8 kg with successful follow ups.
Journal of the Pakistan Medical Association 04/2011; 61(4):421-3.
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International Journal of Collaborative Research on Internal Medicine & Public Health International Journal of Collaborative Research on Internal Medicine & Public Health. 04/2009; 1(1):84-99.
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ABSTRACT: A large number of patients treated at our hospital for endocarditis have negative cultures. Taking into consideration the fact that many of these patients receive antibiotics prior to referral, we decided to study culture-negative endocarditis in Pakistan.
The medical records of all patients admitted to the Aga Khan University Hospital, Pakistan, for the period from 1988 to 2001, with an underlying diagnosis of infective endocarditis (IE) and negative cultures, were reviewed.
Of the 159 patients diagnosed with IE by revised Duke criteria, 86 (54.1%) had persistent negative cultures. More than half of these patients (52%) had received antibiotics before being referred to our center. Patients with culture-negative endocarditis were less likely to be classified as definite endocarditis by revised Duke criteria (p<0.001, 95% CI 0.07-0.3) or to have large vegetations (p=0.021, 95% CI 0.05-0.5), and more likely to have a mitral valve prolapse (p=0.003, 95% CI 1.6-2.3). Definite endocarditis (p=0.042, 95% CI 1.02-7.4), heart failure (p=0.008, 95% CI 1.4-12.7), renal failure (p=0.017, 95% CI 1.16-40.7), embolism (p=0.019, 95% CI 1.2-38.8), and neurological complications (p=0.02, 95% CI 1.16-9.2) were associated with an increased mortality.
Culture-negative endocarditis is very common among patients with IE in Pakistan. The presentation, laboratory findings, and complications are similar to those for culture-positive endocarditis. It is postulated that previous antibiotic treatment is the most common cause of culture-negative endocarditis in our hospital.
International journal of infectious diseases: IJID: official publication of the International Society for Infectious Diseases 02/2009; 13(5):606-12. · 2.17 Impact Factor
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ABSTRACT: 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.
Journal of the Pakistan Medical Association 02/2009; 59(1):43-5.
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ABSTRACT: This study was undertaken to determine the diastolic Doppler echocardiographic correlates of pulmonary vascular resistance calculated on cardiac catheterization in patients with secondary pulmonary arterial hypertension. Thirty-eight consecutive patients with congenital heart disease, pulmonary artery hypertension and pulmonary regurgitation were studied. Continuous-wave Doppler-derived pulmonary artery diastolic gradients were measured at 3 points on the pulmonary regurgitant diastolic velocity slope: peak diastolic, end-diastolic (at the R wave on the electrocardiogram), and mid-diastolic (midway between the peak and end-diastolic points). Catheterization data included oximetry, measurements of pressure in the cardiac chambers and great arteries, and calculation of pulmonary vascular resistance index. Doppler-derived peak, mid, and end-diastolic pulmonary regurgitation gradients correlated best with catheterization-measured pulmonary artery systolic, mean and diastolic pressures, respectively. The best Doppler correlate of pulmonary vascular resistance index was the pulmonary artery end-diastolic gradient. Clinically useful information can be obtained from Doppler pulmonary artery diastolic gradients measured on the pulmonary regurgitant diastolic velocity slope, which can estimate the pulmonary arterial pressure as well as pulmonary vascular resistance obtained on cardiac catheterization.
Asian cardiovascular & thoracic annals 07/2008; 16(3):221-5.
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ABSTRACT: Multiple congenital cardiac defects are usually addressed by cardiac surgery. We present our experience with simultaneous transcatheter treatment of multiple defects in children. Ten children, six females and four males, with multiple defects underwent treatment with interventional technique. The mean age was 4.4 +/- 2.6 years (range, 7 months to 8 years). The cardiac diagnosis was patent ductus arteriosus (PDA) and valvular pulmonary stenosis (PS) in two, atrial septal defect (ASD) and PDA in two, ASD and PS in two, PDA and aortic stenosis (AS) in three (severe left ventricular dysfunction in two), and perimembranous ventricular septal defect (VSD) and valvular PS in one. The ASDs were closed with an Amplatzer septal occluder (mean size, 16 +/- 4 mm), four PDAs were closed with an Amplatzer duct occluder, and three with a Cook's detectable coil. Mean balloon size used to dilate the pulmonary valve was 18 +/- 4 mm, and for the aortic valve this was 12 +/- 2 mm. There was a 70% (+/-15%) postprocedure reduction of gradients across the stenotic valves. The closure rate was 75% for PDAs in the catheterization laboratory, 80% for ASDs, and there was a mild residual intradevice leak in the VSD. In conclusion, interventional technology addressing multiple congenital cardiac defects as a combined procedure in the catheterization laboratory is safe and effective.
Pediatric Cardiology 06/2008; 29(5):890-3. · 1.30 Impact Factor
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ABSTRACT: To determine the clinical profile and assess the outcome of all neonates diagnosed with cyanotic congenital heart disease.
A case series.
The Aga Khan University Hospital from January 1998 to December 2000.
Neonates admitted with diagnosis of cyanotic congenital heart disease were evaluated for clinical diagnosis, survival and mortality.
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's 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.
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.
Journal of the College of Physicians and Surgeons--Pakistan: JCPSP 06/2008; 18(5):290-3. · 0.34 Impact Factor
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ABSTRACT: The modified Blalock-Taussig shunt (MBTS) is the most commonly created systemic-pulmonary shunt in neonates with cyanotic heart disease. Morbidity and mortality after MBTS is associated with several factors including age, pulmonary artery diameter and the baseline cardiac anatomy. The objective of this research was to describe the immediate and short-term follow-up results of MBTS in Pakistani neonates.
A retrospective review of patient charts was done to select 22 neonatal cases of various types of cyanotic heart diseases who had undergone MBTS creation from 1999 to 2005. Clinical and echocardiographic data were collected. Patients were followed up on their post-operative visits. Twenty-two neonates, 14 males and 8 females, mean age 11.2+/-6.9, underwent MBTS surgery during the six-year period of study. Pulmonary artery diameters were 3+/-0.2 and 2.9+/-0.2 for the right and left arteries, respectively. All patients received a 4mm Gor-Tex shunt through a postero-lateral thoracotomy approach. The mean duration of post-operative mechanical ventilation was 3.9+/-4.5 days. Three neonates (13.6%) died within one month of surgery while another three (13.6%) died after three months of surgery. Among these deaths, two were due to shunt occlusion/failure (9%) and the rest were due to non-cardiac causes. Another two patients underwent revision of surgery after shunt failure. Pulmonary atresia with intact interventricular septum was the most common cardiac anomaly in our series.
The mortality rate in neonates is highest during the first post-operative month. Shunt thrombosis and occlusion can be sudden and fatal therefore coagulation profile should be carefully monitored especially in the peri-operative period. PA-IVS was the most common anatomical variant in our limited experience and had high morbidity and mortality rate after surgery.
Heart Lung & Circulation 03/2008; 17(1):54-8. · 1.20 Impact Factor
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ABSTRACT: Cardiac surgery for correction or palliation of congenital cardiac disease in infancy and childhood remains a privilege that is rarely accessible to two-thirds of the world's population. This imbalance has created a unique spectrum of illness in patients with underlying congenital cardiac disease and complicating infective endocarditis in developing countries, including Pakistan. In this study, we characterize endocarditis as seen in such patients presenting in Karachi.
We reviewed retrospectively patients admitted to Aga Khan University with underlying congenitally malformed hearts and endocarditis between 1991 and 2004.
We identified 48 patients with endocarditis according to the modified Duke Criterions, with just over half the cases (54%) classified as definite endocarditis. Of the patients, 23 (49%) patients were more than 16 years old. Uncorrected left-to-right-shunts, tetralogy of Fallot, and congenital mitral valvar disease were the most common underlying defects. Patients with cyanotic defects, particularly of the complex type, were underrepresented (4%). Only 11 (22.9%) of the patients had a previous palliative or corrective surgery. In one-third of the patients (16), streptococcal species were identified as the microbiologic cause of endocarditis, and 22 (45.8%) had culture-negative endocarditis. In contrast, Staphylococcus aureus and enterococci caused endocarditis in only one patient each. There were no differences in mortality or complications between cyanotic and acyanotic congenital defects. Surgery was performed in nine (18.7%) patients with endocarditis, and of these, 13 (27.1%) died.
In contrast to the developed world, endocarditis in the developing countries, such as Pakistan, complicates uncorrected left-to-right shunts and tetralogy of Fallot, probably because patients with complex cyanotic defects fail to survive long after birth due to the lack of available surgery. Almost half of patients had culture-negative endocarditis, likely related to several factors.
Cardiology in the Young 01/2008; 17(6):623-30. · 0.76 Impact Factor
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ABSTRACT: Alternative strategy of non-surgical closure of patent ductus arteriosus (PDA) is presently the first line of therapy. Several devices are being used for transcatheter closure of PDA. A four-year-old girl underwent a second attempt at PDA closure with an amplatzer device. However, after the deployment of the device it got dislodged into the right pulmonary artery hilum. Several attempts by catheter retrieval failed. The girl underwent surgical removal of the device which was complicated by pulmonary artery injury and subsequent repair was performed on cardiopulmonary bypass.
Interactive cardiovascular and thoracic surgery 09/2007; 6(4):572-3.
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ABSTRACT: Transcatheter closure of patent ductus arteriosus (PDA) has been in place for more than three decades. We share our experience with the newer devices.
Ninety-eight patients, with a mean age of 64 +/- 11 months (range 7 months to 54 years), underwent attempted transcatheter closure of PDA. Thirty-seven patients were infants and 10 were adults. Two patients had residual PDA after surgical ligation. There were 66 females and 32 males.
Sixty-six patients were symptomatic and 32 were asymptomatic. Cardiac catheterization showed significant pulmonary hypertension in 18. The mean size of the PDA on aortogram was 3.1 +/- 1.4 mm (range 1.1 to 11 mm). Seven patients were referred for surgical ligation. The PDAs of 37 patients were closed using coil devices, 52 with Amplatzer duct occluders (ADO) and two with Amplatzer muscular VSD devices. One patient had a very large PDA which was embolized and retrieved surgically. One patient required occlusion with two coils. The occlusion rate in the catheterization laboratory with coils was 84 +/- 7%, and 83% with ADO. After 1 week, the occlusion rate for coils was 96%, and 99% with ADO. One patient with a large PDA requiring a 14/12 ADO was left with a mild gradient of 9 mmHg in the aorta at the end of the procedure. None of our patients had stenosis of the left pulmonary artery.
Transcatheter closure of PDA is the preferred alternative to surgical ligation, which should be reserved for small infants and premature babies or large unfavorably-shaped ducts.
The Journal of invasive cardiology 08/2007; 19(7):295-8. · 1.84 Impact Factor
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ABSTRACT: Pulmonary agenesis (PA) is a rare malformation that can be isolated or associated with other anomalies. We report 3 cases of left-sided PA having ipsilateral renal agenesis, facial, and radial ray anomalies. Patients presented in infancy with nonspecific respiratory symptoms and were diagnosed to have PA on chest radiograph and computed tomographic scan. Bronchial compression, by dilated pulmonary artery and associated severe gastroesophageal reflux, aggravated respiratory symptoms and required surgical intervention. The relevant literature is briefly reviewed.
Journal of Pediatric Surgery 07/2006; 41(6):1165-9. · 1.45 Impact Factor
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ABSTRACT: Brain abscess is a serious life-threatening complication of several diseases. The objective of this study was to look at the clinical profile of patients, predisposing conditions, microbiology and outcome of children suffering from brain abscess.
Thirty children aged less than 15 years were reviewed. There were 15 males and 15 females. The mean age of presentation was 5.6+/-4.4 years.
The duration of illness at the time of admission was 17.6+/-24.6 days. Typically patients presented with fever, vomiting, headache and seizures. The predisposing conditions found were cyanotic congenital heart disease in 11 (37%) of children, meningitis in 6 (20%), septicemia in 7 (23%) and no underlying cause was found in 5 (17%) children. The most common microbe in children with cyanotic congenital heart disease was of the Streptococcus milleri group (52%). Computerized tomography confirmed the diagnosis and the most common location of the abscess was the parietal lobe of the cerebral hemisphere. All abscesses were large, more than 2 cm in diameter and were aspirated surgically. Excision was performed in 6 children. Five children expired, one due to a intracranial bleeding and the others due to severe cerebral edema and tentorial herniation. Complications were seen in 20 children and 16 had sequelae, hemiparesis in 11 and seizure disorder in 5.
Brain abscess is a serious infection with poor outcome if diagnosed late. Delayed surgical drainage has high morbidity and mortality. The threshold for diagnosis should be low, particularly in children with a predisposing condition like cyanotic congenital heart disease.
The Indian Journal of Pediatrics 06/2006; 73(5):401-4. · 0.52 Impact Factor
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ABSTRACT: The efficacy and safety of device closure of atrial defects in children with complex congenital heart disease and a hypoplastic right ventricle have not been detailed. The objective of this study was to determine the clinical impact and outcomes of a staged surgical catheter-based management strategy. A retrospective analysis of 17 children with a hypoplastic right ventricle who had undergone cardiac catheterization and attempted device occlusion of an atrial defect was undertaken. Clinical data, anatomical diagnoses, previous surgeries, and interventions were noted. The clinical course and echocardiographic findings were compared before and after defect closure (6 +/- 4 months of follow-up). Nine children had pulmonary atresia with an intact ventricular septum, six had Ebstein's anomaly of the tricuspid valve, and two had isolated right ventricular hypoplasia. The mean age at defect closure was 6.6 +/- 3.4 years. Under general anesthesia, closure of the atrial defect was undertaken after initial temporary test occlusion. The Qp:Qs ratio before closure was 1.1 +/- 0.4. Oxygen saturation improved in all children from 91% +/- 1% to 98% +/- 1% (P < 0.0005). Mean right atrial pressure increased from 9 +/- 3 to 11 +/- 4 mm Hg after closure, but the difference was not statistically significant (P = 0.2). Follow-up revealed an increased right ventricular length (P = 0.009) and Z-score (P = 0.02), although no significant increase in the diameter of the tricuspid valve annulus was observed in children with pulmonary atresia. Right ventricular systolic pressures or systolic function did not change significantly during follow-up (P = 0.5 and 0.29, respectively). Exercise tolerance improved in all children and clinical evidence of right ventricular decompensation was absent. Catheter closure of atrial defects in children with a hypoplastic right ventricle is well tolerated, safe, and effective as a treatment strategy in the staged management.
Catheterization and Cardiovascular Interventions 02/2005; 64(1):112-6. · 2.29 Impact Factor
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ABSTRACT: Hypertrophic cardiomyopathy is an uncommon childhood cardiac disease and can be primary or secondary. Several systemic diseases are known to be associated with this entity. Senger's disease is a mitochondrial disorder causing congenital cataracts lactic acidosis and skeletal and cardiac myopathy. Diagnosis should be kept in mind when routine neonatal eye screening reveals absent red reflex. The authors report a case of Sengers disease and discuss the underlying pathogenetic mechanisms.
The Indian Journal of Pediatrics 06/2004; 71(5):437-40. · 0.52 Impact Factor
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ABSTRACT: 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 airways.
Journal of Ayub Medical College, Abbottabad: JAMC 16(1):66-8.
