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.

Utility of portal vein pulsatility fraction in patients undergoing corrective surgery for tetralogy of Fallot.

BACKGROUND: Right ventricle dysfunction is common after corrective surgery for tetralogy of Fallot and is associated with significant morbidity and mortality. We aimed to determine whether an increased portal vein pulsatility fraction (PVPF) was associated with worse clinical outcomes. METHODS: In a prospective, observational, single-centre study, PVPF and other commonly used parameters of right ventricle function were assessed in patients of all ages undergoing corrective surgery for tetralogy of Fallot intraoperatively, with transesophageal echocardiography, before and after bypass, and post-operatively, with transthoracic echocardiography, at days 1, 2, at extubation, and at ICU discharge. The correlation was tested between PVPF and mechanical ventilation duration, prolonged ICU stay, mortality, and right ventricle function. RESULTS: The study included 52 patients, and mortality was in 3 patients. PVPF measurement was feasible in 96% of the examinations. PVPF in the immediate post-operative period had sensitivity of 73.3% and a specificity of 74.3% in predicting the occurrence of the composite outcome of prolonged mechanical ventilation, ICU stay, or mortality. There was a moderate negative correlation of PVPF with right ventricle fractional area change and right ventricle global longitudinal strain (r = -0.577, p < 0.001 and r = 0.465, p < 0.001, respectively) and a strong positive correlation with abnormal hepatic vein waveform (rho = 0.749, p < 0.001). CONCLUSION: PVPF is an easily obtainable bedside parameter to assess right ventricular dysfunction and predict prolonged mechanical ventilation, prolonged ICU stay, and mortality.

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.

"Y" incision and rectangular patch enlargement of aortic annulus and valve reconstruction with autologous pericardium in congenital aortic stenosis.

Surgical aortic valve replacement in children who have undergone prior balloon or surgical valvuloplasty or both is a formidable challenge. The aortic annulus is small, there is no ideal prosthesis and lifelong anticoagulation is highly undesirable. A "Y" incision and rectangular patch enlargement of the aortic annulus introduced by Dr. Bo Yang in 2020 combined with aortic valve reconstruction introduced by Dr. Shigeyuki Ozaki in 2011, is feasible, as described in this case.

Isolated ventricular inversion with hypoplastic right ventricle and pulmonary stenosis in an adolescent with situs solitus: a rare combination.

Isolated ventricular inversion with situs solitus is a severe and rare congenital cardiac malformation characterised by an atrioventricular discordance but with ventriculo-arterial concordance. Here, we present the rare case of an adolescent with isolated ventricular inversion and hypoplasia of the left-sided morphological right ventricle and pulmonary stenosis, a first of its kind to be reported in the literature.

Rare branching pattern of the aortic arch in a neonate.

Here, we describe the case of a neonate who was found to have a complex pattern of branching on radiological imaging. The right common carotid artery, left common carotid artery and left subclavian artery were arising as a common single trunk from the arch of aorta with the additional presence of an aberrant right subclavian artery from the distal aortic arch on the left side and having a retroesophageal course to reach the right arm.

Implications of left atrial enlargement and patent foramen ovale creation on early surgical outcomes in patients with total anomalous pulmonary venous connection.

INTRODUCTION: In patients with total anomalous pulmonary venous connection (TAPVC), left atrium (LA) is small and suprasystemic pulmonary artery (PA) pressures may be present in some patients. In our study, we studied the relationship between surgical LA enlargement and patent foramen ovale (PFO) creation separately on the outcomes of patients with TAPVC. MATERIALS AND METHODS: Out of the 130 patients operated in our institute between January 2014 and December 2020, LA was enlarged in 60 patients. LA enlargement was done using a larger patch for atrial septal defect (ASD) closure. Thus, the LA volume was increased by shifting the patch towards the right atrium (RA). Suprasystemic or high PA pressures were present in 60 patients. In 33 patients, PFO was created. Early surgical outcomes were determined on the basis of vasoactive inotropic score (VIS), hours of ventilation, hours of inotropic support, intensive care unit (ICU) stay, and hospital stay. RESULT: Between the LA enlarged and nonenlarged group there was statistically significant less VIS score (18 [13-27.5] vs. 24 [18-30], p value .019), hours of ventilation (23 [16-46.5] vs. 26 [18-60], p value .039), hours of inotropic support (45.5 [30-72] vs. 55 [38-84], p value .038), and ICU stay (7 [5-9] vs. 8 [7-10] p value .0352) and statistically nonsignificant less hospital stay (11.5 [9-13] vs. 12 [9-14], p value .424). In patients with preoperative suprasystemic or high PA pressures, there was a statistically significant less VIS score (16 [11-23.5] vs. 18 [13-25], p value .044), hours of ventilation (20 [14-37] vs. 22 [18-39], p value .038), hours of inotropic support (34 [29.5-71] vs. 38 [30-78], p value .042), and hospital stay (9 [5-12] vs. 11 [9-14], p value .038) and statistically nonsignificant less ICU stay (7 [5.5-9] vs. 7 [6-9], p value .886) in the group with a PFO with respect to the other group in which no PFO was created. CONCLUSION: In patients with TAPVC, LA can be enlarged by using a large ASD patch and thus shifting the septum towards RA. Early surgical outcomes were improved with LA enlargement. In patients with suprasystemic or high PA pressures, leaving a PFO improved the postoperative outcomes.

Surgical management of aortopulmonary window with pulmonary artery dilatation causing left main coronary compression.

Aortopulmonary window is a rare congenital heart defect. Left main coronary artery extrinsic compression by an enlarged pulmonary artery is a rare complication and a potential cause for chest pain and sudden cardiac death in patients with pulmonary hypertension. Here, we present the case of a 14-year-old boy with a large aortopulmonary window who was planned for a device closure, but during the procedure, he developed ST-T segment changes while the device was being deployed, and hence the procedure was abandoned. The boy subsequently underwent a successful surgical closure thereafter.

Successful Surgical Management of a Rare Combination of Intrapericardial Teratoma and Thymoma in an Infant.

Primary cardiac tumors are rare, accounting for <0.2% of all childhood tumors. They can be diagnosed prenatally. Intrapericardial teratoma is a rare benign tumor that presents either due to the mass effect of the tumor or secondary pericardial effusion. Thymus is an important part of the immune system in the pediatric age group. Thymic lesions are rare causes of anterior mediastinal pathology. Their occurrence in children is rarer, nevertheless knowledge about their pathologies helps in clinching the correct diagnosis. We report a case of combined intrapericardial teratoma and thymoma that has not been reported previously in the literature.

Ebstein's anomaly of tricuspid valve with aortic stenosis and coarctation of aorta: Successful single-stage repair of a rare adult congenital heart disease.

Ebstein's anomaly of the tricuspid valve is infrequently associated with left heart anomalies. The association of aortic stenosis in Ebstein's anomaly has been reported to be extremely rare and the association of coarctation of aorta is even rarer especially in adults. The combination of all three of these lesions is virtually unknown without any references in literature. We report here an unusual case of Ebstein's anomaly of the tricuspid valve and severe aortic stenosis with coarctation of aorta in an adult who presented to us with exertional dyspnoea in the third decade and underwent a successful single-stage intracardiac repair.

Emergency valve preserving ascending aorta replacement following iatrogenic right coronary artery dissection: A life-saving procedure.

Iatrogenic aortocoronary dissection is a rare but potentially fatal complication of coronary catheterizations. Although the incidence is comparatively low, dissection often leads to procedure failure with increased risk of myocardial infarction and death. Iatrogenic aortocoronary dissection is principally caused by disruption of intima at the ostia of the right or left coronary artery during interventional procedures and appears as luminal filling defects, the persistence of contrast or intimal tear outside the coronary lumen. We present a case of right coronary artery dissection leading to type-A aortic dissection suffered during diagnostic coronary catheterization. This required emergency supracoronary replacement of the ascending aorta with an aortic interposition tube graft and venous grafts to coronary arteries.

Atretic right superior vena cava with left superior vena cava draining directly into left atrium with absent coronary sinus in an adult with partial atrioventricular canal defect and complete heart block.

The association of atretic right superior vena cava with persistent left superior vena cava draining directly into left atrium with absent coronary sinus in atrioventricular canal defect is virtually unknown in adults with no case reported so far. Though atretic right superior vena cava with persistent left superior vena cava is an extremely rare venous anomaly seen in congenital heart disease, it has important clinical implications in cardiac surgery and interventional cardiology. Atrial arrhythmias and right bundle branch block are common with advancing age in partial atrioventricualr canal defect but complete heart block has scarcely been reported in the medical literature.

Benefits of perioperative sildenafil therapy in children with a ventricular septal defect with pulmonary artery hypertension on early surgical outcomes.

OBJECTIVES: Pulmonary hypertension is a common association in children with nonrestrictive ventricular septal defect. It increases perioperative mortality and morbidity. Oral sildenafil is an effective pulmonary vasodilator. In this study, we assessed effects of perioperative oral sildenafil therapy on pulmonary artery pressure and early surgical outcomes. METHODS: This was a single centre, prospective randomized control study. Thirty children with nonrestrictive ventricular septal defects with pulmonary hypertension were divided into two groups. In the sildenafil group (n = 15, mean age 23.3 months), oral sildenafil was administered two weeks before surgery. In the control group (n = 15, mean age 36 months), preoperative sildenafil was not given. Sildenafil was continued postoperatively in both groups, provided the postoperative pulmonary artery pressure was over 50% of systemic pressure. RESULTS: There was no perioperative mortality, pulmonary hypertensive crisis and there were no intolerable side effects related to sildenafil in either group. Mean pulmonary artery pressure showed a reduction in both groups. Sildenafil group showed statistically significant improvement in duration of cardiopulmonary bypass (100.27 ± 21.09 min vs. 125.40 ± 26.83 min, p = .008), mechanical ventilation requirement (22.79 ± 17.13 h vs. 30.53 ± 13.05 h; p = .04), epinephrine requirement (22% patients vs. 48% patients; p = .03) and hospital stay (6.13 ± 1.40 days vs. 7.53 ± 1.92 days; p = .05). CONCLUSION: Oral Sildenafil therapy is an inexpensive and well-tolerated method for reducing pulmonary hypertension secondary to non-restrictive ventricular septal defect. It has noteworthy advantages regarding early surgical outcomes like reduced cardiopulmonary bypass time, improved mechanical ventilation time, lower inotrope requirement and shorter hospital stay if used preoperatively in select patient population.

Outcomes of emergency surgical intervention for complications in the cardiac catheterization laboratory.

Background: In today's era, cardiac catheterization procedures are becoming increasingly safe, but they are still fraught with complications. We aimed to study the outcomes of patients who underwent emergency surgical intervention for complications in the cardiac catheterization laboratory. Methods: A retrospective analysis of patients who required emergency surgical management following a complication in the cardiac catheterization laboratory in our institute from July 2017 to July 2022 was done. Result: A total of 57 patients out of a total of 52,326 patients (0.1%) were included. The average age of presentation was 10.4 years. Congenital heart disease (CHD) constituted the majority of the cases (28/57-49.1%), coronary artery disease (CAD) constituted 19.3% (11/57), and rheumatic heart disease (RHD) constituted 8.8% (5/57) of the cases. Apart from this, 22.80% patients (13/57) were grouped together in the miscellaneous group. In total, 76.9% (10/13) of these patients had pericardial effusion and they developed a right ventricular (RV) rent following an attempted pigtail drainage. Also, one patient each had a RV rent following an attempted permanent pacemaker implantation for heart block and an endocardial biopsy respectively. One patient had a left bronchial rupture following thoracic endovascular aortic repair (TEVAR) for descending thoracic aorta (DTA) aneurysm. Thirty-day mortality was 7% (4/57), and the mean time of shifting the patients from the catheterization laboratory to the operating room was 8.3 h. Conclusion: Cardiac catheterization procedures have become increasingly safe, but complications can still occur, for which the cardiac surgeon should be briefed in a Heart Team meeting before taking up such cases. Even though these complications form a small percentage, the cardiologist should exercise some caution in attempting cases which could have a relatively easier surgical correction.

Main pulmonary artery continuing as right pulmonary artery with trifurcation at its origin and aberrant origin of left pulmonary artery from ascending aorta in a case of tetralogy of Fallot.

Anomalous origin of the left pulmonary artery from the ascending aorta is a rare cardiac malformation described with tetralogy of Fallot. Trifurcation of right pulmonary artery after origin from the main pulmonary artery has not been reported in literature yet.

Early surgical outcomes of a modified infarct exclusion technique in acute post-myocardial infarction ventricular septal rupture: a single-centre experience.

Introduction: Operative mortality in an acute post-myocardial infarction (AMI) ventricular septal rupture (VSR) is high. In addition to ventricular dysfunction, friable myocardium adds to the technical difficulty of the operation. In a modified infarct exclusion technique, the right ventricle is left undisturbed and the free edge of the pericardial patch is incorporated in the sutures while closing the left ventriculotomy. This simplifies the procedure and decreases the chances of right ventricular dysfunction, any residual defect, and bleeding. Study design: A retrospective analysis of patients with VSR following AMI operated in our institute from January 2018 to June 2021 was done. Results: Over the last 3 years, 16 patients with AMI VSR were treated with a modified infarct exclusion technique. Eight patients presented in cardiogenic shock preoperatively and were put on intra-aortic balloon pump support. All patients could be weaned successfully from the cardiopulmonary bypass, no patient had any residual defect, and none of the patients required re-exploration for bleeding. Postoperatively, 5 patients died within the first week and 2 more patients subsequently died due to intractable arrhythmias over the next 30 days. Conclusions: In our centre, the mortality following repair of VSR after AMI was 43%. The modified infarct exclusion technique is a good technique with less chances of postoperative re-exploration and residual defect.

Trileaflet pulmonary valve reconstruction with the Ozaki technique in an adult patient of tetralogy of Fallot with absent pulmonary valve.

Pulmonary valve interventions are one of the most common cardiac interventions that are being performed in patients with a wide variety of congenital heart diseases, more so in adults with congenital heart disease. Despite the introduction of various different reconstructive techniques and conduits, the ideal option still remains elusive. Lack of growth and re-operation for conduit replacement remains an Achilles heel in such conduits. So, surgeons have constantly tried to evolve surgical techniques that would obviate their use and allow age-related growth. The Ozaki procedure has proven to be technically reproducible and have excellent mid-term results in the aortic position in adults. Extending the same principle for reconstruction of the pulmonary valve can prove to be a reasonable alternative. Supplementary Information: The online version contains supplementary material available at 10.1007/s12055-022-01469-1.