Annals of Pediatric Cardiology
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   2012| January-June  | Volume 5 | Issue 1  
    Online since March 12, 2012

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Congenital mitral valve lesions : Correlation between morphology and imaging
Bo Remenyi, Tom L Gentles
January-June 2012, 5(1):3-12
Congenital malformations of the mitral valve are often complex and affect multiple segments of the valve apparatus. They may occur in isolation or in association with other congenital heart defects. The majority of mitral valve malformations are not simply classified, and descriptive terms with historical significance (parachute, mitral, or arcade) often lack the specificity that cardiac surgeons demand as part of preoperative echocardiographic morphological assessment. This paper examines the strengths and limitations of commonly used descriptions and classification systems of congenitally malformed mitral valves. It correlates pathological, surgical, and echocardiographic findings. Finally, it makes recommendations for the systematic evaluation of the congenitally malformed mitral valve using segmental echocardiographic analysis to assist precise communication and optimal surgical management.
  13,142 1,026 8
Pulmonary artery banding: Rationale and possible indications in the current era
Rajesh Sharma
January-June 2012, 5(1):40-43
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Mitral valve operations at a high-volume pediatric heart center: Evolving techniques and improved survival with mitral valve repair versus replacement
Christopher W Baird, Patrick O Myers, Gerald Marx, Pedro J del Nido
January-June 2012, 5(1):13-20
Mitral valve disease is quite variable and can occur as an isolated defect or in association with other complex left sided lesions. These lesions are often best described with detailed pre-operative imaging studies to define the valve anatomy and to access associated left heart disease. Depending on the type of mitral valve disease, various surgical repair techniques have led to improved survival in the recent era. We describe lesion specific approach to mitral valve repair and results.
  8,546 505 6
Approach to postoperative fever in pediatric cardiac patients
Ajay K Gupta, Vishal K Singh, Amit Varma
January-June 2012, 5(1):61-68
Fever in the postoperative period in children undergoing surgery for congenital heart disease is fairly common and tends to cause anxiety to both the surgeon and the patient. Such fever is associated with the metabolic response to trauma, systemic response to the cardiopulmonary bypass, hypothermia, presence of drainage tubes, drugs, blood transfusion as well as infections. Establishing the diagnosis requires proper assessment of the patient with focused history, targeted physical examination and judicious use of investigations with the knowledge of the common causes.
  8,154 609 1
Cor triatriatum dexter: A rare cause of childhood cyanosis
Ahmad Rustam bin Mohd Zainudin, Koh Ghee Tiong, Sharifah A I Mokhtar
January-June 2012, 5(1):92-94
Cor triatriatum dexter is a rare congenital heart anomaly where the right atrium is divided into two chambers by a membrane. We report a boy who had persistent mild cyanosis and diagnosed to have cor triatriatum dexter with secundum atrial septal defect by transoesophageal echocardiography. Interestingly, he had persistent mild cyanosis despite insignificant obstruction to the right ventricular inflow and normal pulmonary artery pressure. The pathophysiology, approach to the diagnosis, and mode of treatment are also discussed.
  7,699 311 8
Anomalous origin of right coronary artery from pulmonary artery
Rajat Gupta, Ashutosh Marwah, Savitri Shrivastva
January-June 2012, 5(1):95-96
Anomalous origin of coronary artery from the pulmonary artery is a rare anomaly that most frequently involves the left coronary artery and very rarely the right coronary artery. These lesions can be missed on echocardiography unless carefully looked for. We describe a case of isolated anomalous origin of right coronary artery from pulmonary artery diagnosed on echocardiography and confirmed by computed tomography (CT) angiography.
  7,026 312 1
Percutaneous pulmonary valve implantation
Sachin Khambadkone
January-June 2012, 5(1):53-60
  5,163 308 8
The Contegra conduit: Late outcomes in right ventricular outflow tract reconstruction
Anthony A Holmes, Steve Co, Derek G Human, Jacques G LeBlanc, Andrew IM Campbell
January-June 2012, 5(1):27-33
Objectives: To report the clinical outcomes (early death, late death, and rate of reintervention) and performance of the Contegra conduit as a right ventricle outflow tract implant and to determine the risk factors for early reintervention. Methods: Forty-nine Contegra conduits were implanted between January 2002 and June 2009. Data collection was retrospective. The mean age and follow-up duration of Contegra recipients was 3.5 ± 4.6 years and 4.2 ± 2.0 years, respectively. Results: There were three deaths (two early, one late), giving a survival rate of 93.9%. The rate of conduit-related reintervention was 19.6% and was most often due to distal conduit stenosis. Age at implantation of <3 months, receipt of a conduit of 12-16 mm diameter, and a diagnosis of truncus arteriosus were each significant contributors to the rate of reintervention. Conclusion: The Contegra is a cost-effective and readily available solution. However, there is a limited range of larger calibers, which means that the homograft conduit (>22 mm) remains the first choice of implant in older children. The rates of reintervention are significantly higher with a diagnosis of truncus arteriosus, age at implantation of <3 months, and implantation of conduits sized 12-16 mm.
  4,918 339 10
Impact of congenital heart disease on brain development in newborn infants
Moustafa M Abdel Raheem, Walid A Mohamed
January-June 2012, 5(1):21-26
Objective: To assess brain development and brain injury in neonates with cyanotic and acyanotic congenital heart disease (CHD). Methods: The study included 52 term infants with CHD who were divided into two groups: Cyanotic (n=21) and acyanotic (n=31). Fifteen healthy neonates of matched age and sex were enrolled in the study as controls. Three-dimensional proton magnetic resonance spectroscopic imaging and diffusion tensor imaging were used to assess brain development and injury. We calculated the ratio of N-acetylaspartate (NAA) to choline (which increases with maturation), average diffusivity (which decreases with maturation), fractional anisotropy of white matter (which increases with maturation), and the ratio of lactate to choline (which increases with brain injury). Results: As compared with control neonates, those with CHD had significant decrease in NAA/choline ratio (P<0.001), significant increase in lactate/choline ratio (P<0.0001), significant increase in average diffusivity (P<0.0001), and significant decrease of white matter fractional anisotropy (P<0.001). Neonates with cyanotic CHD had significant less brain development and more brain injury than those with acyanotic CHD (P<0.05). Conclusions: Newborn infants with cyanotic and acyanotic CHD are at high risk of brain injury and impaired brain maturity.
  4,610 529 3
Occult systemic lupus erythematosus with active lupus nephritis presenting as Libman-Sacks endocarditis
Pankajkumar Ashok Kasar, Milly Mathew, Georgi Abraham, Raghavannair Suresh Kumar
January-June 2012, 5(1):85-88
The diagnosis of systemic lupus erythematosus (SLE) depends on clinical evidence of renal, rheumatologic, cutaneous, and neurologic involvement, supported by serological markers. A previously healthy 14-year-old girl presented with Libman-Sacks endocarditis involving the aortic valve as the first manifestation of SLE. Even though she did not satisfy the American College of Rheumatology criteria for diagnosing SLE, she had anemia, proteinuria, elevated erythrocyte sedimentation rate, low complement 4 (C4) levels, and strongly positive antinuclear antibody titer. A renal biopsy showed stage IV lupus nephritis. Treatment was initiated with immunosuppressants and steroids. This type of presentation may be misdiagnosed as infective endocarditis missing the underlying collagen vascular disease.
  4,957 169 1
Pulmonary artery banding in the current era: Is it still useful?
Maziar Gholampour Dehaki, Ali Sadeghpour Tabaee, Changiz Azadi Ahmadabadi, Alireza Alizadeh Ghavidel, Gholamreza Omra
January-June 2012, 5(1):36-39
Background: The objective of this study was to assess the results of the pulmonary artery (PA) banding in patients with congenital heart defects (CHD) and pulmonary hypertension (PH) in the current era. Methods: We analyzed data from 305 patients who underwent PA banding between April 2005 and April 2010 at our centre. All patients were approached through a left thoracotomy. Twenty percent of patients underwent PA banding based on Trusler's rule (Group 1), 55% of them underwent PA banding based on PA pressure measurement (Group 2), and the rest of them (25%) based on surgeon experience (Group 3). The follow-up period was 39 ± 20 month and 75% of patients (230 cases) had definitive repair at mean interval 23 ± 10 months. Results: The rate of anatomically and functionally effectiveness of PA banding in all groups was high (97% and 92%, respectively). There were no significant differences in anatomically and functionally efficacy rate between all groups (P=0.77, P=0.728, respectively). There was PA bifurcation stenosis in six cases (2%), and pulmonary valve injury in one case (0.3%). The mortality rate in PA banding was 2% and in definitive repair was 3%. Conclusions:We believe that PA banding still plays a role in management of patients with CHD, particularly for infants with medical problems such as sepsis, low body weight, intracranial hemorrhage and associated non cardiac anomalies. PA banding can be done safely with low morbidity and mortality.
  4,237 538 3
An unusual case of left main coronary artery aneurysm with right ventricle fistula
Shweta Nathani, C Reddy, PV Suresh, Sunita Maheshwari
January-June 2012, 5(1):69-71
A 4-year-old boy presented with repeated respiratory tract infections. Echocardiography showed dilation of the left main coronary artery with flow into the Right Ventricular Outflow Tract (RVOT). Diagnosis of Left Coronary Artery Aneurysm (LMCA) with RVOT fistula was made. A surgical repair of LMCA aneurysm by two-patch technique was performed. The patient had an excellent outcome postoperatively and is asymptomatic on follow-up.
  4,165 170 1
Neonatal pulmonary artery thrombosis
Mangesh Jadhav, Ashish Sapre, Swati Garekar, Snehal Kulkarni
January-June 2012, 5(1):44-46
Pulmonary artery thrombosis in neonates is a rare entity. We describe two neonates with this diagnosis; their presentation, evaluation, and management. These cases highlight the importance of this differential diagnosis when evaluating the cyanotic neonate.
  4,012 189 -
Silent embolization of an Amplatzer septal occluder into the left ventricular outflow tract requiring emergent surgical retrieval
Abdelkarim Errahmouni, Mustapha El Hattaoui, Abdennasser Drighil, Drissi Boumzebra
January-June 2012, 5(1):89-91
Percutaneous closure of secundum atrial septal defect is an established safe alternative to surgery with rare complications and high primary success rate. This procedure can be complicated by early or late device embolizations. We report an asymptomatic delayed nonobstructive embolization of an amplatzer septal occluder (ASO) into the left ventricle outflow tract detected by routine transthoracic echocardiography 1 week after implantation, which required emergent surgical retrieval in a stable patient.
  3,693 177 2
"Vanishing" pulmonary valve stenosis
Nofil I Arain, James H Moller, Lee A Pyles, Shanthi Sivanandam
January-June 2012, 5(1):47-50
Objective: Both spontaneous resolution and progression of mild pulmonary valve stenosis (PS) have been reported. We reviewed characteristics of the pulmonary valve (PV) to determine factors that could influence resolution of mild PS. Methods: Fifteen asymptomatic pediatric patients with spontaneous resolution of isolated mild PS were retrospectively reviewed. Results: There was no correlation between the PV gradient, clinical presentation, age at diagnosis, or PV morphology. The PV annulus was small at initial presentation, which normalized at follow up. When corrected for the body surface area (z-score), the PV annulus was normal in all patients, including at initial evaluation. Conclusions: Based on our observation, neither age at diagnosis, nor PV-morphology-influenced resolution of mild PS. The variable clinical presentation makes it difficult to categorize and observe mild PS by auscultation alone. The PV annulus z-score could be a useful adjunct to determine the course and serial observation of mild PS.
  3,610 185 1
Congenital giant cardiac tumor with severe left-ventricular inflow and outflow obstruction and arrhythmia treated with pulmonary artery banding and long-term amiodarone infusion
Daiji Takeuchi, Takeshi Hiramatsu, Toshio Nakanishi
January-June 2012, 5(1):78-80
We report a congenital giant cardiac tumor that occupied the majority of left ventricular cavity with severe left ventricular inflow and outflow obstruction. The hemodynamics were similar to univentricular physiology. He was treated with prostaglandins and bilateral pulmonary artery banding. He had frequent supraventricular tachycardia associated with ventricular pre-excitation that was controlled by long-term administration of intravenous amiodarone. The patient died due to sepsis after 3 months.
  3,387 141 -
The Contegra bovine jugular valved conduit: Living up to expectations?
Krishna S Iyer
January-June 2012, 5(1):34-35
  3,201 207 1
Endovascular stenting of the obstructed vertical vein in a neonate with supracardiac total anomalous pulmonary venous return
Nageswara R Koneti, Hemasree Kandraju, Vasudevan Kanchi, Sreenivas Kumar Arramraju
January-June 2012, 5(1):75-77
A newborn baby presented with respiratory distress, cyanosis and shock within 2 hours of birth. The cardiac evaluation showed supracardiac total anomalous pulmonary venous return with critically obstructed vertical vein. The baby underwent successful stenting of the vertical vein at 12 hours of life.
  3,147 182 -
Atrial septal aneurysm and stroke
Mohd Razaq , Ravi Kumar Parihar, Ghanshyam Saini
January-June 2012, 5(1):98-99
  3,063 140 1
Iatrogenic diversion of IVC to left atrium after surgical closure of ASD
Shreepal A Jain, Robin Pinto, Bharat Dalvi
January-June 2012, 5(1):72-74
Iatrogenic diversion of the inferior vena cava (IVC) to the left atrium during surgical closure of an atrial septal defect (ASD) is a very rare complication in the era of cardiopulmonary bypass. The eustachian valve could be mistaken for the margin of the ASD. We report a case with this complication after closure of a sinus venosus ASD of the IVC type.
  2,909 164 3
Protein losing enteropathy secondary to a pulmonary artery stent
Narayanswami Sreeram, Uwe Trieschmann, Gerardus Bennink
January-June 2012, 5(1):51-52
A 2-year-old patient with hypoplastic left heart syndrome presented 6 months following Fontan completion with protein-losing enteropathy (PLE). He had undergone stent implantation in the left pulmonary artery after the Norwood procedure, followed by redilation of the stent prior to Fontan completion. Combined bronchoscopic and catheterization studies during spontaneous breathing confirmed left bronchial stenosis behind the stent, and diastolic systemic ventricular pressure during expiration of 25 mm Hg. We postulate that the stent acts as a valve, against which the patient generates high expiratory pressures, which are reflected in the ventricular diastolic pressure. This may be the cause of PLE.
  2,922 137 3
Clinical errors
Shyam S Kothari
January-June 2012, 5(1):1-2
  2,567 287 -
Right atrial hemangioma in the newborn: Utility of fetal imaging
Christopher Baird, Shannon Blalock, Resai Bengur, Catherine Ikemba
January-June 2012, 5(1):81-84
We present a rare primary right atrial tumor diagnosed in-utero with fetal echocardiography, and further characterized as a congenital hemangioma with magnetic resonance imaging. Surgical resection was done six days after birth. This case illustrates the complementary roles of evolving advanced imaging techniques for fetuses and infants with congenital heart disease that allows for surgery early in the neonatal period.
  2,676 144 2
Resynchronization therapy in an adolescent with pacemaker-related ventricular dysfunction
Ayhan Cevik, Deniz Oguz, Ayhan Pektas, Murat Ozdemir, Rana Olgunturk
January-June 2012, 5(1):97-98
  2,608 98 -
Selected abstracts presented at the Annual Conference of the Pediatric Cardiac Society of India, 2011

January-June 2012, 5(1):103-109
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Selected Summaries
Sachin Talwar, Anil Singhi
January-June 2012, 5(1):100-102
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