National outcomes of the Fontan operation with endocardial cushion defect.

BACKGROUND: The traditional outcomes of the Fontan operation (FO) in endocardial cushion defect (ECD) patients have been suboptimal. Previous studies have been limited by the smaller number of ECD patients, longer study period with an era effect, and do not directly compare short-term outcomes of FO in ECD patients with non-ECD patients. Our study aims to address these shortcomings. METHODS: A retrospective analysis of the Kids Inpatient Database (2009, 2012, and 2016) for the FO was done. The groups were divided into those who underwent FO with ECD as compared to non-ECD diagnosis. The data were abstracted for demographics, clinical characteristics, and operative outcomes. Standard statistical tests were used. RESULTS: Three thousand three hundred eighty patients underwent the FO of which 360 patients (11%) were FO-ECD. ECD patients were more likely to have Down syndrome, Heterotaxy syndrome, transposition/DORV, and TAPVR as compared to non-ECD patients. FO-ECD had a higher discharge-mortality (2.84% vs. 0.45%, p = .04). The length of stay (16 vs. 13 days, p = .05) and total charges incurred ($283, 280 vs. $234, 106, p = .03) for the admission were higher in the FO-ECD as compared to non-ECD patients. In multivariable analysis, ECD diagnosis, cardiac arrest, acute kidney injury, and postoperative hemorrhage were predictors of mortality. CONCLUSION: Contemporary outcomes for FO are excellent with very low overall operative mortality. However, the outcomes in ECD patients are inferior with higher operative mortality than in non-ECD patients. The occurrence of postoperation complications and a diagnosis of ECD were predictive of a negative outcome.

National Fontan Operation short-term outcomes at or below 2-years-of-age compared to older than 2-years-of-age.

INTRODUCTION: Opinion is divided about optimal early timing of the Fontan Operation (FO). While some studies have suggested 3 years-of-age, others have shown good outcomes below 2 years-of-age. We analyzed the impact of age ≤2 years as compared age >2 years on short-term outcome of the FO using a large national database. METHODS: A retrospective analysis of the Kids Inpatient Database (2009-16) for the FO was done. The groups were divided into those who underwent FO at age ≤2 years (Early FO [EF]) as compared to age >2 years (Late FO [LF]). The data was abstracted for demographics, clinical characteristics, and operative outcomes. Standard statistical tests were used. RESULTS: A total of 3381 patients underwent FO during this period of which 1482 (44%) were EF. The mean ages of the EF and LF were 1.6 and 4.3, respectively (p < .001). LF were more likely to be non-White, female, and have Heterotaxy syndrome. HLHS was more common in EF. There was no difference in the discharge mortality, length of stay, disposition (majority went home), and mean total charges incurred. The overall discharge mortality was low at 0.7% (24/3381). In multivariate analysis: cardiac arrest, acute kidney injury, mechanical ventilation >96 h, endocardial cushion defect and non-White ethnicity were predictors of a mortality and not age. CONCLUSION: Contemporary outcomes for FO are excellent with equivalent short-term outcomes in both the age groups. Occurrence of postoperative complications, non-White ethnicity and endocardial cushion defect diagnosis were predictive of a negative outcome.

Cor triatriatum sinister: Long-term surgical outcomes in children and a proposal for a new classification.

BACKGROUND: Cor Triatriatum Sinister (CTS) is a rare congenital anomaly with an estimated incidence of 0.4%, resulting from abnormal left atrial septation. It may present in isolation or in association with other heart defects. High percentage of patients require surgery with low mortality and recurrence rates. CTS has been classified in the past however we aim to describe a case series with more comprehensive and inclusive classification. METHODS: This was a single-center retrospective cohort study of 16 children with the diagnosis of CTS between 2000 and 2020. Medical records were reviewed for clinical presentations, hospital, and postoperative courses. RESULTS: Sixteen patients (63% female), with a median age at diagnosis of 4.3 months, five (31%) were neonates. Six (38%) had isolated CTS, two (13%) with functional single ventricle (SV), and the remaining eight patients (50%) had other associated heart defects (septal defects in three, coarctation of the aorta in another three, and anomalous pulmonary venous connections in three). Eight patients (50%) presented with obstructed CTS. Twelve patients (75%) underwent surgical intervention. Mortality occurred in three patients (19%) with two surgical (one with total anomalous pulmonary venous connection and another with SV) and one nonsurgical (septal defect with Fanconi anemia). The surgical median follow up was 4.7 years. Recurrence of the membrane occurred in two patients (17%). CONCLUSIONS: This study showed good long-term outcomes for patients with isolated and complex CTS. Complete and proper classification of CTS ensures appropriate diagnosis, surgical planning, and better family counseling which may correlate with outcomes.

Descending thoracic aortic homograft for superior vena caval translocation in the Warden procedure: Report of six cases.

BACKGROUND: Warden procedure has been described for repair of partial anomalous pulmonary venous connections inserted high into the superior vena cava. In the current report, we describe a new modification. METHODS: A total of six patients (five females, 83%) with a mean age of 18.8 ± 10.8 years, underwent a modified Warden procedure with a descending thoracic aortic (DTA) homograft connected to the free wall of the right atrium, rather than to the right atrial appendage in the period between June 2021 and July 2022. RESULTS: All patients had evidence of enlarged right-sided cardiac chambers. Two patients had intact interatrial septum (33.3%), while three patients had sinus venosus (50%) and one had secundum atrial septal defects (16.7%). The procedure was performed via minimally invasive right thoracotomy in half of the patients. Patients were extubated in the operating room or soon after. No early mortality or reoperation. One patient required concomitant aortic root replacement and one needed repair of anomalous left upper pulmonary vein connection to the left innominate vein. Follow-up was complete in all patients with a mean of 6.2 ± 4.6 months. No late mortality or reoperation. All patients were discharged on antiplatelet therapy. Follow-up imaging studies showed satisfactory repair with widely patent superior vena caval and pulmonary venous pathways. CONCLUSIONS: The use of DTA homograft to facilitate superior vena caval translocation in the modified Warden procedure appears to be beneficial as it decreases tension on the anastomosis and avoids the need for anticoagulation.

Successful rapid staged hybrid approach for obstructed total anomalous pulmonary venous connection.

Obstructed total anomalous pulmonary venous connection (TAPVC) represents a true pediatric cardiac emergency. The patient may present in extremis secondary to severe pulmonary hypertension and cardiogenic shock which increases perioperative mortality. We present a neonate who underwent a successful staged hybrid approach for an Infradiaphragmatic obstructed TAPVC.

Alagille Syndrome and Repeat Oxygenator Failure during Cardiopulmonary Bypass: A Word of Caution.

Alagille syndrome is an autosomal dominant disorder that is caused by heterozygous mutation of JAG1 or NOTCH2 gene that impacts several multisystem organs including but may not be limited to the liver, heart, musculoskeletal, skin, and the eyes. The most common congenital heart defect associated with Alagille syndrome is multilevel right ventricular outflow tract obstruction with multiple central and peripheral branch pulmonary arterial stenoses occurring in up to two-thirds of these patients. We report two cases of Alagille syndrome who underwent extensive pulmonary arterial branch rehabilitation and experienced unusual oxygenator failure during cardiopulmonary bypass (CPB). We present lessons learned from these two cases and the changes that we implemented in our practice that facilitated smooth conduct of CPB in other cases that we performed subsequently.

Hirudotherapy for neonatal limb ischemia during ECMO support: A word of caution.

INTRODUCTION: Disordered coagulation, clot formation and distal limb ischemia are complications of extracorporeal membrane oxygenation (ECMO) with significant morbidity and mortality. Medicinal leech therapy (hirudotherapy) has been attempted in plastic and orthopedic surgeries to improve venous congestion and salvage ischemic flaps. To our knowledge this has not been reported in pediatric cardiac surgery or during ECMO support. We present a complex neonate whose ECMO course was complicated by distal limb ischemia for whom leech therapy was attempted. PATIENT AND INTERVENTION: A 2-week-old 2.7 kg infant required ECMO support secondary to perioperative multiorgan system dysfunction following repair of critical coarctation and ventricular septal defect. Despite systemic anticoagulation, his clinical course was complicated by arterial thrombus, vasopressor-induced vascular spasm and bilateral distal limb ischemia. Medicinal leech therapy was tried after initially failing conventional measures. RESULT: Following the third leech application, this patient developed significant hemorrhage from the web space adjacent to the left great toe. An estimated 450 ml of blood loss occurred and more than 300 ml of blood product transfusions were required. He ultimately progressed to irreversible systemic end organ dysfunction and comfort care was provided. CONCLUSION: The use of medicinal leech therapy in pediatric cardiac surgery may be considered to minimize the consequences of advanced limb ischemia and venous congestion. However, this should be used with caution while patients are systemically anticoagulated during ECMO support. A directed review is presented here to assist in determining optimal application and potential course of therapy.

Pulmonary valve replacement via left anterior minithoracotomy: Lessons learned and early experience.

OBJECTIVE: Median sternotomy has been the standard for pulmonary valve replacement (PVR) in patients with free pulmonary regurgitation (PR) and right ventricular enlargement. With the introduction of transcatheter therapy, the search for an alternate to sternotomy is mandated. We present our early experience with a limited anterior left thoracotomy approach. METHODS: We used a left anterior mini-thoracotomy in six male patients (15 ± 1.94 years of age) who developed progressive right ventricular enlargement due to chronic PR. RESULTS: Primary diagnoses were tetralogy of Fallot in five patients and pulmonary atresia with an intact septum in another. Four patients had previous median sternotomy with transannular patch repair. The mean right ventricular end-diastolic volume index was 189 ± 27.13 ml/m2 . The procedure was feasible in all patients. All patients had satisfactory adult size pulmonary bioprosthesis (25 or 27 mm valve), with a mean peak gradient of 18 ± 2.40 mmHg across the prosthesis at discharge. All patients were extubated intraoperatively at the end of the procedure and required no intraoperative transfusions. There were no early or late mortalities. Early morbidities included left hemidiaphragm paralysis in one patient, and re-sternotomy for prosthetic valve endocarditis in one. One patient required late reoperation for a common femoral artery pseudoaneurysm. CONCLUSIONS: Minimally invasive access for PVR is feasible in both primary and repeat settings, through a limited anterior left minithoracotomy in the absence of intracardiac shunts and the need for other concomitant cardiac procedures. Longer-term studies with a larger number of patients are needed to compare the efficacy of this approach to standard sternotomy.

Gore Cardioform atrial septal occluder: deployment procedure and techniques for closing challenging secundum atrial septal defects.

Ostium secundum atrial septal defects are mostly closed in the cardiac catheterisation laboratories using either Amplatzer® (Abbott Laboratories, IL) atrial septal occluder, Gore® Cardioform septal occluder and more recently using the recently approved (US FDA approval June 2019) Gore® Cardioform atrial septal defect occluder (W. L. Gore & Associates, AZ). Similar to any new device in the market, there is a learning curve to the deployment of this device. We therefore aim to report the key features about this new Gore Cardioform atrial septal defect occluder device with special emphasis on technical aspects that can be employed during transcatheter closure of challenging ostium secundum atrial septal defects using this device.

Current Interventional and Surgical Management of Congenital Heart Disease: Specific Focus on Valvular Disease and Cardiac Arrhythmias.

Successful outcome in the care of patients with congenital heart disease depends on a comprehensive multidisciplinary team. Surgery is offered for almost every heart defect, despite complexity. Early mortality for cardiac surgery in the neonatal period is ≈10% and beyond infancy is <5%, with 90% to 95% of patients surviving with a good quality of life into the adult years. Advances in imaging have facilitated accurate diagnosis and planning of interventions and surgical procedures. Similarly, advances in the perioperative medical management of patients, particularly with intensive care, has also contributed to improving outcomes. Arrhythmias and heart failure are the most common late complications for the majority of defects, and reoperation for valvar problems is common. Lifelong surveillance for monitoring of recurrent or residual structural heart defects, as well as periodic assessment of cardiac function and arrhythmia monitoring, is essential for all patients. The field of congenital heart surgery is poised to incorporate new innovations such as bioengineered cells and scaffolds that will iteratively move toward bioengineered patches, conduits, valves, and even whole organs.

Hypoplastic Left Heart Syndrome: An Overview for Primary Care Providers.

Hypoplastic left heart syndrome is one of the most complex congenital heart diseases and requires several cardiac surgeries for survival. The diagnosis is usually established prenatally or shortly after birth. Each stage of surgery poses a unique hemodynamic situation that requires deeper understanding to manage common pediatric problems such as dehydration and respiratory infections. Careful multidisciplinary involvement in the care of these complex patients is improving their outcome; however, morbidity and mortality are still substantial. In this review, we focus on the hemodynamic aspects of various surgical stages that a primary care provider should know to manage these challenging patients.

Outcomes of hospitalization in adults with Fontan palliation: The Mayo Clinic experience.

The outcomes of hospitalization in the Fontan population have not been specifically studied. The purpose of this study was to describe outcomes of hospitalization (frequency and indications for hospitalization, and in-hospital mortality) in this population and to determine how these outcomes differ from those of other adults with congenital heart disease (CHD). METHODS: This was a retrospective study of adult Fontan patients hospitalized at Mayo Clinic Rochester in 1990-2015. We selected age- and gender-matched control group of patients with repaired CHD and biventricular circulation hospitalized within the study period. RESULTS: A total of 367 Fontan patients (age 31±7 years and 259 [71%] with atriopulmonary Fontan) had 853 hospital admissions in 4 years (58 hospitalizations per 100 patient-years). The most common indications were arrhythmia (n=188, 22%), heart failure (n=169, 20%), and cardiac surgery (n=133, 16%). Overall in-hospital mortality was 4% (n=38), and the highest in-hospital mortality occurred in patients hospitalized for cardiac surgery (n=15, 11%) and heart failure (n=13, 8%). In comparison to the repaired CHD and biventricular circulation group, the Fontan group had more frequent hospitalizations (22 vs 58 per 100 patient-years, P<.001) and higher overall in-hospital mortality (1% vs 5%, P<.001), mortality after cardiac surgery (2% vs 11%, P=.01), and mortality for heart failure-related hospitalizations (2% vs 8%, P=.04). CONCLUSIONS: Adults with Fontan palliation had more frequent hospitalization and in-hospital mortality compared to the rest of the CHD population. Arrhythmia and heart failure were the most common indications for hospitalization. Perhaps optimal management of heart failure and arrhythmia may improve outcomes in this population.

Risk stratification and clinical outcomes after surgical pulmonary valve replacement.

BACKGROUND: To determine if RV volume was predictive of survival and cardiovascular adverse event (CAE) after pulmonary valve replacement (PVR). METHODS: We reviewed the MACHD (Mayo Adult Congenital Heart Disease) database for patients with tetralogy of Fallot (TOF) undergoing PVR, 2000-2015. The patients were divided into quartiles based on RV end-diastolic volume index (RVEDVI); those in the lowest quartile (Group A, n = 46) and the top quartile (Group B, n = 42) were selected as the study cohort. RESULTS: In comparison to Group A, Group B patients were older at time of PVR (28 ±â€¯4 vs 33 ±â€¯5 years, P = .011) and had larger RV volumes (RVEDVI 127 [117-138] mL/m2 vs 1 91 [179-208], P < .001; RVESVI 64 [57-73] mL/m2 vs 122 [103-136], P < .001). A total of 28 CAE occurred in 23 patients during 69 (33-94) months follow-up: death (n = 4), heart transplant listing (n = 1), initiation of palliative care (n = 1), heart failure hospitalization (n = 11), stroke (n = 2) and sustained ventricular tachycardia/aborted sudden cardiac death (n = 9). Survival was similar between Groups A and B (95% vs 91% at 10 years, P = .273) but freedom from CAE was significantly lower in Group B (67% vs 36% at 10 years, P = .002). Combination of RVESVI: >95 mL/m2 and tricuspid annular plane systolic excursion/RV systolic pressure (TAPSE/RVSP) <0.4 predicted CAE with sensitivity of 67% and specificity of 92%. CONCLUSION: Patients undergoing PVR at larger RV volumes had similar survival but more overall CAE. A larger study population with a longer follow-up will be required to determine if early PVR provides survival benefit in the long-term.

Role of Doppler echocardiography for cardiac output assessment in Fontan patients.

BACKGROUND: To determine (1) correlation between Doppler stroke volume index (SVI) and cardiac magnetic resonance imaging (CMRI) SVI and (2) association between Doppler SVI and Fontan-associated diseases (FAD) and Fontan failure. METHODS: Review of Fontan patients who underwent same-day CMRI and transthoracic echocardiography (TTE), 2005 to 2015. We defined FAD as cardiac thrombus, protein-losing enteropathy, arrhythmia, and hospitalization for heart failure. Fontan failure was defined as Fontan conversion or revision, heart transplantation or listing, or death. RESULTS: Fifty-three patients with systemic left ventricle (LV) underwent 86 sets of TTE/CMRI. Mean (SD) age 31 (6) years. SVI (45 [16] vs 42 [13] mL/m2), CI (3.0 [1.1] vs 2.8 [0.8] L min-1 m-2), and ejection fraction (53 [4]% vs 51 [5]%) were similar for both modalities (P>.05 for all). Doppler SVI correlated with CMRI (r=0.68; P<.001). Sixteen patients had cirrhosis, and these patients had a higher CI (3.9 [0.9] vs 2.8 [1.0] L min-1 m-2; P<.01). Among the 37 patients without cirrhosis, Doppler SVI <39 mL/m2 was associated with FAD (odds ratio [OR], 2.11; 95% confidence limit, 1.26-3.14; P=.02); Fontan failure was more common in patients with CI was <2.5 L min-1 m-2 (3/9 [33%] vs 0/28 [0%], P=.01). Another 11 patients with systemic right ventricle (RV) underwent 17 sets of TTE/CMRI, mean (SD) age 17 (3) years, and CMRI SVI also correlated with Doppler SVI (r=0.75; P<.001). CONCLUSION: Doppler SVI correlated with CMRI SVI in patients with systemic LV and systemic RV. The association between output measures (SVI and CI) and FAD were seen only in single LV patients (single RV patients not assessed for this outcome due to small numbers). An association between low Doppler CI and Fontan failure was suggested in a small number of single LV patients.

Three-dimensional prototyping for procedural simulation of transcatheter mitral valve replacement in patients with mitral annular calcification.

INTRODUCTION: Three-dimensional (3D) prototyping is a novel technology which can be used to plan and guide complex procedures such as transcatheter mitral valve replacement (TMVR). METHODS: Eight patients with severe mitral annular calcification (MAC) underwent TMVR. 3D digital models with digital balloon expandable valves were created from pre-procedure CT scans using dedicated software. Five models were printed. These models were used to assess prosthesis sizing, anchoring, expansion, paravalvular gaps, left ventricular outflow tract (LVOT) obstruction, and other potential procedure pitfalls. Results of 3D prototyping were then compared to post procedural imaging to determine how closely the achieved procedural result mirrored the 3D modeled result. RESULTS: 3D prototyping simulated LVOT obstruction in one patient who developed it and in another patient who underwent alcohol septal ablation prior to TMVR. Valve sizing correlated with actual placed valve size in six out of the eight patients and more than mild paravalvular leak (PVL) was simulated in two of the three patients who had it. Patients who had mismatch between their modeled valve size and post-procedural imaging were the ones that had anterior leaflet resection which could have altered valve sizing and PVL simulation. 3D printed model of one of the latter patients allowed modification of anterior leaflet to simulate surgical resection and was able to estimate the size and location of the PVL after inserting a valve stent into the physical model. CONCLUSION: 3D prototyping in TMVR for severe MAC is feasible for simulating valve sizing, apposition, expansion, PVL, and LVOT obstruction.

Adult presentation of ALCAPA: Echo and CT diagnosis.

A healthy 20-year-old male presented with aborted sudden death. Both thoracic echocardiography and cardiac computed tomographic angiography demonstrated the characteristic findings of ALCAPA noting a dilated and tortuous right coronary artery, dilated coronary collateral arteries within the interventricular septum or along the surface of the heart, and visualization of the left coronary artery origin from the posterior aspect of the main pulmonary artery. Both imaging modalities demonstrated all three characteristic findings of this rare coronary anomaly and served as complementary imaging studies for surgical correction.

Outcomes Following Three-Factor Inactive Prothrombin Complex Concentrate Versus Recombinant Activated Factor VII Administration During Cardiac Surgery.

OBJECTIVE: To compare outcomes following inactive prothrombin complex concentrate (PCC) or recombinant activated factor VII (rFVIIa) administration during cardiac surgery. DESIGN: Retrospective propensity-matched analysis. SETTING: Academic tertiary-care center. PARTICIPANTS: Patients undergoing cardiac surgery requiring cardiopulmonary bypass who received either rFVIIa or the inactive 3-factor PCC. INTERVENTIONS: Outcomes following intraoperative administration of rFVIIa (263) or factor IX complex (72) as rescue therapy to treat bleeding. MEASUREMENTS AND MAIN RESULTS: In the 24 hours after surgery, propensity-matched patients receiving PCC versus rFVIIa had significantly less chest tube outputs (median difference -464 mL, 95% confidence interval [CI] -819 mL to -110 mL), fresh frozen plasma transfusion rates (17% v 38%, p = 0.028), and platelet transfusion rates (26% v 49%, p = 0.027). There were no significant differences between propensity-matched groups in postoperative stroke, deep venous thrombosis, pulmonary embolism, myocardial infarction, or intracardiac thrombus. Postoperative dialysis was significantly less likely in patients administered PCC versus rFVIIa following propensity matching (odds ratio = 0.3, 95% CI 0.1-0.7). No significant difference in 30-day mortality in patients receiving PCC versus rFVIIa was present following propensity matching. CONCLUSIONS: Use of rFVIIa versus inactive PCCs was significantly associated with renal failure requiring dialysis and increased postoperative bleeding and transfusions.

The Various Applications of 3D Printing in Cardiovascular Diseases.

PURPOSE OF REVIEW: To highlight the various applications of 3D printing in cardiovascular disease and discuss its limitations and future direction. RECENT FINDINGS: Use of handheld 3D printed models of cardiovascular structures has emerged as a facile modality in procedural and surgical planning as well as education and communication. Three-dimensional (3D) printing is a novel imaging modality which involves creating patient-specific models of cardiovascular structures. As percutaneous and surgical therapies evolve, spatial recognition of complex cardiovascular anatomic relationships by cardiologists and cardiovascular surgeons is imperative. Handheld 3D printed models of cardiovascular structures provide a facile and intuitive road map for procedural and surgical planning, complementing conventional imaging modalities. Moreover, 3D printed models are efficacious educational and communication tools. This review highlights the various applications of 3D printing in cardiovascular diseases and discusses its limitations and future directions.

Outcomes of cardiac pacing in adult patients after a Fontan operation.

BACKGROUND: Cardiac pacing can be challenging after a Fontan operation, and limited data exist regarding pacing in adult Fontan patients. The objectives of our study were to determine risk factors for pacing and occurrence of device-related complications (DRCs) and pacemaker reinterventions. METHODS: We performed a retrospective review of Fontan patients from 1994 through 2014. We defined DRCs as lead failure, lead recall, cardiac perforation, lead thrombus/vegetation, or device-related infection, and cardiovascular adverse events (CAEs) as venous thrombosis, stroke, death, or heart transplant. Pacemaker reintervention was defined as lead failure or recall. RESULTS: Of 439 patients, 166 (38%) had pacemakers implanted (79 during childhood; 87, adulthood); 114 patients (69%) received epicardial leads initially, and 52 (31%), endocardial leads. Pacing was initially atrial in 52 patients (31%); ventricular, 30 (18%); or dual chamber, 84 (51%). There were 37 reinterventions (1.9% per year) and 48 DRCs (2.4% per year). Pacemaker implantation during childhood was a risk factor for DRCs (hazard ratio, 2.01 [CI, 1.22-5.63]; P = .03). There were 70 CAEs (venous thrombosis, 5; stroke, 11; transplant, 8; and death, 46), yielding a rate of 3.5% per year. DRCs, CAEs, and reintervention rates were comparable for patients with epicardial or endocardial leads. CONCLUSIONS: More than one-third of adult Fontan patients referred to Mayo Clinic had pacemaker implantation. Epicardial leads were associated with high rate of pacemaker reinterventions but similar DRC rates in comparison to endocardial leads.