Atrioventricular Valve Insufficiency Research Articles

Use of glutaraldehyde-treated autologous pericardial patch in complete atrioventricular septal defect repair.

Synthetic patch materials, such as polytetrafluoroethylene and knitted polyester, are commonly used for the repair of complete atrioventricular septal defects using the double-patch technique. In our study, we investigated the impact of glutaraldehyde-treated autologous pericardial patch on postoperative atrioventricular valve function. In this retrospective study, patients who underwent repair of complete atrioventricular septal defects using the double-patch technique between January 1, 2018, and May 15, 2023, at our pediatric cardiac center were included in the study. Patients were divided into two groups based on the type of patch used for the repair of the ventricular component of the atrioventricular septal defect: autologous pericardial patch (group 1) and polytetrafluoroethylene patch (group 2). Postoperative data of the patients were retrospectively analyzed. Thirty-four patients (57.6%) were female, and 25 (42.4%) were male, with a median age of 7 (5-18) months. The median body weight at the time of surgery was 5.5 (4.1-10) kilograms. Down syndrome was present in 45 (76.3%) patients. The autologous pericardium was used for ventricular septal defect closure in 44 (74.6%) patients (Group 1), while polytetrafluoroethylene was used in 15 (25.4%) patients (Group 2). Permanent cardiac pacemaker placement was required in 4 (6.8%) patients due to postoperative atrioventricular block. Hospital mortality was 11.9% (n=7). Among these cases, 4 (6.8%) patients died from low cardiac output syndrome, and 3 (5.1%) patients died from sepsis. Postoperative echocardiographic evaluation revealed insignificant residual ventricular septal defects in 17 (28.8%) patients. No significant differences were observed between the groups in terms of mortality and postoperative atrioventricular valve insufficiency. The median follow-up duration for discharged patients was 5 months (7 days to 3.9 years). Throughout the follow-up period, there were no reoperations due to valve insufficiency or left ventricular outflow tract obstruction. Glutaraldehyde-treated autologous pericardium is a safe material that can be confidently used for the repair of the ventricular component of complete atrioventricular septal defects. This patch material does not adversely affect atrioventricular valve function and allows for the successful closure of ventricular septal defects.

Caso antemortem de insuficiência valvar atrioventricular em dragão barbudo (Pogona vitticeps) – relato de caso

An atrioventricular valve insufficiency is a common form of cardiac disorder in mammals, whereas it has rarely been reported in reptiles. A two-year-old male bearded dragon (Pogona vitticeps) was presented with acute onset of lethargy, inappetence and general weakness, increased respiratory effort, dark skin color, and arched back. A gastrointestinal foreign body was suspected, but radiography showed coelomic fluid accumulation. Ultrasound heart examination revealed turbulent blood flow at the level of the atrioventricular valves. Electrocardiography by modified Einthoven lead system (lead configuration 1) as described for Squamata, did not show significant changes. A tentative diagnosis of congestive heart failure as a result of valvulopathy was made. Treatment consisted of parenteral furosemide and fluid administration and assisted feeding with appropriate food intended for adult bearded dragons. The patient was weekly evaluated and successfully recovered over the four months. Echocardiography and electrocardiography were repeated 12 months later. The prescribed treatment was successful with complete resolution of clinical signs, and long-term surveillance of the bearded dragon (Pogona vitticeps).

Machine Learning to Predict Interstage Mortality Following Single Ventricle Palliation: A NPC-QIC Database Analysis.

There is high risk of mortality between stage I and stage II palliation of single ventricle heart disease. This study aimed to leverage advanced machine learning algorithms to optimize risk-prediction models and identify features most predictive of interstage mortality. This study utilized retrospective data from the National Pediatric Cardiology Quality Improvement Collaborative and included all patients who underwent stage I palliation and survived to hospital discharge (2008-2019). Multiple machine learning models were evaluated, including logistic regression, random forest, gradient boosting trees, extreme gradient boost trees, and light gradient boosting machines. A total of 3267 patients were included with 208 (6.4%) interstage deaths. Machine learning models were trained on 180 clinical features. Digoxin use at discharge was the most influential factor resulting in a lower risk of interstage mortality (p < 0.0001). Stage I surgery with Blalock-Taussig-Thomas shunt portended higher risk than Sano conduit (7.8% vs 4.4%, p = 0.0002). Non-modifiable risk factors identified with increased risk of interstage mortality included female sex, lower gestational age, and lower birth weight. Post-operative risk factors included the requirement of unplanned catheterization and more severe atrioventricular valve insufficiency at discharge. Light gradient boosting machines demonstrated the best performance with an area under the receiver operative characteristic curve of 0.642. Advanced machine learning algorithms highlight a number of modifiable and non-modifiable risk factors for interstage mortality following stage I palliation. However, model performance remains modest, suggesting the presence of unmeasured confounders that contribute to interstage risk.

Successful Heart Transplantation in a Patient with Neonatal Marfan Syndrome

<h3>Introduction</h3> Neonatal Marfan syndrome (NMS) is a rare subtype of Marfan syndrome associated with more pronounced clinical characteristics. Patients with NMS often have more severe cardiac involvement, including atrioventricular valve prolapse and insufficiency and aortic root pathology. The development of a concurrent myopathy syndrome has not been described. We describe a case of a teenage girl with NMS who developed inotrope-dependent heart failure secondary to dilated cardiomyopathy requiring heart transplantation. <h3>Case Report</h3> The patient is a 16-year-old female with NMS, mitral valve prolapse and regurgitation, and aortopathy who underwent mitral valvuloplasty and valve-sparing aortic root replacement 5 years prior to presentation. Since that time, she had asymptomatic, progressively declining left ventricular systolic ejection fraction without progressive aortic valve or mitral disease. Her left ventricular ejection fraction was 20-25% at its nadir. Additionally, she had severe kyphosis requiring surgery three months prior to presentation and was dependent on GJ feeds for weight management. She presented in winter of 2020 with new onset heart failure requiring admission for milrinone which was unable to be weaned off due to a low cardiac output state and was listed for heart transplant on day 13 of admission. An acceptable heart became available, and she was transplanted on day 17 of admission. Her post-operative course was relatively uncomplicated. She developed ectopic atrial tachycardia, which is well-controlled with atenolol and propafenone. Whole exome sequencing confirmed that she is heterozygous for a ∼13.8 KB deletion within the <i>FBN1</i> gene, confirming her NMS diagnosis, as well as an additional novel deletion of exons 18 to 28 within <i>FBN1.</i> She has had no rejection and marked improvement in her quality of life evident by weaning off tube feedings, exercising regularly with long distance walking and attending senior year of high school. <h3>Summary</h3> The cardiac involvement in NMS and typical Marfan syndrome has generally been described as valve prolapse and aortopathy, although cardiomyopathy in the latter has been reported. Our teenage patient with NMS developed progressive LV systolic heart failure ultimately requiring a heart transplant. We believe patients with NMS that develop end-stage heart failure without treatment options should be considered for heart transplantation.

Surgical management of biatrial myxoma: case report

Heart tumours account for approximately 0.2 % of all tumours: of these, approximately 75 % of all primary heart tumours are benign and 50 % of them are myxomas. Further, myxomas make up 0.0017 % of the general population of patients with cardiovascular disease. Biatrial myxomas, i.e. tumours in both the left and right atria, can be in the form of a ‘butterfly’ or a ‘dumbbell’, and account for &lt; 1 % of all cardiac myxomas. Here we describe the successful surgical management of a rare case of a large biatrial myxoma and concomitant atrioventricular valve insufficiency. Briefly, 2D transthoracic echocardiography findings included an end-diastolic volume of 90 ml, an end-systolic volume of 40 ml and a left ventricular ejection fraction of 55 % (according to Simpson). The fibrous ring of the mitral valve measured 36 mm with a regurgitation degree of 2 while the fibrous ring of the tricuspid valve was 42 mm in size and the regurgitation grade was 3. Lesion size in the cavity of the left and right atrium were 73 × 38 mm and 80 × 42 mm, respectively. These neoplasia were surgically removed under peripheral cardiopulmonary bypass, hypothermia and cold cardioplegia (Custodiol). The myxomas were accessed through the right atrium, according to Giradon and were resected without defragmentation. Next, mitral valve plasty using a soft support ring and annuloplasty of the tricuspid valve were performed according to de Vega. The duration of artificial circulation was 150 minutes and that of myocardial ischaemia was 100 minutes. The patient was extubated 11 hours after surgery, spent 22 hours in the intensive care unit and was discharged on the 14th day after surgery. Surgical resection of biatrial myxomas before the development of serious irreversible or life-threatening complications can provide rapid symptomatic relief in congestive heart failure.Received 30 May 2021. Revised 22 August 2021. Accepted 23 August 2021.Funding: The study did not have sponsorship.Conflict of interest: Authors declare no conflict of interest.Contribution of the authors Literature review: T.A. Simonyan Drafting the article: T.A. Simonyan, I.M. Tsiskaridze Critical revision of the article: T.A. Simonyan, I.Yu. Farulova, E.A. Babajanyan Surgical treatment: I.I. Scopin, I.M. Tsiskaridze Final approval of the version to be published: T.A. Simonyan, I.I. Scopin, I.M. Tsiskaridze, I.Yu. Farulova, E.A. Babajanyan

610 Fontan associated kidney and liver disease: can we predict organ involvement with echocardiographic assessment of systolic function and atrioventricular valve insufficiency?

Abstract Aims Fontan operation represents the surgical palliative option for congenital heart disease with single ventricle physiology. With the improvement of surgical and percutaneous technique, we are facing a growing population of patients with an unique pathophysiology and potential complications. Methods and results Patients that underwent Fontan palliation in our centre between 1993 and 2016 were included in this prospective study. We excluded patients with major congenital renal anomalies, those that underwent cardiac transplantation, and redo-Fontan patients. All the subjects underwent clinical evaluation, laboratory exams with complete renal and hepatic function, transient hepatic elastography, and complete cardiac evaluation. We used Schwartz equation for estimating glomerular filtration rate in patients younger than 18 years, and CDK-EPI equation for adult patients. We enrolled 35 patients, 46% female (N = 16), and 54% male (N = 19). Medium age was 17 years old, median age 15 years old (range: 10–31 years old). Medium time from Fontan completion was 160 months (range: 57–340 months). Regarding to cardiac anatomy, 10 patients had functional single left ventricle (FSLV, 28.5%) and 21 a functional single right ventricle (FSRV, 60%); 4 patients had undetermined single ventricle (11.5%). Total cavo-pulmonary connection (TCPC) with intracardiac lateral tunnel was performed in 7 patients (20%, N = 7), whereas 28 patients had TCPC with external conduit (80%). Data from echocardiographic evaluation showed a medium EF established with Simpson’s method of 60% in patients with FSLV; patients with a FSRV or undetermined single ventricle had a medium FAC of 41.1%, with 15.1% having a reduced FAC &amp;lt; 35%. No FSLV patients had an EF &amp;lt; 50%. When using creatinine-based formula, data about renal function in our population showed a stage 2 chronic kidney disease (eGFR: 60–89 ml/min 1.73 mq) in 11% of total population (N = 4), that became 26% when using cystatin C-based equation (N = 9), with one patient showing a moderate reduced loss of kidney function (eGFR: 40–59 ml/min 1.73 mq). Urinalysis showed 29% (N = 10) of patients having microalbuminuria (microalbumin/creatinine ratio between 30 and 300 mg/g). Statistical analysis demonstrated a negative correlation between systolic function (TAPSE for FSRV) and cystatin C blood levels (Pearson’s R −0.428, P = 0.053), and between systolic function (FAC and Simpson) and microalbuminuria (Pearson’s R −0.414 with P = 0.049 and Pearson’s R −0.754 with P = 0.019, respectively). Transient elastography reported 10 patients (29.4%) with abnormal hepatic stiffness for Fontan patients. That condition appeared to be more frequent in patients with higher grade of AV valve insufficiency (P &amp;lt; 0.05). Conclusions Our population showed an higher prevalence of FSRV Fontan patients, with an expected lower systolic function compared with FSLV. 2D evaluation of systolic function showed a linear inverse correlation with renal function, suggesting that Fontan patients need a closer renal monitoring. Hepatic stiffness, which is a warning sign of potential hepatic cirrhosis need to be monitored in all Fontan patients, especially those with a worse AV valve insufficiency.

Abstract 12589: Fetoscopic Selective Laser Photocoagulation of Placental Anastomoses Leads to Early Reduction of Cardiovascular Burden in the Twin-Twin Transfusion Syndrome

Background: Twin-twin transfusion syndrome (TTTS) occurs with an incidence of 10-15% in monochorionic pregnancies. The Children’s Hospital of Philadelphia (CHOP) TTTS Cardiovascular Score (CVS) is a 20-point scale and encompasses evaluation of five domains: Recipient (R) abnormalities of 1) ventricular hypertrophy/systolic dysfunction, 2) atrioventricular valve insufficiency, 3) diastolic ventricular inflow, ductus venosus and umbilical vein flow patterns and 4) right ventricular outflow/pulmonary obstruction; Donor (D) abnormalities of 5) umbilical arterial (UA) flow. We explore the early impact of selective fetoscopic laser photocoagulation (SLPC) of placental anastomoses on fetal cardiovascular (CV) burden. Methods: Fetal echocardiograms performed prior to and one week post SLPC from 11/2012 to 6/2018 were reviewed. Pulsatility indices (PI) of the UA and middle cerebral artery (MCA), cerebroplacental ratio (CPR) and CHOP CVS elements were analyzed pre and post SLPC using paired t-tests. Results: SLPC was performed in 198 subjects; 17 excluded due to one twin demise post SLPC. Of the 181 analyzed, mean gestational age at SLPC was 19.8 (95% CI: ±0.3) weeks. Following SLPC, R demonstrated an increase in MCA PI and decrease in UA PI, with increase in CPR. D demonstrated a similar magnitude decrease in both MCA PI and UA PI, yielding no change in CPR. Following SLPC, mean CHOP CVS decreased. CHOP pre SLPC CVS was ≥6 in 62 (34%). The magnitude of change was greater in the subgroup with greater pre SLPC CV burden (CVS &gt; 6). Most common elements to improve were systolic dysfunction, tricuspid inflow, ductus venosus and umbilical vein flow patterns; least likely to improve was ventricular hypertrophy. Conclusions: Improvement in CV burden is seen as early as 1 week post SLPC, which supports acute alteration of loading conditions as contributing to the disease. Further study of the trajectory of CV alterations may provide insight into the complex mechanisms underlying TTTS.

870 Surgical Outcomes of Primary and Two-Stage Atrioventricular Septal Defect (AVSD) Repair: A Single Institution Experience

Abstract Objective To examine the surgical outcomes of primary and two-stage repair of complete atrioventricular septal defect(AVSD). Method This retrospective study included 74 patients who underwent operation for balanced complete AVSD between January 2015 and December 2018 in National Heart Centre Kuala Lumpur. Patient demographics, types of procedure, post-op complications and follow-up atrioventricular (AV) valve function were analysed. Results Twenty-one patients (median age: 3 months(2.28-4.32months)), weight: 3.10kg( 2.7-3.82kg)) had Pulmonary Artery Banding (PAB) prior to complete AVSD repair. The post-banding weight of patients rose from 3.1kg to 6.4kg prior to complete repair. The rate of ventilator dependence decreased from 19.8 to 4.8%. There was no worsening of post-banding left AV valve insufficiency (5%) before the complete repair. There was no statistically significant difference in the outcomes after complete AVSD repair in both groups (mortality p = 0.133, morbidities p = 0.471). There was a trend towards higher left AV valve insufficiency in the PAB group over time (at discharge, 10 vs 12%; at 3-months, 12 vs 6%; at 1-year, 14 vs 11%). Similarly, the PAB group also demonstrated a trend towards higher rates of major post- operative complications (33 vs 21%) and in-hospital mortality (9.5 vs 1.9%). Conclusions PAB remained as an effective palliative procedure for patients who are not suitable for primary AVSD repair at the time of presentation. However, it is associated with a higher incidence of left AV valve insufficiency at follow up.

A word on netting of angiotensin-converting enzyme inhibitor therapy in hypoplastic left heart syndrome following stage-I.

NPC-QIC Registry data showed that angiotensin-converting enzyme inhibitors were described in almost 38% for patients with single ventricle physiology after stage-I Norwood palliation. However, mortality and ventricular dysfunction or atrioventricular valve insufficiency seems to be not improved by oral application of angiotensin-converting enzyme inhibitors. The final conclusion was that despite limited evidence of benefit for patients with hypoplastic left heart syndrome, prescription of angiotensin-converting enzyme inhibitors during interstage is still common. Taking into account of the predominant cardiovascular regulation in newborns and young infants by circulating catecholamines, no real improvement is to be expected from angiotensin-converting enzyme inhibitor monotherapy. The goals of drug therapy after stage-I Norwood palliation in hypoplastic left heart syndrome are prevention of systemic right ventricle failure, balancing pulmonary and systemic blood flow, and reduction of oxygen consumption with regard to limitations of oxygen supply by the single ventricle, furthermore, avoiding harmful effects of endogenous catecholamine production in the long term on somatic and cognitive development. In this light of knowledge, we want to recommend the use of a long-acting and highly specific ß1-adrenoreceptor blocker for almost all patients after stage-I Norwood palliation and a combination with angiotensin-converting enzyme inhibitors only by indication after exclusion of potential side effects.

Ethical Issues in the Care of Emerging Adults in Pediatric Intensive Care Units.

Ethical Issues in the Care of Emerging Adults in Pediatric Intensive Care Units.

Implantation of an S-ICD in a Patient with a DDD Pacemaker and Congenitally Corrected Transposition of the Great Arteries

Subcutaneous implantable cardioverter-defibrillator (S-ICD) therapy has become a viable alternative to conventional transvenous ICD implantation. Patients with congenitally corrected transposition of the great arteries (ccTGA) have a high risk of sudden cardiac death due to malignant arrhythmia. The interaction between the S-ICD system and the transvenous pacemaker system is not fully understood. We report a case of S-ICD implantation in a patient with ccTGA and a DDD pacemaker. The patient was a 30-year-old man with a previously placed pacemaker with diagnoses of congenital heart disease, ccTGA (SLL), left atrioventricular valve insufficiency, and third-degree atrioventricular block. He presented with an out-of-hospital cardiac arrest, and an S-ICD was implanted to prevent sudden cardiac death. Defibrillation checks were performed successfully. We tested the compatibility of the DDD pacemaker with the S-ICD and found that there was no interference between them. In conclusion, an S-ICD system is a reasonable and safe option in patients with ccTGA.

ADAMTS19-associated heart valve defects: Novel genetic variants consolidating a recognizable cardiac phenotype.

Recently, ADAMTS19 was identified as a novel causative gene for autosomal recessive heart valve disease (HVD), affecting mainly the aortic and pulmonary valves. Exome sequencing and data repository (CentoMD) analyses were performed to identify patients with ADAMTS19 variants (two families). A third family was recognized based on cardiac phenotypic similarities and SNP array homozygosity. Three novel loss of function (LoF) variants were identified in six patients from three families. Clinically, all patients presented anomalies of the aortic/pulmonary valves, which included thickening of valve leaflets, stenosis and insufficiency. Three patients had (recurrent) subaortic membrane, suggesting that ADAMTS19 is the first gene identified related to discrete subaortic stenosis. One case presented a bi-commissural pulmonary valve. All patients displayed some degree of atrioventricular valve insufficiency. Other cardiac anomalies included atrial/ventricular septal defects, persistent ductus arteriosus, and mild dilated ascending aorta. Our findings confirm that biallelic LoF variants in ADAMTS19 are causative of a specific and recognizable cardiac phenotype. We recommend considering ADAMTS19 genetic testing in all patients with multiple semilunar valve abnormalities, particularly in the presence of subaortic membrane. ADAMTS19 screening in patients with semilunar valve abnormalities is needed to estimate the frequency of the HVD related phenotype, which might be not so rare.

Systolic dysfunction of systemic ventricle in patients who underwent a Fontan operation.

Öner T, Ballı Ş, Hekim Yılmaz E, Bulut MO, Şaşmazel A, Çelebi A. Systolic dysfunction of systemic ventricle in patients who underwent a Fontan operation. Turk J Pediatr 2019; 61: 925-930. The aim of the study was to report the clinical and echocardiographic data of patients who underwent a Fontan operation, and define the group with systolic dysfunction in the systemic ventricle observed during postoperative follow-up. The medical records of 183 patients [mean age: 10.93 ± 5.89 years (range: 2.5-45 years)] who were referred to our center and underwent a Fontan operation were retrospectively reviewed. The clinical, echocardiographic, and postoperative follow-up data of the patients were recorded. Preoperatively, 68 (37.2%) patients experienced pulmonary stenosis, while 41 (22.4%) had pulmonary atresia and 74 (40.4%) had pulmonary hypertension. The most common pathology in patients who were ineligible for biventricular repair was tricuspid atresia, seen in 51 patients (27.9%), followed by double-inlet left ventricle pathologies in 40 patients (21.9%). In total, 38 (20.7%) patients had a biventricular structure; among those with a single ventricular structure, the systemic ventricle involved was the right ventricle in 51 (27.9%) patients and the left ventricle in 94 (51.4%) patients. During follow-up, a total of 31 (16.9%) patients underwent catheterization and ventricular systolic functions were preserved in 168 (91.8%) patients. In Fifteen (8.2%) patients developed systolic dysfunction (ejection fraction < 50%). The mean age of the group developing systolic dysfunction was 15.6 ± 2.63 years (median, 13 years; range: 5-45 years). Of this group, 10 patients had a left ventricular structure of the systemic ventricle, 14 had atrial situs solitus, and 4 had moderate atrioventricular valve insufficiency. Even if the systemic ventricle is in the left ventricular structure, systolic dysfunction in the systemic ventricle develops, especially after the first 10 years, and this makes us think rudimentary ventricle function should also be carefully monitored for intra-univentricular diastolic asynchrony.

A novel totally biodegradable device for effective atrial septal defect closure: A 2‐year study in sheep

To evaluate the feasibility, efficacy, and safety of a fully biodegradable poly lactic acid (PLA)-based occluder for atrial septal defect (ASD) closure in an animal model. ASDs, approximately 12-mm in diameter, were generated in sheep (n = 18) by needle puncture and balloon dilatation. For ASD closure, occluders were implanted by percutaneous transcatheter approach under echocardiographic guidance. Outcomes were evaluated by transthoracic echocardiography, electrocardiography, blood testing, and histology within the follow-up period ranging from 1 month to 2 years. All occluders were successfully implanted. During follow-up, no animal died; rectal temperatures, blood test results, and electrocardiograms were within normal ranges; and transthoracic echocardiograms, macroscopic studies, and histopathological and electron microscopic examination demonstrated that the occluders were well positioned, with no shifting, residual shunts, severe inflammation, thrombus formation, atrioventricular valve insufficiency, cardiac erosion or arrhythmias. The occluders gradually embedded into the endocardial tissue of the hosts with complete endothelialization and disk absorption at 12 months, and a distinct molecular weight decrease of the framework (to 9% of initial) at 24 months after implantation. In a sheep model, the use of totally biodegradable occluders appears feasible, efficacious and safe for ASD closure. Studies in humans are ongoing.

Invited Commentary

Invited Commentary

Cardioband system: a novel percutaneous solution for atrioventricular valve insufficiency

What is known about The Cardioband in the clinical area? Severe secondary mitral valve insufficiency frequently affects high-risk surgical patients. Cardioband system is a novel percutaneous surgical-like device for direct annuloplasty. It is totally implanted in the beating heart, by transvenous femoral access with minimal impact on hemodynamic and cardiac function during the implantation. It has been demonstrated to be safe and feasible in high-risk patients with functional mitral regurgitation, with high rates of implant success, significant annular reduction and regurgitation improvements. That positively impacts directly on ventricular function, functional status and quality of life. Recently, Cardioband has been used also in tricuspid position, with initial promising results. Bibliographic review.

Long-term outcomes in patients who underwent surgical correction for atrioventricular septal defect.

Objective:The follow-up results of patients operated for atrioventricular septal defect (AVSD) during 1996–2016 at Başkent University are presented.Methods:Data obtained from hospital records consists of preoperative echocardiographic and angiographic details, age and weight at surgery, operative details, Down syndrome presence, postoperative care details, early postoperative and latest echocardiographic findings and hospitalization for reintervention.Results:A total of 496 patient-files were reviewed including 314 patients (63.4%) with complete and 181 (36.6%) with partial AVSD (48.4% of all patients had Down syndrome). Atrioventricular (AV) valve morphology was Rastelli type A in 92.2%, B in 6.5%, and C in 1.3% of patients. The operative technique used was single-patch in 21.6% (108), double-patch in 25.8% (128), and modified single-patch (Wilcox) in 52.5% (260) of patients. The follow-up time was 37.79±46.70 (range, 0–198) months. A total of 64 patients (12.9%) had arrhythmias while in the intensive care unit; pacemaker was implanted in 12 patients. A total of 78 patients (15.7%) were treated for pulmonary hypertensive crisis. The early morbidity and mortality in the postoperative first month were calculated as 38% and 10%, and the late morbidity and mortality (>1 month) were calculated as 13.1% and 1.9%, respectively. The rate of reoperation in our cohort was 8.9%.Conclusion:Although the early morbidity and mortality are low in AVSD operations, the rate of reoperations for left AV valve insufficiency are still high. Although Down syndrome is not a risk factor for early mortality, the co-morbid factors, such as longer postoperative mechanical ventilator or inotropic support, lead to higher risk for morbidity. The frequency of pulmonary hypertension and consequent complications are also high.

Acute and midterm results following perventricular device closure of muscular ventricular septal defects: A multicenter PICES investigation.

To describe acute and mid-term results of hybrid perventricular device closure of muscular ventricular septal defects (mVSDs). Perventricular device closure of mVSDs can mitigate technical limitations of percutaneous closure and need for cardiopulmonary bypass or ventriculotomy with a surgical approach. This is a multicenter retrospective cohort study of patients undergoing hybrid perventricular mVSD device closure from 1/2004 to 1/2014. Procedural details, adverse events, outcomes, and follow-up data were collected. Patients were divided into two groups: (1) simple (mVSD closure alone) and (2) complex (mVSD closure with concomitant cardiac surgery). Forty-seven patients (60% female) underwent perventricular mVSD device closure at a median age of 5.2 months (IQR 1.8-8.9) and weight of 5.1 kg (IQR 4.0-6.9). Procedural success was 91% [100% (n = 22) simple and 84% (n = 21/25) complex]. Adverse events occurred in 19% (9/47) [9% (2/22) simple and 28% (7/25) complex]. Hospital length of stay (LOS) was shorter in the simple vs. complex group (4 vs. 14 days, P < 0.01). At mid-term follow-up of 19.2 months (IQR 2.3-43) 90% of pts had complete mVSD closure; none developed late heart block, increased atrioventricular (AV) valve insufficiency or ventricular dysfunction. Perventricular device closure of simple mVSD was associated with a high rate of procedural success, few adverse events, and short hospital LOS. Procedural adverse events were associated with the presence of concomitant complex surgery. Residual mVSD, AV valve insufficiency, or ventricular dysfunction were uncommon at mid-term follow-up. © 2017 Wiley Periodicals, Inc.

Hepatic Fibrosis Is Universal Following Fontan Operation, and Severity is Associated With Time From Surgery: A Liver Biopsy and Hemodynamic Study.

BackgroundCongestive hepatopathy is a recognized complication of Fontan physiology. Data regarding the incidence of hepatopathy and risk factors are lacking.Methods and ResultsLiver biopsies and cardiac catherizations were performed as part of an evaluation offered to all patients ≥10 years after Fontan. Quantitative determination of hepatic fibrosis was performed using Sirius red staining with automated calculation of collagen deposition per slide (%CD). Biopsies from included subjects were compared to stained specimens from controls without known fibrotic liver disease. Patient characteristics, echocardiographic findings, and hemodynamic measures were evaluated as potential risk factors. The cohort consisted of 67 patients (31 female) at mean age of 17.3±4.5 years and mean time from Fontan of 14.9±4.5 years. Right ventricular morphology was present in 37 subjects. Median %CD by Sirius red staining was 21.6% (range 8.7% to 49.4%) compared to 2.6% (range 2.2% to 3.0%) in controls. There was a significant correlation between time from Fontan and degree of Sirius red staining (r=0.33, P<0.01). Serum liver enzymes and platelet count did not correlate with %CD. The median inferior vena cava pressure was 13 mm Hg (range 6‐24 mm Hg) and did not correlate with %CD. There was no difference in %CD based on ventricular morphology or severity of atrioventricular valve insufficiency.ConclusionsIn this cohort of predominantly asymptomatic children and adolescents electively evaluated after a Fontan operation, all exhibited evidence for hepatic fibrosis as measured by collagen deposition in the liver. Time from Fontan was the only factor significantly associated with collagen deposition. These findings demonstrate that liver fibrosis is an inherent feature of Fontan physiology and that the degree of fibrosis increases over time.

Mortality Risk Stratification in Fontan Patients Who Underwent Heart Transplantation

The number of patients who require orthotopic heart transplantation (OHT) for failing Fontan physiology continues to grow; however, the methods and tools to evaluate risk of OHT are limited. This study aimed to identify a set of preoperative variables and characteristics that were associated with a greater risk of postoperative mortality in patients who received OHT for failing Fontan physiology. Thirty-six Fontan patients were identified as having undergone OHT at University of California-Los Angeles Medical Center from 1991 to 2014. Data were collected retrospectively and analyzed. The primary end point was designated as postoperative mortality. After an average follow-up time of 3.5years, 17 (44%) patients suffered postoperative mortality. Patient characteristics including (1) age <18years at the time of OHT, (2) Fontan-OHT interval of <10years, (3) systemic ventricular ejection fraction <20%, (4) moderate-to-severe atrioventricular valve insufficiency, (5) an elevated Model of End-stage Liver Disease, eXcluding INR score, or (6) need for advanced mechanical support before surgery were associated with an increased incidence of postoperative mortality. Using these risk factors, we present a theoretical framework to stratify risk of postoperative death in failing Fontan patients after OHT. In conclusion, a method such as this may aid in the transplantation evaluation and listing process of patients with failing Fontan physiology.