Background: Junctional ectopic tachycardia occurs frequently after congenital cardiac surgery and can be a cause of increased morbidity and mortality. Dexmedetomidine (DEX) is an a2 adrenoreceptor agonist, has properties of controlling tachyarrhythmia by regulating the sympatho-adrenal system. Objective: To evaluate the efficacy of DEX for control of junctional ectopic tachycardia after repair of Tetralogy of Fallot (TOF). Materials and Methods: Two hundred and twenty pediatric cardiac patients with TOFs were enrolled in a prospective randomized control study. Patients underwent correction surgery. They were divided into two groups, i.e., Group 1 (DEX) and Group 2 (control). Heart rate, rhythm, mean arterial pressure (MAP) were recorded after the anesthetic induction (T1), after termination of bypass (T2), after 04 hours (T3), and 08 hours after transferring the patient to intensive care unit (ICU; T4). Results: Heart rate was comparable between two groups before starting the drug but statistically significant after bypass until 08 hours after transferring the patient to ICU. Junctional ectopic tachycardia occurred more in Group-2 (20%) as compared to Group-1 (9.09%; P = 0.022). Junctional ectopic tachycardia occurs early in Group-2 (0.14 ± 0.527 hours) as compared to Group 1 (0.31 ± 1.29 hours; P = 0.042). The duration of junctional ectopic tachycardia was more prolonged in Group-2 (1.63 ± 3.64 hours) as compared to Group-1 (0.382 ± 1.60 hours; P = 0.012). The time to withdraw from mechanical ventilation and ICU stay of Group 1 patient was less than of Group 2 patients (P = <0.001). Conclusion: DEX had a therapeutic role in the prevention of junctional ectopic tachycardia in patients undergoing repair for TOF.

Background: The larger size of the currently available transesophageal echocardiography (TEE) probes limits their use to relatively older infants undergoing cardiac surgery. In very young neonates and infants, epicardial echocardiogram is used to assess postoperative residual defects. Recently, a miniaturized microTEE probe compatible in neonates has been introduced for clinical use. We evaluated the use of this probe in small infants undergoing cardiac surgery. Materials and Methods: Thirty-three consecutive neonates and infants undergoing cardiac surgery at our institution were included in the study. Intraoperative echocardiography with Philips s8-3t microTEE probe done using IE33 platform was utilized to study the preoperative anatomy and assess postoperative results. Results: Thirty-three patients aged 3 days-2 years (mean 5.1 months) and weighing 2.5-11 kg (mean 4.4 kg) underwent perioperative evaluation using the microTEE probe. Good quality two-dimensional and color Doppler images were obtained in all patients. There were no complications related to the probe insertion or manipulation. The findings on microTEE led to revision of surgery in five patients. Certain echocardiographic parameters that could never be recorded with epicardial echocardiogram could be easily seen in microTEE. Conclusion: On preliminary evaluation, the microTEE probe provided good quality images in very small infants who were not amenable for transesophageal echocardiographic evaluation so far. The probe could be used safely in small infants without complications. It appears to be a promising imaging modality in the perioperative assessment of young infants undergoing cardiac surgery, in whom intraoperative epicardial echocardiography is currently the only tool.

Objective To establish normal reference ranges for the left ventricular dimensions in preterm infants and their correlation with gestational age, body weight and chronological age. Materials and Methods In a prospective study, 268 preterm babies, who fulfilled the criteria for inclusion, were examined in Kuwait during the years (2008-2010). Echocardiograms were performed to measure the left ventricular dimensions on 0-6 day(s) of life and at weekly intervals until they reached 36 weeks. The gestational age was grouped into three: 24-27, 28-31 and 32-35 weeks, and body weight into five: ≤999, 1,000-1,499, 1,500-1,999, 2,000-2,499 and ≥2,500 grams. The overall group differences were compared for each period of life: 0-6 days, 1-2, 3-4 and ≥5 weeks. Results The mean gestational age was 29.8 (± 2.38 SD) weeks, ranging between 24 and 35, and the mean body weight 1,479 (± 413 SD) grams, ranging between 588 and 3380. At the first scan (0-6 days of life), all the left ventricular measurements correlated well (P < 0.001) with body weight, and the same was observed with gestational age, except for left ventricular posterior wall thickness at end-systole and end-diastole. A significant gradual increase was noticed in all the dimensions with body weight during each period of life. However, with respect to gestational age, an increase was observed in all the dimensions during first four weeks, but the rate of increase became less after 5 weeks of life. Overall, a progressive and significant increase in all left ventricle measurements was observed during the first nine weeks of life. Conclusion The left ventricular dimension measurements were found to have significant correlation with both gestational age and body weight. The study also provides reference data, which can be used as normal reference tool for left ventricular dimensions for preterm infants against the gestational age, body weight and chronological age.

Management of complex congenital heart disease in patients with Heterotaxy syndrome (HS) has steadily improved. However, there is an insufficient appreciation of various non-cardiac issues that might impact the overall status of these patients. This article briefly reviews the implications of gastrointestinal, immunologic, genitourinary, respiratory, and central nervous system involvement in HS patients with a view to aid in their comprehensive clinical management.

With surgical palliation of hypoplastic left heart syndrome (HLHS), the tricuspid valve (TV) becomes the systemic atrioventricular valve and moderate/severe TV insufficiency (TVI), an adverse risk factor for survival to Fontan, has been reported in up to 35% of patients prior to stage I palliation. Precise echocardiographic identification of the mechanism of TVI cannot be determined by two-dimensional echocardiography. Three-dimensional echocardiography (3DE) can provide significant insight into the mechanisms of TVI. It is the intent of this report to propose a systematic method on how to evaluate and display 3DE images of the TV in HLHS which has not been done previously. TV anatomy, function, and the known mechanisms of insufficiency are reviewed. We defined three regions of the TV (anterior, posterior, septal) that can help define valve "leaflets" that incorporates the many variations of TV anatomy. To determine how the surgeon views the TV, a picture of a pathologic specimen of the TV was placed on a computer screen and rotated until it was oriented as it appears during surgery, the "surgeons view." We have proposed a systematic method for evaluating and displaying the TV using 3DE which can provide significant insight into the mechanisms causing TVI in HLHS. This has the potential to improve both the surgical approach to repairing the valve and, ultimately, patient outcomes.

Kawasaki disease (KD) is a common acute vasculitis in pediatric population that usually involves small- and middle-sized arteries, commonly coronary arteries. Although the incidence and natural course of coronary aneurysms after KD are well documented in studies, related reports on peripheral arterial and aortic aneurysms are scarce. We report the occurrence of a giant aortic aneurysm involving the horizontal part of aortic arch in a 28-month-old boy diagnosed with KD. This complication was managed by steroids therapy in the beginning. Because of mechanical complication and potential risk of rupture, surgery was undertaken.

We present an interesting case of a 2½-year-old child with Tetrology of Fallot with a large intracardiac mass in the left ventricle presenting with fever and bilateral stroke, that resolving with antitubercular therapy alone.

Rhabdomyoma, a benign hamartomatous tumor of the cardiac embryonic myocyte, is the most common intrauterine cardiac tumor and accounts for 0.12% of prenatal fetal studies. Fetal cardiac rhabdomyomas increase in size during second and early third trimester and spontaneously regress postnatally. The clinical presentation is usually benign, however, compromise of the ventricular outflow tract leading to decreased cardiac output and fetal death have been reported. We present a case of large cardiac rhabdomyoma in a fetus that might have caused complete left ventricular outflow tract obstruction and development of hypoplastic left heart syndrome (HLHS) necessitating postnatal single ventricle palliation therapy. The clinical course and outcomes of prenatally diagnosed cardiac rhabdomyoma are reviewed and theories of the development of hypoplastic left heart syndrome are explored.

We report a case of left sinus of Valsalva aneurysm rupture into main pulmonary artery who underwent successful surgical correction. The preoperative diagnosis was facilitated by echocardiography, cardiac catheterization, and computed tomography. The benefits of surgery are sustained at 8 months on clinical and angiographic follow-up.

We describe a new technique for treatment of shunt thrombosis in infants with complex anatomical defects. A 2-month-old girl with Tetralogy of Fallot underwent placement of a modified Blalock-Taussig shunt (MBTS) at day of life (DOL) 6 with revision at DOL 20. Following this surgery, the patient became hypotensive and hypoxic with new evidence of lack of flow through the MBTS on echocardiography. Angiography showed an occluded MBTS and right pulmonary artery with patent distal branches with normal pulmonary venous return. Balloon angioplasty was attempted but failed to fully recanalize the right pulmonary artery (RPA) and MBTS. An AngioJet catheter was then passed through the shunt and RPA to perform rheolyticthrombectomy. Subsequent angiogram showed a widely patent RPA and MBTS. An echocardiogram at 1-month post-thrombectomy showed a widely patent MBTS with continuous flow seen entering both branch pulmonary arteries. The AngioJet system for thrombectomy provides a viable option for complex thrombus removal in patients refractory to other methods.

Goldenhar syndrome (GS) or oculo-auriculo-vertebral dysplasia (OAVD), involves a wide variety of organ systems. Cardiovascular anomalies are among the frequent malformations. The purpose of this report is to introduce a male case of a dizygotic twin pregnancy with GS and right circumflex aortic arch (RCAA), severe coarctation, hypoplastic aortic arch, aberrant right subclavian artery, vascular ring, bilateral renal artery stenosis, and mild Dandy-Walker syndrome. The embryology of RCAA and coarctation is revisited.

We report a case of type-A Coffin-Siris syndrome (CSS) with a unique constellation of congenital heart defects. A 17-year-old Indian boy was referred to our hospital for central cyanosis with features of right heart failure. The cardiac abnormalities included biventricular outflow tract obstruction, small atrial septal defect (ASD), subaortic ventricular septal defect, drainage of left superior venacava to left atrial appendage, and aortic arch anomaly. Patient underwent successful right ventricular infundibular resection, subaortic membrane resection, closure of atrial and ventricular septal defect, rerouting left superior vena cava to left pulmonary artery and aortic valve replacement.

A 22-year-old lady was referred to our institute for the management of pulmonary atresia with hypoplastic pulmonary arteries. Computed tomographic Angiography (CTA) showed right aortic arch with left brachicephalic artery as the first branch, which trifurcated into internal carotid, external carotid and subclavian artery high up in the neck at the level of third cervical vertebra. The left subclavian artery then travelled back caudally and entered into the arm after giving rise to a large collateral artery. This is the first ever-reported case of cervical origin of left subclavian artery (COLSA) in the literature. This anomaly can be explained by the absence of left fourth aortic arch with left subclavian artery arising from the left third aortic arch.

Echocardiography is an important first-line investigation for detection of anomalous origin of a coronary artery from the pulmonary artery (ALCAPA). We report two cases of ALCAPA that illustrate the importance of systematic performance of the echocardiogram, mindful of technical artifacts that may mislead the echocardiographer color Doppler imaging in diagnosis of this condition.

We report a novel technique 'the kissing catheter technique' for retrieval of a broken catheter fragment in a patient undergoing closure of a patent ductus arteriosus.

Scimitar syndrome is a rare congenital anomaly where the right pulmonary veins return to the inferior vena cava (IVC) just below the diaphragm. On chest X-ray (CXR), an IVC catheter will be in a bizarre location outside the heart if it inadvertently passes into the scimitar vein rather than into the right atrium.