Objective: To study the impact of red cell distribution width (RDW) on postoperative recovery after correction of Tetralogy of Fallot (TOF). Background: Increased RDW indicates dysregulated erythropoiesis and predicts survival in critical illnesses that include idiopathic pulmonary artery hypertension and chronic heart failure. Myocardial injury and oxidative stress induced by cardiopulmonary bypass potentially contribute to prolonged recovery in post TOF repair patients. Materials and Methods: Retrospective analysis of data on 94 consecutive children with TOF undergoing corrective repair (January 2010-March 2011) was done. RDW was higher for the study population when compared to acyanotic patients with ventricular septal defect (17.7 ± 3.7 vs. 16.2 ± 4.2; P < 0.001). The mean RDW obtained from 100 separate age-, sex-, and weight-matched TOF patients (17.8) was chosen as a cut-off. Of 93 survivors (median age: 12 (4-204) months, weight: 8.6 (3.2-70) kg), 29 patients with higher RDW (> 17.8) had a longer ICU stay (155.6 ± 71.3 vs. 122.4 ± 61.3 hours, P = 0.02), hospital stay (18.6 ± 10.5 days vs. 13.4 ± 6.5 days, P = 0.01), ventilation time (57.9 ± 41.6 vs. 38.3 ± 30.8 hours, P = 0.01), and more surgical site infection (24.1% vs. 6.2%, P = 0.01). On multivariate analysis only elevated RDW (other variables included age, weight, hemoglobin, hematocrit, and surgical support times) showed a significant association with hospital stay. Conclusions: Elevated RDW appears to be associated with prolonged recovery after TOF repair, the precise underlying mechanisms are worth investigating.

Background: The dilated aorta in adults with bicuspid aortic valve has been shown to have different shapes, but it is not known if this occurs in children. This observational study was performed to determine if there are different shapes of the dilated aorta in children with bicuspid aortic valve and their association with age, gender, hemodynamic alterations, and degree of aortic enlargement. Methods: One hundred and eighty-seven echocardiograms done on pediatric patients (0 - 18 years) for bicuspid aortic valve, during 2008, were reviewed. Aortic valve morphology, shape/size of the aorta, and pertinent hemodynamic alterations were documented. Aortic dilation was felt to be present when at least one aortic segment had a z-score > 2.0; global aortic enlargement was determined by summing the aortic segment z-scores. The aortic shape was assessed by age, gender, valve morphology, and hemodynamic alterations. Results: Aortic dilation was present in 104/187 patients. The aorta had six different shapes designated from S1 through S6. There was no association between the aortic shape and gender, aortic valve morphology, or hemodynamic abnormalities. S3 was the most common after the age of six years and was associated with the most significant degree of global aortic enlargement. Conclusions: The shape of the dilated aorta in children with bicuspid aortic valve does not occur in a uniform manner and multiple shapes are seen. S2 and S3 are most commonly seen. As aortic dilation becomes more significant, a single shape (S3) becomes the dominant pattern.

Background: Amplatzer vascular plugs (AVPs) are devices ideally suited to close medium-to-large vascular communications. There is limited published literature regarding the utility of AVPs in congenital cardiovascular malformations (CCVMs). Aims: To describe the use of AVPs in different CCVMs and to evaluate their safety and efficacy. Materials and Methods: All patients who required an AVP for the closure of CCVM were included in this retrospective review of our catheterization laboratory data. The efficacy and safety of AVPs are reported. Results: A total of 39 AVPs were implanted in 31 patients. Thirteen (33%) were AVP type I and 23 (59%) were AVP type II. AVP type III were implanted in two patients and type IV in one patient. The major indications for their use included closure of pulmonary arteriovenous malformation (AVM) (n = 7), aortopulmonary collaterals (n = 7), closure of a patent Blalock-Taussig shunt (n = 5), systemic AVM (n = 5), coronary AVM (n = 4), patent ductus arteriosus (PDA) (n = 3), pulmonary artery aneurysms (n = 3), and venovenous collaterals (n = 2). Deployment of the AVP was done predominantly via the 5 - 7F Judkin's right coronary guide catheter. Overall 92% of the AVPs could be successfully deployed and resulted in occlusion of the target vessel in all cases, within 10 minutes. No procedure-related or access site complication occurred. Conclusions: AVPs are versatile, easy to use, and effective devices to occlude the vascular communications in a variety of settings. AVP II is especially useful in the closure of tubular structures with a high flow.

Background: Pulmonary valve regurgitation following balloon valvuloplasty for moderate to severe pulmonary valve stenosis is a known late outcome of this procedure. Objective: The aim of the study was to characterise the status of pulmonary regurgitation on follow up after pulmonary valve balloon dilatation (PVBD), and to study the determinant of the severity of PR. Materials and Methods: We retrospectively reviewed 50 consecutive patients, aged 2 days to 18 years, with isolated pulmonary valve stenosis, who had undergone PVBD in 2004-2009 and were assessed with follow-up Doppler echocardiography. The impact of balloon to annulus ratio, age, and valve anatomy on the late development of moderate and severe pulmonary valve regurgitation following balloon valvuloplasty was analysed. Results: Six patients (12%) had no pulmonary valve regurgitation; 32 (64%) had mild, 9 (18%) had moderate, and 3 (6%) had severe pulmonary valve regurgitation at a mean follow-up of 4 years. Balloon to annulus ratio, age, and valve anatomy were not statistically significant predictors for moderate and severe pulmonary valve regurgitation. Conclusions: The majority of patients in our population had mild pulmonary valve regurgitation. Moderate to severe pulmonary valve regurgitation was well tolerated at midterm follow-up. Age, balloon to annulus ratio, and valve anatomy were not statistically significant predictors for the late development of moderate and severe valve regurgitation. Large and longer follow-up studies are needed to address this question.

Complex congenital heart disease (CHD) is associated with significant morbidity worldwide. Managing hypoxemia in these populations can be difficult, particularly in the setting of cyanotic CHD. However, the presence of additional extracardiac shunts secondary to acute respiratory disease can be very challenging to manage. Before understanding how to deal with hypoxemia in patients with dual shunts, one needs to understand the physiology and diagnosis related to the individual shunts and apply this knowledge to the patient as a whole.

A four-month-old female infant presented with heart failure and cyanosis. On echocardiography, there was no outlet to the right atrium except an atrial septal defect and the left atrium drained into the respective ventricles by two atrioventricular valves. The case is reported for its rarity, with a brief literature review.

Double-orifice tricuspid valve (DOTV) is a very rare congenital cardiac anomaly. We report an unusual example of DOTV where one orifice was committed to the left ventricle. This was identified preoperatively and managed surgically.

We describe the echocardiographic features of submitral aneurysm in a 26 week old fetus. The diagnosis was confirmed on neonatal echocardiography.

We report a newborn with an unusual combination of aneurysmally dilated thin-walled right ventricle with hypertrophy of the apical muscles of the right ventricle. There was narrow pulmonary annulus, pulmonary regurgitation, and hypoplasia of the mitral valve and left ventricle. We propose that this heart represents a partial form of Uhl`s anomaly.

Selected children with congenital heart defects undergoing palliative closed heart procedures require a cardiopulmonary bypass (CPB) run only for the purpose of creating an inter-atrial communication. We report a simple technique of atrial septostomy using thoracoscopy scissors under transesophageal echocardiography guidance without the need for CPB.

An eight-year-old boy was evaluated for unexplained hemoptysis and cyanosis. A contrast echocardiogram was suggestive of pulmonary arteriovenous fistula. Further evaluation revealed persistent ductus venosus (PDV) and aortopulmonary collaterals. Both the PDV and aortopulmonary collaterals were closed percutaneously. PDV is amenable for device closure after detailed anatomical evaluation. Prior to closure, it is important to ensure adequate portal vein arborization into the liver and normal portal pressure after test balloon occlusion.

We report a successful perventricular closure of an apical muscular ventricular septal defect (mVSD) by a modified technique. An eight-month-old infant, weighing 6.5 kilograms, presented with refractory heart failure. The transthoracic echocardiogram showed multiple apical mVSDs with the largest one measuring 10 mm. perventricular device closure using a 12 mm Amplatzer mVSD occluder was planned. The left ventricular disk was positioned approximating the interventricular septum; however, the right ventricular (RV) disk was deployed on the free wall of the RV due to an absent apical muscular septum and a small cavity at the apex. The RV disk of the device was covered using an autologous pericardium. His heart failure improved during follow-up.

Left ventricular (LV) myxoma is particularly rare in children and has not been reported in infants. A five-month-old baby presented with a myxoma arising from the anterior, lateral, and superior aspect of the LV, causing severe left ventricular outflow tract obstruction. The LV was accessed through the conal septum after opening the right ventricular outflow. The child had transient complete heart block in the postoperative period. There was no recurrence of tumor at the nine-month follow-up.

Anti-Ro/La negative congenital heart block (CHB) is uncommon. We report one such case of CHB, with no associated structural heart disease or maternal autoantibodies. The heart block reverted to sinus rhythm spontaneously at two weeks of age, and the patient remains in sinus rhythm at a one year followup. Whether patients with antibody negative complete heart block have a different clinical course is conjectural.

The internal jugular vein is not a typical vascular access line during the percutaneous closure of an atrial septal defect. We report the closure of a double atrial septal defect with a single device, using a transjugular venous approach, in a child with an inferior vena cava obstructed by a thrombosis due to previous cardiac catheterization. That the transjugular venous approach can be used as a possible alternative during the transcatheter closure of an atrial septal defect in children, when the inferior vena cava access is not possible.

We report the successful transcatheter closure of right pulmonary artery fistula to left atrium in a six-year-old boy, who had presented with cyanosis and shortness of breath. The two-dimensional echocardiogram with bubble contrast study demonstrated the communication between right pulmonary artery and left atrium. Computerized tomography confirmed the diagnosis and delineated the anatomy. The fistula was closed successfully by a transcatheter trans-septal approach using an 18/20 duct occluder.

We present an unusual cause of progressive cyanosis in a child appearing 2 years after successful Fontan surgery for tetralogy of Fallot with hypoplastic right ventricle. The cause of cyanosis was identified as one large venous channel draining into the left atrium. The channel was closed by Amplatzer vascular plug resulting in improvement of oxygen saturation.

We report a 6-year-old girl with anomalous origin of the right pulmonary artery from the aorta associated with a large ventricular septal defect. The challenges in the assessment of operability and issues in the surgical management are discussed.

We report a case of a six-month-old Hispanic male infant who had Kawasaki disease and coronary artery aneurysms on echocardiography. He died suddenly five months later in spite of aggressive medical therapy. Autopsy showed extensive coronary artery thrombosis. Giant coronary artery aneurysms need diligent follow up as they pose significant risks including risk of thrombus, myocardial infarction and sudden death.

VACTERL association is a non-random association of birth defects of unknown etiology derived from structures of embryonic mesoderm. The common cardiac defects seen with VACTERL association are ventricular septal defects, atrial septal defects, and tetralogy of Fallot. We present a 2-year-old child with VACTERL association in whom we detected double-chambered left ventricle on transthoracic echocardiography.

A quadricuspid aortic valve is rarely diagnosed in children, but it can be associated with significant aortic regurgitation. It is important for pediatric cardiologists to be aware of this pathologic entity. We present a nine-year-old male, diagnosed with a quadricuspid aortic valve and mild aortic regurgitation.

Obstructive lesions in the inflow of the systemic ventricle in congenitally corrected transposition of the great arteries are rare. It is important to identify such lesion which could alter the surgical outcome if not recognized. We report the echocardiographic findings in a patient with supratricuspid obstructive membrane with corrected transposition of the great arteries.