Comparison of Morbidity and Mortality Outcomes between Hybrid Palliation and Norwood Palliation Procedures for Hypoplastic Left Heart Syndrome: Meta-Analysis and Systematic Review.

Background/Objectives: Hybrid palliation (HP) procedures for hypoplastic left heart syndrome (HLHS) are increasing. Our objective was to compare mortality and morbidity following HP and NP (Norwood palliation) procedures. Methods: Systematic review and meta-analysis of HLHS patients of peer-reviewed literature between 2000 and 2023. Mortality and/or heart transplantation in HP versus NP in the neonatal period, interstage period, and at 1, 3 and 5 years of age, and morbidity including completion of Stage II and Stage III palliation, unexpected interventions, pulmonary artery pressures, right ventricle function, neurodevelopmental outcomes and length of hospital stay were evaluated. Results: Twenty-one (meta-analysis: 16; qualitative synthesis: 5) studies evaluating 1182 HLHS patients included. HP patients had higher interstage mortality (RR = 1.61; 95% CI: 1.10-2.33; p = 0.01) and 1-year mortality (RR = 1.22; 95% CI: 1.03-1.43; p = 0.02) compared to NP patients without differences in 3- and 5-years mortality. HP procedure in high-risk HLHS patients had lower mortality (RR = 0.48; 95% CI: 0.27-0.87; p = 0.01) only in the neonatal period. HP patients underwent fewer Stage II (RR = 0.90; 95% CI: 0.81-1.00; p = 0.05) and Stage III palliation (RR = 0.78; 95% CI: 0.69-0.90; p < 0.01), had more unplanned interventions (RR = 3.38; 95% CI: 2.04-5.59; p < 0.01), and longer hospital stay after Stage I palliation (weighted mean difference = 12.88; 95% CI: 1.15-24.62; p = 0.03) compared to NP patients. Conclusions: Our study reveals that HP, compared to NP for HLHS, is associated with increased morbidity risk without an improved survival rate.

One and One-Half Ventricle Repair: Role for Restricting Antegrade Pulmonary Blood Flow.

BACKGROUND: In patients with hypoplastic subpulmonary ventricles, the one and one-half ventricle (1.5V) repair is an alternative to the Fontan procedure. However, in 1.5V-treated patients with pulsatile pulmonary blood flow, superior vena cava (SVC) hypertension or right atrial hypertension may develop. This study aimed to (1) describe patient outcomes after 1.5V repair and (2) determine whether pulmonary artery septation at 1.5V repair confers a lower risk of SVC or right atrial hypertension. METHODS: This study retrospectively reviewed patients who underwent a 1.5V repair between 1989 and 2020. The primary outcome was transplant-free survival. Secondary outcomes were postoperative SVC hypertension (defined by mean Glenn pressures greater than 17 mm Hg, SVC flow reversal or pulsatility, venovenous collateral vessels, or SVC syndrome) and right atrial hypertension (defined as mean right atrial pressures greater than 10 mm Hg with inferior vena cava and hepatic vein dilation or flow reversal). RESULTS: A total of 74 patients underwent 1.5V repair at a median age of 29.6 months (interquartile range, 8.9 to 45.5 months). Median follow-up time was 39.9 months (interquartile range, 11.4 to 178.1 months). Transplant-free survival at 10 years was 92.4%. Among survivors, 12% (8 of 69) had right atrial hypertension and 39% (27 of 69) had SVC hypertension on follow-up. Survivors with unseptated pulmonary arteries had a greater risk of SVC hypertension compared with patients with septated pulmonary arteries (44% vs 10%; P = .04). No difference was found in right atrial hypertension between the 2 groups. CONCLUSIONS: Patients with 1.5V repair avoid Fontan-associated complications with favorable transplant-free survival. However, SVC hypertension remains a significant long-term complication. Pulmonary artery septation at 1.5V repair may reduce the risk of SVC hypertension.

Repair of Anomalous Left Anterior Descending Coronary Artery Arising From the Pulmonary Artery Using a Dacron Interposition Bypass Graft.

An anomalous left anterior descending artery arising from the pulmonary artery (ALADAPA) is exceedingly rare. We present an adult case of ALADAPA that was repaired using a Dacron interposition graft.

Ascending Aortoplasty in Pediatric Patients Undergoing Aortic Valve Procedures.

BACKGROUND: The risks of ascending aortic dilation and indications for intervention in pediatric patients are unclear. Given the concern for aortic size mismatch with growth, larger ascending aortic diameters have been accepted in pediatric patients. The purpose of this study was to evaluate the effectiveness of ascending aortic reduction at the time of aortic valve surgery and its effect on recurrent aortic regurgitation. METHODS: A retrospective 8-year observational review (2010-2018) was conducted in pediatric patients with dilated ascending aorta undergoing aortic valve surgery at a single institution. RESULTS: Forty-seven patients underwent ascending aortoplasty during aortic valve surgery, of which 39 patients with complete data points had preoperative mean ascending aortic Z-scores of 5.35 ± 1.52 reduced to 1.22 ± 1.63 (P < .001) postoperatively. Ascending aortic Z-scores remained similar at 1.37 ± 1.72 at latest follow-up (P = .306). In contrast our matched control group (n = 39) without ascending aortoplasty had preoperative mean ascending aortic Z-scores of 4.15 ± 1.65 reduced to 3.26 ± 2.0 at the midterm follow-up. This reduction was significantly larger in the aortoplasty group, with an average difference in these changes of 3.12 (95% confidence interval, 2.43-3.8; P < .001). Multivariable logistic regression (controlling for aortic valve intervention type and body surface area) showed a 6.84 odds of moderate to severe aortic regurgitation at follow-up in the control group compared with the aortoplasty group (95% confidence interval, 1.43-32.8; P = .016). There were no complications associated with ascending aortoplasty. CONCLUSIONS: Ascending aortoplasty is effective in reducing ascending aortic dimensions and recurrent aortic regurgitation in pediatric patients. Longer-term follow-up is necessary to determine continued rate of growth.

1.5-Ventricle Repair Using Left Ventricle as the Subpulmonary Ventricle.

The use of the left ventricle as the subpulmonary ventricle to achieve a 1.5 or biventricular circulation is feasible in heterotaxy patients with complex intracardiac anatomy and acceptable right ventricular function. It is an alternative in patients who are not ideal candidates for single-ventricle palliation. We highlight 2 cases in which patients were rescued from a failed Fontan palliation and demonstrated improved functional status with normal saturations.

Contemporary Management of Acute Pulmonary Embolism.

Multiple treatment options beyond anticoagulation exist for massive and submassive pulmonary embolism to reduce mortality. For some patients, systemic thrombolytics and catheter-directed thrombolysis are appropriate interventions. For others, surgical pulmonary embolectomy can be life-saving. Extracorporeal life support and right ventricular assist devices can provide hemodynamic support in challenging cases. We propose a management algorithm for the treatment of massive and submassive pulmonary embolism, in conjunction with a multidisciplinary pulmonary embolism response team, to guide clinicians in individualizing treatment for patients in a timely manner.

Inferior mesenteric artery aneurysm in the setting of celiac and superior mesenteric artery occlusion.

Inferior mesenteric artery (IMA) aneurysm is a rare type of visceral aneurysm. We present the case of a 77-year-old woman with an IMA aneurysm in the setting of chronic complete occlusion of the origins of her celiac artery and superior mesenteric artery. The patient was managed successfully with surgical excision of the IMA aneurysm with an end-to-side anastomosis of the IMA to the left common iliac artery. The case report is followed by a discussion based on a literature review of the few previously reported occurrences of IMA aneurysm.

Zone zero hybrid arch exclusion versus open total arch replacement.

Open total aortic arch replacement is one of the most technically demanding operations in cardiothoracic surgery, requiring operator expertise and intraoperative and postoperative teamwork. Despite current advancements in the field of open aortic surgery with regard to intraoperative brain protection and postoperative care, the morbidity and mortality associated with open total arch operations varies. Endovascular and hybrid procedures involving the use of zone 0 as a landing zone allow fair comparison between open total arch and hybrid operations. Hybrid procedures involving all of the other landing zones [1-4] should not be compared with open total arch replacement, as the extent of the pathology is different.

Bovine Jugular Vein Conduit: A Mid- to Long-Term Institutional Review.

BACKGROUND: Since 1999, we have used the bovine jugular vein conduit for right ventricular outflow tract reconstruction in infants and children. Herein, we review their mid- to long-term outcomes. METHODS: Between 1999 and 2016, 315 bovine jugular vein conduits were implanted in 276 patients. Patients were grouped by age at bovine jugular vein conduit implant: group 1: 0 to 1 years (N = 65), group 2: one to ten years (N = 132), and group 3: older than ten years (N = 118). For survival and hemodynamic analysis, additional group stratification was done based on conduit size. Group small: 12 and 14 mm (N = 75), group medium: 16 and 18 mm (N = 84), and group large: 20 and 22 mm (N = 156). RESULTS: Mean follow-up for groups 1, 2, and 3 was 4.0, 4.9, and 5.9 years, respectively. Early mortality was 9%, 0%, and 1% for groups 1, 2, and 3, respectively ( P < .001). Late mortality was 5%, 2%, and 2% for groups 1, 2, and 3, respectively ( P = .337). Group 1 had the lowest ten-year freedom from conduit failure at 13%, versus 53% and 69% for groups 2 and 3, respectively ( P < .001). A total of 21 (6.6%) patients developed endocarditis, 11 (3.5%) patients required reoperation, and 10 (3.2%) patients required antibiotic therapy alone. CONCLUSIONS: The bovine jugular vein conduit is a useful option for right ventricular outflow tract reconstruction given its easy implantability and acceptable midterm durability.

Simple formulas to determine optimal subclavian central venous catheter tip placement in infants and children.

BACKGROUND/PURPOSE: Optimal central venous catheter (CVC) tip location is necessary to decrease the incidence of complications related to their use. We sought to create a practical method to reliably predict the length of catheter to insert into the subclavian vein during CVC placement in children. METHODS: We performed a retrospective review of 727 chest radiographs of children who underwent either left or right subclavian CVC placement. We measured the distance from the subclavian entry site to the to the right atrium/superior vena cava (RA/SVC) junction, following the catheter's course. We analyzed the relationship between that length and patient characteristics, including: age, gender, height, weight and body surface area (BSA). RESULTS: Two derived formulas using the BSA best correlated with the optimal subclavian CVC length. For the left subclavian vein approach, the optimal catheter length was 6.5 BSA+7 cm, and for the right subclavian vein approach it was 5 BSA+6. The use of these formulas correlated in CVC tip placement in a clinically proper location in 92.9% of smaller children and in 95.7% of larger children. CONCLUSION: The optimal length of central venous catheter to insert into the subclavian vein may be determined through the use of a simple formula using the BSA.

High incidence of catheter-associated venous thromboembolic events in patients with long gap esophageal atresia treated with the Foker process.

PURPOSE: To determine the incidence of catheter-associated venous thromboembolic events (VTE) in long gap esophageal atresia (LGEA) patients treated at Boston Children's Hospital (BCH) and to identify possible risk factors associated with their development. METHODS: We performed a retrospective analysis of LGEA patients from 2005 to 2012. Symptomatic VTEs with radiographic confirmation were defined as events. Potential risk factors were assessed by univariate analysis and multivariate logistic regression. Covariates included age, weight, initial gap length, cumulative days of pharmacologic paralysis and paralytic episodes, number and type of central venous catheters (CVCs), and number of operations. RESULTS: Forty-four LGEA patients were identified. The incidence of CVC associated VTE was 34%. Univariate analysis identified age at Foker 1 (P=.03), paralysis duration (P=.01), episodes of paralysis (P=.001), cumulative number of CVC (P=.007) and length of stay (P=.03) as significant. Multivariate logistic regression identified the number of paralytic episodes as the only significant independent risk factor for VTE (P<.0001). CONCLUSIONS: The incidence of symptomatic VTE was 34%, significantly higher than the VTE incidence of 4.5% reported for our other hospitalized children. These data have led to multidisciplinary discussions regarding thromboprophylaxis and development of a consensus-driven protocol. Since the initiation of this protocol, no VTEs have been identified.

Comparison of fenestrated and nonfenestrated patients undergoing extracardiac Fontan.

BACKGROUND: The purpose of this study is to compare morbidity and mortality between fenestrated (F, 61 patients) and nonfenestrated (NF, 54 patients) extracardiac Fontan patients during two eras from July 1995 to December 2010: era 1(1995 to 2004) and era 2 (2005 to 2010). METHODS: Variables evaluated included morphology, hemodynamics, chest tube volume and duration, intensive care and hospital stay, oxygen saturation, neurologic events, rhythm, and readmissions for chylous effusions. Follow-up in 114 hospital survivors was longer in the nonfenestrated cohort (F, 5.0 ± 3.3 years; NF, 7.1 ± 4.6 years; p < 0.005). RESULTS: Cohorts were similar in body size, morphology, and hemodynamics. Fenestration in hypoplastic left heart syndrome was appreciatively higher in era 2. Bypass time (F, 69 ± 27 minutes; NF, 57 ± 21 minutes) and conduit size (F, 18.8 mm; NF, 19.1 mm) were similar. There was 1 early nonfenestrated Fontan death (1 of 54; 2%) and 4 late deaths (F, 2 of 61, 5%; NF, 2 of 53, 4%; p = 0.86). Room air saturation was higher in NF patients (F, 89%; NF, 94%; p < 0.05). Total chest tube volume was similar, but fenestration was associated with greater chest tube drainage among hypoplastic left heart patients (HLHS, 5,582 ± 3,286 mL; non-HLHS, 3,405 ± 2,533 mL; p = 0.06; odds ratio; 2.0). Readmission to treat chylous effusions, loss of sinus rhythm, actuarial freedom from death, all neurologic events, pacemaker insertion, and Fontan takedown were similar in both cohorts. CONCLUSIONS: Fenestration was associated with lower discharge oxygen saturations, but late outcomes in fenestrated and nonfenestrated patients are equivalent.

Atrioesophageal fistula after surgical unipolar radiofrequency atrial ablation for atrial fibrillation.

Atrial fibrillation is the most common arrhythmia in the United States. Procedural treatment options such as the MAZE procedure and radiofrequency ablation are available with reasonable success rates. However, there are risks inherent to these procedures, with atrioesophageal fistula being a rare but devastating complication. We report a patient with atrioesophageal fistula who presented with neurologic symptoms 20 days after her initial MAZE procedure, followed by a quick decline within 24 hours of presentation.