Baiju S. Dharan

Anomalous origin of left coronary artery from pulmonary artery in older children and adults: direct aortic implantation.

BACKGROUND Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) presents rarely in adulthood and is treated by different surgical techniques with varying results. This study was undertaken to evaluate the feasibility of reestablishment of a dual coronary system in older children and adults with emphasis on direct aortic implantation. METHODS From 2002 to 2010, 10 patients aged 10 to 51 years (median age, 24 years) underwent surgical correction of ALCAPA. Median weight was 44.5 kg. All the patients except 2 were symptomatic. Two patients were in atrial fibrillation. Eight patients had varying degrees of mitral regurgitation, which was severe in 2. In all patients, the left coronary artery was arising from the facing sinus of the pulmonary artery. Direct left main coronary implantation into the aorta was feasible in all patients. Mitral valve replacement was done in 4 patients. RESULTS At a median follow-up of 5.5 years there was no new angina or infarction. One patient died 3 months postsurgery due to intracerebral bleed. Echocardiography demonstrated normal antegrade flow in the transferred left coronary artery, with 2 patients having persistent moderate mitral regurgitation. Coronary angiography done on follow-up in one patient revealed a patent transferred left coronary artery with distal development of new atherosclerotic lesion. CONCLUSIONS Direct reimplantation of the ALCAPA is considered technically more difficult and hazardous in adults. With increased experience with coronary transfer technique, direct aortic implantation is feasible in adult cases. This provides a more physiologic correction and reestablishment of a dual coronary system with a better outcome.

Anomalous pulmonary venous connection to superior vena cava: Warden technique.

OBJECTIVE Repair of anomalous pulmonary venous connection (APVC) to superior vena cava (SVC) with internal pericardial patch may be complicated by the obstruction of the SVC or pulmonary veins and sinus node dysfunction. Moreover, the shrinkage of the pericardium may lead to late obstruction. Various techniques are practiced, which have reduced the incidence of obstruction to the venous drainage, but sinus node dysfunction continues to be an issue. We reviewed our experience with the Warden technique, predominantly in children, for various types of APVC to the SVC. METHODS Between 2006 and 2010, 32 patients with anomalous drainage of the pulmonary veins to SVC underwent repair by the Warden technique in our institute. The median age at operation was 4 years (range 3 months-34 years). Partial anomalous pulmonary venous connection (PAPVC) was present in 28 patients, while four patients had total anomalous pulmonary venous connection (TAPVC) to the SVC, where one or more pulmonary veins joined the SVC separately from the entry of the common chamber. Clinical data, echocardiography and operative details were collected from our database, retrospectively. RESULTS At a median follow-up of 24 months, there was no mortality. One patient had transient rhythm disturbance. On follow-up, it was revealed that all patients are in sinus rhythm with no evidence of systemic or pulmonary venous obstruction. CONCLUSION The Warden technique is a simple and effective surgical option, which should be preferred for patients with anomalous drainage of the pulmonary veins to SVC. Though arrhythmias are rare in the early follow-up, longer follow-up is required to rule out their late development.

Multiple Muscular Ventricular Septal Defects: Use of Fluorescein Dye to Identify Residual Defects

Multiple muscular ventricular septal defects remain a challenge for the congenital heart surgeon. The optimal strategy for an infant or neonate with multiple muscular ventricular septal defects is still unclear. Perioperative identification and secure closure of these defects pose significant difficulties. We describe a novel technique of using fluorescein dye to identify small muscular ventricular septal defects.

Anomalous origin of left coronary artery from right pulmonary artery in an infant with coarctation of the aorta

Anomalous origin of the left coronary artery from the right pulmonary artery is a very rare congenital anomaly, and its occurrence with coarctation of the aorta has been reported in very few patients. We report a neonate where the coronary anomaly was missed preoperatively and diagnosed after repair of the coarctation. The patient thereafter underwent ligation of the left anomalous coronary artery and had an uneventful convalescence.

Surgical repair of double-chambered right ventricle in adulthood

The aim of this study was to investigate a rare subset of adult patients with double-chambered right ventricle, pertaining to clinical presentation, rhythm disturbances, surgical approach, and results of surgical repair. A retrospective review of our records revealed that 14 adults had undergone surgical repair of double-chambered right ventricle from 1990 to 2010. Their median age was 25.5 years, and their median weight was 50 kg. Patients presented with various symptoms, the most common being dyspnea on exertion. The mean systolic pressure gradient across the obstruction was 69.8 ± 23.1 mm Hg. The most common associated anomaly was ventricular septal defect. Significant rhythm disturbances were present in 4 patients. Transatrial repair was undertaken in 5 patients, a combined transatrial and transpulmonary approach was used in 4, and a right ventriculotomy in 5. There was no mortality at a median follow-up of 5.1 years. A mild right ventricular outflow tract gradient was noted in 2 patients. Medical management was required for rhythm disturbances in 2 patients. Double-chambered right ventricle can present in adulthood, often with vague symptoms. The midterm results of surgical correction are satisfactory. Long-term follow-up is necessary to detect late arrhythmias.

Early and 1-year outcome and predictors of adverse outcome following monocusp pulmonary valve reconstruction for patients with tetralogy of Fallot: A prospective observational study

Background and Objectives: Repair of tetralogy of Fallot (TOF) with monocusp pulmonary valve reconstruction prevents pulmonary regurgitation (PR) for a variable period. Since postoperative outcome is governed by PR and right ventricular function, we sought to assess the severity of pulmonary regurgitation and right ventricular outflow (RVOT) gradient in the immediate postoperative period and at 1 year and attempted to identify the anatomical substrates responsible for adverse outcomes. Methods: The study included 30 patients. Transthoracic echocardiography was performed before surgery, within 5 days of surgery, and 1 year later. Presence and severity of PR, RVOT gradient, and residual branch pulmonary stenosis were assessed. Right ventricular and monocusp valve functions were studied. Results: Median age was 36.5 months (3-444 months). There were no deaths. Pulmonary regurgitation was mild in 18, moderate in 10, and severe in 2 patients immediately following surgery. At 1 year, 10 patients had severe PR and one had significant RVOT gradient. None of the variables like age, presence of supravalvar pulmonary branch stenosis, main pulmonary artery diameter, or mobility of monocusp valve was found to have any significant association with the progression of PR. McGoon index <1.5 showed a trend toward more PR, while patients with more residual RVOT gradient had lesser regurgitation. Conclusions: Repair of TOF with monocusp pulmonary valve reduces immediate postoperative PR. At 1 year, the monocusp valve underwent loss of function in a significant proportion and PR also progressed. This study could not identify any predictors of progression of PR, though patients with McGoon index <1.5 tended to have more PR while those with more outflow gradient had lesser PR.

Accuracy of a Chest X-Ray–Based Method for Predicting the Depth of Insertion of Endotracheal Tubes in Pediatric Patients Undergoing Cardiac Surgery

OBJECTIVES The incidence of endotracheal tube (ETT) malposition in children with various described methods is 15% to 30%. Chest x-ray (CXR) is the gold standard for confirming appropriate ETT position. The aim of this study was to measure the accuracy of a preoperative CXR-based method in determining depth of insertion of ETTs and to compare it with methods based on the intubation depth mark or formulae (age, height, and ETT internal diameter) in children undergoing cardiac surgery. DESIGN Prospective observational study. SETTING University-affiliated tertiary care hospital. PARTICIPANTS Sixty-six consecutive children scheduled for elective pediatric cardiac surgery. INTERVENTIONS None. MEASUREMENTS AND MAIN RESULTS The distance from carina to mid-trachea was measured for each child preoperatively on the CXR displayed as a computed radiography image in a picture archival and communications system computer. Following intubation, ETTs deliberately were pushed endobronchially and then pulled back to the carina; they were further withdrawn by the previously measured carina to mid-tracheal distance and secured. CXRs postoperatively were repeated to confirm ETT position. The ETT position was measured with other methods using the picture archival and communications system ruler on the postoperative CXR and compared with the CXR method. The proportion of appropriate ETT position with the CXR method was 98.5% (p≤0.001 v other methods). In children younger than 3 years, the appropriate proportion was 97.4%. CONCLUSION The appropriate positioning of ETTs in the trachea by the CXR method is superior to other methods.

Left ventricular regression after balloon atrial septostomy in d-transposition of the great arteries

OBJECTIVES Balloon atrial septostomy (BAS) is an effective palliative procedure in children with transposition of the great arteries and poor intercirculatory mixing. While the subpulmonary left ventricle (LV) is known to regress with time in these newborns due to the declining afterload, it has not been studied how it behaves following BAS and a consequent decrease in preload. The study was designed to examine the effects of BAS on the LV in infants with simple d-transposition of the great arteries by serial 2D echocardiographic monitoring. METHODS This was a prospective echocardiographic follow-up study of all consecutive children with simple d-transposition of the great arteries who underwent BAS for restrictive interatrial communication and oxygen saturation below 75% between January 2014 and June 2015. Left ventricular mass estimation was done by M-mode transthoracic echocardiography before balloon septostomy and serially on follow-up till surgery. RESULTS The median age of the 25 children studied was 4 days (1-95 days) when they underwent BAS. Twenty patients underwent arterial switch operation at a mean of 9 days from balloon septostomy. The mean baseline left ventricular mass was 47.9 g/m2, which decreased to 38.5, 36.2, 32.1, 32.4, 25.7 and 25.2 g/m2 on Days 1, 3, 6, 9, 12 and 15, respectively. The left ventricular mass decreased by 1.5 g/m2 every day during the first 2 weeks following balloon septostomy adjusted for the age of the child in days. Children who underwent BAS beyond 3 weeks of life had faster LV regression than those who underwent the procedure earlier (unstandardized regression coefficient β 0.892, P < 0.001). CONCLUSIONS BAS is associated with accelerated regression of the LV in infants with simple d-transposition of the great arteries in the first 2 weeks after the procedure. Regression of the LV is faster in children who underwent BAS after 3 weeks of age.

Tetralogy of Fallot With Concomitant Morgagni Hernia in Down Syndrome: The Value of Routine Chest Roentgenogram

Mfor 3% to 5% of congenital diaphragmatic hernias. Tetralogy of Fallot (TOF) is a common congenital cyanotic heart disease seen in patients with Down syndrome. However, TOF with associated MH is a very exceptional entity. A 2-year-old boy was diagnosed with congenital heart disease and Down syndrome after birth. A routine chest roentgenogram (posteroanterior view) showed a soft tissue mass with air fluid levels occupying the right cardiophrenic angle (Fig 1A), raising the suspicion of a diaphragmatic hernia. Computed tomography confirmed the diagnosis of congenital diaphragmatic hernia with colon as its contents (Fig 1B). Two-dimensional transesophageal echocardiography revealed TOF with a subaortic ventricular septal defect and right ventricular outflow tract obstruction. The child underwent an operation through a standard transsternal approach owing to congenital heart disease and MH. Intraoperatively, a bulky hernia sac was protruding between the diaphragm and the anterior rectus sheath into themediastinum through a large (9-cm 7-cm) defect in the anterior diaphragm (Fig 2A).

Submitral Aneurysm After Pulmonary Artery Banding—A Case Report

Submitral aneurysms are rare clinical entities occurring predominantly in young adults of African descent. A host of etiologies have been proposed for this entity. We present a unique case of submitral aneurysm which developed after pulmonary artery banding in a three-year-old girl with complex congenital heart disease. The aneurysmal sac was burrowing into the interatrial septum.