[Show abstract][Hide abstract] ABSTRACT: Abstract
Anthracyclines (i.e., doxorubicin, daunorubicin) have significant impact on outcome in many pediatric
chemotherapy protocols and therefore remain the mainstay of treatment. The objective of
this study was to identify the risk factors for anthracycline induced cardiac dysfunction in pediatric
patients. Multiple logistic regression model was applied to assess the risk factors for development
of cardiac dysfunction. 110 pediatric oncology patients were available for final analysis. 75
(66%) children were males and mean age was 74 ± 44 months. ALL (n = 70, 64%) was the most
common primary diagnosis followed by lymphoma (n = 19; 17%) and AML (n = 12, 11%). Daunorubicin
alone or in combination with doxorubicin was used in (n = 94, 85%) patients and cumulative
dose < 300 mg was used in (n = 95; 86%) children. 24 (22%) children received radiation
therapy as per protocol and sepsis were observed in 47 (43%) cases. Post anthracycline, 15 (14%)
children had cardiac dysfunction within a month; out of them 10/15 (67%) had isolated diastolic
dysfunction, while 28 (25%) developed dysfunction within a year. 19 (17%) had pericardial effusion.
11 expired and out of them, 7 had significant cardiac dysfunction. Cumulative dose > 300
mg/m2 (p < 0.001; AOR 2.3), radiation therapy (p = 0.009; AOR 3.5) and sepsis (p = 0.002; AOR 2.6)
were found to be independent risk factors associated anthracycline induced cardiac dysfunction.
At univariant level use of daunorubicin alone or in combination therapy (p < 0.001, OR 7) and
mode of delivery (p 0.048, OR 9.7) were also found statistically significant. In conclusion anthracycline
induced cardiac dysfunction is mostly related to cumulative dose > 300 mg/m2, use of Daunorubicin
alone or in combination with doxorubicin, mode of delivery, radiation therapy and sepsis.
Regular long term follow-up with cardiologist is the key point for early diagnosis and therapy for a
long term survival.
World Journal of Cardiovascular Diseases 06/2014; 4:377-83.
[Show abstract][Hide abstract] ABSTRACT: To evaluate the midterm results of Contegra conduit.
The retrospective study comprised patient record at Aga Khan University Hospital, Karachi, of conduits implanted between May 2007 and June 2012. Data collection was made from the clinical notes and from serial echocardiograms by a single cardiologist. The last followup echocardiography was done at the time of data collection in June 2012. SPSS 19 was used for statistical analysis.
A total of 18 conduits had been implanted (16-22 mm) during the study period. Median age at the time of surgery was 9 years (range: 2.5-16 years). Early mortality was seen in 3 (16.66%) patients, but none was Contegra related. Of the remaining 15 patients, 2 (13.33%) with a diagnosis of Pulmonary Atresia-Ventricular Septal Defect with hypoplastic peripheral Pulmonary Arteries (PA), developed severe distal pressure gradient (50 mmHg) across Contegra over a median period of 18 months (range: 12-24 months), with resultant severe regurgitation and needed percutaneous intervention. There was no thrombosis, calcification, anuerysmal dilation or late deaths.
At midterm followup, Contegra conduit was associated with low re-intervention rates with satisfactory haemodynamic results. However, long-term durability must be determined for this conduit, especially in patients with Pulmonary Atresia-Ventricular Septal Defect with hypoplastic peripheral Pulmonary Arteries.
Journal of the Pakistan Medical Association 10/2013; 63(10):1266-70. · 0.41 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Total anomalous pulmonary venous drainage (TAPVD) accounts for approximately 1.5% of all congenital heart diseases. It is usually diagnosed in the neonatal period and is rarely seen in adults. We report an unusual case of a patient with TAPVD who was successfully treated at the age of 28 years. We believe that this is the oldest person in the South Asian literature to undergo surgical correction of TAPVD.
Annals of Medicine and Surgery. 01/2013; 2(2):60–62.
[Show abstract][Hide abstract] ABSTRACT: Aorto-pulmonary window is a relatively rare congenital cardiac malformation with an overall incidence of 0.1%. Pulmonary hypertension develops quickly if the lesion is left untreated hence early surgical intervention is warranted after diagnosis. The surgery for correction of APW has evolved over years, currently an open repair on cardiopulmonary bypass (CPB) with a single patch technique yields good results. Mortality is affected by association of pulmonary hypertension and other cardiac malformations. We present a case of an infant with a large type II APW with a relatively low pulmonary vascular resistance. Hospital stay was complicated because of pulmonary arterial disease making it an important reason for correction in the first few months of life.
Journal of the Pakistan Medical Association 08/2011; 61(8):836-8. · 0.41 Impact Factor
[Show abstract][Hide abstract] 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.
[Show abstract][Hide abstract] 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.
[Show abstract][Hide abstract] ABSTRACT: Transcatheter closure of atrial septal defect (ASD) and patent ductus arteriosus (PDA) with Amplatzer septal/duct occluder (ASO/ADO) is an established, safe, and efficient procedure with high success. However, device embolization remains a major complication requiring immediate intervention (either percutaneous or surgical) for retrieval and correction of the heart defect. The aim of this study is to share the experience of managing embolized ASO/ADO.
Of the 284 cases of device closure performed from October 2002 to December 2010, four patients (1.4%) had device embolization requiring immediate surgical retrieval. Two adult female patients with secundum ASD had ASO device implanted. One embolized to the right ventricle and the other into the ascending aorta. An eight-month-old boy and a four-year-old girl with hypertensive PDA had device closure. Device embolization occurred into the descending aorta and right pulmonary artery, respectively.
All four devices were retrieved and the defects closed successfully with a low morbidity and no mortality.
Careful consideration should be given to surgical or transcatheter closure of a heart defect. Life-threatening complications although rare can occur. Our experience strongly suggests that these devices should only be inserted in facilities where cardiac surgical support is immediately available.
Journal of Cardiac Surgery 05/2011; 26(3):254-8. · 1.35 Impact Factor
[Show abstract][Hide abstract] 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. · 0.41 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: A patient with a patent ductus arteriosus (PDA) was catheterized for device closure. Anomalous systemic venous drainage was found with interrupted inferior vena cava and persistence of hepatic vessel plexus. Using the superior vena cava route, the PDA was closed successfully.
[Show abstract][Hide abstract] ABSTRACT: Background: The spectrum of infective endocarditis (IE) is significantly different in developed and developing countries. The present study was conducted to study the clinical profile and outcome of infective endocarditis in Pakistan. Methods: A descriptive cross-sectional study with review of medical records for 188 patients admitted to our teaching hospital with a diagnosis of IE from January 1988 to December 2001. One hundred fifty-nine subjects fulfilled the modified Duke diagnostic criteria. Results: Definite IE was found in 59.7% (95/159) patients, while the rest had possible IE. One-third of subjects had acute IE 55/159 (34.5%). Subacute IE was found in 62% (99/159) and Nosocomial IE in 3% of the cases. Eighty six (54%) were classified as having culture- negative endocarditis and 73 (46%) as culture positive. Ninty four patients (59%) had an underlying predisposing factor including congenital heart disease (31%) and rheumatic heart disease (21%). The most frequently isolated organisms were streptococci (52%) and followed by staphylococci (29%). Fourteen (8.1%) patients had right-sided cardiac involvement. Using univariate analysis, patients with heart failure, neurologic or renal complications, septicemia, nosocomial endocarditis, and prosthetic valve endocarditis were at increased risk of death (p ≤ 0.05), however no individual microorganism, or specific site, size, or morphology of vegetation seen on echocardiogram were significantly associated with death. Thirty-seven (23%) patients died of endocarditis or its complications. Conclusion: Endocarditis continues to be an important contributor to morbidity and mortality in Pakistan, especially in young adults. Our patients differ from the west in terms of epidemiology, predisposing factors, microbiology, complications, and outcome.
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.
[Show abstract][Hide abstract] 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. · 0.41 Impact Factor
[Show abstract][Hide abstract] 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
[Show abstract][Hide abstract] 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.
[Show abstract][Hide abstract] 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.
[Show abstract][Hide abstract] 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.
[Show abstract][Hide abstract] 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. · 0.95 Impact Factor
[Show abstract][Hide abstract] 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. · 1.11 Impact Factor
[Show abstract][Hide abstract] 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. · 1.57 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: The aim of this study was to evaluate the spectrum of cardiac involvement and its outcome in beta-thalassaemia major.
There were 75 patients with a mean age of 13.8 (+/- 5.5) years, of whom 33 were male and 42 were female. Clinical history, examination and laboratory investigations were assessed. Electrocardiograms, chest radiographs and echocardiograms were reviewed.
44 patients had cardiac involvement in the form of left ventricular systolic dysfunction in 17, diastolic dysfunction in 22, pericardial effusion in 12 and pulmonary hypertension in 12 patients. With intense chelation therapy and cardiac medications, the condition of 13 of 17 patients with systolic dysfunction, and four of 22 with diastolic dysfunction, improved.
Cardiac disease is a common complication of siderotic disease in thalassaemia major and it can be prevented with regular chelation. This study has shown improved systolic function after regular chelation therapy.
Singapore medical journal 09/2006; 47(8):693-6. · 0.63 Impact Factor
[Show abstract][Hide abstract] 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. · 1.38 Impact Factor