Requiring Pacemaker Placement Research Articles

Modified-Bentall Single-Patch Konno Enlargement Technique for Aortic Stenosis and Prosthesis-Patient Mismatch.

Aortic stenosis and prosthesis-patient mismatch complicate surgery for patients with small left ventricular outflow tracts. We present outcomes of a modified-Bentall single-patch Konno enlargement (BeSPoKE) technique for complex left ventricular outflow tract obstruction in adults. The BeSPoKE technique facilitates a true outflow tract enlargement via an anterior septo-ventriculoplasty, using a single pericardial patch, followed by composite aortic valve-graft root replacement. Postoperative outflow tract geometry and valvular physiology were compared to preoperative measurements using echocardiography and computed tomographic angiography; two-year clinical outcomes were assessed. From October 2017 to March 2022, 25 adults (median age 60 years; 84% female) underwent a BeSPoKE repair. Mean preoperative aortic valve gradient was 44 ± 19mmHg. Twenty-one (84%) patients had previous aortic valve replacements with prosthesis-patient mismatch; median implant size preoperatively was 19mm. Postoperatively, all patients received a prosthesis of at least 21mm, with a median upsizing of 2 (15th-85th Percentile, 2-3 sizes). Mean postoperative aortic valve gradient was 8.5 ± 4.1mmHg (P<.001); mean 2-year gradient was 8.3 ± 1.3mmHg. All patients with bioprosthetic replacements qualified for future transcatheter valve replacements. Postoperative complications included atrial fibrillation (9; 36%) and complete heart block requiring pacemaker placement (8; 32%). There were zero operative mortalities and no reoperations reported. There were 2 late non-cardiac-related deaths; 2-year survival was 92%. The modified-Bentall single-patch Konno enlargement technique facilitates larger prosthesis placement, improves hemodynamics, and enables future transcatheter reinterventions. This approach is a safe treatment for complex left ventricular outflow tract obstruction and prosthesis-patient mismatch in adults.

8746 Primary Prevention and Early Intervention for Cardiovascular Disease in Polyglandular Autoimmune Syndrome Type 2

Abstract Disclosure: V.N. Flores-Robles: None. Y. Fuentes-Rosa: None. M. Sanchez-Cordero: None. D.A. Lopez: None. M. Carrero-Quinones: None. Introduction: Polyglandular autoimmune syndrome type 2 (PAS-2) is an autoimmune syndrome that is known for lymphocytic infiltration causing organ-specific damage. It can include primary adrenal insufficiency (Addison's disease), type 1 diabetes mellitus (T1DM), autoimmune thyroid disease-causing Grave’s disease, or hypothyroidism, and can be diagnosed when two out of these three pathologies are present. Carpenter syndrome, a type of PAS-2, is described as the triad of Addison's disease, autoimmune thyroid disease, and T1DM. Its initial manifestation can be associated with non-endocrine and endocrine disorders as in autoimmune thyroid disease, T1DM, primary hypogonadism, and diabetes insipidus. It is well documented that uncontrolled diabetes mellitus can accelerate the progression of cardiovascular disease of co-existing coronary artery disease, but documentation that Addison’s disease may accelerate the progression is scant with only ten cases documented. On the other hand, there are no documented cases of the presence of coronary artery disease in patients with PAS-2. Case report: This case involves a 66-year-old Hispanic male with a past medical history of hypertension, and PAS-2, presenting with Addison’s disease, type 1 diabetes mellitus, and hypothyroidism. The initial presentation at the hospital was that of a complete atrioventricular block that required pacemaker placement. Cardiology services were consulted as the patient also had a positive stress test and eventually cardiac catheterization was done as further workup. Cardiac catheterization showed obstruction of the left main coronary artery of 80% and left circumflex with mild disease, that required coronary artery bypass (CABG). A 2D ECHO was done for further evaluation of cardiac function and findings were pertinent for an ejection fraction of 65-70%, mild concentric hypertrophy of the left ventricular wall, and left ventricular diastolic function with impaired relaxation pattern. The patient underwent the CABG procedure without any complications. He has been followed up outpatient and remains without any symptoms or pertinent presentation. Early intervention in this patient with PAS-2 prevented any further cardiovascular complications. Conclusion: Patients with PAS-2 may have a predisposition to developing coronary artery disease due to concomitant endocrinopathies that independently increase the risk of cardiovascular disease. This promotes further investigation into the primary prevention of cardiovascular disease in patients presenting with PAS-2. Taking into consideration the increased risk and/or progression of cardiovascular disease in these patients, early intervention and evaluation by cardiology services should be suggested as it may decrease their morbidity or mortality. Presentation: 6/2/2024

Procedural and Intermediate-term Results of the Electroanatomical-guided Cardioneuroablation for the Treatment of Supra-Hisian Second- or Advanced-degree Atrioventricular Block: the PIRECNA multicentre registry.

Prior case series showed promising results for cardioneuroablation in patients with vagally induced atrioventricular blocks (VAVBs). We aimed to examine the acute procedural characteristics and intermediate-term outcomes of electroanatomical-guided cardioneuroablation (EACNA) in patients with VAVB. This international multicentre retrospective registry included data collected from 20 centres. Patients presenting with symptomatic paroxysmal or persistent VAVB were included in the study. All patients underwent EACNA. Procedural success was defined by the acute reversal of atrioventricular blocks (AVBs) and complete abolition of atropine response. The primary outcome was occurrence of syncope and daytime second- or advanced-degree AVB on serial prolonged electrocardiogram monitoring during follow-up. A total of 130 patients underwent EACNA. Acute procedural success was achieved in 96.2% of the cases. During a median follow-up of 300 days (150, 496), the primary outcome occurred in 17/125 (14%) cases with acute procedural success (recurrence of AVB in 9 and new syncope in 8 cases). Operator experience and use of extracardiac vagal stimulation were similar for patients with and without primary outcomes. A history of atrial fibrillation, hypertension, and coronary artery disease was associated with a higher primary outcome occurrence. Only four patients with primary outcome required pacemaker placement during follow-up. This is the largest multicentre study demonstrating the feasibility of EACNA with encouraging intermediate-term outcomes in selected patients with VAVB. Studies investigating the effect on burden of daytime symptoms caused by the AVB are required to confirm these findings.

Postoperative Atrioventricular Block in Pediatric Patients: Impact of Congenital Cardiac Malformations and Medications.

Postoperative atrioventricular block may occur after pediatric cardiac surgery. A small proportion of those who develop atrioventricular block will require pacemaker placement. The primary aim of this study was to determine factors associated with postoperative atrioventricular block. Secondary aims included determining factors associated with pacemaker placement in those with atrioventricular block. Data from the PHIS data were utilized to identify patients under 18 years of age who underwent cardiac surgery. Those who did and did not develop atrioventricular block. Univariable analyses and regression analyses were conducted to determine factors associated with postoperative atrioventricular block. Similar analyses were conducted to determine factors associated with pacemaker placement in those with atrioventricular block. A total of 43,716 admissions were identified. Of these, 2093 (5%) developed atrioventricular block and 480 (1% of total admissions) underwent pacemaker placement. Approximately 70% of those with atrioventricular block received steroids but this was not associated with a decrease in pacemaker placement. Risk factors (congenital malformations of the heart, comorbidities, medications) associated with increased risk of atrioventricular block and pacemaker placement were identified. Postoperative atrioventricular block occurred in 5% of pediatric admissions for cardiac surgery. Of these admissions with postoperative atrioventricular block, 23% required pacemaker placement. Isoproterenol and steroids were not associated with a reduction in the likelihood of pacemaker placement.

The utility and impact of outpatient telemetry monitoring in post-transcatheter aortic valve replacement patients

BackgroundConduction disturbances are a common complication of transcatheter aortic valve replacement (TAVR). Mobile Cardiac Telemetry (MCT) allows for continuous monitoring with near “real time” alerts and has allowed for timely detection of conduction abnormalities and pacemaker placement in small trials. A standardized, systematic approach utilizing MCT devices post TAVR has not been widely implemented, leading to variation in use across hospital systems. ObjectivesOur aim was to evaluate the utility of a standardized, systematic approach utilizing routine MCT to facilitate safe and earlier discharge by identifying conduction disturbances requiring permanent pacemaker (PPM) placement. We also sought to assess the occurrence of actionable arrhythmias in post-TAVR patients. MethodsUsing guidance from the JACC Scientific Expert Panel, a protocol was implemented starting in December 2019 to guide PPM placement post-TAVR across our health system. All patients who underwent TAVR from December 2019 to June 2021 across four hospitals within Northwell Health, who did not receive or have a pre-existing PPM received an MCT device at discharge and were monitored for 30 days. Clinical and follow-up data were collected and compared to pre initiative patients. ResultsDuring the initiative 693 patients were monitored with MCT upon discharge, 21 of whom required PPM placement. Eight of these patients had no conduction abnormality on initial or discharge ECG. 59 (8.6 %) patients were found to have new atrial fibrillation or flutter via MCT monitoring. There were no adverse events in the initiative group. Prior to the initiative, 1281 patients underwent TAVR over a one-year period. The initiative group had significantly shorter length of stay than pre-initiative patients (2.5 ± 4.5 vs 3.0 ± 3.8 days, p < 0.001) and lower overall PPM placement rate within 30 days post-TAVR (16 % vs 20.5 %, P = 0.0125). ConclusionsIn our study, implementation of a standardized, systematic approach utilizing MCT in post-TAVR patients was safe and allowed for timely detection of conduction abnormalities requiring pacemaker placement. This strategy also detected new atrial fibrillation and flutter. Reduction in post TAVR pacemaker rate and length of stay were also noted although this effect is multifactorial.

Transcatheter Aortic Valve Implantation and Cardiac Conduction Abnormalities: Prevalence, Risk Factors and Management.

Over the last decades, transcatheter aortic valve implantation (TAVI) or replacement (TAVR) has become a potential, widely accepted, and effective method of treating aortic stenosis in patients at moderate and high surgical risk and those disqualified from surgery. The method evolved what translates into a noticeable decrease in the incidence of complications and more beneficial clinical outcomes. However, the incidence of conduction abnormalities related to TAVI, including left bundle branch block and complete or second-degree atrioventricular block (AVB), remains high. The occurrence of AVB requiring permanent pacemaker implantation is associated with a worse prognosis in this group of patients. The identification of risk factors for conduction disturbances requiring pacemaker placement and the assessment of their relation to pacing dependence may help to develop methods of optimal care, including preventive measures, for patients undergoing TAVI. This approach is crucial given the emerging evidence of no worse outcomes for intermediate and low-risk patients undergoing TAVI in comparison to surgical aortic valve replacement. This paper comprehensively discusses the mechanisms, risk factors, and consequences of conduction abnormalities and arrhythmias, including AVB, atrial fibrillation, and ventricular arrhythmias associated with aortic stenosis and TAVI, as well as provides insights into optimized patient care, along with the potential of conduction system pacing and cardiac resynchronization therapy, to minimize the risk of unfavorable clinical outcomes.

Early outcomes of the suprasternal transcatheter aortic valve replacement technique.

Transcatheter aortic valve replacement (TAVR) is now an established treatment option for patients with severe aortic stenosis. The most utilized approach remains transfemoral. In patients with difficult femoral access a variety of alternate approaches have been used. Recently, suprasternal access has emerged as a viable alternate approach in patients with very complex vascular access. We describe our 30-day outcomes of patients who underwent suprasternal transcatheter aortic valve replacement (suprasternal [SS]-TAVR), which constitutes the largest single-center cohort to date. From May 2016 to September 2021, 658 patients underwent TAVR at our institution. Of which 29underwent SS-TAVR. We performed a retrospective analysis to evaluate early (30 days) outcomes of this procedure. Main outcomes evaluated included 30-day mortality, stroke and pacemaker rates, length of stay, readmission, and valvular function. All patients were alive 30 days after the procedure. The median hospital length-of-stay was 2 days. Two patients (6.9%) had a stroke on the contra-lateral side of access. Two patients (6.90%) had significant cardiac arrhythmias requiring pacemaker placement. In 30 days, one patient was readmitted (3.45%). Our data confirmthe SS-TAVR as a feasible and safe alternative with comparable results to established approaches in patients who are unsuitable for femoral artery access and offers clinicians another access site in patients with very complex anatomy.

NAFTNet retrospective report on the treatment of anti-Ro/SSA mediated fetal heart block with dexamethasone

Background Complete atrioventricular block (CAVB) is a complication of maternal antibody positivity and treatment of fetal disease is controversial in terms of efficacy and safety. We hypothesized that dexamethasone treatment for fetal anti-Ro/SSA antibody-mediated cardiac disease leads to better pregnancy outcomes than expectant management. Methods A retrospective multi-center cohort study of anti-Ro/SSA antibody positive pregnancies with fetal conduction disease reported by participating North American Fetal Therapy Network (NAFTNet) centers between January 2010 and December 2018. The primary outcomes included: fetal death, oligohydramnios, growth restriction, preterm delivery, and new maternal comorbidities. Secondary outcomes included: pacemaker prior to 28 days, transplantation, and neonatal death in maternal/fetal dyads treated with dexamethasone versus not. Results In 127 anti-Ro/SSA positive pregnancies, 98 were treated with dexamethasone and 29 were not. Of those treated, 61/96 (63.5%) met the primary outcome including 45/91 (49.4%) premature deliveries; 20 mothers developed comorbidities during treatment (fetal death 5, 10 growth restriction, 14 oligohydramnios, two new/worsening gestational diabetes). In the untreated group, 15/25 (60%) met the primary outcome including 11/22 (50%) premature deliveries and four mothers developing comorbidities during their pregnancy (fetal death 3, one growth restriction, one new onset maternal hypertension). Regarding secondary outcomes, 37/96 (43%) treated fetuses required a pacemaker or died by 28 days, while untreated 13/25 (52%) required pacemaker placement, died prior to 28 days or required listing for transplantation. Excluding terminations, survival without transplant was 17 (68%) in untreated and 85 (89%) in treated patients (p<.01). Conclusions While the use of dexamethasone in anti-Ro/SSA positive pregnancies is associated with a high rate of poor pregnancy outcomes, there was an unexpected similarly high rate in untreated positive pregnancies. This suggests that the maternal disease itself is influencing pregnancy complications independent of dexamethasone. Our data, which show that treatment decreases neonatal morbidity and overall mortality without increasing overall pregnancy complications, warrant further study.

Complete Heart Block in a Patient with COVID-19

The severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) pandemic has been reported to cause cardiac complications including myocarditis, acute myocardial infarction (AMI) and various tachyarrhythmias. We present a 63-year-old male who developed nonreversible symptomatic bradycardia due to advanced atrioventricular block (AV) requiring pacemaker placement in the setting of severe SARS-CoV-2 pneumonia.

Senning With Aortic Translocation and Anatomic Repair for Congenitally Corrected Transposition

Anatomic repair for congenitally corrected transposition of the great arteries with ventricular septal defect (VSD) and pulmonic stenosis has been accomplished with atrial switch and Rastelli. Aortic translocation offers a direct left ventricular outflow without an extraanatomic right ventricular-to-pulmonary conduit, which may lead to decreased reoperations. We reviewed our entire experience performing Senning with aortic translocation (SAT). From 2007 to 2017, 8 patients (mean age, 14.1 months; size, 8.86 kg) underwent SAT. Associated anomalies included situs inversus (n= 2), dextrocardia (n= 6), multiple muscular VSDs (n= 2), abnormal or straddling atrioventricular valve chords (n= 5), and branch pulmonary artery stenosis (n= 3). Four of 8 had previous systemic arterial shunts. Mean cardiopulmonary bypass was 487 minutes, and mean cardiac ischemic time was 307 minutes. Additional procedures included repair of branch pulmonary artery stenoses and closure of multiple muscular VSDs. There was no hospital death. One patient was supported with extracorporeal membrane oxygenation because of junctional tachycardia on postoperative day 5. One patient required pacemaker placement for first-degree heart block. Median hospital length of stay was 31 days. Mean length of follow-up was 52 months. All patients remain well with mild or no aortic regurgitation. The first patient underwent a repeat surgical operation for pulmonary venous baffle obstruction 2 years after SAT. Despite the technical complexity, patient outcomes have been satisfactory. We believe SAT provides a superior anatomic repair in these complex defects. Longer-term follow-up is needed regarding late intervention.

CARDIAC AA AMYLOIDOSIS SECONDARY TO FAMILIAL MEDITERRANEAN FEVER PRESENTING AS INTERMITENT COMPLETE HEART BLOCK: A RARE CASE WITH LITERATURE REVIEW

SESSION TITLE: Medical Student/Resident Cardiovascular Disease Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: Cardiac amyloidosis is rare but relatively more frequently seen with ATTR and AL types of amyloidosis. AA amyloidosis related cardiomyopathy or conduction abnormalities are rare owing to less frequent involvement of the myocardium in AA amyloidosis. We present a case of complete heart block in the setting of AA amyloidosis secondary to familial mediterranean fever. CASE PRESENTATION: A 50-year-old female with history of familial mediterranean fever (FMF) presented with syncopal episode. On presentation patient's vitals were stable, labs including TSH were within normal limits. Orthostatic vitals were positive. High-sensitivity troponins were negative with a normal EKG on presentation. Laterally displaced apical beat was noticed on examination. During hospitalization patient was noticed to have complete heart block that was not responsive to atropine and transcutaneous pacing however a response was noticed with epinephrine drip. TTE revealed heart failure with reduced ejection fraction of 41% with severe concentric left ventricular thickening typical for amyloid cardiomyopathy with a well-preserved E/A-wave, which is not usually seen in as thickened ventricle as patients. CRP and SAA were elevated. Patient had endomyocardial biopsy that confirmed amyloid deposition staining positive for amyloid AA and negative for TTR, kappa and lambda and a technetium pyrophosphate scan was also negative. A biventricular pacemaker was placed for complete heart block without adequate escape rhythm, which resulted in resolution of episodes of dizziness associated with bradycardia. Patient's FMF was managed with colchicine and anakinra with the help of rheumatology. Patient was started on diuresis during hospitalization and continued on discharge. Patient followed up with amyloidosis clinic after discharge. DISCUSSION: Systemic amyloidosis is rare and results from deposition of amyloid deposits in various organs and hence a wide range of effects. Prognosis may be vary with the type and extent of amyloidosis. More than 95% of cardiac amyloidosis is secondary to light chain/AL amyloidosis or transthyretin amyloidosis (ATTR), both genetic/hereditary and wild type.[1] Cardiac involvement from AA amyloidosis is very rare. Cardiac amyloidosis usually manifests with syncope or postural hypotension, which may be related with bradyarrhythmias.[2] Like the involvement of cardiovascular system, developments of arrhythmias are also common with ATTR type of amyloidosis as compared to other types and may require pacemaker placement. AA cardiac amyloidosis is rare and bradyarrhythmias or conduction system involvement has also been rarely reported. We report a rare case of complete heart block secondary to AA amyloidosis from familial Mediterranean fever requiring pacemaker placement. CONCLUSIONS: We present cardiac AA amyloidosis as a rare cause of syncope and orthostatic hypotension. Reference #1: Sipe, J.D., et al., Amyloid fibril proteins and amyloidosis: chemical identification and clinical classification International Society of Amyloidosis 2016 Nomenclature Guidelines. Amyloid, 2016. 23(4): p. 209-213. Reference #2: Chamarthi, B., et al., Features and prognosis of exertional syncope in light-chain associated AL cardiac amyloidosis. Am J Cardiol, 1997. 80(9): p. 1242-5 DISCLOSURES: No relevant relationships by Usama Talib, source=Web Response

Clinical outcomes and need for intensive care after non-ST-segment-elevation myocardial infarction

BackgroundThe decision on whether non-ST-segment elevation myocardial infarction (NSTEMI) patients should be admitted to intensive care units (ICU) takes into account several factors including hospital routines. The Acute Coronary Treatment and Intervention Outcomes Network (ACTION) ICU score was developed to predict complications requiring ICU care post-NSTEMI. MethodsWe described patient characteristics and clinical outcomes of 1263 NSTEMI patients admitted to a private hospital in Sao Paulo, Brazil, from 2014 to 2018. We also aimed to retrospectively identify NSTEMI patients who might not have needed to be admitted to the ICU based on the ACTION ICU risk score. We defined complications requiring ICU care post-NSTEMI as cardiac arrest, cardiogenic shock, stroke, re-infarction, death, heart block requiring pacemaker placement, respiratory failure, or sepsis. ResultsMean age was 62.3 years and 35.8% were female. A total of 94.6% of NSTEMI patients were admitted to the ICU. Most NSTEMI patients (91.9%) underwent coronary angiography. Percutaneous coronary intervention was performed in 47.1% and coronary artery bypass graft surgery in 10.3%. Complications requiring ICU care occurred in 62 patients (4.9%). In-hospital mortality rate was 1.3%. Overall, 70.4% had an ACTION ICU score ≤ 5. The C-statistics for the ACTION risk score to predict complications was 0.55 (95% confidence interval 0.47–0.63). ConclusionsComplications requiring ICU care were infrequent in a cohort of NSTEMI patients who were routinely admitted to the ICU over a 4-year period. The ACTION risk score had low accuracy in the prediction of complications requiring ICU care in our population.

Electrocardiographic and clinical predictors of permanent pacemaker insertion following Perceval sutureless aortic valve implantation

BackgroundSutureless aortic valve replacement surgery (AVR) is a reasonable alternative surgical approach in those patients with aortic stenosis who would benefit from reduced cross clamp time, such as elderly and high-risk patients. We sought to evaluate the incidence of pacemaker (PM) implantation following sutureless AVR and to analyse possible pre-operative electrocardiographic and clinical predictors of PM implantation. Methods and resultsBetween November 2013 and March 2015, 58 patients (male 43%, age 77.9 ± 4.9 years) having undergone sutureless AVR with a Perceval prosthesis (Sorin Biomedica Cardio Srl, Saluggia, Italy) were taken into consideration for our analysis. During a mean follow up of 13.8 ± 5.0 months (median 13 months), 14 patients (24.1%) underwent pacemaker (PM) implantation following sutureless AVR procedure. Among these patients, 12 (86%) presented III degree atrioventricular (AV) block, 1 (7%) presented II degree AV block, and remaining one (7%) severe symptomatic bradycardia. The comparison of pre-operative characteristics between PM group and no PM group highlighted that QRS duration, EuroSCORE II index and chronic renal dysfunction were significantly associated with the development of AV conduction abnormalities/symptomatic bradycardia requiring PM implantation (respectively, p = 0.01, p = 0.02 and p = 0.03). ConclusionsThe incidence of PM implantation after sutureless AVR was 24.1% in the present study. The EuroSCORE II, QRS duration and renal dysfunction were significantly associated with higher risk of AV conduction abnormalities/symptomatic bradycardia requiring PM placement.

PACEMAKER FOLLOWING TRANSCATHETER AORTIC VALVE REPLACEMENT ASSOCIATED WITH INCREASED TRICUSPID REGURGITATION

Transcatheter Aortic Valve Replacement (TAVR) therapies have increased in the treatment of aortic disease. As TAVR procedures increase, more data is available on complications, such as the development of conduction abnormalities, often requiring pacemaker placement (PMP). A common complication of

Rastelli Operation for D-Transposition of the Great Arteries, Ventricular Septal Defect, and Pulmonary Stenosis.

Our preferred approach for the surgical treatment of patients with D-transposition of the great arteries, ventricular septal defect, and pulmonary stenosis has been the Rastelli operation. We herein evaluate our 30-year experience with this procedure. Clinical records for patients who underwent the Rastelli operation between 1988 and 2017 at our institution were retrospectively reviewed. Primary outcomes included freedom from death or cardiac transplantation and freedom from conduit reintervention. Forty-seven patients met inclusion criteria. Mean follow-up was 11.7 ± 6.8 years. Forty-three (91.5%) patients received a palliative systemic-to-pulmonary artery shunt and/or atrial septostomy prior to the Rastelli procedure. Five (10.6%) patients required ventricular septal defect enlargement at the time of the Rastelli procedure. The overall mean right ventricle-to-pulmonary artery conduit size was 17.0 mm. Mortalities included one early and three late deaths. Freedom from death or cardiac transplantation was 93% and 84% at 5 and 25 years, respectively. Seven patients required pacemaker placement, two immediately postoperatively and five late. Freedom from conduit replacement was 85% and 25% at 5 and 15 years, respectively. Seven (14.9%) patients required a second conduit intervention. Forty-one (87.2%) patients were New York Heart Association class I or II at the most recent follow-up. The Rastelli operation for D-transposition of the great arteries, ventricular septal defect, and pulmonary stenosis offers excellent mid- to long-term survival. The need for conduit replacement remains the most common indication for reintervention, and further study of the optimal choice of conduit will be useful.

234: NAFTNET retrospective report on dexamethasone and fetal heart block

Complete atrioventricular block (CAVB) occurs in 1-2% of SSA positive pregnancies and the consequences are significant: approximately 20% die and 50% require permanent cardiac pacing by 1 year of age. We hypothesize that dexamethasone (DEX) treatment for fetal anti-SSA/SSB antibody-mediated cardiac disease leads to better pregnancy outcome. Therefore, we assessed recent era experience of treatment with DEX of pregnancies with SSA/SSB-mediated fetal cardiac disease including all degrees of heart block on pregnancy, neonatal outcome and complication rates. We performed a retrospective multi-center cohort study of all SSA/SSB antibody positive pregnancies with fetal conduction system disease reported to the participating NAFTNet centers between January 2010 through December 2017. The primary outcomes included preterm delivery, maternal comorbidities, neonatal complications and secondary outcomes were, need for a pacemaker < 28 days, heart transplantation in the 1st year of life, and intrauterine or neonatal death in maternal/fetal days treated with DEX versus managed expectantly. There was a total of 101 SSA/SSB positive pregnancies of which 75 were treated with DEX and 26 were not. Of those treated, 49/75 (65%) met the primary outcome including 37 premature deliveries; 12 mothers developed comorbidities during treatment (4 with fetal growth restriction, 4 with oligohydramnios, 2 with new or worsening gestational diabetes, and 6 with mood disturbances). In comparison, in the untreated group 11/26 (42%) met the primary outcome of premature delivery. Regarding secondary outcomes, 32/75 (43%) dexamethasone-treated fetuses either required a pacemaker or had a fetal demise/died by 28 days of life, while 13/26 (50%) untreated met the secondary outcome of requiring pacemaker placement or fetal demise/dying prior to 28 days of life, while 1 infant required listing for cardiac transplantation. While the use of dexamethasone in SSA and SSB positive pregnancies is associated with a high rate of poor pregnancy outcomes, there was a similarly high rate in untreated SSA/SSB positive pregnancies suggesting that the maternal disease itself is influencing pregnancy complications. A trend suggesting that treatment decreases neonatal morbidity and progression of conduction disease deserves further study.

A Case of Pneumatosis Intestinalis Caused by Systemic Lupus Erythematous

Introduction: Pneumatosis intestinalis (PI) is a radiographic finding with wide spectrum of presentation, ranging from asymptomatic to perforation and death. There are more than 60 known causes of PI, but systemic lupus erythematous is a rare cause, and infrequently described. Case 42 year old woman was admitted with non-bloody diarrhea and left upper quadrant abdominal cramps for one week. She has a history of GERD (status post fundoplication x3), chronic constipation due to pelvic floor dysfunction, SLE with autonomic instability, resulting in hypotension, syncope, and bradycardia requiring pacemaker placement. Her symptoms occurred in the setting of discontinuing her lupus medications (belimumab and leflunomide), tapering her prednisone, and holding IVIG to repeat testing for autonomic dysfunction, as she had worsening episodes of hypotension and bradycardia in Fall 2016, requiring pacemaker placement. She had a similar presentation in 2015, when she was found to have PI in the splenic flexure. Infectious work up was negative at that time, and symptoms did not improve with a week of IV antibiotics. It then resolved with prednisone taper for presumed SLE exacerbation, resulting in PI. CT abdomen this time showed increased prominence of PI at the splenic flexure, a watershed area. She was placed on bowel rest, given high flow oxygen, and started on metronidazole, but remained symptomatic with nausea, bloating and diarrhea. Colonoscopy showed melanosis coli without evidence of colitis or bowel perforation. Patient then improved with a higher dose of prednisone. Discussion SLE is a rare cause of PI, but could contribute to it through several different mechanisms. A mechanism that was proposed by previous case report was SLE causing enteric vasculitis. Antiphospholipid antibodies and/or lupus anticoagulant may affect the gut mucosa resulting in ischemia, although this patient did not have these antibodies. The patient's autonomic dysfunction may have resulted in hypotensive episodes that could damage the watershed area.

Sinoatrial node toxicity after stereotactic ablative radiation therapy to lung tumors

PurposeStereotactic ablative radiation therapy (SABR) is an established treatment for selected lung tumors. Sinoatrial node (SAN) toxicity after thoracic SABR has not been reported in the literature. We sought to understand the risk of SAN toxicity owing to incidental dose to the SAN from SABR. Methods and materialsWe conducted a retrospective review of patients with early-stage lung cancer or limited pulmonary metastases who underwent thoracic SABR to a right-sided central lung tumor (within 2 cm of the mainstem bronchus or other mediastinal structures) between January 2008 and December 2014, analyzed a subset whose treatment imparted dose to the SAN exceeding 10% of the prescription dose, and examined patient and treatment dosimetric characteristics. Mean follow-up interval was 28 months. Time to toxicity was defined from start of SABR. ResultsOf 47 patients with central tumors in the right lung treated with SABR reviewed, 13 met our study criteria. A contouring atlas of regional cardiac anatomy was created. One patient treated with SABR for non-small cell lung cancer at the right hilum developed symptomatic sick sinus syndrome, requiring pacemaker placement 6 months after treatment. Her acute presentation and short interval between SABR and onset of symptoms suggest that SAN toxicity was likely due to radiation-induced injury. Both her age and mean dose to her SAN were the third highest in our cohort. She remained free from cancer progression at 24 months’ follow-up. Twelve additional patients who received significant dose to the SAN from SABR did not develop toxicity. ConclusionWhile uncommon, SAN toxicity from SABR to right-sided central thoracic tumors should be recognized and followed in future studies.

Sinoatrial node dysfunction after stereotactic ablative radiation therapy in the chest.

132 Background: Sinoatrial node (SAN) injury following stereotactic ablative radiation therapy (SABR) in the chest has not been reported in the literature. We report SAN dysfunction as a potential toxicity of SABR in the chest. Methods: We examined the clinical courses and SABR plans of 47 patients treated for T1 or T2 non-small cell lung cancer of the right upper lobe, middle lobe, lower lobe, or hilum. After developing a contouring atlas for the SAN, based upon the junction between the superior vena cava and the right atrium, dose to the SAN was retrospectively determined for each patient. We identified 13 patients whose treatment imparted significant dose to the SAN, as defined by the SAN encompassed within the 10% prescription isodose (IDL) line. Biologically effective doses (BED), acute and chronic, were correlated with SAN toxicity. Results: Patients underwent SABR to the right lung to a dose of 40-50 Gy in 4 or 5 fractions, with mean acute BED (alpha/beta ratio = 10) of 100.2 Gy and late BED (alpha/beta ratio = 3) of 222.7 Gy. Mean volume of the GTV and PTV were 26.1 and 75.1 mL, respectively. Mean follow-up was 25.5 months. The mean volume of the SAN was 0.37 mL. Average max dose and mean dose to the SAN were 24.7 and 70.7 Gy, respectively. Of the 13 patients whose treatment imparted significant dose to the SAN, one patient without prior arrhythmia developed symptomatic SAN dysfunction requiring pacemaker placement at 6 months after completion of treatment. The sinoatrial node of the patient received a maximum dose of 44.8 Gy in 4 fractions, correlated with an acute BED (alpha/beta ratio = 10) of 90 Gy and late BED (alpha/beta ratio = 3) of 194.1 Gy, and a mean dose of 35.5 Gy in 4 fractions, correlated with an acute BED (alpha/beta ratio = 10) of 71 Gy and late BED (alpha/beta ratio = 3) of 153.8 Gy. This was the third highest max dose and second highest mean dose to SAN in the cohort. Conclusions: We report sinoatrial node dysfunction as a potential toxicity of SABR in the chest for non-small cell lung cancer. Caution is advised when treatment imparts significant dose to the sinoatrial node.

Intermediate-Term Results After Extracardiac Conduit Fontan Palliation in Children and Young Adults with Single Ventricle Physiology-A Single-center Experience.

The Fontan operation and its modifications are currently the standard palliation in patients with single ventricle physiology. The advantages of extracardiac conduit Fontan operation (ECF) have previously been described. We evaluated the outcome of patients who underwent ECF operation at our center since the year 2000 to assess morbidity, mortality and New York Heart Association (NYHA) functional class in the current era. Medical records of 97 patients who underwent ECF operation from November 2000 till October 2013 were reviewed to determine the diagnosis, age at surgery, surgical technique, postoperative complications, NYHA class at last follow-up, echocardiographic parameters, and most recent Holter monitor results. The median age at surgery was 3.4years with 4 total deaths until last follow-up (hospital mortality was 1%, and mortality after hospital discharge was 3%). Seventy-eight of the 97 patients (80%) underwent fenestrated Fontan surgery. Median duration of follow-up was 3.9years (IQR 0.2-12.2years). At the latest follow-up, 62 patients (67%) were determined to be in NYHA class I, 25 patients (27%) in NYHA class II, and 6 (6%) patients in NYHA class III, and the median oxygen saturation was 92% (IQR 86-96). There were 15 patients with history of preoperative arrhythmias and 6 of them required pacemaker placement at the time of surgery. One patient developed ectopic atrial tachycardia, and 1 patient had clinical thromboembolism on follow-up. Systolic function of the single ventricle by transthoracic echocardiogram at the latest follow-up was described as normal in 77 patients (83%), fair in 15 patients (16%), and poor in 1 patient (1%). The results of our study show that patients who undergo extracardiac conduit Fontan operation in the modern era may have significantly less morbidity and mortality when compared to earlier years.