Single-ventricle palliation in children with atrioventricular septal defect and transposition of the great arteries: 45 years of experience

AbstractBackground:The association of atrioventricular septal defect and transposition of the great arteries is very rare. As a rule, these patients have unbalanced ventricles. However, there have been no studies describing the results of single-ventricle palliation in these children.Methods:All children who underwent surgery with a diagnosis of atrioventricular septal defect and transposition of the great arteries were included in the study. Data were obtained from medical records.Results:A total of 38 patients with atrioventricular septal defect and transposition of the great arteries underwent single-ventricle palliation at the study institution between 1971 and 2016. The mean follow-up was 12.4 years (median: 14.6 years, range 2–43.3 years). Most children had unbalanced atrioventricular septal defect (94.7%, 36/38). Survival was 67.6% (95% confidence interval [CI]: 50.0–80.2%) at 10 years and 57.8% (95% CI: 38.0–73.4%) at 20 years. By 10 years, 58.6% (95% CI: 40.8–72.7%) had progressed to Fontan completion, while 32.5% (95% CI: 18.2–47.6%) had died. In patients achieving Fontan completion, 20-year event-free survival was 73.3% (95% CI: 34.8–91.3%), while 5.0% (95% CI: 0.4–20.5%) had undergone cardiac transplantation and 21.7% (95% CI: 3.2–50.8%) had undergone takedown of the Fontan circulation. Freedom from atrioventricular valve surgery was 57.0% (95% CI: 37.2–72.7%) at 10 and 20 years.Conclusions:The association of atrioventricular septal defect and transposition of the great arteries is very rare, and most of these children have unbalanced ventricles. Single-ventricle palliation results in 25-year overall survival of 50%. However, in patients, who had Fontan completion, survival was 75% at 25 years after Fontan operation.

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Echocardiographic preoperative evaluation of unbalanced atrioventricular septal defect

Bulgarian Cardiology ◽

10.3897/bgcardio.26.e52123 ◽

2020 ◽

Vol 26 (2) ◽

pp. 46-58

Author(s):

Zornitsa Vassileva ◽

Anna Kaneva

Keyword(s):

Congenital Heart ◽

Septal Defect ◽

Single Ventricle ◽

Preoperative Evaluation ◽

Atrioventricular Septal Defect ◽

Precise Determination ◽

Moderate Degree ◽

Surgical Strategies ◽

Single Ventricle Palliation

Unbalanced atrioventricular septal defect is seen by 10-15% of the patients with this congenital heart disease and poses serious challenges for the treating physicians regarding the choice of the most suitable operative intervention. The forms with moderate degree of unbalance between the two ventricles, especially when the size of the left ventricle is considered borderline, make the choice between two-ventricle correction and single-ventricle palliation quite difficult. The most important factor when judging the degree of unbalance is not the size of the ventricles but rather the redistribution of blood flow. The use of different echocardiographic measurements could contribute to the more precise determination of the indications for one of the two surgical strategies with resulting improvement of patient outcome.

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Faculty Opinions recommendation of Mid-term outcomes in unbalanced complete atrioventricular septal defect: role of biventricular conversion from single-ventricle palliation.

Faculty Opinions – Post-Publication Peer Review of the Biomedical Literature ◽

10.3410/f.727623526.793545037 ◽

2018 ◽

Author(s):

P Syamasundar Rao

Keyword(s):

Septal Defect ◽

Single Ventricle ◽

Atrioventricular Septal Defect ◽

Complete Atrioventricular Septal Defect ◽

Single Ventricle Palliation ◽

Complete Atrioventricular

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Prenatal diagnosis, associated findings and postnatal outcome of fetuses with double outlet right ventricle (DORV) in a single center

Journal of Perinatal Medicine ◽

10.1515/jpm-2018-0316 ◽

2019 ◽

Vol 47 (3) ◽

pp. 354-364 ◽

Cited By ~ 1

Author(s):

Ingo Gottschalk ◽

Judith S. Abel ◽

Tina Menzel ◽

Ulrike Herberg ◽

Johannes Breuer ◽

...

Keyword(s):

Prenatal Diagnosis ◽

Right Ventricle ◽

Single Ventricle ◽

Double Outlet Right Ventricle ◽

Biventricular Repair ◽

Cardiac Anomalies ◽

Postnatal Outcome ◽

The Mean ◽

Single Ventricle Palliation

Abstract Objective To assess the spectrum of associated anomalies, the intrauterine course, postnatal outcome and management of fetuses with double outlet right ventricle (DORV). Methods All cases of DORV diagnosed prenatally over a period of 8 years were retrospectively collected in a single tertiary referral center. All additional prenatal findings were assessed and correlated with the outcome. The accuracy of prenatal diagnosis was assessed. Results Forty-six cases of DORV were diagnosed prenatally. The mean gestational age at first diagnosis was 21+4 weeks (range, 13–37). A correct prenatal diagnosis of DORV was made in 96.3% of the cases. If the relation of the great arteries, the position of the ventricular septal defect (VSD) and additional cardiac anomalies are taken into account, the prenatal diagnosis was correct in 92.6% of the cases. One case was postnatally classified as transposition of the great arteries with subpulmonary VSD and was excluded from further analysis. A total of 41 (91.1%) fetuses with DORV had major additional cardiac anomalies, 30 (66.7%) had extracardiac anomalies and 13 (28.9%) had chromosomal or syndromal anomalies. Due to their complex additional anomalies, five (11.1%) of our 45 fetuses had multiple malformations and were highly suspicious for non-chromosomal genetic syndromes, although molecular diagnosis could not be provided. Disorders of laterality occurred in 10 (22.2%) fetuses. There were 17 terminations of pregnancy (37.8%), two (4.4%) intrauterine and seven (15.6%) postnatal deaths. Nineteen of 22 (86.4%) live-born children with an intention to treat were alive at last follow-up. The mean follow-up among survivors was 32 months (range, 2–72). Of 21 children who had already undergone postnatal surgery, eight (38.1%) achieved biventricular repair and 13 (61.9%) received univentricular palliation. One recently born child is still waiting for surgery. All children predicted prenatally to need a single ventricle palliation, and all children predicted to achieve biventricular repair, ultimately received the predicted type of surgery. After surgery, 14 of 18 (77.8%) children were healthy without any impairment. Conclusion DORV is a rare and often complex cardiac anomaly that can be diagnosed prenatally with high precision. DORV is frequently associated with major additional anomalies, leading to a high intrauterine and postnatal loss rate due to terminations or declined postnatal therapy. Without additional anomalies, the prognosis is good, although approximately 60% of children will have single ventricle palliation.

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Mid-term outcomes in unbalanced complete atrioventricular septal defect: role of biventricular conversion from single-ventricle palliation†

European Journal of Cardio-Thoracic Surgery ◽

10.1093/ejcts/ezx129 ◽

2017 ◽

Vol 52 (3) ◽

pp. 565-572 ◽

Cited By ~ 5

Author(s):

Meena Nathan ◽

Sitaram Emani ◽

Rinske IJsselhof ◽

Hua Liu ◽

Kimberlee Gauvreau ◽

...

Keyword(s):

Septal Defect ◽

Single Ventricle ◽

Atrioventricular Septal Defect ◽

Complete Atrioventricular Septal Defect ◽

Single Ventricle Palliation ◽

Complete Atrioventricular

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Single Ventricle Palliation in a Developing Sub-Saharan African Country: What Should be Improved?

World Journal for Pediatric and Congenital Heart Surgery ◽

10.1177/2150135118822671 ◽

2019 ◽

Vol 10 (2) ◽

pp. 164-170 ◽

Cited By ~ 3

Author(s):

Valdano Manuel ◽

Humberto Morais ◽

Aida L. R. Turquetto ◽

Gade Miguel ◽

Leonardo A. Miana ◽

...

Keyword(s):

Confidence Interval ◽

African Country ◽

Single Ventricle ◽

Hazard Ratio ◽

Stage I ◽

Stage Ii ◽

Stage Iii ◽

Lost To Follow Up ◽

Single Ventricle Palliation

Introduction: Single ventricle physiology management is challenging, especially in low-income countries. Objective: To report the palliation outcomes of single ventricle patients in a developing African country. Methods: We retrospectively studied 83 consecutive patients subjected to single ventricle palliation in a single center between March 2011 and December 2017. Preoperative data, surgical factors, postoperative results, and survival outcomes were analyzed. The patients were divided by palliation stage: I (pulmonary artery banding [PAB] or Blalock–Taussig shunt [BTS]), II (Glenn procedure), or III (Fontan procedure). Results: Of the 83 patients who underwent palliation (stages I-III), 38 deaths were observed (31 after stage I, six after stage II, and one after stage III) for an overall mortality of 45.7%. The main causes of operative mortality were multiple organ dysfunction due to sepsis, shunt occlusion, and cardiogenic shock. Twenty-eight survivors were lost to follow-up (22 after stage I, six after stage II). Thirteen stage II survivors are still waiting for stage III. The mean follow-up was 366 ± 369 days. Five-year survival was 28.4 % for PAB and 30.1% for BTS, while that for stage II and III was 49.8% and 57.1%, respectively. Age (hazard ratio, 0.61; 95% confidence interval: 0.47-0.7; P = .000) and weight at surgery (hazard ratio, 0.45; 95% confidence interval: 0.31-0.64; P = .002) impacted survival. Conclusion: A high-mortality rate was observed in this initial experience, mainly in stage I patients. A large number of patients were lost to follow-up. A task force to improve outcomes is urgently required.

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P3656Fontan operation performed in adult patients

European Heart Journal ◽

10.1093/eurheartj/ehz745.0513 ◽

2019 ◽

Vol 40 (Supplement_1) ◽

Author(s):

T Santos Monteiro ◽

C Cruz Lamas ◽

M C Terra Cola ◽

A J Oliveira Monteiro ◽

M Machado Melo ◽

...

Keyword(s):

Pulmonary Artery ◽

Fontan Operation ◽

Cardiopulmonary Exercise Testing ◽

Adult Patients ◽

Venous Blood Flow ◽

Inhospital Mortality ◽

Vo2 Max ◽

Venae Cavae ◽

The Mean

Abstract Introduction Treatment of patients with univentricular physiology is based on a sequence of palliative surgeries which end with the Fontan operation, when all venous blood flow is diverted to the lungs, bypassing the heart. Most centers advise to complete this process around 4 years of age, and there are few data about the performance of the Fontan operation in adults. Purpose To describe the results of the Fontan surgery when performed in adult patients. Methods A retrospective review of patients submitted to the Fontan operation between 2014 and 2018, with data collection from charts, regarding their pre-operative state and follow up, including improvement in exercise capacity and hemoglobin levels. Results There were 12 patients submitted to the Fontan operation in the study period, with mean age 24±5 years, 8 female and 4 male. Two patients had no previous surgery, 2 only had bandage of the pulmonary artery, 7 had the Glenn surgery and 1 had the Damus and the Glenn surgery. Five patients had tricuspid atresia (TA) with valvular pulmonary stenosis (PS) or atresia, 1 patient had TA alone, 2 had TA with transposition of the great arteries, 1 patient had double inlet left ventricle (LV) with PS, 2 had double inlet LV with coarctation of the aorta, and 1 patient had hypoplastic right heart. One patient had suspected Noonan Syndrome. The patients who did not have Glenn surgery were submitted to connection of superior and inferior venae cavae with the pulmonary artery in the same procedure (4 patients). Seven patients had the fenestrated Fontan procedure. Six patients had a combined operation. Inhospital mortality was 0%. One patient died 4 months after the surgery due to bilateral subdural hematoma. The immediate post operative complications were tachyarrhythmia (2); important bleeding (2); pericardial effusion (4); pleural effusion (7); provisional pacemaker (1); junctional rhythmn (1); temporary hemodialysis (1); infection of the operative wound (1); fungal endocarditis (1); and mild stroke (1). The mean duration of hospitalization was 41.5±18.7 days. The length of hospital stay after surgery was 31.1±16.2 days. The exercise functional capacity improved in all patients. Before surgery there was 1 patient NYHA II that became NYHA I, 10 were NYHA III and became II or I, and 1 patient who was NYHA IV became II. The average oxygen saturation before surgery was 82% ± 8.2% and after was 91.7% ± 4.7%. The mean hemoglobin went from 17.8 g/dL to 13.9 g/dL. Eight patients performed cardiopulmonary exercise testing (CPX) before surgery, 1 patient was Weber B, 4 patients Weber C, 1 D and 1 E. Mean VO2 max was 11.7 ml/kg.min (± 3.69), and the mean slope was 71.8±35.0. Four patients performed CPX after surgery, mean VO2 max was 16.5±7.3, and mean slope was 39±16.6. Mean follow up was 20.3±17.7 months. Conclusions The Fontan operation is safe in adult patients and may still confer them significant benefits.

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Bifurcated bypass in severe chronic limb threatening ischaemia

Vascular ◽

10.1177/1708538121999856 ◽

2021 ◽

pp. 170853812199985

Author(s):

Daniele Adami ◽

Michele Marconi ◽

Alberto Piaggesi ◽

Davide M Mocellin ◽

Raffaella N Berchiolli ◽

...

Keyword(s):

Survival Rates ◽

Primary Patency ◽

Free Survival ◽

Mortality And Morbidity ◽

Bypass Patency ◽

Stage 4 ◽

Endovascular Approach ◽

The Mean ◽

Clinical Records

Objectives Revascularization according to the angiosome concept is of proven importance for limb salvage in chronic limb threatening ischaemia but it is not always practicable. Bifurcated bypasses could be considered as an option when an endovascular approach is not feasible or has already failed and a single bypass would not allow direct revascularization of the ischaemic area. Bifurcated bypasses are characterized by landing on two different arteries, the main artery (in direct continuity with the foot vessels) and the secondary one (perfusing the angiosome district). The aim of this study is to evaluate the safety and effectiveness of bifurcated bypass in chronic limb threatening ischaemia. Methods Thirty-five patients were consecutively treated with a bifurcated bypass for chronic limb threatening ischaemia from January 2014 to December 2019 in a single vascular surgery centre. Data from clinical records and operative registers were collected prospectively in an electronic database and retrospectively analysed. Primary and primary assisted bypass patency, amputation-free survival, morbidity and mortality rates at 12 and 24 months were analysed. Results Mean follow-up period was 25.1 months (range 2–72 months). Thirty-six bifurcated bypasses were performed on 35 patients (age 75.3 ± 7.2 years; 69.4% were male). According to Wound, Ischemia, foot Infection classification 22.2% belonged to stage 3 and 77.8% to stage 4 and the mean Rutherford’s class was 5.1 ± 0.7. Immediate technical success was 100%. Early mortality and morbidity rates were respectively 5.5%, and 33.3%; foot surgery was performed in 50% of cases with wound healing in all patients. Primary patency and primary assisted bypass patency were 96.7% and 100% at 6 months; 85.2% and 92% at 12 months, 59.9% and 73.4% at 24 months, respectively. Amputation-free survival at 12 and 24 months was, respectively, 95.6% and 78.8%. Overall survival rates at 12 and 24 months were respectively 94.4% and 91.6%. Conclusions Bifurcates bypass can provide good results in patients with chronic limb threatening ischaemia without endovascular option, especially in diabetic ones. Bifurcated bypass is a complex surgical solution, both to be planned and performed, and it is quite invasive for frail patients that should be accurately selected.

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Outcomes of Surface Replacement Arthroplasty in Metacarpophalangeal Joints Affected by Noninflammatory Arthritis

Hand ◽

10.1177/15589447211028917 ◽

2021 ◽

pp. 155894472110289

Author(s):

Matthew R. Claxton ◽

Eric R. Wagner ◽

Marco Rizzo

Keyword(s):

Reoperation Rate ◽

Surface Replacement ◽

Free Survival ◽

Metacarpophalangeal Joints ◽

Surface Replacement Arthroplasty ◽

Replacement Arthroplasty ◽

Pain Ratings ◽

The Mean ◽

Revision Operations

Background The purpose of this study was to investigate the outcomes of surface replacement arthroplasty (SRA) for noninflammatory arthritis of the metacarpophalangeal (MCP) joint. Methods Records from 20 SRAs performed between 1995 and 2017 in 17 patients with noninflammatory arthritis affecting the MCP joint were retrospectively reviewed. The mean follow up was 6.6 years. Results Three arthroplasties (15%) underwent 4 revision operations. The 2-, 5-, 10-, and 15-year rates of survival from surface replacement implant revision were 90%, 90%, 79%, and 79%, respectively. Major complications occurring in revised joints included arthrodesis (n = 1) and amputation (n = 1). The overall reoperation rate was 35%. The 2-, 5-, 10-, and 15-year rates of overall reoperation-free survival were 75%, 69%, 60%, and 60%, respectively. Conclusions Pain ratings and MCP arc of motion significantly improved following arthroplasty. Metacarpophalangeal SRA for noninflammatory arthritis can improve arc of motion and pain. Revision is uncommon; however, 1 in 3 joints requires reoperation.

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Abstract 16154: Baseline Left Ventricular Diameter Predicts Recovery in New Systolic Heart Failure Syndromes

Circulation ◽

10.1161/circ.142.suppl_3.16154 ◽

2020 ◽

Vol 142 (Suppl_3) ◽

Author(s):

Daniel Gold ◽

Nathan Kong ◽

Matthew Gold ◽

Tess Allan ◽

Anand Shah ◽

...

Keyword(s):

Left Ventricular ◽

Medical Decision ◽

Left Ventricular Ejection ◽

Internal Diameter ◽

Event Free Survival ◽

Short Term Treatment ◽

Ventricular Ejection Fraction ◽

Free Survival ◽

Advanced Therapies

Introduction: It is unknown how often patients with very advanced left ventricular (LV) dilation at initial presentation demonstrate meaningful recovery with medical therapy. Understanding short term treatment outcomes may impact medical decision making and counseling. Hypothesis: Patients with left ventricular end diastolic internal diameter (LVEDD) > 6.5cm will be less likely to recover left ventricular ejection fraction (LVEF) as compared to patients with LVEDD < 6.5cm. Methods: Patients were retrospectively identified by a database search of echocardiogram studies obtained at the University of Chicago between 2008-2018. Manual review was performed to ensure new diagnosis of systolic dysfunction with LVEF ≤ 35% and follow up echocardiogram study within 3 to 9 months of index study. LVEDD was determined from parasternal long axis views per routine. LVEF recovery was specified as LVEF > 35%. Chart review was done to assess for composite death, hospice, transplant, left ventricular assist device, and sustained ventricular tachycardia. Chi-square, multivariable logistic regression, and Kaplan-Meier survival were used for analysis. Results: Out of 100 patients included for analysis, mean age was 59.7 years, 41 were female and 82 were African American. 17.7% of patients’ with LVEDD > 6.5 cm had LVEF recovery compared to 53.0% of patients’ with LVEDD ≤ 6.5 cm (p = 0.008). LVEDD > 6.5 cm was associated with less LVEF recovery even when adjusted for age, gender, hypertension, and diabetes (adjusted odds ratio 0.18, 95% CI 0.04 to 0.65). LVEDD > 6.5cm was associated with worse event free survival (p = 0.004) with a median follow-up time of 2.4 years. Conclusions: An LVEDD of > 6.5cm is associated with diminished LVEF recovery and event free survival when compared to those patients with an LVEDD ≤ 6.5cm. Delaying consideration for advanced therapies and device based therapies in hopes of recovery may be inappropriate for many such patients.

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