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.

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.

The American Association for Thoracic Surgery (AATS) 2022 Expert Consensus Document: Management of infants and neonates with tetralogy of Fallot.

OBJECTIVE: Despite decades of experience, aspects of the management of tetralogy of Fallot with pulmonary stenosis (TOF) remain controversial. Practitioners must consider newer, evolving treatment strategies with limited data to guide decision making. Therefore, the TOF Clinical Practice Standards Committee was commissioned by the American Association for Thoracic Surgery to provide a framework on this topic, focused on timing and types of interventions, management of high-risk patients, technical considerations during interventions, and best practices for assessment of outcomes of the interventions. In addition, the group was tasked with identifying pertinent research questions for future investigations. It is recognized that variability in institutional experience could influence the application of this framework to clinical practice. METHODS: The TOF Clinical Practice Standards Committee is a multinational, multidisciplinary group of cardiologists and surgeons with expertise in TOF. With the assistance of a medical librarian, a citation search in PubMed, Embase, Scopus, and Web of Science was performed using key words related to TOF and its management; the search was restricted to the English language and the year 2000 or later. Articles pertaining to pulmonary atresia, absent pulmonary valve, atrioventricular septal defects, and adult patients with TOF were excluded, as well as nonprimary sources such as review articles. This yielded nearly 20,000 results, of which 163 were included. Greater consideration was given to more recent studies, larger studies, and those using comparison groups with randomization or propensity score matching. Expert consensus statements with class of recommendation and level of evidence were developed using a modified Delphi method, requiring 80% of the member votes with 75% agreement on each statement. RESULTS: In asymptomatic infants, complete surgical correction between age 3 and 6 months is reasonable to reduce the length of stay, rate of adverse events, and need for a transannular patch. In the majority of symptomatic neonates, both palliation and primary complete surgical correction are useful treatment options. It is reasonable to consider those with low birth weight or prematurity, small or discontinuous pulmonary arteries, chromosomal anomalies, other congenital anomalies, or other comorbidities such as intracranial hemorrhage, sepsis, or other end-organ compromise as high-risk patients. In these high-risk patients, palliation may be preferred; and, in patients with amenable anatomy, catheter-based procedures may prove favorable over surgical palliation. CONCLUSIONS: Ongoing research will provide further insight into the role of catheter-based interventions. For complete surgical correction, both transatrial and transventricular approaches are effective; however, the smallest possible ventriculotomy should be utilized. When possible, the pulmonary valve should be spared; and if unsalvageable, reconstruction can be considered. At the conclusion of the operation, adequate relief of the right ventricular outflow obstruction should be confirmed, and identification of a significant fixed anatomical obstruction should prompt further intervention. Given our current knowledge and the gaps identified, we propose several key questions to be answered by future research and potentially by a TOF registry: When to palliate or proceed with complete surgical correction, as well as the ideal type of palliation; the optimal surgical approach for complete repair for the best long-term preservation of right ventricular function; and the utility, efficacy, and durability of various pulmonary valve preservation and reconstruction techniques.

Left pulmonary artery reconstruction using cryopreserved pulmonary homograft.

In patients with univentricular heart, the Fontan procedure is the final palliation. This is usually staged. A systemic-to-pulmonary artery shunt is performed in the presence of episodes of cyanotic spells in the neonatal period or in some patients in infancy; a bidirectional superior cardiopulmonary anastomosis is preferred early in life. This is followed by the final Fontan palliation on an elective basis later. For an effective bidirectional superior cavopulmonary anastomosis and Fontan palliation, good-sized confluent pulmonary arteries (PAs) are mandatory in addition to favorable hemodynamic data. Patients with discontinuous PAs that are small in size present a surgical challenge at initial palliation as the one described in this report.

An alternate technique of performing total cavopulmonary connection without cardiopulmonary bypass.

Total cavopulmonary connection (Fontan) without using cardiopulmonary bypass (CPB) may be superior to Fontan on CPB. In the experience of many, a Fontan operation without CPB may be associated with a reduced need for inotropic support, improved outcomes in the postoperative phase including faster time to termination of mechanical ventilatory support, reduced intensive care unit stay, lower volume of pleural and peritoneal effusions, and decreased hospital stay, thereby rendering it more cost-effective. However, the operation is technically more difficult to perform than Fontan on CPB and requires significant modifications of surgical technique and alteration in overall management strategy. In this article, an alternative technique of performing total cavopulmonary connection without CPB is described. Its advantages are briefly discussed.

Neurodevelopmental outcomes in children with cyanotic congenital heart disease following open heart surgery.

Background: Neurodevelopmental abnormalities are common in congenital heart disease (CHD), more so in cyanotic CHDs. Perioperative factors have been known to affect neurodevelopmental outcomes. Aim: We aimed to determine the neurodevelopmental outcomes following open-heart surgery in cyanotic CHD. Methods: In this prospective observational study, eligible infants and children ≤21 months with cyanotic CHD planned for open-heart surgery underwent preoperative neurodevelopmental assessment using Developmental Assessment Scale for Indian Infants (DASII) to look for any motor and/or mental delay. A second neurodevelopmental assessment was performed after 9 months ± 2 weeks of cardiac surgery. Follow-up DASII was conducted through interactive video conferencing in 23 of 60 patients due to COVID-19 pandemic. The univentricular and biventricular repair groups were compared in terms of their neurodevelopmental outcomes. Perioperative factors were compared between neurodevelopmental "delay" and "no delay" groups. Results: Of the 89 children enrolled, preoperative motor and mental delay were present in 29 and 24 children, respectively. Follow-up DASII could be performed in 60 children. At follow-up, motor delay was present in seven and mental delay in four children. Overall, there was a significant improvement in both motor and mental developmental quotient at follow-up. There was no significant difference in either motor or mental domains between univentricular and biventricular groups. Among the perioperative variables, only the postoperative length of stay in intensive care unit was significantly different between neurodevelopmental "delay" and "no delay" groups (P = 0.04). Conclusion: Neurodevelopmental delay occurred substantially among unoperated children with cyanotic CHD. The neurodevelopmental status improved significantly following open-heart surgery among the survivors. Delay was associated with length of stay in intensive care following cardiac surgery.

Thrombus formation in the heart following balloon atrial septostomy in transposition of great arteries.

Thrombotic complications following balloon atrial septostomy (BAS) are unusual. We report a patient with thrombus formation at the site of BAS, extending into the inferior vena cava (IVC), following BAS for transposition of great arteries with intact ventricular septum (TGA-IVS). An urgent arterial switch operation (ASO) with removal of the thrombus was performed. Supplementary Information: The online version contains supplementary material available at 10.1007/s12055-022-01331-4.

Plasmalyte-A Based del Nido Cardioplegia Versus Plain Ringer Based del Nido Cardioplegia: Double-Blind Randomized Trial.

Background: In this prospective randomized controlled trial, we compared the standard del Nido cardioplegia solution (SDN) with the modified del Nido cardioplegia solution (MDN) in which the base solution was the plain Ringer solution. Methods: A total of 80 patients aged < 12 years undergoing intracardiac repair of Tetralogy of Fallot were randomized into SDN (n = 39) or MDN (n = 41) groups. The primary outcome was a change in cardiac index (CI). Secondary outcomes were ventricular arrhythmias after the release of aortic-cross clamp, postoperative inotropic score (IS), time to peripheral rewarming, duration of mechanical ventilation, intensive care unit (ICU) length of stay, and hospital length of stay, and electron microscopic differences between the 2 groups. Cardiac Troponin-I, inflammatory markers tumor necrosis factor-α (TNF-α), and interleukin-L (IL-6) were measured. Results: Applying the noninferiority confidence interval approach, the difference between the changes in CI between the 2 groups was -0.093 L/min/m2 (95% CI: -0.46-0.27 L/min/m2) which was within the noninferiority threshold of -0.5 indicating that CI was similar in both SDN and MDN. Ventricular arrhythmias postclamp release (P = .91), IS (P = .09), duration of mechanical ventilation (P = .27), ICU length of stay (P = .50), hospital length of stay (P = .57), IL-6 (P = .19), TNF-α (P = .17), Troponin-I (P = .15), electron microscopy changes (P > .05) were not different between groups. Conclusion: MDN was shown to be noninferior to the SDN cardioplegia in terms of preservation of cardiac index. In addition, other metrics indicative of myocardial protection were similar between groups. In developing nations where SDN is not available or is expensive, MDN cardioplegia is an acceptable alternative.

Right pulmonary artery to left atrium fistula.

In this report, we describe a 25-year-old patient in whom a fistula between the right pulmonary artery and the left atrium was accurately diagnosed. The successful surgical management of this patient is described. Current diagnostic methods, evaluation, and treatment options for this uncommon cause of cyanosis in an adult patient are discussed.

Modified ultrafiltration and postoperative course in patients undergoing repair of tetralogy of fallot.

BACKGROUND: Expected benefits of modified ultrafiltration (MUF) include increased hematocrit, reduction of total body water and inflammatory mediators, improved left ventricular systolic function, and improved systolic blood pressure and cardiac index (CI) following cardiopulmonary bypass (CPB). This prospective randomized trial tested this hypothesis. METHODS: Seventy-nine patients undergoing intracardiac repair of tetralogy of fallot were randomized to conventional ultrafiltration (CUF) + MUF (n = 39) or only CUF group (n = 40). The primary outcome was a change in hematocrit. Secondary outcomes were changes in peak airway pressures, ventilatory support, blood transfusions, time to peripheral rewarming, mean arterial pressure, central venous pressure, inotrope score (IS), and CI. Serum inflammatory markers were measured. RESULTS: Baseline hematocrit was 50.6 ± 10.02 in the only CUF group whereas it was 43.9 ± 5.55 in the CUF + MUF group (p = .36). Following MUF, the CUF + MUF group had higher hematocrit (44.7 ± 0.50 g/dl) compared to the only CUF group (37.2 ± 0.49 g/dl), p ≤ .001 after adjusting for baseline hematocrit. Central venous pressure (mmHg) immediately following sternal closure was 9.27 ± 3.12 mmHg in the CUF + MUF group and 10.52 ± 2.2 mmHg in the only CUF group (p = .04). In the intensive care unit (ICU), they were 11.52 ± 2.20 mmHg in the only CUF group and 10.84 ± 2.78 mmHg in the CUF + MUF group (p = .02). Time to peripheral rewarming was 6.30 ± 3.91 h in the CUF + MUF group and 13.67 ± 3.91 h in the only CUF group (p = .06). Peak airway pressures in ICU were 17 ± 2 mmHg versus 20.55 ± 2.97 mmHg in CUF + MUF group & only CUF group, respectively, p < .001). Duration of mechanical ventilation was 6.3 ± 2.7 h in CUF + MUF group compared to 14.7 ± 3.5 h in the only CUF group (p = .002). IS was 11.52 ± 2.20 in the only CUF group compared to 10.84 ± 2.78 in CUF + MUFs group. Eight of 39 (20.5%) patients in the CUF + MUF group had IS > 10 compared to 22 of 40 (55%) patients in the only CUF group (p = .02). Serum Troponin-T and interleukin-6 levels were lower in the CUF + MUF group; TNF-α and CPK-MB were similar. ICU and hospital stay were similar. CONCLUSION: Patients undergoing a combination of CUF and MUF had higher postoperative hematocrit, decreased duration of mechanical ventilation, lower need for inotropes and lower interleukin-6 and Troponin-T levels. This group had better postoperative outcomes. This study was registered with the Clinical trials registry of India (CTRI/2017/11/010512) before commencement.

Totally anomalous pulmonary venous connection with intact atrial septum.

BACKGROUND: Total anomalous pulmonary venous connection with intact interatrial septum is extremely rare. AIMS: In these patients, a right to left shunt through a ventricular septal defect or a patent ductus arteriosus is mandatory to maintain the systemic circulation. Mechanical or physiological restriction of shunting through these pathways results in rapid clinical deterioration. DISCUSSION: We describe the anatomical findings with surgical repair in one such 10-day-old baby.

Ivabradine Versus Amiodarone in the Management of Postoperative Junctional Ectopic Tachycardia: A Randomized, Open-Label, Noninferiority Study.

OBJECTIVES: This study sought to compare the efficacy of ivabradine and amiodarone in the management of postoperative junctional ectopic tachycardia (JET) after cardiac surgery in children. BACKGROUND: JET is a serious arrhythmia occurring in children after cardiac surgery and requires aggressive management. Amiodarone has been conventionally used in its treatment. Recent studies have reported the utility of ivabradine in this regard. METHODS: In this open-label randomized controlled trial, 94 children (age ≤18 years) who developed postoperative JET were allocated to receive either amiodarone or ivabradine. The primary endpoint was restoration of normal sinus rhythm. RESULTS: Sinus rhythm was achieved in 43 out of the 46 patients (93.5%) in the amiodarone group and 46 out of the 48 patients (95.8%) in the ivabradine group (mean difference of treatment effect: 2.3%; 95% confidence interval: -6.7% to 11.5%). The median (interquartile range) time taken to achieve sinus rhythm conversion was similar in both the groups: 21.5 (17-30.2) hours versus 22 (13.4-38.5) hours (p = 0.36)]. The time taken to rate control of JET was significantly less in the amiodarone group: median 7.0 (5.5-9.5) hours versus 8.0 (5.8-10.8) hours (p = 0.02)]. No drug-related adverse events were observed in the ivabradine group. CONCLUSIONS: Oral ivabradine is not inferior to intravenous amiodarone in converting postoperative JET to sinus rhythm. There was no difference in time taken to sinus rhythm conversion between the groups, although the rate control was earlier in patients who received amiodarone. Monotherapy with ivabradine may be considered as an alternative to amiodarone in the management of postoperative JET. (Comparison of Two Drugs, Ivabradine and Amiodarone, in the Management of Junctional Ectopic Tachycardia, an Abnormality in Cardiac Rhythm in Patients Under 18 years Who Undergo Cardiac Surgery: CTRI/2018/08/015182).

Tetralogy of Fallot with coronary crossing the right ventricular outflow tract: A tale of a bridge and the artery.

A coronary artery crossing the right ventricular outflow tract is a subset of a larger pathomorphological cohort known as an anomalous coronary artery (ACA) in the tetralogy of Fallot (TOF). The best possible outcome in a patient with TOF and ACA is decided by judicious selection of optimum preoperative investigative information, the timing of surgery, astute assessment of preoperative surgical findings, and appropriate surgical technique from a wide array of choices. In most instances, the choice of surgical technique is determined by the size of the pulmonary annulus and the anatomical relation of ACA to the pulmonary annulus. In the present era, complete, accurate preoperative diagnosis and primary repair is a routine procedure with strategies to avoid a right ventricle-to-pulmonary artery conduit.

Where are we after 50 years of the Fontan operation?

First introduced in 1971, the Fontan procedure is the final common destination for all patients with a functional single ventricle. The procedure itself has evolved tremendously over the last five decades. This review traces this journey and presents the importance, outcomes and future outlook of the procedure in the current era.

Infective endocarditis-induced complete closure of a ventricular septal defect and complete heart block in a child.

We hereby report rare occurrence of irreversible complete heart block in a child with tricuspid valve infective endocarditis. The tricuspid valve vegetation also caused complete closure of perimembranous ventricular septal defect, which was later discovered during surgery.

Simultaneous cardiac and pulmonary central cyanosis in a young girl: the double trouble.

A 14-year-old girl admitted for corrective surgery for tetralogy of Fallot was found to have pulmonary arteriovenous malformations on pre-operative evaluation. In anticipation of a complicated postoperative course, percutaneous closure of the pulmonary arteriovenous malformations was undertaken first followed by a successful surgical outcome. Importance of this rare association and its clinical implication is hereby highlighted.

Audit of homograft valve bank.

INTRODUCTION: Even today, the search for the ideal cardiac valve continues. With advantages of having superior flow dynamics, avoidance of anticoagulation, and resistance to infection, homograft has been shown to have an edge over conventional prosthetic and bioprosthetic valves. But they suffer from disadvantages of limited availability and durability. Our center operates one of the oldest functioning valve banks in the country. We present our experience with homograft valve banking with antibiotic and cryopreserved homografts spread over a quarter century. METHODS: For donor selection, procurement, sterilization, and preservation, the recommendations of the American Association of Tissue Banks are being followed in accordance with statutory provisions of the Transplantation of Human Organs Act, 1994. RESULTS: During 25-year period (1993-2017), 777 hearts were procured. Age of the donors ranged from 2 to 60 years and hearts were procured within 24 h of death. A total of 1646 homografts (774 pulmonary, 774 aortic, 60 mitral valves, 20 descending thoracic aortae, and 18 monocusps) were harvested. A total of 546 (32%) homografts were rejected for various reasons. Nine hundred sixty-seven (56.7%) homografts were used in different procedures. Of these, 478 were pulmonary homografts, 425 were aortic homografts, 39 mitral homografts, 18 monocusps, and 7 descending thoracic aorta homografts. One hundred fifty-four (16%) homografts were antibiotic preserved and the rest 813 (84%) were cryopreserved. CONCLUSIONS: It is possible to run a homograft valve bank with minimum costs. Though, cryopreservation is more expensive, it provides an opportunity to store the valves for an indefinite period and maintain an uninterrupted supply of homografts.

The intra-extracardiac Fontan: preliminary results.

OBJECTIVE: Since the intra-extracardiac Fontan (IECF) was popularized by Jonas in 2008, its claimed advantages over the traditional extracardiac or lateral tunnel Fontan are simplicity, suitability for nearly all subsets, flow characteristics, low risk of sinus node artery injury, and possibly, a lower incidence of arrhythmias. In this paper, we present our early experience with this modification of the Fontan operation. METHODS: Between 2009 and 2018, 10 patients underwent IECF on cardiopulmonary bypass (CPB) and cardioplegic arrest at our institute. Analysis of preoperative, intraoperative, and early follow-up results was performed. A polytetrafluoroethylene (PTFE) graft was sutured proximally to the orifice of the inferior vena cava (IVC) and distally to the ipsilateral bidirectional superior cavopulmonary (BSCP) junction. RESULTS: Nine patients had undergone a previous BSCP connection, and one patient had a primary IECF. Diagnoses were double outlet right ventricle (n = 2), unbalanced atrioventricular septal defect with associated atrioventricular valve regurgitation (n = 3), single ventricle with anomalies of cardiac situs (n = 2), and tricuspid atresia with borderline pulmonary arteries (n = 1) or tricuspid atresia with borderline pulmonary artery pressures (n = 2). Median aortic cross-clamp and CPB times were 42 min and 82 min respectively. There were no early or late deaths. Median intensive care stay was 3 days (1 to 23 days). There were no arrhythmias. Mean duration of pleural effusions was 9.5 (median 5.5) days. There were no arrhythmias at a median follow-up of 5 years (range 1 month to 9.3 years). CONCLUSION: The IECF is simple, particularly at reoperations, in borderline patients and those needing concomitant intracardiac procedures. Early results are promising. These patients need constant surveillance.

Effect of leukoreduction on transfusion-related immunomodulation in patients undergoing cardiac surgery.

OBJECTIVES: In this study, we aimed to determine the consequences of different amounts of leukocyte transfusion on the outcome of patients undergoing cardiac surgery. DESIGN: This was a prospective, single-blinded cohort study conducted for 1 year from July 2018 to June 2019. SETTING: The study setting was the Department of Transfusion Medicine, along with Cardiac Anaesthesia, Cardiac Surgery and Cardiac biochemistry departments in a tertiary care cardiac centre. PARTICIPANTS: A total of 150 patients undergoing cardiac surgery during the study period were divided into three groups (50 in each): Leukofiltered (LR), Buffy coat depleted (BCD) and Non-leukoreduced (NLR). INTERVENTION: The intervention was intra- and postoperative transfusion of packed red blood cells (PRBCs) having different amounts of leukocytes. MEASUREMENTS AND MAIN RESULTS: Patient details about length of intensive care unit (ICU) and hospital stay, blood usage, inotropic drug duration, mechanical ventilation, urine output and infection were recorded from the patient data sheet, whereas patients were followed up for 30 days post-operation, and any mortality was noted. Haematological parameters and biochemical parameters for renal function test were analysed on pre- and post-surgical days 1, 3, 5 and 7, whereas on postoperative days 1 and 7, cytokine-like FAS ligands, Interleukin-10 (IL-10) and Interferon-γ (INF-γ) were tested. Patients in all three groups received an average of four, two and two units of packed red blood cells, platelets and fresh frozen plasma, respectively. There was a statistically significant (P < .05) rise in total leukocyte, neutrophil and lymphocyte count in all three groups from day 0 to day 3, but it reduced to preoperative level on day 5. There was shorter ICU and hospital stay in the LR group of patients (46 ± 19.9 hours and 7.5 ± 2.4 days) compared to NLR (52.1 ± 24.2 hours and 7.9 ± 4.1 days) and BCD (53.3 ± 26.7 hours and 8.8 ± 3.1 days) group of patients, but it was statistically non-significant. The duration of mechanical ventilation was significantly lesser in LR group patients (10.2 ± 6.2 hours) as compared to NLR group (14.7 ± 12.7 hours). On risk ratio calculation of developing postoperative kidney injury, the NLR group had 1.3 and 2.6 times more risk compared to the BCD and LR groups, respectively. On postoperative days 1 and 7, FAS-L levels significantly increased in all three group of patients, whereas IL-10 increased in the NLR and BCD groups and decreased in the LR group non-significantly. The INF-γ levels decreased on day 1 in the NLR and BCD groups but increased in the LR group, but it was inversed on day 7. CONCLUSION: Depletion of leukocytes decreased Transfusion Related Immunomodulation (TRIM) effects in patients undergoing cardiac surgery, but this also depends on the degree of leukoreduction. As found in our study, leukofiltration is more effective compared to buffy-coat depletion only.