Apical Hypertrophic Cardiomyopathy: A Fatal yet Underappreciated Variant of Hypertrophic Cardiomyopathy.

Hypertrophic cardiomyopathy (HCM) is a group of diseases affecting the left ventricle heart muscle that share a common feature of left ventricular hypertrophy without associated cardiac or systemic disorder. It was found to have a genetic basis with autosomal dominant mutations in the sarcomeric protein genes. Apical HCM is a rare subtype and underappreciated variant of HCM that primarily affects the apex of the heart. Apical HCM is dissimilar to classic HCM, with more challenges in diagnosis and inconsistent clinical course than other types. We report a case of a 91-year-old female who presented with a syncopal episode. Workup revealed atypical nonclassic features. Her transthoracic echocardiogram revealed a "spade-like" configuration of the left ventricular cavity at end-diastole consistent with apical hypertrophic cardiomyopathy. The remaining of her workup was consistent with the apical hypertrophic cardiomyopathy as a reason for the syncopal episode on presentation. Apical HCM is a distinct form of HCM that requires more attention among clinicians. In our case, the patient ended up having an implantable cardioverter defibrillator (ICD) for secondary prevention and a prescription of a beta blocker with a good outcome in her case.

Ventricular Noncompaction With Left Ventricular Thrombus: A Case Report.

Noncompaction cardiomyopathy (NCC) is congenital cardiomyopathy characterized by trabeculations of the left ventricle found on echocardiogram and/or cardiac magnetic resonance imaging (CMRI). This rare disease is associated with thromboembolism and an increased risk of ventricular thrombus formation. We present the case of a 73-year-old female who was admitted for a suspected cerebrovascular accident (CVA), later found on echocardiogram and CMRI to have NCC with left ventricular thrombus. She was started on warfarin indefinitely. We highlight the rarity of this phenomenon as well as the unique questions regarding initiation, length, and choice of therapeutic anticoagulation in the absence of atrial fibrillation in these patients. Consideration of this diagnosis should be made in the absence of other cardioembolic etiologies with prompt management based on available guidelines.

Prevalence of abnormalities in electrocardiogram conduction in dialysis patients: a comparative study / Prevalência de anormalidades de condução no eletrocardiograma de pacientes em diálise: um estudo comparativo

ABSTRACT Background: The electrocardiogram (ECG) can aid in identification of chronic kidney disease (CKD) patients at high risk for cardiovascular diseases. Cohort studies describe ECG abnormalities in patients on hemodialysis (HD), but we did not find data comparing ECG abnormalities among patients with normal kidney function or peritoneal dialysis (PD) to those on hemodialysis. We hypothesized that ECG conduction abnormalities would be more common, and cardiac conduction interval times longer, among patients on hemodialysis vs. those on peritoneal dialysis and CKD 1 or 2. Methods: Retrospective review of adult inpatients' charts, comparing those with billing codes for "Hemodialysis" vs. inpatients without those charges, and an outpatient peritoneal dialysis cohort. Patients with CKD 3 or 4 were excluded. Results: One hundred and sixty-seven charts were reviewed. ECG conduction intervals were consistently and statistically longer among hemodialysis patients (n=88) vs. peritoneal dialysis (n=22) and CKD stage 1 and 2 (n=57): PR (175±35 vs 160±44 vs 157±22 msec) (p=0.009), QRS (115±32 vs. 111±31 vs 91±18 msec) (p=0.001), QT (411±71 vs. 403±46 vs 374±55 msec) (p=0.006), QTc (487±49 vs. 464±38 vs 452±52 msec) (p=0.0001). The only significantly different conduction abnormality was prevalence of left bundle branch block: 13.6% among HD patients, 5% in PD, and 2% in CKD 1 and 2 (p=0.03). Conclusion: To our knowledge, this is the first study to report that ECG conduction intervals are significantly longer as one progresses from CKD Stage 1 and 2, to PD, to HD. These and other data support the need for future research to utilize ECG conduction times to identify dialysis patients who could potentially benefit from proactive cardiac evaluations and risk reduction.

RESUMO Introdução: O eletrocardiograma (ECG) pode auxiliar na identificação de pacientes com doença renal crônica (DRC) e alto risco para doenças cardiovasculares. Estudos de coorte descrevem anormalidades no ECG de pacientes em hemodiálise (HD), mas não encontramos dados comparando anormalidades no ECG entre pacientes com função renal normal ou aqueles em diálise peritoneal (DP), com aqueles em hemodiálise. Nossa hipótese foi de que as anormalidades de condução no ECG seriam mais comuns, e o intervalo de condução cardíaca seria mais longo entre os pacientes em hemodiálise comparados àqueles em diálise peritoneal e DRC 1 ou 2. Métodos: revisão retrospectiva dos prontuários de pacientes adultos internados, comparando aqueles com códigos de cobrança para "Hemodiálise" versus pacientes internados sem esses encargos, e uma coorte de pacientes em diálise peritoneal ambulatorial. Pacientes com DRC 3 ou 4 foram excluídos. Resultados: Cento e sessenta e sete prontuários foram revisados. Os intervalos de condução no ECG foram consistente- e estatisticamente mais longos entre os pacientes em hemodiálise (n = 88) vs. em diálise peritoneal (n = 22) e DRC estágios 1 e 2 (n = 57): PR (175 ± 35 vs 160 ± 44 vs 157 ± 22 msec) (p = 0,009); QRS (115 ± 32 vs. 111 ± 31 vs 91 ± 18 ms) (p = 0,001); QT (411 ± 71 vs. 403 ± 46 vs 374 ± 55 ms) (p = 0,006 ), QTc (487 ± 49 vs. 464 ± 38 vs 452 ± 52 ms) (p = 0,0001). A única anormalidade de condução significativamente diferente foi a prevalência de bloqueio do ramo esquerdo: 13,6% nos pacientes em HD, 5% em DP e 2% na DRC 1 e 2 (p = 0,03). Conclusão: Pelo que sabemos, este é o primeiro estudo a relatar que os intervalos de condução no ECG são significativamente maiores à medida que se progride das DRC Estágios 1 e 2, para DP, e para HD. Esses e outros dados corroboram a necessidade de estudos futuros para utilizar os tempos de condução no ECG para identificar pacientes em diálise que poderiam se beneficiar de avaliações cardíacas proativas e assim redução de risco.

Prevalence of abnormalities in electrocardiogram conduction in dialysis patients: a comparative study.

BACKGROUND: The electrocardiogram (ECG) can aid in identification of chronic kidney disease (CKD) patients at high risk for cardiovascular diseases. Cohort studies describe ECG abnormalities in patients on hemodialysis (HD), but we did not find data comparing ECG abnormalities among patients with normal kidney function or peritoneal dialysis (PD) to those on hemodialysis. We hypothesized that ECG conduction abnormalities would be more common, and cardiac conduction interval times longer, among patients on hemodialysis vs. those on peritoneal dialysis and CKD 1 or 2. METHODS: Retrospective review of adult inpatients' charts, comparing those with billing codes for "Hemodialysis" vs. inpatients without those charges, and an outpatient peritoneal dialysis cohort. Patients with CKD 3 or 4 were excluded. RESULTS: One hundred and sixty-seven charts were reviewed. ECG conduction intervals were consistently and statistically longer among hemodialysis patients (n=88) vs. peritoneal dialysis (n=22) and CKD stage 1 and 2 (n=57): PR (175±35 vs 160±44 vs 157±22 msec) (p=0.009), QRS (115±32 vs. 111±31 vs 91±18 msec) (p=0.001), QT (411±71 vs. 403±46 vs 374±55 msec) (p=0.006), QTc (487±49 vs. 464±38 vs 452±52 msec) (p=0.0001). The only significantly different conduction abnormality was prevalence of left bundle branch block: 13.6% among HD patients, 5% in PD, and 2% in CKD 1 and 2 (p=0.03). CONCLUSION: To our knowledge, this is the first study to report that ECG conduction intervals are significantly longer as one progresses from CKD Stage 1 and 2, to PD, to HD. These and other data support the need for future research to utilize ECG conduction times to identify dialysis patients who could potentially benefit from proactive cardiac evaluations and risk reduction.

Chronic Kidney Disease in Patients Undergoing Cardiac Device Placement: Results of a Retrospective Study.

BACKGROUND: Cardiovascular issues (especially arrhythmia and sudden cardiac death) are one of the most common causes of mortality in patients with chronic kidney disease (CKD). To minimize cardiac mortality, these patients frequently require various cardiac devices, such as pacemakers, loop recorders, and defibrillators which can compromise their vascular access. In this study, we aim to determine the prevalence of CKD in patients undergoing cardiac device placement and their progression of CKD. METHODS: Institutional review board approval was obtained for this study. A total of 688 patients undergoing cardiac device placement were included in this study over a 3-year period at Jersey Shore University Medical Center. Demographic characteristics, comorbidities, base-line renal functions during the procedure, types of cardiac devices, sites of vascular access and follow-up renal function when available were assessed retrospectively. Patients were categorized into CKD stages 1 - 5 based on the National Kidney Foundation-Kidney Disease Outcomes Quality Initiative (NKF-KDOQI) guidelines. The patients who were already on hemodialysis were excluded in this study. RESULTS: The average age of the patient were 73.9 years with male predominance (60%). A total of 227 patients (33%) had estimated glomerular filtration rate (eGFR) < 60 mL/min consistent with the evidence of advanced-stage CKD (stages 3 - 5) at the time of cardiac device placement. The most common types of device placements were new insertion/replacement of atrial and ventricular leads (39.5%), loop recorder implantation (21.1%) and generator changes on an already implanted device (11%). Only 4% (28/688) had a leadless cardiac device placement. The most common access sites were subclavian (47.1%), axillary (32.3%) and femoral (12.2%). CONCLUSIONS: The present study demonstrated that nearly one-third of the patient undergoing cardiac device placement had an advanced degree of renal failure. Because CKD is a progressive disease, many of these patients might require renal replacement therapy in the future. Transvenous devices is not a good choice in this group of patients as they will ultimately require an arteriovenous fistula. Subcutaneous leadless cardiac device insertion might be a better option in patients with advanced CKD.

TpTe and TpTe/QT: novel markers to predict sudden cardiac death in ESRD? / TpTe e TpTe/QT: novos marcadores para prever morte súbita cardíaca na DRT?

ABSTRACT Introduction: Reliable markers to predict sudden cardiac death (SCD) in patients with end stage renal disease (ESRD) remain elusive, but echocardiogram (ECG) parameters may help stratify patients. Given their roles as markers for myocardial dispersion especially in high risk populations such as those with Brugada syndrome, we hypothesized that the Tpeak to Tend (TpTe) interval and TpTe/QT are independent risk factors for SCD in ESRD. Methods: Retrospective chart review was conducted on a cohort of patients with ESRD starting hemodialysis. Patients were US veterans who utilized the Veterans Affairs medical centers for health care. Average age of all participants was 66 years and the majority were males, consistent with a US veteran population. ECGs that were performed within 18 months of dialysis initiation were manually evaluated for TpTe and TpTe/QT. The primary outcomes were SCD and all-cause mortality, and these were assessed up to 5 years following dialysis initiation. Results: After exclusion criteria, 205 patients were identified, of whom 94 had a prolonged TpTe, and 61 had a prolonged TpTe/QT interval (not mutually exclusive). Overall mortality was 70.2% at 5 years and SCD was 15.2%. No significant difference was observed in the primary outcomes when examining TpTe (SCD: prolonged 16.0% vs. normal 14.4%, p=0.73; all-cause mortality: prolonged 55.3% vs. normal 47.7%, p=0.43). Likewise, no significant difference was found for TpTe/QT (SCD: prolonged 15.4% vs. normal 15.0%, p=0.51; all-cause mortality: prolonged 80.7% vs. normal 66.7%, p=0.39). Conclusions: In ESRD patients on hemodialysis, prolonged TpTe or TpTe/QT was not associated with a significant increase in SCD or all-cause mortality.

RESUMO Introdução: Marcadores confiáveis para predizer morte súbita cardíaca (MSC) em pacientes com doença renal terminal (DRT) permanecem elusivos, mas os parâmetros do ecocardiograma (ECG) podem ajudar a estratificar os pacientes. Devido a seus papéis como marcadores para a dispersão miocárdica, especialmente em populações de alto risco, como aquelas com síndrome de Brugada, nós hipotetizamos que o intervalo pico da onda T ao final da onda T (TpTe) e TpTe/QT são fatores de risco independentes para MSC na DRT. Métodos: Revisão retrospectiva do prontuário foi realizada em uma coorte de pacientes com DRT iniciando a hemodiálise. Os pacientes eram veteranos de guerra americanos que utilizavam os centros médicos do Veterans Affairs para atendimento médico. A idade média de todos os participantes foi de 66 anos e a maioria era do sexo masculino, consistente com uma população veterana dos EUA. ECGs que foram realizados dentro de 18 meses após o início da diálise, e foram avaliados manualmente para TpTe e TpTe/QT. Os desfechos primários foram MSC e mortalidade por todas as causas, e estes foram avaliados até 5 anos após o início da diálise. Resultados: Após o critério de exclusão, foram identificados 205 pacientes, dos quais 94 com TpTe prolongado e 61 com intervalo TpTe/QT prolongado (não mutuamente exclusivo). A mortalidade geral foi de 70,2% em 5 anos e a MSC foi de 15,2%. Nenhuma diferença significativa foi observada nos desfechos primários ao se avaliar o TpTe (MSC: prolongado 16,0% versus normal 14,4%, p = 0,73; mortalidade por todas as causas: prolongado 55,3% vs. normal 47,7%, p = 0,43). Da mesma forma, nenhuma diferença significativa foi encontrada para TpTe/QT (MSC: prolongado 15,4% vs. normal 15,0%, p = 0,51; mortalidade por todas as causas: prolongado 80,7% vs. normal 66,7%, p = 0,39). Conclusões: Em pacientes com insuficiência renal terminal em hemodiálise, TpTe ou TpTe/QT prolongados não foram associados a um aumento significativo da morte súbita ou mortalidade por todas as causas.

TpTe and TpTe/QT: novel markers to predict sudden cardiac death in ESRD?

INTRODUCTION: Reliable markers to predict sudden cardiac death (SCD) in patients with end stage renal disease (ESRD) remain elusive, but electrocardiogram (ECG) parameters may help stratify patients. Given their roles as markers for myocardial dispersion especially in high risk populations such as those with Brugada syndrome, we hypothesized that the Tpeak to Tend (TpTe) interval and TpTe/QT are independent risk factors for SCD in ESRD. METHODS: Retrospective chart review was conducted on a cohort of patients with ESRD starting hemodialysis. Patients were US veterans who utilized the Veterans Affairs medical centers for health care. Average age of all participants was 66 years and the majority were males, consistent with a US veteran population. ECGs that were performed within 18 months of dialysis initiation were manually evaluated for TpTe and TpTe/QT. The primary outcomes were SCD and all-cause mortality, and these were assessed up to 5 years following dialysis initiation. RESULTS: After exclusion criteria, 205 patients were identified, of whom 94 had a prolonged TpTe, and 61 had a prolonged TpTe/QT interval (not mutually exclusive). Overall mortality was 70.2% at 5 years and SCD was 15.2%. No significant difference was observed in the primary outcomes when examining TpTe (SCD: prolonged 16.0% vs. normal 14.4%, p=0.73; all-cause mortality: prolonged 55.3% vs. normal 47.7%, p=0.43). Likewise, no significant difference was found for TpTe/QT (SCD: prolonged 15.4% vs. normal 15.0%, p=0.51; all-cause mortality: prolonged 80.7% vs. normal 66.7%, p=0.39). CONCLUSIONS: In ESRD patients on hemodialysis, prolonged TpTe or TpTe/QT was not associated with a significant increase in SCD or all-cause mortality.

Dabigatran-Induced Acute Interstitial Nephritis: An Important Complication of Newer Oral Anticoagulation Agents.

Acute kidney injury (AKI) due to an acute interstitial nephritis (AIN) is common and can lead to increased morbidity and mortality. Medications such as antibiotics, nonsteroidal anti-inflammatory drugs (NSAIDs), proton pump inhibitors (PPI) and rifampin are common offending agents. Anticoagulant-associated AIN is more frequently reported with the use of warfarin; however, only few case reports have reported an association with the use of novel oral anticoagulants (NOACs). Herein, we report the case of a 59-year-old male who developed AKI after initiating dabigatran for the treatment of atrial fibrillation. Laboratory data demonstrated elevated blood urea nitrogen (BUN) of 115 mg/dL (baseline = 35 mg/dL) and serum creatinine (Cr) of 5.06 mg/dL (baseline = 1.3 mg/dL). Urinalysis revealed eosinophiluria. Renal biopsy disclosed diffuse tubulointerstitial nephritis and eosinophils and confirmed the diagnosis of AIN. At 1 week, renal function improved (BUN/Cr = 53/2.73 mg/dL) with steroid therapy and discontinuation of dabigatran. With an increasing use of NOACs, it is important to monitor renal function to diagnose AIN in a timely fashion. Early diagnosis and prompt treatment can mitigate serious renal damage induced by dabigatran.