Chylothorax in Infants and Children After Congenital Heart Surgery: Approach and Review.

Chylothorax in infants after surgery for congenital heart disease is associated with significant morbidity and mortality. Numerous management modalities applied alone or in various combinations have been utilized but definitive guidelines have not yet been established. We present two infants who developed refractory chylothorax after congenital heart surgery. We also reviewed evidence for the use of available treatment modalities. In our experience, the use of lymphangiography followed by pleurodesis by povidone-iodine was safe and our impression was that it may have played a decisive role in controlling the lymph leak.

Comparison of Levosimendan Versus Milrinone After the Arterial Switch Operation for Infants ≤3†kg.

Background: Various inotropes and inodilators have been utilized to treat low cardiac output syndrome after the arterial switch operation. The use of levosimendan, a calcium sensitizer has been limited in this setting. This study compares the effects of levosimendan with milrinone in managing low cardiac output after the arterial switch operation. Methods: A retrospective, comparative study was conducted in a tertiary care hospital on patients weighing up to 3 kg undergoing the arterial switch operation between January 2017 and January 2022. Patients received a loading dose followed by continuous infusion of either levosimendan or milrinone. Echocardiographic, hemodynamic and biochemical parameters were compared. Results: Forty-three patients received levosimendan and 42 patients received milrinone as the primary test drug. Cardiac index of less than 2.2 L/min/m2 on postoperative day 1 and 2 was found in 9.3% and 2.3% of patients receiving levosimendan versus 26.2% and 11.9% in those receiving milrinone, respectively (P = .04 and .08, respectively). Early lactate-clearance and better central venous oxygen saturations were noted in the levosimendan group. Prevalence of acute kidney injury was higher in the milrinone group (50% vs 28%; P = .03). Use of peritoneal dialysis in the milrinone group versus levosimendan was 31% and 16.3%, respectively (P = .11). There was no difference in hospital mortality between the groups (milrinone, 3; levosimendan, 2, P = .62). Conclusions: Levosimendan is safe and as effective as milrinone to treat low cardiac output syndrome occurring in neonates after the arterial switch operation. In addition we found that levosimendan was renal protective when compared with milrinone.

"Prolonged" venoarterial extracorporeal membrane oxygenation support for respiratory failure: Outcome in an infant.

Extracorporeal membrane oxygenation (ECMO) is a form of extracorporeal life support which provides cardiorespiratory support to patients with potentially reversible pathophysiological processes. ECMO has evolved over the past few decades as a standard technology for neonatal severe respiratory support. However, its use in the pediatric population has increased only since 2009. We report a case of a 9-month infant who required a prolonged (789 h) venoarterial ECMO for severe acute respiratory distress consequent to pneumonia probably secondary to aspiration. He was discharged after this prolonged ECMO run without any obvious unfavorable outcome and is neurodevelopmentally sound at a 26-month follow-up.

Perfusion Strategy to Prevent Right Ventricular Decompression on Cardiopulmonary Bypass During Extracardiac Fontan for Right Ventricle-Dependent Coronary Circulation.

Early and long-term outcomes in patients with pulmonary atresia-intact ventricular septum undergoing staged univentricular palliations have been known to be adversely affected by the presence of right ventricle-dependent coronary circulation. We describe a surgical technique to circumvent the coronary insufficiency caused by acute decompression of the right heart.

Endovascular stent implantation as a primary method of treatment for blocked modified Blalock‒Taussig shunt.

Modified Blalock‒Taussig shunt is a commonly performed palliative cardiac surgery. Complications like early or late occlusion have been reported in approximately 10% of patients. Thrombosis, stenosis, or kinking are possible mechanisms that lead to shunt obstruction which may be life-threatening and need urgent intervention in form of medical, surgical, or interventional catheterization. Traditionally, these patients are managed in the operating room with shunt revision but with advancing techniques, percutaneous interventions are being increasingly deployed to salvage these patients effectively. We describe our experience of two cases of blocked modified Blalock‒Taussig shunt, which was successfully managed by stenting the shunt.

Aneurysm of Pulmonary Sinus of Valsalva: Successful Management of a Case.

Pulmonary sinus of Valsalva aneurysm is an extremely rare entity, reported only twice in the literature. It can remain asymptomatic or can present with symptoms when associated with pulmonary artery aneurysms. We present a case of pulmonary sinus of Valsalva aneurysm causing severe right ventricular outflow tract obstruction. This report aims to alert the physicians of such an entity when evaluating the causes of subvalvar right ventricular outflow tract obstruction.

Axillary arterial access for balloon dilatation of coarctation of aorta in newborns: a case series.

Balloon dilatation of coarctation of aorta is a standard of care for the patients presenting with severe left ventricular dysfunction. It can be performed through femoral, carotid, and axillary arterial access. Very few case series were available in the literature through axillary arterial access, despite being its advantage as non-end artery and easily palpable in coarctation of aorta. We present our experience with five cases of neonatal coarctation of aorta with severe left ventricular dysfunction where successful balloon dilatation of coarctation of aorta was performed via axillary approach without adverse events.

Techniques to avoid hypothermic circulatory arrest in the management of renal tumor with right atrium extension in children.

Clear cell sarcoma of the kidney is a rare variety of renal tumor accounting for less than 5% of all pediatric renal tumors. Cardiopulmonary bypass along with hypothermic circulatory arrest is frequently used for management of tumor thrombus extending into supra-hepatic inferior vena cava and right atrium. In this paper, we present a strategy of avoiding circulatory arrest and hypothermia and thereby fast-tracking the recovery in managing a case of clear cell sarcoma of the kidney in a 3.5-year-old child with tumor thrombus extending into the right atrium.

Outcomes of primary repair of sternal cleft defects: Providing a "bony cover".

Background: Sternal clefts are rare congenital anterior chest wall defects created by a lack of midline thoracic fusion. Various surgical repairs have been proposed to provide protection to underlying viscera in these defects. Aim: This study aims to perform primary sternal cleft repair using techniques, leading to the provision of a complete bony cover and to assess their outcomes on follow-ups. Materials and Methods: During 2009-2020, seven patients were referred to our unit with sternal defects. Out of them, four infants with sternal clefts underwent primary repair using bilateral perichondrial flap creation of the sternal bars and sliding costal chondrotomy at our institute. In one of them with a wider defect, bilateral "intraperiosteal" sliding clavicular osteotomy was additionally performed to achieve tension-free closure. Results: Satisfactory surgical outcomes were achieved with an uneventful postoperative period. On follow-up, all four patients are thriving well and have a stable anterior chest wall. Those with follow-ups longer than 5 years showed evidence of bone formation. Conclusion: Bony cover to the heart can be provided in all varieties of sternal cleft defects using primary surgical repair early in infancy. The delay in surgical correction increases the complexity of the procedure and may require the use of prosthetic material which has its own disadvantages.

Strategies to mitigate inflammation in management of complex congenital heart disease complicated by "multisystem inflammatory syndrome in children".

A 6-month-old boy, a case of Shone's complex, presented in decompensated state was found to have severe mitral stenosis along with multisystem inflammatory syndrome in children (MISC) warranting urgent surgical intervention. Various modalities including cytokine-adsorbing hemofilter were used to target inflammation. Postoperatively, the child recovered from low cardiac output accompanied by decrease in the levels of inflammatory markers, inopressors, and ventilatory requirements. Open heart surgery in itself is a proinflammatory process and is best avoided during the active inflammatory phase of MISC. In the rare and unavoidable circumstance exemplified by this index case, multipronged strategy targeting inflammation as described can be successfully implemented.

Anesthesia protocols for "bedside" preterm patent ductus arteriosus ligation: A single-institutional experience.

BACKGROUND: Hemodynamically significant patent ductus arteriosus (PDA) is frequently encountered in preterm infants sometimes requiring surgical attention. Although PDA ligation is regularly performed in the operating room, conducting it at the bedside in a neonatal intensive care unit (NICU) and its anesthetic management remains challenging. AIM: We aim to discuss the anesthetic considerations in patients undergoing bedside PDA ligation and describe our experience highlighting the feasibility and safety of this procedure. SETTING AND DESIGN: The study was conducted in the NICU in a tertiary care hospital; This was a retrospective, observational study. METHODS: Preterm infants scheduled for bedside PDA ligation using a predefined anesthesia protocol between August 2005 and October 2020 were included. STATISTICAL ANALYSIS USED: Quantitative data were presented as median with interquartile range and categorical data were presented as numbers and percentage thereof. RESULTS: Sixty-six premature infants underwent bedside PDA ligation. Thirty-day mortality was 4.5% (3 infants), but there were no procedural deaths. One (1.5%) patient had intraoperative endotracheal tube dislodgement. Three (4.5%) infants had postoperative pneumothorax requiring an additional chest tube insertion. Twenty-one (32%) patients required initiation of postoperative inotrope/vasodilator therapy within 6 h. Three postligation cardiac syndromes (≥ Grade-III mitral regurgitation with left ventricular dysfunction and hypotension) occurred. CONCLUSIONS: Although anesthesia for preterm neonates undergoing bedside PDA ligation poses unique challenges, it can be safely conducted by following a predetermined standardized anesthesia protocol. Its successful conduct requires utmost vigilance and pristine understanding of the principles of neonatal and cardiac care.

A review of the Yasui operation with long-term follow-up of a case.

Yasui operation combines Norwood arch reconstruction with Rastelli operation for interrupted or hypoplastic aorta with aortic valvar atresia or hypoplasia with ventricular septal and two adequately sized ventricles, establishing biventricular repair. We present a case of aortic atresia, mitral hypoplasia, and ventricular septal defect (VSD) treated by Yasui procedure, and its long-term (108 months) follow-up and brief review of literature. Review of literature was done using keywords to search on "PubMed" and "Google Scholar." SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1007/s12055-021-01174-5.

Techniques to repair parachute tricuspid valve.

Parachute deformity of atrioventricular valve occurs when chordae tendineae arise either from one papillary muscle or a closely placed muscle group. Preoperative detection is easier with mitral involvement than with tricuspid valve. We present two cases of parachute tricuspid valve (PTV) which were successfully repaired.

Anomalous Left Subclavian Artery from the Left Pulmonary Artery in Transposition of the Great Arteries with Right Aortic Arch.

Anomalous origin of the subclavian artery from the pulmonary artery is a rare but well-described entity. We report a case of anomalous origin of the left subclavian artery from the left pulmonary artery in a patient with transposition of the great arteries. We discuss the safe intraoperative management of this anomaly in a patient in whom the diagnosis was made intraoperatively.

Effect of Albumin Addition to Cardiopulmonary Bypass Prime on Outcomes in Children Undergoing Open-Heart Surgery (EACPO Study)-A Randomized Controlled Trial.

BACKGROUND: There is a paucity of literature regarding the association of high oncotic priming solutions for pediatric cardiopulmonary bypass (CPB) and outcomes, and no consensus exists regarding the composition of optimal CPB priming solution. This study aimed to examine the impact of high oncotic pressure priming by the addition of 20% human albumin on outcomes. METHODS: Double-blinded, randomized controlled study was done in the pediatric cardiac intensive care unit of a tertiary care hospital. Consecutive children with congenital heart diseases admitted for open-heart surgery were randomized into two groups, where the study group received an additional 20% albumin to conventional blood prime before CPB initiation. RESULTS: We enrolled 39 children in the high oncotic prime (added albumin) group and 37 children in the conventional prime group. In the first 24-hour postoperative period, children in the albumin group had significantly lower occurrence of hypotension (28.2% vs 54%, P = .02), requirement of fluid boluses (25.6% vs 54%, P = .006), and lactate clearance time (6 vs 9 hours, P < .001). Albumin group also had significantly higher platelet count (×103/µL) at 24 hours (112 vs 91, P = .02). There was no significant difference in intra-CPB hemodynamic parameters and incidence of acute kidney injury. In subgroup analysis based on risk category, significantly decreased intensive care unit stay (4 vs 5 days, P = .04) and hospital stay (5 vs 7 days, P = .002) were found in the albumin group in low-risk category. CONCLUSION: High oncotic pressure CPB prime using albumin addition might be beneficial over conventional blood prime, and our study does provide a rationale for further studies.

Near-infrared spectroscopy: An important tool during the blalock-taussig shunt.

Near infra red spectroscopy (NIRS) is a noninvasive diagnostic tool for measuring regional oxygen saturation (rSO2). Cerebral oxygenation measured with NIRS is used to corroborate mixed venous oxygenation and hence considered an indicator of tissue perfusion. We describe NIRS guiding an anatomical variation leading to inadequate cerebral circulation or any impairment in cerebral oxygen delivery during Blalock Taussig shunt.

Tricuspid Atresia With Truncus Arteriosus: Off-Pump Stage I Palliation.

Coexistence of tricuspid atresia and truncus arteriosus (common arterial trunk) is an extremely rare combination of anomalies and requires staged palliation. Initial palliation involves disconnection of the pulmonary arteries from the common trunk and creation of a systemic-to-pulmonary artery shunt. This has generally been accomplished with the use of cardiopulmonary bypass. We describe a technique of off-pump palliation using the common trunk and a modified Blalock-Taussig shunt as sources of pulmonary blood flow at various stages of reconstruction. The child has recovered well and has undergone second-stage palliation with bilateral bidirectional superior cavopulmonary anastomoses at one year of age.

Complete transposition of great arteries associated with total anomalous pulmonary venous connection: An unusual cause for early left ventricular myocardial mass regression.

A 24-day-old apparently asymptomatic neonate was found to have complete transposition of great arteries with small patent ductus arteriosus and restrictive patent foramen ovale. The neonate was found to have relatively high saturations (saturations = 88%) despite inadequate mixing communications. Echo findings were suggestive of significant dilatation of right atrium and right ventricle and left ventricular (LV) mass regression. Further echo interrogation revealed coexisting total anomalous pulmonary venous connection (TAPVC) as the cause of relatively high saturations and early LV mass regression. The patient was planned for follow-up and underwent successful Senning repair at the age of 8 months. Hemodynamics and echo findings of this association of TGA with TAPVC have been described in this case report.

Postoperative cerebral oximetry monitoring helps in early detection of diminished flow in Blalock-Taussig shunt.

Cerebral oximeter is a noninvasive device which provides continuous monitoring of the regional cerebral saturation using near-infrared spectroscopy (NIRS). After gaining popularity as an intraoperative monitoring tool, use of NIRS monitoring has also expanded to postoperative period of congenital heart diseases now. Shunt underflow is a known complication after Blalock-Taussig (BT) shunt, which is conventionally detected by a drop in oxygen saturation and metabolic acidosis. We report a case where cerebral regional saturation monitoring by NIRS helped in early detection of low pulmonary flow state during postoperative period of neonatal BT shunt. We observed that the drop in regional cerebral oxygen saturation preceded fall in peripheral oxygen saturation during shunt underflow.