Arterial switch operation and aortic valve replacement for transposition of great arteries in adulthood: two cases from a tertiary care centre in India.

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.

Role of Transesophageal and Epicardial Echocardiography to Assess Surgical Repair in Double-Outlet Left Ventricle.

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.

Aneurysmal right ventricular outflow in an adult patient with unrepaired Tetralogy of Fallot and an absent pulmonary valve.

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.

Outcomes of repair of anomalous origin of pulmonary artery branch from aorta ascendens with autologous tissue: a rare condition revisited.

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.

Valentine on a crab-Type A interrupted aortic arch with type 2 aortopulmonary window.

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.

Bi-ventricular repair of double outlet left ventricle: Experience and review of the literature.

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.

RVOT pseudoaneurysm post biventricular repair for tetralogy of Fallot with single pulmonary artery.

Pseudoaneurysm of the right ventricular outflow tract (RVOT), post repair for tetralogy of Fallot (TOF), is a rare occurrence with few cases reported in literature. TOF with single pulmonary artery is in itself a rare occurrence. RVOT pseudoaneurysm in a case of TOF with single pulmonary artery has not been reported to the best of our knowledge. RVOT pseudoaneurysm is a catastrophic complication which has very few symptoms and has to be picked up early to avoid dire consequences. We have reported such a rare occurrence to highlight the importance of looking out for such complications in rare presentations where anatomy is altered. Supplementary information: The online version contains supplementary material available at 10.1007/s12055-023-01558-9.

Systolic anterior motion of mitral valve-a savior in transposition of great arteries?

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 the mitral valve with a preserved left ventricle (LV) and its management. Supplementary Information: The online version contains supplementary material available at 10.1007/s12055-023-01488-6.

A rare triad in left atrial myxoma.

Atrial myxomas are the most common cardiac tumors, rarely presenting with both systemic embolization and intracranial aneurysms. A 34-year-old woman presented with acute limb ischemia of both lower limbs and right upper limb and on evaluation was diagnosed with left atrial myxoma and intracranial aneurysms. She underwent embolectomy and intracranial aneurysm repair followed by successful excision of left atrial myxoma after 4 weeks. This case is reported for the rare association of myxoma with intracranial aneurysm along with systemic embolization which has not been reported in the literature.

Successful Management of a Very Unusual Coronary Pattern in Transposition of the Great Arteries.

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.

A Rare Case of Giant Atypical Patent Ductus Arteriosus.

A broad window-like patent ductus arteriosus (PDA) arising from the transverse aortic arch proximal to the left subclavian artery and without any associated cardiac anomalies in a one-year-old child is rare. This case is reported for the atypical location and unusually large size of the PDA.

A rare case of synovial sarcoma of diaphragm.

BACKGROUND: Primary diaphragmatic synovial sarcoma is a rare clinical entity with only few cases reported in the literature. It is found mainly in young adults, in the limbs. However, the name is a misnomer as it probably arises from primitive mesenchyme rather than articular surfaces of the joints. CASE: We report a case of 21-year-old patient with synovial sarcoma of the diaphragm, treated by complete surgical excision of the tumor with diaphragmatic reconstruction and confirmed on immunohistopathology. The peculiarity of this case stems from the atypical location of the tumor with complete surgical resection and thereby providing a better quality of life for the patient. CONCLUSION: Synovial sarcoma of the diaphragm is a rare malignancy and more data and research is needed for defining the best management for this tumor.

Nuclear cardiology for a cardiothoracic surgeon.

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.

Congenital unilateral pulmonary vein atresia: A rare disease with successful outcome in a child.

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.

Kissing Lesion of Mitral Valve - A Case Report.

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.

Mitral kissing lesion with anterior mitral leaflet aneurysm in a child.

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.

Midterm results of homografts in pulmonary position: a retrospective single-center study.

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.

An unusual cause of heparin resistance - A case report.

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.

Congenital long QT syndrome and patent ductus arteriosus: A rare surgical scenario.

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.

Correction to: Midterm results of homografts in pulmonary position: a retrospective single-center study.

[This corrects the article DOI: 10.1007/s12055-020-01065-1.].