Pattern of Cleft Lip and Palate Clefts at a Tertiary Care Hospital in Saudi Arabia.

BACKGROUND: Clefts of the lip and palate (CL/P) and cleft palate (CP) are the most common craniofacial congenital anomalies. Clefts are classified as syndromic and nonsyndromic. Nonsyndromic clefts have no known genetic causes. OBJECTIVES: This study combines prospective and retrospective studies to review the patterns of CL/P and CP and associated syndromes and conditions in patients registered for CL/P surgery at a tertiary care pediatric center in our tertiary care hospital in Saudi Arabia. METHODS: It included patient data from May 2015 through April 2023. Patient record forms and SPSS (IBM version 20.0) were used to collect and analyze data. A significance level of 5% was used, with p ≤ 0.05 considered statistically significant. RESULTS: Of the 319 patients who met our inclusion criteria, 175 were male. Of the total, 99 had a left unilateral isolated cleft lip, 61 had a right unilateral isolated cleft lip, 69 had a bilateral cleft lip, and 90 had an isolated CP. Of the total, 140 had CL/P. Around 242 were nonsyndromic. The Chi-square test revealed a significant association between the prevalence of isolated CP and CLP and gender. The prevalence of left unilateral isolated cleft lip and bilateral and isolated CP was significantly associated with syndromic and nonsyndromic cases. CONCLUSION: Males are more likely to be affected by orofacial clefts, which is consistent with the global trend. Isolated CP was the most common orofacial cleft. Within the sample, syndromes' association with orofacial clefts was significantly weaker than that of isolated and bilateral clefts.

Atrial Fibrillation Burden: Impact on Stroke Risk and Beyond.

Atrial fibrillation (AF) is an important independent risk factor for stroke. Current guidelines handle AF as a binary entity with risk driven by the presence of clinical risk factors, which guides the decision to treat with an oral anticoagulant. Recent studies in the literature suggest a dose-response relationship between AF burden and stroke risk, in both clinical AF and subclinical atrial fibrillation (SCAF), which differs from current guidance to disregard burden and utilize clinical risk scores alone. Within clinical classification and at the same risk levels in various scores, the risk of stroke increases with AF burden. This opens the possibility of incorporating burden into risk profiles, which has already shown promise. Long-term rhythm monitoring is needed to elucidate SCAF in patients with stroke. Recent data from randomized trials are controversial regarding whether there is an independent risk from AF episodes with a duration of less than 24 h, including the duration of SCAF greater than six minutes but less than 24 h.

Fusion pacing in patients with right bundle branch block who undergo cardiac resynchronization therapy.

PURPOSE: Patients with right bundle branch block (RBBB) are less likely to respond to cardiac resynchronization therapy (CRT). We aimed to assess whether patients with RBBB respond to CRT with biventricular fusion pacing. METHODS: Consecutive patients with RBBB at a single tertiary care center, who were implanted with a CRT device capable of biventricular fusion pacing using SyncAV programming, were assessed and compared to a historical cohort of CRT patients with RBBB. QRSd was measured and compared during intrinsic conduction, nominal CRT pacing and manual electrocardiogram-based optimized SyncAV programming. Left ventricular ejection fraction (LVEF) was also compared before and 6 months after CRT. RESULTS: We included 8 consecutive patients with RBBB (group 1) who were able to undergo SyncAV programming and 16 patients with RBBB (group 2) from a historical cohort. In group 1, compared to mean intrinsic conduction QRSd (155 ± 13 ms), mean nominally-paced QRSd was 156 ± 15 ms (ΔQRSd 1.3 ± 11.6; p = 0.77) and SyncAV-optimized paced QRSd was 135 ± 14 ms (ΔQRSd -20.0 ± 20.4; p = 0.03 and ΔQRSd -21.3 ± 16.3; p = 0.008; compared to intrinsic conduction and nominal pacing respectively). In group 2, mean QRSd with nominal pacing was 160 ± 24 ms (ΔQRSd 3.8 ± 33.4; p = 0.66 compared to intrinsic conduction). In group 1, baseline LVEF was 22.1 ± 11.5 and after 6 months of follow-up was 27.8 ± 8.6 (p = 0.047). In group 2, the baseline LVEF was 27.2 ± 10.6 and after 6 months of follow-up was 25.0 ± 10.0 (p = 0.45). CONCLUSIONS: CRT programed to allow biventricular fusion pacing significantly improved electrical synchrony and LVEF in patients with RBBB. Larger studies are required to confirm these findings.

Nonalcoholic Fatty Liver Disease and Coronary Artery Disease: Big Brothers in Patients with Acute Coronary Syndrome.

BACKGROUND: The prevalence of nonalcoholic fatty liver disease (NAFLD) has been increasing. This study aimed to evaluate the prevalence of NAFLD, as diagnosed by ultrasound, in patients with acute coronary syndrome (ACS) and to assess whether NAFLD is associated with the severity of coronary obstruction as diagnosed by coronary angiography. METHODS: We performed a prospective single-center study in patients hospitalized due to acute coronary syndrome who underwent diagnostic coronary angiography. Consecutive patients who presented to the emergency room were diagnosed with acute coronary syndrome and were included. All patients underwent ultrasonography of the upper abdomen to determine the presence or absence of NAFLD; NAFLD severity was graded from 0 to 3 based on a previously validated scale. All patients underwent diagnostic coronary angiography in the same hospital, with the same team of interventional cardiologists, who were blinded to the patients' clinical and ultrasonographic data. CAD was then angiographically graded from none to severe based on well-established angiographic criteria. RESULTS: This study included 139 patients, of whom 83 (59.7%) were male, with a mean age of 59.7 years. Of the included patients, 107 (77%) patients had CAD, 63 (45%) with serious injury. Regarding the presence of NAFLD, 76 (55.2%) had NAFLD including 18 (23.6%) with grade III disease. In severe CAD, 47 (60.5%) are associated with NAFLD, and 15 (83.3%) of the patients had severe CAD and NAFLD grade III. CONCLUSIONS: NAFLD is common in patients with ACS. The intensity of NAFLD detected by ultrasonography is strongly associated with the severity of coronary artery obstruction on angiography.

Anticoagulation after catheter ablation of atrial fibrillation: An unnecessary evil? A systematic review and meta-analysis.

BACKGROUND: Anticoagulation in patients with atrial fibrillation (AF) is currently based on clinical parameters (CHA2 DS 2 -VASc score) that have been shown to predict cerebrovascular events (CVE). Controversy exists as to whether CVE risk persists unmodified after successful catheter ablation, as observational studies suggest a lower risk of CVE. Current guidelines recommend continued oral anticoagulation (OAC) based on the CHA 2 DS 2 -VASc score risk profile. METHODS: We conducted a systematic literature review of all studies published up to July 31, 2018, that reported CVE after catheter ablation of AF and compared patients on or off OAC. Random-effects models were used to demonstrate the risk of CVE and major bleeding in on-OAC vs off-OAC patients. This analysis was further stratified by CHADS2 and CHA 2 DS 2 -VASc score. RESULTS: We retained 16 studies, 10 prospective cohort and 6 retrospective cohort, that met inclusion criteria, and which enrolled 25 177 patients: 13 166 off-OAC and 12 011 on-OAC. No significant difference in the incidence of CVE emerged between on-OAC and off-OAC patients after AF ablation (risk ratio, 0.66; confidence interval [CI], 0.38, 1.15). Similar results were found after stratification by CHADS2 and CHA 2 DS 2 -VASc score. Off-OAC patients suffered significantly less bleeding than those on OAC (RR, 0.17; CI, 0.09, 0.34). Of note, the percentage of patients with AF recurrence impacts the treatment effect in the two groups ( P = 0.001). CONCLUSIONS: In this metanalysis, the risk-benefit ratio favored the suspension of OAT after successful AF ablation even in patients at moderate-high risk. Whether the reported results can be extended also to non-vitamin K antagonist oral anticoagulants warrants further investigations.

Anti-arrhythmic therapy in patients with non-ischemic cardiomyopathy.

Implantable cardiac defibrillators (ICD) are the foundation of therapy for the prevention of sudden cardiac death. While ICDs prevent SCD, they do not prevent the occurrence of ventricular arrhythmias which are usually symptomatic. Though catheter ablation has been successful in substrate modification of ventricular tachycardia in patients with ischemic cardiomyopathy, there is much less evidence to support its use in non-ischemic cardiomyopathy. Therefore, anti-arrhythmic drugs (AADs) are an essential adjunctive therapy for secondary prevention of ventricular arrhythmias in patients with non-ischemic cardiomyopathy. In patients with hypertrophic cardiomyopathy (HCM) and dilated cardiomyopathy (DCM), the prevalence of ventricular arrhythmias correlates with the volume of scar as characterized by late gadolinium enhancement. Beta-blockers forms the cornerstone of treatment to prevent ventricular arrhythmias in both HCM and DCM. Disopyramide is an important therapeutic option in HCM as it provides both negative inotropy which reduces obstruction as well as lass I anti-arrhythmic action. In DCM sotalol, through is combined beta-blocking and class III AD effects, significantly reduces the burden of ventricular arrhythmias. Though amiodarone is efficacious in the prevention of ventricular arrhythmias in both HCM and DCM, its use is limited by its side-effects profile. Evidence for AAD therapy for arrhythmogenic right ventricular dysplasia (ARVD) is limited by its low prevalence and lack of studies. ICDs have been shown to reduce SCD regardless of whether patients are receiving AAD therapy.

Anti-arrhythmic drug therapy in implantable cardioverter-defibrillator recipients.

Implantable cardioverter-defibrillators (ICDs) have revolutionized the primary and secondary prevention of patients with ventricular arrhythmias. However, the adverse effects of appropriate or inappropriate shocks may require the adjunctive use of anti-arrhythmic drugs (AADs). Beta blockers are the cornerstone of pharmacological primary and secondary prevention of ventricular arrhythmias. In addition to their established efficacy at reducing the incidence of ventricular arrhythmias, beta-blockers are safe with few side effects. Amiodarone is superior to beta blockers and sotalol for the prevention of ventricular arrhythmia recurrence. However, long-term amiodarone use is associated with significant side effects that limit its utility. Sotalol and mexiletine are the main alternatives to amiodarone with a better side effect profile though they are less efficacious at preventing ventricular arrhythmia recurrence. Dofetilide, azimilide and ranolazine are emerging as therapeutic options for secondary prevention; more studies are needed to assess efficacy and safety in comparison to currently used agents. Beta blockers and amiodarone are the mainstay of therapy in patients experiencing electrical storm; their use reduces the frequency of ventricular arrhythmias and ICD intervention as well as affording time until catheter ablation can be considered.

Catheter ablation for atrial fibrillation in heart failure with reduced ejection fraction: a systematic review and meta-analysis of randomized controlled trials.

BACKGROUND: Previous randomized controlled trials (RCT)s showed similar outcomes in patients with atrial fibrillation (AF) and heart failure with reduced ejection fraction (HFrEF) treated with anti-arrhythmic drugs (AAD) compared to rate control therapy. We sought to evaluate whether catheter ablation is superior to medical therapy in patients with AF and HFrEF. METHODS: We searched electronic databases for all RCTs that compared catheter ablation and medical therapy (with or without use of AAD). We used random-effects models to summarize the studies. The primary end-point was all-cause mortality. Secondary outcomes included heart failure-related hospitalizations and change in left ventricular ejection fraction (LVEF). RESULTS: We retrieved and summarized 7 randomized controlled trials, enrolling 856 patients (429 in the catheter ablation arm and 427 in the medical therapy arm). Compared with medical therapy (including use of AAD), AF catheter ablation was associated with a significant reduction in mortality (risk ratio 0.50; 95% confidence interval [CI]: 0.34 to 0.74; P = 0.0005) and heart failure-related hospitalizations (risk ratio 0.56; 95% CI: 0.44 to 0.71; P < 0.0001). Furthermore, catheter ablation led to significant improvements in LVEF (weighted mean difference, 7.48; 95% CI: 3.71 to 11.26; P < 0.0001). CONCLUSIONS: Compared to medical therapy, including use of AAD, catheter ablation for AF was associated with a significant reduction in mortality and heart failure-related hospitalizations as well as an improvement in LVEF in patients with HFrEF. Larger trials are needed to confirm whether rhythm control with ablation is superior to rate control in patients with AF and heart failure.

Maximizing biventricular pacing in patients with rate-controlled atrial fibrillation using ventricular sense response.

In patients with atrial fibrillation (AF), cardiac resynchronization therapy (CRT) is challenging because the ventricular rate of conducted AF exceeds the biventricular pacing rate. In the current report, we present a patient who received a CRT device that was programmed to ventricular sense response (VSR) on with VVI 40 beats per minute to allow the AF to be paced as fusion beats. We found that the pacing configuration resulting in the narrowest QRS in this patient was VVI 40 with VSR biventricular fusion pacing during AF. VSR mode allows for CRT delivery without the need to artificially increase heart rate.

Predicting response to cardiac resynchronization therapy: Use of strict left bundle branch block criteria.

BACKGROUND: Cardiac resynchronization therapy (CRT) reduces morbidity and mortality in heart failure with reduced ejection fraction (HFrEF). CRT efficacy is greater in left bundle branch block (LBBB). This study aimed to determine if strict LBBB criteria predict an improved QRS duration and left ventricular ejection fraction (LVEF) response after CRT. METHODS: HFrEF patients who received a CRT device at a single quaternary center were included. Patients were divided into three groups based on baseline QRS morphology. Group 1 consisted of patients with strict LBBB. Group 2 had conventional LBBB, and group 3 had non-LBBB morphology. Outcomes assessed included change in QRS duration after CRT, change in LVEF, and all-cause mortality. RESULTS: In 231 patients, 56% of patients were in group 1, 29% were in group 2, and 15% were in group 3. Patients with strict LBBB had a significant reduction in QRS duration (-20.9 ± 12.4 ms) compared to conventional LBBB (6.7 ± 19.4 ms; P < 0.0001) and non-LBBB (3.9 ± 29.3 ms; P < 0.0001). Patients with strict LBBB had a significant increase in LVEF (19.5 ± 10.2) compared to conventional LBBB (5.3 ± 12.6; P < 0.0001) and non-LBBB (-1.3 ± 10.9; P < 0.0001). There was moderate negative correlation between changes in QRS duration and LVEF (correlation coefficient = -0.63, P < 0.0001). Strict LBBB criteria were associated with a significant reduction in mortality compared to conventional LBBB (odds ratio 0.49, 95% confidence interval 0.24 to 0.99; P = 0.046). CONCLUSIONS: Strict LBBB predicted a reduction in QRS duration and an increase in LVEF compared to conventional LBBB and non-LBBB morphology in patients with HFrEF who received CRT.

Cardiac resynchronization therapy reprogramming to improve electrical synchrony in patients with existing devices.

BACKGROUND: Optimal programming of cardiac resynchronization therapy (CRT) has not yet been fully elucidated. A novel algorithm (SyncAV) has been developed to improve electrical synchrony by fusion of the triple wavefronts: intrinsic, right ventricular (RV)-paced, and left ventricular (LV)-paced. METHODS: Consecutive patients at a single tertiary care center with a previously implanted CRT device with SyncAV algorithm (programmable negative AV hysteresis) were evaluated. QRS duration (QRSd) was measured during 1) intrinsic conduction, 2) existing CRT pacing as chronically programmed by treating physician, 3) using the device-based QuickOpt™ algorithm for optimization of AV and VV delays, and 4) ECG-based optimized SyncAV programming. The paced QRSd was assessed and compared to intrinsic conduction and between the different modes of programming. RESULTS: Of 64 consecutive, potentially eligible patients who underwent assessment, 34 patients who were able to undergo SyncAV programming were included. Mean intrinsic conduction QRSd was 163 ±â€¯24 ms. In comparison, the mean QRSd was 152 ±â€¯25 ms (-11.1 ±â€¯19.0) during existing CRT pacing, 160 ±â€¯25 ms (-4.1 ±â€¯25.2) using the QuickOpt™ algorithm and 138 ±â€¯23 (-24.9 ±â€¯17.2) using ECG-based optimized SyncAV programming. SyncAV optimization resulted in significant reductions in QRSd compared to existing CRT pacing (P = 0.02) and QuickOpt™ (P < 0.001). Of the 32% of patients who did not have QRS narrowing with existing CRT, 72% experienced QRS narrowing with SyncAV. CONCLUSION: ECG-based atrio-ventricular delay optimization using SyncAV significantly improved electrical synchrony in patients with a previously implanted CRT. Further studies are needed to assess the impact on long-term outcomes.

Subclinical Atrial Fibrillation and Risk of Stroke: Past, Present and Future.

Subclinical atrial fibrillation (SCAF) describes asymptomatic episodes of atrial fibrillation (AF) that are detected by cardiac implantable electronic devices (CIED). The increased utilization of CIEDs renders our understanding of SCAF important to clinical practice. Furthermore, 20% of AF present initially as a stroke event and prolonged cardiac monitoring of stroke patients is likely to uncover a significant prevalence of SCAF. New evidence has shown that implanting cardiac monitors into patients with no history of atrial fibrillation but with risk factors for stroke will yield an incidence of SCAF approaching 30-40% at around three years. Atrial high rate episodes lasting longer than five minutes are likely to represent SCAF. SCAF has been associated with an increased risk of stroke that is particularly significant when episodes of SCAF are greater than 23 h in duration. Longer episodes of SCAF are incrementally more likely to progress to episodes of SCAF >23 h as time progresses. While only around 30-40% of SCAF events are temporally related to stroke events, the presence of SCAF likely represents an important risk marker for stroke. Ongoing trials of anticoagulation in patients with SCAF durations less than 24 h will inform clinical practice and are highly anticipated. Further studies are needed to clarify the association between SCAF and clinical outcomes as well as the factors that modify this association.

The Role of Cardiovascular and Metabolic Comorbidities in the Link between Atrial Fibrillation and Cognitive Impairment: An Appraisal of Current Scientific Evidence.

Atrial fibrillation (AF) is the most common arrhythmia encountered in clinical practice with implications on long-term outcomes. Metabolic disorders including diabetes mellitus and obesity are independent predictors of atrial fibrillation and present therapeutic targets to reduce both the incidence and duration burden of atrial fibrillation. The presence of pericardial fat in direct contact with cardiac structures, as well the subsequent release of proinflammatory cytokines, may play an important role in this connection. Atrial fibrillation is an independent predictor of cognitive impairment and dementia. While clinical stroke is a major contributor, other factors such as cerebral hypoperfusion and microbleeds play important roles. New evidence suggests that atrial fibrillation and cognitive impairment may be downstream events of atrial cardiomyopathy, which may be caused by several factors including metabolic syndrome, obesity, and obstructive sleep apnea. The mechanisms linking these comorbidities to cognitive impairment are not yet fully elucidated. A clearer understanding of the association of AF with dementia and cognitive impairment is imperative. Future studies should focus on the predictors of cognitive impairment among those with AF and aim to understand the potential mechanisms underlying these associations. This would inform strategies for the management of AF aiming to prevent continued cognitive impairment.

Targeting nonpulmonary vein triggers during atrial fibrillation ablation: is the game worth the candle?

PURPOSE OF REVIEW: Triggers for atrial fibrillation are found outside the pulmonary veins in 12-20% of cases. The role of addressing these triggers during catheter ablations has not been well defined. Therefore, the aim of this review is to summarize the effect of ablation of nonpulmonary vein triggers in addition to pulmonary vein isolation across the spectrum of atrial fibrillation in patients receiving catheter ablation. RECENT FINDINGS: In paroxysmal atrial fibrillation, an inducible nonpulmonary vein trigger is an independent predictor of recurrence. These triggers are inducible by adenosine and isoproterenol infusion. Nonpulmonary vein triggers cause a significant proportion of atrial fibrillation recurrence seen during repeat procedure and addressing them decreases such recurrence. Targeting inducible nonpulmonary vein triggers also decreases recurrence in persistent atrial fibrillation and was associated with a 25-30% relative reduction in arrhythmia recurrence compared with pulmonary vein isolation alone. In persistent atrial fibrillation, the addition of left atrial appendage isolation was associated with 55% reduction in arrhythmia recurrence. There was no benefit to the empirical ablation of the superior vena cava and the addition of extensive linear lines. There was insufficient evidence to assess the effects of empirical ablation of the coronary sinus, crista terminalis, left atrial posterior wall and the vein of Marshall on arrhythmia recurrence. SUMMARY: Evidence suggests that the presence of an inducible nonpulmonary vein trigger is a strong predictor of arrhythmia recurrence. Efforts to detect and ablate nonpulmonary vein triggers are warranted. Further studies are required to fully identify the role nonpulmonary vein trigger ablation.

Warfarin anticoagulation in hemodialysis patients with atrial fibrillation: comparison of nephrologist-led and anticoagulation clinic-led management.

BACKGROUND: There is conflicting evidence of benefit versus harm for warfarin anticoagulation in hemodialysis patients with atrial fibrillation. This equipoise may be explained by suboptimal Time in Therapeutic Range (TTR), which correlates well with thromboembolic and bleeding complications. This study aimed to compare nephrologist-led management of warfarin therapy versus that led by specialized anticoagulation clinic. METHODS: In a retrospective cohort of chronic hemodialysis patients from two institutions (Institution A: Nephrologist-led warfarin management, Institution B: Anticoagulation clinic-led warfarin management), we identified patients with atrial fibrillation who were receiving warfarin for thromboembolic prophylaxis. Mean TTRs, proportion of patients achieving TTR ≥ 60%, and frequency of INR testing were compared using a logistic regression model. RESULTS: In Institution A, 16.7% of hemodialysis patients had atrial fibrillation, of whom 36.8% were on warfarin. In Institution B, 18% of hemodialysis patients had atrial fibrillation, and 55.5% were on warfarin. The mean TTR was 61.8% (SD 14.5) in Institution A, and 60.5% (SD 15.8) in Institution B (p-value 0.95). However, the proportion of patients achieving TTR ≥ 60% was 65% versus 43.3% (Adjusted OR 2.22, CI 0.65-7.63) and mean frequency of INR testing was every 6 days versus every 13.9 days in Institutions A and B respectively. CONCLUSIONS: There was no statistical difference in mean TTR between nephrologist-led management of warfarin and that of clinic-led management. However, the former achieved a trend toward a higher proportion of patients with optimal TTR. This improved therapeutic results was associated with more frequent INR monitoring.

A Historical Perspective on the Role of Functional Lines of Block in the Re-entrant Circuit of Ventricular Tachycardia.

The ablation strategy for ventricular tachycardia (VT) rapidly evolved from an entrainment mapping approach for identification of the critical isthmus of the re-entrant circuit during monomorphic VT, toward a substrate-based approach aiming to ablate surrogate markers of the circuit during sinus rhythm in hemodynamically nontolerated and polymorphic VT. The latter approach implies an assumption that the circuits responsible for the arrhythmia are anatomical or fixed, and present during sinus rhythm. Accordingly, the lines of block delimiting the channels of the circuits are often considered fixed, although there is evidence that they are functional or more frequently a combination of fixed and functional. The electroanatomical substrate-based approach to VT ablation performed during sinus rhythm is increasingly adopted in clinical practice and often described as scar homogenization, scar dechanneling, or core isolation. However, whether the surrogate markers of the VT circuit during sinus rhythm match the circuit during arrhythmias remains to be fully demonstrated. The myocardial scar is a heterogeneous electrophysiological milieu with complex arrhythmogenic mechanisms that potentially coexist simultaneously. Moreover, the scar consists of different areas of diverse refractoriness and conduction. It can be misleading to limit the arrhythmogenic perspective of the myocardial scar to fixed or anatomical barriers held responsible for the re-entry circuit. Greater understanding of the role of functional lines of block in VT and the validity of the surrogate targets being ablated is necessary to further improve the technique and outcome of VT ablation.

An unusual presentation of pacifier thumb duplication with VACTERL association: Case report and review of literature.

INTRODUCTION AND IMPORTANCE: Polydactyly of the hand is a common anomaly among pediatrics, which can present in conjugation with other syndromes or on its own. Various types can be seen, ranging from the involvement of skin only to a completely formed digit. We report the first case of pacifier type thumb duplication presenting with VACTERL association. Herein, we also summarize the existing literature of the distinctive features and management of pacifier polydactyly. CASE PRESENTATION: A premature male infant with intrauterine growth restriction due to maternal type II diabetes was referred for a soft tissue attachment to the left hand. The infant showed VACTERL association signs, including a single kidney, small atrial septal defect, and ventricular septal defect. Examination revealed preaxial polydactyly with a cystic swelling connected to the palm. CLINICAL DISCUSSION: Preaxial polydactyly is the second most common congenital hand anomaly, and its pathology is thought to involve the disruption of apoptosis during embryonic development. Pacifier-type polydactyly is a unique variation characterized by severe edema of the soft tissue digit, believed to be caused by physical damage in utero. The case presented did not require surgical intervention as the duplicated thumb underwent spontaneous autoamputation as opposed to other cases in the literature. CONCLUSION: This is the first reported case of pacifier-type thumb duplication in a patient with VACTERL association. The presentation of this condition adds to the existing body of literature on VACTERL association. Surgical removal is the treatment of choice for pacifier polydactyly, but spontaneous resolution can occur.