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Coronary artery anatomy is the key to a successful arterial switch operation in transposition of the great arteries. We came across an unusual coronary pattern in a child with transposition in which the three major coronary arteries were seen arising from all three aortic sinuses. This coronary pattern is the first of its kind in transposition, and this case report emphasizes the difficulty in translocating such a rare coronary pattern while performing an arterial switch operation.

Unilateral pulmonary vein atresia is a rare congenital anomaly that may occur in association with other congenital heart diseases. We report a rare case of left-sided pulmonary vein atresia in a 2-year-old child who presented with recurrent hemoptysis and was managed surgically with left pneumonectomy.

A double-outlet left ventricle (DOLV) is a congenital cardiac anomaly that rarely is encountered. This case report demonstrates the echocardiographic features of DOLV in the form of the pulmonary artery arising completely from the left ventricle with D-looped ventricles, especially when evaluated by intraoperative transesophageal echocardiography (TEE), along with the correlation of characteristic features with cardiac computerized tomography. The features pertinent to the differentiation of DOLV from double-outlet right ventricle and congenitally corrected transposition of the great arteries by echocardiography have been described. To the authors' knowledge, this was the first report of intraoperative TEE in the case of DOLV. In addition, the use of intraoperative echocardiography to rule out coronary compression as a cause for post-repair ventricular tachycardia, by use of TEE to rule out ventricular dysfunction and regional wall motion abnormalities, as well as epicardial echocardiography to demonstrate normal coronary blood flow, has been reported.

The surgical treatment of transposition of the great arteries, ventricular septal defect, and significant left ventricular outflow tract obstruction continues to evolve. The survival of an unrepaired transposition of the great arteries into late adulthood is a rarity. Even when large intracardiac shunts are present, it remains a lethal cyanotic CHD if it is not surgically corrected soon after birth. We present our experience of two cases, both of whom underwent a single-stage arterial switch operation and an aortic valve replacement for this defect.

Left atrial myxoma is the most common variant of cardiac tumour, rarely presenting with systemic embolization involving 3 limbs and intra cranial bleed simultaneously. Our case aims to discuss appropriate management strategy in such cases. This case presents a 34 year old lady with acute limb ischemia both lower limb and right upper limb with intracranial bleed with no signs of congestive heart failure diagnosed with LA Myxoma on preoperative routine 2d ECHO cardiography. She underwent embolectomy of right brachial artery and bilateral femoral artery, common iliac artery and abdominal aorta with excision of LA Myxoma planned for 4 weeks later. In patients presenting with acute limb ischemia and intracranial bleed, the definitive surgery of LA myxoma excision should be postponed for 4 weeks to prevent risk of systemic heparinisation causing worsening of neurological functions while acute limb ischemia should be addressed immediately.

Background: Anomalous origin of pulmonary artery branch from ascending aorta (APA) in the presence of two separate semilunar valves is an uncommon entity necessitating early diagnosis and surgery to prevent development of irreversible pulmonary vascular disease. We evaluated our experience with the technique and outcome of 11 patients with this condition. Methods: Between January 2000 and December 2019, 11 patients were diagnosed with APA. Echocardiographic data were collected from the records, including the site of origin of the anomalous pulmonary artery, additional defects, pulmonary artery pressures, and biventricular function. Intraoperative charts were reviewed for the details of the surgical procedure and cardiopulmonary bypass. Post-operative data included survival, ventilatory support, and duration of hospital stay. After discharge, children were reviewed at 1 month, 3 months, 6 months, and then at yearly intervals. Results: Of the 11 patients, females were more than males (7:4) with a median age of 6 months (15 days–28 years) and median weight of 5.7 kg (1.8–40 kg). Nine patients underwent direct re-implantation of anomalous pulmonary artery branch to main pulmonary artery. The survival rate was 88.8% in our series. On follow-up, no re-operations or re-interventions were required and all surgically corrected patients were in stable clinical condition. Conclusion: Early- and midterm outcomes of children who underwent surgery for APA is convincing. Early direct re-implantation of the anomalous branch pulmonary artery to main pulmonary artery without any graft material is the optimal surgical strategy for these patients.

BACKGROUND Absent pulmonary valve (PV) is a rare and severe variant seen in only 3% to 6% of patients with tetralogy of Fallot (TOF). Fetuses with this combined condition who survive through birth typically need intervention in infancy or early childhood because of respiratory distress, heart failure, or failure to thrive. DISCUSSION Here, we describe the case of a 37 year old gentleman who presented with a diagnosis of TOF with absent PV, hugely dilated right and left pulmonary arteries and an aneurysmal right ventricular outflow. CONCLUSION TOF with absent PV surviving into adulthood is a rarity. An aneurysmal right ventricular outflow tract in an unrepaired case of TOF has not been described before. Massively dilated left and right pulmonary arteries as in this case can cause significant and even life-threatening airway compromise, thus necessitating early surgical repair.

The arterial switch operation (ASO) is the procedure of choice for the management of d-transposition of the great arteries (TGA). However, the surgical management of infants older than 6 weeks with TGA and intact ventricular septum (IVS) remains contentious. We report a case of late presenting TGA, IVS with Systolic anterior motion(SAM) of mitral valve with preserved left ventricle(LV) and its management.

DISCUSSION – Left brachiocephalic vein is 6 cm long; it begins behind the sternal end of the clavicle, anterior to the cervical pleura by union of the left internal jugular vein and the left subclavian vein. It descends obliquely to the right, behind the upper half of the manubrium sterni, up to the sternal end of the first right costal cartilage, uniting here with right brachiocephalic vein to form SVC. The aortic arch is inferior to this vein. The left retro-aortic bracheocephalic vein is rare entity which was first described by Kerschner.[(1)](#ref-0001) Incidence of left retro-aortic bracheocephalic vein is noted between 0.2 to 1%.[(2)](#ref-0002) Aorto-pulmonary window is a rare cardiac condition, first described by Eliotson in 1830[(3)](#ref-0003) with an incidence of 0.2% - 0.3% of all congenital cardiac lesions.[(4,5)](#ref-0004) Three standard approaches for surgical closure of aorto-pulmonary window have been described in literature include: trans-aortic approach, trans-window approach, trans-pulmonary approach. Trans-window approach is also known as sandwich repair of aorto-pulmonary window.[(6)](#ref-0006) The left retro-aortic brachiocephalic vein with tetralogy of fallot and coarctation of aorta are noted. The left retro-aortic brachiocephalic vein with aorto-pulmonary window is very rare case and has not been reported previously. In our case the patient was admitted for AP window closure after complete pre-operative evaluation. Cardiac computed tomography reported 2.2cm aorto-pulmonary window with the left retro-aortic brachiocephalic vein. After obtaining parental consent for surgery, median sternotomy was performed. Thymus was excised and pericardium was opened longitudinally. By careful dissection, the left brachiocephalic vein was identified behind the distal ascending aorta adjacent to the AP window. Aorta was dissected off the brachiocephalic vein meticulously. Due to left retro-aortic brachiocephalic vein, aortic cannulation had to be done more caudally to avoid obstruction of vein. Aorta was cannulated and clamped without injury to the brachiocephalic vein. Attention was paid to avoid injury of the brachiocephalic vein during encircling the SVC for snaring. Aorto-pulmonary window was closed with PTFE patch through trans-window approach under mild hypothermia.[(6)](#ref-0006) Clinical implication of retro-aortic brachiocephalic vein is very important. More caudal cannulation of SVC is required in left brachiocephalic vein; but in AP window setting, this is more difficult as work space for closure of the defect is also required. Injury may occur to the vein during clamping of aorta. Left retro-aortic brachiocephalic vein may cause technical difficulty during central venous line placement through left arm approach. CONCLUSION- Being the rarest combination of left retro-aortic brachiocephalic vein and aorto-pulmonary window, certain things have to be taken care of like clamping of aorta, SVC cannulation, central vein catheter insertion, snaring of SVC. Pre-operative CT scan is also important to avoid intra-operative surprises.

OBJECTIVE: Valved conduits play a pivotal role in the right ventricular outflow tract (RVOT) reconstruction in patients with congenital heart disease (CHD), and valved homografts have become the most commonly used conduits in pediatric cardiac surgery. This study aimed to assess the midterm performance of aortic and pulmonary homografts used in pulmonary position in patients with CHD. METHODS: Ninety-eight patients underwent surgical RVOT reconstruction with a homograft from January 2012 to December 2017. We collected all patient details from the institute patient record system. Echocardiographic data were obtained from the records. Postoperative data included survival and follow-up echocardiography. The primary endpoints of the study were homograft failure, homograft dysfunction, and freedom from re-intervention. RESULTS: We observed allograft failure in two patients (2%), one each due to pseudo-aneurysm formation and infective endocarditis. There were 8 early deaths (8/98, 8.2%) and 9 late deaths (9/98, 9.2%). The mean survival time was 6.1 years, and the postoperative survival rate at 1 and 5 years was 89.73% and 82.65%, respectively. Homograft regurgitation and homograft dysfunction were higher in the pulmonary homograft group. However, the re-intervention rates were more in the aortic homograft (24.24%) group due to the higher incidence of calcification associated with them. CONCLUSION: Homografts used for RVOT reconstruction provide excellent intermediate-term outcomes, irrespective of the type of homograft used. Pulmonary homografts are more durable than aortic homografts and provide satisfactory results even in infants.

Pseudoaneurysm of the right ventricular outflow tract(RVOT) is an uncommon yet catastrophic complication after intracardiac repair of Tetralogy of Fallot(TOF). We describe a patient diagnosed with RVOT pseudoaneurysm in the immediate postoperative period after complete repair for TOF with single pulmonary artery. The pseudoaneurysm was repaired successfully. This case is reported to emphasise the importance of a high degree of suspicion of this rare entity in these patients for its early diagnosis and management.

Aortic valve endocarditis can lead to secondary involvement of aorto mitral curtain and the adjacent anterior mitral leaflet (AML). The secondary damage to AML is often caused by the infected diastolic jet of aortic regurgitation hitting the ventricular surface of the anterior mitral leaflet, or by the pronounced bacterial vegetation that prolapses from the aortic valve into the left ventricular outflow tract. This is called “kissing lesion”. We describe a case of infective endocarditis of aortic valve in a 13-year-old child causing secondary mitral valve involvement with AML perforation and aneurysm formation.

Cardiac surgeons are commonly faced with issues regarding the balance between the potential risk and the potential benefit of a surgical procedure. Nuclear cardiology procedures such as single-photon emission computed tomography and positron emission tomography provide the surgeon with objective information that augments standard clinical and angiographic assessments related to the diagnosis, prognosis, and potential benefit from any intervention. Myocardial perfusion is imaged with the use of radiopharmaceuticals that accumulate rapidly in the myocardium in proportion to the myocardial blood flow. Radionuclide lung imaging most commonly involves the demonstration of pulmonary perfusion using technetium-99 m macro aggregate albumin (Tc-99 m MAA), as well as the assessment of ventilation using inspired inert gas, usually xenon, or Tc-99 m-labelled aerosols. Nuclear cardiology is extensively used as a part of the work-up of ischemic heart disease and cardiac failure in deciding the optimal therapeutic strategy with its ability to predict the severity of the disease. It has also proved extremely useful in the management of congenital heart disease and the diagnosis of pulmonary embolism, among many other applications. Myocardial perfusion imaging is a basic adjunct to the noninvasive assessment of patients with stable angina, baseline electrocardiogram (ECG) abnormalities, post-revascularisation assessment, and heart failure. This review article covers a summary of basic concepts of nuclear cardiology about what a cardiac surgeon should be aware of. To many, it is just a perfusion test, but the versatility, reliability, and future of the technology are without a doubt.

Sustained ventricular tachycardia (VT) in the early postoperative period following intracardiac repair for tetralogy of Fallot is rare. In stable VT, amiodarone forms the mainstay of management. However, where amiodarone and other antiarrhythmic drugs are contraindicated, suppressive overdrive atrial pacing can be used as a safe and efficient alternative to maintain cardiac output. We present a case of 1-year 5-month-old child who developed VT with low cardiac output syndrome with deranged hepatic function, who was managed efficiently using suppressive atrial pacing to ameliorate the effects of sustained VT.

Aortic valve endocarditis can lead to secondary involvement of aorto-mitral curtain and the adjacent anterior mitral leaflet (AML). The secondary damage to AML is often caused by the infected jet of aortic regurgitation hitting the ventricular surface of the mitral leaflet, or by the pronounced bacterial vegetation that prolapses from the aortic valve into the left ventricular outflow tract. This is called ‘kissing lesion’. We describe a patient with infective endocarditis of the aortic valve causing perforation of both noncoronary cusp of aortic valve and the AML, which is rare.

Echinococcosis is a rare infectious disease in human being that occurs by the larval stages of taeniid cestodes of the genus Echinococcus. Human cystic echinococcosis is the most common presentation. The liver is the most common site of echinococcal cyst, followed by the lungs. The symptoms of lung infestation lead to sudden onset of chest pain, cough, fever, and hemoptysis after a cyst rupture. The diagnosis is confirmed by radiology supplemented with serology. Chest X-ray and computer tomography of chest is the principal investigation for pulmonary hydatid cyst (PHC). The treatment of PHCs is either pharmacotherapy and/or surgery. Surgical intervention is the most preferred treatment of choice; pharmacotherapy is useful in selected patients. Pharmacotherapy includes oral administration of benzimidazoles group of drugs like mebendazole or albendazole.

Valved conduits play a pivotal role in the right ventricular outflow tract (RVOT) reconstruction in patients with congenital heart disease (CHD), and valved homografts have become the most commonly used conduits in pediatric cardiac surgery. This study aimed to assess the midterm performance of aortic and pulmonary homografts used in pulmonary position in patients with CHD.Ninety-eight patients underwent surgical RVOT reconstruction with a homograft from January 2012 to December 2017. We collected all patient details from the institute patient record system. Echocardiographic data were obtained from the records. Postoperative data included survival and follow-up echocardiography. The primary endpoints of the study were homograft failure, homograft dysfunction, and freedom from re-intervention.We observed allograft failure in two patients (2%), one each due to pseudo-aneurysm formation and infective endocarditis. There were 8 early deaths (8/98, 8.2%) and 9 late deaths (9/98, 9.2%). The mean survival time was 6.1 years, and the postoperative survival rate at 1 and 5 years was 89.73% and 82.65%, respectively. Homograft regurgitation and homograft dysfunction were higher in the pulmonary homograft group. However, the re-intervention rates were more in the aortic homograft (24.24%) group due to the higher incidence of calcification associated with them.Homografts used for RVOT reconstruction provide excellent intermediate-term outcomes, irrespective of the type of homograft used. Pulmonary homografts are more durable than aortic homografts and provide satisfactory results even in infants.

Background Double-outlet left ventricle (DOLV) is a rare congenital cardiac anomaly. The aorta and the main pulmonary arterial trunk arises predominantly from the left ventricle and is associated with a malaligned ventricular septal defect, various degrees of hypoplasia of the right ventricle, and presence, or absence of pulmonary stenosis. Bi-ventricular repair is the preferred treatment option whenever possible. Multiple techniques for bi-ventricular repair have been described. The best option for DOLV correction is by translocating the pulmonary root from the left ventricle to the right ventricle. In this series, we report four patients who underwent biventricular repair of DOLV with excellent outcomes. Methods This is a retrospective study of four patients who underwent surgical correction of DOLV between January 2014 and December 2018. We collected all patient details from the institute patient record system. Echocardiographic data were obtained from the records. Intraoperative charts were reviewed for further information on the surgical procedure and cardiopulmonary bypass. Postoperative data included survival, functional status, and followup echocardiography. Results Of the four children, three underwent pulmonary root translocation, and one child underwent a Reparation al etage Ventriculaire (REV) procedure. There was no mortality in our series, and all children are in a stable clinical condition in the recent follow-up, and no re-operations or interventions were required following primary surgical correction. Conclusion DOLV is anatomically and surgically a challenging subset. Pulmonary root translocation in this anatomy is technically challenging but safe and superior option when compared to other alternative surgical procedures. Pulmonary root translocation can be performed with excellent results, even in infants.

Double-outlet left ventricle (DOLV) is a rare congenital cardiac anomaly. The aorta and the main pulmonary arterial trunk arises predominantly from the left ventricle(LV) and is associated with a malaligned ventricular septal defect(VSD), various degrees of hypoplasia of the right ventricle, and presence or absence of pulmonary stenosis. Bi-ventricular repair is the preferred treatment option whenever possible. Various techniques for bi-ventricular repair have been described. The best option for DOLV correction is by translocating the pulmonary root to the right ventricle(RV)[1]. In this series, we report four patients who underwent biventricular repair of DOLV in our institute with excellent outcomes. All patient details were collected from the institute patient record system. Echocardiographic data were obtained from the records. Intraoperative charts were reviewed for further information on the surgical procedure and cardiopulmonary bypass. Postoperative data included survival, functional status and followup echocardiography. Of the four children, three underwent pulmonary root translocation and one child underwent Reparation al etage Ventriculaire(REV) procedure. There was no mortality and all children are in stable clinical condition in the recent follow-up and no re-operations or interventions were required following primary surgical correction. Thus DOLV is anatomically and surgically a challenging subset. Pulmonary root translocation in this anatomy is technically challenging but safe and superior option when compared to other alternative surgical procedures and it can be performed with excellent results, even in infants.

Background: Functional tricuspid regurgitation (FTR) is often neglected. No clear consensus has been reached on superiority of preoperative transthoracic (TTE) or intraoperative transesophageal echocardiography (TEE) in assessment of tricuspid regurgitation (TR) in rheumatic heart disease. There is still debate when to consider for surgical repair of FTR and its benefits. Methods: A total of 50 patients with rheumatic heart disease involving mitral, aortic or both valves with moderate and severe TR were included in the study. TR was assessed using preoperative TTE and intraoperative TEE. 36 out of 50 patients underwent tricuspid valve repair. Two patients died in postoperative period. Results of tricuspid valve repair were studied in 34 patients after six months of surgery. Results: TTE was superior in assessing tricuspid annulus size, central jet area, vena contracta, right atrium size and right ventricle systolic pressure. Patients undergoing tricuspid valve repair had better quality of life with significant improvement in grade of dyspnea, angina, pedal edema and hepatomegaly. The annulus size, jet velocity and right ventricle systolic pressure were significantly lower in patients who underwent tricuspid valve repair. Tricuspid valve should be repaired in all cases of moderate to severe TR with significant annular dilation (>38 mm). Conclusions: FTR is better assessed by TTE compared to intraoperative TEE. Concomitant TR correction (even in moderate degree of TR with tricuspid annulus ≥ 38 mm) should be strongly considered at the time of left side valve surgery resulting in better quality of life. Keywords: Functional tricuspid regurgitation, Preoperative transthoracic echocardiography, Intraoperative transesophageal echocardiography, Tricuspid ring annuloplasty, De Vegas annuloplasty

The ductus arteriosus is rarely used for reconstruction of the aortic arch in neonates. A 2-week-old neonate presented with type A interrupted aortic arch with obstructive supracardiac total anomalous pulmonary venous connection. The ductus arteriosus was used for reconstruction of the aortic arch, and repair of the total anomalous pulmonary venous connection was carried out simultaneously. After 1.5 years of follow-up, there was no ductal narrowing, no gradient in the arch, and a mild gradient across the anastomosis between the common chamber and the left atrium.

The most common cardiac tumour in the pediatric age group is rhabdomyoma. These are usually located in the ventricles, either in the ventricular septum or free wall. Cardiac tumours in early infancy may lead to severely compromised blood flow due to inflow or outflow tract obstruction. The diagnosis of cardiac rhabdomyoma can be established by transthoracic echocardiography (TTE). Rhabdomyomas have a natural history of spontaneous regression; surgical intervention is reserved for patients with symptoms of severe obstruction or hemodynamic instability. In this study, a case of two-year old child who presented with failure to thrive and underwent excision of pedunculated mass from the right ventricular outflow tract was reported.

A 10-day-old infant was evaluated for heart failure and differential cyanosis. Type A interrupted aortic arch with duct-dependent lower body circulation was identified. There was associated type 2 aortopulmonary window which led to a "Valentine on a crab" appearance on echocardiography. Pattern recognition in imaging is useful for early identification of anomalies and for triaging appropriate evaluation and management.

Chronic constrictive pericarditis (CCP) is the most common pericardial pathology. CCP complicating with intracardiac thrombus is a rare entity, the detection of thrombus preoperatively is life-saving in avoiding the risk of pulmonary thromboembolism during anterior pericardiectomy. Transesophageal echocardiography has been shown to have better sensitivity in detecting atrial thrombus than transthoracic echocardiography. Surgical removal of right atrial thrombus under cardiopulmonary bypass should be considered.

Congenital long QT syndrome (LQTS) is a rare cardiac condition characterized by abnormality of either sodium or potassium ion channels resulting in prolongation of QT interval and thereby predisposing to life-threatening arrhythmia. Once the syndrome is diagnosed, measures should be taken to avoid sudden cardiac death. We present a rare case of LQTS associated with patent ductus arteriosus in a child, and a unique approach was used in managing both conditions.

Primary intestinal lymphangiectasia (PIL) is a rare disorder characterized by dilated intestinal lacteals that result in leakage of excessive serum proteins and lymphocytes into the gastrointestinal (GI) tract culminating in protein-losing enteropathy. The GI loss of protein and possible antithrombin III (AT-III) loss creates a prothrombotic environment. The surgical management of congenital heart disease (CHD) in presence of PIL can present with altered heparin response and can impose problems in instituting cardiopulmonary bypass (CPB). We report a case of surgical closure of ventricular septal defect with PIL with altered heparin response. Such an association of PIL with altered heparin response in CHD has not been reported in literature.

Aortic root abscess or aortic annular abscess is a serious complication of aortic valve endocarditis. It usually occurs in patients with prosthetic aortic valve and coagulase-negative staphylococcal endocarditis. However, we came across a young individual of bicuspid aortic valve with no features of Infective Endocarditis (IE) with severe aortic valve regurgitation with sterile aortic root abscess and severe mitral valve regurgitation. He underwent repair of aortic root followed by replacement of both the valves with mechanical valve prosthesis.