IntroductionVasculopathy of the large vessels commonly occurs in sickle cell disease, and as a result cerebral infarction is a well characterized complication of this condition. However, spinal infarction appears to be rare. Spinal infarct is infrequent in the non-sickle cell population as well, and accounts for only about 1 percent of all central nervous system infarcts.Case presentationIn the present work, we report the case of a 19-year-old African-American man with sickle cell disease who experienced an anterior spinal infarct and subsequent quadriplegia. He was incidentally noted to be a heterozygote for factor V Leiden. We also reviewed the literature and found two previous cases of spinal cord infarction and sickle hemoglobin. Our literature search did not demonstrate that heterozygocity for factor V Leiden plays an important role in spinal cord infarction.ConclusionsThe paucity of cases associated with sickle hemoglobin does not allow us to postulate any particular risk factors with sickle cell disease that might predispose patients to spinal cord infarction. Our patient’s case raises the question as to whether spinal cord infarction is being missed in individuals with sickle cell disease and neurologic symptoms.

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Introduction: Advances in surgical and medical therapies have increased the life expectancy for patients with congenital heart disease. Arrhythmias in these patients are common and multi-factorial, often due to a disruption of the hearts’ intrinsic electrical system and degenerative remodeling, secondary to varying pressure and volume loads. The aim of this study is to better characterize the association between type of structural heart disease (SHD) and clinically significant arrhythmias. Methods: We identified all adults with diagnosed congenital heart disease seen at a large tertiary care referral center between 2000 and 2014. Patients were categorized based on gender, SHD, and type of arrhythmia. We used the American Thoracic Society guidelines to group SHD into discrete categories and grouped arrhythmias as tachyarrhythmias (atrial/ventricular) and bradyarrhythmias. Results: We identified 1355 individuals with at least one congenital structural heart defect and at least one arrhythmia. Of those, the most common structural abnormality was left-sided outflow tract obstruction and the least common was congenitally corrected transposition of the great arteries. The most common category of arrhythmia identified was atrial arrhythmia and the least common was sinoatrial node dysfunction (Figure). The most common association between SHD and arrhythmia was atrial arrhythmia in patients with left-sided outflow tract obstruction. Conclusions: This study is one of the largest single-center reports linking specific structural heart defects to type of rhythm disturbance. These data may help providers better anticipate future arrhythmia development based on a patient’s underlying structural defect and offer prognostic information to patients and families. Future studies to more closely evaluate the relationship between arrhythmia patterns and specific risk factors within each category of SHD are needed.

Introduction: Following pacemaker (PM) implant, both atrial fibrillation (AF) and complete atrioventricular block (cAVB), resulting in high burden right ventricular pacing, are independently associated with incident heart failure (HF); however, the combined effect of AF and cAVB in HF development is not well characterized. Methods: A MarketScan ® (MS) Commercial and Medicare Supplemental claims database was used to identify patients ≥18 years old undergoing de novo dual chamber PM implant between 4/2008-3/2014. Baseline cAVB and AF were determined using inpatient and outpatient billing codes in the year prior to PM implant. Patients with cAVB were identified by a diagnosis of 3 rd degree AVB or an AV node ablation, and were compared to those without any AVB (noAVB). Patients with a diagnosis of HF prior to implant were excluded, as were those without continuous MS enrollment for ≥1 year before and after implant. After stratification based on the presence of baseline cAVB and AF, patients were propensity score matched on age, gender, geographic region, implant year and 19 baseline comorbidities. The primary endpoint was incidence of new HF diagnosis following PM implant. Results: The four matched cohorts included: cAVB/AF (n = 2084); noAVB/AF (n = 2084); cAVB/no AF (n = 2075); and noAVB/no AF (n = 2065). Across all groups, mean age was 76 ± 11 years, 56% were male, and median duration of followup after PM implant was 2.28 [IQR 1.59, 3.27] years, without significant differences between groups. The incidence of new HF diagnosis following PM implant (per 100 pt-yrs of follow-up) was highest in the cAVB/AF group (21.5), followed by the noAVB/ AF group (16.7), cAVB/no AF (13.7) and was lowest in the noAVB/no AF group (11.4) (Figure). In multivariate Cox models, the hazard ratio (HR) for new HF diagnosis among those with cAVB compared to those with noAVB was 1.20 [95% confidence interval (CI) 1.06 -1.35, P = .004] and the HR associated with AF compared to no AF was 1.44 [95% CI 1.28 -1.62, P < .001]. There was no significant interaction between the presence of baseline AF and cAVB (P = .440). Conclusion: Both complete AV block and AF are associated with an increased incidence of HF following PM implant. Among patients with both diagnoses, the effect appears to be additive and the risk of developing HF was approximately 50% at 4 years following PM implant. These findings highlight the need for tools to predict which patients are most likely to develop HF following PM implant and strategies to mitigate that risk.

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