Ruptured sinus of valsalva presenting late following repair of tetralogy of fallot.

Rupture of sinus of valsalva following repair of tetralogy of fallot (TOF) is very rare. It should be suspected as a cause of recurrent or prolonged pleural effusion and congestive cardiac failure in patients who have undergone repair of TOF. We report one such patient.

An alternative technique for intracardiac exposure during transatrial repair of tetralogy of fallot.

The commonly used technique to facilitate intracardiac exposure during transatrial repair of tetralogy of fallot involves considerable retraction of the tricuspid valve using retractors. We describe an alternative surgical technique in which it is possible to dispense away with the retractors. The advantages of such a technique are discussed.

Unilateral unidirectional superior cavopulmonary anastomosis in a patient with bilateral superior caval veins and atretic left pulmonary artery.

A unidirectional superior cavopulmonary anastomosis was performed on the right side in a patient with a functionally univentricular heart, atresia of main and left pulmonary artery, bilateral superior caval veins, and a patent arterial duct in the right pulmonary artery. Anastomosis of the left superior caval vein to the right superior caval vein created a neo-innominate vein without using prosthetic material.

Aortopulmonary window: Morphology, diagnosis, and long-term results.

OBJECTIVE: Aortopulmonary window (APW) is a rare congenital heart defect. We reviewed our experience with this condition over the last two decades. METHODS: Between September 1993 and December 2013, 62 patients underwent surgery for APW. Depending on the associated lesions, they were divided into two groups: Simple (Group 1) or complex (Group 2). In the complex group, six patients had a ventricular septal defect, five patients had interrupted aortic arch, three patients had tetralogy of Fallot, two patients had double outlet right ventricle, and one patient had the right pulmonary artery arising from the ascending aorta. RESULTS: Mean age at repair was 21.6 ± 32.02 months (median = 6, range 0.1-144 months). By preoperative echocardiographic assessment 27 out of 62 patients had severe pulmonary artery hypertension (52% of the cohort). Patch repair of APW was performed using the sandwich method (transwindow) (n = 27; 43.5%), transaortic (n = 18; 29%), and transpulmonary artery (n = 5; 8.1%) approaches; 10 patients (16.1%) underwent double ligation and two (3.2%) underwent division and suturing. Overall hospital mortality in group 1 was 6.97% (3/43) and in group 2 it was 21% (4/19), p = 0.085. Mean hospital stay in group 1 was 6.9 ± 2.4 days (median = 7 days) and in group 2 was 12 ± 6.1 days (median = 13 days), p = 0.0001. Follow-up in group 1 was 1.6-9.8 years (median = 6 years); in group 2, it was 1.8-8.9 years (median = 6.5 years). There were no late deaths. Two patients needed reintervention for distortion of the right pulmonary artery origin. All patients were in New York Heart Association Class I/II at last follow up. CONCLUSION: There are multiple acceptable surgical strategies for the treatment of aortopulmonary window. Despite a relatively advanced age and substantial number of patients with severe pulmonary hypertension the outcomes can still be good. Associated anomalies complicate the repair. Patients in the complex group had a protracted hospital course and a higher early mortality but similar late survival.

Bidirectional superior cavopulmonary anastomosis with or without cardiopulmonary bypass: A randomized study.

OBJECTIVES: This study aims to compare the bidirectional superior cavopulmonary anastomosis (BDG) with or without cardiopulmonary bypass (CPB). METHODS: 100 patients undergoing BDG were randomized into two groups: Off-CPB or on-CPB groups. All patients underwent near-infrared spectrophotometry (NIRS) and bispectral index (BIS) monitoring and pre- and postoperative serum 100 beta protein measurements (Sß100) and neuro-cognitive evaluation. Postoperative intensive care unit (ICU) parameters were also studied. RESULTS: The median age of patients in the on-CPB and off-CPB group were 42 and 48 months, respectively (p = 0.11). Median weights in the on-CPB group and off-CPB group were 13.5 (5-50) kg and 15 (7-36) kg, respectively (p = 0.927). There was a significant rise in superior vena cava (SVC) pressure on SVC clamping in the off-CPB group (23.12 ± 6.84 vs 2.98 ± 2.22 mmHg) on-CPB group (p < 0.001). There was a significant fall in NIRS and BIS values from baseline in the off-CPB group during the anastomosis but there was no statistically significant change in serum Sß100from pre-clamp to post-clamp in either group. Inotropic support, duration of ventilation, ICU stay, and hospital stay were significantly less in the off-CPB group (p < 0.001). Assessment of Social Adaptive Functioning revealed no adverse sequelae. There were significant cost savings if surgery was performed off-CPB (p < 0.001). CONCLUSION: Off CPB-BDG is an economical and safe procedure. Duration of inotropic and mechanical ventilatory support, ICU, and hospital stay is significantly less. We did not observe any early adverse neurologic sequelae in patients undergoing off-CPB BDG.

Bidirectional Glenn on cardiopulmonary bypass: A comparison of three techniques.

OBJECTIVE: To analyze the intraoperative and early results of the bidirectional Glenn (BDG) procedure performed on cardiopulmonary bypass (CPB) using three different techniques. METHODS: Between September 2013 and June 2015, 75 consecutive patients (mean age 42 ± 34.4 months) undergoing BDG were randomly assigned to either technique I: open anastomosis or technique II: superior vena cava (SVC) cannulation or technique III: intermittent SVC clamping. We monitored the cerebral near infrared spectrophotometry (NIRS), SVC pressure, CPB time, intensive care unit (ICU) stay, and neurocognitive function. RESULTS: Patients in technique III had abnormal lower NIRS values during the procedure (57 ± 7.4) compared to techniques I and II (64 ± 7.5 and 61 ± 8.0, P = 0.01). Postoperative SVC pressure in technique III was higher than other two groups (17.6 ± 3.7 mmHg vs. 14.2 ± 3.5 mmHg and 15.3 ± 2.0 mmHg in techniques I and II, respectively = 0.0008). CPB time was highest in technique II (44 ± 18 min) compared to techniques I and III (29 ± 14 min and 38 ± 16 min, P = 0.006), respectively. ICU stay was longer in technique III (30 ± 15 h) compared to the other two techniques (22 ± 8.5 h and 27 ± 8.3 h in techniques I and II, respectively = 0.04). No patient experienced significant neurocognitive dysfunction. CONCLUSION: All techniques of BDG provided acceptable results. The open technique was faster and its use in smaller children merits consideration. The technique of intermittent clamping should be used as a last resort.

Resection of subaortic membrane for discrete subaortic stenosis.

BACKGROUND: We reviewed the long-term results of surgery for discrete subaortic membrane (SubAM) from a single institute. METHODS: A retrospective review of medical records of all patients (n = 146) who underwent resection of a SubAM for discrete subaortic stenosis between 1990 and 2015 at the All India Institute of Medical Sciences, New Delhi, India was undertaken. RESULTS: Median age at surgery was 9.0 years (9 months-47 years). There was one early death. Preoperative peak left ventricular outflow tract (LVOT) Doppler gradient was 83.4 ± 26.2 mmHg (range: 34-169 mmHg). On preoperative echocardiography, aortic regurgitation (AR) was absent in 69 (47.3%), mild in 35 (24%), moderate in 30 (20.5%), and severe in 12 (8.2%). After surgery, the LVOT gradient was reduced to 15.1 ± 6.2 mmHg (P < 0.001). Fourteen patients (9.6%) who had residual/recurrent significant gradients are currently being followed-up or awaiting surgery. There was improvement in AR for operated patients with freedom from AR of 92.6 ± 0.03% at 15 years. Kaplan-Meier survival at 25 years was 93.0 ± 3.9% (95% confidence interval: 79.6, 97.7). Freedom from re-operation at 25 years was 96.9 ± 1.8%. CONCLUSIONS: Long-term results of surgery for discrete SubAM are good. Resection of the membrane along with septal myectomy decreases the risk of recurrence.

Arterial switch operation in patients with transposition and a left-sided aorta.

OBJECTIVES: Arterial switch operation is the treatment of choice in infants with transposed arterial trunks. It is technically challenging to perform in patients having usual atrial arrangement and concordant atrioventricular connections but having a left-sided aorta. Correction in this setting requires surgical expertise and precision. Here we review our experience with such patients. METHODS: Between January, 2002 and October, 2013, the arterial switch operation was performed in 20 patients in the combination emphasised above. Patient records were analysed in detail for coronary arterial patterns, and for the techniques used for transfer of the coronary arteries and reconstruction of the great arteries. Outcomes were recorded in terms of in-hospital survival and left ventricular function at the most recent follow-up. RESULTS: All patients survived the procedure. Ages ranged from 3 days to 18 months, with a median of 75 days; the weight of the patients ranged from 3 to 8.8 kg, with a median of 3.85 kg. The LeCompte manoeuvre was performed in only nine patients. The mean cardiopulmonary bypass time was 157.5±24.9, with a median of 161 minutes, and the mean aortic cross-clamp time was 101.2±23.8, with a median of 102 minutes. Subsequently, two patients died: the first due to a sudden onset of ventricular fibrillation and the second during a crisis of severe pulmonary hypertension. At the last follow-up, which ranged from 23 to 41 months, with a mean of 38.04±2.32 and a median of 38.4 months, all 18 survivors were in NYHA class I, with none requiring cardiac medications and all having normal bi-ventricular function without residual defects. CONCLUSION: With appropriate technical modifications, patients with concordant atrioventricular and discordant ventriculo-arterial connections with a left-sided aorta can undergo successful anatomical repair.

Anatomical Repair of an Unusual Combination of Tetralogy of Fallot and Atrioventricular Septal Defect With Unroofed Coronary Sinus.

A 30-month-old female was admitted with recurrent spells and severe cyanosis. Preoperative echocardiography was diagnostic of tetralogy of Fallot with an atrial septal defect of the primum type, unroofed coronary sinus, and a left superior vena cava draining into the left atrium. At surgery the patient was found to have a complete atrioventricular septal defect in addition to these anomalies. Complete anatomical correction was achieved through the right atrial approach.

Alternative approach for repair of total anomalous pulmonary venous connection to the coronary sinus at the time of extracardiac total cavopulmonary connection.

Total anomalous pulmonary venous connection can be encountered in patients with a univentricular heart and must be addressed to at the time of univentricular palliation. We present an alternative technique of re-channeling of the pulmonary venous return toward the left heart in these patients.

Cardiopulmonary exercise performance after total cavopulmonary connection with or without prior superior cavopulmonary connection.

OBJECTIVE: To compare cardiopulmonary exercise capacity of patients undergoing primary total cavopulmonary connection (TCPC) with those undergoing TCPC after a prior bidirectional Glenn (BDG). METHODS: Out of 42 patients in this study, 24 had undergone primary TCPC while 18 had undergone staged TCPC. The peak oxygen consumption (O(2) peak), O(2) pulse, chronotropic index, and other exercise parameters were studied. RESULTS: There were no significant differences in O(2) peak (940 ± 538 vs. 1010 ± 417 mL/min, p=0.42) and O(2) pulse (5.76 ± 3.52 vs. 6.85 ± 2.95) of patients undergoing staged TCPC versus primary TCPC, respectively; however, chronotropic index (0.43 ± 0.23 vs. 0.30 ± 017, p=0.047) was significantly different. Exercise tolerance was the same in fenestrated versus nonfenestrated TCPC groups and age at TCPC less than or more than 7 years. However, O(2) peak and O(2) pulse of patients with extracardiac TCPC was better than patients with lateral tunnel TCPC (p values 0.05, 0.04 and respectively). Some parameters of exercise tolerance of patients with antegrade pulmonary blood flow (APBF) interrupted at the time of BDG were better than those with APBF open. CONCLUSION: There were no differences in the exercise parameters of patients undergoing a staged versus a primary TCPC, fenestrated versus nonfenestrated TCPC, and age at surgery less than or more than 7 years. Exercise parameters were better in the extracardiac conduit group versus lateral tunnel TCPC groups. Patients who had a TCPC after prior interruption of APBF had better exercise parameters.

Pericardiectomy in children <15 years of age.

Between January, 2002 and December, 2011, 27 patients (19 boys) underwent pericardiectomy. The mean age was 9.3 ± 4.96 years (range 0.4 to 15 years) and the mean duration of symptoms was 16.9 ± 22.15 months. In all, 25 patients had dyspnoea; eight were in New York Heart Association (NYHA) class IV; six had bacterial pericarditis; and 18 were on anti-tuberculosis treatment, although only nine had records suggesting tuberculosis. There were nine patients who underwent pre-operative pigtail catheter drainage of pericardial fluid. Surgical procedures were complete pericardiectomy (n = 20), partial pericardiectomy (n = 6), and pleuropericardial window (n = 1).The mean pre-operative right atrial pressure was 20.4 ± 4.93 mmHg. There were six hospital deaths due to low cardiac output (n = 5) and arrhythmia (n = 1). The mean intensive care unit stay was 2.7 ± 1.2 days and mean post-operative stay was 9.9 days. The mean right atrial pressure dropped to 8.7 ± 1.15 mmHg. Adverse outcomes defined as death/prolonged intensive care unit stay, prolonged post-operative stay were not associated with sex, diagnosis of tuberculosis or pyopericardium, or the duration of symptoms or pre-operative right atrial pressure. Younger patients had prolonged intensive care unit stay (p = 0.03) but not increased mortality. Advanced NYHA class predicted death (p = 0.02). The mean follow-up was 23.1 ± 23.8 months. All except one survivor are in NYHA class I and off all cardiac medications. Despite adequate surgery, pericardiectomy in children is associated with a high mortality, which is related to delayed surgery and poor pre-operative general condition. No specific pre-operative variable other than worse pre-operative NYHA class is a predictor of survival. Therefore, early pericardiectomy should be undertaken in such patients.

A simple modification to fix the commissural pillar during right ventricular outflow tract reconstruction during the arterial switch operation.

A simplified technique to fix the commissural pillar of the pulmonary valve at the time of right ventricular outflow tract reconstruction during the arterial switch operation is presented.

Retrospective study of results of Kawashima procedure.

BACKGROUND: Patients with single ventricle physiology and an interrupted inferior caval vein undergo the Kawashima procedure. With increasing follow-up, pulmonary arteriovenous malformations may develop requiring hepatocardiac venous redirection. METHODS: Between January 2005 and December 2012, 15 patients underwent the Kawashima procedure. Preoperative and postoperative characteristics along with functional class, intra-operative details, post-operative course, saturations and haematocrit were recorded. RESULTS: Median age at operation was five years (range 1- 20 years). Five patients had antegrade pulmonary blood flow interrupted and 10 patients had open antegrade flow. There were no deaths. Mean saturation in postoperative period was 90.8 ±1.36%. Median duration of mechanical ventilation and inotropic support was six and 16hours respectively. Median duration of pleural drainage was six days. Median duration of pleural drainage in antegrade flow open and interrupted group was six days and four days respectively. The mean hospital stay was 10.5 days ±7.1 days. Mean follow up was 40.2 ±31 months (median-30 months). Nine patients (60%) were in New York Heart Association (NYHA) functional class 1 and five patients (33.3%) were in NYHA functional class 2 at last follow-up. Only one patient was in class 3 and needs completion Fontan. CONCLUSIONS: The Kawashima procedure can be safely performed with acceptable early results. Although studies have shown the risk of pulmonary arteriovenous malformations after Kawashima procedure in the mid and long-term, our findings do not support this. Kawashima procedure with open antegrade pulmonary blood flow as a definite intervention in such patients is debatable.

Autologous Reconstruction of Anomalous Origin of a Right Pulmonary Artery From the Aorta.

Anomalous origin of a pulmonary artery branch from the aorta is a rare congenital anomaly in which one of the pulmonary arteries arises from the aorta. These patients require early surgery to prevent development of severe irreversible pulmonary arterial hypertension. Multiple techniques have been described for repair of this condition. In this report, we describe a different technique compared with previously described procedures and discuss its advantages.

Repair of anomalous left coronary artery from pulmonary artery (ALCAPA) beyond infancy.

Between January 2002 and December 2012, five patients (4 female) underwent corrective surgery for anomalous left coronary artery from pulmonary artery (ALCAPA). They were older than 1 year (range, 3-56 years). One of the 2 patients younger than 10 years had presented with congestive heart failure, and the other had experienced repeated episodes of lower respiratory tract infection since childhood. Of the remaining 3 adult patients, 2 had experienced angina with effort, and 1 patient had had repeated respiratory tract infections since childhood, with mild dyspnea on effort of New York Heart Association (NYHA) class II. Three patients had the anomalous left coronary artery implanted directly into the ascending aorta via coronary-button transfer, and 2 patients underwent coronary artery bypass with obliteration of the left main ostium. Two patients underwent concomitant mitral valve repair procedures, and 1 patient underwent direct closure of a perimembranous ventricular septal defect. Four patients survived the surgery, and 1 patient died because of a persistently low cardiac output. Follow-up times ranged from 3 months to 4 years. All survivors are in NYHA class I and have left ventricular ejection fractions of 45% to 60%, with moderate (n = 1), mild (n = 1), or no (n = 2) mitral insufficiency. We conclude that a few naturally selected patients with ALCAPA do survive beyond infancy and can undergo establishment of 2 coronary systems with satisfactory results.

Open arch replacement: my way.

With advancement of hybrid and endovascular techniques, there are very few indications for open arch replacement. Major advancements in open arch replacement include antegrade perfusion-based cerebral protection, and an endovascular compliant arch replacement. In the present article, we demonstrate and describe our technique of Bentall's procedure and endovascular compliant arch replacement in a young Marfan's patient with chronic type A dissection and root aneurysm. Supplementary Information: The online version contains supplementary material available at 10.1007/s12055-023-01560-1.

Overview of acute type A aortic dissection in India.

Acute type A aortic dissection (TAAD) is a life-threatening surgical emergency. Though the entity is associated with high mortality and morbidity, with well-executed and timely surgical intervention, mortality and morbidity could be reduced to a reasonable extent. Information about demographics, clinical pattern, and results of management of acute TAAD from the Indian subcontinent largely remains unpublished. There are only a few specialized centers performing aortic operations. Very often, the patients with acute TAAD are operated on by surgeons with limited experience and resources. The surgeon is operating like a "lone warrior" without the support of a specialized radiologist, interventionalist, and specialized anesthetist. In most of the hospitals, facilities for sophisticated monitoring, sealants, specialized grafts, and stent graft are not available. We follow a simple algorithm of diagnosis and surgical management. The goal of treatment is to save the life. We follow a conservative approach best suited to our circumstances. Mild hypothermia, carotid cannulation, and antegrade cerebral perfusion as cerebral protection strategies have yielded satisfactory results. In case of organ malperfusion, with some exceptions, we perform aortic repair first. Our policy, towards arch management is less aggressive. In high-risk cases, we perform an endovascular-compliant hemiarch or arch replacement, followed by stent grafting in the post-operative period.

Innominate vein reconstruction in patients with bilateral superior vena cava undergoing bilateral bidirectional superior cavopulmonary anastomosis.

A 4.5-year-old patient with tricuspid atresia, pulmonary stenosis, bilateral superior vena cava veins with absent innominate vein, and hypoplasia of the left pulmonary artery required a bidirectional superior cavopulmonary anastomosis. An innominate vein was fashioned out of a 6 mm polytetrafluoroethylene graft. The technique is briefly discussed.