Sudden death in the young is rare. About 25% of cases occur during sports. Most young people with sudden cardiac death (SCD) have underlying heart disease, with hypertrophic cardiomyopathy and coronary artery anomalies being commonest in most series. Arrhythmogenic right ventricular dysplasia and long QT syndrome are the most common primary arrhythmic causes of SCD. It is estimated that early cardiopulmonary resuscitation and widespread availability of automatic external defibrillators could prevent about a quarter of pediatric sudden deaths.

Background : Malnourished children suffer several alterations in body composition that could produce cardiac abnormalities. Aim : The aim of the present study was to detect the frequency of myocardial damage in malnourished children as shown by echocardiography and cardiac troponin T (cTnT) level. Methods : Forty-five malnourished infants and young children (mean±SD of age was 11.24 ±7.88 months) were matched with 25 apparently healthy controls (mean±SD of age was 10.78±6.29 months). Blood sample was taken for complete blood picture, liver and kidney function tests, serum sodium, potassium, calcium levels and cTnT. All the malnourished children were subjected to echocardiographic evaluation. Results : Malnourished children showed a significantly lower left ventricular (LV) mass than the control group. The LV systolic functions were significantly impaired in patients with severe malnutrition. The cTnT level was higher than the upper reference limits in 11 (24.44%) of the studied malnourished children and all of them had a severe degree of malnutrition. The cTnT level was significantly higher in patients with anemia, sepsis and electrolyte abnormalities and it correlated negatively with LV ejection fraction (EF). Six of the studied children with high cTnT levels (54.5%) died within 21 days of treatment while only one case (2.9%) with normal level of cTnT died within the same period. Conclusions: LV mass is reduced in malnourished children. Children with severe malnutrition have a significant decrease in LV systolic functions. Elevated cTnT levels in malnourished children has both diagnostic and prognostic significance for cardiomyocyte damage.

Background : Absence of right pulmonary artery (RPA) is a rare congenital abnormality with variable presentation. The diagnosis is often missed in infants Objective : The aim of this study was to describe infantile presentation of isolated absence of RPA along with a brief review of the literature. Methods : The details of five patients diagnosed with isolated absence of RPA from April 2007 to October 2009 were reviewed retrospectively Results : Five patients were identified with this anomaly. The median age of presentation was 86 days (range, 40-120 days) and the median weight was 3.65 kg (range, 3.1-5.5 kg). All patients presented with breathing difficulty and had severe pulmonary hypertension (PHT) along with absent RPA on echocardiography. A multidetector computed tomographic scan was performed to confirm the diagnosis in four cases. Three patients had major aortopulmonary collateral and the hilar RPA was not well developed in all. A correct diagnosis was made before referral in one patient only. Differential vascularity in chest X-ray, a useful clue, was seen in four of five cases. Surgical correction was not considered in view of the small hilar pulmonary artery. The patients were all managed medically with diuretics and Sildenafil. Conclusion : Isolated absence of RPA is a rare congenital abnormality with varied presentation. Infantile presentation is marked with congestive cardiac failure and PHT. Specific diagnostic clue includes differential vascularity on the chest X-ray.

Background and Aim : The main goal of this study was to assess the frequency of recurrent coarctation after repair using different surgical methods. Methods : Surgical results of repairs for coarctation of aorta (Co-A) in 188 patients under the age 14 years who were treated in Rajaee Heart Center, Tehran, Iran, were evaluated retrospectively. The most common methods included patch-graft aortoplasty (59%), resection with end-to-end anastomosis (20.7%) and subclavian flap aortoplasty (SCFA) (16.5%). The remaining patients underwent bypass tube graft and excision with placement of a tube graft. Seventy eight percent had discrete stenosis while 22% had long segment narrowing. The patients were followed for 81.6±32.8 months. Results : The overall mortality rate was 2.6%. The highest incidence rate of recoarctation was found in the patch-graft aortoplasty group (12.7%) and the lowest was found in SCFA (3.2%). The incidence of recoarctation in long-segment lesions was significantly higher than that in the discrete ones (30% vs. 4%, P<0.001). In patients <1 year, the incidence of recoarctation was lower than that in the other age groups. Conclusion : The patch-graft aortoplasty technique had the highest incidence of recoarctation and SCFA had the lowest rate. Long-segment Co-A had a higher chance of recoarctation. In contrast to some previous reports, the incidence of recoarctation was not higher in the age below 1 year.

Occlusions of major vessels in patients with congenital heart disease may occur due to a variety of factors. These occlusions are often felt to be best addressed surgically; however, we and others have been successful in recanalizing most of these vessels in the catheterization laboratory. Most of these patients will require multiple procedures in the catheterization laboratory to ensure vessel patency and to facilitate vessel growth. Physicians performing the procedure should have a thorough understanding of the anatomic considerations for the intended procedure and have access to a variety of devices and equipment to optimize the result of the procedure. In this article, we review some of the technical aspects that are vital for the success of the procedure.

A transient and reversible reduction in cardiac output-low cardiac output state (LCOS) often occurs following surgery for congenital heart disease. Inappropriately managed LCOS is a risk factor for increased morbidity and death. LCOS may occasionally be progressive and refractory needing a period of "myocardial rest" with extracorporeal life support (ECLS). ECLS is currently considered a routine tool available for rapid deployment in most industrialized countries. Accumulated experience and refinements in technology have led to improving survivals - discharge survivals of 35%−50%, with almost 100% survival in select groups on elective left ventricular assist device. Thus, there is an increasing trend to initiate ECLS "early or electively in the operating room" in high-risk patients. India has a huge potential need for ECLS given the large number of infants presenting late with preexisting ventricular dysfunction or in circulatory collapse. ECLS is an expensive and resource consuming treatment modality and is not a viable therapeutic option in our country. The purpose of this paper is to reiterate an anticipatory, proactive approach to LCOS: (1) methods for early detection of evolving LCOS and (2) timely initiation of individualized therapy. This paper also explores what is feasible with the refinement of "simple, conventional, inexpensive strategies" for the management of LCOS. Therapy for LCOS should be multimodal based on the type of circulation and physiology. Our approach to LCOS includes: (1) intraoperative strategies, (2) aggressive afterload reduction, (3) lusitropy, (4) exclusion of structural defects, (5) harnessing cardiopulmonary interactions, and (6) addressing metabolic and endocrine abnormalities. We have achieved a discharge survival rate of greater than 97% with these simple methods.

Cardiac myxomas (CMs) account for nearly half of the primary cardiac tumors in the elderly. They arise from sub-endocardial "reserve" or "lepidic" cells, which may show divergent differentiation. We describe a CM with glandular differentiation in the right atrium of a 10-year-old child who presented with respiratory distress on exertion, of 2 months duration. On echocardiography, two large interconnected masses measuring 34Χ30 mm and 20Χ17 mm were seen to arise from the free wall of the right atrium. Cut surface of the excised mass was myxoid with areas of calcification. On microscopy, there were typical features of a myxoma with prominent glandular differentiation and characteristic immunophenotype. The case is being reported due to its rarity in pediatric age group as well as its glandular differentiation, which must be recognized as a spectrum of histomorphologic diversity and must not be mistaken for a metastatic adenocarcinoma.

The window ductus represents a very rare anatomical anomaly. Only a few cases of successful surgical repair have been reported. We present a 12 year old boy in whom window ductus was diagnosed and successfully operated. The operation included closure of the large (2.5 cmΧ0.5 cm) connection between the main pulmonary artery and the aortic arch using cardiopulmonary bypass and hypothermia with circulatory arrest.

The prenatal ultrasonographic diagnosis of ectopia cordis associated with a complex intra-cardiac defect (common atrium, common atrioventricular valve with single ventricle) is illustrated in a 32-week gestation fetus. The fetus showed associated features of amniotic band disruption sequence. The cardiac autopsy findings correlated with the antenatal diagnosis. The association of ectopia cordis with amniotic band disruption is rare and infrequently reported in literature.

We present a three-dimensional reconstructed image of vascular ring in a 2.5-month-old patient, which was obtained using multidetector computed tomography (MDCT). MDCT angiography made an accurate diagnosis of this life-threatening, but correctable, anomaly in an infant with a stridor, repeated respiratory infections and episodes of apnea.

A 57-year-old lady presenting with angina was found to have multiple coronary arterio venous fistulae (CAVF) arising from both left and the right coronary arteries and draining into the pulmonary artery. She underwent successful surgical closure of these CAVF.

Fungal endocarditis (FE) is rare in children and does not usually occur in structurally normal hearts. The commonest causative agent is Candida albicans. We report a 5-year-old female child presenting with high-grade fever and cardiac failure. Anemia, leukocytosis and high CRP were found, but bacterial blood culture was sterile. There was no response to antimicrobial agents. Two-dimensional echocardiography revealed a large heterogeneous mass attached to the right ventricle and tricuspid valve. Provisional diagnosis of FE was made, which was confirmed by growth of Candida tropicalis in blood culture. Liposomal amphotericin B was started, followed by radical curative surgery including excision of the entire vegetation with total tricuspid valve excision. Histopathology and culture of the resected vegetation confirmed the diagnosis. The patient was given antifungal therapy for a total of 7 weeks, including 2 weeks of post-operative treatment, following which she was afebrile.

A 22-year-old man was referred for evaluation of exertional fatigue. On examination, there were no overt signs of congestive heart failure. Transthoracic and transesophageal echocardiography revealed rupture of the right coronary aortic sinus of Valsalva into the right ventricle. It was successfully closed with a 12 X 10 Amplatzer duct occluder.

A 13-day-old baby girl presenting with features of congestive cardiac failure was found to have coarctation of the aorta (CoA) and patent ductus arteriosus (PDA) by echocardiography. Doppler spectral display revealed moderate CoA. Echocardiogram, 12 hours later, showed severe juxtaductal aortic coarctation with spontaneous closure of PDA. This case emphasises the need to keep a close watch on the progress of CoA in the neonatal period, even if the duct has narrowed to a small size thus demonstrating the role of constriction of juxtaductal aorta in pathogenesis of coaractation. Closure of even asmall PDA can cause acute progression CoA in the presence of posterior shelf.

Severe pulmonary hypertension in a teenager with end-stage renal disease on chronic hemodialysis via arteriovenous access is reported. Clinical presentation included persistent volume overload and pericardial effusion. Serial hemodynamic data obtained at cardiac catheterization confirmed the diagnosis. In addition, detailed biochemical and imaging data (echo- Doppler, computed tomography of chest, computed tomographic pulmonary angiography, VQ lung scan, etc.) were obtained to find out the mechanism. The exact cause of pulmonary hypertension remains unclear, and a multi- factorial mechanism is postulated. This rare case is presented to highlight the role of aggressive dialysis, pericardiocentesis, and use of sildenafil and bosentan in the management.

Anatomically corrected malposition of great arteries (ACMGA) is a rare form of congenital heart disease in which the great arteries arise above the anatomically correct ventricles but have abnormal spatial relationship. We report the case of a 26-year-old female with ACMGA and tunnel type of subaortic obstruction. The abnormal relationship and segmental arrangement necessitates systematic approach in evaluation for proper diagnosis and surgical repair. This unusual case is reported for its rarity and to highlight the need for awareness to differentiate it from other more common conditions.

A 6-year-old girl, a known case of Kawasaki disease, presented with acute thrombotic occlusion of right coronary artery (RCA) with symptoms of acute angina and myocardial dysfunction. She underwent beating heart off-pump coronary artery bypass graft (CABG) surgery with right internal mammary artery (RIMA) grafted to distal RCA. Follow-up computed tomography angiogram revealed well-flowing RIMA with no obstruction or kink. This case highlights the importance of CABG as a safe and life-saving procedure in expert hands, even for children in emergent conditions.

Pulmonary arterio venous malformations are one of the rare vascular anomalies. Usually single large pulmonary arteriovenous fistula (PAVF) can be closed through transcatheter route but multiple PAVF require surgical lobectomy. We describe a case of an 8-year-old boy with multiple PAVF, who underwent successful transcatheter closure with multiple coils.

One of the most interesting congenital malformations is that of conjoined twins. We report echocardiographic features of twin heart in dicephalus, tribrachius, dispinous, thoracoomphalopagus twin. It showed two hearts fused at atrial level. Right-sided heart had single atrial chamber with a single ventricle. A single great vessel, aorta, originated from it. Left-sided heart was well developed with two atria and two ventricles. There was a small mid muscular ventricular septal defect and a small patent ductus arteriosus. Great arteries had normal origins.