Superior cavopulmonary shunt via right vertical axillary mini-thoracotomy.

We present 2 cases of patients with univentricular heart physiology of different anatomic variants who successfully underwent minimally invasive bidirectional cavopulmonary anastomosis via the right vertical axillary mini-thoracotomy.

Extremely Long-Term Follow-Up of Dr William Glenn's Original Patient Cohort With Superior Cavopulmonary Anastomosis (1958-1990).

BACKGROUND: Historically, Dr William Glenn performed the first classic superior cavopulmonary anastomosis in a seven-year-old child at Yale in 1958. By 1990, this operation was performed consecutively in over 90 patients. With over 60 years of follow-up, this is the longest survival record of early Glenn patients from the first 30 years. METHODS: We performed a single center, retrospective evaluation of patients undergoing a Glenn operation. A collected list of surviving patients, previously updated in 1988, included demographics, age at procedure, and underlying diagnosis. Follow-up data were obtained in May 2022 using electronic medical records to determine survival, age of survivors, and age of deceased. RESULTS: Ninety-five patients underwent the Glenn operation from 1958 to 1990: 58.9% (n = 56) were male and 41.1% (n = 39) female. Fifteen patients were lost to follow-up, but 12 were alive in 1988. Sixty patients were deceased (68.1%), with an average age of 33.5 ± 18.3(range, 2-78, excluding seven early deaths) years. The oldest patient who passed away was a 78-year-old male with tetralogy of Fallot. Twenty patients remain alive, with an average age of 47.5 (range, 32-66) years. Four patients who are still alive today (20% survivors) are older than 60 years. CONCLUSIONS: Since Dr Glenn's original operation, the technique, timing, and indications have been modified (ie, bidirectional Glenn) to adapt to the current era. By following this initial group of patients, we can approach completion of the survival rates for adult congenital patients who were some of the first pediatric patients to receive this ground-breaking palliative procedure.

Textbook Outcome for Superior Cavopulmonary Connection: A Metric for Single Ventricle Heart Surgery.

Background: To develop a more holistic measure of congenital heart center performance beyond mortality, we created a composite "textbook outcome" (TO) for the Glenn operation. We hypothesized that meeting TO would have a positive prognostic and financial impact. Methods: This was a single center retrospective study of patients undergoing superior cavopulmonary connection (bidirectional Glenn or Kawashima ± concomitant procedures) from 2005 to 2021. Textbook outcome was defined as freedom from operative mortality, reintervention, 30-day readmission, extracorporeal membrane oxygenation, major thrombotic complication, length of stay (LOS) >75th percentile (17d), and mechanical ventilation duration >75th percentile (2d). Multivariable logistic regression and Cox proportional hazards modeling were used. Results: Fifty-one percent (137/269) of patients met TO. Common reasons for TO failure were prolonged LOS (78/132, 59%) and ventilator duration (67/132, 51%). In multivariable analysis, higher weight [odds ratio, OR: 1.44 (95% confidence interval, CI: 1.15-1.84), P = .002] was a positive predictor of TO achievement while right ventricular dominance [OR 0.47 (0.27-0.81), P = .007] and higher preoperative pulmonary vascular resistance [OR 0.58 (0.40-0.82), P = .003] were negative predictors. After controlling for preoperative factors and excluding operative mortalities, TO achievement was independently associated with a decreased risk of death over long-term follow-up [hazard ratio: 0.50 (0.25-0.99), P = .049]. Textbook outcome achievement was also associated with lower direct cost of care [$137,626 (59,333-167,523) vs $262,299 (114,200-358,844), P < .0001]. Conclusion: Achievement of the Glenn TO is associated with long-term survival and lower costs and can be predicted by certain risk factors. As outcomes continue to improve within congenital heart surgery, operative mortality will become a less informative metric. Textbook outcome analysis may represent a more balanced measure of a successful outcome.

The Evolution of Longitudinal Strain Across Various Univentricular Morphologies Prior to Superior Cavopulmonary Anastomosis.

BACKGROUND: Infants with single-ventricle physiology have increased morbidity, mortality, and ventricular dysfunction in the pre-superior cavopulmonary anastomosis (pre-SCPA) period. Echocardiography-derived longitudinal strain (LS) is emerging as a reliable marker of single-ventricle performance. We aim to assess evolution of LS during the pre-SCPA period across univentricular morphologies and explore associations of LS with modifiable and nonmodifiable factors. METHODS: Ninety-four term infants (36 females) with univentricular physiology who were discharged home prior to stage 2 palliation were serially analyzed for LS (single apical view) and other echo measures at initial hospital discharge and the last pre-SCPA encounter. Ventricular myocardium was tracked for strain along the septum and respective lateral wall for single right ventricular (RV) and left ventricular (LV) groups, and along both right and left lateral walls in functionally univentricular hearts with biventricular (BiV) morphology. Clinical data were obtained from the medical record. RESULTS: Longitudinal strain improved in the total cohort during the pre-SCPA period (16.48% ± 3.31% to 17.57% ± 3.81%, P = .003). Longitudinal strain improved between encounters in the single LV (P = .04) and BiV groups (P = .02). However, LS failed to improve in the RV group (P = .7) with lower LS at both visits compared with the other groups. The RV group, mostly composed of hypoplastic left heart syndrome patients-87% of cases-had a higher incidence of arrhythmias (57%) and unplanned reinterventions (60%), most of which were arch reinterventions. A subanalysis based on arch reintervention showed that LS improves between encounters in the single LV group needing arch reintervention (P = .05) compared with the single RV group needing arch reintervention (P = .89). Lower LS was independently associated with unplanned reinterventions at both encounters (P = .008 and .02). CONCLUSIONS: Single-ventricle LS evolves differently across ventricular morphologies during the pre-SCPA period and is related to the need for unplanned cardiac reinterventions. Lower LS is noted in the single RV group, who mostly have hypoplastic left heart syndrome.

Contemporary Early Postoperative Cone Repair Outcomes for Patients With Ebstein Anomaly.

OBJECTIVE: To describe the early postoperative outcomes after cone repair (CR) for Ebstein anomaly (EA) across the age spectrum. PATIENTS AND METHODS: For this study, 284 patients from 1 to 73 years of age who underwent CR at Mayo Clinic from June 1, 2007, to December 21, 2018, were separated by age group (1-<4, 4-<19, 19-<40, and 40+ years) and by disease severity for analysis. Outcomes of interest included death, reoperation, readmission, early postoperative complications, cardiac intensive care unit and hospital length of stay, and need for superior cavopulmonary anastomosis. RESULTS: Mortality within 30 days was 0%. The reoperation rate was 4.9% (n=14) and the median hospital length of stay was 5 days, with no statistical difference between ages at time of CR or severity groups. The readmission rate was 2% (n=6). Postoperative complications were seen in 8.8% (n=25) of cases overall, with higher rates in the youngest age group (21%, P<.001). Superior cavopulmonary anastomosis was most common in the youngest age group (37% vs 17% overall, P<.001) and in those with severe disease (35%, P<.001). CONCLUSION: Children and adults with Ebstein anomaly have very good early postoperative outcomes with a less than 10% complication and reoperation rate and very low mortality following cone reconstruction. In the setting of good and stable right ventricle function and no symptoms of heart failure or cyanosis, waiting for CR until 4 years of age may minimize early postoperative complications and need for superior cavopulmonary anastomosis.

Common arterial trunk in functionally univentricular hearts: a case series.

INTRODUCTION: The association of a univentricular heart defect with common arterial trunk is extremely rare. There is a lack of population-based outcome studies reported in the literature. METHODS: The hospital records, echocardiographic and other imaging modality data, outpatients' records, operation notes, and other electronic data were reviewed. Patients were reviewed, and the final outcomes of surgery were observed. RESULTS: Six cases (two males) with common arterial trunk presented over a 30-year period. Five had a complete unbalanced atrioventricular septal defect (83%) and one (17%) had tricuspid atresia associated with common arterial trunk. All had antenatal diagnosis. Two cases (33%) were excluded from initial surgical palliation due to Trisomy 21 in one and severe truncal valve regurgitation in one. Initial surgical palliation was performed in four cases (67%) at median age of 31 days (2-60) and consisted of disconnection and reconstruction of the pulmonary arteries and establishing controlled pulmonary blood flow. There were no early deaths. Conversion to cavopulmonary shunt was not possible in two due to severe airway problems in one and pulmonary arteries anatomy in one. They died at 11 and 16 months, respectively. Two patients (33%) underwent cavopulmonary shunt with 1 (17%) being alive at 18 months - 12 months after cavopulmonary shunt. The second patient proceeded to Fontan completion at 19 months but required catheter takedown 3 months later and died 3.5 years later. CONCLUSIONS: Univentricular hearts with common arterial trunk carry extremely poor short- to medium-term outcomes. This should inform antenatal and postnatal counselling and decision-making.

HeartMate 3 VAD in a Single-Ventricle Patient Palliated With a Superior Cavopulmonary Anastomosis.

We describe a 9-year-old, 25-kg child with failing single-ventricle palliation with a superior cavopulmonary anastomosis successfully supported with a HeartMate 3 ventricular assist device (Abbott) as a bridge to transplantation.

Increased Circulating Endothelin 1 Is Associated With Postoperative Hypoxemia in Infants With Single-Ventricle Heart Disease Undergoing Superior Cavopulmonary Anastomosis.

Background Inadequate pulmonary vascular growth results in morbidity for many children with single-ventricle heart disease (SVHD). Endothelin 1 (ET1) is a potent vasoconstrictor and stimulator of pulmonary artery smooth muscle proliferation. Circulating ET1 levels and their association with outcomes have not been studied during early SVHD palliation. We aimed to define circulating levels of ET1 in patients with SVHD undergoing stage 2 palliation and evaluate their relationship to postoperative hypoxemia. We hypothesized that patients with SVHD with higher ET1 concentration would have a greater post-stage 2 hypoxemia. Methods and Results Prospective cohort study of 55 subjects with SVHD undergoing stage 2 palliation and 50 controls. Samples for ET1 analysis were collected at preoperation (systemic and pulmonary vein) and 2, 24, and 48 hours postoperation for cases and a single time point for controls. The primary outcome was percentage of first 48 postoperative hours with clinically significant hypoxemia (saturation, <70%). ET1 concentration was lower in preoperative cases than controls (2.2 versus 2.7 pg/mL; P=0.0015) and in the pulmonary vein than systemic vein (1.7 versus 2.2 pg/mL; P<0.001). ET1 level increased by 2 hours postoperation and trended back to baseline by 48 hours. Higher preoperative pulmonary vein ET1 and 2 hours postoperative ET1 were associated with larger hypoxemia burden (10.6% versus 2.7% [P=0.0081]; and 7.6% versus 3.2% [P=0.01], respectively). Multivariable testing demonstrated ET1 concentration and cardiopulmonary bypass time were associated with hypoxemia, whereas catheterization measurements and clinical variables were not. Conclusions Infants with SVHD with higher perioperative ET1 concentration experience more post-stage 2 hypoxemia. ET1 activity may be a modifiable risk factor of pulmonary vascular inadequacy for stage 2 palliation.

Combined Norwood and cavopulmonary shunt as the first palliation in late presenters with hypoplastic left heart syndrome and single-ventricle lesions.

OBJECTIVE: A primary cavopulmonary shunt as a component of the initial Norwood palliation could be an option in patients with hypoplastic left heart syndrome and single-ventricle lesions. We present our initial experience with this approach in carefully selected patients with unrestricted pulmonary blood flow and low pulmonary vascular resistance. METHODS: The study included 16 patients; the mean age was 137.9 ± 84.2 days. All patients underwent a Norwood palliation consisting of atrial septectomy, Damus-Kaye-Stansel connection, and arch augmentation in addition to the cavopulmonary shunt as the initial palliation. RESULTS: The mean preoperative pulmonary to systemic blood flow (Qp/Qs) ratio on room air (n = 9) and with 100% oxygen (n = 8) was 5.3 ± 3.2 and 8.6 ± 4.3, respectively. The mean pulmonary vascular resistance on room air (n = 10) and 100% oxygen (n = 9) was 4.8 ± 3.1 and 1.7 ± 0.97 WU/m2, respectively. Delayed chest closure was needed in 12 patients, and 6 patients required postoperative inhaled nitric oxide. One patient underwent takedown of the cavopulmonary shunt and construction of the right ventricle to pulmonary artery conduit after 1 month. The mean intensive care unit stay was 18.9 ± 15.4 days. There were 2 in-hospital deaths (48 hours and 8 days after surgery) and 2 postdischarge deaths (6 months and 2 years after hospital discharge). Seven patients have undergone the Fontan completion successfully, and 5 patients await further surgery. CONCLUSIONS: First-stage Norwood palliation with cavopulmonary shunt for patients with hypoplastic left heart syndrome or single-ventricle lesions is feasible in late presenters with low pulmonary vascular resistance.

Purpose-Built Transcatheter Cavopulmonary Anastomosis Device Requirements: Multi-Modality Imaging Evaluation.

BACKGROUND/PURPOSE: Patients with a functional single ventricle undergo multiple, palliative open-heart surgeries. This includes a superior cavopulmonary anastomosis or bidirectional Glenn shunt. A less-invasive transcatheter approach may reduce morbidity. METHODS/MATERIALS: We analyzed pre-Glenn X-ray contrast angiography (XA), cardiac computed tomography (CT), and cardiac magnetic resonance (CMR) studies. RESULTS: Over an eleven-year period (1/2007 - 6/2017), 139 Glenn surgeries were performed at our institution. The typical age range at surgery was 59 - 371 days (median = 163; IQR = 138 - 203). Eight-nine XA, ten CT, and ten CMR studies obtained from these patients were analyzed. Cephalad SVC measurements (millimeters) were 7.3 ± 1.7 (XA), 7.7 ± 1.6 (CT) and 6.9 ± 1.8 (CMR). RPA measurements were 7.3 ± 1.9 (XA), 7.4 ± 1.6 (CT) and 6.6 ± 1.9 (CMR). Potential device lengths were 10.9 ± 6 - 17.4 ± 6.4 (XA), 10.1 ± 2.1 - 17.7 ± 2.4 (CT) and 17.3 ± 4. - 23.7 ± 5.5 (CMR). SVC-RPA angle (degrees) was 132.9 ± 13.2 (CT) and 140 ± 10.2 (MRI). Image quality of all CT (100%), almost all XA (SVC 100%, RPA 99%), and most MRI (SVC 80%, RPA 90%) were deemed sufficient. Parametric modeling virtual fit device with 10 mm diameter and 20 - 25 mm length was ideal. CONCLUSIONS: Ideal transcatheter cavopulmonary shunt device for the typical patient would be 10 mm in diameter and 20-25 mm in length.

Factors associated with mortality or transplantation versus Fontan completion after cavopulmonary shunt for patients with tricuspid atresia.

OBJECTIVE: Tricuspid atresia with normally related great vessels (TA) is considered the optimal substrate for the Fontan pathway. The factors associated with death or transplantation after cavopulmonary shunt (CPS) are underappreciated. We aimed to determine factors associated with CPS-Fontan interstage death/transplantation versus transition to Fontan in TA. METHODS: A total of 417 infants younger than 3 months of age with TA were enrolled (January 1999 to February 2020) from 40 institutions into the Congenital Heart Surgeons' Society TA cohort. Parametric competing risk methodology was used to determine factors associated with the competing end points of death/transplantation without Fontan completion, and transition to Fontan. RESULTS: CPS was performed in 382 patients with TA; of those, 5% died or underwent transplantation without transition to Fontan and 91% transitioned to Fontan by 5 years after CPS. Prenatal diagnosis (hazard ratio [HR], 0.74; P < .001) and pulmonary artery band (PAB) at CPS (HR, 0.50; P < .001) were negatively associated with Fontan completion. Preoperative moderate or greater mitral valve regurgitation (HR, 3.0; P < .001), concomitant mitral valve repair (HR, 11.0; P < .001), PAB at CPS (HR, 3.0; P < .001), postoperative superior vena cava interventions (HR, 9.0; P < .001), and CPS takedown (HR, 40.0; P < .001) were associated with death/transplantation. CONCLUSIONS: The mortality rate after CPS in patients with TA is notable. Those with preoperative mitral valve regurgitation remain a high-risk group. PAB at the time of CPS being associated with both increased risk of death and decreased Fontan completion may represent a deleterious effect of antegrade pulmonary blood flow in the CPS circulation.

Exercise capacity after total cavopulmonary anastomosis: a longitudinal paediatric and adult study.

AIMS: Fontan palliation is a surgical strategy for patients with complex congenital heart disease, in whom biventricular circulation cannot be achieved. Long-term survival is negatively affected by the absence of sub-pulmonary ventricle and increased systemic venous pressure. Exercise capacity is a known predictor of overall survival and quality of life in congenital heart defects. We aim to track individual trends of peak oxygen uptake (V̇O2 peak) after total cavopulmonary connection (TCPC), identify predictors of deterioration, and derive a disease-specific reference V̇O2 peak dataset. METHODS AND RESULTS: A retrospective study of serial cardiopulmonary exercise testing (CPET) data, gathered from all patients who underwent TCPC in the Czech Republic between 1992 and 2016. Of 354 consecutive patients with TCPC, 288 (81.4%) patients underwent one or more CPETs yielding 786 unique V̇O2 peak values used as a reference dataset. Longitudinal data were available in 206 (58.2%) patients, who underwent a median (inter-quartile range) of 3.0 (2.0-5.0) CPETs over a mean (standard deviation) of 8.9 (5.5) years. The decline of exercise capacity with age was linear and not faster than in healthy peers (P = 0.47), but relative values of V̇O2 peak in TCPC patients were 12.6 mL/min/kg lower. Single ventricular morphology and pulmonary artery size had no significant influence on the exercise capacity dynamics. V̇O2 peak decline correlated negatively with the trend of body mass index z-score (P = 0.006) and was faster in women than men (P = 0.008). CONCLUSIONS: Total cavopulmonary connection patients have significantly reduced exercise capacity. The age-related decline paralleled the healthy population and correlated negatively with the body mass index trend. The presented V̇O2 peak reference dataset may help the clinicians to grade the severity of exercise capacity impairment in individual TCPC patients.

Miniaturized Fontan Circulation Assist Device: Chronic In Vivo Evaluation.

We have miniaturized and optimized our implantable rotary blood pump developed to provide long-term mechanical right heart support for patients who have failing Fontan circulation. The objective of this study was to evaluate the miniaturized Fontan circulation assist device (mini-FCAD) during 30-day sheep studies (n = 5). A complete right heart bypass was performed and all return flow was supported by the pump. Postoperatively, unfractionated heparin was given to maintain thromboelastography R times of 2× normal. The first two studies were terminated on day 0 and day 4 due to complications. In the final three studies, the animals remained healthy and were electively terminated at 30 ± 2 days. Pump flow was between 5 and 7 lpm, left atrial pressure remained normal, and inlet pressures were between 3 and 18 mm Hg with no incidents of suction. There was no evidence of hemolysis, end organ or pulmonary dysfunction, thromboembolic events, nor thermal damage to the surrounding tissue. Explanted devices from two studies were free of thrombi and in the third study there were unattached thrombi on the SVC inlet of the rotor. The mini-FCAD was successfully tested in vivo as a right heart replacement device demonstrating adequate circulatory support and normal physiologic pulmonary and venous pressures.

Glenn surgery: a safe procedure in the path of univentricular correction / Cirugía de Glenn: un procedimiento seguro en el camino de la corrección univentricular

Abstract Background: This study describes 35 years of experience in a tertiary care level hospital that treats cardiac patients with univentricular heart physiology who underwent Glenn surgery. Methods: The study consisted of a retrospective analysis of patients who underwent Glenn surgery, including variables related to pre-operative, intra-operative, and post-operative morbidity and mortality. Results: From 1980 to 2015, 204 Glenn surgeries were performed. The most common heart disease was tricuspid atresia IB (19.2%). In 48.1% of the cases, the procedure was performed with antegrade flow. A bilateral Glenn procedure was performed in 12.5% of the cases and 10.3% were carried out without using a cardiopulmonary bypass pump. Reported complications included infections, bleeding, arrhythmias, chylothorax, neurological alterations, and pleural effusion. The mortality rate was 2.9% Conclusions: Glenn surgery is a palliative surgery with good results. It significantly improves patient quality of life over a long period until a total cavopulmonary shunt is performed. The complications observed are few, and the mortality rate is low. Therefore, it is a safe surgery that should be used for univentricular congenital heart disease.

Resumen Introducción: El objetivo de este trabajo fue describir la experiencia de 35 años en un hospital de tercer nivel con pacientes cardiópatas con fisiología univentricular que fueron sometidos a cirugía de Glenn. Métodos: Se presenta un análisis retrospectivo de los pacientes sometidos a cirugía de Glenn. Se incluyeron variables relacionadas con la morbilidad y la mortalidad preoperatorias, transoperatorias y posoperatorias. Resultados: Desde 1980 hasta 2015 se realizaron 204 cirugías de Glenn para la corrección de cardiopatías. De ellas, la más frecuente fue la atresia tricuspídea IB (19.2%); en el 48.1% se realizó Glenn con flujo anterógrado, en el 12.5% Glenn bilateral y el 10.3% de las cirugías fueron sin apoyo de bomba de circulación extracorpórea. Las complicaciones reportadas fueron infecciones, sangrado, arritmias, quilotórax, alteraciones neurológicas y derrame pleural. La mortalidad fue del 2.9%. Conclusiones: La cirugía de Glenn es un procedimiento paliativo que se ha utilizado con buenos resultados. Mejora en forma importante la calidad de vida del paciente durante un largo periodo mientras que se realiza la derivación cavopulmonar total. Presenta pocas complicaciones y muy baja mortalidad, por lo que es una cirugía segura que debe ser utilizada para cardiopatías congénitas univentriculares.