Annals of Pediatric Cardiology
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   2014| May-August  | Volume 7 | Issue 2  
    Online since May 14, 2014

 
 
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REVIEW ARTICLE
Familial hypercholesterolemia: A review
Mithun J Varghese
May-August 2014, 7(2):107-117
DOI:10.4103/0974-2069.132478  PMID:24987256
Familial hypercholesterolemia (FH) is a genetic disorder of lipoprotein metabolism resulting in elevated serum low-density lipoprotein (LDL) cholesterol levels leading to increased risk for premature cardiovascular diseases (CVDs). The diagnosis of this condition is based on clinical features, family history, and elevated LDL-cholesterol levels aided more recently by genetic testing. As the atherosclerotic burden is dependent on the degree and duration of exposure to raised LDL-cholesterol levels, early diagnosis and initiation of treatment is paramount. Statins are presently the mainstay in the management of these patients, although newer drugs, LDL apheresis, and other investigational therapies may play a role in certain subsets of FH, which are challenging to treat. Together these novel treatments have notably improved the prognosis of FH, especially that of the heterozygous patients. Despite these achievements, a majority of children fail to attain targeted lipid goals owing to persistent shortcomings in diagnosis, monitoring, and treatment. This review aims to highlight the screening, diagnosis, goals of therapy, and management options in patients with FH.
  5 9,953 686
CASE REPORTS
Giant coronary-pulmonary fistula with pulmonary atresia, ventricular septal defect, and coronary anomaly: A case report and review of literature
PS Seetharama Bhat, SL Girish Gowda, Jayaranganath Mahimarangaiah, Cholenahally Nanjappa Manjunath
May-August 2014, 7(2):142-144
DOI:10.4103/0974-2069.132496  PMID:24987263
Congenital coronary-pulmonary artery fistula is a rare condition and is usually associated with pulmonary atresia. We present a 10-year-old girl with circumflex coronary artery to pulmonary artery (PA) fistula with a giant dilated circumflex coronary artery, ventricular septal defect (VSD), pulmonary atresia, and anomalous originofleft anterior descending (LAD) artery from right coronary sinus. The patient underwent surgical correction. We report the investigation, treatment, and review of literatureof this rare congenital anomaly associated with dilated circumflex coronary artery.
  2 3,125 158
ORIGINAL ARTICLES
Prevalence of congenital anomalies in newborns with congenital heart disease diagnosis
Alexander Egbe, Simon Lee, Deborah Ho, Santosh Uppu, Shubhika Srivastava
May-August 2014, 7(2):86-91
DOI:10.4103/0974-2069.132474  PMID:24987252
Background: There is a known association between congenital heart disease (CHD) and other congenital anomalies (CA). These associations have been altered by changes in prenatal factors in recent time. We reviewed the largest database of inpatient hospitalization information and analyzed the current association between common CHD diagnoses and other congenital anomalies. Materials and Methods: Case-control study design. We reviewed the Nationwide Inpatient Sample (NIS) database from 1998 to 2008 and identified all live births with CHD diagnosis (case) and live births without CHD diagnosis (control). We compared prevalence of associated congenital anomalies between the case and control groups. Results: Our cohort consisted of 97,154 and 12,078,482 subjects in the case and control groups, respectively. In the CHD population, prevalence of non-syndromic congenital anomaly (NSCA), genetic syndrome (GS), and overall extra-cardiac congenital anomaly (CA) were 11.4, 2.2, and 13.6%, respectively. In the control group, prevalence of NSCA, GS, and CA were 6.7, 0.3, and 7.0%, respectively. NSCA (odds ratio (OR): 1.88, confidence interval (CI): 1.73-1.94), GS (OR 2.52, CI 2.44-2.61), and overall CA (OR: 2.01, CI: 1.97-2.14) were strongly associated with CHD. Prevalence of GS and multiple organ-system CA decreased significantly over the study period. Conclusions: This is the largest and most comprehensive population-based study evaluating association between CHD and extra-cardiac malformation (ECM) in newborns. There was significant decrease in prevalence of GS and multiple CA over the study period.
  2 5,485 565
BRIEF COMMUNICATIONS
Rescrewing the embolized duct occluder using the delivery cable
Nageswara Rao Koneti, Shweta Bakhru, Raghava Raju Penumatsa, Krishna Mohan Lalukota
May-August 2014, 7(2):103-106
DOI:10.4103/0974-2069.132477  PMID:24987255
We report the successful re-screwing of the embolized duct occluder (DO) in three children for retrieval and to attempt redeployment. The DO was embolized into descending aorta immediately after the deployment in one child and within 24 h after the procedure in two further patients. The DO was re-screwed back by the DO delivery cable, using "sheath in sheath" in all three cases; however, successful retrieval could be done only in two. Repositioning in the patent ductus arteriosus (PDA) was done using the same device in those two children and surgical removal was needed in third child with perimembranous ventricular septal defect.
  1 3,137 261
CASE REPORTS
Amplatzer vascular plug IV for occlusion of pulmonary arteriovenous malformations in a patient with cryptogenic stroke
Surendranath R Veeram Reddy, Vishal G Patel, Sarah K Gualano
May-August 2014, 7(2):145-147
DOI:10.4103/0974-2069.132498  PMID:24987264
Paradoxical embolism resulting in cryptogenic stroke has received much attention recently, with the primary focus on patent foramen ovale (PFO). However, it is essential to be vigilant in the search for other causes of paradoxical embolic events, such as pulmonary arteriovenous malformations (PAVM). We describe successful closure of pulmonary AVM with a St Jude Medical (Plymouth, MN) Amplatzer TM vascular plug IV. The newer AVP-IV devices can be used for successful embolization of tortuous pulmonary AVM in remote locations where use of other traditional devices may be technically challenging.
  1 3,194 144
Transcatheter closure of hypertensive ductus with amplatzer post infarction muscular VSD occluder after percutaneous retrieval of embolized amplatzer duct occluder
Milind S Phadke, Satish Karur, Prafulla G Kerkar
May-August 2014, 7(2):126-129
DOI:10.4103/0974-2069.132481  PMID:24987259
Transcatheter closure of a large hypertensive patent ductus arteriosus is challenging with scant data about it. Even more challenging is retrieval of an embolized Amplatzer duct occluder. We report successful closure of a 12 mm large ductus with the Amplatzer muscular VSD occluder (post myocardial infarction) after percutaneous retrieval of the embolized, largest available, 16/14 mm Amplatzer duct occluder.
  1 2,786 153
IMAGES
The role of cardiac MRI in the diagnosis and management of sinus venosus atrial septal defect
Madhusudan Ganigara, David Tanous, David Celermajer, Rajesh Puranik
May-August 2014, 7(2):160-162
DOI:10.4103/0974-2069.132509  PMID:24987269
Sinus venosus atrial septal defects (SV-ASDs) are inter-atrial communications caused by a deficiency of the common wall between the superior or inferior vena cava and the right-sided pulmonary veins. The diagnosis can be challenging, especially in adults with delayed presentation. We present images that illustrate an example of the role of cardiac magnetic resonance imaging (CMRI) in the diagnosis and follow-up of a patient with SV-ASD.
  1 3,846 228
ORIGINAL ARTICLES
Non-invasive estimation of pulmonary vascular resistance in patients of pulmonary hypertension in congenital heart disease with unobstructed pulmonary flow
Arindam Pande, Achyut Sarkar, Imran Ahmed, GS Naveen Chandra, Shailesh Kumar Patil, Chanchal Kumar Kundu, Rahul Arora, Ajanta Samanta
May-August 2014, 7(2):92-97
DOI:10.4103/0974-2069.132475  PMID:24987253
Context: Pulmonary vascular resistance (PVR) is a critical and essential parameter during the assessment and selection of modality of treatment in patients with congenital heart disease accompanied by pulmonary arterial hypertension. Aim: The present study was planned to evaluate non-invasive echocardiographic parameters to assess pulmonary vascular resistance. Settings and Design: This prospective observational study included 44 patients admitted in the cardiology and pediatric cardiology ward of our institution for diagnostic or pre-operative catheter based evaluation of pulmonary arterial pressure and PVR. Materials and Methods: Detailed echocardiographic evaluation was carried out including tricuspid regurgitation velocity (TRV) and velocity time integral of the right-ventricular outflow tract (VTI RVOT ). These parameters were correlated with catheter-based measurements of PVR. Results: The TRV/VTI RVOT ratio correlated well with PVR measured at catheterization (PVRcath) (r = 0.896, 95% confidence interval [CI] 0.816 to 0.9423, P < 0.001). Using the Bland-Altman analysis, PVR measurements derived from Doppler data showed satisfactory limits of agreement with catheterization estimated PVR. For a PVR of 6 Wood units (WU), a TRV/VTI RVOT value of 0.14 provided a sensitivity of 96.67% and a specificity of 92.86% (area under the curve 0.963, 95% confidence interval 0.858 to 0.997) and for PVR of 8 WU a TRV/VTI RVOT value of 0.17 provided a sensitivity of 79.17% and a specificity of 95% (area under the curve 0. 0.923, 95% confidence interval 0.801 to 0.982). Conclusions: Doppler-derived ratio of TRV/VTI RVOT is a simple, non-invasive index, which can be used to estimate PVR.
  1 4,939 493
BRIEF COMMUNICATIONS
Total anomalous systemic venous drainage to the left atrium: An entity reviewed and investigated
Neeraj Awasthy, Sitaraman Radhakrishnan, Sunil Kaushal, Rajesh Sharma
May-August 2014, 7(2):98-102
DOI:10.4103/0974-2069.132476  PMID:24987254
We present three unusual cases of total anomalous systemic venous drainage (TASVD) where all the systemic veins were abnormally connected to the atrium that drained into the left ventricle (LV). All three patients had features consistent with left atrial isomerism. Based on the available evidence and review of literature we propose that TASVD be included as a part of the syndrome of left atrial isomerism. A classification for TASVD is also proposed with surgical implications.
  - 3,140 298
CASE REPORTS
Giant aneurysm of the atrial appendages in infants
Sean M Lang, Abbas Haider Zaidi, Tal Haider Geva, Stephen P Sanders, Pedro Nido, E Kevin Hall
May-August 2014, 7(2):130-134
DOI:10.4103/0974-2069.132489  PMID:24987260
This case series describe two patients with giant aneurysms of the atrial appendages. This report discusses the clinical symptoms, imaging modalities, indications for surgical intervention, and histology of atrial appendage aneurysms.
  - 2,704 159
Surgical exclusion of postsurgical pseudoaneurysm of the ascending aorta
Ramachandra Barik, Amar Narayana Patnaik, Ravintula Venkata Kumar, Rudra Prasad Mohapatra, Vikas Medep, Lalita Nemani
May-August 2014, 7(2):135-137
DOI:10.4103/0974-2069.132490  PMID:24987261
Pseudoaneurysm of ascending aorta after cardiac surgery is rare in children. We report a case of successful surgical exclusion of ascending aortic pseudoaneurysm in a 15-year-old boy. The neck of the aneurysm was in close proximity to the right coronary artery (RCA).
  - 2,722 136
Mycotic aneurysm in a child with history of coarctation of the aorta repair
M Santiago Restrepo, Joseph W Turek, Benjamin Reinking, Nicholas Von Bergen
May-August 2014, 7(2):138-141
DOI:10.4103/0974-2069.132493  PMID:24987262
A mycotic aneurysm is a rare condition occasionally seen in patients with a history of prior cardiac or vascular surgery. Here we report the presentation of a mycotic aneurysm in a pediatric patient at the site of prior aortic coarctation repair. This patient's initial presentation suggested rheumatologic or oncologic disease, and after diagnosis he continued to show evidence of splenic, renal and vascular injury distal to the mycotic aneurysm site while being treated with antibiotics. We discuss the diagnosis, treatment and management of this condition.
  - 2,825 139
Right aortic arch with isolation of the left innominate artery in a case of double chamber right ventricle and ventricular septal defect
Chirantan Mangukia, Sonali Sethi, Saket Agarwal, Smita Mishra, Deepak Kumar Satsangi
May-August 2014, 7(2):148-151
DOI:10.4103/0974-2069.132500  PMID:24987265
Herein, we report an unusual case of right aortic arch with isolation of the left innominate artery in a case of double chamber right ventricle with ventricular septal defect. The blood supply to the innominate artery was by a collateral arising from the descending aorta. The embryological development of this anomaly can be explained by the hypothetical double aortic arch model proposed by Edwards with interruption of the arch at two levels.
  - 3,252 150
EDITORIAL
Professor Rajendra Tandon: Passing of a legend
Shyam Sunder Kothari, R Krishna Kumar
May-August 2014, 7(2):83-85
DOI:10.4103/0974-2069.132472  PMID:24987251
  - 3,407 251
IMAGES
Giant coronary artery aneurysms in a 58-year-old
Anil Kumar Singhi, Sreeja Pavithran, Kothandam Sivakumar
May-August 2014, 7(2):152-154
DOI:10.4103/0974-2069.132502  PMID:24987266
All giant Kawasaki aneurysms may not regress fully; some may eventually calcify, undergo thrombosis, and get detected in asymptomatic adults at later age. Tomisaku Kawasaki initially described this illness as mucocutaneous lymph node syndrome in childhood in 1967 and coronary arteritis was recognized later. We present a 58-year-old male, possibly one of the oldest surviving patients with giant coronary aneurysms who presented with large secundum atrial septal defect (ASD) with heart failure. This indicates that the disease was perhaps prevalent outside Japan even before the first Kawasaki's description.
  - 2,547 139
Circular shunt in a pulmonary artery to right atrial tunnel, an anomaly unreported so far
Anil Kumar Singhi, Kothandam Sivakumar
May-August 2014, 7(2):155-157
DOI:10.4103/0974-2069.132504  PMID:24987267
While aortico-right atrial tunnels with left to right shunt from aorta to right atrium are reported widely, pulmonary artery to right atrial tunnels have not been described so far. Such a tunnel will lead to a circular shunt with a recirculation of blood in the right sided cardiac chambers repeatedly bypassing the pulmonary capillary bed. This newly described pulmonary artery to right atrial tunnel was closed nonsurgically with a duct occluder after angiographic delineation.
  - 2,738 124
Multimodality imaging of a subtotally obstructive right ventricular myxoma in an asymptomatic 10-year-old girl
Gesa Wiegand, Ludger Sieverding, Ulrich Kramer, Susanne Haen, Michael Hofbeck
May-August 2014, 7(2):158-159
DOI:10.4103/0974-2069.132519  PMID:24987268
A 10-year-old girl presented with a supraventricular tachycardia. A heart murmur was detected during a clinical examination; therefore, echocardiography was performed. This revealed a giant right ventricular myxoma that subtotally obstructed the right ventricular outflow tract. A myxoma in the right ventricle is uncommon in children. This rare intracardiac tumor was examined using multimodality imaging, and the patient underwent surgical resection. The pathologic features confirmed our suspicion and revealed an encapsulated tumor with myxomatoid stroma containing focal hemorrhages.
  - 2,813 101
IMAGES IN PEDIATRIC CARDIOLOGY
Xanthoma tuberosum in homozygous familial hypercholesterolemia
Nagaraja Moorthy, Rajiv Ananthakrishna, Prabhavathi Bhat, Manjunath C Nanjappa
May-August 2014, 7(2):118-119
DOI:10.4103/0974-2069.132479  PMID:24987257
Familial homozygous hypercholesterolemia is one of the high risk factors that can result in premature coronary arterial disease leading to severe morbidity and premature death in children and young adults. We describe a rare example of extensive xanthoma tuberosum in a case of familial homozygous hypercholesterolemia.
  - 2,507 142
REVIEW ARTICLE
The Hemi-Fontan operation: A critical overview
Sachin Talwar, Vinitha Viswambharan Nair, Shiv Kumar Choudhary, Balram Airan
May-August 2014, 7(2):120-125
DOI:10.4103/0974-2069.132480  PMID:24987258
The hemi-Fontan (HF) operation is a staging procedure in the journey towards an ultimate Fontan palliation. Although popular in the Western world, it has found limited application in the developing world. In this review we discuss the indications, techniques, merits, and demerits of this procedure along with its present day role in developing world where there is lack of awareness about this operation.
  - 4,498 441