Annals of Pediatric Cardiology
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   2015| September-December  | Volume 8 | Issue 3  
    Online since September 7, 2015

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Inherited arrhythmias: The cardiac channelopathies
Shashank P Behere, Steven N Weindling
September-December 2015, 8(3):210-220
DOI:10.4103/0974-2069.164695  PMID:26556967
Ion channels in the myocardial cellular membrane are responsible for allowing the cardiac action potential. Genetic abnormalities in these channels can predispose to life-threatening arrhythmias. We discuss the basic science of the cardiac action potential; outline the different clinical entities, including information regarding overlapping diagnoses, touching upon relevant genetics, new innovations in screening, diagnosis, risk stratification, and management. The special considerations of sudden unexplained death and sudden infant death syndrome are discussed. Scientists and clinicians continue to reconcile the rapidly growing body of knowledge regarding the molecular mechanisms and genetics while continuing to improve our understanding of the various clinical entities and their diagnosis and management in clinical setting. Two separate searches were run on the National Center for Biotechnology Information's website. The first using the term cardiac channelopathies was run on the PubMed database using filters for time (published in past 5 years) and age (birth-18 years), yielding 47 results. The second search using the medical subject headings (MeSH) database with the search terms "Long QT Syndrome" (MeSH) and "Short QT Syndrome" (MeSH) and "Brugada Syndrome" (MeSH) and "Catecholaminergic Polymorphic Ventricular Tachycardia" (MeSH), applying the same filters yielded 467 results. The abstracts of these articles were studied, and the articles were categorized and organized. Articles of relevance were read in full. As and where applicable, relevant references and citations from the primary articles where further explored and read in full.
  7,678 851 8
Plastic bronchitis
Anil Kumar Singhi, Bharathi Vinoth, Sarah Kuruvilla, Kothandam Sivakumar
September-December 2015, 8(3):246-248
DOI:10.4103/0974-2069.164682  PMID:26556975
Plastic bronchitis, a rare but serious clinical condition, commonly seen after Fontan surgeries in children, may be a manifestation of suboptimal adaptation to the cavopulmonary circulation with unfavorable hemodynamics. They are ominous with poor prognosis. Sometimes, infection or airway reactivity may provoke cast bronchitis as a two-step insult on a vulnerable vascular bed. In such instances, aggressive management leads to longer survival. This report of cast bronchitis discusses its current understanding.
  5,262 295 7
Demonstration of circular shunt in fetal Ebstein anomaly
Deepa Sasikumar, Bijulal Sasidharan, Sivasankaran Sivasubramanian
September-December 2015, 8(3):249-250
DOI:10.4103/0974-2069.154155  PMID:26556976
Ebstein's anomaly was diagnosed in a fetus at 24 weeks of gestation. There was significant cardiomegaly and severe tricuspid regurgitation (TR). There was functional pulmonary atresia with severe pulmonary regurgitation (PR) and this was causing a circular shunt. There was no fetal hydrops.
  5,260 293 -
Clarifying the atrioventricular junctional anatomy in the setting of double outlet right atrium
Saurabh Kumar Gupta, Anunay Gupta, Sivasubramanian Ramakrishnan, Robert H Anderson
September-December 2015, 8(3):233-239
DOI:10.4103/0974-2069.164688  PMID:26556972
Double outlet atrium is a rare cardiac anomaly wherein one of the atriums, most frequently the right atrium, opens into both the ventricles. Although seen more commonly in the setting of atrioventricular septal defect, this arrangement can also be found when one of the atrioventricular connections is atretic due to absence of the atrioventricular connection and the other atrioventricular valve straddles the muscular ventricular septum. It is the specific anatomy and connections of the atrioventricular junction that clarifies the situation and distinguishes between these two types of double outlet atrium. In this report, we present a case of double outlet right atrium co-existing with the absence of left atrioventricular connection. We then discuss the morphologic aspects of this interesting anomaly.
  4,575 293 3
Universal heart coverage for children with heart disease in India
Raman Krishna Kumar
September-December 2015, 8(3):177-183
DOI:10.4103/0974-2069.164674  PMID:26556962
  4,454 311 2
A novel snare assistance safeguards against early embolization of devices and facilitates quick retrieval of malpositioned devices in atrial septal defects with deficient margins
Sreeja Pavithran, Kothandam Sivakumar
September-December 2015, 8(3):189-195
DOI:10.4103/0974-2069.164685  PMID:26556964
Background : Embolization might complicate device closure of large atrial septal defects (ASDs) with deficient margins. When margins are deficient, a precariously placed device can appear to be held in good position by the rigid delivery cable. Once the cable is unscrewed, the device adopts the natural lie of the interatrial septum. This can occasionally expose the inadequately captured margins and lead to device embolization. Most embolizations occur immediately after release. Retrieval of the embolized device required prolonged fluoroscopy and sometimes open heart surgery. Objective : To evolve a new strategy of retrieval of a malpositioned device after unscrewing the cable before impending embolization. Materials and Methods : After deploying the device in place, a snare is passed through the delivery sheath around the cable to grip the screw on the right atrial disc of the device. With the snare holding the screw end, the device is released by unscrewing the cable. The device position is reconfirmed on echocardiography. The snare is subsequently removed if the device was stable. In case of device migration, the same snare is used to retrieve the device before it embolizes completely. Results : Snare assistance was used in 24 patients considered as high-risk for device embolization. Its usefulness was demonstrated in two patients with deficient posterior margin and small inferior margin where the device got malpositioned immediately after release. As the snare was still holding on to the screw end, the device could be retrieved into the sheath easily. Conclusion : This novel snare assisted device release strategy safeguards against device embolization in large ASDs with deficient rims and allows simplified retrieval.
  4,449 301 3
Endomyocardial fibrosis in Sub Saharan Africa: The geographical origin, socioeconomic status, and dietary habits of cases reported in Yaounde, Cameroon
David Chelo, Felicitee Nguefack, Hubert D Mbassi Awa, Samuel Kingue
September-December 2015, 8(3):202-209
DOI:10.4103/0974-2069.164693  PMID:26556966
Background : Endomyocardial fibrosis (EMF) is a neglected heart condition of the inter-tropical regions. Numerous hypotheses suggest a relationship between its geographical distribution in the affected regions and other etio-pathogenic factors such as dietary habits, infectious causes, and geochemical causes. Knowledge of its epidemiology in Cameroon remains limited, which is why we decided to describe the profile of a paediatric series of EMF in Yaoundé. Patients and Methods : A retrospective study was carried out on EMF in 54 patients diagnosed from 1 January 2006-31 December 2014 in a Paediatric Centre of Yaoundé. Diagnosis was mainly echocardiographic. We compiled data on the geographic origins of the patients, their dietary habits and the socioeconomic profile of their families. Results : The patients' ages ranged from 2 to 17 years, most of whom (83.3%) were between 5 and 15 years. For geographical distribution, all came from three tropical forest zones where they have lived since their childhood. These were Center (32/54), South (12/54), and East (10/54). All families had a moderate income, consumed tubers at least twice a week especially cassava (43/54) and had low sources of proteins. Conclusion : Apart from geographical similarities all patients of our series shared the same dietary habits. Our study was conducted in a hospital setting; therefore a screening of the disease in the whole national territory would enable a more reliable mapping.
  4,398 197 3
An adult with central cyanosis and differential pulmonary vascularity
Rajiv Ananthakrishna, Nagaraja Moorthy, Dattatreya PV Rao, Manjunath C Nanjappa
September-December 2015, 8(3):253-254
DOI:10.4103/0974-2069.150701  PMID:26556978
A 22-year-old male patient presented with progressive effort intolerance of 2-years duration. Clinical findings and investigations were suggestive of Tetralogy of Fallot (TOF). In addition, there was a conspicuous difference in the pulmonary vascularity with oligemia on the left side and relative hypervascularity on the right side. The right pulmonary artery was arising from the proximal ascending aorta and the main pulmonary artery was continuing as the left pulmonary artery. The anomalous origin of a branch pulmonary artery from the aorta (AOPA) is a rare cardiac anomaly. We report this condition in association with TOF, highlighting the differential pulmonary vascularity.
  4,144 205 -
Use of echocardiographic subxiphoid five-sixth area length (bullet) method in evaluation of adequacy of borderline left ventricle in hypoplastic left heart complex
Justin T Tretter, Sujata Chakravarti, Puneet Bhatla
September-December 2015, 8(3):243-245
DOI:10.4103/0974-2069.158520  PMID:26556974
Indexed left ventricular end-diastolic volume (ILVEDV) is commonly used in evaluating "borderline left ventricle (LV)" in hypoplastic left heart complex (HLHC) to determine if the LV can sustain adequate systemic cardiac output. Commonly used quantification methods include biplane Simpson or the traditional five-sixth area length "bullet" methods, which have been shown to underestimate true LV volumes, when septal position is mildly abnormal. Subxiphoid five-sixth area length method is proposed as a more accurate estimate of true LV volume in the evaluation of borderline LV.
  4,128 173 1
Transcatheter closure of patent vertical vein after repair of total anomalous pulmonary venous connection
Sudeep Verma, Anand Subramanian, Rajan Saileela, Nageswara Rao Koneti
September-December 2015, 8(3):221-224
DOI:10.4103/0974-2069.164692  PMID:26556968
Background: Vertical vein is left patent in some cases of supra-cardiac total anomalous pulmonary venous connection (TAPVC) when there is hemodynamic instability due to noncompliant left atrium and ventricle. After the peri-operative period, this results in features of pre-tricuspid shunt. Materials and Methods: Three cases with patent vertical vein following repair of supra-cardiac TAPVC presented with features of pre-tricuspid shunt on follow-up. Trans-catheter closure of patent vertical vein was performed using vascular plug in all three subjects. Results: The procedure was technically successful in all the patients. There was a complication related to catheter tip breakage in one of them, which was successfully managed. There was no impingement on pulmonary vein in any of the patients. Conclusion: Patent vertical vein following TAPVC repair results in features of pre-tricuspid shunt. Transcatheter closure of the patent vein is feasible.
  4,069 191 1
Percutaneous closure of patent arterial ducts in patients from high altitude: a sub-Saharan experience
Endale Tefera, Shakeel A Qureshi, Ramon Bermudez-Canete, Lola Rubio
September-December 2015, 8(3):196-201
DOI:10.4103/0974-2069.164690  PMID:26556965
Background : At high altitude, patent arterial ducts tend to be larger and associated with pulmonary hypertension. Patent ductus arteriosus device closure in this background could be challenging. Objectives : We report our experience with percutaneous closure of patent arterial ducts using a variety of devices in patients residing in a high altitude. Patients and Methods : This is a retrospective review of the case records of 145 patients (age 9 months-20 years, mean 5.6 ± 3.9 years, and weight 7-54 kg, mean 17.7 ± 9.4) with duct sizes ranging between 2 and 21 mm, (mean, 5.8 ± 2.7) who underwent percutaneous closure of patent arterial ducts. One hundred thirty-six (93.8%) of the patients were from a geographic area of 2100-2800 m above sea level. Results : Successful device closure was achieved in 143 cases. It was difficult to achieve device stability in two patients with expansile ducts. Therefore, they were treated surgically. The devices used were various types of duct occluder devices in 131 patients, while atrial and ventricular septal occluders were used in eight patients. For the group, mean systolic pulmonary artery (PA) pressure decreased from 47.0 ± 16.7 mmHg before occlusion to 29.0 ± 7.4 mmHg after occlusion (P ≤ 0.001)., mean diastolic PA pressure from 25.0 ± 10.9 mmHg to 14.8 ± 6.0 mmHg and the average mean PA pressure decreased from 35.9 ± 13.5 mmHg to 21.1 ± 6.5 mmHg. Complications (4.8%) included device and coil embolization, bleeding, and pulse loss. On follow-up (mean duration of 36.1 ± 12.1 months, range 12-62 months), 137 patients were in functional class 1, 3 had residual shunt, 2 had device migration and one patient had persisting pulse loss. Conclusions: Successful duct closure was achieved in the vast majority of patients, even though the ducts were larger and significant number of them had pulmonary hypertension in this high altitude group. There was a relatively higher incidence of residual shunts and device migration in this series, generally due to the nonavailability of optimal device and surgical support. Long-term follow-up is required before we can draw conclusions with regard to the sustainability of drop in PA pressures. Septal Occluder devices may be a possible alternative for large tubular or window-type ducts with severe pulmonary hypertension, where there may be concerns about the size and stability of duct occluder devices.
  3,881 235 -
"Classical Blalock-Taussig shunt" gone wrong: Confusing the right common carotid with right subclavian artery
A Mohammed Idhrees, Vijay Thomas Cherian, Sabarinath Menon, Thomas Mathew, Baiju S Dharan, Karunakaran Jayakumar
September-December 2015, 8(3):228-229
DOI:10.4103/0974-2069.164686  PMID:26556970
A 14-year-old girl underwent classical Blalock-Taussig shunt at 5 months of age. Computed tomography evaluation showed "Adachi type H" pattern of aortic arch vessels with the right common carotid artery being anastomosed to the right pulmonary artery mistaking it for the right subclavian artery.
  3,905 165 2
Assessment and feasibility of the four landmarks of the aortic root in a cohort of very preterm infants
Sam Phillips, Koert A de Waal
September-December 2015, 8(3):184-188
DOI:10.4103/0974-2069.164683  PMID:26556963
Background : The diameter of the aortic root is used as a parameter to calculate blood flow in very preterm infants. There are considerable differences in diameter of the four anatomical landmarks of the aortic root in children and adults, but limited data are available for the very preterm population. The aim of this study was to obtain reference and reliability data on two-dimensional measurements of the aortic root in very preterm infants <30 weeks gestation. Materials and Methods : Fifty long axis echocardiograms were reviewed and re-analyzed for measurements at the four anatomical landmarks of the aortic root; the aortic annulus, sinus of Valsalva (SV), sinotubular junction, and the proximal ascending aorta (PAA). A subjective visual scoring system was developed to quantify image quality. A random sample of images was blindly re-measured to assess intra- and inter-observer reliability. Results : Significant differences in diameter were found between the four landmarks, except between SV and PAA. Inter-observer coefficients showed marginal variation ranging from 5.0% to 8.2%, with slightly lower intra-observer variability. Overall image quality score was poorest for PAA on standard long axis images but improved when the probe was adjusted along the outflow tract. Conclusion : We present reliability and reference values for all four anatomic landmarks of the aortic root in very preterm infants and demonstrated the importance of standardizing and reporting cardiac output measurements in preterm infants.
  3,808 244 1
Pacemaker lead design masquerading as lead fracture
Madhu Gangadhara, Charles Peebles, James Gnanapragasam
September-December 2015, 8(3):251-252
DOI:10.4103/0974-2069.158521  PMID:26556977
Performing chest X-ray is an important annual investigation to check pacemaker lead integrity during follow-up of patients with pacemakers. Understanding lead design is vital to the correct interpretation of X-rays to prevent inappropriate interventions for patients as highlighted in this case.
  3,813 121 -
Neurogenic stunned myocardium associated with pediatric brain tumor may not be catecholamine-induced
Shigeta Moriya, Joji Inamasu, Motoki Oheda, Yuichi Hirose
September-December 2015, 8(3):240-242
DOI:10.4103/0974-2069.164689  PMID:26556973
A rare case of pediatric neurogenic stunned myocardium (NSM) associated with a brain tumor is reported. A previously healthy 6-year-old boy presented with coma, and imaging studies revealed a brain tumor. On hospitalization day 3, he developed NSM and neurogenic pulmonary edema necessitating intensive cardiopulmonary support. Although blood marker levels of cardiac injury were elevated, his plasma and urinary norepinephrine levels were within normal limits. His cardiorespiratory functions markedly improved by hospitalization day 8. This case report may be one of the first to document plasma and urinary catecholamine levels in pediatric NSM. While solid conclusion cannot be drawn based on experience from a single case, these results suggest that pediatric NSM may not be catecholamine-induced.
  3,619 139 2
Percutaneous tricuspid valve replacement in childhood
Mathias Emmel, Nalini Sreeram, Gerardus Bennink, Narayanswami Sreeram
September-December 2015, 8(3):230-232
DOI:10.4103/0974-2069.164687  PMID:26556971
Percutaneous replacement of the tricuspid valve with a bovine jugular venous valve (melody valve) was successfully undertaken in a 9-year-old boy. The patient had a previous history of bacterial endocarditis of the native tricuspid valve in infancy. Initially, a pericardial patch valve was created, followed by surgical replacement of the valve using a biological tissue valve at 4 years of age. Progressive stenosis and regurgitation of the biological valve, with severe venous congestion and resulting hepatic dysfunction prompted percutaneous valve replacement.
  3,455 179 1
Acute endocarditis of a percutaneously placed pulmonary valve
Karthik V Ramakrishnan, Laura Olivieri, Richard A Jonas
September-December 2015, 8(3):225-227
DOI:10.4103/0974-2069.164684  PMID:26556969
Endocarditis of percutaneously placed pulmonary valve is increasingly being recognized and reported as a potentially life-threatening complication. In this report, we discuss a 17-year-old male who presented with septic shock secondary to staphylococcal endocarditis of a percutaneously placed pulmonary valve.
  3,434 124 1
Surgical correction of obstructed total anomalous pulmonary venous return soon after birth
Nageswara Rao Koneti, Pallavi Kathare, Tapan Kumar Dash, Srinivas Murki
September-December 2015, 8(3):255-256
DOI:10.4103/0974-2069.164680  PMID:26556979
  3,329 141 -
The future of Ross procedure
Francesco Nappi, Cristiano Spadaccio, Christophe Acar
September-December 2015, 8(3):256-257
DOI:10.4103/0974-2069.164681  PMID:26556980
  3,283 137 -